Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was reviewed for 42 children over a 34-year period. The main surgical techniques were coronary reimplantation and an intrapulmonary baffle. Early mortality was 2.4% and long-term survival was 98%. Left ventricular function normalized in most patients by 9.5 months. Concomitant mitral valve repair was performed in 21% of patients, with 71% freedom from reoperation at 10 years. Late mitral valve intervention was required in 19% of patients overall. Surgical repair of ALCAPA can be achieved with good long-term outcomes, though mitral regurgitation and late mitral valve

1.
JOURNAL PRESENTATION
Dr. ANUJ MEHTA

2. Surgical Intervention for Anomalous Origin of Left Coronary Artery
From the Pulmonary Artery in Children : A Long-Term Follow Up
• THE ANNALS OF THORACIC SURGERY : Volume 101 . Number 5 . May 2016
• Authors :Phillip S. Naimo ,MD Tyson A. Fricke, Yves d’Udekem, MD ,PhD,Andrew D. Cochrane
MBBS, Christian P. Brizard, MD et al.
• Institutions:
• Department of cardiothoracic surgery ,Royal Children’s Hospital Melbourne;
• Department of Paediatrics, University of Melbourne,
• Murdoch Children’s Research Institute ,Melbourne.
• Monash Medical Centre,Melbourne
• Princess Margaret Hospital for Children,Perth
• Women’s and Children’s Hospital ,Adelaide .

3. INTRODUCTION
• Anomalous left coronary artery from the pulmonary artery is a rare
congenital anomaly (1:30,000 to 300,000 )
• If untreated mortality at 1 year of age is 90%
• Left ventricular dysfunction and ischaemic mitral regurgitation
secondary to mitral annular dilatation and ischaemic papillary
muscle dysfunction

4. Different surgical techniques
• Intrapulmonary artery baffle or Takeuchi repair
• Coronary reimplantation to the aorta
• Ligation of the anomalous artery
• Bypass grafting

5. Intrapulmonary artery baffle or Takeuchi repair

6. Coronary Reimplantation

7. • The decision to repair the Mitral valve remains controversial .
• After revascularisation of anterior wall MV may become reasonable
despite severe pre operative MR
• Early outcomes of ALCAPA repair are good
• Previously reported mid term results.[J Thorac Cardiovasc Surg
1999;117:332-42]

8. AIMS
• To review the surgical management of ALCAPA repair
• To evaluate associated MV disease , its management and
• Long term outcomes at Royal children’s hospital

9. MATERIALS AND METHODOLOGY
Approval
• The Human ethics committee at the Royal Children’s Hospital
• Study duration - 1980 to 2014
• Sample size - 42
• Retrospective data collection from medical records from first
admission to last follow-up

10. Definitions
• Early mortality- death within 30 days of operation or before hospital
discharge
• all others considered late deaths
• Severity of MR using conventional guidelines

11. Data Analysis
• Stata , version 12 (StataCorp LP, College Station ,TX)
• Descriptive statistics for —
continuous data were expressed as mean +- standard deviations
skewed continuous data as median.
• Categorical data as - frequencies and percentages
• Kaplan Meier curve was used to analyse and plot time-related end points.
p<0.05 significant.

12. RESULTS
• FEBRUARY 1980 - MARCH
2014 - 42 Children
underwent ALCAPA repair
• Median age - 140 days
34 <1 year of age
• Median weight - 5.8 kg
PRE OPERATIVE CHARACTERISTICS

13. • Reimplantation - 29 (69% [29 of 42] )
• Intrapulmonary baffle (Takeuchi repair) - 12 (29% [12 of 42])
• Ligation of anomalous left circumflex artery arising from PA -1 (2%)
• Concomitant MV repair - 9 (21% [9 of 42]
• 8 - REIMPLANTATION 1 TAKEUCHI REPAIR
• Additional repair -1 (incipient LV rupture through a transmural
infarct -resected and reconstructed)

14. MV REPAIR NO REPAIR p
MEDIAN AGE 147 days 141days 0.87
MEAN CPB
TIME
184+/-50 min 137+/-79 min 0.09
MEAN ACC
TIME
112+/-22 70+/- 39 0.006

15. • 15 patients required LVAD at completion of surgery
• 12 of 15 were noted to have significant rise in LA pressure and
reduction in MAP after weaning off CPB
• 3 of 15 were put electively on LVAD
• ACC without LVAD - 79+/- 45 min with LVAD 95+/- 40 min
[p=0.35]
• LVAD removed at mean of 4 +/- 2 days (range 2-8 days)
• ECMO (n=1) on POD-2 (cardiac arrest and respiratory failure)
wean-off next day.

16. Mortality
• Early mortality - 2.4% (1 of 42)
• 11 month old girl child 6.5 kg
• CCF with mild MR, severe LV dysfunction
• ALCAPA repair through reimplantation
• post op LV remained poor supported with LVAD
• POD-4 developed cerebral infarct secondary to thromboembolism and died
• NO late deaths
• Overall Survival - 98% +/- 2 % at 5 , 10 and 20 years follow up

17. Management of the Mitral Valve
• Pre operatively
• mild - 13 (31%)
• moderate -18 (43%)
• severe - 11 (26%)
26%
43%
31%

18. • Concomitant MV repair - 9 (21% [9 of 42]
• severe MR - 5
• moderate MR - 4
• Suture annuloplasty -6
• plication -2
• ring annuloplasty - 1
suture annuloplasty
plication
ring annuloplasty

19. • Two of MV repair patients required additional MV repair at 70 days and 4.1 years
• One patient undergone 2 redo MV repairs - persisting MR
• Freedom from MV reoperation with concomitant MV repair - 71 % +/- 18% at 10
years
• additional 6 patients undergone late MV operation at median age of 3 years (11
months - 25 years)
• Post ALCAPA repair - 3 had persisting severe MR
• worsening of MR to severe - 3
• 4- repair 2- replacement

20. • one patient has undergone
2 replacements at 1.5 and
22 years of age
• 3- reimplantation
• 2-Takeuchi repair
• 1- ligation of anomalous
coronary
5 year 10 year 20 years
without
concomitant
MV repair
86+/- 6 % 86+/- 6 % 81+/- 8 %
FREEDOM FROM LATE MV REPAIR

21. • SEVERE MR - 11 (pre operatively )
• all patients with preoperative severe
MR have normal LV function
• No MR - 2
• mild MR - 9
• Last follow up
• among all ,16 free of MR
• 24 mild MR
mild MR
replacement
Late Repair
concomitant repair

22. Outcomes of MV without concomitant Reapir

23. Outcomes of MV with concomitant Reapir

24. Reoperations
• Patients- 10
• Operations- 11
• Mean age 9 years (2 months-25 years)
• MV Repair- 6
• MV Replacement - 2
• Pericardial patch augmentation of MPA (stenosis ) - 1 (Takeuchi
procedure)

25. LV Function
• Serial post operative Echo - 32 patients
• LV function normalised based on Ejection Fraction at a median
time of 9.5 months (2.3-15.8 months)
• Last follow up Echo available for all 40 patients - LV function mildly
reduced in 4 patients (10% [4 of 40])
• all others have normal LV function.

26. Patency of the Coronary Artery
• All patients were asymptomatic
• No routine CAG was done
• Takeuchi repair (n=12) were asymptomatic at mean follow - up of
22 years
• 2 of these 12 underwent late MV Repair (n=1) or replacement (n=1)
did not have any evidence of ischaemia

27. • Coronary reimplantation (n=28) all asymptomatic at mean follow-
up of 9 years .
• One asymptomatic patient had CAG - normal coronaries
• Three had stress test at 8,9,15 years after surgery - normal
• Late MV Repair (4) , Replacement (1) - no evidence of ischaemia
• All patients have patent LCA confirmed by Echo during most recent
follow-up.

28. Follow -Up
• 100% for local patients
• One international patient lost follow -up .
• Median follow-up - 14 years. range (4 months- 31 years )
• Thirty-seven (93%[37 of 40]) patients being followed-up in past five years
• Three (7%[3 of 40]) did not have follow up in last 5 years . they have 7,10,19 years
follow -up respectively
• Twenty-seven (68% [27 0f 40%] )had at least 10 years follow-up
• Thirty-four (85% [34 of 40] ) had at least 5 years follow-up.

29. Comments
• Surgical repair of ALCAPA can be achieved with good results.
• Takeuchi repair - patients may experience
• supra valvular pulmonary stenosis ,
• baffle obstruction ,baffle leaks
• and AR
• one patient required reoperation for stenosis of MPA after 7.5 years
• No patient encountered baffle obstruction in 22 year follow up.

30. • After 1999 all patients have undergone coronary reimplantation
• post 9 year follow up none has encountered stenosis of the
implanted coronary.
• Early mortality has been reported 0% to 16%
• Late mortality is rare.10 year survival 86% to 100%
• Only one early death at age of 140 days . no late death and
survival of 98% at 20 years.

31. • Edwin and associates reported 27 patients between 1994-2011 with
hospital mortality of 3.7% (1 of 27).
• Seven required LVAD
• fractional shortening of <20% and ACC of > 56 minutes predicted
more than 80% LVAD use.
• In Melbourne group - 12 of 15 required LVAD because of difficulty
in weaning off from CPB (rise in LA pressure and reduced MAP)

32. To repair MV or not ???
• Several studies reported that concomitant MV Repair increases ACC time
in already ischaemic myocardium.
• Although ACC is higher in ALCAPA with MV intervention , this had no
effect on normalisation of LV function or other post operative outcomes.
• As MR improves once coronary blood flow is restored some advocate no
intervention on the MV regardless of severity.
• Kudumula and colleagues- addressed only structurally defective MV ,19
out of 25 had moderate -severe MR ,4 underwent Repair for structural
lesion. No deaths after 8 years and 4 have moderate MR .

33. • Isomatsu and colleagues - 29 patients (1982-2000)
• 24 underwent annuloplasty
• 2 early deaths in severe MR .
• No late death
• survival 93.1 % at 10 years
• only 1 out of 24 required reoperation.
• Some centres do MV repair only if ischaemic lesion of papillary muscle evident
intraoperatively. OR Severe MR in older children .

34. • 11 had severe MR . 5 underwent concomitant repair
• 1 had reoperation
• Out of 6 who had severe MR but did not underwent repair - 3 required
repair/replacement at a later date.
• Thus overall 8 of 11 underwent MV intervention.
• It seems reasonable to intervene for MV with severe degree
• In others MR decreased as ventricular function improved , so
intervention does not seem justified

35. Limitations
• Retrospective study
• Perioperative techniques have evolved over time during the study
period
• Limited statistical analysis of risk factors for mortality because of
small number of patients and outcomes.

36. Conclusions
• ALCAPA can be operated on with good outcomes.
• Persistant MR and a moderate rate of late MV repair warrants close
follow - up.

37. THANK YOU