Chronic thromboembolic pulmonary hypertension complicated with homocystinuria.
A 17-year-old boy with homocystinuria was found to have a systolic murmur during a routine examination. Echocardiography demonstrated pulmonary hypertension (PH), and computer tomography angiography showed pulmonary thrombi. Although 12-month anticoagulation treatment reduced the thrombotic material within the main branch, it failed to clear thrombotic materials in the left and right lobar branches. Two years later, the patient was admitted to our hospital due to a worsening of PH. Treatment with bosentan, sildenafil and beraprost, in addition to anti-coagulant therapy, did not improve his PH. Balloon pulmonary angioplasty (BPA) was performed to remove the pulmonary thrombi. BPA markedly improved the patient's hemodynamics and exercise capacity. Close follow-up is scheduled to prevent any potential future thrombotic complications.
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Japan.
Shinpei
S
Ogawa
Tetsuji
T
Katayama
Koichi
K
Kaikita
Masayo
M
Tsukamoto
Eiichiro
E
Yamamoto
Megumi
M
Yamamuro
Tomoko
T
Tanaka
Kenichi
K
Tsujita
Sunao
S
Kojima
Shinji
S
Tayama
Seiji
S
Hokimoto
Hiroshige
H
Yamabe
Yasuhiro
Y
Indo
Fumio
F
Endo
Hiromi
H
Matsubara
Hisao
H
Ogawa
D000293:Adolescent; D000800:Angioplasty, Balloon / Q000379:methods; D002908:Chronic Disease; D004452:Echocardiography; D006439:Hemodynamics; D006712:Homocystinuria / Q000453:epidemiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology*; D008297:Male; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011655:Pulmonary Embolism / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery*
D058986:Phosphodiesterase 5 Inhibitors; D010975:Platelet Aggregation Inhibitors