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A 60-year-old male hypertensive was suspected to have post myocardial infarction left ventricular aneurysm. His transthoracic echocardiogram performed elsewhere reported an inferior wall left ventricular aneurysm. He was referred for coronary angiography. His coronary angiogram and repeat transthoracic echocardiogram demonstrated an accessory chamber arising from mid-inferior wall synchronously contracting with the left ventricle suggesting a diverticulum. In addition, he had anomalous coronary arteries with coronary artery disease. His electrocardiogram did not reveal any features suggestive of a post infarct aneurysm. This case illustrates the importance of a comprehensive clinical and echocardiographic examination with a broad differential thought process.
A 60-year-old male was referred to our institute for coronary angiography. He was known to be hypertensive with a recent history of hospital admission for uncontrolled hypertension, acute pulmonary edema and non-ST-elevation myocardial infarction. His echocardiogram was reported as concentric left ventricular (LV) hypertrophy, global hypokinesia of LV with severe LV systolic dysfunction and an inferior wall post infarct aneurysm. He denied any angina or dyspnea. His cardiovascular examination revealed a heaving apex, S4 with no signs of heart failure. The ECG reviewed prior to coronary angiogram showed LV hypertrophy with strain pattern, slurring of S wave in leads II and AVF without any evidence of QS waves or persistent ST elevation in the inferior leads (Fig. 1). Coronary angiogram done showed absent left main, with selective left anterior descending artery injection showing tight lesion in the mid-segment (Fig. 2A, arrowheads). The left circumflex artery was arising from right sinus originating from a common ostium along with right coronary artery (Fig. 2B). The right coronary artery was totally occluded proximally with tight lesion in mid-left circumflex artery (Fig. 2B, arrowheads). On left ventriculogram, an accessory chamber was seen as an oblong out pouching at the mid-inferior wall that filled rapidly with contrast medium. When first observed it seemed to be inferior wall aneurysm, but was synchronously contracting with the LV suggesting being a diverticulum. The image resembled a ‘kangaroo carry’ pouch with the larger LV carrying the diverticulum that mimicked a smaller version of the LV (Fig. 3A and B, arrowheads movie). Following this, transthoracic echocardiogram was done which confirmed severe concentric LV hypertrophy, mild mitral regurgitation, and global hypokinesia of LV with an ejection fraction of 25%. In addition, there was a contractile accessory chamber (tongue like) originating from mid-inferior wall of the LV with a narrow neck (5 mm) and measuring 27 mm in length (Fig. 4A and B, arrowheads) with no thrombus. Color Doppler showed blood flow entering the diverticulum from LV during diastole. The appearance on echocardiography confirmed LV diverticulum. Patient was advised surgery, but he refused.
Congenital ventricular diverticulum is a rare cardiac anomaly in adults. It is commonly detected in children along with midline thoracoabdominal defects . In one-third of the cases, LV diverticulum is not associated with congenital malformations and is referred to as isolated LV diverticulum . Congenital LV diverticulum consists of a localized out pouching from the free wall of the LV that contains endocardium, myocardium, and pericardium . The prevalence is 0.42% among 12,271 adult patients undergoing left ventriculogram . The patients are generally asymptomatic, but may develop thromboembolism, heart failure, arrhythmias, and rarely, rupture [1,2]. Diagnosis can be made by echocardiography, computed tomography angiography, magnetic resonance imaging, and cineangiography . LV diverticulum needs to be differentiated from a ventricular aneurysm/pseudoaneurysm. A narrow neck and synchronous contractility along with all three ventricular layers indicate a diverticulum . In contrast, aneurysm (congenital or acquired) or pseudo aneurysm shows akinesia or paradoxical contractility of the out pouching with a broad neck/thinned myocardium in an aneurysm and a narrow neck/pericardium in a pseudo aneurysm. Congenital LV diverticulum on echocardiography appears as a tongue like or digitiform out pouching of the ventricle, commonly occurring in the LV apex with similar acoustic properties to the ventricular wall .
The exact etiology of congenital LV diverticulum is not known. It is a developmental anomaly, starting in the 4th embryonic week . It is postulated that a maldevelopment of the myocardial intratrabecular sinusoids along with hemodynamic factors may contribute to its formation . Treatment is surgical resection if the diverticulum is large, symptomatic or if associated with cardiac anomalies requiring surgery . In addition, anticoagulation is advised after systemic embolization, radiofrequency ablation or implantation of an implantable cardioverter-defibrillator is advocated in case of symptomatic ventricular tachycardia, with or without class I or III antiarrhythmic drugs . Recently, percutaneous closure of LV diverticula has been described .
Ohlow et al.  reported coronary artery anomalies in nearly 60% of 117 patients with isolated congenital LV aneurysm or diverticulum. These coronary anomalies were common in patients with inferior or non-apical location, as noted in this patient. In their study, nearly 40% of the patients with LV diverticulum had right coronary artery anomalies with absent left main in 3.4% and left circumflex arising from right cusp in 1.8% of the patients similar to the patient described in this report .
In the case reported, the initial echocardiogram was reported as LV aneurysm even though he did not sustain an ST-elevation myocardial infarction, nor did his ECG showed any evidence of QS waves or persistent ST elevation in the inferior leads. After review of his ECG, we did have doubt regarding aneurysm diagnosis even though ECG has a sensitivity of 51% and specificity of 84% in diagnosing ventricular aneurysm . However, coronary angiogram and transthoracic echocardiogram demonstrated absence of akinetic or dyskinetic movement along with a typical synchronous movement of the accessory chamber with the LV clinching a diagnosis of diverticulum. This case illustrates a rare combination of congenital LV diverticulum with anomalous coronary artery and coronary artery disease in an adult patient. In addition, this case illustrates the importance of a comprehensive clinical and echocardiographic examination with a broad differential thought process. The diagnosis of LV diverticulum was based on overall integration of all the data.
There is no conflict of interest or financial disclosure for our article.