Adriano Caputo1, Mario Giordano1, Carola Iacono2, Guido Oppido3, Maria Giovanna Russo1
1 Department of Paediatric Cardiology, University of Campania “Luigi Vanvitelli”, “Ospedali dei Colli”, Monaldi Hospital, Naples, Italy
2 Department of Paediatric Cardiology, University of Campania “Luigi Vanvitelli”, “Ospedali dei Colli”; Department of Paediatric Cardiac Surgery, “Ospedali dei Colli”, Monaldi Hospital, Naples, Italy
3 Department of Paediatric Cardiac Surgery, “Ospedali dei Colli”, Monaldi Hospital, Naples, Italy
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Date of Submission02-Sep-2021Date of Decision30-Nov-2021Date of Acceptance08-May-2022Date of Web Publication06-Jan-2023 Abstract
Cor triatriatum dexter is an extremely rare congenital anomaly that is caused by the partitioning of the right atrium by a broad sheet of tissue due to the persistence of the right sinus venosus valve. We describe a rare case of prominent right sinus venosus valve (cor triatriatum dexter) with consequent right-to-left shunt across the patent foramen ovale in a newborn with significant cyanosis.
Keywords: Cor triatriatum dexter, cyanosis, newborn
How to cite this article:A term male newborn (weight 3.500 kg) was referred to our department 11 h after delivery due to respiratory distress. The child was hyporeactive, hypotonic and peripheral perfusion was adequate. Respiratory and heart rates were 50 and 150 bpm, respectively. Peripheral blood saturation was 80%–82%. No cardiac or lung pathologic sounds were detected at the physical exam. Echocardiography highlighted a severe dilation of the right atrium (RA) with a large mobile membrane compatible with an exuberant Eustachian valve More Details versus cor triatriatum dexter.[1] The finding of a large fenestrated membrane separating the RA into two cavities confirmed the diagnosis of cor triatriatum dexter.[2] This prominent membrane prolapsed into the right ventricle during the diastole and moved back into the RA in systole [Figure 1]. This “sailing-like effect” was responsible for both partial tricuspid valve obstruction and right-to-left shunt across the patent foramen ovale (PFO) [Figure 2].
Figure 1: Echocardiography, subcostal view. The prominent redundant membrane (cor triatriatum dexter) (*) inside the RA. LA: Left atrium, RA: Right atriumFigure 2: Echocardiography color Doppler, subcostal view. The redundant membrane (cor triatriatum dexter) (*) causes a significant right-to-left interatrial shunt via the PFO. LA: Left atrium, RA: Right atrium, SVC: Superior vena cava, PFO: Patent foramen ovaleIn the following days, despite an invasive ventilation with high FiO2, the arterial blood saturation ranged from 80% to 85%. The cor triatriatum dexter was considered the cause of the cyanosis, and therefore, it was decided to remove it surgically. The surgical operation confirmed the preoperative diagnosis of cor triatriatum dexter by the finding of a membrane at the junction between the inferior vena cava and the RA, with insertions along the crista terminalis up to the atrial septum. The prominent membrane was surgically removed and the PFO was sutured. The patient had an uncomplicated postoperative course and was discharged 10 days after the procedure with a normal arterial saturation (99%).
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References
Correspondence Address:
Dr. Mario Giordano
Via Panoramica, 33, 80041-Boscoreale
Italy
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/apc.apc_173_21
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