Targeting Local Recurrence After Surgery With MRI Imaging for Prostate Cancer in the Setting of Salvage Radiation Therapy.

Magnetic resonance imaging (MRI) is being increasingly used for imaging suspected recurrence in prostate cancer therapy. Functional MRI with diffusion and perfusion imaging has the potential to demonstrate local recurrence even at low PSA value. Detection of recurrence can modify the management of postprostatectomy biochemical recurrence. MRI scan acquired before salvage radiotherapy is useful for the localization of recurrent tumors and also in the delineation of the target volume. The objective of this review is to assess the role and potential impact of MRI in targeting local recurrence after surgery for prostate cancer in the setting of salvage radiation therapy.

[Cardiac glomus tumor: An unusual localization of pericytic (perivascular) tumor]. / Tumeur glomique intracardiaque : une tumeur péricytaire (périvasculaire) de localisation inhabituelle.

Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization. However visceral locations have been described. Cardiac location is exceptional with only one case reported in literature. Here, we report the case of cardiac glomus tumor (glomangiomyoma) developed in the left ventricle in a 34 year-old patient, diagnosed after chest pain. The length of tumor was 4cm in greatest dimension. Histologically, the tumor concerned both round glomus cells and smooth muscle cells with prominent branching thin-walled vessels. By immunohistochemistry, these two contingents exhibited diffuse expression of smooth muscle actin and heterogeneous expression of H-caldesmone whereas cytokeratins, melanocytic markers and chomogranine were negative. Next Generation molecular analysis using RNA sequencing highlighted the characteristic MIR143-NOTCH gene fusion witch supports the diagnosis of glomus tumor. In this observation, we recall histological and immunohistochemistry features of glomus tumor and we make a synthesis concerning the molecular data recently described in sporadic glomus tumor.

Use of a Biodegradable, Contrast-Filled Rectal Spacer Balloon in Intensity-Modulated Radiotherapy for Intermediate-Risk Prostate Cancer Patients: Dosimetric Gains in the BioPro-RCMI-1505 Study.

BACKGROUND/PURPOSE: Dose-escalated external beam radiotherapy (RT) is effective in the control of prostate cancer but is associated with a greater incidence of rectal adverse events. We assessed the dosimetric gain and safety profile associated with implantation of a new biodegradable rectal spacer balloon. MATERIALS/METHODS: Patients scheduled for image-guided, intensity-modulated RT for intermediate-risk prostate cancer were prospectively included in the French multicenter BioPro-RCMI-1505 study (NCT02478112). We evaluated the dosimetric gain, implantation feasibility, adverse events (AEs), and prostate-cancer-specific quality of life associated with use of the balloon spacer. RESULTS: After a scheduled review of the initial recruitment target of 50 patients by the study's independent data monitoring committee (IDMC), a total of 24 patients (including 22 with dosimetry data) were included by a single center between November 2016 and May 2018. The interventional radiologist who implanted the balloons considered that 86% of the procedures were easy. 20 of the 24 patients (83.3%) received IMRT and 4 (16.7%) received volumetric modulated arc therapy (78-80 Gy delivered in 39 fractions). The dosimetric gains associated with spacer implantation were highly significant (p<0.001) for most variables. For the rectum, the median (range) relative gain ranged from 15.4% (-9.2-47.5) for D20cc to 91.4% (36.8-100.0) for V70 Gy (%). 15 patients (62%) experienced an acute grade 1 AE, 8 (33%) experienced a late grade 1 AE, 1 (4.2%) experienced an acute grade 2 AE, and 3 experienced a late grade 2 AE. No grade 3 AEs were reported. Quality of life was good at baseline (except for sexual activity) and did not markedly worsen during RT and up to 24 months afterwards. CONCLUSION: The use of a biodegradable rectal spacer balloon is safe, effective and associated with dosimetric gains in modern RT for intermediate-risk prostate cancer.

Single pulmonary valve cusp agenesis first detected by computed tomography reconstruction.

We present the case, with tomographic three-dimensional reconstructions, of an adult patient affected by congenital absence of one pulmonary valve cusp with completely normal morphology of the other two cusps.

Independent Evaluation of the Respective Predictive Values for High-Grade Prostate Cancer of Clinical Information and RNA Biomarkers after Upfront MRI and Image-Guided Biopsies.

Upfront MRI is taking the lead in the diagnosis of clinically significant prostate cancer, while few image-guided biopsies (IGBs) fail to demonstrate clinically significant prostate cancer. The added value of innovative biomarkers is not confirmed in this context. We analysed SelectMDx-v2 (MDx-2) in a cohort of upfront MRI and image-guided biopsy patients. Participants included patients who received a trans-rectal elastic-fusion registration IGB on the basis of DRE, PSA, PCA3, and PCPT-2.0 risk evaluation. Pre-biopsy MRI DICOM archives were reviewed according to PI-RADS-v2. Post-massage first-void urine samples stored in the institutional registered bio-repository were commercially addressed to MDxHealth to obtain MDx-2 scores. Univariate and multivariate analyses were conducted with the detection on IGB of high-grade (ISUP 2 and higher) as the dependent variable. High-grade cancer was demonstrated in 32/117 (27.4%) patients (8/2010-8/2018). Age, prostate volume, biopsy history, MDx-2, and PI-RADS-v2 scores significantly related to the detection of high-grade cancer. MDx-2 scores and the clinical variables embedded into MDx-2 scores were analysed in multivariate analysis to complement PI-RADS-v2 scores. The two combinations outperformed PI-RADS-v2 alone (AUC-ROC 0.67 vs. 0.73 and 0.80, respectively, p < 0.05) and calibration curves confirmed an adequate prediction. Similar discrimination (C-statistics, p = 0.22) was observed in the prediction of high-grade cancer, thereby questioning the respective inputs and added values of biomarkers and clinical predictors in MDx-2 scores. Based on the results of this study, we can conclude that instruments of prediction developed for systematic prostate biopsies, including those that incorporate innovative biomarkers, must be reassessed and eventually confirmed in the context of upfront MRI and IGB.

Accuracy of the preoperative PSA level for predicting clinically significant incidental transitional zone-prostate cancer before endoscopic enucleation of very large adenoma.

OBJECTIVE: To analyse the accuracy of high preoperative PSA levels for predicting transitional zone incidental PCa (TZ-PCa) in men with very large prostates. MATERIALS AND METHODS: Perioperative data from 375 consecutive patients who underwent endoscopic enucleation of the prostate (EEP) for benign prostatic obstruction between July 2013 and December 2018 were retrospectively reviewed. Patients were stratified into three groups according to the preoperative PSA level: low-PSA (< 4 ng/mL), intermediate-PSA (4 ≤ PSA < 10 ng/mL) and high-PSA (≥ 10 ng/mL). Men in each group were propensity score matched by age, 5α-reductase inhibitor (5-ARI) use, prostate volume and mpMRI. The TZ-PCa incidence rate was retrospectively compared by preoperative PSA level in a propensity score model including all predetermined variables. RESULTS: Age, prostate volume, 5-ARI use were similar between patient groups. The median PSA levels in the low-, intermediate- and high-PSA groups were 3 [2.3; 3.4], 6.6 [5.3; 8.1] and 12.7 [11; 16.7] ng/mL, respectively. The median prostate volume was > 100 grams in all groups (108, 105 and 120 cc, respectively). The T1a-Gleason 6 incidental TZ-PCa rate was statistically comparable between the three groups (3.4, 5.1 and 8.6% in the low-, intermediate- and high-PSA groups, respectively). The detection rate of clinically significant TZ-PCa was low for preoperative PSA levels > 4 ng/mL (1.7%); with no difference between the intermediate- and high-PSA groups. CONCLUSION: In men with large glands, the clinically significant incidental TZ-PCa detection rate was similar regardless of the preoperative PSA level stratum. Such details may help with patient counselling during BPH surgical management.

Is mean heart dose a relevant surrogate parameter of left ventricle and coronary arteries exposure during breast cancer radiotherapy: a dosimetric evaluation based on individually-determined radiation dose (BACCARAT study).

BACKGROUND: Intra-individual heterogeneity of cardiac exposure is an issue in breast cancer (BC) radiotherapy that was poorly considered in previous cardiotoxicity studies mainly based on mean heart dose (MHD). This dosimetric study analyzes the distribution of individually-determined radiation doses to the heart and its substructures including coronary arteries and evaluate whether MHD is a relevant surrogate parameter of dose for these substructures. METHODS: Data were collected from the BACCARAT prospective study that included left or right unilateral BC patients treated with 3D-Conformal Radiotherapy (RT) between 2015 and 2017 and followed-up for 2 years with repeated cardiac imaging examinations. A coronary computed tomography angiography (CCTA) was performed before RT for all patients. Registration of the planning CT and CCTA images allowed delineation of the coronary arteries on the planning CT images. Using the 3D dose matrix generated during treatment planning and the added coronary contours, dose distributions were generated for whole heart and the following substructures: left ventricle (LV), left main coronary artery (LMCA), left anterior descending artery (LAD), left circumflex artery (LCX) and right coronary artery (RCA). A descriptive analysis of the physical doses in Gray (Gy) was performed, Dmean was the volume-weighted mean dose. RESULTS: Dose distributions were generated for 89 left-sided BC patients (MHD = 2.9 ± 1.5 Gy, Dmean_LAD = 15.7 ± 3.1 Gy) and 15 right-sided BC patients (MHD = 0.5 ± 0.1 Gy; Dmean_RCA = 1.2 ± 0.4 Gy). For left-sided BC patients, the ratio Dmean_LAD/MHD was around 5. Pearson correlation coefficients between MHD and Dmean for delineated substructures were all statistically significant. However, for all substructures, the coefficient of determination R2 indicated that the proportion of the variance in Dmean of the substructure predictable from MHD was moderate to low (in particular R2 = 0.45 for LAD). Among left-sided BC patients with MHD < 3Gy, 56% of patients could nevertheless receive LAD doses above 40Gy (V40 > 0). CONCLUSION: Our study illustrates that MHD is not enough to predict with confidence individual patient dose to the LV and coronary arteries, in particular the LAD. For precise radiotherapy-induced cardiotoxicity studies it would be necessary to consider the distribution of doses within these cardiac substructures rather than just the MHD. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02605512 , Registered 6 November 2015 - Retrospectively registered.

Left atrial appendage occlusion simulation based on three-dimensional printing: new insights into outcome and technique.

AIMS: The aim of this study was to assess the predictive value of simulation based on 3D-printed models before left atrial appendage occlusion (LAAO) for peri-device leaks (PDL) and the impact on procedural outcomes compared to conventional imaging. METHODS AND RESULTS: Seventy-six patients referred for LAAO with double disc device underwent construction of a 3D-printed LA model using flexible photopolymer resin. An implant simulation was performed retrospectively in Group 1 (55 patients), blinded to implanted device size, and prospectively in Group 2 (21 patients). In Group 1, an off-axis device position occurred in 14 patients (25%) and the incidence of PDL was 27% (15 patients); mismatch between model and device size was the best predictor (area under the curve 0.88, CI: 0.77-0.99). When using 3D simulation prospectively, mean prosthesis number per patient (1.05±0.21 vs. 1.20±0.52, p=0.04) and incidence of leaks (5% vs. 27%, p<0.01) were reduced compared to conventional imaging alone, as well as fluoroscopy time (19 mins [13.4-23] vs. 13.5 mins [11.1-15], p=0.012) and total fluoroscopy dose (7,291 [1,811-12,734] vs. 1,978 (1,548-4,800) mGy·cm2, p=0.029). CONCLUSIONS: 3D-printed patient-specific adaptive and flexible LA models improve LAAO double disc device sizing. This can potentially reduce procedure time and the number of prostheses employed per patient.

Precision Matters in MR Imaging-targeted Prostate Biopsies: Evidence from a Prospective Study of Cognitive and Elastic Fusion Registration Transrectal Biopsies.

Purpose To measure the precision in placement of a biopsy needle in a magnetic resonance (MR) imaging-detected target with transrectal ultrasonography (US), to document the clinical relevance of precision, and to report on the precision of cognitive and software-based registrations. Materials and Methods This prospective study was approved by the institutional review board and performed between June 2013 and September 2013. Patients provided informed verbal consent. Two cores each were obtained with cognitive and fusion techniques in 88 patients with a Prostate Imaging Reporting and Data System version 1 score of at least 3. Precision was measured with Euclidian geometry by using the Digital Imaging and Communications in Medicine archives of the biopsy as the distance from the core to the center (dCC) and the distance from the core to the surface of the target modeled as a sphere. To address clustering of data from multiple cores in the same patients, analyses of precision focused on the best shot for a patient or a technique. The Welch unequal variance t test and Yates corrected χ2 test were used as appropriate. Results Mean precision was 2.5 mm (95% confidence interval: 1.8 mm, 3.3 mm). Positive cores were closer to the center than were negative cores (dCC: 1.7 mm vs 3.1 mm, respectively; P = .025). More cancers were detected with on-target than off-target cores (33 of 71 cores [46.5%] vs three of 17 cores [17.6%]; P = .03). Cores obtained with the fusion technique achieved a higher precision than did cores obtained with the cognitive technique (dCC: 2.8 mm vs 7.1 mm, respectively; P < .0001). Targeted cores demonstrated cancer in 44 patients. Fewer cancers were detected with the cognitive technique than with the fusion technique (31 of 44 patients [70.5%] vs 40 of 44 patients [90.9%]; P = .03). Conclusion A deformable MR imaging/transrectal US image registration system achieved a higher precision and depicted cancer in more patients than did the cognitive freehand technique. © RSNA, 2018.

Early detection and prediction of cardiotoxicity after radiation therapy for breast cancer: the BACCARAT prospective cohort study.

BACKGROUND: Radiotherapy (RT) for breast cancer presents a benefit in terms of reducing local recurrence and deaths resulting from breast cancer but it can lead to secondary effects due to the presence of neighboring cardiac normal tissues within the irradiation field. Breast RT has been shown to be associated with long-term increased risk of heart failure, coronary artery disease, myocardial infarction and finally cardiovascular death more than 10 years after RT. However, there is still a lack of knowledge for early cardiotoxicity induced by breast RT that can appear long before the onset of clinically significant cardiac events. Based on a 2-year follow-up prospective cohort of patients treated with breast RT, the BACCARAT (BreAst Cancer and CArdiotoxicity Induced by RAdioTherapy) study aims to enhance knowledge on detection and prediction of early subclinical cardiac dysfunction and lesions induced by breast RT and on biological mechanisms potentially involved, based on functional and anatomical cardiac imaging combined with simultaneous assessment of multiple circulating biomarkers and accurate heart dosimetry. METHODS/DESIGN: BACCARAT study consists in a monocentric prospective cohort study that will finally include 120 women treated with adjuvant 3D CRT for breast cancer, and followed for 2 years after RT. Women aged 50 to 70 years, treated for breast cancer and for whom adjuvant 3D CRT is indicated, without chemotherapy are eligible for the study. Baseline (before RT) and follow-up data include measurements of functional myocardial dysfunction including strain and strain rate based on 2D-speckle tracking echocardiography, anatomical coronary lesions including description of plaques in segments of coronary arteries based on Coronary computed tomography angiography, and a wide panel of circulating biomarkers. The absorbed dose is evaluated for the whole heart and its substructures, in particular the coronary arteries. Analysis on occurrence and evolution of subclinical cardiac lesions and biomarkers will be performed and completed with dose-response relationship. Multivariate model of normal tissue complication probability (NTCP) will also be proposed. DISCUSSION: Tools and results developed in the BACCARAT study should allow improving prediction and prevention of potential lesions to cardiac normal tissues surrounding tumors and ultimately enhance patients' care and quality of life. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02605512.

Are Magnetic Resonance Imaging-Transrectal Ultrasound Guided Targeted Biopsies Noninferior to Transrectal Ultrasound Guided Systematic Biopsies for the Detection of Prostate Cancer?

PURPOSE: In men with suspicion of prostate cancer the standard of cancer detection is transrectal ultrasound guided 10 to 12-core systematic biopsy. The targeted biopsy only strategy using magnetic resonance imaging-transrectal ultrasound image registration is gaining in popularity. We assessed the noninferiority of targeted vs systematic biopsy. MATERIALS AND METHODS: Between June and October 2014 a total of 108 biopsy naïve patients with prostate specific antigen between 4 and 20 ng/ml, normal rectal examination and a single suspicious image on magnetic resonance imaging were included in study at 7 centers. Patients underwent systematic biopsy by a first operator blinded to magnetic resonance imaging, immediately followed by 3 targeted biopsies within the suspicious image by a second operator. The primary end point was the cancer detection rate. The noninferiority margin was set at -5%. The secondary end points were the detection rate of clinically significant prostate cancer (maximum cancer core length 5 mm or greater for Gleason 6 or any Gleason 7 or greater disease) and procedure duration. RESULTS: Systematic and targeted biopsies detected cancer in 66 (61.1%) and 61 patients (56.5%), respectively. The mean difference was -4.5% with a 95% CI lower bound of -11.8%. A total of 13 patients with protocol violations were excluded from the per protocol analysis, which showed a mean difference of -5.2% with a 95% CI lower bound of -13.1%. Clinically significant prostate cancer was detected in 50 (46.2%) and 52 patients (48.1%) with systematic and targeted biopsies, respectively (p = 0.69). The mean ± SD duration of image fusion plus targeted biopsy was 16.7 ± 7 minutes vs 7.4 ± 3 for systematic biopsy (p <0.001). CONCLUSIONS: Targeted biopsy seemed to be inferior to systematic biopsy for overall cancer detection. Detection of clinically significant prostate cancer did not differ between targeted and systematic biopsies.

Prostate Imaging Reporting and Data System and Likert Scoring System: Multiparametric MR Imaging Validation Study to Screen Patients for Initial Biopsy.

PURPOSE: To compare the diagnostic performance of the magnetic resonance (MR) imaging-based Prostate Imaging Reporting and Data System (PI-RADS) and a Likert scale in the detection of prostate cancer in a cohort of patients undergoing initial prostate biopsy. MATERIALS AND METHODS: This institutional review board-approved two-center prospective study included 118 patients with normal digital rectal examination (DRE) results but elevated prostate-specific antigen (PSA) levels (4-20 ng/mL) who were referred for initial prostate biopsies and had one suspicious (Likert scale score, ≥3) focus at prebiopsy 1.5-T multiparametric MR imaging performed with T2-weighted, diffusion-weighted [DW], and dynamic contrast material-enhanced imaging. Targeted core biopsies and random systematic core biopsies were performed. The elementary unit for analysis was the core. Relationships were assessed by using the Mann-Whitney U test. Yates corrected and Pearson χ(2) tests were used to evaluate categoric variables. A training set was randomly drawn to construct the receiver operating characteristic curves for the summed PI-RADS scores and for the Likert scale scores. The thresholds to recommend biopsy were obtained from the Youden J statistics and were tested in the remaining validation set in terms of predictive characteristics. Interobserver variability was analyzed by using weighed κ statistics in a random set of 50 patients. RESULTS: Higher T2-weighted, DW, and dynamic contrast-enhanced imaging PI-RADS scores were observed in areas that yielded cancer-positive cores. The percentage of positive cores increased with the sum of scores aggregated in five classes as follows: For summed PI-RADS scores of 3-5, the percentage of positive cores was 2.3%; for scores of 6-8, it was 5.8%; for scores of 9 or 10, it was 24.7%; for scores of 11 or 12, it was 51.8%; and for scores of 13-15, it was 72.1% (P for trend, <.0001). For the threshold of summed PI-RADS scores of 9 or greater, sensitivity was 86.6%, specificity was 82.4%, the positive predictive value was 52.4%, the negative predictive value was 96.5%, and accuracy was 83.2%. The respective data for Likert scale scores of 3 or greater were 93.8%, 73.6%, 44.3%, 98.1%, and 73.3%. Good interobserver agreement was observed for the Likert scale (κ = 0.80) and the summed PI-RADS (κ = 0.73) scoring systems. CONCLUSION: PI-RADS provided the site-specific stratified risk of cancer-positive cores in biopsy-naive men with normal DRE results and elevated PSA levels. There was no significant difference between summed PI-RADS scores of 9 or greater and Likert scale scores of 3 or greater in the detection of cancer in the peripheral zone.

Assessment of the learning curves for photoselective vaporization of the prostate using GreenLight™ 180-Watt-XPS laser therapy: defining the intra-operative parameters within a prospective cohort.

PURPOSE: To assess the learning curves for the intra-operative parameters of the GreenLight™ 180-W XPS for photoselective vaporization of the prostate (PVP). METHODS: A prospective study was conducted on 200 men who underwent PVP using the GreenLight™ 180-W XPS over 20 months. The population was divided into four consecutive equal groups. Evolution of lasing parameters was the main endpoint to reach an average energy of 5 kJ per prostate volume and to reach a lasing time/operative time (LT/OT) ratio of 66-80 %. Changes in the IPSS and prostate volume were also evaluated 12 weeks later. RESULTS: Total energy delivered (energy/ml of prostate) and the LT/OT ratio significantly increased over time (p < 0.05). Urinary function significantly improved from baseline in all groups. The first lasing parameter endpoint was reached after the 75th patient (group 1) and the second endpoint (LT/OT ratio) after the 125th patient (group 3). Only the PSA level (p = 0.04) and prostate volume (p < 0.0001) decreased significantly in the 3rd and the 4th group. Post-operative complications occurred in 20 % of patients, which were primarily Clavien-Dindo grades 1 and 2, though there were no statistical differences between the four groups (p = 0.62). In-hospital stay and time to catheter removal were significantly shorter in the 3rd and 4th group. CONCLUSIONS: The current study assessed the PVP learning curves within multiple intra-operative parameters. The PVP learning curves required at least 120 procedures until it met all intra-operative parameters of experts in this field.

Evaluation of aortic valve stenosis using cardiovascular magnetic resonance: comparison of an original semiautomated analysis of phase-contrast cardiovascular magnetic resonance with Doppler echocardiography.

BACKGROUND: Accurate quantification of aortic valve stenosis (AVS) is needed for relevant management decisions. However, transthoracic Doppler echocardiography (TTE) remains inconclusive in a significant number of patients. Previous studies demonstrated the usefulness of phase-contrast cardiovascular magnetic resonance (PC-CMR) in noninvasive AVS evaluation. We hypothesized that semiautomated analysis of aortic hemodynamics from PC-CMR might provide reproducible and accurate evaluation of aortic valve area (AVA), aortic velocities, and gradients in agreement with TTE. METHODS AND RESULTS: We studied 53 AVS patients (AVA(TTE)=0.87±0.44 cm(2)) and 21 controls (AVA(TTE)=2.96±0.59 cm(2)) who had TTE and PC-CMR of aortic valve and left ventricular outflow tract on the same day. PC-CMR data analysis included left ventricular outflow tract and aortic valve segmentation, and extraction of velocities, gradients, and flow rates. Three AVA measures were performed: AVA(CMR1) based on Hakki formula, AVA(CMR2) based on continuity equation, AVA(CMR3) simplified continuity equation=left ventricular outflow tract peak flow rate/aortic peak velocity. Our analysis was reproducible, as reflected by low interoperator variability (<4.56±4.40%). Comparison of PC-CMR and TTE aortic peak velocities and mean gradients resulted in good agreement (r=0.92 with mean bias=-29±62 cm/s and r=0.86 with mean bias=-12±15 mm Hg, respectively). Although good agreement was found between TTE and continuity equation-based CMR-AVA (r>0.94 and mean bias=-0.01±0.38 cm(2) for AVA(CMR2), -0.09±0.28 cm(2) for AVA(CMR3)), AVA(CMR1) values were lower than AVA(TTE) especially for higher AVA (mean bias=-0.45±0.52 cm(2)). Besides, ability of PC-CMR to detect severe AVS, defined by TTE, provided the best results for continuity equation-based methods (accuracy >94%). CONCLUSIONS: Our PC-CMR semiautomated AVS evaluation provided reproducible measurements that accurately detected severe AVS and were in good agreement with TTE.

Hypertension and aortorenal disease in Alagille syndrome.

Alagille syndrome is a rare congenital multisystem disorder that may involve heart disease and pulmonary or peripheral artery stenosis. We report the clinical and radiological presentation of five adult patients with Alagille syndrome, hypertension and renal artery stenosis. All had systolic hypertension and a narrowing of the abdominal aorta, corresponding to a secondary midaortic syndrome. Renovascular disease progressed during follow-up, with increases in blood pressure, decreases in glomerular filtration rate and/or kidney atrophy. A literature review identified several anecdotal reports of Alagille syndrome associated with hypertension, renal artery stenosis and/or midaortic syndrome. We discuss this condition, focusing on diagnosis, differential diagnosis, associated conditions and management. Cardiologists, nephrologists and radiologists should be aware of this rare cause of renovascular hypertension and of the need for clinical, biological and echographic follow-up.

Combined retrograde stent-graft placement and surgical repair for a Stanford type A aortic dissection.

PURPOSE: To report a case of retrograde acute Stanford type A aortic dissection treated without hypothermic circulatory arrest. CASE REPORT: A 55-year-old man presented with a retrograde acute type A aortic dissection with an entry tear 30 mm below the left subclavian artery. A concurrent emergent endovascular and surgical treatment was performed, excluding the entry tear with retrograde delivery of a stent-graft and replacing the ascending aorta with a Dacron tube without circulatory arrest. CONCLUSION: Avoiding hypothermic circulatory arrest was the main advantage of this hybrid therapeutic choice. This combined technique may be of interest in acute retrograde type A dissections that present complications such as impending rupture or visceral malperfusion. A close collaboration between endovascular specialists and cardiac surgeons is essential for such a hybrid strategy.