Abstract 17140: Impact of Balloon Pulmonary Angioplasty on Chronic Thromboembolic Pulmonary Hypertension Complicated With Homocystinuria
A 17-year-old male with homocystinuria was detected systolic murmur at a regular medical examination. Echocardiography demonstrated pulmonary hypertension (PH) [57/17 mmHg in estimated pulmonary artery pressure(PAP)], and the computer tomography (CT) angiography showed thrombotic materials within the main branch of the right pulmonary artery, the branches of right lower lobe, and branches of left lower and middle lobe. Though 1-year anticoagulation treatment improved thrombotic material within the main branch, the thrombotic materials within the both side of lobar branches were remained. At the age of 19, the patient was admitted to our department because of the aggravation of PH. Though the patient did not complain shortness of breath, we observed the tachypnea in daily life conversation. Physical examination showed jugular vein dilatation, a split S2 sound and a systolic murmur with Levine II/XI on the left sternal border between the second rib. Electrocardiography revealed sinus rhythm with right ventricular hypertrophy. Chest radiograph showed mild enlargement in right ventricle. Echocardiography showed the aggravation of PH (96/24 mmHg in estimated PAP). In the CT angiography, the thrombotic materials within the both side of lobar branches were remained. The ventilation-perfusion scanning demonstrated diffuse perfusion deficits. We diagnosed the patient as chronic thromboembolic PH (CTEPH) complicated with homocystinuria. As the administration of bosentan, sildenafil and beraprost in addition to the anti-coagulant therapy did not improve PH, we selected balloon pulmonary angioplasty (BPA) for mechanical removal of the pulmonary thrombi. Right heart catheterization showed the elevation of PAP (102/39/59 mmHg). BPA improved the mean PAP from 59 to 19 mmHg. At 1 year after BPA, mean PAP was kept less than 25 mmHg. Besides, BPA ameliorated 6 minutes walking test from 360m to 475m. This is the first case describing that CTEPH associated with homocystinuria was effectively treated with BPA. Based on his underlying disease (CTEPH with homocystinuria), we selected BPA that was less invasive and repeatable. In conclusion, BPA markedly improved the hemodynamics and exercise capacity in a case of CTEPH associated with homocystinuria.
- © 2013 by American Heart Association, Inc.