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Published on: April 8, 2013
Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology
Gunnar Erikssen1, Jamil Aboulhosn2, Jeannette Lin2
1ACHD Unit, Department of Cardiology, Oslo University Hospital Rikshospitalet, Oslo, Norway.
Insights
Single right ventricular (SRV) morphology is a significant predictor of mortality in patients with univentricular hearts (UVH), primarily due to heart failure. This finding holds true for SRV patients with or without hypoplastic left heart syndrome (HLHS).
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease Research
Background:
- Univentricular hearts (UVH) present a high mortality risk, necessitating improved patient stratification.
- Distinguishing between single right ventricular (SRV) and single left ventricular (SLV) morphology is crucial for risk assessment.
Purpose of the Study:
- To investigate the prognostic significance of SRV versus SLV morphology in patients with UVH.
- To enhance risk stratification for UVH patients by analyzing ventricular morphology.
Main Methods:
- A prospective study included 395 UVH patients operated between 1972 and 2016.
- Data collected included UVH morphology, surgical procedures, and outcomes (death or heart transplantation - HTX).
- Survival analysis compared SRV and SLV groups, considering heart failure as a cause of death/HTX.
Main Results:
- SRV morphology was associated with significantly higher mortality/HTX rates compared to SLV in both non-Fontan and Fontan patient groups.
- Heart failure was a more frequent cause of death/HTX in SRV patients.
- SRV morphology strongly predicted adverse outcomes in multivariable analyses (RR 3.3).
Conclusions:
- SRV morphology is a robust predictor of both short-term and long-term survival in UVH patients.
- The increased mortality in SRV patients is primarily attributed to higher rates of heart failure.
- These findings are applicable to SRV patients regardless of the presence of hypoplastic left heart syndrome (HLHS).
Objective:
Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV).
Methods:
All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX.
Results:
Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6).
Conclusion:
SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.
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