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  • Title: Surgical correction of tetralogy of Fallot with unilateral absence of pulmonary artery.
    Author: Bockeria LA, Podzolkov VP, Makhachev OA, Zelenikin MA, Alekian BG, Ilyin VN, Gadjiev AA, Shatalov KV, Kakuchaya TT, Khiriev TKh, Zaets SB.
    Journal: Ann Thorac Surg; 2007 Feb; 83(2):613-8. PubMed ID: 17257996.
    Abstract:
    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.
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