• G B Luciani, W J Wells, A Khong, and V A Starnes.
• Division of Cardiothoracic Surgery, Children's Hospital Los Angeles, Calif, USA.
• J. Thorac. Cardiovasc. Surg. 1997 Mar 1; 113 (3): 443-52.

BackgroundPatients with tetralogy of Fallot/pulmonary atresia often have bilateral pulmonary artery lesions, including diminutive central and peripheral vessels, major aortopulmonary collaterals, and distortion from previous operations. Staged procedures through lateral thoracotomies and median sternotomies have traditionally been used for repair.MethodsBetween October 1993 and December 1995, 10 patients 3 months to 15 years old with complex tetralogy of Fallot/pulmonary atresia underwent repair via a clamshell approach. Nine had undergone a mean of 2.8 +/- 0.8 previous operations (range 1 to 4). Indications for operation were repair of pulmonary artery arborization anomalies in 10 (4 unilateral, 6 bilateral), with unifocalization in 6 (2 unilateral, 4 bilateral).ResultsEight of 10 patients had concomitant complete repair. There were no deaths at a mean follow-up of 17.1 +/- 4.0 months (range 12 to 26). Mean ventilation time was 3.7 +/- 2.1 days (range 1 to 14) and hospital stay 8.7 +/- 4.6 days (range 4 to 19). At follow-up, the peak right ventricular/left ventricular pressure ratio in patients who received complete repair was 0.44 +/- 0.13 (0.30 to 0.67). One patient (10%) required reoperation because of pseudoaneurysm of the main pulmonary artery 14 months after repair, and one had successful stent placement because of recurrent left and right pulmonary artery stenosis 8 months after repair. Two infants who underwent complete unifocalization and central pulmonary artery reconstruction are awaiting completion of repair.ConclusionsThe clamshell approach to complex tetralogy of Fallot/ pulmonary atresia provides simultaneous exposure of bilateral central and peripheral pulmonary artery lesions and intracardiac pathologic conditions. This procedure appears safe and may decrease the number of operations required to complete repair of tetralogy of Fallot/pulmonary atresia in selected patients.

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