Large Cohort of Cardiac Amyloid Patients with Defined Hemodynamics Suggests That Pulmonary Hypertension Is Common

Analysis of subtypes of pulmonary hypertension, and potential association with specific biomarkers and survival, suggests further studies are required.

In many cardiomyopathies, the presence of pulmonary hypertension foreshadows a poor prognosis; there is a dearth of data on the impact of pulmonary hypertension in cases of cardiac amyloid specifically. Longinow and colleagues at the Cleveland Clinic looked at the prevalence of pulmonary hypertension in patients with cardiac amyloidosis, as well as the subtypes of pulmonary hypertension, and analyzed the potential association with specific biomarkers and survival. Hemodynamics were ascribed to these specific categories: no pulmonary hypertension; precapillary pulmonary hypertension (pulmonary capillary wedge pressure [PCWP] <15, pulmonary vascular resistance [PVR] >3); isolated postcapillary (Ipc) pulmonary hypertension (PCWP >15, PVR <3); and combined post- and precapillary (CpC) pulmonary hypertension (PCWP >15, PVR >3).

The investigators conducted a retrospective analysis of patients included in the Cleveland Clinic Heart Failure Database from 2000 to 2019. These patients had undergone right heart catheterization. Pulmonary hypertension was defined as outlined at the 6th World Symposium, with a mean pulmonary artery pressure of >20 mm Hg being the cutoff for pulmonary hypertension. Baseline demographic data were analyzed and stratified by pulmonary hypertension subgroup. Among the 132 patients with cardiac amyloid, there were similarities in baseline demographics, laboratory values, and hemodynamics between the light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) patient cohorts. These included age (64 and 72 years of age), and measured NT-proBNP, which was 4211 ± 5394 compared with 8509 ± 12,466 in the AL and ATTR groups, respectively.

In total, 102 patients had some form of pulmonary hypertension. Of these patients, 32 were found to have combined precapillary and postcapillary pulmonary hypertension; 3 were found to have precapillary pulmonary hypertension (but no postcapillary pulmonary hypertension); and 67 were found to have isolated postcapillary pulmonary hypertension. Interestingly, between the 4 pulmonary hypertension subgroups, differences in cardiac index, creatine, and biomarkers such as troponin-T and proBNP were not found to be significant. Mortality was not statistically different.

This study, which represents a large cohort of cardiac amyloid patients with defined hemodynamics, suggests that pulmonary hypertension is common. However, there was no difference in survival detected during the follow-up period between Ipc and CpC; therefore, pulmonary hypertension itself may be a poor prognostic indicator of survival in patients with cardiac amyloidosis. While one cohort was small, to date, this is one of the largest studies of its kind. These findings are unusual compared with other cardiomyopathies such as hypertrophic cardiomyopathy and heart failure with preserved ejection fraction. To corroborate these findings, further research is warranted.

Source: Longinow J, Bhattacharya S, Buggey J, et al. Prevalence and significance of pulmonary hypertension in patients with cardiac amyloidosis. Presented at: American College of Cardiology 70th Annual Scientific Session, May 15-17, 2021.

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