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28. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: Unifocalization brings no long-term benefits.
Yves D'udekem, Nelson Alphonso, Martin Agge Nǿrgaard, Andrew Donald Cochrane, Glenda Rolley, Jim L Wilkinson, Christian Brizard; Melbourne, Australia
Objectives:
To evaluate the contribution of unifocalization procedures in the management of patients with pulmonary atresia, ventricular septal defects (VSD), and major aortopulmonary collateral (MAPCAs) dependant lung circulation.
Methods:
From 1975 to 1995, 82 consecutive patients were entered in a multistage approach and had 67 sternotomies and 122 thoracotomies, in order to perform 63 central shunts, 11 RV to PA conduits, 44 peripheral shunts, 130 MAPCA transplantations, and 76 MAPCA ligations. The serial angiographies and the follow-up of these patients were reviewed. Pre- and postoperative variables were analysed by logistic regression and entered in a Cox regression analysis.
Results:
Concurrent follow-up rate was 80 %. The hospital mortality of the preliminary procedures was 9% (7/82). Seven pts died between these procedures and the final repair, 14 are still alive but not suitable for repair, and 53 (65%) had a complete repair. The hospital mortality of the repair was 8 % (4/53), and 13 late deaths occurred after repair, all cardiac related. The overall survival of the patients at 30 years, irrespective of the operations they had was 58 ± 7%. The probability of being in NYHA Class I or II at the age of 30 was 51 ± 8%, and in class I only was 29 ± 6%. Twelve years after complete repair, the survival was 51 ± 14%; the probability to be alive and in class I was 32 ± 10%; the freedom from reoperation for conduit replacement was 62 ± 8%. Cox proportional analysis failed to identify a factor predictive of late mortality.
On angiography, the central shunt promoted growth of the central pulmonary arteries in all cases (29 pts). Three left (10%) and 11 right (38%) pulmonary arteries developed stenosis greater than 50 %. The left pulmonary artery grew from 3 ± 1 to 6 ± 2 mm (p< 0.001) in the 2 years interval following the completion of the shunt. Sixty unifocalized MAPCAs were identified on serial angiographies in 31 pts. After a mean of 3.2 ± 4 years, 26 had thrombosed and 12 presented with a stenosis greater than 50%. Serial measurements of 29 MAPCAs were obtained. Over a mean time interval of 3.5 years during which the weight of the patients increased from 12 ± 9 to 25 ± 21 kgs (p<0.001), the size of the MAPCAs went from 3.6 ± 1 mm to 4 ± 2 mm (p=0.25).
Conclusion:
Long-term survival into adulthood of patients with pulmonary atresia, VSD and MAPCAs has been achieved with a multistage approach. However, late survival depends exclusively on the growth of the native pulmonary circulation. The few unifocalised MAPCAs that did not thrombose failed to grow.
Because unifocalization procedures do not increase the pulmonary vasculature, preparation of patients with pulmonary atresia, VSD and MAPCAs should aim at achieving growth of the native pulmonary arteries by shunting rather than unifocalization.
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