Heart's hidden storm

Description of Clinical Presentation: 53-year-old lady, diagnosed case of Crohn’s disease underwent intestinal segmental surgical resection with stoma. During her postoperative recovery in the ICU, she had severe chest pain and cardiogenic shock. ECG was suggestive of STEMI and elevated serum troponins. Hence she underwent urgent catheter angiography. Catheter angiography was normal with no evidence of obstructive disease. Further workup was performed with cardiac MRI.

Diagnostic Techniques and Their Most Important Findings: CMR revealed severely reduced LV function (LVEF 27%, LV GLS -11.6%). No evidence of regional wall motion abnormality was seen. Patchy areas of Myocardial oedema were seen with regional increase in T1 and T2 mapping values. Late gadolinium enhancement revealed ring like mid myocardial late gadolinium enhancement involving septum and inferior wall. It was predominantly mid myocardial in septum and epicardial along inferior wall. These features suggested genetic cause of cardiomyopathy and hence further workup with genetic testing was performed which confirmed two genetic mutations.

Learning Points from this Case: Inflammatory bowel disease has immune mediated pathogenesis. Recent history of surgery aggravated underlying genetic abnormality and resulted in hot phase of genetic cardiomyopathy.It is important to be aware of such clinical presentations and treat this patient’s accordingly.