Long term results of surgical management of anomalous origin of the left coronary artery ( from pulmonary artery ( ALCAPA and how to improve it ,Howaida AlQethamy, MD, Adel Ragheb, MD , Wael Hussain, MRCS, Yahia Alfaraidi, MD .Roberto M Di Donato MD
,Department of pediatric cardiac surgery , Prince Sultan Cardiac Center, Riyadh .Saudia Arabia
ALCAPA is a rare congenital abnormality that affects .of every 300,000 live births and accounts for 0.25%–0.5% of all CHD 1
, It usually manifests as an isolated defect .in 5% of cases it may be associated with ASD,VSD, and aortic coarctation .Children with ALCAPA usually present in infancy ,As the pulmonary vascular resistance drops in the first few weeks of life myocardial ischemia or infarction, or both, may occur depending on the degree of . coronary artery collateral development Ischemic papillary muscle dysfunction results in progressive mitral regurgitation, and infants may present with congestive heart failure or cardiogenic shock Early diagnosis and prompt surgical intervention with the aim of restoring a two-coronary-artery circulatory system have excellent results and lead to gradual myocardial recovery
Anomalous origin of the left coronary artery from (the pulmonary artery (ALCAPA
Pathophysiology In fetal and early neonatal life, ALCAPA is well tolerated because pulmonary arterial pressure equals systemic pressure, which leads to antegrade flow in both the anomalous LCA and the normal right coronary (. artery (RCA When pulmonary arterial pressure decreases, flow in the LCA decreases and then reverses, which leads to myocardial ischemia and infarction due to coronary steal and unoxygenated blood and if left untreated, up to 90% .of patients with ALCAPA die within the 1st year of life The extent of acquired collateral circulation between the RCA and LCA during the critical period, when pulmonary arterial pressure gradually .decreases, determines the extent of myocardial ischemia In patients who live to adulthood, ALCAPA syndrome may cause myocardial infarction, left ventricular dysfunction and mitral regurgitation, or silent myocardial ischemia, which can lead to sudden . cardiac death
the spectrum of pathophysiologic changes that take place .after birth in patients with ALCAPA syndrome
Diagnosis of ALCAPA syndrome ( .electrocardiogram (ECGchest X-ray- . . D ECHO-2cardiac catheterization- . .CT angiography( . magnetic resonance (MR.D reconstruction of CT study-3-
Oblique VR image shows a dilated RCA and the LAD artery with multiple collateral vessels at the right ventricular wall. The image demonstrate the anomalous origin of the LCA from the pulmonary trunk .
Same image after removal of cardiac chambers with manual editing, it clearly demonstrate the anomalous origin of the LCA from the pulmonary trunk, along with multiple collateral vessels within the interventricular septum and the dilated RCA
Surgical principles Early repair enables correction of chronic subendocardial ischemia, recovery of ventricular . function, and reduces the risk for malignant dysrhythmias and sudden death : Surgical approaches classified into two groups Single-coronary-system repair-1 , Includes ligation of the anomalous LCA at its pulmonary origin .aprocedure that is currently avoided due to the high rate of complications
-
Two-coronary -system repairs are preferred which is-2 ( . Coronary button transfer (Reimplantation . Takeuchi procedure placement of a coronary artery bypass graft (CABG( combined with ligation of the . origin of the LCA Button transfer is considered to be the most anatomic correction, and it has excellent long. term results. It is the preferred method of treatment in infants Cardiac transplantation in cases of significant left ventricular dysfunction and percutaneous . transcatheter closure of the ALCAPA are another choices
-
Takeuchi procedure In this procedure, a transpulmonary baffle between the coronary ostium in the pulmonary artery (PA) and the aorta (Ao) is created. supravalvular Because the pulmonary artery wall is used to create the baffle, .pulmonary stenosis could happen
Diagram shows the .coronary button transfer
The purpose To determine the early and late outcomes of all patients presenting with ALCAPA who had repair by the aortic . reimplantation technique ,Factors associated with mortality ,need for postoperative ventricular support . and need for reoperation are defined as well as surgical decision if there is MR also discussed Serial and late assessment of: . ventricular function and size.status of the mitral valvefunctional capacity of patients undergoing physiologicsurgical correction of ALCAPA by aortic reimplantation .are evaluated
-
PATIENTS AND METHODS patients presenting with ALCAPA who had 47 repair in our center with different techniques in the period from Jan 1985 to July 2012
TABLE 1 DISTRIBUTION OF ASSOCIATED LESIONS
TYPE OF LESIONS
NO. OF PATIENTS
PERCENTAGE
ISOLATED ALCAPA
40
85.11%
ALCAPA WITH ASD
2
4.25%
ALCAPA WITH VSD
2
4.25%
ALCAPA WITH PDA
1
2.12%
ALCAPA WITH COA & PDA
1
2.12%
ALCAPA WITH SCIMITAR SYNDROME, PAPVD AND CORTRIATRIATUM
1
2.12%
OPERATIVE TECHNIQUES Ligation of anomalous left coronary artery was performed in four cases (8.51%(, Takeuchi’s repair was done for three cases (6.38%( and 40 cases (85.1%( underwent direct aortic implantation of left coronary artery
Mitral valve repair was adopted in three cases (6.38( and mitral valve replacement (. was performed in one case (2.12%
DIFFERENT TECHNIQUES DONE IN OUR STUDY
.(Total No. (47 pts .(Group A
(40 pts
(85.1%( .(Group B
(3 pts
(6.38%( .(Group C (8.51%(
(4 pts
Postoperative Complications The postoperative course is variable and may require a period of left ventricular support to allow for recovery of both stunned and hibernating myocardium. Midterm . and long-term ventricular function and clinical status improve over the ensuing months Each type of surgical repair is associated with specific complications. CABG placement combined with ligation of the anomalous LCA is associated with potential graft . occlusion and stenosis coronary button transfer carries the risk for tearing of the anomalous LCA and massive bleeding due to increased friability and diminished elasticity when the anomalous LCA . is mobilized for repair In the Takeuchi procedure, a transpulmonary baffle made from the pulmonary arterial wall is used to create a coronary tunnel to connect the anomalous LCA to the aorta. Complications such as supravalvular pulmonary stenosis and baffle obstruction or . leakage have been reported Simple ligation of the anomalous LCA is no longer performed due to recanalization of the ALCAPA, a greater risk of atherosclerosis, severe mitral regurgitation resulting from ischemic cardiomyopathy, and a persistent risk of sudden death due to silent . ischemia
Mitral valve function 4 patients 2\4 of these patients
had severe MR upon cessation of bypass. required return to bypass and mitral valve
reconstruction for severe MR with hemodynamic instability. Residual MR at discharge was graded as 0 (n=6(, (n=5(, II窶的II (n=1( III窶的V (n=0(. One patient required mitral valve re-repair at 16 months postoperatively. The original post-mitral repair echocardiogram performed in the operating room revealed grade I窶的I MR. The discharge echo showed grade II residual MR. Subsequent follow-up echocardiograms displayed worsening MR. At reoperation a complex repair was performed. Discharge echo showed no residual MR and at follow-up three months postoperatively MR remains absent. MR at three months, six months and at most recent follow-up showed the same
RESULTS Early postoperative echo showed moderate to severe mitral regurge with severe LV . dysfunction in 8 cases (17.02%( which improved in the first year of follow up ,cases (8.51%( needed ECMO support in operating room 4 successfully weaned from ECMO and the other 2 died 4\2 . two patients needed ECMO support in intensive care unit (4.25%(, one case weaned from ECMO successfully and the other one died. 15 cases ( 31.91%( transferred to ICU with open chest. Mortality rate was 17.02% (eight cases(. One case (2.12%( returned back after one year with left coronary artery origin stenosis after Takeuchi’s repair, PCI done successfully for this patient.
TABLE 2 SHOWS RESULTS IN DIFFERENT GROUPS variables
Total number
Group A
Group B
Group C
TABLE 2 SHOWS RESULTS IN DIFFERENT GROUPS 47 pt. 40 pt. 3 pt.
4 pt.
.Mortality(op(
8 ((17.02%
(15%( 6
(33.3%( 1
1(25%(
Open chest
(31.9%( 15
13(32.5%(
2 (66.7%(
0 (0%(
ECMO support
6 ((12.8%
5(12.5%(
1 (33.3%(
0 (0%(
Mitral valve repair
(6.4%( 3
3(7.5% (
0 (0%(
0(0%(
Mitral valve replacement
(2.1% ( 1
1(2.5%(
0 (0%(
0(0%(
Conclusion Most of our mortality occurred in high age group and so, early diagnosis and repair of ALCAPA improve outcome of surgery . Aortic implantation of left coronary artery is the best technique of ALCAPA repair and can be done easily. Although severe LV dysfunction and severe mitral regurgitation has a high impact on the outcome of ALCAPA repair regarding mortality , the need for mechanical support and long hospital stay, it is better not to touch the mitral valve , as the repair is very technically demanding especially in small babies without expected good results and consumes a lot of cross clamp time with subsequent bad impact on surgical outcome. Mechanical support should be decided according to the degree of LV dysfunction and severity of mitral valve regurgitation.
THANK YOU