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Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
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Atherosclerosis III: Management01:26

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Management of atherosclerosis involves an integrated strategy encompassing pharmacological treatment, surgical interventions, lifestyle changes, and nutrition therapy to address the multifactorial nature of the disease.Pharmacological TherapyA cornerstone of atherosclerosis management is the use of pharmacological agents. Statins, such as atorvastatin, are pivotal in inhibiting HMG-CoA reductase, an enzyme that catalyzes an initial step in cholesterol synthesis in the liver. This reduction in...
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Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.

Ryan Osgueritchian1, Vivek P Jani2, Hoda Mombeini1

  • 1Division of Cardiology, Johns Hopkins University, Baltimore, Maryland.

Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography
|July 31, 2025
PubMed
Summary
This summary is machine-generated.

Established risk scores offer limited prognostication for systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH). Echocardiographic RV-PA coupling metrics significantly improve mortality risk prediction in SSc-PAH patients.

Keywords:
EchocardiographyMortalityPulmonary arterial hypertensionRight ventricleScleroderma

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Area of Science:

  • Cardiology
  • Pulmonology
  • Rheumatology

Background:

  • Pulmonary arterial hypertension (PAH) significantly increases morbidity and mortality, especially in patients with systemic sclerosis (SSc).
  • Right ventricular (RV) adaptation to pulmonary artery (PA) afterload is critical for outcomes in SSc-associated PAH (SSc-PAH).
  • Existing clinical risk scores have unclear performance in prognosticating SSc-PAH.

Purpose of the Study:

  • To evaluate the association of established PAH risk scores with mortality in SSc-PAH.
  • To determine if echocardiographic RV-PA coupling metrics enhance risk discrimination in SSc-PAH.

Main Methods:

  • Retrospective analysis of 174 SSc-PAH patients with echocardiography.
  • Assessment of COMPERA 2.0, REVEAL 2.0, REVEAL Lite 2, and French noninvasive criteria.
  • Evaluation of RV global longitudinal strain and RV free wall strain (RVFWS) normalized to PA systolic pressure (PASP) for RV-PA coupling.

Main Results:

  • Established risk scores showed modest discrimination for 1-year mortality (C-indices 0.664–0.751).
  • Incorporating RV-PA coupling metrics (RV/PASP) significantly improved mortality prediction (P < .05).
  • RV-PA coupling parameters yielded the greatest increase in predictive accuracy across all assessed risk scores.

Conclusions:

  • Current risk scores have limited ability to predict 1-year mortality in SSc-PAH.
  • Echocardiographic RV-PA coupling parameters significantly enhance risk assessment in SSc-PAH.
  • This noninvasive approach refines prognostication for this high-risk patient group.