Abstract
The early development of pulmonary vascular disease in cardiac patients with Down's syndrome has been suspected but few hemodynamic studies have been reported. We have studied by cardiac catheterization 47 patients aged seven weeks to 22 years with a variety of cardiac defects including 22 with some form of an A-V cushion defect (AVC), 17 with a ventricular septal defect (VSD), four with Tetralogy of Fallot, three with isolated patent ductus arteriosus (PDA) and one with total anomalous pulmonary venous return to the coronary sinus. Pulmonary artery hypertension (PAH) was present in 41 of the 47 patients. The pulmonary vascular resistance (PVR) was at least moderately elevated in 23 patients and normal or slightly raised in 18.
Of interest was the demonstration of a PDA in 11 patients. Systemic PAH was found in one of the three with an isolated PDA while all eight patients, including six under age six years with a VSD (4 of 17) or AVC (4 of 21), had severe PAH with a mean PA pressure ranging from 60-84 mm Hg. and an elevated pulmonary vascular resistance. This unexpected high incidence of associated PDA in patients with Down's syndrome may be one explanation for the frequent and early development of pulmonary vascular disease. Significant pulmonary disease was a problem in seven patients but was not clearly related to a high PVR.
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Noonan, J., Walters, L. HEMODYNAMIC STUDIES IN DOWN'S SYNDROME PATIENTS WITH CONGENITAL HEART DISEASE. Pediatr Res 8, 353 (1974). https://doi.org/10.1203/00006450-197404000-00077
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DOI: https://doi.org/10.1203/00006450-197404000-00077