title: Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management.
abstract: We report a successfully treated case of unilateral absence of a pulmonary artery, associated with an atrial septal defect and chronic lung disease with severe pulmonary hypertension. Because this severe pulmonary hypertension could jeopardize postoperative hemodynamic, the ingenuity of surgical strategy was required. The atrial septal defect was left open as a safety "pop-off" valve, and prosthetic graft was chosen as a reconstructive material to avoid excessive dilatation from exposure to unpredictable postoperative pulmonary hypertension.
authors.affiliation: Department of Cardiovascular Surgery and cardiology, Nagano Children's Hospital, 3100, Toyoshina, Azumino, Nagano 399-8288, Japan. kentaro.umezu@gmail.com
authors.forename: Kentaro
authors.initials: K
authors.lastname: Umezu
authors.forename: Yorikazu
authors.initials: Y
authors.lastname: Harada
authors.forename: Takahiko
authors.initials: T
authors.lastname: Sakamoto
authors.forename: Kiyohiro
authors.initials: K
authors.lastname: Takigiku
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yasukochi
mesh_terms: D000015:Abnormalities, Multiple; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000295:instrumentation; D006328:Cardiac Catheterization; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D055420:Perfusion Imaging; D011474:Prosthesis Design; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D011859:Radiography; D012720:Severity of Illness Index; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*