Anomalous Left Coronary Artery Connected to the Pulmonary Artery Associated With Other Cardiac Defects: A Difficult Joint Diagnosis

Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) can be associated rarely with other congenital heart defects. The preoperative joint diagnosis is challenging. From 1987 to 2012, a retrospective bicentric assessment of 12 patients with ALCAPA related to other cardiac defects focused on the associated heart defect, the moment of complete diagnosis related to surgery, and outcome. Coarctation was the most frequently associated heart defect (n = 5) followed by tetralogy of Fallot with or without pulmonary atresia (n = 3). The study group comprised one case of hypoplastic left heart syndrome, one right aortic arch, one congenital mitral malformation, and one infant with divided left atrium and anomalous pulmonary venous return. Only four patients had a complete diagnosis of both the cardiac defect and the coronary abnormality before surgery. In two cases, the coronary anomaly was discovered during surgery performed for another cardiac defect and treated at the same time. The diagnosis of the six remaining patients was determined after cardiac repair. Of the 12 patients, 7 (58 %) died after surgery. Half of these patients died within the first 30 days after repair. At this writing, the remaining patients are in good health after a median follow-up period of 5.4 years (range, 2.1–8.5 years). This study confirmed that ALCAPA associated with other cardiac defects often is misdiagnosed before surgery, mostly due to specific hemodynamics masking myocardial ischemia preoperatively. Survival was compromised due to the unrecognized diagnosis of an associated coronary abnormality but also because of midterm complications related to the other cardiac defects.     

Major coronary anomalies in childhood

Major coronary artery anomalies are extremely rare in childhood. We wanted to assess the historical and diagnostic features and the therapeutic options of three distinct types of coronary artery anomalies: abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary fistula and coronary stenosis. In a retrospective study, 33 children with these types of coronary artery disease were identified, 15 with ALCAPA, 12 with fistula and six with coronary stenosis. History, physical examination, ECG, X-ray, echocardiography, angiography, therapy and outcome were reviewed. ALCAPA showed distinct typical echocardiographic characteristics. Coronary artery fistula could be identified by a typical murmur and echocardiographic evidence of coronary dilatations. Coronary stenosis should be suspected by the clinical picture in a specific context. CONCLUSION: rare coronary artery anomalies can be accurately diagnosed in childhood. Timely therapy yields good prognosis.     

儿童先天性冠状动脉心腔瘘的诊断及外科治疗

目的：探讨先天性冠状动脉瘘的诊断及手术治疗方法。方法：全组22例术前均经心电图、X线、选择性冠状动脉造影、B超检查确诊，并施行外科手术治疗。结果：本组19例术前行选择性冠状动脉造影，17例行B超检查，对照符合率 94.2%。选择性冠状动脉造影与手术对照符合率 99.8%。21例术后恢复顺利，出院时心前区杂音消失。术后(19/21例)随访15月至23年，心功能I级。死亡1例，死于术后低心排综合征。结论：近年来随着超声心动图检查经验的积累及技术提高，冠状动脉瘘的诊断并不困难，但确诊目前仍依赖于选择性冠状动脉造影或核磁共振心脏扫描。手术难度不大，但应注意修补瘘口，避免残余分流，如冠状动脉瘘远端仍有血管分支时，应注意冠状动脉远端的血液供应。     

Increased Incidence of Coronary Artery Origin Anomalies Associated With Isolated Patent Ductus Arteriosus

Coronary artery anomalies may increase the risk of sudden death. Despite awareness of this association with certain congenital heart anomalies such as tetralogy of Fallot and transposition of the great arteries, it is thought to be an infrequent finding in cases of isolated patent ductus arteriosus (PDA). The authors report their experience with coronary anomalies in PDA patients. This study aimed to estimate the incidence of coronary artery anomalies in patients with PDA. The study reviewed 206 angiograms of PDA patients obtained between 1999 and 2011 to determine the origin of the coronary arteries. In 102 angiograms (49.5 %), the origin of the coronary arteries could be adequately visualized. An anomalous origin of coronary arteries was detected in 11 of the 102 patients (10.8 %). Seven of these patients had a single common coronary artery origin (6.8 %). One patient had an aberrant origin of the left coronary artery from the noncoronary sinus, and three patients had an aberrant origin of the right coronary artery: two from the left coronary sinus and one from the noncoronary sinus. These findings suggest that the incidence of coronary artery anomalies in association with an isolated PDA may be considerably higher than expected and previously reported. In view of the increased risk for sudden death with coronary anomalies, a reasonable approach is to determine the coronary artery origin and pathway after the diagnosis of an isolated PDA.     

Electrocardiogram of Anomalous Left Coronary Artery From the Pulmonary Artery in Infants

The electrocardiogram of an infant with anomalous left coronary artery from the pulmonary artery (ALCAPA, Bland-White-Garland syndrome) usually shows typical signs of an anterolateral myocardial infarction, manifested by abnormal Q waves in leads I, avL, V5, and V6, as well as by transient ST changes in these leads. Because 20–45 % of such patients do not show abnormal Q waves, the diagnosis should be strongly suspected if there is an abnormal R wave progression in the chest leads.     

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目的 探讨小儿左冠状动脉起源于肺动脉(ALCAPA)所致心脏扩大病例的误诊原因,总结诊断经验.方法 回顾性分析1996年7月至2009年1月广东省心血管病研究所22例小儿ALCAPA所致心脏扩大的误诊病例.均行心电图、X线胸片、超声心动图检查,8例行心脏CT检查,14例行心导管检查及造影,21例进行了外科手术治疗.结果 ≤1岁患儿10例中,6例误诊为心内膜弹力纤维增生痘,3例误诊为扩张型心肌病,1例误诊为先天性二尖瓣脱垂并关闭不全.>1岁患儿12例中,4例误诊为扩张型心肌病,3例误诊为心内膜弹力纤维增生症,2例误诊为先天性二尖瓣脱垂并关闭不全,1例误诊为右冠状动脉右心室瘘,1例误诊为川崎病,1例误诊为动脉导管未闭.结论 小儿ALCAPA所致心脏增大病例容易误诊为心内膜弹力纤维增生症、扩张型心肌病、先天性二尖瓣脱垂等疾病,左冠状动脉起源于肺动脉后方偏右的病例更易误诊.提高对ALCAPA的认识、超声或CT检查中仔细探查冠状动脉及其起源对避免误诊至关重要.     

左冠状动脉起源于肺动脉的术前诊断与治疗

目的 探讨左冠状动脉起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery,ALCAPA)的术前诊断,手术治疗和疗效.方法 对山西省儿童医院2010年6月至2014年3月收治的9例ALCAPA患儿的术前诊断及手术方式进行总结.结果 本组患儿共9例,男3例,女6例,年龄4个月～4岁6个月,平均(1.14±1.33)岁;体重5.6～18.0 kg,平均(8.06±3.99)kg.术前行心脏彩超、心脏增强CT检查,明确诊断,其中有1例患儿行心脏造影检查.9例患儿中合并二尖瓣轻度反流2例,二尖瓣轻-中度反流5例,中-重度反流2例.心电图表现:9例患儿在导联Ⅰ、AVL及V4-6有不同程度的异常Q波,ST段改变或T波倒置.胸部X线检查示心影明显增大,心胸比例0.56～0.73,平均0.61 ±0.05,左心缘饱满隆起,向左下方突起,心脏彩超示左冠状动脉起源于肺动脉,左心室明显扩大,心肌收缩力减弱,射血分数低于50％,8例患儿左心室舒张末径均＞35 mm,1例患儿合并心尖部室壁瘤.1例患儿行心脏彩超及CT检查后怀疑ALCAPA,后经造影检查明确诊断.6例患儿术前行放射性核素心肌显像检查,均伴有不同程度的心尖、前壁、侧壁心肌梗死,部分心肌存活.本组患儿均在全身麻醉体外循环下行左冠状动脉移植术,7例患儿加做二尖瓣成形术.全组患儿早期死亡1例,死亡原因为严重低心排综合征,余均治愈出院.其中,围术期合并低心排综合征2例,肺炎3例,室上性心动过速1例.术后随访3个月～2年,超声心动图检查提示冠状动脉血流通畅,射血分数较术前提高,二尖瓣反流减轻.患儿生长发育良好,无心力衰竭表现,心功能恢复良好.结论 超声心动图对ALCAPA的诊断有重要价值,心脏增强CT及造影检查可确诊,术前核素心肌显像对存活心肌的判定、手术及预后有重要意义.冠状动脉移植术是最理想的手术方法.     

左冠状动脉起源于肺动脉的临床分析

目的：左冠状动脉起源于肺动脉（anomalous origin of the left coronary artery from the pulmomary artery, ALCAPA)是临床上一种少见的先天性冠状动脉异常性疾病，目前国内报道的资料不多。该研究对其临床特点与治疗进行探讨。方法：对10例诊断为左冠状动脉起源于肺动脉患儿的临床表现、心电图特征、心脏超声、心导管资料、手术治疗及转归进行分析。结果：患儿都有不同程度的多汗、烦躁、气促、拒食等心绞痛及心功能不全的表现；心电图、彩色超声心动图及心导管检查有较特征性的改变；4例患儿行肺动脉内隧道术，4例行直接左冠状动脉移植术，2例行左冠状动脉重建术。术后8例症状改善，2例死亡。结论：掌握ALCAPA的病变特点和相关的诊断线索，辅以彩色超声心动图和心导管检查，可提高ALCAPA的检出率，并早期手术治疗。［中国当代儿科杂志，2007，9（1）：25-27］     

Single Coronary Artery Anomaly: Classification and Evaluation Using Multidetector Computed Tomography and Magnetic Resonance Angiography

The aim of this study was to use multidetector computed tomography (MDCT) and magnetic resonance (MRA) angiography to illustrate the classification and clinical characteristics of single coronary artery anomaly (SCAA). Retrospective evaluation of 22 adult and pediatric patients with SCAA by way of a medical archiving system was performed between June 2001 and August 2012. Imaging modalities used for coronary artery evaluation included MRA and MDCT angiography. Of the 22 patients, the majority (n = 8; 36 %) showed an interarterial course, the subtype having the worst prognosis. The retroaortic course (n = 3; 14 %) and course anterior to the pulmonary trunk (n = 3; 14 %) were the next most frequent patterns. Additional types (n = 8; 36 %) included the following: L-I, R-III, septal, and combined. Four patients (18 %) showed atherosclerotic involvement. SCAA anomaly was diagnosed as an incidental finding in the majority of patients evaluated initially for cardiovascular diseases (n = 19; 86 %). Two patients (9 %) required surgical interventions solely for their anomaly. Nine patients (41 %) were found to have coexisting congenital heart disease. Although conventional catheter angiography is responsible for the current classification of SCAA, advanced imaging modalities are useful in the evaluation of morphological and clinical characteristics of single coronary arteries.     

A Unique Aortic Arterial Meshwork With a Major Aortopulmonary Collateral Artery, a Major Arteriovenous Collateral Artery, and A Coronary Arteriovenous Fistula in a Young Man With a Bicuspid Aortic Valve

This report describes a case of an asymptomatic 18-year-old boy with a grade 1/6 ejection systolic murmur in the aortic area referred for an echocardiographic study to rule out a congenital heart disease. The patient had situs solitus, levocardia, bicuspid aortic valve, dilation of the proximal portions of the right coronary artery, and arteriovenous coronary fistula of the anomalous coronary branch originating from the anterior descending coronary artery into the main pulmonary artery. In addition he had a unique aortic arterial meshwork constituted by arteries originating from the inferior and posterior aspect of the distal portion of the transverse aortic arch, intersegmentary arteries originating from the lateral and anterior aspect of the proximal portion of the descending aorta, and the true bronchial artery with a major arteriovenous collateral artery draining into the superior vena cava and the superior division of the left lower pulmonary vein as well as a major aortopulmonary collateral artery anastomosed with a bronchial artery and draining into the posterior aspect of the right pulmonary artery. The use of transthoracic two-dimensional color flow Doppler echocardiography, magnetic resonance angiography and multislice computed tomography to establish a detailed morphologic analysis and the presence of associated anomalies in patients with a bicuspid aortic valve are discussed.     

Non-invasive diagnosis and management of coronary arteriovenous fistula. A case report

Coronary arteriovenous fistulas are rare anomalies resulting in abnormal communication between the coronary artery and any chamber of the heart. An asymptomatic patient was referred for evaluation of her murmur. Two-dimensional and color Doppler echocardiographic evaluation revealed an enlarged left main coronary artery. A retrograde, eccentric small jet was found within the right ventricular outflow tract at the pulmonary artery valvular level allowing us to detect the entrance site of the fistula. The diagnosis was confirmed by cardiac catheterization and angiocardiography. Although our case was asymptomatic, the decision to perform cardiac surgery was made because of the aneurysmatic appearance of the left coronary artery. In our opinion, visualization of coronary arteries by two-dimensional echocardiography, together with additional information obtained from the Doppler examination, provides an excellent technique for the noninvasive diagnosis of coronary artery fistula.     

Transcatheter Closure of Abnormal Vessels and Arteriovenous Fistulas With the Amplatzer Vascular Plug 4 in Patients With Congenital Heart Disease

Although vascular plugs allow the interventional closure of medium-sized to large abnormal vessels, their application is limited by the need for long sheaths or large guiding catheters. The authors report their experience with the new Amplatzer vascular plug 4 (AVP 4), a self-expanding spindle-shaped occluder made of Nitinol wire mash, which can be placed through 4-Fr catheters with an internal diameter of 0.038 in. or larger. From October 2009 until June 2012, 14 AVP 4 devices were deployed in 12 patients (ages, 0.3–48.8 years). Nine patients had venovenous or arteriovenous collaterals in functional univentricular hearts. One patient had pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries, and one patient had a pulmonary arteriovenous fistula. One child had a large coronary artery fistula to the right atrium. The authors used AVP 4 devices with diameters of 4–8 mm. In all the patients, the AVP 4 was implanted successfully. No occluder dislocations and no complications related to the procedure occurred. Complete vessel occlusion was achieved in seven cases. In seven additional cases, a residual shunt was present at the end of the procedure while the patients were still fully heparinized. In 2 of 14 vessels, the decision was made to place additional devices to abolish residual shunting. According to the authors’ experience, the AVP 4 allows safe and effective occlusion of medium-size and large abnormal vessels. It is also well suited for tortuous high-flow vessels such as coronary or pulmonary arteriovenous fistulas. In case of a suboptimal position, it is possible to reposition the occluder with ease. Further studies are needed to determine whether initial residual shunting in heparinized patients disappears during follow-up care. The AVP 4 represents a valuable new device for the interventional treatment of complex congenital vessel malformations.     

Isolierte kongenitale Koronaranomalien

Background

Isolated coronary anomalies are rare congenital malformations. The anomalous origin of the coronary artery from the pulmonary artery (ALCAPA, ARCAPA), the anomalous origination of the coronary ostium from the opposite, facing “coronary” sinus, coronary fistulas, and coronary aneurysms have to be mentioned.

Diagnosis

The symptoms vary from life-threatening events (e.g., myocardial infarction, cardiac syncope and ventricular dysrhythmia) to atrial or ventricular volume overload and asymptomatic courses. Echocardiography is used as a screening method; noninvasive modalities such as magnetic resonance imaging and computed tomography are additionally used for diagnosis, but coronary angiography is the gold standard to depict the morphology discussed in the individual case.

Therapy

The indication for conservative, interventional, or operative treatment must be decided for each patient individually.     

Diagnosis and Diagnostic Modalities in Pediatric Patients with Elevated Troponin

Care of adults with coronary artery disease focuses on troponins to rapidly move patients to catheterization. Troponins are increasingly drawn in children, but emergent catheterization may not be indicted. We sought to establish etiologies of troponin elevation and ascertain the yield of diagnostic tests, in this population. Retrospective review of patients from January 1, 2002, to December 31, 2011, who had any elevated troponin during the study period. Patients were excluded for recent cardiac surgery, “significant” congenital heart disease, if they were neonates in the NICU or were on ECMO. Twenty-four patients made up our study group: 17/24 (71 %) had myocarditis or cardiomyopathy. Three had coronary-related diagnoses: 1 ALCAPA and 2 Kawasaki syndrome. The most useful testing for making or confirming the diagnoses included ECG, CXR and ECHO. Fourteen had right heart catheterization which was useful in 10/14. Nine had MRI which was useful in 7/9 (all five cases of suspected myocarditis). Left heart catheterization was completed in 10/24 cases, but in no case made or changed the diagnosis. This study confirms that children with elevated troponins differ from adults. The most common cause is myocarditis or cardiomyopathy, whereas coronary-related ischemia is rare. Diagnosis with ECG, CXR and ECHO is typically adequate. Focused use of right heart catheterization and MRI may be useful. In pediatric patients with elevated troponins, left heart catheterization and coronary angiography should be reserved for a highly selective group, and adult “door-to-balloon time” protocols should not be applied routinely.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: 15 Years Experience

This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdisgnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.     

Association of Common Origin of the Carotid Arteries with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

Common origin of the carotid arteries (COCA) is a common pattern of aortic arch vessels and is the single most common cause of tracheobronchial compression by a congenital cardiovascular anomaly. By no means all affected patients are symptomatic. Symptoms may range from recurrent pulmonary infections and “noisy respiration” to stridor and apneic spells. In our study of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) we found a highly significant association of COCA with ALCAPA (85%), although no patient with ALCAPA in this study had evidence of tracheal stenosis documented in the hospital chart. As COCA is easily correctable, we suggest consideration of COCA during evaluation and surgery of patients with ALCAPA so that, if the patient also has symptoms possibly related to COCA, the artery can be suspended from the posterior wall of the sternum.     

Resolution of pathologic Q wave,left ventricular dysfunction and mitral regurgitation after dual coronary repair of the anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect that usually presents as dilated cardiomyopathy in infancy. From 1984 to 2005, 13 (five males and eight females, 0.13%) out of 9,950 patients with congenital heart disease were identified as ALCAPA at our institute. Corrective surgery was performed at a median age of 9 months (range: 2 months to 5 years). Eleven patients underwent direct reimplantation of the left coronary artery (LCA) to the aorta, while two received extrapulmonary baffling. The overall survival rate was 92%. Only one patient died 5 months after reimplantation of the LCA due to acute myocardial ischaemia. Left ventricular ejection fraction (LVEF) was significantly lower in the eight (62%) patients operated during infancy than in those operated beyond 5 months (median: 35% vs. 75%). Left ventricle function was normalised in 11 patients (85%). Among the eight patients without concomitant mitral annuloplasty, mitral regurgitation (MR) improved to a mild or trivial degree in six patients and remained at the pre-operative level in two patients. Pathologic Q wave was noted in 11 patients, which eventually regressed in all except two cases. The median interval of recovery was 16 days, 6 months and 24 months for MR, LVEF and electrocardiogram (ECG) changes, respectively. In conclusion, ALCAPA is also a rare disease in Asian countries, such as Taiwan. The subsequent recovery of MR, left ventricular (LV) function and even pathologic Q wave can be expected after dual coronary repair, regardless of the age at repair.     

Pulmonary arteriovenous fistula in the newborn: a case report of Rendu-Osler-Weber syndrome and a review of the literature.

In most instances, congenital arteriovenous fistula is only one manifestation of a more widespread abnormality; 60% of patients also have hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber syndrome). Among those with congenital pulmonary arteriovenous fistula, the diagnosis is made during infancy in only 15% of patients. We present a case of pulmonary arteriovenous fistula in a newborn and review the literature. This rare condition of newborns can be treated with different surgical procedures. Only 17 cases of newborn pulmonary arteriovenous fistula/have been reported, and only two of those had associated Rendu-Osler-Weber syndrome. The results of surgical procedures were good in most of these cases. We treated our case with lobectomy successfully.     

Congenital H-type tracheoesophageal fistula: a national multicenter study

Background

Congenital H-type tracheoesophageal fistula (TEF) is very rare and represents <5 % of all congenital tracheoesophageal malformations. This is a national, multicenter review of our experience with isolated H-type TEF outlining clinical presentation, methods of diagnosis, associated anomalies, treatment and outcome

Patients and methods

The medical records of all patients with the diagnosis of congenital H-type TEF treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, associated anomalies, method of diagnosis, treatment and outcome.

Results

During the study period (January 1998–December 2013), 435 infants and children with the diagnosis of esophageal atresia with or without TEF were treated. Among these, 23 (5.3 %) had isolated TEF. There were 11 males and 12 females. Their age at presentation ranged from 5 days to 3 years and 7 months but the majority (90 %) were diagnosed during their first year of life. Their clinical presentation included: chocking and coughing during feeds in 12 (52.2 %), recurrent chest infection in 16 (69.6 %) and cyanosis in 10 (43.5 %). One presented with abdominal distension also. The diagnosis was made using esophagogram. In 11 (47.8 %), a single study confirmed the diagnosis, 8 (34.8 %) required two studies while 4 (17.4 %) required three studies. Nineteen (82.6 %) had preoperative bronchoscopy and in 13 (56.5 %), a catheter was used to cannulate the fistula. All were operated through a right cervical incision except one who underwent thoracoscopic ligation and division of the fistula. In one, the fistula was only transfixed and tied without being divided. This patient developed a recurrent fistula. Two patients developed postoperative stridor secondary to recurrent laryngeal nerve palsy. In both of them, there was complete recovery.

Conclusions

H-type TEF is very rare and commonly presents with recurrent chest infection, chocking and coughing during feeds and cyanosis. Physicians caring for these patients should be aware of this and a high index of suspicion is of paramount importance to avoid delay in diagnosis with its associated morbidity. A contrast esophagogram is valuable in confirming the diagnosis. The study however may need to be repeated. Preoperative bronchoscopy is valuable to localize and cannulate the fistula for easier access during surgery. Surgical repair is the treatment of choice and this should be performed through a right cervical incision or thoracotomy for low fistulae. Thoracoscopic ligation and division of a low H-type fistula is an alternative and less invasive approach when compared to thoracotomy.