Hypoplastic Left Heart Stage I

Abstract

E ndoluminal pulmonary banding and duct stenting are the basis for nonsurgi-cal, transcatheter stage I in newborns with hypoplastic left heart syndrome (HLHS). We report a novel, nonsurgical palliation for newborns with HLHS and variants. 1 The aim of our transcatheter approach is to replace the Norwood and open-chest hybrid procedures, 2 as well as complex neonatal biventricular repairs, to enable comprehensive stage II or biventricular repair with a lower overall risk by postponing surgeries to later infancy. Following institutional guidelines, this compassionate percutaneous transcatheter approach was performed with written parental consent and was approved by our ethics committee. Spontaneously breathing, sedated newborns received an endoluminal pulmonary artery (PA) branch banding with US Food and Drug Administration-approved, CE-marked Medtronic Micro Vascular Plug (MVP) devices, manually modified to a pulmonary flow restrictor (PFR), as previously described. 3 After bilateral PFR placement, the arterial duct was stented, as is routine in the Giessen hybrid approach. 4 Briefly, after local anesthesia , the femoral vein and artery were punctured, and 4F sheaths were placed. Heparin was administered intravenously in a single dose of 100 U/kg, followed by 50 U/kg after placement of PFRs. On the basis of angiographically determined diameters of the left and right PAs, custom-made MVPs were chosen: MVP-5Q for a vessel size up to 5 mm, 7Q for up to 7 mm, and 9Q for up to 9 mm. Cineangiograms were performed in 20° to 25° cranial and 25° to 30° left oblique or 25° to 30° right oblique planes. Contrast medium was injected manually through a 4F Terumo Cobra shaped catheter positioned within pulmonary branches. The same diagnostic catheter was used for PFR placement. The thin polytetrafluoroethylene-covered, nitinol-framed, self-expandable MVPs were manually converted from a plug device to a flow restrictor. Considering that the MVP-5Q consists of 7 and the 7Q and 9Q consist of 10 covered segments, the covering of 1 or 2 segments was carefully removed by scalpel, generating perforations of ≈3 to 4.5 mm (Figure). After loading of the modified MVP within the Cobra 4F (0.038-in) catheter, the PFRs were placed within the PA branches under fluoroscopy guidance. After bilateral PFR placement, the arterial duct was stented with a Formula balloon-expandable stent (COOK, Copenhagen, Denmark) for treating a narrowed duct and a self-expandable Sinus-Superflex-DS stent (OptiMed, Karlsruhe, Germany) for stenting a wide-open arterial duct. Follow-up drug treatment consisted of continuous infusion of heparin (400 U·kg −1 ·d −1) for 2 days, followed by oral clopidogrel (0.2 mg·kg −1 ·d −1) and ace-tylsalicylic acid (1-2 mg·kg −1 ·d −1) together with low-dose bisoprolol/lisinopril/ spironolactone.