title: [Operative outcome in children with total anomalous pulmonary venous connection].
abstract: The authors present their experience in surgical treatment of children with total anomalous pulmonary venous connection. A retrospective analysis was carried out in 42 patients operated on in the years 1979-1999. The group included 30 boys aged 7 days to 7 years (mean 170 +/- 480 days) and 12 girls aged 14 days to 7 months (mean 86 +/- 80 days). Diagnostic studies allowed to determine that 24 patients (57%) had supracardiac type (type I), nine (21%)--cardiac (type II), five (12%)--infracardiac (type III) and four (10%)--mixed (type IV). The diagnosis was based on clinical examinations and echocardiography, supplemented in 17 patients (40%) by hemodynamic studies and angiocardiography. Preoperative pulmonary hypertension was noted in 34 children (81%), including 15 patients (36%) diagnosed by hemodynamic studies and 19 children (45%) by echocardiography. All children were operated on in deep hypothermia with circulatory arrest. In the early postoperative period (30 days), seven children (17%) died, while four more (9.5%) died in the latter period. The former were characterized by significantly lower birth weight values (p = 0.023). The total mortality rate was higher in children with type II and III anomalies. No statistically significant association was found between preoperative pulmonary hypertension and early mortality. Thirty-one children (74%) remain in late follow-up. All of them are in NYHA (New York Heart Association) class I and develop normally. Total anomalous pulmonary venous connection is a rare congenital anomaly that can be fully repaired with improving early and late results.
authors.affiliation: Klinika Kardiochirurgii Dzieciecej Katedry Chirurgii Pediatrycznej, Uniwersytetu Jagiellońskiego w Krakowie. mimalec@cyf-kr.edu.pl
authors.forename: E
authors.initials: E
authors.lastname: Malec
authors.forename: J
authors.initials: J
authors.lastname: Kołcz
authors.forename: K
authors.initials: K
authors.lastname: Januszewska
authors.forename: J
authors.initials: J
authors.lastname: Pajak
authors.forename: T
authors.initials: T
authors.lastname: Mroczek
mesh_terms: D000792:Angiography; D006348:Cardiac Surgical Procedures; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D007036:Hypothermia, Induced; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate