This document discusses coarctation of the aorta, including its development, presentation, management, and outcomes. It begins with an overview of the anatomy and embryological development of the aorta. Coarctation results from abnormal development of the 4th and 6th aortic arches. Presentation and management in the delivery room and NICU is then reviewed. Undiagnosed cases may present with signs of heart failure. Timing of ductal closure is also an important factor. Surgical intervention, outcomes, complications, and long-term issues are then described. Neurologic abnormalities can occur pre- and post-operatively. Overall, outcomes have significantly improved with early diagnosis and surgical repair.

1. Coarctation of the AortaCoarctation of the Aorta
Liz Wetzel, MDLiz Wetzel, MD
CICU RoundsCICU Rounds
November 19, 2010November 19, 2010

2. ObjectivesObjectives
• Review Anatomy/Lesion DevelopmentReview Anatomy/Lesion Development
• Discuss DR Presentation and ManagementDiscuss DR Presentation and Management
• Review Post-natal Evaluation and TransportReview Post-natal Evaluation and Transport
• Describe possible presentation in non-prenatallyDescribe possible presentation in non-prenatally
diagnosed cases and timing of duct closurediagnosed cases and timing of duct closure
• Discuss potential CICU Course and outcomesDiscuss potential CICU Course and outcomes

3. Development of the aortic archDevelopment of the aortic arch
The left fourth arch vessel becomes the arch of the aorta. The left 6The left fourth arch vessel becomes the arch of the aorta. The left 6thth
becomes part of the left pulmonary artery and the ductus arteriosis.becomes part of the left pulmonary artery and the ductus arteriosis.
Sadler, TW. Langman’s Medical Embryology 8th
edition. Philadelphia: Lippincott Williams& Wilkins,2000: 239-243.

4. Development continuedDevelopment continued
Gittenberger-De Groot, A.C. Bartelings, M.M. Deruiter, M.C. Poelmann, R.E. Basics of Cardiac Development for the Understanding of Congenital Heart Malformations. Pediatric Research.
2005; 57 (2): 169-176.
Molin, D. DeRuiter, M.C, Wisse, L.J, Azhar, M., Doetschman, T., Poelmann, R. E., Gittenberger-de Groot, A. C. Altered apoptosis pattern during pharyngeal arch artery
remodeling is associated with aortic arch malformations in Tgfβ2 knock-out mice. Cardiovascular Research. 2002; 56: 312-322.

5. Development of CoarctationDevelopment of Coarctation
• Abnormal development ofAbnormal development of
left 4left 4thth
and 6and 6thth
aortic archesaortic arches
• Represents 5-10% of allRepresents 5-10% of all
congenital cardiac lesionscongenital cardiac lesions
• More common in boys thanMore common in boys than
girlsgirls
• No real impact prior to birthNo real impact prior to birth
due to presence of PDAdue to presence of PDA
unless there is fetal closureunless there is fetal closure

6. Ductus Tissue TheoryDuctus Tissue Theory
• Due to a migration ofDue to a migration of
ductus smooth muscle cellsductus smooth muscle cells
into the periductal aortainto the periductal aorta
with subsequentwith subsequent
constriction and narrowingconstriction and narrowing
of the aortic lumenof the aortic lumen
• Evident when ductus closesEvident when ductus closes
Hemodynamic TheoryHemodynamic Theory
• Reduced intrauterineReduced intrauterine
blood flow causesblood flow causes
underdevelopment ofunderdevelopment of
aortic archaortic arch
• Results from reducedResults from reduced
volume of blood flowvolume of blood flow
through the fetal aorticthrough the fetal aortic
arch and isthmusarch and isthmus

7. Other TheoriesOther Theories
• May be due to a defect in the vascular wall of theMay be due to a defect in the vascular wall of the
ascending aortaascending aorta
• Vascular apoptosis may have a roleVascular apoptosis may have a role (Molin et al 2002)(Molin et al 2002)
• Recessive genetic mutation found in zebrafishRecessive genetic mutation found in zebrafish (Weinstein et(Weinstein et
al 1995)al 1995)
• Autosomal dominant inheritance of non-syndromic leftAutosomal dominant inheritance of non-syndromic left
ventricular outflow tract obstructionventricular outflow tract obstruction (Wessels et al 2005)(Wessels et al 2005)

8. DR Presentation & ManagementDR Presentation & Management
• Follow normal NRP guidelines for resuscitation and beFollow normal NRP guidelines for resuscitation and be
sure to have a stable airwaysure to have a stable airway
• This is not a lesion where you would expect acuteThis is not a lesion where you would expect acute
delivery room decompensationdelivery room decompensation
• Should not be a blue baby due to the heart defectShould not be a blue baby due to the heart defect
• Admit to the NICUAdmit to the NICU

9. Management in the NICUManagement in the NICU
• Support the airway (intubation if necessary)Support the airway (intubation if necessary)
• Echocardiogram and CXREchocardiogram and CXR
• Ideally establish umbilical accessIdeally establish umbilical access
• PGEPGE11 infusioninfusion (0.03 to 0.05 mcg/kg/min)(0.03 to 0.05 mcg/kg/min)
• Correct acidosis and electrolyte abnormalitiesCorrect acidosis and electrolyte abnormalities
• Blood pressure support as indicatedBlood pressure support as indicated
• Main goal is to stabilize the patient and get themMain goal is to stabilize the patient and get them
transferred to a cardiac intensive care unittransferred to a cardiac intensive care unit

10. Transport Issues and Hand-off to cardsTransport Issues and Hand-off to cards
• Be confident you have a stable airwayBe confident you have a stable airway
– Risk of apnea with PGERisk of apnea with PGE11 infusion, consider caffeine?infusion, consider caffeine?
• Have secure IV access with fluids runningHave secure IV access with fluids running
– PGEPGE11 infusion can cause vasodilatation and can result ininfusion can cause vasodilatation and can result in
relative hypovolemia in neonatesrelative hypovolemia in neonates
• Report recent blood gas with electrolytes including ionizedReport recent blood gas with electrolytes including ionized
calcium and potentially a lactatecalcium and potentially a lactate
• Full set of vitals including 4 extremity blood pressuresFull set of vitals including 4 extremity blood pressures

11. Undiagnosed Coarctation PresentationUndiagnosed Coarctation Presentation
• Decreased or absent femoral pulses,Decreased or absent femoral pulses,
tachypnea, grunting, poor feeding,tachypnea, grunting, poor feeding,
signs of CHF, abnormal 4 extremitysigns of CHF, abnormal 4 extremity
blood pressuresblood pressures
• If coming from home can present to ED in shock with multi-If coming from home can present to ED in shock with multi-
organ dysfunction and severe metabolic acidosisorgan dysfunction and severe metabolic acidosis
• CXR with cardiomegaly, pulmonary congestionCXR with cardiomegaly, pulmonary congestion
Sharland, G.K, Chan, KY, Allen, LD. Coarctation of the aorta: difficulties in prenatal diagnosis. British Heart Journal. 1994; 71: 70-75.
http://www.heartonline.org/congenital.htm

12. Potential CXR ProgressionPotential CXR Progression
DAY FIVE OF LIFEDAY FIVE OF LIFEDAY THREE OF LIFEDAY THREE OF LIFE

13. Closure of the ductClosure of the duct
• Functional and Anatomic Closure of DuctFunctional and Anatomic Closure of Duct
– Closure occurs in three steps:Closure occurs in three steps:
1.1.constriction of ductal smooth muscle;constriction of ductal smooth muscle;
2.2.hypoxia/ischemia of medial smooth muscle;hypoxia/ischemia of medial smooth muscle;
3.3.remodeling resulting in permanent closure (Koch et. alremodeling resulting in permanent closure (Koch et. al
2006)2006)
•In term infantsIn term infants functional closurefunctional closure can occur as early ascan occur as early as
12-1512-15 hours of age,hours of age, if greater than 72 hours it isif greater than 72 hours it is
considered persistent,considered persistent, truetrue anatomicanatomic closureclosure can takecan take
weeksweeks
Neoreviews Controversies in the Management of PDA (Gien 2008)

14. Timing of Ductal ClosureTiming of Ductal Closure
• In >95% of neonates >1500g closure usually begins within 96 hours
(Koch et. al 2006)
• Spontaneous closure occurs in >34% of ELBW neonates (Koch et. al
2006)

15. Potential surgical interventionPotential surgical intervention
• First surgery was done experimentally in animals in 1944First surgery was done experimentally in animals in 1944
– Blalock and Park– Blalock and Park
• 1. Resection with end-to-end anastomosis1. Resection with end-to-end anastomosis
• 2. Patch aortoplasty2. Patch aortoplasty
• 3. Left subclavian patch aortoplasty3. Left subclavian patch aortoplasty
• 4. Bypass grafts between ascending and descending4. Bypass grafts between ascending and descending
aortaaorta
Rothman, Abraham. Coarctation of the Aorta: An Update. Current Problems in Pediatrics. 1998; 37-60.

16. Cincinnati Children’s ExperienceCincinnati Children’s Experience
• Preferred approach here is end-to-end anastomosisPreferred approach here is end-to-end anastomosis
• Most important determinant of outcome is how fast it isMost important determinant of outcome is how fast it is
detected and how soon they head to the ORdetected and how soon they head to the OR
• Typically in the OR within 12 hours of admission to CICUTypically in the OR within 12 hours of admission to CICU
• Usual length of stay is 2 days in CICU and a total of 5Usual length of stay is 2 days in CICU and a total of 5
days in the hospitaldays in the hospital (unless very sick prior to OR)(unless very sick prior to OR)
– Less than 5% need re-interventionLess than 5% need re-intervention
Courtesy of Dr. Angela Lorts; Cardiac Critical Care Staff; Cincinnati Children’s Heart Institute

17. Surgical outcomesSurgical outcomes
• Acute mortality ranged from 3% to 32%, strongly correlated withAcute mortality ranged from 3% to 32%, strongly correlated with
complexity of associated cardiovascular lesionscomplexity of associated cardiovascular lesions
• Lowest in those with isolated coarcation (<2%)Lowest in those with isolated coarcation (<2%)
• Restenosis rate was 3-41%Restenosis rate was 3-41%
Rothman, Abraham. Coarctation of the Aorta: An Update. Current Problems in Pediatrics. 1998; 37-60.

18. Survival DataSurvival Data
• Quaegegbeur et alQuaegegbeur et al. reported on a multi-institutional study that looked. reported on a multi-institutional study that looked
at 326 severely symptomatic neonates with coarctation and with orat 326 severely symptomatic neonates with coarctation and with or
without VSD.without VSD.
• The 1 month survival was 93% and the 24 month survival was 84%.The 1 month survival was 93% and the 24 month survival was 84%.
Quaegebeur, J.M, Jonas, R.A, Weinberg, A.D, Blackstone, E.H, Kirklin, J.W. Outcomes in seriously ill neonates with coarctation of the aorta, A multiinstitutional study. The
Journal of Thoracic and Cardiovascular Surgery. 1994; 108: 841-854.

19. Post-operative complicationsPost-operative complications
• HoarsenessHoarseness
• Ipsilateral diaphragm paralysisIpsilateral diaphragm paralysis
• ChylothoraxChylothorax
• Vessel injury/bleedingVessel injury/bleeding
• Rebound HTNRebound HTN
• Post-coartectomy syndromePost-coartectomy syndrome
• Paralysis due to spinal cord ischemiaParalysis due to spinal cord ischemia

20. Long term complicationsLong term complications
• Re-stenosis: influenced by presence of residualRe-stenosis: influenced by presence of residual
ductal tissue within the aortaductal tissue within the aorta
• Hypertension: more likely in repair at a later ageHypertension: more likely in repair at a later age
• Neurologic abnormalitiesNeurologic abnormalities
– Ultrasound abnormalitiesUltrasound abnormalities
– microcephalymicrocephaly

21. Neurologic abnormalitiesNeurologic abnormalities
Preoperative neurobehavioral abnormalities: abnormalPreoperative neurobehavioral abnormalities: abnormal
tone, posturing, weak cry, poor suck, poor auditory andtone, posturing, weak cry, poor suck, poor auditory and
visual orientingvisual orienting
Abnormal ultrasound findings: ventriculomegaly, IVH, basalAbnormal ultrasound findings: ventriculomegaly, IVH, basal
ganglia calcification, widened subarachnoid spaces foundganglia calcification, widened subarachnoid spaces found
preoperativelypreoperatively
Limperopoulos C, Majnemer A, Shevell M, Rosenblatt, Rohlicek C, Tchervenkov C. Neurologic Status of Newborns With Congenital Heart Defects Before Open
Heart Surgery.Pediatrics. 1999; 103(2): 402-408.

22. THANK YOU TO MY ADVISOR
DR. KRAWCZESKI
ANY QUESTIONS?????

23. References
• Brouwer, R.M, Erasmsus, M.E, Ebels, T, Eijgelaar, A. Influence of age on sruvival, late hypertension, and recoarctation in elective aortic coarctationBrouwer, R.M, Erasmsus, M.E, Ebels, T, Eijgelaar, A. Influence of age on sruvival, late hypertension, and recoarctation in elective aortic coarctation
repair: Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years.repair: Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. The Journal of Thoracic andThe Journal of Thoracic and
Cardiovascular SurgeryCardiovascular Surgery. 1994; 108: 525-531.. 1994; 108: 525-531.
• Chang RK, Gurvitz M, rodriguez S. Missed Diagnosis of Critical Congenital Heart Disease.Chang RK, Gurvitz M, rodriguez S. Missed Diagnosis of Critical Congenital Heart Disease. Arch Pediatr Adolesc Med.Arch Pediatr Adolesc Med. 2008; 162(10): 969-974..2008; 162(10): 969-974..
• De-Wahl Granelli, A, Mellander M, Sunnegardh J, Sandberg K, Ostman-Smith I. Screening for duct-dependent congential heart diseaswe with pulseDe-Wahl Granelli, A, Mellander M, Sunnegardh J, Sandberg K, Ostman-Smith I. Screening for duct-dependent congential heart diseaswe with pulse
oximetry: A critical evaluation of strategies to maximize sensitivity.oximetry: A critical evaluation of strategies to maximize sensitivity. Acta PediatricaActa Pediatrica. 2005;94: 1590-1596. 2005;94: 1590-1596
• Gittenberger-De Groot, A.C. Bartelings, M.M. Deruiter, M.C. Poelmann, R.E. Basics of Cardiac Development for the Understanding of Congenital HeartGittenberger-De Groot, A.C. Bartelings, M.M. Deruiter, M.C. Poelmann, R.E. Basics of Cardiac Development for the Understanding of Congenital Heart
Malformations.Malformations. Pediatric Research.Pediatric Research. 2005; 57 (2): 169-176.2005; 57 (2): 169-176.
• Johnson BA and Ades A. Delivery Room and Early Postnatal Management of Neonates Who Have Prenatally Diagnosed Congenital Heart DiseaseJohnson BA and Ades A. Delivery Room and Early Postnatal Management of Neonates Who Have Prenatally Diagnosed Congenital Heart Disease. Clinics. Clinics
in Perinatologin Perinatology. 2005; 32: 921-946.y. 2005; 32: 921-946.
• Limperopoulos C, Majnemer A, Shevell M, Rosenblatt, Rohlicek C, Tchervenkov C. Neurologic Status of Newborns With Congential Heart Defects BeforeLimperopoulos C, Majnemer A, Shevell M, Rosenblatt, Rohlicek C, Tchervenkov C. Neurologic Status of Newborns With Congential Heart Defects Before
Open Heart Surgery.Open Heart Surgery.Pediatrics.Pediatrics. 1999; 103(2): 402-408.1999; 103(2): 402-408.
• Molin, D. DeRuiter, M.C, Wisse, L.J, Azhar, M., Doetschman, T., Poelmann, R. E., Gittenberger-de Groot, A. C. Altered apoptosis pattern during
pharyngeal arch artery remodelling is associated with aortic arch malformations in Tgfβ2 knock-out mice. Cardiovascular Research. 2002; 56: 312-322.
• Polin,Fox,Abman. Fetal and Neonatal Physiology 3rd
edition. Mechanisms Regulating Closure of the Ductus Arteriosis. Saunders. Pennsylvania 2004:
743-747.
• Quaegebeur, J.M, Jonas, RShultz A, Localio A, Clark B, Ravishankar C, Videon N, Kimmel S. Epidemiadn ologic Features of the Presentation of Critical
Congenital Heart Disease: Implications for Screening. Pediatrics. 2008;121(4) 751-757.
• .A, Weinberg, A.D, Blackstone, E.H, Kirklin, J.W. Outcomes in seriously ill neonates with coarctation of the aorta, A multiinstitutional study. The Journal
of Thoracic and Cardiovascular Surgery. 1994; 108: 841-854.
• Rothman, Abraham. Coarctation of the Aorta: An Update. Current Problems in Pediatrics. 1998; 37-60.

24. References
• Sadler, TW. Langman’s Medical Embryology 8th
edition. Philedelphia: Lippincott Williams& Wilkins,2000: 239-243.
• Sharland, G.K, Chan, KY, Allen, LD. Coarctation of the aorta: difficulties in prenatal diagnosis.Sharland, G.K, Chan, KY, Allen, LD. Coarctation of the aorta: difficulties in prenatal diagnosis. British Heart JournalBritish Heart Journal. 1994; 71: 70-75.. 1994; 71: 70-75.
• Weinstein, BM, Stemple, DL, Dreiver W, Fishman, MC. Gridlock, a localized heritable vascular patterning defect in the zebrafish.Weinstein, BM, Stemple, DL, Dreiver W, Fishman, MC. Gridlock, a localized heritable vascular patterning defect in the zebrafish. Nat Med.Nat Med. Nov 1995;Nov 1995;
1(11): 1143-1147.1(11): 1143-1147.
• Wessels, M.W, Berger, R, Frohn-Mulder, I, Roos-Hesselink, J.W, Hoogeboom, J, Mancini, G.S, Bartelings, M.M, De Krijger, R, Wladimiroff, J.W,Wessels, M.W, Berger, R, Frohn-Mulder, I, Roos-Hesselink, J.W, Hoogeboom, J, Mancini, G.S, Bartelings, M.M, De Krijger, R, Wladimiroff, J.W,
Niermeijer, M.F, Grossfeld, P, Willems, P.J. Autosomal Dominant Inheritance of Left Ventricular Outflow Tract Obstruction.Niermeijer, M.F, Grossfeld, P, Willems, P.J. Autosomal Dominant Inheritance of Left Ventricular Outflow Tract Obstruction. American Journal ofAmerican Journal of
Medical GeneticsMedical Genetics. 2005; 134A: 171-179.. 2005; 134A: 171-179.
• Zehr K, Gillinov M, Redmond M, Greene PS, Kan J, Gardner TJ, Reitz B, Cameron D. Repair of Coarctation of the Aorta in Neonates and Infants: AZehr K, Gillinov M, Redmond M, Greene PS, Kan J, Gardner TJ, Reitz B, Cameron D. Repair of Coarctation of the Aorta in Neonates and Infants: A
Thirty-Year Experience.Thirty-Year Experience. Annals of ThoracicAnnals of Thoracic Surgery. 1995; 59: 33-41.Surgery. 1995; 59: 33-41.
• http://www.heartonline.org/congenital.htm
• Title page image: www.odlarmad.com

25. Survival Data based on AgeSurvival Data based on Age
Brouwer, R.M, Erasmsus, M.E, Ebels, T, Eijgelaar, A. Influence of age on sruvival, late hypertension, and recoarctation in elective aortic coarctation
repair: Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. The Journal of Thoracic and
Cardiovascular Surgery. 1994; 108: 525-531.