Cardiovascular involvement in systemic rheumatic diseases: An integrated view for the treating physicians

Systemic autoimmune diseases can affect various kinds of organs including the kidney, the skin, soft tissue and the bone. Among others, cardiovascular involvement in rheumatic diseases has been shown to affect myocardium, pericardium, cardiac vessels, conduction system and valves, eventually leading to increased mortality. In general, underlying chronic inflammation leads to premature atherosclerosis, but also other manifestations such as arrhythmia and heart failure may have a ‘silent’ progress. Traditional cardiovascular risk factors play a secondary role, while disease-specific factors (i.e. disease duration, severity, antibody positivity, persistent disease activity) can directly influence the cardiovascular system. Therefore, early diagnosis is critical to optimize management and to control inflammatory activity and recent data suggest that risk factors (i.e. hypercholesterolemia and hypertension) need intensive treatment as well. With the advent of immunosuppressive agents, most rheumatic diseases are well controlled on treatment, but information related to their cardioprotective efficacy is not well-defined. In this review, we focus on cardiovascular involvement in rheumatic diseases and highlight current evidence which should be of help for the treating physicians. Moreover, cardiotoxicity of immunosuppressive drugs is a rare issue and such potential adverse events will be briefly discussed.     

Familial pseudo-Wolff-Parkinson-White syndrome

INTRODUCTION: PRKAG2 plays a role in regulating metabolic pathways, and mutations in this gene are associated with familial ventricular preexcitation, hypertrophic cardiomyopathy, and atrioventricular conduction disturbances. Clinico-pathologic and experimental data suggest the hypothesis of a glycogen storage disease. OBJECTIVE: To report a unique pattern of clinical features observed in individuals with a mutant PRKAG2 from two unrelated families. METHODS AND RESULTS: We studied two large families and found a total of 20 affected individuals showing a combination of sinus bradycardia, short PR interval, RBBB, intra and infrahisian conduction disturbances often requiring a pacemaker, and atrial tachyarrhythmias. Three individuals died suddenly at a young age. No patient had the Wolff-Parkinson-White (WPW) syndrome, and only two patients (10%) had myocardial hypertrophy. We performed screening of the exons and exon-intron boundaries of PRKAG2. Genetic analysis revealed a missense mutation (Arg302Gln) in the affected individuals from both families. This mutation had been described before and has been associated with the familial form of the WPW syndrome and with a high prevalence of left ventricular hypertrophy. CONCLUSION: PRKAG2 mutations are responsible for a diverse phenotype and not only the familial form of the WPW syndrome. Familial occurrence of right bundle branch block, sinus bradycardia, and short PR interval should raise suspicion of a mutant PRKAG2 gene.     

Genetics,Clinical Features,and Long-Term Outcome of Noncompaction Cardiomyopathy

Background

The clinical outcomes of noncompaction cardiomyopathy (NCCM) range from asymptomatic to heart failure, arrhythmias, and sudden cardiac death. Genetics play an important role in NCCM.

Novel criterion for the differential diagnosis of wide QRS complexes and wide complex tachycardia using the initial activation of QRS on leads V1 and V2: Differential diagnosis of wide QRS based on V1-V2

Background

Many diagnostic criteria for the differential diagnosis of wide complex tachycardia (WCT) are complex and not completely accurate. Incorrect diagnosis is also related to error in applying criteria.

Ambulatory Rhythm Monitoring to Detect Late High-Grade Atrioventricular Block Following Transcatheter Aortic Valve Replacement

BackgroundHigh-grade atrioventricular block (H-AVB) is a well-described in-hospital complication of transcatheter aortic valve replacement (TAVR). Delayed high-grade atrioventricular block (DH-AVB) has not been systematically studied among outpatients post-TAVR, using latest-generation TAVR technology and in the early post-TAVR discharge era.ObjectivesThe purpose of this study was to assess utility of ambulatory event monitoring (AEM) in identifying post-TAVR DH-AVB and associated risk factors.MethodsPatients without pre-existing pacing device undergoing TAVR at the University of Colorado Hospital from October 2016 to March 2018, and who did not require permanent pacemaker implantation pre-discharge, were discharged with 30-day AEM to assess for DH-AVB (≥2 days post-TAVR). Clinical and follow-up data were collected and compared among those without incident H-AVB.ResultsAmong 150 consecutive TAVR patients without a prior pacing device, 18 (12%) developed H-AVB necessitating permanent pacemaker <2 days post-TAVR, 1 died pre-discharge, and 13 declined AEM; 118 had 30-day AEM data. DH-AVB occurred in 12 (10% of AEM patients, 8% of total cohort) a median of 6 days (range 3 to 24 days) post-TAVR. DH-AVB versus non-AVB patients were more likely to have hypertension and right bundle branch block (RBBB). Sensitivity and specificity of RBBB in predicting DH-AVB was 27% and 94%, respectively.ConclusionsDH-AVB is an underappreciated complication of TAVR among patients without pre-procedure pacing devices, occurring at rates similar to in-hospital, acute post-TAVR H-AVB. RBBB is a risk factor for DH-AVB but has poor sensitivity, and other predictors remain unclear. In this single-center analysis, AEM was helpful in expeditious identification and treatment of 10% of post-TAVR outpatients. Prospective study is needed to clarify incidence, risk factors, and patient selection for outpatient monitoring.     

Cardiac Resynchronization Defibrillator Therapy for Nonspecific Intraventricular Conduction Delay Versus Right Bundle Branch Block

BackgroundThe benefits of cardiac resynchronization therapy (CRT) in patients with non-left bundle branch block (LBBB) conduction abnormality have not been fully explored.ObjectivesThis study sought to evaluate clinical outcomes among Medicare-aged patients with nonspecific intraventricular conduction delay (NICD) versus right bundle branch block (RBBB) in patients eligible for implantation with a CRT with defibrillator (CRT-D).MethodsUsing the National Cardiovascular Data Registry implantable cardioverter-defibrillator (ICD) registry data between 2010 and 2013, the authors compared outcomes in CRT-eligible patients implanted with CRT-D versus ICD-only therapy among patients with NICD and RBBB. Also, among all CRT-D–implanted patients, the authors compared outcomes in those with NICD versus RBBB. Survival curves and multivariable adjusted hazard ratios (HRs) were used to assess outcomes including hospitalization and death.ResultsIn 11,505 non-LBBB CRT-eligible patients, after multivariable adjustment, among patients with RBBB, CRT-D was not associated with better outcomes, compared with ICD alone, regardless of QRS duration. Among patients with NICD and a QRS ≥150 ms, CRT-D was associated with decreased mortality at 3 years compared with ICD alone (HR: 0.602; 95% confidence interval [CI]: 0.416 to 0.871; p = 0.0071). Among 5,954 CRT-D–implanted patients, after multivariable adjustment NICD compared with RBBB was associated with lower mortality at 3 years in those with a QRS duration of ≥150 ms (HR: 0.757; 95% CI: 0.625 to 0.917; p = 0.0044).ConclusionsAmong non-LBBB CRT-D–eligible patients, CRT-D implantation was associated with better outcomes compared with ICD alone specifically in NICD patients with a QRS duration of ≥150 ms. Careful patient selection should be considered for CRT-D implantation in patients with non-LBBB conduction.