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CLINICAL RESEARCH: CONGENITAL HEART DISEASE: EDITORIAL COMMENT

Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome

Mio Taketazu, MD^*, Catherine Barrea, MD^*, Jeffrey F. Smallhorn, MD^*, Gregory J. Wilson, MD and Lisa K. Hornberger, MD^*,*

^* Fetal Cardiac Program, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Cardiovascular Research, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

Manuscript received May 21, 2003; revised manuscript received August 22, 2003, accepted January 12, 2004.

^* Reprint requests and correspondence: Dr. Lisa K. Hornberger, Fetal Cardiovascular Program, Pediatric Echocardiography Laboratory, UCSF Medical Center, 505 Parnassus Avenue, San Francisco, California, USA 94143-0214.
lhornberger{at}pedcard.ucsf.edu

OBJECTIVES: We sought to determine whether direct foramen ovale (FO) assessment or pulmonary venous (PV) flow patterns in fetal hypoplastic left heart syndrome (HLHS) correlate with clinical markers of postnatal left atrial (LA) hypertension severity associated with restrictive FO.

BACKGROUND: Restrictive FO places a newborn with HLHS at high risk of mortality and morbidity.

METHODS: We reviewed the prenatal and postnatal echocardiograms and outcomes of 45 fetuses with variants of HLHS diagnosed since May 1999 to determine whether direct FO assessment or PV flow patterns correlate with clinical LA hypertension after birth.

RESULTS: Direct FO assessment in utero showed a poor correlation with postnatal FO size, PaO₂, base excess, and the need for atrial septoplasty (p > 0.05). In 40 fetuses with available PV spectra, three PV flow patterns were observed: 1) continuous forward flow with a small a-wave reversal (velocity time integral [VTI] for reverse/forward flow [VTIR/VTIF ratio <0.18]); 2) continuous forward flow with increased a-wave reversal (VTIR/VTIF ratio 0.18); and 3) brief to-and-fro flow. Among 19 live-borns, the postnatal FO diameter was smaller in patients with type B than in those with type A flow (1.6 ± 1.6 mm and 4.5 ± 2.1 mm, respectively; p = 0.0015), and all patients with type C flow had an intact atrial septum. All three patients with type C flow were critically ill at birth, requiring emergent atrial septoplasty, and two died after heart transplantation, whereas patients with type A or B flow were clinically stable, with only one postoperative death.

CONCLUSIONS: Prenatal PV flow patterns in HLHS identify the fetus at risk of severe LA hypertension at birth.

Abbreviations and Acronyms   FO = foramen ovale   HLHS = hypoplastic left heart syndrome   LA = left atrial/atrium   PA = pulmonary artery   PV = pulmonary vein/venous   VTI = velocity-time integral

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