Congenital Left Ventricular Aneurysm

This paper presents the case of a 30-year-old man who was diagnosed with an apical-lateral wall left ventricular aneurysm with scarring, prominent left ventricular trabeculations, and mildly diminished systolic function. Working diagnosis was a congenital left ventricular aneurysm in the setting of left ventricular noncompaction, yet with a questionable defect of the pericardium.

with wall thinning and severe hypokinesis with a thin layer of subendocardial late gadolinium enhancement of the apical lateral and true apical segments.Prominent trabeculations were seen within the viable part of the LV myocardium (Figure 2).

PAST MEDICAL HISTORY
Born at term without known maternal pregnancy complications, the patient reported normal growth during childhood, but had chronically reduced functional capacity and inability to participate in regular exercise activities limited by dyspnea.Additional medical history included anxiety and depression, for which the patient was treated with a selectiveserotonin reuptake inhibitor.

LEARNING OBJECTIVES
To understand that congenital LVAs are a rare abnormality, typically of the apex of the left ventricle; they are a nidus for ventricular arrhythmias.
To consider neurohormonal blockade initiation given high mortality rates from heart failure in this population.To understand that arrhythmia monitoring should be part of the initial evaluation of patients with congenital LVAs.To establish that currently the pathophysiology is unclear, LDB3 and ZASP are genes of interest.The pericardium appeared significantly thinned to absent at the aneurysmal site (Figure 4).
In addition to LVA, the diagnosis of congenital partial absence of the pericardium was considered, and in consultation with cardiothoracic surgery, the patient underwent video-assisted thoracoscopic surgery for direct pericardial visualization with plan for repair if a defect was found, the area of pericardial and myocardial thinning can be seen in Figure 5.
Video-assisted thoracoscopic surgery revealed a thin yet intact pericardium with bulging of the pericardium where the LV apical aneurysm was located.
Final considerations included a LVA in the setting of a myopathy within the scope of LV noncompaction, given the prominent trabeculations noted on cardiac magnetic resonance.

MANAGEMENT (MEDICAL/INTERVENTIONS)
The patient was initiated on aspirin and neurohormonal blockade including metoprolol succinate, valsartan-sacubitril, and empagliflozin, and had the aforementioned placement of an ICD.

DISCUSSION
LV structural abnormalities have been reported to develop from the fourth week of embryonic life. 1 Isolated LVAs are commonly an incidental cardiac finding with a reported history of normal pregnancy, yet LVAs may have catastrophic in utero outcomes presenting with fetal arrhythmia, hydrops fetalis, and fetal demise. 1      Congenital Ventricular Aneurysms ISSN 2666-0849 https://doi.org/10.1016/j.jaccas.2024.102453DIFFERENTIAL DIAGNOSIS The differential diagnosis included congenital coronary artery abnormalities, congenital partial absence of the pericardium with ventricular herniation, and congenital cardiomyopathy.The myocardium morphology suggests a congenital myopathy within the LV noncompaction spectrum with course trabeculations, yet the discrete apical-lateral LV aneurysm located in a noncoronary distribution and subendocardial scarring raises the diagnosis of a congenital left ventricular aneurysm (LVA).The dilemma was whether the focal thinning of the pericardium around the aneurysm could also represent a pericardial defect with potential herniation.INVESTIGATIONS Active ischemia was ruled out using a single-photon emission computed tomography exercise stress testing, yet revealed a large size fixed defect at the aneurysmal site seen in Figure 3. Seven-day event monitor showed scattered premature ventricular complexes and an electrophysiology-induced rapid ventricular arrhythmia at the aneurysm site for which the patient received a subcutaneous internal cardioverter-defibrillator (ICD).A computed tomography angiography of the coronaries redemonstrated the unusual myocardial architecture and revealed normal coronary origin and course, without stenosis, yet paucity of coronary distribution to the aneurysm.
Ohlow et al 2 reported that among those with LVA, 62% are found in the apex, 15% are in the apical-lateral wall, 8% are lateral, 8% are septal, and 4% are posterolateral.Associated abnormalities are common and include intracardiac shunts, coronary anomalies, and extracardiac thoracoabdominal abnormalities.Histology reveals fibrous replacement of the normal 3-layered myocardium.There are also reports of multilobulated bulging arising from the left ventricle.In a retrospective adult cohort, the prevalence of LVA was found to be 0.34%, commonly

FIGURE 2 3
FIGURE 2 3 Different Slices From the Same Cardiac Magnetic Resonance Sequence

FIGURE 3
FIGURE 3 Stress Test Results

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A C C : C A S E R E P O R T S , V O L . 2