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

ABSTRACT: 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.) Central Illustration

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PROVIDER: S-EPMC9588453 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature

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Double chambered right ventricle with ventricular septal defect in adults: case series and review of the literature.

Project description:Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

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A rare case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve.

Project description: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.

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Project description: Not available

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Transcatheter ventricular septal defect (VSD) creation for restrictive VSD in double-outlet right ventricle.

Project description:BackgroundDouble-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum.Methods/resultsA full-term infant girl (2.9 kg) referred for hypoxia (80% with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation.ConclusionsThis report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.

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Congenital Double-Chambered Left Ventricle Presenting as Monomorphic Ventricular Tachycardia.

Project description: Not available

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Accessory Left Ventricle: A Mistaken Case of Ventricular Septal Defect!

Project description:Physical examination is becoming a lost art. We describe a case of a patient who was referred to us with an initial diagnosis of ventricular septal defect. Discordance between imaging findings and the physical examination led to a diagnosis of an accessory left ventricle, a rare but benign congenital cardiac condition. (Level of Difficulty: Beginner.).

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Project description:An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care.

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Unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm: A case report.

Project description:A 54-year-old male with a history of unrepaired ventricular septal defect (VSD) suffered from easy fatigability on exertion. A Levine grade V/VI continuous murmur was auscultated. Transthoracic echocardiogram showed a ruptured sinus of Valsalva aneurysm (SVA) and a significant left-to-right shunting from the ascending aorta to the right ventricle (RV). In addition, a 36 mmHg of pressure gradient was observed between the inflow and outflow tract in the RV, suggesting double-chambered RV (DCRV). Cardiac catheterization also revealed 33 mmHg of the pressure gradient in the mid-potion of the RV, which was coincident with DCRV. A calculated pulmonary-to-systemic flow ratio was 3.0. Therefore, the patient was offered surgical repair of the ruptured SVA and VSD, which was successfully performed. During the surgery, an anomalous muscle band, which is usually the cause of DCRV, was not found, instead, a thickened RV free-wall due to the exposure of the left-to-right shunt flow, so-named jet lesion, was found. Therefore, surgical resection of the anomalous muscle band was not required. The protruded SVA toward the RV, the jet lesion, and the increased RV stroke volume, which could induce relative stenosis, were the causes of the unusual DCRV. <Learning objective: A mechanism of an unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm (SVA) is as follows. One is a morphological stenosis in the right ventricle (RV) due to a protruded SVA toward the RV. The second is a jet lesion; a thickened RV wall induced by the exposure of the shunt flow from the ascending aorta. The third is a relative stenosis due to the increased stroke volume of the RV.>.

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Revisiting Folger's pouch in double-chambered right ventricle.

Project description:Folger's pouch is a right ventricular outflow pouch arising from the distal low-pressure chamber in double-chambered right ventricle. It is rarely described on echocardiogram due to difficult visualization and lack of knowledge of its existence. Retrospective assessment of echocardiogram after an angiocardiographic evaluation demonstrates the pouch in a modified high parasternal view.

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Isolated Double-Chambered Right Ventricle - A Rare Entity.

Project description:A double-chambered right ventricle (DCRV) is a rare congenital heart disease and an uncommon cause of congestive heart failure. An anomalous muscle band divides the right ventricle into two cavities: the proximal high-pressure chamber and the distal low-pressure chamber. Most cases are diagnosed and treated during childhood. Furthermore, there is a tendency for progression, if not treated early. Echocardiography is considered useful for the diagnosis of this ailment. Most of the patients have associated congenital anomalies, such as ventricular septal defect, pulmonary stenosis, and subaortic stenosis. Isolated DCRV is a rare entity. Hence, we report a case of an isolated DCRV in an adult patient.

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