diagnosis of exclusion: idiopathic pulmonary arterial hypertension presenting as intractable shortness of breath - a case report and review of the subject

A 35 year old house wife was evaluated for gradual onset and progressively worsening dyspnea. She had normal vitals, but raised JVP, loud P2, normal vesicular breathing in chest and normal systemic examination. Her chest radiograph showed cardiomegaly and echocardiography was consistent with severe pulmonary hypertension of non cardiac origin. Extensive investigations to find any cardiovascular, respiratory or systemic disorders were negative ruling out an underlying cause of occult pulmonary hypertension and she was finally diagnosed as having idiopathic pulmonary hypertension [IPAH]. She responded well to treatment with pulmonary vasodilator drugs and was advised regular follow ups in pulmonary out patients department