In patients with cardiac amyloidosis, comorbid pulmonary hypertension was associated with significantly higher all-cause mortality compared to those without (29.5% vs 18.9%; HR 1.70; P<0.001).
Cohort (n=20,134)
Yes
Does comorbid pulmonary hypertension worsen cardiovascular and survival outcomes in adult patients with cardiac amyloidosis?
In patients with cardiac amyloidosis, comorbid pulmonary hypertension is independently associated with significantly increased risks of mortality, right ventricular failure, and major adverse cardiovascular events.
Effect estimate: HR 1.70 (95% CI 1.61-1.80)
Absolute Event Rate: 29.5% vs 18.9%
p-value: p=<0.001
Abstract Rationale Pulmonary hypertension (PH) frequently complicates cardiac amyloidosis, reflecting progressive pulmonary vascular and right ventricular remodeling. Despite its clinical relevance, large-scale evidence quantifying the prognostic impact of PH in this population remains limited. Methods We conducted a retrospective, multicenter cohort analysis using the TriNetX U.S. Collaborative Network, encompassing de-identified electronic health records from 109 healthcare organizations. Adult patients with cardiac amyloidosis were stratified by the presence or absence of comorbid PH. Propensity-score matching (1:1) was performed to balance demographics, comorbidities, and cardiovascular medication use, resulting in 10,067 patients per cohort. Outcomes within five years of diagnosis included right ventricular (RV) failure, major adverse cardiovascular events (MACE), and all-cause mortality. Measures of association and survival analyses were generated using TriNetX analytic tools. Results After matching, the mean age was 77.7 ± 11 years, 39% were female, and 62% were White. The presence of PH was associated with markedly higher rates of RV failure (67.9% vs 39.0%; odds ratio OR 3.32, 95% confidence interval CI 3.13-3.52; hazard ratio HR 2.70, 95% CI 2.59-2.81; p 0.001), MACE (39.0% vs 31.8%; OR 1.37, 95% CI 1.30-1.46; HR 1.29, 95% CI 1.23-1.35; p 0.001), and all-cause mortality (29.5% vs 18.9%; OR 1.80, 95% CI 1.68-1.92; HR 1.70, 95% CI 1.61-1.80; p 0.001). Median survival was significantly shorter among patients with PH (1677 days vs not reached; log-rank p 0.001), with five-year survival probabilities of 47.7% versus 63.8%, respectively. Conclusions In this large, real-world cohort of cardiac amyloidosis patients, pulmonary hypertension was independently associated with excess mortality, right ventricular failure, and major cardiovascular events, even after rigorous matching. These findings highlight PH as a key prognostic determinant in cardiac amyloidosis and emphasize the need for early recognition and hemodynamic optimization in this high-risk population. This abstract is funded by: None
Campoli et al. (Fri,) conducted a cohort in Cardiac amyloidosis (n=20,134). Pulmonary hypertension vs. No pulmonary hypertension was evaluated on All-cause mortality (HR 1.70, 95% CI 1.61-1.80, p=<0.001). In patients with cardiac amyloidosis, comorbid pulmonary hypertension was associated with significantly higher all-cause mortality compared to those without (29.5% vs 18.9%; HR 1.70; P<0.001).