Double-chambered Left Ventricle Research Articles

Double-chambered left ventricle: diagnosis by CMR and review of the literature

BackgroundA double-chambered left ventricle (DCLV) is an extremely rare congenital malformation. The exact prevalence of DCLV is not known, although studies have reported prevalence of 0.04–0.42%. This abnormality is characterized by the sub-division of left ventricle into two chambers, the main left ventricular chamber (MLVC) and the accessory chamber (AC) by a septum or muscle band.Case presentationWe are reporting two cases of DCLV, one in an adult male and an infant, who were referred for undergoing cardiac magnetic resonance (CMR) imaging. The adult patient was asymptomatic, whereas the infant had the diagnosis of left ventricular aneurysm on fetal echocardiography. On CMR, we confirmed the diagnosis of DCLV in both patients, as well as moderate aortic insufficiency in the adult patient. Both patients were lost to follow-up.ConclusionsThe double-chambered left ventricle (DCLV) is commonly detected in infancy or childhood. Although echocardiography can help detect double-chambered ventricles, MRI provides a better knowledge of this problem and can also be used to diagnose other related heart disorders.

Double-chambered left ventricle in a patient with incomplete Shone complex

Double-chambered left ventricle in a patient with incomplete Shone complex

From Double-Chambered Right Ventricle to Double-Chambered Left Ventricle: Unusual Evolution of a Ventricular Septal Defect

We present the first imaging registry of the progressive isolation of an apical chamber of the right ventricle caused by the hypertrophy of the moderator band generated from the hemodynamic effect of a ventricular septal defect, leaving the apex of the right ventricle as an accessory chamber of the left ventricle. (Level of Difficulty: Advanced.)

Double chambered left ventricle-Two chambers are not better than one.

In this month's Journal,1 Ali and colleagues present a case series consisting of two adults who underwent surgical resection for a double chamber left ventricle (DCLV). The first case was an 18-year-old female with several years of progressive chest pain and dyspnea who was ultimately found to have both a double chamber right ventricle (DCRV) and DCLV after transthoracic echocardiography (TTE) and cardiac catheterization. She underwent surgical resection of the fibromuscular bands in both ventricles with an uncomplicated postoperative course. The second case was a 27-year-old male with a history of supravalvular aortic stenosis that had been previously addressed who on TTE was found to have aortic regurgitation, aortic stenosis, and DCLV with elevated left ventricular outflow tract and accessory chamber (AC) gradients. He underwent mechanical aortic valve replacement, ascending aorta replacement, and muscle bundle excision with a course notable for delayed sternal closure and retroperitoneal hematoma. Both patients had technically successful operations with band resection and appropriate biventricular function confirmed on TTE. Both survived their index operations and had resolution of their symptoms, with the second patient dying at 6 months due to complications from anticoagulation. The authors should be congratulated for the successful identification and operative treatment of these patients. DCLV is an incredibly rare diagnosis and is even less common than its counterpart DCRV—which is seen in less than 2% of all congenital cardiac anomalies.2 Ali and colleagues successfully differentiated DCLV from other similar pathologies such as left ventricular aneurysms and left ventricular diverticulum. This is critical given their difference in etiology and overall management. Those with asymptomatic DCLV do not require surgical intervention. Symptoms are rare but can present as fatal tachyarrhythmias or heart failure. Patients can also be found to have mid-ventricular obstructions, particularly those with superior–inferior arrangements according to Bao et al.3 Those who are intervened upon typically undergo resection with patch reconstruction or cardiac transplant for large communications.4 Furthermore, diagnosing DCLV can be a challenge in and of itself. Some patients, including the second case of this study, have previous imaging that does not confirm the presence of DCLV.5 This just reiterates the importance of having a high clinical suspicion. Nevertheless, TTE is typically the first test performed and serves as a fairly reliable starting point. Bao et al.3 showed that in their series of nine patients, eight were correctly diagnosed during their initial TTE. Although a tissue diagnosis would be the best confirmatory test, a second imaging modality is typically used to confirm the diagnosis. The authors used cardiac catheterization and magnetic resonance imaging (MRI) in their first and second case, respectively, as their second modality of choice. The advantages of cardiac MRI are stated throughout the literature. It offers high inherent contrast resolution, good spatial and temporal resolutions, a wide field of view, multiplanar imaging capabilities, and the absence of radiation.4, 6 Gadolinium is particularly useful as a similar enhancement of the AC wall compared to the remainder of the left ventricular myocardium supports the diagnosis of DCLV.7 We (Figure 1) commend the authors on their work with this complex patient population. Both cases presented were unique and challenging. The authors demonstrated the ability to address DCLV in addition to the patient's existing congenital abnormalities. Furthermore, they demonstrate the ability to do so through both a transaortic and transmitral approach. DCLV is an incredibly rare diagnosis, making it difficult to establish practice guidelines. One should always pause when deciding when and how to operate on these patients. Still, Ali and colleagues add to the body of literature that suggests operating on DCLV can be done safely and that patients may derive a meaningful benefit. This work was supported by a research grant from NHLBI/NIH (No. T32HL007849). The content is solely the responsibility of the authors and does not represent the official views of the National Institutes of Health. The authors declare no conflict of interest.

A TALE OF TWO CHAMBERS: DOUBLE-CHAMBERED LEFT VENTRICLE

A TALE OF TWO CHAMBERS: DOUBLE-CHAMBERED LEFT VENTRICLE

Rare Case of an Adult With Double-Chambered Left Ventricle.

The rare case of an adult with a double-chambered left ventricle was revealed using multimodality imaging using echocardiography and cardiac magnetic resonance imaging in a 38-year-old asymptomatic male patient. The congenital malformation was dominated by a second, coarsely trabeculated muscular shelf dividing the left ventricle into 2 chambers without signs for left ventricular inflow or outflow tract obstruction. The partition wall did not show any signs for intramyocardial fibrosis in late gadolinium enhancement cardiovascular magnetic resonance imaging. Flow measurements excluded a relevant intracardial shunt across the additive perimembranous ventricular septal defect. There were no signs for global right and left ventricular dysfunction with left and right ventricular volumes and ejection fraction within normal limits. A conservative approach was recommended. In summary, we are able to present the case of an adult with a double-chambered left ventricle with a second muscular "septum" partially dividing the left ventricular cavity without causing a relevant impact on cardiac function or clinical signs for heart failure.

Repeated chest tightness—A case of double‐chambered left ventricle in an adult

Double-chambered left ventricle (DCLV) is a rare congenital heart disease. A hypertrophic muscle bundle in the left ventricle may cause varying degrees of obstruction in the middle of the left ventricle, resulting in different clinical symptoms. Here, we report a patient with a history of repeated chest tightness who was misdiagnosed with coronary heart disease and ventricular aneurysm. After repeated ultrasound examinations, the patient was eventually diagnosed with DCLV, which was confirmed by magnetic resonance imaging and coronary angiography. This case highlights the necessity to improve the accuracy of DCLV diagnosis via echocardiogram.

Diagnosis of Double-chambered Left Ventricle of Superior-inferior Type by Echocardiography: A Retrospective Study of 9 Subjects across Two Heart Centers.

Double-chambered left ventricle (DCLV) is extremely rare. Challenges remain in accurate diagnosis of DCLV by echocardiography, because it is easily confused with left ventricular diverticulum, left ventricular aneurysm, ventricular septal defect, etc. Herein, we reviewed echocardiographic characteristics of DCLV and evaluated the accuracy of echocardiographic diagnosis. Clinical and echocardiographical databases of two heart centers were reviewed. Nine children with DCLV of superior-inferior arrangement were studied retrospectively. Normal elliptical geometry of LV cavity is preserved in DCLV. Fibromuscluar bundles separate LV into the main chamber and the accessory chamber. The associated malformations included ventricular septal defects, mitral regurgitation, mitral valve stenosis, pulmonary stenosis, and left ventricular noncompaction in our study. Eight of nine subjects with DCLV of superior-inferior arrangement were diagnosed correctly by initial echocardiographic examination, and one case was mistaken as left ventricular diverticulum. Double-chambered left ventricle could be diagnosed and followed up by echocardiography. The morphological classification is a simplified way to improve diagnostic accuracy.

Double-chambered left ventricle: An unusual finding in a middle-aged woman.

Double-chambered left ventricle (DCLV) is a rare congenital abnormality and is characterized by the subdivision of the left ventricle into two separate chambers by an abnormal septum or muscle band. This rare congenital entity is usually diagnosed in the neonatal or pediatric age. Nevertheless, it can be asymptomatic and incidentally found in the adulthood. A comprehensive multimodality imaging approach is crucial for the correct final diagnosis.

Accessory left ventricular chamber in a cat: multimodality imaging description by cardiac magnetic resonance imaging and echocardiography

A left ventricular accessory chamber is a complex and uncommon phenotype consisting in a subdivision of the left ventricle by a thick-walled muscle bundle or septum into two cavities. Multiple aetiologies such as congenital double-chambered left ventricle and acquired endomyocardial form of restrictive cardiomyopathy have been reported.The endomyocardial form of restrictive cardiomyopathy, owing to its structural heterogeneity, can present a similar phenotype to the congenital abnormality with intraventricular lesions bridging the ventricular septum and left ventricular free wall. Conventional echocardiography is the first-line, accepted, non-invasive imaging modality to investigate underlying cardiac disease but presents limitations for the overall assessment of myocardial tissue. This report describes the use of cardiac magnetic resonance imaging as an additional imaging modality to provide multiplanar morphological, structural, and functional information of the myocardium. In the presented images, hyperintensities on cardiac magnetic resonance imaging within the myocardium along with hyperechoic and heterogeneous myocardial tissue on echocardiography and elevated troponin I were suggestive of a presumptive acquired pathological process such as feline endomyocarditis–left ventricular endomyocardial fibrosis complex, rather than a sole congenital anomaly. Although the diagnosis was not confirmed, this case exemplifies the advantages of using complementary multimodality imaging in a cat presented with a left ventricular accessory chamber.

P1336 Sometimes the heart could appear different if seen under a magnetic resonance scanner: misdiagnosed cases of isolated left ventricle apical hypoplasia and double chambered left ventricle

Abstract Introduction Congenital heart diseases (CHD) are detected in 1% of children, often usually the first year of life; however, many defects are diagnosed later or remain undiagnosed. Both congenital and acquired disorders could affect the left ventricle (LV). First-line assessment includes echocardiography; nonetheless, because of intrinsic or technical limitations and artefacts, further investigation may be required. Cardiac Magnetic Resonance (CMR) is capable of providing anatomical and functional information without many of limitations and drawbacks of echocardiography. We describe CMR findings of misdiagnosed cases of two rare congenital LV abnormalities: isolated LV apical hypoplasia (ILVAH) and double-chambered LV (DCLV). Case report 1 (image A,B,D) An 18 yrs girl diagnosed with dilated cardiomyopathy (DCM) was submitted to our CMR Lab for a functional assessment. After birth, deep Q waves in the inferior leads were noted on the ecg, and an echocardiographic examination showed a dilated and hypokinetic LV. At 1 year of age, she underwent cardiac catheterization that excluded coronary arteries anomalies and confirmed a reduced LV systolic function. A diagnosis of idiopathic DCM was formulated and she was initiated with anticongestive therapy. During the follow-up she felt well with normal exercise tolerance, longitudinal echocardiography did not show any substantial modification over the years. In 2018, a CMR study was performed. Surprisingly, the cardiac apex was formed exclusively by the right ventricle, wrapped around the LV. The LV appeared spherical and truncated inferiorly, and the apical portion was missing; LV volumes and ejection fraction were normal; regional akinesia and subendocardial late gadolinium enhancement (LGE) were evident at the inferior wall. These findings were consistent of ILVAH. Case Report 2 (image C,E,F) A 24 yrs old boy with prenatal diagnosis of LV diverticulum came to our observation for a CMR study. He was asymptomatic, particularly, no palpitations or syncope were referred, nor arrhythmias were detected during the follow-up; exercise tolerance was normal. CMR showed a coarse muscle band in the LV cavity, extending from the apex to the posterior papillary muscle, thus delimiting a contractile accessory chamber. LV volumes and global systolic function were normal. The LV accessory chamber presented a normal structured free lateral wall with a normal systolic thikening, except at the apical infero-lateral segment where it appeared thinned and akinetic and showed LGE with a subendocardial pattern. These findings were consistent of DCLV. Conclusions the LV could be affected by many diseases with different etiological, clinical and morphological features. Compared to other imaging diagnostic modalities, CMR allows better definition of LV morphology, function and tissue characterization, becoming essential for LV abnormalities diagnosis and follow-up. Abstract P1336 Figure.

P1337 Left ventricle outpouchings. Assesing diagnostic complexity

Abstract Congenital left ventricle outpouchings (LVO) are a very rare condition that include left ventricle accessory chamber, left ventricle aneurysm (LVA), left ventricle diverticulum (LVD), double-chambered left ventricle and accessory left ventricle, without well-stablished diagnostic criteria. A recent article by Elaheh Malakan et al tries to show this difficulties and propose an algorithm to help define this different pathologies. We present the case of a 26-day-old boy that was admitted to our Hospital with suspicion of dilated cardiomyopathy. In his physical exam a systolic murmur was detected, and his mother described tiredness during intakes and tachypnea. His ECG showed a sinus rhythm, with an 80º QRS axis. No signs of hypertrophy or dilated cavities were detected. As part of the study a TTE was performed, detecting a dilated left ventricle with a tract crossing the apical side from the LV lateral wall. The MRI confirmed the presence of an incomplete muscular septum from de LV lateral wall up to the apex encompassing the papillary muscles in the mid left ventricle, dividing the LV in two chambers with telediastolic size of 3.5x3cm and 2.6x2.4cm respectively. This secondary appendix, with a pseudoaneurysm morphology, distended in systole with a dyskinetic motion. Globally, the left ventricle was dilated and had mild systolic disfunction. The right cavities were morphological and functionally normal. In this case initially a common dilated cardiomyopathy was suspected, being able to confirm the presence of a muscular tract crossing the mid left ventricle. The MRI described the image as an LV pseudoaneurysm, however the TTE images and the diagnostic algorithm proposed by Elaheh Malakan et al would include this case as a two-chamber left ventricle, denoting the still present difficulties in the diagnostic process in these cases, and its impact in their therapeutic approach. Abstract P1337 Figure.

Diagnosis of congenital double-chambered left ventricle by Doppler echocardiography

Objective To review the imaging characteristics and evaluate the diagnostic value of echocardiography for congenital double-chambered left ventricle(DCLV) in children, and improve the diagnostic accuracy of initial echocardiography. Methods Doppler echocardiography was performed and these images were compared retrospectively with operative and computed tomography angiography findings in 8 children with DCLV. Results DCLV was characterized by the presence of a main left ventricular chamber and an auxiliary chamber seperated by abnormally hypertrophic muscle and/or fiber bundles. Six cases were diagnosed with type A, and 2 cases with type B by Li Jun′s classification. The associated malformations included ventricular septal defect (3 cases), mitral regurgitation (2 cases), mitral valve stenosis (1 case). Six of 8 cases(75.0%) were diagnosed correctly using Doppler echocardiography, in the remaining 2 cases (25.0%), 1 case was missed, 1 case misdiagnosed as left ventricular diverticulum. Conclusions DCLV could be correctedly diagnosed by Doppler echocardiography. This malformation should be differentiated from left ventricular diverticulum, left ventricular aneurysm, left ventricular non-compaction, and ventricular septal defect. Key words: Echocardiography, Doppler; Double-chambered left ventricle

Bands in the Heart: Multimodality Imaging Review.

Multiple bands and bandlike structures can be found within the cardiac chambers, which can be evaluated with various imaging modalities including echocardiography, CT, MRI, and invasive angiography. These bands can be classified as normal structures or normal variants, aberrant structures, or pathologic entities. Normal structures include the crista terminalis, taenia sagittalis, Chiari network, coumadin ridge, moderator band, papillary muscles, and chordae tendineae. Aberrant structures include aberrant papillary muscles, accessory chordae, false tendons, and accessory mitral valve tissue. Pathologic entities include double-chambered right ventricle, double-chambered left ventricle, cor triatriatum, and subaortic stenosis. Several types of bands are incidental findings discovered at imaging and do not produce clinical symptoms. However, some bands can mimic cardiac diseases, including masses. More importantly, some bands are pathologic entities that produce symptoms owing to hemodynamic consequences. Performing multimodality imaging helps the radiologist (a) identify, localize, and characterize the bands; (b) determine if they are normal structures, abnormal structures, or pathologic entities; (c) distinguish them from cardiac pathologic conditions; and (d) evaluate the secondary consequences of pathologic entities. This article reviews the various bands visualized within the cardiac chambers, as well as the role of imaging in depicting the bands, their appearances across various imaging modalities, and their clinical significance. Online supplemental material is available for this article. ©RSNA, 2019.

Двухкамерный левый желудочек при сложном врожденном пороке сердца у младенца: крайне редкая патология

Двухкамерный левый желудочек при сложном врожденном пороке сердца у младенца: крайне редкая патология

An uncommon variant of double-chambered right ventricle masquerading as double-chambered left ventricle

We present a rare case of a double-chambered right ventricle masquerading as a double-chambered left ventricle, which was found incidentally on cardiac imaging in an adult female patient with atypical chest pain. The most common form of double-chambered right ventricle is characterized by compartmentalization of the right ventricle by muscular bands into 2 distinct chambers. The main features of this malformation are a pressure gradient between the 2 compartments, and the frequent (up to 90%) association with a membranous ventricular septal defect. In our case, the muscular band dividing the right ventricle was located in the inferoseptal part of the latter, creating a diminutive cavity that had no communication with the main right ventricle but communicated with the left ventricle creating the false impression of a double-chambered left ventricle. This constitutes a rare variant of double-chambered right ventricle with unknown clinical implications.

Five-year follow-up two-dimensional speckle tracking echocardiography in a juvenile with a double-chambered left ventricle

Double-chambered left ventricle (DCLV) is a particularly rare congenital entity characterized by the presence of two ventricular cavities separated by an abnormal muscle band. An asymptomatic 15-year-old boy was referred to our hospital because of electrocardiographic (ECG) abnormalities. His initial transthoracic echocardiography (TTE) demonstrated a DCLV with mild left ventricular systolic dysfunction. During a 5-year follow-up period, he remained symptom free with no changes in ECG and conventional TTE findings. However, two-dimensional speckle tracking echocardiography revealed a subtle progressive deterioration of left ventricular systolic function during the 5-year follow-up.

Double-chambered Left Ventricle in Adult Patient with Asymptomatic Evolution

Double-chambered Left Ventricle in Adult Patient with Asymptomatic Evolution

Cardiac hydatid cyst presenting as double-chambered left ventricle

A 55-year-old woman who lived in a village was admitted with dyspnea that had begun over 3 months prior. Initial examination was unremarkable. Chest x-ray and 12-lead electrocardiogram were normal. Transthoracic e c h o c a r d i o g r a p h y (TTE) was performed. Two-dimensional and 3-dimensional echocardiogram showed presence of a large intramural cystic mass attached to the posterolateral wall of the left ventricular cavity. No significant blood flow into the cyst was observed on Doppler echocardiography (Figures). Subsequently, a serological test for specific Echinococcus antibodies was performed, the result of which was consistent with Echinococcus granulosus. Ultimately, the patient was referred to cardiac surgery for resection of cyst, with diagnosis of cardiac cyst hydatid. In spite of advice, the patient refused surgical treatment. Albendazole 800 mg/day was prescribed. Echo monitoring every 6 months was recommended. Hydatid cysts may locate in any organ and present with various non-specific symptoms. Therefore, cardiac cyst hydatid should always be kept in mind when patients from endemic areas present with cardiac or paracardiac cystic masses and complain of non-specific cardiac symptoms. 350

Double-chambered left ventricle in a cat

Double-chambered left ventricle is a rare congenital disorder in which the left ventricular cavity is subdivided into two cavities by an anomalous septum or muscle band. We describe a case of double-chambered left ventricle, most likely caused by the presence of excessive left ventricular bands, in an asymptomatic cat.