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

• Published in: Circulation (New York, N.Y.) N.Y.), 2014-08, Vol.130 (8), p.638-645
• Main Authors: Freud, Lindsay R, McElhinney, Doff B, Marshall, Audrey C, Marx, Gerald R, Friedman, Kevin G, del Nido, Pedro J, Emani, Sitaram M, Lafranchi, Terra, Silva, Virginia, Wilkins-Haug, Louise E, Benson, Carol B, Lock, James E, Tworetzky, Wayne
• Format: Article
• Language: English
• Subjects: Aortic Valve Stenosis - diagnostic imaging; Aortic Valve Stenosis - mortality; Aortic Valve Stenosis - surgery; Balloon Valvuloplasty - methods; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Coronary Circulation; Disease Progression; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Female; Fetal Diseases - diagnostic imaging; Fetal Diseases - mortality; Fetal Diseases - surgery; Follow-Up Studies; Gestational Age; Heart; Humans; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - prevention & control; Infant, Newborn; Kaplan-Meier Estimate; Medical sciences; Pregnancy; Pregnancy Outcome; Ultrasonography, Prenatal - methods
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.114.009032

BACKGROUND—Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported. METHODS AND RESULTS—We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P