Short and long-term outcomes after balloon pulmonary valvuloplasty in congenital pulmonary stenosis

Balloon pulmonary valvuloplasty [BPV] has emerged as the treatment of choice for patients with valvular pulmonary stenosis [PS]. We report here our short and long-term outcomes of BPV in 64 patients with isolated native PS. From February 1996 to February 2006, sixty-four patients with PS [pressure gradients ?40 mm Hg] were enrolled in this retrospective study. The hemodynamic data at catheterization revealed that the RV - PA pressure gradient before BPV ranged from 40 to 240 mmHg [mean +/- SD=93.2 +/- 43.4 mmHg]. The above gradient immediately after BPV ranged from 5 to 163 mmHg [mean +/- SD=30.3 +/- 27.7 mmHg], and the difference was significant [p=0.0037]. Twenty-three patients had regular follow-up. The duration of follow- up ranged from 1-120 months with a mean of 38.5 +/- 31.3 months. The transvalvar pressure gradient during the above period ranged from 10 to 140 mmHg with a mean of 35.9 +/- 27.9 mmHg and showed a significant difference [p=0.0032] with the pressure gradients before BPV. BPV provides short and mid-term relief of pulmonary valve obstruction in the majority of patients

Supravalvular and valvular aortic stenosis in heterozygous familial hypercholesterolemia

To report calcified aortic stenosis due to hyper-cholesterolemia in two siblings. Case Presentation and Intervention: A 13-year-old boy with a history of dyspnea on exertion and a systolic murmur of aortic stenosis was referred to our center. Echocardiography showed combined valvular and supravalvular aortic stenoses with a good left ventricle systolic function and severe left ventricular hypertrophy. Two years later his 17-year-old sister was referred to the clinic with similar symptoms. Severe valvular aortic stenosis was detected by echocardiography. Selective coronary angiography showed significant involvement. The father had a history of hypercholesterolemia and confirmed coronary artery disease involving 3 vessels. Angiography showed anterioapical and inferiobasal hypokinesis with preserved left ventricle systolic function. The mother and the other two siblings did not have hyperlipidemia, thereby indicating heterozygous familial hypercholesterolemia in the two affected siblings. The siblings were managed with atrovastatin and nicotinic acid and cholestyramine was added stepwise. The father was treated with lovostatin. This report shows that severe hyperlipidemia in very young patients may be a risk factor for valvulopathy