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When coronary arteries need systolic pressure: surgical considerations

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Abstract
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. National multicentre retrospective analysis over 15 years (1985-2010) in a population of similar to 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. 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. 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.
Keywords
ABNORMALITIES, MORTALITY, DETERMINANTS, CONNECTIONS, REVISED CLASSIFICATION, CIRCULATION, MANAGEMENT, Coronary abnormality, INTACT VENTRICULAR SEPTUM, PULMONARY-ATRESIA, Right ventricular dependence, Shunt, Sinusoids, Congenital heart

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Chicago
Cools, Bjorn, Stephen C Brown, Thierry Sluysmans, Daniël De Wolf, Hughes Dessy, and Marc Gewillig. 2013. “When Coronary Arteries Need Systolic Pressure: Surgical Considerations.” European Journal of Cardio-thoracic Surgery 43 (4): 737–742.
APA
Cools, B., Brown, S. C., Sluysmans, T., De Wolf, D., Dessy, H., & Gewillig, M. (2013). When coronary arteries need systolic pressure: surgical considerations. EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY, 43(4), 737–742.
Vancouver
1.
Cools B, Brown SC, Sluysmans T, De Wolf D, Dessy H, Gewillig M. When coronary arteries need systolic pressure: surgical considerations. EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY. 2013;43(4):737–42.
MLA
Cools, Bjorn, Stephen C Brown, Thierry Sluysmans, et al. “When Coronary Arteries Need Systolic Pressure: Surgical Considerations.” EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY 43.4 (2013): 737–742. Print.
@article{3109135,
  abstract     = {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.
National multicentre retrospective analysis over 15 years (1985-2010) in a population of similar to 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.
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.
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.},
  author       = {Cools, Bjorn and Brown, Stephen C and Sluysmans, Thierry and De Wolf, Daniël and Dessy, Hughes and Gewillig, Marc},
  issn         = {1010-7940},
  journal      = {EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY},
  keywords     = {ABNORMALITIES,MORTALITY,DETERMINANTS,CONNECTIONS,REVISED CLASSIFICATION,CIRCULATION,MANAGEMENT,Coronary abnormality,INTACT VENTRICULAR SEPTUM,PULMONARY-ATRESIA,Right ventricular dependence,Shunt,Sinusoids,Congenital heart},
  language     = {eng},
  number       = {4},
  pages        = {737--742},
  title        = {When coronary arteries need systolic pressure: surgical considerations},
  url          = {http://dx.doi.org/10.1093/ejcts/ezs531},
  volume       = {43},
  year         = {2013},
}

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