Review of the Myelodysplastic Syndrome as a Cause of Group 5 Pulmonary Arterial Hypertension: An Orphan Disease in an Orphan Pulmonary Hypertension Group.

The coexistence of MDS and pulmonary hypertension (PH) is not a common finding and often goes unnoticed because symptoms such as dyspnea can be confused with the underlying pathology. The annual incidence of idiopathic pulmonary arterial hypertension (PAH) is only around 0.2 cases per 100,000 inhabitants, while MDS is 1 to 8 cases per 100,000 inhabitants. This review summarizes the clinical manifestations, functional respiratory tests, hemodynamic parameters using right heart catheterization, and imaging findings using echocardiography and tomography of pulmonary hypertension in myelodysplastic syndrome. We centered our discussion on the diagnosis of these patients within the hematologic disorders, especially in patients with the detriment of the functional class, as we were not used to looking for this diagnosis as a first choice. Several specialties dealing with patients with hematologic disorders (internists, hematologists, family physicians, geriatrics, oncologists) will find helpful the contents of this review.