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(Circulation. 2004;110:253-258.)
© 2004 American Heart Association, Inc.

Original Articles

Creation of an Atrial Septal Defect In Utero for Fetuses With Hypoplastic Left Heart Syndrome and Intact or Highly Restrictive Atrial Septum

Audrey C. Marshall, MD; Mary E. van der Velde, MD; Wayne Tworetzky, MD; Carlen A. Gomez, MD; Louise Wilkins-Haug, MD, PhD; Carol B. Benson, MD; Russell W. Jennings, MD; James E. Lock, MD

From the Departments of Cardiology, Children’s Hospital, and Pediatrics, Harvard Medical School, Boston, Mass (A.C.M., W.T., J.E.L.); Division of Pediatric Cardiology, C.S. Mott Children’s Hospital, Ann Arbor, Mich (M.E.v.d.V., C.A.G.); Departments of Obstetrics and Gynecology (L.W.-H.) and Radiology (C.B.B.), Brigham and Women’s Hospital and Harvard Medical School, Boston, Mass; and Department of Surgery, Children’s Hospital and Harvard Medical School, Boston, Mass (R.W.J.).

Correspondence to Audrey C. Marshall, MD, Department of Cardiology, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail Audrey.marshall{at}cardio.chboston.org

Received September 30, 2003; de novo received December 16, 2003; revision received March 25, 2004; accepted March 26, 2004.

Background— Infants born with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum face a neonatal mortality of at least 48% despite early postnatal left atrial decompression and palliative surgery. Prenatal left atrial decompression has been suggested as a means of improving these outcomes. This study reports the feasibility of fetal catheterization to create an interatrial communication and describes technical considerations.

Methods and Results— Seven fetuses at 26 to 34 weeks’ gestation with hypoplastic left heart syndrome and intact or highly restrictive atrial septum underwent attempted prenatal intervention. Under ultrasound guidance, the atrial septum was approached with a needle introduced percutaneously from the maternal abdominal surface. In 6 of 7 fetuses, the atrial septum was successfully perforated, with balloon dilation of this iatrogenic defect resulting in a small but persistent interatrial communication. There were no maternal complications. One fetus died after the procedure. The remaining fetuses were liveborn at term, although 4 died as neonates.

Conclusions— Ultrasound-guided fetal atrial septoplasty consisting of septal puncture and balloon dilation is feasible and can be performed percutaneously to minimize maternal risk. Although we have not demonstrated any positive clinical impact to date, it is our hope that further technical evolution will ultimately enable prenatal left atrial decompression and improvement of outcomes in fetuses with hypoplastic left heart syndrome and intact atrial septum.

Key Words: catheterization • heart defects, congenital • pregnancy

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