This handout is for acute hf — hemochromatosis / iron-overload cardiomyopathy. Your care team identified this based on: ferritin >1000 ng/ml + transferrin saturation >50% + new hf symptoms (dyspnea, edema, fatigue) → suspect iron-overload cardiomyopathy.
Other reasons your team may use this plan: known hfe c282y homozygote or compound heterozygote with new hf symptoms — likely cardiac iron deposition; cardiac mri t2* needed; chronic transfusion-dependent patient (β-thalassemia major, sickle cell, mds, aplastic anemia) with 20+ lifetime units + new hf — transfusional iron-overload cardiomyopathy; cardiac mri t2* <20 ms (especially <10 ms severe; <6 ms imminent hf risk) per anderson pmid 11713075 + new lv dysfunction → confirmed cardiac iron-overload cardiomyopathy.
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 |
|---|---|---|---|---|
| furosemide | 40-80 mg IV (diuretic-naive); 2.5x outpatient PO dose IV if on chronic loop (DOSE-trial guided) | IV | q12h titrate | DOSE PMID 21366472 — high-dose IV bolus or continuous infusion equivalent; titrate to UOP + symptom resolution; transition to PO before discharge |
| nitroglycerin | 5-20 µg/min IV titrate | IV | continuous | Preload + modest afterload reduction for hypertensive ADHF; AVOID if SBP <100 or RV-predominant restrictive phase |
| sacubitril_valsartan | 24/26 mg PO BID (titrate to 49/51 then 97/103 BID) | PO | BID | PIONEER-HF PMID 30403955 — in-hospital initiation; PARADIGM-HF — superior to ACEi for HFrEF; standard GDMT in iron-overload-related HFrEF; 36h washout from ACEi required |
| carvedilol | 3.125 mg PO BID titrate | PO | BID | CAPRICORN PMID 11356436 + COPERNICUS PMID 11386262 — beta-blocker mortality benefit in HFrEF; standard 4-pillar GDMT; safe in iron-overload cardiomyopathy |
| spironolactone | 12.5-25 mg PO daily | PO | daily | RALES PMID 10471456 — mortality benefit in HFrEF; monitor K + eGFR; renal dose-adjust; standard 4-pillar GDMT |
| dapagliflozin | 10 mg PO daily | PO | daily | DAPA-HF PMID 31535829 — mortality + HF hospitalization benefit; 4th pillar GDMT; safe in iron-overload cardiomyopathy; especially useful given DM comorbidity common in HH |
| empagliflozin | 10 mg PO daily | PO | daily | EMPULSE PMID 35347356 — in-hospital initiation safe + improves clinical benefit; alternative to dapagliflozin |
| deferasirox | 21 mg/kg PO daily (Jadenu) — start lower 14 mg/kg if frail or eGFR borderline | PO | daily | EHA-EBMT 2022 consensus PMID 35139194 — first-line oral chelator with excellent cardiac penetration; monitor eGFR (renal toxicity), LFTs (hepatotoxicity), CBC (cytopenias); dose-adjust per renal function |
| deferoxamine | 40-60 mg/kg/day SC infusion 8-12 h/day 5-7 d/week | SC | continuous infusion | Historic standard chelator; reserved for severe iron overload requiring maximal cardiac iron mobilization; combination with deferiprone for cardiac iron clearance per Tanner PMID 17389272 TIC trials; poor compliance due to infusion burden |
| deferiprone | 75-100 mg/kg/day PO TID | PO | TID | Pennell EHJ 2001 PMID 11713075 + 2006 PMID 16735649 — best cardiac iron clearance per T2* studies; risk of agranulocytosis (need WEEKLY CBC monitoring); often combined with deferoxamine for severe cardiac iron overload (T2* <6 ms) per Tanner PMID 17389272 TIC trials |
| warfarin | 5 mg PO daily INR target 2-3 | PO | daily | AC for AFib (very common in iron-overload — atrial iron deposition); AC for LV thrombus per AHA 2022 Class IIa; preferred over DOAC if cirrhosis with hepatic synthetic dysfunction |
| apixaban | 5 mg PO BID (or 2.5 mg BID per dose-reduction criteria) | PO | BID | ACC/AHA 2023 AFib PMID 38033089 — DOAC preferred over warfarin in most cases; AVOID if Child-Pugh C cirrhosis from iron-overload hepatopathy; ARISTOTLE PMID 21870978 |
Plan: Iron-overload cardiomyopathy ADHF — supportive ADHF + GDMT 4-pillar + PHLEBOTOMY (HH) or IRON CHELATION (transfusion-dependent or HH-intolerant); coordinate with hematology + hepatology + endocrinology (EASL 2022 PMID 36064151; AASLD 2011 PMID 21452290; EHA-EBMT 2022 PMID 35139194; Anderson PMID 11713075)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Cardiology follow-up at 1-2 wks + 3 mo + 6 mo + 12 mo; phlebotomy (HH) every 1-2 wks until ferritin <50, then maintenance 2-6/year; chelation (transfusion-dependent) lifelong with q3mo ferritin trend + annual T2* MRI; family genotype screening (siblings + first-degree relatives per EASL 2022); endocrinology follow-up (DM, hypogonadism, hypothyroidism); hepatology follow-up with annual US + AFP for HCC surveillance per AASLD 2011 (cirrhosis present); transplant evaluation if end-stage despite optimized the four foundational heart-failure medications + iron removal
Guideline: EASL 2022 hereditary hemochromatosis CPG (PMID 36064151) + AASLD 2011 hemochromatosis practice guideline (Bacon PMID 21452290) + EHA-EBMT 2022 iron chelation consensus (PMID 35139194) + Anderson EHJ 2001 cardiac MRI T2* gold standard (PMID 11713075) + 2022 ACC/AHA HF Guideline (PMID 35363499)