Cor Vasa 2014, 56(6):e449-e455 | DOI: 10.1016/j.crvasa.2014.07.006

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

Pavel Vojtovič^*, Tomáš Tláskal, Roman Gebauer, Oleg Reich, Václav Chaloupecký, Viktor Tomek, Sylvia Krupičková, Tomáš Matějka, Petr Hecht, Jan Janoušek

Dětské kardiocentrum, Fakultní nemocnice v Motole, Praha, Česká republika

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.
Patients: Fifty-two consecutive patients (pts.) operated for HLHS from 1999 to 2012 were evaluated retrospectively.

Methods and 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.

Keywords: Children; Hypoplastic left heart syndrome; Long-term follow up; Staged palliation

Received: March 31, 2014; Revised: July 28, 2014; Accepted: July 31, 2014; Published: December 1, 2014  Show citation