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The Journal of Thoracic and Cardiovascular Surgery, Vol 105, 112-119, Copyright © 1993 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
AJ Muster, VR Zales, MN Ilbawi, CL Backer, CE Duffy and C Mavroudis
The right ventricle in patients with severe outflow obstruction or atresia
and a small tricuspid valve often remains too hypoplastic even after
optimal palliation to tolerate biventricular repair with closure of the
atrial septal defect. In these patients, nonpulsatile cavopulmonary (Glenn)
anastomosis has traditionally facilitated biventricular repair. In 1989,
Billingsley and associates reported the addition of a bidirectional
cavopulmonary anastomosis to the definitive biventricular repair in
patients with hypoplastic right ventricle, pulmonary atresia, and intact
ventricular septum. The atrial septal defect was left open with an
adjustable snare for later closure. We report five patients with
hypoplastic right ventricle (mean diastolic volume 48.4%, mean stroke
volume 40.2% of predicted value) who had the atrial septal defect closed at
the time of the biventricular repair. Four patients, who had the
bidirectional cavopulmonary anastomosis supplementing the biventricular
repair, had no evidence of excessive right atrial or superior vena cava
hypertension postoperatively. One patient, who had atypical tetralogy of
Fallot with tricuspid stenosis, developed recurrent pericardial tamponade
and marked hepatomegaly following conventional tetralogy repair with
closure of the atrial septal defect. These complications were controlled
with the addition of bidirectional cavopulmonary anastomosis 2 months
later. Postoperative hemodynamic or Doppler studies in these patients
revealed pulsatile flow in the entire pulmonary artery system, including
the artery distal to the Glenn anastomosis. This modification of
biventricular repair allows primary closure of the atrial septal defect and
provides pulsatile arterial flow in the entire pulmonary artery, even when
the right ventricle is significantly hypoplastic.
ARTICLES
Biventricular repair of hypoplastic right ventricle assisted by pulsatile bidirectional cavopulmonary anastomosis
Division of Cardiology and Cardiovascular Surgery, Children's Memorial Hospital, Chicago, IL 60614.
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