Heart failure clinics
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Heart failure clinics · Jul 2012
ReviewPulmonary arterial hypertension in connective tissue diseases.
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs). Approximately 10% of patients with systemic sclerosis develop PAH, the prevalence being much lower in other CTDs. ⋯ Despite similarities in presentation, hemodynamic perturbations, and pathogenesis, patients with CTD-associated PAH (CTD-PAH) usually have a poorer response to PAH-specific medications and poorer prognosis than patients with idiopathic PAH (IPAH). Select patients with CTD-PAH may be candidates for lung transplantation, but results are less favorable than for IPAH because of comorbidities and complications specifically associated with CTD.
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The right ventricle (RV) is in charge of pumping blood to the lungs for oxygenation. Pulmonary arterial hypertension (PAH) is characterized by high pulmonary vascular resistance and vascular remodeling, which results in a striking increase in RV afterload and subsequent failure. There is still unexploited potential for therapies that directly target the RV with the aim of supporting and protecting the right side of the heart, striving to prolong survival in patients with PAH.
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Exercise stress tests of the pulmonary circulation show promise for the detection of early or latent pulmonary vascular disease and may help us understand the clinical evolution and effects of treatments in patients with established disease. Exercise stresses the pulmonary circulation through increases in cardiac output and left atrial pressure. Recent studies have shown that exercise-induced increase in pulmonary artery pressure is associated with dyspnea-fatigue symptomatology, validating the notion of exercise-induced pulmonary hypertension. Exercise in established pulmonary hypertension has no diagnostic relevance, but may help in the understanding of changes in functional state and the effects of therapies.