Persistent Eustachian valve and transient arterial hypoxemia in a newborn: a clinical observation

The Eustachian valve (EV) is located in the orifice of inferior vena cava and belongs to structures of the normal heart. It plays an important role in the fetal blood flow by directing the flow of blood from inferior vena cava through an open foramen ovale to the left atrium, thereby ensuring the systemic flow in a fetus and bypassing the pulmonary circulation. After birth and upon closure of the foramen ovale, the valve ceases to function and tends to regress. Usually, a prominent EV is a clinically non-significant ultrasound finding. In isolated cases, however, it can cause significant hemodynamic abnormalities and subsequent rhythm disorders, delayed fetal development and transient hypoxemia in newborns. It can extremely rare be a cause of blood right-toleft shunting through the foramen ovale leading to desaturation. Clinically it can manifest by central cyanosis in newborns and infants. The differential diagnosis is made in neonatal intensive care units. We present a case of transient arterial hypoxemia in a newborn with prominent EV and inter-atrial shunt. A one-month old infant was transferred from the Department of Pediatric Cardiology with a history of transient hypoxemic spells related to right-to-left shunting via atrial septal defect caused by obstruction of the tricuspid valve by the prominent EV. The instrumental findings including contrast-enhanced tomography supported this hypothesis. The patient was stable for subsequent 10 days of the follow-up, which allowed for further conservative managements until the conventional time point for children with an atrial septal defect. Potential regress of the prominent EV, as well as natural growth of an infant and his/hers intracardiac structures, provide mostly favorable outcome without a surgical intervention. This was clearly illustrated in the clinical case.

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Prominent Eustachian Valve Mimicking Inferior Rim of Atrial Septum Causing Iatrogenic Inferior Vena Cava Type Sinus Venosus Atrial Septal Defect

Cureus ◽

10.7759/cureus.15387 ◽

2021 ◽

Author(s):

Annie T Wang ◽

Uoo Kim

Keyword(s):

Inferior Vena Cava ◽

Atrial Septal Defect ◽

Septal Defect ◽

Inferior Vena ◽

Vena Cava ◽

Atrial Septum ◽

Sinus Venosus ◽

Eustachian Valve

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P1246 The Eustachian valve: a structure not so innocent

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.700 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

T E Graca Rodrigues ◽

R Placido ◽

C David ◽

S Goncalves ◽

F J Pinto ◽

...

Keyword(s):

Atrial Fibrillation ◽

Heart Rate ◽

Inferior Vena Cava ◽

Atrial Septal Defect ◽

Right Atrium ◽

Septal Defect ◽

Inferior Vena ◽

Vena Cava ◽

Eustachian Valve ◽

The Right

Abstract Background The eustachian valve is an embryological remnant of the inferior vena cava (IVC) valve. It is usually absent or inconspicuous and has no known function in the normal adult. However, there are reports suggesting that the EV is not as innocent as we thought. The EV can be a site of infective vegetations or be mistaken for a thrombus or tumor. The EV can also make interventional procedures (closure of ASD (atrial septal defect) or ablation for atrial flutter) more laborious. Clinical Case A 80 year-old female patient was referred to the emergency department after an episode of acute palpitations, right chest pain, followed by syncope. She had a previous medical history of type 2 diabetes and epilepsy. On admission she was normotensive (104/67mmHg). The ECG showed atrial fibrillation with heart rate of 180 beats per minute. The echocardiogram show slight dilation of the right ventricle and bilateral atrial dilatation. The blood tests documented an elevated D-dimers 2,73 μg/mL, troponin (hs-TNT 27ng/L) and NT-proBNP 4339pg/ml, with a normal renal function. Computed tomographic (CT) pulmonary angiography revealed filling defects on the segmentar and subsegmentar inferior lobes, compatible with the diagnosis of pulmonary embolism and also revealed a dilatation of the main pulmonary trunk (35mm). Based on this result the patient start anticoagulation with low molecular weight heparin and heart rate control with bisoprolol. However besides of this strategy the patient remain in atrial fibrillation without a controlled hear rate and an transesophageal echocardiography (TOE) was performed. The TOE shown a small freely mobile mass – thombi - at the inferior vena cava-right atrium (IVC-RA) junction tethered at the eustachian valve; a small atrial septal defect (4mm) – ostium secundum type – with a left to right shunt; and a preserved biventricular function (figure 1). Based on this exams we optimize the therapy with digoxin and amiodarone with a good control of the heart rate and patient was discharge on warfarine, bisoprolol and digoxin asymptomatic and to be reevaluated on cardiology clinic. Discussion/Conclusion: The eustachian valve in adult is an embryological remainder of the fetal venous sinus valve. This structure is located in the right atrium and is a common, not pathological, echocardiographic finding. It is known that a small percentage of the pulmonary embolisms, thrombi are discovered in the right side of the heart; however, their presence on the Eustachian valve is very unusual. Besides this in our particular case in the present we will have also to ensure that the anticoagulation is effective to avoid that the trombi migrates into the systemic circulation by the atrial septal defect. Abstract P1246 Figure.

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Atrial Septal Defect Closure via Left Subclavian Vein Approach in a Patient With Absent Inferior Vena Cava

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135120983807 ◽

2021 ◽

pp. 215013512098380

Author(s):

Yusuke Enta ◽

Shunsuke Tatebe ◽

Yoshikatsu Saiki ◽

Norio Tada

Keyword(s):

Inferior Vena Cava ◽

Atrial Septal Defect ◽

Subclavian Vein ◽

Septal Defect ◽

Inferior Vena ◽

Transcatheter Closure ◽

Vena Cava ◽

Atrial Septal Defect Closure ◽

Azygos Continuation ◽

Left Subclavian Vein

Without the femoral venous approach, transcatheter closure of an atrial septal defect is challenging. We performed percutaneous closure via the left subclavian vein in a patient with absence of the inferior vena cava with azygos continuation. Considering that inferior vena cava anomalies are not extremely rare among those with congenital heart disease, the left subclavian vein approach can be an alternative to the femoral approach.

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