RESUMEN
BACKGROUND: Group 3 pulmonary hypertension (PH) is a common complication in patients with lung diseases but there are currently no FDA-approved therapies. The data is conflicting, but a few small studies suggest potential benefits in using Group 1 PH therapies in these patients, particularly in severe PH with right ventricular (RV) dysfunction. METHODS: A retrospective cohort study of patients with severe Group 3 PH with RV dysfunction who received parenteral prostanoids from 2007-2018 at our institution was undertaken. Severe PH was defined as mean pulmonary arterial pressure (mPAP) ≥35 mmHg or mPAP 25-34 with cardiac index (CI) <2.4 L/min/m2. Routine prognostic studies including N-terminal prohormone of brain natriuretic peptide (NT-proBNP), 6-minute walk distance (6MWD), WHO Functional Class assessment, oxygen requirement, arterial oxygen saturation, right ventricular systolic pressure (RVSP) and right heart catheterization (RHC) pressures, were obtained before initiation of parenteral therapy and at first clinical follow-up. RESULTS: Nine patients were included. Five were female (55.6%) with a median [interquartile range (IQR)] of 69 [54-71] years. Median CI was 1.8 (1.6-2.4) L/min/m2 and median pulmonary vascular resistance (PVR) was 14.7 (10.7-17.1) Wood units (WU). We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class. Resting oxygen requirement worsened from 4 to 6 L/min (P=0.04) and exertional oxygen saturation nadir worsened from 90% to 83% (P=0.01) despite the increase in FiO2 with exertion. Overall results were heterogenous: several patients demonstrated clinical stabilization, with two undergoing lung transplantation and one showing long-term stability with medical therapy. Symptoms remained severe for most: three patients discontinued prostanoid therapy, choosing to pursue hospice care. CONCLUSIONS: We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class, while oxygen requirement at rest and oxygen saturation during exertion significantly worsened. Our results suggest that parenteral prostanoids should not generally be considered in the treatment of Group 3 PH patients.
RESUMEN
An 86-year-old woman presented with marked blistering of her left index fingertip and ulceration of the left middle fingertip, with a 2-year history of recurrent blistering and ulceration of her fingers bilaterally. She denied any preceding finger trauma, although she reported frequent gardening. She denied systemic symptoms. Her medical history was significant for a 2-year history of atrial fibrillation on carvedilol, amiodarone and apixaban, and she was a lifetime non-smoker. On admission, she had elevated inflammatory markers but unremarkable autoantibodies. Radiograph of the hand revealed diffuse soft tissue fullness and subtle irregularities at the tuft of the index finger, but all other investigations were unremarkable. The lesion was incised and drained, revealing blood-tinged purulent fluid. Wound biopsy revealed spongiosis with neutrophils, consistent with a diagnosis of blistering distal dactylitis.