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Ann Thorac Surg 1995;60:1762-1764
© 1995 The Society of Thoracic Surgeons

Original Articles: Cardiovascular

Pulmonary Atresia With Intact Ventricular Septum: Long-Term Results of ``One and a Half Ventricular Repair''

Kagami Miyaji, MD, Munehiro Shimada, MD, Akihiko Sekiguchi, MD, Akira Ishizawa, MD, Takayoshi Isoda, MD, Minoru Tsunemoto, MD

Departments of Cardiovascular Surgery and Pediatric Cardiology, National Children's Hospital, Tokyo, Japan

Accepted for publication August 8, 1995.

Background. Between 1982 and 1984, we successfully performed ``one and a half ventricular repair'' using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results.

Methods. In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years postoperatively.

Results. All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient.

Conclusions. Although the lower limits of the tricuspid valve diameter for ``one and a half ventricular repair'' using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.




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