Management of Adult with Single Ventricle, Absence of Left Pulmonary Artery, and Severe Stenosis of the BT Shunt

Background: Adults with complex congenital heart disease and status post palliation with classical BT-Shunt (CBTS) are a very rare group. Management and treatment of these patients is very challenging and requires an interdisciplinary approach.

Methods: A 40-year-old female patient with tricuspid valve atresia and status post classical BT-Shunt (CBTS) was admitted to our department with increasing exercise intolerance and dyspnea at rest (NYHA IV). Oxygen saturation was 65–72% at rest and 31% after 120 m 6 minute walk test (6MWT). The performed echocardiogram shows a severe stenosis of the CBTS. The CT scan shows no central left pulmonary artery, the left lung was supplied through small aortopulmonary collaterals. A cardiac catheterization was performed and showed a severe distal stenosis (4 mm) of the CBTS (16 mm) with a large RPA aneurysm, mean pressure in the RPA was 30 mm Hg. A covered CP-Stent was mounted on a 12 mm BiB-Balloonand and implanted in the CBTS stenosis with an hourglass configuration to reduce complications of hyperperfusion syndrome. The diameter obtained of the stenosis after stent implantation was 8 mm. 2 days after stent implantation, the patient’s condition steadily deteriorated with dyspnea and intercostal retractions. After intubation and increase of diuretics and antibiotic treatment the patient’s condition improved continuous. After 5 days the patient was extubated and switched to NIV support. 5 weeks after stent implantation in CBTS the patient was discharged with improved condition. At follow up 3 months after discharge patient’s condition still improved with oxygen saturation 80–85% at rest and 62% after 180 m 6 minute walk test.

Conclusion: Percutaneous treatment of CBTS stenosis in adults with single ventricle anatomy palliated with CBTS is safe and feasible and represents a low risk alternative to surgery. The development of hyperperfusion syndrome remains a main risk and requires a close patient monitoring.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
13 February 2024