title: When coronary arteries need systolic pressure: surgical considerations.
abstract: OBJECTIVE
  Patients with pulmonary atresia and intact ventricular septum (PA-IVS) may have coronary sinusoids connected to a hypertensive right ventricle. Coronary perfusion may then completely depend on the right ventricular systolic pressure wave; decompression of the right ventricle can be deleterious in these patients. This study was set to investigate the treatment strategies and outcomes of patients with coronary sinusoids.
  
  
  METHODS
  National multicentre retrospective analysis over 15 years (1985-2010) in a population of ~10.2 million people. All patients with PA-IVS and coronary sinusoids were identified from local databases. All angiograms, echocardiograms, surgical reports and outcome data were reviewed.
  
  
  RESULTS
  Thirty patients were identified. Right ventricular-dependent coronary circulation was present in nine patients (30%). A systemic-to-pulmonary artery shunt was created in 23 patients (77%) at a median age of 13.0 days (range: 1-479). A bidirectional Glenn was performed in 20 children at a median age of 7.8 months (range: 2.1-112.9) and 11 children proceeded to a Fontan repair at a median age of 3.6 years (range: 2.1-19.6). Pulmonary valve perforation and angioplasty were performed in six children and in three a simultaneous decompression procedure was also done. Ten patients died (33%). Four died at the time of systemic-to-pulmonary artery shunt and three during the bidirectional Glenn shunt. In all these patients myocardial ischaemia was reported.
  
  
  CONCLUSIONS
  Mortality in PA-IVS with sinusoids is high. Right ventricular coronary dependence may lead to early death if the right ventricular systolic pressure wave is interrupted. Adequate understanding of the underlying pathology and pathophysiology is essential before attempting any procedure in this subgroup of children.
authors.affiliation: Pediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Bjorn
authors.initials: B
authors.lastname: Cools
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Brown
authors.forename: Thierry
authors.initials: T
authors.lastname: Sluysmans
authors.forename: Daniel
authors.initials: D
authors.lastname: DeWolf
authors.forename: Hughes
authors.initials: H
authors.lastname: Dessy
authors.forename: Marc
authors.initials: M
authors.lastname: Gewillig
mesh_terms: D000293:Adolescent; D017130:Angioplasty; D001794:Blood Pressure / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D023921:Coronary Stenosis / Q000601:surgery*; D003331:Coronary Vessels / Q000502:physiology / Q000601:surgery*; D016208:Databases, Factual; D005260:Female; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D018633:Pulmonary Atresia / Q000601:surgery*; D011664:Pulmonary Valve / Q000601:surgery; D012189:Retrospective Studies; D055815:Young Adult