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.

Clinical analysis of 208 children with congenital coronary artery origin abnormalities / 中国小儿急救医学

Objective:To describe the clinical features and prognosis of congenital anomalous origin of coronary artery(AOCA) in children to increase our understanding of the disease.Methods:This retrospective study included children diagnosed with AOCA using computed tomography coronary angiography(CTCA) admitted to the Department of Cardiology, Beijing Children′s Hospital, Capital Medical University, from January 1, 2014 to December 31, 2019.The clinical presentations, laboratory results, imaging analyses, treatments, and prognoses of these patients were analyzed.Results:A total of 208 children, including 105 boys and 103 girls, we evaluated the ages(9.03±4.18)years old with AOCA.Of these, 157 cases(75.5%) presented with cardiac symptoms, such as chest tightness, palpitations, dizziness, syncope, fatigue, and decreased endurance.Three cases(1.4%) had atypical symptoms of paroxysmal crying, dyspnoea and cyanosis, and 48 cases(23.1%) were asymptomatic.Levels of serological markers of myocardial injury were elevated in 59 cases(28.4%), and 140 cases(67.3%) had predominant ST-T abnormalities on electrocardiograms.Transthoracic echocardiography identified 27 cases (13%) with cardiac enlargement and ten cases(4.8%) with left ventricular systolic dysfunction.There were 126 cases(60.6%) with the anomalous origin of the left coronary artery revealed by CTCA, 50 cases(24.0%) with the anomalous origin of the right coronary artery and 32 cases(15.4%) with bilateral coronary arteries of anomalous origin.Five children underwent surgical treatment, and the remaining 203 children were treated conservatively with drugs.The whole group was successfully treated, and no death case was recorded during the follow-up period.Conclusion:AOCA may cause different degrees of myocardial ischemia.Diverse clinical presentations and diagnostic limitations of transthoracic echocardiography often lead to missed diagnosis or misdiagnosis.In contrast, CTCA has high diagnostic accuracy and can be used to identify the location and course of the coronary ostia.Hence, the management of AOCA should be tailored on a case-to-case basis, taking into consideration of the specific type of coronary origin, with surgical intervention being warranted if necessary.

A Surgical Case of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in Early Infancy / 日本心臓血管外科学会雑誌

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital defect, and few cases have been reported in the pediatric age group. Unlike the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), patients with ARCAPA are usually asymptomatic and often diagnosed incidentally. Here, we present a surgical case of a 2-month-old male who was suspected of having ARCAPA in the neonatal period. The patient was suspected of having ARCAPA due to the detection of a heart murmur and the results from an echocardiogram taken the 4th day after birth. Coronary angiography on the 37th day after birth showed the contrast flow to the pulmonary artery through the right coronary artery from the left coronary artery; thus, we diagnosed ARCAPA. Surgical intervention should be performed before the appearance of myocardial ischemia due to decreasing pulmonary artery pressure, so we performed reimplantation of the right coronary artery to the ascending aorta and patch plasty of the pulmonary artery at 2 months after birth. The patient was discharged from the hospital on the 16th day after the operation, and a 6-month postoperative coronary angiography revealed good right coronary flow and the disappearance of collaterals.

A Case of Surgical Treatment of Anomalous Origin of the Single Coronary Artery from the Pulmonary Artery / 日本心臓血管外科学会雑誌

A 2-month-old male infant was transferred to our hospital for suspected cardiomyopathy because he had livedo reticularis and peripheral coldness. An electrocardiogram showed ischemic change and an echocardiogram showed a dilated, poorly functioning left ventricle. Therefore, we performed urgent cardiac catheterization. The coronary artery was not visualized by aortography, but the single coronary artery arising from the pulmonary artery was depicted by pulmonary angiography. Therefore, we performed emergent surgery. The whole coronary arteries traveled directly inferiorly from the left side of the pulmonary trunk. A patent ductus arteriosus (PDA) was closed at the operation. We established cardiopulmonary bypass using two arterial cannulae through both ascending aortas and pulmonary trunk to maintain coronary blood flow. Direct implantation is difficult because the coronary ostium is far from the ascending aorta. Therefore, we chose to perform the Takeuchi procedure using an intrapulmonary artery tunnel. The patient's postoperative course was good, and he was discharged on postoperative day 22. A case of an anomalous origin of a single coronary artery from the pulmonary artery without any other heart disease is extremely rare. Management of cardiopulmonary bypass and myocardial protection in this abnormality is discussed.

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)

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.

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 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.

Clinical characteristics of anomalous origin of coronary artery accompanied with myocardial damage in children:A report of 72 cases / 中国实用儿科杂志

OBJECTIVE: To investigate the clinical characteristics and treatment strategies of anomalous origin of the cor⁃onary artery accompanied with myocardial damage,so as to improve the level of clinicians' diagnosis and treatment of this disease. METHODS: The clinical data of 72 cases of anomalous origin of the coronary artery accompanied with myo⁃cardial damage admitted in Department of Cardiology,Beijing Children's Hospital,Capital Medical University from2015 to 2017 were analyzed retrospectively. The clinical characteristics,diagnosis and treatment methods were analyzed.RESULTS: In 42 cases(58.3%),chest pain,chest tightness and palpitation were the main clinical symptoms. ECG abnor⁃malities were mainly ST-T changes(62.5%). Echocardiography showed 13 cases(18.1%)of cardiac enlargement,4 cas⁃es(5.6%)of left ventricular systolic dysfunction. Coronary artery CT angiography(CTA)showed that the anomalous ori⁃gin of the left coronary artery was the most frequent(73.6%),followed by the anomalous origin of bilateral coronary arter⁃ies(13.9%),and the anomalous origin of the right coronary artery was the least frequent(12.5%). The whole group was successfully treated with no death. CONCLUSION: There might be a correlation between the anomalous origin of coronary artery and ischemic myocardial damage. In the examination of patients,pediatricians should pay more attention to the judgments of the position and movement of the coronary artery by CTA,and attach importance to the differential diagno⁃sis between ischemic myocardial damage and myocarditis.

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.

Improvement of the Ischemic Area after Surgical Treatment for Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery / 日本心臓血管外科学会雑誌

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly. Although asymptomatic in most cases, with the anomaly only being detected incidentally, surgical correction should be considered before onset of severe myocardial ischemia in such cases. Here, we present a 70-year-old man who was referred to our department due to chest pain on effort and was given a diagnosis of ARCAPA concomitant with mild aortic stenosis. As the symptoms and the degree of aortic stenosis deteriorated during follow-up, the patient underwent direct re-implantation of the right coronary artery into the ascending aorta and aortic valve replacement. The patient's postoperative course was uneventful, and the symptoms disappeared. Postoperative myocardial perfusion scintigraphy revealed improvement of the myocardial ischemic area.

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta and unilateral absence of pulmonary artery by echocardiography / 中华超声影像学杂志

Objective@#To improve the prenatal diagnosis and differential diagnosis ability for anomalous origin of one pulmonary artery branch from the aorta (AOPA) and unilateral absence of pulmonary artery (UAPA) by comparing and analyzing different types of fetal echocardiography features.@*Methods@#Fetal echocardiographic features were analyzed retrospectively in fetus with prenatal diagnosis of UAPA or AOPA between June 2012 and December 2018, and the different types of fetal echocardiography characteristics and main points of identification were summarized.@*Results@#A total of 6 fetuses with AOPA and 6 fetuses with UAPA were diagnosed.Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and 1 case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with an absent pulmonary valve. All 6 cases of AOPA had proximal form. Among the 6 fetuses with UAPA, 4 cases were isolated unilateral absence of right pulmonary artery, 1 case was isolated unilateral absence of left pulmonary artery and 1 case was unilateral absence of left pulmonary artery combined with tetralogy of Fallot (TOF). The main common characteristic sonographical findings included: there was no confluence at the bifurcation of the main pulmonary artery on multiple views, and the affected pulmonary branch was not attached to the main pulmonary artery. The different characteristic sonographical findings in fetus included: the affected pulmonary artery of AOPA arosed from the right posterior or left posterior portion of the ascending aorta on three vessels and trachea view (3VT) and long axial view of left ventricle, and 3VT and the coronary view of brachiocephalic trunk artery showed that the abnormal origin of pulmonary artery branch of UAPA connected with the root of brachiocephalic trunk artery by the ipsilateral vertical arterial duct (DA), and color Doppler flow imaging showed that the blood flow of the affected pulmonary artery branch was isolated in the pulmonary field.@*Conclusions@#AOPA and UAPA have many similarities in the characteristics of fetal echocardiography. 3VT and long axial view of left ventricle are the best views for displaying proximal AOPA, 3VT and the coronary views of the root of brachiocephalic trunk artery are the best views for displaying UAPA.

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta and unilateral absence of pulmonary artery by echocardiography / 中华超声影像学杂志

Objective To improve the prenatal diagnosis and differential diagnosis ability for anomalous origin of one pulmonary artery branch from the aorta ( AOPA ) and unilateral absence of pulmonary artery ( U APA ) by comparing and analyzing different types of fetal echocardiography features . Methods Fetal echocardiographic features were analyzed retrospectively in fetus with prenatal diagnosis of U APA or AO PA between June 2012 and December 2018 ,and the different types of fetal echocardiography characteristics and main points of identification were summarized . Results A total of 6 fetuses with AOPA and 6 fetuses with U APA were diagnosed .Among the 6 fetuses with AOPA ,5 cases were anomalous origin of right pulmonary artery from the aorta ( AORPA ) ,including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome ,and 1 case was anomalous origin of left pulmonary artery from the aorta ( AOLPA ) with an absent pulmonary valve . All 6 cases of AOPA had proximal form . Among the 6 fetuses with U APA ,4 cases were isolated unilateral absence of right pulmonary artery ,1 case was isolated unilateral absence of left pulmonary artery and 1 case was unilateral absence of left pulmonary artery combined with tetralogy of Fallot ( T OF) . T he main common characteristic sonographical findings included :there was no confluence at the bifurcation of the main pulmonary artery on multiple view s ,and the affected pulmonary branch was not attached to the main pulmonary artery . T he different characteristic sonographical findings in fetus included :the affected pulmonary artery of AO PA arosed from the right posterior or left posterior portion of the ascending aorta on three vessels and trachea view ( 3V T ) and long axial view of left ventricle , and 3V T and the coronary view of brachiocephalic trunk artery showed that the abnormal origin of pulmonary artery branch of U APA connected with the root of brachiocephalic trunk artery by the ipsilateral vertical arterial duct ( DA ) ,and color Doppler flow imaging showed that the blood flow of the affected pulmonary artery branch was isolated in the pulmonary field . Conclusions AOPA and UAPA have many similarities in the characteristics of fetal echocardiography . 3V T and long axial view of left ventricle are the best view s for displaying proximal AOPA ,3VT and the coronary view s of the root of brachiocephalic trunk artery are the best view s for displaying UAPA .

Acute coronary syndrome associated with anomalous origin of circumflex artery. Case report and literature review / Síndrome coronario agudo asociado al origen anómalo de la arteria circunfleja. Reporte de caso y revisión de literatura

Abstract: We present a case of an acute coronary ischemic syndrome associated with anomalous origin of the circumflex artery, with a right coronary sinus origin independent of the right coronary artery, which has a prevalence of 0.1 to 8.4%. This type of anomaly is classified according to the international guidelines as low-risk, however, at high risk of presenting atheroma, coronary complications,(1) and acute coronary ischemic syndrome due to its occasionally intraarterial pathway. The treatment depends on the complexity of the coronary anatomy. In this case with the presence of total obstruction the correct therapy was to perform a percutaneous coronary angioplasty with drug-eluting stent (DES) placement, which improves the survival and is therapeutic at the acute phase, as in the case of this patient.

Resumen: Presentamos un caso de síndrome isquémico coronario agudo asociado al origen anómalo de la arteria circunfleja, a partir del seno coronario derecho independiente de la arteria coronaria derecha, que tiene una prevalencia de 0.1 a 8.4%. Este tipo de anomalía se clasifica según las guías internacionales como de bajo riesgo; sin embargo, con alto riesgo de presentar desarrollo de ateroma y complicaciones coronarias, síndrome isquémico coronario agudo debido a su trayecto en ocasiones intraarterial. El tratamiento depende de la complejidad de la anatomía coronaria. En este caso de obstrucción total, la terapéutica correcta fue realizar angioplastia coronaria percutánea con la colocación de stent fármaco activo, lo que mejora la sobrevida y es terapéutico en el momento agudo, como en el caso de este paciente.

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.

Prenatal ultrasonic diagnosis of anomalous origin of one pulmonary artery from the aorta and literature review / 中华超声影像学杂志

Objective To summarize the prenatal ultrasonographic characteristics of anomalous origin of one pulmonary artery from the aorta( AOPA ) ,and describe the diagnostic and clinical outcomes of fetal AOPA . Methods Echocardiographic characteristics of 3 fetuses with AOPA were reviewed . The ultrasonographic features were comparatively analyzed with postpartum autopsy findings . The relevant literature were reviewed and the experience of prenatal diagnosis of AOPA were summarized . Results In 3 cases with AOPA ,2 cases were the proximal type and anomalous origin of right pulmonary artery from the aorta ,of them ,one was accompanied with coarctation of the aorta , the other was accompanied with aortopulmonary window . One case was the distal type and anomalous origin of left pulmonary artery from the left innominate aorta ,it was only associated with mirror right aortic arch and right ductus arteriosus . Ultrasound characteristics were no bifurcation in distal pulmonary artery ,main trunk of pulmonary artery extends directly to one branch ,the other branch originated from the ascending aorta or left innominate artery . Three cases were confirmed by postpartum autopsy . Conclusions There are some characteristic signs on ultrasonic features of AOPA . Prenatal ultrasound has important value for diagnosis of AOPA .

The value of MSCTA in detecting anomalous origin of coronary artery / 实用放射学杂志

Objective To evaluate the value of multi-slice spiral computed tomography angiography(MSCTA)scanning and reconstruction technology in detecting anomalous origin of coronary artery(AOCA).Methods Retrospective analysis was done in 3 856 patients who accepted MSCTA.Volume rendering(VR),multi-planar reformation(MPR),curved planar reformation(CPR)and maximum intensity projection(MIP)were used to observe the origin and course of coronary artery.Results 42 patients with AOCA were detected among 3 856 objects,and the detection rate was 1.09%.The detection rates had no statistically significant difference between male(1.17%)and female(0.98%).The rate of patients with anomalous origin of left coronary artery was 30.95%(13/42), and 9 objects(69.23%,9/13)of them had the anomalous origin of left circumflex.The rate of patients with anomalous origin of right coronary was 66.67%(28/42),and 35.71% of them(17/28)were found to have the anomalous origin of right coronary artery from the left sinus of valsalva.Conclusion MSCTA scanning and reconstruction technology is noninvasive,rapid,accurate and intuitive.

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. A further imaging work-up, including computed tomography or magnetic resonance imaging, might be helpful.

An Adult Surgical Case of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery / 日本心臓血管外科学会雑誌

Anomalous origin of the coronary artery from the pulmonary artery (ACAPA) is a rare congenital heart disease. A woman in her 60s was diagnosed as ARCAPA during examination for angina. A magnetic resonance image showed myocardial ischemia in a small area of the right coronary artery (RCA) and left anterior descending artery. Reimplantation of the RCA to the ascending aorta and patch plasty of the pulmonary artery were performed. Repositioning of the RCA orientation was necessary for relief of the kinking. The postoperative course was uneventful. She is now free from angina 15 months after surgery.

Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis / 日本心臓血管外科学会雑誌

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.