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.
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
Graphical Abstract
Keywords
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Article info
Publication history
Published online: May 04, 2022
Accepted:
April 28,
2022
Received in revised form:
April 22,
2022
Received:
January 24,
2022
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
