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Published on: December 11, 2017
Mid-term outcomes of classical hypoplastic left heart syndrome after Fontan procedure
Takashi Nagase1, Satoshi Fujita1, Takeaki Harada1
1Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka, Japan.
Insights
Patients with classical hypoplastic left heart syndrome (cH) show comparable survival after extracardiac total cavopulmonary connection. However, cH patients experience reduced reintervention freedom and faster exercise tolerance decline.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Cardiovascular Outcomes Research
Background:
- Extracardiac total cavopulmonary connection (ECPCC) is a palliative procedure for complex congenital heart disease.
- Classical hypoplastic left heart syndrome (cH) presents unique challenges post-Fontan palliation.
- Long-term outcomes comparing cH to other univentricular conditions after ECPCC require detailed analysis.
Purpose of the Study:
- To evaluate the outcomes of patients with classical hypoplastic left heart syndrome (cH) following extracardiac total cavopulmonary connection (ECPCC).
- To compare survival, Fontan-related events, liver function, hemodynamics, reintervention rates, and exercise tolerance between cH patients and other univentricular conditions.
Main Methods:
- Retrospective analysis of 812 patients undergoing ECPCC from 1994-2022.
- Comparison of outcomes in 109 cH patients versus 205 heterotaxy (Hx) and 498 other univentricular (O) conditions.
- Median follow-up of 10.1 years, assessing survival, Fontan events, liver function, hemodynamics, reintervention, and exercise tolerance.
Main Results:
- Survival rates at 10 and 20 years were high and similar across all groups (97.2% for cH).
- Fontan-related event rates and liver function were comparable; no liver cirrhosis observed.
- cH group showed lower pulmonary artery index, reduced freedom from reintervention, and faster decline in exercise tolerance compared to the O group.
Conclusions:
- ECPCC provides comparable survival for cH patients to other univentricular conditions, with good hemodynamics and low mid-term comorbidities.
- Concerns remain regarding the lower pulmonary artery index, decreased freedom from reintervention, and progressive decline in exercise tolerance in cH patients.
- Further research is needed to address these specific challenges and optimize long-term management for cH patients post-Fontan procedure.
Objectives:
To examine the outcomes of patients with classical hypoplastic left heart syndrome following extracardiac total cavopulmonary connection.
Methods:
We retrospectively analysed 812 cases that underwent extracardiac total cavopulmonary connection at our hospital between 1994 and 2022. With a median follow-up of 10.1 years, we compared the survival rate, Fontan-related events, liver function, postoperative haemodynamics, freedom from reintervention and exercise tolerance in 109 patients with classical hypoplastic left heart syndrome (cH group), 205 patients with heterotaxy (Hx group) and 498 patients with other univentricular conditions (O group).
Results:
The survival rates (97.2% at 10 and 20 years for the cH group) and freedom rates from all Fontan-related events (81.2% and 68.1%) were similar across groups. Liver enzyme variables did not significantly differ, and liver cirrhosis was not observed. Postoperative catheter examinations showed similar Fontan pressure, end-diastolic pressure, cardiac index and pulmonary vascular resistance across groups, with a lower pulmonary artery index in the cH group. The rates of freedom from reintervention at 10 and 20 years were lower in the cH group (80.5% and 55.7%). Maximal oxygen consumption during cardiopulmonary exercise testing declined faster in the cH group (-2.5% per year) than in the O group (-0.9% per year), with no difference with the Hx group (-2.4% per year).
Conclusions:
The overall survival rate of patients with classical hypoplastic left heart syndrome after Fontan procedure was comparable to that of patients with other univentricular syndromes, showing good haemodynamics and lower mid-term comorbidities. However, lower pulmonary artery index, reduced freedom from reintervention and progressive decline in exercise tolerance are remaining concerns.
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