A rare cause of cyanosis in neonatal age: Cor triatriatum dexter

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  Table of Contents    IMAGE   Year : 2022  |  Volume : 15  |  Issue : 4  |  Page : 429-430 A rare cause of cyanosis in neonatal age: Cor triatriatum dexter

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:
Caputo A, Giordano M, Iacono C, Oppido G, Russo MG. A rare cause of cyanosis in neonatal age: Cor triatriatum dexter. Ann Pediatr Card 2022;15:429-30
How to cite this URL:
Caputo A, Giordano M, Iacono C, Oppido G, Russo MG. A rare cause of cyanosis in neonatal age: Cor triatriatum dexter. Ann Pediatr Card [serial online] 2022 [cited 2023 Jan 7];15:429-30. Available from: 
https://www.annalspc.com/text.asp?2022/15/4/429/367288    Images in Congenital Heart Disease Top

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 atrium

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Figure 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 ovale

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In 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 Top
1.Martínez-Quintana E, Rodríguez-González F. Cor triatriatum dexter: More than a simple membrane. J Thorac Cardiovasc Surg 2016;151:276-7.  Back to cited text no. 1
    2.Moral S, Ballesteros E, Huguet M, Panaro A, Palet J, Evangelista A. Differential diagnosis and clinical implications of remnants of the right valve of the sinus venosus. J Am Soc Echocardiogr 2016;29:183-94.  Back to cited text no. 2
    

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Correspondence Address:
Dr. Mario Giordano
Via Panoramica, 33, 80041-Boscoreale
Italy
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Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/apc.apc_173_21

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