Abstract Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Echocardiographic examination showed a giant Eustachian valve moving towards the tricuspid valve during late systole and leading to complete obstruction of the inflow. Blood flow was redirected through the persistent foramen ovale, producing a right-to-left shunt. Cyanosis, Eustachian valve, Tricuspid valve INTRODUCTION A 4-day-old female neonate born after an uneventful pregnancy with a birth weight of 4.4 kg and an Apgar score of 9/10/10, presented after normal initial postnatal adaptation. Desaturation and a cyanotic skin colour progressively occurred on the second day of life. Initial echocardiographic examination showed a tricuspid atresia with duct-depending pulmonary circulation. An infusion of prostaglandin E1 was started, and the child was referred for further management to our institution. On admission, a saturation of 55% was reported for the child. The other vital signs were within the normal range, and except for the cyanosis, the physical examination was unremarkable. Echocardiography revealed no reliable flow across the tricuspid valve during diastole. Remarkably, further assessment showed a right atrial mass that moved in the late systole of the right ventricle in the direction of the tricuspid valve and prolapsed into the tricuspid valve orifice in early diastole, leading to complete obstruction and functional tricuspid atresia. A large right-to-left shunt through a patent foramen ovale (PFO) was detectable. Ductus arteriosus was restrictive so that the dosage of prostaglandin infusion was increased. On echocardiographic examination, the exact morphology of this probably giant Eustachian valve (EV) remained uncertain. In particular, the attachment of the abnormal structure to the tricuspid valve could not be clearly delineated. Below this structure, the tricuspid valve seemed to be of normal size, with normal leaflets and normal chordal attachment (Fig. 1). No other abnormal findings were found and there was a good biventricular function. Figure 1: View largeDownload slide EV (A, B) moving to TV in the late systole of the right ventricle and prolapsing into the tricuspid valve orifice in early diastole (C), leading to complete obstruction and functional tricuspid atresia (D). EV: Eustachian valve; RA: right atrium; RV: right ventricle; TV: tricuspid valve. Figure 1: View largeDownload slide EV (A, B) moving to TV in the late systole of the right ventricle and prolapsing into the tricuspid valve orifice in early diastole (C), leading to complete obstruction and functional tricuspid atresia (D). EV: Eustachian valve; RA: right atrium; RV: right ventricle; TV: tricuspid valve. Although prostaglandin dose was increased, the duct remained small, and the oxygen saturation did not reach more than 70%. On the 4th day of life, the operation was performed through a median sternotomy, and cardiopulmonary bypass was established through aortic and bicaval cannulation, using mild hypothermia at 34°C. The right atrium was opened under cardioplegic arrest. The intraoperative findings showed a huge and thickened prolapsing EV, which was located at the usual place and was attached along the tricuspid annulus obstructing the tricuspid valve in systole (Fig. 2A). The tricuspid valve was inconspicuous with regular size and normal function. The abnormal structure was resected (Fig. 2B), and the PFO was closed with a suture. The child had an uneventful postoperative course and was discharged on the 7th postoperative day with normal oxygen saturation. Figure 2: View largeDownload slide (A) An asterisk indicates the attachment line; IVC: inferior vena cava; SVC: superior vena cava. (B) An asterisk indicates patent foramen ovale, and double asterisks indicate a resection line. TV: tricuspid valve. Figure 2: View largeDownload slide (A) An asterisk indicates the attachment line; IVC: inferior vena cava; SVC: superior vena cava. (B) An asterisk indicates patent foramen ovale, and double asterisks indicate a resection line. TV: tricuspid valve. DISCUSSION The resection of the giant EV lead to the cure of this unusual and rare case of functional tricuspid atresia. During development, the right valve of the systemic venous sinus divided the right atrium and then regressed and moved caudally and finally ended up leaving the crista terminalis, EV and Thebesian valve . Very few cases of an obstruction of the right ventricular inflow as a result of persistence of the right valve of the systemic venous sinus are mentioned in literature. It can be presented as an isolated malformation or as a part of complex right-sided heart defects . Certainly, one of the main problems is the uniform description and categorization of this pathology. The descriptions range from the giant EV and cor triatriatum dexter (CTD) to the pseudo-CTD and Chiari’s network, all of them with the common denominator of some type of obstruction of right ventricular inflow and cyanosis due to right-to-left shunt [1, 3–5]. The treatment of these patients is inconsistent and depends on the specific anomaly. Bharucha et al.  reported that the term CTD is not absolutely correct. The persistence of the right valve primarily leads to a subdivision of the right atrium but not to complete division . This statement underlines the complexity of the description of this pathology, which leads to the fact that in other publications such structures are described as pseudo-CTD. However, in CTD, surgical resection is the treatment of choice . In most cases, the term giant EV describes the simplest form of a persistent valve of systemic venous sinus. For the treatment of these cases, both surgical and non-surgical procedures were described . The decision on the specific procedure depends on the degree of right ventricular inflow obstruction and the cyanosis resulting from a right-to-left shunt across the PFO. In certain cases with intermittent episodes of cyanosis and oxygen desaturation due to an intermitting shunting across the PFO, the admission to the intensive care unit and administration of oxygen led to normal saturation levels. In all cases, the infants did not require any cardiac intervention and could be discharged . In another case, the obstructing giant EV was resected by surgery, as in our case. As opposed to colleagues who have used the term pseudo-CTD , we decided to describe the defect as the giant EV. We report this case to point on the giant EV as a potential cause of increasing cyanosis in the first days of life. Echocardiographic examination reveals the lesion. Alternatively, magnetic resonance imaging may also confirm the findings. In this case, we could not preoperatively rule out a possible fusion between the EV and the tricuspid valve. Because of the cyanosis and restrictive remaining duct, surgery was the only available option. In this particular case, the decision to operate on the child was retrospectively justified because the tricuspid valve was not affected. Our findings showed a giant EV as a remnant of a persistent right valve of the systemic venous sinus, which normally has no connection with the tricuspid valve. In cases where the tricuspid valve is impaired, the outcome might be worse, but surgery is still the method of choice. Nevertheless, we conclude that in neonates with a giant EV causing relevant haemodynamic obstruction, successful resection of the giant EV is possible and has an excellent outcome. Conflict of interest: none declared. REFERENCES 1 Bharucha T , Spicer DE , Mohun TJ , Black D , Henry GW , Anderson RH. Cor triatriatum or divided atriums: which approach provides the better understanding? Cardiol Young 2015 ; 25 : 193 – 207 . Google Scholar CrossRef Search ADS PubMed 2 Trento A , Zuberbuhler JR , Anderson RH , Park SC , Siewers RD. Divided right atrium (prominence of the Eustachian and Thebesian valves) . J Thorac Cardiovasc Surg 1988 ; 96 : 457 – 63 . Google Scholar PubMed 3 Alghamdi MH. Cor triatriatum dexter: a rare cause of cyanosis during neonatal period . Ann Pediatr Cardiol 2016 ; 9 : 46 – 8 . Google Scholar CrossRef Search ADS PubMed 4 Gad A , Mannan J , Chhabra M , Zhang XX , Narula P , Hoang D. Prominent Eustachian valve in newborns: a report of four cases . AJP Rep 2016 ; 6 : e33 – 7 . Google Scholar PubMed 5 Yerebakan C , Valeske K , Esmaeili A , Akintuerk H. Giant remnant of fetal circulation leading to cyanosis: pseudo-cor triatriatum dexter . Interact CardioVasc Thorac Surg 2014 ; 18 : 690 – 2 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
Interactive CardioVascular and Thoracic Surgery – Oxford University Press
Published: Jan 18, 2018
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