Although the prognosis for patients with Pulmonary Hypertension (PH) has improved over recent decades, it is still difficult to predict clinical outcome in individual patients. In all forms of the disease, right ventricular (RV) function remains the most important determinant of survival and the difficulties encountered in predicting prognosis in individuals reflects the heterogeneous response of the RV. Our understanding of the mechanisms that underpin RV failure or adaptation in response to PH has greatly improved over recent years, as has our ability to accurately identify prognostically significant RV dysfunction in patients with PH. These advances have been driven by significant improvements in established RV imaging tools, such as 2-D echocardiography, and by the development of entirely new techniques, such as 3-D echocardiography, tissue Doppler imaging and cardiovascular magnetic resonance imaging. The purpose of this article is to review these techniques and to summarize these recent advances.

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The authors provide histopthological images of pulmonary vascular lesions from patients with diseases of several etiologies and clinically presenting with pulmonary hypertension. The sources are lung for the purpose of presurgical evaluation in children with congenital heart disease, lung explants in cases of lung transplantation, and lung tissue from necropsies. Lesions are classified as nonspecific or specific, according to the histopathological features that may allow the recognition of etiology or a specific form of presentation.

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[FULL TEXT]  [PDF] [CITATIONS]