Multicentric clinical evaluation of a computed tomography-based fully automated deep neural network for aortic maximum diameter and volumetric measurements.

OBJECTIVE: This study aims to evaluate a fully automatic deep learning-based method (augmented radiology for vascular aneurysm [ARVA]) for aortic segmentation and simultaneous diameter and volume measurements. METHODS: A clinical validation dataset was constructed from preoperative and postoperative aortic computed tomography angiography (CTA) scans for assessing these functions. The dataset totaled 350 computed tomography angiography scans from 216 patients treated at two different hospitals. ARVA's ability to segment the aorta into seven morphologically based aortic segments and measure maximum outer-to-outer wall transverse diameters and compute volumes for each was compared with the measurements of six experts (ground truth) and thirteen clinicians. RESULTS: Ground truth (experts') measurements of diameters and volumes were manually performed for all aortic segments. The median absolute diameter difference between ground truth and ARVA was 1.6 mm (95% confidence interval [CI], 1.5-1.7; and 1.6 mm [95% CI, 1.6-1.7]) between ground truth and clinicians. ARVA produced measurements within the clinical acceptable range with a proportion of 85.5% (95% CI, 83.5-86.3) compared with the clinicians' 86.0% (95% CI, 83.9-86.0). The median volume similarity error ranged from 0.93 to 0.95 in the main trunk and achieved 0.88 in the iliac arteries. CONCLUSIONS: This study demonstrates the reliability of a fully automated artificial intelligence-driven solution capable of quick aortic segmentation and analysis of both diameter and volume for each segment.

4D flow cardiac MRI to assess pulmonary blood flow in patients with pulmonary arterial hypertension associated with congenital heart disease.

PURPOSE: The purpose of this study was to evaluate the accuracy of four-dimensional flow cardiac magnetic resonance imaging (4D flow MRI) compared to right heart catheterization in measuring pulmonary flow (Qp), systemic flow (Qs) and pulmonary-to-systemic flow ratio (Qp/Qs) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). MATERIALS AND METHODS: The study was registered on Clinical-trial.gov (NCT03928002). Sixty-four patients with PAH-CHD who underwent 4D flow MRI were included. There were 16 men and 48 women with a mean age of 45.3 ± 13.7 (standard deviation [SD]) years (age range: 21-77 years). Fifty patients (50/64; 78%) presented with pre-tricuspid shunt. Qp (L/min), Qs (L/min) and Qp/Qs were measured invasively using direct Fick method during right heart catheterization and compared with measurements assessed by 4D flow MRI within a 24-48-hour window. RESULTS: The average mean pulmonary artery pressure was 51 ± 17 (SD) mm Hg with median pulmonary vascular resistance of 8.8 Wood units (Q1, Q3: 5.3, 11.7). A strong linear correlation was found between Qp measurements obtained with 4D flow MRI and those obtained with the Fick method (r = 0.96; P < 0.001). Bland Altman analysis indicated a mean difference of 0.15 ± 0.48 (SD) L/min between Qp estimated by 4D flow MRI and by right heart catheterization. A strong correlation was found between Qs and Qp/Qs measured by 4D flow MRI and those obtained with the direct Fick method (r = 0.85 and r = 0.92; P < 0.001 for both). CONCLUSION: Qp as measured by 4D flow MRI shows a strong correlation with measurements derived from the direct Fick method. Further investigation is needed to develop less complex and standardized methods for measuring essential PAH parameters, such as pulmonary arterial pressures and pulmonary vascular resistance.

Evaluación multiparamétrica de la función ventricular derecha en la hipertensión arterial pulmonar asociada a cardiopatías congénitas / Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease

Introducción y objetivos El pronóstico de los pacientes con cardiopatía congénita e hipertensión arterial pulmonar (HTP) está estrechamente relacionado con la función del ventrículo derecho (VD). Los parámetros de ecocardiografía bidimensional como la deformación miocárdica o el índice de remodelado VD telesistólico (IRVDTS) han surgido para cuantificar la función del VD. Métodos Se estudió de forma prospectiva a 30 paciente de edad 48± 12 años con shunt pretricuspídeo e HTP para investigar la precisión de diversos parámetros ecocardiográficos de estudio de la función del VD (extrusión sistólica del anillo tricúspide, velocidad sistólica pico del anillo tricúspide, ratio de duración sistodiastólica del VD, área de la aurícula derecha, cambio de área fraccional del ventrículo derecho, deformación miocárdica global del ventrículo derecho e IRVDTS) respecto a la fracción de eyección del ventrículo derecho medida por resonancia cardiaca. Resultados En 13 pacientes (43,3%) se observó una fracción de eyección del VD <45%. La deformación longitudinal global del VD (ρ [coeficiente de correlación de Spearman] = −0,75, p=0,001; R2=0,58, p=0,001), área de la aurícula derecha (ρ=−0,74, p <0,0001; R2=0,56, p <0,0001); IRVDTS (ρ=−0,64, p < 0,0001; R2=0,47, p <0,0001), ratio de duración sistodiastólica (ρ=−0,62, p=0,0004; R2=0,47, p <0,0001) y cambio de área fraccional del VD (ρ=0,48, p=0,01; R2=0,37, p <0,0001) se correlacionaron con la fracción de eyección del VD. La deformación longitudinal global del VD, IRVDTS y área de la aurícula derecha fueron predictores de fracción de eyección <45% con la mayor área bajo la curva (0,88; IC95%, 0,71-1,00; 0,88; IC95%, 0,76-1,00 y 0,89; IC95%, 0,77-1,00, respectivamente). Una deformación longitudinal global del VD> −16%, IRVDTS ≥ 1,7 y un área de la aurícula derecha ≥ 22 cm2 predijeron una fracción de eyección del VD <45% con una sensibilidad y especificidad del 87,5 y 85,7%; 76,9 y 88,3%; 92,3 y 82,4%, respectivamente (AU)

Introduction and objectives Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardiographic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function. Methods We prospectively studied 30 patients aged 48±12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance. Results RV ejection fraction <45% was observed in 13 patients (43.3%). RV global longitudinal strain (ρ [Spearman's correlation coefficient]=−0.75; P=.001; R2=0.58; P=.001), right atrium area (ρ=−0.74; P <.0001; R2=0.56; P <.0001), RVESRI (ρ=−0.64; P <.0001; R2=0.47; P <.0001), systolic-to-diastolic duration ratio (ρ=−0.62; P=.0004; R2=0.47; P <.0001) and RV fractional area change (ρ=0.48; P=.01; R2=0.37; P <.0001) were correlated with RV ejection fraction. RV global longitudinal strain, RVESRI and right atrium area predicted RV ejection fraction <45% with the greatest area under curve (0.88; 95%CI, 0.71-1.00; 0.88; 95%CI, 0.76-1.00, and 0.89; 95%CI, 0.77-1.00, respectively). RV global longitudinal strain >−16%, RVESRI ≥ 1.7 and right atrial area ≥ 22 cm2 predicted RV ejection fraction <45% with a sensitivity and specificity of 87.5% and 85.7%; 76.9% and 88.3%; 92.3% and 82.4%, respectively. Conclusions RVESRI, right atrial area and RV global longitudinal strain are strong markers of RV dysfunction in patients with pretricuspid shunt and PAH (AU)

Fusión de imágenes de tomografía computarizada cardiaca y ecocardiografía: un nuevo enfoque en las cardiopatías congénitas / Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease

Introducción y objetivos: El diagnóstico y manejo terapéutico de pacientes con cardiopatías congénitas se basa en gran medida en los resultados de la ecocardiografía. Las diferentes técnicas de fusión de imagen han supuesto un reciente avance en la obtención de imágenes cardíacas. Nuestro objetivo ha sido el de evaluar la viabilidad de la fusión de la tomografía computarizada (TC) y la ecocardiografía transtorácica (ETT) 3D en niños y adultos con cardiopatías congénitas. Métodos: Se incluyeron de forma prospectiva 13 pacientes con cardiopatías congénitas y 1 paciente con corazón normal los cuales se sometieron a TC y ETT 3D como parte de su seguimiento habitual. Se describieron los pasos necesarios para completar el proceso de fusión (alineación, puntos de referencia y superposición), la navegación y la evaluación de las imágenes. Resultados: La edad media fue de 9,5 años [2,7-15,7], el 57% eran varones y la superficie corporal media fue de 0,9 m2 [0,6-1,7]. Se clasificaron las cardiopatías como simples (n=4, 29%), moderadas (n=4, 29%) o complejas (n=6, 42%). La fusión 3D ETT-TC fue exitosa en todos los pacientes. La mediana del tiempo total para completar el proceso de fusión fue de 735 segundos [628-1163], sin diferencias significativas según el grado de complejidad de la cardiopatía. Los puntos de referencia se modificaron significativamente en las cardiopatías congénitas complejas. Conclusiones: La técnica de fusión 3D ETT-TC en una población de niños y adultos con diversas cardiopatías congénitas es viable y precisa según demostramos. La visualización simultánea de diferentes estructuras cardíacas podría ayudar a comprender las características anatómicas de las cardiopatías congénitas sin limitaciones en cuanto a la edad, el peso o la complejidad.(AU)

Introduction and objectives: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. Methods: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. Results: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. Conclusions: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.(AU)

Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease.

INTRODUCTION AND OBJECTIVES: Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardiographic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function. METHODS: We prospectively studied 30 patients aged 48±12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance. RESULTS: RV ejection fraction <45% was observed in 13 patients (43.3%). RV global longitudinal strain (ρ [Spearman's correlation coefficient]=-0.75; P=.001; R2=0.58; P=.001), right atrium area (ρ=-0.74; P <.0001; R2=0.56; P <.0001), RVESRI (ρ=-0.64; P <.0001; R2=0.47; P <.0001), systolic-to-diastolic duration ratio (ρ=-0.62; P=.0004; R2=0.47; P <.0001) and RV fractional area change (ρ=0.48; P=.01; R2=0.37; P <.0001) were correlated with RV ejection fraction. RV global longitudinal strain, RVESRI and right atrium area predicted RV ejection fraction <45% with the greatest area under curve (0.88; 95%CI, 0.71-1.00; 0.88; 95%CI, 0.76-1.00, and 0.89; 95%CI, 0.77-1.00, respectively). RV global longitudinal strain >-16%, RVESRI ≥ 1.7 and right atrial area ≥ 22 cm2 predicted RV ejection fraction <45% with a sensitivity and specificity of 87.5% and 85.7%; 76.9% and 88.3%; 92.3% and 82.4%, respectively. CONCLUSIONS: RVESRI, right atrial area and RV global longitudinal strain are strong markers of RV dysfunction in patients with pretricuspid shunt and PAH.

Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease.

INTRODUCTION AND OBJECTIVES: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease. METHODS: We prospectively included 14 patients, 13 of whom had congenital heart disease, and who underwent both CT and 3D TTE as part of their usual follow-up. We described the steps required to complete the fusion process (alignment, landmarks, and superimposition), navigation, and image evaluation. RESULTS: Median age was 9.5 [2.7-15.7] years, 57% were male, and median body surface area was 0.9 m2 [0.6-1.7]. Congenital heart disease was classified as simple (n=4, 29%), moderate (n=4, 29%), or complex (n=6, 42%). 3D TTE-CT fusion was successful in all patients. Median total time to complete the fusion process was 735 [628-1163] seconds, with no significant difference according to the degree of complexity of the defects. Landmarks were significantly modified in complex congenital heart disease. CONCLUSIONS: We established the feasibility and accuracy of 3D TTE-CT fusion in a population of children and adults with a variety of congenital heart diseases. The simultaneous visualization of many intracardiac structures may help to understand the anatomical features of congenital heart disease without limitations regarding age, weight, or complexity of the congenital defects.

Unexpected Cardiotoxicity in Patients With HER2-Mutant NSCLC Treated With Trastuzumab Deruxtecan: A Case Report.

Antibody-drug conjugates targeting receptor tyrosine-protein kinase erbB-2 (ERBB2, HER2) have emerged as promising targeted options for HER2-mutant NSCLC. Among antibody-drug conjugates targeting HER2, trastuzumab deruxtecan was found to have the most impressive efficacy and is a potential new standard of care. Drug-related interstitial lung disease remains a serious unpredictable identified risk for patients treated with trastuzumab deruxtecan, requiring careful monitoring and multidisciplinary management. We report the first two cases of drug-related cardiotoxicity with acute myocarditis that developed after the first trastuzumab deruxtecan cycle. Routine cardiovascular risk screening is advisable, with close collaboration between cardiology specialists and oncologists.

4D flow cardiovascular magnetic resonance recovery profiles following pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension.

BACKGROUND: Four-dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) allows comprehensive assessment of pulmonary artery (PA) flow dynamics. Few studies have characterized longitudinal changes in pulmonary flow dynamics and right ventricular (RV) recovery following a pulmonary endarterectomy (PEA) for patients with chronic thromboembolic pulmonary hypertension (CTEPH). This can provide novel insights of RV and PA dynamics during recovery. We investigated the longitudinal trajectory of 4D flow metrics following a PEA including velocity, vorticity, helicity, and PA vessel wall stiffness. METHODS: Twenty patients with CTEPH underwent pre-PEA and > 6 months post-PEA CMR imaging including 4D flow CMR; right heart catheter measurements were performed in 18 of these patients. We developed a semi-automated pipeline to extract integrated 4D flow-derived main, left, and right PA (MPA, LPA, RPA) volumes, velocity flow profiles, and secondary flow profiles. We focused on secondary flow metrics of vorticity, volume fraction of positive helicity (clockwise rotation), and the helical flow index (HFI) that measures helicity intensity. RESULTS: Mean PA pressures (mPAP), total pulmonary resistance (TPR), and normalized RV end-systolic volume (RVESV) decreased significantly post-PEA (P < 0.002). 4D flow-derived PA volumes decreased (P < 0.001) and stiffness, velocity, and vorticity increased (P < 0.01) post-PEA. Longitudinal improvements from pre- to post-PEA in mPAP were associated with longitudinal decreases in MPA area (r = 0.68, P = 0.002). Longitudinal improvements in TPR were associated with longitudinal increases in the maximum RPA HFI (r=-0.85, P < 0.001). Longitudinal improvements in RVESV were associated with longitudinal decreases in MPA fraction of positive helicity (r = 0.75, P = 0.003) and minimum MPA HFI (r=-0.72, P = 0.005). CONCLUSION: We developed a semi-automated pipeline for analyzing 4D flow metrics of vessel stiffness and flow profiles. PEA was associated with changes in 4D flow metrics of PA flow profiles and vessel stiffness. Longitudinal analysis revealed that PA helicity was associated with pulmonary remodeling and RV reverse remodeling following a PEA.

Cardiac functional imaging.

During the last 20 years, cardiac imaging has drastically evolved. Positron emission tomography (PET), fast three-dimensional (3D) imaging with the latest generations of echocardiography & multi-detector computed tomography (CT), stress perfusion assessed by magnetic resonance imaging (MRI), blood flow analysis using four-dimensional (4D) flow MRI, all these techniques offer new trends for optimal noninvasive functional cardiac imaging. Dynamic functional imaging is obtained by acquiring images of the heart at different phases of the cardiac cycle, allowing assessment of cardiac motion, function, and perfusion. Between CT and Cardiac MRI (CMR), CMR has the best temporal resolution, which is suitable for functional imaging while cardiac CT provides higher spatial resolution with isotropic data that have an identical resolution in the three dimensions of the space. The latest generations of CT scanners enable whole heart assessment in one beat, offering also an acceptable temporal resolution with the possibility to display the images in a dynamic mode. Another rapidly growing technique using functional and molecular imaging for the assessment of biological and metabolic pathways is the PET using radio-labeled tracers. Meanwhile, the oldest cardiac imaging tool with doppler ultrasound technology has never stopped evolving. Echocardiography today performs 3D imaging, stress perfusion, and myocardial strain assessment, with high temporal resolution. It still is the first line and more accessible exam for the patient. These different modalities are complementary and may be even combined into PET-CT or PET-MRI. The ability to combine the functional/molecular data with anatomical images may implement a new dimension to our diagnostic tools.

Myocardial involvement in children with post-COVID multisystem inflammatory syndrome: a cardiovascular magnetic resonance based multicenter international study-the CARDOVID registry.

BACKGROUND: Recent evidence shows an association between coronavirus disease 2019 (COVID-19) infection and a severe inflammatory syndrome in children. Cardiovascular magnetic resonance (CMR) data about myocardial injury in children are limited to small cohorts. The aim of this multicenter, international registry is to describe clinical and cardiac characteristics of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 using CMR so as to better understand the real extent of myocardial damage in this vulnerable cohort. METHODS AND RESULTS: Hundred-eleven patients meeting the World Health Organization criteria for MIS-C associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), having clinical cardiac involvement and having received CMR imaging scan were included from 17 centers. Median age at disease onset was 10.0 years (IQR 7.0-13.8). The majority of children had COVID-19 serology positive (98%) with 27% of children still having both, positive serology and polymerase chain reaction (PCR). CMR was performed at a median of 28 days (19-47) after onset of symptoms. Twenty out of 111 (18%) patients had CMR criteria for acute myocarditis (as defined by the Lake Louise Criteria) with 18/20 showing subepicardial late gadolinium enhancement (LGE). CMR myocarditis was significantly associated with New York Heart Association class IV (p = 0.005, OR 6.56 (95%-CI 1.87-23.00)) and the need for mechanical support (p = 0.039, OR 4.98 (95%-CI 1.18-21.02)). At discharge, 11/111 (10%) patients still had left ventricular systolic dysfunction. CONCLUSION: No CMR evidence of myocardial damage was found in most of our MIS-C cohort. Nevertheless, acute myocarditis is a possible manifestation of MIS-C associated with SARS-CoV-2 with CMR evidence of myocardial necrosis in 18% of our cohort. CMR may be an important diagnostic tool to identify a subset of patients at risk for cardiac sequelae and more prone to myocardial damage. CLINICAL TRIAL REGISTRATION: The study has been registered on ClinicalTrials.gov, Identifier NCT04455347, registered on 01/07/2020, retrospectively registered.

Pre-surgical and Post-surgical Aortic Aneurysm Maximum Diameter Measurement: Full Automation by Artificial Intelligence.

OBJECTIVE: The aim of this study was to evaluate an automatic, deep learning based method (Augmented Radiology for Vascular Aneurysm [ARVA]), to detect and assess maximum aortic diameter, providing cross sectional outer to outer aortic wall measurements. METHODS: Accurate external aortic wall diameter measurement is performed along the entire aorta, from the ascending aorta to the iliac bifurcations, on both pre- and post-operative contrast enhanced computed tomography angiography (CTA) scans. A training database of 489 CTAs was used to train a pipeline of neural networks for automatic external aortic wall measurements. Another database of 62 CTAs, including controls, aneurysmal aortas, and aortic dissections scanned before and/or after endovascular or open repair, was used for validation. The measurements of maximum external aortic wall diameter made by ARVA were compared with those of seven clinicians on this validation dataset. RESULTS: The median absolute difference with respect to expert's measurements ranged from 1 mm to 2 mm among all annotators, while ARVA reported a median absolute difference of 1.2 mm. CONCLUSION: The performance of the automatic maximum aortic diameter method falls within the interannotator variability, making it a potentially reliable solution for assisting clinical practice.

Interventricular septum mass presenting as a late acute coronary syndrome with ST-segment elevation: a case report.

BACKGROUND: Intracardiac masses are relatively rare but the diagnosis can be challenging for the cardiologist and the clinical presentation can be misleading. While most of the cardiac masses are benign, malignant masses are mostly metastatic tumours. CASE SUMMARY: An 81-year-old man was admitted to the cardiology department for congestive heart failure with the complaint of recent dyspnoea. The initial electrocardiogram was suggestive of a late presentation of an anterior myocardial infarction. Blood test showed mild and stable elevation of troponin and brain natriuretic peptide. Doppler-echocardiography revealed an interventricular septal thickening. Contrast echocardiography revealed a mass with a possibly necrotic centre and peripheral hypervascularization. Cardiac computed tomography (CT) confirmed the existence of a cardiac tumour with a hypodense centre and also revealed the presence of a large tumour of the lung's left lower lobe with multiple enlarged lymph nodes associated with possible left adrenal gland metastasis. Computed tomography-guided percutaneous biopsy of the pulmonary mass demonstrated a squamous cell lung cancer which was likely the primary cancer. The patient was discharged home waiting for chemotherapy to start but died a few days later at home of an unknown cause. DISCUSSION: Diagnosis of intracardiac mass is difficult, often requiring multiple imaging modalities. Contrast-enhanced echocardiography may help early diagnosis and can be easily implemented with other imaging modalities such as cardiac magnetic resonance imaging or CT.

Male Sex Is Associated With Cervical Artery Dissection in Patients With Fibromuscular Dysplasia.

Background Cervical artery dissection (CeAD) is a frequent manifestation of fibromuscular dysplasia (FMD). However, risk factors for CeAD are unknown. We investigated factors associated with CeAD in the ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry. Methods and Results The ARCADIA registry includes women or men aged ≥18 years, with a diagnosis of renal, cervical, or intracranial artery FMD, who were prospectively recruited at 16 university hospitals in France and Belgium. Diagnosis of acute or past CeAD at inclusion was established on imaging according to standard diagnostic criteria. Associations between potential determinants and CeAD were assessed by logistic regression analyses. Among 469 patients (75 men) with FMD, 65 (13.9%) had CeAD. Patients with CeAD were younger, more likely to be men, have a history of migraine, and less likely to have a history of hypertension than patients without CeAD. In the multivariable analysis, male sex (odds ratio [OR], 2.66; 95% CI, 1.34-5.25), history of migraine (OR, 1.90; 95% CI, 1.06-3.39), age ≥50 years (OR, 0.41; 95% CI, 0.23-0.73), history of hypertension (OR, 0.35; 95% CI, 0.20-0.64), and involvement of ≥3 vascular beds (OR, 2.49; 95% CI, 1.15-5.40) were significantly associated with CeAD. To validate the association between CeAD and sex, we performed a systematic review. We collected additional data on sex from 2 published studies and unpublished data from the US Registry for Fibromuscular Dysplasia and the European/International FMD Registry. In the pooled analysis (289 CeAD, 1933 patients), male sex was significantly associated with CeAD (OR, 2.04; 95% CI, 1.41-2.95; I2=0%). Conclusions In patients with FMD, male sex and multisite involvement are associated with CeAD, in addition to other previously known risk factors. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT02884141.

Renal Outcome and New-Onset Renal and Extrarenal Dissections in Patients With Nontrauma Renal Artery Dissection Associated With Renal Infarction.

[Figure: see text].

Rare loss-of-function mutations of PTGIR are enriched in fibromuscular dysplasia.

AIMS: Fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection (SCAD) are related, non-atherosclerotic arterial diseases mainly affecting middle-aged women. Little is known about their physiopathological mechanisms. We aimed to identify rare genetic causes to elucidate molecular mechanisms implicated in FMD and SCAD. METHODS AND RESULTS: We analysed 29 exomes that included familial and sporadic FMD. We identified one rare loss-of-function variant (LoF) (frequencygnomAD = 0.000075) shared by two FMD sisters in the prostaglandin I2 receptor gene (PTGIR), a key player in vascular remodelling. Follow-up was conducted by targeted or Sanger sequencing (1071 FMD and 363 SCAD patients) or lookups in exome (264 FMD) or genome sequences (480 SCAD), all independent and unrelated. It revealed four additional LoF allele carriers, in addition to several rare missense variants, among FMD patients, and two LoF allele carriers among SCAD patients, including one carrying a rare splicing mutation (c.768 + 1C>G). We used burden test to test for enrichment in patients compared to gnomAD controls, which detected a putative enrichment in FMD (PTRAPD = 8 × 10-4), but not a significant enrichment (PTRAPD = 0.12) in SCAD. The biological effects of variants on human prostaclycin receptor (hIP) signalling and protein expression were characterized using transient overexpression in human cells. We confirmed the LoFs (Q163X and P17RfsX6) and one missense (L67P), identified in one FMD and one SCAD patient, to severely impair hIP function in vitro. CONCLUSIONS: Our study shows that rare genetic mutations in PTGIR are enriched among FMD patients and found in SCAD patients, suggesting a role for prostacyclin signalling in non-atherosclerotic stenosis and dissection.

Four-dimensional Flow MRI: Principles and Cardiovascular Applications.

In-plane phase-contrast (PC) imaging is now a routine component of MRI of regional blood flow in the heart and great vessels. In-plane PC MRI provides a volumetric, isotropic, time-resolved cine sequence that enables three-directional velocity encoding, a technique known as four-dimensional (4D) flow MRI. Recent advances in 4D flow MRI have shortened imaging times, while progress in big-data processing has improved dataset pre- and postprocessing, thereby increasing the feasibility of 4D flow MRI in clinical practice. Important technical issues include selection of the optimal velocity-encoding sensitivity before acquisition and preprocessing of the raw data for phase-offset corrections. Four-dimensional flow MRI provides unprecedented capabilities for comprehensive analysis of complex blood flow patterns using new visualization tools such as streamlines and velocity vectors. Retrospective multiplanar navigation enables flexible retrospective flow quantification through any plane across the volume with good accuracy. Current flow parameters include forward flow, reverse flow, regurgitation fraction, and peak velocity. Four-dimensional flow MRI also supplies advanced flow parameters of use for research, such as wall shear stress. The vigorous burgeoning of new applications indicates that 4D flow MRI is becoming an important imaging modality for cardiovascular disorders. This article reviews the main technical issues of 4D flow MRI and the different parameters provided by it and describes the main applications in cardiovascular diseases, including congenital heart disease, cardiac valvular disease, aortic disease, and pulmonary hypertension. Online supplemental material is available for this article. ©RSNA, 2019 See discussion on this article by Ordovas .

Presumption of pericardial endometriosis using MRI: Case report and review of the literature.

Endometriosis is a condition where hormonal-responsive endometrial tissue grows outside the uterus usually within the pelvic cavity. Extra-pelvic endometriosis is rare and may involve the pericardium. We report the first case of pericardial endometriosis suspected using cardiac magnetic resonance imaging.