Review of Mavacamten for Obstructive Hypertrophic Cardiomyopathy and Future Directions.

Hypertrophic cardiomyopathy (HCM) is a condition with abnormal hypertrophy of the left ventricle in the absence of common causes. The most common form involves the basal septum and can lead to obstruction of the left ventricular outflow tract. Patients can experience exertional symptoms such as chest pain, dyspnea and syncope. Traditional treatment has included beta blockers and nondihydropyridine calcium channel blockers with second-line therapy being disopyramide. Recently, mavacamten, a cardiac myosin inhibitor, has demonstrated improvement in quantitative measures of obstruction and symptom relief to such a degree that patients were able to defer invasive management of the disease. This review focuses on the pharmacology of mavacamten, its clinical trial data and guidance on how to incorporate this drug into clinical practice. Furthermore, it discusses emerging therapies currently being investigated for HCM.

Advances in multi-modality imaging for constrictive pericarditis and pericardial inflammation: role of imaging-guided therapy.

INTRODUCTION: Constrictive pericarditis (CP) can result from uncontrolled inflammation of the pericardium. This can be due to various etiologies. CP can lead to both left- and right-sided heart failure with associated poor quality of life, so early recognition is key. The evolving role of multimodality cardiac imaging allows for earlier diagnosis and facilitates management to help mitigate this adverse outcome. AREAS COVERED: This review discusses the pathophysiology of constrictive pericarditis, chronic inflammation and autoimmune etiologies, clinical presentation of CP, and advances in multimodality cardiac imaging for diagnosis and management. Echocardiography and cardiac magnetic resonance (CMR) imaging remain cornerstone modalities to evaluate this condition, whereas additional imaging modalities such as computed tomography and FDG-positron emission tomography can provide complementary information. EXPERT OPINION: Advances in multimodality imaging allow for a more precision diagnosis of constrictive pericarditis. There has been a paradigm shift in pericardial disease management with advances in multimodality imaging, especially CMR, to detect subacute and chronic inflammation. This has enabled imaging-guided therapy (IGT) to both help prevent and potentially reverse established constrictive pericarditis.

Characteristics and immediate outcomes of patients who underwent percutaneous balloon mitral valvuloplasty at the Jakaya Kikwete Cardiac Institute, Tanzania.

BACKGROUND: For rheumatic mitral stenosis (MS), a multidisciplinary evaluation is mandatory to determine the optimal treatment: medical, percutaneous balloon mitral valvuloplasty (PBMV) or valve surgery. Clinical and imaging evaluations are essential for procedural risk assessment and outcomes. PBMV interventions are increasingly available in Africa and are feasible options for selected candidates. Enhancing PBMV training/skills transfer across most of African countries is possible. OBJECTIVES: The aim of this study was to provide insight into the clinical practice of patients with rheumatic MS evaluated for PBMV in a Tanzanian teaching hospital and to define the role of imaging, and evaluate the heart team and training/skills transfer in PBMV interventions. METHODS: From August 2019 to May 2022, 290 patients with rheumatic MS were recruited consecutively in the Tanzania Mitral Stenosis study. In total, 43 (14.8%) patients were initially evaluated for eligibility for PBMV by a heart team. We carried out the clinical assessment, laboratory investigations, transthoracic/oesophageal echocardiography (TTE/TEE) and electrocardiography. RESULTS: The median age was 31 years (range 11-68), and two-thirds of the patients were female (four diagnosed during pregnancy). Two patients had symptomatic MS at six and eight years. Nine patients had atrial fibrillation with left atrial thrombus in three, and two were detected by TEE. Nine patients in normal sinus rhythm had spontaneous echo contrast. The mean Wilkins score was 8.6 (range 8-12). With re-evaluation by the local and visiting team, 17 patients were found to have unfavourable characteristics: Bi-commissural calcification (four), ≥ grade 2/4 mitral regurgitation (six), high scores and left atrial thrombus (three), left atrial thrombus (two), and severe pulmonary hypertension (two). Three patients died before the planned PBMV. Eleven patients were on a waiting list. We performed PBMV in 12 patients, with success in 10 of these, and good short-term outcomes [mean pre-PBMV (16.03 ± 5.52 mmHg) and post-PBMV gradients (3.08 ± 0.44 mmHg, p < 0.001)]. There were no complications. CONCLUSIONS: PBMV had good outcomes for selected candidates. TEE is mandatory in pre-PBMV screening and for procedural guidance. In our cohort, patients with Wilkins score of up to 11 underwent successful PBMV. We encourage PBMV skills expansion in low- and middle-income countries, concentrating on expertise centres.

Fibromuscular dysplasia in a middle-aged transgender man: The role of hormones in disease pathogenesis.

Fibromuscular dysplasia is an uncommon non-inflammatory arteriopathy. Hormonal factors are believed to play a role in disease pathogenesis given the overwhelming female predominance of this disease. We describe a case of a 56-year-old transgender man on prolonged testosterone therapy diagnosed with renal fibromuscular dysplasia after presenting with hypertensive urgency.

Quality of Diabetes Care in the USA.

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the current quality of diabetes care in the USA, discuss barriers to guideline-recommended treatment adherence, and outline strategies for the improvement in care. RECENT FINDINGS: Current treatment guidelines highlight the importance of glycemic control, use of novel medications with proven cardiovascular efficacy, and multifactorial cardiovascular risk factor intervention for the treatment of diabetes and associated complications. Albeit proven evidence for these guidelines, the vast majority of patients with diabetes remain insufficiently treated. Interventions to improve outcomes require focus on care delivery systems, physician behavior, and patient-centered approaches. De-fragmenting care systems to form collaborative, multi-specialty teams, use of standardized and comprehensive treatment algorithms, development of quality assessment tools, avoiding physician therapeutic inertia, and addressing patient barriers, including lack of perceived benefit, insufficient diabetes education and access to care, and medication costs, represent key objectives to improve diabetes care and outcomes. Clinical research in standardized trials has proven the feasibility to reduce morbidity and mortality associated with diabetes. Implementing models of care to disseminate these encouraging research findings to the wider population and to overcome barriers to achieving guideline-recommended treatment goals should be the objective to improve our current quality of diabetes care in the USA.

Treatment of Hypertension in Patients with Diabetes Mellitus: a Contemporary Approach.

PURPOSE OF REVIEW: Blood pressure guidelines worldwide have changed their recommended blood pressure goals multiple times over the past decade due to an evolving understanding of the treatment of hypertension in patients with diabetes mellitus. While it is evident through randomized trials that treatment of hypertension in diabetes mellitus prevents complications, the optimal blood pressure goal is not clear. RECENT FINDINGS: Post hoc analyses of the Action to Control Cardiovascular Risk in Diabetes-Blood Pressure (ACCORD-BP) trial, its long-term follow along study ACCORDION, and Systolic Blood Pressure Intervention Trial (SPRINT) suggest that patients with diabetes have a reduced risk of adverse cardiovascular events when aiming for more intensive blood pressure targets. High-quality data support guideline recommendations for more aggressive blood pressure targets in patients with diabetes mellitus. Reasoning for a return to more aggressive blood pressure goals in this at-risk population is discussed, and treatment strategies encompassing contemporary therapeutic options are recommended.

Improving the accuracy of heart failure diagnosis in low-resource settings through task sharing and decentralization.

Background: Task sharing of TTE may improve capacity for heart failure diagnosis and management in patients in remote, low-resource settings but the impact on diagnostic accuracy and patient outcomes has not been studied.Objectives: Determine feasibility and impact of non-expert training in transthoracic echocardiography (TTE) to improve the diagnosis and outcomes of patients with suspected heart failure in Uganda.Methods: This two-part study examined an innovative training program to develop TTE competency among non-experts and used a pre-post design to determine the impact of decentralized TTE. Four of 8 non-experts (50%) passed a three-part training course. The training comprised of distance learning through a web-based curriculum, a 2-day hands-on workshop with cardiologists, and independent practice with remote mentorship. Continuous measures were compared (pre- vs. post-TTE) using t-tests or Wilcoxon rank-sum tests as distributionally appropriate and categorical variables assessed through chi-square testing. Sensitivity and specificity were calculated according to standard methodology comparing diagnosis pre- and post-TTE during phase 2.Results: Performance in the post-training phase showed good agreement with expert categorization (κ = 0.80) with diagnostic concordance in 421 of 454 studies (92.7%). TTE changed the preliminary diagnosis in 81% of patients, showing low specificity of clinical decision-making alone (14.2%; 95% CI 10.1-19.2%). Dilated cardiomyopathy, hypertensive heart disease with preserved systolic function, and right heart failure were the most underdiagnosed conditions prior to TTE while hypertensive heart disease with decreased systolic function was the most over-diagnosed condition.Conclusions: In conclusion, non-expert providers can achieve a high level of proficiency for the categorization of heart failure using handheld TTE in low-resource settings and use of telemedicine and remote mentorship may improve performance and feasibility. The addition of TTE resulted in substantial improvement in etiological specificity. Further study is needed to understand implications of this strategy on healthcare utilization, long-term patient outcomes, and cost.

Evaluation and Treatment of Severe Rhabdomyolysis in a Patient with Legionnaires' Disease.

A 53-year-old man with alcoholism and a three-day history of diarrhea and abdominal pain was hospitalized with mild acute kidney injury (AKI) and rhabdomyolysis after a fall where he was down for a short duration. Subsequent testing revealed patchy right lower lobe infiltrates on chest X-ray and a positive urinary Legionella antigen test. Creatinine phosphokinase (CPK) peaked at 85,780 U/L (normal 0-250) on hospital day two and remained markedly elevated for five days despite aggressive intravenous (IV) hydration and appropriate antibiotic treatment. When the patient defervesced and showed clinical signs of resolution of pneumonia, the CPK level declined rapidly, and renal function returned to baseline. Rhabdomyolysis with AKI is a rare but serious complication of Legionella pneumonia, with most patients requiring dialysis. Our patient's complete recovery without renal replacement therapy can probably be attributed to his normal baseline renal function, timely diagnosis of his Legionella-associated rhabdomyolysis, and prompt treatment with aggressive IV hydration and appropriate antibiotics. Legionella infection should be considered in acutely ill patients with rhabdomyolysis of unclear etiology.

Coronary artery calcium, HIV and inflammation in Uganda compared with the USA.

Objectives: To compare the prevalence of detectable coronary artery calcium (CAC) among higher risk, older people living with HIV (PLWH) and uninfected persons in Uganda versus the USA, and second to explore associations of CAC with HIV-specific variables and biomarkers of inflammation. Methods: This cross-sectional study of 430 total subjects compared 100 PLWH on antiretroviral therapy and 100 age-matched and sex-matched HIV-uninfected controls in Uganda with 167 PLWH on antiretroviral therapy and 63 uninfected controls in the USA. Multivariable logistic regression was used to examine associations with detectable CAC (CAC >0). Results: Compared with US subjects, Ugandans were older (mean age 56 vs 52 years) and were more likely to have diabetes (36% vs 3%) and hypertension (85% vs 36%), but were less likely to be male (38% vs 74%) or smokers (4% vs 56%). After adjustment for HIV serostatus, age, sex and traditional risk factors, Ugandans had substantially lower odds of CAC >0 (adjusted OR 0.07 (95% CI 0.03 to 0.17), p<0.001). HIV was not associated with CAC >0 in either country (p>0.1). Among all PLWH, nadir CD4 count was associated with the presence of CAC, and among Ugandans soluble intercellular adhesion molecule (p=0.044), soluble CD163 (p=0.004) and oxidised low-density lipoprotein (p=0.043) were all associated with the presence of CAC. Conclusions: Ugandans had a dramatically lower prevalence of any coronary calcification compared with US subjects. The role of HIV infection and inflammation as risk factors for subclinical coronary disease in sub-Saharan Africa merits further investigation.

Renal infarction in vascular Ehlers-Danlos syndrome masquerading as pyelonephritis.

Symptoms associated with numerous diseases can be indistinguishable from those of the urinary system disorders because receptors of many visceral organs as well as the body wall transmit sensation through pain fibers shared with the kidneys. Disregarding important family background of genetic disorder can be detrimental for some patients.