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← Section VI · Miscellaneous Topics (Role of Echo)
VI.D

Systemic Diseases

10 cards

Notes

Amyloidosis

  • AL (light chain) - associated with plasma cell dyscrasia (myeloma).
  • ATTR (transthyretin) - wild-type (senile, elderly men) or hereditary.
  • AA (secondary) - chronic inflammatory disease. Rare cardiac involvement.
  • Echo: concentric LVH, biatrial enlargement, small pericardial effusion, apical-sparing GLS, low ECG voltage.

Sarcoidosis

  • Non-caseating granulomatous inflammation.
  • Cardiac involvement: patchy - mimics DCM, HCM (basal septal thinning), or restrictive.
  • Common: AV block, VT, LV dysfunction.
  • Diagnosis: PET or cMRI (LGE); biopsy.

Hemochromatosis

  • Iron overload cardiomyopathy - initially restrictive, later dilated.
  • T2* on cMRI is the imaging gold standard.

Systemic lupus erythematosus (SLE)

  • Pericarditis (most common).
  • Libman-Sacks endocarditis (sterile verrucous valvular vegetations).
  • Coronary vasculitis / accelerated atherosclerosis.
  • Myocarditis.

Rheumatoid arthritis

  • Pericardial effusion.
  • Nodular myocardial or valvular involvement (rare).
  • Increased CV mortality from accelerated atherosclerosis.

Ankylosing spondylitis / seronegative spondyloarthritis

  • Aortic root dilation and aortic regurgitation.
  • Conduction disease (AV block).

Scleroderma

  • Pulmonary hypertension (WHO Group 1).
  • Diastolic dysfunction from myocardial fibrosis.
  • Pericarditis.

Diabetes

  • Diastolic dysfunction (early manifestation).
  • Diabetic cardiomyopathy (LV hypertrophy and dysfunction independent of CAD/HTN).

HIV

  • Dilated cardiomyopathy (viral or antiretroviral toxicity).
  • Pericardial effusion.
  • Pulmonary hypertension.
  • Accelerated CAD.

Chagas disease

  • Trypanosoma cruzi.
  • Apical LV aneurysm with preserved basal function.
  • Conduction disease (RBBB + LAFB).

Endocrine

  • Hyperthyroidism - high-output state, AF, mitral regurg from annular dilation.
  • Hypothyroidism - pericardial effusion, bradycardia, diastolic dysfunction.
  • Acromegaly - LVH, diastolic dysfunction; late dilated CM.
  • Cushing syndrome / pheochromocytoma - hypertensive LVH.

Carcinoid syndrome

  • Serotonin-producing tumor of the mid-gut with hepatic metastases.
  • Right-sided plaque-like fibrous deposits → tricuspid and pulmonic valve thickening, retraction, immobility → mixed regurgitation and stenosis.
  • Left-sided involvement only if right-to-left shunt (PFO) or bronchial primary.

Marfan and other connective tissue disease

  • Marfan (fibrillin-1): aortic root dilation, MVP, ectopia lentis, tall stature.
  • Loeys-Dietz (TGFβ-R): more aggressive aortic dilation, bifid uvula, hypertelorism.
  • Ehlers-Danlos type IV: arterial rupture, less commonly aortic root dilation.

Cards

  • basicVI.D-001
    Cardiac manifestations of SLE?
    → 1) Pericarditis (most common cardiac manifestation). 2) Libman-Sacks endocarditis (sterile verrucous vegetations on valves). 3) Coronary vasculitis / accelerated atherosclerosis. 4) Myocarditis.
  • basicVI.D-002
    Which sero-negative rheumatologic disease classically causes aortic root dilation and AR?
    → Ankylosing spondylitis (and related seronegative spondyloarthritis). Also causes conduction disease (AV block).
  • basicVI.D-003
    Which two valves are typically affected by carcinoid heart disease, and how?
    → Tricuspid valve and pulmonic valve — thickened, retracted, immobile leaflets producing mixed regurgitation and stenosis. Left-sided involvement only occurs with a right-to-left shunt (PFO) or bronchial carcinoid.
  • basicVI.D-004
    Marfan syndrome — genetic basis and classic cardiovascular echo findings?
    → Fibrillin-1 (FBN1) mutation on chromosome 15. Findings: aortic root (sinus of Valsalva) dilation, mitral valve prolapse, sometimes tricuspid prolapse. Extracardiac: ectopia lentis, tall stature.
  • basicVI.D-005
    How does Loeys-Dietz syndrome differ from Marfan?
    → Loeys-Dietz: TGFβ receptor mutations. More aggressive aortic dilation and earlier surgical thresholds (root repair at smaller diameters). Characteristic bifid uvula, hypertelorism, arterial tortuosity throughout body.
  • basicVI.D-006
    Chagas cardiomyopathy — classic echo and ECG features?
    → Apical LV aneurysm (dyskinetic apex with preserved basal function) plus conduction disease (RBBB + left anterior fascicular block). Caused by chronic Trypanosoma cruzi infection.
  • basicVI.D-007
    Hemochromatosis cardiomyopathy — imaging characteristics?
    → Initially restrictive phenotype from myocardial iron deposition, later transitioning to dilated cardiomyopathy. Cardiac T2* by cMRI is the gold-standard imaging biomarker.
  • basicVI.D-008
    Which endocrine disorder classically causes a pericardial effusion, bradycardia, and diastolic dysfunction?
    → Hypothyroidism.
  • basicVI.D-009
    Cardiac manifestations of hyperthyroidism?
    → High-output state, sinus tachycardia or atrial fibrillation, functional mitral regurgitation from annular dilation, and (long-term) LV dysfunction from tachycardia-mediated cardiomyopathy.
  • basicVI.D-010
    Cardiac manifestations of HIV/AIDS?
    → Dilated cardiomyopathy (viral or antiretroviral toxicity), pericardial effusion, pulmonary hypertension, accelerated coronary artery disease.