ALCAPA Repair Research Articles

Cardiac Surgery and Covid-19 Insight into Peri-Operative Management

Background: Health care is seriously affected by the corona virus disease 2019 (COVID-19) pandemic with alarming effects upon conduct of cardiac surgery. The initial resource conservation strategy has to modify for handling the surging case load due to deference of routine care in the face of pandemic. Methods: The cardiac surgical practice during the lockdown period (from 25th March till 30TH November 2020) at a tertiary care centre was observed. The cardiac diagnosis of the ones operated, conduct algorithm, and working policy were analyzed. Descriptive statistics was applied to calculate the percentages of different case subsets in both adult and pediatrics groups. Results: Total of 333 emergency cases were operated on during the time frame from 25thMarch 2020 to 30thNovember 2020. Cases were divided into adult and pediatric cases. Total adult cases were 160 (48%) in number. Adult cases were further classified into aortic cases, coronary artery disease, valvular pathologies and miscellaneous ones. Total 11 aortic cases (6.8%), 43 CABG cases (26.8%), 42 valvular cases (26.2%) and 83 miscellaneous cases (51.8%) were operated. In the pediatric population, a total of 173 cases (52%) were operated, with 42 cases (24.2%) being arterial switch operations, one case of ALCAPA repair (0.5%), 25 cases (14.4%) of TAPVC repair and others (60.6%). All cases were preoperatively COVID-19 negative. However postoperatively 10 patients (3%) showed symptoms of COVID-19 and on testing turned out to be COVID-19 positive. 8 patients (80%) were transferred to dedicated COVID-19 facility for management and later 6 (60%) were discharged after successful management. There was no COVID-19-related mortality and none of the health-care workers developed COVID-19 in the entire study period. Conclusion: The initial phase of resource conservation has undermined the routine cardiac surgical practice. The study showed that strict adherence to management algorithm is necessary for persisting smooth continuation of cardiac surgical practice with provision of optimum critical care. The strategic comeback against COVID-19 would urge institutional development of protocols to aid the post-surge period.

Midterm Surgical Outcomes for ALCAPA Repair in Infants and Children.

Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function. From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients. There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention. The repair of ALCAPA can be accomplished by establishment of a dual-coronary system, which offers an acceptable mortality rate and will rarely require a second surgery. Left ventricular (LV) recovery is a progressive process, especially for infants with impaired LV function. Concomitant MV annuloplasty is safe and reliable and can be performed as necessary in patients with moderate or severe mitral valve regurgitation.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Long-Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (ALCAPA) in Children After Cardiac Surgery: A Single-Center Experience.

BackgroundThe anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly. It induces left ventricular (LV) dysfunction and mitral valve regurgitation (MR). If untreated, survival beyond infancy is rare. The aim of our study was to analyze the outcome in children with ALCAPA after cardiac surgery.MethodsWe retrospectively reviewed all patients who were diagnosed at our institution with ALCAPA and underwent surgical repair from 1999 to the end of 2018 (for 20 years). We followed LV dimensions, function, the progress of MV regurgitation, and the somatic growth of children after surgical repair.ResultsTwenty-nine patients underwent ALCAPA repair while 15 (52%) patients were male. The median age at surgical repair was 5.3 (IQR: 3.8-7.4) months and the mean weight was 5.5±2 kg. Surgical repair was performed in form of coronary reimplantation in 26 (90%) patients and Takeuchi repair in three (10%) patients. Intensive care unit (ICU) stay was eight (IQR: 6-17) days and hospital stay was 15 (IQR: 12-21) days. The follow-up duration was 5±3.6 years. Echocardiographic parameters started to improve by six weeks after the repair, and they normalized by one year. At the time of surgery ejection fraction (EF) was 34±17%, fractional shortening (FS) was 15±10%, and LV inner diameter in diastole (LVIDD) z score was 5.7±2.8. These parameters improved by one year after surgery to 66±7%, 34±6%, and 0±1.3, respectively. However, somatic growth started to improve six months after surgical repair. MR was moderate to severe in seven (24%) patients at the time of surgery and regressed to no more moderate nor severe MR at the last follow-up. None of the 29 patients died.ConclusionsLV systolic function and dimensions start to improve by six weeks after surgery and reach normal values by one year. MR regresses without intervention in correspondence with the regression of LV dimensional parameters. The somatic growth of children improves six months after repair.

Unexpected mechanism of mitral regurgitation in a patient post ALCAPA repair: Added value of three-dimensional echocardiography.

Accurate assessment of etiology of mitral regurgitation (MR) is one of the key steps in the decision-making process and further clinical management of patients with severe MR. Our clinical case illustrates the added value of three-dimensional echocardiography (3DE) in assessment of mitral valve morphology and identification of an unexpected mechanism of MR which was not previously diagnosed using conventional echocardiography. 3DE helped to choose appropriate management strategy in this patient.

Risk factors for early fluid overload following repair in pediatric patients with ALCAPA complicated with moderate or severe left ventricular dysfunction and the effect on clinical outcomes

Objective To identify the risk factors for early fluid overload(FO)following repair in the pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) complicated with moderate or severe left ventricular dysfunction (left ventricular ejection fraction [LVEF]<50%) and evaluate the effect on clinical outcomes. Methods Forty-three pediatric patients with ALCAPA complicated with moderate or severe left ventricular dysfunction, aged 2-128 months, weighing 4.5-34.5 kg, with New York Heart Association Ⅲ or Ⅳ, undergoing ALCAPA repair, were enrolled in this study.The pediatric patients were divided into FO≥5% group (n=14) and FO<5% group (n=29) according to the FO developed within 24 h after operation. The pediatric Risk, Injury, Failure, Loss, and End-Stage Renal Disease criterion was used to diagnose acute kidney injury developed after operation. Factors including age, height, weight, preoperative LVEF, preoperative biomarkers, operative data, postoperative ventilation time, duration of intensive care unit(ICU)stay and related postoperative clinical outcome parameters were recorded.The risk factors of which P values were less than 0.05 would enter the multivariate logistic regression analysis to stratify the risk factors for FO≥5% developed within 24 h after operation.The effect of FO≥5% on postoperative severe acute kidney injury (Injury and Failure), ventilation time, duration of ICU stay and etc. was assessed. Results Fourteen cases developed early postoperative FO≥5%, and the incidence was 33%.The results of the logistic regression analysis showed that lower preoperative LVEF was an independent risk factor for early postoperative FO≥5% (P 0.05). Conclusion Lower preoperative LVEF is a risk factor for early postoperative FO in pediatric patients with ALCAPA complicated with a moderate or severe left ventricular dysfunction undergoing repair, and it is not helpful for clinical outcomes in pediatric patients when postoperative early FO≥5% occurs. Key words: Coronary artery disease; Pulmonary artery; Risk factors; Prognosis; Child; Fluid overload

The outcome of surgical repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants

BackgroundAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation in infants presenting with angina, dyspnea, and excessive perspiration invoked by crying and feeding. The aim of this study was to evaluate the factors affecting morbidity and the use of extracorporeal membrane oxygenation (ECMO) and to assess the quality of life post-repair.MethodsIn this retrospective study, information about five infants who underwent ALCAPA repair was collected from the databases of two tertiary referral cardiac centers (King Abdulaziz University Hospital and King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia) from 2011 to 2018. The patients were diagnosed using echocardiography, and data including mitral insufficiency and ejection fraction were assessed preoperatively and postoperatively. Quality of life was assessed using a questionnaire-based interview.ResultsThe median (range) age at the time of repair was 95 (34–144) days, and the median weight was 4.9 (3–5.7) kg. Two patients underwent the Takeuchi procedure, and three patients underwent left coronary artery reimplantation and translocation to the aorta. The median preoperative ejection fraction was 25 (12.5–45)%, and at the last follow-up, the median EF of the three survivors was 59 (50–70)%. There was no significant change in mitral insufficiency grade postoperatively. Two patients had ECMO support and died postoperatively. The infants who died were younger (< 75 days) and had lower weight (< 4.5 kg) at the time of intervention. Patients who survived showed good quality of life were asymptomatic and had heart function within the normal range.ConclusionEchocardiography and CT angiogram are effective tools for ALCAPA diagnosis in pediatric patients. Low weight at time or repair was associated with increased morbidity and ECMO did not increase survival.

Mid-Term Outcome for Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

This study is a single-centre experience with surgical intervention of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was performed of patients who underwent ALCAPA repair between November 1991 and October 2017. A total of 138 patients with a median age of 36 months underwent ALCAPA repair. The median follow-up period was 3.5 years. Overall, 114 (82.6%) patients received coronary reimplantation, 21 (15.2%) underwent Takeuchi repair, and three (2.1%) were treated by closure of the ALCAPA plus coronary artery bypass graft. Concomitant mitral repair was performed in 50 patients with moderate-to-severe mitral regurgitation (MR). Three (2.1%) patients died in hospital. Three (2.1%) patients needed extracorporeal membrane oxygenation (ECMO) support. Seven (5.0%) patients needed delayed sternal closure. In patients with decreased preoperative left ventricular ejection fraction (LVEF) (<50%), the LVEF significantly improved from 25% to 33% at discharge (p = 0.001). The percentage of more than moderately significant MR decreased from 36.2% to 5.2% (p = 0.02). There was no late mortality. Two patients needed reoperation because of baffle leaks after Takeuchi repair. Three patients had severe MR. All patients had normal LVEF at last follow-up. The median normalisation time of left ventricular function was 6 months. Short-term and mid-term outcomes of surgical treatment for ALCAPA patients were excellent. The concomitant mitral valve repair did not increase the perioperative risk but had satisfactory mid-term outcomes. Normalisation of left ventricular function is expected within the first 6 months.

Epidemiology of acute kidney injury among paediatric patients after repair of anomalous origin of the left coronary artery from the pulmonary artery.

Acute kidney injury (AKI) is a prevalent complication after the surgical repair of paediatric cardiac defects and is associated with poor outcomes. Insufficient renal perfusion secondary to severe myocardial dysfunction in neonates is most likely an independent risk factor in patients undergoing repair for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). We retrospectively investigated the epidemiology and outcomes of children with ALCAPA who developed AKI after repair. Eighty-nine children underwent left coronary reimplantation. The paediatric-modified risk, injury, failure, loss and end-stage (p-RIFLE) criteria were used to diagnose AKI. The incidence of AKI was 67.4% (60/89) in our study. Among the patient cohort with AKI, 23 (38.3%) were diagnosed with acute kidney injury/failure (I/F) (20 with acute kidney injury and 3 with acute kidney failure). Poor cardiac function (left ventricular ejection fraction < 35%) prior to surgery was a significant contributing factor associated with the onset of AKI [odds ratio (OR) 5.55, 95% confidential interval (CI) 1.39-22.13; P = 0.015], while a longer duration from diagnosis to surgical repair (OR 0.97, 95% CI 0.95-1.00; P = 0.049) and a higher preoperative albumin level (OR 0.83, 95% CI 0.70-0.99; P = 0.041) were found to lower the risk of AKI. Neither the severity of preoperative mitral regurgitation nor mitral annuloplasty was associated with the onset of AKI. After reimplantation, there was 1 death in the no-AKI group and 2 deaths in the AKI/F group (P = 0.356); the remaining patients survived until hospital discharge. The median follow-up time was 46.5 months (34.0-63.25). During follow-up, patients in the AKI cohort were seen more often by specialists and reassessed more often by echocardiography. Paediatric AKI after ALCAPA repair occurs at a relatively higher incidence than that suggested by previous reports and is linked to poor clinical outcomes. Preoperative cardiac dysfunction (left ventricular ejection fraction < 35%) is strongly associated with AKI. The beneficial effect of delaying surgery seen in some of our cases warrants further investigation, as it is not concordant with standard teaching regarding the timing of surgery for ALCAPA.

Diagnosis and treatment of abnormal left coronary artery originating from the pulmonary artery: A single-center experience.

Objective:We aimed to review symptoms, findings, surgical treatment options, short- and mid-term outcomes, and reoperation rate of patients diagnosed with of left coronary artery from the pulmonary artery (ALCAPA) of an anomalous origin in our institution.Methods:From May 2000 to March 2018, 33 patients who had left coronary artery originating from the pulmonary artery were retrospectively examined. The clinical features of patients, diagnostic tools and their efficacy, outcomes of surgical repair, and problems during follow-up were evaluated.Results:Thirty-three patients (22 females, 11 males) were included in the study. At the time of surgery, the median age and weight of patients were 6 months (minimum/maximum, 1–166 months) and 6.5 kg (minimum/maximum, 3–38.5 kg), respectively. The mean follow-up was 5±3.5 years (range, 1–16 years). Dyspnea, tachypnea, diaphoresis, prolonged feeding time, and developmental delay were common presenting signs and symptoms. It was determined that all the patients who were diagnosed at another center reached our center for surgical treatment within 1 month. Twenty-three (69.7%) patients had pathologic Q wave with anterior and/or anterolateral myocardial infarction signs on an electrocardiogram (ECG), whereas 22 (66.6%) patients had ST-T segment changes. Twenty-one (63.6%) patients had cardiomegaly on the telecardiogram. A reimplantation surgery was performed to 22 patients and 10 patients underwent the Takeuchi procedure. In addition to ALCAPA repair, 5 patients needed mitral valve plasty. Atrial septal defect (ASD) and ventricular septal defect (VSD) were closed in one patient, and Tetralogy of Fallot was totally corrected in another. At discharge, there was a significant improvement in left ventricular (LV) systolic functions. At the last visit, all patients had normal LV systolic functions except four who had mild dysfunction. The mean follow-up of the four patients was 2.8 years. In the early postoperative period, complications were seen in 10 patients. Five patients died in the early postoperative period, while one patient died 9 months after the ALCAPA surgery because of low cardiac output syndrome that developed after mitral repair.Conclusion:Patients with ALCAPA commonly present with congestive heart failure symptoms. When the diagnosis is confirmed in these patients, surgical treatment should not be delayed. The availability of surgical center and surgery outcomes for ALCAPA diagnosed patients are comparable with other countries, but the delay in the diagnosis of disease is still a problem in our country.

Surgical Outcome in Adolescents and Adults With Anomalous Left Coronary Artery From Pulmonary Artery

The outcomes of different repair strategies of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in adolescent and adult patients are uncertain. The long-term outcomes of reimplantation and Takeuchi repair were compared in this study. We conducted a retrospective review of data collected from patients receiving ALCAPA repair at our institute from January 2005 to December 2016. Short- and long-term outcomes of reimplantation and Takeuchi repair were compared. A total of 50 consecutive patients underwent ALCAPA repair, with an average age of 31.6 ± 15.6 years and 66% women. No significant differences were found in short-term outcomes between the 2 groups. However, at a median of 65.7 months' follow-up, the major adverse cardiovascular event (MACE) (including all-cause death, admission due to heart failure, new-onset acute myocardial infarction, and repeated revascularization) rate of the Takeuchi repair group was significantly lower than that of the reimplantation group (hazard ratio, 0.21; 95% confidence interval, 0.04 to 0.97). Furthermore, the preoperative glucose level was significantly associated with increased MACE rate (hazard ratio, 10.82; 95% confidence interval, 1.20 to 97.54). Left ventricular end-diastolic diameter and ejection fraction significantly improved in both groups. However, mitral valvuloplasty did not predict long-term recovery of left ventricular function. Although short-term outcomes were satisfactory in both groups, a higher MACE rate was observed in reimplantation group than Takeuchi repair group; mitral valvuloplasty was not significantly associated with improved prognosis and left ventricular reverse remodeling. Elevation of preoperative blood glucose level was significantly associated with increasing long-term MACE rate.

Surgical Outcomes of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: An Echocardiography Follow-up.

Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography.Methods:From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up.Results:The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range: 0.5–36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients. All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up.Conclusions:Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.

9 Long-term outcomes of adults with alcapa repair

Background Anomalous left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital abnormality, which, if untreated, can cause complications such as myocardial infarction, heart failure and death; only few untreated patients survive to adulthood. We sought to determine long-term outcomes of these patients in our Adult Congenital Heart Disease service. Methods A retrospective review of case notes of patients with ALCAPA was performed, reviewing clinic letters, operation details, cardiac imaging and exercise tests. Results We identified 8 patients (6 female) with mean age 26.3±6.2 years. Mean follow-up was 4.1 years (range, 18 months to 12 years). All patients have had surgical repair; one patient required concomitant mitral valve repair. Mean age at operation was 9.7 years (range, 3 months to 34 years old); 3 patients (38%) were operated as adults. At last clinic review, one patient had NYHA III symptoms, the rest were well (NYHA I). Mean peak oxygen consumption on cardiopulmonary exercise testing was 36±2.4 ml/kg/min (range 33.4 to 38.8 ml/kg/min, mean 93%±15.9% predicted, range 78% to 112%). All patients were in sinus rhythm and no ischaemia or arrhythmias were identified. 7 patients (88%) had good left ventricular function (mean EF 61%); 1 patient had mildly impaired function (EF 50%) due to an apical transmural infarction. Moderate mitral regurgitation was seen in 3 patients (38%) and all had normal aortic root size. Conclusion We describe long-term outcomes of patients with ALCAPA syndrome. Postoperatively, the majority remain asymptomatic with good exercise capacity. Their left ventricular systolic function is good. Life-long follow up is warranted.

70. Surgical mitral valve replacement with modified Melody valve in children

Objective We describe a case of mitral valve (MV) replacement using modified Melody valve implantation. Methods A 2.5 year-old girl,with a history of ALCAPA repair in April 2012, had undergone MV repair by ring annuloplasty at 1 year of age. She came back with severe MV stenosis (mean gradient, 8 mmHg) and regurgitation. She had a hugely dilated left atrium and a MV annulus of 14 mm. She, then, underwent surgical MV replacement with a modified Melody valve. Results The Melody valve was prepared before the cardiopulmonary bypass. The procedure included stent shortening and adding a bovine pericardial sewing ring. Through a trans-septal approach, the previous MV ring was removed, the pericardial ring patch of the Melody valve was secured to the mitral annulus and the ventricular end of the valve was fixed to the posterior–inferior wall of the left ventricle. The prosthesis was then inflated to size 16 mm. Testing of the valve showed good leaflet coaptation. The atrial septum was closed by fenestrated (4 mm) bovine pericardial patch. TEE showed good valve function with a tiny paravalvular leak and no left ventricular outflow obstruction. The pulmonary veins were also unobstructed. Conclusions The modified Melody valve is a viable option for children MV annular diameters, providing a valid alternative to existing prostheses. The technique is relatively easy and the short term result is very good. This prosthesis will be particularly attractive if maintaining competence after subsequent dilations as the child grows.

Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up.

Little is known about preoperative factors affecting postoperative morbidity following anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) repair. This study aimed at identifying predictors of postoperative outcomes, including mortality and left ventricle (LV) normalization. A retrospective, single institution review was conducted on forty-four ALCAPA repairs from April 1991 to November 2012. Preoperative clinical data and echocardiograms were analyzed. Postoperative outcomes included duration of intensive care supports and mortality. Time to normalization of LV end diastolic dimension (LVEDD) and function were assessed. Logistic regression and Cox proportional hazard analyses were used to correlate preoperative variables to postoperative outcomes. Forty-four patients underwent ALCAPA repair. No in-hospital or late mortality was observed. LVEDD, weight, and LV shortening fraction (SF) independently predicted duration of postoperative inotropic support. LVEDD and body surface area independently predicted the duration of postoperative intubation. For the infant majority, younger age predicted longer duration of postoperative intubation (p=0.048) and LVEDD Z-score independently predicted duration of postoperative IV inotropic support (p=0.042). LV function normalized in all patients for whom follow-up data was available. LVEDD Z-score independently predicted time to normalization of LV function (p=0.013). ALCAPA repair in the current era has excellent outcomes, with no mortality in our cohort. Immediate postoperative morbidities are influenced by patient size, LVEDD, and preoperative SF. Outcomes of infantile ALCAPA are influenced by the degree of LV dilation. Time to normalization of LV function is related to LVEDD. Limitations included retrospective evaluation of LV function.

Reoperation and Mechanical Circulatory Support After Repair of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Twenty-Year Experience

Although outcomes for repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) have improved, early postoperative mechanical circulatory support is occasionally still required. This study was undertaken to determine whether long-term outcomes for children supported with extracorporeal membrane oxygenation (ECMO) after ALCAPA repair differ from those in children who did not require ECMO. Between 1989 (when our ECMO program began) and 2010, 26 consecutive patients (median age of 0.26 years) underwent surgical repair of ALCAPA mainly with a strategy to produce a dual coronary system. Among the 26 patients, 21 did not require ECMO postoperatively (non-ECMO group) and 5 were supported by ECMO (ECMO group). Hospital and clinic records were reviewed to determine endpoints of early or late death, cardiac transplantation, and late reoperation. There were no early or late deaths in either study group, at a mean follow-up of 6.5±6.5 years. Mean duration of ECMO support was 10.7±6.7 days. There was no difference in age or weight between the two groups. Two patients, one in each group, required cardiac transplantation at 6 days and 21 months, respectively. Four other patients required 6 reoperations (5 for mitral regurgitation and 1 for an atrial septal defect with pulmonary stenosis). Actuarial freedom from cardiac transplantation or reoperation at 5 years was 0% in the ECMO group and 92% in the non-ECMO group (p<0.001; log-rank test). Overall survival is excellent after ALCAPA repair. However, those patients who require mechanical support after repair appear to be at higher risk for transplantation or reoperation, typically for mitral regurgitation.

P157: Results of anomalous origin of the left coronary artery from the pulmonary artery in children

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) causes severe myocardial ischemia, which can produce papillary muscle dysfunction, annular dilatation secondary to global left ventricular (LV) dysfunction, and frank myocardial infarction with papillary muscle rupture. Mitral regurgitation (MR) secondary to the LV and/or papillary muscle dysfunction will usually improve in the absence of continuing ischemia. The aim of this study is to determine the influence of mitral valve (MV) pathology on early and late outcomes of patients with ALCAPA who underwent a surgical repair. Methods: Twenty-five patients underwent operations over a 30-year period (median age, 4 months; range, 1 month-16 years) with a median follow-up of 7 years (range, 2 months-30 years). Before repair, 11 infants (44%) presented in extremis requiring ventilatory and inotropic support, and 16 infants (64%) presented in heart failure. MR was present in 18 patients (72%); mild in 10, moderate in 5, and severe in 3. None of the patients underwent MV repair at the same time as the ALCAPA repair. Results: Hospital survival was 96%. Three patients underwent MV repair at the mean time of 3.5 years (all with preoperative severe MR). The degree of MR gradually improved in all patients with preoperative mild and moderate MR. Echocardiographic studies demonstrated improvement in LV function in all children. None of the patients showed any evidence of supravalvar pulmonary stenosis. Conclusion: Long-term clinical outcome and LV function are good, despite severe LV dysfunction at presentation. Mitral valve repair or replacement is not generally necessary at the time of the initial operation. Residual or recurrent MR is present in some of patients and can be managed accordingly to its degree of severity.