Current evidence in predictors of aortic growth and events in acute type B aortic dissection.

OBJECTIVES: Acute type B aortic dissection can have a stable course or evolve into aneurysm and subsequent adverse events. The aim of this systematic review was to analyze the morphologic predictors of an adverse course to establish their validity based on consistency of results. METHODS: Fifty-one studies were included in this review, reporting on aortic size, false lumen (FL) size, primary entry tear (ET) size and location, status of FL thrombosis, number of ETs, branch vessels involvement, and FL longitudinal extent. RESULTS: Some predictors showed good consistency, whereas others did not. Aortic size was the most investigated predictor. A larger diameter at presentation predicted worse outcomes, with few exceptions. Both FL size and size relative to true lumen size also predicted an adverse course, although a standardized measurement method was not used. Regarding primary ET size and location, evidence was sparse and somewhat conflicting. Although FL complete thrombosis was consistently associated with a more benign course, the role of partial thrombosis remained unclear and the concept of FL saccular formation might account for the inconsistency, but further evidence is needed. A higher number of re-entry tears was considered to be protective against false channel expansion, but results need to be confirmed. The predictive role of branch vessels involvement and FL longitudinal extent remain controversial. CONCLUSIONS: Among several predictors of aortic growth and events in acute type B aortic dissection, controversial and even conflicting results have been described. Consistent evidence has been demonstrated only for two predictors: aortic size at presentation is associated with adverse events and total FL thrombosis has a protective role.

Left ventricular endocardial longitudinal dysfunction persists after acute myocarditis with preserved ejection fraction.

BACKGROUND: The aim of present study was to assess left ventricular (LV) myocardial deformation and changes over time in patients with acute myocarditis (AM) with preserved ejection fraction detected by late gadolinium enhancement (LGE) magnetic resonance imaging. METHODS: Thirty-five male patients with AM diagnoses and preserved systolic function based on cardiac magnetic resonance imaging (MRI) were prospectively enrolled. On admission, echocardiography with measurements of global and segmental longitudinal (LS) strains was performed both at the endocardial (ENDO) and epicardial (EPI) levels. Findings were compared to 25 control subjects. Twenty-six patients were also monitored over a 22-month follow-up (FU group). RESULTS: On admission, global ENDO-LS was poorer in magnitude in AM (-19.2 ± 3.1) than in controls (-24.0 ± 1.05) (P < 0.0001), whereas EPI-LS was not different (-20.6 ± 3.4 vs -19.7 ± 6 P = NS). A functional increase in magnitude in both ENDO-LS (-20.8 ± 5.4, P = NS) and EPI-LS (-22.6 ± 4.6, P = 0.02) was found in FU vs AM patients. CONCLUSIONS: The present study demonstrates a steady ENDO-LS impairment in infarct-like AM during a 2-year follow-up period, despite a preserved LV ejection fraction.

Advanced Non-invasive Imaging Techniques in Chronic Heart Failure and Cardiomyopathies : Focus on Cardiac Magnetic Resonance Imaging and Computed Tomographic.

Cardiomyopathies (Cs) are a heterogeneous group of myocardial diseases with structural and/or functional abnormalities.The aetiology is due to genetic-family substrate in most cases, however, the correct and detailed analysis of morphofunctional abnormalities (severity and distribution of hypertrophy, ventricular dilatation, ventricular dysfunction) and tissue characteristics (myocardial fibrosis, myocardial infiltration) are a crucial element for a definite diagnosis.Among the different diagnostic imaging modalities applied in clinical practice (echocardiography, nuclear medicine), cardiac magnetic resonance (CMR) has emerged as a non-invasive diagnostic tool having high ability to quantify systolic function and tissue abnormalities that represent the substrates of many Cs.The main added value of CMR is the ability to identify cardiomyopathies with respect to ischemic heart disease and, above all, to discriminate the major types of cardiomyopathies based on morpho-functional presentation patterns and the presence and location of myocardial fibrosis.Many CMR elements allow increasing diagnostic accuracy but CMR data should be integrated with an appropriate clinical and instrumental context.Computed Tomographic (CT) scan technology has showed a complementary role in patients having Cs and HF.In this chapter, the diagnostic, pathophysiologic and prognostic value of CMR and CT in heart failure due to the most common cardiomyopathies will be discussed.

Left Atrial Morphology, Size and Function in Patients With Transthyretin Cardiac Amyloidosis and Primary Hypertrophic Cardiomyopathy　- Comparative Strain Imaging Study.

BACKGROUND: We sought to assess left atrial (LA) morphology and function in patients with transthyretin cardiac amyloidosis (TTR-CA) and hypertrophic cardiomyopathy (HCM). Primarily, longitudinal deformation (reservoir) and pump function were the focus of vector-velocity strain echocardiography imaging. METHODS AND RESULTS: The study group comprised 32 patients (mean age 57.7±15.4 years, 16 in each group), and 15 healthy controls. Diagnosis of TTR-CA was based on echocardiography and either gadolinium-enhanced (LGE) cardiac magnetic resonance (cMRI) or radionuclide imaging. At baseline, there were no differences in age, body surface area, blood pressure and risk factors among the groups. Left ventricular (LV) mass was greater in patients than in controls, and slight LA dilatation was found in the TTR-CA group. LA reservoir was 14.1±4.7% in TTR-CA, 20.0±5.6% in HCM, and 34.0±11.8% in controls (<0.001). In addition, LA pump function chiefly was impaired in the former group, irrespective of LA chamber size and LV ejection fraction. LGE in the atrial wall was seen in 9/10 TTR-CA versus 0/8 HCM patients undergoing cMRI (P<0.001). LA reservoir ≤19% and pump function ≤-1.1% best discriminated TTR-CA from HCM patients in the receiver-operating characteristic analysis. CONCLUSIONS: LA reservoir and pump function were significantly impaired in both TTR-CA and HCM patients compared with controls, but mainly in the former group, irrespective of LA volume and LV ejection fraction, likely caused by a more altered LA wall structure. (Circ J 2016; 80: 1830-1837).

Extrinsic pulmonary stenosis in primary mediastinal B-cellular lymphoma.

We describe the case of a 34-year-old man with a history of asthenia and excessive fatigability. Transthoracic echocardiography showed a mass in the right ventricular outflow tract with a peak systolic gradient of 52 mmHg. Contrast-enhanced CT confirmed the presence of a lobulated mass, which extensively filled the anterior mediastinum, infiltrating the pulmonary artery trunk up to occupying the right ventricular outflow tract. CT-guided biopsy revealed primary mediastinal B-cellular lymphoma. The patient underwent chemotherapy, achieving complete remission of the disease at the 12-month follow-up, while the gradient across the pulmonary artery dropped from 52 mmHg to 14 mmHg.

Late acquired coronary aneurysm and restenosis after bioresorbable vascular scaffold implantation: a case report.

Background: Although the technology of bioresorbable vascular scaffold (BVS) aroused the peak of interest a few years ago and currently remains available only as part of experimental research, patients who have had BVS implanted should be still carefully monitored to detect possible long-term complications. Case summary: We present the case of a 47-year-old man who had received BVS implantation for ST-segment elevation myocardial infarction. Six years later, computed tomography coronary angiography (CTCA) demonstrated in-segment restenosis in between two newly formed coronary aneurysms at the site of the implanted BVS. The patient received successful optical coherence tomography-guided percutaneous intervention with a new metallic drug-eluting stent implantation. Discussion: Our case demonstrates that coronary aneurysms can be well characterized with CTCA and are often incidentally discovered as they cause no symptoms. The incidence of coronary aneurysm at the site of a previously implanted BVS is not defined, and little is known about the pathophysiology and evolution of these lesions. Therefore, the decision to proceed with conservative management or intervention must be tailored to the clinical conditions of the patient, the anatomy, the rapidity of growth, and the possible thrombotic burden.

Comparison between (99m)Tc-diphosphonate imaging and MRI with late gadolinium enhancement in evaluating cardiac involvement in patients with transthyretin familial amyloid polyneuropathy.

OBJECTIVE: Cardiac involvement is not rare in systemic amyloidosis and is associated with poor prognosis. Both (99m)Tc-diphosphonate imaging and cardiac MRI with late gadolinium enhancement are considered valuable tools in revealing amyloid deposition in the myocardium; however, to our knowledge, no comparative study between the two techniques exists. We compared findings of these two techniques in patients with transthyretin-familial amyloid polyneuropathy (FAP). SUBJECTS AND METHODS: Eighteen patients with transthyretin-FAP underwent (99m)Tc-diphosphonate imaging and MRI with late gadolinium enhancement. Images were visually evaluated by independent readers to determine the presence of radiotracer accumulation or late gadolinium enhancement-positive areas at the level of cardiac chambers. RESULTS: Interobserver agreement ranged from moderate to very good for (99m)Tc-diphosphonate imaging findings and was very good for findings of MRI with late gadolinium enhancement. Left ventricle (LV) radiotracer uptake was found in 10 of 18 patients, whereas LV late gadolinium enhancement-positive areas were found in eight of 18 patients (χ(2) = 0.9; p = 0.343). One hundred fifty-nine LV segments showed (99m)Tc-diphosphonate accumulation, and 57 LV segments were late gadolinium enhancement positive (p < 0.0001). Radiotracer uptake was found in the right ventricle (RV) in eight patients and in both atria in five patients, whereas MRI showed that RV was involved in three patients and both atria in six patients; the differences were not statistically significant (RV, p = 0.07; atria, p = 1). Intermodality agreement between (99m)Tc-diphosphonate imaging and MRI ranged from fair to good. CONCLUSION: Our study shows that, although (99m)Tc-diphosphonate imaging and MRI with late gadolinium enhancement have similar capabilities to identify patients with myocardial amyloid deposition, cardiac amyloid infiltration burden can be significantly underestimated by visual analysis of MRI with late gadolinium enhancement compared with (99m)Tc-diphosphonate imaging.

Constrictive Pericarditis: An Update on Noninvasive Multimodal Diagnosis.

Constrictive pericarditis (CP) is a rare condition that can affect the pericardium after every pericardial disease process and has been described even after SARS-CoV-2 infection or vaccine. In CP, the affected pericardium, usually the inner layer, is noncompliant, constraining the heart to a fixed maximum volume and impairing the diastolic function. This leads to several clinical features, that, however, can be pleomorphic. In its difficult diagnostic workup, noninvasive multimodal imaging plays a central role, providing important morphological and functional data, like the enhanced ventricular interdependence and the dissociation between intrathoracic and intracardiac pressures. An early and proper diagnosis is crucial to set an appropriate therapy, changing the prognosis of patients affected by CP. In this review, we cover in detail the main elements of each imaging technique, after a reminder of pathophysiology useful for understanding the diagnostic findings.

Dual-source dual-energy CT evaluation of complex cystic renal masses.

OBJECTIVE: The purpose of this study was to assess the value of dual-source dual-energy CT in the evaluation of complex cystic renal masses. SUBJECTS AND METHODS: Seventy patients underwent contrast-enhanced dual-energy CT that included true unenhanced images acquired in single-energy mode, corticomedullary phase images acquired in dual-energy mode, and nephrographic phase images acquired in single-energy mode. Virtual unenhanced, blended weighted-average, and color-coded iodine overlay images were reconstructed. The acceptance level and image quality of virtual and true unenhanced images were evaluated. Contrast enhancement on both true unenhanced or blended weighted-average images and color-coded iodine overlay images was evaluated with both calculation in regions of interest and use of confidence level scales. Radiation dose parameters were estimated. RESULTS: Virtual unenhanced images of 70 lesions (97.2%) and true unenhanced images of 72 lesions (100%) were judged acceptable (p = 0.5). The mean quality score of virtual unenhanced images was 2.0 ± 0.7 and of true unenhanced images was 1.5 ± 0.5 (p < 0.001). Mean contrast enhancement measured on true unenhanced and blended weighted-average images was 45.9 ± 15.9 HU (range, 21-78 HU) and on color-coded iodine overlay images was 47.3 ± 16.8 HU (range, 22-75 HU) with no significant differences. Enhancement was excluded on color-coded iodine overlay images with a significantly (p < 0.03) higher level of confidence than it was on true unenhanced and blended weighted-average images. The mean dose reduction with use of a combined dual- and single-energy dual-phase CT protocol was 29.1% ± 11.9% (p < 0.001). CONCLUSION: Dual-source dual-energy CT is a reliable imaging technique in the evaluation of complex cystic renal masses. True unenhanced images can be replaced by virtual unenhanced images with considerable radiation dose reduction. The color-coded iodine overlay technique is a useful tool for both excluding and identifying endocystic enhancement.

MRI findings, patterns of disease distribution, and muscle fat fraction calculation in five patients with Charcot-Marie-Tooth type 2 F disease.

PURPOSE: To describe the magnetic resonance imaging (MRI) pattern of muscle involvement and disease progression in five patients with late-onset Charcot-Marie-Tooth (CMT) disease type 2 F, due to a previously unknown mutation. MATERIALS AND METHODS: Five patients (three males, two females) underwent MRI of the lower limbs to define the pattern of muscle involvement and evaluate the muscle fat fraction (MFF) of residual thigh muscle with gradient-echo (GRE) dual-echo dual-flip angle technique. Evaluation of fatty infiltration both by visual inspection and MFF calculation was performed. RESULTS: A proximal-to-distal gradient of muscle involvement was depicted in male patients with extensive muscle wasting of lower legs, less severe impairment of distal thigh muscles, and sparing of proximal thigh muscles. A peculiar phenotype finding was that no or only slight muscle abnormalities could be found in the two female patients. CONCLUSION: We described the pattern of muscle involvement and disease progression in a family with CMT disease type 2 F. GRE dual-echo dual-flip angle MRI technique is a valuable technique to obtain a rapid quantification of MFF.

Right ventricular ischemic injury in patients with acute ST-segment elevation myocardial infarction: characterization with cardiovascular magnetic resonance.

BACKGROUND: Experimental data show that the right ventricle (RV) is more resistant to ischemia than the left ventricle. To date, limited data are available in humans because of the difficulty of discriminating reversible from irreversible ischemic damage. We sought to characterize RV ischemic injury in patients with reperfused myocardial infarction using cardiovascular magnetic resonance. METHODS AND RESULTS: In 3 tertiary centers, 242 consecutive patients with reperfused acute ST-segment elevation myocardial infarction were studied with cardiovascular magnetic resonance at 1 week and 4 months after myocardial infarction. T2-weighted and postcontrast cardiovascular magnetic resonance scans were used to depict myocardial edema and late gadolinium enhancement, respectively. Early after infarction, RV edema was common (51% of patients), often associated with late gadolinium enhancement (31% of patients). Remarkably, RV edema and late gadolinium enhancement were found in 33% and 12% of anterior left ventricular infarcts, respectively. Baseline regional and global RV functions were inversely related to the presence and extent of RV edema and RV late gadolinium enhancement. At follow-up, a significant decrease in frequency (25/242 patients; 10%) and extent of RV late gadolinium enhancement was observed (P<0.001). With the use of multivariable analysis, the presence of RV edema was an independent predictor of RV global function improvement during follow-up (ß-coefficient=0.221, P=0.003). CONCLUSIONS: Early postinfarction RV ischemic injury is common and is characterized by the presence of myocardial edema, late gadolinium enhancement, and functional abnormalities. RV injury is not limited to inferior infarcts but is commonly found in anterior infarcts as well. Cardiovascular magnetic resonance findings suggest reversibility of acute RV dysfunction with limited permanent myocardial damage at 4-month follow-up.

Tuberculous aortitis in an human immunodeficiency virus-positive Ivorian migrant: A case report.

We report an unusual case of a 35-year-old Ivorian migrant with an abdominal mass and medical history relevant for human immunodeficiency virus-2 positivity with a CD4/CD8 ratio of 0.63; Mantoux and lymphocyte stimulation tests (QuantiFERON) were positive. 3D-CT images revealed a voluminous non-homogeneous retroperitoneal mass in the right abdominal region presenting no significant contrast impregnation. Thoraco-abdominal aorta presented diffuse-altered morphology with multiple ectasias throughout its course and an aneurysm at the level of the subrenal tract. The patient underwent vascular surgery. Mycobacterium tuberculosis complex was detected by polymerase chain reaction performed on intraoperative tissue specimens. Postoperative course was uneventful. After surgery, 3D-CT images showed no signs of malfunction of the prosthesis. At last, at 6-month follow-up, the patient was well. Cross-sectional imaging techniques, such as contrasted-CT, are essential and allow for making diagnosis, assessing disease activity, and evaluating post-treatment condition. 3D reconstruction permits an appropriate patient care by means of an excellent visualization and staging of the disease process.

Prognostic Role of Left Ventricular Intramyocardial Fatty Metaplasia in Patients With Previous Myocarditis (MYOFAT Study).

Left ventricular intramyocardial fat (LV-IMF) is often found in patients with previous irreversible myocardial damage and may be detected by cardiac magnetic resonance (CMR). No data are currently available about the prevalence of LV-IMF in patients with previous myocarditis. Our aim was to assess the prevalence of LV-IMF in patients with previous myocarditis by repeating after >3 years a follow-up CMR examination and to evaluate its clinical and prognostic role. Patients with clinical suspected myocarditis who underwent CMR within the first week from the onset of their symptoms and underwent repeated CMR were enrolled. LV-IMF was detected as areas of left ventricular intramyocardial "India ink" black boundary with or without a hyperintense core. Overall, in 235 patients with a definitive diagnosis of acute myocarditis, CMR was repeated after a median of 4 (3 to 6) years from symptom onset. LV-IMF positive patients (n = 35, 15%) presented greater ventricular volumes and more frequently a mid-wall late gadolinium enhancement than those without LV-IMF (both p < 0.05). Patients presenting major cardiac events (sudden cardiac deaths, resuscitated cardiac arrest, and appropriate implantable cardioverter-defibrillator-firing) at follow-up had a greater prevalence of LV-IMF than those without (55% vs 11%, p < 0.001). Patients with LV-IMF had a higher incidence myocarditis relapse (27% vs 9%, p = 0.003) and a greater risk of major cardiac events (p < 0.0001) than those without. At logistic regression analysis, LV-IMF was an independent predictor of major cardiac events. In conclusion, LV-IMF is not an uncommon finding in patients with previous myocarditis and is associated with worse ventricular remodeling and prognosis.

Early Impairment of Right Ventricular Morphology and Function in Transthyretin-Related Cardiac Amyloidosis.

BACKGROUND: Our study aimed to evaluate right ventricular (RV) morphology and strain (S) in the early stage of familial transthyretin (TTR) cardiac amyloidosis (CA). METHODS AND RESULTS: Thirty-seven patients with transthyretin mutation underwent 99mTc-3,3-diphosphono-1,2 propanodicarboxylic acid (99mTc-DPD) scans and/or cardiac magnetic resonance (CMR) to identify TTR CA. Each patient underwent echocardiography to quantify RV dimensions, tricuspid annular plane systolic excursion (TAPSE), systolic pulmonary artery pressure (sPAP), longitudinal (L) strain of the RV free wall, left ventricular (LV) septal thickness (ST), ejection fraction, E/E', LV global (G) L, radial (R), and circumferential (C) S. 99mTc-DPD and CMR revealed the accumulation in 22 of 37 patients (CA group) and no accumulation in 15 patients (no-CA group). Left ventricular (LV) septal thickness (ST) was higher (P < 0.0001) while LV ejection fraction and E/E' were lower (P < 0.05) in the CA group than the no-CA group. LV-global longitudinal strain (LS) was lower (P < 0.0001) in the CA-group than the no CA-group, whereas LV-global circumferential strain and LV-global radial strain were similar. The CA group showed higher values of RV dimensions (P < 0.05) and sPAP (0.02) and a lower (P = 0.002) TAPSE. Globally, RV-LS was lower (P = 0.005) in the CA group than the no-CA group. Basal and mid segments of the RV free wall showed a lower LS in the CA group than the no-CA group (P < 0.01), while apical S was similar between groups. CONCLUSIONS: RV deformation, particularly in basal and mid segments, is early impaired in CA.

Nuck canal endometriosis: MR imaging findings and clinical features.

OBJECTIVE: The purpose of this study was to describe the MR imaging findings of Nuck canal endometriosis (NCE). MATERIALS AND METHODS: In a 10-year period, 486 out of 612 patients, with laparoscopically and/or surgically proven diagnosis of pelvic endometriosis, underwent MR imaging examination. The examinations were reviewed by two urogenital experienced radiologists working in consensus. Data analysis included: lesions location, size, morphological and signal intensity pattern, involvement of the adjacent muscles, and tendons. RESULTS: In 372 out of 486 patients an MRI diagnosis of endometriosis was made. NCE was found in eight patients. All the lesions were located on the right side. The mean size of the lesions was 2.5 cm (range 1.5-4.5 cm). Two patterns of NCE were found: type 1, prevalently cystic (n = 2); and type 2, prevalently solid with small scattered cysts within lesion (n = 6). In all the patients, hemorrhagic hyperintense cysts could be seen on T1-weighted images. In four patients, the lesions involved the inguinal canal, and in another four patients, the lesions were only outside the inguinal canal. Involvement of the abdominis rectus muscle was seen in two patients, and of the adductor common tendon in two patients. CONCLUSION: MR imaging permits the diagnosis of NCE as well as the evaluation of exact extension of the disease.

Semiautomatic quantification of left ventricular function by two-dimensional feature tracking imaging echocardiography. A comparison study with cardiac magnetic resonance imaging.

OBJECTIVE: To evaluate the accuracy of a semiautomatic quantification of left ventricular (LV) volumes and ejection fraction (EF) using two-dimensional (2D) feature tracking imaging (FTI). METHODS: Thirty-four consecutive subjects (11 patients with dilated cardiomyopathy, 13 with hypertrophic cardiomyopathy, and 10 subjects with no cardiac disease) underwent, on the same day, trans-thoracic echocardiography (TTE) examination, FTI, and cardiac magnetic resonance imaging (MRI), as gold standard, in order to quantify LV volumes and EF. The echocardiographic quantification of LV volumes and EF was determined from four- and two-chamber views using both standard TTE Biplane Simpson's method and a semiautomatic border detection based on FTI. Furthermore, the time for data analysis for each method was measured. RESULTS: The time required for semiautomatic analysis of volumes and EF was significantly lower (P < 0.0001) by FTI (71 seconds) in comparison with standard biplane Simpson's method (93 seconds). LV volumes obtained by FTI were significant underestimated (P < 0.001) in comparison with MRI. Bland-Altman analysis of EDV and ESV using FTI and cardiac MRI showed a low level of agreement for EDV (mean difference = 40.8; SD = 39) and ESV (mean difference = 38.1; SD = 42). On the contrary, no significant difference between FTI and MRI in assessing the LVEF was found; furthermore, a very low bias (2 ± 12) by Bland-Altman analysis was found between FTI and cardiac MRI for the quantification of EF. CONCLUSION: Semiautomatic quantification of LV volumes using FTI allows an accurate, rapid, easy and reliable assessment of LV EF and a rough estimation of LV volumes.

[Role of non invasive cardiac imaging in myocarditis]. / Ruolo dell'imaging cardiaco non invasivo nelle miocarditi.

Myocarditis is a cardiac disease characterized by inflammation of myocardial tissue. Despite clear definition, diagnosis and therapy of myocarditis remain a problem in clinical practice. In this paper, the role of non-invasive cardiac imaging techniques in identifying patients with acute and chronic myocarditis is reviewed.

Role of right ventricular involvement in acute myocarditis, assessed by cardiac magnetic resonance.

OBJECTIVES: Right ventricular (RV) myocarditis (MY) is unrecognized, and its prevalence is unknown. We evaluated the prevalence of RV involvement in acute MY and its association with cardiac events (cardiac death, cardiac arrest, ventricular assist device, transplantation, and appropriate ICD intervention). METHODS: We enrolled 151 patients who underwent cardiac magnetic resonance for clinical suspicion of acute MY. The CMR protocol included T2-STIR images for edema detection, post-contrast cine-SSFP for hyperemia detection and late gadolinium enhancement (LGE) images. RESULTS: Signs of RV MY were found in 27 patients (17.8%): RV edema at T2-STIR in all of these 27 patients; RV LGE was detected in 11 patients (7.3%). The median RV myocardial segment involved was 2 (1-3). In 13 patients, RV edema was in direct continuity with LV edema of septum and inferior wall or with anterior septum and anterior wall. In 2 patients RV myocarditis was found without any signs of LV involvement. Patients with RV MY had higher RV end-diastolic volume index (p = 0.04) and RV mass index (p = 0.03), and lower RV ejection fraction (p < 0.001) than others. At Kaplan-Meier survival curve patients with RV MY had more cardiac events than those without RV involvement (p = 0.015). RV involvement, anteroseptal LGE and RV LGE were associated with cardiac events. CONCLUSION: RV involvement in acute MY is more frequent than previously hypothesized. RV MY was associated with cardiac events.

Coronary Involvement in Marfan Syndrome: The Role of Electrocardiographically Gated Computed Tomography Angiography.

We report a case of coronary artery and aortic root aneurysms in a 50-year-old man with Marfan syndrome. The coexistence of these findings is uncommon and rarely reported. We underline the role of electrocardiographically gated computed tomography angiography in the diagnosis of coronary aneurysm in this category of patients.

Left atrial function in cardiac amyloidosis.

AIMS: Left atrium can be involved by amyloid deposition in familial amyloid polyneuropathy (FAP). The aim of our study is to assess left atrium function in atrial amyloidosis. METHODS: Twenty-eight FAP patients (53 ±â€Š12 years) and a control group of 22 asymptomatic individuals (49 ±â€Š11 years) underwent strain echocardiography and cardiac magnetic resonance (CMR). CMR by late gadolinium enhancement (LGE) was used to assess the left atrium amyloid deposition, whereas strain echocardiography was used to quantify the left atrium deformation. The following atrial longitudinal strain (ALS) parameters were assessed: peak at the end of ventricular systole (peak-ALS), peak at early diastole (early-ALS), negative peak in late diastole, precontraction (prec)-ALS (difference between peak-ALS and early-ALS), and late ALS (sum of negative peak and prec-ALS). RESULTS: CMR showed atrial LGE in 14 FAP patients (LGE-atrial group), whereas 14 FAP patients showed no LGE (no-LGE-atrial group). Peak-ALS was significantly lower in the LGE-atrial group (22.8 ±â€Š13%) compared with the no-LGE-atrial group (59.6 ±â€Š33.1%; P = 0.001) and controls (47.4 ±â€Š16.4%; P = 0.001). Early-ALS was lower in the LGE-atrial group (10.2 ±â€Š6.2%) compared with the controls (26.3 ±â€Š11.9%; P = 0.02) and the no-LGE-atrial group (30.2 ±â€Š22.4%; P = 0.01). Prec-ALS was lower (P = 0.001) in the LGE-atrial group (12.6 ±â€Š7.8%) compared with the no-LGE-atrial group (26.2 ±â€Š15%). Conversely, late-ALS was higher (P = 0.04) in the no-LGE-atrial group (22.8 ±â€Š12.3%) compared with the controls (13.9 ±â€Š9%); no significant differences were found in the negative peak among groups. CONCLUSIONS: Patients with atrial amyloidosis have an adverse left atrium remodeling associated with left atrium dysfunction. Left atrium assessment may provide useful information in the clinical and prognostic stratification of amyloidotic patients.