296.1 🎓 醫孞生版

296.1.0.1 📌 䞀頁重點

296.1.0.1.1 Autoimmune / Connective Tissue Disease
296.1.0.1.1.1 Systemic Lupus Erythematosus (SLE)
  • Pericarditis (most common cardiac manifestation, ~ 30%): friction rub, effusion, tamponade
  • Myocarditis (rare but severe): LV dysfunction, arrhythmia
  • Libman-Sacks endocarditis: sterile vegetations on mitral valve (less commonly aortic); often in APS overlap
  • Premature atherosclerosis: ~ 5x risk of CAD; from inflammation + steroids
  • APS overlap: thrombosis, valve abnormalities (Ch281)
  • Treatment: HCQ + steroids + immunosuppressants; aggressive CV risk modification
296.1.0.1.1.2 Rheumatoid Arthritis (RA)
  • Pericarditis (often asymptomatic, found on echo)
  • Pericardial effusion with constrictive features
  • Accelerated atherosclerosis (RA = CAD risk equivalent)
  • Rheumatoid heart valve disease (rare)
  • Conduction abnormalities (rare)
  • Treatment: aggressive CV prevention (statin, ASA in CAD), DMARDs
296.1.0.1.1.3 Systemic Sclerosis (Scleroderma)
  • Myocardial fibrosis (focal, patchy)
  • Pulmonary hypertension (group 1 PAH); annual DETECT screening
  • Conduction abnormalities
  • Pericarditis / effusion
  • Right HF from PAH
  • Treatment: PAH-specific therapy (Ch282), nifedipine for Raynaud, immunosuppression
296.1.0.1.1.4 Antiphospholipid Syndrome (APS)
  • Valve abnormalities (Libman-Sacks-like)
  • Coronary thrombosis
  • Cardiac valve dysfunction
  • PAH
  • See Ch281 for AC management
296.1.0.1.1.5 Sjögren’s Syndrome
  • Pericarditis (rare)
  • Congenital heart block in babies of SSA/SSB-positive mothers
296.1.0.1.1.6 Polymyositis / Dermatomyositis (PM/DM)
  • Myocarditis (~ 30%); ↑ CK, troponin
  • Conduction abnormalities
  • HF
  • Treatment: steroids + immunosuppression
296.1.0.1.2 Vasculitis
296.1.0.1.2.1 Takayasu Arteritis (Large Vessel)
  • Aorta + main branches
  • “Pulseless disease” (subclavian stenosis)
  • Coronary ostial stenosis (causes angina, MI)
  • Aortic regurgitation (root dilation)
  • HTN (renal artery stenosis)
  • Aneurysm + dissection
  • Young Asian women
  • Treatment: steroids, MTX, biologics (tocilizumab); angiography reserved for severe
296.1.0.1.2.2 Giant Cell Arteritis (GCA) (Large Vessel)
  • Elderly
  • Aortic involvement (thoracic aneurysm in 15-20%)
  • Aortic regurgitation
  • Coronary ostial (rare)
  • Treatment: steroids; tocilizumab
296.1.0.1.2.3 Polyarteritis Nodosa (PAN) (Medium Vessel)
  • Coronary aneurysms
  • Pericarditis, myocarditis
  • HBV association
  • Treatment: steroids + cyclophosphamide
296.1.0.1.2.4 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
  • ANCA-associated; eosinophilic vasculitis
  • Myocarditis + endomyocardial fibrosis (Löffler)
  • HFrEF, restrictive features
  • Eosinophilia + asthma + neuropathy
  • Treatment: steroids + cyclophosphamide; mepolizumab
296.1.0.1.2.5 Granulomatosis with Polyangiitis (Wegener’s)
  • Less commonly cardiac
  • Pericarditis, valvulitis, coronary involvement
  • ANCA-positive (PR3 / c-ANCA)
296.1.0.1.2.6 Kawasaki Disease
  • Pediatric; coronary aneurysm
  • 5-day fever + mucocutaneous + lymphadenopathy
  • Treatment: IVIG + ASA; serial echo + cardiology follow-up
296.1.0.1.3 Infiltrative / Storage Diseases
296.1.0.1.3.1 Amyloidosis
  • AL (light chain): plasma cell dyscrasia (MM); systemic; cardiac, renal, hepatic, GI, neurologic
  • ATTR-wild type (senile): elderly; cardiac predominant
  • ATTR-hereditary (familial): TTR mutation; familial; neurologic + cardiac
  • AA (secondary): chronic inflammation; cardiac rare
  • Cardiac features: HFpEF → HFrEF, conduction issues, AF, arrhythmia, sudden death
  • Diagnosis: PYP scan (ATTR), Congo red biopsy, mass spec
  • Treatment:
    • AL: daratumumab + CyBorD (ANDROMEDA)
    • ATTR: tafamidis (ATTR-ACT); patisiran (transthyretin gene silencer)
    • Supportive: diuretics, AC for AF; CAUTION digoxin + CCB (binding to amyloid → toxicity)
296.1.0.1.3.2 Sarcoidosis
  • Granulomatous infiltration of heart
  • 5-25% of systemic sarcoidosis
  • AV block (most common cardiac presentation)
  • Cardiomyopathy (HFrEF, HFpEF)
  • Ventricular arrhythmia / SCD
  • Restrictive cardiomyopathy
  • Diagnosis: cardiac MRI + PET-CT + EMB
  • Treatment: steroids + immunosuppressants (MTX, AZA, infliximab); ICD; pacemaker; HF therapy
296.1.0.1.3.3 Hemochromatosis
  • Hereditary (HFE mutation) or secondary (transfusion-dependent thalassemia, MDS)
  • Iron overload cardiomyopathy — DCM phenotype, HFrEF
  • Conduction abnormalities
  • AF
  • Diagnosis: ferritin, transferrin sat, CMR T2* (cardiac iron quantification)
  • Treatment: phlebotomy (hereditary) or chelation (transfusion-dependent — deferasirox, deferiprone)
296.1.0.1.3.4 Fabry Disease
  • X-linked α-galactosidase A deficiency
  • Glycosphingolipid accumulation
  • LVH (mimics HCM)
  • Renal failure, neuropathy, angiokeratomas
  • Newer: cardiac variant predominant
  • Diagnosis: enzyme activity, genetic testing
  • Treatment: enzyme replacement (agalsidase α/β), migalastat (chaperone), pegunigalsidase
296.1.0.1.3.5 Glycogen Storage Disease
  • Pompe (acid α-glucosidase deficiency): infantile + late-onset; HCM phenotype
  • Cori, Andersen, Forbes others
  • ERT (enzyme replacement therapy) for Pompe
296.1.0.1.4 Endocrine
296.1.0.1.4.1 Thyroid Disease

Hyperthyroidism: - Sinus tachycardia, AF (10-15%) - HF (high-output) - LVH (long-standing) - Increased CV mortality - Treatment: anti-thyroid (methimazole, PTU), β-blocker, RAI

Hypothyroidism: - Bradycardia - Pericardial effusion (rare myxedema) - Diastolic dysfunction - ↓ Contractility - Lipid abnormalities - Treatment: levothyroxine (cautious in CAD)

296.1.0.1.4.2 Cushing’s Syndrome (Ch277)
  • HTN, DM, dyslipidemia
  • Accelerated atherosclerosis
  • Treatment: address cortisol excess + CV risk
296.1.0.1.4.3 Acromegaly
  • LVH, HF
  • HTN, DM, OSA
  • Diastolic dysfunction
  • Treatment: surgery, somatostatin analogs
296.1.0.1.4.4 Pheochromocytoma (Ch277)
  • HTN, episodic
  • Cardiomyopathy (catecholamine-induced)
  • Treatment: α-blockade → β-blockade → surgery
296.1.0.1.4.5 Diabetes Mellitus
  • Diabetic cardiomyopathy (independent of CAD)
  • Premature CAD
  • Microvascular
  • HFpEF + HFrEF
  • Autonomic neuropathy → arrhythmia
  • Treatment: SGLT2i, GLP-1 RA, ACEi/ARB, statin, multifactorial CV RF management
296.1.0.1.4.6 Hyperparathyroidism (Primary)
  • HTN
  • LVH
  • Conduction abnormalities
  • Vascular calcification
  • Surgery (parathyroidectomy) for severe disease
296.1.0.1.5 Infectious
296.1.0.1.5.1 Syphilis (Tertiary)
  • Syphilitic aortitis: ascending aorta + arch
  • Aortic regurgitation + aortic aneurysm + aortic stenosis
  • Coronary ostial stenosis (less common)
  • VDRL / RPR + FTA-ABS / TPPA
  • Treatment: penicillin G + valve surgery if severe AR
296.1.0.1.5.2 Tuberculosis
  • Pericarditis (most common cardiac TB)
  • Effusive → effusive-constrictive → constrictive
  • High ADA in pericardial fluid (> 40)
  • Treatment: 4-drug TB regimen + steroids (debated) + pericardiectomy if constrictive
296.1.0.1.5.3 HIV
  • Pericardial effusion (common)
  • Cardiomyopathy (HFrEF — direct viral effect, opportunistic infections, antiretroviral side effects)
  • Pulmonary HTN (group 1)
  • Accelerated atherosclerosis
  • ART-related: abacavir → MI risk (?); other RX side effects
  • HIV-associated MI: ↑ in HIV
  • Statins more aggressively in HIV
296.1.0.1.5.4 Chagas Disease (Trypanosoma cruzi)
  • Latin America endemic
  • Late: cardiomyopathy + apical aneurysm
  • Conduction (RBBB common, AV block)
  • Heart failure
  • Sudden death
  • Treatment: benznidazole / nifurtimox (acute); HF therapy + ICD (chronic)
296.1.0.1.5.5 Lyme Disease (Borrelia burgdorferi)
  • Acute: myopericarditis with AV block (most common)
  • Late: rare cardiomyopathy
  • Treatment: doxycycline, ceftriaxone if severe
296.1.0.1.5.6 Endocarditis (Ch297-298)
  • Infective endocarditis with cardiac manifestations
296.1.0.1.6 Hematologic
296.1.0.1.6.1 Sickle Cell Disease
  • Cardiomyopathy (HFpEF/HFrEF features)
  • Pulmonary HTN (group 5)
  • MI (vaso-occlusive)
  • TR + LVH common
  • Treatment: hydroxyurea, transfusion, iron chelation if needed, HF therapy
296.1.0.1.6.2 Thalassemia
  • Iron overload cardiomyopathy (transfusion-dependent)
  • CMR T2* for cardiac iron
  • Treatment: chelation (deferasirox, deferiprone, deferoxamine)
296.1.0.1.6.3 Myeloproliferative Neoplasms (MPN)
  • JAK2-positive: thrombosis risk (CV + venous + arterial)
  • ↑ Hyperviscosity
  • Treatment: aspirin + cytoreduction (hydroxyurea, anagrelide)
296.1.0.1.6.4 Iron Deficiency Anemia
  • High-output HF (rare, severe)
  • Treatment: iron supplementation; CONFIRM-HF: IV iron in HFrEF + iron deficiency
296.1.0.1.7 Genetic / Neuromuscular
296.1.0.1.7.1 Marfan, Loeys-Dietz, vEDS (Ch280)
296.1.0.1.7.2 Friedreich’s Ataxia
  • GAA repeat in FXN gene
  • HCM phenotype (concentric)
  • LV dysfunction → HFrEF
  • Treatment: idebenone (debated), HF therapy
296.1.0.1.7.3 Duchenne Muscular Dystrophy
  • X-linked dystrophin mutation
  • Dilated cardiomyopathy (HFrEF)
  • Diagnosis in adolescence
  • Treatment: ACEi early; β-blocker
  • Cardiac MRI early
  • Trends: emerging gene therapy (Casimersen, Vyondys 53, Vyvgart, etc.)
296.1.0.1.7.4 Myotonic Dystrophy
  • DMPK CTG repeat (DM1)
  • Conduction abnormalities (AV block, common reason for SCD)
  • Cardiomyopathy
  • Pacemaker / ICD frequently needed
296.1.0.1.7.5 Becker Muscular Dystrophy
  • Milder DMD allelic
  • DCM also common
296.1.0.1.7.6 Emery-Dreifuss Muscular Dystrophy
  • LMNA or emerin mutation
  • DCM + AV block
  • ICD primary prevention often indicated
296.1.0.1.8 COVID-19 — Long-Term
296.1.0.1.8.1 Acute Cardiac
  • Myocarditis (uncommon but recognized)
  • Arrhythmia
  • Acute coronary syndromes (cytokine storm, hypercoagulable)
  • HF decompensation
296.1.0.1.8.2 Post-Acute Sequelae (PASC, “Long COVID”)
  • Persistent dyspnea
  • POTS-like syndrome
  • Microvascular dysfunction
  • ↑ Future CV risk (some studies)

296.1.0.2 🩺 床邊速查

  • SLE: pericarditis common, Libman-Sacks; premature CAD
  • Scleroderma: PAH + myocardial fibrosis; annual DETECT screening
  • Vasculitis: Takayasu (coronary ostia, aortitis), Churg-Strauss (myocarditis)
  • Amyloid: AL (daratumumab + CyBorD), ATTR-wild type (tafamidis), PYP scan
  • Sarcoid: AV block + HFrEF; steroid + immunosuppressant + ICD
  • Hemochromatosis: iron overload DCM; phlebotomy + chelation
  • Fabry: HCM phenotype + renal/neuropathy; ERT or migalastat
  • Friedreich, Duchenne, Myotonic, Emery-Dreifuss: each has specific cardiac phenotype