Coarctation of the AortaCoarctation of the Aorta

Liz Wetzel, MDLiz Wetzel, MD

CICU RoundsCICU Rounds

November 19, 2010November 19, 2010

ObjectivesObjectives• Review Anatomy/Lesion DevelopmentReview Anatomy/Lesion Development

• Discuss DR Presentation and ManagementDiscuss DR Presentation and Management

• Review Post-natal Evaluation and Transport Review Post-natal Evaluation and Transport

• Describe possible presentation in non-prenatally Describe 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

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.

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.

Development of CoarctationDevelopment of Coarctation• Abnormal development of Abnormal development of

left 4left 4thth and 6 and 6thth aortic arches aortic arches

• Represents 5-10% of all Represents 5-10% of all congenital cardiac lesions congenital cardiac lesions

• More common in boys than More common in boys than girlsgirls

• No real impact prior to birth No real impact prior to birth due to presence of PDA due to presence of PDA unless there is fetal closureunless there is fetal closure

Ductus Tissue TheoryDuctus Tissue Theory

• Due to a migration of Due to a migration of ductus smooth muscle cells ductus smooth muscle cells into the periductal aorta into the periductal aorta with subsequent with subsequent constriction and narrowing constriction and narrowing of the aortic lumenof the aortic lumen

• Evident when ductus closesEvident when ductus closes

Hemodynamic TheoryHemodynamic Theory

• Reduced intrauterine Reduced intrauterine blood flow causes blood flow causes underdevelopment of underdevelopment of aortic archaortic arch

• Results from reduced Results from reduced volume of blood flow volume of blood flow through the fetal aortic through the fetal aortic arch and isthmus arch and isthmus

Other TheoriesOther Theories• May be due to a defect in the vascular wall of the May be due to a defect in the vascular wall of the

ascending aortaascending aorta

• Vascular apoptosis may have a role Vascular apoptosis may have a role (Molin et al 2002)(Molin et al 2002)

• Recessive genetic mutation found in zebrafish Recessive genetic mutation found in zebrafish (Weinstein et (Weinstein et al 1995)al 1995)

• Autosomal dominant inheritance of non-syndromic left Autosomal dominant inheritance of non-syndromic left ventricular outflow tract obstruction ventricular outflow tract obstruction (Wessels et al 2005)(Wessels et al 2005)

DR Presentation & ManagementDR Presentation & Management

• Follow normal NRP guidelines for resuscitation and be Follow 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 acute This 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

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 infusion infusion (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 them Main goal is to stabilize the patient and get them transferred to a cardiac intensive care unittransferred to a cardiac intensive care unit

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 in infusion can cause vasodilatation and can result in relative hypovolemia in neonatesrelative hypovolemia in neonates

• Report recent blood gas with electrolytes including ionized Report 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

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 extremity signs 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

Potential CXR ProgressionPotential CXR Progression

DAY FIVE OF LIFEDAY FIVE OF LIFEDAY THREE OF LIFEDAY THREE OF LIFE

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. al remodeling resulting in permanent closure (Koch et. al 2006)2006)

•In term infants In term infants functional closure functional closure can occur as early as can occur as early as 12-1512-15 hours of age,hours of age, if greater than 72 hours it is if greater than 72 hours it is considered persistent, considered persistent, truetrue anatomicanatomic closureclosure can take can take weeksweeks

Neoreviews Controversies in the Management of PDA (Gien 2008)

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)

Potential surgical interventionPotential surgical intervention

• First surgery was done experimentally in animals in 1944 First 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 descending 4. Bypass grafts between ascending and descending

aortaaorta

Rothman, Abraham. Coarctation of the Aorta: An Update. Current Problems in Pediatrics. 1998; 37-60.

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 is Most 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 5 Usual length of stay is 2 days in CICU and a total of 5 days in the hospital days 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

Surgical outcomesSurgical outcomes

• Acute mortality ranged from 3% to 32%, strongly correlated with Acute 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.

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 or at 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.

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

Long term complicationsLong term complications

• Re-stenosis: influenced by presence of residual Re-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

Neurologic abnormalitiesNeurologic abnormalities

Preoperative neurobehavioral abnormalities: abnormal Preoperative neurobehavioral abnormalities: abnormal tone, posturing, weak cry, poor suck, poor auditory and tone, posturing, weak cry, poor suck, poor auditory and visual orienting visual orienting

Abnormal ultrasound findings: ventriculomegaly, IVH, basal Abnormal ultrasound findings: ventriculomegaly, IVH, basal ganglia calcification, widened subarachnoid spaces found ganglia 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.

THANK YOU TO MY ADVISOR

DR. KRAWCZESKI

ANY QUESTIONS?????

References• Brouwer, R.M, Erasmsus, M.E, Ebels, T, Eijgelaar, A. Influence of age on sruvival, late hypertension, and recoarctation in elective aortic coarctation 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. repair: Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. The Journal of Thoracic and The 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 pulse De-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 Heart 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. 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 Before Limperopoulos 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.

References• Sadler, TW. Langman’s Medical Embryology 8 th 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 of American 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: A 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: A Thirty-Year Experience. Thirty-Year Experience. Annals of Thoracic Annals of Thoracic Surgery. 1995; 59: 33-41.Surgery. 1995; 59: 33-41.

• http://www.heartonline.org/congenital.htm

• Title page image: www.odlarmad.com

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.