Society for Cardiovascular Magnetic Resonance 2022 Cases of SCMR case series.

"Cases of SCMR" is a case series on the SCMR website (https://www.scmr.org) for the purpose of education. The cases reflect the clinical presentation, and the use of cardiovascular magnetic resonance (CMR) in the diagnosis and management of cardiovascular disease. The 2022 digital collection of cases are presented in this manuscript.

IgG4-Related disease with diffuse myopericardial involvement- value of CMR: a case report and literature review of cardiac involvement.

BACKGROUND: IgG4-related disease is a fibro-inflammatory disorder with an unknown etiology, which can affect multiple organ systems, including the cardiovascular system. While most reported cases of cardiovascular involvement are primarily associated with the aorta, there have been sporadic reports of isolated cardiac involvement. CASE PRESENTATION: This paper presents a documented case of IgG4-related systemic disease with symptoms indicative of restrictive cardiomyopathy. Subsequent Cardiac Magnetic Resonance imaging revealed diffuse myopericardial involvement, characterized by pericardial thickening and enhancement, accompanied by subepicardial and myocardial infiltration. Considering the rarity of cardiac involvement in our case, we conducted a thorough review of the existing literature pertaining to various patterns of cardiac involvement in IgG4-related disease, as well as the diagnostic modalities that can be employed for accurate identification and assessment. CONCLUSIONS: This case report sheds light on the importance of recognizing and evaluating cardiac manifestations in IgG4-related systemic disease to facilitate timely diagnosis and appropriate management.

Aortic Arch Debranching and Thoracic Endovascular Aortic Repair (TEVAR) for Type B Aortic Dissection.

BACKGROUND: Since its introduction, thoracic endovascular aortic repair (TEVAR) has revolutionized the treatment of type B aortic dissections (TBADs). However, the proximal aspect of the aortic pathology treated may infringe on the origin of the left subclavian artery or even more proximally. Hence, to ensure durable outcomes, the origin of these vessels needs to be covered, but an extra-anatomical bypass is required to perfuse vital branches, known as aortic arch debranching. This series aims to describe and delineate the disparities of aortic arch debranching during TEVAR for TBAD. METHODS: A retrospective review and analysis of a multicenter international database was conducted to identify patients with TBAD treated with TEVAR between 2005 and 2021. Data analyzed included patient demographics, disease characteristics, operative characteristics, and postoperative outcomes with follow-up on mortality and reintervention. All statistical analyses were carried out using IBM SPSS 26. Patient survival was calculated using a Kaplan-Meier survival analysis, and a P value of less than 0.05 was considered statistically significant. RESULTS: A total of 58 patients were included in the analysis, of which 27 (46.6%) presented with complicated disease and 31 were uncomplicated, of which 10 (17.2%) were classed as high risk and 21 (36.2%) low risk. Zone 2 was the most common proximal landing zone for the stent graft. Left subclavian artery bypass was performed selectively (26%), with 1 stroke occurring, likely due to embolic reasons. A further 6 underwent more proximal aortic debranching before TEVAR (10%) and was a significant risk factor for mortality and the number of stents deployed. The overall rates of reintervention and mortality were 17.2% (n = 10) and 29.3% (n = 17). CONCLUSIONS: Aortic arch debranching and TEVAR for TBAD is associated with significant mortality. Future developments to treat aortic arch pathology could incorporate branched graft devices, eliminating the need for debranching, improving stroke rates, and reducing future reinterventions.

Feature tracking cardiac magnetic resonance imaging to assess cardiac manifestations of systemic diseases.

Feature-tracking cardiac magnetic resonance (FT-CMR), with the ability to quantify myocardial deformation, has a unique role in the evaluation of subclinical myocardial abnormalities. This review aimed to evaluate the clinical use of cardiac FT-CMR-based myocardial strain in patients with various systemic diseases with cardiac involvement, such as hypertension, diabetes, cancer-therapy-related toxicities, amyloidosis, systemic scleroderma, myopathies, rheumatoid arthritis, thalassemia major, and coronavirus disease 2019 (COVID-19). We concluded that FT-CMR-derived strain can improve the accuracy of risk stratification and predict cardiac outcomes in patients with systemic diseases prior to symptomatic cardiac dysfunction. Furthermore, FT-CMR is particularly useful for patients with diseases or conditions which are associated with subtle myocardial dysfunction that may not be accurately detected with traditional methods. Compared to patients with cardiovascular diseases, patients with systemic diseases are less likely to undergo regular cardiovascular imaging to detect cardiac defects, whereas cardiac involvement in these patients can lead to major adverse outcomes; hence, the importance of cardiac imaging modalities might be underestimated in this group of patients. In this review, we gathered currently available data on the newly introduced role of FT-CMR in the diagnosis and prognosis of various systemic conditions. Further research is needed to define reference values and establish the role of this sensitive imaging modality, as a robust marker in predicting outcomes across a wide spectrum of patients.

Increased prevalence of thyroid dysfunction in Tehran - HAMRAH study.

BACKGROUND: The aim of the current study is to assess the prevalence of different categories of thyroid dysfunction and their associated risk factors among the modern urban population of Tehran, the capital of Iran. METHODS: The present investigation is a sub-study of the HAMRAH study, a population-based prospective study designed to assess the prevalence of traditional cardiovascular risk factors and their changes through a 10-year follow-up. 2228 (61% female) adults aged between 30 and 75 years old and with no overt cardiovascular diseases were selected through a multistage cluster randomized sampling. Blood levels of thyroid-stimulating hormone (TSH), thyroxin (T4), and triiodothyronine (T3) were measured with the aim of assessing the prevalence of abnormal thyroid function status among the modern urban Iranian population, and in order to report the total prevalence of participants with clinical hypo- or hyperthyroidism, the number of individuals taking thyroid-related drugs were added to the ones with overt thyroid dysfunction. A subgroup analysis was also performed to determine the associated risk factors of thyroid dysfunction. RESULTS: The prevalence of thyroid dysfunction among the total population was 7% (95%CI: 5.9 - 8%) and 0.4% (95% CI: 0.1 - 0.6%) for subclinical and overt hypothyroidism, and 1.6% (95% CI: 1 - 2%) and 0.2% (95% CI: 0 - 0.3%) for subclinical and overt hyperthyroidism, respectively. Clinical thyroid dysfunction was detected in 10.3% of the study population (9.4% had clinical hypo- and 0.9% had clinical hyperthyroidism). In the subgroup analysis, thyroid dysfunction was significantly more prevalent among the female participants (P-value = 0.029). CONCLUSIONS: In the current study, the prevalence of different categories of abnormal thyroid status, and also the rate of clinical hypo- and hyperthyroidism was assessed using the data collected from the first phase of the HAMRAH Study. In this study, we detected a higher prevalence of clinical and subclinical hypothyroidism among the Iranian population compared to the previous studies.

Ethnic & Sex Disparities in Type B Aortic Dissection Patients Undergoing Thoracic Endovascular Aortic Repair: An International Perspective.

BACKGROUND: There has been a revolution in the management of type B aortic dissection (TBAD) over the last 2 decades due to the increasing development of endovascular techniques, with the prime example being thoracic endovascular aortic repair (TEVAR). However, many controversies concerning the use of TEVAR in TBAD exist. For instance, there is little available evidence suggesting any differences in demographics, disease characteristics, intervention technicalities, and clinical outcomes between males and females as well as different ethnicities when undergoing TEVAR for TBAD, both in the short and long term. Also, there is no risk prediction model/tool available. The objective of this international study is to describe and delineate the disparities between male and female patients of different ethnicities in terms of demographics, disease and interventional characteristics, and clinical outcomes. METHODS: Over 17 years a total of 58 TBAD patients were admitted to 2 tertiary vascular centres and treated using TEVAR. Mortality, postoperative complications and reintervention data were recorded for the first 30 days after the procedure as well as during follow-up. Follow-up for all patients lasted until the study endpoint or until a patient's death. Data were analysed retrospectively using IBM SPSS statistical package 26 for Windows. RESULTS: The mean age was similar between the 2 groups, with the majority of patients in both being Caucasian. More than half of the males had complicated TBAD, while the majority of females were classed as having uncomplicated disease. The most commonly utilized proximal and distal landing zone for the TEVAR stent graft was zone 2 and zone 4, respectively. The mean proximal stent diameter was significantly larger in males compared to females (P = 0.004). The difference in mean distal diameter between the 2 groups was insignificant (P = 0.721). The mean total stent coverage of the thoracic aorta was 251.3 mm in male patients compared to 291.2 mm in females (P = 0.203). A total of 32 patients underwent adjunctive procedures. Seven (17%) of the males had one or more postoperative complications compared to 2 (12%) females. Nine (22%) males underwent a reintervention procedure post TEVAR compared to only 1 (6%) female (P = 0.136). Overall, males had a mortality rate of 24% (n = 10) compared to 41% of females (n = 7) (P = 0.201). Estimated survival of males and females post TEVAR was 80.4 ± 9.6 months and 69.7 ± 14.4 months (P = 0.428). For the total population, the number of stent grafts was inversely correlated with mortality. CONCLUSIONS: Despite the favourable clinical outcomes achieved by TEVAR in TBAD, there remains a grey area concerning its management. Thus, it is important to perform risk stratification of individual patients using their demographics and comorbidities, particularly scrutinizing patient sex and ethnic origin, when considering intervention for TBAD to achieve optimum results.

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.

Determinants of perivascular adipose tissue stranding as a novel imaging marker and its relation to inflammatory biomarker high-sensitivity C-reactive protein.

Purpose: This study aimed to examine the relationship of perivascular adipose tissue (PVAT) stranding in coronary computed tomography angiography (CCTA) with high-sensitivity C-reactive protein (hsCRP) and the determinants of PVAT stranding in coronary artery disease (CAD) patients. Material and methods: This retrospective cross-sectional study was done by collecting data from CAD patients who were referred to Rajaie Cardiovascular Centre between January 2018 and September 2020, with CCTA and hsCRP test 72 hours apart from the CCTA. PVAT stranding was defined as irregular obscuration of PVAT adjacent to the coronary arteries. An attempt was made to find a correlation between included variables and PVAT stranding by comparing them between 2 groups: patients with and without PVAT stranding. Results: From 92 patients, 31 participants had PVAT stranding, and statistically significant higher levels of hsCRP were detected in them (p = 0.007). We demonstrated significantly higher prevalence of history of hyperlipidaemia (OR = 3.83, p = 0.029), high-risk plaque features (OR = 11.80, p = 0.015), and obstructive coronary luminal stenosis (OR = 3.25, p = 0.025) in patients with PVAT stranding. Also, significantly higher PVAT attenuation was detected in patients with PVAT stranding (p < 0.001) independently from mean attenuation of epicardial fat. Conclusion: PVAT stranding could be used as a novel non-invasive marker in CCTA of CAD patients. More studies focusing on patient outcomes are required to better evaluate the reliability and prognostic value of this marker.

Re-emphasis on the role of echocardiography in diagnosis of aortic root surgery complications.

BACKGROUND: Aortic pseudoaneurysm is an infrequent complication of ascending aorta surgery. CASES: This is a report of two cases that underwent the Bentall procedure that presented with large pseudoaneurysms. CONCLUSION: Use of multimodality imaging after Bentall surgery has been advocated for the detection of surgical complications. Although 3D CT angiography has the highest diagnostic accuracy, familiarity with transthoracic echocardiographic finding in these patients will lead to earlier diagnosis.

Acute coronary syndrome following arteriovenous fistula creation in a post CABG patient: A steal phenomenon from coronary artery to subclavian artery.

A 70-year-old man with a history of coronary artery bypass grafting 15 years back and arteriovenous (AV) fistula creation in the left arm 1 month back presented with acute coronary syndrome (ACS). He had not received dialysis before his referral. We felt the most likely etiology for these complaints was increased cardiac oxygen demand from an increased cardiac output related to the newly formed left AV fistula. Coronary angiography was done to detect any significant stenosis in the native or grafted vessels. This revealed that the left subclavian artery was totally occluded in the ostioproximal segment and the coronary arteries did not have occlusions to explain the ACS setting. CT angiography confirmed the angiographic findings of the totally occluded left subclavian artery followed by a well-developed and patent left internal mammary artery to left anterior descending artery. This led to the consideration of a steal syndrome from the coronary artery by the subclavian artery distal to the occlusion. A successful percutaneous endovascular intervention on the left subclavian artery occlusion was performed. Subsequently, the patient became asymptomatic and experienced a dramatic increase in left ventricular ejection fraction.

Multimodality imaging of constrictive pericarditis in H syndrome.

This is the first report of constrictive pericarditis (CP) in a 16-year-old boy with H syndrome with pericardial involvement predominantly over the right ventricle with favorable response to anti-inflammatory treatment. H syndrome, first reported in 2008, is a new auto-inflammatory syndrome with multiorgan involvement due to mutation in the SLC29A3 gene. We described the echocardiographic characteristics of asymmetric pericardial involvement and presented the cardiac computed tomography angiographic and magnetic resonance imaging findings. We reviewed the echocardiographic signs of CP, introduced tricuspid E/A respiratory alternans as a novel echocardiographic sign of right ventricular dominant CP, and explained the underlying mechanism.

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.

Abnormal congenital aorto-cardiac communications (CACC) are a heterogeneous constellation of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. The current terminology of CACC has significant errors and shortcomings including inconsistent and interchangeable use of terms of fistula and tunnel and lack of an inclusive classification with practical information on therapeutic management. The aims of this study were threefold: firstly, to perform a concise narrative review of congenital pathologic connections between the aortic root and cardiac chambers which include rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications; secondly, to investigate the differentiating features of the so-called aorta right atrial tunnel (ARAT), with and without coronary artery take-off from the tunnel, and coronary cameral fistula (CCF) by applying a differential diagnostic assistance toolbox to two groups of patients with ARAT and CCF; and lastly, to propose a practical and inclusive anatomic-therapeutic classification for CACCs. The two main cornerstones of the proposed classification are the type of the connector between the aorta and cardiac chamber (hole versus passage) and the nature of the connecting passage ( anatomic versus extra-anatomic). We classified CACCs into three types. Depending on the intramural versus extramural course of the extra-anatomic connecting passage, type 3 is further subdivided into type 3A and type 3B.

The Role of Exercise Stress Echocardiography for Determination of Subclinical Cardiac Involvement in ß-Thalassemia Major.

ß-Thalassemia major (ß-TM) patients are at increased risk for cardiovascular diseases. Determination of subclinical cardiac involvement is essential for preventive measures. Thus, we aimed to evaluate the role of stress echocardiography for identification of subclinical cardiac dysfunction in ß-TM patients. In this prospective study, 45 ß-TM patients who were referred for cardiac evaluation, were enrolled. Exclusion criteria included non sinus rhythm and overt cardiac disease. Stress echocardiography levels and cardiac magnetic resonance imaging (MRI) results were obtained from ß-TM patients. Patients were divided into two groups of normal vs. iron overload from cardiac T2* greater or less than 20 msec, respectively. Resting and peak exercise right ventricular stroke volume (RVSV) and left ventricular SV (LVSV) were significantly lower in iron overload vs. normal ß-TM patients, respectively (p value <0.05). At peak LV global longitudinal strain (GLS) and myocardial performance index (MPI) were significantly decreased and increased compared with resting in iron overload vs. normal ß-TM patients, respectively (p value <0.05). There was a significant relationship between inappropriate hemodynamic response to exercise and lower age (p value = 0.032). Resting LVSV and RVSV seemed better prognosticators for iron overload than LV ejection fraction (LVEF). Decreased GLS and increased MPI at peak exercise could also predict the presence of cardiac iron overload. These measurements by stress echocardiography could be evaluated when cardiac T2* could not be determined.

Unusual collateral vessel from right subclavian vein to left atrium, a rare complication of superior vena cava obstruction.

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49-year-old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi-lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.

Self-Expandable Stent for Repairing Coarctation of the Left-Circumferential Aortic Arch with Right-sided Descending Aorta and Aberrant Right Subclavian Artery with Kommerell's Aneurysm.

Endovascular treatment offers a great advantage in the management of main arteries stenoses. However, simultaneous presence of a group of anomalies may complicate the situation. Here we present a case of 21-year-old man with aortic coarctation. Radiographic imaging and angiography demonstrated aortic coarctation of the left-circumferential aortic arch, right-sided descending aorta, and Kommerell's diverticulum at the origin of right subclavian artery. These anomalies have rarely been reported to concurrently exist in the same case and the treatment is challenging. Percutaneous treatment for repair of aortic coarctation was successfully performed with deployment of self-expanding nitinol stents. Follow-up demonstrated the correction of blood pressure and improvement of the symptoms. It appears that deployment of self-expandable nitinol stents present a viable option for the management of coarcted aorta in patients having all or some of these anomalies together.

Congenital unruptured aneurysm of sinus of Valsalva, quadricuspid aortic valve, and ascending aortic aneurysm associated with genetic leukoencephalopathy in an infant: A new syndrome?

We report a 15-month-old female with unruptured noncoronary sinus of Valsalva anreurysm (SOVA) associated with quadricuspid aortic valve, ascending aortic aneurysm (AAA), moderate aortic insufficiency, mild mitral insufficiency, genetic leukoencepaholpathy, developmental delay and mild mixed aminoaciduria. She was referred for evaluation of a cardiac murmur. Initial echocardiographic examination showed the aneurysm as a round cyst in the right atrium which changed in size during systole and diastole. As to the best of our knowledge, this is the first report of association of SOVA, quadricuspid aortic valve, AAA and genetic leukoencephalopathy in an infant. These associations may suggest a new syndrome. We describe the echocardiographic and computed tomographic angiography findings. We also review 641 cases in the literature and the current six classification systems for SOVA.

Different Presentation of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Adults: Case Reports.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac malformation. We report three cases of ALCAPA who survived to adulthood. The first case was a 51-year-old woman who complained of typical chest pain that was diagnosed with ALCAPA using cardiac catheterization and coronary computed tomographic angiography (CTA). The second case was a 30-year-old woman with a history of surgery for atrial septal defect at 10 years old who presented with progressive exertional dyspnea. Cardiac catheterization confirmed the diagnosis of ALCAPA. The third case was a 19-year-old man who was brought to our clinic due to aborted sudden cardiac death on the previous day. Cardiac catheterization and coronary CTA confirmed the diagnosis. They underwent the closure of orifice of the anomalous left coronary artery and grafting the left anterior descending artery concomitantly with mitral valve repair. All patients were followed up during a mean of 8.7 months and they were asymptomatic.

Comparison of diagnostic accuracy of dual-source CT and conventional angiography in detecting congenital heart diseases.

BACKGROUND: Cardiac dual-source computed tomography (DSCT) is primarily used for coronary arteries. There are limited studies about the application of DSCT for congenital heart diseases. The aim of this study was to determine the diagnostic value of DSCT in the cardiac anomalies. MATERIAL/METHODS: The images of DSCTs and conventional angiographies of 36 patients (21 male; mean age: 8.5 month) with congenital heart diseases were reviewed and the parameters of diagnostic value of these methods were compared. Cardiac surgery was the gold standard. RESULTS: A total of 105 cardiac anomalies were diagnosed at surgery. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of DSCT were 98.25%, 97.9%, 98.1%, 99.07%, and 98.2%, respectively. The corresponding values of angiography were 95.04%, 98.7%, 97.8%, 98.1%, and 98%, respectively. Only one atrial septal defect (ASD) and two patent ductus arteriosus (PDA) were missed by DSCT. Angiography missed two ASD and two PDA. DSCT also provided important additional findings (n=35) about the intrathoracic or intraabdominal organs. CONCLUSIONS: DSCT is a highly accurate diagnostic modality for congenital heart diseases, obviating the need for invasive modalities. Beside its noninvasive nature, the advantage of DSCT over the angiography is its ability to provide detailed anatomical information about the heart, vessels, lungs and intraabdominal organs.

The value of coronary artery calcium score assessed by dual-source computed tomography coronary angiography for predicting presence and severity of coronary artery disease.

BACKGROUND: Measuring coronary artery calcium score (CACS) using a dual-source CT scanner is recognized as a major indicator for assessing coronary artery disease. The present study aimed to validate the clinical significance of CACS in predicting coronary artery stenosis and its severity. MATERIAL/METHODS: This prospective study was conducted on 202 consecutive patients who underwent both conventional coronary angiography and dual-source (256-slice) computed tomography coronary angiography (CTA) for any reason in our cardiac imaging center from March to September 2013. CACS was measured by Agatston algorithm on non-enhanced CT. The severity of coronary artery disease was assessed by Gensini score on conventional angiography. RESULTS: There was a significant relationship between the number of diseased coronary vessels and mean calcium score, i.e. the mean calcium score was 202.25±450.06 in normal coronary status, 427.50±607.24 in single-vessel disease, 590.03±511.34 in two-vessel disease, and 953.35±1023.45 in three-vessel disease (p<0.001). There was a positive association between calcium score and Gensini score (r=0.636, p<0.001). In a linear regression model, calcium score was a strong determinant of the severity of coronary artery disease. Calcium scoring had an acceptable value for discriminating coronary disease from normal condition with optimal cutoff point of 350, yielding a sensitivity and specificity of 83% and 70%, respectively. CONCLUSIONS: Our study confirmed the strong relationship between the coronary artery calcium score and the presence and severity of stenosis in coronary arteries assessed by both the number of diseased coronary vessels and also by the Gnesini score.