ABSTRACT
Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/ hemodynamic criteria that are a consequence of several etiologies. Confirmation of pulmonary hypertension is based on right heart catheterization. Pulmonary hypertension is a devastating condition that can lead to considerable morbidity and premature mortality. In the last few decades, significant advancement in the pharmacotherapy of pulmonary hypertension has resulted from better understanding of the complex pathogenesis and pathophysiology of this dreaded disease. Despite these accomplishments, pharmacotherapy of pulmonary hypertension is still far from perfect, and the mortality in this modern treatment era is still unacceptably high. We report a complex clinical presentation characterized by severe pulmonary hypertension secondary to concomitant mitral stenosis with veno-occlusive disease in the context of systemic sclerosis. Our case highlights the importance of a systematic and comprehensive diagnostic approach to avoid missing an underlying pathology.