Anomalous aortic origin of the left coronary artery / Origen aórtico anómalo de la arteria coronaria izquierda

Abstract We present an unusual case of an anomalous left coronary artery arising from the contralateral sinus of Valsalva: a 63-year-old male patient who consulted to our emergency department with 1-week history of progressive dyspnea on exertion with clinical signs of heart failure, associated with lung congestion on the chest X ray and elevated NT-proBNP levels (2000 pg/ml; normal value <150). Doppler echocardiography showed severe dilation of both left atrium and left ventricle, with severe deterioration of LV systolic function (Ejection fraction of 26%), global hypokinesia and a moderate mitral regurgitation with central jet. A cardiac cath eterization was performed, which evidenced an anomalous origin of the left main coronary artery from the right coronary sinus with a proximal lesion of nearly 50%. A coronary computed tomographic angiography confirmed the diagnosis of an anomalous origin with an intramyocardial path at the level of the interventricular septum, as sociated with moderate extrinsic compression. To determine the degree of functional ischemia presented by the left main coronary artery lesion we performed a fractional flow reserve evaluation, resulting in 0.75, which was ranked as significant. An angioplasty with implantation of a drug-eluting stent (with Everolimus) was performed successfully to the target lesion. The patient evolved favorably during hospitalization and was discharged from the medical center to continue outpatient follow-up. Patient remained asymptomatic at 1-month and 6 months, during clinical evaluation, without evidence of ischemia on noninvasive functional assessment.

Resumen Presentamos un raro caso de nacimiento anómalo de arteria coronaria izquierda en el seno de Valsalva contralateral. Se trata de un hombre de 63 años que consultó al servicio de emergencias de nuestro centro por disnea progresiva de una semana de evolución, con signos clínicos de insuficiencia cardíaca, aso ciado a signos de congestión en la radiografía de tórax, y valores de NT-proBNP elevados (2000 pg/ml; valor normal <150). El ecocardiograma Doppler evidenció dilatación grave de la aurícula y del ventrículo izquierdo, con deterioro grave de la función sistólica (fracción de eyección de 26%), hipoquinesia global e insuficiencia mitral moderada con jet central. Se realizó una cinecoronariografía que evidenció el nacimiento anómalo del tronco de arteria coronaria izquierda desde el seno coronario derecho, con una lesión cercana al 50%. Una angiotomografía coronaria confirmó el origen anómalo del vaso coronario, con trayecto intramiocárdico a nivel del septum interventricular asociado a compresión extrínseca moderada. Para determinar el grado de isquemia funcional que presentaba la lesión del tronco coronario izquierdo se evaluó la reserva de flujo fraccional, que arrojó un resultado de 0.75 el cual se consideró significativo, prosiguiendo a angioplastia con implante de stent liberador de droga (con Everolimus) a dicha lesión. El paciente evolucionó favorablemente durante la internación en el hospital, egresando de la institución para continuar seguimiento ambulatorio. Persistió asintomático en los controles realizados al mes y a los 6 meses, sin evidencia de isquemia en la evaluación funcional no invasiva.

Analysis of the risk factors for extracorporeal membrane oxygenation use after surgical repair in patients with anomalous origin of the left coronary artery from the pulmonary artery combined with severe left ventricular dysfunction / 中国胸心血管外科临床杂志

@#Objective    To analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients. Methods    The clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed. Results    A total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58± 19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min. Conclusion    ACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.

Clustered Origin of the Left Coronary Artery from the Left Sinus of Valsalva: Uncharacteristic Developmental Event in the Temporally Regulated Process

Coronary artery development is a sequential and progressively regulated process. However, there are a number of reports available on the disturbance to this temporal process resulting in variations of the artery. In these instances, the function of the coronary arteries was normal in providing blood supply to the heart. The normal and abnormal coronary artery development has been studied using a variety of approaches and the knowledge continues to evolve. The cadaveric specimen was obtained from the “willed body program” for the purpose of student dissection. During the cadaveric dissection of a 94-year old male Caucasian we observed that the left coronary artery arose from the coronary ostium in a cluster of multiple branches. A detailed study of the origin of the left coronary artery showed that there is one ostium from which multiple branches arose. The right coronary artery arose normally from the right coronary ostium. The coronary veins were normal and returned the blood through the coronary sinus to the right atrium. The anomalous finding is discussed in the light of what is already known about the normal coronary artery development and rationalize a possible explanation. We conclude that the abnormal development of the coronary artery is rarely detrimental and provides adequate blood circulation to the heart. However, knowledge of such malformation is important in clinical practice.

Diameter of the orifice of the left coronary artery: angiographic study / Diámetro del ostio de la arteria coronaria izquierda: estudio angiográfico

SUMMARY: The ongoing advances in the technology of coronary artery angiograms have put high demands on the basic knowledge of coronary arteries. This study describes the angiographic morphology of the ostium (orifice) of the left coronary artery among Sudanese with respect to sex, age, length, and BMI. The study design is a cross-sectional retrospective hospital-based conducted from 2014 to 2016. The inclusion criterion of participants is adult males and females presented to the cardiac centers for elective angiograms. The exclusion criteria are age below 18 years, documented congenital heart disease, and previous coronary bypass. Angiograms were done using a digital radiographic system. Data was collected through a predesigned data collection sheet. The data were entered and analyzed using SPSS v27. A test of correlation was done between the different variables. The data were presented in the form of tables. A P-value of <0.05 was considered statistically significant. The total number of participants was 441; males and females represent 42.9 % and 57,1 %, respectively. The mean age of participants was 56.24±8.68 years. The left coronary artery originated from the left aortic sinus. The mean diameter and length of the left coronary artery were 3.8±0.70 mm and 8.1576±4.32 mm, respectively. A significant negative correlation was reported between the diameter of the orifice and both age and length of the left coronary artery. A non-significant difference between males and females in the diameter of the left coronary artery at ostium (P=0.058) and a significant difference in the length (P=0.00). Sudanese have the smallest diameter of the orifice of the left coronary artery among Africans. Sudanese males have a smaller diameter of orifice than females; females have the longest arteries. A wider orifice of the left coronary artery is associated with a short arterial length.

RESUMEN: Los avances en la tecnología de los angiogramas de las arterias coronarias constituyen importantes exigencias al conocimiento básico de las arterias coronarias. Este estudio describe la morfología angiográfica del ostio de la arteria coronaria izquierda (ACI) entre los sudaneses respecto al sexo, la edad, la longitud y el IMC. El estudio es un diseño hospitalario retrospectivo transversal realizado entre 2014 y 2016. El criterio de inclusión de los participantes, hombres y mujeres adultos, fue aquellos que se realizaron angiografías electivas en los centros cardiológicos Los criterios de exclusión fueron: edad menor de 18 años, cardiopatía congénita documentada y bypass coronario previo. Los angiogramas se realizaron utilizando un sistema radiográfico digital. Los datos se obtuvieron a través de una ficha de datos prediseñada. Estos fueron ingresados y analizados con SPSS v27. Se realizó una prueba de correlación entre las diferentes variables. Los datos se presentaron en forma de tablas. Un valor P de <0,05 se consideró estadísticamente significativo. El número total de participantes fue de 441; Hombres y mujeres representanron el 42,9 % y 57,1 %, respectivamente. La edad media de los participantes fue de 56,24±8,68 años. La arteria coronaria izquierda se originaba en el seno aórtico izquierdo. El diámetro medio y la longitud de la arteria coronaria izquierda fueron 3,8±0,70 mm y 8,1576±4,32 mm, respectivamente. Se encontró una correlación negativa significativa entre el diámetro del ostio, la edad y la ACI. Además se encontró una diferencia no significativa entre hombres y mujeres en el diámetro del ostio de la ACI (P=0,058) y una diferencia significativa en la longitud (P=0,00). Los sudaneses tienen el diámetro del ostio de la arteria coronaria izquierda más pequeño entre los africanos. Los hombres sudaneses tienen un diámetro del ostio de la arteria coronaria izquierda más pequeña y las mujeres tienen las arterias más largas. Un ostio más ancho de la arteria coronaria izquierda se asocia con una longitud arterial corta.

Expression analysis of myocardial strain index after repair of abnormal origin of left coronary artery of pulmonary artery in children / 中华胸心血管外科杂志

Objective:To study the expression characteristics of myocardial strain index after the abnormal origin of the left coronary artery of the pulmonary artery in children was repaired.Methods:The data of 30 children (study group) with abnormal origin of pulmonary artery left coronary artery repair from August 2017 to August 2021 were analyzed. In addition, healthy children during the same period were selected as the control group, and the study group was compared before and after treatment and the control group. Circumferential and radial peak myocardial strain index, post-contraction strain index.Results:The longitudinal, circumferential, and radial overall peak strain indexes of the study group before and after treatment were significantly lower than those of the control group, and the longitudinal, circumferential, and radial overall peak strain indexes of the study group after treatment were significantly higher than those before treatment ( P<0.05); The longitudinal, circumferential, and radial peak strain indexes of the study group before treatment were significantly lower than those of the control group. After treatment in the study group, the middle section of the longitudinal inferior wall, the middle section of the anterior wall, the basal section of the anterior wall, the apex, and the circumferential direction were significantly lower The peak strain index of the basal segment of the inferior wall and the middle segment of the inferior wall was significantly lower than that of the control group; and the longitudinal, circumferential, and radial peak strain indexes of the study group after treatment were significantly higher than those before treatment ( P<0.05); the study group children before treatment Longitudinal, circumferential, and radial PSI indexes were significantly lower than those of the control group. After treatment, the study group was treated in the longitudinal inferior wall, septal apical segment, circumferential inferior wall basal segment, inferior wall middle segment, and radial PSI anterior wall basal segment, apex. The part was significantly higher than that of the control group; and the longitudinal, circumferential, and radial PSI of the study group after treatment were significantly lower than before treatment ( P<0.05). Conclusion:After ALCAPA repair, the overall and regional strain and overall synchronization are improved, indicating that the resting myocardium has recovered, but the strain of certain segments supplied by the abnormal left coronary artery fails to normalize after ALCAPA repair. Persistent myocardial injury is consistent, which can provide some guidance for the prognosis assessment of children with ALCAPA.

Origen anómalo de la arteria coronaria izquierda a partir de la arteria pulmonar / Anomalous origin of left coronary artery from the pulmonary artery

Introducción: El origen anómalo de la arteria coronaria izquierda que nace en la arteria pulmonar, conocido como síndrome de Bland, White y Garland, es una enfermedad cardiaca congénita, de baja incidencia, espectro clínico variable y no es exclusiva de la edad pediátrica. Tiene una alta mortalidad en el primer año de vida si no se diagnostica y trata oportunamente. Objetivo: Describir las características del diagnóstico del síndrome de Bland, White y Garland. Métodos: Se realizó la caracterización de la entidad en Cuba en un decenio (2005 a 2015). Se incluyeron todos los pacientes con manifestaciones clínicas sugerentes de síndrome de Bland, White y Garland y confirmación ecocardiográfica. Se realizó el cálculo de la prevalencia de la entidad y se empleó el porcentaje en las variables cualitativas y la prueba exacta de Fisher. La validación estadística de los resultados de la investigación adoptó nivel de significación menor a 5 por ciento. Resultados: La prevalencia estimada en esa década en Cuba estaba alrededor de 2,9 por 300 000 nacidos vivos. Se demuestra la variabilidad en sus manifestaciones clínicas y amplio rango de edad de presentación. El tratamiento es quirúrgico con excelente supervivencia y pronóstico. Conclusiones: El diagnóstico del síndrome de Bland, White y Garland es difícil mediante el método clínico debido a la diversidad en su presentación. La prevalencia estimada en Cuba es mayor que lo registrado en el mundo actual. El diagnóstico temprano y tratamiento oportuno mejora de forma significativa la supervivencia y el pronóstico de los pacientes aquejados por esta dolencia(AU)

Introduction: The anomalous origin of left coronary artery that originates in the pulmonary artery, known as Bland, White and Garland is a congenital heart disease, with low incidence, variable clinical spectrum and is not exclusive of the pediatric age group. It has a high mortality in the first year of life if not diagnosed and treated early. Objective: To describe the characteristics of the diagnosis of Bland, White and Garland syndrome. Methods: It was made the characterization of the entity in Cuba in a decade (2005 to 2015). There were included all the patients with clinical manifestations suggestive to Bland, White and Garland syndrome and echocardiographic confirmation. It was made the calculation of this entity´s prevalence and it was used the percentage in the qualitative variables and Fisher's exact test. The statistical validation of research results had a significance level lower than 5 percent. Results: The estimated prevalence in that decade in Cuba was around 2.9 per 300 000 live births. It was demonstrated the variability in its clinical manifestations and wide range of onset´s age. The treatment is surgical with excellent survival and prognosis. Conclusions: The diagnosis of Bland, White and Garland syndrome is difficult through the clinical method due to the diversity in its presentation. The estimated prevalence in Cuba is greater than what was registered in the world today. Early diagnosis and prompt treatment significantly improves the survival and prognosis of patients suffering from this condition(AU)

A case of dual left anterior descending artery with myocardial infarction

The Left anterior descending artery (LAD) is a branch of the left main coronary artery which runs obliquely towards the apex of the heart in the anterior interventricular sulcus. Among all of the coronary arteries, the LAD artery has the most constant course. Amongst the anomalies of coronaries, the duplication or bifurcation of the LAD artery is infrequent. The classification of the bifurcation of the LAD has been extensively described in various reports with the widespread use of CT Angiography. We describe herein, an anomalous LAD detected on autopsy. This unusual case highlights the gross autopsy finding of Type-I anomalous dual LAD coronary artery in a young adult who died of Myocardial Infarction.

Hemodynamic Analysis on Anomalous Origin of the Right Coronary Artery from the Left Coronary Artery Sinus / 医用生物力学

Objective To analyze the hemodynamic parameters of anomalous origin of the right coronary artery from the left coronary artery sinus (AORL) based on computational fluid dynamics (CFD), so as to make an evaluation of the disease. Methods A normal right coronary artery (RCA) case and an AORL case were selected. Two models were reconstructed in Mimics software and imported into ANSYS CFX software for hemodynamics simulation. The hemodynamics of normal RCA model and AORL model were compared. Results AORL model had a smaller volume flow (9.35 cm3/s), which might lead to insufficient blood supply downstream of the RCA; the pressure at the acute corner of AORL model (13.78 kPa) was lower than normal RCA model (14.9 kPa); the wall shear stress (WSS) of AORL model (12.83 Pa) was larger than that of normal RCA model (9.74 Pa); the total deformation of AORL model was relatively large. Conclusions The entrance velocity and pressure of AORL were lower than those of normal RCA, which might lead to ischemic symptoms. The research findings are of theoretical significance for the effective evaluation of ischemia and other diseases in clinic.

An Infant Case of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Successfully Treated with Modified Spiral Cuff Technique / 日本心臓血管外科学会雑誌

The patient was a six-month-old girl with an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). She had fever and visited a family physician at 5 months of age. Because of poor oxygenation, she was referred to our pediatric department and intubated soon after hospitalization. Echocardiography showed that the orifice of left coronary artery was just above pulmonary commissure, the left ventricular ejection fraction was 9%, and the level of mitral regurgitation was moderate. Right coronary angiography showed that the left coronary artery contrasted against the collateral arteries. The left coronary artery originated from the left side of the pulmonary trunk. After recovery of the general condition with medical therapy, the patient underwent coronary artery reconstruction by the modified spiral cuff technique on the 21st day of hospitalization. The temporary detachment of pulmonary valve and its commissure for making a margin around the left coronary artery enabled us to make the spiral cuff in almost the usual manner. She was moved to the intensive care unit with the support of extracorporeal membrane oxygenation (VA-ECMO) and was successfully weaned off the VA-ECMO 5 days after the surgery. The postoperative course was good, and she was discharged from our hospital 3 months after the surgery. The echocardiogram one year after the surgery showed a left ventricular ejection fraction of 30%, mild mitral regurgitation, and mild pulmonary regurgitation. Our experience indicates that the spiral cuff technique is a useful coronary reconstruction method for the treatment of ALCAPA, especially in cases presenting a considerable distance between the origin of the left coronary artery and the transplantation site. There are few reports regarding the surgical treatment of infantile ALCAPA showing reduced left ventricular function. Coronary artery reconstruction using the spiral cuff method and planned VA-ECMO are useful surgical procedures in such cases. Our experience also suggests that the establishment of a treatment strategy including mechanical support is essential to improve the results in severe ALCAPA cases.

Cadaveric Study On Normal And Variant Anatomy Of Left Coronary Artery

Background: Human heart is supplied by coronary arteries – Right and Left coronary artery. The coronary arteriesarise from the aortic sinuses and the left coronary artery from the left posterior aortic sinus. The left coronaryartery has two branches, the anterior interventricular and circumflex arteries. The anterior interventricularbranch is the continuation of left coronary artery, gives off septal branches, right and left ventricular branches.The left ventricular branches are called diagonal arteries. The left circumflex artery gives off left atrial and leftventricular branches. One of these atrial branches supply the sinoatrial node in 35% of subjects and AV node in10-20% of the subjects.Materials and Methods: The study was carried out in the department of anatomy, Kasturba Medical College,Manipal, India. The study was performed on 50 formalin fixed human hearts of unknown sex and age. The leftcoronary artery and their branches were carefully dissected. The origin, branches & branching pattern of leftcoronary artery was observed, noted and photographed.Results: In present study, 49 samples (98%) showed the origin of left coronary artery from left posterior aorticsinus while 01 sample (02%) had no trunk of left coronary artery. Sino-atrial nodal artery was originating formcircumflex artery in 13 samples (26%) and atrio-ventricular nodal artery from the circumflex artery in 05samples (10%). The trunk of left coronary artery was bifurcating in 37 samples (74%) and trifurcating in 12samples (24%) with one sample (02%) showing absent trunk of left coronary artery. The median artery waspresent in 12 samples (24%) and posterior interventricular artery was originating from circumflex artery in 05samples (10%).Conclusion: Left coronary artery commonly originated from left posterior aortic sinus with very few variations.Sino-atrial nodal artery and atrioventricular nodal artery commonly originates from right coronary artery.Bifurcation of left coronary artery is commoner than trifurcation. The present study is useful in better understandingof the normal and variant anatomy of left coronary artery

Clustered Origin Of Left Coronary Artery From The Left Sinus Of Valsalva: Uncharacteristic Developmental Event In The Temporally Regulated Process

Background: The coronary artery development is a sequential and progressively regulated process. However,there are number of reports available on the disturbance to this temporal process resulting in variations of theartery. In these instances, the function of the coronary arteries was normal in providing blood supply to theheart. The normal and abnormal coronary artery development has been studied using variety of approaches andthe knowledge continues to evolve.Materials and Methods: The cadaveric specimen was obtained from the “willed body program” for the purpose ofstudent dissection. We report here an anomaly found in a 94-year old Caucasian male died of cerebral arteryinfarction.Results and Observations: We observed that the left coronary artery arose from the coronary ostium in a clusterof multiple branches. The right coronary artery arose normally from the right coronary ostium. Further dissectionshowed the co-dominant nature of the left and right coronary arteries in providing blood supply to the posterioraspect of the heart and the interventricular septum.Conclusions: Knowledge of coronary artery and its disposition is important in clinical practice especially incoronary artery stent placement, coronary artery bypass surgery and coronary sinus catheterization.

Adult anomalous origin of left coronary artery from pulmonary artery: A case report and literature review / 吉林大学学报(医学版)

Objective: To explore the pathogenesis, clinical characteristics, diagnosis and treatment methods of anomalous origin of left coronary artery from pulmonary artery (A L C A P A), and to improve the clinicians'understanding of the disease. Methods: The clinical data of an adult patient with ALCAPA who had been misdiagnosed as endocardial fiborelastosis (E F E) were retrospectively analyzed; in the meanwhile, the related literatures were reviewed. Results: The patient admitted to hospital because of her intermittent precordial pain, chest tightness and shortness of breath, and suffered from more than 2 months, aggravated for 3 d. The patient was preliminarily diagnosed as cardiomyopathy (considering the great possibility of E F E), atrial fibrillation, N Y H A E. After treatment, paroxysmal retrosternal pain still occurred, and ventricular fibrillation occurred one time. Finally, the patient was diagnosed as ALCAPA by coronary artery CTA, and treated by coronary artery bypass grafting. After operation, the patient recovered well. Conclusion: ALCAPA is relatively rare, and its clinical manifestations lack specificity, so it is easy to misdiagnose. Coronary artery CTA is an effective diagnostic method, and operation is the first choice for its treatment.

Clinical characteristics of anomalous origin of the left coronary artery from the pulmonary artery in 91 children / 中华儿科杂志

Objective@#To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) .@*Methods@#A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t-test and a Chi-square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient.@*Results@#(1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54) , whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0±7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ2=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow-up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function.@*Conclusions@#Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long-term follow-up treatment.

Clinical study on surgical repair of severe ALCAPA in infants and children / 中华胸心血管外科杂志

Objective To discuss the surgical treatment of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA) and the risk factors of postoperative recovery in infants and children.Methods A retrospective review of all patients who underwent surgical treatment of ALCAPA in Shanghai Children Medical Center(2003.1-2018.1) was conducted.Patients were divided into early surgery group (2003.1-2012.12) and late surgery group (2013.1-2018.1) according to the operation time,a preoperative left ventricular ejection fraction(LVEF) ＜0.35 was defined as severe group and LVEF≥0.35 as the control group in both surgery group.Meantime,and the clinical data among the groups were analyzed and compared.Results 102 patients were included in our study.According to the operation time and preoperative LVEF grouping:10 cases in the early severe group,early death in 4 cases(40％);28 cases in the early control group,and 3 cases died(10.7％)in hospital.Preoperative LVEF(0.29 ± 0.06 vs.0.53 ± 0.12),surgical age [(8.0 ± 7.9) months vs.(23.3 ± 27.7)months],and cardiopulmonary bypass time [(131.1 ± 39.6) min vs.(103.8 ± 29.8) min] were statistically different between the early two groups.The results of the late surgery had been improved:24 cases in the late severe group,4 cases died in hospital(16.7％);40 cases in the late control group,and early death in 2 cases (5％).In the late surgery groups,there was a statistically significant difference in preoperative LVEF(0.28 ±0.05 vs.0.59 ±0.12),left ventricular end-diastolic diameter(LVDD) Z-score(3.09 ±1.16 vs.2.11 ±0.95),and surgical age [(5.3 ±3.0) months vs.(24.8 ±30.5)months],clamping time [(67.1 ± 15.5) min vs.(82.7 ± 28.4) min].In the severe group,there was no significant difference in preoperative clinical data between early and late patients,and the early mortality decreased from 40％ in the early period to 16.7％ in the late period.In this study,13 cases(38.2％) of children with severe ALCAPA underwent mechanical circulation support(MCS).One patient died during MCS support and 2 died after weaning.Conclusion The early mortality severe ALCAPA remains high,which may be related to severe cardiac ischemia,left ventricular enlargement and age at surgical time.The modify of surgery technology and the use of MCS in the early clinical stage can improve the early survival rate.

Surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery / 中国胸心血管外科临床杂志

@#Objective     To review the experience of the surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods     A retrospective, single institution review was conducted on thirty-six adult patients with ALCAPA surgical treatment from November 1991 to November 2017 in Fuwai Hospital. Of these patients, nine were males and twenty-seven were females. The mean age was 36.6±13.3 years. The mean weight was 60.0±9.4 kg. The preoperative echocardiography showed the mean left ventricular ejection fraction (LVEF) was 57%±6% and the mean left ventricular end-diastolic dimension (LVEDD) was 52.3±6.3 mm. Severe mitral regurgitation (MR) was seen in one patient, moderate in five patients. The operative procedures included coronary artery re-implantation in seventeen patients, Takeuchi operation in sixteen patients, ligation of left coronary artery plus coronary artery bypass graft in three patients. In addition, six patients underwent mitral valve repair. Results     There was no in-hospital mortality. The mean cardiopulmonary bypass time was 152.5±72.9 min and aortic cross clamp time was 101.9±43.6 min, respectively. The mean mechanical ventilation time and ICU time was 17.3±16.3 h and 43.1±30.7 h, respectively. The mean postoperative LVEF was 59%±6%, which did not significantly improve compared with preoperative LVEF. However, the mean postoperative LVEDD of 46.9±5.9 mm had significant reduction compared with the preoperative LVEDD. Of the six patients with mitral valve repair, one was mild and the other five were trivial. Thirty-five patients (97.2%) completed the follow-up with a mean time of 5.5 years. All the patients survived with New York Heart Association class Ⅰor Ⅱ. Two patients needed interventional occlusion or re-operation due to the fistula of internal tunnel within the pulmonary artery. At the latest echocardiography, the mean LVEF of 69%±7% improved significantly compared with the preoperative LVEF. Mild MR was detected in ten patients, moderate in two patients during the follow-up period. Conclusion     The surgical treatment of adult patients with ALCAPA has satisfactory short- and long-term results. The patients who underwent Takeuchi procedure may need re-operation due to fistula of internal tunnel within the pulmonary artery during the long-term follow-up.

Clinically Relevant Morphometric Study Of Left Coronary Artery In Adult Human Cadavers

Background: The anatomical variations of left coronary artery [LCA] determine the course in the pathogenesis ofatherosclerosis, mechanical stress and hemodynamic change.Aim: To study the gross anatomy of left coronary artery [LCA] in terms of its origin, termination, branchingpattern, dominance pattern, external diameter at origin, length of main trunk of left coronary artery, variationsand/ anomalies if present.Materials and Methods: After an ethical approval, 150 adult human cadaveric hearts were collected fromDepartment of Anatomy, B.V.D.U. Medical College and Hospital, Sangli and Pune. The careful dissection wascarried out to note details about left coronary artery and data was analyzed using SPSS software.Results: The origin of left coronary artery was observed in the left posterior aortic sinus 100%. The incidence ofbifurcation, trifurcation and quadrifurcation was 69.33, 28% and 2.67% respectively. SA nodal artery was directlyarising from main trunk of left coronary artery in 2 hearts (1.33%). Circumflex branch of left coronary artery gaveSA nodal artery, AV nodal artery and posterior interventricular artery in 18.66%, 16% and 16% hearts respectively.In one case (0.66%), we found a hyperdominant left anterior descending artery which continued as posteriorinterventricular artery [PDA] occupying entire posterior interventricular sulcus and terminated at crux of theheart by giving AV nodal artery. Hence left dominance was observed in total 16.66% cases. The mean externaldiameter of left coronary artery at its origin was 5.02 ±1.0328. Length of main trunk of left coronary artery wasranging from 4 mm to 22 mm with mean length of 11.66±3.529 mm.Conclusion: Short or long main trunk of left coronary artery, small diameter of main trunk, additional terminalbranches of left coronary artery, left coronary artery dominance, Mouchet’s posterior recurrent interventricularartery, hyperdominanant left anterior descending artery are the significant anatomical factors which decide theextent of coronary insufficiency, its functional impact and may create challenges during the interventionalcoronary care.

A Study On Variation In The Termination Of Right And Left Coronary Arteries And Their Clinical Significance

Aim: This current study is done to throw light upon the, distribution and termination of right coronary artery andleft anterior descending branch of left coronary artery and to correlate the clinical significance of the variationsobservedMaterials and methods: 30 human hearts from the embalmed cadavers from Department of Anatomy, GreatEastern Medical School were collected during routine dissection. The heart is taken out after incising the fibrouspericardium and great vessels .The course of right and left coronary arteries were traced from the Ostia. Thespecimens were duly numbered, preserved in 5% formaldehyde solution. Photographs of each specimen weretaken by digital camera and the arterial pattern is coloured red digitally and labeled.Results: In the present study the right coronary artery terminates at crux or beyond the crux in 57%, before cruxin 23% and right border in 13%. Left anterior descending artery terminates beyond apex in the lower 1/3rd of theinter-ventricular groove in 87% and at the apex in 13%.Conclusion: Right and left coronary arteries showed significant variation in their mode of termination. Betteranatomical knowledge about the branches of coronary artery and its variation is essential for cardiologists andinterpretation of coronary angiograms by radiologist.

Utilidad de la ecocardiografía en la detección de la insuficiencia cardiaca en un adulto joven con síndrome de origen anómalo de la arteria coronaria izquierda del tronco de la arteria pulmonar y válvula mitral asimétrica similar al paracaídas / Use of echocardiography in the detection of heart failure in a young adult with anomalous left coronary artery from the pulmonary artery syndrome andparachute-like asymmetric mitral valve

Resumen Objetivo: describir un caso de un paciente joven con insuficiencia cardiaca, secundaria a dos malformaciones cardiacas infrecuentes, síndrome de ALCAPA y válvula mitral asimétrica, parecida al paracaídas, resaltando la utilidad de la ecocardiografía. Métodos: se analiza el caso a la luz de la literatura médica. Conclusiones: El origen anómalo de la arteria coronaria izquierda del tronco de la arteria pulmonar y la válvula mitral asimétrica parecida al paracaídas, son malformaciones raras, asociadas a insuficiencia mitral severa e insuficiencia cardiaca. No se encontraron reportes en la literatura acerca de la coexistencia de las dos patologías en un paciente.

Abstract Objective: To describe a case of a young adult with heart failure, secondary to two rare cardiac malformations, anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome and parachute-like asymmetric mitral valve, highlighting the use of echocardiography. Material and methods: A case is analysed along with a search in the medical literature. Conclusions: The anomalous origin of left coronary artery from the pulmonary artery and parachute-like asymmetric mitral valve are rare malformations associated with severe mitral insufficiency and heart failure. No reports were found in the literature as regards the existence of these two diseases in a patient.

Clinical study of 62 cases of anomalous left coronary artery from the pulmonary artery / 临床儿科杂志

Objective To explore the clinical manifestation, treatment and prognosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). Method The clinical data of patients with ALCAPA from January 2011 to September 2016 were retrospectively analyzed. All the patients were divided into infant group (<12 months) and child group (≥12 months). Results In the 62 patients (38 infants and 24 children) with ALCAPA, the median ΔLVDD (actually measured LVDD – the normal upper limit of LVDD in this age group) of infant group and child group was 13.6 (8.4～17.5) mm and 8.5 (3.3～13.7) mm respectively. The mean LVEF of the infant group was 40.6±2.4 ％, lower than that of the children group (59.0±2.9) ％, and the difference was statistically significant (P<0.01). Sixty children were treated with surgery. The mean cardiopulmonary bypass duration of infant group (137.1±8.4 min) was longer than that of the children group (105.9±6.6 min), and the difference was statistically significant (P=0.010). The median mechanical ventilation time after operation in infant group (6 d, 3～7 d), was longer than that in the children group (2 d, 1～4 d), and the difference was statistically significant (P<0.01). The median follow-up time of 60 patients was 38 months. Six patients died within 1 month after surgery (5 cases in the infant group and 1 in the children group), and there were no deaths more than 1 month after surgery. In 22 cases, the flow rate of pulmonary valve increased rapidly within 1 week after operation (>1.8 m/s). Conclusion ALCAPA is easily misdiagnosed as dilated cardiomyopathy. The clinical manifestations of infants were more severe than those of children. The mortality was high within 1 month after surgery, and the long-term prognosis was better.

Clinical efficacy of reimplantation of anomalous origin of the left coronary artery from the pulmonary artery: A case control study / 中国胸心血管外科临床杂志

@#Objective    To analyze the clinical efficacy and mid-term outcomes of reimplantation of anomalous origin of left coronary artery from the pulmonary artery (ALCAPA), and to evaluate whether concomitant management of mitral regurgitation (MR) during ALCAPA repair is needed. Methods    Between March 2005 and March 2015, 52 consecutive patients (20 males and 32 females with a median age of 10 months ranging 2-193 months) underwent reimplantation of ALCAPA at Department of Cardiac Surgery, Guangdong Cardiovascular Institute. There were 14 males and 21 females with a mean age of 35.4±42.8 months not receiving mitral valvuloplasty (a Non-MVP group), and 6 males and 11 females with a mean age of 13.5±11.0 months receiving mitral valvuloplasty (a MVP group). In order to facilitate the analysis, degree of MR was graded by number: 0.0=none, 1.0=trivial, 2.0=mild, 2.5=mild-moderate, 3.0=moderate, 3.5=moderate-severe and 4.0=severe. Results    The left ventricular fractional shortening (LVFS) and left ventricular end diastolic diameter (LVEDD) demonstrated significant improvement between preoperation and discharge (28.6%±9.6% vs.   32.1%±10.1%, P=0.023; 38.4±5.6 mm vs. 30.5±5.7 mm, P<0.001), and there was also significant improvement in the mean MR grade between preoperation and discharge (2.9±1.2, vs. 2.4±1.2, P=0.001). There were 4 in-hospital deaths (7.7%). The median follow-up was 21.0 months (ranging 1.5-111.0 months). Three patients (5.8%) were lost to follow-up, 1 patient required reoperation for mitral valve replacement and there was no death during follow-up. Significant improvement was seen in LVFS between discharge and final follow-up (32.1%±10.1% vs. 38.0%±6.0%, P=0.001); however, there was no significant difference in the degree of MR between discharge and final follow-up (2.4±1.2 vs. 2.3±1.2, P=0.541). There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, mechanical ventilation time or hospital stay between the two groups. Conclusion    Creation of a dual coronary system with reimplantation of the left coronary artery results in complete recovery of left ventricular function. However, concomitant management of MR during ALCAPA repair remains controversial. Concomitant mitral valve repair for ALCAPA patients with moderate-severe and severe MR is helpful to early function recovery of mitral valve.