Journal of Rare Cardiovascular Diseases

Aims: The aim of this study was to analyze the incidence of long-term complications observed during follow-up of patients who had undergone the Ross procedure in childhood. Methods: The study engaged a cohort of 9 patients, all of whom were between 19 to 32 years old. Patients had been in the care of the Centre for Rare Cardiovascular Diseases since the age of 18. Clinical and echocardiographic data were collected from the follow-up period. Results: 78% of patients had preserved global systolic function of the left ventricle, and 56% had dilatation of the ascending aorta. Due to the aneurysm of the ascending aorta one patient required the Bentall de Bono procedure. Another patient underwent a reoperation because of endocarditis of the pulmonary homograft with severe aortic and pulmonary regurgitation. 1/3 of the studied patients were being considered for a reoperation. 23% of patients developed severe pulmonary valve regurgitation, 33% moderate. 67% of patients developed mild to moderate pulmonary valve stenosis. Most patients were in NYHA class I-II. Conclusion: Late complications are frequent in this group of patients, and hence they require surveillance in specialized Centers for Grown-up Congenital Heart Diseases. JRCD 2016; 3 (1): 9–13

rare disease; Ross procedure; aortic valve replacement; pulmonary valve replacement; echocardiography

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