[Prostacyclin oral internal therapy for severe pulmonary hypertension after total correction of cor triatriatum with partial anomalous pulmonary venous return; report of a case].
A 37-day-old girl having cor triatriatum with partial anomalous pulmonary venous return was found on emergent admission. Echocardiography and angiography revealed severe pulmonary hypertension with anomalous pulmonary venous return to inominate vein. She still was in respiratory failure after emergent surgical repair because of severe pulmonary hypertension following pulmonary venous obstruction. Prostacyclin is the first Food and Drug Administration (FDA)-approved treatment for advanced primary pulmonary hypertension, which has antithrombotic properties related to its effect on platelets and is a potent vasodilator of both the systemic and pulmonary arteries, and has positive inotropic properties. We started prostacyclin oral internal therapy for her and she recovered from respiratory problems very quickly. Prostacyclin might be effective for pulmonary hypertension before and after surgical repair as primary pulmonary hypertension.
Department of Cardiovascular Surgery, National Nagasaki Medical Center, Omura, Japan.
M
M
Hamawaki
T
T
Tomino
H
H
Sato
M
M
Imura
K
K
Suzuki
K
K
Nakashima
Y
Y
Okamoto
D000284:Administration, Oral; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D003310:Cor Triatriatum / Q000150:complications / Q000601:surgery*; D011464:Epoprostenol / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D007223:Infant; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage*; D011183:Postoperative Complications; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery*; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*
D000959:Antihypertensive Agents; D010975:Platelet Aggregation Inhibitors; D014665:Vasodilator Agents; D011464:Epoprostenol