Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery.
BACKGROUND
Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital heart defect that still represents a surgical challenge. The purpose of this study is to summarize our experience of surgical treatment of this complex lesion.


METHODS
From 1983 to 2003, 27 patients with tetralogy of Fallot and unilateral absence of the left (n = 25) or right (n = 2) pulmonary artery underwent different surgical interventions. The age of patients ranged from 40 days to 37 years (median, 5.3 years). Pulmonary arterial Nakata index and Nakata index Z-score were used for the quantitative assessment of the contralateral pulmonary artery. Twenty patients underwent various palliative procedures, namely Blalock-Taussig or Gore-Tex shunt, transluminal balloon pulmonary valvuloplasty, and reconstruction of right ventricular outflow tract without ventricular septal defect closure. At a median interval of 3.6 years after palliation, 13 patients underwent complete repair of tetralogy of Fallot. In the other 7 patients, complete repair was performed as a primary intervention.


RESULTS
Hospital mortality after palliation and after a complete repair was the same and reached 5%. Sixteen patients with the Nakata index greater than 200 mm2/m2 and Z-score greater than -4 survived after a complete repair. One of 4 patients with Nakata index less than 200 mm2/m2 and Z-score less than -4 died after surgery.


CONCLUSIONS
Majority of patients with tetralogy of Fallot and unilateral absence of the pulmonary artery require palliative intervention as a first step of surgical treatment. Nakata index greater than 200 mm2/m2 and Nakata index Z-score greater than -4 are criteria for a successful complete repair.
Bockeria
Leo A
LA
Bakoulev Scientific Center for Cardiovascular Surgery, Moscow, Russia.
Podzolkov
Vladimir P
VP
Makhachev
Osman A
OA
Zelenikin
Mikhail A
MA
Alekian
Bagrat G
BG
Ilyin
Vladimir N
VN
Gadjiev
Ali A
AA
Shatalov
Konstantin V
KV
Kakuchaya
Teya T
TT
Khiriev
Titalav Kh
TKh
Zaets
Sergey B
SB
D000293:Adolescent; D000328:Adult; D013504:Cardiovascular Surgical Procedures / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000503:physiopathology; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care; D011651:Pulmonary Artery / Q000002:abnormalities*; D012720:Severity of Illness Index; D016019:Survival Analysis; D013771:Tetralogy of Fallot / Q000601:surgery*