Palliative surgical treatment of congenital heart defects associated with unilateral absence of the pulmonary artery.

OBJECTIVES: Experience with the palliative treatment of congenital heart defects (CHDs) associated with unilateral absence of the pulmonary artery (UAPA) is limited. There is a description of 32 interventions in the available literature. The aim of this retrospective study was to present our experience with palliative interventions in patients with cyanotic CHDs associated with UAPA and to suggest a rational surgical strategy. METHODS: Twenty-nine patients were subjected to palliative interventions. All of them had the following cyanotic CHDs with agenesis of the left pulmonary artery: tetralogy of Fallot (n = 26) or double outlet right ventricle (n = 3). Twenty patients were subjected to a single and 9 to multiple staged palliative operations. Patients were grouped according to the type of initial palliation to assess the clinical and haemodynamic results of each surgical intervention: Group 1: systemic-to-pulmonary shunts (n = 13); Group 2: transluminal balloon pulmonary valvuloplasty (n = 5); Group 3: palliative reconstruction of the right ventricular outflow tract (n = 11). The median age of patients at the initial palliative intervention was 2.6 years. Twenty-three of 27 discharged patients were followed up for a median period of 3 years. RESULTS: Hospital mortality in our series reached 7% (2 of 29 patients). Both lethal outcomes occurred after palliative reconstruction of the right ventricular outflow tract was performed as a sole intervention. The assessment of angiographic parameters has shown that palliative reconstruction of the right ventricular outflow tract provided more significant and uniform enlargement of the pulmonary artery than systemic-to-pulmonary shunts or transluminal balloon pulmonary valvuloplasty. Fifty-nine percent of patients (17 of 29 patients) were subjected to complete repair of CHDs during the follow-up. CONCLUSIONS: Palliative surgical treatment of CHDs associated with UAPA can be performed with a relatively low risk. Systemic-to-pulmonary artery shunt and transluminal balloon pulmonary valvuloplasty are methods of choice in patients with non-severe hypoplasia of the single pulmonary artery. The intravascular intervention is indicated more in patients with a prevailing valvular component of the pulmonary stenosis. Palliative reconstruction of the right ventricular outflow tract is a more favourable procedure for patients with a severe hypoplasia of the single pulmonary artery.

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.