Isolated congenital left ventricular diverticula: A rare cardiac anomaly, Nahid Rezaeian, Homa Najafi Archives of Cardiovascular Imaging 2017 5(2):32-34 Ventricular diverticula are a rare congenital abnormality characterized by synchronized contractility. Isolated diverticula mostly remain asymptomatic until adulthood. In this case report, we describe a 22-year-old woman with a history of pain in her arms and cold-induced skin discoloration. She was evaluated with echocardiography and cardiac magnetic resonance imaging, which illustrated a muscular basal diverticulum in the left ventricle.

Introduction

Congenital ventricular diverticula constitute a rare cardiac malformation that affects 0.26%–0.4% of people. They occur alone or in association with other anomalies.^[1],[2] Left ventricular (LV) diverticula are characterized as apical (the most common site) and nonapical.^[3],[4] In this case report, we describe a 22-year-old woman with a nonapical LV diverticulum.

Case Report

A 22-year-old woman referred to us with a history of pain in her arms and cold-induced skin discoloration, compatible with Raynaud's phenomenon, from which she had been suffering for 2 years. She denied any history of trauma. Her neurologic and cardiologic examinations were normal. She had no remarkable diseases or major cardiovascular risk factors, and nor was there any known significant family history. The electrocardiogram was nondiagnostic. Echocardiography was performed, and it showed an outpouching in the basal and mid-anterolateral wall of the LV, with normal contractility and flow, which was supplied from the LV, suggesting an LV diverticulum. Nevertheless, the other echocardiographic findings were normal. Cardiac magnetic resonance imaging (CMR) confirmed an LV muscular diverticulum by showing a localized intramyocardial space (maximal length = 30 mm and maximal width = 17 mm) in the basal and mid-LV anterolateral segments, which had a direct connection into the LV cavity via small endocardial defects. This space was surrounded by two myocardial rims. The inner rim included a thin contractile myocardium, whereas the outer rim was a thicker myocardial layer in the subepicardial aspect [[Figure 1], Videos 1 and 2].

Figure 1: (a) Steady-state free precession function shows left ventricular dysfunction, (b) Perfusion imaging shows left ventricular dysfunction, (c) Late gadolinium enhancement shows left ventricular dysfunction, (d) Short tau inversion recovery sequence shows left ventricular dysfunction Click here to view

At 1-year follow-up, echocardiography and CMR were repeated, and the findings were unchanged.

Discussion

Congenital LV outpouchings comprise five myocardial abnormalities: LV diverticula, LV aneurysms and pseudoaneurysms, accessory LVs, LV accessory chambers, and double-chambered LVs.^[5] The primary and most common types of ventricular outpouching are congenital ventricular diverticula and congenital ventricular aneurysms.

Owing to the variety of possible treatments and diagnoses, distinguishing between congenital ventricular diverticula and congenital ventricular aneurysms is very important. Congenital ventricular diverticula are often small and circular and communicate with the ventricle through a narrow neck, whereas congenital ventricular aneurysms are mostly large and saccular and have a broad base.^{[1],[2],[4],[6]} The wall of a congenital ventricular diverticulum displays synchronous contractions in accordance with the heart's rhythm; conversely, the contraction of a congenital ventricular aneurysm is paradoxical and dyskinetic.^{[2],[4],[5],[6],[7]} On histologic examination, true congenital ventricular diverticula (muscular) are formed by all the three layers (i.e., myocardium, epicardium, and endocardium), whereas congenital ventricular aneurysms consist of no or disrupted myocardium with fibrous tissue.^{[2],[4],[5],[6],[7]}

Congenital ventricular diverticula are characterized as apical and nonapical. Apical congenital ventricular diverticula mostly accompany midline thoracoabdominal malformations and can be a part of Cantrell's syndrome, whereas nonapical congenital ventricular diverticula and congenital ventricular aneurysms are isolated.^[1],[4],[7] In our patient, the small size of the protrusion with narrow rims, normal contraction, and without any anomalies suggested a nonapical-isolated congenital ventricular diverticulum.

The exact etiology of LV diverticula is unknown, but possible etiologies are intrinsic abnormalities during embryogenesis or acquired in uteromalformations such as coronary artery abnormalities, viral infections, or arrhythmia-related vascular accidents.^[8]

The clinical manifestations of ventricular diverticula depend on the associated anomalies and complications. They can present as cardiomegaly or pericardial effusion during the intrauterine period and after birth, they can also present as pericardial effusion, shock, or cardiac arrest as a result of spontaneous rupture of the diverticulum, severe respiratory failure, abnormal electrocardiography, or cardiac shadow on X-ray. Most diverticula in adults are found incidentally during a routine assessment, and some are complicated by systemic ventricular tachyarrhythmias, systemic embolisms, sudden death, spontaneous rupture, severe valvular regurgitation, congestive heart failure, and endocarditis.^[9]

Several diagnostic tools are available for detecting LV diverticula. The fastest and most common method is echocardiography. Echocardiography can assess the morphology and location of the diverticulum; nonetheless, because of technical limitations and technical artifacts, usually further investigation is needed to confirm the diagnosis.^[3],[6] CMR, multi-slice computed tomography scanning, two-dimensional echocardiography, transesophageal echocardiography, and angiographic ventriculography are the other diagnostic tools that help diagnose LV diverticula.^[2] CMR is the gold standard for diagnosing LV diverticula. This imaging modality can also differentiate LV diverticula from ventricular aneurysms and other congenital structural diseases and recommends long-term follow-ups.^[6],[7] In addition, CMR is an effective method for analyzing LV volumes and ejection fractions.^[10]

How best to manage asymptomatic patients with an isolated congenital ventricular diverticulum is controversial. Some researchers recommend surgical treatment, especially for fibrous diverticula, because of the high incidence of spontaneous rupture; others recommend conservative treatment with close follow-up with echocardiography or CMR, particularly in muscular diverticula. Nevertheless, whenever refractory symptoms, significant complications, or a rapid increase in size during follow-up develop, elective surgery should be considered.^[2],[8] Our patient had a small muscular diverticulum without any major symptoms or complications; accordingly, annual follow-up with echocardiography and CMR should be maintained.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 

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