Cardiac sarcoidosis outcome differences: A comparison of patients with de novo cardiac versus known extracardiac sarcoidosis at presentation. (July 2022)

• Record Type: Journal Article
• Authors: Rosen, Natalie S.; Pavlovic, Noelle; Duvall, Chloe; Wand, Alison L.; Griffin, Jan M.; Okada, David R.; Chrispin, Jonathan; Tandri, Harikrishna; Mathai, Stephen C.; Stern, Barney; Pardo, Carlos A.; Kasper, Edward K.; Sharp, Michelle; Chen, Edward S.; Gilotra, Nisha A.
• Is Part Of: Respiratory medicine. Volume 198(2022)
• Publication Date: 2022-07
• Subjects: Cardiomyopathy -- Arrhythmia -- Cardiac sarcoidosis -- Pulmonary sarcoidosis -- Immunosuppression; Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2022.106864
• Languages: English
• ISSNs: 0954-6111

Abstract

Abstract: Background: Sarcoidosis is a systemic disease characterized by granulomatous inflammation. Cardiac involvement is associated with increased morbidity. However, differences in clinical characteristics and outcomes based on initial sarcoidosis organ manifestation in patients with cardiac sarcoidosis (CS) have not been described. Methods: A retrospective cohort of 252 patients with CS at an urban, quaternary medical center was studied. Presentation, treatment and outcomes of de novo CS and prior ECS groups were compared. Survival free of primary composite outcome (left ventricular assist device implantation, orthotopic heart transplantation (OHT), or death) was assessed. Results: There were 124 de novo CS patients and 128 with prior ECS at time of CS diagnosis. De novo CS patients were younger at CS diagnosis (p = 0.020). De novo CS patients had a more advanced cardiac presentation: lower left ventricular ejection fraction (LVEF) (p < 0.001), more frequent sustained ventricular arrhythmias (VA) (p = 0.001), and complete heart block (p = 0.001). During follow-up, new VA (p < 0.001), ventricular tachycardia ablation (p < 0.001), and OHT (p = 0.003) were more common in the de novo CS group. Outcome free survival was significantly shorter for de novo CS patients (p = 0.005), with increased hazard of primary composite outcome (p = 0.034) and development of new VA (p = 0.027) when compared to ECS patients. Overall mortality was similar between groups. Conclusion: Patients presenting with CS as their first recognized organ manifestation of sarcoidosis have an increased risk of adverse cardiac outcomes as compared to those with a prior history of ECS. Improved awareness and diagnosis of CS is warranted for earlier recognition. Graphical abstract: Image 1 Highlights: De novo cardiac sarcoidosis (CS) presents with more advanced cardiomyopathy, ventricular arrhythmia and heart block. De novo CS patients have increased adverse cardiac outcomes compared to those with a prior extracardiac sarcoidosis (ECS). In those with CS, de novo and prior ECS patients have similar mortality. Improved awareness and diagnosis of CS is warranted for earlier recognition.