Faculty Bibliography

Over the past few years an increasing number of prospective controlled sarcoidosis treatment trials have been completed. Unfortunately, these studies utilize different endpoints making comparisons between studies difficult. At the recent World Association of Sarcoidosis and other Granulomatous disease (WASOG) meeting, a session was dedicated to the evaluation of clinical endpoints for various disease manifestations. These included pulmonary, pulmonary hypertension, fatigue, cutaneous, and a classification of clinical disease phenotypes. Based on the available literature and our current understanding of the disease, recommendations for clinical evaluation were proposed for each disease category. For example, it was recommended that pulmonary studies should include changes in the forced vital capacity. Additionally, it was recommended that all trials should incorporate measurement of quality of life.

Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap with mechanisms of pulmonary arterial hypertension (PAH). This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH.

Using cardiac magnetic resonance, the presence of myocardial delayed contrast enhancement (DCE) has been described in the ventricular septum at the level of the right ventricular insertion points in patients with pulmonary hypertension (PH). The aim of this study was to investigate the prevalence, extent, and correlates of this finding. Septal DCE was evaluated in 55 patients with known or suspected PH of various causes. The extent of DCE was estimated visually with an insertion enhancement score (range 0 to 4) and quantified as DCE mass. The results were correlated with cine magnetic resonance and right-sided cardiac catheterization. Predictors of DCE were investigated using multivariate analysis. PH at rest was present in 42 patients (group 1) and absent in 13 (group 2). DCE was noted in 41 patients (97%) in group 1 and 3 (23%) in group 2 (p <0.0001). The extent of DCE was higher in group 1 than group 2 (median insertion enhancement score 3 vs 0, median DCE mass 8.7 vs 0 g, respectively; p <0.0001 for both). The extent of DCE showed moderate to good univariate correlations (r = 0.5 to 0.73) with pulmonary pressures and with right ventricular volumes, mass, and ejection fractions. In multivariate analysis, systolic pulmonary pressure was the only predictor of DCE. In conclusion, the presence of septal DCE at the right ventricular insertion points is common in PH of different causes, and the level of systolic pulmonary pressure elevation appears to be the main determinant of this finding.

PURPOSE: To retrospectively evaluate the accuracy and reproducibility of the cardiac magnetic resonance (MR) imaging-derived left ventricular septal-to-free wall curvature ratio for prediction of the right ventricular systolic pressure (RVSP) in patients clinically known to have or suspected of having pulmonary hypertension (PH), with same-day right-side heart catheterization (RHC) as the reference standard. MATERIALS AND METHODS: Institutional review board approval was received for this HIPAA-compliant study. Sixty-one patients clinically known or suspected of having PH underwent cardiac MR and RHC on the same day. Interventricular septal curvature (C(IVS)) and left ventricular free wall curvature (C(FW)) measured at end systole were used to derive the curvature ratio (C(IVS)/C(FW)). Effective distending transmural pressure (dP(FW)) and transseptal pressure gradient (dP(IVS)) were assumed to be equivalent, respectively, to the systolic blood pressure (SBP) and the difference between SBP and RVSP. Curvature ratio and SBP were used to noninvasively estimate RVSP. Linear regression analysis was performed to assess the difference between curvature ratio and rate of pressure rise (dP) ratio (dP(IVS)/dP(FW)). The accuracy of the dichotomized curvature ratio in PH detection was analyzed by using receiver operating characteristic (ROC) curves. RESULTS: PH, defined as RVSP higher than 40 mm Hg, was confirmed with RHC in 46 patients. A direct linear correlation between dP ratio and curvature ratio was observed (r = 0.85, P < .001). Bland-Altman analysis revealed moderate agreement between cardiac MR- and RHC-derived RVSPs (mean difference, -1.1 mm Hg +/- 15.9 [standard deviation]). ROC analysis of the accuracy of the curvature ratio for detection of increased RVSP revealed 87% sensitivity and 100% specificity (area under ROC curve, 0.95; P < .001). Intraobserver (r = 0.97) and interobserver (r = 0.95) curvature ratio measurements were closely correlated. CONCLUSION: In patients clinically known to have or suspected of having PH, cardiac MR-derived curvature ratio, as compared with RHC measurement, was an accurate and reproducible index for estimation of RVSP.