While randomized trials of pulmonary arterial hypertension (PAH) therapy exclude patients with an elevated pulmonary capillary wedge pressure (PCWP), 25 percent of our referred pulmonary hypertension (PH) population has a PCWP > 15 mmHg. These patients often otherwise meet WHO criteria for PAH. We sought to better characterize these patients.
We reviewed PH patients referred to our center from 2001 to 2005 to determine the characteristics of those patients with an elevated PCWP. We included those with a mPAP > 25 mmHg, and PCWP > 15 mmHg. Those with PH and abnormal LV systolic function, or pulmonary vascular resistance (PVR) < 3 were excluded. Baseline characteristics, diagnosis, disease severity, and therapy were analyzed.
114 patients had normal LV function, mPAP > 25 and PCWP > 15; 29 with PVR < 3 were excluded. The remaining 85 patients had multiple comorbidities; 73.8% had hypertension, 54.1% had LV hypertrophy, and 57.6% were obese. Approximately 1/3 had one or more of the following: atrial fibrillation, coronary disease, chronic obstructive pulmonary disease or diabetes. Mean hemodynamics were as follows: mPAP 52.9 mmHg (29-138), PCWP 21.5 mmHg (16-40), PVR 7.9 Wood Units (WU) (3.1-32.9), and cardiac index (CI) 2.4 L/min/m2 (1.3-4.34). The mPAP was >55 mmHg in 36.4%, right atrial pressure >20 mmHg in 25%, and CI <2.0 L/min/m2 in 20%. Mild PH (PVR 3 to 5 WU)was present in 40%. Specific PAH therapy (e.g., prostacyclins or endothelin antagonist)was safely administered in 39/51 patients with more severe PH. Major diagnoses included PAH, (8 idiopathic, 7 connective tissue disease, 1 portopulmonary, 6 congenital heart) while left-heart / valvular disease, or primary lung or sleep disorders appeared to contribute in 30 and 23 patients respectively. The remainder had chronic thromboembolism, sarcoidosis, sickle cell, and high output disorders.
Patients with PH and an elevated PCWP but with normal LV systolic function have significant comorbidities complicating their management.
Future controlled clinical trials should study specific PAH therapy in this population.
Terry Fortin, None.