Severe Congenital Pulmonary Valve Stenosis Diagnosed in Adulthood: A Case Report.

Pulmonary valve stenosis is a congenital heart disease that is usually diagnosed during childhood, while presentation in adulthood is uncommon and may lead to delayed diagnosis. We report the case of a 21-year-old patient with no significant medical history who presented with a six-month history of exertional dyspnea and constrictive chest pain. Electrocardiography showed right axis deviation with signs of right ventricular hypertrophy, and chest radiography revealed dilatation of the left pulmonary artery. Transthoracic echocardiography demonstrated severe valvular pulmonary stenosis with a peak velocity of 4.53 m/s and a maximum systolic gradient of 82.23 mmHg, associated with right ventricular hypertrophy and preserved systolic function, without evidence of pulmonary hypertension. Computed tomography pulmonary angiography confirmed focal post-stenotic dilatation of the left pulmonary artery. After a multidisciplinary discussion, balloon pulmonary valvuloplasty was recommended. This case highlights the importance of considering congenital pulmonary valve stenosis in young adults presenting with exertional symptoms and underscores the challenges of access to advanced interventional therapies in resource-limited settings.