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 !