Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle-Type Congenital Heart Disease: The Philadelphia Fetus-to-Fontan Cohort Study

Abstract

Background Prenatal diagnosis of single ventricle-type congenital heart disease is associated with improved clinical courses. Prenatal counseling allows for optimal delivery preparations and opportunity for prenatal intervention. Expectant parents frequently ask what the likelihood of survival through staged palliation is and the factors that influence outcome. Our goal was specifically to quantify peri- and postnatal outcomes in this population. Methods and Results We identified all patients with a prenatal diagnosis of single ventricle-type congenital heart disease presenting between July 2004 and December 2011 at our institution. Maternal data, fetal characteristics, and data from the postnatal clinical course were collected for each patient. Kaplan-Meier curves and multivariate analysis with logistic regression were used to evaluate variables associated with decreased transplant-free survival. Five hundred two patients were identified, consisting of 381 (76%) right ventricle- and 121 left ventricle-dominant lesions. After prenatal diagnosis, 42 patients did not follow up at our center; 79 (16%) chose termination of pregnancy, and 11 had intrauterine demise with 370 (74%) surviving to birth. Twenty-two (6%) underwent palliative care at birth. Among 348 surviving to birth with intention to treat, 234 (67%) survived to at least 6 months post-Fontan palliation. Presence of fetal hydrops, right ventricle dominance, presence of extracardiac anomalies, and low birthweight were significantly associated with decreased transplant-free survival. Conclusions In patients with a prenatal diagnosis of single ventricle-type congenital heart disease and intention to treat, 67% survive transplant-free to at least 6 months beyond Fontan operation. An additional 5% survive to 4 years of age without transplant or Fontan completion. Fetuses with right ventricle-dominant lesions, extracardiac anomalies, hydrops, or low birthweights have decreased transplant-free survival.

Keywords: fetal; fetal echocardiography; outcomes research; risk factor; single ventricle.

Figure 1

Longitudinal outcomes of all prenatally diagnosed fetuses with single ventricle‐type congenital heart disease (SVCHD). 2V indicates biventricular; IUFD, intrauterine fetal demise; OHT, orthotopic heart transplant; PD, prenatal diagnosis; TOP, termination of pregnancy.

Figure 2

Kaplan–Meier survival curve for overall survival of fetuses with single ventricle‐type congenital heart disease with intention to treat.

Figure 3

Kaplan–Meier survival curves for dominant right ventricle vs dominant left ventricle anatomy.

Figure 4

Kaplan–Meier survival curves in patients with extracardiac defects vs without.