Journal of Heart and Cardiovascular Medicine · Cyanosis in the Newborn Ryan M Serrano1* and Brandy Hattendorf2 1Riley Hospital for Children, Pediatric Cardiology, Indianapolis, Indiana,

Somato Publications

Journal of Heart and Cardiovascular Medicine

Journal of Heart and Cardiovascular Medicine© 2018 Somato Publications. All rights reserved. 014 Volume 1 Issue 1 - 1004

Case Report

Cor triatriatum Dexter: A Rare Cause of Intermittent Cyanosis in the Newborn

Ryan M Serrano1* and Brandy Hattendorf2

1Riley Hospital for Children, Pediatric Cardiology, Indianapolis, Indiana, USA

2Children’s Hospital of Los Angeles, Pediatric Cardiology, Los Angeles, California, USA

*Address for Correspondence: Ryan M. Serrano, Pediatric Cardiology, 705 Riley Hospital Drive, RR 127, Indianapolis, IN

46202, USA, Tel: (317) 274-8906; Fax: (317) 274-4022; E-mail: [email protected]

Received: 20 October 2018; Accepted: 04 November 2018; Published: 04 November 2018

Citation of this article: Serrano, RM., Hattendorf, B. (2018) Cor triatriatum Dexter: A Rare Cause of Intermittent Cyanosis in the

Newborn. J Heart Cardiovasc Med, 1(1): 014-016.

Copyright: © 2018 Serrano, RM, et al. This is an open access article distributed under the Creative Commons Attribution License,

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

IntroductionCor triatriatum sinister is a well-described cardiac anomaly in

which the left atrium is divided into two compartments by a fibro-muscular septum [1]. Patients can be asymptomatic or present with signs of pulmonary arterial and venous hypertension and its sequelae. Cor triatriatum dexter, on the other hand, occurs when the right atrium is divided into two compartments secondary to persistence of the right valve of the embryonic systemic sinus venosus [2]. It accounts for only 0.1% of all congenital heart malformations and is often associated with other congenital anomalies [2,3]. The clinical manifestations described range from an asymptomatic, incidental finding to elevated central venous pressures and right-sided heart failure due to obstruction of the tricuspid valve and inferior vena cava [2].

Case ReportA one day old infant was born full term via an uncomplicated,

normal spontaneous vaginal delivery with a birth weight of 3.27

kilograms and Apgar scores of 9 at 1 minute and 9 at 5 minutes. Prenatally, the right ventricle appeared smaller than the left ventricle, although it was considered in the low normal range for size at that time. Nevertheless, because of this discrepancy, a postnatal echocardiogram was obtained and revealed normal biventricular size and systolic function. Surprisingly, a large, mobile membrane was seen superior to the tricuspid valve, attaching to the atrial septum. It was noted to traverse across the tricuspid valve during diastole and regress back into the atrium during systole without restriction of flow across the tricuspid valve. An atrial septal defect was also present (Figure 1). Chest x-ray revealed a normal cardiothymic silhouette and clear lungs.

At 12 hours of life, the infant developed circumoral cyanosis with a pulse oximeter reading of 84 percent. This deficit resolved with blow by oxygen. She was transferred to the neonatal intensive care unit for evaluation. The cyanotic episode was suspected to be secondary to sepsis, so a work up was completed and empiric antibiotics started. She continued to have intermittent, limited episodes of desaturations.

ABSTRACTWe present the case of an infant who was prenatally diagnosed with a size discrepancy between the right and left ventricles, but post-natally was found to have cor triatriatum dexter. The patient was initially noted by fetal echocardiogram to have a small right heart. Post-natally, the infant had intermittent mild cyanosis without signs of obstructive heart failure, secondary to restriction of tricuspid flow by the membrane. The infant eventually developed persistent cyanosis. This prompted surgical repair with removal of the cor membrane, which was causing preferential right to left shunting across an atrial septal defect. In conclusion, cor triatriatum dexter should be considered in the fetal differential for an underdeveloped right ventricle. It should also be considered in any newborn presenting with neonatal cyanosis with or without accompanying signs or symptoms of heart failure.

Keywords: Cor triatriatum dexter, Cyanosis, Fetal echocardiogram

Citation: Serrano, RM., Hattendorf, B. (2018) Cor triatriatum Dexter: A Rare Cause of Intermittent Cyanosis in the Newborn. J Heart Cardiovasc Med, 1(1): 014-016.

Journal of Heart and Cardiovascular Medicine© 2018 Somato Publications. All rights reserved. 015 Volume 1 Issue 1 - 1004

A repeat echocardiogram on day of life three confirmed a mobile membrane superior to the tricuspid valve without restriction of blood flow across the valve, as well as an atrial septal defect with right to left flow. Electrocardiogram was normal with sinus rhythm.

The infant was monitored in the neonatal intensive care unit where she remained clinically well and was eventually discharged home with a pulse oximeter and oxygen as needed. At home, she was initially well with no tachypnea, feeding intolerance or persistent cyanosis, but with time her parents reported a steady decline in her baseline saturations. By two weeks of life, the baseline saturation had reached the low 80s, which prompted surgical removal of the cortriatriatum membrane. Intraoperative findings demonstrated that the membrane spanned from the outlet of the superior vena cava down to the Eustachian valve and over to the septal leaflet of the tricuspid valve. While there was no obstruction at the level of the tricuspid valve, the cor membrane preferentially shunted blood flow right to left across an 8 mm secundum atrial septal defect. The membrane was removed and the atrial septal defect was closed primarily. Post-operative transesophageal echocardiogram demonstrated no residual membrane, no residual interatrial shunt, normal inferior and superior vena cava flow, normal left and right ventricular size and function, and normal tricuspid valve. After an uncomplicated postoperative course, the patient was discharged home on postoperative day three with normal saturations and no oxygen requirement.

DiscussionDuring fetal life, the function of the Eustachian valve of the sinus

venosus is to divert oxygenated blood from the inferior vena cava to the left atrium via the foramen ovale. With development of the heart, the sinus venosus is typically incorporated into the right atrium. Cor triatriatum dexter is a rare entity in which persistence of the right-sided valve creates a division of the right atrium [4]. Often, patients are asymptomatic because the membrane is fenestrated or small enough that systemic venous flow passes unobstructed to the outflow chamber. If complications requiring surgical intervention do occur, they are usually secondary to right sided obstruction causing signs and symptoms of heart failure or intractable arrhythmia [5]. In addition, one report describes the case of a 22 year old woman who suffered

from a myocardial infarction and pulmonary embolism thought to be secondary to a thrombus found on the thick and calcified ridge within the right atrium [6].

For our patient, the cor membrane preferentially diverted deoxygenated blood through a secundum atrial septal defect, resulting in intermittent desaturations that evolved to persistent cyanosis. This is important to keep in mind in the context of a lack of tricuspid valve obstruction or clinical signs of heart failure. León et al. [7] described a similar case of a newborn with a persistent Eustachian valve, but the Eustachian valve is embryologically different and a much more common finding than cor triatriatum dexter. Also, that patient did not require surgical intervention. While there is one reported case of persistent mild cyanosis associated with cor triatriatum dexter and a secundum atrial septal defect in a nine year old [8], our patient represents the first case of cor triatriatum dexter presenting as neonatal cyanosis without signs of heart failure. We suspect the cor membrane may have been difficult to identify prenatally given its lack of rigidity within the atrial chamber. Additionally, the borderline small right heart structures demonstrated on fetal echocardiogram were likely secondary to the decreased flow across the tricuspid valve due to the cor membrane. Although rare, cor triatriatum dexter should be considered in both the differential for fetal echocardiograms that suggest small right sided structures, as well as neonatal cyanosis with or without accompanying signs or symptoms of heart failure.

Financial SupportThis research received no specific grant from any funding agency,

commercial or not-for-profit sectors.

Conflicts of InterestThe authors have no financial or non-financial conflicts of interest

to disclose. No affiliations or any personal, racial and intellectual properties to disclose.

Contributor’s Statement Dr. Ryan Serrano drafted the initial manuscript and approved the

final manuscript as submitted.

Dr. Brandy Hattendorf reviewed and revised the manuscript, assisted with image collection, and approved the final manuscript as submitted.

Ethical StandardsThe authors assert that all procedures contributing to this work

comply with the ethical standards of the relevant national guidelines on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the institutional committee.

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2. Choudhary, D., Sivasankaran, S., Venkateshwaran, S., Sasidharan, B. (2013) Cor Triatiratum Dexter: A Rare Cause of Isolated Right Atrial Enlargement. Pediatr Cardiol, 34(1): 198-199

3. Aliyahu, I., Ibrahim, FZ. (2018) Cor-triatriatum dexter with associated cyanosis in a 3-month-old girl. J Cardiovasc Echogr, 28(2): 143-145.

Figure 1: Apical four chamber view via transthoracic echocardiogram demonstrating the core membrane superior to the tricuspid valve and attached to the atrial septum.

Citation: Serrano, RM., Hattendorf, B. (2018) Cor triatriatum Dexter: A Rare Cause of Intermittent Cyanosis in the Newborn. J Heart Cardiovasc Med, 1(1): 014-016.

Journal of Heart and Cardiovascular Medicine© 2018 Somato Publications. All rights reserved. 016 Volume 1 Issue 1 - 1004

4. Gussenhoven, WJ., Essed, CE., Bos, E. (1982) Persistent right sinus venosus valve. Br Heart J, 47(2): 183-185.

5. Suijker, M., Hazekamp, GM., Rammelloo, AJL., Boon, R., Hruda, J. (2008) Persistent sinus venosus valve requiring surgery in children. Congenital Heart Disease, 3(4): 250-253.

6. Hussain, ST., Mawulawde, K., Stewart, RD., Pettersson, GB. (2015) Cor triatriatum dexter: A rare cause of myocardial infarction and

pulmonary embolism in a young adult. J Thorac Cardiovasc Surg, 149(3): e48-50.

7. León, RL., Zaban, NB., Schamberger, MS., Ho CY, Mietzsch, U. (2018) Cyanosis and stroke due to functional cor triatriatum dexter in a neonate. Neonatology, 113(3): 231-234.

8. Zainudin, AR., Tion, KG., Mokhtar, SA. (2012) Cor triatriatum dexter: A rare cause of childhood cyanosis. Ann Pediatr Cardiol, 5(1): 92-94.