coarctation of the aorta - lieberman's...
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Raymond LiuGillian Lieberman, MDRaymond LiuGillian Lieberman, MD May 2001
Coarctation of the AortaRaymond Liu, HMS IIIGillian Lieberman, MD
Raymond LiuGillian Lieberman, MD
Patient L.D.
• 19 y.o. female who presents with HTN
• HTN first noted at age 12 with no further work-up
• Recently took own BP at 180/120
• Has experienced frequent HA with minimal exertion
• PMH: Noncontributory
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Raymond LiuGillian Lieberman, MD
L.D. Physical Exam
• VS: Pulse: 69 BP: Right arm:145/88 Left arm: 148/90 Doppler lower extremity: 90
• Cor: Nl S1 S2. II/VI systolic ejection murmur that radiates to intrascapular region. No diastolic murmur, no S3/S4. Femoral pulses barely palpable. Lower extremity pulses nonexistant
• Rest of physical exam noncontributory
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Raymond LiuGillian Lieberman, MD
Differential Diagnosis of Pediatric HTN
• Infants• Renal artery
thrombosis • Congenital renal dz.• Coarctation of the
aorta• Bronchopulmonary
dysplasia• Patent ductus
arteriosus• IVH
•1-10yrs•Renal Disease•Coarctation of the aorta•Pheochromocytoma•Mineralcorticoid excess•Hyperthyroidism•Neurogenic tumors
•>10yrs•Renal Disease•Essential HTN
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Raymond LiuGillian Lieberman, MD
Background of Coarctation of the Aorta
•7% of children with congenital heart disease •7th most common congenital heart disease• Males > Females by factor of 2• Associated with Turner’s syndrome, Sturge-Weber
syndrome, Neurofibromatosis, and William’s syndrome• Complications include aneurysms, dissections, or
endocarditis• Unoperated coarctation has a 90% mortality by age 50
average age of death at 35 yrs.
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Raymond LiuGillian Lieberman, MD
Anatomy of Coarctation of the Aorta• Juxtaductal coarctation(Adult type)
• Narrowing of thoracic descending aorta just beyond remnant of ductusarteriosus
• 95% of all coarctation cases• Net left-to-right shunting occurs• Collaterals develop • 70% of pts have bicuspid aortic valve
• Tubular hypoplasia(Infantile type)• More diffuse narrowing of transverse
aorta and aortic isthmus• Proximal to patent ductus arteriosus
Courtesy of www.vh.org6
Raymond LiuGillian Lieberman, MD
Anatomy of Coarctation of the Aorta
NEJM Volume 342(4) 27 January 2000
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Raymond LiuGillian Lieberman, MD
Pathology of Coarctation of the Aorta
Cotran et al. Robbins Pathological Basis of Disease. 1999.
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Coarctation
Raymond LiuGillian Lieberman, MD
Embryology of Coarctation• 4th arch persists on left
to connect dorsal aortaand ventral aorta and form aortic arch
• 6th arch develops distally into ductusarteriosus
• Coarctation results from4th and 6th arch development problem
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Raymond LiuGillian Lieberman, MD
Imaging Modalities
• Plain Film
•Echocardiogram
•Magnetic Resonance Imaging
•Angiography
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Raymond LiuGillian Lieberman, MD
Plain Film Imaging
• Advantages• Ease of access• Relative ease of interpretation• Sensitive
• Limitations• Nonspecific• Lack of quantitative data
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Raymond LiuGillian Lieberman, MD
Plain Film Findings
• Classic “3” sign• Rib notching of
posteriorthird of ribs 3-8
• “E” sign on esophagram
• Infantile cardiomegaly
• Infantile pulmonary congestion
Courtesy of Children’s Hospital, Boston12
Raymond LiuGillian Lieberman, MD
Classic “3” Sign
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Raymond LiuGillian Lieberman, MD
Rib Notching
NEJM 1/27/00 Vol. 342, No. 4
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Raymond LiuGillian Lieberman, MD
“E” Sign on EsophogramLAO View
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Coarctation
Raymond LiuGillian Lieberman, MD
Infantile Cardiomegaly
Courtesy of Children’sHospital, Boston
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Raymond LiuGillian Lieberman, MD
Infantile Pulmonary Congestion
Courtesy of Children’s Hospital, Boston17
Raymond LiuGillian Lieberman, MD
Echocardiogram Imaging
• Advantages• First-line diagnostic imaging• Ease of accessibility• Low cost• Quantitative data: pressure gradients, LVH, anatomical
measurements
• Limitations• Limited by narrow field of view• Limited accuracy in quantitative analysis
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Raymond LiuGillian Lieberman, MD
Echocardiographic Findings
Multimedia EncylopediaOf Congenital Heart Disease, 1996
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Raymond LiuGillian Lieberman, MD
Echocardiographic Findings
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Raymond LiuGillian Lieberman, MD
Echocardiographic Findings: Doppler Measurements
Courtesy of Yale School of Medicine
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Raymond LiuGillian Lieberman, MD
Bicuspid Aortic Valve
NEJM 1/27/00 Vol. 342, No. 4
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Raymond LiuGillian Lieberman, MD
Magnetic Resonance Imaging
• Advantages• Allows tomographic imaging in arbitrary anatomic
sections• Accurate quantitative data: Gradients, Anatomical
Measurements• No ionizing radiation or contrast medium needed
• Limitations• Slow scanning process• Claustrophobia is issue for children• High expense
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Raymond LiuGillian Lieberman, MD
Types of MRI
• Spin echo(SE) pulse sequences• Mainstay of morphologic diagnosis• Provides highest contrast resolution with limited temporal
resolution• Blood is black with internal structures contrasted against
signal void of heart chambers
• Gradient reversal echo(GRE) pulse sequences• Can acquire images within short cardiac time interval• Blood is white providing noninvansive contrast medium
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Raymond LiuGillian Lieberman, MD
Spin Echo MRI of Patient L.D.
Courtesy of Children’s Hospital, Boston25
Coarctation
Raymond LiuGillian Lieberman, MD
SE MRI of Infantile Coarctation• Narrowing of aortic
isthmus with multiple collaterals
Courtesy of Children’s Hospital, Boston26
Coarctation
Raymond LiuGillian Lieberman, MD
GRE MRI of L.D.
Courtesy of Children’sHospital, Boston27
Left Ventricular Hypertrophy
Raymond LiuGillian Lieberman, MD
Gadolinium Enhanced MR Angiogram
Courtesy of Children’s Hospital, Boston28
Raymond LiuGillian Lieberman, MD
3D MRI Reconstruction of L.D.
Courtsey of Children’s Hospital, Boston29
Raymond LiuGillian Lieberman, MD
MRI of Coarctation
• T1 Weighted Spin Echo
• GRE Phase Contrast Cine Study
•Contrast Enhanced 3D Reconstruction
Courtesy of Dr. Vrachiolitis, BIDMC30
Raymond LiuGillian Lieberman, MD
Angiography of Coarctation of the Aorta
• Advantages• Gold standard for visualization of lesion• Defines valvular and anatomic disease• Delineates collateral circulation• Offers opportunity for therapeutic intervention
• Limitations• Invasive procedure requiring ionizing radiation• Limited x-ray tube power and intensifier size for babies
and young children
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Raymond LiuGillian Lieberman, MD
Thereaputic Options for Coarctation of the Aorta
• Medical• Surgical• Catheterization
• Balloon angioplasty• Balloon-expandable stents
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Raymond LiuGillian Lieberman, MD
Surgical Intervention
• Resection and end-to-end anastomosis is gold standard
• Indicated for pts. with transcoarctation pressure gradient >30 mmHg
• 90% children are normotensive with 5yr f/u• Residual HTN develops in ½ of pts. >40yrs
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Raymond LiuGillian Lieberman, MD
Catheterization
• Balloon angioplasty• Indicated for both postoperative restenosis and
dilatation of native coaractation• Mortality of angioplasty ranges from 0.0-2.5%
compared with mortality of surgery from 4-25%• 50% restenosis rates• Limited by higher incidence of aortic aneurysm and
recurrent coarctation
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Raymond LiuGillian Lieberman, MD
Catheterization
• Balloon-expandable stents• Flow to major branches of aortic arch maintained
without obstruction at 3 yr f/u• 32/33 patients with native or recurrent coarctation had
complete relief of the obstruction occurred in 32. • Comparable morbidity and mortality results with
balloon angioplasty• Limited by small amount of data
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Raymond LiuGillian Lieberman, MD
Pre-Dilatation Cath for L.D.
Courtesy of Children’s Hospital, Boston
Raymond LiuGillian Lieberman, MD
Balloon Angioplasty for L.D.
Courtesy of Children’s Hospital, Boston37
Raymond LiuGillian Lieberman, MD
Post-Dilatation Catherization for L.D.
Courtesy of Children’s Hospital, Boston
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Raymond LiuGillian Lieberman, MD
Post Procedure CXR
Courtesy of Children’s Hospital, Boston39
Post-ProcedureDecrease in Dilation
Pre-ProcedureDilation
Raymond LiuGillian Lieberman, MD
Catheterization Stents
Radiologic Clinics of North AmericaVol. 37 Num. 2 3/9940
Raymond LiuGillian Lieberman, MD
Catheterization Stents
Courtesy of Yale School of Medicine41
Raymond LiuGillian Lieberman, MD
Summary
• Coarctation of the aorta is narrowing of thoracic descending aorta located distal(juxtaductal) or proximal(infantile) to remnant of ductus arteriosus
• Imaging modalities include plain film, echocardiogram, MRI, or angiography.
• Cardiac catheterization offers opportunity for both radiological interpretation and therapeutic intervention
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Raymond LiuGillian Lieberman, MD
References
Behrman. Nelson Textbook of Pediatrics. 2000.Braunwald, E. Heart Disease: A Textbook of Cardiovascular Medicine 2001Brickner, M. Elizabeth. Hillis, L. David. Lange, Richard A.. “Medical Progress: Congenital
Heart Disease in Adults: First of Two Parts. NEJM. 2000; 342: 256-263.Daniel, Werner G.. Mugge, Andreas.. “Medical Progress: Transesophageal
Echocardiography.” NEJM. 1995; 332:1268-1279. Ing, F. “Early diagnosis of coarctation of the aorta in children: a continuing dilemma”
Pediatrics 1996; 98: 378-82.Sinaiko, Alan. “Current Concepts: Hypertension in Children.” NEJM. 1996; 335:1968-
1973.www.vh.org
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Raymond LiuGillian Lieberman, MD
Acknowledgements• Michael Landzberg, Children’s Hospital• Dr. Senna, Children’s Hospital• Larry Barbaras, Cara Lyn D’amour, Webmasters• Thomas Vrachiolitis, BIDMC• Daniel Saurborn, BIDMC• Eric Niendork, BIDMC• Gillian Lieberman, BIDMC• Lynn Sosa, HMS• Yi-Bin Chen, HMS
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