Der Internist
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The thin-walled right heart is characterized by a low mass-volume-relation. Right ventricular function is influenced basically by loading conditions (afterload and preload), myocardial perfusion, contractility and heart rate. Afterload is determined by intrathoracal and pulmonary vascular pressure/resistance. ⋯ So the normal, not hypertrophied right ventricle is extremely sensitive to increasing pulmonary artery pressure. Otherwise, minor reductions in afterload lead to a substantial decrease of right ventricular wall stress, myocardial oxygen demand and likely the risk of arrhythmia. Therefore clinical consequences and complications of pulmonary hypertension are substantially dependent on right ventricular dynamics.
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Chronic thrombembolic pulmonary hypertension (CTEPH) represents a common type of pulmonary hypertension and is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction. Unresolved thrombemboli and possible in situ thrombosis lead to obstruction of pulmonary arteries resulting in elevated pressures in those areas of the vasculature that were spared from thromboembolic occlusion. ⋯ Pulmonary endarterectomy of the obstructing thromboembolic material should be performed in patients who are accessible to surgery. In patients who are judged inoperable or with persistent pulmonary hypertension treatment with pulmonary arterial hypertension medications can be considered. This could contribute to improved clinical outcome and survival, whereas further controlled studies are required addressing this question.
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The prevalence of pulmonary hypertension in interstitial lung disease (ILD) is high (30-40%). However, diagnosis of pulmonary hypertension in ILD is often delayed. Pulmonary hypertension can occur in the absence of advanced pulmonary dysfunction or severe hypoxia and is associated with a worse prognosis. ⋯ Treatment options of pulmonary hypertension in ILD are limited to the treatment of the underlying diseases. Newer vasodilating drugs may improve the prognosis but have first to be evaluated in clinical trials. Lung or lung and heart transplantation is the therapeutic option in end stage disease.