MODERN DAY APPROACH TO AORTIC COARCTATION

1
MODERN DAY APPROACH TO AORTIC COARCTATION
SUSAN VOSLOO CHRISTIAAN BARNARD MEMORIAL
HOSPITAL CAPE TOWN
2
HISTORY

• 1760 Morgagni
• Congenital narrowing of aorta adjacent to
  attachment of ductus
• Uncommon between LCA LSA, or in lower thoracic
  or abdominal aorta

3
MORPHOLOGY
4
COARCTATION SEGMENT
5
FETAL CIRCULATION
6
CO-EXISTING LEFT HEART 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

7
MAJOR COLLATERAL CHANNELS
8
AGES AT PRESENTATION
1ST OPERATION (92)
RECOARCTATION (8)
(2.2)
2
19 (20.6)
40 (43.5)
3
3
31 (33.7)
2
9
AGES AT CLINICAL PRESENTATION

• NEONATAL PERIOD (40) first month of life (12
  pre-op vent, inotropes incl 5 isolated coarct, 7
  co-existing lesions)
• INFANCY (34) from 1 month - 1 year
• CHILDHOOD (21) age 1 14 years
• ADOLESCENTS AND ADULTS (5) beyond 14 years

10
SPECIAL INVESTIGATIONS

• ECHOCARDIOGRAPHY
• CARDIAC CATHETERIZATION OR AORTOGRAPHY
• MRI
• CT

11
MR AORTIC COARCTATION
12
CT AORTIC COARCTATION
13
PRIMARY ANGIOPLASTY vs SURGERY

• OLDER PATIENTS Primary angioplasty stenting gt
  surgery with comparable if not superior risk
  recurrence rates
• HIGH RISK INFANTS Still better served with
  surgery

14
Do High-Risk Infants Have a Poorer Outcome From
Primary Repair of Coarctation? Analysis of 192
Infants Over 20 yrs (JG McGuinness,et al, Our
Ladys Childrens Hospital, Dublin, Ireland,
AnnThorac Surg 2010 902023-2027)

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

15
Do High-Risk Infants Have a Poorer Outcome From
Primary Repair of Coarctation? Analysis of 192
Infants Over 20 yrs (JG McGuinness,et al, Our
Ladys Childrens Hospital, Dublin, Ireland,
AnnThorac Surg 2010 902023-2027)

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

16
SURGICAL HISTORY

• 1944 Crafoord Nylin
• 1945 Gross
• Original technique resection with end-to-end
  anastomosis (REE)
• Other techniques followed
• Choice of technique mostly based on individual
  preference

17
SURGICAL APPROACH
LEFT THORACOTOMY
18
SURGICAL TECHNIQUES
ALL OPERATIONS (n100)

3
10
14
73
19
SURGICAL TECHNIQUES
FIRST OPERATION (92)
RECOARCTATION (8)
7
14
2
3
71
3
M/s (9)
M/s (2)
20
SIMPLE RESECTION END-END ANASTOMOSIS (SEE)
21
MONITORING PRE-REPAIR
22
MONITORING POST-REPAIR
23
EXTENDED RESECTION END-END ANASTOMOSIS (Amato
1977)
24
GROWTH ARCH RE-INTERVENTION FACTORS

• Mortality (8/36) and arch re-intervention (5/36)
  common in neonates weighing lt 2.5 kgs
• SEE (2/3) EEE (3/16) SCF (7/15) patch
  aortoplasty (1/2)
• Catch-up growth of transverse arch and isthmus
  does occur post coarctation repair, especially in
  smallest arch parameters, where EEE was favoured
• This may be increased using extended rather than
  simple resection and end-to-end anastomosis
• (T Karamlou et al Hosp for Sick
  Children,Toronto J Thorac Cardiovasc Surg 2009
  137 1163-7)

25
ALTERNATIVE SURGICAL TECHNIQUES

• Subclavian flap reversed subclavian flap
• Patch aortoplasty (indirect aortoplasty) Direct
  aortoplasty
• Interposition or Bypass grafts

26
SUBCLAVIAN FLAPWaldhausen Nahrwold 1966
27
REVERSED SUBCLAVIAN FLAP
28
DIRECT ISTHMOPLASTYVosschulte 1957
29
PATCH AORTOPLASTYIndirect Isthmoplasty
30
CAUSES OF ANEURYSM

• 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

31
ANEURYSMS POST COARCTATION REPAIR
Predictors of aneurysm formation after surgical
correction of aortic coarctation (Y von
Kodolitsch, Hamburg, Germany, J Am Coll Cardiol,
2002 39617-624) 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
32
INTERPOSITION GRAFTS Schusler 1962 Brom 1965
33
BYPASS GRAFTSWeldon 1973 Edeie 1975
34
MID-TERM OUTCOMES OF RESECTION EEE

• 201 pts coarctation without/with VSD (14)
• Neonates (53) pre-op shock(20)
• Sternotomy 44 pts (22) thoracotomy 157 pts
  (78)
• Early mortality 2 (PHTCDH, MAS, MOF, RSV)
• Re-intervention 8 pts (3 balloon angioplasty 5
  re-ops 75 in 1st po yr)
• (S Kaushal Childrens Memorial Hosp, Chicago
  Ann Thor Surg 2009 88 1932-8)

35
OUTCOME - MORTALITY

• No deaths lt 1 month or gt 1 year
• 2 early deaths (both hospitalized since birth)
• 1. F, ex-prem, 6 weeks, 1.8 kg, pre-op vent,
  Coarctation AP Window, po pneumonia, ECMO day
  5-19, off ECMO, recurrent pneumonia week later,
  died respiratory failure
• 2. F, ex-prem, 3 months, 2.1 kg, large
  hydrocephalus, massive pericardial effusion,
  Klebsiella septicaemia, died day 7 po
• No late deaths, including all subsequent surgery
  for intracardiac repairs post palliation

36
OUTCOME EARLY MORBIDITY

• Transient Hypertension common
• PO Ventilation gt 3 days (3 2 died)
• Phrenic Nerve injury(2) Both required
  diaphragmatic plication
• Chylothorax (2) 1 thoracic duct ligation
• No postop bleeding, spinal cord complications

37
FACTORS DETERMINING SPINAL CORD INJURY RISK

• The location and length of narrowing
• The presence of the collateral circulation
• The clamping time required for the procedure

38
OUTCOME LATE MORBIDITY

• PPM (2) LV dysfunction at 1 4 yrs
• Late Aneurysms nil
• Hypertension continuous anti-HT therapy (2)
• Recoarctation ( 8 single balloon angioplasty lt
  6m 2 at 4 6 yrs po 1 redo surgery REE patch
  at 6m)

39
CAUSES AORTIC RECOARCTATION
40
PATIENTS (n100)

• ISOLATED COARCTATION (66) including 12 pts with
  stable left heart obstructive lesions, being
  observed
• CO-EXISTING CARDIAC LESIONS (34)
• M 58 F 42
• PRIMARY OPERATION (92)
• RECOARCTATION (8)

41
CO-EXISTING CARDIAC DEFECTS (n46/100)

• Bicuspid Aortic Valve (8)
• Stable Shone complex (4) (12)
• Significant LVOTO (5) (34)
• VSD (16)
• Other (13)
• DORV (4) TGAVSD (2) UVH (5) AP-window (1) IHD
  (1)

42
COARCTATION PLUS SIGNIFICANT LVOTO (n 5)

• AORTIC VALVOTOMY (3)
• Aortic valvotomy with aortic coarctation (1),
  Aortic valvotomy at 3 5 months post coarct (2)
• PROGRESSIVE LVOTO POST-COARCT REPAIR
• Ross procedure at 5 yrs (1)
• Resection Subaortic stenosis at 4 yrs,then
  Ross-Konno at 10 yrs (1)

43
COARCTATION PLUS VSD (n 16)

• RECOARCTATION (4)
• Primary VSD coarctation (2)
• PAB coarctation later VSD closure (2)
• PRIMARY VSD COARCTATION (3)
• PAB COARCTATION (9)
• CBMH later VSD closure _at_ 4-22m age (5)
• RXH all awaiting definitive procedures (4)

44
COARCTATION WITH OTHER CARDIAC DEFECTS (n13)

• Primary repair with coarctation (5)
• - APW (1),
• - IHD (LIMA LAD) (1)
• - TGA VSD primary ASO VSD (1),
• - DORV (2)
• Palliation PAB (8)
• TGA VSD at 11m (1),
• DORV at 11 15 m(2)
• UVH Glenn (3/5), TCPC (1/3) - Awaiting
  repairs(2)

45
THANK YOU!