Ischemic Stroke as the First Sign of Decompensated LVNC

A 61-year-old male with a known diagnosis of left ventricular non-compaction (LVNC) cardiomyopathy — established five years earlier by cardiac MRI — presented to the emergency department reporting two days of decreased left-sided peripheral vision. He had been exercising when the visual disturbance began. He had no history of chest pain, dyspnea, or fluid retention, and no prior cardiovascular events. His most recent MUGA scan, performed 10 months earlier, had shown a left ventricular ejection fraction (LVEF) of 55% with no intracardiac thrombus.

On examination, vital signs were within normal limits. Neurological testing revealed left homonymous hemianopia with no other cranial nerve, motor, or sensory deficits. Routine labs — CBC and comprehensive metabolic panel — were unremarkable. The ECG showed normal sinus rhythm with left axis deviation and left bundle branch block (LBBB), suggestive of left ventricular hypertrophy.

Figure 1

ECG: Normal Sinus Rhythm with Left Axis Deviation and LBBB

The LBBB pattern on presentation is a key clue to underlying structural disease. In LVNC, conduction abnormalities arise because the prominent trabeculations are directly involved in developing the Purkinje system, predisposing patients to a spectrum of arrhythmias and conduction delays. Note the broad, notched QRS morphology in the lateral leads.

CT head without contrast showed an area of low attenuation with loss of gray-white differentiation in the right occipital lobe, consistent with acute to subacute infarction. Brain MRI confirmed marked hyperintensity with vasogenic edema in the right occipital and right posterior temporal regions, with imaging appearance consistent with acute embolic ischemic stroke.

Figure 2

Diffusion-Weighted MRI: Right Occipital and Posterior Temporal Infarction

Increased signal intensity in the right occipital and right posterior temporal region on DWI confirms restricted diffusion consistent with acute ischemic stroke. The posterior distribution involving both the temporal and occipital lobes localizes to the posterior cerebral artery territory, strongly suggesting a cardioembolic rather than large-vessel atherosclerotic mechanism, given the multi-lobar involvement.

Figure 3

T2-Weighted MRI: Vasogenic Edema in Right Occipital and Posterior Temporal Lobes

T2 hyperintensity with surrounding vasogenic edema extends beyond the core DWI lesion, reflecting peri-infarct injury. The combination of DWI and T2 signal changes in complementary vascular distributions supports the embolic etiology and helps gauge the age of the infarct — findings clinically relevant to anticoagulation timing decisions.

A transesophageal echocardiogram (TEE) obtained as part of the stroke workup revealed an ejection fraction of only 28% — a dramatic decline from 55% just 10 months earlier — with multiple apical LV trabeculations consistent with LVNC and no intracardiac thrombus identified. The patient had never shown atrial fibrillation on prior rhythm monitoring, and he denied any symptoms of heart failure.

He was started on goal-directed medical therapy for HFrEF (carvedilol and sacubitril-valsartan) and initiated on apixaban monotherapy for anticoagulation, with aspirin discontinued. A regadenoson stress test showed no reversible ischemia. On follow-up two months later, repeat MUGA scan demonstrated LVEF improvement to 41%, and his visual field deficits fully resolved.

Discussion

This case illustrates a critical surveillance gap inherent to LVNC: ejection fraction can deteriorate rapidly and asymptomatically, leaving patients vulnerable to cardioembolic events before clinical heart failure develops. The 27-point LVEF decline in 10 months — from a preserved 55% to a reduced 28% — occurred without dyspnea, edema, or fatigue. In LVNC, the spongy, trabeculated myocardium creates intertrabecular recesses that can harbor sluggish flow and predispose to thrombus formation even in the absence of an identified clot on imaging. The current consensus supports therapeutic anticoagulation when LVEF falls below 40%, when atrial fibrillation develops, or after a thromboembolic event — in this patient, the stroke triggered anticoagulation that the silent EF decline alone had not prompted.

The neurological presentation also offers a clinical teaching point: posterior circulation embolic stroke causing homonymous hemianopia without other focal deficits can be initially misattributed to a benign visual complaint. When LVNC is a known diagnosis, any new neurological symptom should prompt urgent cardiac imaging to reassess EF and evaluate for intracardiac thrombus, even in patients who are regularly followed. This case supports the argument that annual LVEF surveillance intervals may be insufficient for LVNC patients who are clinically silent — a more dynamic approach, including symptom-guided echocardiography, should be considered for this population.