Commentary: An opportunity to better characterize coronary ostial stenosis in patients with Williams syndrome and other elastin arteriopathies

Central MessagePreoperative testing can help identify coronary artery lesions in patients with Williams syndrome and other elastin arteriopathies.See Article page 212. Preoperative arteriopathies. See A group from Stanford has analyzed outcomes of arteriopathies who underwent relief ostial stenosis along supravalvular aortic pulmonary stenosis.2Mainwaring R.D. Collins R.T. Patrick W.L. Martin E. MacMillen K.L. Hanley F.L. Surgical repair arteriopathy syndromes.J Thorac Cardiovasc Surg. 2021; 162: 212-219Abstract Full Text PDF Scopus (3) Google Scholar The also published their results branch this difficult patient population.3Martin Mainwaring II, peripheral without or Alagille syndromes.Semin January 17, 2020; ([Epub ahead print])Abstract (6) As we understand more about the pathophysiology vascular obstruction related to gene deletion at 7q11.23,4Pober B.R. Johnson M. Urban Z. Mechanisms treatment cardiovascular disease Williams-Beuren syndrome.J Clin Invest. 2008; 118: 1606-1615Crossref PubMed (139) ultimate for these would be potentially therapy that could enhance formation prevent smooth muscle proliferation. These have debilitating obstructions result ventricular hypertrophy, dysfunction, insufficiency. Catheter-based therapies not proven very durable, hence surgical been gold standard. There are multiple phenotypes variable areas obstruction, including region valve, arteries, arch, arterial origin. presents experience conjunction and/or stenosis. This included 16 whom 11 had 5 arteriopathy. Of patients, 7 bilateral remainder either right left ostium. technique consists utilizing a contiguous homograft patch augment ostium address techniques similar those reported by Boston Children's Hospital.1Anderson N.D. Borisuk M.J. Hoganson D.M. Rathod R.H. Baird C.W. Three-patch root reconstruction extended main augmentation neonates infants.Semin 2019; 31: 99-101Abstract Scholar,5Wu F. Mondal A. del Nido P.J. Gauvreau K. Emani S.M. et al.Long-term prognosis primary repair.Ann 108: 1202-1209Abstract (12) indication intervention report manifold: preoperative evidence myocardial ischemia, imaging intraoperative finally insufficiency after is clear opportunity here complex population. exists period; origins flow measurements intracoronary ultrasound detect any limiting lesions. helpful operative planning. If there greater than mild limitation may benefit on because significant hypertrophy present vast majority patients. clinicians, it imperative pay close attention ostia proximal arteries so accurately addressed time surgery. indicates, something done excellent outcomes. syndromesThe Journal Thoracic Cardiovascular SurgeryVol. 162Issue 1PreviewPatients syndromes often cardiac characterized supravalvar (SVAS), (PPAS), (CAOS). SVAS PPAS marked increase oxygen demand, whereas CAOS limit supply. combination predisposes ischemic events sudden arrest. purpose study was review our syndromes. Full-Text