Anomalous Muscle Bundle Research Articles

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.

A six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect

BackgroundCor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers.Case presentationHere, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair.ConclusionsThe association of CTS with DCRV forming a “6-chambered heart” is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair.

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

Anomalous muscle bundle in the right atrium; Implication to trans atrial device closure

Intracavitary muscle bands or aberrant bands have been well described in all four chambers of the heart but rarely seen thick muscular band crossing right atrium. We report a case of devisable secundum atrial septal defect with an intra-atrial anomalous muscular band, crossing right atrial wall to the rim of the secundum atrial septal defect warranting surgical closure.

Successful Surgical Treatment of the Superior Vena Cava Syndrome with Pulmonary Embolism Caused by Congenital Anomalous Muscle Bundle in the Right Atrium

Successful Surgical Treatment of the Superior Vena Cava Syndrome with Pulmonary Embolism Caused by Congenital Anomalous Muscle Bundle in the Right Atrium

Isolated double chambered right ventricle as a rare cause of sudden death in infancy

Isolated double-chambered right ventricle (DCRV) is a rare form of congenital heart disorder in which the right ventricle is divided into a high-pressure inlet portion and a low-pressure outlet portion by an anomalous muscle bundle. Rarely, autopsy cases describing this congenital heart disease have been reported in the world literature.We report the case of a 50-day-old male who died seven days after been suffering from fever and dyspnea. The autopsy revealed essentially an isolated double-chambered right ventricle (DCRV) and an important pulmonary edema.In this paper, we present the first reported case of sudden unanticipated death in infancy due to isolated double-chambered right ventricle.

Outcome of the Repair of Double-Chambered Right Ventricle: A single centre experience

Background: The presence of anomalous muscle bundles may pro-duce a pressure gradient between the inflow and outflow portions of the right ventricle, resulting in double-chambered right ventricle. We reviewed the outcomes of double chambered right ventricle surgical repair.Methods: Between December 2012 and January 2014, 6 patients under went surgical repair of a double-chambered right ventricle. The patients ranged in age from 3 years to 20 years (mean 8.2±5.9 yrs). Right ventricular outflow tract pressure gradients were from 60 to 120 mm Hg (mean 63.3±40.3). An associated ventricular septal defect was present in 4 patients (66.66%).Results: There were no hospital or late deaths. Mean postsurgical follow up was 3.8±0.8 months). No patient required further surgery to relieve obstruction of right ventricular outflow tract.Conclusions: Surgical repair of a double chambered right ventricle yield excellent hemodynamic and functional results.Cardiovasc. j. 2016; 8(2): 165-168

Excision of anomalous muscle bundles as an important addition to extended septal myectomy for treatment of left ventricular outflow tract obstruction

ObjectiveThis study aims to report our preliminary experience and anatomic findings in the surgical treatment and postoperative management of hypertrophic obstructive cardiomyopathy. MethodsThis study included 277 patients with hypertrophic obstructive cardiomyopathy (168 [60.65%] were male), with a median age of 47 years (interquartile range, 35-54 years), who underwent surgical myectomy performed by 1 surgeon in Fuwai Hospital between May 2010 and April 2015. The median follow-up was 14 months (interquartile range, 7-24 months). ResultsA total of 127 patients (45.85%) underwent concomitant procedures, and 2 patients (0.72%) died in the early perioperative days. The left ventricular outflow gradient decreased from 78 mm Hg (interquartile range, 61-100 mm Hg) to 11 mm Hg (interquartile range, 8-15 mm Hg) when discharged (P < .001). Of the 228 patients with well-documented anatomic description, more than 80% had various intraventricular anomalies. The cumulative survival was 99.28% (95% confidence interval, 97.15-99.82) at 1 year and 96.98% (95% confidence interval, 92.56-98.79) at 5 years. Of the surviving 272 patients, 268 (98.53%) were categorized with functional class I and II of the New York Heart Association classification at the latest evaluation. ConclusionsAnomalous muscle bundles are common in hypertrophic obstructive cardiomyopathy, and they may lead to middle-apical obstruction. Surgical myectomy provides excellent clinical outcomes with low risk for sufficient relief of obstruction and radical correction of intraventricular anomalies in patients with hypertrophic obstructive cardiomyopathy.

0182: Double-chambered right ventricle with intact interventricular septum in adults

The double-chambered right ventricle (DCRV) is a rare congenital heart disease in which anomalous muscle bundles divide the right ventricle into two cavities, causing variable degrees of obstruction. Typically, DCRV is diagnosed at childhood or adolescence, and most DCRV patients have associated congenital anomalies, such as ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The aim of this study is to determinate the clinical presentation, the echocardiographic patterns and the outcome of DCRV with intact ventricular septum in 4 adults. : They were 3 men and one woman. The mean age was 29 years [17;43]. The clinical manifestations were a class II of NYHA dyspnea in 3 patients and right congestive heart failure was observed in one patient. A systolic ejection murmur was heard on the left parasternal border in all cases. Electrocardiogram revealed atrial fibrillation in 2 cases, incomplete right bundle block in 3 patients and right ventricular hypertrophy in all patients. Transthoracic echocardiography established the diagnosis in all cases. It objectified right cavities enlargement in 2 patients and right ventricular hypertrophy in all patients. Moderate tricuspid regurgitation was found in one patient and it was severe in one other patient. The pressure gradient in the right ventricle was evaluated at 72, 80, 80 and 75 mmHg. DCRV was an isolated lesion in all patients. Cardiac catheterization was performed in all patients; it confirmed echocardiographic findings in them. The 4 patients were referred to surgery. Surgical inspection confirmed echocardiographic and catheterisation data in all subjects. They underwent a resection of the obstructing muscle bundles. The postoperative course was uneventful in all patients with no death. DCRV should be suspected in adults when there is a RV outflow tract obstruction with unusual symptoms. Echocardiography is considered as a useful method for the diagnosis of isolated DCRV. Cardiac catheterisation is performed in difficult cases. DCRV should in general be treated surgically, because the obstruction is progressive and ends in heart failure. Operative results and long-term outcomes are usually excellent.

Accessory Tricuspid Valve Leaflet and an Anomalous Muscle Bundle in the Right Ventricular Outflow Tract in a Patient with Double‐Outlet Right Ventricle: A Rare Case Report

Double-outlet right ventricle (DORV), a clinically important congenital heart disease, occurs in 1-3% of persons with congenital heart disease. It may occur as an isolated cardiac defect, together with other cardiac lesions, or in association with extracardiac anomalies. Other rare cardiac anomalies include an anomalous muscle bundle (AMB) in the right ventricular outflow tract (RVOT) and an accessory tricuspid valve leaflet. We report a very rare case of concomitant DORV, AMB in the RVOT and accessory tricuspid valve leaflet in a 17-year-old male patient. The patient eventually died from severe decompensated heart failure. To the best of our knowledge, such a case has not been previously reported in the literature.

Erişkin bir hastada çift odacıklı sağ ventrikül ve ciddi koroner arter hastalığı: Olgu sunumu

Double-chambered right ventricle is a congenital cardiac anomaly in which the right ventricle is divided into two portions by anomalous muscle bundles. We discuss a patient aged 46 who had a double-chambered right ventricle, with coronary artery stenosis and a bicuspid valve. Our patient underwent successful surgical resection of the obstruction by a right ventriculotomy and the infundibular stenosis was expanded with a Gore-tex patch. Also, a left internal mammary artery-left anterior descending coronary artery anostomosis was performed for the left anterior descending stenosis. Although, double-chambered right ventricles are extremely rare in adults, the example of double-chambered right ventricle with coronary artery stenosis which we diagnosed and operated on was the third case reported according to our review of the literature.

A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

Double-chambered right ventricle (DCRV) is a rare congenital heart disorder involving 2 different right ventricle (RV) pressure compartments that is often associated with ventricular septal defect (VSD). Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.

Double-chambered right ventricle presenting significant outflow tract obstruction only in the right decubitus position.

A 75-year-old man was referred to our echocardiography laboratory for presurgical evaluation. His past history included ventricular septal defect closure. He reported chest discomfort in the right decubitus position. Two-dimensional and Doppler echocardiography revealed an anomalous muscle bundle with accelerated blood flow in the right ventricle. The peak flow velocity of the flow increased from 1.4m/s in the left decubitus position to 2.9m/s in the right decubitus position. Double-chamber right ventricle with intermittent mid-ventricular obstruction during the right decubitus position was confirmed by transesophageal echocardiography.

Multimodality cardiac imaging of a double chambered right ventricle with intrapulmonary shunting: a case report

BackgroundDouble chambered right ventricle (DCRV) is a relatively rare congenital heart disease, characterized by the abnormal division of the right ventricle into a high-pressure inlet and low-pressure outlet by anomalous muscle bundles. Extra-cardiac right-to-left shunts may present with clinical symptoms in adulthood and should be sought in patients with previous cavo-pulmonary shunt procedures.Case presentationWe report a case of DCRV in a 29 year old Caucasian male presenting in adulthood with a right-to-left shunt secondary to venous collaterals, following cavopulmonary anastomosis for congenital pulmonary atresia and hypoplastic right ventricle.ConclusionMultimodality cardiac imaging using echocardiography, cardiac CT, cardiac MRI and cardiac catheterization is often required for complete characterization of complex congenital heart anomalies in adulthood.

Not All Obstructive Cardiac Lesions Are Created Equal: Double‐Chamber Right Ventricle In Pregnancy

Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy.

Double chambered right ventricle with severe calcification of the tricuspid valve in an elderly woman: a case report

IntroductionDouble chambered right ventricle is a rare congenital cardiac anomaly in which the right ventricle is divided into two chambers by an anomalous muscle bundle. The diagnosis of this disorder is difficult in adults. Calcification of the tricuspid valve is extremely rare, and very few cases have been reported. Most cases of tricuspid valve calcification had a congenital disorder with high pressure in the right ventricle.Case presentationWe report a rare case of a 71-year-old Japanese woman who presented with chest discomfort, and was found to have a double chambered right ventricle with severe calcification of the tricuspid valve. This abnormality was found by echocardiography, and the diagnosis was confirmed by multislice cardiac computerized tomography, cardiac magnetic resonance imaging, and cardiac catheterization. Our patient rejected surgical repair, and medical therapy with carvedilol was effective to reduce her symptoms.ConclusionCalcification of the tricuspid valve is extremely rare, and considered to be due to high pressure in the right ventricle. To the best of our knowledge, there are no other reported cases of this combination of double chambered right ventricle and calcification of the tricuspid valve.

Isolated Double-Chambered Right Ventricle in a Young Adult

Double-chambered right ventricle (DCRV) is a rare congenital heart disorder in which the right ventricle is divided by an anomalous muscle bundle into a high pressure inlet portion and a low pressure outlet portion. We report a case of isolated DCRV without symptoms in adulthood, diagnosed through echocardiography, cardiac catheterization and cardiac magnetic resonance imaging.

Double-Chambered Right Ventricle Associated with Infective Endocarditis Complication of the Pulmonary Valve: Surgical Management

Double-chambered right ventricle (DCRV) is a rare congenital heart disease characterized by the presence of anomalous muscle bundles, which divide the right ventricle into two chambers: a high-pressure proximal chamber and a low-pressure distal chamber. Most DCRV patients are diagnosed and treated during childhood, and presentation in adulthood is not common. Many congenital heart diseases are often associated with other complications such as infective endocarditis (IE). Right-side endocarditis, which usually involves infection of the tricuspid valve, is uncommon, and endocarditis of the pulmonary valve is extremely rare. We report a 51-year-old woman with undiagnosed DCRV and ventricular septal defect associated with pulmonary valve endocarditis. The diagnostic evaluation and the surgical management are discussed.

Ventriculoseptal Defect Detected After Surgical Repair of Double-Chamber Right Ventricle

Ventriculoseptal Defect Detected After Surgical Repair of Double-Chamber Right Ventricle

Spatial Relationship between the Ventricular Septal Defect and the Anomalous Muscle Bundle in a Double-chambered Right Ventricle

The hemodynamic profile and surgical approach to a double-chambered right ventricle (RV) are influenced by the position of a ventricular septal defect (VSD) in relation to the obstructing muscle bundle. This relationship, however, has not been systematically evaluated, with available literature yielding conflicting results. The objective of this study was to describe this relationship in a series of consecutive patients. This is a retrospective study of patients with double-chambered RV at a single institution between 1999 and 2005. The location of the VSD in relation to the obstructing muscle bundle was established by a review of echocardiograms, cardiac catheterizations, and surgical notes. Twenty-seven patients were identified, and 4 excluded because of inadequate data. There was no disagreement among the methods to determine the relationship between the VSD and the muscle bundle(s). The median age was 65 months (8-204 months). An associated VSD was seen in 21 patients (91%); 18 perimembranous (86%), 2 muscular (9%), and 1 supracristal (5%). The VSD was proximal to the obstructing muscle bundle in 13 (62%), and distal to the bundle in 9 (38%); the supracristal defect was distal, the 2 muscular were proximal, and 11 (61%) of the perimembranous defect were proximal to the muscle bundle. The position of the VSD in relation to the anomalous muscle bundle in a double-chambered RV is heterogeneous. The majority of defects communicate with the high-pressure chamber proximal to the muscle bundle(s).