Severe Cyanosis Due to Atrial Septal Defect and an Anomalous Inferior Vena Caval Valve

Abstract

Uncomplicated atrial septal defect is often associated with mild arterial desaturation. A case is reported with severe cyanosis and life-threatening hypoxemia due to an atrial septal defect. Cardiac catheterization revealed normal right heart pressures and an atrial septal defect with shunting of inferior vena caval blood into the left atrium. At surgery an anomalous inferior vena caval valve was found directing inferior vena caval blood into the left atrium. The embryologic basis for this association is discussed. The delayed onset of severe cyanosis may have been contributed to by the thoracic scoliosis.

Anomalous drainage of the inferior caval vein to the left atrium

“…Thus, most case reports of such anomalous connection of the inferior caval vein to the left atrium have described an abnormally large right valve of the systemic venosus sinus ("sinus venosus") coexisting with an atrial septal defect or patent oval foramen. 1,2,4 During early embryologic development, the right and left venous valves separate the systemic venous sinus from the primary part of the right atrium. 9 The left venous valve subsequently fuses with the atrial septum.…”

“…When seen, most cases are associated with atrial septal defects. 1,2 In these patients, it is the occurrence of severe cyanosis despite the presence of the atrial septal defect with normal right ventricular pressures that points to the correct diagnosis. Indeed, drainage of the inferior caval vein to the left atrium should always be considered as a potential diagnosis when confronted with the triad of cyanosis, clubbing of the fingers and toes, and the absence of other pathological findings in the cardiac examination.…”

Persistent redundant Eustachian valve interfering with Amplatzer PFO occluder placement: Anatomico‐clinical and technical implications

“…Some authors mentioned the prominent Eustachian valve in association with a right‐to‐left shunting through an atrial septal defect [2–7]. In these cases, the large valve subdividing totally or partially the right atrium causes a right‐to‐left shunt by favoring the IVC blood flow into the left atrium via the septal defect, without changes in right heart pressure.…”

“…Some reports in the literature showed an association of EV with atrial septal defect (ASD), but few with PFO. In these reports, the large redundant EV may even contribute to the right‐to‐left shunting in the presence of normal right heart pressures [2–7].…”

Eventos catastróficos associados ao tratamento da comunicação interatrial tipo ostium secundum: razões para não se subestimar este tipo de cardiopatia congênita

“…A bibliographical investigation of the database MEDLINE revealed fewer than 50 cases since 1966. This anomaly is due to the failure of the embryonic development of the right sinus venous valve, septum secundum and the dorsal interatrial septum portion [12].…”