STATE-OF-THE-ART PAPER
Clinical and Mechanistic Issues in Early Repolarization
Of Normal Variants and Lethal Arrhythmia Syndromes
Begoña Benito, MD, Eduard Guasch, MD, Lena Rivard, MD and Stanley Nattel, MD*
Research Center and Department of Medicine, Montreal Heart Institute and Université de Montréal, Montreal, Quebec, Canada
J Am Coll Cardiol, 2010; 56:1177-1186
Early repolarization, involving ST-segment elevation and, sometimes, prominent J waves at the QRS-ST junction, has been considered a normal electrocardiographic variant for over 60 years. A growing number of case reports and case-control studies indicate that in some instances, early repolarization patterns are associated with increased risk of idiopathic ventricular fibrillation. Epidemiological evidence indicates a dose effect for the risk of cardiac and sudden death with the extent of J-point elevation. This paper reviews present knowledge regarding the epidemiology, presentation, therapeutic response, and mechanisms characteristic of early repolarization. We highlight major unanswered questions relating to our limited ability to determine which individuals with this common electrocardiographic variant are at risk for sudden death, our incomplete understanding of underlying mechanisms, the inadequate information regarding genetic determinants and therapeutic responses, and the unclear relationship between early repolarization and other conditions involving accelerated repolarization and sudden arrhythmic death such as Brugada and short-QT syndromes. This review paper intends to inform the practicing physician about important clinical issues and to stimulate investigators to address the many unresolved questions in this rapidly evolving field.
Thursday, September 30, 2010
History: Norwood Procedure
JTCVS 1981 Oct;82(4):511-9
&
N Engl J Med 1983; 308:23-26January 6, 1983
Physiologic Repair of Aortic Atresia–Hypoplastic Left Heart Syndrome
William I. Norwood, M.D., Peter Lang, M.D., and Dolly D. Hansen, M.D.
&
N Engl J Med 1983; 308:23-26January 6, 1983
Physiologic Repair of Aortic Atresia–Hypoplastic Left Heart Syndrome
William I. Norwood, M.D., Peter Lang, M.D., and Dolly D. Hansen, M.D.
Monday, September 27, 2010
Trisomy-13 and 18 - Cardiac Surgery
Effectiveness of Cardiac Surgery in Trisomies 13 and 18 (From the Pediatric Cardiac Care Consortium).
Eric Graham, Scott Bradley, Girish Shirali, Christine Hills and Andrew Atz.
Am J Cardiol 2004;93:801-3
1982-2000.
Multicenter Pediatric Cardiac Care Consortium - Cardiac Registry
70 were identified, only 35 had genetic confirmation.
Study group: n=35
5 cardiac diagnostic categories (based on the most-significant lesion):
VSD - 20
TOF - 6
Coarctation - 4
PDA - 3
AVSD - 2.
Weight range: 2.1 - 16 kg (Median 3.7 kg).
Gender: 14/21
Age at surgery: 4 - 2479 days (Median 128 days)
Hospital stay: 3 - 48 days (Median 10 days)
Hospital survival: 32 (91%)
Morbidity: Among 26 patients who were either intubated for surgery or intubated for less than 2 days prior to surgery: Among 9 patients who were intubated for > 2 days prior to surgery, 3 were extubated prior to discharge. One died. 5 were still intubated at discharge.
Follow-up: None, after hospital discharge.
Justification for surgery: To improve quality of life...was not addressed in this study.
Other references:
Japan: Am J Med Genet 2008;146A(11):1372-80 (& Editorial on p.1369-71)
Gp A - 2000-2: Offered no treatment (n=13)
Gp B - 2002-3: Offered pharmacologic treatment for PDA only (n-9)
Gp C - After Nov 2003 - 2005: Pharmacologic and Surgical treatment offered (n=9)
Median length of survival:
Gp A - 7 days
Eric Graham, Scott Bradley, Girish Shirali, Christine Hills and Andrew Atz.
Am J Cardiol 2004;93:801-3
1982-2000.
Multicenter Pediatric Cardiac Care Consortium - Cardiac Registry
70 were identified, only 35 had genetic confirmation.
Study group: n=35
5 cardiac diagnostic categories (based on the most-significant lesion):
VSD - 20
TOF - 6
Coarctation - 4
PDA - 3
AVSD - 2.
Weight range: 2.1 - 16 kg (Median 3.7 kg).
Gender: 14/21
Age at surgery: 4 - 2479 days (Median 128 days)
Hospital stay: 3 - 48 days (Median 10 days)
Hospital survival: 32 (91%)
Morbidity: Among 26 patients who were either intubated for surgery or intubated for less than 2 days prior to surgery: Among 9 patients who were intubated for > 2 days prior to surgery, 3 were extubated prior to discharge. One died. 5 were still intubated at discharge.
Follow-up: None, after hospital discharge.
Justification for surgery: To improve quality of life...was not addressed in this study.
Other references:
Japan: Am J Med Genet 2008;146A(11):1372-80 (& Editorial on p.1369-71)
Gp A - 2000-2: Offered no treatment (n=13)
Gp B - 2002-3: Offered pharmacologic treatment for PDA only (n-9)
Gp C - After Nov 2003 - 2005: Pharmacologic and Surgical treatment offered (n=9)
Median length of survival:
Gp A - 7 days
Gp B - 24 days
Gp C - 243 days
Utah: Am J Med Genet 2006;140:937-44 & 1994;49:175-88Training: Spectrum of Consult Requests for Pediatric Cardiology
Conditions Leading to Pediatric Cardiology Consultation in a Tertiary Academic Hospital
Robert L. Geggel, MD
PEDIATRICS Vol. 114 No. 4 October 2004, pp. e409-e417
Objective. To determine the basis for cardiac consultations for pediatric patients in an academic hospital setting.
Methods. The activities of the cardiology consultation service were tabulated for 12 months, from July 2001 to June 2002. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained by the consultation service, encounter forms submitted to the billing office, consultation notes maintained in a central file, and a departmental list of echocardiography studies. Patients who required clearance for noncardiac surgical procedures were generally evaluated in the cardiology clinic and not by the consultation service. Patient data were obtained from consultation and echocardiography reports and from hospital computer-based records for discharge summaries for inpatient admissions, emergency department encounter summaries, and laboratory reports. For each patient, consultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate admissions, or for different clinical concerns during a single admission.
Results.
A total of 2071 consultations were performed for 1724 patients. The age at the time of consultation was 6.6 ± 9.3 years (median: 1.2 years; range: 1 day to 60.6 years).
A total of 1507 patients (87.4%) had a single consultation; 217 patients (12.6%) had multiple encounters, ranging from 2 to 9, accounting for 564 consultations (27.2%).
Clinical concerns included murmurs (18.5%), cardiac function (12.7%), arrhythmias (12.7%), intercurrent illnesses among cardiac patients (11.3%), cyanosis (6.3%), syndromes (5.7%), chest pain (5.2%), syncope/dizziness (4.5%), subacute endocarditis (4.4%), follow-up evaluations of fetal diagnoses (4.3%), Kawasaki disease (3.4%), cor pulmonale (3%), recent cardiac surgery or catheterization (1.6%), cerebrovascular accidents (1.2%), and miscellaneous conditions.
Four diagnoses accounted for 91% of murmur evaluations, ie, patent ductus arteriosus, ventricular septal defects, innocent murmurs, and pulmonary branch murmur of infancy.
The most common murmur diagnosis in the neonatal intensive care unit was patent ductus arteriosus (68%), in the well-child nursery was ventricular septal defect (64%), and on the medical ward was innocent murmur (62%).
The most common basis for evaluation of function was oncologic disease. Among patients evaluated for function, there were 3 new diagnoses of structural congenital heart disease, all involving neonates with aortic arch obstruction.
Approximately two-thirds of arrhythmias were supraventricular in origin. The most common arrhythmias requiring treatment were supraventricular tachycardia and atrial flutter/fibrillation, the latter occurring mainly among older patients with structural heart disease.
Diagnoses made with fetal echocardiography accounted for 14.3% of newborn consultations and included 83% of patients with cyanotic cardiac disease.
Three syndromes accounted for 57% of consultations for this indication, ie, VACTERL association (vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal abnormality, and limb anomalies), trisomy 21, and infant of diabetic mother.
Chest pain and syncope/dizziness were frequently evaluated in the emergency department and, in this setting, accounted for 13 and 10% of all evaluations and 19 and 25% of evaluations for new patients, respectively. For patients evaluated for chest pain, the most common basis was musculoskeletal/costochondritic (42%) or idiopathic (22%). There was a cardiac or pericardial basis in 11% of cases; these patients either had known heart disease associated with this complication or systemic symptoms, abnormal cardiac auscultatory findings, and electrocardiographic features of pericarditis.
Syncope/dizziness most commonly had a vasovagal (50.5%) or orthostatic (24.7%) basis. There was a cardiac basis in 5.4% of cases; these patients were more likely to have symptoms associated with exercise.
Although endocarditis was a frequent clinical concern (91 patients), only 3 cases were identified, involving 2 patients with structural heart disease and 1 neonate with an indwelling intracardiac catheter. Two other patients had central venous lines, intravascular thrombus, and fungemia.
Kawasaki disease was the most common acquired condition leading to consultation.
Cor pulmonale was most commonly screened among patients with congenital diaphragmatic hernia, chronic lung disease of prematurity, pneumonitis, reactive airway disease, or cystic fibrosis.
Patients with recent cardiac surgery or cardiac catheterization typically had postpericardiotomy syndrome or complications associated with vascular access.
Approximately 20% of cases of cerebrovascular accidents had a cardiac basis.
Conclusions. Although a variety of conditions were assessed, some were encountered more frequently. Future educational curricula developed for cardiac training of pediatric residents should appropriately emphasize conditions necessitating consultation.
Robert L. Geggel, MD
PEDIATRICS Vol. 114 No. 4 October 2004, pp. e409-e417
Objective. To determine the basis for cardiac consultations for pediatric patients in an academic hospital setting.
Methods. The activities of the cardiology consultation service were tabulated for 12 months, from July 2001 to June 2002. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained by the consultation service, encounter forms submitted to the billing office, consultation notes maintained in a central file, and a departmental list of echocardiography studies. Patients who required clearance for noncardiac surgical procedures were generally evaluated in the cardiology clinic and not by the consultation service. Patient data were obtained from consultation and echocardiography reports and from hospital computer-based records for discharge summaries for inpatient admissions, emergency department encounter summaries, and laboratory reports. For each patient, consultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate admissions, or for different clinical concerns during a single admission.
Results.
A total of 2071 consultations were performed for 1724 patients. The age at the time of consultation was 6.6 ± 9.3 years (median: 1.2 years; range: 1 day to 60.6 years).
A total of 1507 patients (87.4%) had a single consultation; 217 patients (12.6%) had multiple encounters, ranging from 2 to 9, accounting for 564 consultations (27.2%).
Clinical concerns included murmurs (18.5%), cardiac function (12.7%), arrhythmias (12.7%), intercurrent illnesses among cardiac patients (11.3%), cyanosis (6.3%), syndromes (5.7%), chest pain (5.2%), syncope/dizziness (4.5%), subacute endocarditis (4.4%), follow-up evaluations of fetal diagnoses (4.3%), Kawasaki disease (3.4%), cor pulmonale (3%), recent cardiac surgery or catheterization (1.6%), cerebrovascular accidents (1.2%), and miscellaneous conditions.
Four diagnoses accounted for 91% of murmur evaluations, ie, patent ductus arteriosus, ventricular septal defects, innocent murmurs, and pulmonary branch murmur of infancy.
The most common murmur diagnosis in the neonatal intensive care unit was patent ductus arteriosus (68%), in the well-child nursery was ventricular septal defect (64%), and on the medical ward was innocent murmur (62%).
The most common basis for evaluation of function was oncologic disease. Among patients evaluated for function, there were 3 new diagnoses of structural congenital heart disease, all involving neonates with aortic arch obstruction.
Approximately two-thirds of arrhythmias were supraventricular in origin. The most common arrhythmias requiring treatment were supraventricular tachycardia and atrial flutter/fibrillation, the latter occurring mainly among older patients with structural heart disease.
Diagnoses made with fetal echocardiography accounted for 14.3% of newborn consultations and included 83% of patients with cyanotic cardiac disease.
Three syndromes accounted for 57% of consultations for this indication, ie, VACTERL association (vertebral anomalies, anal atresia, congenital heart disease, tracheoesophageal fistula, renal abnormality, and limb anomalies), trisomy 21, and infant of diabetic mother.
Chest pain and syncope/dizziness were frequently evaluated in the emergency department and, in this setting, accounted for 13 and 10% of all evaluations and 19 and 25% of evaluations for new patients, respectively. For patients evaluated for chest pain, the most common basis was musculoskeletal/costochondritic (42%) or idiopathic (22%). There was a cardiac or pericardial basis in 11% of cases; these patients either had known heart disease associated with this complication or systemic symptoms, abnormal cardiac auscultatory findings, and electrocardiographic features of pericarditis.
Syncope/dizziness most commonly had a vasovagal (50.5%) or orthostatic (24.7%) basis. There was a cardiac basis in 5.4% of cases; these patients were more likely to have symptoms associated with exercise.
Although endocarditis was a frequent clinical concern (91 patients), only 3 cases were identified, involving 2 patients with structural heart disease and 1 neonate with an indwelling intracardiac catheter. Two other patients had central venous lines, intravascular thrombus, and fungemia.
Kawasaki disease was the most common acquired condition leading to consultation.
Cor pulmonale was most commonly screened among patients with congenital diaphragmatic hernia, chronic lung disease of prematurity, pneumonitis, reactive airway disease, or cystic fibrosis.
Patients with recent cardiac surgery or cardiac catheterization typically had postpericardiotomy syndrome or complications associated with vascular access.
Approximately 20% of cases of cerebrovascular accidents had a cardiac basis.
Conclusions. Although a variety of conditions were assessed, some were encountered more frequently. Future educational curricula developed for cardiac training of pediatric residents should appropriately emphasize conditions necessitating consultation.
Thursday, September 23, 2010
Ethics & Training: Time to stop.
PCCM Perspective
In think it is time to stop
Annie Janvier MD, PhD.
Pediatr Crit Care Med 2010;11:624-5.
An article about including "stopping" CPR in the training for CPR.
In think it is time to stop
Annie Janvier MD, PhD.
Pediatr Crit Care Med 2010;11:624-5.
An article about including "stopping" CPR in the training for CPR.
Wednesday, September 22, 2010
PFO morphology & Stroke (Infarct) size on MRI
Circulation: Cardiovascular Interventions. 2010
The Association of Patent Foramen Ovale Morphology and Stroke Size in Patients With Paradoxical Embolism
Andre Akhondi, MD; Rubine Gevorgyan, MD; Chi-Hong Tseng, PhD; Leo Slavin, MD; Catherine Dao, MD; David S. Liebeskind, MD and Jonathan M. Tobis, MD
Background Patent foramen ovale (PFO) has been implicated in the pathogenesis of cryptogenic stroke through paradoxical embolization to the cerebral circulation. This study evaluated the relationship between the morphological and functional size of the PFO by echocardiography compared with cerebral infarct volume identified on MRI.
Methods and Results Patients who were referred to interventional cardiology with the diagnosis of cryptogenic stroke were included and had either a transesophageal echocardiogram or an intracardiac echo and a brain MRI at the time of stroke. Transesophageal echocardiogram or intracardiac echo was used to obtain PFO measurements. MRI of the brain with 3 sequences (T2, diffusion-weighted imaging, and fluid-attenuated inversion recovery) was used to diagnose acute stroke and measure the infarct volume. In the 72 patients studied, the median measured stroke volume was 4.3 cm3 on diffusion-weighted imaging, 4.1 cm3 on T2, and 3.5 cm3 on fluid-attenuated inversion recovery. There was no significant correlation between the PFO height, length, septum secundum thickness, or echo bubble grade and the infarct volume measured from the 3 MRI sequences. There was a significant correlation between septal excursion distance and infarct volume (r=0.35; P=0.005), but the 12 patients with atrial septal aneurysm did not have the largest strokes.
Conclusions This analysis revealed that septal excursion distance correlates with stroke size by MRI. However, smaller PFO size without the presence of atrial septal aneurysm may still be associated with significant strokes. There was no significant association between PFO height, length by echo, or shunt grade by transcranial Doppler study and brain infarct volume. Therefore, PFO size or morphology should not be the only criteria to decide whether a PFO should be closed.
The Association of Patent Foramen Ovale Morphology and Stroke Size in Patients With Paradoxical Embolism
Andre Akhondi, MD; Rubine Gevorgyan, MD; Chi-Hong Tseng, PhD; Leo Slavin, MD; Catherine Dao, MD; David S. Liebeskind, MD and Jonathan M. Tobis, MD
Background Patent foramen ovale (PFO) has been implicated in the pathogenesis of cryptogenic stroke through paradoxical embolization to the cerebral circulation. This study evaluated the relationship between the morphological and functional size of the PFO by echocardiography compared with cerebral infarct volume identified on MRI.
Methods and Results Patients who were referred to interventional cardiology with the diagnosis of cryptogenic stroke were included and had either a transesophageal echocardiogram or an intracardiac echo and a brain MRI at the time of stroke. Transesophageal echocardiogram or intracardiac echo was used to obtain PFO measurements. MRI of the brain with 3 sequences (T2, diffusion-weighted imaging, and fluid-attenuated inversion recovery) was used to diagnose acute stroke and measure the infarct volume. In the 72 patients studied, the median measured stroke volume was 4.3 cm3 on diffusion-weighted imaging, 4.1 cm3 on T2, and 3.5 cm3 on fluid-attenuated inversion recovery. There was no significant correlation between the PFO height, length, septum secundum thickness, or echo bubble grade and the infarct volume measured from the 3 MRI sequences. There was a significant correlation between septal excursion distance and infarct volume (r=0.35; P=0.005), but the 12 patients with atrial septal aneurysm did not have the largest strokes.
Conclusions This analysis revealed that septal excursion distance correlates with stroke size by MRI. However, smaller PFO size without the presence of atrial septal aneurysm may still be associated with significant strokes. There was no significant association between PFO height, length by echo, or shunt grade by transcranial Doppler study and brain infarct volume. Therefore, PFO size or morphology should not be the only criteria to decide whether a PFO should be closed.
Tuesday, September 21, 2010
Troponin in Heart Failure - Review article (Adult)
J Am Coll Cardiol, 2010; 56:1071-1078
STATE-OF-THE-ART PAPER
Troponin Elevation in Heart Failure
Prevalence, Mechanisms, and Clinical Implications
Robb D. Kociol, MD*, Peter S. Pang, MD, Mihai Gheorghiade, MD, Gregg C. Fonarow, MD, Christopher M. O'Connor, MD* and G. Michael Felker, MD, MHS*,*
Circulating biomarkers have become increasingly important in diagnosing and risk stratifying patients with heart failure (HF). While the natriuretic peptides have received much focus, there is increasing interest in the role of circulating cardiac troponin (cTn) in detecting myocardial injury (often subclinical) in those with HF. Accumulating evidence suggests that patients with chronic and acute HF may have measurable levels of circulating cTn, whose detection and magnitude may have prognostic implications. Furthermore, as new, more sensitive cTn assays are being developed, larger numbers of HF patients are found to have detectable cTn with a persistent relationship between magnitude and outcome. This knowledge improves our ability to understand the mechanism of worsening HF, improve risk stratification, and detect potential injury related to new therapeutics in HF. As investigators begin to understand the relationship of detectable cTn to HF outcomes, as well as temporal changes in its magnitude, and its relationship to other circulating biomarkers, more insight may be gained into the progressive nature of cardiac dysfunction and the transition from chronic compensated to acute decompensated HF. Ultimately, this information might allow physicians to guide therapy, choose appropriate therapeutics, and improve HF outcomes.
STATE-OF-THE-ART PAPER
Troponin Elevation in Heart Failure
Prevalence, Mechanisms, and Clinical Implications
Robb D. Kociol, MD*, Peter S. Pang, MD, Mihai Gheorghiade, MD, Gregg C. Fonarow, MD, Christopher M. O'Connor, MD* and G. Michael Felker, MD, MHS*,*
Circulating biomarkers have become increasingly important in diagnosing and risk stratifying patients with heart failure (HF). While the natriuretic peptides have received much focus, there is increasing interest in the role of circulating cardiac troponin (cTn) in detecting myocardial injury (often subclinical) in those with HF. Accumulating evidence suggests that patients with chronic and acute HF may have measurable levels of circulating cTn, whose detection and magnitude may have prognostic implications. Furthermore, as new, more sensitive cTn assays are being developed, larger numbers of HF patients are found to have detectable cTn with a persistent relationship between magnitude and outcome. This knowledge improves our ability to understand the mechanism of worsening HF, improve risk stratification, and detect potential injury related to new therapeutics in HF. As investigators begin to understand the relationship of detectable cTn to HF outcomes, as well as temporal changes in its magnitude, and its relationship to other circulating biomarkers, more insight may be gained into the progressive nature of cardiac dysfunction and the transition from chronic compensated to acute decompensated HF. Ultimately, this information might allow physicians to guide therapy, choose appropriate therapeutics, and improve HF outcomes.
Changing Mortality in Congenital Heart Disease
J Am Coll Cardiol, 2010; 56:1149-1157
Changing Mortality in Congenital Heart DiseasePaul Khairy, MD, PhD*, Raluca Ionescu-Ittu, MSc,, Andrew S. Mackie, MD, SM, Michal Abrahamowicz, PhD, Louise Pilote, MD, MPH, PhD, and Ariane J. Marelli, MD,*
Objectives: This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD).
Background: Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends.
Methods: We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years.
Results: The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval [CI]: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes.
Conclusions: Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.
Changing Mortality in Congenital Heart DiseasePaul Khairy, MD, PhD*, Raluca Ionescu-Ittu, MSc,, Andrew S. Mackie, MD, SM, Michal Abrahamowicz, PhD, Louise Pilote, MD, MPH, PhD, and Ariane J. Marelli, MD,*
Objectives: This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD).
Background: Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends.
Methods: We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years.
Results: The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval [CI]: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes.
Conclusions: Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.
Figure 1 Distribution of Age at Death in Patients With Congenital Heart Disease in 1987 to 1988 and 2004 to 2005
Histogram bars depict the proportion of all deaths (x-axis) according to age at death (y-axis) in our cohort of patients with congenital heart disease in the first (1987 to 1988; left) and final (2004 to 2005; right) years of observation. Bold black curves with diamonds represent the corresponding age at death distribution in the general Quebec population during the same periods of observation.
Also read this related article:
European Heart Journal 2010;31:1220-29
Mortality in adult congenital heart disease. Verheugt CL, et al.
Article from Netherlands. The Dutch CONCOR Regional Registry.
6933 pts, 197 (2.8%) died. F-up of 24,865 patient-years were analyzed for cause of death. Higher mortality compared to general population. Median age of death was 48.8 yrs. 77% of deaths were cardiovascular in origin (CHF or Sudden death - 45%). Predictors of mortality were age, gender, severity of defect, number of interventions and number of complications (i.e. endocarditis, SVT, VT, conduction disturbances, MI and PAH).
Overall, nothing unexpected. But, they have data to confirm this common sense thought.
Ross Procedure: Outcome
Circulation. 2010;122:1153-1158
Spectrum and Outcome of Reoperations After the Ross Procedure
John M. Stulak, MD; Harold M. Burkhart, MD; Thoralf M. Sundt, III, MD; Heidi M. Connolly, MD; Rakesh M. Suri, MD,, DPhil; Hartzell V. Schaff, MD; Joseph A. Dearani, MD
Background— Proposed advantages to the Ross procedure included presumed increased freedom from reoperation and simpler reoperation for pulmonary conduit replacement if needed. It is increasingly apparent, however, that reoperations are frequent after the Ross procedure and that when required, they may be more complex than previously thought.
Methods and Results— Between September 1991 and August 2008, 56 patients underwent reoperation at our institution after a Ross procedure performed by ourselves (n=13) or elsewhere (n=43). Median age at first reoperation at our institution was 26 years (range 1 to 69 years). The 4 most common indications for reoperation were isolated autograft (neoaortic) regurgitation in 11 cases (20), isolated pulmonary conduit regurgitation/stenosis in 9 (16), combined autograft regurgitation/dilatation in 8 (14), and combined autograft regurgitation and pulmonary conduit regurgitation/stenosis in 6 (11). A total of 144 procedures were performed in these 56 patients during first reoperation at our institution. The autograft valve required replacement in 21 cases (38) and aortic root replacement in 21 (38), with ascending aortic/arch reconstruction in 13 (23) and mitral valve surgery in 5 (9). The pulmonary valve was replaced in 33 cases (59) and the tricuspid valve was repaired/replaced in 10 (18). Early mortality was 1.8 (1 of 56 patients), and morbidity included 6 patients with respiratory failure and 3 who required postcardiotomy extracorporeal membrane oxygenation. There were 4 late deaths during the median follow-up of 8 months (range 1 to 179 months).
Conclusions— A broad spectrum of complex reoperations may be required after the Ross procedure. Patients and family members considering the procedure should be informed of the potential for associated morbidity should reoperation be necessary.
Editorial
Circulation. 2010;122:1139-1140.
Reoperations After the Ross ProcedureTirone E. David, MD
Spectrum and Outcome of Reoperations After the Ross Procedure
John M. Stulak, MD; Harold M. Burkhart, MD; Thoralf M. Sundt, III, MD; Heidi M. Connolly, MD; Rakesh M. Suri, MD,, DPhil; Hartzell V. Schaff, MD; Joseph A. Dearani, MD
Background— Proposed advantages to the Ross procedure included presumed increased freedom from reoperation and simpler reoperation for pulmonary conduit replacement if needed. It is increasingly apparent, however, that reoperations are frequent after the Ross procedure and that when required, they may be more complex than previously thought.
Methods and Results— Between September 1991 and August 2008, 56 patients underwent reoperation at our institution after a Ross procedure performed by ourselves (n=13) or elsewhere (n=43). Median age at first reoperation at our institution was 26 years (range 1 to 69 years). The 4 most common indications for reoperation were isolated autograft (neoaortic) regurgitation in 11 cases (20), isolated pulmonary conduit regurgitation/stenosis in 9 (16), combined autograft regurgitation/dilatation in 8 (14), and combined autograft regurgitation and pulmonary conduit regurgitation/stenosis in 6 (11). A total of 144 procedures were performed in these 56 patients during first reoperation at our institution. The autograft valve required replacement in 21 cases (38) and aortic root replacement in 21 (38), with ascending aortic/arch reconstruction in 13 (23) and mitral valve surgery in 5 (9). The pulmonary valve was replaced in 33 cases (59) and the tricuspid valve was repaired/replaced in 10 (18). Early mortality was 1.8 (1 of 56 patients), and morbidity included 6 patients with respiratory failure and 3 who required postcardiotomy extracorporeal membrane oxygenation. There were 4 late deaths during the median follow-up of 8 months (range 1 to 179 months).
Conclusions— A broad spectrum of complex reoperations may be required after the Ross procedure. Patients and family members considering the procedure should be informed of the potential for associated morbidity should reoperation be necessary.
Editorial
Circulation. 2010;122:1139-1140.
Reoperations After the Ross ProcedureTirone E. David, MD
Scimitar Syndrome: European Study
Circulation. 2010;122:1159-
Scimitar Syndrome. A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study
Vladimiro L. Vida, MD, PhD; Massimo A. Padalino, MD, PhD; Giovanna Boccuzzo, MPH; Erjon Tarja, MD; Hakan Berggren, MD; Thierry Carrel, MD; Sertaç Çiçek, MD; Giancarlo Crupi, MD; Duccio Di Carlo, MD; Roberto Di Donato, MD; José Fragata, MD; Mark Hazekamp, MD; Viktor Hraska, MD; Bohdan Maruszewski, MD; Dominique Metras, MD; Marco Pozzi, MD; Rene Pretre, MD; Jean Rubay, MD; Heikki Sairanen, MD; George Sarris, MD, PhD; Christian Schreiber, MD; Bart Meyns, MD; Tomas Tlaskal, MD; Andreas Urban, MD; Gaetano Thiene, MD; Giovanni Stellin, MD
Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study.
Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis.
Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.
Scimitar Syndrome. A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study
Vladimiro L. Vida, MD, PhD; Massimo A. Padalino, MD, PhD; Giovanna Boccuzzo, MPH; Erjon Tarja, MD; Hakan Berggren, MD; Thierry Carrel, MD; Sertaç Çiçek, MD; Giancarlo Crupi, MD; Duccio Di Carlo, MD; Roberto Di Donato, MD; José Fragata, MD; Mark Hazekamp, MD; Viktor Hraska, MD; Bohdan Maruszewski, MD; Dominique Metras, MD; Marco Pozzi, MD; Rene Pretre, MD; Jean Rubay, MD; Heikki Sairanen, MD; George Sarris, MD, PhD; Christian Schreiber, MD; Bart Meyns, MD; Tomas Tlaskal, MD; Andreas Urban, MD; Gaetano Thiene, MD; Giovanni Stellin, MD
Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study.
Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis.
Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.
Stroke & PFO - New devices
J Am Coll Cardiol Intv, 2010; 3:963-967
Patent Foramen Ovale Closure With the SeptRx Device. Initial Experience With the First "In-Tunnel" Device.
Wibke J. Zimmermann, MD, Corinna Heinisch, MD, Nicolas Majunke, MD, Stephan Staubach, MD, Scott Russell, Nina Wunderlich, MD, Horst Sievert, MD*
CardioVascular Center, Sankt Katharinen, Frankfurt, Germany
Objectives: The purpose of this study was to evaluate the safety and effectiveness of the SeptRx patent foramen ovale (PFO) closure device (SeptRx, Inc., Fremont, California).
Background: A PFO is a relatively common remnant of the fetal circulation that can be associated with cryptogenic stroke, transient ischemic attack, migraine, or decompression sickness. Percutaneous PFO closure with different devices has been performed for many years. However, most of the common devices leave a relatively large amount of material in the left and right atria. The SeptRx PFO device (SeptRx, Inc.) is the first PFO closure device designed to fit directly into the pocket of the PFO.
Methods: From July 2006 to May 2007, 13 patients between 18 and 65 years of age with a history of cryptogenic stroke or transient ischemic attack were included into this first-in-man trial. All patients received 100 mg aspirin and 75 mg clopidogrel for 6 months. Follow-up was done at 1 and 6 months after procedure with transesophageal echocardiography and transcranial Doppler.
Results: In 11 of 13 patients, PFO closure with the SeptRx device was successfully performed. In 2 patients, PFO closure with this device was not possible due to the anatomy of the PFO; 1 device was retrieved before release, and the other was recaptured with a snare. After 30 days, 6 of the 11 PFOs were closed; after 6 months, all were closed. No adverse events occurred.
Conclusions: The SeptRx PFO closure device appears to be safe and effective. The advantage of this occluder is that there is only minimal foreign material on the left and right sides of the interatrial septum. This is the first such "in-tunnel" PFO closure device.
Patent Foramen Ovale Closure With the SeptRx Device. Initial Experience With the First "In-Tunnel" Device.
Wibke J. Zimmermann, MD, Corinna Heinisch, MD, Nicolas Majunke, MD, Stephan Staubach, MD, Scott Russell, Nina Wunderlich, MD, Horst Sievert, MD*
CardioVascular Center, Sankt Katharinen, Frankfurt, Germany
Objectives: The purpose of this study was to evaluate the safety and effectiveness of the SeptRx patent foramen ovale (PFO) closure device (SeptRx, Inc., Fremont, California).
Background: A PFO is a relatively common remnant of the fetal circulation that can be associated with cryptogenic stroke, transient ischemic attack, migraine, or decompression sickness. Percutaneous PFO closure with different devices has been performed for many years. However, most of the common devices leave a relatively large amount of material in the left and right atria. The SeptRx PFO device (SeptRx, Inc.) is the first PFO closure device designed to fit directly into the pocket of the PFO.
Methods: From July 2006 to May 2007, 13 patients between 18 and 65 years of age with a history of cryptogenic stroke or transient ischemic attack were included into this first-in-man trial. All patients received 100 mg aspirin and 75 mg clopidogrel for 6 months. Follow-up was done at 1 and 6 months after procedure with transesophageal echocardiography and transcranial Doppler.
Results: In 11 of 13 patients, PFO closure with the SeptRx device was successfully performed. In 2 patients, PFO closure with this device was not possible due to the anatomy of the PFO; 1 device was retrieved before release, and the other was recaptured with a snare. After 30 days, 6 of the 11 PFOs were closed; after 6 months, all were closed. No adverse events occurred.
Conclusions: The SeptRx PFO closure device appears to be safe and effective. The advantage of this occluder is that there is only minimal foreign material on the left and right sides of the interatrial septum. This is the first such "in-tunnel" PFO closure device.
The longer anchors are on the left atrial side and are designed to uncoil to accommodate a range of patent foramen ovale (PFO) tunnel lengths. The interior of the device contains a fine-wire braided Nitinol mesh, which provides an immediate barrier to the conduction of emboli and is intended to stimulate the body‘s natural adhesion response for permanent closure of the PFO.
J Am Coll Cardiol Intv, 2010; 3:968-973
Patent Foramen Ovale Closure Using a Bioabsorbable Closure Device
Safety and Efficacy at 6-Month Follow-Up
Ben J. Van den Branden, MD*, Martijn C. Post, MD, PhD, Herbert W. Plokker, MD, PhD, Jurriën M. ten Berg, MD, PhD, Maarten J. Suttorp, MD, PhD
Department of Interventional Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands
Objectives: The aim of this study was to assess the mid-term safety and efficacy of percutaneous patent foramen ovale (PFO) closure using a bioabsorbable device (BioSTAR, NMT Medical, Boston, Massachusetts).
Background: Closure of PFO in patients with cryptogenic stroke has proven to be safe and effective using different types of permanent devices.
Methods: All consecutive patients who underwent percutaneous PFO closure with the bioabsorbable closure device between November 2007 and January 2009 were included. Residual shunt was assessed using contrast transthoracic echocardiography.
Results: Sixty-two patients (55% women, mean age 47.7 ± 11.8 years) underwent PFO closure. The in-hospital complications were a surgical device retrieval in 2 patients (3.2%), device reposition in 1 (1.6%), and a minimal groin hematoma in 6 patients (9.7%). The short-term complications at 1-month follow-up (n = 60) were a transient ischemic attack in the presence of a residual shunt in 1 patient and new supraventricular tachycardia in 7 patients (11.3%). At 6-month follow-up (n = 60), 1 patient without residual shunt developed a transient ischemic attack and 1 developed atrial fibrillation. A mild or moderate residual shunt was noted in 51.7%, 33.9%, and 23.7% after 1-day, 1-month, and 6-month follow-up, respectively. A large shunt was present in 8.3%, 3.4%, and 0% after 1-day, 1-month, and 6-month follow-up.
Conclusions: Closure of PFO using the bioabsorbable device is associated with a low complication rate and a low recurrence rate of embolic events. However, a relatively high percentage of mild or moderate residual shunting is still present at 6-month follow-up.
Sunday, September 19, 2010
Innovation: Parental Use of External Defibrillator for Infant with LQTS
Pediatrics 2006:118:e526-e529
Abhay Divekar and Reeni Soni
Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.
Abhay Divekar and Reeni Soni
Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.
Additional Reading:
T wave morphology in LQTS
Circulation 2000;102:2849-2855
Very useful article to identify genotype based on EKG findings.
Thursday, September 16, 2010
Echo: 3D imaging of ASD & Right Atrium
Anatomy of Right Atrial Structures by Real-Time 3D Transesophageal Echocardiography
Francesco F. Faletra, Siew Y. Ho, and Angelo Auricchio
J Am Coll Cardiol Img 2010;3 966-975
3D Echocardiography of the Atrial Septum: Anatomical Features and Landmarks for the Echocardiographer
Kuberan Pushparajah, Owen I. Miller, and John M. Simpson
J Am Coll Cardiol Img 2010;3 981-984
Francesco F. Faletra, Siew Y. Ho, and Angelo Auricchio
J Am Coll Cardiol Img 2010;3 966-975
3D Echocardiography of the Atrial Septum: Anatomical Features and Landmarks for the Echocardiographer
Kuberan Pushparajah, Owen I. Miller, and John M. Simpson
J Am Coll Cardiol Img 2010;3 981-984
ICU: ECMO for CPR
Circulation. 2010;122:S241-S248
Rapid-Response Extracorporeal Membrane Oxygenation to Support Cardiopulmonary Resuscitation in Children With Cardiac Disease
David A. Kane, MD; Ravi R. Thiagarajan, MBBS, MPH; David Wypij, PhD; Mark A. Scheurer, MD; Francis Fynn-Thompson, MD; Sitaram Emani, MD; Pedro J. del Nido, MD; Peter Betit, RRT; Peter C. Laussen, MBBS
Background—Survival of children with in-hospital cardiac arrest that does not respond to conventional cardiopulmonary resuscitation (CPR) is poor. We report on survival and early neurological outcomes of children with heart disease supported with rapid-response extracorporeal membrane oxygenation (ECMO) to aid cardiopulmonary resuscitation (ECPR).
Methods and Results—Children with heart disease supported with ECPR were identified from our ECMO database. Demographic, CPR, and ECMO details associated with mortality were evaluated using multivariable logistic regression. Pediatric overall performance category and pediatric cerebral performance category scores were assigned to ECPR survivors to assess neurological outcomes. There were 180 ECPR runs in 172 patients. Eighty-eight patients (51%) survived to discharge. Survival in patients who underwent ECPR after cardiac surgery (54%) did not differ from nonsurgical patients (46%). Survival did not vary by cardiac diagnosis and CPR duration did not differ between survivors and nonsurvivors. Factors associated with mortality included noncardiac structural or chromosomal abnormalities (OR, 3.2; 95% CI, 1.3–7.9), use of blood-primed ECMO circuit (OR, 7.1; 95% CI, 1.4–36), and arterial pH <7.00 after ECMO deployment (OR, 6.0; 95% CI, 2.1–17.4). Development of end-organ injury on ECMO and longer ECMO duration were associated with increased mortality. Of pediatric overall performance category/pediatric cerebral performance category scores assigned to survivors, 75% had scores 2, indicating no to mild neurological injury.
Conclusions—ECPR may promote survival in children with cardiac disease experiencing cardiac arrest unresponsive to conventional CPR with favorable early neurological outcomes. CPR duration was not associated with mortality, whereas patients with metabolic acidosis and noncardiac structural or chromosomal anomalies had higher mortality.
Rapid-Response Extracorporeal Membrane Oxygenation to Support Cardiopulmonary Resuscitation in Children With Cardiac Disease
David A. Kane, MD; Ravi R. Thiagarajan, MBBS, MPH; David Wypij, PhD; Mark A. Scheurer, MD; Francis Fynn-Thompson, MD; Sitaram Emani, MD; Pedro J. del Nido, MD; Peter Betit, RRT; Peter C. Laussen, MBBS
Background—Survival of children with in-hospital cardiac arrest that does not respond to conventional cardiopulmonary resuscitation (CPR) is poor. We report on survival and early neurological outcomes of children with heart disease supported with rapid-response extracorporeal membrane oxygenation (ECMO) to aid cardiopulmonary resuscitation (ECPR).
Methods and Results—Children with heart disease supported with ECPR were identified from our ECMO database. Demographic, CPR, and ECMO details associated with mortality were evaluated using multivariable logistic regression. Pediatric overall performance category and pediatric cerebral performance category scores were assigned to ECPR survivors to assess neurological outcomes. There were 180 ECPR runs in 172 patients. Eighty-eight patients (51%) survived to discharge. Survival in patients who underwent ECPR after cardiac surgery (54%) did not differ from nonsurgical patients (46%). Survival did not vary by cardiac diagnosis and CPR duration did not differ between survivors and nonsurvivors. Factors associated with mortality included noncardiac structural or chromosomal abnormalities (OR, 3.2; 95% CI, 1.3–7.9), use of blood-primed ECMO circuit (OR, 7.1; 95% CI, 1.4–36), and arterial pH <7.00 after ECMO deployment (OR, 6.0; 95% CI, 2.1–17.4). Development of end-organ injury on ECMO and longer ECMO duration were associated with increased mortality. Of pediatric overall performance category/pediatric cerebral performance category scores assigned to survivors, 75% had scores 2, indicating no to mild neurological injury.
Conclusions—ECPR may promote survival in children with cardiac disease experiencing cardiac arrest unresponsive to conventional CPR with favorable early neurological outcomes. CPR duration was not associated with mortality, whereas patients with metabolic acidosis and noncardiac structural or chromosomal anomalies had higher mortality.
Gender difference in mortality after congenital heart surgery
Circulation. 2010;122:S234-S240
Sex Differences in Mortality in Children Undergoing Congenital Heart Disease Surgery
A United States Population–Based Study
Ariane Marelli, MD; Kimberlee Gauvreau, ScD; Mike Landzberg, MD; Kathy Jenkins, MD, MPH
Background—The changing demographics of the adult congenital heart disease (CHD) population requires an understanding of the factors that impact patient survival to adulthood. We sought to investigate sex differences in CHD surgical mortality in children.
Methods and Results—Children <18 years old hospitalized for CHD surgery were identified using the Kids’ Inpatient Database in 2000, 2003, and 2006. Demographic, diagnostic, and procedural variables were grouped according to RACHS-1 (Risk Adjustment for Congenital Heart Surgery) method. Logistic regression was used to determine the odds ratio of death in females versus males adjusting for RACHS-1 risk category, age, prematurity, major noncardiac anomalies, and multiple procedures. Analyses were stratified by RACHS-1 risk categories and age. Of 33 848 hospitalizations for CHD surgery, 54.7% were in males. Males were more likely than females to have CHD surgery in infancy, high-risk CHD surgery, and multiple CHD procedures. Females had more major noncardiac structural anomalies and more low-risk procedures. However, the adjusted risk of in-hospital death was higher in females (odds ratio, 1.21; 95% confidence interval, 1.08 to 1.36) on account of the subgroup with high-risk surgeries who were <1 year of age (odds ratio, 1.39; 95% confidence interval, 1.16 to 1.67).
Conclusions—In this large US population study, more male children underwent CHD surgery and had high-risk procedures. Female infants who had high-risk procedures were at higher risk for death, but this accounted for a small proportion of females and is therefore unlikely to have a major impact on the changing demographics in adults in CHD.
Sex Differences in Mortality in Children Undergoing Congenital Heart Disease Surgery
A United States Population–Based Study
Ariane Marelli, MD; Kimberlee Gauvreau, ScD; Mike Landzberg, MD; Kathy Jenkins, MD, MPH
Background—The changing demographics of the adult congenital heart disease (CHD) population requires an understanding of the factors that impact patient survival to adulthood. We sought to investigate sex differences in CHD surgical mortality in children.
Methods and Results—Children <18 years old hospitalized for CHD surgery were identified using the Kids’ Inpatient Database in 2000, 2003, and 2006. Demographic, diagnostic, and procedural variables were grouped according to RACHS-1 (Risk Adjustment for Congenital Heart Surgery) method. Logistic regression was used to determine the odds ratio of death in females versus males adjusting for RACHS-1 risk category, age, prematurity, major noncardiac anomalies, and multiple procedures. Analyses were stratified by RACHS-1 risk categories and age. Of 33 848 hospitalizations for CHD surgery, 54.7% were in males. Males were more likely than females to have CHD surgery in infancy, high-risk CHD surgery, and multiple CHD procedures. Females had more major noncardiac structural anomalies and more low-risk procedures. However, the adjusted risk of in-hospital death was higher in females (odds ratio, 1.21; 95% confidence interval, 1.08 to 1.36) on account of the subgroup with high-risk surgeries who were <1 year of age (odds ratio, 1.39; 95% confidence interval, 1.16 to 1.67).
Conclusions—In this large US population study, more male children underwent CHD surgery and had high-risk procedures. Female infants who had high-risk procedures were at higher risk for death, but this accounted for a small proportion of females and is therefore unlikely to have a major impact on the changing demographics in adults in CHD.
ICU: T3 therapy after congenital heart surgery
Circulation. 2010;122:S224-S233
Triiodothyronine Supplementation in Infants and Children Undergoing Cardiopulmonary Bypass (TRICC)
A Multicenter Placebo-Controlled Randomized Trial: Age Analysis
Michael A. Portman, MD; April Slee, MS; Aaron K. Olson, MD; Gordon Cohen, MD, PhD; Tom Karl, MD; Elizabeth Tong, PhD; Laura Hastings, MD; Hitendra Patel, MD; Olaf Reinhartz, MD; Antonio R. Mott, MD; Richard Mainwaring, MD; Justin Linam, MA; Sara Danzi, PhD, for the TRICC Investigators
Background—Triiodothyronine levels decrease in infants and children after cardiopulmonary bypass. We tested the primary hypothesis that triiodothyronine (T3) repletion is safe in this population and produces improvements in postoperative clinical outcome.
Methods and Results—The TRICC study was a prospective, multicenter, double-blind, randomized, placebo-controlled trial in children younger than 2 years old undergoing heart surgery with cardiopulmonary bypass. Enrollment was stratified by surgical diagnosis. Time to extubation (TTE) was the primary outcome. Patients received intravenous T3 as Triostat (n=98) or placebo (n=95), and data were analyzed using Cox proportional hazards. Overall, TTE was similar between groups. There were no differences in adverse event rates, including arrhythmia. Prespecified analyses showed a significant interaction between age and treatment (P=0.0012). For patients younger than 5 months, the hazard ratio (chance of extubation) for Triostat was 1.72. (P=0.0216). Placebo median TTE was 98 hours with 95% confidence interval (CI) of 71 to 142 compared to Triostat TTE at 55 hours with CI of 44 to 92. TTE shortening corresponded to a reduction in inotropic agent use and improvement in cardiac function. For children 5 months of age, or older, Triostat produced a significant delay in median TTE: 16 hours (CI, 7–22) for placebo and 20 hours (CI, 16–45) for Triostat and (hazard ratio, 0.60; P=0.0220).
Conclusions—T3 supplementation is safe. Analyses using age stratification indicate that T3 supplementation provides clinical advantages in patients younger than 5 months and no benefit for those older than 5 months.
Triiodothyronine Supplementation in Infants and Children Undergoing Cardiopulmonary Bypass (TRICC)
A Multicenter Placebo-Controlled Randomized Trial: Age Analysis
Michael A. Portman, MD; April Slee, MS; Aaron K. Olson, MD; Gordon Cohen, MD, PhD; Tom Karl, MD; Elizabeth Tong, PhD; Laura Hastings, MD; Hitendra Patel, MD; Olaf Reinhartz, MD; Antonio R. Mott, MD; Richard Mainwaring, MD; Justin Linam, MA; Sara Danzi, PhD, for the TRICC Investigators
Background—Triiodothyronine levels decrease in infants and children after cardiopulmonary bypass. We tested the primary hypothesis that triiodothyronine (T3) repletion is safe in this population and produces improvements in postoperative clinical outcome.
Methods and Results—The TRICC study was a prospective, multicenter, double-blind, randomized, placebo-controlled trial in children younger than 2 years old undergoing heart surgery with cardiopulmonary bypass. Enrollment was stratified by surgical diagnosis. Time to extubation (TTE) was the primary outcome. Patients received intravenous T3 as Triostat (n=98) or placebo (n=95), and data were analyzed using Cox proportional hazards. Overall, TTE was similar between groups. There were no differences in adverse event rates, including arrhythmia. Prespecified analyses showed a significant interaction between age and treatment (P=0.0012). For patients younger than 5 months, the hazard ratio (chance of extubation) for Triostat was 1.72. (P=0.0216). Placebo median TTE was 98 hours with 95% confidence interval (CI) of 71 to 142 compared to Triostat TTE at 55 hours with CI of 44 to 92. TTE shortening corresponded to a reduction in inotropic agent use and improvement in cardiac function. For children 5 months of age, or older, Triostat produced a significant delay in median TTE: 16 hours (CI, 7–22) for placebo and 20 hours (CI, 16–45) for Triostat and (hazard ratio, 0.60; P=0.0220).
Conclusions—T3 supplementation is safe. Analyses using age stratification indicate that T3 supplementation provides clinical advantages in patients younger than 5 months and no benefit for those older than 5 months.
Umbalanced AV septal defect: Surgical decision making based on echo
Circulation. 2010;122:S209-S215
Echocardiographic Definition and Surgical Decision-Making in Unbalanced Atrioventricular Septal Defect
A Congenital Heart Surgeons’ Society Multiinstitutional Study
Anusha Jegatheeswaran, MD; Christian Pizarro, MD; Christopher A. Caldarone, MD; Meryl S. Cohen, MD; Jeanne M. Baffa, MD; David B. Gremmels, MD; Luc Mertens, MD, PhD; Victor O. Morell, MD; William G. Williams, MD; Eugene H. Blackstone, MD; Brian W. McCrindle, MD, MPH; David M. Overman, MD
Background—Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes.
Methods and Results—Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons’ Society (CHSS) institutions (2000–2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI0.4 (right dominant) or 0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed.
The majority of patients had balanced AVSD (avvi between 0.4 and 0.6)
Conclusions—AVVI effectively characterizes the transition between balanced and unbalanced AVSD with important correlation to anatomic substrate and selected surgical strategy.
Echocardiographic Definition and Surgical Decision-Making in Unbalanced Atrioventricular Septal Defect
A Congenital Heart Surgeons’ Society Multiinstitutional Study
Anusha Jegatheeswaran, MD; Christian Pizarro, MD; Christopher A. Caldarone, MD; Meryl S. Cohen, MD; Jeanne M. Baffa, MD; David B. Gremmels, MD; Luc Mertens, MD, PhD; Victor O. Morell, MD; William G. Williams, MD; Eugene H. Blackstone, MD; Brian W. McCrindle, MD, MPH; David M. Overman, MD
Background—Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes.
Methods and Results—Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons’ Society (CHSS) institutions (2000–2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI0.4 (right dominant) or 0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed.
The majority of patients had balanced AVSD (avvi between 0.4 and 0.6)
Conclusions—AVVI effectively characterizes the transition between balanced and unbalanced AVSD with important correlation to anatomic substrate and selected surgical strategy.
RVOT remodeling during Pulm. valve replacement
Circulation. 2010;122:S201-S208.
Randomized Trial of Pulmonary Valve Replacement With and Without Right Ventricular Remodeling Surgery
Tal Geva, MD; Kimberlee Gauvreau, ScD; Andrew J. Powell, MD; Frank Cecchin, MD; Jonathan Rhodes, MD; Judith Geva, MSW; Pedro del Nido, MD
Background—Although pulmonary valve replacement (PVR) is effective in reducing right ventricular (RV) volume overload in patients with chronic pulmonary regurgitation, persistent RV dysfunction and subsequent adverse clinical outcomes have been reported. This trial was conducted to investigate whether the addition of surgical RV remodeling with exclusion of scar tissue to PVR would result in improved RV function and laboratory and clinical parameters, as compared with PVR alone.
Methods and Results—Between February 2004 and October 2008, 64 patients who underwent RV outflow tract procedures in early childhood had more than or equal to moderate pulmonary regurgitation, and fulfilled defined criteria for PVR were randomly assigned to undergo either PVR alone (n=34) or PVR with surgical RV remodeling (n=30). No significant difference was observed in the primary outcome (change in RV ejection fraction, –2±7% in the PVR alone group and –1±7% in the PVR with RV remodeling group; P=0.38) or in any of the secondary outcomes at 6-month postoperative follow-up. Multivariable analysis of the entire cohort identified preoperative RV end-systolic volume index <90 mL/m2 and QRS duration <140 ms to be associated with optimal postoperative outcome (normal RV size and function), and RV ejection fraction <45% and QRS duration 160 ms to be associated with suboptimal postoperative outcome (RV dilatation and dysfunction).
Conclusion—The addition of surgical remodeling of the RV to PVR in patients with chronic pulmonary regurgitation did not result in a measurable early benefit. Referral to PVR based on QRS duration, RV end-systolic volume, or RV ejection fraction may be beneficial.
Randomized Trial of Pulmonary Valve Replacement With and Without Right Ventricular Remodeling Surgery
Tal Geva, MD; Kimberlee Gauvreau, ScD; Andrew J. Powell, MD; Frank Cecchin, MD; Jonathan Rhodes, MD; Judith Geva, MSW; Pedro del Nido, MD
Background—Although pulmonary valve replacement (PVR) is effective in reducing right ventricular (RV) volume overload in patients with chronic pulmonary regurgitation, persistent RV dysfunction and subsequent adverse clinical outcomes have been reported. This trial was conducted to investigate whether the addition of surgical RV remodeling with exclusion of scar tissue to PVR would result in improved RV function and laboratory and clinical parameters, as compared with PVR alone.
Methods and Results—Between February 2004 and October 2008, 64 patients who underwent RV outflow tract procedures in early childhood had more than or equal to moderate pulmonary regurgitation, and fulfilled defined criteria for PVR were randomly assigned to undergo either PVR alone (n=34) or PVR with surgical RV remodeling (n=30). No significant difference was observed in the primary outcome (change in RV ejection fraction, –2±7% in the PVR alone group and –1±7% in the PVR with RV remodeling group; P=0.38) or in any of the secondary outcomes at 6-month postoperative follow-up. Multivariable analysis of the entire cohort identified preoperative RV end-systolic volume index <90 mL/m2 and QRS duration <140 ms to be associated with optimal postoperative outcome (normal RV size and function), and RV ejection fraction <45% and QRS duration 160 ms to be associated with suboptimal postoperative outcome (RV dilatation and dysfunction).
Conclusion—The addition of surgical remodeling of the RV to PVR in patients with chronic pulmonary regurgitation did not result in a measurable early benefit. Referral to PVR based on QRS duration, RV end-systolic volume, or RV ejection fraction may be beneficial.
Recoarctation - Surgery vs. Cath Intervention
Circulation 2010;122:S81-S84
Late Outcomes of Reintervention on the Descending Aorta After Repair of Aortic Coarctation
Morgan L. Brown, MD; Harold M. Burkhart, MD; Heidi M. Connolly, MD; Joseph A. Dearani, MD; Donald J. Hagler, MD; Hartzell V. Schaff, MD
Background—After repair of aortic coarctation, patients may develop restenosis, aneurysms, and pseudoaneurysms at the site of prior repair. We assessed the outcomes of late reintervention on the descending aorta after aortic coarctation repair.
Methods and Results—From March 1954 to July 2008, 130 patients had operations or endovascular procedures on the descending aorta after previous coarctation repair. We excluded patients who had complex left-sided cardiac lesions or interrupted aortic arch. Mean age at reintervention was 32±24 years and 28% were female. The interval between coarctation repair and reintervention was 17±13 years. Seventy-four percent of patients had hypertension. Reasons for reintervention were restenosis (n=122 [94%]), aneurysm (n=4 [3%]), and pseudoaneurysm (n=4 [3%]). Ninety-five patients (73%) underwent operative procedures including an extra-anatomic conduit (n=41), patch repair (n=32), interposition graft (n=14), end–end anastomosis (n=6), and subclavian flap (n=2). Thirty-five patients underwent endovascular treatment (balloon dilatation, n=22 or stenting, n=13). There was no early mortality. In the surgical group, 5 patients required early reoperation for bleeding and 5 patients had early vocal cord paralysis. One patient in the endovascular group had aortic rupture at the time of intervention requiring urgent operation. Survival was 97% at 10 years. At 5 years, freedom from a second repeat procedure on the descending aorta was 96% in the surgical group and 72% in the endovascular group (P<0.001). Five years after reintervention, fewer patients required treatment for hypertension (57% versus 74%, P<0.001) and a median of 1 antihypertensive medication was prescribed compared with a median of 2 medications preintervention.
Conclusions—Operative and endovascular management of recoarctation can be performed safely with good late outcomes.
Late Outcomes of Reintervention on the Descending Aorta After Repair of Aortic Coarctation
Morgan L. Brown, MD; Harold M. Burkhart, MD; Heidi M. Connolly, MD; Joseph A. Dearani, MD; Donald J. Hagler, MD; Hartzell V. Schaff, MD
Background—After repair of aortic coarctation, patients may develop restenosis, aneurysms, and pseudoaneurysms at the site of prior repair. We assessed the outcomes of late reintervention on the descending aorta after aortic coarctation repair.
Methods and Results—From March 1954 to July 2008, 130 patients had operations or endovascular procedures on the descending aorta after previous coarctation repair. We excluded patients who had complex left-sided cardiac lesions or interrupted aortic arch. Mean age at reintervention was 32±24 years and 28% were female. The interval between coarctation repair and reintervention was 17±13 years. Seventy-four percent of patients had hypertension. Reasons for reintervention were restenosis (n=122 [94%]), aneurysm (n=4 [3%]), and pseudoaneurysm (n=4 [3%]). Ninety-five patients (73%) underwent operative procedures including an extra-anatomic conduit (n=41), patch repair (n=32), interposition graft (n=14), end–end anastomosis (n=6), and subclavian flap (n=2). Thirty-five patients underwent endovascular treatment (balloon dilatation, n=22 or stenting, n=13). There was no early mortality. In the surgical group, 5 patients required early reoperation for bleeding and 5 patients had early vocal cord paralysis. One patient in the endovascular group had aortic rupture at the time of intervention requiring urgent operation. Survival was 97% at 10 years. At 5 years, freedom from a second repeat procedure on the descending aorta was 96% in the surgical group and 72% in the endovascular group (P<0.001). Five years after reintervention, fewer patients required treatment for hypertension (57% versus 74%, P<0.001) and a median of 1 antihypertensive medication was prescribed compared with a median of 2 medications preintervention.
Conclusions—Operative and endovascular management of recoarctation can be performed safely with good late outcomes.
Quatitation of Aortic Regurgitation using echocardiography
Circulation: Cardiovascular Imaging. 2010;3:542-549
An Echocardiographic Model Predicting Severity of Aortic Regurgitation in Congenital Heart Disease
Rebecca S. Beroukhim, MD; Dionne A. Graham, PhD; Renee Margossian, MD; David W. Brown, MD; Tal Geva, MD and Steven D. Colan, MD
Background—Multiple echocardiographic parameters have been identified to predict the severity of aortic regurgitation (AR) with variable reliability. This study was performed to identify which echocardiographic parameters best predict the severity of AR in a cohort of patients with congenital heart disease, using cardiovascular MRI quantification as a reference standard.
Methods and Results—The study involved 2 phases. In phase 1, predictive models were developed on the basis of multivariable analysis of various morphometric and Doppler variables obtained from 174 echocardiograms that best predicted the severity of AR as defined by paired cardiovascular MRI examinations. A nonlinear estimate of regurgitation fraction, using the variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral, was identified through multivariable analysis as the best predictive model for AR fraction. In phase 2, the predictive models were prospectively tested on 43 echocardiographic examinations for which a paired cardiovascular MRI was performed. The agreement between the observed and predicted AR fraction was assessed using Bland-Altman analysis. For the 30 studies of the validation data set that had adequate quality images of both the parasternal vena contracta width and the abdominal aorta flow profile, the predicted AR values had a mean bias±SD of 0.4±7.3% (P=0.80).
Conclusions—A model using the 2 variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral can predict AR severity in patients with a wide variety of congenital heart disease.
An Echocardiographic Model Predicting Severity of Aortic Regurgitation in Congenital Heart Disease
Rebecca S. Beroukhim, MD; Dionne A. Graham, PhD; Renee Margossian, MD; David W. Brown, MD; Tal Geva, MD and Steven D. Colan, MD
Background—Multiple echocardiographic parameters have been identified to predict the severity of aortic regurgitation (AR) with variable reliability. This study was performed to identify which echocardiographic parameters best predict the severity of AR in a cohort of patients with congenital heart disease, using cardiovascular MRI quantification as a reference standard.
Methods and Results—The study involved 2 phases. In phase 1, predictive models were developed on the basis of multivariable analysis of various morphometric and Doppler variables obtained from 174 echocardiograms that best predicted the severity of AR as defined by paired cardiovascular MRI examinations. A nonlinear estimate of regurgitation fraction, using the variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral, was identified through multivariable analysis as the best predictive model for AR fraction. In phase 2, the predictive models were prospectively tested on 43 echocardiographic examinations for which a paired cardiovascular MRI was performed. The agreement between the observed and predicted AR fraction was assessed using Bland-Altman analysis. For the 30 studies of the validation data set that had adequate quality images of both the parasternal vena contracta width and the abdominal aorta flow profile, the predicted AR values had a mean bias±SD of 0.4±7.3% (P=0.80).
Conclusions—A model using the 2 variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral can predict AR severity in patients with a wide variety of congenital heart disease.
BNP level predicts outcome of pediatric heart failure.
Circulation: Heart Failure 2010;3:606-11
BNP Levels Predict Outcome in Pediatric Heart Failure Patients
Post Hoc Analysis of the Pediatric Carvedilol Trial
Scott R. Auerbach, MD; Marc E. Richmond, MD; Jacqueline M. Lamour, MD; Elizabeth D. Blume, MD; Linda J. Addonizio, MD; Robert E. Shaddy, MD; Lynn Mahony, MD; Elfriede Pahl, MD and Daphne T. Hsu, MD
Background—The ability of serum B-type natriuretic peptide levels (BNP) to predict outcomes in children with heart failure (HF) has not been well demonstrated. This study was designed to determine whether BNP levels predict outcomes in patients with moderate symptomatic HF.
Methods and Results—We investigated whether enrollment BNP levels for the Pediatric Carvedilol Trial were associated with baseline characteristics. Freedom from a composite end point of HF hospitalization, death, or transplantation at 9 months was compared using a threshold BNP level identified using receiver operating curve analysis. Median BNP level was 110 pg/mL (interquartile range, 22.4 to 342.0 pg/mL) in 138 subjects. Median age was 3.4 years (interquartile range, 1.1 to 11.0 years). Diagnoses were cardiomyopathy (60%) and congenital heart disease (40%); 73% had a systemic left ventricle. BNP levels correlated moderately with left ventricular ejection fraction (R=0.39, P<0.001) r="0.19)." p="0.002)">2 years (hazard ratio, 4.45; 95% confidence interval, 1.68 to 12.04; P=0.003) were independently associated with worse outcomes.
Conclusions—In children with moderately symptomatic HF, BNP 140 pg/mL and age >2 years identified subjects at higher risk for worse outcome. Further validation is needed to determine the BNP levels necessary to stratify risk in other pediatric cohorts.
BNP Levels Predict Outcome in Pediatric Heart Failure Patients
Post Hoc Analysis of the Pediatric Carvedilol Trial
Scott R. Auerbach, MD; Marc E. Richmond, MD; Jacqueline M. Lamour, MD; Elizabeth D. Blume, MD; Linda J. Addonizio, MD; Robert E. Shaddy, MD; Lynn Mahony, MD; Elfriede Pahl, MD and Daphne T. Hsu, MD
Background—The ability of serum B-type natriuretic peptide levels (BNP) to predict outcomes in children with heart failure (HF) has not been well demonstrated. This study was designed to determine whether BNP levels predict outcomes in patients with moderate symptomatic HF.
Methods and Results—We investigated whether enrollment BNP levels for the Pediatric Carvedilol Trial were associated with baseline characteristics. Freedom from a composite end point of HF hospitalization, death, or transplantation at 9 months was compared using a threshold BNP level identified using receiver operating curve analysis. Median BNP level was 110 pg/mL (interquartile range, 22.4 to 342.0 pg/mL) in 138 subjects. Median age was 3.4 years (interquartile range, 1.1 to 11.0 years). Diagnoses were cardiomyopathy (60%) and congenital heart disease (40%); 73% had a systemic left ventricle. BNP levels correlated moderately with left ventricular ejection fraction (R=0.39, P<0.001) r="0.19)." p="0.002)">2 years (hazard ratio, 4.45; 95% confidence interval, 1.68 to 12.04; P=0.003) were independently associated with worse outcomes.
Conclusions—In children with moderately symptomatic HF, BNP 140 pg/mL and age >2 years identified subjects at higher risk for worse outcome. Further validation is needed to determine the BNP levels necessary to stratify risk in other pediatric cohorts.
History: Blalock-Taussig Shunt Original 1945 JAMA Paper
The surgical treatment of malformations of the heart
in which there is pulmonary stenosis or pulmonary atresia
in which there is pulmonary stenosis or pulmonary atresia
Alfred Blalock & Helen B. Taussig
They reported 3 cases in whom BT shunt was performed. First patient E.M.S. had surgery on Nov 29, 1944.
Related article:
Has the time come to rename the Blalock-Taussig shunt?
Thomas Brogan & George Alfieris
Tuesday, September 14, 2010
Interventional Catheterization Performed in the Early Postoperative Period After Congenital Heart Surgery in Children
JACC 2004;43:1264-9
LPA stenosis - stent:
BT shunt occlusion - balloon:
Notable comments in the article:
Evan Zahn et al.
1995-2001.
62 patients underwent 66 catheterizations.
Median postop day 9 (0-42 days).
35 cases involved 50 interventions.
Median age 4 mo (2 days - 11 years)
Median weight 4.7 kg (2.3 - 45 kg)
Success rate:
Angioplasty 100%
Stent implantation 87%
Vascular/Septal occlusion 100%
Palliative pulmonary valvotomy 75%
30 procedures were Angioplasty or Stent placement. 26 of these were at recent suture lines.
Complications: Stent migration 1, CVA 1, LPA stenosis 1
30-day survival: 83% (No procedure related mortality)
BT shunt occlusion - balloon:
Notable comments in the article:
1) Balloon/Stenosis ratio - 2.5 (range 1.4 - 4.4)...similar to ratio used in Rosales et al. 2002 study (Cath Cardiovasc Interv 2002;56:272-7). Therefore, reason for 20% mortality from vessel disruption may be due to intrinsic difference in technique.
2) ...A variety of freshly sutured materials can undergo angioplasty and stent placement without vessel disruption.
Potential reasons:
a. Prolene sutures - elongates as much as 34% before breaking
b. An enlargement of the circumference of the suture line (increased distance between parallel throws) in response to balloon inflation - prevents suture breakage....we believe continuous prolene suture lines can be safely and effectively expanded with balloon/stenosis ratios of 2.5 or less...since angioplasty needs higher ratio, stent placement is preferable in early postop period.
Saturday, September 11, 2010
Enalapril in Fontan - No significant advantage
Enalapril (target dose - 0.4 mg/kg/day) vs. Placebo
Enrolled 230 infants with single ventricle physiology
Followed up to 14 months of age
Primary end point: Weight at 14 months
185 completed trial(Rest were lost to either withdrawal from trial or death/transplant - 13%)
Other measures: Height, Ross score, BNP, Bayley Scale of infant development & Ventricular ejection fraction.
Result: No difference between the two group in any of the parameters studied.
Death or Transplant occurred in 13% in either group.
Adverse reactions occurred in 88 of Enalapril group and 87 of Placebo group.
Intraoperative Monitoring of Rec. Laryngeal Nerve
JTCVS 2010;140:578-82
Can we try to do this in neonates?
Will the instrumentation lend itself to application in neonates?
Will the instrumentation lend itself to application in neonates?
Postop. Neonatal Nutrition
Nutrition in Clinical Practice 2009;24:242-9.
Nutrition Support After Neonatal Cardiac Surgery
Joyce L. Owens, RD,CD, CANS
Ndidiamaka Musa, MD
Medical College of Wisconsin, Pediatrics: Critical Care, Milwaukee, Wisconsin.
Abstract
Congenital heart disease is the most common birth defect in the United States, with an estimated frequency of approximately 12–14 of 1000 live births per year. Neonates with congenital heart disease often need palliative or corrective surgery requiring cardiopulmonary bypass during the first weeks of life. The neonate undergoing cardiopulmonary bypass surgery experiences a more profound metabolic response to stress than that seen in older children and adults undergoing surgery. However, compared with older children and adults, the neonate has less metabolic reserves and is extremely vulnerable to the negative metabolic impact induced by stress, which can lead to suboptimal wound healing and growth failure. There are complications associated with the metabolic derangements of neonatal surgery requiring cardiopulmonary bypass, including but not limited to acute renal failure, chylothorax, and neurological dysfunction. This article discusses the importance of nutrition and metabolic support for the neonate undergoing cardiopulmonary bypass and the immediate postoperative nutrition needs of such a patient. Also, this article uses a case study to examine the feeding methodology used at one particular institution after neonatal cardiac surgery. The purpose of the case study is to provide an illustration of the many factors and obstacles that clinicians often face in the provision and timing of nutrition support.
Nutrition Support After Neonatal Cardiac Surgery
Joyce L. Owens, RD,CD, CANS
Ndidiamaka Musa, MD
Medical College of Wisconsin, Pediatrics: Critical Care, Milwaukee, Wisconsin.
Abstract
Congenital heart disease is the most common birth defect in the United States, with an estimated frequency of approximately 12–14 of 1000 live births per year. Neonates with congenital heart disease often need palliative or corrective surgery requiring cardiopulmonary bypass during the first weeks of life. The neonate undergoing cardiopulmonary bypass surgery experiences a more profound metabolic response to stress than that seen in older children and adults undergoing surgery. However, compared with older children and adults, the neonate has less metabolic reserves and is extremely vulnerable to the negative metabolic impact induced by stress, which can lead to suboptimal wound healing and growth failure. There are complications associated with the metabolic derangements of neonatal surgery requiring cardiopulmonary bypass, including but not limited to acute renal failure, chylothorax, and neurological dysfunction. This article discusses the importance of nutrition and metabolic support for the neonate undergoing cardiopulmonary bypass and the immediate postoperative nutrition needs of such a patient. Also, this article uses a case study to examine the feeding methodology used at one particular institution after neonatal cardiac surgery. The purpose of the case study is to provide an illustration of the many factors and obstacles that clinicians often face in the provision and timing of nutrition support.
Delayed Sternal Closure
J Thorac Cardiovasc Surg 1997;113:886-893
Objective: The purpose of this study was to assess morbidity and mortality associated with delayed sternal closure after pediatric cardiac operations. Methods: Hospital records were reviewed of all patients with an open sternum after a cardiac operation at Children's Hospital, Boston, from January 1992 to December 1995. Results: A total of 178 patients had delayed sternal closure with an overall mortality rate of 19%. The most common diagnosis of patients with delayed sternal closure was hypoplastic left heart syndrome (29%). Although myocardial distention or chest wall edema (n = 47) was a common indication to delay sternal closure, in many patients (n = 47) the sternum was left open electively to avoid postoperative cardiac or respiratory compromise. Successful sternal closure was achieved in 158 patients (89%) at a mean of 3.4 ± 1.8 days after opening. There were significant increases in left atrial pressure (7.7 ± 3.4 to 9.8 ± 4.1 mm Hg, p = 0.00001) and right atrial pressure (8 ± 3.2 to 10.1 ± 3.3 mm Hg, p = 0.00001) with sternal closure. There was a small but statistically significant drop in pH (7.44 ± 0.05 to 7.41 ± 0.08, p < 0.0001) during sternal closure. The peak inspiratory pressure, delivered breaths per minute, and fraction of inspired oxygen all significantly increased during sternal closure. Clinical evidence of surgical site infection occurred in 12 (6.7%) of the patients with delayed sternal closure; mediastinitis developed in 7 (3.9%) patients. Conclusions: Although delayed sternal closure after complex operations for congenital heart disease is often necessary in the operating room because of edema, unstable hemodynamic conditions, or bleeding, it can also be used electively to aid in hemodynamic and respiratory stability in the initial postoperative period. Our review supports a low morbidity associated with delayed sternal closure in a pediatric population.
Delayed Sternal Closure is life-saving:
Children - European Journal of Cardio-thoracic Surgery 2002;21(5):787-93
Adult - Annals of Thoracic and Cardiovascular Surgery 2002;8:220-3
Nursing Consideration in Delayed Sternal Closure in Children: Critical Care Nurse June 2010;30:50-61.
Decrease in rSO2 (cerebral & somatic) after Delayed Sternal Closure - JTCVS Apr 2010;139:894-900
Ventricular remodeling after Myocarditis vs. Dilated Cardiomyopathy in Children
Circulatation: Heart Failure 2010
Published online Sep 10, 2010
Ventricular Remodeling and Survival are More Favorable for Myocarditis Than For Idiopathic Dilated Cardiomyopathy in Childhood: An Outcomes Study from the Pediatric Cardiomyopathy Registry
Susan R. Foerster ; Charles E. Canter ; Amy Cinar ; Lynn A. Sleeper ; Steven A. Webber ; Elfriede Pahl ;Paul F. Kantor ; Jorge A. Alvarez ; Steven D. Colan ; John L. Jefferies ; Jacqueline M. Lamour ;Renee Margossian ; Jane E. Messere ;Paolo G. Rusconi ; Robert E. Shaddy ; Jeffrey A. Towbin ;James D. Wilkinson and Steven E. Lipshultz
Background—Myocarditis is one cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function.
Methods and Results—The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (BCM, n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (PM, n=253) were compared to children with idiopathic dilated cardiomyopathy (IDCM, n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the BCM and PM groups did not differ (P
0.5), but both groups differed significantly from the IDCM group (all P
0.003). In children with myocarditis,lower LVFS z-score at presentation predicted greater mortality (hazard ratio [HR]=0.85, 95% CI 0.73-0.98, P=0.03) and greater LV posterior wall thickness predicted transplantation (HR=1.17, 95% CI 1.02-1.35, P=0.03). In those with decreased LVFS at presentation, independent predictors of echocardiographic normalization were presentation with an LVEDD z-score >2 (HR=0.36, 95% CI 0.22-0.58, P <0.001)> (HR=1.16, 95% CI 1.01-1.34, P=0.04).
Conclusions—Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplant, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with IDCM. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation.
Subscribe to:
Posts (Atom)