A Case of Pediatric Heart Failure Caused by Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Case Report and Literature Review

A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.

Consideration of the Necessity of Prophylactic Bypass Grafting for Anomalous Origin of the Right Coronary Artery—Based on a Case with Concomitant Left Main Trunk Disease Resuscitated from Cardiopulmonary Arrest

Anomalous origin of the right coronary artery is a rare congenital anomaly, but is associated with sudden death. Originating from the opposite sinus of Valsalva, an interarterial?course and an intramural course are especially considered as the risk factor for fatal cardiac events. Surgical indication remains controversial because many patients are asymptomatic. A 52-year-old man with anomalous origin of the right coronary artery with an interarterial?course concomitant with the left main trunk disease was resuscitated from cardiopulmonary arrest. It was likely to be attributed to the left main trunk disease, but anatomical structure of the right coronary artery suggests its possible involvement. Prophylactic bypass grafting for the right coronary artery was performed using saphenous vein graft without ligating native vessel to prevent future cardiac events, as well as revascularization of the left main trunk disease. All grafts were patent in one-year follow-up coronary angiography. Any cardiac event has not occurred.

“Treat-Repair-Treat”:Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

Anomalous origin of left pulmonary artery branch from the aorta with Fallot's tetralogy:one case report

我们报导经常与呼吸窘迫介绍了的一个十岁的男孩的案例后来出生。胸 X 光检查显示出一个扩大右室。Echocardiography 证明 Fallot 的四联剧和左肺的动脉的典型特征不能被设想。然而，心导管插入术透露左肺的动脉在上升主动脉有异常起源。病人经历了缺点的全部的外科的修正。九天以后，他被解除。我们在场发生的文学评论的结果， physiopathological，这稀罕疾病的临床、诊断、外科的特征。

Chest Pain on Exertion: Origin of the Right Coronary Artery from the Left Coronary Sinus, through the Aortic Pulmonary Sulcus with Squeezed, or Anterior Descending Branch Borderline Coronary Lesion?

The prognosis of patients in whom the right coronary artery (RCA) arises from the left coronary sinus (LCS) is unequal. An initial intramural course of the coronary artery within the aortic media is considered to cause myocardial ischemia in cases of coronary anomalies. In this case report, we present one patient admitted due to chest pain on exertion that suggested angina. Her only risk factor for coronary artery disease was hypertension. Clinical examination findings that the origin of the right coronary artery from the LCS, through the aortic pulmonary sulcus with atherosclerotic narrowing and squeezed of the RCA critical ostial, stenosis at the ostium of the RCA and the middle of left anterior descending (LAD) coronary, intravascular ultrasonography (IVUS) showed LAD and RCA minimal lumen area were 3.9 mm2, 5.9 mm2;plaque burden 66%, 65% respectively. We inserted EXCEL 3.0 × 18 mm stent in LAD stenosis. No complaints and adverse events were noticed during a 1-year follow-up.

Urgent Off-Pump Revascularisation in ACS and Cardiogenic Shock with Anomalous Origin of Right Coronary Artery from Left Anterior Descending Artery: A Case Report

A 55-year-old female known case of essential hypertension and diabetes was brought to the Emergency Department in a drowsy state with cardiogenic shock. At presentation systolic BP was 70 mm Hg, pulse rate was 44/min and random blood sugar was 239 mg/dl. Troponin I was 8.07. Electrocardiography showed junctional rhythm and bradycardia. Ejection Fraction (LVEF) was 15% - 20%. Coronary angiography was done which revealed single vessel disease in LAD with anomalous origin of RCA from midsegment of LAD. Patient was immediately taken up for Off-pump Coronary Artery Bypass Grafting (OPCAB). Right saphenous vein graft was harvested and anastomosed proximally to aorta and distally to LAD on beating heart using Medtronic Octopus heart stabilizer and coronary shunts. Wound closed in layers after achieving complete hemostasis. Patient was taken off ventilatory support on first post-operative day and discharged in a stable condition on fourth post-operative day. Post-operative Echocardiography showed LVEF increased from 15% - 20% at time of admission to 38% and 52% after 3 months and 12 months of surgery respectively.

Percutaneous revascularization in a patient with anomalous origin of left main coronary artery

Anomalous origin of the coronary artery from opposite coronary sinus is infrequently observed during coronary angiography. Percutaneous coronary intervention (PCI) of anomalous coronary artery is technically difficult and challenging. It requires appropriate selection of guide catheters for adequate stability, coaxial alignment and backup support during the intervention. We hereby report a rare case of anomalous origin of left main coronary artery (LM) from the right coronary sinus, having a retro-aortic course to the left side before its bifurcation into left anterior descending (LAD) and circumflex artery. The 59-year-old man had successful PCI of atherosclerotic LAD lesions. A 64-slice Multi-Detector Computed Tomography (MDCT) performed at 4 years of follow-up demonstrated patency of coronary stents and also delineated the origin and course of the anomalous LM. The case illustrates the rarity of anomalous LM, and describes technical issues during PCI and the role of MDCT in coronary anomaly imaging.

Anomalous left coronary artery from pulmonary artery: Case series and brief review

Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 - 12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiome-galy with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.

Compression of Left Main Coronary Artery in Patients with Pulmonary Artery Aneurysm and Pulmonary Hypertension

Background: Pulmonary artery aneurysm (PAA) is an unusual finding and its association with left main coronary (LMCA) compression is even more infrequent. Cardiac CT evaluates of presence and size of PAA and the degree of LMCA compression. The aim of this study is to describe two cases of adults with compression of LMCA with PAA associated with PDA and pulmonary hypertension. Case presentation: The first case is a 27-year-old man with PAA (78 mm diameter) and LMCA compression of 70% between the aortic sinus and the PAA. He presented angina as a manifestation of the LMCA compression. During follow-up the patient died. The second case is a 28-year-old man with PAA (110 mm diameter) that compresses LMCA in 55%, he rejected surgical treatment, but he is in close follow-up with medical treatment. Conclusion: Cardiac computed tomography played an important role both in the diagnosis and identification of high-risk PAA patients.

Anomalous Origin and Course of Coronary Artery—Presentation of Three Cases

Anomalous origin of coronary arteries may be encountered coincidentally in the presence of unrelated pathology or when these are affected directly. This may directly be responsible for affecting the procedure or outcome. Various types of anomalies of origin,?as well as course of coronary arteries,?have been classified in the past. Here we report 3 cases of anomalous origin of coronary arteries in different scenarios. First case had anomalous coronary with bicuspid aortic valve with dilated ascending aorta for which Bentall’s procedure was done, while the second and third cases were anomalous coronaries with coronary artery disease for which coronary artery bypass grafting was done.

Nursing cooperation in corrective surgery of anomalous origin of the left coronary artery from the pulmonary artery in adult

Background Anomalous Origin of Coronary Artery （AOCA）, a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It＇ s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include： understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.

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

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

Occult anomalous origin of the left coronary artery from the pulmonary artery with atrial septal defect initially visualized by transthoracic echocardiography

Anomalous origin of left coronary artery from the pulmonary artery （ALCAPA） is a rare congenitalcardiac malformation. Here we report a case of ALCAPA with atrial septal defect （ASD） initially visualized by transthoracic echocardiography, subsequently confirmed by the coronary angiography.

Aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery:A ten-year single center experience

Background At present, creation of a dual coronary system with direct aortic implantation is the preferred management for anomalous origin of left coronary artery from the pulmonary artery （ALCAPA）. However, controversy still exists over the initial management of mitral regurgitation （MR） at the time of ALCAPA repair. Methods Between March 2005 and March 2015, 52 consecutive patients （20 males and 32 females） underwent direct aortic implantation of ALCAPA. Median age at surgery was 10 months （range, 2 to 193）. Mean weight was 9.7_＋7.8 kg. In order to facilitate the analysis, description of MR was given a numeric grade： 0 = none, 1 = trivial, 2 = mild, 2.5 = mild-moderate, 3 = moderate, 3.5 = moderate-severe, and 4 = severe. Results The LV shortening fraction （LVFS） demonstrated significant improvement between presentation and discharge （32.1±10.1% vs. 28.6±9.6%, P = 0.023）, and there was also significant improvement in the mean MR grade between presentation and discharge （2.40＋1.16 vs. 2.91＋ 1.19, P = 0.001）. There were 4 hospital deaths （7.7%）. The median follow-up was 21 months （range 1.5 to 111）, 3 patients （5.8%） were lost to follow-up, 1 patient required reoperation for mitral valve replacement. Significant improvement was seen in LVFS between discharge and late follow-up （38.0±6.0% vs. 32.1±10.1%, P = 0.001）, however, the degree of MR did not dem- onstrate further change （2.29_＋1.20 vs. 2.40＋1.16, P = 0.541）. Conclusion Creation of a dual coronary system with direct aortic implantation of the left coronary artery results in complete recovery of left ventricular function. Concomitant mitral valve （MV） repair for ALCAPA patients with moderate-severe and severe MR is helpful to early MV function recovery.

Early variation of left ventricular dimension and mitral regurgitation after correction for anomalous origin of the left coronary artery from the pulmonary artery

Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction （33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty）. Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% （2 in-hospital deaths）. Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, （P 〈 0.01） and 23.04 ±5.87 mm （P 〈 0.01） respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 （P 〈 0.05） in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 （P 〈 0.05）. Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.

Postoperative management of patients with repair of anomalous origin of the left coronary artery from the pulmonary arterya report of 30 cases

Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare congenital anomaly It demonstrated the combined effects of the absence of a normal coronary flow with a coronary steal and the profound ischemia that can produce left ventricular dysfunction and mitral regurgitation. We here introduce the postoperative management of patients with repair of anomalous ori artery from the pulmonary artery, with an emphasis on its outcome. Methods Reco gin rds of the left coronary of 31 patients with anomalous origin of the left coronary artery from the pulmonary artery receiving surgery from 1998 to 2010 were reviewed retrospectively, 10 of which were treated with the mitral valve surgically at the same time. The age of patients was 4 months to 16 years （m kilograms）, all of which were diagno edian, sed of anomalous origin of the left coronary artery from the pulmonary by echocardiography and cardiac catheterization. After surgery, electrocardiogram, echocardiography, arterial blood pressure, transcutaneous oxygen saturation and central venous pressure were monitored. Common postoperative complications in our group were analysed. And preoperative and postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter were obtained. Cardiopulmonary bypass time and mechanical ventilation time of postoperative patients with no pneumonia were compared with those with pneumonia. Binary logistic regression was applied for the analysis of the risk factors of postoperative pneumonia. Results Of 31 patients, 30 survived after surgery with early mortality of 3.23%. One patient died of severe low cardiac output syndrome. Mechanical ventilation time was 4 hours to 168 hours hours （mean, 39.68 ± 50.52 hours; median, 18 hours）. ICU stay was 16 hours to 425 hours （mean, 111.65 ± 127.03 hours; median, 44 hours）. In our group, common postoperative complications were myocardial ischemia（n = 12, 36.4%）, infection（n = 11, 33.3%） including pneumonia （n = 10, 30.3%）, postoperative tachyarrhythmia（n = 5, 15.2%）, low postoperative cardiac output（n = 2, 6.1%）, endocarditis（n = 1, 3.0%）. Compared with preop-erative data, postoperative data including area of mitral regurgitation, left ventricular systolic diameter and left ventricular distolic diameter decreased remarkably（P 〈 0.01）, with left ventricular ejection fraction significantly improved （P 〈 0.05）. Compared with mechanical ventilation time of patients with no postoperative pneumonia, time of those with pneumonia apparently prolonged（P 〈 0.01 ） while cardiopulmonary bypass time extended （P 〈 0.05）. Mechanical ventilation time was a risk factor of postoperative pneumonia （OR = 1.041, OR 95%CI = （1.010, 1.073）. Conclusions Proper therapy strategies according to the knowledge of the ALCAPA disease and the change of postoperative pathology and physiology play an important role in avoiding the postoperative complications and improving postoperative outcomes.

Secondary coronary artery ostial lesions:Three case reports

BACKGROUND Atherosclerosis is one of the main causes of coronary artery ostial lesions seen clinically.Secondary coronary artery ostial lesions are rare,and cases reported previously were associated with syphilitic vasculitis and aortic dissection.Here,we report three rare cases of secondary coronary ostial lesions.Due to their rareness,these lesions can easily be neglected,which may lead to misdiagnosis and missed diagnosis.CASE SUMMARY We present three patients with acute myocardial infarction and unstable angina caused by secondary coronary artery ostial lesions.In Case 1,coronary angiography(CAG)revealed 90%stenosis of the left main coronary ostium.Chest contrast computed tomography(CT)suggested thymic carcinoma invading the left main coronary ostium.Coronary artery bypass grafting and tumor resection were performed.In Case 2,echocardiography revealed a sinus of Valsalva aneurysm(SVA)-like dilatation.CAG showed a right coronary sinus giant aneurysm and complete obstruction of the right coronary artery(RCA)ostium.Aortic contrast CT confirmed these findings.The Bentall procedure was performed.In Case 3,CT CAG identified an anomalous origin of the right coronary artery(AORCA)from the left sinus of Valsalva coursing between the aorta and pulmonary trunk,causing severe RCA ostium stenosis by compression.Surgical correction of the AORCA was performed.CONCLUSION The cases reported here suggest that we should consider other causes of coronary ostial lesions other than atherosclerosis.

成人冠状动脉异常主动脉起源病例特点及经胸超声心动图漏诊原因分析

目的:分析成人冠状动脉异常主动脉起源(anomalous aortic origin of coronary artery,AAOCA)的病例特点,总结经胸超声心动图(transthoracic echocardiography,TTE)漏诊原因,为提高TTE诊断成人AAOCA的准确性提供参考。方法:选择2019年1月至2022年12月在某院经冠状动脉CT血管造影和/或有创冠状动脉造影确诊AAOCA的患者37例,统计患者临床症状、12导联心电图、心肌酶检查及TTE检查结果;将患者按照冠状动脉异常起源部位进行分型,分析TTE漏诊原因。采用卡方检验比较TTE漏诊差异情况。结果:37例患者中,无症状或轻症者共31例;12导联心电图检查、心肌酶检查、心腔大小变化、节段性室壁运动异常及左室收缩功能结果均以阴性者居多。以右冠状动脉起源于左冠状动脉窦(anomalous origin of the right coronary artery from left sinus,ARCA-L)患者最多,占比为59.45%(22/37);TTE 2次检查共确诊21例患者,均为冠状动脉异常起源于对侧冠状动脉窦(anomalous origin of coronary artery arising from the opposite sinus,ACAOS),其中ARCA-L 19例,TTE对ACAOS的检出率达87.5%;TTE检查13例起源于分支、高位开口的患者全部漏诊。TTE对ACAOS的检出率要显著高于冠状动脉异常起源于分支和起源于高位开口的检出率,差异有统计学意义(21/24 vs 0/13,P<0.001)。结论:成人AAOCA患者症状表现及相关检查结果以阴性居多、缺乏特异性;TTE对ACAOS的诊断检出率较高,但对冠状动脉异常起源于分支、高位开口的患者易漏诊。超声医师需进行多切面多角度联合扫查,正确应用彩色多普勒血流显像甄别假阴性,以降低漏诊率。

体外膜氧合成功救治左冠状动脉异常起源于肺动脉术后低心排血量综合征患儿1例

左冠状动脉异常起源于肺动脉(anomalous left coronary artery origin from pulmonary artery,ALCAPA)是一种发病率低、预后较差的先天性心脏畸形,常在婴儿期需要外科手术矫治。低心排血量综合征是指各种原因导致心排出量降低的一种病理异常表现,是心脏术后导致患者死亡的主要原因之一。体外膜氧合(extracorporeal membrane oxygenation,ECMO)是一种可以临时替代心肺功能的装置,在重症及急救领域应用广泛,但在ALCAPA术中及术后应用相关经验报道较少,本文分享一例ECMO成功救治ALCAPA术后低心排血量综合征的案例。

Three Cases of ALCAPA with Associated Anomalies

There are several potential issues that affect the treatment and diagnostic pattern of anomalous left coronary artery arising from the pulmonary artery. We report three cases of infants who presented with anomalous left coronary artery arising from the pulmonary artery with severe left ventricular dysfunction and severe mitral regurgitation along with associated anomalies. One patient had congenital lobar emphysema of the right midde lobe. Another patient had left main stem bronchus compression, collapse of basal segments of left lower lobe and panlobular emphysema in medial basal segment of right lower lobe. The third patient had cleft lip and palate. All patients underwent successful repair. The hemodynamic stability was compromised when the infant with congenital lobar emphysema had spontaneous pneumothorax after extubation and she needed an intercostal drainage. The infant with lung collapse had to be reintubated on the second day since she became hypoxic due to recollapse of the lung once the airway positive pressure was removed. She needed chest physiotherapy, vigorous endotracheal suctioning and inhaled bronchodilator therapy. The patient who had cleft palate succumbed to aspiration pneumonitis in the postoperative period. Follow-up of other two patients after three months showed very good improvement in left ventricular systolic function.