Heart failure overview & cardiomyopathies

HF is a clinical diagnosis (congestion + a structural/functional ventricular problem). Cardiomyopathy is an imaging/structural label and does not require volume overload.

Frederick Lang5 min6 questions7 trials

What you are diagnosing

Heart failure is a clinical diagnosis: signs and symptoms of congestion (orthopnea, edema, crackles, ↑ JVP) attributable to a structural or functional problem with ventricular ejection or filling (). Cardiomyopathy is an imaging/structural label — abnormal myocardium — and does not require volume overload. Heart failure is the final common pathway of every cardiomyopathy.

Name the syndrome, then the ventricle

HF
Clinical syndrome
Congestion plus a ventricular problem. Treat the congestion and the remodeling — not the echo label alone.
CM
Myocardial disease
Dilated, hypertrophic, or restrictive on imaging. Can be silent. Work up the cause even when the patient is euvolemic.
EF
LVEF class
HFrEF ≤40%. HFmrEF 41–49%. HFpEF ≥50%. HFimpEF = prior ≤40% now >40%. GDMT follows the class, not the admission weight.

ACC/AHA stage, then NYHA class

A
At risk
HTN, CAD, DM, obesity, cardiotoxins, family history. No structural disease, no symptoms. Prevent.
B
Pre-HF
Structural or functional disease (low EF, LVH, valve, raised natriuretic peptide) without symptoms.
C
Symptomatic HF
Current or prior congestion. This is where GDMT, devices, and most admissions live.
D
Advanced HF
Rest symptoms, recurrent hospitalizations, inotropes, MCS, transplant. NYHA IV until proven otherwise.

NYHA is functional class inside stages C/D: I none, II ordinary activity, III less-than-ordinary, IV at rest. Stage never goes backwards; NYHA can.

Etiologies

Morphology on imaging is a separate axis: dilated, hypertrophic, or restrictive.

Category	Think
Most common	Ischemic, valvular, cardiometabolic (obesity, HTN, DM, HLD)
Metabolic	Nutritional deficiency (beriberi)
High-output	Sepsis, peripartum, hyperthyroid, anemia, liver failure, Paget’s, AV shunts
Toxins / drugs	Alcohol, cocaine / amphetamines, chemotherapy, immune checkpoint inhibitors
Infectious	HIV, coxsackie, echovirus, EBV, CMV, Lyme, Chagas, tuberculosis
Infiltrative	Amyloid (ATTR or AL), hemochromatosis, sarcoidosis, Löffler’s
Genetic	HCM / HOCM, ARVC, familial neuromuscular disease, glycogen storage, LV noncompaction
Inflammatory	Connective-tissue disease, giant-cell myocarditis
Other	Cor pulmonale, Takotsubo, tachyarrhythmia-induced, constriction / effusion, radiation, carcinoid, idiopathic

Workup

Everyone: exam, troponin, NT-proBNP, ECG, telemetry, CXR, TTE.
Ischemic lane: lipids, A1c, stress ± nuclear perfusion, coronary CTA, angiogram.
Non-ischemic lane: family history, meds, substances, tox screen, β-hCG, iron studies, SPEP / sFLC, TSH, HIV, ANA.
Extended, when the first pass fails: chest CT, cardiac MRI, right-heart catheterization, endomyocardial biopsy, PYP scan, directed infectious and metabolic serologies.

Management skeleton

Treat the cardiomyopathy and the acute trigger.
Diurese to decongest and drop preload.
Start GDMT to stop remodeling: HFrEF / HFmrEF / HFimpEF = ARNi (or ACE / ARB) + evidence-based β-blocker + MRA + SGLT2i. HFpEF = MRA + SGLT2i (± GLP-1 RA if obese).
Ask about coronaries, valves, ICD / CRT, inotropes, MCS, and transplant before discharge — not at the third readmission.

Select cardiomyopathies

Hypertrophic cardiomyopathy

LV / RV hypertrophy ± dynamic LVOT obstruction (HOCM), high SCD risk, a sarcomere mutation in ~70% ().

Exam: SEM at LLSB / apex that gets louder with Valsalva; paradoxical S2 split; S4.
ECG: giant precordial T inversions (apical HCM), high voltage, inferior / lateral Q waves.
TTE: wall ≥15 mm, SAM of the mitral valve, gradient that worsens with Valsalva.
Disease-specific: aficamten, mavacamten. Refractory obstruction → septal ablation or surgical myectomy.
Rhythm: disopyramide, β-blocker > verapamil. ICD if prior VT / SCD / unexplained syncope, SCD in a first-degree relative, LVH >30 mm, NSVT on Holter, abnormal BP response to exercise, heavy LGE on cMRI, or EF <50%.
Send genetics; consider sports restriction. Volume depletion worsens obstruction.

Cardiac amyloid (AL, ATTR)

Clues: bilateral carpal tunnel, lumbar stenosis, autonomic failure, neuropathy, AV block, low-voltage QRS discordant with LVH on TTE, stubborn low-level troponin, high ATTR-CM score.
TTE: walls >12 mm, apical-sparing strain. cMRI: ↑ extracellular volume, diffuse LGE.
ATTR: tafamidis or acoramidis (TTR stabilizers), vutrisiran (TTR silencer).
AL: chemotherapy (Dara-CyBorD), transplant when indicated.
Narrow euvolemia window. β-blockers and ARNi are often poorly tolerated; SGLT2i and MRA may still help (; ).

Hemochromatosis

Clues: known hemoglobinopathy, supraventricular arrhythmias, liver disease, arthritis, hypogonadism.
Diagnosis: cMRI with T2* to quantify iron.
Treatment: scheduled phlebotomy, iron chelators.

Sarcoidosis

Clues: AV block or a pseudoinfarct ECG (pathologic Q waves, no prior MI), unexplained VT storms, patchy basal / LV wall LGE on cMRI.
Treatment: prednisone, then steroid-sparing (azathioprine, MTX), anti-TNF agents.

Ischemic cardiomyopathy

Revascularize when there is left-main or multivessel disease, EF ≤35%, ACS, or angina that limits life.
PCI does not beat medical therapy in stable extensive CAD with low EF (REVIVED). CABG still has a long-term signal in selected patients.

Valvular disease

Pills rarely fix the lesion. Prioritize TAVR, transcatheter edge-to-edge repair (MitraClip), or surgery.
Severe AS in : phenylephrine, same logic as HOCM.