Anomalous Origin Of Left Coronary Artery Research Articles

Transcatheter Pulmonary Valve Implantation in Repaired Tetralogy of Fallot and Anomalous Left Coronary Artery Origin

We describe a rare and extremely challenging case of transcatheter pulmonary valve implantation in repaired tetralogy of Fallot and anomalous origin of the left main coronary artery from the right coronary sinus. Procedural planning based on advanced multimodality imaging and 3-dimensional technology proved to be the key to procedural success.

Anomalous origin of the left coronary artery from the right sinus of Valsalva with separate ostia and inter-arterial course: MDCT angiography depiction.

We describe a case of anomalous left coronary artery origin from the right coronary sinus with separate ostia of right and left coronary arteries detected by 128-slice computed tomography (CT) coronary angiography. Apart from separate ostia, the left coronary artery had inter-arterial course between the aorta and the pulmonary trunk, also called as a malignant course.

Abstract 15578: A Rare Case of Anomalous Left Main Coronary Artery Origin Presenting as ST Elevation Myocardial Infarction

Background: ST-segment elevation myocardial infarctions (STEMI) are uncommon presentations of coronary artery anomalies and pose challenges in the emergent setting of percutaneous coronary interventions. We describe an extremely rare case of left main coronary artery (LMCA) originating off the right coronary artery (RCA) in a patient who presents with STEMI and was found to have Medina 1,1,1 lesion at the origin of the anomalous (LMCA). Case Presentation: An 81-year old male, with a past medical history significant for aortic stenosis and hyperlipidemia, presents to the emergency room for substernal non-radiating chest pressure. His electrocardiogram showed ST segment elevations in inferior and anterior leads. He was taken emergently to the cardiac cath lab. Coronary angiogram revealed a single right coronary ostium with an anomalous LMCA to mid left anterior descending (LAD) artery via dual insertion within a myocardial bridge. There was severe 90% Medina 1,1,1 bifurcating proximal RCA stenosis and 90% proximal LMCA stenosis. The entire left coronary system was fed by the anomalous LMCA, which was inserted into the mid-LAD in a dual insertion, between which there was a myocardial bridge. Cardiothoracic surgery team was consulted, and decision was made to pursue coronary artery bypass grafting (CABG). An intra-aortic balloon pump was placed via right femoral artery and patient was taken for emergent surgery, where he underwent 3 vessel CABG (left internal mammary artery to LAD, saphenous venous graft to obtuse marginal branch and posterior descending artery). Discussion: A broad spectrum of coronary anomalies have been reported, but their incidence in STEMI is rare. We discuss a very rare anomaly in which the entire left coronary system branches off the RCA, and culprit lesion was found to be a Medina 1,1,1 lesion. We aim to add to the sparce data pool of STEMI management in patients with anomalous coronary arteries.

Unique family clustering of anomalous left main coronary artery origin from the right sinus of Valsalva: a case for echocardiographic screening and genetic determination.

Unique family clustering of anomalous left main coronary artery origin from the right sinus of Valsalva: a case for echocardiographic screening and genetic determination.

Anomalous Origins of All Three Coronary Arteries from Separate Ostia within the Right Aortic Cusp: A Case Report and Review of the Literature.

Coronary artery anomalies are congenital defects which are found incidentally or after cardiac events. While these are rare abnormalities with the majority of patients remain asymptomatic and largely undiagnosed, it remains to be a major cause of sudden cardiac death (SCD). Anomalous origin of left coronary artery (ALCA) from the opposite right aortic sinus is extremely rare with less than 100 cases reported to-date. These patients are at increased risk for significant cardiac events, including SCD. In this report, we present a 48-year-old man with hypertension and marijuana use who was admitted initially with multi-lobar pneumonia and acute kidney injury, developed respiratory failure and sustained ST elevation myocardial infarction (STEMI). Coronary angiography demonstrated anomalous origin of all three main coronary arteries arising from right aortic sinus. In this report we also discuss the genesis of this rare and potentially fatal congenital abnormality and we highlight the diagnostic and management strategies available to-date.

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

A case of anomalous left coronary artery origin from pulmonary artery (ALCAPA) in an asymptomatic adult

A case of anomalous left coronary artery origin from pulmonary artery (ALCAPA) in an asymptomatic adult

The Incidence and Clinical Relevance of Coronary Artery Anomalies Detected on Multidetector Computed Tomography in Sarawak

Background: Coronary artery anomalies (CAAs) are rare. Some anomalies are associated with myocardial ischaemia, heart failure and sudden cardiac death. Objectives: The aims of this study were to determine the incidence of CAAs detected on multidetector computed tomography (MDCT) and their clinical relevance. Methods: We reviewed our center’s MDCT database from January 2005 to December 2015. Results: 76 out of 5677 (incidence 0.01%) patients were reported to have CAAs. They consisted of 44 patients (57.9%) with anomalous origin of right coronary artery (RCA), 7 (9.2%) with anomalous origin of left coronary artery (LCA), 3 (3.9%) with anomalous origin of the left circumflex artery (LCX), 1 (1.3%) with abnormal course of LCX, 15 (19.7%) with coronary artery fistulas, 3 (3.9%) with single coronary artery, 3 (3.9%) with anomalous left coronary artery from pulmonary artery (ALCAPA).We were able to retrieved 26 patients’ (mean age 49 ± 13 years, 17 male) case folder. They consisted of 11 patients with anomalous origin of the RCA (10 from left coronary sinus), 4 with anomalous origin of LCA from right coronary sinus (3 interarterial course), 7 with coronary fistulas (2 large fistulas), 1 with single coronary artery (Lipton LII, anterior course) , 3 with ALCAPA. Out of the 26 patients, 24 (92.3%) were alive and 2 were lost to follow-up. The commonest presenting symptom was chest pain (65.4%), followed by dyspnea (34.6%) and heart failure (11.5%). 3 patients underwent surgery and 1 underwent transcatheter coiling of fistula. 4 patients had positive functional test (2 anomalous origin of RCA, 1 anomalous origin of LCA from right coronary sinus and 1 ALCAPA). Only 1 patient who had positive functional test underwent surgery. The remaining 3 who did not undergo surgery were still alive. The patient with single coronary artery presented with heart failure and remained alive with pharmacotherapy. All 3 ALCAPA patients were alive, with the oldest patient survived to age 71 years. None of them had surgery performed. Conclusions: CAAs are rare. Majority of cases may be benign. Largescale studies are needed to better define the prognosis and optimal treatment of individual forms of CAAs.

False Negative Echocardiography in an Adolescent with Anomalous Left Main Coronary Artery Origin Presenting with Cardiac Arrest

False Negative Echocardiography in an Adolescent with Anomalous Left Main Coronary Artery Origin Presenting with Cardiac Arrest

Successful percutaneous coronary intervention in the setting of an aberrant left coronary artery arising from the right coronary cusp in a patient with acute coronary syndrome: a case report

BackgroundAn aberrant origin of the left coronary artery (LCA) from the right coronary cusp (RCC) is an extremely rare congenital anomaly. We here report on successful percutaneous coronary intervention (PCI) in a patient presenting with acute coronary syndrome and an aberrant origin of the LCA from the RCC.Case presentationA 50-year-old man presented at our emergency department with recurrent resting chest pain. Following unsuccessful attempts at visualizing the left coronary artery using Judkins left and Amplatz catheters, an aortogram using a pigtail catheter suggested anomalous left coronary artery origin and showed a significant occlusive lesion at proximal left anterior descending artery. A Judkins right 4 guiding catheter was placed around the left coronary ostium and exchanged for a Judkins left 3.5 guiding catheter after introducing a .014" coronary long wire into the left circumflex artery. With excellent angiographic visualization and guide support, a drug-eluting stent was then successfully implanted. Cardiac computed tomography (CT) demonstrated left coronary artery origin from right coronary cusp.ConclusionThis report presents a case of LCA originating from the RCC accompanied with acute coronary syndrome that was treated with successful PCI.

Anomalous Aortic Origin of Left Coronary Artery Leading to Sudden Cardiac Arrest in Young Female

Purpose: Anomalous aortic origin of the coronary artery(AAOCA) is an uncommon but important cause of sudden death in young patients. Vigilance is required to ascertain a prompt diagnosis and expedite surgery to prevent further fatal events. We describe a case of a 20-year-old female with an anomalous left coronary artery origin, whose presentation and path to diagnosis and treatment is outlined. Methodology: A 20-year-old female presented following an out of hospital cardiac arrest (OOHCA) whilst swimming. ROSC was achieved with CPR and a single defibrillation from an AED. History revealed recurrent episodes of chest pain and syncope over the previous 4 years. She was evaluated 3 years prior by cardiology; TTE and EST were found to be normal and EP review for prolonged QTS stratified her as a low risk for QTS. She was discharged without further investigations. CTCA performed following her arrest revealed an anomalous left coronary artery, arising from the right coronary sinus, passing intramurally between the aortic root and pulmonary trunk. This was repaired by deroofing the left coronary artery across the top of the left and right commisure of the aortic valve. The aortic valve commisure was re-suspended with good outcome. Results: Surgery provided excellent immediate results and exercise stress test at 6 months was negative. She remained asymptomatic with no further episodes of chest pain or syncope and is now able to perform strenuous activities. Conclusion: The AAOCA arising from the opposite sinus of Valsalva is a rare but an important condition which targets children and adolescents with an often fatal outcome if diagnosis is missed.

Anomalous origin of the left coronary artery from the pulmonary artery: diagnosis and follow-up evaluation by echocardiography

Objective To explore the value of the echocardiography in the diagnosis and follow-up evaluation of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA). Methods Sixty-five patients with ALCAPA were reviewed retrospectively and followed-up after operations. Results Four cases were misdiagnosed. Two of infant-type patients were dead during the follow-up period.Postoperatively, echocardiography demonstrated that left ventricular end diastolic dimension (LVEDD) significantly narrowed in all infant-type patients in short-term and medial-term followed-up [(42.6±1.3)mm vs (36.0±1.5)mm vs (34.2±1.6)mm, P<0.001], left ventricular ejection function(LVEF) improved in short-term follow-up[(30.0±1.6)% vs (37.2±3.0)%, P=0.04] and significantly improved in a median follow-up[(37.2±3.0)% vs (58.6±2.4)%, P<0.001]. Adult-type also had a significant narrowed LVEDD (P<0.001), and none of the patients showed any significant residual mitral insufficiency. Conclusions Echocardiography has important clinical value in diagnosis of ALCAPA, postoperative and follow-up evaluation. Key words: Echocardiography; Heart defects, congenital; Anomalous origin of left coronary artery from pulmonary artery

Preoperative diagnosis and surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery

Objective To investigate the preoperative diagnosis, surgical treatment and results of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA). Methods We retrospectively analyzed the clinical data of 9 patients with ALCAPA who had undergone surgical treatment in Shanxi Children′s Hospital from June 2010 to March 2014. Results There were 3 males and 6 females, aged from 4 months to 4 years old and 6 months, average age(1.14±1.33)years, body weight from 5.6 to 18.0 kg, average weight(8.06±3.99) kg.Echocardiography and spiral CT angiography were performed in all patients, one of which underwent cardiac catheter angiography.The cases of mitral regurgitation were mild in 2 cases, mild to moderate in 5 cases, and moderate to severe in 2 cases.Electrocardiogram showed abnormal Q wave, ST segment change and T wave inversion in lead Ⅰ, AVL and V4-6 in 9 cases.The X-ray sign was significant heart enlargement(C/T ratio 0.56-0.73, mean 0.61±0.05). Echocardiography indicated ALCAPA, significant enlargement of left ventricle and decreased myocardial contractility(ejection fraction less than 50%). End diastolic diameter of left ventricle were more than 35 mm in 8 cases and aneurysm formation of left ventricular apex in 1 case.One case was confirmed ALCAPA by catheter angiography.Six cases had radioactive nuclide myocardial imaging, which showed different degree of myocardial infarction.All patients underwent reimplantation of left coronary artery into the aorta, mitral valvoplasty in 7 patients.One case died for low cardiac output syndrome.Others recovered and discharged.Postoperative complications included low cardiac output syndrome in 2 cases, pneumonia in 3 cases and supraventricular tachycardia in 1 case.During a follow-up of 3 months to 2 years, well development, good heart function, mitral valve regurgitation and ejection fraction improved in all case. Conclusion Echocardiography has important value in diagnosis of ALCAPA, which are confirmed by spiral CT angiography and catheter angiography.Preoperative nuclide examination for determinant of survival myocardium is helpful for surgery and prognosis.Reimplantation of left coronary artery into the aorta is the most ideal surgical procedure. Key words: Anomalous origin of the left coronary artery from the pulmonary artery; Preoperative diagnosis; Surgical treatment

Percutaneous Coronary Intervention for the Anomalous Left Coronary Artery Originating from the Noncoronary Cusp.

Percutaneous coronary intervention (PCI) for anomalous left coronary artery (LCA) originating from the noncoronary cusp (NCC) is challenging, as it poses difficulties with the engagement of the guiding catheter and the establishment of backup support. This report examines the case of a 69-year-old woman with unstable angina of anomalous LCA origin. The computed tomography showed a diffuse plaque in the middle of the left anterior descending (LAD) artery and an anomalous LCA originating from the NCC. After successful engagement of a straightened Judkins-Left diagnostic catheter, the angiography revealed a diffuse plaque in the middle of the LAD artery. We then engaged a Judkins-Right guiding catheter. Due to the weak backup support of the guiding catheter, we used another wire to stabilize it, and the stent was then implanted successfully. To our knowledge, this is the first case report of PCI for an anomalous LCA originating from the NCC.

Anomalous origin of the coronary arteries

The arterial supply of the heart is provided by the left and right coronary arteries, which arise from the ascending aorta immediately above the aortic valve. An anomalous origin of the left coronary artery from the right aortic sinus and vice versa is reported to be between 0.6% - 5.6% and is a clinical relevant congenital coronary malformation as it can lead to sudden death. Coronary anomalies are a poorly understood topic in modern cardiology especially in developing countries like Tanzania. Anomalous origins of coronary arteries may cause potentially dangerous symptoms and even sudden death during strenuous activity. A cadaveric study in an unsuspected population provides a basis for understanding the normal variants, which may facilitate determination of the prevalence of anomalies and evaluation of the value of screening for such anomalies. The present study describes the normal and variant anatomy of coronary arteries in a Tanzanian population. A total of 75 hearts were studied, where 77.3% of them were obtained from male cadaverous specimens. Results revealed that in 2.7% of the hearts the left and right coronary artery had originated from a common ostium in the left aortic sinus. These variations have been reported in several case reports and studies. However, there are no such extensive studies being conducted among Tanzanians. The present study was undertaken to shed more light on this topic and it provides a basis for understanding the normal variants, for determining the incidence of anomalies, and for evaluating the value of screening for such anomalies.

Altered course of non-affected left coronary artery as a reason for symptoms of coronary artery disease.

Anomalies of coronary arteries are rare (about 0.3–1.3% of patients undergoing a coronary angiography procedure), and left coronary artery (LCA) going from the right sinus of Valsalva (RSV) is described in 0.09–0.15% of cases. We present the case of a 61-year-old female who complained of typical angina symptoms for two years. A cardiac stress test was performed with positive result. The patient was admitted to hospital for coronary catheterisation. Electrocardiogram (ECG) revealed: sinus rhythm 66/min, with T-wave inversion in leads III and aVF and ST depression in leads V4–V6. Echocardiography showed: no segmental wall motion abnormalities and ejection fraction 65%. A coronary angiography was performed. Anomalous origin of LCA from the RSV was observed. Apart from that, the coronary arteries were not affected. Because coronary angiography reveals only two-dimensional views, coronary computed tomography angiography was performed to evaluate the detailed anatomy and course of the coronary arteries. A study was performed with ECG gating at 56–61 bpm using a retrospective acquisition technique. Anatomy and patency of coronary arteries was evaluated using dedicated coronary software. Coronary arteries and their braches were free of plaques, which might have caused significant stenoses and related symptoms. RSV was a source of short common trunk, which bifurcated into the right coronary artery (RCA) and LCA (Fig. 1). The RCA followed its typical anatomical course, and the LCA ran to the left, initially between the right ventricular outflow tract and ascending aorta, and subsequently within the basal anterior segment of the intraventricular septum (Fig. 2A, B). Intramuscular segment supplied the branch corresponding with the diagonal (D1) branch of left anterior descending artery (LAD) and a tiny branch that seemed to supply the intraventricular septum. Further segments of the LCA anatomically corresponding to the LAD ran in epicardial fat tissue giving an origin to the left circumflex artery and its obtuse marginal branches. The type of anomalous course of LCA described in our patient (inter-arterial) has the worst prognosis and is associated with sudden cardiac death (> 50%), especially connected with exercise. For that reason the case of our patient is also unusual. As a young woman she practiced athletics for several years. Her professional work was connected with great physical effort. And finally she gave birth to three children (forces of nature). None of these situations provoked any symptoms. At the time of writing, the state of the patient is stable, so she has been offered optimal medical treatment.

Anomalous origin of the left coronary artery from the pulmonary artery in infants: clinical features and the perioperative treatment strategies

To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed. The patients' cardiac function was evaluated with echocardiography during follow-up. Most patients presented with symptoms of heart failure, such as tachypnea, diaphoresis, poor feeding, failure to thrive etc. Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients, endocardial hyperplasia in 5 patients, dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients. The left ventricular ejection fraction (LVEF) was (45.5±13.9)% (25%-77%). The left ventricular fractional shortening (LVFS) was (22.0±7.3)% (12%-38%). Twenty one patients underwent cardiovascular CT scan. Left coronary artery originated from left posterior sinus in 9 patients, from right posterior sinus in 1 patient, from lower main pulmonary artery in 5 patients, from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital, only 1 case who underwent angiography was diagnosed correctly. Two patients were misdiagnosed in 8 patients first-presented in our hospital. Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan. The preoperative therapies included using inotropic agents, diuretics and vasodilators according to cardiac function. Two patients underwent left coronary artery orifice ligation. Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system. Patients of NYHA IV with moderate mitral regurgitation (MR) and NYHA III with severe MR underwent mitral annuloplasty. If LVEF was less than 30% after weaning from cardiopulmonary bypass, blood pressure could not be maintained with medication, or lactates increased progressively, extracorporeal membrane oxygenation (ECMO) was demanded. Two patients showed low cardiac output syndrome immediately after surgical procedures, died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries, duration of ventilation was 7-500 hours, 11 of them were supported with ventilator for less than 60 hours. The mean length of hospital stay was (23.4±13.9) d (8-65 d). The follow-up duration ranged from 1-91 months (median 28.5 months). One case was lost to follow up. The patient died from infection 3 months after discharge. The cardiac functions of the remaining 22 patients were improved. The size of left ventricle of 14 patients recovered to normal. LVEF increased to the normal level in 20 cases. No patient underwent redo procedure. The accurate diagnosis can be made based on history, electrocardiogram, echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants. Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery syndrome in an adult and his eight-year follow-up results

Bland-White-Garland sendromu olarak da bilinen sol koroner arterin pulmoner arterden cikis anomalisi (ALCAPA), cok nadir gorulen bir dogustan anomalidir. Tedavi edilmedigi takdirde, ALCAPA sendromu cogunlukla miyokard iskemisi ve kalp yetmezligi nedeniyle yasamin ilk yilinda yuksek mortalite ile seyreder. Eriskin hastalarin bircogu asemptomatik olabilir ya da bu hastalarda anjina, nefes darligi, bayginlik, miyokard enfarktusu, aritmi veya ani kardiyak olum gorulebilir. Malign ventrikuler aritmilere sekonder ani kardiyak olum, bu hasta grubunda en sik gorulen durumdur. Bu yazida, aktif bir sporcu olan ve atipik yakinmalari olan 36 yasinda bir erkek olgu sunuldu. Transtorasik ekokardiyografide pulmoner arterde tanimlanamayan turbulans tespit edilmesi uzerine yapilan koroner anjiyografide ALCAPA sendromu saptandi. Anah tar soz cuk ler: Eriskin; koroner anjiyografi; koroner arter anomalisi. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) which is also known as Bland-White-Garland syndrome, is a rare congenital anomaly. If it is left untreated, ALCAPA syndrome has a high mortality in the first year of life due to mostly myocardial ischemia and heart failure. Most adult patients may be asymptomatic or present with angina, dyspnea, syncope, myocardial infarction, arrhythmia or sudden cardiac death. Sudden cardiac death secondary to malign ventricular arrhythmias is the most common presentation in this patient population. In this article, we report a 36-yearold male case who was an active sportsman and presented with atypical complaints. Transthoracic echocardiography revealed an indefinite turbulence in pulmonary artery and coronary angiography showed ALCAPA syndrome.

Anomalous left coronary artery origin on postmortem imaging in correlation with autopsy

A case of an anomalous origin of the left coronary artery from the right coronary cusp with interarterial course between the aorta and pulmonary artery, detected by autopsy and retrospectively on postmortem computed tomography (PMCT) and magnetic resonance (PMMR) imaging is presented. According to the literature, this type of coronary artery anomaly is highly related with sudden death in young people. Encountering this anomaly in a 75 years old individual as in the case presented here is considered rare, since the anomaly remains mainly undiagnosed if no symptoms are present. The deceased underwent both, PMCT and PMMR examination, prior to autopsy. The autopsy finding could be confirmed on PMCT and PMMR and a three-dimensional segmentation-based reconstruction was performed. With this case report we present a rare coronary artery anomaly which was survived 75 years long and found by coincidence in autopsy, and discuss the distinct advantage of re-evaluation of postmortem images by experts.

Preoperative evaluation of children with anomalous origin of left coronary artery from the pulmonary artery and the follow-up after surgery

Objective To retrospectively study 50 children with anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and find the simple and practical indexes that may predict myocardial viability and the possible risk factors that may affect the choice of operation and the recovery after surgery. Methods A retrospective study was performed in 50 children with ALCAPA[29 male, 21 female, aged from 4 months to 18 years, average (4.49±4.30) years] between Apr.1999 and Mar. 2013. The preoperative examination included electrocardiogram(ECG), echocardiography, chest X-ray, mutislice spiral computerized tomography(MSCT), angiocardiography et al. Follow-up data were obtained by reviewing the records made in out-patient and recent telephone call. According to myocardial 18F-FDG imaging, the extent of myocardial viability of 15 patients with ALCAPA was classified into 4 grades.0 normal myocardial; 1 viable myocardial; 2 partial viable myocardial; 3 myocardial infarction. Meanwhile, the global clinical scores were calculated and divided into 10 levels according to the deviations of clinical manifestations which included abnormal Q waves, left ventricular ejection fraction(LVEF), left ventricular end-diastolic dimension(LVED), cardiothoracic ratio(CTR), intercoronary collaterals(ICC), mitral regurgitation(MR) and aneurysm. The relationship of the extent of myocardial viability and clinical features were studied. All patients with ALCAPA were classified into groups by the global clinical scores and the preoperative, perioperative and post operative clinical manifestations were compared among groups. Results The extent of myocardial viability was related well to the global clinical scores(r=0.936, P 0.05). The results showed that grade 0-1 equaled scores 0-3, grade 2 equaled scores 3-5, grade 3 equaled scores>5. Children with less viable myocardium had more severe clinical symptoms. Among abnormal Q waves, LVEF 0.65, ICC dysplasia, median to severe MR and the global clinical scores>3, the global clinical scores>3 and LVEF 3) in all patients. More infants and toddlers, abnormal Q waves, ICC dysplasia, RCA/AO<0.2, larger CTR and lower LVEF were found in group B than those in group A. The time of cross-clamp and CPB was not different between the 2 groups, but the postoperative ventilation time and postoperative ICU stay were longer in group B than in group A. Forty-seven patients underwent operation and there were no operative deaths. One infant with scores 9 died while waiting heart transplantation. Follow-ups from 1 to 168 months were conducted in 38 patients(80.9%, 38/47 cases) and 1 patient with ligation of the left coronary artery(LCA) had a sudden death after 8 months of surgery. All patients had gotten smaller LVED after surgery. Nineteen patients had mild MR and 3 patients had mild supravalvular pulmonary stenosis in following-up. Of the 16 patients with LVEF<50%, 14 had a recovery of LVEF, 2 patients still had LVEF<50%. Conclusions In children with ALCAPA, the extent of myocardial viability evaluated by myocardial 18F-FDG imaging is related closely to the preoperative clinical manifestations. The global clinical scores>3 and LVEF<50% have a good predict of myocardial infarction. Even in young children with severely depressed left ventricular function, higher global clinical scores and more myocardial infarction, median and long-term follow-ups showed satisfactory recovery of cardiac function after successful restoration of a dual coronary arterial system. Key words: Anomalous origin of the left coronary artery from the pulmonary artery; Preoperative evaluation; Follow-up; Myocardial viability