What is the clinical evidence from this study?

Study design: Case Report. Population: Hypertrophic cardiomyopathy (n=1). Intervention: Dual-chamber ICD and antiarrhythmic therapy.

What question did this study set out to answer?

Describe a rare clinical manifestation of hypertrophic cardiomyopathy involving co-existing ventricular fibrillation and sinoatrial node arrest associated with an MYH7 gene mutation.

November 3, 2023Frontiers in Cardiovascular MedicineOpen Access

Case Report: Hypertrophic cardiomyopathy with recurrent episodes of ventricular fibrillation and concurrent sinus arrest

View Full Paper

Ask AI

Bookmark

Key result

MYH7-variant HCM presents with recurrent VF and concurrent SA node arrest requiring a dual-chamber ICD.

Why the study?

Sinoatrial node arrest is uncommon in hypertrophic cardiomyopathy and has not been previously described occurring during ventricular fibrillation.

Population

18-year-old woman with non-obstructive HCM and sudden cardiac arrest due to VF

Design

Case report

Authors

JHJassin HamidiUniversity Hospital Münster JWJoachim WinterDüsseldorf University Hospital RWRene WeberAugusta Krankenhaus

Discussion

Member takes

Implication

Supports considering dual-chamber ICDs in HCM with mixed ventricular and sinus arrhythmias; leaves open broader applicability.

Key Points

Describe a rare clinical manifestation of hypertrophic cardiomyopathy involving co-existing ventricular fibrillation and sinoatrial node arrest associated with an MYH7 gene mutation.
Clinical, electrophysiological, and genetic evaluation of an 18-year-old female presenting with sudden cardiac arrest from non-obstructive hypertrophic cardiomyopathy (N=1).
Analysis of cardiac rhythm recordings and device diagnostics across subcutaneous and dual-chamber implantable cardioverter-defibrillator systems.
Interrogation of the initial subcutaneous cardioverter-defibrillator confirmed recurrent ventricular fibrillation combined with severe bradycardia and sinoatrial node arrest.
Genetic analysis identified a heterozygous, pathogenic missense variant in the MYH7 gene (c.2155C>T; p.Arg719Trp), an alteration associated with early cardiac death.
Conversion from a subcutaneous defibrillator to a dual-chamber transvenous system resolved arrhythmic instability by delivering necessary antibradycardia pacing and defibrillation.

Study Design

Type

Case Report (n=1)

Structured PICO

Population

18-year-old woman with non-obstructive hypertrophic cardiomyopathy (HCM) presenting with sudden cardiac arrest due to ventricular fibrillation (VF) and concurrent sinoatrial node (SAN) arrest, carrying a heterozygous pathogenic variant of the MYH7 gene (c.2155C>T; p.Arg719Trp).

Intervention

Subcutaneous implantable cardioverter-defibrillator (S-ICD) followed by device exchange to a dual-chamber transvenous ICD, along with medical therapy (bisoprolol, ramipril, amiodarone, diltiazem).

Outcome

Recurrence of ventricular fibrillation and concurrent sinus arrest, and successful termination by ICD.

This case highlights the rare co-occurrence of ventricular fibrillation and sinus arrest in a patient with hypertrophic cardiomyopathy and a pathogenic MYH7 variant, emphasizing the potential need for a dual-chamber ICD.

Limitations

Sinoatrial node (SAN) functional parameters, in particular SNRT and SACT, HR parameters, and HRV were not available.
Evaluation of SAN function upon adenosine bolus and subsequent aminophylline or theophylline treatment could not be analyzed.
SAN arrest cannot be distinguished from SAN exit block and conduction impairments due to the lack of specific SAN function data.
SAN functional parameters (SNRT, SACT, HR parameters, HRV) were not available
Evaluation of SAN function upon adenosine bolus and subsequent aminophylline/theophylline or atropine treatment could not be analyzed
Cannot distinguish SAN arrest from SAN exit block and conduction impairments

Cite This Study

Hamidi et al. (2023) conducted a case report in Hypertrophic cardiomyopathy (n=1). Dual-chamber ICD and antiarrhythmic therapy was evaluated. An 18-year-old female with hypertrophic cardiomyopathy and a pathogenic MYH7 variant experienced recurrent ventricular fibrillation with concurrent sinoatrial node arrest, requiring a dual-chamber ICD.

synapsesocial.com/papers/6a160d45ec4058fde8c5bdc6 https://doi.org/10.3389/fcvm.2023.1240189

View Full Paper

Ask AI

Bookmark

Case Report: Hypertrophic cardiomyopathy with recurrent episodes of ventricular fibrillation and concurrent sinus arrest

Discussion

Member takes

Implication

Key Points

Study Design

Structured PICO

Limitations

Cite This Study

Case Report: Hypertrophic cardiomyopathy with recurrent episodes of ventricular fibrillation and concurrent sinus arrest

Discussion

Member takes

Implication

Key Points

Study Design

Structured PICO

Limitations

Cite This Study

Also Consider

Also Consider