Severe pulmonary arterial hypertension in a patient with small ventricular septal defect

Description

A boy aged 16 years presented to our hospital with a history of dyspnoea on exertion and haemoptysis. His cardiovascular examination revealed features of severe pulmonary artery hypertension (PAH). He had no cyanosis. His transthoracic echocardiogram showed dilated right atrium and ventricle with the interventricular septum bulging to the left side. There was a small perimembraneous ventricular septal defect (VSD) with right-to-left shunt (figure 1 and video 1). The left atrium and ventricle were of normal size. Continuous wave Doppler evaluation showed right-to-left shunting with a peak gradient of 33 mm Hg, thus indicating suprasystemic PAH. Doppler evaluation of the tricuspid regurgitation (TR) jet showed a gradient of 120 mm Hg (figure 2). The pulmonary artery (PA) was hugely dilated with spontaneous echocontrast, but no thrombus (figure 2 and video 2). In spite of extensive evaluation, we could not find a...

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