Syncopal episodes unveiling biventricular arrhythmogenic cardiomyopathy: A case report in a Filipino patient
Tóm tắt
Background
Inherited cardiac disorders are important causes of arrhythmias, congenital heart diseases, hypertension and cadiomyopathies; however this is underreported and underrecognized in many regions in Asia. We report a case of a young female diagnosed with a rare form of cardiomyopathy after presenting with recurrent syncopal attacks
Case
The patient is a 31 year old, Filipino female, a chronic smoker and has no recorded co-morbid illnesses. She presented to the emergency department due to second episode of syncopal attack within 2 months, followed by brief episodes of seizure preceded by premonitory symptom of palpitation. ECG showed T wave inversions on precordial leads and right axis deviation with negative troponin I and 24 hour Holter. Echocardiogram, showed RA and RV dilatation with signs of high probability of pulmonary hypertension. CT pulmonary angiogram was negative for pulmonary thromboembolism or interstitial lung disease . Cardiac MRI was done with noted RV dilatation with microaneurysms, dyskinesia and signs of pressure overload. There was late gadolinium enhancement not attributable to ischemic mediated injury on post intravenous gadolinium contrast, indicative of fibrofatty replacement. The same late gadolinium enhancement was seen in the left ventricle. Hence patient fulfilled the Padua criteria and diagnosis of biventricular arrythmogenic cardiomyopathy was made.
Conclusion
Arrhythmogenic cardiomyopathy is an inherited cardiac muscle disease that poses risk for fatal arrhythmia and sudden cardiac death. There are very few available reported cases of ACM among Asians decent, this maybe attributed to underdiagnosis or misdiagnosis of common clinical manifestations, or unavailability of conventional cardiac diagnostics especially in rural areas of low income Asian countries. A high index of suspicion should raise the possibility of heritable diseases in young patients.
