Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis

Slivnick, Jeremy; Zaręba, Karolina M.; Varghese, Juliet; Truong, Vien T.; Wallner, Alexander; Tong, Matthew; Hummel, Christopher; Mazur, Wojciech; Rajpal, Saurabh

doi:10.1136/openhrt-2021-001808

Cited by 9 publications

(8 citation statements)

References 34 publications

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“…However, it is difficult to differentiate between these two pathophysiological mechanisms. 21 Additionally, secondary hepatic lesions occur from monoclonal light chain or associated fragment deposits and this may best be reflected by ALP levels. 22 This concurs with recent Chinese studies that found total bilirubin was independently associated with mortality.…”

Section: Discussionmentioning

confidence: 99%

“…Also, altered hepatic function in amyloidosis is multifactorial and complex but is principally caused by cardiac failure and hepatic amyloid infiltration. However, it is difficult to differentiate between these two pathophysiological mechanisms 21 . Additionally, secondary hepatic lesions occur from monoclonal light chain or associated fragment deposits and this may best be reflected by ALP levels 22 …”

Section: Discussionmentioning

confidence: 99%

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Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Zaroui,

Kharoubi,

Gounot

et al. 2024

ESC Heart Failure

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AimsPredicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage.Methods and resultsThis prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision‐making regarding therapy. Over the 12‐year study period, among the 233 patients included, 133 were NYHA III‐IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT‐proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut‐off values for Stage 1: hsTnT ≤ 107 ng/L and NT‐proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT‐proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT‐proBNP then CB 4 μmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 μmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2–24]. At 1 year, 102 (44%) patients died and the Kaplan–Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11–18]) and stage 3, 6.6 months (95% CI [1–13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2.ConclusionsThe Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Zaroui,

Kharoubi,

Gounot

et al. 2024

ESC Heart Failure

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“…Only a limited amount of studies evaluated the relation between hemodynamics and clinical outcome in CA, showing that RAP was independently associated with clinical outcome. 6,7 No studies have previously analyzed if the classic hemodynamic cutoffs, heavily embedded in clinical practice, convey risk information. We specifically looked at the prognostic impact of CI <2.2 L/min per m² and PCWP >18 mm Hg, a cutoff that was validated in 1976 in patients with an acute myocardial infarction in the seminal paper of Forrester and Diamond and colleagues.…”

Section: Discussionmentioning

confidence: 99%

“…Limited studies have reported on the cardiac hemodynamic profiles in CA and the limited data show conflicting results as to whether ATTR-CA and AL-CA are affected by a same degree of hemodynamic alteration. [4][5][6][7] There is a need to understand the prognostic value of hemodynamic variables and pulmonary hypertension (PH) in CA.…”

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confidence: 99%

Hemodynamic Profiling and Prognosis in Cardiac Amyloidosis

Martens

Bhattacharya

Longinow

et al. 2023

Circ: Heart Failure

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BACKGROUND: Little information is available on the prognostic relevance of cardiac hemodynamic cutoffs in cardiac amyloidosis (CA) and its subtypes. METHODS: Consecutive patients diagnose with light chain-CA or transthyretin CA undergoing right heart catheterization were analyzed. Prognostic relevance of classic hemodynamic cutoffs of cardiac index (CI <2.2 L/min per m 2 ), pulmonary capillary wedge pressure (>18 mm Hg), right atrial pressure (>8 mm Hg), and mean pulmonary artery pressure (≥25 mm Hg or pulmonary hypertension) with the combined end point of cardiac transplant/left ventricular assist device and death and heart failure admissions separately was assessed. RESULTS: A total of 469 CA patients underwent right heart catheterization (light chain CA=42% and transthyretin CA=52%) of whom 69%, 64%, and 79% had elevated right atrial pressure, pulmonary capillary wedge pressure, and pulmonary hypertension, respectively. The classic hemodynamic cutoffs for right atrial pressure (hazard ratio, 1.26 [0.98–1.62]) and mean pulmonary artery pressure (hazard ratio, 1.28 [0.96–1.71]) did not identify patients at higher risk for adverse outcome; however, cutoffs of 14 mm Hg for right atrial pressure (hazard ratio, 1.59 [1.26–2.00]) and 35 mm Hg for mean pulmonary artery pressure (hazard ratio, 1.30 [1.01–1.66]) performed better to detect worse outcome ( P <0.05 for both). Reduced CI occurred in 55% of patients and was the strongest variable associated with the risk for cardiac transplant/left ventricular assist device and death, heart failure admissions, and reduced functional capacity. Reduced CI independently predicted risk on top of the Mayo-score in light chain CA and National Amyloid Center score in transthyretin CA ( P <0.05 for both). Patients with light chain CA had higher pulmonary capillary wedge pressure and lower stroke volume index but maintained CI through a higher heart rate. CONCLUSIONS: Hemodynamic variables are grossly abnormal in CA, but elevated filling pressures are prognostic at significantly higher threshold values than classic cutoff values. CI is the hemodynamic variable most strongly associated with outcome and functionality in CA.

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“…The cardinal symptom is exertional dyspnoea 5,6 . Isolated post‐capillary PH and combined pre‐ and post‐capillary PH are prevalent in these patients 7,8 . and associated with adverse outcomes 9–11 .…”

Section: Introductionmentioning

confidence: 99%

Invasive haemodynamics at rest and exercise in cardiac amyloidosis

Holt,

Flø,

Ravnestad

et al. 2023

ESC Heart Failure

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AimsOur aim was to investigate haemodynamics at rest and during exercise in patients with transthyretin cardiomyopathy (ATTR‐CM) in light of the 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines on pulmonary hypertension (PH).Methods and resultsWe performed right heart catheterization (RHC) in 57 subjects with ATTR‐CM. The proportion of patients with PH was 77% according to the 2022 guidelines versus 47% when applying the 2015 guidelines. Isolated post‐capillary PH and combined pre‐ and post‐capillary PH were most prevalent. Thirty‐six patients underwent a supine bicycle cardiopulmonary exercise test during RHC. Exercise‐induced PH was defined as an increase in mean pulmonary arterial pressure from rest to exercise per increase in cardiac output (ΔmPAP/ΔCO) of > 3 mmHg/L/min. An increase in pulmonary arterial wedge pressure per change in cardiac output (ΔPAWP/ΔCO) from rest to exercise >2 mmHg/L/min was considered suggestive of post‐capillary exercise‐induced PH. All but two patients who exercised during RHC developed exercise‐induced PH. The median ΔmPAP/ΔCO was 7.2 mmHg/L/min and ΔPAWP/ΔCO was 5.1 mmHg/L/min. The median ΔRAP/ΔCO was 3.6 mmHg/L/min and ΔRAP/ΔPAWP was 0.6 mmHg/L/min.ConclusionsMost patients with ATTR‐CM have isolated post‐capillary or combined pre‐ and post‐capillary PH at rest, and almost all patients develop exercise‐induced PH with a large post‐capillary component. There was a pronounced, but balanced increase in atrial pressures on exercise.

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Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis

Cited by 9 publications

References 34 publications

Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Prognostic mortality factors in advanced light chain cardiac amyloidosis: A prospective cohort study

Hemodynamic Profiling and Prognosis in Cardiac Amyloidosis

Invasive haemodynamics at rest and exercise in cardiac amyloidosis

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