What is the initial approach to a teenage patient presenting with a left ventricular (LV) aneurysm?

Left Ventricular Aneurysm in a Teenage Patient: Diagnostic Approach

In a teenage patient with LV aneurysm, the most critical first step is to determine whether this is a true aneurysm versus pseudoaneurysm, as pseudoaneurysms are prone to rupture and require urgent surgical intervention, while simultaneously investigating non-ischemic etiologies since coronary artery disease is exceedingly rare in this age group.

Primary Etiologic Considerations in Adolescents

Non-Ischemic Causes (Most Likely)

Hypertrophic cardiomyopathy (HCM) with apical aneurysm is a critical diagnosis to consider, as this represents a discrete thin-walled dyskinetic or akinetic segment with transmural scar at the LV apex and is associated with significantly increased sudden cardiac death risk 1.

• Congenital structural abnormalities including:

  • Bicuspid aortic valve with associated aortic regurgitation leading to chronic volume overload and LV remodeling 1
  • Discrete subaortic obstruction 1
  • Ventricular septal defect with prolapse of aortic cusp 1
  • Post-surgical complications from congenital heart disease repair 1

• Infectious etiologies are particularly important in pediatric populations:

  • Mycotic (infectious) aneurysms from infective endocarditis, especially in patients with congenital or rheumatic heart disease 2
  • These result from arterial damage caused by infective emboli 2

• Connective tissue disorders must be systematically excluded:

  • Ehlers-Danlos syndrome type IV (though this typically affects intracranial vessels, cardiac involvement can occur) 2
  • Marfan syndrome or other collagen vascular diseases 1

Rare Ischemic Causes in Adolescents

• Anomalous coronary arteries with resultant myocardial infarction 3
• Kawasaki disease with coronary artery aneurysms and subsequent thrombosis 1
• Multisystem Inflammatory Syndrome in Children (MIS-C) with coronary involvement and LV dysfunction 1

Critical Diagnostic Algorithm

Immediate Imaging Assessment

Transthoracic echocardiography is the first-line diagnostic modality to:

• Differentiate true aneurysm (wide neck, contains all three myocardial layers) from pseudoaneurysm (narrow neck, contained rupture) 4, 5
• Assess LV systolic function (ejection fraction <50% indicates high-risk pathology) 1
• Evaluate for apical aneurysm specifically in HCM context 1
• Identify valvular abnormalities (bicuspid aortic valve, aortic regurgitation, mitral regurgitation) 1
• Measure LV dimensions (end-diastolic dimension >4 standard deviations above normal is significant) 1

Advanced Cardiac Imaging

Cardiovascular magnetic resonance (CMR) imaging should be obtained to:

• Quantify late gadolinium enhancement (LGE ≥15% of LV mass indicates extensive fibrosis and increased sudden death risk in HCM) 1
• Precisely characterize aneurysm morphology and wall thickness 1
• Assess for transmural scar at the aneurysm site 1
• Evaluate maximal wall thickness (≥30 mm or z-score ≥20 in pediatrics suggests massive LVH) 1

Coronary Assessment

Coronary angiography is recommended before any surgical intervention in adolescents to:

• Exclude anomalous coronary anatomy 1
• Identify coronary artery aneurysms from Kawasaki disease or MIS-C 1
• Rule out atherosclerotic disease (though extremely rare in this age group) 4
• Plan surgical approach if Ross operation or other complex repair is contemplated 1

Risk Stratification for Sudden Cardiac Death

Comprehensive SCD risk assessment must be performed every 1-2 years in adolescents with LV aneurysm, evaluating 1:

• Personal history of cardiac arrest, sustained ventricular arrhythmias, or unexplained syncope
• Family history of premature HCM-related sudden death in close relatives
• Massive LVH with wall thickness ≥28-30 mm (or z-score ≥20 in pediatrics)
• LV systolic dysfunction with ejection fraction <50%
• Apical aneurysm presence (discrete thin-walled dyskinetic segment)
• Extensive LGE comprising ≥15% of LV mass on CMR
• Nonsustained ventricular tachycardia on 24-48 hour ambulatory monitoring (≥3 beats at rate exceeding baseline sinus rate by >20%)
• Genotype status if genetic testing performed

Laboratory Evaluation

Obtain the following to identify underlying systemic conditions:

• Inflammatory markers (ESR, CRP) to assess for active infection or inflammatory process 1
• Troponin and BNP/NT-proBNP to evaluate myocardial injury and heart failure 3
• Blood cultures if infectious etiology suspected 2
• Genetic testing for cardiomyopathy panels and connective tissue disorders 1

Critical Management Pitfalls to Avoid

Do not assume "normal" vital signs exclude serious pathology - adolescents with complex cardiac disease can maintain adequate blood pressure and oxygen saturation despite significant cardiac dysfunction 3.

Pseudoaneurysms have high mortality regardless of treatment, with especially poor outcomes in non-surgical management 4. Any suspicion of pseudoaneurysm (narrow neck on imaging) mandates urgent cardiothoracic surgery consultation 5.

Physical examination findings are often nonspecific - a murmur is present in only 70% of cases, and many patients may be asymptomatic despite life-threatening pathology 4.

ECG findings are typically nonspecific - only 20% show ST segment elevation; most demonstrate nonspecific ST-T wave changes 4. Do not be falsely reassured by a relatively normal ECG.

Surgical Indications in Adolescents

Symptomatic patients require intervention when presenting with 1:

• Angina, syncope, or dyspnea on exertion
• NYHA functional class III or IV symptoms
• Signs of heart failure

Asymptomatic patients require intervention when demonstrating 1:

• LV systolic dysfunction (ejection fraction <50%) on serial studies 1-3 months apart
• Progressive LV enlargement (end-diastolic dimension >4 standard deviations above normal)
• Apical aneurysm with high-risk features in HCM context

Surgical approach depends on underlying etiology: valve repair/replacement for valvular disease 1, aneurysm resection with linear closure for true aneurysms 6, or urgent repair for pseudoaneurysms 4, 5.