Using all-cause mortality to define severe RV dilation with RV/LV volume ratio.

Right ventricular (RV) end-diastolic volume (EDV) to left ventricular (LV) EDV ratio using cardiovascular magnetic resonance imaging (CMR) is an important parameter for RV size evaluation in additional to indexed EDV. We explore the severity partition for RV dilation using mortality in a population of 62 patients with pulmonary hypertension (PH). Cine short-axis images were acquired with a 1.5 T MR scanner using a steady-state free precession sequence. The optimal cutoff to classify severe RV dilation was determined by a receiver-operating curve (ROC) analysis based on mortality. We further defined mild and moderate categories by the standard deviation distance between normal and severely dilated and found the categories RV dilation by RV/LV volume ratio to be "mild" (1.27-1.69), "moderate" (1.70-2.29) and "severe" (≥2.30). There were significant differences in RVEDV and RV ejection fraction between "mild", "moderate" and "severe" groups (p < 0.001). The "severe" category had a significantly higher mortality when compared to the "non-severe" categories (p < 0.001) while there was no difference among the "non-severe" dilated groups. We have shown that severe RV dilation partition can be defined using mortality with RV/LV volume ratio, which offers an outcome based grading of the "severe" category of RV dilation.

Cardiac MRI right ventricle / left ventricle (RV/LV) volume ratio improves detection of RV enlargement.

PURPOSE: To determine the normal range of the ratio of right ventricular (RV) end-diastolic volume to left ventricular (LV) end-diastolic volume by magnetic resonance imaging (MRI) and examine whether combining this volume ratio with RVEDV indexed to body surface area (RVEDVi) increased the detection of RV dilation in patients with pulmonary arterial hypertension (PAH). MATERIALS AND METHODS: MRI-derived ventricular function and volumes were measured in a control group (n = 152) and in patients with PAH (n = 46). Images were acquired with a 1.5T Siemens or a 1.5T Philips scanner using a steady-state free procession sequence. Proposed criteria for the detection of RV enlargement, including RVEDVi alone, RV/LV volume ratio alone, and combining both criteria, were evaluated in both groups. RESULTS: The range (mean ± 2 standard deviations) for the volume ratio in the normal population was found to be 0.906-1.266; there was no difference between genders (P = 0.70). Combining this ratio with RVEDVi detected RV enlargement in 21.7% (P < 0.001) PAH patients (volume ratio ≥1.27) who were not identified by the RVEDVi alone (>104 mL/m(2) for females and >113 mL/m(2) for males). CONCLUSION: Combining RV/LV volume ratio with indexed RVEDV increased detection of RV enlargement in a PAH population. This result may have potential impact in RV size assessment. J. Magn. Reson. Imaging 2016;43:1379-1385.

RV mass measurement at end-systole: Improved accuracy, Reproducibility, and reduced segmentation time.

PURPOSE: To evaluate the accuracy, reproducibility, and contouring time of RV mass in end-systole (ES) and end-diastole (ED). Magnetic resonance imaging (MRI) has been shown to be accurate and reproducible for the evaluation of right ventricular (RV) volume and function. RV mass, assessed in end-diastolic (ED) phase, is one of the least reproducible variables. The choice of end-systolic (ES) phase could offer an alternative to improve reproducibility, since the selection of the basal slice and the visualization of the usually thin RV wall are easier in this phase. MATERIALS AND METHODS: To evaluate accuracy, 11 sheep were imaged in vivo and their RV free walls were weighed after removing epicardial fat. To evaluate reproducibility, 30 normal subjects and 30 subjects with pulmonary arterial hypertension (PAH) were imaged and interobserver and intraobserver variabilities were assessed in the ES and the ED. Segmentation time was recorded after visual selection of ES and ED phases. RESULTS: ES RV mass measurement has less absolute variability (5.2% ± 3.2) compared to ED (10.6% ± 6.3) using weighed RV mass in sheep as the gold standard (P < 0.001). ES segmentation yielded higher intraobserver (intraclass correlation coefficients [ICC] = 0.94-0.99; coefficient of variability [CoV] = 6-7.3%) and interobserver (ICC = 0.85-0.98; CoV = 10.9-11.7%) reproducibility than ED segmentation. Segmentation time in humans was 25-28% faster in ES (P < 0.001). CONCLUSION: The MRI assessment of RV mass is more accurate, reproducible, and faster in the ES phase.

Encephalocele-radial, cardiac, gastrointestinal, anal/renal anomalies: novel evidence for a new condition?

The association between encephalocele and radial defects is considered uncommon. These features have been occasionally described separately in certain recurrent conditions such as VACTERL association, oculo-auriculo-vertebral spectrum and Edwards syndrome (trisomy 18). DK-phocomelia is a rare syndrome characterized by both findings. However, Froster-Iskenius and Meinecke [1992, Clin Dysmorphol 1: 37-41] and Kunze et al. [1992, Eur J Pediatr 151: 467-468] reported patients presenting similar malformations. We proposed, through the description of an additional case, that these last patients present the same condition and thus represent a new syndrome. The fetus presented a cranial vault deformity associated with an exuberant herniation of brain content, compatible with occipital encephalocele. Other uncommon features were also identified: microtia of the left ear with atresia of the external auditory canal; radial defect with aplasia of left radius and thumb; findings suggestive of a congenital heart defect and esophageal atresia; hypoplastic lungs and adrenals; thoracolumbar scoliosis; atrophic right kidney; and single umbilical artery. Thus, based on our review, we believe that these patients represent a new condition characterized by encephalocele and radial defects associated with multiple malformations. We propose, that the name "Encephalocele-radial, cardiac, gastrointestinal, anal/renal anomalies," as suggested by the London Medical Database, or even the name, "Froster-Iskenius and Meinecke syndrome" should be used to indicate these cases. © 2014 Wiley Periodicals, Inc.