Related Experiment Video
Updated: Jan 6, 2026
Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Incremental Utility of Right Ventricular Dysfunction in Patients With Myeloproliferative Neoplasm-Associated
Jiwon Kim1, Spencer Krichevsky2, Lola Xie3
1Greenberg Cardiology Division, Department of Medicine, Weill Cornell Medical College, New York, New York.
Background:
Myeloproliferative neoplasm (MPN) has been associated with pulmonary hypertension (PH) on the basis of small observational studies, but the mechanism and clinical significance of PH in MPN are not well established. The aims of this study were to expand understanding of PH in a well-characterized MPN cohort via study of PH-related symptoms, mortality risk, and cardiac remodeling sequalae of PH using quantitative echocardiographic methods.
Methods:
The population comprised a retrospective cohort of patients with MPN who underwent transthoracic echocardiography: Doppler-derived pulmonary arterial systolic pressure applied established cutoffs for PH (≥35 mm Hg) and advanced PH (≥50 mm Hg); right ventricular (RV) performance was assessed via conventional indices (tricuspid annular plane systolic excursion [TAPSE], S') and global longitudinal strain. Symptoms and mortality were discerned via standardized review.
Results:
Three hundred one patients were studied; 56% had echocardiography-demonstrated PH (20% advanced) paralleling a high prevalence (67%) among patients with invasively quantified PASP. PH was associated with adverse left ventricular (LV) remodeling indices, including increased myocardial mass and diastolic dysfunction (P ≤ .001 for all): LV mass and filling pressure (P < .01) were associated with PH independent of LV ejection fraction. RV dysfunction by strain and TAPSE and S' increased in relation to PH (P ≤ .001) and was about threefold greater among patients with advanced PH compared with those without PH. Patients with RV dysfunction were more likely to report dyspnea, as were those with advanced PH (P < .05). During median follow-up of 2.2 years, all-cause mortality was 27%. PH grade (hazard ratio, 1.9; 95% CI, 1.1-3.0; P = .012) and TAPSE- and S'-demonstrated RV dysfunction (hazard ratio, 3.3; 95% CI, 1.3-8.2; P = .01) were independently associated with mortality; substitution of global longitudinal strain for TAPSE and S' yielded similar associations of RV dysfunction with death (hazard ratio, 3.2; 95% CI, 1.5-6.7; P = .003) independent of PH.
Conclusions:
PH is highly prevalent in patients with MPN and is linked to LV diastolic dysfunction; echocardiography-quantified RV dysfunction augments risk for mortality independent of PH.
Insights
Pulmonary hypertension (PH) is common in myeloproliferative neoplasm (MPN) and linked to left ventricular diastolic dysfunction. Echocardiography-assessed right ventricular dysfunction in MPN patients independently predicts mortality.
Area of Science:
- Cardiology
- Hematology
- Pulmonology
Background:
- Pulmonary hypertension (PH) is observed in myeloproliferative neoplasms (MPN), but mechanisms and clinical significance require further study.
- Previous research on PH in MPN is limited to small observational studies.
- Understanding PH in MPN is crucial for patient management and risk stratification.
Purpose of the Study:
- To investigate the prevalence and clinical implications of PH in a well-characterized MPN cohort.
- To assess PH-related symptoms, mortality risk, and cardiac remodeling using quantitative echocardiography.
- To elucidate the relationship between PH, left ventricular (LV) and right ventricular (RV) function, and patient outcomes.
Main Methods:
- Retrospective analysis of 191 MPN patients undergoing transthoracic echocardiography.
- PH diagnosis based on Doppler-derived pulmonary arterial systolic pressure (PASP ≥35 mm Hg).
- Assessment of RV performance using conventional indices (TAPSE, S') and global longitudinal strain; LV remodeling and diastolic function evaluated.
Main Results:
- PH was detected in 56% of MPN patients (20% advanced PH).
- PH associated with adverse LV remodeling (increased mass, diastolic dysfunction) and impaired RV function (P ≤ 0.001).
- RV dysfunction and PH grade independently predicted all-cause mortality (HR 1.9-3.3; P ≤ 0.012).
Conclusions:
- PH is highly prevalent in MPN patients and associated with LV diastolic dysfunction.
- Echocardiography-quantified RV dysfunction is a significant independent predictor of mortality in MPN.
- RV global longitudinal strain offers valuable prognostic information, similar to conventional RV indices.
More Related Videos
07:38Comprehensive Echocardiographic Assessment of Right Ventricle Function in a Rat Model of Pulmonary Arterial Hypertension
Published on: January 20, 2023
09:22Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Related Concept Videos
01:20Mitral Regurgitation I: Introduction
01:22Mitral Stenosis I: Introduction
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:23Mitral Stenosis II: Clinical features and Diagnostic Tests
01:26Heart Failure III: Clinical Manifestations
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy