Bony Breathlessness: Reversible Pulmonary Hypertension in Melnick-Needles Syndrome Using Noninvasive Ventilation.

In this patient, echocardiography demonstrated a normal left heart, confirmed by low pulmonary capillary wedge pressure at RHC, and both computed tomography pulmonary angiography and V/Q excluded thromboembolic disease, ruling out groups 2 and 4 PH, respectively. Current PH guidelines suggest the treatment for group 3 PH is to optimize underlying lung disease, in this case, with overnight NIV. However, the hemodynamic severity was such that her mortality risk was so high that the PH multidisciplinary team decided to add sildenafil, a type 5 phosphodiesterase-5 inhibitor with antiremodeling effects licensed for the treatment of PAH.[4] If this was considered to be PAH without the lung disease component (rather than group 3 PH) with such severe pulmonary hemodynamics, a patient would be initiated on at least combination oral PAH therapy, and may be considered for systemic prostacyclin therapy. Work by Corral and others[5] found that PH, left ventricular hypertrophy, and left ventricular mass improved in patients with obesity hypoventilation syndrome by using NIV, but not continuous positive airway pressure. [Extracted from the article]