What is the initial approach to managing a left ventricular (LV) aneurysm in a young adult?

Causes of Left Ventricular Aneurysm in a 19-Year-Old Male

In a 19-year-old male with left ventricular aneurysm, the most critical diagnosis to consider is hypertrophic cardiomyopathy with apical aneurysm, which carries significantly increased sudden cardiac death risk, followed by congenital structural abnormalities, infectious etiologies (particularly mycotic aneurysms from endocarditis), and connective tissue disorders. 1

Primary Etiologic Categories

Hypertrophic Cardiomyopathy with Apical Aneurysm

• This represents the highest-risk etiology in adolescents and young adults with LV aneurysm, associated with markedly elevated sudden cardiac death risk 1
• Apical aneurysms in HCM context require immediate comprehensive risk stratification 1

Congenital Structural Abnormalities

The following congenital lesions must be systematically evaluated 1:

• Bicuspid aortic valve with associated aortic regurgitation - AR can develop in adolescents with bicuspid valves and may lead to progressive LV dysfunction and aneurysm formation 2
• Discrete subaortic obstruction with secondary LV remodeling 1
• Ventricular septal defect with prolapse of aortic cusp into the defect 2, 1
• Post-surgical complications from congenital heart disease repair - particularly in patients who underwent repair of atrioventricular septal defects or other complex lesions in childhood 2, 1

Infectious Etiologies

• Mycotic aneurysms from infective endocarditis are particularly important in pediatric populations 1
• Inflammatory diseases including Kawasaki disease can cause coronary artery aneurysms leading to myocardial infarction and subsequent LV aneurysm 2
• Post-infectious myocarditis with subsequent scar formation 3

Connective Tissue Disorders

The following must be systematically excluded 1:

• Ehlers-Danlos syndrome type IV - associated with vascular fragility and aneurysm formation 1
• Marfan syndrome - can cause aortic root dilation and secondary cardiac complications 1

Idiopathic LV Aneurysm

• Life-threatening ventricular arrhythmias may be the first manifestation of idiopathic LV aneurysm in young adults 3
• These aneurysms can present with ventricular tachycardia, ventricular fibrillation, or sudden cardiac arrest without prior symptoms 3
• Defects of sympathetic innervation and metabolism are documented in the aneurysm area, potentially contributing to arrhythmogenesis 3

Initial Diagnostic Approach

First-Line Imaging

• Transthoracic echocardiography is the mandatory first-line diagnostic modality to differentiate true aneurysm from pseudoaneurysm, assess LV systolic function, evaluate for apical aneurysm in HCM context, and assess valve function 1, 4
• Obtain both parasternal and apical views to fully characterize the aneurysm location and morphology 1

Advanced Imaging

• Cardiovascular magnetic resonance (CMR) imaging should be obtained to quantify late gadolinium enhancement, precisely characterize aneurysm morphology, assess for transmural scar at the aneurysm site, and evaluate myocardial viability 1, 3
• Coronary angiography is recommended before any surgical intervention to exclude anomalous coronary anatomy, identify coronary artery aneurysms, and rule out atherosclerotic disease (though rare at this age) 2, 1

Arrhythmia Assessment

• Perform 24-48 hour ambulatory (Holter) monitoring to assess for nonsustained ventricular tachycardia 1
• Consider event recorder for patients with syncope or palpitations 2
• Electrophysiology testing may be indicated in high-risk patients or those with documented clinical arrhythmias 2, 3

Laboratory Evaluation

• Assess inflammatory markers (ESR, CRP) to evaluate for active infection or inflammatory process 1
• Consider screening for hemochromatosis, HIV, and rheumatologic conditions in selected cases 4
• Measure BNP or NT-proBNP if heart failure is suspected 4

Risk Stratification for Sudden Cardiac Death

Comprehensive SCD risk assessment must be performed every 1-2 years evaluating the following 1:

• Personal history of syncope, cardiac arrest, or sustained ventricular arrhythmias 1
• Family history of sudden cardiac death or cardiomyopathy 1
• Massive left ventricular hypertrophy (if HCM present) 1
• Left ventricular systolic dysfunction 1
• Presence of apical aneurysm 1
• Genotype status (if genetic testing performed) 1

Critical Pitfalls to Avoid

• Do not assume coronary artery disease as the primary etiology - while the vast majority of LV aneurysms in adults are secondary to myocardial infarction 5, this is exceedingly rare in a 19-year-old without risk factors
• Do not delay echocardiography - it is the definitive initial test to confirm the aneurysm and guide further workup 4
• Do not overlook the possibility of contained rupture - patients on antithrombotic therapy or with recent trauma may present with hemopericardium 6
• Do not miss HCM with apical aneurysm - this carries the highest sudden death risk and requires specific management strategies 1