Long-term results of children operated for hypoplastic left heart syndrome in Children's Heart Centre

Abstract

c o r e t v a s a 5 6 ( 2 0 1 4 ) e 4 4 9 -e 4 5 5 Children Long-term follow up a b s t r a c t Introduction: Hypoplastic left heart syndrome (HLHS), one of the most serious congenital heart defects, can be surgically paliated using 3 subsequent stages. Long-term results of this approach are reviewed in this study. Methods: Fifty-two consecutive patients (pts.) operated for HLHS from 1999 to 2012 were evaluated retrospectively. Results: Norwood stage I operation was performed at the median age of 6.5 days with a total mortality of 19%. Significant risk factor for death was lower weight at surgery (Cox proportional risk per 1 g increase = 0.997, CI 0.995-0.990, P < 0.001). Between stages I and II, 15 catheter/surgical reintervention had to be carried out in 13 pts. (aortic arch narrowing in 10/13). Forty-two pts. underwent stage II operation at the median age of 6.8 months with a total mortality of 4.8% and 18 subsequent reinterventions in 13 patients. Finally, 26 patients aged median 3.9 years underwent stage III operation (total cavopulmonary connection) with a total mortality of 8%. The probability of survival at 1/5/10 years of age was 77/77/71%. At long-term follow up (median 7.8 years) 37 of the 38 surviving patients are in NYHA functional class I or II. Conclusions: Despite a highly centralized care, surgical treatment of HLHS is still associated with a significant mortality and morbidity. Long-term survivals, however, have an acceptable functional status during childhood corresponding to other groups of patients after surgical palliation for functionally single ventricle.

Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study

“…7 From the Czech Republic, it was reported that 52 patients born 1999-2012 had a survival of 73% including survival after heart transplantation. 11 In a recent national population-based study from New Zealand, Cloete et al reported 56% survival to 1 year of age in hypoplastic left heart syndrome with intention to treat. 12 The improved survival between the early and late birth period was mainly explained by improved survival between stages I and II.…”

A systematic review examining the clinical and health-care outcomes for congenital heart disease patients using home monitoring programmes

“…mortality that occurs in patients between S1P and S2P) is reported in the literature to be between 11% and 19%. 33 – 35 Reported mortality rates compare favourably to the literature, with 10 demonstrating lower mortality. 13 – 16 , 18 , 20 , 27 , 28 , 30 , 31 A 15% mortality was noted by one study, 17 while the remaining studies were <11% when home monitoring was used.…”