Fulminant Necrotizing Eosinophilic Myocarditis Successfully Treated With Veno-Arterial Extracorporeal Membrane Oxygenation and High-Dose Steroids

Abstract Fulminant necrotizing eosinophilic myocarditis (FNEM) is a rare form of EM characterized by biventricular heart failure with hemodynamic deterioration, often requiring inotropes or mechanical circulatory support. Here, we report a case of a 43-year-old healthy woman with FNEM who was admitted with acute heart failure that rapidly progressed to cardiogenic shock and electrical storm, culminating in cardiac arrest. Early diagnosis and prompt administration of corticosteroids in combination with veno-arterial extracorporeal membrane oxygenation allowed complete recovery of biventricular systolic function.

Mitral annular disjunction: Beyond mitral valve prolapse.

Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. The present article offers a review of the literature regarding the arrhythmic risk of MAD and mitral valve prolapse, as well as a review of risk stratification in these patients.

Diagnostic Challenges of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a disease characterized by clinical heterogeneity with unpredictable course. Several disease endotypes have been identified, including SLE with antiphospholipid syndrome (APS). We report a case of a pregnant woman with hypertension and proteinuria, diagnosed with APS, Libman-Sacks endocarditis that led to moderate to severe mitral valve insufficiency, and SLE. We describe the diagnostic steps, evolution, and complications. This case highlights the asynchrony behavior of SLE, emphasizing the importance of a multidisciplinary approach to an early diagnosis.

Left ventricular noncompaction associated with a pathogenic mutation in the MYH7 gene: Known mutation, different phenotype.

Left ventricular noncompaction (LVNC) is a genetically heterogeneous cardiomyopathy, with familial and sporadic forms, but genetic testing only identifies a pathogenic mutation in a minority of cases. The main complications are heart failure, embolism and dysrhythmias. Herein we report a familial case of LVNC associated with a mutation in the MYH7 gene and review the literature regarding controversies in LVNC. A 50-year-old woman was referred to the cardiology clinic for palpitations. She underwent echocardiography and cardiac magnetic resonance imaging that revealed mild left ventricular systolic dysfunction and LVNC criteria. She had several episodes of non-sustained ventricular tachycardia and received an implantable cardioverter-defibrillator (ICD). Genetic testing revealed the c.1003G>C (p.Ala335Pro) mutation in the MYH7 gene. Familial screening showed clear genotype-phenotype cosegregation, which provided strong evidence for the pathogenic role of this mutation. To the best of our knowledge, this is the first report of LVNC associated with the p.Ala335Pro mutation in the MYH7 gene. This mutation has been described in hypertrophic cardiomyopathy, suggesting that the same pathogenic sarcomere mutation may be associated with different cardiomyopathies. This case also highlights the current difficulties regarding decisions on ICD implantation for primary prevention of sudden cardiac death in LVNC.

Perimyocarditis Following COVID-19 Vaccination.

A 39-year-old male was admitted in the emergency room with chest pain. He had been given the second dose of Pfizer-BioNTech COVID-19 vaccine 3 days before. The patient denied taking any other medication beyond the usual. He didn't feel sick in the previous days/weeks. Laboratory studies revealed elevated serum levels of troponin and C-reactive protein. An autoantibody screen and a serologic panel to detect common viruses were negative. A cardiac MRI showed myocardial edema/inflammation and confirmed the diagnosis of perimyocarditis which was considered to be a consequence of COVID-19 vaccination. Physicians should be aware of the possibility of cardiovascular complications after COVID-19 vaccination.

Brugada syndrome: Eligibility for subcutaneous implantable cardioverter-defibrillator after exercise stress test.

INTRODUCTION: Brugada syndrome (BrS) is a channelopathy associated with ventricular arrhythmias and sudden cardiac death. In patients at high risk of sudden death, an implantable cardioverter-defibrillator is indicated. Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are an alternative to transvenous systems, with reduced risk of infection and complications associated with system extraction or explantation. OBJECTIVE: To test electrocardiographic eligibility for S-ICD placement after exercise stress testing (EST) in patients with BrS. METHODS: The sample included 35 consecutive patients with BrS. Electrocardiographic eligibility was assessed using the Boston Scientific model 2889 EMBLEM™ S-ICD automated screening tool, in four phases: decubitus and orthostatism, and before and after EST. Those who had at least one acceptable vector in the four measurements were considered eligible. RESULTS: In this study, 71.4% of patients were male and mean age was 53.86±12 years. In screening prior to EST, 14.3% of patients (n=5) were not eligible for an S-ICD. There was a statistically significant association between ineligibility and presence of complete right bundle branch block and history of syncope. After EST, 16.7% of initially eligible patients no longer had eligible vectors (n=5). CONCLUSION: In this study, 16.7% of patients previously eligible for an S-ICD were no longer eligible after EST. This result demonstrates the importance of screening after EST in all patients with BrS and with indication for an S-ICD, and may influence decisions concerning which ICD to implant or whether to institute pharmacological measures that avoid inappropriate therapies.

Inverted takotsubo syndrome complicated with cardiogenic shock requiring veno-arterial extracorporeal membrane oxygenation in a patient with bilateral pheochromocytoma: a case report.

BACKGROUND: Takotsubo syndrome (TS) is characterized by a transient left ventricular (LV) dysfunction and rarely presents with cardiogenic shock (CS). Inverted TS (ITS) is a rare entity associated with the presence of a pheochromocytoma. CASE SUMMARY: We present a case of a young woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breathlessness. An ITS secondary to a pheochromocytoma crisis presenting with CS was diagnosed. The patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of LV function. On the 35th day of hospitalization, open bilateral adrenalectomy was performed. DISCUSSION: Takotsubo syndrome patients presenting with CS are challenging and clinicians should be aware of underlying causes. Specific triggers such as pheochromocytoma should systematically be considered particularly if ITS was presented. Extracorporeal life support devices could provide temporary mechanical circulatory support in patients with TS on refractory CS and help to manage complex cases with TS due to pheochromocytoma.

Post mortem genetic test, the clinical diagnosis is not fade with the death of the patient. / Teste genético post mortem, o diagnóstico clínico não se esgota com a morte do doente.

In up to one-third of cases of sudden death, the medico-legal autopsy finding is inconclusive, and the option to perform a molecular autopsy is covered in international guidelines. The importance of postmortem genetic testing lies in its ability to identify hereditary diseases, often those with an autosomal dominant transmission pattern, and, through consultations and screening of relatives, to identify family members with a pathogenic mutation, who are often asymptomatic, providing an opportunity to change the course of their lives. The authors present three clinical cases that highlight the importance of postmortem genetic studies and family studies, as well as the integration of the data obtained in a cardiology consultation, which may be for arrhythmology, coronary disease or cardiomyopathy, depending on the specific condition. This could modify the course of the disease in many relatives.