Protein losing enteropathy after Fontan operation - Improved survival

JACC 2014;64(1):54-62.

Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan Operation.
Anitha S. John, et al.

From Mayo clinic.

Fetal aortic valvuloplasty for evolving HLHS

Circulation 2014;130:638-45

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

Postnatal Outcomes of the First 100 Patients

Linday R. Freud et al. from Boston

Background—Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.

Methods and Results—We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0 .01="" 10="" 5.4="" 5="" 84="" 96="" a="" after="" all="" among="" and="" at="" better="" bv="" cardiac="" death="" em="" follow-up="" freedom="" from="" hlhs="" log-rank="" median="" of="" patients="" than="" was="" which="" years="">P

=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction.

Conclusions—Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted. 

• • Valvular Heart Disease

Heart Valve Prostheses in Pregnancy: Outcomes for Women and Their Infants

1. Claire M. Lawley, et al.
2. 1. JAHA 2014;3:e000953
   2. 1. 
         

1. ¹Clinical Population Perinatal Health Research, Kolling Institute, University of Sydney, Sydney, Australia (C.M.L., C.S.A., J.B.F., C.L.R.)
2. ²Department of Cardiology, Royal North Shore Hospital, St Leonards, Australia (C.M.L., G.A.F.)
3. ³Department of Obstetrics and Gynaecology, Royal North Shore Hospital, St Leonards, Australia (T.A.N.)

1. Correspondence to:
   Claire M. Lawley, BSc(Med)Hons, MBBS(Hons), Clinical Population Perinatal Health Research Group, The Kolling Institute, University of Sydney at Royal North Shore Hospital, Sydney, Australia. E‐mail: claw2317@uni.sydney.edu.au

Abstract

Background As the prognosis of women with prosthetic heart valves improves, an increasing number are contemplating and undertaking pregnancy. Accurate knowledge of perinatal outcomes is essential, assisting counseling and guiding care. The aims of this study were to assess outcomes in a contemporary population of women with heart valve prostheses undertaking pregnancy and to compare outcomes for women with mechanical and bioprosthetic prostheses.

Methods and Results Longitudinally linked population health data sets containing birth and hospital admissions data were obtained for all women giving birth in New South Wales, Australia, 2000–2011. This included information identifying presence of maternal prosthetic heart valve. Cardiovascular and birth outcomes were evaluated. Among 1 144 156 pregnancies, 136 involved women with a heart valve prosthesis (1 per 10 000). No maternal mortality was seen among these women, although the relative risk for an adverse event was higher than the general population, including severe maternal morbidity (139 versus 14 per 1000 births, rate ratio [RR]=9.96, 95% CI 6.32 to 15.7), major maternal cardiovascular event (44 versus 1 per 1000, RR 34.6, 95% CI 14.6 to 81.6), preterm birth (183 versus 66 per 1000, RR=2.77, 95% CI 1.88 to 4.07), and small‐for‐gestational‐age infants (193 versus 95 per 1000, RR=2.03, 95% CI 1.40 to 2.96). There was a trend toward increased maternal and perinatal morbidity in women with a mechanical valve compared with those with a bioprosthetic valve.

Conclusions Pregnancies in women with a prosthetic heart valve demonstrate an increased risk of an adverse outcome, for both mothers and infants, compared with pregnancies in the absence of heart valve prostheses. In this contemporary population, the risk was lower than previously reported.the

Congenital Heart Surgery Risk Scores

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

Clinical outcome and improved survival in patients with protein-losing enteropathy after Fontan operation

Anitha John, et al.
From Mayo Clinic
JACC 2014;64(1):54-62.

Also see, Editorial JACC 2014;64(1):63-64.

Spectrum and outcome of primary cardiomyopathies diagnosed during fetal life

R Weber, ..., E. Jaeggi.
From Toronto group
JACC Heart Failure 2014;2(4):403-411