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HEART FAILURE > SARCOID > Flashcards

SARCOID Flashcards

1
Q

What is sarcoidosis?

A

Sarcoidosis is an idiopathic granulomatous inflammatory disease with multisystem involvement, believed to be caused by an immune response to an environmental antigenic trigger in genetically susceptible individuals.

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2
Q

Pathological hallmark of sarcoidosis?

A

The presence of non-caseating granulomas is the signature histologic finding.

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3
Q

Most common organ involved in sarcoidosis?

A

Pulmonary system

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4
Q

Extra-pulmonary sites that can be affected in sarcoidosis?

A

Heart, liver, spleen, lymph nodes, eyes, cranial nerves and skin may be affected.

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5
Q

Prevelance of cardiac involvement in sarcoidosis patients?

A

About 25%
(although some patients with such involvement may be asymptomatic)

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6
Q

Demographics of sarcoidosis?

A

The incidence of sarcoidosis is highest in Scandinavian countries and among African Americans.

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7
Q

Mean age of onset of sarcoidosis?

A

The mean age of onset is 40-55 years, with a younger mean age of diagnosis in men compared with women.

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8
Q

Most important predictor of mortality in cardiac sarcoidosis?

A

Left ventricular dysfunction is the most important predictor of mortality in patients with cardiac sarcoidosis

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9
Q

Predictors of ventricular tachycardia in patients with cardiac sarcoidosis?

A

Abnormal late gadolinium enhancement on cardiac magnetic resonance and abnormalities on positron emission tomography (i.e., perfusion defect and increased fluorodeoxyglucose [FDG] uptake, or having focal FDG uptake by the right ventricle) are predictors of ventricular tachycardia, even among individuals with normal ejection fraction.

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10
Q

Cardiac sites involved in sarcoidosis?

A

Left ventricle (LV) and right ventricle (RV) are most commonly involved
Other sites may include the atria, papillary muscles, valves, coronary arteries, and pericardium.

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11
Q

Pathogenesis of sarcoidosis?

A

The pathogenesis of CS includes: (1) focal inflammation leading to fibrosis and scar formation by innate immune activation of macrophages and dendritic cells, (2) upregulation of major histocompatibility complex expression that affects the adaptive immune response, (3) upregulation of the rapamycin complex 1 with changes in metabolic and immune pathways, and (4) enhanced effector T-cell responses in combination with impaired regulatory T-cell response. These processes lead to cytokine and chemokine activation with epithelioid granulomatous inflammation.

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12
Q

Common manifestations of cardiac sarcoidosis?

A

Cardiac sarcoidosis most commonly manifests as atrial or ventricular arrhythmias, conduction abnormalities including heart block, right ventricular dysfunction, or left ventricular dysfunction.

1) AV block
2) Heart failure
3) Ventricular tachycardia
4) Frequent PVCs
5) Sudden cardiac death

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13
Q

Conduction abnormalities in sarcoidosis?

A

P-R prolongation
Sinus node arrest
AV block > syncope or SCD
IVCDs

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14
Q

Tachyarrhythmias in sarcoidosis?

A

Ventricular tachyarrythmias are common and could be due to re-entrant in areas of scar or abnormal automaticity in areas of granulomas
SVTs include Atrial fibrillation. Atrial flutter and Atrial tachycardia

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15
Q

Most common cause of death in cardiac sarcoidosis?

A

Sudden cardiac death (tachyarrhythmias or AV block)
Risk stratification is important as it is MCC of death

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16
Q

Cardiomyopathy in cardiac sarcoidosis?

A

Can cause dilated or restrictive cardiomyopathy

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17
Q

Coronary artery involvement in cardiac sarcoidosis?

A

CS rarely leads to coronary artery involvement from vasculitis but prevelance of atherosclerotic CAD is high in patients with cardiac sarcoidosis

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18
Q

Screening in patients with KNOWN extracardiac sarcoidosis?
Questions to ask?

A

Assess for symptoms or signs of cardiac involvement such as syncope, presyncope, lighthadedness, palpitations or heart failure symptoms

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19
Q

Screening in patients with KNOWN extracardiac sarcoidosis?
Should we get EKG?

A

If patients do not have any symptoms and have extracardiac sarcoidosis we should get yearly EKGs to check for PR prolongation or conduction abnormalities

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20
Q

In patients with extra cardiac sarcoidosis with symptoms suggestive of cardiac involvement next diagnostic step?

A

CMR preferrably or FDG-PET

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21
Q

Echo findings suspicious for sarcoidosis?

A

1) Focal wall thinning or thickness (MC is basal anteroseptum in PLAX view)
2) Unexplained aneurysms
3) LV dysfunction

22
Q

Imaging findings that are suspicious for sarcoidosis?

A

Echo: Focal wall thinning or thickness, unexplained aneursyms or LV dysfunction
CMR: LGE
FDG-PET: Resting perfusion defects

23
Q

When should we send patients with extra-cardiac sarcoidosis for CMR or FDG-PET?

A

1) Symptoms such as palpitations, syncope, pre-syncope
2) Abnormal EKG with conduction abnormalities
3) Abnormal suspicious echocardiogram

24
Q

When shoud we use imaging with CMR or FDG-PET in patients with no prior history of extra-cardiac sarcoidosis?

A

1) Unexplained Mobitz II or 3rd degree AV block in young < 60 patients
2) Sustained monomorphic VT of unknown etiology

25
Q

How can a definitive diagnosis of cardiac sarcoid be established?

A

Histology through endomyocardial biopsy showing non caseating granulomas

26
Q

Limitations of EMB?

A

The sensitivity of a standard EMB random sample is limited (~30%), and thus a negative EMB does not rule out CS. The limited sensitivity is due to the patchy involvement in CS, as well as the fact that most times EMB is only obtained from the subendocardial layer of the midseptum.

27
Q

Does a negative EMB rule out cardiac sarcoidosis?

A

No

28
Q

Clinical criteria for diagnosis of sarcoidosis in the absence of histological findings?

A

Heart Rhythm Society (HRS) criteria for diagnosis of cardiac sarcoidosis (CS) in the absence of histological findings include:
- histologic diagnosis of extracardiac sarcoidosis with cardiac findings suspicious for CS (including steroid-responsive left ventricular [LV] dysfunction or heart block), unexplained LV dysfunction, unexplained ventricular tachycardia, high-grade heart block, patchy uptake on cardiac fluorodeoxyglucose–positron emission tomography, late gadolinium enhancement on cardiac magnetic resonance consistent with CS, or positive gallium scan.

29
Q

Limitations of clinical diagnostic criteria for sarcoidosis?

A

Clinical criteria for the diagnosis of CS in the absence of histologic confirmation are available but have important limitations. For example, the Heart Rhythm Society (HRS) suggests that a clinical diagnosis of “probable CS” (>50% likelihood) can be established, but only when there is histologic confirmation of extracardiac sarcoidosis (Table 2). However, isolated CS without extracardiac involvement is possible in approximately 25% of CS cases.

When the diagnosis of CS remains uncertain based on current clinical criteria, integration of data from both cardiac magnetic resonance (CMR) imaging and cardiac positron emission tomography (PET) may be helpful.

30
Q

ECG findings in sarcoidosis?

A

ECG findings in CS are nonspecific but can include P-R interval prolongation, second- or third-degree atrioventricular (AV) block, QRS prolongation, frequent premature ventricular complexes, atrial arrhythmias, pathologic Q waves, and nonspecific ST-segment and T-wave abnormalities.

31
Q

Echocardiographic findings in sarcoidosis?

A

Among patients who have CS, echocardiography is useful for identifying the extent of cardiac involvement—including assessment of valvular function and pericardial involvement—and can be used for serial evaluation of LV systolic function. Findings may include focal areas of edema leading to increased wall thickness, or focal areas of akinesis, dyskinesis, and aneurysms in more advanced disease. LV global longitudinal strain is often reduced and lower values correlate with adverse cardiovascular events. Patients may have reduced or preserved ejection fraction (EF). The RV is frequently involved. Diastolic dysfunction is a nonspecific finding. Pulmonary hypertension is infrequently observed.

32
Q

First line imaging in patients with suspected cardiac sarcoidosis?

A

CMR : LGE can be focal or extensive

33
Q

What does negative CMR mean for cardiac sarcoidosis?

A

No LGE on CMR

34
Q

No LGE on CMR means?

A

Very high negative predicted value of CS

35
Q

When to perform Cardiac PET in patients with suspected cardiac sarcoidosis?

A

1) In patients who cannot undergo CMR, or when CMR results are inconclusive (i.e., nonspecific LGE pattern)

2) When CMR findings are positive, PET is complementary and can help: 1) evaluate the presence and amount of active myocardial inflammation—and thus identify the potential role of immunosuppressive therapies; 2) identify the extent of extracardiac inflammation, which may be a factor in the choice and intensity of medical therapy; and 3) identify potential extracardiac biopsy sites.

36
Q

When should EMB be considered for suspected cardiac sarcoidosis?

A

EMB may be most helpful in the following scenarios: 1) absence of accessible extracardiac biopsy sites; 2) after a negative extracardiac biopsy, if suspicion for CS persists; and 3) in cases in which disease is confined to the heart.

37
Q

Most common CMR finding in sarcoidosis?

A

Multifocal subepicardial and midwall LGE is the most common finding in CS . The basal LV wall, lateral wall, and septum are most commonly involved. Cine CMR provides accurate assessment of LV volumes, EF, and regional wall motion.

38
Q

Limitations of CMR in diagnosing cardiac sarcoidosis?

A

There are limitations to CMR. LGE is not specific for CS. In rare cases, LGE can be absent in cases of myocardial inflammation; therefore, when clinical suspicion is high, a negative LGE study is insufficient to rule out CS. In addition, because LGE is most commonly due to scar, it cannot be used to determine whether inflammation is present. Techniques such as T2 imaging can detect inflammation by identifying increased water content by the myocardium, but as of yet are not well validated in CS and have limited correlation with FDG-PET.

39
Q

Gladolinium should be avoided when?

A

Gadolinium should be avoided in cases of estimated glomerular filtration rate <30 mL/min/1.73 m2, dialysis, and acute kidney injury due to the risk of nephrogenic systemic fibrosis.

40
Q

FDG-PET in diagnosing cardiac sarcoidosis?

A

FDG-PET can aid with diagnosis, prognosis, and response to therapy by quantifying active myocardial inflammation. FDG-PET imaging does not require stress testing and usually involves three components: 1) cardiac FDG images; 2) rest myocardial perfusion imaging using single-photon emission computed tomography or PET; and 3) limited whole-body FDG images to identify extracardiac involvement.

41
Q

Typical findings in FDG-PET in cardiac sarcoidosis?

A

Typical findings in CS include areas of perfusion defect associated with focal FDG uptake. The images need to be interpreted in the relevant clinical context, as hibernating myocardium from obstructive coronary artery disease may also result in similar abnormalities. Importantly, the presence of FDG uptake is not specific to CS and may be seen in other forms of myocarditis or in other inflammatory cardiomyopathies.

42
Q

Clinical scenarios in which Cardiac PET maybe useful in suspected of Known CS?

A
43
Q

Clinical features in cardiac sarcoidosis associated with a worse prognosis?

A

HF symptoms, depressed LVEF, and sustained ventricular tachycardia (VT). Moreover, LV dysfunction is the most important predictor of mortality in patients with CS

44
Q

Imaging findings in cardiac sarcoidosis associated with a worse prognosis?

A

1) cardiac FDG-PET findings, which can identify patients at higher risk for death or VT; 2) focal FDG uptake in the RV on FDG-PET, which is associated with worse outcomes; and 3) presence of LGE on CMR, which is associated with adverse cardiac outcomes

45
Q

Management for patients with cardiac sarcoidosis?

A

HF treatment: Patients with CS and HF should receive standard guideline-directed medical therapy for HF with reduced EF or HF with preserved EF.

Immunosuppression: is recommended in cases of probable or definite CS when there is imaging evidence of active myocardial inflammation. The presence of inflammation in other organs—as assessed by a limited whole-body PET—may indicate greater benefit of systemic anti-inflammatory treatment and warrants referral to pulmonary or rheumatology for comanagement of other organ systems.

46
Q

First-line Immunosuppressive therapy for treatment of cardiac sarcoidosis?

A

Glucocorticoids are most commonly used. Optimal dose, duration of therapy, and when to use other agents is uncertain. Recommendations are based on consensus, as there have been no randomized clinical trials of steroid therapy in CS. It is reasonable to initiate prednisone at 40-60 mg/day followed by a slow taper after 1-3 months to a maintenance dose of 10-15 mg/day for 1 year. Duration of therapy is controversial and often depends on repeat imaging and clinical course. Serial imaging with FDG-PET (e.g., after 6 months of initiating therapy) may aid management and ensure appropriate response

47
Q

Second-line Immunosuppressive therapy for treatment of cardiac sarcoidosis?

A

Steroid-sparing agents such as methotrexate, azathioprine, infliximab, or mycophenolate mofetil may be used when there is inadequate response to glucocorticoids alone or intolerable side effects to glucocorticoids. However, data on these agents in CS are limited to observational studies or case reports.

48
Q

Third-line Immunosuppressive therapy for treatment of cardiac sarcoidosis?

A

Tumor necrosis factor (TNF) antagonists such as infliximab may worsen HF and should be used with caution in patients who have symptomatic HF. Given toxicities associated with anti-TNF agents, these agents are not first-line options in CS.

49
Q

Pacemaker indications in patients with cardiac sarcoidosis?

A

Permanent pacing is reasonable for patients with sarcoidosis who have second-degree Mobitz type II AV block, high-grade AV block, or third-degree AV block. Current guidelines recommend that ICD placement can be beneficial for patients with CS who have indications for permanent pacing

50
Q

ICD indications in patients with cardiac sarcoidosis?

A

Note: For patients for whom it is uncertain if an ICD can be helpful, an EP study may help risk-stratify the patient.

51
Q

Atrial arrhythmias in cardiac sarcoidosis?

A

While anticoagulation in patients with CS and atrial fibrillation should be guided by risk estimated by the CHA2DS2-VASc score, several observational studies have suggested that patients with CS have a higher incidence of atrial fibrillation. Class I antiarrhythmic agents are contraindicated for the treatment of arrhythmias associated with CS.

52
Q

Screening for cardiac sarcoidosis?

A

Patients with a history of biopsy-proven extracardiac sarcoidosis should have a clinical evaluation for CS, including history, examination, and a 12-lead electrocardiogram (ECG). History of syncope, presyncope, and significant palpitations should prompt advanced imaging testing to screen for disease, preferably using CMR or cardiac PET

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