Patient handout

Restrictive cardiomyopathy (chronic — etiology hunt + RCM-vs-CP pivot)

PRODUCTION

1. Your condition

This handout is for restrictive cardiomyopathy (chronic — etiology hunt + rcm-vs-cp pivot). Your care team identified this based on: echo: restrictive filling + biatrial enlargement + non-dilated ventricles + normal ef.

Other reasons your team may use this plan: right-heart congestion (edema, ascites, elevated jvp) with preserved ef; exertional dyspnea / fatigue out of proportion to ef; family history of rcm / known storage or infiltrative disease.

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
pericardiectomy referral (if constrictive pericarditis)	—	—	—	CP is potentially curable by pericardiectomy — must not be mislabeled RCM (2023 ESC Cardiomyopathy)

Plan: RCM etiology routing + cautious symptomatic HF (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)

3. When to call your provider

Contact your care team if any of the following happen:

Constrictive pericarditis identified → surgical pericardiectomy — 2023 ESC Cardiomyopathy
End-stage idiopathic/familial RCM → transplant — 2023 ESC Cardiomyopathy
Decompensation → cautious acute pathway — 2022 ACC/AHA HF

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Hemodynamics/imaging indicate constrictive pericarditis rather than RCM — pericardiectomy is potentially CURATIVE; do not mislabel RCM — 2023 ESC Cardiomyopathy
Amyloid as the RCM etiology (FLC/PYP) — route to ATTR or AL engine for disease-modifying therapy — Gillmore Circulation 2016
Fabry disease (low α-Gal in males / GLA variant) — enzyme replacement / migalastat; route metabolic pathway — 2023 ESC Cardiomyopathy
Iron-overload cardiomyopathy (high ferritin/TSAT, low cardiac T2*) — phlebotomy/chelation; route iron-overload pathway — 2023 ESC Cardiomyopathy
Loeffler endomyocardial disease / hypereosinophilic syndrome — corticosteroids ± imatinib (FIP1L1-PDGFRA); anticoagulate for endomyocardial thrombus — 2023 ESC Cardiomyopathy
Idiopathic/familial (sarcomeric/desmin/TNNI3) RCM — poor prognosis; genetics + transplant evaluation — 2023 ESC Cardiomyopathy
AF with marked biatrial enlargement — high intracardiac-thrombus/embolic risk → anticoagulate; gentle rate control preserving filling time — ESC 2024 AF
Pregnancy with RCM — restrictive physiology poorly tolerates volume shifts; high maternal risk; cardio-obstetric, careful diuresis, avoid ACEi/ARB — ESC 2018 Pregnancy

5. Follow-up

Family cascade if familial; etiology-specific long-term care

6. Sources

Guideline: 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; RCM-vs-constrictive-pericarditis hemodynamic literature