09 l pasquini anelli vascolari
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Transcript of 09 l pasquini anelli vascolari
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Fetal Diagnosis
of Complete Vascular Ring
in Right Aortic Arch and
Left Ductus Arteriosus
LUCIANO PASQUINI MDDirector Perinatal Cardiology
Bambino Gesu’ Hospital Rome
April 18-19 2015
“ La Sapianza” University , Rome
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Anatomy of Ductus Arteriosus in Right Aortic Arch
Anatomic Patterns
L.Knight et al. Right Aortic Arch. Circulation 1974
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Complete Vascular Rings
DAA RAA, LDA, ALSA RAA, LDA
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Embriology
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Anatomy
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Postnatal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
• Symptoms
• CTScan / MRI
• Rare
Kommerell Diverticulum
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PA
T
Normal “V” Shape
RAAsvc
RAA and a LDA ,”U” Shape
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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STUDY POPULATION
• January 2008 - April 2014
• 19 pts
• 7 with prenatal diagnosis
(ga 26.9±5.28 weeks)
• 12 with postnatal diagnosis
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
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Methods
• Fetal or Postnatal Diagnosis
• Prenatal and Postnatal Echocardiography
• CTScan / MRI
• Prenatal and Postnatal Medical, Genetic and Surgical Reports
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
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FETAL POPULATION
• all Asyntomatic
• 2 Surgery
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
RESULTS 1
POSTNATAL POPULATION
• 9/12 Syntomatic
• 9/12 Surgery
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• 11/19 Tracheal Compression by CT scan
(9 symtomatic)
9/12 postnatal
2/7 fetal
• 11/19 Surgical Resection Left Ligamentum
(only one with residual significant
Tracheomalacy)
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
RESULTS 2
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
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• 17/19 Normal Inracardiac Anatomy
• 2/19 Associated VSD
• 3/19 Genetic Syndrome
(2 DiGeorge S.,1 Goldenhar S.)
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
RESULTS 3
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Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
• Definition
• Medial Necrosis
• Primary Resection
Kommerell Diverticulum
Trends in vascular ring surgery , C.L. Backer et al JTCVS 2005
Reoperation afther Vascular Ring Repair , C.L. Backer et al STCSPCSA 2014
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• Frequency is unknown but is certainly higher than
previously reported
• Postnatal Diagnosis can be difficult while Fetal
Diagnosis is much easier
• CT/MRI is recommended
• Long Term Follow-up is necessary
• There is a small subgroup of asyntomatic patients
with severe tracheal stenosis
• Kommerell Diverticulum Resection is recommanded
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Conclusions
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Thank you all !!
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THANK YOU ALL !!
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Grazie a Tutti !