Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair".
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 post-operatively.


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.
Miyaji
K
K
Department of Cardiovascular Surgery, National Children's Hospital, Tokyo, Japan.
Shimada
M
M
Sekiguchi
A
A
Ishizawa
A
A
Isoda
T
T
Tsunemoto
M
M
D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006346:Heart Septum / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010166:Palliative Care; D018633:Pulmonary Atresia / Q000473:pathology / Q000601:surgery*; D014261:Tricuspid Valve / Q000473:pathology