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  • Title: [Natural history of atrial septal defect with pulmonary hypertension: follow-up of medically treated patients over 16 years of age].
    Author: Hayakawa H, Momma K, Takao A.
    Journal: J Cardiol; 1994; 24(2):107-13. PubMed ID: 8164141.
    Abstract:
    The clinical course and prognosis of 20 consecutive patients aged over 16 years with atrial septal defect and pulmonary hypertension were analyzed. All patients, six men and 14 women, underwent cardiac catheterization at an average age of 43 years and received medical treatment. All patients with the ostium secundum type had a systolic pulmonary arterial pressure of 87 +/- 18 mmHg (mean +/- SD) and a pulmonary arteriolar resistance of 17 +/- 11 U.m2. At a mean follow-up period of 8 years, nine patients had died of cardiac causes at a mean age of 51 years, 10 were alive, and one was lost to follow-up. Five out of 13 patients with a pulmonary arteriolar resistance of 14 U.m2 or more had died of progression of the pulmonary vascular obstructive disease. Two of the seven patients with a pulmonary arteriolar resistance less than 13 U.m2 had died of pulmonary vascular obstructive disease and another two died of congestive heart failure. Although 25% of the patients had become symptomatic before 20 years of age, the diagnosis of atrial septal defect was not made until a mean age of 39 years. Eighteen patients became more severely symptomatic, but the time until the development of the symptoms varied. The mean age of patients in the New York Heart Association class I, II, III-IV were 27, 38, and 48 years of age, respectively. The percent survivals at 50 and 60 years of age were 77% and 54%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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