Presentation
Progressive dyspnea
Patient Data
Prominent pulmonary arterial tree, with relative peripheral pruning.
Cardiomegaly with bulging of the left heart border is consistent with right ventricular enlargement.
Case Discussion
6-minute walk test revealed poor endurance, having to take frequent breaks, and desaturation down to 84% on room air. Ventilation/Perfusion scan (V/Q) excluded pulmonary emboli. Full blood examination, renal and liver function tests and autoimmune screen were all normal. Transthoracic echocardiogram found an enlarged right ventricle with impaired systolic function and estimated pulmonary artery pressure (PAP) of 67mmHg.
Right heart catheterization recorded elevated pulmonary artery pressure of 55mmHg on average and pulmonary vascular resistance of 11.9 Woods units by thermodilution. This confirmed a diagnosis of idiopathic pulmonary hypertension.
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