title: [Long-surviving patient with isolated absent pulmonary valve syndrome: a case report].
abstract: A 79-year-old woman was admitted for exertional dyspnea in September 2001. She had begun to experience unusual fatigue from the age of 40 years. Cardiac examination revealed a single S2, Levine grade II/VI presystolic murmur in the 5th left sternal border, and a right parasternal impulse. Echocardiography showed dilated right chambers and absence of pulmonary valve leaflets. Doppler echocardiography at the pulmonary annulus revealed a 'to and fro' pattern. Cardiac catheterization indicated the same diastolic pressures in the pulmonary artery and right ventricle. The diagnosis was absent pulmonary valve syndrome. Administration of a diuretic agent resulted in almost immediate improvement of symptoms. Absent pulmonary valve syndrome, generally associated with tetralogy of Fallot, often causes severe respiratory failure or right heart failure during infancy. A case of such long survival without associated cardiac anomalies is very rare.
authors.affiliation: Department of Internal Medicine, Izumi City Hospital, Fuchu-cho 4-10-10, Izumi, Osaka 594-0071. okajimakazue@aol.com
authors.forename: Kazue
authors.initials: K
authors.lastname: Okajima
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Kawase
authors.forename: Takao
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Katsunori
authors.initials: K
authors.lastname: Hato
authors.forename: Masaki
authors.initials: M
authors.lastname: Nishimoto
authors.forename: Junichi
authors.initials: J
authors.lastname: Yoshikawa
mesh_terms: D000368:Aged; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D010701:Phonocardiography; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D017741:Survivors