The impact of extracorporeal life support (ECLS) on hypoplastic left heart syndrome (HLHS) patients long term survival: A 10-year experience

Background —There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results —Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival ( P <0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality ( P =0.004 and P =0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions —Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.

Download Full-text

Congenital Brain Anomalies Associated With the Hypoplastic Left Heart Syndrome

PEDIATRICS ◽

10.1542/peds.85.6.984 ◽

1990 ◽

Vol 85 (6) ◽

pp. 984-990 ◽

Cited By ~ 1

Author(s):

Tracy A. Glauser ◽

Lucy B. Rorke ◽

Paul M. Weinberg ◽

Robert R. Clancy

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Brain Anomalies ◽

Physical Features ◽

Left Heart Syndrome

This study details the type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Overall, 29% of the infants had either a major or minor central nervous system abnormality. Overt central nervous system malformations, including 3 cases of agenesis of the corpus callosum and 1 case of holoprosencephaly, were seen in 4 infants (10%). Micrencephaly (brain weight at autopsy more than 2 SDs below the mean for age) was found in 27% of the infants. An immature cortical mantle was seen in 21% of the study group. Seven infants (17%) had specific recognizable patterns of malformation. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malformations. Conversely, the presence of dysmorphic features did not reliably indicate an underlying brain anomaly. Infants who had hypoplastic left heart syndrome as one of multiple nonneurologic malformations were more likely to have micrencephaly than those infants with hypoplastic left heart syndrome as an isolated abnormality. Occurrence of developmental neuropathology was elevated in those infants with hypoplastic left heart syndrome who did not have a recognizable pattern of malformation but who were small for gestational age, microcephalic, or had ocular abnormalities. Infants with hypoplastic left heart syndrome deserve careful genetic, ophthalmologic, and neurologic evaluations, imaging of their intracranial anatomy, and long-term neurologic follow-up.

Download Full-text

The genetics of hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951100005722 ◽

1999 ◽

Vol 9 (6) ◽

pp. 627-632 ◽

Cited By ~ 31

Author(s):

Paul D. Grossfeld

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Heart Defects ◽

Left Ventricular ◽

Left Ventricular Cavity ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Term Survival ◽

Surgical Options ◽

Systemic Ventricle ◽

Left Heart Syndrome

Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.

Download Full-text

Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezz376 ◽

2020 ◽

Vol 57 (6) ◽

pp. 1098-1104

Author(s):

Katarzyna Januszewska ◽

Pawel Nawrocki ◽

Anja Lehner ◽

Julia Stegger ◽

Felix Kleinerueschkamp ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Fontan Operation ◽

Pulmonary Arteries ◽

Norwood Procedure ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Second Stage ◽

The Right ◽

Left Heart Syndrome ◽

The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

Download Full-text