Abstract 1059 A case series of double chambered left ventricle detected by cardiovascular magnetic resonance

Background

Double chambered left ventricle (DCLV) is a rare congenital condition and only few case reports are mentioned in literature, so entity, clinical course and prognosis remains unclear. Cardiovascular magnetic resonance (CMR) is widely accepted to characterize congenital heart disease and can be a great imaging tool in rare phenomena.

Case summary

We identified 3 patients with the diagnosis of a DCLV over a period of 2 years within approximately 7000 MRI scans in total, suggesting that this cardiac abnormality is not as rare as believed so far and confirming that CMR plays a unique role compared to other imaging modalities in congenital heart disease. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis in each patient was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography. The repeatedly mentioned connection with non-compaction CMP is important, and an atypical trabecularization was also present in one of our cases. A strong connection with other congenital diseases has not yet been described, although Tetralogy of Fallot was present in one of our cases.

Discussion

DCLV, 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, because until adulthood, there is no restriction in functionality and no increased thrombogenicity, and there was no need for a tailored therapy so far in our present cases. In summary, we recommend follow-ups with CMR, which plays a significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Moreover, 3D-wholeheart images allow additional views from any direction due to the sampling of comprehensive 3D data of the thorax, which is not possible with e.g. echocardiography due to its limited thoracic window. In addition, we expect more diagnoses in the future due to the wider availability of CMR.

Clin Res Cardiol (2023). https://doi.org/10.1007/s00392-023-02180-w
A case series of double chambered left ventricle detected by cardiovascular magnetic resonance
C. Meier¹, M. Bietenbeck², S. Drakos², B. Chamling², V. Vehof², R. M. Radke¹, D. Bulut³, P. Stalling¹, M. Theofanidou², A. Yilmaz²
¹Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster; ²Herz-MRT-Zentrum, Universitätsklinikum Münster, Münster; ³Gemeinschaftspraxis Dr. Weis/ Dr. Bulut, Arnsberg;
Background Double chambered left ventricle (DCLV) is a rare congenital condition and only few case reports are mentioned in literature, so entity, clinical course and prognosis remains unclear. Cardiovascular magnetic resonance (CMR) is widely accepted to characterize congenital heart disease and can be a great imaging tool in rare phenomena. Case summary We identified 3 patients with the diagnosis of a DCLV over a period of 2 years within approximately 7000 MRI scans in total, suggesting that this cardiac abnormality is not as rare as believed so far and confirming that CMR plays a unique role compared to other imaging modalities in congenital heart disease. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis in each patient was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography. The repeatedly mentioned connection with non-compaction CMP is important, and an atypical trabecularization was also present in one of our cases. A strong connection with other congenital diseases has not yet been described, although Tetralogy of Fallot was present in one of our cases. Discussion DCLV, 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, because until adulthood, there is no restriction in functionality and no increased thrombogenicity, and there was no need for a tailored therapy so far in our present cases. In summary, we recommend follow-ups with CMR, which plays a significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Moreover, 3D-wholeheart images allow additional views from any direction due to the sampling of comprehensive 3D data of the thorax, which is not possible with e.g. echocardiography due to its limited thoracic window. In addition, we expect more diagnoses in the future due to the wider availability of CMR.
https://dgk.org/kongress_programme/jt2023/aV1076.html