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 Table of Contents  
REVIEW ARTICLE
Year : 2019  |  Volume : 16  |  Issue : 4  |  Page : 105-110

Submitral left ventricular aneurysm: Characteristics, diagnosis, management, and outcome


1 Department of Surgery, Cardiothoracic Unit, School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia
2 Department of Internal Medicine, Division of Cardiology, School of Medicine, College of Health Sciences, Addis Ababa University, Addis Ababa, Ethiopia

Date of Web Publication22-Oct-2019

Correspondence Address:
Dr. Senbeta Guteta Abdissa
Division of Cardiology, Department of Internal Medicine, School of Medicine, College of Health Sciences, Addis Ababa University, P.O. Box 28287/1000, Addis Ababa
Ethiopia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcls.jcls_99_18

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  Abstract 

Left ventricular (LV) aneurysm (LVA) is an uncommon cardiac disorder. There is no documented report from Ethiopia. Our objective was to discuss a case of LVA we encountered and analyze the clinical characteristics, diagnosis, management modalities, and outcome of patients with LVA from published studies over the last 200 years. in addition to the case we encountered, we searched PubMed for publications on patients with LVA and analyzed those reported since 1816. We identified 369 patients with LVA. The mean age at diagnosis was 31.7 ± 21.7 years, and 52.3% of them were males. It was more frequently reported from Africa (32.0%) and Europe (31.4%). The inferior part of the left ventricle including the submitral area is the most frequently (51.8%) reported location for LVA. Diagnosis was made coincidentally in most (40.1%) of the patients. The presenting features in those symptomatic patients were dyspnea or heart failure (HF) (23.8%), rhythm disturbances (18.2%), chest pain (10.6%), syncope (8.4%), thrombus (10.8%), or embolic events (5.4%). The universally used diagnostic modality was echocardiography. Electrocardiography (61%), chest X-ray (31.2%), cardiac catheterization (42.8%), computed tomography (1.3%), and magnetic resonance imaging (1.6%) were used as confirmatory or complementary imaging tests. During the follow-up period, death from cardiac causes occurred in 13.1% of the patients, with the leading cause of the cardiac death being congestive HF (51.9%). LVA has varied clinical features with potentially deleterious outcomes. While close follow-up with echocardiography in such patients is necessary, building the local expertise in surgical management of such patients is recommended as it has implications on individual patient, proper health-care utilization, and health-care costs.

Keywords: Diagnosis, management, outcome, submitral left ventricular aneurysm


How to cite this article:
Nega B, Goshu DY, Abdissa SG. Submitral left ventricular aneurysm: Characteristics, diagnosis, management, and outcome. J Clin Sci 2019;16:105-10

How to cite this URL:
Nega B, Goshu DY, Abdissa SG. Submitral left ventricular aneurysm: Characteristics, diagnosis, management, and outcome. J Clin Sci [serial online] 2019 [cited 2019 Dec 10];16:105-10. Available from: http://www.jcsjournal.org/text.asp?2019/16/4/105/269723


  Introduction Top


Left ventricular (LV) aneurysm (LVA), first described in 1816, is an uncommon cardiac disorder. It is a sac-like outpouching that arises from the wall of the left ventricle. Although LVA was thought to be a condition that had exclusively occurred in the population of Africa, many cases have been described from various areas outside Africa.[1],[2]

Patients with LVA are predominantly asymptomatic, and it is discovered coincidentally when diagnostic procedures are done for other reasons. However, some LVA can cause significant morbidity and mortality due to heart failure (HF), rupture of the lesion, thromboembolism, and sudden cardiac death.[3],[4]

The improvement of echocardiography and the advent of other advanced cardiac diagnostic methods have led to an earlier diagnosis.[5],[6],[7],[8] Most of the publications to date are case reports, and there is a small number of case series and a few number of reviews.[2],[9],[10]

In addition to the discussion of the case our group encountered, we retrospectively reviewed literature that provided sufficient data to provide a clear picture of this rare condition. The purpose of our study was to collect all published data on LVA and then examine the clinical characteristics, diagnosis, management modalities, and outcome of such patients.


  Case Report Top


A 29-year-old male patient (consent obtained) presented with a 1-month history of palpitation and left chest pain on exertion that radiated to the back and left arm. He developed a progressive breathlessness and a low-grade intermittent fever for 2 weeks.

On general examination, heart rate was 124/min, blood pressure was 110/70 mmHg, temperature was 36.9°C, respiratory rate was 22/min, and weight was 79 kg. Cardiovascular examination revealed soft heart sounds without any audible added sounds.

Investigation done showed hemoglobin of 12.5 g/dl, total white blood cell count of 9600/mm 3 of blood, and platelet count of 334,000/mm 3. Serology test for both HIV and Venereal Disease Research Laboratory was nonreactive. There was no electrolyte abnormality, and cardiac-specific enzymes were normal.

Resting electrocardiography (ECG) showed evidence of sinus tachycardia [Figure 1].
Figure 1: Electrocardiography of the patient showing sinus tachycardia

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Chest X-ray (CXR) (posteroanterior and lateral views) of the patient showed cardiomegaly, predominantly on the left lower border as depicted in [Figure 2].
Figure 2: Chest X-ray films of the patient (a) Postero–anterior. (b) Lateral. Postero–anterior view shows enlargement of the cardiac silhouette primarily at its left lower border

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Transthoracic echocardiography (TTE) revealed a large cavity (6 cm × 7.5 cm) arising immediately below the posterior mitral leaflet and communicating with the ventricle through a 10-mm wide ostium [Figure 3]a. Color Doppler flow imaging demonstrated systolic flow into the aneurysm [Figures 3b] and mild mitral regurgitation. As open-heart surgery could not be performed, due to lack of appropriate setup and an expert, the patient was referred abroad for surgical treatment.
Figure 3: Echocardiography of the patient. (a) Apical four-chamber view showing the aneurysm. (b) Color Doppler flow imaging showing systolic flow into the aneurysm. LA = Left atrium, RA = Right atrium, LV = Left ventricle, RV = Right ventricle, LVA = Submitral left ventricular aneurysm

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  Methods Top


In addition to the earlier case report, we performed a PubMed search to identify all articles published with the description of LVA. Medical Subject Headings and keywords' searches included the terms “submitral,” “ventricular aneurysm/aneurysms” ([“heart ventricles”(MeSH Terms) OR “heart” (All Fields) AND “ventricles” (All Fields)] OR “heart ventricles” [All Fields] AND [“aneurysm” (MeSH Terms) OR “aneurysm” (All Fields)]). We limited our literature search to researches done on humans and publications in English. This yielded a total of 2752 articles. Reference lists of the selected articles were reviewed for other potentially relevant citations. All retrieved studies were examined to eliminate potential duplicates or overlapping data, and we finally analyzed data of 369 patients with LVA.

Study selection and data extraction

We included an article if it reported about LVA. We recorded baseline demographics, clinical and morphological characteristics, diagnostic methods, management, and outcome. The authors of this manuscript have verified that they abide by the principles of ethical publishing.

Definitions

Left ventricular aneurysm

The definition of LVA was adopted from the respective article.

Location of the left ventricular aneurysm

Nomenclature of the location of LVA was adopted from Austen et al.[11] and included the following: (1) anterobasal, (2) anterolateral, (3) apical, (4) diaphragmal, (5) posterobasal, (6) septal, (7) lateral, and (8) LV outflow tract. “Inferior” was defined as diaphragmal plus posterobasal plus submitral location.

Statistical analysis

We reported continuous variables as mean value ± standard deviation (SD) and categorical variables as absolute (n) and relative (%) frequencies. Clinical outcomes (cardiac death, noncardiac death, HF, rupture, arrhythmic events, embolic events, increase in the size of the LVA, and operative repair of LVA) were presented. The results from the included studies were combined for each outcome to give an overall estimate. The overall mean and SD were obtained by using the pooled mean and SD.[12] Statistical analysis was performed using Excel and SPSS software version 23.0. (Armonk, NY, USA: IBM Corp).


  Results Top


We extracted the data of 369 patients with LVA since the first publication in 1816. Until 1950, there were only ten patients with LVA published. By the year 2000, the number of literature reached 223, basically on individual patient cases. Since then, there were 146 new patients with LVA published [Figure 4].
Figure 4: Cumulative number of publications of patients with left ventricular aneurysm over a period of about two centurie

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LVA was more frequently reported from Africa (118/369 [32.0%]) and Europe (116/369 [31.4%]) followed by America (93/369 [25.2%]) and Asia (42/369 [11.4%]). Males constituted 193/369 (52.3%) patients diagnosed with LVA. The mean age at diagnosis was 31.7 ± 21.7 years. Twenty-one patients (5.7%) were diagnosed prenatally.

Clinical presentation

Patients found to have LVA presented with a wide range of clinical features ranging from coincidental diagnosis to disabling symptoms.

Coincidental diagnosis

One hundred and forty-eight (40.1%) patients did not have a symptom and were found coincidentally when diagnostic procedures were performed for other reasons.

Dyspnea on exertion or heart failure and other symptoms

The most frequent symptom as the cause of evaluation was dyspnea. A history of dyspnea on exertion or HF was noted in 88 (23.8%) LVA patients. Complaints of typical angina or atypical chest pain were described in 39 (10.6%) patients. Four (1.1%) patients from India were reported to have developed LVA after tuberculosis or inflammation.

Syncope and rhythm disturbances

The 2nd most frequent cause for evaluation was syncope and rhythm disturbances. The incidence of atrial or ventricular tachyarrhythmic events was 67 (18.2%) and that of syncope was 31 (8.4%).

Thrombus, embolic events, and rupture at presentation

Thrombus or spontaneous echo contrast in LVA was reported in 40 (10.8%) patients, whereas 20 (5.4%) patients presented with cerebral and/or peripheral embolic events. Rupture as the cause of presentation was reported in 14 (3.8%) patients, with 92.9% (13/14) of the patients being younger than 18 years.

Diagnostic studies

Echocardiography

Diagnosis of LVA in all patients was initially made by TTE. Transesophageal echocardiography (TEE) in 12 (3.3%) and three-dimensional echocardiography (3D Echo) in 3 (0.8%) patients were used to confirm the diagnosis of LVA.

Electrocardiography (ECG)

Electrocardiography was reported in 225 (61%) patients with LVA. The range of ECG alterations included sinus rhythm in 91 (40.4%), alterations of depolarization (e.g., Q-waves in 31 [13.8%]) and repolarization (e.g., T-wave abnormalities in 73 [32.3%]), bundle branch block in 16 (7.16%), and arrhythmia in 67/225 (18.2%) patients.

Chest radiography

Chest radiography was available in 115 (31.2%) patients diagnosed with LVA. The descriptions included cardiomegaly or alteration of the cardiac silhouette in 87 (75.6%) and local accumulation of calcium at the site of LVA in 14 (12.2%) patients.

Cardiac catheterization, cardiac computed tomography, and cardiac magnetic resonance imaging

Cardiac catheterization was performed in 158 (42.8%) patients, whereas cardiac computed tomography (CT) and cardiac magnetic resonance imaging (MRI) (CMR) were performed in five (1.3%) and six (1.6%) patients, respectively.

Morphologic characteristics

Location, size, and contractility of the left ventricular aneurysm

Most of the LVA, 191 (51.8%), were localized “inferiorly” (submitral, posterobasal, and diaphragmal), whereas 102 (27.6%) cases were localized at the LV apex. The mean size of LVA was 1498 ± 506 mm 2, and 290 (78.6) LVA patients were found to be hypo- or akinetic.

Histology

Histological examination was performed in 64 patients with LVA. The wall of the aneurysm was found to be fibrous in 57 patients (90.1%), with isolated/atrophic residual myocardial fibers in 11.1% (7/63). In the remaining 7 (9.9%) patients, the wall of the lesion showed no microscopic features distinct from the LV myocardium. The other histological findings were vacuolization (3.6%), calcifications (10.4%), and giant cells (3.6%).

Treatment and outcome of patients with left ventricular aneurysm

Operative treatment and mortality and morbidity during follow-up are shown in [Table 1]. Follow-up was available in 206/369 (55.8%) patients diagnosed with LVA (the longest follow-up was 18 years). The mean follow-up duration was 62.7 ± 48.3 months, resulting in a cumulative follow-up of 1076 patient-years. Nearly 39% of the patients underwent surgical management.
Table 1: Treatment and outcome of patients with left ventricular aneurysm during follow-up (n=206)

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Surgical management

The surgical technique depended on the location and size of the LVA and was determined by a resection of the diverticulum or aneurysm. It included aneurysmectomy alone in 62/81 (76.5%) or combined with correction of accompanying mitral regurgitation in 6 (7.5%), or congenital heart defects in 13 (16%) patients.

Mortality and morbidity during follow-up

Cardiac death occurred in 27 (13.1%) patients. HF as a cause of cardiac death was reported in 14 (51.9%) patients. Rupture of the aneurysm was reported in six (22.2%) patients and occurred only in patients younger than 8 years of age. The incidence of sudden cardiac death reported was 7 (25.9%).


  Discussion Top


This study is one of the largest reviews specific to cases of LVA. It provides a full description of this uncommon, but potentially serious, cardiac pathology.

Prevalence

There is no prospective study done regarding the epidemiology of LVA. A retrospective echocardiographic study described 8 LVA out of 43,000 consecutive adult patients, resulting in a prevalence of 0.02%.[13] A publication by Ohlow et al. demonstrated a prevalence of 0.46% in 12,271 consecutive adult patients undergoing coronary angiography.[9] The low prevalence on echocardiographic study might be attributed to the underestimation of the true number of LVA due to difficult visualization, particularly of the LV apex. Even though it was more frequently reported from Africa (32.0%), publications of cases from other regions of the world have been increasing despite previous reports claim that it is mainly a disease of the Black population.[1],[14]

Clinical presentation

LVAs are often diagnosed coincidentally when diagnostic procedures are performed for other reasons and are commonly asymptomatic. This may explain the late diagnosis of, on an average, 31.7 years. The most common clinical features in the symptomatic patients were dyspnea or HF, syncope or rhythm disturbances, and embolic events.

Diagnostic studies

There is no specific laboratory test for LVA. Diagnosis is mainly based on imaging studies.

Electrocardiography (ECG)

Even though there is no specific feature, a wide range of ECG features were described in patients with LVA. The findings included sinus rhythm, alterations of depolarization and repolarization, bundle branch block, and ventricular or atrial arrhythmia.

Chest X-ray

Depending on the size of the LVA, CXR may show cardiomegaly. The other finding is localized calcifications.[15]

Echocardiography

TTE, TEE, and more recently 3D-Echo are an indispensable and universal noninvasive method of diagnosing LVA.[16] In addition, they help in identifying accompanying malformations, characterizing the LVA, and provide information about the contractility and myocardial perfusion of LVA.[17],[18],[19],[20],[21]

Other imaging studies

Cardiac CT (CCT), CMR, coronary angiography, LV angiography, and radionuclide studies are used as confirmatory or complementary imaging studies. CT demonstrated relevant anatomical details in patients with LVA although only few data systematically analyzed the relevance of CT.[22],[23],[24] MRI is particularly important in visualizing associated complex congenital cardiac anomalies. Its role for diagnosing LVA, however, needs to be exactly defined.[2]

Coronary angiography is helpful to exclude the possibility of coronary artery disease as an underlying cause for LVA when there is clinical suspicion.[25] LV angiography discloses the typical changes of a more or less contracting aneurysm or diverticulum with a small or wide communication with the left ventricle.[3]

Radionuclide studies show the amount of active myocardium depending on the level of uptake in the wall of the LVA and thus, might be helpful to assess the risk of rupture of the aneurysm although not been established as a routine procedure yet.[26]

Management

Treatment modalities of LVA are determined by the clinical presentation and findings of the individual patient. The clinical conditions, size of the aneurysm, or related findings determine whether to follow a nonsurgical strategy with careful follow-up or perform surgery which is the definitive management. Surgical treatment includes aneurysmectomy alone or aneurysmectomy combined with correction of mitral valve or accompanying congenital heart defects.[27],[28],[29]

Outcomes

Cardiac death occurred usually early, before the 9th year of life, and the most frequent causes in descending order were congestive HF, rupture, and sudden cardiac death. Congestive HF in LVA patients can be explained in the context of chronic volume overload caused by the LVA. Rupture of the ventricle at the site of the lesion as the cause of death is thought to be because of the thinner wall with transmural fibrosis and paradoxical contractions causing progressive dilatation and exposing the aneurysm to the risk of rupture.[27],[30],[31] Rupture in general appears to be a problem of the younger age groups as the median age at the time of rupture is in the perinatal period.

The other outcomes in patients with LVA during follow-up include arrhythmic events with or without syncope and sudden cardiac death. The majority of the patients with ventricular tachycardia (VT) had morphologies corresponding to the anatomical location of the LVA. Reproducible inducibility of the monomorphic VT during electrophysiologic testing suggests re-entry as the underlying mechanism of these arrhythmias.[32]


  Conclusion Top


LVAs are a rare cardiac malformation and mainly asymptomatic. However, they may cause HF, VT, sudden cardiac death, ventricular wall rupture, and systemic embolization. The index of suspicion should be high in young patients presenting with the symptoms and signs of HF and mitral regurgitation, particularly with normal valve morphology. Diagnosis is mainly made by TTE. TEE, cardiac CT, and MRI help in confirming the diagnosis of LVA when required. The treatment of choice depends on individual patient, but surgery is the definitive management. When surgery is delayed, close follow-up with echocardiography is necessary. Building the capacity of local expertise in the surgical management of such patients is recommended as it has implications on the individual patient and proper health-care utilization and health-care costs.

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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