Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach.
BACKGROUND
The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels.


RESULTS
Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications.


CONCLUSIONS
Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.
Abella
Raul F
RF
Division of Cardiovascular Surgery, Hospital San Donato, San Donato Milanese, Italy. r_abella@hotmail.com
De La Torre
Teresa
T
Mastropietro
Gerardo
G
Morici
Nuccia
N
Cipriani
Adriano
A
Marcelletti
Carlo
C
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery*; D000714:Anastomosis, Surgical; D000792:Angiography; D002315:Cardiopulmonary Bypass / Q000379:methods*; D002675:Child, Preschool; D003097:Collateral Circulation; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery; D006334:Heart Function Tests; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D018633:Pulmonary Atresia / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods*