Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD.

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD.

Serum concentration of procollagen type III amino-terminal peptide is increased in patients with successfully repaired coarctation of the aorta with left ventricular hypertrophy.

Pathological left ventricular hypertrophy (LVH) with myocardial fibrosis is an independent risk factor for cardiovascular mortality. Previous studies indicated that patients with coarctation of the aorta (CoA) have increased left ventricular mass (LVM) including LVH, even after successful CoA repair. It is unclear whether the increased LVM is pathological one with cardiac fibrosis.