Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients.
OBJECTIVE
This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries.


METHODS
A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach. In Group I (2007-09), the surgeon-preferred procedure (71%-one-stage total repair) was used. In Group II (2010-14), a surgical algorithm based on pulmonary arterial tree morphology was applied.


RESULTS
Median follow-up was 5.04 years and 98% complete. The overall survival rate was 92.59%. Cumulative operative mortality decreased from 10.7% in Group I to 0% in Group II ( P  = 0.036). Successful complete repair was performed in 23 (82%) of 28 patients in Group I (median follow-up, 7.99 years) and in 33 (60%) of 55 patients in Group II (median follow-up, 3.85 years). The difference in total survival was not significant between Groups I and II but was markedly influenced by the presence of the 22q11 deletion. The survival of 23 patients with confirmed 22q11 deletion was 73.91%; the survival of 28 patients with confirmed absence of the 22q11 deletion was 100%; and the survival of 32 patients not tested for the 22q11 deletion was 100% ( P  = 0.0001).


CONCLUSIONS
Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.
Department of Cardiac Surgery, Ukrainian Children's Cardiac Center, Kyiv, Ukraine.
Oleksandr D
OD
Babliak
Department of Cardiac Surgery, Ukrainian Children's Cardiac Center, Kyiv, Ukraine.
Yaroslav B
YB
Mykychak
Department of Interventional Cardiology, Ukrainian Children's Cardiac Center, Kyiv, Ukraine.
Oleksandra O
OO
Motrechko
Department of Cardiac Surgery, Ukrainian Children's Cardiac Center, Kyiv, Ukraine.
Illya M
IM
Yemets
D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D011652:Pulmonary Circulation / Q000502:physiology; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D014455:Ukraine / Q000453:epidemiology; D054079:Vascular Malformations / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods*
Major aortopulmonary collateral arteries; Pulmonary atresia and ventricular septal defect; Surgical algorithm; Unifocalization