MITRAL AND AORTIC REGURGITATION IN 84 PATIENTS WITH MUCOPOLYSACCHARIDOSES

In echocardiographic and necropsy studies nodular thickening of the mi tral valve and, less frequently, of the aortic valve has been found in 60%-90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. I n this study 84 unselected patients with different enzymatically prove n MPS and 84 age and sex matched, healthy persons were studied prospec tively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the va lve into the left atrium or the left ventricular outflow tract, respec tively, with peak velocities exceeding 2.5 m/s. Of the 84 patients wit h satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our pat ients. None of the control persons showed mitral or aortic regurgitati on. Conclusion Aortic and mitral regurgitation are more frequent in pa tients with MPS than previously thought and that therefore these patie nts should have regular colour Doppler flow mapping and antibiotic pro phylaxis when required.