Severe congestive heart failure in mild pulmonic stenosis due to dysplastic pulmonary valve associated with cardiomyopathy

Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary valve without associated shunt lesions. We describe two patients with mild pulmonic stenosis due to valvular dysplasia associated with cardiomyopathy who developed severe congestive heart failure. Since the small pressure gradients across the pulmonary valve cannot cause this complication, we suggest that it resulted from the associated hypertrophic non-obstructive cardiomyopathy. The presence of cardiomyopathy may alter the clinical presentation and prognosis of patients with dysplastic pulmonary valve. In some cases, like our two cases, the cardiomyopathy rather than the valvular lesion should be considered the main disease.