PURPOSE: PAH is usually managed in university-based centers. We describe an experience of an Out Patient PAH Center set in a large multi-disciplinary private practice over a 20 month period.
METHODS: A registry of 52 suspected PAH patients was maintained, 35 female, 17 male, mean age 65. The management team included a Nurse Practitioner and 2 Pulmonary Physicians.
RESULTS: 54% of patients presented with dyspnea, NYHA class III and IV (67%), fatigue (11%), cough (8%). A total of 29% had previously been diagnosed with PAH, 23% were obese (BMI > 30), 17% had OSA. The predominant etiology was idiopathic followed by left heart disease, CTD, ILD, thromboembolic, and multifactorial (31%, 25%, 21%, 8%, 6%, 35%). Workup included right heart catheterization 25% (mPAP of 40mm Hg), echocardiogram 73% (estimated SPAP mean 58mm Hg), 6MWT (46%), PSG (17%). Patients were treated with Bosentan, Sildenifil, Iloprost, Epoprostenol, CCB, combination drug therapy (21%, 8%, 6%, 6%, 17%, 44%) and CPAP (17%). Mortality: 4 patients died (8%), 2 ILD, 1 CTD, 1 Multi. 5 patients improved NYHA Class (10%). The majority remained stable on and off treatment.
CONCLUSION: Most patients remain stable over a short follow-up period especially when PAH is related to left heart causes. Short-term mortality of PAH of all etiologies is low. Many patients with clinically significant PAH are older than previously reported.
CLINICAL IMPLICATIONS: Management of pulmonary hypertension is feasible in an outpatient private practice setting. Obesity and Sleep Apnea need to be considered as significant co-morbid illnesses. Management protocols in PAH may need to be modified to deal with a geriatric population.
DISCLOSURE: Susan Smith, No Financial Disclosure Information; No Product/Research Disclosure Information