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Case Reports

A Symptomatic Neonate With Tetralogy of Fallot, an Absent Pulmonary Valve, and a Single Coronary Artery With a Major Fistula

^a Department of Cardiovascular Surgery, Kanagawa Children's Medical Center, Yokohama, Japan
^b Department of Pediatric Cardiology, Kanagawa Children's Medical Center, Yokohama, Japan

Accepted for publication April 2, 2009.

^_* Address correspondence to Dr Kajihara, 2-138-4 Mutsukawa, Minami-ku, Yokohama, 232-8555, Japan (Email: n-c.kaji{at}f6.dion.ne.jp).

We present a case with a rare combination of tetralogy of Fallot with an absent pulmonary valve, and a single coronary artery with a major fistula to the main pulmonary artery. Myocardial ischemia developed in response to increasing shunt flow through the coronary fistula, resulting in heart failure. We ligated the coronary fistula and plicated the anterior wall of the dilated pulmonary arteries during the neonatal period. Complete repair through a transatrial-transpulmonary approach was performed at the age of 17 months. The postoperative course was excellent and the patient maintained a stable hemodynamic and respiratory state with no evidence of myocardial ischemia.