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Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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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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Pulmonary hypertension: screening and evaluation in scleroderma.

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  • 1Arthritis Center, Boston University School of Medicine, Boston, Massachusetts, USA.

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Systemic sclerosis is linked to frequent pulmonary hypertension, a major cause of death. Early suspicion and accurate diagnosis are key, as new treatments offer improved outcomes for scleroderma-related pulmonary arterial hypertension.

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Area of Science:

  • Rheumatology
  • Cardiology
  • Pulmonology

Background:

  • Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in patients with systemic sclerosis (SSc).
  • Understanding the epidemiology, pathogenesis, and risk factors of SSc-associated PH is crucial for timely diagnosis and management.
  • Recent advancements in screening and treatment offer hope for improved patient outcomes.

Purpose of the Study:

  • To provide an updated review of pulmonary arterial hypertension (PAH) in systemic sclerosis.
  • To discuss current understanding of epidemiology, pathogenesis, risk factors, screening, and treatment.
  • To highlight recent changes in PH classification and their implications for rheumatologists.

Main Methods:

  • Literature review of recent studies on SSc-associated PH.
  • Analysis of current screening methods, including noninvasive tests and biomarkers.
  • Evaluation of emerging treatment strategies for PAH.

Main Results:

  • Several noninvasive screening methods have shown utility in detecting PAH in SSc patients.
  • New treatments are emerging, potentially improving survival beyond the current median of 1 year.
  • Right heart catheterization remains the gold standard for diagnosing PH and differentiating subtypes.

Conclusions:

  • High clinical suspicion for PH is essential, even in early stages of SSc.
  • Accurate differentiation between pulmonary veno-occlusive disease and PAH is critical due to differential treatment responses.
  • The role of screening remains controversial, with ongoing debate regarding serum biomarkers and noninvasive testing.