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Fatal Cardiac Hemochromatosis in a Patient with Hereditary Spherocytosis
- Source :
- International Heart Journal. 59:427-430
- Publication Year :
- 2018
- Publisher :
- International Heart Journal (Japanese Heart Journal), 2018.
-
Abstract
- A 31-year-old man was admitted to our hospital with atrial tachycardia and cardiogenic shock. He had been diagnosed with hereditary spherocytosis (HS) during childhood, but he never received any red blood cell transfusions. Right ventricular endomyocardial biopsy revealed multiple myocardial hemosiderin deposits, and he was diagnosed with cardiac hemochromatosis. In addition to the iron deposition in the heart, the loss of myocyte and severe interstitial fibrosis were present. His cardiac function did not improve even after the cardioversion for atrial tachycardia, and he suffered from recurrent heart failure. Despite intensive medical treatment for heart failure and arrhythmias in combination with iron chelation therapy, he eventually died of progressive and refractory heart failure. Hemochromatosis is a systemic disorder characterized by the excessive deposition of iron in multiple organs. The occurrence of hemochromatosis in HS is extremely rare, and previous reports have shown that the coexistence of heterozygosity for the HFE gene mutation in HS patients causes excess iron storage. The prognosis is poor due to progressive congestive heart failure and refractory arrhythmias. Here we report a rare case of fatal cardiac hemochromatosis associated with HS. The possibility of cardiac hemochromatosis needs to be considered in cases of heart failure or arrhythmia in patients with HS.
- Subjects :
- Adult
Male
Cardiac function curve
medicine.medical_specialty
medicine.medical_treatment
Spherocytosis, Hereditary
030204 cardiovascular system & hematology
Cardioversion
Hereditary spherocytosis
03 medical and health sciences
Fatal Outcome
0302 clinical medicine
Internal medicine
medicine
Humans
Atrial tachycardia
Hemochromatosis
Heart Failure
business.industry
Cardiogenic shock
General Medicine
medicine.disease
030220 oncology & carcinogenesis
Heart failure
Hemosiderin
cardiovascular system
Cardiology
medicine.symptom
Cardiology and Cardiovascular Medicine
business
Subjects
Details
- ISSN :
- 13493299 and 13492365
- Volume :
- 59
- Database :
- OpenAIRE
- Journal :
- International Heart Journal
- Accession number :
- edsair.doi.dedup.....0948a96f7f1cc927f9b24e7075ca1897