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.

Pregnancy and early post-natal outcomes of fetuses with functionally univentricular heart in a low-and-middle-income country

“…5,6,8,9 The overall low utilisation of cardiac care in the live-born babies in this study is different from reports from the developed world where the majority receive specialised cardiac care. 21,22 This is despite the fact that all palliative surgeries for univentricular heart were being performed in our centre from the inception of the study. A recent report had highlighted the feasibility of single ventricle palliations in the setting of low-and-middle-income countries with encouraging early outcomes.…”

“…7 We observed that intention-to-treat and cardiac procedures were more common in patients with systemic left ventricle, which has been shown to be associated with better long-term Fontan outcomes. 21,22 The medical costs of the staged single ventricle palliation pathway will consume about 5 times the average per capita annual income of a family in Kerala. 31 In addition, the annual clinic visits, evaluation and medications will exceed an average monthly income.…”

“…[18][19][20] Recent studies from developed countries reported longitudinal follow-up until Fontan operation after prenatal diagnosis of univentricular heart and identified risk factors for adverse perinatal outcomes in these patients. 21,22 However, despite the availability of guidelines for mid-trimester anomaly (18-20 weeks) scans in India, evaluation of the fetal heart is seldom performed in mass screening programs. 23 The Medical termination of pregnancy act of India was introduced in 1971 before the era of routine fetal ultrasound and it provides guidelines for termination of pregnancy in India until the gestational age limit of 20 weeks.…”

Maternal Outcomes of Ongoing Pregnancies Complicated by Fetal Life-Limiting Conditions

“…[19][20][21] The HLHS diagnosis constituted close to a third of the cases included in our analysis. Even though at least 67% of prenatally diagnosed HLHS survive transplant-free to at least 6 months beyond the Fontan operation, which is the last surgical step in the single ventricle pathway approach to postnatal HLHS management, 22 in our cohort we included women with diagnosis of fetal HLHS who chose to pursue palliative care at birth. Therefore, in this analysis, they were considered as having a fetal life-limiting condition.…”

Regenerative therapies in young hearts with structural or congenital heart disease

“…BMCs have been studied extensively and are known to be safe in several scenarios of bone marrow reconstitution in ischemic HF of the adult. The unique possibility to yield high numbers of CDC in patients operated for CHD in the neonatal period and myocardial repopulation during a planned surgery at an early phase of myocardial dysfunction in infancy might be preventive for the known deterioration process in univentricular hearts (34,35).…”