Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager

Abstract

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

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

“…The cyanosis in these patients was attributed to persistent, large inferior vena caval Eustachian valves [2]. While few case reports have described similar veno-arterial shunting in patients with Eustachian valve and ASD [2][3][4][6][7], the cause was identified properly and surgically treated in a timely manner at a relatively young age. For our case, however, the Eustachian valve was misidentified incorrectly as the inferior rim of the ASD during the initial surgery.…”

“…The embryologic remnant can present differently on echocardiogram, ranging in thickness, length, and shape. While the valve is usually benign, veno-arterial shunting is a very rare phenomenon that has been described in a few case reports [2][3][4]. It can occur in patients with prominent valves and atrial septal defects (ASD) without pulmonary hypertension or right ventricular outflow obstruction [2][3][4].…”

“…While the valve is usually benign, veno-arterial shunting is a very rare phenomenon that has been described in a few case reports [2][3][4]. It can occur in patients with prominent valves and atrial septal defects (ASD) without pulmonary hypertension or right ventricular outflow obstruction [2][3][4]. We report a case of a patient with iatrogenic residual IVC type sinus venosus ASD secondary to a prominent Eustachian valve that was misinterpreted as the inferior rim of the atrial septum during the initial ASD repair.…”

Platypnea Orthodeoxia Syndrome Secondary to a Persistent Eustachian Valve

“…The embryologic remnant can present differently on echocardiography, ranging in thickness, length, and shape. While the valve is usually benign, veno-arterial shunting is a rare phenomenon described in a few case reports [1][2][3]. The increase in the right atrial pressure increases shunting, but in patients with persistent eustachian valves, shunting can occur despite the normal right atrial pressure through the PFO that is positional in nature [4].…”

“…Eustachian valve forms commonly associated with POS have associated PFOs and ASDs. They occur in patients with prominent valves and ASDs without pulmonary hypertension or right ventricular outflow obstruction [1][2][3]. The functional component promotes shunting when the patient rises to an upright position.…”