Dataset Information

The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

ABSTRACT:

Background

Congenital aneurysms of the atrium are very rare malformations. Known complications are therapy-resistant arrhythmias. Different treatments such as medical therapy, electrophysiological ablation, and surgery have been proposed. However, there are no guidelines on treatment.

Case summary

We describe the case of a neonate with bi-atrial aneurysms causing atrial arrhythmia. Arrhythmia was first observed in the 28th week of gestation. Maternal digoxin treatment did not show any effect. After birth, bi-atrial aneurysms were diagnosed and determined as the probable cause of the atrial tachycardia and later of atrial flutter. Antiarrhythmic drug treatment was initiated. However, only frequency control could be achieved. At the age of 7?months, the patient underwent surgical resection. Since surgery, sinus rhythm is present.

Conclusion

Atrial aneurysms are rare malformations, known complications are atrial arrhythmia. If medical treatment fails, surgery correction appears to be indicated going along with low operative risk and a high probability of successful termination of arrhythmia.

SUBMITTER: Stoller F

PROVIDER: S-EPMC7873803 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

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Publications

The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

Stoller Fabienne F Glöckler Martin M Kadner Alexander A Widenka Hannah H

European heart journal. Case reports 20201228 2

<h4>Background</h4>Congenital aneurysms of the atrium are very rare malformations. Known complications are therapy-resistant arrhythmias. Different treatments such as medical therapy, electrophysiological ablation, and surgery have been proposed. However, there are no guidelines on treatment.<h4>Case summary</h4>We describe the case of a neonate with bi-atrial aneurysms causing atrial arrhythmia. Arrhythmia was first observed in the 28th week of gestation. Maternal digoxin treatment did not show ...[more]

PMID: 33598610

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Project description:Defects of atrial and ventricular septation are the most frequent form of congenital heart disease, accounting for almost 50% of all cases. We previously reported that a heterozygous G296S missense mutation of GATA4 caused atrial and ventricular septal defects and pulmonary valve stenosis in humans. GATA4 encodes a cardiac transcription factor, and when deleted in mice it results in cardiac bifida and lethality by embryonic day (E)9.5. In vitro, the mutant GATA4 protein has a reduced DNA binding affinity and transcriptional activity and abolishes a physical interaction with TBX5, a transcription factor critical for normal heart formation. To characterize the mutation in vivo, we generated mice harboring the same mutation, Gata4 G295S. Mice homozygous for the Gata4 G295S mutant allele have normal ventral body patterning and heart looping, but have a thin ventricular myocardium, single ventricular chamber, and lethality by E11.5. While heterozygous Gata4 G295S mutant mice are viable, a subset of these mice have semilunar valve stenosis and small defects of the atrial septum. Gene expression studies of homozygous mutant mice suggest the G295S protein can sufficiently activate downstream targets of Gata4 in the endoderm but not in the developing heart. Cardiomyocyte proliferation deficits and decreased cardiac expression of CCND2, a member of the cyclin family and a direct target of Gata4, were found in embryos both homozygous and heterozygous for the Gata4 G295S allele. To further define functions of the Gata4 G295S mutation in vivo, compound mutant mice were generated in which specific cell lineages harbored both the Gata4 G295S mutant and Gata4 null alleles. Examination of these mice demonstrated that the Gata4 G295S protein has functional deficits in early myocardial development. In summary, the Gata4 G295S mutation functions as a hypomorph in vivo and leads to defects in cardiomyocyte proliferation during embryogenesis, which may contribute to the development of congenital heart defects in humans.

Dataset Information

The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

Background

Case summary

Conclusion

Publications

The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

Similar Datasets

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