Congenital Left Ventricular Aneurysm Research Articles

English

Left ventricular aneurysms are not uncommon and mostly occur as a sequel of acute myocardial infarction. Congenital left ventricular aneurysms (CLVA) especially submitral ones are very rare and only few cases are reported in literature. Here we report a unique case of congenital sub mitral aneurysm, in a 16 year old patient presenting as mitral regurgitation with absent lower

Management of the congenital aneurysm of the left ventricle associated with mitral insufficiency in a child: a case report.

The combination of congenital left ventricular aneurysm associated with mitral insufficiency is rare. We describe the case of a girl aged 11 years, bearing these two entities simultaneously. Aneurysmal resection of the left ventricle was performed with Dor technic to allow remodelation of the anatomy of the left ventricle. Mitral annuloplasty was performed through a transseptal approach. Three months after surgery, the child presents a good myocardial contractility without mitral regurgitation and normal ejection fraction.

Left Ventricular Aneurysm in a Four-Year-Old Child

We describe spontaneous rupture of a congenital left ventricular (LV) aneurysm with subsequent tamponade and cardiac arrest in a 4-year-old male with staphylococcal septicemia. Emergency resuscitation, thoracotomy, and oversewing were successfully undertaken in the pediatric intensive care unit. There was complete cardiovascular recovery without adverse neurodevelopmental sequelae. This article details the difficulties in determining the etiology of ventricular aneurysms but highlights the importance of attempting to do so, particularly in distinguishing between congenital and acquired forms. Congenital aneurysms are usually a stable pathology; mycotic aneurysms are not and should be managed emergently, as survival after rupture is rare.

Congenital left ventricular wall abnormalities in adults detected by gated cardiac multidetector computed tomography: Clefts, aneurysms, diverticula and terminology problems

ObjectivesOur aim was to evaluate congenital left ventricular wall abnormalities (clefts, aneurysms and diverticula), describe and illustrate imaging features, discuss terminology problems and determine their prevalence detected by cardiac CT in a single center. Materials and methodsCoronary CT angiography images of 2093 adult patients were evaluated retrospectively in order to determine congenital left ventricular wall abnormalities. ResultsThe incidence of left ventricular clefts (LVC) was 6.7% (141 patients) and statistically significant difference was not detected between the sexes regarding LVC (P=0.5). LVCs were single in 65.2% and multiple in 34.8% of patients. They were located at the basal to mid inferoseptal segment of the left ventricle in 55.4%, the basal to mid anteroseptal segment in 24.1%, basal to mid inferior segment in 17% and septal–apical septal segment in 3.5% of cases. The cleft length ranged from 5 to 22mm (mean 10.5mm) and they had a narrow connection with the left ventricle (mean 2.5mm). They were contractile with the left ventricle and obliterated during systole. Congenital left ventricular septal aneurysm that was located just under the aortic valve was detected in two patients (0.1%). No case of congenital left ventricular diverticulum was detected. ConclusionCardiac CT allows us to recognize congenital left ventricular wall abnormalities which have been previously overlooked in adults. LVC is a congenital structural variant of the myocardium, is seen more frequently than previously reported and should be differentiated from aneurysm and diverticulum for possible catastrophic complications of the latter two.

Long-Term Prognosis of Adult Patients With Isolated Congenital Left Ventricular Aneurysm or Diverticulum and Abnormal Electrocardiogram Patterns

Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies frequently associated with electrocardiogram (ECG) abnormalities. The aim of this study was to evaluate the long-term prognosis in such patients. A total of 108 patients with LVA or LVD having ECG-abnormalities were assessed. The patients were classified into 2 groups according to ECG abnormalities: a distinct ECG group (8 ECG patterns known to be frequently associated with LVA/LVD); and a control group (all other ECG abnormalities). The primary endpoint was a composite of cardiac death, rhythm disturbances, syncope, embolic events, and hospitalization for cardiovascular events. Mean patient age was 64±10 years; 45 (42%) were male; median follow-up (FU) was 50 months. The primary endpoint occurred in 12/27 patients from the distinct ECG group and in 15/81 patients in the control group (44% vs. 19%; P=0.01). Cardiac event rate per year (CER) was 1.8% vs. 0.8%, respectively. There were no cardiac deaths during FU. Symptoms (arrhythmia-related symptoms, syncope, and embolic events) at time of diagnosis increased the incidence of adverse events during FU (70% vs. 28%; P=0.05; CER 2.9% vs. 1.1%). Age ≥64 years, presence of LVD, gender, and location of the anomaly did not affect the incidence of adverse events. The incidence of adverse events in symptomatic patients with isolated LVA or LVD and distinct abnormal ECG patterns is increased during long-term FU. None of the present patients, however, experienced cardiac death.

Arrhythmic Manifestations in Patients With Congenital Left Ventricular Aneurysms and Diverticula

Congenital left ventricular aneurysms and diverticula (LVA/Ds) are rare cardiac malformations that can be detected using echocardiography or other imaging techniques. Some of these patients present with ventricular arrhythmias. This study investigated clinical characteristics of patients with congenital LVA/D presenting with arrhythmic manifestations. Over the previous 20 years 250 patients were diagnosed to have congenital LVA/D at our institution. Diagnosis was made using echocardiography after exclusion of coronary artery disease, local cardiac inflammatory processes, traumatic causes, or cardiomyopathies. At initial presentation 32 of the 250 patients (13%, average age 45 years, range 25 to 65, 21 men and 11 women) exhibited arrhythmias. At least 2 LVA/Ds were present in 6 of these patients. LVA/Ds were localized at the posterobasal, apical, anteroseptal, and anterolateral walls in 12, 11, 4, and 5 patients, respectively. The most common complaints at presentation were syncope or presyncope in 18 patients and palpitations in 11 patients. One patient had survived sudden cardiac death. Long-term electrocardiographic recordings showed ventricular tachycardia (VT) or ventricular fibrillation in 17 patients (53%). Twelve patients underwent electrophysiologic testing. Nine patients had inducible ventricular tachyarrhythmia, whereas induced tachycardia was similar to that during spontaneous arrhythmia in 7 patients. In conclusion, patients with congenital LVA/Ds who present with arrhythmic manifestations commonly have VT. Electrophysiologic testing can reproduce clinical VT in most of these patients.

Fetal Diagnosis of Left Ventricular Aneurysm: A Case Report

Congenital left ventricular aneurysm is a rare but potentially lethal condition. We describe a case of isolated congenital left ventricular aneurysm diagnosed at 28 weeks' gestation. In addition to standard imaging, we utilized color-coded transthoracic tissue Doppler techniques to further evaluate the aneurysm postnatally.

Prevalence and Spectrum of Coronary Artery Anomalies in Patients With an Isolated Congenital Left Ventricular Aneurysm or Diverticulum

Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies and frequently associated with other cardiac anomalies. The objective of our study was to investigate the prevalence and the spectrum of coronary anomalies in such patients. The incidence of coronary anomalies is increased in patients with LVA or LVD. We assessed 117 patients with isolated LVA or LVD for the prevalence of coronary anomalies and compared the findings with an age- and sex-matched control group (n = 117) without the diagnosis of LVA or LVD. Coronary anomalies were present in 58.1% of the study population (68 of 117). The median age of affected patients was 64 years, and 45 (38.5%) were male. Coronary anomalies were more prevalent in patients with LVA or LVD (58.1% vs 6.8%, P < 0.001), male patients (89% vs 57%, P = 0.0002), and in patients with nonapical location of LVA or LVD (24% vs 45%, P = 0.02) compared with control, whereas age and type (LVA vs LVD) had no influence (57% vs 57.6%, P = 0.4; and 58.8% vs 57.6%, P = 0.87, respectively). The number of adverse cardiac events was similar in both groups during a 4.2-year follow-up period (29% vs 19%, P = 0.09). None of the patients in our series had major coronary anomalies with potential lethal consequences. This large single-center study suggests that the prevalence of abnormal coronary-artery anatomy in patients with isolated LVA or LVD is as high as 58.1%. However, we did not identify major coronary anomalies with potential lethal consequences, and the clinical course during follow-up was not influenced by the presence or absence of coronary anomalies.

Modified Damus-Kaye-Stansel/Dor Procedure for a Newborn With Severe Left Ventricular Aneurysm

Congenital left ventricular aneurysm (CVA) is a rare cardiac malformation. The prognosis is variable, depending on such factors as the size in comparison to the ventricular cavity, signs of heart failure, arrhythmia and so on. Most infants and young children with large aneurysm showed poor clinical outcomes. Here, we report the case of patient who was prenatally diagnosed with a large CVA, who had severe left ventricular dysfunction at 21 weeks' gestation for which she successfully underwent a modified Damus-Kaye-Stansel/Dor procedure.

Normal Coronary Artery Patient Presenting with Left Ventricular Aneurysm

Left ventricular aneurysm (LVA) is one of the most important complications of myocardial infarction LVA is strictly defined as a distinct area of abnormal left ventricular diastolic contour with systolic dyskinesia or paradoxical bulging. LVA usually results from myocardial infarction. Other rare aetiologies of LVA include hypertrophic cardiomyopathy, Chagas' disease, sarcoidosis, congenital LVA, and idiopathic However, LVA formation in patients with idiopathic dilated cardiomyopathy is rarely reported, and the incidence, clinical features, and pathogenesis of LVA formation in patients with idiopathic dilated cardiomyopathy is not well understood. Here, we present a 45 years old, idiopathic dilated cardiomyopathy patient with LVA and normal coronary arteries The pathogenesis of LVA formation in patients with idiopathic dilated cardiomyopathy is not clear. One acceptable hypothesis is that coronary artery emboli originate from mural thrombi, present in some patients with idiopathic dilated cardiomyopathy, which develop due to local wall infarction and fibrosis. The local myocardial perfusion differences could be seen in idiopathic dilated cardiomyopathy and predominantly found in the anteroposterior axis of the left ventricle. Local fibrosis occurs more frequently on the anterior wall or posterior wall, and less frequently on the lateral or septal wall. In our patient, LVA existed in the septal segments.We could not define the exact mechanism of the septal aneurysm in our patient but we decided to present this abnormal case, which is different from cases thus far reported in the literature.

Congenital left ventricular aneurysm: a cause of impaired myocardial torsion and peripheral thrombo-embolic events

Congenital aneurysms of the left ventricle (LV) are rare cardiac abnormalities and in most instances, are asymptomatic. However, some patients may present cardiac rupture, tamponade, ventricular arrhythmias, and eventually sudden death. Herein, we describe a case of a 64-year-old male patient who was hospitalized for critical limb ischaemia because of an acute embolic event. Transthoracic contrast echocardiography revealed a congenital aneurysm of the LV apex with a small thrombus and a reduced LV ejection fraction. Speckle tracking imaging showed an impaired myocardial torsion. Diagnosis was confirmed after surgical resection of the aneurysm. LV torsion and ejection fraction were normalized after surgery.

Congenital Subaortic Left Ventricular Aneurysm Diagnosed by 64-slice Multidetector-Row Computed Tomography

In this report, we describe a very rare case of a congenital subaortic left ventricular aneurysm in an 80-year-old patient. Cardiac computed tomography imaging is a reliable and noninvasive tool for diagnosis and follow-up of subaortic aneurysms.

Echocardiographic Findings on Congenital Left Ventricular Diverticulum and Aneurysm: Two Rare Cases and Literature Review

Congenital diverticulum and aneurysm are rare cardiac abnormalities. Given their very low incidence especially in adult patients and the few cases reported, the diagnosis might be ignored when the cardiac malformation is seen for the first time. This article presents two adult cases of congenital diverticulum and aneurysm, which were first detected by echocardiography. The relevant literature is reviewed.

No value of the ECG in congenital left ventricular aneurysms and diverticula?

This editorial refers to ‘Prevalence and spectrum of abnormal electrocardiograms in patients with an isolated congenital left ventricular aneurysm or diverticulum’ by M.-A. Ohlow et al. ,o n page 1689. In this issue of the journal, Ohlow et al. 1 present their experience regarding the value of the electrocardiogram (ECG) in diagnosing congenital left ventricular diverticulum or aneurysm. Both are rare disorders of the left ventricular wall; the former being a finger- or a hook-like appendix emerging from the wall of the left ventricle, contracting in synchrony with the left ventricle and with a narrow connection to the left ventricular cavity. 2 In contrast, congenital aneurysms have a wide connection to the left ventricle and look similar to acquired aneurysms of the ventricular wall. Their size may vary from 0.5 to 9 cm and they are located most frequently in the apex or the perivalvular area. There is a frequent association with other congenital cardiac malformations, most frequently ventricular and atrial septal defect, patent foramen ovale and tricuspid atresia, and vascular or non-vascular extracardiac abnormalities. The clinical course of isolated congenital aneurysms and diverticula is usually benign, but ventricular arrhythmias and sudden cardiac death, systemic emboli, rupture with cardiac tamponade, mitral regurgitation, or compression of the left coronary artery in case of perivalvular localization have been reported. Recent data from the same authors 3 suggest a 20-fold higher prevalence than reported in the literature and a male preponderance for aneurysms; women were more likely to have diverticula. Embolic events were more frequent in patients with diverticula. Arrhythmias occurred more frequently in both conditions than in the control group. In this study, the ECG abnormalities in congenital aneurysms and diverticula are described. For this purpose, standard 12-lead ECGs of 125 patients were studied and compared with ECGs of age- and gender-matched controls. In this relatively old population (the mean age was 66 years), ECG abnormalities were found in 56.8% of cases. More specifically, inverted T waves . 2m m in � 2 leads, abnormal Q waves, slow R progression in the precordial leads, (in)complete right bundle branch block, an early repolarization pattern, a prolonged PR-interval, and atrial fibrillation were more prevalent in the study group. More frequently, abnormalities were found in diverticula (38.2 vs. 15.8%) and in the apical localization of the aneurysm (32 vs. 16%), and there was a trend for more abnormal ECGs at older age. This study, reporting on the ECG in congenital left ventricular aneurysms and diverticula, is important, being the largest one until now and based on an impressive, single centre, coronary angiography database. Although ECG findings were abnormal in the majority of cases, the sensitivity, specificity, and positive and negative predictive value were disappointing. The authors conclude that the ECG has little value as a screening tool to diagnose congenital aneurysms or diverticula. These findings, however, are not unexpected: although an ECG is an excellent tool to diagnose arrhythmias, ischaemia, and electrical disease (such as long QT syndrome and Brugada syndrome), it is much less appropriate to diagnose structural changes of the heart. This does not mean, however, an ECG is not useful for identifying structural or prognostic features. Unfortunately, in the present study, no detailed information on electrocardiographic and structural or clinical associations was provided. Other study limitations, some acknowledged by the authors, are partly due to the retrospective study design. The study population has been retrieved from the coronary angiography database, leading to inclusion bias because individuals with anginal or arrhythmia complaints were more likely to be included. It is, therefore, unclear how representative the results are for both anomalies in the general population. Abnormal ECGs might have further contributed to the selection of patients for coronary angiography, influencing the results obtained in the study. The study population consists of adults with a mean age of 66 years; therefore, children are not represented. Because more pronounced manifestations of both conditions exist in children, including those that could result in more frequent and possibly more specific ECG abnormalities than described in the present study. Inherent to the patient selection is the underrepresentation

Prevalence and spectrum of abnormal electrocardiograms in patients with an isolated congenital left ventricular aneurysm or diverticulum

Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies and can be associated with ECG abnormalities and rhythm disturbances. We sought to investigate the prevalence and the spectrum of ECG abnormalities in such patients. We assessed 125 patients with isolated LVA or LVD for the prevalence of ECG abnormalities and compared the findings to an age- and gender-matched control group. The 12-lead ECG patterns were evaluated according to commonly used criteria and were classified into three subgroups (distinct, mildly, and minor). Fifty-four of the 125 patients (43.2%) had normal and 71 (56.8%) abnormal ECGs. Mean age was 66 years. Forty-nine (39.2%) were male. Distinct abnormal ECG patterns were more prevalent in patients with LVD (38.2 vs. 15.8%, P = 0.04), and apical location of the anomaly (36.6 vs. 16.6%, P = 0.02). Older age (>66 years) was associated with a trend for a higher prevalence of abnormal ECG pattern (33 vs. 18%, P = 0.06), whereas gender had no influence (32 vs. 16%, P = 0.14). This study also shows that the sensitivity, specificity, positive predictive value and negative predictive value of a 12-lead ECG for the diagnosis of LVA or LVD are low. This large single-centre study suggests that the prevalence of abnormal ECG patterns in patients with isolated LVA or LVD is as high as 56.8%. However, ECG is not specific and sensitive to be used as a screening tool in such patients.

Evaluation of congenital left ventricular aneurysm in the fetus by velocity vector imaging.

We report a case of left ventricular aneurysm evaluated using the velocity vector imaging (VVI) technique, an angle-independent method used to calculate the tissue velocity strain and strain rate on routine gray scale two-dimensional images and display the velocities of endocardial points as vectors overlaid on B-mode images. It uses a combination of speckle tracking and complex geometric analysis, allowing tracing of myocardial activity throughout the cardiac cycle. In this case, evaluation of the myocardial dynamics in the fetus by VVI was started at 27weeks of gestation, and both the systolic and diastolic velocities and the ejection fraction in the aneurysmal segment were less than in the other segments. VVI imaging at 35weeks of gestation showed this difference even more clearly. Postnatal VVI examination showed no significant difference between results for the systolic and diastolic velocities or ejection fraction and those obtained at the prenatal VVI examination. VVI seemed to be as useful at this time, especially for assessing ventricular systolic function, as it was for postnatal examination.

Response to Letter Regarding Article, “Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy”

We thank Dr Giuseppe Ando et al for their interest in our description of left ventricular (LV) apical aneurysms occurring in patients with hypertrophic cardiomyopathy (HCM), a newly recognized and important subgroup within this heterogeneous disease spectrum.1 Ando et al have raised a number of important issues relative to nomenclature, mechanism, and the treatment of LV apical aneurysms in HCM patients. With respect to nomenclature, we agree that the term diverticula should be …

Congenital left ventricular subaortic aneurysm in an asymptomatic child

A newborn was referred to our department, due to the presence of a cardiac murmur. Echocardiographic study showed a small membranous ventricular septal defect and a yuxta-aortic mass. In the follow-up, patient remained asymptomatic, ventricular septal defect closed, and yuxta-aortic mass increase in size with somatic growth. A transesophageal echocardiography was carried out, showing a fibrotic and dyskinetic left ventricular subaortic aneurysm of 19×18 mm. Given that the patient remained asymptomatic, a conservative approach was adopted. Congenital left ventricular subaortic aneurysm is an infrequent entity, and rupture, endocarditis, coronary compression, thromboembolism, arrhythmias and aortic insufficiency are some of its complications, but, as in our patient, if aneurysm is not associated with complications, patient is asymptomatic, and aneurysm growth is not very important, a conservative approach can be adopted, with a close surveillance.

Prevalence and Outcome of Congenital Left Ventricular Aneurysms and Diverticula in an Adult Population

Objectives: We sought to investigate the prevalence and clinical outcome of congenital left ventricular aneurysms (LVAs) and diverticula (LVD) in a large adult population. Methods: We retrospectively studied the left ventricular angiograms of 12,271 consecutive patients undergoing cardiac catheterization at our institution and analyzed the medical records of the patients. Results: The overall prevalence of LVA/LVD was 0.76% (94 patients): there were 42 patients with LVA (0.34%) and 52 patients with LVD (0.42%). Men were more likely to have LVA (62 vs. 38%), and women were more likely to have LVD (75 vs. 25%; p = 0.001 for both). The mean time of follow-up was 56 ± 6 months and was focused on clinical events, rehospitalization and survival. Mortality was 6% with no cardiac death. Patients with LVD were significantly more likely to have embolic events (p = 0.04). Patients with LVA and LVD were more likely to have rhythm disturbances compared with controls (p = 0.01 for both). Incidence of syncope was not different in both groups (p = 0.4 and 0.12, respectively). There was no reported incidence of rupture. Conclusion: This large single-center study suggests that the prevalence of LVA/LVD in adults is up to 20-fold higher than previously reported. One third of the affected patients in our series had nonfatal cardiovascular events during follow-up, with a predominance of embolic events in the LVD group.

Usefulness of Magnetic Resonance Imaging Evaluation of Congenital Left Ventricular Aneurysms

Congenital left ventricular (LV) aneurysm is a rare malformation of unknown cause that is often associated with a poor prognosis. This study was undertaken to evaluate the usefulness of cardiac magnetic resonance imaging in patients with congenital LV aneurysms and to determine the relation between clinical manifestations and the morphologic and functional characteristics of the aneurysms. Among the 26 consecutive patients with congenital LV aneurysms included, the anomalies involved the apex or free wall in 15 and the submitral myocardium in 11. Cardiac magnetic resonance provided detailed anatomic and functional assessment of the aneurysms in all patients. Compared with submitral aneurysms, apical or free-wall aneurysms were larger (24 +/- 29 vs 3 +/- 2 ml/m(2), p = 0.02), were more frequently associated with scar tissue by myocardial delayed enhancement imaging (71% vs 0%, p = 0.03), and tended to be more commonly associated with symptoms (53% vs 18%, p = 0.08). Aneurysm volume but not location correlated with LV size (r = 0.735, p <0.0001) and the ejection fraction (r = 0.774, p <0.0001). Apical or free-wall aneurysms were resected in 5 patients, with no mortality. There was 1 death after aortic valve replacement in a patient with type B Niemann-Pick disease, which was not clearly related to the LV aneurysm. The remaining 25 patients are alive at a median age of 13.5 years. In conclusion, the experience with this cohort illustrates that cardiac magnetic resonance is well suited for the morphologic and functional evaluation of congenital LV aneurysms.