Long-Term Survival Trends Among Patients Born With Single Ventricle Heart Disease

Among children with single ventricle (SV) heart disease, survival was higher for those with left ventricular (LV) dominance and those who attained 2-ventricle circulation, according to results of a study published in The Annals of Thoracic Surgery.

For this study, patients (N=381) who received care for SV heart disease at Duke University Medical Center in the United States between 2005 and 2020 were evaluated for outcomes. Patients were stratified into 8 diagnostic groups: aortic atresia or aortic stenosis, double inlet left ventricle (DILV), double outlet right ventricle, hypoplastic left heart syndrome (HLHS), pulmonary atresia with intact ventricular septum, tricuspid atresia, unbalanced complete atrioventricular canal, or other. Patients were also stratified into 11 surgical groups: aortic arch repair or pulmonary artery banding, biventricular repair, ductal stent, heart transplant, hybrid Norwood procedure, Norwood procedure, pulmonary artery banding, systemic-pulmonary shunt, total anomalous pulmonary venous return repair (TAPVR), other, or no surgical procedure.

Over half of patients (56%) were boys, mean birth weight was 3.0±0.6 kg, gestational age at birth was 38±1.9 weeks, 16% had a chromosomal or syndromic abnormality, 58% had right ventricular dominance, the most common diagnosis was HLHS (36%), and the most common surgical procedure was Norwood (45%).

During a mean follow-up of 4.1±4.3 years, the mortality rate was 29% and at 10 years, the survival rate was 65%±3%.

Stratified by fundamental diagnosis, the best 10-year survival was observed among patients with DILV (89%±7%) and the poorest among patients with HLHS (55%±5%). Stratified by procedure, the highest survival rate was observed among those who received ductal stent (100%±0%) and poorest survival for patients that received TAPVR (33%±18%). Right ventricular dominance associated with poorer survival compared with left ventricular dominance (58% vs 78%; P =.002).

At the time of censure, 83% of patients had SV physiology, 11% biventricular circulation, and 6% had received a heart transplant. Patients who achieved biventricular circulation were associated with improved survival compared with SV physiology (87% vs 63%; P =.04). Heart transplant associated with poorer survival (59%), in which the surgical mortality rate during primary heart transplantation was 50% and 13.3% for salvage transplantation.

In general, procedures performed earlier during the study (2005-2012) were associated with lower 8-year survival rates (60% vs 74%; P =.04).

Risk for mortality was associated with chromosomal or syndromic abnormality (P =.0002), right ventricular dominance (P =.004), undergoing a hybrid procedure (P =.01), undergoing surgery earlier in the study period (P =.01), and lower weight at stage 1 operation (P =.02).

The trends observed in this study may not be generalizable to other centers.

“LV dominance, more recent intervention, and attainment of a 2-ventricle circulation were associated with improved survival,” the study authors wrote. “These data can provide important guideposts for families and clinicians for purposes of prognostication, patient counseling, and identification of high-risk patient subsets.”

Reference

Zhu A, Meza JM, Prabhu NK, et al. Survival after intervention for single ventricle heart disease over 15 Years at a single institution. Ann Thorac Surg. Published online April 13, 2022. doi:10.1016/j.athoracsur.2022.03.060