Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart disease.
BACKGROUND
When total anomalous pulmonary venous connection is associated with other complex cardiac malformations, early and late postsurgical morbidity and mortality are excessive.


METHODS
In an attempt to modify this outcome, twelve children (4 days to 6.8 years of age) with total anomalous pulmonary venous connection and various congenital cardiac defects were treated with orthotopic heart transplantation. Associated cardiac diagnoses included the following: hypoplastic left heart syndrome (n = 2), unbalanced atrioventricular canal with pulmonary atresia (n = 2), and single ventricle with severe pulmonary stenosis (n = 3) or atresia (n = 5). Two patients had situs inversus, and two had dextrocardia with situs ambiguous. Eight patients had asplenia and one had polysplenia. Palliative pretransplantation procedures in five patients included the following: systemic to pulmonary artery shunt (n = 5), atrioventricular valve annuloplasty (n = 1) and classical Glenn shunt (n = 1). The donor left atrium was anastomosed directly to a common pulmonary venous pool in nine patients; whereas three children required complex reconstruction to baffle the pulmonary venous flow to the donor left atrium.


RESULTS
There was one operative death related to an oversized heart and vena caval thrombosis. Follow-up ranged from 16 months to 4.5 years (average 3 years). In two patients (18%) pulmonary venous obstruction developed 3 and 4 months after transplantation. Reoperation to relieve the obstruction was successful in one patient. The second patient underwent three such reoperations and died of sepsis 10 months after orthotopic heart transplantation.


CONCLUSIONS
Orthotopic transplantation is a viable option for children with complex total anomalous pulmonary venous connection that precludes a biventricular repair. Transplantation may improve the dismal prognosis of those children, but it does not eliminate the potential for late pulmonary venous obstruction.
Razzouk
A J
AJ
Loma Linda University Children's Hospital, Calif., USA.
Gundry
S R
SR
Chinnock
R E
RE
Larsen
R L
RL
Ruiz
C
C
Zuppan
C W
CW
Bailey
L L
LL
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000379:methods* / Q000401:mortality / Q000502:physiology; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D011183:Postoperative Complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D011668:Pulmonary Veno-Occlusive Disease / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate; D016896:Treatment Outcome