Marfan Syndrome beyond Aortic Root-Phenotyping Using Cardiovascular Magnetic Resonance Imaging and Clinical Implications.

Marfan syndrome (MFS) is an inherited autosomal-dominant connective tissue disorder with multiorgan involvement including musculoskeletal, respiratory, cardiovascular, ocular, and skin manifestations. Life expectancy in patients with MFS is primarily determined by the degree of cardiovascular involvement. Aortic disease is the major cardiovascular manifestation of MFS. However, non-aortic cardiac diseases, such as impaired myocardial function and arrhythmia, have been increasingly acknowledged as additional causes of morbidity and mortality. We present two cases demonstrating the phenotypical variation in patients with MFS and how CMR (Cardiovascular Magnetic Resonance) could serve as a "one stop shop" to retrieveS all the necessary information regarding aortic/vascular pathology as well as any potential underlying arrhythmogenic substrate or cardiomyopathic process.

Cardiovascular magnetic resonance for the evaluation of patients with cardiovascular disease: An overview of current indications, limitations, and procedures.

Cardiovascular disease (CVD) is the most common cause of morbidity/mortality worldwide. Early diagnosis is the key to improve CVD prognosis, and cardiovascular imaging plays a crucial role in this direction. Echocardiography is the most commonly used imaging modality. However, the need for early diagnosis/treatment favors the development of modalities providing information about tissue characterization beyond echocardiography. In this context, the rapid evolution of cardiovascular magnetic resonance (CMR) led to the coexistence of cardiologists and radiologists in the CMR field. Our aim was to provide an overview of indications, sequences, and reporting of CMR findings in various CVDs. The indications/limitations of CMR as well as the pathophysiological significance of various sequences in adult/pediatric CVDs are presented and discussed in detail. The role of CMR indices in the evaluation of the most common clinical scenarios in cardiology and their impact on CVD diagnosis/prognosis were analyzed in detail. Additionally, the comparison of CMR versus other imaging modalities is also discussed. Finally, future research directions are presented. CMR can provide cardiac tissue characterization and biventricular/biatrial functional assessment in the same examination, allowing for early and accurate identification of important subclinical abnormalities, before clinically overt CVD takes place.

Percutaneous Navigation under Local Anesthesia for Computed Tomography-Guided Microwave Ablation of Malignant Liver Lesions Located in the Hepatic Dome.

Background and Objectives: The aim of the present study was to report the safety and efficacy of percutaneous navigation under local anesthesia for computed tomography-guided microwave ablation of malignant liver lesions located in the hepatic dome. Patients with primary and secondary malignant liver lesions located in the hepatic dome who underwent percutaneous computed tomography-guided microwave ablation using a computer-assisted navigation system under local anesthesia were prospectively evaluated. The primary objective was technical success. Materials and Methods: The sample consisted of 10 participants (16 lesions) with a mean age of 60.60 years (SD = 9.25 years) and a mean size of 20.37 ± 7.29 cm, and the mean follow-up time was 3.4 months (SD = 1.41) months. Results: Primary technical success was 93.75%. Tumor remnant was noticed at one month follow-up in a single metastatic lesion, which was re-treated with an ablation session, and no tumor remnant was depicted in the subsequent imaging follow-up (secondary technical success 100%). Grade I self-limited complications (according to the CIRSE classification system) included small pleural effusion (n = 1) and minor bleeding post antenna removal (n = 1) requiring nothing but observation. Conclusions: the findings of the present study indicate that percutaneous navigation under local anesthesia is a safe and efficacious approach for computed tomography-guided microwave ablation of malignant liver lesions located in the hepatic dome. Large randomized controlled studies are warranted to observe treatment effectiveness and compare the results with those of other options.

Efficacy and safety of percutaneous computed tomography-guided microwave ablation for colorectal cancer, oligometastatic liver-only disease: a single center's experience.

BACKGROUND: We retrospectively evaluated the effectiveness and safety of computed tomography-guided percutaneous microwave ablation (MWA) of metastatic liver disease in terms of 5-year overall survival and 5-year disease-free survival. METHODS: Institutional database research identified 32 colorectal cancer patients with oligometastatic liver-only disease who underwent percutaneous computed tomography-guided MWA. Contrast-enhanced computed tomography or magnetic resonance imaging was used for post-ablation follow up. Patient and tumor characteristics, MWA technique and complications were evaluated. In addition, the 5-year overall survival, the 5-year disease-free survival, and the potential factors affecting the survival of these patients were analyzed. RESULTS: Mean patient age was 72 years (male: female 21:11). In total 58 lesions were treated in 45 ablation sessions. Average lesion size was 2 cm (range 0.8-3.9 cm). The primary tumor for the majority of patients was in the colon (n=27), while in 5 patients it was located in the rectum. The majority of patients had 1 liver metastasis (n=16), 11 patients had 2, 4 patients had 3, and 1 patient had 4. Primary local tumor control was achieved in 91.3% (53/58) of the ablated lesions. Overall survival at 1, 3 and 5 years was 96.8%, 68.7% and 34.3%, respectively. CONCLUSION: Computed tomography-guided percutaneous MWA for metastatic liver-only disease in oligometastatic patients is a feasible, safe and effective therapy with satisfactory long-term survival rates.

A double shunt technique for the prevention of ischaemia of a congenital, solitary, pelvic kidney during abdominal aortic aneurysm repair: a case report.

INTRODUCTION: Congenital solitary pelvic kidney is a rare condition, and its association with an abdominal aortic aneurysm is even more unusual. To the best of our knowledge, only two such cases have been reported in the literature to date. CASE PRESENTATION: We report the case of a 59-year-old Caucasian man with a congenital solitary pelvic kidney, who was found to have an abdominal aortic aneurysm 83 mm in diameter. Abdominal computed tomography angiography clearly identified two renal arteries, one originating from the aortic bifurcation. and the other from the proximal portion of the right common iliac artery. At surgery, renal ischaemia was prevented by introduction of an axillofemoral shunt (consisting of two femoral cannulas and a vent tube of extracorporeal circulation) from the right axillary to the right femoral artery, and a second Argyle shunt from the right common iliac artery to the origin of the left renal artery. A 20 mm Dacron tube graft was then implanted. Our patient's postoperative renal function was normal. CONCLUSION: The renal preservation double shunt technique used in this case seems to be effective during abdominal aortic aneurysm repair.

Enhancement performance of a 64-slice triple rule-out protocol vs 16-slice and 10-slice multidetector CT-angiography protocols for evaluation of aortic and pulmonary vasculature.

OBJECTIVE: To compare the enhancement of the pulmonary and aortic vasculature between a biphasic injection 64-slice, a single-phase injection 16-slice, and a single-phase injection 10-slice multidetector computed tomographic (CT) angiography (CTA) protocols. METHODS: With institutional review board approval and Health Insurance Portability and Accountability Act compliance, 50 patients (16 men, 34 women; mean age, 51.5 years; range, 30-75 years) with atypical chest pain from the emergency department were scanned using a triple rule-out protocol on a 64-slice CT scanner. Pulmonary enhancement was compared with 50 patients (21 men, 29 women; mean age, 65.6 years; range, 38-90 years) imaged with a single-phase 16-slice pulmonary angiography protocol. Aortic enhancement was compared with 24 patients (12 men, 12 women; mean age, 66.1; range, 34-92 years) who were imaged with a 16-slice aortic dissection CTA protocol and to 25 patients (15 men, 10 women; mean age, 50.8 years; range, 20-83 years) imaged with a 10-slice aortic dissection CTA protocol. A 2-tailed Student t test or sign test was used to assess significant differences from a vascular attenuation cutoff value of 250 Hounsfield units (HU). RESULTS: Individual mean pulmonary arterial and aortic attenuation values were statistically significantly less than 250 HU for the 16- and 10-slice protocols and statistically significantly more than 250 HU for the 64-slice protocols (P < 0.05). Mean pooled pulmonary attenuation values were more than 250 HU in 18% (9/50) of the 16-slice and in 93% (39/42) of the 64-slice protocols. Mean pooled aortic attenuation values were more than 250 HU in 18.4% (9/49) of the 10- and 16- and in 100% (42/42) of the 64-slice protocols. CONCLUSIONS: The triple rule-out 64-slice biphasic injection breath hold CTA protocol provides significantly higher attenuation of aortic and pulmonary vasculature compared with our current 10- and 16-slice protocols.

Atypical chest pain: coronary, aortic, and pulmonary vasculature enhancement at biphasic single-injection 64-section CT angiography.

PURPOSE: To prospectively evaluate the enhancement of coronary, pulmonary, and thoracic aortic vasculature by using biphasic single-acquisition 64-section computed tomographic (CT) angiography and to prospectively evaluate if differences in right side of the heart and coronary venous enhancement interfere with interpretation of coronary arteries. MATERIALS AND METHODS: With internal review board approval and HIPAA compliance, 50 patients (16 men, 34 women; mean age, 51.5 years; range, 30-75 years) with atypical chest pain were referred from the emergency department and were imaged with a 64-section CT scanner after premedication with oral atenolol and/or intravenous metoprolol. Thoracic CT angiography with retrospective gating was subsequently performed with a single biphasic injection of 130 mL of iso-osmolar contrast material (100 mL at 5 mL/sec and 30 mL at 3 mL/sec) in caudal-to-cranial acquisition. Coronary, aortic, and pulmonary arterial attenuation values were obtained. Coronary venous and right atrial enhancement were evaluated to assess whether there was interference with coronary artery evaluation. A two-tailed Friedman test was used to evaluate differences among segments within each artery. RESULTS: Mean coronary arterial, pulmonary arterial, and aortic attenuation values were significantly higher than the 250-HU threshold (P < .05). Mean pooled coronary arterial (288.9 HU +/- 64.8), pulmonary arterial (316.4 HU +/- 79.9), and aortic (329.9 HU +/- 63.3) attenuation values were significantly higher than the 250-HU threshold (P < .0001). Coronary venous enhancement did not affect depiction or interpretation of coronary arteries. Right atrial streak artifact focally traversed the right coronary artery in only one study. CONCLUSION: The aforementioned thoracic CT angiographic protocol provides enhancement of coronary, aortic, and pulmonary vasculature in a single breath hold without interference from right side of the heart streak artifact or coronary venous enhancement.

Large recesses associated with left ventricular noncompaction: an unusual presentation of the disease.

Left ventricular noncompaction (LVNC) represents an arrest of myocardial morphogenesis, resulting in persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses. We report a case of a 30-year-old patient who was referred to our hospital due to familial history of cardiomyopathy. The patient underwent transthoracic echocardiography, cardiac catheterization and contrast ventriculography and cardiac magnetic resonance imaging. All imaging techniques showed the characteristic pattern of noncompaction together with two unusually large recesses in the inferior wall of myocardium. This unusual form of LVNC may have an impact on the clinical course of the disease and may require anticoagulation therapy.

Covered stents to treat partial recanalization of onyx-occluded giant intracavernous carotid aneurysm.

PURPOSE: To present the application of a covered stent for the management of a left internal carotid artery (ICA) aneurysm that had recanalized following embolization with Onyx. CASE REPORT: A 54-year-old man had a giant intracavernous aneurysm of the left ICA successfully occluded with Onyx. Recurrence of symptoms 5 months later prompted control angiography, which showed partial recanalization of the aneurysm. The aneurysm neck was successfully sealed by placing 2 polytetrafluoroethylene-covered stents across it. Control angiography performed at 12 months after stent placement showed no stenosis or signs of recanalization of the aneurysm. CONCLUSIONS: Recanalization of giant intracavernous carotid aneurysms post-Onyx treatment may be safely treated with placement of covered stents across the aneurysm neck.

Percutaneous management of extensive clot trapped in a temporary vena cava filter.

PURPOSE: To present percutaneous management of extensive clot trapped in a temporary inferior vena cava (IVC) filter. CASE REPORT: A 20-year-old man with a large sacral tumor and left external iliac vein thrombosis had a wire-mounted Günther temporary filter (GTF) prophylactically placed in the infrarenal IVC prior to tumor resection. The 7-day postsurgical venogram prior to filter removal showed extensive clot trapped by the filter, as well as interval cephalic migration, with the filter tip now at the level of the renal veins. A Günther Tulip MReye (GTM) filter was deployed but not released above the renal veins to prevent clot migration during caudal withdrawal of the wire-mounted GTF. After creating enough space in the infrarenal IVC, the GTM was repositioned and fully released with its apex now below the renal veins. The GTF was then uneventfully removed. There was no clinical evidence for significant pulmonary embolism 12 months after placement. CONCLUSIONS: Cephalic migration of a clotted temporary IVC filter can be managed with intraprocedural protection against pulmonary embolism by first deploying a Günther Tulip filter above the renal veins, with subsequent advancement below the renal veins before temporary filter removal.

Transmitted cardiac pulsations as an indicator of transjugular intrahepatic portosystemic shunt function: initial observations.

PURPOSE: To determine if cardiac pulsations are visible and quantifiable on spectral waveforms during Doppler ultrasonographic (US) evaluation of transjugular intrahepatic portosystemic shunts (TIPS), and if so, whether their magnitude declines with shunt dysfunction. MATERIALS AND METHODS: Baseline and pre-revision US images obtained in 15 patients with venographically confirmed TIPS malfunction were retrospectively examined for spectral waveform pulsation. Cardiac pulsatility was quantified by using the venous pulsatility index (VPI), the venous equivalent of resistive index. VPIs were obtained at four locations from the main portal vein to the stent-hepatic venous junction. Baseline and follow-up examination results in 11 patients with functional TIPS acted as controls and were evaluated similarly. Baseline and follow-up mean VPIs at all four locations were compared for both sets of patients by using the Newman-Keuls pairwise multiple sample comparison test. The chi(2) test was used to determine if a VPI threshold that would result in an acceptable sensitivity and specificity for shunt dysfunction existed. RESULTS: One hundred twenty mean VPIs were obtained in the study group, and 88 mean VPIs were obtained in the control group. Pre-revision VPIs at each location were significantly lower (P <.01) than all baseline values and than the follow-up values in the control group. A VPI less than 0.16 was 94% sensitive and 87% specific for shunt dysfunction. CONCLUSION: The VPI, a quantitative measure of cardiac pulsation obtained with Doppler US, may be a useful parameter for assessing TIPS function.

Contrast-enhanced three-dimensional MR angiography of the pulmonary vascular tree.

Contrast-enhanced three-dimensional MR angiography has evolved into a promising technique in the study of the pulmonary vasculature. Both congenital and acquired entities can be now morphologically demonstrated in a non-invasive manner obviating the need for conventional pulmonary angiography. Due to spatial resolution limitations, however, it is still premature to routinely apply the method in the detection of small subsegmental emboli, in cases of suspected pulmonary embolism, and further technical developments will be required. In this paper we present a spectrum of congenital and acquired disorders affecting the pulmonary vascular tree as demonstrated with contrast-enhanced three-dimensional MR angiography.