Back to the Roots: Fetal Circulation as an Urgent Therapy for Severe Aortic Stenosis and Depressed Left Ventricular Function in Neonates

Objectives: Initial treatment of critical aortic stenosis is still debated. Many centers use urgent balloon aortic valvuloplasty (BAV) or surgical therapy with high mortality and morbidity especially in cardiogenic shock. To stabilize these critical neonates instead of using mechanical circulatory support systems, we use surgical bilateral pulmonary artery banding (bPAB), catheter interventional ductal stenting (DS), and restrictive ASD creation. Using parallel circulation, the left ventricle is decompressed, and the right ventricle supports systemic circulation. Therefore, left ventricular function can be recovered. After recovery, biventricular correction can be performed in infancy.

Methods: Between 2002 and 2017, seven patients were treated with hybrid strategy. Six patients presented in cardiogenic shock and one patient needed high inotropic support. All patients had initial BAV DS and five patients had Rashkind ASD creation. After that all patients received bPAB. Three patients were preterm and under 2,500 g. Hypoplastic aortic arch was present in three patients, and endocardial fibroelastosis in six patients. Three of seven patients had borderline left ventricle.

Results: After the catheter interventions, bPAB was performed with a median interval of 6 days (1–21 days) and 3,500 g (2,050–3,800 g). All patients recovered from shock and survived after the hybrid procedure. Six of seven patients received biventricular correction with a median age of 178 days (range 135–303 days). One patient was palliated univentricularly because of inadequate left ventricular size and function. Five patients received aortic valve reconstruction; three of these patients had additional aortic isthmic resection and aortic arch reconstruction. One patient underwent Ross–Konno operation. Median follow-up of survivors after biventricular repair is 39 months (4–189 months). During the follow-up time, only one patient had aortic reinterventions. This patient received two times subaortic resection and finally Ross–Konno operation. Two patients needed catheter interventions for right pulmonary artery and pulmonary valve after biventricular repair. No late mortality was observed.

Conclusion: Creating fetal circulation with bPAB, DS, and restrictive ASD creation can be successfully used to treat severe sick neonates with cardiogenic shock. These patients can further achieve biventricular repair. Reintervention rates for aortic valve and pulmonary arteries are low.

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