MODERN DAY APPROACH MODERN DAY APPROACH TO AORTIC COARCTATIONTO AORTIC COARCTATION

SUSAN VOSLOO CHRISTIAAN BARNARD MEMORIAL HOSPITAL

CAPE TOWN

HISTORYHISTORY

1760 Morgagni 1760 Morgagni Congenital narrowing of aorta Congenital narrowing of aorta

adjacent to attachment of ductusadjacent to attachment of ductus

Uncommon between LCA & LSA, Uncommon between LCA & LSA, or in lower thoracic or abdominal or in lower thoracic or abdominal aortaaorta

AORTIC COARCTATION

MORPHOLOGYMORPHOLOGY

AORTIC COARCTATION

COARCTATION COARCTATION SEGMENTSEGMENT

AORTIC COARCTATION

FETAL CIRCULATIONFETAL CIRCULATION

AORTIC COARCTATION

CO-EXISTING LEFT HEART CO-EXISTING LEFT HEART ANOMALIES (up to 50%)ANOMALIES (up to 50%)

Supravalvar mitral ring Mitral stenosis with or without a single

papillary muscle (parachute mitral valve) Endomyocardial fibrosis Left ventricular hypoplasia or hypertrophy Aortic atresia and hypoplasia of ascending

aorta Supra-valvar, valvar, sub-valvar aortic

stenosis or hypoplasia

AORTIC COARCTATION

MAJOR COLLATERAL MAJOR COLLATERAL CHANNELSCHANNELS

AORTIC COARCTATION

AGES AT PRESENTATIONAGES AT PRESENTATION

AORTIC COARCTATION

1ST OPERATION (92) RECOARCTATION (8)

3

2

3 40(43.5%)

31(33.7%)

19(20.6%)

2(2.2%)

AGES AT CLINICAL AGES AT CLINICAL PRESENTATIONPRESENTATION

NEONATAL PERIOD NEONATAL PERIOD (40) (40) first month of first month of life (12 pre-op vent, inotropes incl 5 life (12 pre-op vent, inotropes incl 5 isolated coarct, 7 co-existing lesions) isolated coarct, 7 co-existing lesions)

INFANCY INFANCY (34) (34) from 1 month - 1 yearfrom 1 month - 1 yearCHILDHOOD CHILDHOOD (21) (21) age 1 – 14 yearsage 1 – 14 yearsADOLESCENTS AND ADULTS ADOLESCENTS AND ADULTS (5)(5)

beyond 14 years beyond 14 years

AORTIC COARCTATION

SPECIAL SPECIAL INVESTIGATIONSINVESTIGATIONS

ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

CARDIAC CATHETERIZATION OR CARDIAC CATHETERIZATION OR AORTOGRAPHYAORTOGRAPHY

MRIMRICTCT

AORTIC COARCTATION

MR AORTIC COARCTATIONMR AORTIC COARCTATION

AORTIC COARCTATION

CT AORTIC COARCTATIONCT AORTIC COARCTATION

AORTIC COARCTATION

PRIMARY ANGIOPLASTY PRIMARY ANGIOPLASTY vs SURGERY vs SURGERY

OLDER PATIENTS:OLDER PATIENTS: Primary Primary angioplasty & stenting > surgery angioplasty & stenting > surgery with comparable if not superior risk with comparable if not superior risk & recurrence rates& recurrence rates

HIGH RISK INFANTS: HIGH RISK INFANTS: Still better Still better served with surgeryserved with surgery

AORTIC COARCTATION

Do High-Risk Infants Have a Poorer Outcome From Do High-Risk Infants Have a Poorer Outcome From Primary Repair of Coarctation? Primary Repair of Coarctation? Analysis of 192 Infants Analysis of 192 Infants

Over 20 yrs Over 20 yrs (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin, (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin,

Ireland, AnnThorac Surg 2010; 90:2023-2027)Ireland, AnnThorac Surg 2010; 90:2023-2027)

Primary angioplasty reports Primary angioplasty reports ( 8 studies last 10 yrs):( 8 studies last 10 yrs):6 studies represented only low risk pts, no initial 6 studies represented only low risk pts, no initial mortality, re-intervention rate of 14-83%mortality, re-intervention rate of 14-83%2 studies included high risk patients:2 studies included high risk patients:- mortality 17 & 21%mortality 17 & 21%- re-intervention 73% in 10 days, 77% by 12 yrsre-intervention 73% in 10 days, 77% by 12 yrs Both studies reported lost femoral pulses 12-18%, Both studies reported lost femoral pulses 12-18%, long term sequelae unknown long term sequelae unknown

AORTIC COARCTATION

Do High-Risk Infants Have a Poorer Outcome From Do High-Risk Infants Have a Poorer Outcome From Primary Repair of Coarctation? Analysis of 192 Infants Primary Repair of Coarctation? Analysis of 192 Infants

Over 20 yrs Over 20 yrs (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin, Ireland, (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin, Ireland,

AnnThorac Surg 2010; 90:2023-2027)AnnThorac Surg 2010; 90:2023-2027)

Higher vs lower risk surgical pts Higher vs lower risk surgical pts (pre-op PG, (pre-op PG, ventilation, LV dysfunction, inotropic support) were: ventilation, LV dysfunction, inotropic support) were: -Smaller (3.3 vs 4.2 kg), younger (18 vs 57 days), Smaller (3.3 vs 4.2 kg), younger (18 vs 57 days), PAB (25 vs 15%),PAB (25 vs 15%),- same technique, similar X-clamp times same technique, similar X-clamp times -mortality(7 vs 3%), recurrence (11%) mortality(7 vs 3%), recurrence (11%) -treated easily with single balloon angioplasty,mean treated easily with single balloon angioplasty,mean 3.8 yrs later3.8 yrs later

AORTIC COARCTATION

SURGICAL HISTORYSURGICAL HISTORY

1944 Crafoord & Nylin1944 Crafoord & Nylin1945 Gross1945 GrossOriginal technique resection with Original technique resection with

end-to-end anastomosis (REE)end-to-end anastomosis (REE)Other techniques followedOther techniques followedChoice of technique mostly based Choice of technique mostly based

on individual preferenceon individual preference

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SURGICAL APPROACHSURGICAL APPROACH

AORTIC COARCTATION

LEFT THORACOTOMY

SURGICAL TECHNIQUESSURGICAL TECHNIQUES

AORTIC COARCTATION

ALL OPERATIONS (n=100)

73

14

103

SURGICAL TECHNIQUESSURGICAL TECHNIQUES

AORTIC COARCTATION

FIRST OPERATION (92) RECOARCTATION (8)

23

3

14

71

7

M/s (9) M/s (2)

SIMPLE RESECTION & END-SIMPLE RESECTION & END-END ANASTOMOSIS (SEE)END ANASTOMOSIS (SEE)

AORTIC COARCTATION

MONITORING PRE-REPAIRMONITORING PRE-REPAIR

AORTIC COARCTATION

MONITORING POST-REPAIRMONITORING POST-REPAIR

AORTIC COARCTATION

EXTENDED RESECTION & END-EXTENDED RESECTION & END-END ANASTOMOSIS (Amato END ANASTOMOSIS (Amato

1977) 1977)

AORTIC COARCTATION

GROWTH & ARCH RE-GROWTH & ARCH RE-INTERVENTION INTERVENTION

FACTORSFACTORS

AORTIC COARCTATION

Mortality (8/36) and arch re-intervention (5/36) Mortality (8/36) and arch re-intervention (5/36) common in neonates weighing < 2.5 kgscommon in neonates weighing < 2.5 kgs

SEE (2/3); EEE (3/16); SCF (7/15); patch aortoplasty SEE (2/3); EEE (3/16); SCF (7/15); patch aortoplasty (1/2)(1/2)

Catch-up growth of transverse arch and isthmus Catch-up growth of transverse arch and isthmus does occur post coarctation repair, especially in does occur post coarctation repair, especially in smallest arch parameters, where EEE was favouredsmallest arch parameters, where EEE was favoured

This may be increased using extended rather than This may be increased using extended rather than simple resection and end-to-end anastomosissimple resection and end-to-end anastomosis

(T Karamlou et al: Hosp for Sick Children,Toronto; J Thorac (T Karamlou et al: Hosp for Sick Children,Toronto; J Thorac Cardiovasc Surg 2009; 137: 1163-7)Cardiovasc Surg 2009; 137: 1163-7)

ALTERNATIVE ALTERNATIVE SURGICAL TECHNIQUESSURGICAL TECHNIQUES

Subclavian flap & reversed Subclavian flap & reversed subclavian flapsubclavian flap

Patch aortoplasty (indirect Patch aortoplasty (indirect aortoplasty) & Direct aortoplastyaortoplasty) & Direct aortoplasty

Interposition or Bypass graftsInterposition or Bypass grafts

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SUBCLAVIAN FLAPSUBCLAVIAN FLAPWaldhausen & Nahrwold 1966Waldhausen & Nahrwold 1966

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REVERSED SUBCLAVIAN REVERSED SUBCLAVIAN FLAP FLAP

AORTIC COARCTATION

DIRECT ISTHMOPLASTYDIRECT ISTHMOPLASTYVosschulte 1957Vosschulte 1957

AORTIC COARCTATION

PATCH AORTOPLASTYPATCH AORTOPLASTYIndirect IsthmoplastyIndirect Isthmoplasty

AORTIC COARCTATION

CAUSES OF ANEURYSMCAUSES OF ANEURYSM

AORTIC COARCTATION

• Accelerated proximal aortic wall growth due to compliance mismatch

• Cystic medial necrosis in aortic wall adjacent to coarctation

• Disruption of intima or sub-intima with or without patch aortoplasty

• Infection

ANEURYSMS POST ANEURYSMS POST COARCTATION REPAIRCOARCTATION REPAIR

AORTIC COARCTATION

Predictors of aneurysm formation after surgical correction of aortic coarctation(Y von Kodolitsch, Hamburg, Germany, J Am Coll Cardiol, 2002; 39:617-624) Reported 25 aneurysms (9% of coarctation repairs),8 ascending, 17 local aneurysms, with 36% mortality if left untreated Independent predictors for aneurysm formation:* Higher age at repair (72% had surgery after age 13.5 yrs) * Patch graft technique* Higher pre-op gradient & bicuspid aortic valve favoured ascending aneurysm formation

INTERPOSITION INTERPOSITION GRAFTS GRAFTS Schusler 1962 Brom Schusler 1962 Brom

19651965

AORTIC COARCTATION

BYPASS GRAFTSBYPASS GRAFTSWeldon 1973 Edeie 1975Weldon 1973 Edeie 1975

AORTIC COARCTATION

MID-TERM OUTCOMES MID-TERM OUTCOMES OF RESECTION & EEEOF RESECTION & EEE

201 pts coarctation without/with VSD (14%)201 pts coarctation without/with VSD (14%) Neonates (53%); pre-op shock(20%)Neonates (53%); pre-op shock(20%) Sternotomy 44 pts (22%); thoracotomy 157 Sternotomy 44 pts (22%); thoracotomy 157

pts (78%)pts (78%) Early mortality 2% (PHT&CDH, MAS, MOF, Early mortality 2% (PHT&CDH, MAS, MOF,

RSV)RSV) Re-intervention 8 pts (3 balloon angioplasty; 5 Re-intervention 8 pts (3 balloon angioplasty; 5

re-ops; 75% in 1re-ops; 75% in 1stst po yr) po yr)(S Kaushal; Children’s Memorial Hosp, Chicago; Ann Thor (S Kaushal; Children’s Memorial Hosp, Chicago; Ann Thor

Surg 2009; 88: 1932-8)Surg 2009; 88: 1932-8)AORTIC COARCTATION

OUTCOME - MORTALITYOUTCOME - MORTALITY

No deaths < 1 month or > 1 yearNo deaths < 1 month or > 1 year 2 early deaths (both hospitalized since birth)2 early deaths (both hospitalized since birth)1. F, ex-prem, 6 weeks, 1.8 kg, pre-op vent, Coarctation & AP 1. F, ex-prem, 6 weeks, 1.8 kg, pre-op vent, Coarctation & AP

Window, po pneumonia, ECMO day 5-19, off ECMO, Window, po pneumonia, ECMO day 5-19, off ECMO, recurrent pneumonia week later, died respiratory failurerecurrent pneumonia week later, died respiratory failure

2. F, ex-prem, 3 months, 2.1 kg, large hydrocephalus, 2. F, ex-prem, 3 months, 2.1 kg, large hydrocephalus, massive pericardial effusion, Klebsiella septicaemia, died massive pericardial effusion, Klebsiella septicaemia, died day 7 poday 7 po

No late deaths, No late deaths, including all subsequent surgery for including all subsequent surgery for intracardiac repairs post palliationintracardiac repairs post palliation

AORTIC COARCTATION

OUTCOME – EARLY OUTCOME – EARLY MORBIDITYMORBIDITY

Transient Hypertension commonTransient Hypertension commonPO Ventilation > 3 days (3 – PO Ventilation > 3 days (3 – 2 died2 died) ) Phrenic Nerve injury(2)Phrenic Nerve injury(2); Both required ; Both required

diaphragmatic plicationdiaphragmatic plicationChylothorax (2); Chylothorax (2); 1 thoracic duct ligation1 thoracic duct ligationNo postop bleeding, spinal cord No postop bleeding, spinal cord

complicationscomplications

AORTIC COARCTATION

FACTORS DETERMINING FACTORS DETERMINING SPINAL CORD INJURY RISKSPINAL CORD INJURY RISK

The location and length of narrowing

The presence of the collateral circulation

The clamping time required for the procedure

AORTIC COARCTATION

OUTCOME – LATE OUTCOME – LATE MORBIDITYMORBIDITY

PPM (2) – LV dysfunction at 1 & 4 yrs PPM (2) – LV dysfunction at 1 & 4 yrs Late Aneurysms Late Aneurysms – nil – nil Hypertension – Hypertension – continuous anti-HT continuous anti-HT

therapy (2)therapy (2)

RecoarctationRecoarctation ( 8 single balloon ( 8 single balloon angioplasty < 6m; 2 at 4 & 6 yrs po; angioplasty < 6m; 2 at 4 & 6 yrs po; 1 redo surgery REE – patch at 6m)1 redo surgery REE – patch at 6m)

AORTIC COARCTATION

CAUSES AORTIC CAUSES AORTIC RECOARCTATIONRECOARCTATION

AORTIC COARCTATION

PATIENTS (n=100)PATIENTS (n=100)

ISOLATED COARCTATION ISOLATED COARCTATION (66) including (66) including 12 pts with stable left heart obstructive 12 pts with stable left heart obstructive lesions, being observedlesions, being observed

CO-EXISTING CARDIAC LESIONSCO-EXISTING CARDIAC LESIONS (34) (34)

M 58; F 42M 58; F 42

PRIMARY OPERATION (92)PRIMARY OPERATION (92)RECOARCTATION (8)RECOARCTATION (8)

AORTIC COARCTATION

CO-EXISTING CARDIAC CO-EXISTING CARDIAC DEFECTS (n=46/100)DEFECTS (n=46/100)

Bicuspid Aortic Valve (8)Bicuspid Aortic Valve (8)Stable Shone complex (4) Stable Shone complex (4) (12)(12)

Significant LVOTO (5) Significant LVOTO (5) (34)(34)VSD (16)VSD (16)Other (13)Other (13)

DORV (4) TGA&VSD (2) UVH (5) AP-DORV (4) TGA&VSD (2) UVH (5) AP-window (1) IHD (1) window (1) IHD (1)

AORTIC COARCTATION

COARCTATION PLUS COARCTATION PLUS SIGNIFICANT LVOTOSIGNIFICANT LVOTO (n =5) (n =5)

AORTIC VALVOTOMY (3) AORTIC VALVOTOMY (3)

Aortic valvotomy with aortic coarctation (1), Aortic valvotomy with aortic coarctation (1), Aortic valvotomy at 3 & 5 months post coarct Aortic valvotomy at 3 & 5 months post coarct (2)(2)

PROGRESSIVE LVOTO POST-COARCT REPAIR PROGRESSIVE LVOTO POST-COARCT REPAIR

Ross procedure at 5 yrs (1) Ross procedure at 5 yrs (1)

Resection Subaortic stenosis at 4 yrs,then Ross-Resection Subaortic stenosis at 4 yrs,then Ross-Konno at 10 yrs (1)Konno at 10 yrs (1)

AORTIC COARCTATION

COARCTATION PLUS COARCTATION PLUS VSDVSD

(n = 16)(n = 16) RECOARCTATIONRECOARCTATION (4) (4)

Primary VSD & coarctation (2)Primary VSD & coarctation (2)

PAB & coarctation; later VSD closure (2)PAB & coarctation; later VSD closure (2) PRIMARY VSD & COARCTATION PRIMARY VSD & COARCTATION (3)(3) PAB & COARCTATION PAB & COARCTATION (9)(9)

CBMH; later VSD closure @ 4-22m age (5)CBMH; later VSD closure @ 4-22m age (5)

RXH; all awaiting definitive procedures (4)RXH; all awaiting definitive procedures (4)

AORTIC COARCTATION

COARCTATION WITH OTHER COARCTATION WITH OTHER CARDIAC DEFECTS (n=13)CARDIAC DEFECTS (n=13)

Primary repair with coarctation (5)Primary repair with coarctation (5)- APW (1), - APW (1),

- IHD (LIMA – LAD) (1); - IHD (LIMA – LAD) (1);

- TGA & VSD primary ASO & VSD (1), - TGA & VSD primary ASO & VSD (1),

- DORV (2)- DORV (2)

Palliation PAB (8)Palliation PAB (8)- TGA & VSD at 11m (1), TGA & VSD at 11m (1), - DORV at 11 & 15 m(2) DORV at 11 & 15 m(2) - UVH: Glenn (3/5), TCPC (1/3) - Awaiting repairs(2)UVH: Glenn (3/5), TCPC (1/3) - Awaiting repairs(2)

AORTIC COARCTATION

THANK YOU!THANK YOU!