Published Online
on March 13, 2006

A more recent version of this article appeared on March 21, 2006

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Submitted on September 14, 2005
Revised on January 3, 2006
Accepted on January 13, 2006

Fetal Aortic Valve Stenosis and the Evolution of Hypoplastic Left Heart Syndrome. Patient Selection for Fetal Intervention

Kaarin Mäkikallio MD, PhD, Doff B. McElhinney MD, Jami C. Levine MD, Gerald R. Marx MD, Steven D. Colan MD, Audrey C. Marshall MD, James E. Lock MD, Edward N. Marcus MSc, and Wayne Tworetzky MD^*

From the Department of Cardiology (K.M., D.B.M., J.C.L., G.R.M., S.D.C., A.C.M., J.E.L., E.N.M., W.T.), Children’s Hospital, and Harvard Medical School, Boston, Mass, and the Department of Obstetrics and Gynecology (K.M.), University of Oulu, Oulu, Finland.

^* To whom correspondence should be addressed. E-mail: wayne.tworetzky{at}cardio.chboston.org.

Background--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.

Methods and Results--Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at 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.

Conclusions--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.

Key words: aorticvalve stenosis • echocardiography, Doppler • fetal monitoring • hypoplastic left heart syndrome • valvuloplasty

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