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Quick Fire 3- Miscellaneous III

SECONDARY HEMOCHROMATOSIS IN A PATIENT WITH SUPRAVENTRICULAR TACHYCARDIA AND NEW DECOMPENSATED HEART FAILURE (QF_FR_087)

Friday, January 31, 2025
8:20 AM – 8:30 AM East Coast USA Time
Location: Blue Room PreFunction
Description of Clinical Presentation: A 26-year-old male with a history of sickle cell disease, acute chest syndrome, prior stroke, and recurrent blood transfusions presented with worsening lower extremity edema, abdominal pain, and an eighteen-pound weight gain over one week. On admission, vitals were notable for tachycardia with a heart rate up to 130s. Physical examination revealed jugular venous distension, abdominal distension, bibasilar crackles, and 2+ bilateral lower extremity edema. Labs were notable for elevated BNP (4,072 pg/mL), elevated ferritin (9,828 ng/mL), and anemia (Hgb 5.7 g/dL). ECG demonstrated supraventricular tachycardia at a rate of 130 bpm.

Diagnostic Techniques and Their Most Important Findings: Transthoracic echocardiography (TTE) demonstrated a left ventricular ejection fraction (EF) by 3D of 68%, left ventricular end-diastolic diameter of 5.3 cm, septal and posterior wall thickness of 0.9 cm, mildly dilated left atrium (38 mL/m2) and normal right ventricular cavity size and systolic function. Cardiac magnetic resonance (CMR) imaging was ordered for evaluation of heart failure with preserved EF and showed mildly depressed left ventricular systolic function (LVEF of 55%), mildly enlarged left ventricular size (indexed LVEDV 111 mL/m2), severely enlarged right ventricular size (indexed RVEDV 154 mL/m2) and moderately depressed right ventricular systolic function (EF of 39%). The left atrium was mildly enlarged and the right atrium was moderately enlarged. Post-contrast images demonstrated sub-epicardial late gadolinium enhancement in the basal to apical inferior, inferolateral, and anterolateral segments, in a non-ischemic pattern (Figure 1). Extracellular volume fraction was elevated at 37%, global native T2 time was low at 30 +/- 5 ms, and the liver was noted to be hypointense (Figure 2). Therefore, subsequent T2* imaging was obtained, which revealed a reduced relaxation time of 12.6 ms (Figure 3), consistent with marked myocardial iron overload (< 20 ms).

Learning Points from this Case: Hemochromatosis can lead to multiple cardiac complications including heart failure, dilated cardiomyopathy, restrictive cardiomyopathy, and diastolic dysfunction. It can often be difficult to distinguish from myocarditis. Fortunately, CMR has become a valuable tool in identifying iron overload. For this case, in the setting of a normal TTE, CMR was imperative for the diagnosis of cardiomyopathy and myocardial iron overload given its accurate quantification of chamber size, function, and iron deposition. In patients with severe myocardial iron overload, fibrosis can eventually develop and be detected using late gadolinium enhancement (LGE) and extracellular volume quantification. The mechanism of LGE in cardiac iron overload involves the accumulation of iron within cardiomyocytes, leading to cellular damage and replacement fibrosis. As the extracellular space expands due to fibrosis, there is increased retention of gadolinium contrast in these areas, resulting in hyperenhancement on LGE imaging. However, it is important to note that LGE may not be present in early stages of iron overload cardiomyopathy, and T2* mapping remains the gold standard for detecting and quantifying myocardial iron deposition.