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scientific articles

Repair of Cardiac Defect in Patient with Ehlers-Danlos Syndrome and Deficiency of Hageman Factor

key information

source: British Medical Journal

year: 1961

authors: Fantl P, Morris KN, Sawers RJ


A boy aged 11 years had been regarded as normal until the age of 8 years, when a heart murmur was detected by the school medical officer and he was referred to the cardiothoracic unit of the Alfred Hospital. He had the clinical features of an atrial septal defect with left axis deviation in the electrocardiogram, suggesting the diagnosis of partial persistent atrio-ventricular canal. Two and a half years later his poor muscular development was noted, and he had developed symptoms of exertional dyspnoea. Cardiac catheterization and femoral-artery puncture performed without incident indicated a left-to-right shunt at both the atrial and ventricular levels, and the pulmonary blood flow to be four times greater than the systemic flow. The original diagnosis was retained, and he was admitted to hospital for repair of the defect three years after the first examination.

organization: Baker Medical Research Institute, Alfred Hospital

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