Congenital Left Ventricular Aneurysm Research Articles

Abstract P1115: Epicardial Signaling Regulates Myocardial Contractility Via Mirna

Background: A congenital left ventricular aneurysm is a rare cardiac malformation that maycause heartfailure, ventricular wall rupture, ventricular arrythmia or sudden cardiac death. However, mechanisms of congenital left ventricular aneurysm are not known. Paracrinesignals from cardiomyocytes, such as a cardiokine, prokineticin-2, can activate EPDCsdifferentiation in adult heart to promote neovascularization. How the reciprocal interaction viaprokineticin and miRs from epicardium to cardiomyocytes affect heart growth and function,and how EMT-related events contributecardiomyocyte proliferation and function areunknown. MicroRNA (miR124) has an important role in the adult heart, however, its role incardiac development is unknown. Results: Here in this study, by deletion of a prokineticin-2 receptor PKR1 through epicardialcell linage tracing at the different developmental stages of mice embryos, we found that thesemice exhibited ventricular rupture and embryonic lethality. Indeed, miR-124 was significantlyupregulated in PKR1 -deficient epicardial cells both in vitro and in vivo , whereas ectopicexpression of these miRs in epicardial cells resulted in the reduced expression of SNAI2 andsubsequently led to impaired EMT and EMT-related events such impaired migration andincreased apoptosis. Wild type mice cardiomyocytes that were cultured in condition mediumderived from PKR1-deficient epicardial cells had a lower beating rate and contractility. Treatment of the cardiomyocytes with miR-124mimic exhibited the same phenotype, that wasrestored by miR-124 inhibitor, indicating involvement of mir124 in epicardium-cardiomyocyte communication and regulation of cardiomyocyte contractility. Conclusion: These finding reveals a key signaling pathway in which a cardiokine,prokineticin-2 promotes EMT to form EPDCs and their migration in an autocrine manner, andsupports myocardial growth and contractility through repressing miR124. Our study mayprovide novel insight in etiology of congenital defects and new strategies to promotemyocardial regeneration.

In Utero Presentation of Left Ventricular Aneurysm.

Congenital left ventricular aneurysm, pseudoaneurysm, and diverticulum are rare entities. These diagnoses can be made pre- and/or postnatally. Although these entities overlap clinically and morphologically, important distinctions can allow for accurate diagnoses. Appropriate diagnosis can be imperative for risk stratification and guidance of prenatal and postnatal management. The case described in the present report highlights a challenging case of a fetal left ventricular aneurysm, management during the prenatal and postnatal periods, and important differentiating features from a ventricular diverticulum and pseudoaneurysm.

Ballooning without Compaction Left Ventricular aneurysms in Noncompaction Cardiomyopathy

Left ventricular non-compaction cardiomyopathy (LVNC) is a congenital cardiomyopathy that occurs as a result of disruption in the early stages of cardiac development. It can occur in isolation, or in conjunction with other rare congenital disorders such as a congenital left ventricular aneurysm. In the presence of LVNC, left ventircular aneurysms are thought to occur due to lack of capillary formation. Overall, there are very limited cases documenting both together. Both conditions can result in arrhythmias, thromboembolic disease, ventricular dysfunction and sudden cardiac death. Diagnosis can be made with echocardiography, although cardiac magnestic resoncnance imaging has become increasingly used for diagnosis. Treatment involves treating heart failure symptoms, arrhythmias and implantation of cardioverter-defibrillators when appropriate. Despite the few case reports on the presence of both conditions occuring simulatenously, recommedenations on treatment, including the use of therapeutic anticoagulation in this patient population needs to be looked into further.

Cauliflower Heart: Congenital Submitral Aneurysm

HomeRadiology: Cardiothoracic ImagingVol. 5, No. 2 PreviousNext Images in Cardiothoracic ImagingCauliflower Heart: Congenital Submitral AneurysmAakash Vijay, Pranav Gupta, Faraz A. Farooqui Aakash Vijay, Pranav Gupta, Faraz A. Farooqui Author AffiliationsFrom the Departments of Cardiology (A.V., F.A.F.) and Radiodiagnosis (P.G.), Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi 110029, India.Address correspondence to F.A.F. (email: [email protected]).Aakash VijayPranav GuptaFaraz A. Farooqui Published Online:Mar 23 2023https://doi.org/10.1148/ryct.220271MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Reference1. Chi NH, Yu HY, Chang CI, Lin FY, Wang SS. Clinical surgical experience of congenital submitral left ventricular aneurysm. Thorac Cardiovasc Surg 2004;52(2):115–116. Crossref, Medline, Google ScholarArticle HistoryReceived: Nov 7 2022Revision requested: Nov 23 2022Revision received: Dec 29 2022Accepted: Feb 8 2023Published online: Mar 23 2023 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 5, No. 2 Metrics Altmetric Score PDF download

Congenital cardiac left ventricular aneurysm revealed by a hydrops foetalis

Prenatal detection of fetal left ventricular aneurysm is rare condition defined by a unilobulated or multilobulated bulging of a ventricular wall segment. The clinical profile, natural course, and treatment remain unclear because these malformations are rare. In view of their infrequency, it would be interesting to report cases in order to have more data concerning the diagnosis, the prognosis and the management. We present a case of left ventricular aneurysm with a hydrops foetalis diagnosed at 21 weeks’ gestation in a 23-year-old primiparous woman who was referred for routine screening ultrasonography. A fetal echocardiogram showed an abnormal four-chambers view with a large cystic lesion on the anterolateral wall of the left ventricle. We found a severe left ventricular dysfunction with a severe mitral regurgitation with an hydrops foetalis with a pericardial effusion, large volume septate ascite and a bilateral pleural effusion. Fetal MRI, in T2 Trufi sequences (axial, coronal and sagittal sections), shows a large segment of the apical left ventricular wall that exhibited a thin-walled outpouching with a wide communication to the left ventricular cavity ( Fig. 1 ). Congenital LV aneurysms (LVA) are rare and poorly understood. Twenty-six reported cases were published until 2014 ( Fig. 2 ). The differential diagnosis is congenital left ventricular diverticula. The prognosis of congenital left ventricular aneurysms is quite variable, depending on size, growth over time, the presence or absence of compression of the fetal lung and signs of cardiac failure.

A rare case report: Intrauterine diagnosis of fetal left ventricular aneurysm

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 of gestation. In addition to standard imaging, we utilized color-coded transthoracic tissue Doppler techniques to further evaluate the aneurysm postnatally. Keywords: Fetal echocardiography, Ventricular aneurysm, Congenital heart disease, Prenatal ultrasound.

VP16.05: Congenital left ventricular aneurysm associated with fetal arrhythmia: evaluation with advanced echocardiographic modalities

VP16.05: Congenital left ventricular aneurysm associated with fetal arrhythmia: evaluation with advanced echocardiographic modalities

Four-dimensional echocardiographic and pathologic findings of congenital left ventricular aneurysm: A case report.

The sonographic and histopathologic features of a rare case of left ventricular aneurysm are reported, detailing the method of prenatal diagnosis and suggesting possible etiology of congenital ventricular aneurysm.

Congenital left ventricular aneurysms and diverticula: an entity in search of an identity.

Congenital left ventricular aneurysm or diverticulum are rare cardiac malformations described in 809 cases since the first description in 1816, being associated with other cardiac, vascular or thoraco-abdominal abnormalities in about 70%. It appears to be a developmental anomaly, starting in the 4th embryonic week. In an experimental study, targeted knockdown of cardiac troponin T in the chick was performed at day 3, after the heart tube has formed. Morpholino treatment of gene TNNT2 at this stage led to the development of left ventricular diverticula (LVD) in the primitive left ventricular wall. Diagnosis of left ventricular aneurysms (LVA)/LVD can be made after exclusion of coronary artery disease, local or systemic inflammation or traumatic causes as well as cardiomyopathies. Clinically, most of LVA and LVD are asymptomatic or may cause systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies (echocardiography, magnetic resonance imaging or left ventricular angiography) visualizing the structural changes and accompanying abnormalities. Mode of treatment has to be individually tailored and depends on clinical presentation, accompanying abnormalities and possible complications, options include surgical resection (especially in symptomatic patients), anticoagulation after systemic embolization, radiofrequency ablation or implantation of an implantable cardioverter defibrillator (ICD) in case of symptomatic ventricular tachycardias, and occasionally combined with class I- or III-antiarrhythmic drugs. Cardiac death occurs usually in childhood, is significantly more frequent in LVA patients and caused by congestive heart failure in most of the cases, whereas patients diagnosed with LVD died more frequently from rupture of the LVD.

Coexistence of congenital left ventricular aneurysm and prominent left ventricular trabeculation in a patient with LDB3 mutation: a case report

BackgroundThe coexistence of congenital left ventricular aneurysm and abnormal cardiac trabeculation with gene mutation has not been reported previously. Here, we report a case of coexisting congenital left ventricular aneurysm and prominent left ventricular trabeculation in a patient with LIM domain binding 3 gene mutation.Case presentationA 30-year-old Asian man showed paroxysmal sinus tachycardia and Q waves in an electrocardiogram health check. There were no specific findings in physical examinations and serological tests. A coronary-computed tomography angiography check showed normal coronary artery and no coronary stenosis. Both left ventricle contrast echocardiography and cardiac magnetic resonance showed rare patterns of a combination of an apical aneurysm-like out-pouching structure with a wide connection to the left ventricle and prominent left ventricular trabecular meshwork. High-throughput sequencing examinations showed a novel mutation in the LDB3 gene (c.C793>T; p.Arg265Cys).ConclusionsOur finding indicates that the phenotypic expression of two heart conditions, congenital left ventricular aneurysm and prominent left ventricular trabeculation, although rare, can occur simultaneously with LDB3 gene mutation. Congenital left ventricular aneurysm and prominent left ventricular trabeculation may share the same genetic background.

P2105Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: analysis of 809 cases published since 1816

P2105Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: analysis of 809 cases published since 1816

Submitral Congenital Left Ventricular Aneurysm: You Recognize Only What You Know.

Submitral Congenital Left Ventricular Aneurysm: You Recognize Only What You Know.

Heart beats: not to be beaten.

Heart beats: not to be beaten.

Endovascular catheter ablation of ventricular tachycardia in a patient with a surgically repaired congenital left ventricular aneurysm.

We present a patient with a congenital left ventricular aneurysm who visited our outpatient clinic for a routine check-up and, during this visit, lost consciousness due to sustained ventricular tachycardia. In our patient, endocardial mapping revealed extensive conduction abnormalities, and successful ablation was accomplished at the endocardial surface.

Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: Analysis of 809 cases published since 1816

BackgroundCongenital left ventricular aneurysm (LVA) or diverticulum (LVD) is rare cardiac anomalies. We aimed to analyse the clinical characteristics and outcome in all ever published patients. MethodsMEDLINE, Web of science, Google and EMBASE, and reference lists of relevant articles were searched for publications reporting on LVA or LVD patients. ResultsWe identified 809 patients published since 1816 [354 (49.1%) LVA, 453 (50.6%) LVD, 2 (0.3%) both]. Mean age at diagnosis was 34.1±27 (LVA) and 29.7±27.6years (LVD; p=0.05). 48.9% were male. LVA was larger (38.7±22.5mm versus 31.4±21.2mm; p=0.002) and frequently found in submitral location (33% versus 4.9%; p<0.001), LVD was frequently located at the LV-apex (61.2% versus 28.7%; p<0.001). LVD was often associated with cardiac (34.2% versus 11%; p<0.001) or extracardiac anomalies (32.7% versus 3%; p<0.001). LVA patients presented more frequently with ventricular tachycardia/fibrillation (18.1% versus 13.1%; p=0.01), the incidences of rupture (4% versus 4.5%; p=0.9), syncope (8.3% versus 5.1%; p=0.1), and embolic events (4.9% versus 3.6%; p=0.4) at presentation were not different between LVA and LVD.Mean follow-up was 56.3±43months. Cardiac death occurred more frequently in the LVA group (11.5% versus 5.0%; p=0.05) at a median age of 0.8 [LVA] and 2.5 [LVD] years. The leading cause of cardiac death was congestive heart failure in the LVA-group (50.0% versus 0.0%; p=0.01), and rupture in the LVD-group (75.0% versus 27.3%; p=0.04). ConclusionsLVA and LVD are distinct congenital anomalies with different clinical and morphological characteristics. The prognosis of LVA is significantly worse during long-term follow-up.

Incidence and Prognosis of Ventricular Arrhythmias in Patients with Congenital Left Ventricular Aneurysms or Diverticula

BackgroundPatients with congenital left ventricular aneurysms and diverticula may present with arrhythmia. The incidence of ventricular arrhythmias and the clinical outcome of these patients have not been reported to date. MethodsAmong 250 consecutive patients with congenital left ventricular aneurysms and diverticula detected by echocardiography, the clinical outcome of patients who presented with ventricular arrhythmias or associated symptoms was investigated. ResultsOf 250 patients with congenital left ventricular aneurysms and diverticula, 30 had ventricular arrhythmias or syncope at initial presentation. During a follow-up of 85 months, spontaneous ventricular tachycardia occurred in 17 of these patients (57%). Ventricular tachycardia was sustained in 13, with a monomorphic pattern in 9 patients. In 82% (11 patients), ventricular tachycardia was inducible during electrophysiologic testing. In 7 patients a sustained monomorphic ventricular tachycardia with a right bundle branch block pattern similar to the clinical tachycardia was induced. Twenty patients were treated with antiarrhythmic agents. Eleven patients received an implantable cardioverter defibrillator. Appropriate device discharges were observed in 73% during a follow-up of 61 months. One patient underwent surgical resection of a congenital left ventricular aneurysm. Three patients underwent successful catheter ablation for incessant ventricular tachycardia. Of these, 2 were free of any clinically relevant arrhythmia during follow-up. Three patients died (10, 41, and 89 months after initial presentation). In 2 of them, the cause of death was attributed to ventricular arrhythmia. ConclusionThe clinical outcome of patients with congenital left ventricular aneurysms and diverticula and arrhythmia is variable. Clinical ventricular tachycardia in these patients is often monomorphic and usually inducible during electrophysiologic study, indicating a role for this test in risk stratification. Appropriate discharges are frequent in implantable cardioverter defibrillator recipients with congenital left ventricular aneurysms and diverticula.

Unusual cause of chest pain mimicking acute myocardial infarction: congenital left ventricular aneurysm.

A 36-year-old man without any cardiac history presented to the emergency room with ongoing chest pain. The Electrocardiography (ECG) showed diffuse ST segment elevations on the anterior leads compatible with acute anterolateral wall myocardial infarction [Table/Fig-1]. The patient was a current smoker with a family history of coronary artery disease (CAD). He had a stable hemodynamic status with normal blood pressure and pulse rate. Initial examination revealed mild systolic murmur along the left sternal border as well as lateral displacement of the apical pulse. Lung auscultation was completely normal and all peripheral pulses were palpable. He was evaluated with transthoracic echocardiography that showed reduced ejection fraction of 38% with hypokinesis of the anterior wall along with a diffuse aneurysmal apical segment [Table/Fig-2]. Depending on the high clinical suspicion of acute myocardial infarction, the patient underwent emergency coronary angiography that revealed normal coronary arteries. In order to further define the anatomy, cardiac magnetic resonance imaging (MRI) was performed [Table/Fig-3][Video-1 and 2]. Left ventricular cavity was seen to expand at the apical level associated with thinning of the myocardium concordant with a true aneurysm. The aneurysmal pouch had a size of 4x5 cm without any thrombus inside. The myocardium did not display contrast enhancement excluding evidence for fibrosis or scar tissue. The wall thickness at the apex was measured as 2 mm showing a risk for ventricular rupture. Since there was no history of trauma or recent infarction, the patient was estimated as having a congenital aneurysm. As the aneurysmal sac was a substrate for arrhythmia and thrombus formation, it was decided to be surgically repaired. The patient underwent a Dor procedure represented by reconstruction of the ventricular cavity with endoventricular patch-plasty [1]. Perioperative course was free of complications. The patient was discharged uneventfully provided that the left ventricular volumes were reduced along with improvement in systolic function. [Table/Fig-1]: 12-lead ECG of the patient on presentation showing marked ST elevations on the D1-aVL and V4-6 derivations [Table/Fig-2]: Transthoracic echocardiographic image of the apical aneurysm shown by asterisk in the apical four-chamber view [Table/Fig-3]: Cardiac magnetic resonance image showing thinning of the myocardium at the apex (arrows) along with an aneurysmal pouch (asterisk) This case appears to call attention to a rare cardiac malformation that can present to the emergency room with typical signs and symptoms of acute myocardial infarction. In the literature, congenital left ventricular aneurysms have a wide-spectrum of presentations [2,3]. Although mostly asymptomatic [4], life-threatening ventricular arrhythmias [5], embolic events due to thrombus formation [6] and sudden cardiac death due to rupture [7] have been reported so far. In our case, CAD risk factors of the patient as well as the ECG findings led to an initial misdiagnosis of acute myocardial infarction.The precise diagnosis of a congenital left ventricular aneurysm in such a case necessitates demonstration of the true aneurysm by cardiac MRI and exclusion of CAD by angiography.

Usefulness of 3D Transthoracic Echocardiography in the Diagnosis of Congenital Left Ventricular Aneurysm

A 29 year-old male without any risk factors for coronary artery disease presented with symptomatic sustained ventricular tachycardia. 2-Dimensional (2 D) transthoracic echocardiogram(TTE) did not reveal any abnormality. When we did a 3-Dimensional(3 D) echocardiogram, a small left ventricular(LV) aneurysm was detected near the outflow tract. After catheter ablation, the patient was discharged without any anti-arrhythmic therapy.

Congenital Left Ventricular Outpouchings: A Systematic Review of 839 Cases and Introduction of a Novel Classification after Two Centuries

Congenital left ventricular outpouchings (LVOs) are reported under five overlapping and poorly defined terms including left ventricular accessory chamber, left ventricular aneurysm (LVA), left ventricular diverticulum (LVD), double-chambered LV, and accessory left ventricle. Diagnostic criteria are frequently mixed and not mutually exclusive. They convey no information regarding treatment strategy and prognosis. The aim of this systematic review is to provide a clear and inclusive classification, with therapeutic and prognostic implications, for congenital LVOs. We performed three separate sets of search on three subjects including "congenital left ventricular outpouchings," "important and simply measurable markers of left ventricular function," and "relationship of mechanics of intraventricular blood flow and optimal vortex formation in left ventricle and elliptical geometry of LV." We enrolled case series, review articles, and case reports with literature review. All types of acquired LVO's were excluded. We studied the abstracts of all searched articles. We focused on diagnostic criteria and patients' outcome. To examine the validity and reliability of the novel classification, fifteen previous studies were revisited using the novel classification. A total of 20 papers from 11 countries fulfilled our inclusion criteria. The age of patients ranged from prenatal age to geriatric age range. Diagnostic criteria were clearly stated only for two of the above five terms (i.e., congenital LVA and congenital LVD). Cases with mixed diagnostic criteria were frequent.Elliptical geometry of left ventricle was found to have significant impact on effective blood flow mechanics in LV. A simple inclusive classification for congenital LVOs, with therapeutic and prognostic implications, was introduced. The cornerstone of this classification is elliptical LV geometry. Large-type IIc LVO have dismal prognosis, if left untreated. LVO type I and small LVO type IIa have the best prognosis.

Characteristics and outcome of primary congenital left ventricular aneurysm and diverticulum: analysis of cases from the literature.

Congenital left ventricular aneurysm (LVA) and diverticulum (LVD) are rare cardiac anomalies. We aimed to analyse the characteristics and long-term outcome of prenatal diagnosed patients with LVA/LVD. We performed a systematic review of the literature to identify and analyse patients diagnosed prenatally with LVA/LVD. Twenty-six fetuses with prenatal diagnosis of LVA (50% male subjects) and 16 with LVD (38% male subjects) were identified. Fetal age at diagnosis was 23.8 ± 6.1 weeks. Mean maternal age was 30.6 ± 6.1 years old. LVA was larger (375 ± 237 vs 60 ± 71 mm(2); p = 0.002), and LVA/LVD was frequently observed in an apical location (62%). Associated cardiac/extracardiac defects were reported in 13/42 (31%). Symptomatic patients presented with arrhythmias (LVA 16% vs LVD 0%), hydrops fetalis (LVA 27% vs LVD 18.8%), or rupture (LVA 4% vs LVD 38%; p = 0.02). Size changes of the LVA/LVD during pregnancy were reported in 17%. Fetal death occurred in nine cases (21.4%); termination of pregnancy was performed in six cases (14.3%). All other babies were delivered at term. During follow-up (mean 29.1 ± 38.2 months), the rate of adverse events was significantly increased in patients with LVA (40 vs 0%; p = 0.04) CONCLUSION: The rate of fetal complications including fetal death is high. Long-term outcome among the survivors is significantly worse in patients with LVA.