The harm of delayed diagnosis of arrhythmogenic cardiac sarcoidosis: a case series.

AIMS: Cardiac sarcoidosis (CS) is a known cause of ventricular tachycardia (VT). However, an arrhythmogenic presentation may not prompt immediate comprehensive evaluation. We aimed to assess the diagnostic and disease course of patients with arrhythmogenic cardiac sarcoidosis (ACS). METHODS AND RESULTS: From the Leiden VT-ablation-registry, consecutive patients with CS as underlying aetiology were retrospectively included. Data on clinical presentation, time-to-diagnosis, cardiac function, and clinical outcomes were collected. Patients were divided in early (<6 months from first cardiac presentation) and late diagnosis. After exclusion of patients with known causes of non-ischaemic cardiomyopathy (NICM), 15 (12%) out of 129 patients with idiopathic NICM were ultimately diagnosed with CS and included. Five patients were diagnosed early; all had early presentation with VTs. Ten patients had a late diagnosis with a median delay of 24 (IQR 15-44) months, despite presentation with VT (n = 5) and atrioventricular block (n = 4). In 6 of 10 patients, reason for suspicion of ACS was the electroanatomical scar pattern. In patients with early diagnosis, immunosuppressive therapy was immediately initiated with stable cardiac function during follow-up. Adversely, in 7 of 10 patients with late diagnosis, cardiac function deteriorated before diagnosis, and in only one cardiac function recovered with immunosuppressive therapy. Six (40%) patients died (five of six with late diagnosis). CONCLUSION: Arrhythmogenic cardiac sarcoidosis is an important differential diagnosis in NICM patients referred for VT ablation. Importantly, the diagnosis is frequently delayed, which leads to a severe disease course, including irreversible cardiac dysfunction and death. Early recognition, which can be facilitated by electroanatomical mapping, is crucial.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

Isolated potentials and pace-mapping as guides for ablation of ventricular tachycardia in various types of nonischemic cardiomyopathy.

BACKGROUND: In patients with prior infarction, isolated potentials (IPs) during sinus rhythm reflect fixed scar and often indicate sites critical for ventricular tachycardia (VT). The purpose of this study was to determine the value of IPs in conjunction with pace-mapping to guide VT ablation in patients with various types of nonischemic cardiomyopathy. METHODS: Mapping and ablation of VT were performed in 35 consecutive patients (26 men, age 55 ± 13 years, ejection fraction 0.31 ± 0.14) with VT and various etiologies of nonischemic cardiomyopathy. Pace-mapping was performed at sites with low voltage. Radiofrequency energy was delivered at sites with concealed entrainment or matching pace-maps. RESULTS: One hundred ninety-five VTs (mean cycle length 363 ± 88 ms) were induced. Sites with prespecified ablation criteria displaying IPs during sinus rhythm were recorded in 21 of 35 patients (60%, IP-positive). In these patients, a total of 216 sites meeting prespecified ablation criteria were identified and 146 of 216 sites (68%) displayed IPs. Fifteen of 21 IP-positive patients (71%) no longer had inducible VT after ablation. In 14 of 35 patients, no sites with IPs where prespecified ablation criteria were met were identified (IP-negative) despite combined endocardial and epicardial mapping in 7 of 14 patients. Only 1 of 14 IP-negative patients (7%) no longer had inducible VT at the end of the ablation procedure. During a mean follow-up of 18 ± 13 months, 14 of 21 IP-positive patients (67%) remained arrhythmia-free, compared to 1 of 14 IP-negative patients (7%; P < 0.01). Half of the IP-negative patients had major adverse events due to recurrent arrhythmias, compared to none in IP-positive patients. CONCLUSION: IPs in conjunction with pace-mapping are helpful for identifying critical isthmus areas for ablation of VT in patients with various types of nonischemic cardiomyopathy. Patients with nonischemic cardiomyopathy in whom the arrhythmogenic substrate is characterized by IPs have a more favorable outcome than patients in whom IPs are absent.