PT  - JOURNAL ARTICLE
AU  - Slivnick, Jeremy
AU  - Zareba, Karolina M
AU  - Varghese, Juliet
AU  - Truong, Vien
AU  - Wallner, Alexander L
AU  - Tong, Matthew S
AU  - Hummel, Christopher
AU  - Mazur, Wojciech
AU  - Rajpal, Saurabh
TI  - Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis
AID  - 10.1136/openhrt-2021-001808
DP  - 2022 Mar 01
TA  - Open Heart
PG  - e001808
VI  - 9
IP  - 1
4099  - http://openheart.bmj.com/content/9/1/e001808.short
4100  - http://openheart.bmj.com/content/9/1/e001808.full
SO  - Open Heart2022 Mar 01; 9
AB  - Objectives While cardiac amyloidosis (CA) classically involves the left ventricle (LV), less is known about its impact on the right ventricle (RV) and pulmonary vasculature. We performed a retrospective analysis to identify the prevalence and types of pulmonary hypertension (PH) profiles in CA and to determine haemodynamic and cardiovascular magnetic resonance (CMR) predictors of major adverse cardiovascular events (MACE).Methods Patients with CA who underwent CMR and right heart catheterisation (RHC) within 1 year between 2010 and 2019 were included. Patients were assigned the following haemodynamic profiles based on RHC: no PH, precapillary PH, isolated postcapillary PH (IPCPH), or combined precapillary and postcapillary PH (CPCPH). The relationship between PH profile and MACE (death, heart failure hospitalisation) was assessed using survival analysis. CMR and RV parameters were correlated with MACE using Cox-regression analysis.Results A total of 52 patients were included (age 69±9 years, 85% men). RHC was performed during biopsy in 44 (85%) and for clinical indications in 8 (15%) patients. Rates of no PH, precapillary PH, IPCPH and CPCPH were 5 (10%), 3 (6%), 29 (55%) and 15 (29%), respectively. Haemodynamic PH profile did not correlate with risk of death (p=0.98) or MACE (p=0.67). Transpulmonary gradient (TPG) (HR 0.88, CI 0.80 to 0.97), RV, (HR 0.95, CI 0.92 to 0.98) and LV ejection fraction (HR 0.95, CI 0.92 to 0.98) were significantly associated with MACE.Conclusions PH is highly prevalent in CA, even at the time of diagnosis. While IPCPH was most common, CPCPH is not infrequent. TPG and RV ejection fraction (RVEF) are prognostic markers in this population.Data are available upon reasonable request. The authors of this manuscript have agreed to make the de-identified data available upon reasonable request.