Transplantation as a primary treatment for hypoplastic left heart syndrome: Intermediate-term results

Razzouk, Anees J.; Chinnock, Richard; Gundry, Steven R.; Johnston, Joyce K.; Larsen, Ranae L.; Baum, Marti; Mulla, Neda F.; Bailey, Leonard L.

doi:10.1016/0003-4975(96)00295-0

Cited by 192 publications

(92 citation statements)

References 14 publications

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“…These infants are usually very unstable, with severe hypoxemia and acidosis. 1,2 Management usually consists of either emergent surgical atrial septectomy 2,26 or catheter-based atrial septoplasty 1,3,8 in the immediate newborn period. Even with expectant management, the presurgical mortality in infants awaiting a firststage Norwood procedure 3 or a cardiac transplant 6 is high.…”

Section: Discussionmentioning

confidence: 99%

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

et al. 2005

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Background-Pulmonary venous Doppler (PVD) flow patterns in the fetus with hypoplastic left heart syndrome (HLHS) have been correlated with restrictive interatrial communication or intact atrial septum (RAS) postnatally; however, the ability of PVD to identify the neonate requiring emergent atrial septoplasty (EAS) for severe left atrial hypertension and hypoxemia has not been critically evaluated. It was the purpose of this study to determine the predictive power of fetal PVD in identifying the need for EAS in newborns with HLHS and RAS. Methods and Results-Forty-one patients with fetal PVD flow analysis and postnatally confirmed HLHS were studied.Pulsed-wave assessment of PVD flow included S-, D-, and A-wave velocity, time-velocity integral (VTI) of forward and reverse flow, and S/D velocity and forward/reverse VTI ratio. Neonatal EAS was used as the primary clinical outcome variable. Receiver operating characteristic curves were used to determine cutpoints at which PVD indices best predicted EAS. Cutpoints were evaluated for clinical accuracy and usefulness by use of Bayesian analysis. Eight of 41 subjects underwent EAS. Need for EAS was most accurately predicted by forward/reverse VTI ratio Ͻ5 (sensitivity, 0.88, 95% CI, 0.49 to 0.99; specificity, 0.97, 95% CI, 0.82 to 0.99), which, when present, increases the posttest likelihood of EAS to 74%, assuming a pretest prevalence of 10%. Accuracy and usefulness of other PVD indices were affected by false-positive results. Conclusions-In the fetus with HLHS, a PVD forward/reverse VTI ratio of Ͻ5 is the strongest predictor of the need for EAS in the newborn period. These observations should improve our ability to identify and expectantly manage the fetus with HLHS and RAS.

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Section: Discussionmentioning

confidence: 99%

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

et al. 2005

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“…It was a successful transplant, becoming the patient with the highest survival time to date 4 . Bailey obtained excellent results in patients with left heart hypoplasia, showing that pediatric cardiac transplantation is a valid option, with results perfectly comparable to transplantation in adults 5 .…”

Section: Introductionmentioning

confidence: 85%

Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades

Becker

Besa

Riveros

et al. 2017

Rev. chil. pediatr.

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Introduction: Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. Objectives: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Material and Methods: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Results: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33,3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. Conclusions: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed urgently and 34.5% died on the waiting list.

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“…Data from several centers show an 11-14% early mortality following the procedure (Chrisant et al, 2005;Razzouk et al, 1996), with a post-transplant actuarial survival of 84% (Razzouk et al, 1996), 72-76% (Chrisant et al, 2005;Razzouk et al, 1996) and 70% (Razzouk et al, 1996) at 1, 5 and 7 years, respectively. Innovations in peri-transplant management such as the development of new immunosuppressive strategies and the realization that ABO incompatibility is possible in neonatal transplantation because the immune response is not mature, have contributed to significantly improve its outcomes.…”

Section: Cardiac Transplantationmentioning

confidence: 99%

“…Innovations in peri-transplant management such as the development of new immunosuppressive strategies and the realization that ABO incompatibility is possible in neonatal transplantation because the immune response is not mature, have contributed to significantly improve its outcomes. Unfortunately, 20-25% of the patients die while awaiting for suitable donors (Jenkins et al, 2000;Razzouk et al, 1996), decreasing the rate of actuarial survival to 54-55% at 5 years when also accounting for these deaths (Chrisant et al, 2005;Jenkins et al, 2000). Although the benefit of a biventricular physiology produces better quality of life in children who receive a successful transplant compared with age-matched palliative-staged patients, major disadvantages of cardiac transplantation include high mortality on the waiting list as well as the immunosuppressant side-effects and morbidities.…”

Section: Cardiac Transplantationmentioning

confidence: 99%

Congenital Aortic Stenosis in Childhood

Crespo¹,

Dahdah²

2011

Aortic Valve Stenosis - Current View on Diagnostics and Treatment

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Transplantation as a primary treatment for hypoplastic left heart syndrome: Intermediate-term results

Cited by 192 publications

References 14 publications

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

Predictive Value of Fetal Pulmonary Venous Flow Patterns in Identifying the Need for Atrial Septoplasty in the Newborn With Hypoplastic Left Ventricle

Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades

Congenital Aortic Stenosis in Childhood

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