Transcript
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
Anatomy of Ductus Arteriosus in Right Aortic Arch
Anatomic Patterns
L.Knight et al. Right Aortic Arch. Circulation 1974
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Complete Vascular Rings
DAA RAA, LDA, ALSA RAA, LDA
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Embriology
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
Anatomy
Postnatal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
• Symptoms
• CTScan / MRI
• Rare
Kommerell Diverticulum
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PA
T
Normal “V” Shape
RAAsvc
RAA and a LDA ,”U” Shape
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
PAT
RAA with Aberrant LSA and LDA
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
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
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
• 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
Fetal Diagnosis Right Aortic Arch and Left Ductus Arteriosus
• 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
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
• 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
Thank you all !!
THANK YOU ALL !!
Grazie a Tutti !
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