Clinical and imaging characteristics of patients with cardiac amyloidosis- a single center observational study.

Amyloidosis is a disease characterized by the deposition of protein fibrils. Cardiac involvement is a significant factor in determining prognosis. This study aimed to examine the clinical profile, outcomes, and long-term mortality rates in patients with transthyretin (ATTR) and amyloid light-chain (AL) amyloidosis. The retrospective cohort study included 94 patients with amyloidosis (69 with AL and 25 with ATTR amyloidosis) diagnosed between 2010 and 2022. The study involved multimodality imaging (ECG, echocardiography and cardiac magnetic resonance (CMR) data and survival analyses. Patients with ATTR amyloidosis were older and had a higher proportion of males compared to those with AL amyloidosis. Cardiac involvement was more prevalent in the ATTR group, including atrial fibrillation (AF), while pleural and pericardial effusion were more frequent in the AL group. Biomarkers such as NT-proBNP and troponin T were significantly elevated in both groups and were associated with all-cause mortality only in univariate analyses. CMR data, especially typical late gadolinium enhancement (LGE) was not associated with increased mortality, while pleural effusion and left atrial dilatation on echocardiography were identified as powerful predictors of mortality. In conclusion, both AL and ATTR amyloidosis exhibited poor outcomes. Cardiac involvement, particularly dilated left atrium and pleural effusion on echocardiography were associated with an increased risk of mortality, while typical LGE on CMR was not.

Complete recovery after fulminant myocarditis in a patient with COVID-19.

The clinical spectrum of Coronavirus disease 2019 (COVID-19) varies from asymptomatic infection to severe disease with multiorgan dysfunction. Cardiovascular involvement is common and in rare cases can lead to serious complications, such as fulminant myocarditis. The clinical course of COVID-19 myocarditis varies from complete recovery to death in rare cases. The pathophysiology of COVID-19-related myocarditis is still unclear but is believed to involve direct viral injury and cardiac damage due to the host's immune response. Guidelines on the management of COVID-19-related myocarditis are yet to be established. We present here the case of a male patient in his early fifties admitted with life-threatening myocarditis in the course of COVID-19 infection who was successfully treated and recovered without any sequelae.

The Zanzibar Heart Survey: A special report from a humanitarian cardiology program at the Mnazi Mmoja referral hospital, Zanzibar, United Republic of Tanzania by Haukeland University Hospital in Bergen, Norway.

A Norwegian cardiology delegation comprised of Cardiologists and Researchers travelled voluntarily to Zanzibar to undertake 4 humanitarian missions in 2022. The principal aims of this were to: 1) Train local cardiologists in transthoracic echocardiography and perform echocardiographic screening in patients with cardiac symptoms who had not undergone any prior cardiac imaging, 2) Conduct a hypertension survey to improve awareness, treatment and control of hypertension and 3) Implant permanent pacemakers in patients with significant bradyarrhythmias for the first time in the Archipelago. The current report details our experience at the Mnazi Mmoja Referral Hospital. We describe the challenges in managing common cardiovascular conditions such as hypertension, cardiomyopathies, coronary artery disease and rhythm disturbances. Furthermore, we propose that improvement to care may be achieved by implementing systematic access to echocardiography and hypertension services to the island. In our survey, we found that hypertension and hypertension-mediated target organ damage were highly prevalent and hypertension was poorly controlled in Zanzibar. The common reasons for poor BP control were reported to be partly the issue of cost, affordability and availability of antihypertensive medications, and partly due to lack of awareness. Women were on average 10 years younger than men and were more likely to be obese, while men had higher burden of established cardiovascular disease (CAD, stroke, chronic kidney disease, and atrial fibrillation). Humanitarian healthcare missions by Western countries provide invaluable contributions to the healthcare of patients elsewhere in the world. Although their impact can be felt immediately, there is the propensity for these benefits to dissipate rapidly following the departure of visiting delegations. There is a need for more sustainable solutions whereby local healthcare systems are empowered to develop their own local capacities and initiate a system whereby local training can occur, the utilisation of facilities can be maximised and new skills can be transferred to health care practitioners to ensure universal access to diagnostics and treatments of cardiovascular diseases in Zanzibar. Our report indicates that measurable changes can be achieved in a relatively short time frame. These may in turn translate to improvements in access and quality of healthcare to the local population.

"Ion-Flux": The natural course of ECG changes in severe hypokalemia.

Whereas the electrocardiogram (ECG) changes in hypokalemia are well known, they often receive less attention than the more striking features of hyperkalemia. Furthermore, there is a need for further discussion as to the subtleties of ECG changes that can aid in the differential diagnoses. This case study presents the ECG changes of a patient with severe hypokalemia due to diarrhea. It highlights how bifid T-waves in hypokalemia can be distinguished from other conditions such as coronary artery disease or pericarditis. Furthermore, it also shows the gradual reversal of ECG changes in the same patient when potassium is normalized.

Association between Covid-19 infection and platypnea-orthodeoxia syndrome.

Introduction and importance: Platypnea-orthodeoxia syndrome is defined as dyspnoea and deoxygenation when changing from a recumbent to an upright position. Post-Covid-19 sequelae can induce or exacerbate pulmonary hypertension and thereby render a previously mild and asymptomatic platypnea-orthodeoxia syndrome to manifest with new or worsening symptoms. Case presentation: The authors present the case of an 80-year-old man who following an episode of moderate-severe Covid-19 infection developed type I respiratory failure that required hospital discharge with long-term oxygen therapy. He had a background history of postural paroxysmal hypoxaemia which had previously raised the suspicion of a right-to-left shunt through either a patent foramen ovale, atrial septal defect or an intrapulmonary arteriovenous malformation. However, given the low burden of symptoms this was not explored further. Following recovery from Covid-19 infection, the patient experienced marked dyspnoea and oxygen desaturation in an upright position that was relieved by a return to a supine position. Discussion and conclusion: Persistent dyspnoea and hypoxia are common symptoms in patients who experience post-Covid-19 syndrome. However, when patients with prior moderate-to-severe Covid-19 illness present with new onset breathlessness and/or desaturation that is worsened in an upright position, platypnea-orthodeoxia syndrome should be considered.

Impact of aortic valve stenosis on myocardial deformation in different left ventricular levels: A three-dimensional speckle tracking echocardiography study.

BACKGROUND: Global systolic left ventricular (LV) myocardial function progressively declines as degenerative aortic valve stenosis (AS) progresses. Whether this results in uniformly distributed deformation changes from base to apex has not been investigated. METHODS: Eighty-five AS patients underwent three-dimensional (3D) echocardiography in this cross-sectional study. Patients were grouped by peak jet velocity into mild (n = 32), moderate (n = 31), and severe (n = 22) AS. 3D speckle tracking derived strain, rotation, twist, and torsion were obtained to assess global LV function and myocardial function at the apical, mid, and basal levels. RESULTS: Global longitudinal strain (GLS) was lower in patients with severe AS (-16.1 ± 2.4% in mild, -15.5 ± 2.5% in moderate, and -13.5 ± 3.0% in severe AS [all p < .01]). Peak basal and mid longitudinal strain (LS), basal rotation and twist from apical to basal level followed the same pattern, while peak apical LS was higher in moderate AS compared to severe AS (all p < .05). In multivariate analyses, lower GLS was particularly associated with male sex, higher body mass index and peak aortic jet velocity, lower basal LS with higher filling pressure (E/e') and LV mass, lower mid LS with higher RWT and presence of AS symptoms, and lower apical LS with male sex and higher systolic blood pressure, respectively (all p < .05). CONCLUSION: Using 3D speckle tracking echocardiography reveals regional and global changes in LV mechanics in AS related to the severity of AS, LV remodeling and presence of cardiovascular risk factors.

Echocardiography Assessment of Rheumatic Heart Disease: Implications for Percutaneous Balloon Mitral Valvuloplasty.

Echocardiography is an important diagnostic imaging modality in recognizing rheumatic heart disease, a chronic sequelae of acute rheumatic fever. Left-sided heart valves, especially the mitral valve is typically affected, with stenosis or regurgitation as a consequence. Although assessment of valve area by 2D planimetry is the reference method for mitral stenosis severity, 3D planimetry provides more accurate measurement and diagnostic value. Careful selection of patients in terms of echocardiographic criteria is essential to ensure safety and success of the intervention and better long-term outcomes. Several echocardiographic scores based upon mitral valve mobility, thickening, calcification, and subvalvular thickening are developed to assess mitral valve anatomy and the feasibility of percutaneous mitral commissurotomy. 3D transesophageal echocardiography (TEE) provides detailed information of the mitral anatomy (commissural fusions, and subvalvular apparatus) before intervention. In addition, 3D TEE planimetry provides a more accurate measurement of the valve area compared with 2D echocardiography. Generally, huge annular calcification and lack of commissural fusion are unfavorable echocardiographic markers that increase the risk of complications and preclude the feasibility of percutaneous balloon mitral valvuloplasty. More contemporary prospective echocardiography research studies on patients with RHD from low- and middle-income countries are needed.

Troponin T and N-Terminal Pro-Brain Natriuretic Peptide Are Associated with Long-Term All-Cause Mortality in Patients with Post-Sternotomy Mediastinitis following Coronary Artery Bypass Grafting: A 15-Year Follow-Up Study.

BACKGROUND: Deep sternal wound/mediastinitis is a rare but feared complication in coronary artery bypass grafting (CABG) patients and seems to increase the risk of cardiac death, and is also associated with the risk of early internal mammary artery (IMA) graft obstruction. The pathological mechanism explaining the link between mediastinitis and IMA graft obstruction and the impact on mortality is complex, multifactorial, and not fully investigated. OBJECTIVES: Mediastinitis has been associated with increased concentrations of N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin T (TnT) at mid-term follow-up, representing persistent low-grade myocardial injury and impaired cardiac function. However, whether mediastinitis is associated with all-cause mortality, or whether the association is driven by these cardiac-specific biomarkers (NT-proBNP and TnT), is not investigated. METHODS: The present study provides the longest and most complete follow-up data in 82 patients undergoing CABG, including 41 with post-sternotomy mediastinitis. RESULTS: The annualized incidence rate of mediastinitis was 0.14%/year and remained stable at 0.14% throughout the study period. During a mean follow-up of 12.7 ± 3.5 years, a total of 42 deaths occurred (27 [65.9%] in mediastinitis and 15 [36.6%] in non-mediastinitis group, p = 0.008). No association was found between IMA or saphenous vein graft obstruction with all-cause mortality. Mediastinitis was associated with a 1.9-fold increased risk of all-cause mortality. However, in the multivariable-adjusted models, age and higher TnT and NT-proBNP levels, but not mediastinitis per se were associated with all-cause mortality. CONCLUSIONS: Mediastinitis after CABG surgery was associated with a poor prognosis during a 15-year follow-up, showing a nearly two-fold higher frequency of all-cause mortality compared with non-mediastinitis group, with the differences in mortality rate occurring primarily after 10 years. The association between mediastinitis and all-cause mortality was modulated by subclinical myocardial damage and stretch, reflected by elevated TnT and NT-proBNP, measured at 2.7-year follow-up, underscoring that these could represent prognostic markers in CABG patients.

A BRASH Diagnosis With a Timely Intervention.

The BRASH (bradycardia, renal failure, atrioventricular block, shock, and hyperkalaemia) syndrome is a recently recognized condition which may lead to life-threatening complications if not correctly identified and treated early. We report here the case of a 74-year-old woman with type 2 diabetes, hypertension and atrial flutter who presented to the emergency department with 2-day history of dizziness, presyncope, and bradycardia, and a junctional rhythm at 61 beat per minute on initial ECG. She was on apixaban, digoxin, prazosin, and telmisartan. Serum biochemistry revealed severe hyperkalaemia with a potassium 8.4 mmol/L, creatinine 161 mmol/L, glucose 15.3 mmol/L and an upper normal digoxin level of 1.2 mmol/L (ref. 0.6-1.2). Arterial blood pH was 7.2. Given the constellation of biochemical and clinical findings a diagnosis of BRASH syndrome was made, though her blood pressure values at presentation were rather high (180/65-179/59 mmHg). The patient was rapidly stabilised with the administration of intravenous insulin and dextrose, fluid resuscitation, and zirconium cyclosilicate (SZC), followed by haemodialysis. Following the correction of the serum potassium to 4.7 mmol/L, a further ECG performed 6 hours later, showed a restoration of sinus rhythm with a rate of 65 bpm, normalization of the QRS duration. The digoxin and telmisartan were discontinued, and the patient was commenced on a calcium channel antagonist for hypertension. Clinicians should be alerted to patients who present with either a BRASH (shock) or BRAHH (hypertensive manifestation) where timely intervention is essential to avoid life-threatening brady-and tachyarrhythmias in these patients.

Long-term multimodality imaging follow-up of ST-segment elevation myocardial infarction patients treated with bioresorbable vascular scaffold: advantages and challenges.

BACKGROUND: Multislice computed tomography (MSCT) offers a non-invasive method of imaging bioresorbable scaffolds (BRS). OBJECTIVES: To investigate the advantages and challenges using MSCT in the follow-up after BRS implantation. METHOD: The BRS cohort consisting of 31 patients in the 'BRS in STEMI' trial was examined by multimodality imaging and followed long-term. Minimum lumen area (MLA) and average lumen area (ALA) were examined 12 and 36 months after BRS implantation with MSCT. Optical coherence tomography (OCT) at 12 months was used as a reference. RESULTS: Measured by MSCT, the mean MLA was 0.05 ±â€…1.32 mm² ( P  = 0.85), but ALA was 1.32 (±2.59 mm², P  = 0.015) greater than by OCT. ALA and MLA did not change significantly from 12 to 36 months. MSCT identified all cases of restenosis but missed one patient with massive malapposition. CONCLUSION: Our data support using MSCT in the follow-up after BRS implantation. Invasive investigation should still be considered for patients with unexplained symptoms.

Cardiac Involvement in Systemic and Local Vasculitides: The Value of Noninvasive Multimodality Imaging.

Despite significant advances in managing systemic vasculitides, cardiovascular morbidity, and mortality are still of primary concern. Advances in noninvasive imaging have broadened our understanding of the clinical heterogeneity of cardiac involvement in vasculitides. Common cardiovascular complications in primary or secondary vasculitides are; coronary artery aneurysms, acute coronary syndromes, myocarditis, pericarditis, endocarditis, and valvular dysfunction. Echocardiography, cardiac magnetic resonance , positron emission tomography, and computed tomography angiography are essential in identifying cardiac involvement and guiding treatment. Here, we present our experiences of cardiac involvement in systemic vasculitides, covering most aspects of common cardiac complications based on a multi-modality approach to challenging (real-world) cases. As many cardiac manifestations are clinically silent, heart function should be systemically assessed by a multimodality imaging-based approach, including ECG, serial echocardiograms with strain imaging and 3D, and cardiac magnetic resonance to detect early signs of cardiac manifestations. This enables timely intervention and optimal medical treatment, which is essential for a better prognosis. There is a need for better and closer collaboration in clinical practice and research fields between cardiologists and rheumatologists.

B-vitamin Treatment Modifies the Mortality Risk Associated with Calcium Channel Blockers in Patients with Suspected Stable Angina Pectoris: A Prospective Cohort Study.

BACKGROUND: Calcium channel blockers (CCBs) are used for the treatment of cardiovascular disease (CVD), including angina pectoris, and hypertension; however, the effect on survival remains uncertain. CCBs impair fibrinolysis and have been linked to elevated plasma homocysteine (Hcy), a CVD risk marker. OBJECTIVE: We explored the association between CCB use and mortality in a large prospective cohort of patients with suspected stable angina pectoris (SAP), and potential effect modifications by Hcy-lowering B-vitamin treatment (folic acid, B12, and/or B6) as 61.8% of the patients participated in a randomized placebo-controlled B-vitamin intervention trial. METHODS: Patient baseline continuous characteristics according to CCB treatment were tested by linear regression. Hazard ratios (HRs) for mortality associated with CCB treatment, also according to B-vitamin intervention, were examined using Cox regression analysis. The multivariable model included CVD risk factors, medical histories, and the use of CVD medications. RESULTS: A total of 3991 patients (71.5 % men) were included, of whom 907 were prescribed CCBs at discharge. During 10.3 years of median follow-up, 20.6% died and 8.9% from cardiovascular- and 11.7% from non-cardiovascular causes. Patients treated with CCBs had higher plasma Hcy, fibrinogen levels, and erythrocyte sedimentation rate (all P<0.001). Furthermore, CCB use was positively associated with mortality, also after multivariable adjustments (HRs [95% CIs]: 1.34 [1.15,1.57], 1.35 [1.08,1.70], and 1.33 [1.09,1.64] for total, CVD, and non-CVD death, respectively). Numerically stronger associations were observed among patients not treated with B-vitamins (HR [95% CI]: 1.54 [1.25, 1.88], 1.69 [1.25, 2.30], and 1.41 [1.06, 1.86] for total, CVD deaths, and non-CVD deaths, respectively), whereas no association was seen in patients treated with B-vitamins (HR [95% CI]: 1.15 [0.91, 1.46], 1.09 [0.76, 1.57], and 1.20 [0.88, 1.65]). CONCLUSIONS: In patients with suspected SAP, CCB treatment was associated with increased mortality risk primarily among patients not treated with B-vitamins.

Natural Course of Electrocardiogram Changes and the Value of Multimodality Imaging in Acute Pericarditis.

BACKGROUND: ECG is the initial diagnostic tool that in combination with typical symptoms often raises the suspicion of pericarditis. Echocardiography remains the first-line imaging modality for assessment of pericardial diseases, particularly effusion/tamponade, constrictive physiology, and assessment of regional wall motion abnormalities as differential diagnoses. However, cardiac CT and cardiac magnetic resonance may be necessary in complicated cases and to identify pericardial inflammation in specific settings (atypical presentation, new onset constriction), as well as myocardial involvement and monitoring the disease activity. SUMMARY: In acute pericarditis, the most commonly used ECG criteria recommended by international guidelines are the widespread ST-segment elevation or PR depression. However, the classic ECG pattern of widespread ST-segment elevation or PR depression can be seen in less than 60% of patients. In addition, ECG changes are often temporally dynamic, evolve rapidly during the course of disease, and may be influenced by a number of factors such as disease severity, time (stage) of presentation, degree of myocardial involvement, and the treatment initiated. Overall, temporal dynamic changes on ECG during acute pericarditis or myopericarditis have received limited attention. Hence, the aim of this brief clinical review was to increase awareness about the various ECG changes observed during the course of acute pericarditis. KEY MESSAGES: ECG may be normal at presentation or for days after the index episode of chest pain, but serial ECGs can reveal specific patterns of temporally dynamic ST elevation in patients with pericarditis or myopericarditis, particularly during new episodes of chest pain.