MODERN DAY APPROACH TO AORTIC COARCTATION SUSAN VOSLOO

MODERN DAY APPROACH TO AORTIC COARCTATION SUSAN VOSLOO

MODERN DAY APPROACH TO AORTIC COARCTATION SUSAN VOSLOO CHRISTIAAN BARNARD MEMORIAL HOSPITAL CAPE TOWN HISTORY 1760 Morgagni Congenital narrowing of aorta adjacent to attachment of ductus

Uncommon between LCA & LSA, or in lower thoracic or abdominal aorta AORTIC COARCTATION MORPHOLOGY AORTIC COARCTATION COARCTATION

SEGMENT AORTIC COARCTATION FETAL CIRCULATION AORTIC COARCTATION 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 AORTIC COARCTATION

MAJOR COLLATERAL CHANNELS AORTIC COARCTATION AGES AT PRESENTATION 1ST OPERATION (92) RECOARCTATION (8)

(2.2%) 2 19 40 (20.6%) (43.5%) 3 31

(33.7%) 3 2 AORTIC COARCTATION 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 AORTIC COARCTATION SPECIAL INVESTIGATIONS

ECHOCARDIOGRAPHY CARDIAC CATHETERIZATION OR AORTOGRAPHY MRI CT AORTIC COARCTATION MR AORTIC COARCTATION AORTIC COARCTATION

CT AORTIC COARCTATION AORTIC COARCTATION PRIMARY ANGIOPLASTY vs SURGERY OLDER PATIENTS: Primary angioplasty & stenting > surgery with comparable if not superior risk

& recurrence rates HIGH RISK INFANTS: Still better served with surgery AORTIC COARCTATION 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; 90:2023-2027) 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

AORTIC COARCTATION 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; 90:2023-2027) 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 AORTIC COARCTATION

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 AORTIC COARCTATION

SURGICAL APPROACH LEFT THORACOTOMY AORTIC COARCTATION SURGICAL TECHNIQUES ALL OPERATIONS (n=100) 10 3

14 73 AORTIC COARCTATION SURGICAL TECHNIQUES FIRST OPERATION (92) RECOARCTATION (8)

7 14 REE SCF 71 M/s (9)

2 3 Patch Graf t AORTIC COARCTATION

3 M/s (2) SIMPLE RESECTION & ENDEND ANASTOMOSIS (SEE) AORTIC COARCTATION MONITORING PRE-REPAIR

AORTIC COARCTATION MONITORING POST-REPAIR AORTIC COARCTATION EXTENDED RESECTION & ENDEND ANASTOMOSIS (Amato 1977) AORTIC COARCTATION

GROWTH & ARCH REINTERVENTION Mortality (8/36) and arch re-intervention (5/36) FACTORS common in neonates weighing < 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) AORTIC COARCTATION ALTERNATIVE SURGICAL TECHNIQUES Subclavian flap & reversed

subclavian flap Patch aortoplasty (indirect aortoplasty) & Direct aortoplasty Interposition or Bypass grafts AORTIC COARCTATION SUBCLAVIAN FLAP Waldhausen & Nahrwold 1966

AORTIC COARCTATION REVERSED SUBCLAVIAN FLAP AORTIC COARCTATION DIRECT ISTHMOPLASTY Vosschulte 1957

AORTIC COARCTATION PATCH AORTOPLASTY Indirect Isthmoplasty AORTIC COARCTATION 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 AORTIC COARCTATION ANEURYSMS POST

COARCTATION REPAIR 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 AORTIC COARCTATION favoured ascending aneurysm formation INTERPOSITION GRAFTS Schusler 1962 Brom 1965

AORTIC COARCTATION BYPASS GRAFTS Weldon 1973 Edeie 1975 AORTIC COARCTATION 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% (PHT&CDH, 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)

AORTIC COARCTATION OUTCOME - MORTALITY No deaths < 1 month or > 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 AORTIC COARCTATION OUTCOME EARLY MORBIDITY Transient Hypertension common PO Ventilation > 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 AORTIC COARCTATION 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 AORTIC COARCTATION 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 < 6m; 2 at 4 & 6 yrs po; 1 redo surgery REE patch at 6m) AORTIC COARCTATION

CAUSES AORTIC RECOARCTATION AORTIC COARCTATION PATIENTS (n=100) 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) AORTIC COARCTATION CO-EXISTING CARDIAC DEFECTS (n=46/100) Bicuspid Aortic Valve (8) Stable Shone complex (4) Significant LVOTO (5)

VSD (16) Other (13) (12) (34) DORV (4) TGA&VSD (2) UVH (5) APwindow (1) IHD (1) AORTIC COARCTATION 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 RossKonno at 10 yrs (1) AORTIC COARCTATION COARCTATION PLUS

VSD RECOARCTATION (n =(4) 16) Primary VSD & coarctation (2) PAB & coarctation; later VSD closure (2) PRIMARY VSD & COARCTATION (3) PAB & COARCTATION (9)

CBMH; later VSD closure @ 4-22m age (5) RXH; all awaiting definitive procedures (4) AORTIC COARCTATION COARCTATION WITH OTHER CARDIAC DEFECTS (n=13) 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)

AORTIC COARCTATION THANK YOU!

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