RESUMEN
Atrial fibrillation (AF) often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature. Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can contribute to the development and maintenance of AF. Atrial fibrillation may occur as a consequence of disease-specific defects, non-specific cardiac chamber changes secondary to the primary illness, or a combination thereof. The presence of AF can denote a turning point in the progression of the disease, promoting clinical deterioration and increasing morbidity and mortality. Furthermore, the management of AF can be particularly challenging in patients with inherited cardiomyopathies. In this article, we review the current information on the prevalence, pathophysiology, risk factors, and treatment of AF in three different inherited cardiomyopathies: hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia/cardiomyopathy, familial dilated cardiomyopathy, and left ventricular non-compaction cardiomyopathy.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica/genética , Fibrilación Atrial/genética , Cardiomiopatía Dilatada/genética , Cardiomiopatía Hipertrófica/genética , Frecuencia Cardíaca/genética , No Compactación Aislada del Miocardio Ventricular/genética , Displasia Ventricular Derecha Arritmogénica/epidemiología , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/terapia , Fibrilación Atrial/epidemiología , Fibrilación Atrial/fisiopatología , Fibrilación Atrial/terapia , Cardiomiopatía Dilatada/epidemiología , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Hipertrófica/epidemiología , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/terapia , Predisposición Genética a la Enfermedad , Humanos , No Compactación Aislada del Miocardio Ventricular/epidemiología , No Compactación Aislada del Miocardio Ventricular/fisiopatología , No Compactación Aislada del Miocardio Ventricular/terapia , Fenotipo , Prevalencia , Pronóstico , Medición de Riesgo , Factores de RiesgoRESUMEN
Canada has seen a sixfold increase in Lyme disease since being nationally notifiable in 2009. This is the first Canadian series on Lyme carditis manifested as high-degree atrioventricular block. We report 5 recent cases presented over a 2-year period. The variation of nonspecific presentations requires a high index of suspicion for prompt diagnosis and correct management. Recognizing this early would curtail the progression of conduction disorders and potentially avoid permanent pacemaker implantation.
Asunto(s)
Bloqueo Atrioventricular/fisiopatología , Enfermedad de Lyme/fisiopatología , Miocarditis/fisiopatología , Adolescente , Adulto , Antibacterianos/uso terapéutico , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/tratamiento farmacológico , Canadá , Ceftriaxona/uso terapéutico , Doxiciclina/uso terapéutico , Ecocardiografía , Electrocardiografía , Eritema Crónico Migrans , Prueba de Esfuerzo , Humanos , Enfermedad de Lyme/diagnóstico , Enfermedad de Lyme/tratamiento farmacológico , Masculino , Miocarditis/diagnóstico , Miocarditis/tratamiento farmacológico , Índice de Severidad de la Enfermedad , Mordeduras de Garrapatas , Adulto JovenRESUMEN
BACKGROUND: Lyme carditis (LC), an early manifestation of Lyme disease that most commonly presents as high-degree atrioventricular block (AVB), usually resolves with antibiotic treatment. When LC is not identified as the cause of AVB, a permanent pacemaker may be inappropriately implanted in a reversible cardiac conduction disorder. HYPOTHESIS: The likelihood that a patient's high-degree AVB is caused by LC can be evaluated by clinical characteristics incorporated into a risk stratification tool. METHODS: A systematic review of all published cases of LC with high-degree AVB, and five cases from the authors' experience, was conducted. The results informed the development of a new risk stratification tool, the Suspicious Index in LC (SILC) score. The SILC score was then applied to each case included in the review. RESULTS: Of the 88 cases included, 51 (58%) were high-risk, 31 (35.2%) intermediate-risk, and 6 (6.8%) low-risk for LC according to the SILC score (sensitivity 93.2%). For the subset of 32 cases that reported on all SILC variables, 24 (75%) cases were classified as high-risk, 8 (25%) intermediate-risk, and 0 low-risk (sensitivity 100%). Specificity could not be assessed (no control group). Notably, 6 of the 11 patients who received permanent pacemakers had reversal of AVB with antibiotic treatment. CONCLUSION: The SILC risk score and COSTAR mnemonic (constitutional symptoms; outdoor activity; sex = male; tick bite; age < 50; rash = erythema migrans) may help to identify LC in patients presenting with high-degree AVB, and ultimately, minimize the implantation of unnecessary permanent pacemakers.