Hypoplastic Left Heart Stage I

Circulation, vol. 142, pp. 1402–1404

Abstract

hypoplastic left heart syndrome ◼ Norwood procedures E ndoluminal pulmonary banding and duct stenting are the basis for nonsurgical, 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 polytetrafluoroethylenecovered, 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 balloonexpandable 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 acetylsalicylic acid (1-2 mg•kg -1 •d -1 ) together with low-dose bisoprolol/lisinopril/ spironolactone.

Authors 5

1. Dietmar Schranz

   Justus-Liebig-Universität Gießen · University Hospital Frankfurt

   Affiliation as printed

   Department of Pediatric Cardiology University Clinic Frankfurt, Germany (D.S., A.E., R.S.)

   Pediatric Heart Center, Justus-Liebig University, Giessen, Germany (D.S., H.A.)

2. Anoosh Esmaeili

   University Hospital Frankfurt

   Affiliation as printed

   Department of Pediatric Cardiology University Clinic Frankfurt, Germany (D.S., A.E., R.S.)

   Anoosh Esmaeili https://orcid.org/0000-0002-8075-1060 Department of Pediatric Cardiology University Clinic Frankfurt, Germany (D.S., A.E., R.S.)

3. Roland Schrewe

   University Hospital Frankfurt

   Affiliation as printed

   Department of Pediatric Cardiology University Clinic Frankfurt, Germany (D.S., A.E., R.S.)

   Roland Schrewe https://orcid.org/0000-0002-6521-0135 Department of Pediatric Cardiology University Clinic Frankfurt, Germany (D.S., A.E., R.S.)

4. Gunter Kerst Aachen Department of Pediatric Cardiology

   Universitätsklinikum Aachen · RWTH Aachen University

   Affiliation as printed

   Department of Pediatric Cardiology, University Clinic Aachen, Germany (G.K.)

   Gunter Kerst https://orcid.org/0000-0002-2359-5612 Department of Pediatric Cardiology, University Clinic Aachen, Germany (G.K.)

5. Hakan Akintuerk

   Justus-Liebig-Universität Gießen

   Affiliation as printed

   Pediatric Heart Center, Justus-Liebig University, Giessen, Germany (D.S., H.A.)

   Hakan Akintuerk https://orcid.org/0000-0002-9276-7350 Pediatric Heart Center, Justus-Liebig University, Giessen, Germany (D.S., H.A.)