Graphical Abstract Graphical abstract In cardiac sarcoidosis (CS), inflammatory granulomas invade the heart leading to injury and fibrosis (yellow stars in the section of a sarcoidotic heart in the middle of the graph). CS is often subclinical, but, when clinically manifest, presents commonly with slow or fast arrhythmias or heart failure. On the left of the figure, positron emission tomography (PET) exposes focal septal uptake of 18-F fluorodeoxyglucose suggesting active inflammation (white arrow), and contrast-enhanced cardiac magnetic resonance (CMR) shows septal late gadolinium enhancement (arrows) indicating replacement fibrosis. Both constitute major diagnostic criteria for CS and entitle probable CS diagnosis if accompanied by confirmed extracardiac histology of sarcoidosis. Yet, the only way to definite diagnosis, demonstrated on the right, is myocardial biopsy showing non-necrotic granulomas (black arrow). The therapy of CS is based on immunosuppression and management of heart block, ventricular arrhythmias, and heart failure. The risk of sudden cardiac death (SCD) needs assessment and consideration of an implantable cardioverter-defibrillator (ICD). With current therapy, expected 5-year survival is well above 90% as shown by the Kaplan–Meier graph of a 398-patient Finnish CS cohort.

/pdf/cardiac-sarcoidosis-phenotypes-diagnosis-treatment-and-20vaabo6.pdf

Cardiac sarcoidosis: phenotypes, diagnosis, treatment, and prognosis

Background Isolated cardiac sarcoid (iCS) is reported to have more severe clinical presentation and greater risk of adverse events compared with cardiac sarcoid (CS) with extracardiac involvement (nonisolated CS). Delays in diagnosing specific organ involvement may play a role in these described differences. Methods and Results A retrospective observational study of patients with CS over a 20‐year period was conducted. Objective evidence of organ involvement and time of onset based on consensus criteria were identified. CS was confirmed by histology in all patients from myocardium only (iCS) or extracardiac tissue (nonisolated CS). The primary end point was a composite of mortality, orthotopic heart transplant, and durable left ventricular assist device implantation. CS was isolated in 9 of 50 patients (18%). Among baseline characteristics, iCS and nonisolated CS differed significantly only in the frequency of sustained ventricular tachycardia at presentation (78% versus 37%; P=0.03) and delay in CS diagnosis >6 months (67% versus 5%; P<0.01). A nonsignificant trend toward lower left ventricular ejection fraction and more frequent heart failure in iCS was observed. Over a median follow‐up of 9.7 years (95% CI, 6.8–10.8), 18 patients reached the primary end point (13 deaths, 2 orthotopic heart transplants, and 3 durable left ventricular assist device implantations). The 1‐, 5‐, and 10‐year event‐free survival rates were 96% (95% CI, 85%–99%), 79% (95% CI, 64%–88%), and 58% (95% CI, 40%–73%), respectively, without differences between groups. There were no significant predictors of the primary end point, including delayed CS diagnosis. Conclusions Long‐term outcomes were similar between iCS and nonisolated CS in patients with histologically documented sarcoid. Diagnostic delays may contribute to differences in the dominant clinical presentation, despite similar outcomes.

Long‐Term Outcomes of Cardiac Sarcoid: Prognostic Implications of Isolated Cardiac Involvement and Impact of Diagnostic Delays

Outcomes of heart transplantation in cardiac amyloidosis: an updated systematic review

Sarcoidosis is an inflammatory multisystemic disease of unknown etiology characterized by the formation of non-caseating granulomas.Sarcoidosis can affect any organ, predominantly the lungs, lymphatic system, skin and eyes.While >90% of patients with sarcoidosis have lung involvement, an estimated 5% of patients with sarcoidosis have clinically manifest cardiac sarcoidosis (CS), whereas approximately 25% have asymptomatic, clinically silent cardiac involvement verified by autopsy or imaging studies.CS can present with conduction disturbances, ventricular arrhythmias, heart failure or sudden cardiac death.Approximately 30% of <60-year-old patients presenting with unexplained high degree atrioventricular (AV) block or ventricular tachycardia are diagnosed with CS, therefore CS should be strongly considered in such patients.CS is the second leading cause of death among patients affected by sarcoidosis after pulmonary sarcoidosis, therefore its early recognition is important, because early treatment may prevent death from cardiovascular involvement.The establishment of isolated CS diagnosis sometimes can be quite difficult, when extracardiac disease cannot be verified.The other reason for the difficulty to diagnose CS is that CS is a chameleon of cardiology and it can mimic (completely or almost completely) different cardiac diseases, such as arrhythmogenic cardiomyopathy, giant cell myocarditis, dilated, restrictive and hypertrophic cardiomyopathies.In this review article we will discuss the current diagnosis and management of CS and delineate the potential difficulties and pitfalls of establishing the diagnosis in atypical cases of isolated CS. 

Cardiac Sarcoidosis: A Comprehensive Clinical Review

Cardiac transplantation as surgical treatment for cardiac sarcoidosis.

Background Cardiac sarcoidosis is an increasingly common indication for a heart transplant, but there is a paucity of knowledge with regard to long-term outcomes following transplant. Methods We utilized the Organ Procurement and Transplantation Network database to retrospectively analyze adult patients undergoing first-time, single-organ heart transplant between January 1999 and March 2020. Results Of the 41,447 patients that underwent heart transplant during the study period, 289 (0.7%) were transplanted for a primary diagnosis of restrictive cardiomyopathy due to cardiac sarcoidosis (RCM-Sarcoidosis). RCM-Sarcoidosis was associated with 33% reduced risk of mortality over 10 years compared to non-RCM indications in a multivariable Cox proportional hazards model (p = .03). Ten-year survival functions were improved among RCM-Sarcoidosis compared to this reference group (73.4% [64.2%-80.6%] vs. 59.5% [58.8%-60.1%], p = .002). Among patients transplanted after 1999 who had at least 10 years of follow-up (n = 19,489), median survival of RCM-Sarcoidosis patients was 11.9 [8.3-14.6] years while that of non-RCM patients was 9.9 [4.0-13.1] years. RCM-Sarcoidosis was not associated with an increased risk of secondary outcomes such as graft failure, rejection, or infection. The incidence of retransplant was comparable between RCM-Sarcoidosis and non-RCM patients (1.38% vs. 1.50%, p = .93). Conclusions These data suggest that long-term outcomes following transplant for cardiac sarcoidosis are favorable compared to heart transplant for other indications.

Long-term survival after heart transplantation for cardiac sarcoidosis.

BACKGROUND The incidence of systemic amyloidosis is rising, and there is a concomitant rise in heart transplant for an indication of cardiac amyloidosis. METHODS We utilized the Organ Procurement and Transplantation Network (OPTN) database to retrospectively assess survival and outcomes in adult patients undergoing heart transplant for cardiac amyloidosis from 1999 to 2019. We also compared survival among four distinct time periods: 1999-2001, 2002-2008, 2008-2015, 2016-2019. RESULTS Of 41,103 patients, 425 (1.03%) were transplanted for an indication of restrictive cardiomyopathy due to cardiac amyloidosis (RCM-Amyloidosis). The percent of all transplants occurring for RCM-Amyloidosis increased from 0.25% in the 1999-2001 era to 1.74% in the 2015-2019 era (p < .001). Across eras, Kaplan-Meier survival functions were comparable between RCM-Amyloidosis and non-RCM patients at 1 year (88% vs. 89%, p = .56) and at 5 years (72% vs. 77%, p = .092), but worse for RCM-Amyloidosis patients at 10 years (44% vs. 59%, p = .002). With adjustment for other clinical variables in multivariable Cox regression model, RCM-Amyloidosis was not associated with increased risk of death at 1 year (hazard ratio [HR] = 1.11, p = .56) or at 5 years (HR = 1.20, p = .18), but it was associated with increased risk of death at 10 years (HR = 1.35, p = .01). Cardiac amyloidosis was not associated with any morbidity outcomes following transplant, including graft failure, acute rejection, or hospitalization for infection or rejection. CONCLUSIONS Our data suggest a trend of improving survival among RCM-Amyloidosis patients compared with non-RCM patients across transplant eras, with current similarities in 1- and 5-year survival but a persistent, increased risk of mortality at 10 years.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/jocs.15796

Improving contemporary outcomes following heart transplantation for cardiac amyloidosis.

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Long-term survival after heart transplantation for cardiac sarcoidosis.

Improving contemporary outcomes following heart transplantation for cardiac amyloidosis.