Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome : Patient selection for fetal intervention

• Published in: Circulation (New York, N.Y.) Vol. 113; no. 11; pp. 1401 - 1405
• Main Authors: MÄKIKALLIO, Kaarin, MCELHINNEY, Doff B, LEVINE, Jami C, MARX, Gerald R, COLAN, Steven D, MARSHALL, Audrey C, LOCK, James E, MARCUS, Edward N, TWORETZKY, Wayne
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 21-03-2006
• Subjects: Abortion, Therapeutic - statistics & numerical data; Adolescent; Adult; Aortic Valve Stenosis - complications; Aortic Valve Stenosis - diagnostic imaging; Aortic Valve Stenosis - embryology; Aortic Valve Stenosis - therapy; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Catheterization - methods; Catheterization - statistics & numerical data; Decision Making; Diseases of the aorta; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Female; Fetal Death; Fetal Heart - diagnostic imaging; Fetal Heart - pathology; Fetal Therapies - methods; Fetal Therapies - statistics & numerical data; Gestational Age; Hemodynamics; Humans; Hypoplastic Left Heart Syndrome - embryology; Hypoplastic Left Heart Syndrome - etiology; Hypoplastic Left Heart Syndrome - prevention & control; Hypoplastic Left Heart Syndrome - surgery; Infant, Newborn; Medical sciences; Patient Selection; Pregnancy; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Treatment Outcome; Ultrasonography, Prenatal; Human; Aortic stenosis; hypoplastic left heart syndrome; echocardiography; fetal monitoring; Cardiovascular disease; Left Heart Hypoplasia; Congenital disease; valvuloplasty; Cardiac valvular disease; Evolution; aorticvalve stenosis; Aortic valve; Doppler
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.105.588194

Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS.
Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS.
In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.