Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery

Tetralogy of Fallot with unilateral absence of the pulmonary artery (UAPA) is a rare congenital heart disease. The aim of the present study was to examine the surgical strategy for Tetralogy of Fallot with UAPA by summarizing our experience of its treatment. We retrospectively evaluated 17 patients admitted to our hospital for treatment between 2006 and 2017. All patients were diagnosed with absence of the left pulmonary artery. The Nakata Index (NI), NI Z score, and McGoon ratio of the existing pulmonary artery were calculated. All patients were divided into one-stage complete repair (group A) or palliative procedure (group B) groups according to these criteria and surgical treatments. There were nine treated patients in group A, with a mean NI of 595.6 ± 690.32 mm2/m2 (169.3–2433 mm2/m2) and a mean NI Z score of − 1.57 ± 3.02 (− 4.60 to 5.27). There were eight treated patients in group B, with a mean NI of 107.61 ± 49.49 mm2/m2 (53.15–216.39 mm2/m2) and a mean NI Z score of − 6.27 ± 1.56 (− 8.22 to − 3.54). The mean follow-up time in group A was 5.58 ± 3.42 years (1–11.7 years), while that in group B was 5.4 ± 3.42 years (0.6–12.1 years). No hospital deaths occurred and the follow-up results were good in both groups. In conclusion, development of a single existing pulmonary artery can be evaluated using the NI and the NI Z score. These evaluations can be used to select complete repair or palliative procedures for patients and achieve good results.