Hemodynamics of Pulmonary Hypertension in the Setting of Hereditary Hemorrhagic Telangiectasia

To determine the hemodynamic profile of patients with hereditary hemorrhagic telangiectasia (HHT) referred for right heart catheterization and estimate the incidence of pulmonary hypertension (PH) and/or high output states in this setting. Single center cohort study of all patients with HHT and suspected PH who underwent right heart catheterization between 1/1/1995- 9/1/2013. Data reported as mean ± standard deviation. High output state was defined as cardiac index > 4.2 L/min/m2. Of 62 patients who underwent right heart catheterization for suspected HHT and PH, 38 patients (61 %) met Curacao criteria for HHT. The mean age at time of PH diagnosis was 56 ± 16 years. Baseline NT-BNP was 558 ± 1855 pg/ml and baseline hemoglobin was 11.6 ± 2.4 g/dL. At the time of right heart catheterization, 13 patients (34%) were World Health Organization functional class III/IV while 14 patients (37%) had class II symptoms. Pulmonary arteriovenous malformations, hepatic arteriovenous malformations, and gastrointestinal malformations were present in 17 (45 %), 29 (76 %), and 3 (8 %) respectively. Of those 38 patients with criteria-confirmed HHT, 24 (63%) had a pulmonary artery pressure > 25 mmHg and a cardiac index < 4.2 L/min/m2. The mean cardiac index for these patients was 3.25 ± 1.02 L/min/m2. Five (13%) had PAP >25 mmHg and cardiac index > 4.2 L/min/m2. The mean cardiac index for these patients was 4.65 ± 0.28 L/min/m2. Four (11%) of the 38 patients did not meet criteria for PH, but did have high output states with an index of > 4.2 L/min/m2. The mean cardiac index for these patients was 6.08 ± 0.78 L/min/m2. Five patients (13%) had neither PH nor high output states. Mean pulmonary vascular resistance for patients with both PH and HHT was 4.54 ± 4.19 Wood Units. In patients with hereditary hemorrhagic telangiectasia the coexistence of PH is common with the majority having hemodynamics compatible with pulmonary arterial hypertension rather than high-output mediated PH.