Recommendations for cardiovascular magnetic resonance and computed tomography in congenital heart disease: a consensus paper from the CMR/CCT working group of the Italian Society of Pediatric Cardiology (SICP) and the Italian College of Cardiac Radiology endorsed by the Italian Society of Medical and Interventional Radiology (SIRM) Part I.

Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. Nevertheless, correct execution and interpretation require in-depth knowledge of all technical and clinical aspects of CHD, a careful assessment of risks and benefits before each exam, proper imaging protocols to maximize diagnostic information, minimizing harm. This position paper, written by experts from the Working Group of the Italian Society of Pediatric Cardiology and from the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, is intended as a practical guide for applying CCT and CMR in children and adults with CHD, wishing to support Radiologists, Pediatricians, Cardiologists and Cardiac Surgeons in the multimodality diagnostic approach to these patients. The first part provides a review of the most relevant literature in the field, describes each modality's advantage and drawback, making considerations on the main applications, image quality, and safety issues. The second part focuses on clinical indications and appropriateness criteria for CMR and CCT, considering the level of CHD complexity, the clinical and logistic setting and the operator expertise.

Corona mortis variant of the obturator artery: a systematic study of 300 hemipelvises by means of computed tomography angiography.

PURPOSE: The purpose of the present study is to systematically assess the prevalence and characteristics of the corona mortis (CM) variant of the obturator artery by means of computerised tomography angiography (CTA). MATERIAL AND METHODS: A total of 150 consecutive patients (112 males, average age 73 years) referred to CTA for lower limb arterial evaluation were included in this retrospective study. Patient demographics, anastomosis incidence, artery diameter, distance from the symphysis pubis, Kellgren-Lawrence score, and pelvic size were evaluated. RESULTS: In this study 40.6% of patients presented at least one competent CM arterial anastomosis. In the 300 evaluated arteries, 90 arterial anastomoses were found (30%), 40 on the right side (average diameter 1.63 mm) and 50 on the left side (1.78 mm). In 32 cases there was only one anastomosis, while in 29 cases the CM was bilateral. No anastomoses were detected in 89 patients. Mean distance from the symphysis was 50 mm. No relevant association with hip osteoarthrosis was found for CM. Mean pelvic size was 213 mm. CONCLUSIONS: The evidence from this study suggests that CM is a common variant that needs to be acknowledged before pelvic intervention.

Endovascular Repair of an Unusually Complex Anastomotic Pseudoaneurysm of an Aorto-Bisiliac Graft.

BACKGROUND: Anastomotic pseudoaneurysm is an underestimated complication of aorto-iliac grafts. CASE REPORT: This case report describes an unusual presentation of a pseudoaneurysm with a particularly complex anatomy involving both the left iliac branches, which hindered the interpretation of diagnostic studies and therapeutic management in a patient with multiple comorbidities. CONCLUSIONS: The manuscript describes a successful management of such a complication by means of an elective endovascular approach.

Successful Treatment of Resistant Hypertension by Means of Chronic Renal Artery Occlusion Revascularization in a Fragile Patient.

BACKGROUND: Renal artery stenosis is a common cause of secondary hypertension refractory to medical therapy. Percutaneous angioplasty along with metallic stent placement has been described as an effective treatment for revascularization of the renal artery. CASE REPORT: A 54-year-old woman affected by paranoid schizophrenia with a history of hypertension and chronic renal failure due to renal artery occlusion was treated by endovascular recanalization and stent placement that resulted in improvement of renal function and control of hypertension. Laboratory studies 4 months after the revascularization revealed blood creatinine decrease gradually from 8.57 mg/dL to 3 mg/dL. CONCLUSIONS: Revascularization with stenting has proven to be a safe and effective procedure in the treatment of total renal artery occlusion which also led to a significant relief at a psychological level.

Treatment of popliteal artery aneurysms by means of cryopreserved homograft.

BACKGROUND: Autologous saphenous vein is considered the gold standard conduit in the femoral-popliteal revascularization for popliteal artery aneurysms (PAAs). In several cases, it may be absent or unsuitable for length or diameter and so it may be considered unfit for a conduit. In such patients, a synthetic graft or the endovascular correction can be useful, but results are controversial. In this retrospective case series, we have analyzed the safety and efficacy of the cryopreserved homograft (CHg) as a conduit in the PAA revascularization. METHODS: In the period between January 2005 and December 2013, 54 PAAs have been treated with an arterial CHg. Indications to surgery were asymptomatic aneurysm with >25-mm diameter in 30 cases (55.6%), ischemic symptoms in 20 (37%), and compressive symptoms in 4 (7.4%). An urgent treatment was performed in 8 cases (14.8%) for severe ischemic symptoms. All cases have been operated by the same vascular team: a posterior surgical approach was preferred whenever possible (33 cases, 61.1%). All patients received a clinical and radiologic follow-up, and all data were collected in a specific database. A >10-mm diameter increase of the conduit was considered significant for an aneurysmatic degeneration. RESULTS: The primary outcomes were complications and reoperation rate, limb salvage, and primary and secondary patency. Secondary outcome was the incidence of aneurysmatic degeneration of the CHg. Mean diameter of the homografts was 6.3 mm (range, 4-8 mm). In the 30-day postoperative period, 7 PAAs (13%) developed a complication: 3 wound infections, 3 graft thrombosis, and 1 anastomotic bleeding. Six cases needed a reoperation: 2 surgical revascularizations, 2 wound debridement, and 1 anastomotic hemostasis; 1 major amputation was necessary for graft failure after the revascularization. Mean follow-up was 34.8 months (range, 1-96). Five cases (9.3%) developed a graft complication with 4 reoperations necessary (7.4%): 2 grafts required a percutaneous transluminal angioplasty for a perigraft stenosis and 2 underwent a femorodistal bypass in CHg for severe ischemic symptoms after CHg occlusion. Another one CHg occlusion was treated conservatively because it was clinically asymptomatic. Primary patency was 96.3%, 93.9%, and 88.3% at 12, 36, and 60 months, respectively; secondary patency was 98.1% at 12, 36, and 60 months. Freedom from amputation resulted in 98.1%. No aneurysmatic degeneration was observed. Univariate and multivariate analysis showed urgent surgery to be independent risk factors for complications, reoperations, and CHg occlusion (P < 0.05). CONCLUSIONS: In this retrospective study, the use of CHg showed to be safe and effective in the surgery of PAAs, either in the short or in the long-term follow-up. Urgent surgery can be considered predictive of graft failure. In our experience, CHg can be considered a good alternative conduit to the autologous saphenous vein.

Image-Based Computational Fluid Dynamics to Compare Two Repair Techniques for Mitral Valve Prolapse.

OBJECTIVE : The treatment of mitral valve prolapse involves two distinct repair techniques: chordal replacement (Neochordae technique) and leaflet resection (Resection technique). However, there is still a debate in the literature about which is the optimal one. In this context, we performed an image-based computational fluid dynamic study to evaluate blood dynamics in the two surgical techniques. METHODS : We considered a healthy subject (H) and two patients (N and R) who underwent surgery for prolapse of the posterior leaflet and were operated with the Neochordae and Resection technique, respectively. Computational Fluid Dynamics (CFD) was employed with prescribed motion of the entire left heart coming from cine-MRI images, with a Large Eddy Simulation model to describe the transition to turbulence and a resistive method for managing valve dynamics. We created three different virtual scenarios where the operated mitral valves were inserted in the same left heart geometry of the healthy subject to study the differences attributed only to the two techniques. RESULTS : We compared the three scenarios by quantitatively analyzing ventricular velocity patterns and pressures, transition to turbulence, and the ventricle ability to prevent thrombi formation. From these results, we found that the operative techniques affected the ventricular blood dynamics in different ways, with variations attributed to the reduced mobility of the Resection posterior leaflet. Specifically, the Resection technique resulted in turbulent forces, related with the risk of hemolysis formation, up to 640 Pa, while the other two scenarios exhibited a maximum of 240 Pa. Moreover, in correspondence of the ventricular apex, the Resection technique reduced the areas with low velocity to 15%, whereas the healthy case and the Neochordae case maintained these areas at 30 and 48%, respectively. Our findings suggest that the Neochordae technique developed a more physiological flow with respect to the Resection technique. CONCLUSION: Resection technique gives rise to a different direction of the mitral jet during diastole increasing the ability to washout the ventricular apex preventing from thrombi formation, but at the same time it promotes turbulence formation that is associated with ventricular effort and risk of hemolysis.

Recommendations for cardiovascular magnetic resonance and computed tomography in congenital heart disease: a consensus paper from the CMR/CCT Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology endorsed by the Italian Society of Medical and Interventional Radiology (Part II).

Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. This is the second of two complementary documents, endorsed by experts from the Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, aimed at giving updated indications on the appropriate use of CMR and CCT in different clinical CHD settings, in both pediatrics and adults. In this article, support is also given to radiologists, pediatricians, cardiologists, and cardiac surgeons for indications and appropriateness criteria for CMR and CCT in the most referred CHD, following the proposed new criteria presented and discussed in the first document. This second document also examines the impact of devices and prostheses for CMR and CCT in CHD and additionally presents some indications for CMR and CCT exams when sedation or narcosis is needed.

Guidelines on the diagnosis, treatment and management of visceral and renal arteries aneurysms: a joint assessment by the Italian Societies of Vascular and Endovascular Surgery (SICVE) and Medical and Interventional Radiology (SIRM).

The objective of these Guidelines is to provide recommendations for the classification, indication, treatment and management of patients suffering from aneurysmal pathology of the visceral and renal arteries. The methodology applied was the GRADE-SIGN version, and followed the instructions of the AGREE quality of reporting checklist. Clinical questions, structured according to the PICO (Population, Intervention, Comparator, Outcome) model, were formulated, and systematic literature reviews were carried out according to them. Selected articles were evaluated through specific methodological checklists. Considered Judgments were compiled for each clinical question in which the characteristics of the body of available evidence were evaluated in order to establish recommendations. Overall, 79 clinical practice recommendations were proposed. Indications for treatment and therapeutic options were discussed for each arterial district, as well as follow-up and medical management, in both candidate patients for conservative therapy and patients who underwent treatment. The recommendations provided by these guidelines simplify and improve decision-making processes and diagnostic-therapeutic pathways of patients with visceral and renal arteries aneurysms. Their widespread use is recommended.

An Image-Based Computational Fluid Dynamics Study of Mitral Regurgitation in Presence of Prolapse.

PURPOSE: In this work we performed an imaged-based computational study of the systolic fluid dynamics in presence of mitral valve regurgitation (MVR). In particular, we compared healthy and different regurgitant scenarios with the aim of quantifying different hemodynamic quantities. METHODS: We performed computational fluid dynamic (CFD) simulations in the left ventricle, left atrium and aortic root, with a resistive immersed method, a turbulence model, and with imposed systolic wall motion reconstructed from Cine-MRI images, which allowed us to segment also the mitral valve. For the regurgitant scenarios we considered an increase of the heart rate and a dilation of the left ventricle. RESULTS: Our results highlighted that MVR gave rise to regurgitant jets through the mitral orifice impinging against the atrial walls and scratching against the mitral valve leading to high values of wall shear stresses (WSSs) with respect to the healthy case. CONCLUSION: CFD with prescribed wall motion and immersed mitral valve revealed to be an effective tool to quantitatively describe hemodynamics in case of MVR and to compare different regurgitant scenarios. Our findings highlighted in particular the presence of transition to turbulence in the atrium and allowed us to quantify some important cardiac indices such as cardiac output and WSS.

Turbulent blood dynamics in the left heart in the presence of mitral regurgitation: a computational study based on multi-series cine-MRI.

In this work, we performed a computational image-based study of blood dynamics in the whole left heart, both in a healthy subject and in a patient with mitral valve regurgitation. We elaborated multi-series cine-MRI with the aim of reconstructing the geometry and the corresponding motion of left ventricle, left atrium, mitral and aortic valves, and aortic root of the subjects. This allowed us to prescribe such motion to computational blood dynamics simulations where, for the first time, the whole left heart motion of the subject is considered, allowing us to obtain reliable subject-specific information. The final aim is to investigate and compare between the subjects the occurrence of turbulence and the risk of hemolysis and of thrombi formation. In particular, we modeled blood with the Navier-Stokes equations in the arbitrary Lagrangian-Eulerian framework, with a large eddy simulation model to describe the transition to turbulence and a resistive method to manage the valve dynamics, and we used a finite element discretization implemented in an in-house code for the numerical solution.

Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Background: Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized. Design: The CMR/CT WG of the Italian Pediatric Cardiology Society set up a multicenter observational clinical database of repaired TOF evaluations. This registry will enroll patients retrospectively and prospectively assessed by CMR for clinical indication in many congenital heart diseases (CHD) Italian centers. Data collection in a dedicated platform will include surgical history, clinical data, imaging data, and adverse cardiac events at 6 years of follow-up. Summary: The multicenter repaired TOF clinical database will collect data on patients evaluated by CMR in many CHD centers in Italy. The registry has been set up to allow future research studies in this population to improve clinical/surgical management and risk stratification of this population.

Pilot clinical study on the prevention of complications after lung biopsy by the MIPP kit PNX device.

Background: Pneumothorax (PNX), pulmonary hemorrhage, hemothorax and chest wall hematoma are the most commonly reported complications of percutaneous lung biopsy (PLB). Sealing the biopsy tract with different types of materials is an emerging way to prevent PLB complications. Methods: To investigate the safety and efficacy of a new device, Minimally Invasive Percutaneous Procedure Kit for Pneumothorax (MIPP-Kit PNX), when used in association with a resorbable bio-compatible glue in the prevention of PLB complications. A prospective, multicenter, open-label, single-arm study was performed to evaluate the complication rate after glue administration by the new investigational device during PLBs. Results: Fourty-three patients were enrolled after informed consent signature (40 underwent PLB, while three were screening failures). Only 3 patients (7.5%, 95% CI: 0.0-15.7%) developed complications within 48 h after glue injection during PLB: two developed minor pneumothoraces and one a pulmonary hemorrhage. No patients who showed procedural complications before glue administration were reported with any recurrent or new complications after glue administration. Conclusions: In comparison with the data reported in the literature, this trial results support the safe and effective use of the MIPP kit PNX in the prevention of PLB complications. These promising preliminary results warrant further confirmation in larger clinical trials. Trial Registration: ClinicalTrials.gov identifier: NCT04071509.

Aortic root physiology late after a "perfect" ross operation: magnetic resonance imaging study of three operative techniques.

In order to define physiological properties of the autograft root, magnetic resonance imaging (MRI) findings relative to three different operative techniques were compared with those of control subjects. Twenty-three patients, 18/5 M/F, aged 32 ± 9 years, underwent MRI assessment of the aortic root. Patients with normally functioning autograft valve and at least 4 years of follow-up (average 5.6 ± 1.9, range 4-10 years) were selected for each technique: six subcoronary (Group 1), nine inclusion (Group 2), and eight freestanding root (Group 3). Results were compared among patient groups and with seven control subjects, 6/1 M/F, aged 30 ± 2 years (P = ns). Morphological and functional properties were defined using transverse and paracoronal views, during systole and diastole. Mean aortic size in each group was greater than control, except for the LV-aortic junction and the sinus of Valsalva in Group 1 (26 ± 5 vs. 23 ± 3 mm, P = 0.2 and 33 ± 6 vs. 30 ± 5 mm, P = 0.3). Aortic valve plane rotation (P = 0.02) and root dilatation (P = 0.02) were more common in Group 3. Altered valve opening dynamics and asymmetrical aortic flow profile were also more common in Group 2 (P = 0.03) and Group 3 (P = 0.04). Distensibility was significantly reduced at sinus level in Group 2 and 3 compared with control (4.1 ± 3.5% and 3.6 ± 4.4% vs. 9.0 ± 4.7%, P = 0.03). Asymmetrical aortic flow profile was more common in patients with aortic dilatation (P = 0.05) and with severely reduced (<4%) root elasticity (P = 0.06). Among the three techniques, only subcoronary grafting allows preservation of physiological autograft valve dynamics, aortic flow and distensibility, at all root levels, late after operation. These findings may have relevant implications in the selection of the ideal Ross technique.

Comparative finite element model analysis of ascending aortic flow in bicuspid and tricuspid aortic valve.

In bicuspid aortic valve (BAV) disease, the role of genetic and hemodynamic factors influencing ascending aortic pathology is controversial. To test the effect of BAV geometry on ascending aortic flow, a finite element analysis was undertaken. A surface model of aortic root and ascending aorta was obtained from magnetic resonance images of patients with BAV and tricuspid aortic valve using segmentation facilities of the image processing code Vascular Modeling Toolkit (developed at the Mario Negri Institute). Analytical models of bicuspid (antero-posterior [AP], type 1 and latero-lateral, type 2 commissures) and tricuspid orifices were mathematically defined and turned into a volumetric mesh of linear tetrahedra for computational fluid dynamics simulations. Numerical simulations were performed with the finite element code LifeV. Flow velocity fields were assessed for four levels: aortic annulus, sinus of Valsalva, sinotubular junction, and ascending aorta. Comparison of finite element analysis of bicuspid and tricuspid aortic valve showed different blood flow velocity pattern. Flow in bicuspid configurations showed asymmetrical distribution of velocity field toward the convexity of mid-ascending aorta returning symmetrical in distal ascending aorta. On the contrary, tricuspid flow was symmetrical in each aortic segment. Comparing type 1 BAV with type 2 BAV, more pronounced recirculation zones were noticed in the latter. Finally, we found that in both BAV configurations, maximum wall shear stress is highly localized at the convex portion of the mid-ascending aorta level. Comparison between models showed asymmetrical and higher flow velocity in bicuspid models, in particular in the AP configuration. Asymmetry was more pronounced at the aortic level known to be more exposed to aneurysm formation in bicuspid patients. This supports the hypothesis that hemodynamic factors may contribute to ascending aortic pathology in this subset of patients.

Diagnosis and Management of Iatrogenic Hemobilia Secondary to Transjugular Intrahepatic Portosystemic Shunt Procedure.

A patient with end-stage liver disease and subsequent refractory ascites was treated with a transjugular intrahepatic portosystemic shunt (TIPS) procedure. The procedure was complicated by massive gastrointestinal hemorrhage with associated rettorragia. Computed tomography angiography (CT-A) was performed and revealed haemobilia due to an artero-biliary fistula between the right hepatic artery and an intrahepatic biliary branch. The patient underwent an attempt at percutaneous embolization. Bleeding was successfully stopped by the embolization of the fistula with coils. Hemobilia is a rare cause of upper gastrointestinal bleeding with an increasing incidence due to the widespread use of invasive hepatobiliary procedures. Hemobilia is an uncommon complication of TIPS procedures. Nowadays, transcatheter embolization is the gold standard in the management of hemobilia.

Spleen stiffness measurement for assessing the response to ß-blockers therapy for high-risk esophageal varices patients.

BACKGROUND: Non-selective ß-blocker (NSBB) therapy is the treatment of choice for primary prophylaxis of cirrhotic patients with high-bleeding risk esophageal varices (HRV). The hemodynamic response to NSBB is assessed by the measurement of the hepatic venous pressure gradient (HVPG). Recently, liver and spleen stiffness measurements (LSM and SSM) were proposed as non-invasive surrogates of HVPG. We aimed to evaluate LSM and SSM changes for assessing hemodynamic response in these patients. METHODS: Cirrhotic patients with HRV were prospectively enrolled and evaluated at our Department before starting NSBB and after 3 months. Correlation between changes (delta) of HVPG after NSBB treatment and those of LSM or SSM by transient elastography was performed. RESULTS: From the initial 59 patients considered for the study, 20 were finally included in the analysis. Fifteen (15) patients reached hemodynamic response to NSBB according to HVPG. Changes in LSM did not correlate with changes in HVPG (r = 0.107, p value = 0.655), unlike changes in SSM (r = 0.784, p value < 0.0001). Delta SSM presented excellent accuracy in identifying HVPG responders (AUROC 0.973; 95% CI 0.912-1). The best cut-off for delta SSM to identify responders was -10% (sensitivity 100%, specificity 60%, NPV 100% and PPV 90%). CONCLUSIONS: SSM could be a reliable non-invasive test for the assessment of hemodynamic response to NSBB therapy as primary prophylaxis for HRV. Similar to HVPG, SSM reduction ≥ 10% is able to assess hemodynamic response.

Total Endovascular Aortic Arch Repair with Branched Graft.

In selected cases, the utilization of branched endografts for the treatment of aortic arch aneurysms could be a safe and advantageous alternative to high-risk procedures such as open total aortic arch replacement or hybrid arch repair. We present the case of a 70-year-old man with saccular aneurysm of a bovine aortic arch which was endovascularly treated using a double-branched custom-made aortic endoprosthesis based on the Relay NBS (Non-Bare Stent) Plus platform intended for zone 0 deployment. The postoperative clinical course was uneventful. The postoperative computed tomography scan showed a good result of the implant. The patient was discharged 6 days after the procedure.

Dynamic and quantitative evaluation of degenerative mitral valve disease: a dedicated framework based on cardiac magnetic resonance imaging.

BACKGROUND: Accurate quantification of mitral valve (MV) morphology and dynamic behavior over the cardiac cycle is crucial to understand the mechanisms of degenerative MV dysfunction and to guide the surgical intervention. Cardiac magnetic resonance (CMR) imaging has progressively been adopted to evaluate MV pathophysiology, although a dedicated framework is required to perform a quantitative assessment of the functional MV anatomy. METHODS: We investigated MV dynamic behavior in subjects with normal MV anatomy (n=10) and patients referred to surgery due to degenerative MV prolapse, classified as fibro-elastic deficiency (FED, n=9) and Barlow's disease (BD, n=10). A CMR-dedicated framework was adopted to evaluate prolapse height and volume and quantitatively assess valvular morphology and papillary muscles (PAPs) function over the cardiac cycle. Multiple comparison was used to investigate the hallmarks associated to MV degenerative prolapse and evaluate the feasibility of anatomical and functional distinction between FED and BD phenotypes. RESULTS: On average, annular dimensions were significantly (P<0.05) larger in BD than in FED and normal subjects while no significant differences were noticed between FED and normal. MV eccentricity progressively decreased passing from normal to FED and BD, with the latter exhibiting a rounder annulus shape. Over the cardiac cycle, we noticed significant differences for BD during systole with an abnormal annular enlargement between mid and late systole (LS) (P<0.001 vs. normal); the PAPs dynamics remained comparable in the three groups. Prolapse height and volume highlighted significant differences among normal, FED and BD valves. CONCLUSIONS: Our CMR-dedicated framework allows for the quantitative and dynamic evaluation of MV apparatus, with quantifiable annular alterations representing the primary hallmark of severe MV degeneration. This may aid surgeons in the evaluation of the severity of MV dysfunction and the selection of the appropriate MV treatment.

Uncorrected Ebstein's anomaly with atrial septal defect complicated by brain abscess in an adult patient.

: We report the case of a 56-year-old male patient affected by a severe form of Ebstein's anomaly (type C of Carpentier classification) with secundum atrial septal defect, who presented to the emergency department with impaired consciousness, seizures, and trismus. The brain computed tomography scan showed evidence of a mass located in the frontal lobe, confirmed by brain MRI consistent with brain abscess. Both echocardiography and cardiac MRI showed no evidence of valvular vegetation. This case shows how the combination of increased atrial pressure and bidirectional shunt through atrial septal defect may lead to paradoxical embolization.