BackgroundLate gadolinium enhancement (LGE) occurs at the right ventricular (RV) insertion point (RVIP) in patients with pulmonary hypertension (PH) and has been shown to correlate with cardiovascular magnetic resonance (CMR) derived RV indices. However, the prognostic role of RVIP-LGE and other CMR-derived parameters of RV function are not well established. Our aim was to evaluate the predictive value of contrast-enhanced CMR in patients with PH.MethodsRV size, ejection fraction (RVEF), and the presence of RVIP-LGE were determined in 58 patients with PH referred for CMR. All patients underwent right heart catheterization, exercise testing, and N-terminal pro-brain natriuretic peptide (NT-proBNP) evaluation; results of which were included in the final analysis if performed within 4 months of the CMR study. Patients were followed for the primary endpoint of time to clinical worsening (death, decompensated right ventricular heart failure, initiation of prostacyclin, or lung transplantation).ResultsOverall, 40/58 (69%) of patients had RVIP-LGE. Patients with RVIP- LGE had larger right ventricular volume index, lower RVEF, and higher mean pulmonary artery pressure (mPAP), all p < 0.05. During the follow-up period of 10.2 ± 6.3 months, 19 patients reached the primary endpoint. In a univariate analysis, RVIP-LGE was a predictor for adverse outcomes (p = 0.026). In a multivariate analysis, CMR-derived RVEF was an independent predictor of clinical worsening (p = 0.036) along with well-established prognostic parameters such as exercise capacity (p = 0.010) and mPAP (p = 0.001).ConclusionsThe presence of RVIP-LGE in patients with PH is a marker for more advanced disease and poor prognosis. In addition, this study reveals for the first time that CMR-derived RVEF is an independent non-invasive imaging predictor of adverse outcomes in this patient population.

Background-Presurgical planning of mitral valve (MV) repair in patients with Barlow disease (BD) and fibroelastic deficiency (FED) is challenging because of the inability to assess accurately the complexity of MV prolapse. We hypothesized that the etiology of degenerative MV disease (DMVD) could be objectively and accurately ascertained using parameters of MV geometry obtained by morphological analysis of real-time 3D echocardiographic (RT3DE) images. Methods and Results-Seventy-seven patients underwent transesophageal RT3DE study: 57 patients with DMVD studied intraoperatively (28 BD, 29 FED classified during surgery) and 20 patients with normal MV who were used as control subjects (NL). MVQ software (Philips) was used to measure parameters of annular dimensions and geometry and leaflet surface area, including billowing volume and height. The Student t test and multinomial logistic regression was performed to identify parameters best differentiating DMVD patients from normal as well as FED from BD. Morphological analysis in the DMVD group revealed a progressive increase in multiple parameters from NL to FED to BD, allowing for accurate diagnosis of these entities. The strongest predictors of the presence of DMVD included billowing height and volume. Three-dimensional billowing height with a cutoff value of 1.0 mm differentiated DMVD from NL without overlap, and billowing volume with a cutoff value 1.15 mL differentiated between FED and BD without overlap. Conclusions-Morphological analysis as a form of decision support in assessing MV billowing revealed significant quantifiable differences between NL, FED, and BD patients, allowing accurate classification of the etiology of MV prolapse and determination of the anticipated complexity of repair. (Circ Cardiovasc Imaging. 2011;4:24-32.)

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AimsLate gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) is a valuable test to detect myocardial damage in patients with sarcoidosis; however, the clinical significance of LGE in sarcoidosis patients with preserved left ventricular ejection fraction (LVEF) is not defined. We aim to characterize the prevalence of LGE, its associated cardiac findings, and its clinical implications in sarcoidosis patients with preserved LVEF. Methods and resultsOne hundred and fifty-two patients with biopsy proven extra-cardiac sarcoidosis, no known cardiac sarcoidosis, and LVEF ≥50% referred for LGE-CMR were included in this observational study. The presence of LGE in the left ventricular myocardium was considered diagnostic for cardiac sarcoidosis. The cohort was divided into two groups based on the presence or absence of LGE. Twenty-nine patients (19%) had LGE involving 11 + 9% of the left ventricle. The modified Japanese Ministry of Health and Welfare (JMHW) criteria for diagnosing cardiac sarcoidosis only had a sensitivity of 52% and specificity of 83% for identifying myocardial LGE in these patients. Compared with those patients without LGE, those with LGE had a higher heart rate (84 + 19 vs. 76 + 18 b.p.m., P ¼ 0.002), greater prevalence of an abnormal electrocardiogram (76 vs. 31%, P , 0.001), diastolic dysfunction (67 vs. 33%, P ¼ 0.05), reduced right ventricular ejection fraction (49 + 8 vs. 55 + 6%, P ¼ 0.012), and evidence of non-sustained ventricular tachycardia (33 vs. 6%). ConclusionsIn patients with sarcoidosis and preserved systolic function, myocardial damage is commonly present and may increase the risk of ventricular tachy-arrhythmias. The JMHW Criteria were neither sensitive nor specific for predicting the presence of myocardial LGE.--