Neonatal Cyanosis: The Eustachian Valve as a Rare Culprit

Background: Reasons for neonatal cyanosis may be respiratory, infectious diseases, as well as cardiac defects. The most common cyanotic congenital cardiac defects are transposition of the great arteries, Fallot-type lesions, univentricular circulation, and total anomalous pulmonary venous drainage. We report a female neonate who presented with an unusual cause of cyanosis.

Methods: The patient presented with low oxygen saturation after birth. Except for hypothyreosis, gestational diabetes, and obesity of the mother, the pregnancy was uneventful. The child was admitted to the Department of Neonatology and put on continuous positive airway pressure (CPAP) and oxygen therapy up to 50%, which resulted in transcutaneous oxygen saturation (TcO₂) of approximately 85%. Infectious and respiratory reasons for cyanosis were excluded. The following echocardiographic examination showed an elevated right ventricular pressure measured by tricuspid regurgitation and a right to left shunt via an atrial septal defect type II (ASD II) with no signs of tricuspid valve stenosis. There were no signs of major congenital heart disease.

Results: After admission to our facility, a thorough examination revealed an elongated eustachian valve that resulted in partial obstruction of the tricuspid valve and persistent right to left shunt via ASD mimicking a cor triatriatum dexter. After eustachian valve resection and ASD closure, the patient was released from the hospital 4 days after the procedure with a good echocardiographic result and a TcO₂ of 98%.

Conclusion: The causes of neonatal cyanosis may lie beyond the usual spectrum of congenital heart disease. Careful examination is imminent, and even an Eustachian valve, when abnormally structured, can be identified as a rare culprit.

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Artikel online veröffentlicht:
11. Februar 2025