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Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: Effect of sildenafil, beraprost and bosentan
Author(s) -
Kodama Yoshihiko,
Tao Katsuo,
Ishida Fumihiko,
Kawakami Tadashi,
Tsuchiya Keiji,
Ishida Kazuo,
Takemura Tamiko,
Nakazawa Atsuko,
Matsuoka Kentaro,
Yoda Hitoshi
Publication year - 2012
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.2012.03712.x
Subject(s) - medicine , bronchopulmonary dysplasia , sildenafil , bosentan , inhalation , apgar score , anesthesia , pulmonary hypertension , ductus arteriosus , nitric oxide , pulmonary atresia , surgery , cardiology , gastroenterology , gestational age , ventricle , pregnancy , endothelin receptor , genetics , receptor , biology
The case is described herein of a patient with alveolar capillary dysplasia with double‐outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237th day of life as a result of worsening PH associated with infection.