A lady presents with complaints of dysnea. Pulse rate is 70/min while BP=110/70 mm Hg. On examination there is an early-diastolic murmur. CXR is given here. Diagnosis of the patient is?

The striking feature in this chest X-ray is the remarkably prominent main pulmonary aery segment (MPA), which appears to be dilated. This X-ray also shows a prominent right atrial contour, indicating right atrial dilatation as a consequence of pulmonary hypeension and right ventricular hyperophy. In view of prominent pulmonary aery segment with early diastolic murmur the clinical diagnosis is pulmonary aery hypeension causing a mild pulmonary regurgitation. The female gender is suggestive of a connective tissue disorder that can lead to pulmonary aery hypeension.