Significance of Pulmonary Hypertension in Cardiac Amyloidosis

Pulmonary hypertension (PH) portends a poor prognosis in chronic heart failure and within distinct cardiomyopathies. There is a paucity of data on the impact of PH in patients with light-chain (AL) and transthyretin (ATTR) cardiac amyloidosis (CA). We sought to define the prevalence and significance of PH and PH subtypes in CA. We retrospectively identified patients with a diagnosis of CA who underwent right-sided cardiac catheterization (RHC) from January 2000 to December 2019. PH was defined as mean pulmonary artery pressure >20 mm Hg. PH was phenotyped as precapillary PH (PC-PH; pulmonary capillary wedge pressure [PCWP] <15, pulmonary vascular resistance [PVR] ≥3), isolated postcapillary PH (IpC-PH; PCWP >15, PVR <3), and combined postcapillary and precapillary PH (CpC-PH; PCWP >15 and PVR ≥3). Survival was assessed in those with CA and PH and for PH phenotypes. A total of 132 patients were included, 69 with AL CA and 63 with ATTR CA. A total of 75% (N = 99) had PH (76% of patients with AL and 73% of patients with ATTR, p = 0.615) and the predominant PH phenotype was IpC-PH. The degree of PH was comparable between ATTR CA and AL CA, and PH was observed in advanced stage disease (National Amyloid Center or Mayo stage II or greater). The overall survival for patients with CA and PH was similar to to those without PH. Higher mean pulmonary artery pressure independently predicted mortality in CA with PH (odds ratio 1.06, confidence interval 1.01 to 1.12, p = 0.03). In conclusion, PH was seen frequently in CA and tended to be IpC-PH; however, its presence did not significantly impact survival.