Patient handout

Genetic / familial dilated cardiomyopathy (LMNA/FLNC/TTN/RBM20/DSP, chronic)

PRODUCTION

1. Your condition

This handout is for genetic / familial dilated cardiomyopathy (lmna/flnc/ttn/rbm20/dsp, chronic). Your care team identified this based on: echo: dilated lv + systolic dysfunction (non-ischemic).

Other reasons your team may use this plan: family history of dcm / unexplained scd / known pathogenic variant; av block / conduction disease with dcm (lmna flag); ventricular arrhythmia / palpitations with dcm.

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
sacubitril/valsartan	24/26→97/103 mg	PO	BID	Standard HFrEF GDMT applies — TTN-truncating DCM is particularly GDMT-responsive (PARADIGM-HF; 2022 ACC/AHA HF)
carvedilol	3.125→25 mg	PO	BID	Evidence-based BB pillar (2022 ACC/AHA HF)
spironolactone	12.5–25 mg	PO	once daily	MRA pillar (2022 ACC/AHA HF)
dapagliflozin	10 mg	PO	once daily	SGLT2i pillar (2022 ACC/AHA HF)

Plan: Genetic DCM — HFrEF GDMT + genotype-specific ICD (2022 AHA/ACC/HFSA; 2023 ESC Cardiomyopathy)

3. When to call your provider

Contact your care team if any of the following happen:

High-grade AV block in LMNA → ICD-capable device (not PPM-only) — 2023 ESC Cardiomyopathy
Sustained VT/VF → EP + ICD — 2023 ESC Cardiomyopathy
Progressive end-stage (esp. LMNA) → transplant/LVAD — 2023 ESC Cardiomyopathy

4. When to seek emergency care

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

LMNA cardiomyopathy — conduction disease + malignant VT often before LV dysfunction; ICD by LMNA risk model even at EF >35; if pacing indicated use ICD-capable device — Wahbi JACC 2019(life-threatening)
FLNC-truncating / DSP / RBM20 / PLN / TMEM43 — arrhythmic DCM, high LGE/SCD; low ICD threshold; DSP "hot phases" mimic myocarditis — 2023 ESC Cardiomyopathy
Recovered EF on the four foundational heart-failure medications in genetic DCM — do NOT withdraw therapy (TRED-HF relapse, genetic substrate persists) → route HF-improved engine — TRED-HF Lancet 2019
Pregnancy with genetic DCM — peripartum unmasking/decompensation; STOP ACEi/ARB/ARNi/SGLT2i → BB ± hydralazine; LMNA arrhythmic risk; cardio-obstetric — ESC 2018 Pregnancy
End-stage genetic DCM (LMNA often progresses despite the four foundational heart-failure medications) — transplant / durable LVAD evaluation — 2023 ESC Cardiomyopathy(life-threatening)

5. Follow-up

First-degree family cascade screening + serial evaluation of gene-positive relatives; lifelong genotype-specific care

6. Sources

Guideline: 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; Wahbi LMNA risk model; TRED-HF