Ventricular Tachycardia Crisis: Assessing Norepinephrine vs. Stress Steroids in the Battle Against Waterhouse-Friderichsen Syndrome With Distributive Shock.

Waterhouse-Friderichsen syndrome (WFS) is a rare but life-threatening complication associated with acute hemorrhagic necrosis of the adrenal glands, primarily linked to meningococcal infection. This report details the case of a 62-year-old female with HIV/AIDS and substance misuse who presented with ventricular tachycardia and hemodynamic instability. Subsequent evaluation revealed WFS in the context of disseminated meningococcal infection. The case highlights the diversity of WFS manifestations and the diagnostic challenges, particularly in patients with comorbidities. Managing WFS involves a delicate balance of steroids and vasopressors, necessitating a multidisciplinary approach. Timely diagnosis and intervention are critical in mitigating the high mortality associated with this syndrome.

Heart's Dangerous Symphony: Torsade De Pointes Unleashed by Gitelman Syndrome-Induced Hypomagnesemia.

Gitelman syndrome (GS) is a rare autosomal recessive salt-losing renal tubular disorder associated with a mutation of SLC12A3 or CLCNKB genes which encodes the thiazide-sensitive sodium-chloride co-transporter (NCCT) in the distal renal tubule. It is inherited as an autosomal recessive disorder. Hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria, and renin-angiotensin-aldosterone system (RAAS) activation are characteristics of GS. GS is often misdiagnosed or underdiagnosed owing to its low incidence and lack of awareness. Its prevalence is estimated to be around 1-10 per 40,000 people. We report a case of cardiac arrest secondary to torsade de pointes (TdP) because of GS-induced hypomagnesemia. Our case highlights the importance of clinicians being aware of the potential electrolyte abnormalities and complications associated with GS, as it can lead to catastrophic consequences if not identified and corrected earlier.

Autoantibodies for Cardiac Channels and Sudden Cardiac Death and its Relationship to Autoimmune Disorders.

BACKGROUND: Sudden Cardiac Death (SCD) is an unexpected death caused by heart dysfunction. Autoantibodies against cardiac proteins may be potentially involved in the occurrence and progression of cardiac disease and SCD. The first report on the role of autoantibodies in idiopathic dilated cardiomyopathy appeared in the 1980s. In recent years new studies on the effects of the presence of specific autoantibodies and their relationship to ventricular arrhythmias and SCD were published. The purpose of the current mini-review is to analyze the results of the research studies focused on the relationship between anti-cardiomyocyte autoantibodies and SCD with respect to autoimmune disorders. CONCLUSION: According to our analysis, more research is needed to understand the role of these autoantibodies against cardiac proteins in the SCD pathogenesis, and potentially employ this knowledge for improving prognosis of SCD.

A common single nucleotide polymorphism can exacerbate long-QT type 2 syndrome leading to sudden infant death.

BACKGROUND: Identification of infants at risk for sudden arrhythmic death remains one of the leading challenges of modern medicine. We present a family in which a common polymorphism (single nucleotide polymorphism) inherited from the father, combined with a stop codon mutation inherited from the mother (both asymptomatic), led to 2 cases of sudden infant death. METHODS AND RESULTS: KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, CACNA1c, CACNB2b, and KCNJ2 genes were amplified and analyzed by direct sequencing. Functional electrophysiological studies were performed with the single nucleotide polymorphism and mutation expressed singly and in combination in Chinese ovary (CHO-K1) and COS-1 cells. An asymptomatic woman presenting after the death of her 2-day-old infant and spontaneous abortion of a second baby in the first trimester was referred for genetic analysis. The newborn infant had nearly incessant ventricular tachycardia while in utero and a prolonged QTc (560 ms). The mother was asymptomatic but displayed a prolonged QTc. Genetic screening of the mother revealed a heterozygous nonsense mutation (P926AfsX14) in KCNH2, predicting a stop codon. The father was asymptomatic with a normal QTc but had a heterozygous polymorphism (K897T) in KCNH2. The baby who died at 2 days of age and the aborted fetus inherited both K897T and P926AfsX14. Heterologous coexpression of K897T and P926AfsX14 led to loss of function of HERG current much greater than expression of K897T or P926AfsX14 alone. CONCLUSIONS: Our data suggest that a common polymorphism (K897T) can markedly accentuate the loss of function of mildly defective HERG channels, leading to long-QT syndrome-mediated arrhythmias and sudden infant death.

Twisting until it breaks: a rare cause of ICD lead failure.

We describe a rare case of Twiddler's syndrome that resulted in a complex ICD lead fracture involving both the insulation and the conductor. The conductor fracture resulted in noise artefact that was interpreted by the device as ventricular fibrillation, but the patient had not received any shocks because the "episodes" were non-sustained. The patient did not have traditional risk factors for Twiddler's syndrome.

Management of high defibrillation threshold.

The implantable cardioverter defibrillator (ICD) has become the primary therapy for the treatment of potentially lethal ventricular arrhythmias. Ventricular arrhythmias encompass a spectrum of rhythm disturbances ranging from the occasional monomorphic ventricular premature complex to the almost universally fatal ventricular fibrillation. Our understanding of the mechanisms of ventricular fibrillation and defibrillation is still in evolution. At present, the most common ICD configuration consists of a pectoral pulse generator (active-can) with a bipolar transvenous dual coil lead. A transvenous system with an active-can has improved defibrillation thresholds and the ease of implantation. However, there are various clinical scenarios in which patients with high defibrillation threshold (DFT) are encountered. Although the incidence of high DFT patients is low, it is of significant concern since it may account for sudden cardiac death in patients with ICDs. At present, there are few clinical trials that are rigorous and well designed, and which can define a perfect methodology for the treatment of high DFT patients. In this review, in the context of commonly encountered clinical scenarios, we discuss therapeutic strategies to help manage patients with high DFT.