Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome.

AIM: In Marfan syndrome, various cardiovascular pathologies, such as aortic dilatation and mitral valve pathologies, already occur in childhood and determine course of the disease. This study aimed to establish additional cardiovascular risk markers for severe Marfan phenotypes. We investigated tricuspid valve prolapse (TVP) and its predictive value for outcome of paediatric Marfan disease. METHODS: In this retrospective, observational cohort study, we identified 130 paediatric Marfan patients (10.7 ± 4.8 years) with FBN1 variants. We divided patients into two groups based on TVP presence and performed a cross-sectional analysis to investigate the association of TVP with other cardiovascular, ocular and systemic pathologies, at first and last visit. A longitudinal analysis was performed with follow-up data. RESULTS: At baseline, patients with TVP had higher incidence of aortic root dilatation (p = 0.013), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.025) than patients without TVP. At follow-up, previous presence of TVP predicted higher probability of aortic root dilatation (p = 0.002), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.002). CONCLUSION: This shows that TVP is linked to both cardiac and extracardiac Marfan manifestations and TVP is an important marker for a disease severity in these children. Therefore, TVP should be assessed routinely using echocardiography in paediatric Marfan patients.

4D flow cardiovascular magnetic resonance for monitoring of aortic valve repair in bicuspid aortic valve disease.

BACKGROUND: Aortic valve repair has become a treatment option for adults with symptomatic bicuspid (BAV) or unicuspid (UAV) aortic valve insufficiency. Our aim was to demonstrate the feasibility of 4D flow cardiovascular magnetic resonance (CMR) to assess the impact of aortic valve repair on changes in blood flow dynamics in patients with symptomatic BAV or UAV. METHODS: Twenty patients with adult congenital heart disease (median 35 years, range 18-64; 16 male) and symptomatic aortic valve regurgitation (15 BAV, 5 UAV) were prospectively studied. All patients underwent 4D flow CMR before and after aortic valve repair. Aortic valve regurgitant fraction and systolic peak velocity were estimated. The degree of helical and vortical flow was evaluated according to a 3-point scale. Relative flow displacement and wall shear stress (WSS) were quantified at predefined levels in the thoracic aorta. RESULTS: All patients underwent successful aortic valve repair with a significant reduction of aortic valve regurgitation (16.7 ± 9.8% to 6.4 ± 4.4%, p < 0.001) and systolic peak velocity (2.3 ± 0.9 to 1.9 ± 0.4 m/s, p = 0.014). Both helical flow (1.6 ± 0.6 vs. 0.9 ± 0.5, p < 0.001) and vortical flow (1.2 ± 0.8 vs. 0.5 ± 0.6, p = 0.002) as well as both flow displacement (0.3 ± 0.1 vs. 0.25 ± 0.1, p = 0.031) and WSS (0.8 ± 0.2 N/m2 vs. 0.5 ± 0.2 N/m2, p < 0.001) in the ascending aorta were significantly reduced after aortic valve repair. CONCLUSIONS: 4D flow CMR allows assessment of the impact of aortic valve repair on changes in blood flow dynamics in patients with bicuspid aortic valve disease.

Accuracy of multiparametric MR imaging with PI-RADS V2 assessment in detecting infiltration of the neurovascular bundles prior to prostatectomy.

OBJECTIVES: To evaluate the accuracy of assessment of neurovascular bundle (NVB) infiltration using multiparametric magnetic resonance imaging (mpMRI) and PI-RADS V2 prior to prostatectomy. METHODS: The ethics committee approved this retrospective study with waiver of informed consent. N=198 consecutive patients with biopsy proved cancer underwent standardized mpMRI at 3T prior to surgery. NVB infiltration was assessed for each side (a total of 396). Maximum PI-RADS V2 scores were determined for the posterolateral areas adjacent to the NVBs. Imaging results were correlated with postoperative pathology and standard descriptive statistics were calculated. RESULTS: Overall T-staging sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of mpMRI were 64.4%, 89.2%, 82.4%, 76.2% and 78.3%, respectively. In 396 cases NVB infiltration was predicted with 75.3%, 94.0%, 80.2%, 92.1 % and 89.4 % sensitivity, specificity, PPV, NPV and accuracy, respectively. Analyses of 396 NVB and their adjacent PI-RADS V2 scores with pathology revealed significantly more NVB-infiltrations in suspect scores of 5 and 4 vs. uncertain scores of 3-1 (81/264 vs. 16/132, p=0.0001). Considering scores higher than 3 as a criterion of infiltration demonstrated moderate sensitivity and poor specificity (83.5% and 38.8%, respectively). Interobserver agreement of a second reading of a random sample was good (κ=0.64) for NVB infiltrations and moderate (κ=0.59) for PI-RADS V2. CONCLUSIONS: Assessment of infiltration of the neurovascular bundles using mpMRI has valuable diagnostic performance, yet PI-RADS V2 Scores demonstrate limited eligibility. Combined findings offer crucial information for the planning of prostatectomy.

Myocardial Fibrosis in Competitive Triathletes Detected by Contrast-Enhanced CMR Correlates With Exercise-Induced Hypertension and Competition History.

OBJECTIVES: This study analyzed the presence of myocardial fibrosis detected by late gadolinium-enhancement (LGE) cardiac magnetic resonance (CMR) in correlation with the performance of competitive triathletes objectified by an exercise test and individual competition history. BACKGROUND: Myocardial fibrosis detected by LGE CMR has been reported to occur in 0% to 50% of asymptomatic athletes. However, the cause and mechanisms of myocardial fibrosis are unclear. METHODS: Eighty-three asymptomatic triathletes undergoing >10 training h per week (43 ± 10 years of age; 65% male) and 36 sedentary controls were studied by using LGE and extracellular volume (ECV) CMR. Parameters of physical fitness were measured by spiroergometry. Triathletes reported their lifetime competition results. RESULTS: LGE CMR revealed focal nonischemic myocardial fibrosis in 9 of 54 (17%) male triathletes (LGE+) but in none of the female triathletes (p < 0.05). LGE+ triathletes had higher peak exercise systolic blood pressure (213 ± 24 mm Hg) than LGE- triathletes (194 ± 26 mm Hg; p < 0.05). Furthermore, left ventricular mass index was higher in LGE+ triathletes (93 ± 7 g/m2) than in LGE- triathletes (84 ± 11 g/m2; p < 0.05). ECV in LGE- myocardium was higher in LGE+ triathletes (26.3 ± 1.8%) than in LGE- triathletes (24.4 ± 2.2%; p < 0.05). LGE+ triathletes completed longer cumulative distances in swimming and cycling races and participated more often in middle and Iron Man distances than LGE- triathletes. A cycling race distance of >1,880 km completed during competition had the highest accuracy to predict LGE, with an area under the curve value of 0.876 (p < 0.0001), resulting in high sensitivity (89%) and specificity (79%). Multivariate analysis identified peak exercise systolic blood pressure (p < 0.05) and the swimming race distance (p < 0.01) as independent predictors of LGE presence. CONCLUSIONS: Myocardial fibrosis in asymptomatic triathletes seems to be associated with exercise-induced hypertension and the race distances. There appears to be a safe upper limit, beyond which exercise may result in myocardial fibrosis.

Diagnosing acute pancreatitis-Clinical and radiological characterisation of patients without threefold increase of serum lipase.

OBJECTIVES: Diagnosing acute pancreatitis (AP) may be challenging in patients with acute abdominal pain but missing threefold increased serum lipase levels (Lip-). This studyaims both to characterize these patients using clinical, radiological and mortality data, and to assess the group of patients who need contrast-enhanced computed tomography (CECT). METHODS: In this retrospective, IRB approved study 234 consecutive patients with AP were investigated. Inclusion criteria were single (SAP) and recurrent attacks (RAP) of AP and CECT ≥72h after onset of symptoms. Severity of AP was assessed by C-reactive protein at 48h after hospital admission and using 3 CT-based scores (CTSI, mCTSI, EPIC) by 2 observers. Mortality rates from pancreatic and non-pancreatic causes were noted with regard to lipase increase. Results were compared with paired t-test and Wilcoxon signed-rank test. RESULTS: 64/234 (27%) patients belonged to Lip- group and 170/234 (73%) patients were allocated to Lip+ group. Significantly more male patients (78% in Lip- vs. 63% in Lip+, p<0.05) with RAP (63% in Lip- vs. 21% in Lip+, p<0.001) were observed in the Lip- group. EPIC was significantly lower in Lip- group compared to Lip+ group (3(IQR 2-5) vs. 2(IQR 1-3), p<0.001). Mortality from pancreatic and non-pancreatic causes was comparable in Lip- and Lip+ group (pancreatic causes: 9% vs. 6%, p=0.60; nonpancreatic causes: 8% vs. 5%, p=0.58). CONCLUSIONS: 27% of all patients with AP presented without threefold increase of lipase levels. Thus, they would be underdiagnosed without confirming CECT, which revealed significantly lower counts of pleural effusions and ascites. Male patients with RAP were found significantly more often among the Lip- group. Hence, they would benefit the most from CECT for diagnosing AP.

MDCT of acute pancreatitis: Intraindividual comparison of single-phase versus dual-phase MDCT for initial assessment of acute pancreatitis using different CT scoring systems.

OBJECTIVES: The purpose of the retrospective study was to evaluate the additional value of dual-phase multidetector computed tomography (MDCT) protocols over a single-phase protocol on initial MDCT in patients with acute pancreatitis using three CT-based pancreatitis severity scores with regard to radiation dose. METHODS: In this retrospective, IRB approved study MDCT was performed in 102 consecutive patients (73 males; 55years, IQR48-64) with acute pancreatitis. Inclusion criteria were CT findings of interstitial edematous pancreatitis (IP) or necrotizing pancreatitis (NP) and a contrast-enhanced dual-phase (arterial phase and portal-venous phase) abdominal CT performed at ≥72h after onset of symptoms. The severity of pancreatic and extrapancreatic changes was independently assessed by 2 observers using 3 validated CT-based scoring systems (CTSI, mCTSI, EPIC). All scores were applied to arterial phase and portal venous phase scans and compared to score results of portal venous phase scans, assessed ≥14days after initial evaluation. For effective dose estimation, volume CT dose index (CTDIvol) and dose length product (DLP) were recorded in all examinations. RESULTS: In neither of the CT severity scores a significant difference was observed after application of a dual-phase protocol compared with a single-phase protocol (IP: CTSI: 2.7 vs. 2.5, p=0.25; mCTSI: 4.0 vs. 4.0, p=0.10; EPIC: 2.0 vs. 2.0, p=0.41; NP: CTSI: 8.0 vs. 7.0, p=0.64; mCTSI: 8.0 vs. 8.0, p=0.10; EPIC: 3.0 vs. 3.0, p=0.06). The application of a single-phase CT protocol was associated with a median effective dose reduction of 36% (mean dose reduction 31%) compared to a dual-phase CT scan. CONCLUSIONS: An initial dual-phase abdominal CT after ≥72h after onset of symptoms of acute pancreatitis was not superior to a single-phase protocol for evaluation of the severity of pancreatic and extrapancreatic changes. However, the effective radiation dose may be reduced by 36% using a single-phase protocol.