'Matrioska left ventricle': a case of double-chambered left ventricle with double mitral valve apparatus.
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ABSTRACT:
SUBMITTER: Rovera C
PROVIDER: S-EPMC9303002 | biostudies-literature | 2022 Jul
REPOSITORIES: biostudies-literature
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'Matrioska left ventricle': a case of double-chambered left ventricle with double mitral valve apparatus.
Rovera Chiara C Valli Federica F Volpe Alessandra A Guazzi Marco M Pontone Gianluca G
European heart journal. Cardiovascular Imaging 20220701 8
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Project description: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.
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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: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:BackgroundA double-chambered left ventricle (DCLV) is an extremely rare congenital disease that is often asymptomatic and undiagnosed until adulthood. The incidence of a double-chambered right ventricle is estimated to be 1 in 36000 patients, while the incidence of DCLV is certainly even lower. To date, only a handful of cases of DCLV have been reported.Case summaryA 4-year-old boy was admitted to the local hospital in 2019 due to chest discomfort. He had mild tachypnoea and wheezing. Upon physical examination, his heart was found to be enlarged without any obvious cardiac murmur. Cardiac percussion also revealed an enlargement of the heart, and further echocardiography confirmed a diagnosis of a 'dual-chamber left ventricle'. No other cardiac or systemic abnormalities were observed. In January 2022, the patient came to our hospital for further diagnosis and treatment. The laboratory results including coagulation testing showed no obvious abnormality. The 24-hour Holter monitor revealed a sinus rhythm with a left bundle branch block (I°). The bedside chest X-ray indicated an abnormal protrusion of the left margin of the heart. Transthoracic echocardiography showed that the left ventricle was divided into main and accessory chambers by a thick muscle bundle in the middle of the left ventricular cavity. Cardiac magnetic resonance (CMR) imaging confirmed this, and additionally found a giant thrombus in the accessory cavity.DiscussionA DCLV is an extremely rare congenital heart disease that is often asymptomatic and undiagnosed until adulthood. The aetiology of DCLV is still unclear; however, some reports have suggested that it may be related to a hypoplasia of the regional myocardial intra-trabecular sinusoids or an intra-myocardial aneurysm during the embryonic period. Additionally, some cases have indicated that DCLV may be a subtype of genetic cardiomyopathies. A DCLV is characterized by a subdivision of the left ventricle into two chambers by an abnormal septum or by muscle bands. This case report introduces a patient with DCLV and a giant thrombus, in which CMR imaging plays an important role in both diagnosis and differential diagnosis.
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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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