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Rare Case of an Adult With Double-Chambered Left Ventricle.

ABSTRACT: 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.

SUBMITTER: Frick M

PROVIDER: S-EPMC10642131 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature

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Rare Case of an Adult With Double-Chambered Left Ventricle.

Frick Michael M Schütt Katharina K Kerst Gunter G Marx Nikolaus N Lebherz Corinna C

CJC pediatric and congenital heart disease 20220202 1

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 g ...[more]

PMID: 37969559

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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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Rare Case of an Adult With Double-Chambered Left Ventricle.

Publications

Rare Case of an Adult With Double-Chambered Left Ventricle.

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