Background: Therapy for hypoplastic left heart syndrome and complex (HLHS and HLHC, respectively)
is still associated with significant morbidity and mortality. Our aim is to improve
the outcome of these vulnerable patient groups by minimizing neonatal surgical trauma
and postponing surgeries to later infancy. Here, we describe four patients who underwent
a compassionate stage-1 procedure (S1P) using a novel, fully transcatheter approach.
Method: Currently, the transcatheter technique was performed in three prenatally diagnosed
newborns with HLHS and one with HLHC with associated aortic atresia and inlet ventricular
septal defect. Nonsurgical S1P was performed in four sedated, spontaneously breathing
newborns with a median weight of 3,345 g and a median age of 5 days. First, an endoluminal
pulmonary artery (PA) banding of the right and left PA was performed. For this purpose,
microvascular plug (MVP) devices were manually modified to pulmonary flow restrictors
(PFR's). PFR's were manually created by removing 1 of the 10 PTFE-covered end cells
of the MVP with a scalpel. Thus, a perforation of approximately 3 to 4 mm was achieved.
Following placement of bilateral PFR's, the arterial duct was stented with a self-expandable
sinus-superflex-DS. At discharge, all patients received bisoprolol and clopidogrel.
The parents were introduced in the surveillance, and all infants were closely followed
by our out-clinic department aiming for a palliative or corrective stage II.
Results: Nonsurgical S1P were performed without mortality and complications. Taking the learning-curve
into account, the median length of hospital stay was 16 days. During the interstage,
three reinterventions were necessary, all of which were performed in advance. It included
stenting of the atrial septum in one, dilatation of left-sided pulmonary vein (PV)
stenosis in another, and a partial dilatation of the right PFR (3-mm coronary balloon)
followed by placement of a coronary stent within a slowly developing aortic coarctation
in the third patient with HLHC. Two patients already received a surgical comprehensive
stage II at the age of 4 months. The removal of the PFR's went smoothly with only
slight injury of the pulmonary intimal tissue and surgical reconstruction of the PAs
before the Glenn anastomosis was uneventful.
Conclusion: Transcatheter S1P was successful in all four newborns. A close follow-up during interstage
is mandatory as initial morphologies and functional status can suddenly change. A
prospective, multicenter study is provided to compare the transcatheter with the hybrid
approach.
