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Patients with hypertrophic cardiomyopathy (HCM) are commonly affected by ventricular tachyarrhythmias such as ventricular tachycardia, leading to syncope and sudden cardiac death (SCD). Complete atrioventricular (AV) block in patients with HCM is very unusual but may also lead to syncope and SCD. We report a 52-year-old male who presented with recurrent episodes of pre-syncope and effort intolerance. A 12-lead ECG demonstrated deep T-wave inversion in the precordial leads with complete AV dissociation, and a two-dimensional echocardiogram revealed HCM without resting or provoked left ventricular outflow tract obstruction. The patient initially got a temporary transvenous pacemaker, followed by a dual-chamber rate-responsive pacemaker, which was subsequently upgraded to a dual-chamber implantable cardioverter-defibrillator after further risk stratification. Although rare, there have been a few reported cases of HCM complicated by atrioventricular block. This case should alert physicians to the possibility of atrioventricular block in patients with HCM, which could influence the management outcomes.
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http://dx.doi.org/10.7759/cureus.89041 | DOI Listing |
JACC Case Rep
September 2025
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Background: Cardiac sarcoidosis (CS) usually affects the left ventricle and presents with nonspecific features like conduction abnormalities and ventricular arrhythmias. However, right ventricle (RV)-dominant involvement has been increasingly reported, making diagnosis difficult.
Case Summary: A 55-year-old man presented with palpitations.
Mol Genet Genomic Med
September 2025
Department of Maternal-Fetal Medicine, Augusta University, Augusta, Georgia, USA.
Introduction: Spinal muscular atrophy (SMA), caused by pathogenic variants in the survival motor neuron (SMN) gene, is the most common genetic cause of mortality in children under the age of two. Prior reports of obstetric sonograms performed in pregnancies with severe forms of fetal SMA have discrepant findings that may stem from a failure to account for the SMN2 copy number.
Methods: We present a neonate diagnosed with SMA type 0 postnatally (0SMN1/1SMN2 genotype).
Open Access Rheumatol
August 2025
Department of Rheumatology and Immunology, the First Affiliated Hospital, Jinan University, Guangzhou, 510632, People's Republic of China.
Objective: To evaluate the efficacy of baricitinib in combination therapy for managing refractory, rapidly progressive systemic sclerosis (SSc) with severe cardiac conduction defects and interstitial lung disease (ILD).
Methods: A 48-year-old male patient with SSc complicated by significant cardiac enlargement, third-degree atrioventricular block, heart failure, progressive ILD, and partial intestinal obstruction was included in the study. Prior treatments with mycophenolate mofetil (MMF), tacrolimus, and cyclophosphamide (CTX) had shown limited efficacy.
Eur Heart J Case Rep
September 2025
Duke University Medical Center, Division of Cardiology, Box 3182, Durham, NC 27710, USA.
Background: Genetic aetiologies of early-onset arrhythmias and cardiomyopathy (CM) are common, but timely diagnosis requires a high index of suspicion.
Case Summary: An asymptomatic 47-year-old man presented to cardiology clinic for smartwatch low-rate alarms. His brother had exertional syncope and died in his 20s from heart failure.
Heart Rhythm O2
August 2025
Department of Cardiology, Rouen University Hospital, UNIROUEN, INSERM U1096, Rouen, France.
Background: A high burden of right ventricular pacing (RVP) increases the risk of hospitalization because of heart failure. Data on predictive factors for high burden of RVP in patients with permanent pacemaker implantation (PPI) after transcatheter aortic valve replacement (TAVR) are limited.
Objective: This study aimed to identify predictors of high RVP burden in patients with current indications for PPI after TAVR.