Double-Chambered Right Ventricle in Adulthood: A Case Series.
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ABSTRACT:
SUBMITTER: Malone RJ
PROVIDER: S-EPMC10954581 | biostudies-literature | 2024 Mar
REPOSITORIES: biostudies-literature
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Double-Chambered Right Ventricle in Adulthood: A Case Series.
Malone Richard Joseph RJ Henderson Everett Randall ER Wilson Zachary Ryan ZR McMullan Matthew R MR Skelton Thomas N TN Campbell William F WF McMullan Michael R MR
CASE (Philadelphia, Pa.) 20240308 3Part A
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Project description:A 55-year-old woman admitted with symptoms of right heart failure received a diagnosis of double-chambered right ventricle and a ventricular septal defect with aortic and pulmonic valve endocarditis. This case highlights the use of multimodality imaging and importance of adult congenital heart disease expertise in the diagnosis and treatment of such patients. (Level of Difficulty: Intermediate.).
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Project description:BackgroundDouble chambered left ventricle (DCLV) is a rare anatomical variant of the left ventricular structure characterized by the division of the left ventricle into two distinct chambers due to abnormal muscle bundles or septa. This anomaly typically results in primary and secondary chambers within the left ventricle. Due to the lack of conspicuous clinical symptoms, DCLV is often overlooked.Case summaryThe first patient was a 69-year-old male experiencing intermittent chest tightness for several weeks. Transthoracic echocardiography indicated the presence of Type A DCLV, which was subsequently confirmed by 3D echocardiography and cardiac magnetic resonance imaging. An additional case is also worthy of note, during a routine transthoracic echocardiographic examination of a 48-year-old male, we unexpectedly discovered Type B DCLV. His unique cardiac structure was clearly demonstrated through 3D echocardiography.DiscussionDouble chambered left ventricle is a rare structural heart malformation often overlooked due to atypical symptoms. This paper presents two cases of DCLV, illustrating two distinct classifications. Comprehensive understanding of the imaging manifestations of DCLV is imperative for accurate clinical diagnosis.
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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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Project description: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.).
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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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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.
| S-EPMC7799069 | biostudies-literature
Project description:BackgroundDouble-chambered left ventricle (DCLV) is a rare congenital condition, and few case reports are mentioned in literature. Entity, clinical course, and prognosis remain unclear. Cardiovascular magnetic resonance (CMR) is often used for characterization of various congenital heart diseases and can be particularly useful for imaging rare phenomena.Case summaryThree cases of DCLV were detected by CMR within 2 years in our CMR centre with and without associated congenital heart disease or hypertrabecularization. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis of DCLV was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography.DiscussionDouble-chambered left ventricle, synonymous with the terminus 'cor triventriculare sinistrum', has been previously perceived as a rare phenomenon compared with double-chambered right ventricle. It has to be distinguished from ventricular aneurysm or cardiac diverticulum and is characterized by an additional contractile septum with normal wall structure that divides the LV cavum into two (rather) same-sized chambers. The prognosis seems to be benign, since there is no restriction in functionality and no increased thrombogenicity until adulthood. Consequently, there is (presumably) no need for a tailored therapy-at least in the cases present here. Accordingly, we recommend follow-up CMR examinations for progress monitoring and recognize CMR's significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Due to its broader availability, we expect further cases of DLVC in the future.
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Project description:To introduce image characteristics of double-chambered right ventricle on cardiac computed tomography and set a diagnostic criterion for the diagnosis. We retrospectively collected and measured the right ventricular constrictive ratio on computed tomography images in children who had simple ventricular septal defects in the past 10 years, because double-chambered right ventricle is often associated with ventricular septal defects. The right ventricular constrictive ratio was defined as the subinfundibular cross-sectional intraluminal area during end-systole divided by the area during end-diastole in the same patient. We compared the right ventricular constrictive ratio between subjects with concomitant double-chambered right ventricle and those without. 52 children were included, and 23 (44.2%) of them have concomitant double-chambered right ventricle. In most cases (n = 21; 91.3%), the hypertrophied muscular bundles occur just inferior to the level of the supraventricular crest in the right ventricle. Mean right ventricular constrictive ratio in patients with double-chambered right ventricle (15%) was significantly smaller than that without (29%). A cut-off value of a right ventricular constrictive ratio less than 20.1% was established to diagnose double-chambered right ventricle with an 89.7% sensitivity and 78. 3% specificity. Right ventricular constrictive ratio can be a valuable asset for the preoperative diagnosis of double-chambered right ventricle with cardiac computed tomography.
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