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ICU TopicsCardiovascular / cardiomyopathy

ICU · Cardiovascular / cardiomyopathy

Cardiomyopathies & Myocarditis

Also known as Cardiomyopathy · Dilated cardiomyopathy · Hypertrophic cardiomyopathy · Restrictive cardiomyopathy · Arrhythmogenic right ventricular cardiomyopathy · ARVC · Takotsubo cardiomyopathy · Stress cardiomyopathy · Broken heart syndrome · Myocarditis · Giant cell myocarditis · Eosinophilic myocarditis · Fulminant myocarditis · Cardiac amyloidosis · SAM · Systolic anterior motion · LVOT obstruction · Mavacamten · Tafamidis · Cardiac MRI late gadolinium enhancement · Lake Louise criteria · Dallas criteria · HCM Risk-SCD

The four cardiomyopathies are dilated (the commonest — an enlarged LV with a reduced EF, treated with heart-failure therapy), hypertrophic (asymmetric septal LVH with a dynamic LVOT obstruction and a risk of sudden cardiac death — treat with beta-blockers, avoid vasodilators, an ICD for prevention), restrictive (amyloid, sarcoid — stiff ventricles with impaired filling, treated for the cause), and arrhythmogenic (a genetic right-ventricular cardiomyopathy). Takotsubo is a transient stress-mediated apical ballooning that mimics an anterior STEMI. Myocarditis is an inflammation of the myocardium (most commonly viral — coxsackie, parvovirus, SARS-CoV-2), presenting with chest pain, arrhythmia, heart failure, and a rising troponin with normal coronary arteries. The cardiac MRI (mid-wall late gadolinium enhancement) and the biopsy (the Dallas criteria) confirm. Treatment is supportive; most recover, but giant-cell and a fulminant case may need immunosuppression, mechanical support or a transplant.

high20 referencesUpdated 26 July 202626 min readVerification in progress

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  • In HCM never give a vasodilator, a diuretic, or an inotrope — they worsen the dynamic LVOT obstruction; use a beta-blocker and a pure alpha-vasopressor (phenylephrine)
  • Myocarditis mimics an ACS — a rising troponin with normal coronaries; the MRI mid-wall LGE distinguishes it from the subendocardial LGE of an infarct; do not thrombolyse or stent a myocarditis
  • Giant cell myocarditis is universally fatal without immunosuppression (cyclosporine/steroids) and/or transplant — biopsy any fulminant case in a middle-aged adult
  • Fulminant myocarditis may need mechanical support (VA-ECMO, Impella) or a transplant as a bridge to recovery — and paradoxically has a BETTER long-term prognosis than non-fulminant acute myocarditis
  • Restriction vs constriction — the pericardium is the key: restriction has a normal pericardium and a thick infiltrated LV (treat medically); constriction has a thickened pericardium and a normal LV (pericardiectomy)
  • Takotsubo can cause LVOT obstruction, ventricular thrombus, free-wall rupture and Torsades — anticoagulate if apical thrombus, avoid inotropes if LVOT obstruction
  • Cardiac amyloidosis — never give ACE inhibitors/ARBs or calcium-channel blockers (the 'doubleGamma sign' of intolerance); avoid digoxin (binds amyloid fibrils)
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Practise this topic8 MCQs with explanations

Target exams

CICMFFICMEDIC

Red flags

  • In HCM never give a vasodilator, a diuretic, or an inotrope — they worsen the dynamic LVOT obstruction; use a beta-blocker and a pure alpha-vasopressor (phenylephrine)
  • Myocarditis mimics an ACS — a rising troponin with normal coronaries; the MRI mid-wall LGE distinguishes it from the subendocardial LGE of an infarct; do not thrombolyse or stent a myocarditis
  • Giant cell myocarditis is universally fatal without immunosuppression (cyclosporine/steroids) and/or transplant — biopsy any fulminant case in a middle-aged adult
  • Fulminant myocarditis may need mechanical support (VA-ECMO, Impella) or a transplant as a bridge to recovery — and paradoxically has a BETTER long-term prognosis than non-fulminant acute myocarditis
  • Restriction vs constriction — the pericardium is the key: restriction has a normal pericardium and a thick infiltrated LV (treat medically); constriction has a thickened pericardium and a normal LV (pericardiectomy)
  • Takotsubo can cause LVOT obstruction, ventricular thrombus, free-wall rupture and Torsades — anticoagulate if apical thrombus, avoid inotropes if LVOT obstruction
  • Cardiac amyloidosis — never give ACE inhibitors/ARBs or calcium-channel blockers (the 'doubleGamma sign' of intolerance); avoid digoxin (binds amyloid fibrils)

Overview & definition

The cardiomyopathies are diseases of the heart muscle, classified into four morphological types — dilated (the commonest), hypertrophic, restrictive, and arrhythmogenic right ventricular — to which Takotsubo (stress) cardiomyopathy is often added as a fifth, reversible, mimic. Myocarditis is an inflammation of the myocardium (most commonly viral) that can mimic an ACS and may progress to a dilated cardiomyopathy.[1]

The 2023 ESC cardiomyopathy guideline reframes these around a phenotype-first, genotype-driven approach: define the phenotype (dilated, hypertrophic, restrictive, non-compaction, ARVC) from imaging, then hunt systematically for the cause (genetic, acquired), and cascade-screen first-degree relatives.[1]

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References20ShowHide
  1. [1]Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies Eur Heart J, 2023.PMID 37622657
  2. [2]Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary Circulation, 2020.PMID 33215938
  3. [3]O'Mahony C, Jichi F, Pavlou M, et al. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD) Eur Heart J, 2014.PMID 24126876
  4. [4]Olivotto I, Oreziak A, Barriales-Villa R, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial Lancet, 2020.PMID 32871100
  5. [5]Maurer MS, Schwartz JH, Gundapaneni B, et al. Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy N Engl J Med, 2018.PMID 30145929
  6. [6]McDonagh TA, Metra M, Adamo M, et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure Eur Heart J, 2021.PMID 34447992
  7. [7]Templin C, Ghadri JR, Diekmann J, et al. Clinical Features and Outcomes of Takotsubo (Stress) Cardiomyopathy N Engl J Med, 2015.PMID 26332547
  8. [8]McCarthy RE 3rd, Boehmer JP, Hruban RH, et al. Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis N Engl J Med, 2000.PMID 10706898
  9. [9]Tschöpe C, Ammirati E, Bozkurt B, et al. Myocarditis and inflammatory cardiomyopathy: current evidence and future directions Nat Rev Cardiol, 2021.PMID 33046850
  10. [10]Ferreira VM, Schulz-Menger J, Holmvang G, et al. Cardiovascular Magnetic Resonance in Nonischemic Myocardial Inflammation: Expert Recommendations J Am Coll Cardiol, 2018.PMID 30545455
  11. [11]Kandolin R, Lehtonen J, Salmenkivi K, et al. Diagnosis, treatment, and outcome of giant-cell myocarditis in the era of combined immunosuppression Circ Heart Fail, 2013.PMID 23149495
  12. [12]Bauersachs J, König T, van der Meer P, et al. Pathophysiology, diagnosis and management of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy Eur J Heart Fail, 2019.PMID 31243866
  13. [13]Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy Heart Rhythm, 2019.PMID 31078652
  14. [14]Oster ME, Shay DK, Su JR, et al. Myocarditis Cases Reported After mRNA-Based COVID-19 Vaccination in the US From December 2020 to August 2021 JAMA, 2022.PMID 35076665
  15. [15]Sliwa K, Petrie MC, van der Meer P, et al. Clinical presentation, management, and 6-month outcomes in women with peripartum cardiomyopathy: an ESC EORP registry Eur Heart J, 2020.PMID 32840318
  16. [16]Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy Eur J Heart Fail, 2010.PMID 20675664
  17. [17]van der Meer P, van Essen BJ, Viljoen C, et al. Bromocriptine treatment and outcomes in peripartum cardiomyopathy: the EORP PPCM registry Eur Heart J, 2025.PMID 39221911
  18. [18]Prameswari HS, et al. Recurrence of Peripartum Cardiomyopathy in Subsequent Pregnancy Stratified by Left Ventricular Function: A Systematic Review and Meta-Analysis Eur J Heart Fail, 2026.PMID 41795865
  19. [19]Rigato I, Bauce B, Rampazzo A, et al. Compound and digenic heterozygosity predicts lifetime arrhythmic outcome and sudden cardiac death in desmosomal gene-related arrhythmogenic right ventricular cardiomyopathy Circ Cardiovasc Genet, 2013.PMID 24070718
  20. [20]Tan JL, Tan BE, Cheung JW, et al. Update on cardiac sarcoidosis Trends Cardiovasc Med, 2023.PMID 35504422

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