Abstract Number ‐ 235: Stroke Management in Left Ventricular Non‐Compaction Syndrome (LVNC): Report of 2 Cases

Introduction Left Ventricular Non‐Compaction syndrome is a rare congenital cardiomyopathy that results in prominent endomyocardial trabeculations which increases the risk of left ventricular dysfunction, malignant arrhythmias and thromboembolic events. Its diagnosis is based on morphological changes seen on trans‐thoracic echocardiograph (TTE), which can often be missed as it is user dependent. Objective: To discuss two cases of stroke associated with LVNC at our institution as well as offer further diagnostic and therapeutic recommendations. Methods Case Report Results Case1: A 29‐year‐old gentleman with a history dilated cardiomyopathy of unclear etiology and prior stroke presented to our institution complaining of dizziness and right sided headache. He was found to have a right M2 occlusion resulting in an insular infarct. Patient was out of the window for any chemical or mechanical thrombectomy. Initial TTE showed a depressed ejection fraction of 45‐50‐% with a 6mm LV apical thrombus. Patient was started on anticoagulation with coumadin and follow up TTE 3 months later showed the same EF, but with resolution of the apical thrombus. Given the unclear etiology of his heart failure, he was sent for cardiac MRI which demonstrated deep endomyocardial trabeculae consistent with Left Ventricular Non‐Compaction Syndrome. Given new findings, patient was recommended to continue anticoagulation with coumadin indefinitely. Case 2: A 12‐year old boy with a history of Cerebral Palsy and cardiomyopathy due to a formal diagnosis of Left Ventricular Non‐Compaction syndrome shortly after birth presented with sudden right sided weakness. Patient was admitted to the PICU team for elevated level of care. He underwent an MRI of the brain and found to have a left MCA ischemic stroke. He was out of the window for any mechanical or chemical thrombectomy. He was started on anticoagulation after 4 days with a stable CT scan of the stroke. Conclusions In the first case, several TTE’s in the outpatient setting and two done while the patient was hospitalized did not identify the pathogenic trabeculae. The diagnosis was only made after a cardiac MRI was done, posing a delay in diagnosis and possible treatment that could have prevented thrombogenic environment in the patient’s LV leading to an acute stroke. In the second case, the patient did have a diagnosis of LVNC, but anticoagulation was never started. Currently, there there exists no current accepted guidelines for diagnoses and management of LVNC. We recommend a cardiac MRI if there is a concern for stroke in a patient with cardiac myopathy of unclear etiology. Furthermore, professional opinion based on several meta‐analysis suggests that anticoagulation should be started when LVEF is less than 40% or presence of a prior stroke in patient with LVNC. Given the rarity of this syndrome and the challenge with its diagnosis, further studies are needed to better evaluate its timely diagnosis and proper management.