Double-chambered Right Ventricle Research Articles

Abstract 4143915: A Case of Double Chambered Right Ventricle Associated with Ventricular Septal Defect- an Overlooked Congenital Heart Disease

Case description: A 68-year-old man with history of prostate cancer, anal and scrotal squamous cell carcinoma presented to the cardiology clinic with dyspnea on exertion with exercise tolerance limited to 2 flights of stairs. Physical examination showed grade 3/6 holosystolic murmur best heard at the left lower sternal edge. A transthoracic echocardiograph (TTE) revealed an ejection fraction of 65%, prominent muscle bound right ventricular outflow tract obstruction (RVOT) consistent with double chambered right ventricle (DCRV), and a Windsock deformity of the membranous interventricular septum through which there was a VSD with left to right shunting. The maximal pressure gradient across the VSD was approximately 116mmHg. The pulmonary artery systolic pressure was 61mmHg; consistent with severe pulmonary artery hypertension. Discussion: DCRV is a rare condition accounting for 0.5 to 2% of congenital heart diseases. It is characterized by abnormal muscle bands that divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber, leading to progressive right ventricular outflow tract obstruction (RVOT). This condition rarely occurs in isolation and is often ventricular septal defect (VSD), which is seen in up to 90% of DCRV cases. DCRV may go undiagnosed until adulthood. As the degree of RVOT increases, patients present with shortness of breath and decreased exercise tolerance. TTE is an invaluable tool for diagnosing and determining the severity of obstruction through pulsed and continuous wave doppler to measure the gradient across the hypertrophied muscle bundles. Current guidelines recommend surgery in asymptomatic patients with severe right ventricular obstruction with a pressure gradient greater than 40 mmHg between the proximal and distal compartments within the right ventricle. Even though our patient was mildly symptomatic, his significant pressure gradient of 116 mmHg warrants surgical evaluation. Conclusion: DCRV is a rare and often overlooked congenital heart disease. The patient, despite mild symptoms, exhibited classic features of DCRV and associated VSD, highlighting the potential for this condition to persist undiagnosed into late adulthood. The presence of severe pulmonary hypertension further emphasizes the importance of early detection and ongoing management to prevent severe complications such as heart failure and sudden cardiac death.

Mid-term Results of Double Chamber Right Ventricle in Association with Genetic Syndromes

Double chamber right ventricle (DCRV) is a congenital disease in which a hypertrophied muscle band divides the right ventricle chamber into two. The early-mid-term follow-up of 6 patients with DCRV and distant genetic syndromes is reported in this paper. A retrospective analysis was performed of 6 DRCV patients with a mean age of 3.9±1.4 years. Concomitant cardiac anomalies were perimembranous ventricular septal defect (n=3), atrial septal defect (n=1), mild aortic regurgitation (n=1), discrete subaortic membrane (n=1). Associated genetic syndromes were Costello (n=1), Seckel (n=1) and Down syndromes (n=4). The mean follow-up period was 4.86±4.6 years. Mean systolic pressure gradient in the right ventricle in the postoperative was 18.5±11.5 mmHg. No mortality occurred and there was no requirement for reintervention. To the best of our knowledge, this is the first report in literature of concomitant DCRV with Costello and Seckel syndromes.

A case series of three patients of double-chambered right ventricle with distinctive features

Double-chambered right ventricle (DCRV) is an extremely rare condition caused by anomalous muscular bundle (AMB), dividing the RV into high- and low-pressure chambers. We are highlighting a case series of three rare patients of DCRV, presenting with diverse clinical and echocardiographic features. Case 1 is manifesting as isolated DCRV in an asymptomatic young female adult, with the AMB located in the subvalvular region of the right ventricular outflow tract. Case 2 is of a significantly symptomatic male infant, afflicted with DCRV, and associated with a large apical muscular ventricular septal defect (VSD). The AMB was situated near the RV apex, just proximal to the VSD. Case 3 represented an asymptomatic DCRV in an adolescent female along with a coexisting small hemodynamically insignificant VSD. Individually, all the three patients are exceedingly rare and their clinical and echocardiographic features were peculiarly distinctive, hence making this case series an interesting and exemplary manuscript.

Isolated Double-Chambered Right Ventricle: A Rare Congenital Heart Disease

Double-chambered right ventricle (DCRV) is an uncommon congenital heart condition characterized by a progressive obstruction in the right ventricular outflow tract. This obstruction is caused by anomalous muscles or fibrous tissues that divide the right ventricle into two cavities: a proximal high-pressure chamber (anatomically lower) and a distal low-pressure chamber (anatomically higher). We present a case of a middle-aged man with a medical history of recurrent symptomatic dyspnea. Upon presentation, there were no signs of congestive cardiac failure. The diagnosis was confirmed using transthoracic two-dimensional (2-D) echocardiography and Transesophageal echocardiography (TEE) for more precise characterization. The primary objective of this case report is to highlight the rarity of this congenital heart disease, particularly in adults.

Importance of the Multimodality Evaluation of a Double-chambered Right Ventricle for Surgical Indications on Admission for Acute Myocardial Infarction.

We treated a female patient known to have a double-chambered right ventricle (DCRV) who presented with symptoms of an acute myocardial infarction (AMI). Emergent coronary artery catheterization revealed acute right coronary artery (RCA) occlusion and proximal left anterior descending (LAD) stenosis. We performed percutaneous coronary intervention (PCI) for the RCA occlusion. Right heart catheterization revealed a pressure gradient across the mid-RV of 58 mmHg. Computed tomography and magnetic resonance imaging revealed no other congenital cardiac abnormalities. She underwent surgical repair of the RV stenosis and coronary artery bypass surgery for LAD stenosis.

724 AN UNUSUAL CASE OF ECHOCARDIOGRAPHY-ESTIMATED PULMONARY HYPERTENSION

Abstract Case Presentation 48-years-old woman was diagnosed with severe pulmonary hypertension at transthoracic echocardiography during cardiological evaluation for bariatric surgery. PAPs was estimated 110 mmHg, mild-moderate tricuspidal insufficiency with no dilatation of right chambers and LVEF preserved (65%). The patient had history of heart murmur since childhood and now presented with type 2 diabetes mellitus on metformin treatment, third grade obesity (BMI 55) and dyslipidemia (LDL col. 120 mg/dL). Diagnostic Workup: The patient was symptomatic dyspnea for mild-moderate exertion (NYHA II-III). Heart-beat was rhythmic with 4/6 ubiquitous systolic sharp murmur. Lung auscultation was normal. Bilateral pretibial edema was noted. The cardiac catheterization showed: A transesophageal echocardiography confirmed post-tricuspidal right intraventricular obstruction due to mid-ventricular fibro-muscular rims and disclosed a small restrictive membranous defect on interventricular septum with minimum left-to-right shunt. A cardiac-MRI confirmed an hypertrophic moderator band with obstruction of the outflow tract. The patients was discharged with a program of sleeve-gastrectomy and after surgical correction of cardiac congenital defects. Conclusion The double-chambered right ventricle (DCRV) is an uncommon congenital anomaly: the right ventricle (RV) is divided into two chambers due to the presence of an abnormally located muscular band or anomalous muscle hypertrophy in the sub-infundibular part of RV outflow tract, with a variable grade of obstruction. This anomaly may be missed by the estimation of high pulmonary pressure through tricuspidal regurgitation. Due to limitations of echocardiography in adult patients additional imaging methods, such as MRI, combined with cardiac catheterization data are required for a definitive diagnosis.

1116 DOUBLE-CHAMBERED RIGHT VENTRICLE AND VENTRICULAR SEPTAL DEFECT IN AN ELDERLY WOMAN: A RARE CASE.

Abstract Introduction Double-chambered right ventricle (DCRV) is a rare congenital heart defect with right ventricular outflow tract (RVOT) obstruction. The right ventricle (RV) is divided into anatomically proximal high-pressure and distal low-pressure chambers by abnormal muscle bundle. DCRV is frequently associated with others congenital heart defects, particularly ventricular septal defects (VSDs). Although its typically presents during childhood and adolescence, it can also present in adulthood. Case Presentation An 84-years-old woman was admitted to our hospital, in emergency department, with a 30-days history of worsening dyspnea and exercise intolerance. She was Ukrainian and did not speak Italian or English. The patient past medical history was unknow except for untreated bilateral glaucoma complicated by blindness. Vital signs were notable for tachycardia, tachypnea (respiratory rate, 28/minute), blood pressure of 118/76 mm Hg, SpO2 of 91%. Physical exam revealed left-sided parasternal systolic murmurs, abolished vesicular murmur at lung bases and jugular vein distension with hepatojugular reflux. The ECG showed atrial fibrillation. Chest X-ray showed moderate cardiomegaly, bilateral pleural effusions, and pulmonary congestion. Transthoracic echocardiography (TTE) was performed and revealed a normal-sized left ventricle with mildly reduced left ventricle ejection fraction (EF 48%), left atrial enlargement, biventricular hypertrophy with asymmetrical interventricular septal hypertrophy. Also, we found massive right atrium and enlarged right ventricle with reduced longitudinal contractility (TAPSE of 13 mm and tricuspid annular tissue Doppler S’ velocity = 7.0 cm/sec). Color flow Doppler in parasternal short-axis view revealed a turbulent systolic flow into the right ventricle. Continuous-wave spectral Doppler analysis showed a peak velocity of 5.6 m/ sec corresponding to a peak gradient of 120 mmHg. Real time 3D-TTE confirmed the of mid-ventricular obstruction due to abnormal trabecular tissue. Therapy including diuretics, beta-blockers and anticoagulants was started. Subsequently, a transesophageal echocardiography (TOE) confirmed the presence of an anomalous mid-ventricular muscle bundle and revealed an associated small sub-aortic ventricular septal defect (VSD) leading to the diagnosis of acute RV failure due to double-chambered RV with VSD and atrial fibrillation. Due to the high risk of complications, patient was considered not amenable for surgery. She was discharged on medical therapy. Conclusions We report a rare case of DCRV and VSD diagnosed in an elderly patient. Due to its rarity, DCRV continues to be misdiagnosed, especially in adulthood. Three-dimensional echocardiography and TOE were most useful tool to define diagnosis and pathophysiology in such an elderly and non-compliant patient.

Double chambered right ventricle with ruptured sinus of Valsalva: rare entity

Valsalva sinus aneurysm (SVAs) and double-chambered right ventricle (DCRV) is an uncommon congenital heart defect and are usually associated with ventricular septal defect (VSD) in 50% to 60% of patient’s population. The current case report had documented a 59 years old hypertensive asymptomatic male with a known case of SVAs presented to our hospital following device closure for 6 months. On examination, the patient was diagnosed to have DCRV induced by SVAs prior to the closure along with other factors. However, to the best of our knowledge, this is the first case report on DCRV induced by SVAs in synergy with other associated multifactors and without VSD among the Indian population.

Double Chambered Right Ventricle with Small Ventricular Septal Defect: Detection and Evaluation by Color Doppler Echocardiography - A Rare Case Report

Double-chambered right ventricle (DCRV) is a rare condition seen only in 0.5-2.0 % of all cases of congenital heart disease (CHD). 80-90 % of DCRV cases are associated with various other congenital heart defects. In particular, perimembranous large ventricular septal defect (VSD) is the most common anomaly associated with DCRV. Anomalous muscular bundle (AMB) typically divides the right ventricle (RV) into a proximal high pressure and distal low pressure chamber. This aberration is often diagnosed during childhood and adolescence. Here we are presenting an exceedingly rare case of asymptomatic DCRV in an adolescent female with a small perimembranous VSD. Keywords: Double Chambered Right Ventricle, small perimembranous VSD, DCRV in an adolescent, facial anomalies, cleft upper lip

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.

Double chambered right ventricle with perimembranous defect and severe aortic aneurism: A case in an adult patient

Double-chambered right ventricle (DCRV) is a rare congenital heart disease characterized by the division of the right ventricular

Double chambered right ventricle with perimembranous defect and severe aortic aneurism: A case in an adult patient

Double-chambered right ventricle (DCRV) is a rare congenital heart disease characterized by the division of the right ventricular cavity

Isolated double-chambered right ventricle in a young female – a rare congenital heart disease: Evaluation by transthoracic color echocardiography and cardiac computed tomography

A double-chambered right ventricle (DCRV) is a rare congenital heart disease and an uncommon cause of congestive cardiac failure. An anomalous muscle band divides the right ventricle into two cavities, the proximal high-pressure chamber, and distal low-pressurechamber. Its origin is debated. Most cases are diagnosed and treated during childhood. Furthermore, there is tendency for progression, if not treated. Echocardiography is considered useful for diagnosis. About 80–90% patients have associated congenital anomalies, such as ventricular septal defect, pulmonary stenosis, and subaortic stenosis. Isolated DCRV is exceptionally rare. Hence, we are reporting a case of an isolated DCRV in an asymptomatic young female patient.

Endothelial-to-Mesenchymal Transition as Underlying Mechanism for the Formation of Double-Chambered Right Ventricle

Double-chambered right ventricle (DCRV) is a progressive division of the right ventricular outflow tract (RVOT) often associated with a subaortic ventricular defect (VSD). The septation is caused by a mixture of hypertrophied muscle bundles and fibrous tissue, whereof the latter is of unclear pathogenesis. Our group has previously reported that flow disturbances lead to formation of fibroelastic tissue through a process called endothelial-to-mesenchymal transition (EndMT) but it is unclear whether the same mechanism exists in the RV. Tissue from patients undergoing repair of DCRV was examined to identify the histomorphological substrate of this tissue. Demographic and pre-/post-operative echocardiographic data were collected from nine patients undergoing surgery for DCRV. RVOTO tissue samples were histologically analyzed for myocardial hypertrophy, fibrosis, elastin content, and active EndMT (immunohistochemical double-staining for endothelial and mesenchymal markers and transcription factors Slug/Snail) and compared to four healthy controls. Indication for surgery were symptoms and progressive RVOT gradients. A highly turbulent flow jet through the RVOTO and VSD was observed in all patients with a preoperative median RVOT peak gradient of 77 mmHg (IQR 55.0–91.5), improved to 6 mmHg (IQR 4.5–17) postoperatively. Histological analysis revealed muscle and thick infiltratively growing fibroelastic tissue. EndMT was confirmed as underlying patho-mechanism of this fibroelastic tissue but the degree of myocardial hypertrophy was not different compared to controls (P = 0.08). This study shows for the first time that an invasive fibroelastic remodeling processes of the endocardium into the underlying myocardium through activation of EndMT contributes to the septation of the RVOT.

A case of double‐chambered right ventricle due to cap‐like fibrous tissue and aberrant chordal insertion of tricuspid valve in adult

A double-chambered right ventricle is a rare congenital heart disease where an anomalous structure divides the right ventricle into two cavities. A 78-year-old man with dyspnea was referred to our institution for a double-chambered right ventricle (DCRV) and ventricular septal defect (VSD). Echocardiography showed normal left ventricular function, right ventricular hypertrophy, and mild tricuspid regurgitation. On parasternal short-axis views, color-flow Doppler studies showed a mosaic pattern through the stenotic right ventricular outflow tract (RVOT). Cardiac catheterization showed a 122 mmHg pressure gradient between the high-pressure chamber and the low-pressure chamber of the RVOT. Computed tomography showed a cap-like structure consisting of fibrous tissue, with moderate stenosis, which divided the right ventricle into two cavities. The aberrant tendinous chords supporting the anterior leaflet of the tricuspid valve were found inserted into the anterior wall of the RV (Figure 1). Resection of the cap-like fibrous tissue and abnormal muscle (Figure 2), detachment of aberrant tendinous chords of the tricuspid valve, closure of VSD by direct suture, and tricuspid valve plasty (TVP) were performed. TVP was achieved using an artificial chordae replacement with expanded polytetrafluoroethylene (CV-5) suture and a 32-mm Physio Tricuspid annuloplasty ring (Edwards Lifeline). Postoperative echocardiography revealed no RVOT pressure gradient and a normal right ventricular pressure of 21 mmHg. In conclusion, we report a rare case of DCRV secondary to a cap-like structure fibrous tissue with anomalous chordal insertion of a tricuspid valve leaflet.

P1726 A case of double chamber right ventricle with membranous ventricular septal defect: multimodality approach before and after surgical repair

Abstract 21 year old male admitted with progressive dyspnea. Transthoracic echocardiogram(TTE) showed membranous type ventricular septal defect(VSD).Additionally there was an evident turbulence and nearly 120mm Hg (5.5m/s maximal velocity) gradient in the mid region of right ventricle(RV) a muscle bundle was restricting the flow. Double chamber right ventricle(DCRV) and VSD have been confirmed also with right heart catheterisation.Measured RV pressures were 90/0/5 mmHg and pulmonary artery pressures were 17/4(mean:8mmHg).Qp/Qs was 1.3. Cardiac magnetic resonance imaging (CMRİ) excluded other congenital defects like atrial septal defect,abnormal pulmonary venous return or aortic coarctation. Cardiac BT also clearly showed the hypertrophied muscular bundle which is close to the apex; was dividing the right ventricle cavity into two parts. Patient has been referred for surgery.On follow up, patient was doing well and TTE nicely showed a clear decrease of gradient as measured maximal velocity was 2m/s and no residual shunt of VSD. Control CMRİ also showed good RV function with a mild residual muscle structure. Our case was a nice example of VSD without pulmonary hypertension because of restricted blood flow of pulmonary artery by a muscle bundle. If VSD is presented with a restricted RV outflow ; instead of a dilated RV chamber as been expected; a concomitant DCRV should be kept in mind. Abstract P1726 Figure. video 1

Double-chambered Right Ventricle after Ventricular Septal Defect Operation Presenting as Syncope

Double-chambered right ventricle (DCRV) is a cardiac disease of the right ventricular outflow tract obstruction characterized by anomalous muscle bundles that divide the right ventricle into two chambers. It may be also develop over time as an acquired lesion in patients with an abnormally short distance between the moderator band and the pulmonary valve. This report highlights the case of a man with double-chambered right ventricle after ventricular septal defect operation, who presented with syncope
 J MEDICINE JAN 2020; 21 (1) : 65-66

A RARE ASSOCIATION OF PERSISTENT VENTRICULAR TACHYCARDIA AND DOUBLE CHAMBERED RIGHT VENTRICLE

Double chambered right ventricle (DCRV) is characterized by the division of the right ventricle into two different chambers by anomalous muscular bands—a proximal high-pressure chamber and a distal low-pressure chamber. A 70-year-old-man with a medical history of diabetes mellitus presented with

DEVELOPMENT OF DOUBLE-CHAMBERED RIGHT VENTRICLE AFTER BILATERAL LUNG TRANSPLANTATION

Double-chambered right ventricle (DCRV) is a rare congenital cardiac defect in which the right ventricle (RV) is partitioned into a proximal high-pressure and distal low-pressure chamber by hypertrophied muscle bundles. We present a case of de novo development of DCRV after bilateral lung

MULTIMODALITY APPROACH IN THE DIAGNOSIS OF DOUBLE-CHAMBERED RIGHT VENTRICLE IN AN ADULT

Double-chambered right ventricle (DCRV) seldom presents in adulthood. Diagnosis of this condition can be challenging, and requires a multimodality approach. A 38 year old woman with a history of ventricular septal defect (VSD) presented with exertional chest pain and dyspnea. A harsh systolic