Chagas Cardiomyopathy: A First for Ireland (QF_SA_124)

A 52-year-old Brazilian woman presented to our institution with a 4-hour history of transient expressive dysphasia. She also described several weeks of NYHA II dyspnoea. She was a smoker with a history of dyslipidaemia. Clinical findings were consistent with hypervolemia, serum pro-BNP was elevated 935ng/L and her chest XR demonstrated venous congestion. Serial ECG showed non-specific TWI and inferior Q waves. CT Brain confirmed an established temporal lobe infarction. MRI Brain demonstrated multifocal infarcts suggesting a cardioembolic phenomenon.
Trans-thoracic echocardiography revealed biventricular failure (LVEF 15%) and a large apical thrombus, without apical aneurysmal dilatation and moderate mitral regurgitation. Cardiac MRI, demonstrated myocardial inflammation with 50% antero-apical sub-endomyocardial fibrosis, and late gadolinium enhancement consistent with Chagas cardiomyopathy (CM). Ischemic cardiomyopathy remained within the differential until invasive coronary angiography demonstrated mild, non-obstructive coronary artery disease. Serological testing confirmed the diagnosis. Chagas CM has not been documented for an Irish patient, as far as we are aware.
Our busy primary PCI centre serves a population of over 380,000 people but without access to CMRI on site. Further funding and provision of CMRI is needed to allow quicker and more effective diagnosis. Our patient now remains well with NYHA II dyspnoea and has been appropriately titrated on guideline directed medical therapy (GDMT) with Entresto, Dapagliflozin, Eplerenone and Bisoprolol.

Diagnostic Techniques and Their Most Important Findings:

Echocardiography demonstrated severe biventricular failure, moderate mitral regurgitation with a large apical thrombus in the left ventricle. Coronary angiography showed mild coronary arterial disease.
This was a challenging case as there was inconsistency between echocardiographic and the angiographic findings and the CMR findings were not the typical subepicardial distribution routinely seen in myocarditis. Following the discrepancy between the CMR and angiographic findings, Chagas serology was completed which ultimately confirmed Chagas CM. Although there is little evidence for treating the underlying Chagas infection in adults, it does change management regarding consideration for device therapy as patients often develop potentially life threatening brady and tachyarrhythmias. The recommended treatment is similar for those with biventricular dysfunction and typically, they may remain euvolemic with good functional capacity on GDMT.  However, these patients require closer follow up as there is little evidence regarding response to GDMT and overall prognosis can be quite poor.
Notably, our centre is unable to provide cardiac MRI, and despite being a primary PCI centre and a university teaching hospital we routinely refer patients for CMRI in a centre which is180Km away (360Km round trip).

Learning Points from this Case:

This case highlights the importance and usefulness of CMR in the diagnosis of Chagas CM. However, Chagas CM is typically a disease of poorer countries which often do not have access to CMR creating further potential barriers to rapid detection. As confirmation of Chagas CM changes the general management of the condition particularly from a device and surveillance perspective, it highlights how lack of access to expensive imaging modalities such as CMR may disadvantage this patient cohort further.