RACHS
RACHS-1
Aristotle Comprehensive Complexity Score (ACC)
STAT score (STS-EACTS)
Paper comparing RACHS-1 and ACC: Ann Thoracic Surgery 2011;92(3):949-56.
The ACC was a better predictor of operative mortality and length of intensive care unit stay than RACHS-1. In order to achieve similar performance, regression models including RACHS-1 need to be further adjusted on age, prematurity, and major extracardiac abnormalities.
Paper using STAT score: JTCVS 2014;147:666-71. Adult congenital heart surgery.
RACHS categories - partial list
Showing posts with label Outcome. Show all posts
Showing posts with label Outcome. Show all posts
Saturday, August 16, 2014
Saturday, March 24, 2012
Fundoplication & Gastrostomy ...outcome of single ventricle
Keating JJ et al. JTCVS 2012;143:891-895
1999=2007
n=155
32 (21%) had fundoplication &/or G-tube (24 had both. 7 had G-tube only).
65 (42%) were HLHS. 24 of 65 had Fundos &/or G-tube.
Need for fundo &/or Gtube was associated with lower transplant-free survival.
Wednesday, June 22, 2011
Adult CHD: Risk factors for death in pediatric hospitals
Risk Factors for Death After Adult Congenital Heart Surgery in Pediatric Hospitals
- Yuli Y. Kim, MD,
- Kimberlee Gauvreau, ScD,
- Emile A. Bacha, MD,
- Michael J. Landzberg, MD and
- Oscar J. Benavidez, MD, MPP
- CIRCOUTCOMES.110.958256
- Correspondence to Oscar J. Benavidez, MD, Department of Cardiology, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115. E-mailOscar.Benavidez@cardio.chboston.org
Abstract
Background Despite the central role that pediatric hospitals play in the surgical treatment of congenital heart disease, little is known about outcomes of adult congenital cardiac surgical care in pediatric hospitals. Risk factors for inpatient death, including adult congenital heart (ACH) surgery volume, are poorly described.
Methods and Results We obtained inpatient data from 42 free-standing pediatric hospitals using the Pediatric Health Information System data base 2000 to 2008 and selected ACH surgery admissions (ages 18 to 49 years). We examined admission characteristics and hospital surgery volume. Of 97 563 total (pediatric and adult) congenital heart surgery admissions, 3061 (3.1%) were ACH surgery admissions. Median adult age was 22 years and 39% were between ages 25 to 49 years. Most frequent surgical procedures were pulmonary valve replacement, secundum atrial septal defect repair, and aortic valve replacement. Adult mortality rate was 2.2% at discharge. Multivariable analyses identified the following risk factors for death: age 25 to 34 years (adjusted odds ratio [AOR], 2.1; P=0.009), age 35 to 49 years (AOR, 3.2; P=0.001), male sex (AOR, 1.8; P=0.04), government-sponsored insurance (AOR, 1.8; P=0.03), and higher surgical risk categories 4+ (AOR, 21.5; P=0.001). After adjusting for case mix, pediatric hospitals with high ACH surgery volume had reduced odds for death (AOR, 0.4; P=0.003). There was no relationship between total congenital heart surgery volume and ACH inpatient mortality.
Conclusions Older adults, male sex, government-sponsored insurance, and greater surgical case complexity have the highest likelihood of in-hospital death when adult congenital surgery is performed in free-standing pediatric hospitals. After risk-adjustment, pediatric hospitals with high ACH surgery volume have the lowest inpatient mortality.
Wednesday, May 18, 2011
Surgery: TAPVR - Primary Sutureless Repair Results
The Journal of Thoracic and Cardiovascular Surgery Volume 141, Issue 6, June 2011, Pages 1346-1354
Congenital heart disease
Primary sutureless repair for “simple” total anomalous pulmonary venous connection: Midterm results in a single institution
Bobby Yanagawa MD, PhDa, Abdullah A. Alghamdi MD, MSca, Andreea Dragulescu MDb, Nicola Viola MDa, Osman O. Al-Radi MDa, Luc L. Mertens MD, PhDb, John G. Coles MDa, Christopher A. Caldarone MDa and Glen S. Van Arsdell MDa, ,
Objective
We have previously reported the use of an atriopericardial or “sutureless” repair for surgical management of postoperative pulmonary vein stenosis. The potential of avoiding geometric distortion of pulmonary venous suture lines and preventing post-repair pulmonary vein stenosis encouraged us to extend the use of this technique for primary “simple” total anomalous pulmonary venous connection repair.
Methods
Between January 1997 and July 2009, 57 consecutive patients (median age, 15 days; median weight, 3.4 kg) underwent sutureless or conventional total anomalous pulmonary venous connection repair.
Results
Types of total anomalous pulmonary venous connection included supracardiac in 31 patients (54%), cardiac in 15 patients (26%), and infracardiac in 11 patients (19%). Median follow-up time was 2.9 years. Preoperative mean pulmonary vein score, a composite measure of stenosis in all 4 pulmonary veins, was 0.3/0–12, and vertical vein obstruction was found in 35 patients (61.4%). A primary sutureless repair was carried out in 21 patients (36.8%; supracardiac, n = 12; cardiac, n = 4; infracardiac, n = 5). The sutureless repair group had proportionally greater high-risk infracardiac total anomalous pulmonary venous connection (24% vs 16%, P = .05). Primary outcomes of death or reoperation for pulmonary vein stenosis and postoperative pulmonary vein scores (0.2 ± 0.7 vs 0.7 ± 1.7, P = .26) were not different between the techniques.
Conclusions
The sutureless repair group had proportionally more infracardiac total anomalous pulmonary venous connection and a higher rate of decline in postoperative right ventricular systolic pressure. Despite increased preoperative risk, no difference was observed in primary outcomes of death and reoperation in the conventional repair group.
Monday, April 25, 2011
Out of Hospital Cardiac Arrest in Children - Outcomes
J Am Coll Cardiol, 2011; 57:1822-1828, doi:10.1016/j.jacc.2010.11.054
© 2011 by the American College of Cardiology Foundation
Incidence, Causes, and Outcomes of Out-of-Hospital Cardiac Arrest in Children
A Comprehensive, Prospective, Population-Based Study in the Netherlands
Abdennasser Bardai, MD et al.
Email: h.l.tan@amc.uva.nl).
Objectives: This study sought to determine comprehensively the incidence of pediatric out-of-hospital cardiac arrest (OHCA) and its contribution to total pediatric mortality, the causes of pediatric OHCA, and the outcome of resuscitation of pediatric OHCA patients.
Background: There is a paucity of complete studies on incidence, causes, and outcomes of pediatric OHCA.
Methods: In this prospective, population-based study, OHCA victims younger than age 21 years in 1 province of the Netherlands were registered through both emergency medical services and coroners over a period of 4.3 years. Death certificate data on total pediatric mortality, survival status, and neurological outcome at hospital discharge also were obtained.
Results: With a total mortality of 923 during the study period and 233 victims of OHCA (including 221 who died and 12 who survived), OHCA caused 24% (221 of 923) of total pediatric mortality. Natural causes of OHCA amounted to 115 (49%) cases, with cardiac causes being most prevalent (n = 90, 39%). The incidence of pediatric OHCA was 9.0 per 100,000 pediatric person-years (95% confidence interval: 7.8 to 10.3), whereas the incidence of pediatric OHCA from cardiac causes was 3.2 (95% confidence interval: 2.5 to 3.9). Of 51 resuscitated patients, 12 (24%) survived; among survivors, 10 (83%) had a neurologically intact outcome.
Conclusions: Out-of-hospital cardiac arrest accounts for a significant proportion of pediatric mortality, and cardiac causes are the most prevalent causes of OHCA. The vast majority of OHCA survivors have a neurologically intact outcome.
Editorial:
J Am Coll Cardiol, 2011; 57:1829-1830, doi:10.1016/j.jacc.2010.11.055
Improved Outcomes for Cardiac Arrest in Children
Share the Baton With the Bystander*
Sumeet S. Chugh, MD*
Heart Institute, Cedars-Sinai Medical Center, Los Angeles, California
* Reprint requests and correspondence: Dr. Sumeet S. Chugh, The Heart Institute, 5702 South Tower, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California 90048 (Email: sumeet.chugh@cshs.org).
Key Words: bystander • cardiac arrest • death • pediatric • population • sudden • survival
We continue to grapple with the complexities of preventing and resuscitating out-of-hospital cardiac arrests (OHCAs). Of these, cardiac arrests among children clearly have the most devastating effect on communities. The study from Bardai et al. (1) based in North Holland, the Netherlands, is an important investigation with some caveats and several lessons that merit some discussion.
This was a prospective study in a community of 2.4 million people, with 588,389 residents younger than 21 years of age. It is important to recognize that the manner in which Bardai et al. (1) presented their data tends to overestimate the actual incidence of pediatric OHCA. Their definition of OHCA was very broad and included natural causes (cardiac and noncardiac) as well as all unnatural causes of OHCA, such as traffic accidents and violent trauma. Another reason for possible overestimation relates to their inclusion of subjects 20 years of age or younger (the 18- to 20-year age group represented 25% of total OHCAs). Nonetheless, their findings indicate that similar to adults, there is likely to be geographic variation in the annual incidence of pediatric OHCA. Even if we focus on the subgroup of natural OHCA of likely cardiac cause, the annual incidence ranges from 3.2 per 100,000 children in North Holland, the Netherlands, to 7.5 per 100,000 in Portland, Oregon (2). For the purpose of reporting, it is helpful to make a clear separation between OHCA resulting from natural causes versus that resulting from unnatural causes, because the approach to prevention is distinctly different for each category. Although childhood cardiac arrests invariably are the most devastating of the OHCAs, fortunately, they are relatively uncommon, representing 1.3% and 2.8% of all OHCAs in North Holland, the Netherlands, and Portland, Oregon, respectively.
Similar to adults, the vast majority of pediatric OHCAs (78% in the North Holland study) are related to cardiac causes. However, the age distribution of North Holland pediatric OHCAs in this subgroup is distinct from that of earlier reports. The significantly lower proportion of infants (39%) is a departure from the expected highest rates in the younger than 1 year age group compared with older pediatric age groups, and the authors point us to published data that suggests a 5.7-fold higher incidence of sudden infant death syndrome (SIDS) in the United States compared with the Netherlands (3). Because they do not provide us with any information regarding what proportion of infants had a diagnosis of SIDS, we have to assume that the rates of SIDS are lower compared with those of other communities. Although successful SIDS prevention through education and modification of behavior and societal or cultural practices is a well-established strategy, the North Holland findings could indicate that there is significant room for improvement in prevention of SIDS in other parts of the world.
The most striking findings of the North Holland study relate to the outcome of resuscitated pediatric OHCAs, both in terms of survival to hospital discharge as well as neurologic recovery. The overall rate of survival to hospital discharge was 24% (12 of 51), and the overall rate of neurologic recovery was 83%. When taken in the context of the existing literature (6.4% survival in a recent large North American experience [4]), this is an enviable rate of survival and neurologic recovery for pediatric OHCAs. OHCAs with ventricular tachycardia or ventricular fibrillation, as opposed to pulseless electrical activity or asystole, are significantly more likely to survive, and Bardai et al. (1) report high rates of ventricular tachycardia or ventricular fibrillation (36% overall, with 83% among adolescents). However, these rates do not seem to be explained by the somewhat modest response times that are in the range of 11 to 12 min. It is likely, therefore, that this favorable outcome is attributable to the high rates of witnessed collapse, bystander cardiopulmonary resuscitation, and use of automated external defibrillators. Although there are other factors, such as population density and overall education level of residents, that likely contribute to the survival advantage enjoyed by North Holland pediatric residents, this solid outcome clearly reflects successful bystander education in a well-deployed emergency medical response system.
Bardai et al. (1) need to be congratulated for their impressive work in a sizable community. Although their community may have some specific characteristics that are not transferable to other regions, the excellent survival and neurologic recovery rates are unmistakable and demonstrate the feasibility of attaining better outcomes for pediatric OHCA, especially by increasing awareness within the community. Improved bystander CPR, as well as use of automated external defibrillators, are likely to be of particular benefit for resuscitation in children. Simultaneously, because most naturally occurring pediatric sudden deaths are likely to be cardiac, we also must continue to focus our efforts on prevention. Although SIDS is acknowledged to be a complex disorder with multiple causes, major inroads have also been made by focused community-based educational interventions (3). In addition, the younger the age, the higher the likelihood of a genetic cause. A renewed emphasis needs to be placed on community health care providers obtaining detailed family histories with appropriate referral for genetic screening and counseling. In the event of unexplained sudden deaths, a molecular autopsy (5,6) should become part of the community forensic investigation as a critical means of enhancing prevention for family members who are left behind.
Footnotes
Dr. Chugh is the Pauline and Harold Price Professor of Cardiac Electrophysiology at the Cedars-Sinai Heart Institute, Los Angeles, California; there are no other relationships to disclose.
* Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.
References
1. Bardai A, Berdowski J, van der Werf C, et al. Incidence, causes, and outcomes of out-of-hospital cardiac arrest in children: a comprehensive, prospective, population-based study in the Netherlands J Am Coll Cardiol 2011;57:1822-1828.[Abstract/Free Full Text]
2. Chugh SS, Reinier K, Balaji S, et al. Population-based analysis of sudden death in children: the Oregon Sudden Unexpected Death Study Heart Rhythm 2009;6:1618-1622.[CrossRef][Web of Science][Medline]
3. Moon RY, Horne RS, Hauck FR. Sudden infant death syndrome Lancet 2007;370:1578-1587.[CrossRef][Web of Science][Medline]
4. Atkins DL, Everson-Stewart S, Sears GK, et al. Epidemiology and outcomes from out-of-hospital cardiac arrest in children: the Resuscitation Outcomes Consortium Epistry-Cardiac Arrest Circulation 2009;119:1484-1491.[Abstract/Free Full Text]
5. Chugh SS, Senashova O, Watts A, et al. Postmortem molecular screening in unexplained sudden death J Am Coll Cardiol 2004;43:1625-1629.[Abstract/Free Full Text]
6. Tan HL, Hofman N, van Langen IM, van der Wal AC, Wilde AA. Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives Circulation 2005;112:207-213.[Abstract/Free Full Text]
© 2011 by the American College of Cardiology Foundation
Incidence, Causes, and Outcomes of Out-of-Hospital Cardiac Arrest in Children
A Comprehensive, Prospective, Population-Based Study in the Netherlands
Abdennasser Bardai, MD et al.
Email: h.l.tan@amc.uva.nl).
Objectives: This study sought to determine comprehensively the incidence of pediatric out-of-hospital cardiac arrest (OHCA) and its contribution to total pediatric mortality, the causes of pediatric OHCA, and the outcome of resuscitation of pediatric OHCA patients.
Background: There is a paucity of complete studies on incidence, causes, and outcomes of pediatric OHCA.
Methods: In this prospective, population-based study, OHCA victims younger than age 21 years in 1 province of the Netherlands were registered through both emergency medical services and coroners over a period of 4.3 years. Death certificate data on total pediatric mortality, survival status, and neurological outcome at hospital discharge also were obtained.
Results: With a total mortality of 923 during the study period and 233 victims of OHCA (including 221 who died and 12 who survived), OHCA caused 24% (221 of 923) of total pediatric mortality. Natural causes of OHCA amounted to 115 (49%) cases, with cardiac causes being most prevalent (n = 90, 39%). The incidence of pediatric OHCA was 9.0 per 100,000 pediatric person-years (95% confidence interval: 7.8 to 10.3), whereas the incidence of pediatric OHCA from cardiac causes was 3.2 (95% confidence interval: 2.5 to 3.9). Of 51 resuscitated patients, 12 (24%) survived; among survivors, 10 (83%) had a neurologically intact outcome.
Conclusions: Out-of-hospital cardiac arrest accounts for a significant proportion of pediatric mortality, and cardiac causes are the most prevalent causes of OHCA. The vast majority of OHCA survivors have a neurologically intact outcome.
Editorial:
J Am Coll Cardiol, 2011; 57:1829-1830, doi:10.1016/j.jacc.2010.11.055
Improved Outcomes for Cardiac Arrest in Children
Share the Baton With the Bystander*
Sumeet S. Chugh, MD*
Heart Institute, Cedars-Sinai Medical Center, Los Angeles, California
* Reprint requests and correspondence: Dr. Sumeet S. Chugh, The Heart Institute, 5702 South Tower, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California 90048 (Email: sumeet.chugh@cshs.org).
Key Words: bystander • cardiac arrest • death • pediatric • population • sudden • survival
We continue to grapple with the complexities of preventing and resuscitating out-of-hospital cardiac arrests (OHCAs). Of these, cardiac arrests among children clearly have the most devastating effect on communities. The study from Bardai et al. (1) based in North Holland, the Netherlands, is an important investigation with some caveats and several lessons that merit some discussion.
This was a prospective study in a community of 2.4 million people, with 588,389 residents younger than 21 years of age. It is important to recognize that the manner in which Bardai et al. (1) presented their data tends to overestimate the actual incidence of pediatric OHCA. Their definition of OHCA was very broad and included natural causes (cardiac and noncardiac) as well as all unnatural causes of OHCA, such as traffic accidents and violent trauma. Another reason for possible overestimation relates to their inclusion of subjects 20 years of age or younger (the 18- to 20-year age group represented 25% of total OHCAs). Nonetheless, their findings indicate that similar to adults, there is likely to be geographic variation in the annual incidence of pediatric OHCA. Even if we focus on the subgroup of natural OHCA of likely cardiac cause, the annual incidence ranges from 3.2 per 100,000 children in North Holland, the Netherlands, to 7.5 per 100,000 in Portland, Oregon (2). For the purpose of reporting, it is helpful to make a clear separation between OHCA resulting from natural causes versus that resulting from unnatural causes, because the approach to prevention is distinctly different for each category. Although childhood cardiac arrests invariably are the most devastating of the OHCAs, fortunately, they are relatively uncommon, representing 1.3% and 2.8% of all OHCAs in North Holland, the Netherlands, and Portland, Oregon, respectively.
Similar to adults, the vast majority of pediatric OHCAs (78% in the North Holland study) are related to cardiac causes. However, the age distribution of North Holland pediatric OHCAs in this subgroup is distinct from that of earlier reports. The significantly lower proportion of infants (39%) is a departure from the expected highest rates in the younger than 1 year age group compared with older pediatric age groups, and the authors point us to published data that suggests a 5.7-fold higher incidence of sudden infant death syndrome (SIDS) in the United States compared with the Netherlands (3). Because they do not provide us with any information regarding what proportion of infants had a diagnosis of SIDS, we have to assume that the rates of SIDS are lower compared with those of other communities. Although successful SIDS prevention through education and modification of behavior and societal or cultural practices is a well-established strategy, the North Holland findings could indicate that there is significant room for improvement in prevention of SIDS in other parts of the world.
The most striking findings of the North Holland study relate to the outcome of resuscitated pediatric OHCAs, both in terms of survival to hospital discharge as well as neurologic recovery. The overall rate of survival to hospital discharge was 24% (12 of 51), and the overall rate of neurologic recovery was 83%. When taken in the context of the existing literature (6.4% survival in a recent large North American experience [4]), this is an enviable rate of survival and neurologic recovery for pediatric OHCAs. OHCAs with ventricular tachycardia or ventricular fibrillation, as opposed to pulseless electrical activity or asystole, are significantly more likely to survive, and Bardai et al. (1) report high rates of ventricular tachycardia or ventricular fibrillation (36% overall, with 83% among adolescents). However, these rates do not seem to be explained by the somewhat modest response times that are in the range of 11 to 12 min. It is likely, therefore, that this favorable outcome is attributable to the high rates of witnessed collapse, bystander cardiopulmonary resuscitation, and use of automated external defibrillators. Although there are other factors, such as population density and overall education level of residents, that likely contribute to the survival advantage enjoyed by North Holland pediatric residents, this solid outcome clearly reflects successful bystander education in a well-deployed emergency medical response system.
Bardai et al. (1) need to be congratulated for their impressive work in a sizable community. Although their community may have some specific characteristics that are not transferable to other regions, the excellent survival and neurologic recovery rates are unmistakable and demonstrate the feasibility of attaining better outcomes for pediatric OHCA, especially by increasing awareness within the community. Improved bystander CPR, as well as use of automated external defibrillators, are likely to be of particular benefit for resuscitation in children. Simultaneously, because most naturally occurring pediatric sudden deaths are likely to be cardiac, we also must continue to focus our efforts on prevention. Although SIDS is acknowledged to be a complex disorder with multiple causes, major inroads have also been made by focused community-based educational interventions (3). In addition, the younger the age, the higher the likelihood of a genetic cause. A renewed emphasis needs to be placed on community health care providers obtaining detailed family histories with appropriate referral for genetic screening and counseling. In the event of unexplained sudden deaths, a molecular autopsy (5,6) should become part of the community forensic investigation as a critical means of enhancing prevention for family members who are left behind.
Footnotes
Dr. Chugh is the Pauline and Harold Price Professor of Cardiac Electrophysiology at the Cedars-Sinai Heart Institute, Los Angeles, California; there are no other relationships to disclose.
* Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.
References
1. Bardai A, Berdowski J, van der Werf C, et al. Incidence, causes, and outcomes of out-of-hospital cardiac arrest in children: a comprehensive, prospective, population-based study in the Netherlands J Am Coll Cardiol 2011;57:1822-1828.[Abstract/Free Full Text]
2. Chugh SS, Reinier K, Balaji S, et al. Population-based analysis of sudden death in children: the Oregon Sudden Unexpected Death Study Heart Rhythm 2009;6:1618-1622.[CrossRef][Web of Science][Medline]
3. Moon RY, Horne RS, Hauck FR. Sudden infant death syndrome Lancet 2007;370:1578-1587.[CrossRef][Web of Science][Medline]
4. Atkins DL, Everson-Stewart S, Sears GK, et al. Epidemiology and outcomes from out-of-hospital cardiac arrest in children: the Resuscitation Outcomes Consortium Epistry-Cardiac Arrest Circulation 2009;119:1484-1491.[Abstract/Free Full Text]
5. Chugh SS, Senashova O, Watts A, et al. Postmortem molecular screening in unexplained sudden death J Am Coll Cardiol 2004;43:1625-1629.[Abstract/Free Full Text]
6. Tan HL, Hofman N, van Langen IM, van der Wal AC, Wilde AA. Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives Circulation 2005;112:207-213.[Abstract/Free Full Text]
Wednesday, April 20, 2011
Survival difference based on ethnicity
Published online April 18, 2011
PEDIATRICS (doi:10.1542/peds.2010-2702)
Racial/Ethnic Disparities in Risk of Early Childhood Mortality Among Children With Congenital Heart Defects
Wendy N. Nembhard, PhD, Jason L. Salemi, MPH, Mary K. Ethen, MPH, David E. Fixler, MD, MSc, Angela DiMaggio, BS, Mark A. Canfield, PhD
BACKGROUND Infants with congenital heart defects (CHDs) have increased risk of childhood morbidity and mortality. However, little is known about racial/ethnic differences in early childhood mortality.
PATIENTS AND METHODS We conducted a retrospective cohort study with data from the Texas Birth Defect Registry on 19 530 singleton, live-born infants with a CHD and born January 1, 1996, to December 31, 2003, to non-Hispanic (NH) white, NH black, and Hispanic women. Texas Birth Defect Registry data were linked to Texas death records and the National Death Index to ascertain deaths between January 1, 1996, and December 31, 2005. Kaplan-Meier survival estimates were computed, and hazard ratios (HRs) and 95% confidence intervals (CIs) were calculated from multivariable Cox-proportional hazard regression models to determine the effect of maternal race/ethnicity on mortality for selected CHD phenotypes.
RESULTS After adjusting for covariates, compared with NH white children, NH black children had increased early childhood mortality risk for transposition of the great arteries (HR: 2.04 [95% CI: 1.40–2.97]), tetralogy of Fallot (HR: 1.85 [95% CI: 1.09–3.12]), pulmonary valve atresia without ventricular septal defect (VSD) (HR: 2.60 [95% CI: 1.32–5.12]), VSD (HR: 1.56 [95% CI: 1.19–2.03]), and atrial septal defect (HR: 1.34 [95% CI: 1.08–1.66]). Hispanic children had higher mortality risk for pulmonary valve atresia without VSD (HR: 1.76 [95% CI: 1.06–2.91]) and hypoplastic left heart syndrome (HR: 1.51 [95% CI: 1.13–2.02]).
CONCLUSIONS We provide evidence that supports racial/ethnic disparities in early childhood mortality among infants with CHDs. Identifying infants with the greatest risk of early childhood mortality will facilitate development of interventions and policies to mitigate these risks.
PEDIATRICS (doi:10.1542/peds.2010-2702)
Racial/Ethnic Disparities in Risk of Early Childhood Mortality Among Children With Congenital Heart Defects
Wendy N. Nembhard, PhD, Jason L. Salemi, MPH, Mary K. Ethen, MPH, David E. Fixler, MD, MSc, Angela DiMaggio, BS, Mark A. Canfield, PhD
BACKGROUND Infants with congenital heart defects (CHDs) have increased risk of childhood morbidity and mortality. However, little is known about racial/ethnic differences in early childhood mortality.
PATIENTS AND METHODS We conducted a retrospective cohort study with data from the Texas Birth Defect Registry on 19 530 singleton, live-born infants with a CHD and born January 1, 1996, to December 31, 2003, to non-Hispanic (NH) white, NH black, and Hispanic women. Texas Birth Defect Registry data were linked to Texas death records and the National Death Index to ascertain deaths between January 1, 1996, and December 31, 2005. Kaplan-Meier survival estimates were computed, and hazard ratios (HRs) and 95% confidence intervals (CIs) were calculated from multivariable Cox-proportional hazard regression models to determine the effect of maternal race/ethnicity on mortality for selected CHD phenotypes.
RESULTS After adjusting for covariates, compared with NH white children, NH black children had increased early childhood mortality risk for transposition of the great arteries (HR: 2.04 [95% CI: 1.40–2.97]), tetralogy of Fallot (HR: 1.85 [95% CI: 1.09–3.12]), pulmonary valve atresia without ventricular septal defect (VSD) (HR: 2.60 [95% CI: 1.32–5.12]), VSD (HR: 1.56 [95% CI: 1.19–2.03]), and atrial septal defect (HR: 1.34 [95% CI: 1.08–1.66]). Hispanic children had higher mortality risk for pulmonary valve atresia without VSD (HR: 1.76 [95% CI: 1.06–2.91]) and hypoplastic left heart syndrome (HR: 1.51 [95% CI: 1.13–2.02]).
CONCLUSIONS We provide evidence that supports racial/ethnic disparities in early childhood mortality among infants with CHDs. Identifying infants with the greatest risk of early childhood mortality will facilitate development of interventions and policies to mitigate these risks.
Interruprtions of Warfarin for procedures
Circulation: Cardiovascular Quality and Outcomes
2011;4(2):206-210
Guideline-Discordant Periprocedural Interruptions in Warfarin Therapy
Skolarus, Lesli E. MD, MS; Morgenstern, Lewis B. MD; Froehlich, James B. MD, MPH; Lisabeth, Lynda D. PhD; Brown, Devin L. MD, MS
Abstract
Background—: Periprocedural interruptions in warfarin therapy increase thromboembolic risks to patients and are not indicated for all procedures. We sought to determine the frequency and guideline concordance of periprocedural warfarin interruptions to inform a future educational intervention.
Methods and Results—: In October and November of 2009, an anonymous postal survey was sent to all patients followed for more than 1 year by the University of Michigan Anticoagulation service. Patients were asked how many times in the prior year they were requested to interrupt warfarin therapy for a medical or dental procedure or test and the specific indication for the requested interruption in warfarin therapy. A total of 1686 of 2133 (79%) subjects responded. The mean age of respondents was 69 years (SD=14 years). The majority were men (56%) and white (93%). Atrial fibrillation was the most common indication for warfarin therapy (n=966, 57%). At least 1 request to interrupt warfarin therapy in the prior year was given by 819 of 1648 (50%) respondents, including 481 of the 947 (51%) respondents taking warfarin for atrial fibrillation. Forty-eight percent of requests to interrupt warfarin among all respondents and 50% of requests to interrupt warfarin among those taking warfarin, specifically for atrial fibrillation, were for indications not supported by guideline statements.
Conclusions—: Periprocedural requests to interrupt warfarin therapy are common and are often discordant with current guidelines. Educational interventions may decrease risk of periprocedural thromboembolic complications.
2011;4(2):206-210
Guideline-Discordant Periprocedural Interruptions in Warfarin Therapy
Skolarus, Lesli E. MD, MS; Morgenstern, Lewis B. MD; Froehlich, James B. MD, MPH; Lisabeth, Lynda D. PhD; Brown, Devin L. MD, MS
Abstract
Background—: Periprocedural interruptions in warfarin therapy increase thromboembolic risks to patients and are not indicated for all procedures. We sought to determine the frequency and guideline concordance of periprocedural warfarin interruptions to inform a future educational intervention.
Methods and Results—: In October and November of 2009, an anonymous postal survey was sent to all patients followed for more than 1 year by the University of Michigan Anticoagulation service. Patients were asked how many times in the prior year they were requested to interrupt warfarin therapy for a medical or dental procedure or test and the specific indication for the requested interruption in warfarin therapy. A total of 1686 of 2133 (79%) subjects responded. The mean age of respondents was 69 years (SD=14 years). The majority were men (56%) and white (93%). Atrial fibrillation was the most common indication for warfarin therapy (n=966, 57%). At least 1 request to interrupt warfarin therapy in the prior year was given by 819 of 1648 (50%) respondents, including 481 of the 947 (51%) respondents taking warfarin for atrial fibrillation. Forty-eight percent of requests to interrupt warfarin among all respondents and 50% of requests to interrupt warfarin among those taking warfarin, specifically for atrial fibrillation, were for indications not supported by guideline statements.
Conclusions—: Periprocedural requests to interrupt warfarin therapy are common and are often discordant with current guidelines. Educational interventions may decrease risk of periprocedural thromboembolic complications.
Wednesday, March 9, 2011
ICU: Prolonged stay after cardiac surgery
Eur J Cardiothorac Surg. 2011 Jan 10. [Epub ahead of print]
Predictors of long intensive care unit stay following cardiac surgery in children.
Pagowska-Klimek I, Pychynska-Pokorska M, Krajewski W, Moll JJ.
The Department of Anesthesiology and Intensive Care, Polish Mother's Memorial Hospital Institute, Lodz, ul. Rzgowska 288/293, Poland.
Objective: Prolonged length of stay in intensive care units after congenital heart disease surgery is associated with poor outcome, places a considerable burden on the financial resources of hospitals, and is an organizational challenge as well. This research discusses the impact of perioperative factors on prolonged stay in intensive care units.
Methods: This is a retrospective study examining the determinants of prolonged intensive care length of stay in 693 children after cardiac surgery. Univariate and multivariate analyses were performed for an intensive care unit stay over 3 and over 14 days.
Results: Neonatal age, preoperative mechanical ventilation and preoperative myocardial dysfunction, complexity and duration of procedures, as well as postoperative complications (low cardiac output syndrome, bleeding, re-operation, acute kidney injury, sepsis, respiratory insufficiency, pulmonary hypertension, pneumothorax, postoperative cardiac arrest, pneumonia, and delayed sternum closure) prolong intensive care unit hospitalization over 3 days. Patients with acute kidney injury requiring renal replacement therapy, pneumothorax, pulmonary hypertension, need for re-operation during the same admission, and myocardial dysfunction prior to surgery are at high risk of intensive care unit stay over 14 days.
Conclusions: Some patients with a risk of prolonged hospitalization may be identified preoperatively, the others just after the operation. Optimizing preoperative status and aggressive treatment of complications may have significant influence on the duration of hospitalization in intensive care units. The knowledge of risk factors may facilitate organizational procedures and rational bed management.
Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
Predictors of long intensive care unit stay following cardiac surgery in children.
Pagowska-Klimek I, Pychynska-Pokorska M, Krajewski W, Moll JJ.
The Department of Anesthesiology and Intensive Care, Polish Mother's Memorial Hospital Institute, Lodz, ul. Rzgowska 288/293, Poland.
Objective: Prolonged length of stay in intensive care units after congenital heart disease surgery is associated with poor outcome, places a considerable burden on the financial resources of hospitals, and is an organizational challenge as well. This research discusses the impact of perioperative factors on prolonged stay in intensive care units.
Methods: This is a retrospective study examining the determinants of prolonged intensive care length of stay in 693 children after cardiac surgery. Univariate and multivariate analyses were performed for an intensive care unit stay over 3 and over 14 days.
Results: Neonatal age, preoperative mechanical ventilation and preoperative myocardial dysfunction, complexity and duration of procedures, as well as postoperative complications (low cardiac output syndrome, bleeding, re-operation, acute kidney injury, sepsis, respiratory insufficiency, pulmonary hypertension, pneumothorax, postoperative cardiac arrest, pneumonia, and delayed sternum closure) prolong intensive care unit hospitalization over 3 days. Patients with acute kidney injury requiring renal replacement therapy, pneumothorax, pulmonary hypertension, need for re-operation during the same admission, and myocardial dysfunction prior to surgery are at high risk of intensive care unit stay over 14 days.
Conclusions: Some patients with a risk of prolonged hospitalization may be identified preoperatively, the others just after the operation. Optimizing preoperative status and aggressive treatment of complications may have significant influence on the duration of hospitalization in intensive care units. The knowledge of risk factors may facilitate organizational procedures and rational bed management.
Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
Saturday, January 22, 2011
Cath lab: Retinal Emboli after Cardiac Cath (Adult)
Clinical significance of retinal emboli during diagnostic and therapeutic cardiac catheterization in patient with coronary artery disease
Javad Kojuri 
BMC Cardiovascular Disorders 2011, 11:5doi:10.1186/1471-2261-11-5
| Published: | 21 January 2011 |
Abstract (provisional)
Background
Cardiac catheterization may cause retinal embolization, a risk factor for cerebrovascular emboli and stroke. We describe the incidence of clinically silent and apparent retinal emboli following diagnostic and interventional coronary catheterization and associated risk factors.
Methods
Three hundred selected patients attending a tertiary referral center for diagnostic and therapeutic cardiac catheterization were studied. Direct retinal examination and examination of the visual field and acuity were done before and after catheterization by a retinal specialist.
Results
There was 5 case of retinal embolus before catheterization, and 19 patients (incidence 6.3%) developed new retinal arteriolar emboli after catheterization. Only 1 patient developed clinically apparent changes in vision. Two conventional risk factors (age and hypertension) were significantly associated with new retinal emboli. The risk of retinal emboli was also significantly associated with operator expertise.
Conclusions
Retinal embolism was found after coronary catheterization in 6.3% of our patients. This finding indicates that the retinal, and possibly the cerebral circulation, may be compromised more frequently than is clinically apparent as a complication of coronary catheterization. Age and hypertension are independent predictors of retinal embolism (clinical trial registrationNCT01157338)
Thursday, November 11, 2010
Modification to Extra-cardiac lateral tunnel Fontan - In situ pedicled pericardial tunnel.
JTCVS Volume 140, Issue 5, Pages 1076-1083 (November 2010)
In situ pericardial extracardiac lateral tunnel Fontan operation: Fifteen-year experience
Read at the 90th Annual Meeting of The American Association for Thoracic Surgery, Toronto, Ontario, Canada, May 1–5, 2010.
Nahidh W. Hasaniya, MD, PhDa, Anees J. Razzouk, MDa, Neda F. Mulla, MDb, Ranae L. Larsen, MDb, Leonard L. Bailey, MDa
Background
The study purpose is to evaluate the long-term outcome of the in situ pericardial extracardiac lateral tunnel Fontan operation.
Methods
From June 1994 to August 2009, 160 patients with single ventricle (boys, n = 96, 60%, median age = 39 months, mean weight 15.5 kg) underwent the pedicled pericardial extracardiac lateral tunnel operation. Patients' charts were reviewed for perioperative and long-term follow-up data, outcome, and mortality. The potential growth of these tunnels was evaluated.
Results
The main diagnoses included tricuspid atresia (n = 44, 27%); double-outlet right ventricle (n = 29, 18%), and hypoplastic left heart syndrome (n = 26, 16%). The mean follow-up was 6.5 ± 3.7 years (range: 0.1–15 years). There were 2 (1.3%) operative and 6 (3.7%) late deaths. Actuarial survival at 14 years was 93%. Early complications included prolonged effusions (n = 35, 22%), chylothorax (n = 5, 3.1%), readmissions (n = 35, 22%), cerebrovascular accidents (n = 8, 5%), contralateral phrenic nerve palsy (n = 1, 0.8%), and transient arrhythmias (n = 5, 3.1%). No pacemaker was needed. Late complications included tunnel stenosis (n = 3, 1.8%) managed with balloon dilatation and stenting in 2 patients and surgical revision in 1; tunnel thrombosis (n = 2, 1.2%) causing death in both patients; and protein losing-enteropathy (n = 4, 2.5%). Follow-up echocardiography of 10 patients showed laminar flow, no turbulence/gradient at the inferior vena cava and mid-tunnel levels. The diameter indexed to body surface area showed growth, reduction, or no change depending on flow demands.
Conclusions
The construction of the extracardiac lateral tunnel Fontan conduit using viable pedicled pericardium is a relatively simple, durable, and safe operation. Long-term follow-up confirms low morbidity and mortality. Fenestration is unnecessary in most patients. This viable tunnel adapts to physiologic flow demands.
In situ pericardial extracardiac lateral tunnel Fontan operation: Fifteen-year experience
Read at the 90th Annual Meeting of The American Association for Thoracic Surgery, Toronto, Ontario, Canada, May 1–5, 2010.
Nahidh W. Hasaniya, MD, PhDa, Anees J. Razzouk, MDa, Neda F. Mulla, MDb, Ranae L. Larsen, MDb, Leonard L. Bailey, MDa
Background
The study purpose is to evaluate the long-term outcome of the in situ pericardial extracardiac lateral tunnel Fontan operation.
Methods
From June 1994 to August 2009, 160 patients with single ventricle (boys, n = 96, 60%, median age = 39 months, mean weight 15.5 kg) underwent the pedicled pericardial extracardiac lateral tunnel operation. Patients' charts were reviewed for perioperative and long-term follow-up data, outcome, and mortality. The potential growth of these tunnels was evaluated.
Results
The main diagnoses included tricuspid atresia (n = 44, 27%); double-outlet right ventricle (n = 29, 18%), and hypoplastic left heart syndrome (n = 26, 16%). The mean follow-up was 6.5 ± 3.7 years (range: 0.1–15 years). There were 2 (1.3%) operative and 6 (3.7%) late deaths. Actuarial survival at 14 years was 93%. Early complications included prolonged effusions (n = 35, 22%), chylothorax (n = 5, 3.1%), readmissions (n = 35, 22%), cerebrovascular accidents (n = 8, 5%), contralateral phrenic nerve palsy (n = 1, 0.8%), and transient arrhythmias (n = 5, 3.1%). No pacemaker was needed. Late complications included tunnel stenosis (n = 3, 1.8%) managed with balloon dilatation and stenting in 2 patients and surgical revision in 1; tunnel thrombosis (n = 2, 1.2%) causing death in both patients; and protein losing-enteropathy (n = 4, 2.5%). Follow-up echocardiography of 10 patients showed laminar flow, no turbulence/gradient at the inferior vena cava and mid-tunnel levels. The diameter indexed to body surface area showed growth, reduction, or no change depending on flow demands.
Conclusions
The construction of the extracardiac lateral tunnel Fontan conduit using viable pedicled pericardium is a relatively simple, durable, and safe operation. Long-term follow-up confirms low morbidity and mortality. Fenestration is unnecessary in most patients. This viable tunnel adapts to physiologic flow demands.
Thursday, October 7, 2010
BT shunt: Sternotomy vs. Thoracotomy
Heart Lung Circ. 2010 Aug;19(8):460-4.
Surgical approaches to the blalock shunt: does the approach matter?
Shauq A, Agarwal V, Karunaratne A, Gladman G, Pozzi M, Kaarne M, Ladusans EJ.
Department of Paediatric Cardiology, Alder Hey Royal Children Hospital, Eaton Rd, Liverpool L12 2AP, United Kingdom. shauq7@yahoo.com
Abstract
OBJECTIVE: The Blalock-Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality.
METHODS: Forty-five modified BT shunts in 41 patients, mean age 93 days (1-1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot.
RESULTS: Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183 h vs. 53 h, P<0.001)>inotropic requirements (33.3% vs. 4.2%, P<0.05)>longer intensive care unit stay (mean 9.14 days vs. 3.3 days, P<0.05)>hospital stay (mean 14.59 days vs. 5 days P<0.005).
CONCLUSIONS: Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.
PMID: 20434951 [PubMed - in process]
Surgical approaches to the blalock shunt: does the approach matter?
Shauq A, Agarwal V, Karunaratne A, Gladman G, Pozzi M, Kaarne M, Ladusans EJ.
Department of Paediatric Cardiology, Alder Hey Royal Children Hospital, Eaton Rd, Liverpool L12 2AP, United Kingdom. shauq7@yahoo.com
Abstract
OBJECTIVE: The Blalock-Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality.
METHODS: Forty-five modified BT shunts in 41 patients, mean age 93 days (1-1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot.
RESULTS: Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183 h vs. 53 h, P<0.001)>inotropic requirements (33.3% vs. 4.2%, P<0.05)>longer intensive care unit stay (mean 9.14 days vs. 3.3 days, P<0.05)>hospital stay (mean 14.59 days vs. 5 days P<0.005).
CONCLUSIONS: Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.
PMID: 20434951 [PubMed - in process]
Neonatal Repair of Tetralogy of Fallot
Symptomatic neonatal Tetralogy of Fallot: Repair or Shunt?
Kanter et al. Ann Thorac Surgery 2010;89:858-63
Neonatal repair of TOF results in improved pulmonary artery development without increased need for reintervention.
Kolcz J & Pizarro, C. Eur J Cardio-thorac Surg 2005;394-99
Long-term results in right ventricular outflow tract reconstruction in neonatal cardiac surgery: Options and outcomes.
Kaza AK, et al. 2009;138:911-16
Poor outcome was reported from Boston Children's Hospital in 1991: 14 symptomatic babies had complete repair of TOF at less than 30 days. 4/14 (28.6%) died (JTCVS 1991;101:126-37).
Better results from Michigan. (Hennein HA et al. JTCVS 1995:109:332-44). n-30, No hospital death. 25% reoperation rate in mean f-up of 15 months.
Updated data from Michigan in 2000 (Hirsch JC et al. 2000;232:508-14), report 61% freedom from reoperation at 5 yrs.
Melbourne BT shunt experience.
Twelve year experience with the modified Blalock-Taussig shunt in neonates. Eur J Thorac Cardiovasc Surg 1992;6:586-9. Nearly 100 neonates with 1 death in 10 years. Find the paper and read it.
Sunday, October 3, 2010
Outcome: LBW babies with CHD
Outcome Analysis of Major Cardiac Operations in Low Birthweight Neonates.
Bove et al. Ann Thoracic Surg 2004;78:181-7.
1995-2003; Belgium, Less than 2.5 kg.
n=49 (Corrective Surgery 31, Palliative Surgery 18)
Weight: 1.3 - 2.5 (mean = 2.19) kg
Age at operation: 1 - 90 (mean = 15.2) days
Lesions: VSD 10, TOF 8, CoA 8, TGA 7, Single V 4, PA-IVS 4, IAA 3, TAPVR 3, CAVSD 2.
Overall mortality: 18% (4/31 & 5/18)
Mean f-up: 2.8 yrs
Survival: 87% for corrective surgery gp. 54% for palliative surgery gp.
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;49:175-88Tuesday, September 21, 2010
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.
Thursday, September 16, 2010
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.
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.
Sunday, July 11, 2010
Dilated Cardiomyopathy - Treatment and Outcome
The impact of changing medical treatment on transplant-free survival in pediatric dilated cardiomyopathy. Paul F Kantor, Jonathan R. Abraham, Anne I. Dipchand, Lee N. Benson, Andrew N. Redington. JACC 2010;55:1377-84.
Single institution. Retrospective review of all patients over 30 years (1976-2005).
n-189, 44 died. 24 had transplant. 10 were lost to follow up. So, the study cohort n=111.
2-yr & 5-yr transplant-free survival = 63.6% and 56.3% respectively.
5-yr survival:
Digoxin only - 67.5%
Digoxin & ACEi - 57.2%
ACEi & BB - 58.5%
Multivariate analysis:
Low LVEF at presentation is associated with increased risk of death or transplantation.
End point was not influenced by time era or treatment strategy.
Single institution. Retrospective review of all patients over 30 years (1976-2005).
n-189, 44 died. 24 had transplant. 10 were lost to follow up. So, the study cohort n=111.
2-yr & 5-yr transplant-free survival = 63.6% and 56.3% respectively.
5-yr survival:
Digoxin only - 67.5%
Digoxin & ACEi - 57.2%
ACEi & BB - 58.5%
Multivariate analysis:
Low LVEF at presentation is associated with increased risk of death or transplantation.
End point was not influenced by time era or treatment strategy.
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