Can sarcoidosis cause concentric heart failure?

Can Sarcoidosis Cause Heart Failure?

Yes, sarcoidosis can cause heart failure through myocardial infiltration with noncaseating granulomas, leading to both systolic and diastolic dysfunction, though the pattern is typically NOT concentric—it more commonly presents with regional wall motion abnormalities, ventricular dilation, or restrictive physiology rather than concentric hypertrophy. 1

Cardiac Involvement Patterns in Sarcoidosis

Prevalence and Clinical Manifestations

• Autopsy studies demonstrate cardiac involvement in 20-50% of patients with systemic sarcoidosis, though only 5-8% develop clinically manifest cardiac disease 1, 2
• An additional 20-25% have asymptomatic (clinically silent) cardiac involvement detectable only by advanced imaging 2, 3
• Cardiac sarcoidosis presents with three primary manifestations: conduction abnormalities, ventricular arrhythmias, and heart failure—NOT typically concentric hypertrophy 1

Myocardial Involvement Characteristics

• The disease causes myocardial infiltration with noncaseating granulomas that preferentially affects the basal septum and lateral walls in mid-myocardial or subepicardial distributions 4
• After the early granulomatous inflammation stage, sarcoidosis can progress to end-organ fibrosis, creating a substrate for both systolic dysfunction and arrhythmias 1
• The pattern is infiltrative rather than concentric—echocardiography typically shows wall motion abnormalities, abnormal septal thickness (not uniform concentric thickening), and abnormal Doppler filling patterns 1

Ventricular Dysfunction and Heart Failure

Left Ventricular Dysfunction

• Marked LV dysfunction at diagnosis (LVEF <35%) predicts adverse outcomes in a 25-year Finnish study of 110 cardiac sarcoidosis patients 1
• The extent of left ventricular dysfunction is the most important mortality predictor in clinically manifest cardiac sarcoidosis 2, 3
• Global reductions in LVEF can occur, but this represents dilated or restrictive cardiomyopathy patterns, not concentric hypertrophy 1

Right Ventricular Involvement

• RV involvement can occur and may mimic arrhythmogenic right ventricular cardiomyopathy 1
• The presence of RV involvement on PET scanning is associated with increased risk of death 1

Distinguishing from Concentric Hypertrophy

Key Imaging Differences

• Cardiac MRI shows late gadolinium enhancement in mid-myocardial and subepicardial distributions, particularly in basal septum and lateral walls—this is pathognomonic for sarcoidosis and distinct from the diffuse, symmetric pattern of concentric hypertrophy 4
• Late gadolinium enhancement has 75-100% sensitivity and 75-77% specificity for cardiac sarcoidosis 4
• T2-weighted imaging shows high signal in acute stages due to myocardial edema and inflammation 4

Infiltrative Disease Classification

• Sarcoidosis is classified as an infiltrative cardiomyopathy, but acute sarcoidosis results in myocardial thickening while chronic sarcoidosis results in myocardial thinning (not concentric hypertrophy) 1
• This distinguishes it from true concentric hypertrophy seen in hypertensive heart disease or hypertrophic cardiomyopathy 1

Prognostic Implications

Risk Stratification

• The presence of late gadolinium enhancement is associated with 4.9% per year risk of death or VT compared to 0.24% per year when absent 4, 1
• Extensive LV and RV involvement on imaging represents a particularly high-risk feature 1
• Ventricular arrhythmias and sudden cardiac death can occur even with preserved LVEF, emphasizing that heart failure is not the only cardiac manifestation 1

Mortality Risk

• Cardiac involvement contributes greatly to higher mortality in cardiac sarcoidosis compared to sarcoidosis without cardiac involvement 1
• Cardiac disease is one of the more common causes of death in sarcoidosis, though this mortality risk may be preventable with appropriate therapies 1

Clinical Pitfalls to Avoid

• Do not assume normal LVEF excludes significant cardiac sarcoidosis—ventricular arrhythmias and conduction disease can occur with preserved systolic function 1
• Do not confuse the infiltrative pattern of sarcoidosis with concentric hypertrophy—the distribution of involvement is characteristically patchy and regional, not uniform 4
• Conduction abnormalities (including complete heart block) occur in approximately 25-30% of cardiac sarcoidosis patients and may be the presenting feature 1
• Isolated cardiac sarcoidosis occurs in up to two-thirds of clinically manifest cases, so absence of pulmonary or systemic findings does not exclude the diagnosis 3