Supporting Role of Adequate Affected Tissue Biopsy in the Diagnostic Algorithm for Cardiac Amyloidosis.

Amyloidosis is a pathology that occurs as a result of the accumulation of various misfolded proteins in the extracellular space. It is a significant cause of morbidity and mortality due to multi-organ involvement. One of the most important determinants of mortality and morbidity is cardiac involvement. Cardiac amyloidosis (CA) may present with a variety of clinical findings. In this article, we aim to demonstrate the supportive role of cardiac and extra-cardiac tissue in the routine diagnostic pathway for CA.

A Cardiac Magnetic Resonance Study: Comparison of Biventricular Longitudinal Function in Hypertrophic Cardiomyopathy Patients and Normal Individuals.

OBJECTIVE:  Hypertrophic cardiomyopathy (HCM) is a genetic disease with an incidence of 0.2%-0.5%. It has a wide range of clinical presentations varying from coincidental diagnoses to heart failure, ventricular arrhythmias and sudden cardiac death. Mitral annular plane systolic excursion (MAPSE) and tricuspid annular plane systolic excursion (TAPSE) are M-mode-derived practical and reproducible measurements of systolic longitudinal displacement of the annular plane. These two measures may be used as markers of the left ventricular and right ventricular longitudinal functions. Currently, there are only a few studies on cardiac magnetic resonance (CMR)-derived TAPSE and MAPSE measurement comparison between the HCM group and normal control group. The aim of our study is to show the differences in CMR-derived TAPSE and MAPSE values between the HCM and normal population. METHODS:  We evaluated CMR exams of patients diagnosed with HCM and of normal individuals scanned between 2020 and 2021 retrospectively. The patients were from our own institution's and other hospitals' in- and out-patient departments. Data was collected on 36 HCM patients and 34 adults with no known history of cardiac and non-cardiac diseases. All CMR exams were performed on a 1.5 T (Magnetom Avanto, Siemens Healthcare, Erlangen, Germany) scanner. CMR-derived MAPSE and TAPSE were measured on standard four-chamber steady-state free precession (SSFP) cine images and given in millimeters. RESULTS:  From February 2020 to December 2021, a total of 150 patients were diagnosed with hypertrophic cardiomyopathy. After exclusion, 36 patients with HCM were included in the study and the normal control group comprised 34 individuals. The mean age of the HCM group was 43.2 + 13.5 years, while it was 37.5 + 11.3 in the control group. The female ratio of the HCM group was found to be 36%, while it was 56% in the healthy control group. MAPSE values were significantly higher in the normal control group when compared to the HCM patient group (MAPSE: 14.5 ± 2.9 mm vs. 11.7 ± 3.2 mm; p<0.001), while TAPSE values did not depict a significant difference between the two groups (p=0.627). CONCLUSIONS:  This study suggests that MAPSE values are significantly lower in the HCM patient group in comparison with the normal control group on CMR scans. Although not statistically significant, TAPSE values are also lower in the HCM group.

High take off left main coronary artery accompanied by multicryptic left ventricle myocardium detected by cardiac computerized tomography in a young male: case report.

BACKGROUND: Myocardial crypts are discrete, narrow, blood filled invaginations within the left ventricular myocardium and high-take-off coronary artery are rare manifestations where coronary arteries originate above the sinotubuler junction. CASE PRESENTATION: A 41-year-old man with multiple coronary artery disease risk factors admitted to our outpatient department with progressive dyspnea and atypical chest pain. Physical examination revealed no pathological findings. His blood examination revealed only mild to moderately high IgE and LDL levels. Transthoracic echocardiography (TTE) was normal. His treadmill test was normal, yet in the 3rd stage of the test he had an atypically located chest pain which was relieved in the resting period. As he had multiple cardiovascular risk factors, we performed a coronary CT angiography to exclude coronary artery disease. Coronary CT angiography(CCTA) demonstrated multiple myocardial crypts, a muscular VSD like defect which were not detectable with TTE and a high take off left main coronary artery (LMCA). After CCTA, we repeated the TTE to investigate the crypts and VSD-like defect which were clear on CCTA, yet a precise TTE hardly showed crypts and didn't confirm a shunt between the left and right ventricle. We defined the defect as 'spontaneously closed muscular VSD'. None of these pathologies were clinically relevant with the patient's symptoms, thus pneumonology started a montelukast therapy for 1 year and we decided to follow up the patient, as multiple crypts may indicate an early phase hypertrophic cardiomyopathy. CONCLUSIONS: Considering that a high take-off LMCA is a congenital anomaly, encountering multiple crypts which are also congenital pathologies, is plausible, as congenital anomalies may accompany eachother. Echocardiography is a very useful, practical imaging tool but regrettably may be suboptimal due to various patient and method related reasons. Target combination of different cardiovascular imaging tools like echocardiography, cardiac CT(CCT), may be utilized in order to ensure a comprehensive diagnosis particularly.

Acute pulmonary embolism in a patient with hepatitis C virus infection and hepatocellular carcinoma: a case report.

BACKGROUND: Cardiac metastases in hepatocellular carcinoma patients are infrequently encountered and usually associated with a very poor prognosis. CASE PRESENTATION: Hereby, we report a case of an acute pulmonary embolism (PE) on top of HCC with direct cardiac invasion to the right atrium (RA) through the inferior vena cava with another metastasis to the right ventricular apex in the form of highly mobile cauliflower mass protruding through the tricuspid valve into RA and nearly obliterating right ventricular outflow tract in a multi-centric hepatocellular carcinoma patient. CONCLUSION: Acute dyspnea in a patient with a long history of hepatitis C virus infection raises the suspicion of acute PE due to either hypercoagulable state induced by malignancy or by cardiac extension of the tumor which usually carries high mortality rates. To the best of our knowledge, this case is the first case in the literature to show cardiac metastases in HCC with two different pathological mechanisms.

A 19-Year-Old Pregnant Woman With Pulmonary Hypertension With Progressive Dyspnea.

CASE PRESENTATION: A 19-year-old pregnant woman at week 32 of gestation was referred to our clinic with progressive shortness of breath for the further evaluation and treatment of high-risk pregnancy. Her complaints had been existing since her childhood. Two years prior to her admission, she had been diagnosed with heart failure with preserved ejection fraction due to cardiomyopathy and associated pulmonary hypertension. The patient had no family history of any cardiac disease. She had never smoked or drunk alcohol. Her clinical condition had deteriorated progressively with the pregnancy.

Comprehensive Assessment of Heart Failure with Preserved Ejection Fraction Using Cardiac MRI.

Heart failure with preserved ejection fraction (HFpEF) burden is increasing. Its diagnostic process is challenging and imprecise due to absence of a single diagnostic marker, and the multiparametric echocardiography evaluation needed. Left ventricular (LV) ejection fraction (LVEF) is a limited marker of LV function; thus, allocating HF phenotypes based on LVEF can be misleading. HFpEF encompasses a broad spectrum of causes, and its diagnostic criteria give a central role to echocardiography, a first-line technique with inherent limitations related to ultrasound capabilities. Conversely, cardiac magnetic resonance provides superior anatomic and functional assessment, enabling tissue characterization, offering unprecedented diagnostic precision.

An indirect effect of covid-19 on the heart: A case report.

Covid-19 virus has been recognized to develop major cardiac complications. The indirect effect of the virus though, in terms of fear for hospital admissions, constitutes a greater threat. In this case we present a 69-year old male patient who suffered from a myocardial infarction that has not been given the proper attention due to the fear of in-hospital contact with covid patients. The result was the delayed revascularization and eventually the development of heart failure. This case presents the full range of covid-19 affection to the heart and raises the public awareness for not underestimating symptoms suggesting life-threatening conditions.

Myocardial Work: Methodology and Clinical Applications.

A precise and accurate assessment of left ventricular (LV) contractility is of utmost importance in terms of prognosis in most cardiac pathologies. Given the limitations of ejection fraction (EF) and global longitudinal strain (GLS) due to their load dependency, a novel imaging tool called myocardial work (MW) has emerged as a promising method for LV performance evaluation. MW is a novel, less load-dependent method based on computation of myocardial strain-arterial blood pressure curves. This method provides a more detailed assessment of segmental and global LV function incorporating the patient's LV pressure and is derived by brachial artery pressure utilizing an empiric reference curve adjusted to the duration of the isovolumic and ejection phases as determined by echocardiography. The clinical implications of this unique method have been expanding in the last few years, which attest to the robust additive role of MW in routine practice.

Interarm blood pressure differences and 2-year mortality in acute coronary syndrome patients.

BACKGROUND AND AIMS: Interarm blood pressure difference (IABPD) was associated with increased cardiovascular and all-cause mortality in various cohorts previously. In this study, we planned to explore the association between the IABPD obtained with simultaneous measurements in both arms and the risk of mortality over a 2-year follow-up of patients with acute coronary syndrome (ACS). METHODS: Simultaneous blood pressure (BP) measurements were performed during initial admission in patients with ACS. Systolic ≥10 mmHg and diastolic ≥5 mmHg absolute IABPD was defined as cutoff values in this study. The relationship of IABPD and all-cause mortality was assessed using Kaplan-Meier curves and Cox analysis. RESULTS: A total of 532 patients with ACS were included in the study. Mean age of the study participants was 60.1 ± 12.6. Patients included in the study were followed for 23.2 ± 7.2 months (median 25.3, min: 0, max: 28.7 months). Survival was assessed using Kaplan-Meier curves. Patients with systolic IABPD ≥ 10 mmHg and systolic IABPD < 10 mmHg had an average survival time of 25.94 ± 0.84 and 25.92 ± 0.38 months (P = 0.925), respectively. Survival times of diastolic IABPD ≥5 mmHg and diastolic IABPD <5 mmHg were 26.44 ± 0.62 and 25.71 ± 0.41 (P = 0.251) months, respectively. CONCLUSIONS: In the current study, we did not find a significant association between IABPD and all-cause mortality in patients with ACS in 2-years follow-up. Future studies may be required for further evaluation of the prognostic importance of IABPD in patients with ACS.

Emergency TAVI in a critically ill patient: A case report.

Transcatheter aortic valve implantation is a safe procedure even in inoperable patients with multi-organ failure and cardiogenic shock. In such cases, the heart team should be prepared to proceed to emergent implantation for timely and successful management of the patient.

Ascending aorta mycotic aneurysm in a child secondary to subacute infective endocarditis on the basis of congenital aortic stenosis demonstrated by cardiac computerized tomography.

Infective endocarditis (IE) is rarely seen in children, and it can cause significant morbidity and mortality. Congenital heart disease is a predisposing cardiac condition for IE and has an increasing prevalence. A mycotic aortic aneurysm is a rare disease due to infection and it has been reported that it may be a result of IE. We present a 10-year-old boy who was referred to our cardiovascular surgery department due to severe aortic stenosis (AS). Transthoracic echocardiography revealed calcific severe AS and computerized tomography (CT) angiography was performed to better evaluate the ascending aorta. CT angiography demonstrated a saccular aneurysm of the ascending aorta. The underlying pathology was considered as chronic IE on top of congenital AS with an unknown number of cusps. Histologic findings supported our diagnosis and accompanied by a saccular aneurysm. The patient underwent a successful aortic root enlargement, mechanical aortic valve replacement, and ascending aorta repair surgery.

Differential diagnosis of apical hypertrophic cardiomyopathy and apical displacement of the papillary muscles: a multimodality imaging point of view.

Apical hypertrophic cardiomyopathy (ApHCM) and apical displacement of papillary muscles (ADPM) are two different pathologies with a number of similar imaging findings that may hamper adequate diagnosis. While ApHCM is associated with increased rate of mortality, ADPM commonly presents with a benign course and differential diagnosis is of great importance. Clinical assessment and 2D echocardiography cannot sufficiently differentiate these conditions, however, and advanced echocardiographic methods may facilitate diagnosis. Although echocardiography is the first-line imaging method in the diagnostic algorithm, cardiac magnetic resonance imaging (CMRI) is the gold standard for evaluating patients due to good spatial resolution and myocardial tissue characterization abilities. When CMRI is contraindicated, cardiac computed tomography may be an alternative reliable method that can also give information about the coronary anatomy. Nuclear imaging may also provide supplementary data regarding hypertrophy and coronary arteries when there is a suspicion of ischemia.

Gender-related clinical and management differences in patients with chronic heart failure with reduced ejection fraction.

AIM: Gender-related differences have been described in the clinical characteristics and management of patients with chronic heart failure with reduced ejection fraction (HFrEF). However, published data are conflictive in this regard. METHODS: We investigated differences in clinical and management variables between male and female patients from the ATA study, a prospective, multicentre, observational study that included 1462 outpatients with chronic HFrEF between January and June 2019. RESULTS: Study population was predominantly male (70.1%). In comparison to men, women with chronic HFrEF were older (66 ± 11 years vs 69 ± 12 years, P < .001), suffered more hospitalisations and presented more frequently with NYHA class III or IV symptoms. Ischaemic heart disease was more frequent in men, whereas anaemia, thyroid disease and depression were more frequent in women. No difference was seen between genders in the use rate of renin-angiotensin system inhibitors, beta-blockers, mineralocorticoid receptor antagonists, or ivabradine, or in the proportion of patients achieving target doses of these drugs. Regarding device therapies, men were more often treated with an implantable cardioverter-defibrillator (ICD) and women received more cardiac resynchronisation therapy. CONCLUSION: In summary, although management seemed to be equivalent between genders, women tended to present with more symptoms, require hospitalisation more frequently and have different comorbidities than men. These results highlight the importance of gender-related differences in HFrEF and call for further research to clarify the causes of these disparities. Gender-specific recommendations should be included in future guidelines in HFrEF.

A real-life correlation between clinical SYNTAX score II and carotid intima-media thickness in patients with stable coronary artery disease.

BACKGROUND: SYNTAX score II (SSII) is an update of the established SYNTAX score (SS) that uses clinical variables such as age, sex, creatinine clearance, left ventricular ejection fraction, chronic obstructive pulmonary disease, and peripheral arterial disease. Furthermore, SSII has been proven to be a more powerful predictive tool than SS in patients with complex coronary artery disease (CAD). Carotid õntima-media thickness (CIMT) is a widely used noninvasive evidence for subclinical or early atherosclerosis, and it was proved to be an independent predictor for cardiovascular events. Most of the previously published articles studied the association between the CIMT with old cardiovascular scoring systems such as SSI and Gensini score with debatable data about their correlation. AIM: To study the correlation between SSII and CIMT in stable CAD patients undergoing elective coronary angiography (CA). METHOD AND PATIENTS: A prospective study including 155 patients undergoing elective CA for stable CAD excluding patients with history of acute coronary syndrome, previous coronary revascularization either by percutaneous coronary intervention or coronary artery bypass grafting, and previous cerebrovascular stroke. RESULTS: The mean age of patients was 58.25 ± 16.46 years, and 79 patients (50.96%) were men. The mean SSII score was 10.23 ± 11.36 and mean CIMT was 0.85 ± 0.24. The correlation between SSII and CIMT using Spearman correlation showed a strong correlation between SSII score and CIMT with correlation coefficient r = 0.752. CONCLUSION: The study showed a strong positive correlation between SSII and CIMT in stable CAD patients undergoing elective CA.

Association of abnormal coronary sinus reflux with coronary slow flow and importance of the Thebesian valve.

BACKGROUND: The relationship between coronary slow flow (CSF) and coronary sinus (CS) reflux has not been previously studied. This study aimed to investigate the relationship between CSF and CS reflux and Thebesian valve presence as well as the relationship between CS reflux and serum annexin-V and ICAM-1 levels. METHODS: In this case-control study, patients were divided into two groups: CSF (n = 13) and control (n = 7) groups. CS flow parameters and Thebesian valve presence were evaluated by cardiac magnetic resonance (CMR). Moreover, serum ICAM-1 and annexin-V levels were measured. RESULTS: Regurgitation volume and regurgitation fraction, indicators of reflux flow in CS, were higher in the CSF group than in the control group (p = 0.039 and p = 0.019). Fewer Thebesian valves were observed in the CSF group than in the control group (p = 0.022). Furthermore, a positive correlation was found between regurgitation volume and regurgitation fraction and serum annexin-V and ICAM-1 levels (r = 0.813, p < 0.001 and r = 0.996, p < 0.001; r = 0.817, p < 0.001 and r = 0.993, p < 0.001, respectively). CONCLUSIONS: This study revealed the significant relationship between CSF and reflux flow in CS. The fact that the patients in the CSF group have fewer Thebesian valves suggests the importance of the valve in preventing backward flow from the coronary vein. A positive correlation between serum ICAM-1 and annexin-V levels with regurgitation volume and regurgitation fraction indicates that after a certain threshold, CS reflux should be considered an abnormal condition.

Adherence to guideline-directed medical and device Therapy in outpAtients with heart failure with reduced ejection fraction: The ATA study.

OBJECTIVE: Despite recommendations from heart failure guidelines on the use of pharmacologic and device therapy in patients with heart failure with reduced ejection fraction (HFrEF), important inconsistencies in guideline adherence persist in practice. The aim of this study was to assess adherence to guideline-directed medical and device therapy for the treatment of patients with chronic HFrEF (left ventricular ejection fraction ≤40%). METHODS: The Adherence to guideline-directed medical and device Therapy in outpAtients with HFrEF (ATA) study is a prospective, multicenter, observational study conducted in 24 centers from January 2019 to June 2019. RESULTS: The study included 1462 outpatients (male: 70.1%, mean age: 67±11 years, mean LVEF: 30%±6%) with chronic HFrEF. Renin-angiotensin system (RAS) inhibitors, beta-blockers, mineralocorticoid receptor antagonists (MRAs), and ivabradin were used in 78.2%, 90.2%, 55.4%, and 12.1% of patients, respectively. The proportion of patients receiving target doses of medical treatments was 24.6% for RAS inhibitors, 9.9% for beta-blockers, and 10.5% for MRAs. Among patients who met the criteria for implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy (CRT), only 16.9% of patients received an ICD (167 of 983) and 34% (95 of 279) of patients underwent CRT (95 of 279). CONCLUSION: The ATA study shows that most HFrEF outpatients receive RAS inhibitors and beta-blockers but not MRAs or ivabradin when the medical reasons for nonuse, such as drug intolerance or contraindications, are taken into account. In addition, most eligible patients with HFrEF do not receive target doses of pharmacological treatments or guideline-recommended device therapy.

Assessment of atrial electromechanical delay and P wave dispersion in patients with chronic obstructive pulmonary disease.

OBJECTIVE: Chronic obstructive pulmonary disease (COPD) is associated with atrial fibrillation (AF) and reduced forced expiratory volume (FEV1) is an independent predictor for new onset AF. The aims of this study were (1) to analyze the atrial electromechanical delay (AEMD) and P wave dispersion which are two predictors of AF development in patients with COPD and without any cardiovascular disease, and (2) to assess the relationship of those with pulmonary functions as quantified by FEV1 measurements. METHODS: The study included 41 patients with COPD (33 male; mean age: 51 years) and 32 healthy controls. P wave dispersion was calculated as the difference between the maximum and minimum P wave duration in a 12-lead surface electrocardiography (ECG) recording. AEMD, defined as the time interval from the P wave onset on the ECG to the initiation of the late diastolic (Am) wave using a tissue Doppler examination, was measured from the lateral mitral annulus (LAEMD), septal annulus (SAEMD), and tricuspid lateral annulus (TAEMD). RESULTS: P wave dispersion was significantly longer in the COPD group than those in the controls (76±19 ms vs. 45±10 ms; p<0.001). All of the AEMD measurements demonstrated significant prolongation in patients with COPD (LAEMD: 74±9 ms vs. 64±11 ms; SAEMD: 66±10 ms vs. 57±12 ms; and TAEMD: 65±9 ms vs. 46±7 ms; p<0.001 for all). The only correlation with FEV1 was observed in the TAEMD values of the COPD group (rs: -401; p<0.009). CONCLUSION: Both P wave dispersion and AEMD parameters were significantly longer in COPD patients without any established structural or functional cardiac abnormalities, indicating an increased tendency for AF development, beginning from the initial stages of the disease.

Frequency of de novo atrial fibrillation in patients presenting with acute ischemic cerebrovascular stroke.

BACKGROUND: Atrial fibrillation (AF) affects millions of people worldwide and can remain undiagnosed for years. It is a major cause of cerebrovascular stroke (CVS); hence, early detection is extremely important in order to decrease the risk of CVS. We conducted a retrospective observational study looking into the prevalence of silent AF in 3299 patients admitted from January 2014 to December 2017 in a tertiary care stroke specialized center. Ischemic CVS was confirmed either by using multislice computed tomography (MSCT) or magnetic resonance imaging (MRI) of the brain. AF was diagnosed by electrocardiography (ECG) at the time of admission or during the hospital stay. Patients with a history of AF were excluded from the study. RESULTS: Of the 3299 patients admitted by acute ischemic CVS, 707 (21.43%) had a history of AF and thus were excluded from the study. Of the remaining 2592 patients eligible for the study, 1666 (64.27%) were males with a mean age of 56.06 years (± 16.01). A total of 2313 (89.24%) patients remained in sinus rhythm throughout the hospital stay, 211 (8.14%) patients were in AF on admission, and 68 (2.62 %) patients developed AF during their hospital stay. The total number of newly diagnosed patients with AF was 279 (10.76%). CONCLUSION: The prevalence of de novo atrial fibrillation in patients presented with acute cerebrovascular stroke is high. The implementation of good screening programs can significantly reduce the risk of disabilities and morbidities.