Talks
Presenting Author Academic/Professional Position
Resident
Academic Level (Author 1)
Resident
Discipline/Specialty (Author 1)
Internal Medicine
Academic Level (Author 2)
Faculty
Discipline/Specialty (Author 2)
Internal Medicine
Presentation Type
Oral Presentation
Discipline Track
Clinical Science
Abstract Type
Case Report
Abstract
Background: Left ventricular noncompaction (LVNC) is a rare structural abnormality of the left ventricular myocardium, characterized by prominent trabeculations and deep intertrabecular recesses due to incomplete compaction during embryogenesis. Clinical presentation is highly variable, ranging from asymptomatic cases to arrhythmias, thromboembolism, heart failure, and sudden cardiac death. Diagnosis is primarily imaging-based, with transthoracic echocardiography (TTE) using the Jenni criteria as standard. These criteria include excessive apical or midventricular trabeculations with a noncompacted-to-compacted ratio >2:1 at end-systole, a thin compacted epicardial layer (≤8.1 mm), and color Doppler flow within the recesses. When echocardiography is inconclusive, cardiovascular magnetic resonance (CMR) is recommended. LVNC may be familial, sporadic, acquired, or transiently associated with conditions such as myocarditis, amyloidosis, or systemic lupus erythematosus (SLE). We report a case of SLE-induced perimyocarditis presenting with cardiomyopathy resembling LVNC.
Case Presentation: A 44-year-old Hispanic male with no significant medical history presented with four weeks of dyspnea, lower extremity swelling, and pleuritic chest pain. Examination revealed bilateral basal crackles and 3+ pedal edema. Laboratory studies demonstrated severe anemia and thrombocytopenia, elevated ESR, CRP, and BNP of 1055. Baseline EKG was unremarkable, and high-sensitivity troponin was elevated without a dynamic rise, making acute coronary syndrome less likely. TTE showed an ejection fraction of 30%, global hypokinesia, and severe pericardial effusion, consistent with perimyocarditis. Additionally, 4–5 trabeculations with deep recesses were noted at the apex and posterolateral wall of the left ventricle, with blood flow into the intertrabecular recesses visualized on color Doppler. The non-compacted to compacted myocardium ratio at end-systole was >2:1, supporting a diagnosis of LVNC. Mild concentric left ventricular hypertrophy was also present, while heart valves and morphology were normal, with no congenital anomalies. Autoimmune testing revealed active SLE, after which high-dose prednisone (1 mg/kg/day) was initiated. Supportive treatment with intravenous diuretics and guideline-directed medical therapy for heart failure resulted in clinical improvement. The patient experienced no significant arrhythmias during hospitalization. Due to concern for evolving tamponade, he underwent a pericardial window procedure and recovered uneventfully, with discharge and planned cardiology and rheumatology follow-up.
Conclusion: LVNC, formerly called spongy myocardium or hypertrabeculation syndrome, is classified as an "unclassified" cardiomyopathy by the World Health Organization and diagnosed using the Jenni TTE criteria. The presence of noncompacted myocardium alone does not always indicate disease, as LVNC can represent a morphological variant rather than a pathological condition. This case highlights SLE-related myocarditis producing imaging findings that mimic LVNC through myocardial edema and wall thickening. Given its nonspecific presentation, LVNC should be considered in patients with prominent trabeculations on cardiac imaging, even if asymptomatic. Trabeculations may develop in adulthood, warranting monitoring and, when indicated, antiarrhythmic, heart failure, or anticoagulation therapy, though risk factors and the need for anticoagulation remain uncertain. With increasing detection of hypertrabeculation via TTE and CMR, careful evaluation is essential to distinguish true LVNC from acquired or secondary causes and avoid misdiagnosis. Clinicians should maintain a high index of suspicion for LVNC in patients presenting with new-onset heart failure, interpreting imaging findings within the broader clinical context.
Recommended Citation
Singh, Ripudaman and Flores, Eduardo D. MD, FACC, "Acquired Left Ventricular Noncompaction Physiology in Lupus Myocarditis: An Unclassified Cardiomyopathy Phenotype" (2026). Research Symposium. 2.
https://scholarworks.utrgv.edu/somrs/2026/talks/2
Included in
Acquired Left Ventricular Noncompaction Physiology in Lupus Myocarditis: An Unclassified Cardiomyopathy Phenotype
Background: Left ventricular noncompaction (LVNC) is a rare structural abnormality of the left ventricular myocardium, characterized by prominent trabeculations and deep intertrabecular recesses due to incomplete compaction during embryogenesis. Clinical presentation is highly variable, ranging from asymptomatic cases to arrhythmias, thromboembolism, heart failure, and sudden cardiac death. Diagnosis is primarily imaging-based, with transthoracic echocardiography (TTE) using the Jenni criteria as standard. These criteria include excessive apical or midventricular trabeculations with a noncompacted-to-compacted ratio >2:1 at end-systole, a thin compacted epicardial layer (≤8.1 mm), and color Doppler flow within the recesses. When echocardiography is inconclusive, cardiovascular magnetic resonance (CMR) is recommended. LVNC may be familial, sporadic, acquired, or transiently associated with conditions such as myocarditis, amyloidosis, or systemic lupus erythematosus (SLE). We report a case of SLE-induced perimyocarditis presenting with cardiomyopathy resembling LVNC.
Case Presentation: A 44-year-old Hispanic male with no significant medical history presented with four weeks of dyspnea, lower extremity swelling, and pleuritic chest pain. Examination revealed bilateral basal crackles and 3+ pedal edema. Laboratory studies demonstrated severe anemia and thrombocytopenia, elevated ESR, CRP, and BNP of 1055. Baseline EKG was unremarkable, and high-sensitivity troponin was elevated without a dynamic rise, making acute coronary syndrome less likely. TTE showed an ejection fraction of 30%, global hypokinesia, and severe pericardial effusion, consistent with perimyocarditis. Additionally, 4–5 trabeculations with deep recesses were noted at the apex and posterolateral wall of the left ventricle, with blood flow into the intertrabecular recesses visualized on color Doppler. The non-compacted to compacted myocardium ratio at end-systole was >2:1, supporting a diagnosis of LVNC. Mild concentric left ventricular hypertrophy was also present, while heart valves and morphology were normal, with no congenital anomalies. Autoimmune testing revealed active SLE, after which high-dose prednisone (1 mg/kg/day) was initiated. Supportive treatment with intravenous diuretics and guideline-directed medical therapy for heart failure resulted in clinical improvement. The patient experienced no significant arrhythmias during hospitalization. Due to concern for evolving tamponade, he underwent a pericardial window procedure and recovered uneventfully, with discharge and planned cardiology and rheumatology follow-up.
Conclusion: LVNC, formerly called spongy myocardium or hypertrabeculation syndrome, is classified as an "unclassified" cardiomyopathy by the World Health Organization and diagnosed using the Jenni TTE criteria. The presence of noncompacted myocardium alone does not always indicate disease, as LVNC can represent a morphological variant rather than a pathological condition. This case highlights SLE-related myocarditis producing imaging findings that mimic LVNC through myocardial edema and wall thickening. Given its nonspecific presentation, LVNC should be considered in patients with prominent trabeculations on cardiac imaging, even if asymptomatic. Trabeculations may develop in adulthood, warranting monitoring and, when indicated, antiarrhythmic, heart failure, or anticoagulation therapy, though risk factors and the need for anticoagulation remain uncertain. With increasing detection of hypertrabeculation via TTE and CMR, careful evaluation is essential to distinguish true LVNC from acquired or secondary causes and avoid misdiagnosis. Clinicians should maintain a high index of suspicion for LVNC in patients presenting with new-onset heart failure, interpreting imaging findings within the broader clinical context.
