Coarctation of the Aorta: Diagnosis and Management.

Coarctation of the aorta (CoA) accounts for approximately 5-8% of all congenital heart defects. Depending on the severity of the CoA and the presence of associated cardiac lesions, the clinical presentation and age vary. Developments in diagnosis and management have improved outcomes in this patient population. Even after timely repair, it is important to regularly screen for hypertension. Patients with CoA require lifelong follow-up with a congenital heart disease specialist as these patients may develop recoarctation and complications at the repair site and remain at enhanced cardiovascular risk throughout their lifetime.

Pre-operative Considerations in Adult Mucopolysaccharidosis Patients Planned for Cardiac Intervention.

Mucopolysaccharidoses (MPS) are rare lysosomal storage diseases characterized by multiorgan involvement and shortened longevity. Due to advances in therapies such as enzyme replacement therapy and haematopoietic stem cell therapy, life expectancy has increased posing newer challenges to patients and health professionals. One such challenge is cardiovascular manifestations of MPS, which can be life limiting and cause reduction in quality of life. Any cardiovascular intervention mandates comprehensive, multi-systemic work-up by specialist teams to optimize outcome. We highlight the importance of multidisciplinary evaluation of adult MPS patients requiring cardiovascular intervention. Clinical assessments and investigations are discussed, with a focus on the cardiac, anesthetic, airway, respiratory, radiological and psychosocial factors.

Society for Cardiovascular Magnetic Resonance 2020 Case of the Week series.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). Case of the week is a case series hosted on the SCMR website ( https://www.scmr.org ) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. Each case consists of the clinical presentation and a discussion of the condition and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2020 Case of the Week series of 11 cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Revisiting the pathogenic role of insulin resistance in Duchenne muscular dystrophy cardiomyopathy subphenotypes.

INTRODUCTION: Duchenne muscular dystrophy (DMD) is known to impact the subepicardial layer of the myocardium through chronic inflammation. Recent animal studies have shown predominant subendocardial involvement in rats with DMD. The primary outcome parameter was to determine by cardiovascular MRI (CMR) if two differential patterns of myocardial involvements exist in DMD; the secondary outcome parameters were to correlate the observed pattern with metabolic markers such as insulin resistance measures. METHODS: Forty patients with DMD were screened using CMR to determine which of them had predominantly subendocardial dysfunction (SENDO group), or subepicardial/midmyocardial involvement (SEPMI group). Patients were subjected to body mass index measurement, serum creatinine kinase, serum lactate dehydrogenase enzyme, fasting glucose-insulin ratio (FGIR), full lipid profile, left ventricular ejection fraction (LVEF), left ventricle E/E´ ratio (the ratio of early mitral inflow velocity to average early diastolic velocities of the basal septum and mitral annulus) for left ventricle diastolic function, and myocardial layer strain discriminating echocardiography (MLSD-STE). Results: 26 patients displayed SENDO while 34 displayed SEPMI. SENDO group displayed overt insulin resistance; (FGIR (SENDO: 7 ± 1 vs. SEPMI: 5 ± 1, P < 0.001). FGIR was negatively correlated with Subendocardial Global Longitudinal Strain (ENDO-LS) with r = -0.75. CONCLUSION: DMD does not seem to influence the heart uniformly; DMD cardiomyopathy probably has two separate phenotypes with different mechanisms. Insulin resistance might be implicated in its pathogenesis and its reversal may help to slow disease progression.

Assessment of aortic annulus dimensions for transcatheter aortic valve replacement (TAVR) with high-pitch dual-source CT: Comparison of systolic high-pitch vs. multiphasic data acquisition.

OBJECTIVES: To evaluate a systolic ECG-gated high-pitch aortoiliac computed tomography (CT) angiography for planning transcatheter aortic valve implantation (TAVI). METHODS: Patients referred for TAVI underwent a combined CT imaging with retrospective, multiphasic ECG-gating of the heart and systolic ECG-gated high-pitch aortoiliac CT angiography. Consecutive patients were retrospectively included in this study group. Heart rate (HR) and heart rate variability (HRV) were assessed during the high-pitch ECG prediction phase. Aortic annulus area (AAA) was planimetrically quantified on both datasets. While only one moment of cardiac cycle was available for measurements in the high-pitch CT, the point of time in the multiphasic CT was chosen, where AAA yielded maximum size. Hypothetical prosthesis sizing was compared between multiphasic vs. high-pitch CT. RESULTS: Among 61 patients (44.2 % men, mean age: 83.3 ± 5.5 years) average heart rate and HRV were 71.0 ± 13.4 bpm and 7.3 ± 8.5 bpm. 20 patients (32.7 %) had atrial fibrillation at the time of image acquisition. There was a strong correlation of AAA as derived from multiphasic vs. the high-pitch CT (r = 0.98). The difference in AAA was 10.5 ± 17.1mm2 (455.1 ± 83.0 mm2 for multiphasic vs. 444.5 ± 87.2 mm2 for high-pitch CT) and did not reach statistical significance (p = 0.08). Hypothetical prosthesis sizing showed an agreement in 55 of 61 patients (90.2 %). A sizing based on the high-pitch CT resulted in smaller prosthesis choice in 6 patients, all of them suffering from atrial fibrillation. Mean effective radiation dose was 10.9 ± 6.1 mSv for cardiac CTA and 4.1 ± 1.0 mSv for high-pitch CTA. CONCLUSION: For patients with sinus rhythm, systolic high-pitch aortoiliac CTA provides adequate prosthesis size selection as compared with multiphasic ECG-gated cardiac CTA and may result in significantly reduced radiation exposition.

The Egyptian Collaborative Cardiac Genomics (ECCO-GEN) Project: defining a healthy volunteer cohort.

The integration of comprehensive genomic and phenotypic data from diverse ethnic populations offers unprecedented opportunities toward advancements in precision medicine and novel diagnostic technologies. Current reference genomic databases are not representative of the global human population, making variant interpretation challenging, especially in underrepresented populations, such as the North African population. To address this, the Egyptian Collaborative Cardiac Genomics (ECCO-GEN) Project launched a study comprising 1000 individuals free of cardiovascular disease (CVD). Here, we present the first 391 Egyptian healthy volunteers recruited to establish a pilot phenotyped control cohort. All individuals underwent detailed clinical investigation, including cardiac magnetic resonance imaging (MRI), and were sequenced using a targeted panel of 174 genes with reported roles in inherited cardiac conditions. We identified 1262 variants in 27 cardiomyopathy genes of which 15.1% were not captured in current global and regional genetic reference databases (here: gnomAD and Great Middle Eastern Variome). The ECCO-GEN project aims at defining the genetic landscape of an understudied population and providing individual-level genetic and phenotypic data to support future studies in CVD and population genetics.

Can myocardial remodeling be a useful surrogate predictor of myocardial iron load? A 3D echocardiographic multicentric study.

The relationship between myocardial iron load and eccentric myocardial remodeling remains an under-investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI. 3D derived sphericity index was significantly higher in patients with myocardial iron load. It was correlated with T2* with a 100% sensitivity and specificity (cut-off value of 0.34) to discriminate between patients with and without myocardial iron overload.

Emergency Repair of a Perforated Mycotic Aneurysm with a Self-made Pericardial Tube-graft.

A 63-year-old female with a history of kidney transplantation was admitted for emergency repair of a perforated mycotic aneurysm of the right subclavian artery (RSA) in combination with a paravertebral and posterior mediastinal abscess. After resection of the aneurysm and after radical local debridement, orthotopic repair was performed with a self-made pericardial tube graft from the brachiocephalic bifurcation to the thoracic outlet. The paravertebral and posterior mediastinal abscess was drained. The postoperative course was uneventful. Using a self-made readily available pericardial neo-tube enlarges the armamentarium of handling complex infective surgical scenarios and presents a smart alternative to alloplastic vascular reconstruction.

Long-Standing Cyanosis in Congenital Heart Disease Does not Cause Diffuse Myocardial Fibrosis.

The assumption of the presence of diffuse myocardial fibrosis in long-standing cyanotic congenital heart disease (CHD) inspired us to noninvasively determine the myocardial extracellular volume (ECV) using contrast CMR. T1 maps were measured pre and 10 min after the injection of 0.15 mmol/kg of gadolinium in 25 subjects. Seven patients with long-standing cyanotic CHD and no previous cardiac surgery (aged 16-53 years and oxygen saturations of 69-90%), nine normal subjects (aged 14-49 years), and nine patients with previously cyanotic CHD, who had been corrected by open heart surgery (aged 2 months-58 years, mean 9 years). Late gadolinium enhancement was performed to exclude scar areas. The T1 values were measured in the interventricular septum and in the left lateral or inferior ventricular wall, such that same areas were assessed in every patient in the pre- and post-contrast T1 scan. ECV was calculated according to ΔR1myocardium/ΔR1blood * (1 - hematocrit). Cyanotic patients had significantly lower ECV percentage than the previous cyanotic patients (septum: 22 ± 2.7% vs 35 ± 4.6%, p = 0.002; LV wall: 22 ± 2.2% vs 30 ± 3.7%, p = 0.01, respectively). No significant differences were found between cyanotic patients and normal controls (septum: 22 ± 2.7% vs 24 ± 1.4%, p = 0.44; LV wall: 22 ± 2.2% vs 24 ± 2%, p = 0.57, respectively). Long-standing cyanosis in CHD without cardiac surgery does not cause diffuse myocardial fibrosis or expansion of the myocardial ECV.

Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters.

Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn't correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.

Validation of Noninvasive Measurement of Cardiac Output Using Inert Gas Rebreathing in a Cohort of Patients With Heart Failure and Reduced Ejection Fraction.

BACKGROUND: Cardiac output (CO) is a key indicator of cardiac function in patients with heart failure. No completely accurate method is available for measuring CO in all patients. The objective of this study was to validate CO measurement using the inert gas rebreathing (IGR) method against other noninvasive and invasive methods of CO quantification in a cohort of patients with heart failure and reduced ejection fraction. METHODS AND RESULTS: The study included 97 patients with heart failure and reduced ejection fraction (age 42±15.5 years; 64 patients (65.9%) had idiopathic dilated cardiomyopathy and 21 patients (21.6%) had ischemic heart disease). Median left ventricle ejection fraction was 24% (10%-36%). Patients with atrial fibrillation were excluded. CO was measured using 4 methods (IGR, cardiac magnetic resonance imaging, cardiac catheterization, and echocardiography) and indexed to body surface area (cardiac index [CI]). All studies were performed within 48 hours. Median CI measured by IGR was 1.75, by cardiac magnetic resonance imaging was 1.82, by cardiac catheterization was 1.65, and by echo was 1.7 L·min-1·m-2. There were significant modest linear correlations between IGR-derived CI and cardiac magnetic resonance imaging-derived CI (r=0.7; P<0.001), as well as cardiac catheterization-derived CI (r=0.6; P<0.001). Using Bland-Altman analysis, the agreement between the IGR method and the other methods was as good as the agreement between any 2 other methods with each other. CONCLUSIONS: The IGR method is a simple, accurate, and reproducible noninvasive method for quantification of CO in patients with advanced heart failure. The prognostic value of this simple measurement needs to be studied prospectively.

Cardiovascular magnetic resonance is successfully feasible in many patients aged 3 to 8years without general anesthesia or sedation.

OBJECTIVES: Patients younger than 8 years are usually examined by cardiovascular magnetic resonance (CMR) under general anesthesia (GA) or sedation without intubation. Therefore, we sought to study the feasibility of CMR in patients aged 3 to 8years without GA or sedation. PATIENTS: Data sets of 71 consecutive patients aged 3 to 8years were studied retrospectively. DESIGN: The total cohort was divided into 2 groups: a no-GA or sedation without intubation group (no-GA or sedation) and a GA or sedation without intubation group (GA or sedation). MEASUREMENTS: The patients' age, scan durations for each group, successfully answered clinical question, and number of sequences per study were compared between both groups. MAIN RESULTS: Scan duration in the no-GA or sedation group (n=44) was 35± 20minutes, and that in the GA or sedation group (n=27) was 60± 31minutes (P<.001). The percentage of successful reports was 95% (42/44) in the no-GA or sedation group and 89% (24 of 27) in the GA or sedation group (P=.29). CONCLUSION: CMR in patients aged 3 to 8years is usually successfully feasible without GA or sedation.

An interatrial tunnel: a rare form of atrial septal defects.

Atrial septal defects (ASDs) account for approximately 6%-10% of congenital heart defects. The well-known types of atrial septal communications are the ostium secundum, ostium primum, sinus venosus types, and coronary sinus defects. A 50-year-old female was referred for TEE for better assessment of MR severity and mechanism. 2D/3D-TEE showed a rare combination of different abnormalities; bi-leaflet mitral valve prolapse, cleft P2, cor triatriatum sinister, and a tunnel-shaped IAS communication. To the best of our knowledge, this is a very rare case with a rare form of atrial septal defect that was not described before. We named this defect an interatrial tunnel.

PLATFORM at 90 days: Evaluating the clinical utility of FFRCT.

The advancement that took place in assessing fractional flow reserve (FFR) using various competing modalities led to numerous research trials to evaluate the clinical impact of each. Among those trials is the recently published PLATFORM study. The data was designed to compare two clinical scenarios; a combination of computed fluid dynamics with computed tomographic angiography (CTA-guided strategy) in non-obstructive coronary artery disease (CAD) on one arm, compared to the standard practice representing the other clinical arm. The study's results were evaluated for further evidence and clinical insights.

Cardiac MRI findings of endomyocardial fibrosis (Loeffler's endocarditis) in a patient with rheumatoid arthritis.

Loeffler's endocarditis and cardiac manifestations of the hypereosinophilic syndrome (HES) are rare and difficult to diagnose. We report a case of in a 36 year-old female with a history of rheumatoid arthritis with disabling dyspnea. The transthoracic echocardiogram demonstrated normal systolic cardiac functions and a left ventricular apical thrombus. However, using cardiovascular magnetic resonance (CMR) with inversion-recovery (IR) delayed enhancement, and cine steady-state free precession (SSFP) sequences, we were able to clearly demonstrate endocardial fibrosis, tissue inflammation, apical ventricular hypertrophy, and LV thrombus that correlate with clinical findings. We believe cardiac MRI is more useful than transthoracic echocardiography in the diagnosis and management of HES and ultimately it obviated the need for biopsy to confirm the diagnosis.