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Patient handout

Catecholaminergic polymorphic VT (CPVT, chronic)

PRODUCTION

1. Your condition

This handout is for catecholaminergic polymorphic vt (cpvt, chronic). Your care team identified this based on: exertional / emotional syncope with structurally normal heart.

Other reasons your team may use this plan: bidirectional / polymorphic vt on exercise stress test; aborted scd in a young patient with normal heart/qt; family history of cpvt / exertional young scd.

2. Your medications

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

MedicationStarting doseHowWhenWhat it does
nadolol1–2 mg/kg/day (typical adult 40–80 mg)POonce–BID2022 ESC VA — nadolol is the preferred BB in CPVT (best exercise-VT suppression); lifelong, including gene-positive
propranolol2–3 mg/kg/dayPOTIDPropranolol alternative non-selective BB (2017 AHA/ACC/HRS VA)

Plan: CPVT arrhythmia-suppression (2013 HRS/EHRA/APHRS; 2017 AHA/ACC/HRS VA; 2022 ESC VA)

3. When to call your provider

Contact your care team if any of the following happen:

  • VT storm / arrest → ED + acute CPVT pathway — 2022 ESC VA
  • Breakthrough on BB+flecainide → LCSD — 2022 ESC VA
  • ICD shock storm → intensify BB/flecainide/LCSD (NOT just more shocks) — 2022 ESC VA

4. When to seek emergency care

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

  • Aborted SCD / sustained VT — secondary-prevention ICD but ALWAYS combined with BB+flecainide±LCSD (ICD shocks pro-arrhythmic in CPVT) — 2022 ESC VA(life-threatening)
  • Events / residual exercise VT despite adequate BB + flecainide — LCSD — 2022 ESC VA
  • ICD shock storm in CPVT — shocks are pro-arrhythmic (catecholamine surge); intensify BB/flecainide + sedation + LCSD, NOT more shocks alone — 2022 ESC VA(life-threatening)
  • Competitive/intense exercise exposure — primary catecholamine trigger; restriction (shared-decision recreational) — 2022 ESC VA
  • Pediatric CPVT — common presentation; weight-based nadolol/flecainide, school AED + emergency action plan — 2013 consensus
  • Pregnancy with CPVT — continue nadolol (± flecainide), peripartum catecholamine-surge vigilance; cardio-obstetric — ESC 2018 Pregnancy

5. Follow-up

First-degree family cascade (exercise test + genetics); lifelong BB + exercise counseling

6. Sources

Guideline: 2013 HRS/EHRA/APHRS Inherited Arrhythmia Expert Consensus + 2017 AHA/ACC/HRS VA Guideline + 2022 ESC Ventricular Arrhythmia Guideline

  1. pubmed.ncbi.nlm.nih.gov/23994779
  2. pubmed.ncbi.nlm.nih.gov/29084731
  3. pubmed.ncbi.nlm.nih.gov/36017572