Early outcomes of total pulmonary arterial reconstruction in patients with arterial tortuosity syndrome.

BACKGROUND: Arterial Tortuosity Syndrome (ATS) is an autosomal recessive connective tissue disease that can present with complex pulmonary arterial stenosis, causing right ventricular (RV) hypertension and dysfunction. In this study, we review our experience and early outcomes with single stage surgical repair.
METHODS: From 2008 to 2010, 7 patients with ATS and severe bilateral pulmonary artery stenosis underwent surgical repair that involved extensive reconstruction of the pulmonary arterial tree bilaterally, including the central, lobar and segmental branches. The clinical records of these patients were reviewed for preoperative data, repair techniques and early clinical outcomes, including reinterventions and recent echocardiographic studies.
RESULTS: All 7 patients had bilateral peripheral and central pulmonary artery stenosis with systemic to supra-systemic RV pressure and severe RV dysfunction. Median age at the time of operation was 72 months (range=7 to 120 months). All patients had low RV pressure (≤40% systemic) after the procedure with significant improvement in RV function to normal before hospital discharge. The mean right ventricular/left ventricular pressure ratio decreased from 1.27±0.19 to 0.31±0.06 postoperatively (p<0.001). There was no mortality. All patients continued regular follow-up at our institution for a mean follow-up period of 17.6±9.1 months. All patients were alive and asymptomatic with normal RV function and no evidence of elevated RV pressure on most recent echocardiography.
CONCLUSIONS: Complete surgical reconstruction of the pulmonary arterial tree in advanced cases of ATS is a successful intervention with excellent early clinical and hemodynamic outcomes.