Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome: Postnatal Outcomes of the First 100 Patients | Journal Scan

Study Questions:

What are the outcomes of patients achieving biventricular circulation after fetal aortic valvuloplasty for severe aortic stenosis?

Methods:

A retrospective review was performed at a single center. Postnatal outcomes and survival for patients with biventricular circulation were assessed and compared with those managed as hypoplastic left heart syndrome (HLHS).

Results:

A total of 100 patients underwent attempted balloon valvuloplasty over a period of approximately 12 years. The procedure was technically successful in 77 cases. Eighty-eight infants were live-born. Thirty-one infants were managed as biventricular circulation from birth, and an additional seven infants were eventually converted from univentricular circulation to biventricular circulation. After a median follow-up of 5.4 years, freedom from cardiac death among all biventricular repair patients was 96 ± 4% at 5 years and 84 ± 12% at 10 years. There was no cardiac mortality in patients managed with biventricular circulation from birth. All but one of the patients with biventricular circulation required postnatal intervention, including 90% with balloon dilatation of the aortic valve. Forty-two percent of patients required aortic or mitral valve replacement. On most recent echocardiogram, 80% of patients had normal ejection fraction.

Conclusions:

The authors concluded that short- and intermediate-term survival for patients achieving biventricular circulation after fetal aortic valvuloplasty is encouraging, but that continued follow-up is necessary because of morbidity and ongoing need for interventions.

Perspective:

This study shows favorable results for patients achieving biventricular circulation after fetal aortic valvuloplasty. Fetal intervention is a resource-intensive and technically demanding proposition, regarding coordination of multiple disciplines. Patient selection is of critical importance, as well as identification of the optimal postnatal management strategy. A significant proportion (over half of the initial group) did not achieve a biventricular circulation. Of additional concern is the 11% fetal loss rate after the procedure, in the setting of the extremely low rate in utero demise for fetuses with HLHS. Finally, fetal intervention is an option only for a small minority of patients, specifically those with aortic stenosis and left ventricular dilatation. It is possible that earlier fetal diagnosis may allow for intervention in a larger proportion of patients. Although there are insufficient data to support routine fetal intervention for evolving HLHS, these results are promising and continue to lay the groundwork for future efforts.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Valvular Heart Disease, Congenital Heart Disease, CHD & Pediatrics and Imaging, Echocardiography/Ultrasound

Keywords: Balloon Valvuloplasty, Catheterization, Dilatation, Echocardiography, Fetus, Patient Selection, Hypoplastic Left Heart Syndrome, Mitral Valve, Aortic Valve Stenosis


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