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DOI: 10.1055/s-0036-1571944
Stenting a Stenotic Levoatriocardinal Vein in a Hypoplastic Left Heart Syndrome with An Intact Atrial Septum
Objective: The levoatriocardinal vein is a pulmonary-systemic connection that provides an alternative drainage for pulmonary venous blood in left-sided obstructive lesions. It is thought to result from the persistence of anastomotic channels that connect the capillary plexus of the embryonic foregut to the cardinal veins. There are only few reports about successful preoperative stent placement in the obstructed levoatriocardinal vein or in an obstructed vertical vein at HLHS with an IAS and a total anomalous pulmonary venous connection (TAPVC) as a bridge to surgery.
Patient: We present a case about an infant with a Hypoplastic Left Heart Syndrome (HLHS) and an intact atrial septum (IAS) that was diagnosed prenatally. Postnatal ECHO confirmed the diagnosis and left atrial decompression was only possible by an obstructed levoatriocardinal vein into the innominate vein. Shortly after birth the patient developed severe respiratory distress with a bilateral pulmonary edema. Bedside balloon atrioseptostomy failed due to a complete closure of the IAS and therefore catheter investigation was initiated for palliative measures.
Management and Result: Catheter investigation revealed a subatretic stenosis of the levoatrial vein with a diameter of ∼1 mm and a normal connection of the pulmonary veins to the hypoplastic left atrium. There was a long segment hypoplasia of the LAV and a tight stenosis. After stent implantation (Plamaz Blue 6 mm × 18 mm) immediate decompression was achieved and the clinical condition rapidly improved. The patient was extubated 18 hour after the intervention and subsequently breathed spontaneously without respiratory support. Uneventful Norwood I operation with creation of a broad communication between the left and right atrium was performed 4 days later.
Conclusion: A stenotic levoatriocardinal vein in a patient with HLHS and IAS presents as a life threatening critical condition resulting in severe bilateral pulmonary edema. Interventional treatment by rapid stenting adequately can decompress the left atrium and stabilize the hemodynamics and clinical symptoms. This treatment modality may be a safe method for restoring normal lung function and bridging to the first Norwood palliation. When this combination is diagnosed prenatally, immediate postnatal catheter management should be planned in advance.