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When coronary arteries need systolic pressure: surgical considerations.
MedLine Citation:
PMID:  23096456     Owner:  NLM     Status:  Publisher    
OBJECTIVES: 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.
Bjorn Cools; Stephen C Brown; Thierry Sluysmans; Daniel Dewolf; Hughes Dessy; Marc Gewillig
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-10-24
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  -     ISSN:  1873-734X     ISO Abbreviation:  Eur J Cardiothorac Surg     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-10-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Pediatric and Congenital Cardiology, University Hospitals Leuven, Leuven, Belgium.
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