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A 40-year-old man, newly diagnosed with cardiac sarcoidosis (CS) presented with symptomatic ventricular tachycardia three days after starting steroid-based immunosuppressive therapy (IT). There was no clear guideline indication for implantable cardioverter-defibrillator (ICD) before the initiation of IT. Shortly after ICD implantation and the initiation of anti-arrhythmic drugs, recurring ventricular arrhythmias required titration of the anti-arrhythmic drug therapy. One-year follow-up assessment showed no significant arrhythmias and complete PET scan FDG uptake suppression. This case, along with recent publications, suggests transient pro-arrhythmic effects of steroids in patients with CS, which are not appropriately addressed in the current guidelines. We believe ICD implantation should be considered in clinically manifest CS before initiating IT, particularly in cases with heterogeneous and/or extensive FDG uptake on PET scans., (© 2024 The Authors. Pacing and Clinical Electrophysiology published by Wiley Periodicals LLC.)

Background: Although positron emission tomography (PET) imaging is well established for its diagnostic role in cardiac sarcoidosis, less is known about the prognostic value of PET and its use in risk stratification for major adverse cardiac events (MACE)., Objectives: The goal of this study was to perform a systematic review and meta-analysis looking at the prognostic value of PET imaging in patients with cardiac sarcoidosis., Methods: Study investigators systematically searched EMBASE (Excerpta Medica dataBASE), MEDLINE, PubMed, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, CINAHL (Cumulative Index to Nursing and Allied Health Literature), ClinicalTrials.gov, and the European Union Clinical Trial Registry for cardiac sarcoidosis and PET imaging. The primary outcome of interest was MACE., Results: The search revealed 3,010 records, of which 55 studies were included. This represented 5,250 patients. Factors associated with MACE included the following: the combination of abnormal fluorodeoxyglucose (FDG) uptake and perfusion defect, which had an OR of 2.86 (95% CI: 1.74-4.71; P < 0.0001); abnormal perfusion or FDG uptake, which had an OR of 2.69 (95% CI: 1.67-4.33); abnormal FDG uptake, which had an OR of 2.61 (95% CI: 1.51-4.50); focal abnormal right ventricular uptake, which had an OR of 6.27 (95% CI: 3.19-12.32; P < 0.00001); and a lack of response to immunosuppression on serial PET, which had an OR of 8.43 (95% CI: 3.25-21.85; P < 0.0001). A QUIPS (Quality in Prognostic Studies) tool analysis found a low to moderate risk of bias, particularly given the small sample sizes in the individual studies., Conclusions: Multiple cardiac PET parameters provide risk stratification value in cardiac sarcoidosis. Focal right ventricular uptake and a lack of response to immunosuppressive therapy on serial PET imaging were particularly predictive of MACE., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Sarcoidosis, a granulomatous disorder of unknown etiology affecting multiple organs. It is often a benign disease but can have significant morbidity and mortality when the heart is involved (often presenting with clinical manifestations such as conduction irregularities and heart failure). This study addresses a critical gap in cardiac sarcoidosis (CS) research by developing a robust animal model. The absence of a reliable animal model for cardiac sarcoidosis is a significant obstacle in advancing understanding and treatment of this condition. The proposed model utilizes carbon nanotube injection and transverse aortic constriction as stressors. Intramyocardial injection of carbon nanotubes induces histiocytes typical of sarcoid granulomas in the heart but shows limited effects on fibrosis or cardiac function. Priming the immune system with transverse aortic constriction prior to intramyocardial injection of carbon nanotubes enhances cardiac fibrosis, diminishes cardiac function, and impairs cardiac conduction. This novel, easily executable model may serve as a valuable tool for disease profiling, biomarker identification, and therapeutic exploration., (© 2024 The Author(s). Advanced Biology published by Wiley‐VCH GmbH.)

Cardiac sarcoidosis is an infiltrative cardiomyopathy that results from granulomatous inflammation of the myocardium and may present with high-grade conduction disease, ventricular arrhythmias, and right or left ventricular dysfunction. Over the past several decades, the prevalence of cardiac sarcoidosis has increased. Definitive histological confirmation is often not possible, so clinicians frequently face uncertainty about the accuracy of diagnosis. Hence, the likelihood of cardiac sarcoidosis should be thought of as a continuum (definite, highly probable, probable, possible, low probability, unlikely) rather than in a binary fashion. Treatment should be initiated in individuals with clinical manifestations and active inflammation in a tiered approach, with corticosteroids as first-line treatment. The lack of randomized clinical trials in cardiac sarcoidosis has led to treatment decisions based on cohort studies and consensus opinions, with substantial variation observed across centers. This scientific statement is intended to guide clinical practice and to facilitate management conformity by providing a framework for the diagnosis and management of cardiac sarcoidosis.

Background: clinically silent cardiac sarcoidosis (CS) may be associated with adverse outcomes, hence the rationale for screening patients with extracardiac sarcoidosis. The optimal screening strategy has not been clearly defined., Methods: patients with extra-cardiac sarcoidosis were prospectively included and underwent screening consisting of symptom history, electrocardiography (ECG), transthoracic echocardiogram, Holter, and signal-averaged ECG (SAECG). Cardiac magnetic resonance (CMR) was performed in all patients. Clinically silent CS was defined as CMR demonstrating late gadolinium enhancement (LGE) in a pattern compatible with CS according to a majority of independent and blinded CMR experts. Significant cardiac involvement was defined as the presence of LGE ≥6% and/or a positive fluorodeoxyglucose-positron emission tomography., Results: among the 129 patients included, clinically silent CS was diagnosed in 29/129 (22.5%), and 19/129 patients (14.7%) were classified as CS with significant cardiac involvement. There was a strong association between hypertension and CS (p < 0.05). Individual screening tools provided low diagnostic yield; however, combination of tests performed better, for example, a normal Holter and a normal SAECG had negative predictive values of 91.7%. We found consistently better diagnostic accuracy for the detection of CS with significant cardiac involvement., Conclusion: clinically silent CS and CS with significant cardiac involvement were found in 22.5% and 14.7% of patients with extra-cardiac sarcoidosis. The association with hypertension raises the possibility that some cases of hypertensive cardiomyopathy may be mistaken for CS. Screening with readily available tools, for example Holter and SAECG, may help identifying patients without CS where additional CMR is not needed., Competing Interests: Declaration of competing interest Dr. David H Birnie discloses being on the advisory board for Star Therapeutics and Kinevant/Roivant Sciences. Dr. Daniel Juneau discloses being a consultant for Novartis. No conflict of interest is pertinent to this work., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Cardiac sarcoidosis (CS) is a potentially serious form of infiltrative cardiomyopathy. Despite scarce evidence, immunosuppressive treatment is generally recommended, but local routines may vary significantly. We sought to survey the clinical practices in the treatment of CS, with the aim that the results may suggest future research priorities. We conducted a web-based survey focused on treatment-naive patients with CS. We subclassified CS according to the presence/absence of overt cardiac presentation (clinically manifest/silent) and to the presence/absence of active inflammation (metabolically active/inactive by fluorodeoxyglucose positron emission tomography). The survey was developed jointly by the authors and administered to expert clinicians (n = 79) involved in CS treatment. An agreement threshold was set at 70%. A total of 62 of 79 respondents (78.5%) from 12 countries completed the survey. The agreement threshold was reached for: (1) always treating clinically manifest, metabolically active CS, 57 of 62 (91.9%), (2) never treating clinically silent, metabolically inactive CS, 44 of 62 (71.0%), (3) not requiring histopathologic confirmation of sarcoidosis before treatment initiation, (49 of 62, 79.0%), (4) using fluorodeoxyglucose positron emission tomography for assessing treatment indication (44 of 62, 71.0%) and treatment response (44 of 62, 71.0%), and (5) using prednisone as a first-line agent (100%), although respondents were divided on monotherapy (69.4%) or combination with methotrexate 25.8%. The approach to particular scenarios, tapering, and duration of treatment showed the greatest variation in response. In conclusion, in this survey of clinical practice, important aspects of CS treatment reached the agreement threshold, whereas others showed a great degree of clinical equipoise., Competing Interests: Declaration of Competing Interest Dr. Birnie discloses being on the advisory board for Star Therapeutics and Kinevant/Roivant Sciences. The remaining authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

About 5% of sarcoidosis patients develop clinically manifest cardiac features. Cardiac sarcoidosis (CS) typically presents with conduction abnormalities, ventricular arrhythmias and heart failure. Its diagnosis is challenging and requires a substantial degree of clinical suspicion as well as expertise in advanced cardiac imaging. Adverse events, particularly malignant arrhythmias and development of heart failure, are common among CS patients. A timely diagnosis is paramount to ameliorating outcomes for these patients. Despite weak evidence, immunosuppression (primarily with corticosteroids) is generally recommended in the presence of active inflammation in the myocardium. The burden of malignant arrhythmias remains important regardless of treatment, thus leading to the recommended use of an implantable cardioverter defibrillator in most patients with clinically manifest CS.

Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. It is estimated that another 20 to 25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement ( clinically silent disease). Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest cardiac sarcoidosis (CS) despite minimal data supporting it. Positron emission tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators (ICDs). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In the current era of earlier diagnosis, modern heart failure treatment, and use of ICD therapy, the prognosis from CS is much improved. In a recent Finnish nationwide study, 10-year cardiac survival was 92.5% in 102 patients., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Patients with sarcoidosis can present with cardiac symptoms as the first manifestation of disease in any organ. In these patients, the use of chest imaging modalities may serve as an initial screening tool towards the diagnosis of sarcoidosis through identification of pulmonary/mediastinal involvement; however, the use of chest imaging for this purpose has not been well studied. We assessed the utility of different chest imaging modalities for initial screening for cardiac sarcoidosis (CS)., Methods and Results: All patients were investigated with chest x-ray, chest computed tomography (CT) and/or cardiac/thorax magnetic resonance imaging (MRI). We then used the final diagnosis (CS versus no CS) and adjudicated imaging reports (normal versus abnormal) to calculate the sensitivity and specificity of individual and combinations of chest imaging modalities. We identified 44 patients (mean age 54 (±8) years, 35.4% female) and a diagnosis of CS was made in 18/44 patients (41%). The sensitivity and specificity for screening for sarcoidosis were 35% and 85% for chest x-ray, respectively (AUC 0.60; 95%CI 0.42-0.78; p value=0.27); 94% and 86% for chest CT (AUC 0.90; 95%CI 0.80-1.00; p value <0.001); 100% and 50% for cardiac/thorax MRI (AUC 0.75; 95%CI 0.56-0.94; p value=0.04)., Conclusions: During the initial diagnostic workup of patients with suspected CS, chest x-ray was suboptimal as a screening test. In contrast CT chest and cardiac/thorax MRI had excellent sensitivity. Chest CT has the highest specificity among imaging modalities. Cardiac/thorax MRI or chest CT could be used as an initial screening test, depending on local availability., (Copyright: © 2019.)

Background: Sarcoidosis is a systemic inflammatory disease which can involve nearly any organ. Clinically manifest cardiac involvement occurs in perhaps 5% of patients with sarcoidosis. The reported prevalence of isolated cardiac sarcoidosis (CS) varies widely with reported rates of 27-54%. The explanation for this variability is likely multi-factorial but perhaps mostly related to the diagnostic method(s) for assessing extra-cardiac involvement. The primary aim of this study was to assess the rate of isolated CS in a homogeneous, prospectively recruited cohort of patients with clinically manifest CS, using whole body FDG PET-CT imaging as a gold standard. A secondary aim was to describe the extent and distribution of extra-cardiac sarcoidosis at the time of first presentation of clinically manifest CS., Methods: Patients were prospectively recruited at the time of first presentation with cardiac symptoms. All patients underwent whole-body and cardiac 18 F-FDG PET-CT. All patients were examined for presence of skin sarcoidosis and were assessed by an ophthalmologist., Results: 31 patients were included (mean age 56±8years, 17 female, 100% Caucasian). Patients had limited extra-cardiac involvement (mean of 2.2 organs) however using the most precise definition, only 1/31 (3.2%) patients had isolated CS. There were marked differences in right ventricular (RV) and atrial involvement between patients presenting with CS as first presentation compared to patients presenting initially with extra-cardiac disease., Conclusions: Most patients had limited extra-cardiac involvement at the time of presentation of manifest CS however, isolated CS, using the proposed gold standard, was only observed in one patient., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Cardiac symptoms are usually dominant over extra-cardiac as most patients with clinically manifest disease have minimal extra-cardiac disease and up to two-thirds have isolated cardiac sarcoidosis (CS). It is estimated that another 20-25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In addition, the extent of myocardial late gadolinium enhancement is emerging as an important prognostic factor. The literature shows some controversy regarding outcomes for patients with clinically silent CS and larger studies are needed. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest CS despite minimal data supporting it. Fluorodeoxyglucose Positron Emission Tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

Premature ventricular contraction (PVC)-induced cardiomyopathy is increasingly being recognized as a reversible cause of left ventricular (LV) systolic dysfunction (LVSD). The diagnosis of PVC-induced cardiomyopathy is considered in subjects with high PVC burdens (> 10,000 per 24 hours) after excluding other known causes of LVSD. PVC suppression is the mainstay of the management of PVC-induced cardiomyopathy, in addition to proven evidence-based medical therapy recommended for subjects with LVSD. Management strategies for PVC-induced cardiomyopathy include medical therapy and/or catheter ablation, with an increasing role for catheter ablation as a first-line therapy in view of the potential for permanent suppression of PVCs. Recovery of LVSD is typically a gradual process over months after effective suppression of PVCs. Last, asymptomatic patients with high PVC burdens and preserved LV systolic function appear to be at low risk over the intermediate term for developing LVSD. However, it is prudent to monitor LV function periodically because of the potential for deterioration of LV function observed during long-term follow-up in some subjects., (Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Clinically manifest cardiac involvement occurs in perhaps 5% of patients with sarcoidosis. The 3 principal manifestations of cardiac sarcoidosis (CS) are conduction abnormalities, ventricular arrhythmias, and heart failure. An estimated 20% to 25% of patients with pulmonary/systemic sarcoidosis have asymptomatic cardiac involvement (clinically silent disease). In 2014, the first international guideline for the diagnosis and management of CS was published. In patients with clinically manifest CS, the extent of left ventricular dysfunction seems to be the most important predictor of prognosis. There is controversy in published reports as to the outcome of patients with clinically silent CS. Despite a paucity of data, immunosuppression therapy (primarily with corticosteroids) has been advocated for the treatment of clinically manifest CS. Device therapy, primarily with implantable cardioverter-defibrillators, is often recommended for patients with clinically manifest disease., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Purpose: Cardiac sarcoidosis (CS) is a cause of conduction system disease (CSD). (18)F-Fluorodeoxyglucose-positron emission tomography (FDG PET) and cardiac magnetic resonance (CMR) are used for detection of CS. The relative diagnostic value of these has not been well studied. The aim was to compare these imaging modalities in this population., Methods: We recruited steroid-naive patients with newly diagnosed CSD due to CS. All CS patients underwent both imaging studies within 12 weeks of each other. Patients were classified into two groups: group A with chronic mild CSD (right bundle branch block and/or axis deviation), and group B with new-onset atrioventricular block (AVB, Mobitz type II or third-degree AVB)., Results: Thirty patients were included. Positive findings on both imaging studies were seen in 72 % of patients (13/18) in group A and in 58 % of patients (7/12) in group B. The remainder (28 %) of the patients in group A were positive only on CMR. Of the patients in group B, 8 % were positive only on CMR and 33 % were positive only on FDG PET. Patients in group A were more likely to be positive only on CMR, and patients in group B were more likely to be positive only on FDG PET (p = 0.02). Patients in group B positive only on FDG PET underwent CMR earlier relative to their symptomatology than patients positive only on CMR (median 7.0, IQR 1.5 - 34.3, vs. 72.0, IQR 25.0 - 79.5 days; p = 0.03)., Conclusion: The number of positive FDG PET and CMR studies was different in patients with CSD depending on their clinical presentation. This study demonstrated that CMR can adequately detect cardiac involvement associated with chronic mild CSD. In patients presenting with new-onset AVB and a negative CMR study, FDG PET may be useful for detecting cardiac involvement due to CS.

Introduction: Atrioventricular block (AVB) can be caused by several conditions, including cardiac sarcoidosis (CS). The prevalence of CS causing this presentation in a North American population has not been investigated and was the purpose of this study., Methods: We prospectively evaluated patients aged 18-60 years presenting with unexplained 2nd or 3rd degree AVB and no previous history of sarcoidosis in any organ. All patients had fluorodeoxyglucose-positron emission tomography (FDG-PET) scans for the evaluation of CS. Japanese Ministry of Health Welfare (JMHW) criteria and biopsy results were used to confirm the diagnosis of CS. Subjects with advanced imaging suggestive of CS were investigated for extracardiac involvement. Patients were followed for major adverse cardiac events., Results: Thirty-two patients presenting with unexplained AVB underwent cardiac and whole body FDG-PET for the investigation of CS from February 2010 to June 2013. Mean age was 52.8 ± 6.2 years, and 20 were male. CS was diagnosed in 11/32 (34%) subjects and 11/11 were subsequently diagnosed with extra-CS. Average follow-up was 21 ± 9 months. Adverse events were observed in 3 subjects with CS but none in subjects with idiopathic AVB. All 3 patients presented with heart failure, 2 also had recurrent VT resulting in ICD shocks., Conclusions: In this prospective study of consecutive patients aged ≤60 years presenting with unexplained AVB, we found that 11/32 (34%) had previously undiagnosed CS. Among patients with CS, 3/11 had adverse clinical outcomes compared with 0/21 (P = 0.011). Our data suggest that all patients aged ≤60 years with unexplained AVB should be investigated for CS. Moreover, patients diagnosed with CS should be closely followed., (© 2014 Wiley Periodicals, Inc.)

Background: There are no published clinical consensus guidelines or systematic evaluation supporting the use of corticosteroids for the treatment of cardiac sarcoidosis. The purpose of this study was to systematically review the published data on corticosteroid treatment of cardiac sarcoidosis., Methods: Studies were identified from MEDLINE, EMBASE, Cochrane Controlled Trials Register, Cochrane Database of Systematic Reviews, and National Institutes of Health Clinical Trials.gov database. The quality of included articles was rated using Scottish Intercollegiate Guidelines Network 50. Outcomes examined were atrioventricular (AV) conduction, left ventricular function, ventricular arrhythmias, and mortality., Results: A total of 1491 references were retrieved and 10 publications met the inclusion criteria. There were no randomized trials and all publications were of poor to fair quality. In the 10 reports, 257 patients received corticosteroids and 42 patients did not. There were 57 patients with AV conduction disease treated with corticosteroids, with 27/57 (47.4%) improving. In contrast, 16 patients were not treated with corticosteroids and 0/16 improved. Four publications reported on left ventricular function recovery, 2 reported on ventricular arrhythmia burden, and 9 reported on mortality. However, the data quality were too limited to draw conclusions for any of these outcomes., Conclusions: Our systematic review identified 10 publications reporting outcomes after corticosteroid therapy. The best data relates to AV conduction recovery and corticosteroids appeared to be beneficial. It is not possible to draw clear conclusions about the utility of corticosteroids for the other outcomes. There is a clear need for large multicentre prospective registries and trials in this patient population., (Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Positron emission tomography using (18)F-Fluorodeoxyglucose (FDG) is an emerging modality for diagnosis of cardiac sarcoidosis (CS). We compared the location and degree of FDG uptake in CS patients presenting with either advanced atrioventricular block (AVB) or ventricular tachycardia (VT)., Methods and Results: We included consecutive patients who presented with either AVB or VT with a diagnosis of CS. A cohort of patients with clinically silent CS was included as controls. FDG activity was quantified as standardized uptake values (SUV) and both the overall mean left ventricular (LV) SUV as well as the Maximum Mean Segmental SUV was recorded for each patient. Receiver operator characteristic (ROC) analysis was performed to identify cutoff SUV values that best identified patients with VT. A total of 27 patients with CS were included (13 females; mean age, 56 ± 8 years; 8 VT, 12 AVB, and 7 controls). Both mean LV SUV and Max SUV in CS patients presenting with VT were significantly higher compared with those with AVB (mean SUV: VT median 5.33, range 4.7-9.35 versus AVB median 2.48, range 0.86-8.59, P=0.016; max SUV: VT median 11.07, range 9.24-14.4 versus AVB median 5.63, range 3.42-15.71, P=0.005) and compared with controls. There was no significant difference in SUV values between AVB patients and controls. ROC analysis for identification of patients with VT showed AUCs of 0.93 and 0.895 for a mean LV SUV of >3.42 and a max SUV >8.56, respectively (P<0.001)., Conclusions: CS patients with VT displayed significantly higher FDG uptake when compared with those with AVB and asymptomatic controls. Further prospective studies are required to evaluate this finding.

Purpose of Review: Sarcoidosis is a granulomatous disease of unclear cause and variable presentation. Cardiac involvement can result in life-threatening conditions including heart block, ventricular tachycardia, sudden cardiac death, and heart failure. There is no consensus on the diagnosis and management of cardiac sarcoidosis and a practical update is needed to provide clinicians with guidance., Recent Findings: Three recent studies have described cardiac manifestations as the first presentation of sarcoidosis. In one study, cardiac sarcoidosis was found to be the underlying cause in 19% of adults aged less than 55 years presenting with new onset unexplained atrioventricular block. Also, there are increasing reports of patients with isolated cardiac sarcoidosis (i.e., without sarcoid in other organs). Finally, advances in imaging have enhanced our ability to detect myocardial involvement and perhaps follow response to treatment., Summary: Cardiac sarcoidosis should be considered in patients aged less than 55 years presenting with unexplained atrioventricular block and in patients with idiopathic cardiomyopathy and sustained ventricular tachycardia. Much remains to be learned about the condition, including the role of steroids and devices in treatment, and the place of advanced imaging in following the response to treatment. Collaborative multicenter studies are required to answer these important clinical questions.

Background: There are few data on the frequency and predictors of tachycardia-induced cardiomyopathy (TICM) in patients with persistent atrial flutter., Objectives: To examine the incidence of TICM in patients undergoing ablation for persistent atrial flutter, and to examine predictors for the development of TICM., Methods and Results: One hundred eleven patients met the inclusion criteria for the present study. Twenty-eight of 111 (25%) patients had cardiomyopathy before ablation. Sixteen of 28 (57%) patients showed significant improvement in their left ventricular (LV) function postablation. LV function improved to normal in 12 of 16 (75%) patients. Nineteen of 28 (68%) cardiomyopathy patients had preablation LV function in the range in which they would be considered for an implantable cardioverter defibrillator for primary prevention of sudden cardiac death. In nine of 19 (47%) patients, the ejection fraction improved such that an implantable cardioverter defibrillator was no longer indicated. In multivariate analysis, average ventricular rate during atrial flutter was the only independent predictor of reversibility of cardiomyopathy (P=0.013)., Conclusions: Sixteen of 28 (57%) cardiomyopathy patients with persistent atrial flutter had significantly improved LV function postablation. In 75% of these patients, LV function improved to normal.

The authors report a case of transient left ventricular apical ballooning after a prolonged electrophysiology procedure. Interventional electrophysiologists should be aware of this syndrome as a potential complication.

Reports from case series have estimated the incidence of peripartum cardiomyopathy (PC) at 1 case/1,485 live births to 1 case/15,000 live births and probable mortality rates of 7% to 60%. The objective of this study was to produce the first population-based study of the incidence, mortality, and risk factors for PC. The National Hospital Discharge Survey was used. Discharge information was available for 3.6 million patient discharges from 1990 to 2002. There were an estimated 16,296 cases of PC from 1990 to 2002. During this period, there were 51,966,560 live births in the United States. Thus, the incidence of PC was 1 case/3,189 live births. There was a trend toward an increase in PC incidence during the study period, with an estimate for the years 2000 to 2002 of 1 case/2,289 live births. The in-hospital mortality rate was 1.36% (95% confidence interval 0% to 10.2%). The total mortality rate was 2.05% (95% confidence interval 0.29% to 10.8%). Patients with PC were older (mean age 29.7 vs 26.9 years), were more likely to be black (32.2% vs 15.7%), and had a higher incidence of pregnancy associated hypertensive disorders (22.5% vs 5.87%) compared with national data. In conclusion, the incidence of PC is relatively uncommon, occurring at an average frequency of 1 case/3,189 live births from 1990 to 2002. The estimated mortality of 1.36% to 2.05% (95% confidence interval 0.29% to 10.8%) is less than previously reported from most case series.