Treatment of adult congenital anal atresia with rectovestibular fistula:A rare case report

BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed.Diagnosis was made based on the patient's clinical symptoms,signs,imaging showing the fistula,X-ray and magnetic resonance imaging results.The preoperative examination was improved.Anorectoplasty was performed.The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.CONCLUSION Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.

Current characteristics of congenital coronary artery fistulas in adults:A decade of global experience

AIM:To describe the characteristics of coronary artery fistulas(CAFs) in adults,including donor vessels and whether termination was cameral or vascular. METHODS:A PubMed search was performed for articles between 2000 and 2010 to describe the current characteristics of congenital CAFs in adults.A group of 304 adults was collected.Clinical data,presentations,diagnostic modalities,angiographic fistula findings and treatment strategies were gathered and analyzed.With regard to CAF origin,the subjects were tabulated into unilateral,bilateral or multilateral fistulas and compared.The group was stratified into two major subsets according to the mode of termination;coronary-cameral fistulas(CCFs) and coronary-vascular fistulas(CVFs) . A comparison was made between the two subsets. Fistula-related major complications[aneurysm formation,infective endocarditis(IE) ,myocardial infarction(MI) ,rupture,pericardial effusion(PE) and tamponade] were described.Coronary artery-ventricular multiple micro-fistulas and acquired CAFs were excluded as well as anomalous origin of the coronary arteries from the pulmonary artery(PA) . RESULTS:A total of 304 adult subjects(47%male) with congenital CAFs were included.The mean age was51.4 years(range,18-86 years) ,with 20%older than 65 years of age.Dyspnea(31%) ,chest pain(23%) and angina pectoris(21%) were the prevalent clinical presentations.Continuous cardiac murmur was heard in 82%of the subjects.Of the applied diagnostic modalities,chest X-ray showed an abnormal shadow in 4%of the subjects.The cornerstone in establishing the diagnosis was echocardiography(68%) ,and conventional contrast coronary angiography(97%) .However,multislice detector computed tomography was performed in 16%.The unilateral fistula originated from the left in 69%and from the right coronary artery in 31%of the subjects.Most patients(80%) had unilateral fistulas,18%presented with bilateral fistulas and 2%with multilateral fistulas.Termination into the PA was reported in unilateral(44%) ,bilateral(73%) and multilateral(75%) fistulas.Fistulas with multiple origins(bilateral and multilateral) terminated more frequently into the PA(29%) than into other sites(10.6%)(P=0.000) . Aneurysmal formation was found in 14%of all subjects.Spontaneous rupture,PE and tamponade were reported in 2%of all subjects.In CCFs,the mean age was 46.2 years whereas in CVFs mean age was 55.6 years(P=0.003) .IE(4%) was exclusively associated with CCFs,while MI(2%) was only found in subjects with CVFs.Surgical ligation was frequently chosen for unilateral(57%) ,bilateral(51%) and multilateral fistulas(66%) ,but percutaneous therapeutic embolization(PTE) was increasingly reported(23%,17%and 17%,respectively) . CONCLUSION:Congenital CAFs are currently detected in elderly patients.Bilateral fistulas are more frequently reported and PTE is more frequently applied as a therapeutic strategy in adults.

Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography

Tracheoesophageal fistula （TEF） or bronchoesophageal fistula may be congenital, inflammatory, neoplastic, or secondary to trauma. Congenital TEF or bronchoesophageal fistula is usually associated with esophageal atresia and is readily diagnosed in infancy. But if it is not associated with esophageal atresia, it may persist until adulthood. Some theories have been proposed to explain this delay in diagnosis. We present a case of a 70-year-old man with congenital TEF. The TEF was successfully diagnosed by multidetector-row CT esophagography.

Congenital solitary coronary artery fistulas characterized by their drainage sites

Last centuries have witnessed tremendous sophistication and progress in the detection,diagnosis and treatment of coronary artery fistulas(CAFs).In many countries,CAFs were reported to be visualized and treated using several imaging techniques and different management strategies.Reports from nearly all continents of the globe have contributed to the description of CAFs,not only in Asia and Europe but also throughout North and Latin America.However,these reports have to be cautiously analyzed as many of them were published as a case report and careful interpretation is warranted due to possible publication bias.A literature search was performed using PubMed search interface to select papers dealing with congenital CAFs in adult population between 2000-2009.A total of 233 subjects were collected,and analysed according to their drainage site and treatment modality.They were divided into two subgroups:percutaneous transluminal embolization group(PTE group,n=122)and surgical ligation group(SL group,n=111).In the SL group,atherosclerotic coronary artery disease(19%)and associated congenital lesions(23%)were more prevalent compared with the PTE group(9%and 8%),respectively.Infective endocarditis was more frequently seen in the SL group besides syncope,congestive heart failure and hemopericardium.In both groups multimodality diagnostic workup composed of several non-invasive and invasive imaging techniques for fistula visualization were performed and drainage sites into the different cardiac chambers and intrathoracic great vessels were similarly distributed in the two groups.

Congenital bronchobiliary fistula: A case report and review of the literature

BACKGROUND Congenital bronchobiliary fistula is a rare developmental abnormality with an abnormal fistula between the respiratory system and biliary tract. The aim of this report is to analyze and summarize the clinical features and experience of diagnosing and treating congenital bronchobiliary fistula(CBBF) occurring in the neonatal period.CASE SUMMARY The onset of symptoms was 3 d after birth in our patient with progressive cyanosis and respiratory distress, and a large amount of green fluid was noticed in her respiratory secretion. We performed computed tomography(CT),fiberoptic bronchoscopy, and cholangiography to make a diagnosis, as well as fistulography with a bronchoscope for the first time. These examinations provided us with valuable images to make a correct diagnosis. The fistula was dissected and removed with excellent results. Surgical removal of the fistula was successful, and the baby recovered well and was discharged. She has been followed for 4 mo without any signs of discomfort.CONCLUSION The main symptom of CBBF is bile-like sputum. CT, bronchoscopy,fistulography, and intraoperative cholangiography can provide important evidence for diagnosis. Surgical resection of the fistula is the first choice of treatment.

A large congenital and solitary intrahepatic arterioportal fistula in an old woman

Arterioportal fistula(APF)is a rare cause of portal hypertension and may lead to death.APF can be congenital,post-traumatic,iatrogenic(transhepatic intervention or biopsy)or related to ruptured hepatic artery aneurysms.Congenital APF is a rare condition even in children.In this case report,we describe a 73-year-old woman diagnosed as APF by ultrasonography,computed tomography,and hepatic artery selective arteriography.The fistula was embolized twice but failed,and she still suffered from alimentary tract hemorrhage.Then,selective arteriography of the hepatic artery was performed again and venae coronaria ventriculi and short gastric vein were embolized.During the 2-year follow-up,the patient remained asymptomatic.We therefore argue that embolization of venae coronaria ventriculi and short gastric vein may be an effective treatment modality for intrahepatic APF with severe upper gastrointestinal bleeding.

Incidental congenital coronary artery vascular fistulas in adults:Evaluation with adenosine-13N-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas(CAFs) using adenosine stress ^(13)N-ammonia positron emission tomography computed tomography(PET-CT).METHODS Congenital CAFs were incidentally detected during coronary angiography(CAG) procedures in 11 adult patients(six males and five females) with a mean age of 64.3 years(range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas(origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress ^(13)N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery(n = 8), right coronary artery(n = 2) and circumflex(n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery(n = 11) or coronary sinus(n = 1). The CAG delineated the characteristics of the fistula(origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas(8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components(23/36, 64%). Adenosine pharmacologic stress ^(13)N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction.CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.

Congenital bronchoesophageal fistula in an adult: A case report

Bronchoesophageal fistulas are usually diagnosed in the neonatal period. As such, the condition is rare in adults. We present a case of a congenital bronchoesophageal fistula in a 62-year-old man with the complaint of severe bouts of cough and choking after swallowing liquid. His workup included a barium esophagogram that revealed a fistula between the esophagus and a right lower lobe bronchus. The diagnosis should be considered in certain individuals with suggestive symptomatology and unexplained respiratory pathology. The fistula was divided and resected, The patient had an uneventful recovery.

Congenital bronchoesophageal fistula in adults

AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fi stulae in adults. METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded. RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively.The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery. CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Congenital Coronary Artery Fistula in Children:A Review of 28 Cases with Clinical and Imaging Outcomes

Background:Congenital coronary artery fistula(CCAF)is a rare anomaly.Treatment strategies tend to close the defect with a symptomatic and significant shunt,primarily based on expert consensus and case series.Results for long-term follow-up in children are limited Methods:We conducted a retrospective study to assess clinical and imaging outcomes of children with CCAF at Siriraj Hospital,Thailand during 2000–2020.Patients with single ventricle were excluded.Treatment strategies[surgical closure(SC),and percutaneous closure(PC)]were classified and the clinical outcomes at the follow-up in 2021,including coronary thrombosis,myocardial ischemia,and the results of cardiovascular imaging were reviewed.Results:Twenty-eight children with CCAF were included in the study.The median age at diagnosis was 2.5 years(2 days–18 years).Presenting symptoms were audible murmur(82%)and heart failure(35%).Most of fistulae arose from the right coronary artery(12/28)and exited at the right atrium(11/28).In recent visits(0.5–14 years follow-up),six patients with asymptomatic small CCAF were managed by watchful follow-up without complications.PC was primarily treated in 11 children:7 underwent successful procedures;1 had a residual shunt and required re-intervention;1 had ischemic symptoms immediately after the procedure with left coronary occlusion that required device removal plus SC and 2 were technically unable to place the device,requiring SC.Four patients were waiting for interventions(1 PC and 3 SC).Cardiovascular imaging surveillance that followed closure demonstrated asymptomatic thrombus formation in three patients(1 PC and 2 SC).No mortality presented.Conclusion:CCAF with significant shunt is indicated to close either SC or PC.Ischemic events are rare but have been reported after closure.In addition,thrombus formation should be watched for post-intervention.Surveillance with cardiovascular imaging is recommended after defect closure(ideally 1–5 years post closure),or at interval follow-ups in patients with symptoms to evaluate possible recanalization,thrombus,or ischemia.Life-long clinical and echocardiographic follow-up is warranted.Watchful follow-up is acceptable for hemodynamically insignificant fistula without complication in the series.

Surgical Correction of Coronary Artery Ectasia Combining Congenital Coronary Artery Fistula

Background:Coronary artery ectasia(CAE)complicated with concomitant congenital coronary artery fistula(CCAF)is rare.This study characterizes the clinical characteristics of CAE combining CCAF,and reports a single-institution experience with surgical correction of CAE combining CCAF.Methods:A total of 24 symptomatic patients(8 males,median 52.5 years old)who underwent surgical correction of CAE combining CCAF in this center were reviewed.Based on the size of ectatic segment,the CAE were classified as a giant CAE(>20 mm,n=14)and a non-giant CAE(≤20 mm,n=10).Individualized surgical approaches were chosen.The patients were followed up for a median of 3.8 years.Results:The overwhelming majority of CAEs were solitary,and only 4.2%of CAEs were associated with multiple lesions.CAEs were predominantly located in the right coronary artery with predilection to women more than to men(2:1).95.8%of patients with the CCAF had single fistula defect.The right atrium was the most frequent drainage site(33.3%)followed by the left ventricle(25.0%).Surgical mortality was 4.2%.All 22 follow-up patients survived with recovery from symptoms and New York Heart Association(NYHA)functional class I-II.In 10 patients with non-giant CAEs undergoing closure of fistula alone,favorable in-hospital outcomes were recorded,but residual fistula(one patient)and acute inferior wall myocardial infarction related to intracoronary thrombosis(one patient)were observed at follow-up.In 11 patients with giant CAEs undergoing aneurysm resection plus distal bypass grafting at the time of closure of fistula,favorable in-hospital outcomes and encouraging midterm results were recorded.Additionally,in 3 patients with giant CAEs undergoing closure of fistula plus aneurysmal plication,adverse events occurred,including surgical death related to rupture of the ectatic segment(one patient),perioperative myocardial infarction caused by acute thromboembolism(one patient),nonfatal inferior wall myocardial infarction related to intracoronary thrombosis(one patient)at follow-up.Conclusion:Individualized surgical approaches based on the size and the location of ectatic coronary artery as well as fistula should be offered to symptomatic patients with CAE combining CCAF.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula

Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate diagnostic and treatment strategies for this rare condition.Methods:We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.Results:Among 386 patients with EA/TEF,14(3.6%)had type D EA/TEF.Only two patients were diagnosed with proximal TEF preoperatively.Seven patients were diagnosed intraoperatively.Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy.During the neonatal period,seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy.Due to missed diagnosis and other reasons,the other 7 patients underwent two-stage surgery for repair of the proximal TEF,including cervical incision and thoracoscopy.Ten of the 14 patients experienced postoperative complications including anastomotic leakage,pneumothorax,esophageal stricture,and recurrence.Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak(4/7).In contrast,only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation:Type D EA/TEF is a rare condition,and proximal TEFs are easily missed.Bronchoscopy may aim to diagnose and determine the correct surgical approach.A cervical approach may be more suitable for repairing the proximal TEF.

Application of MPVR and TL-VR with 64-row MDCT in neonates with congenital EA and distal TEF

AIM:To assess the application of multiple planar volume reconstruction(MPVR) and three-dimensional (3D) transparency lung volume rendering(TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula(TEF).METHODS:Twenty neonates(17 boys,3 girls) with EA and distal TEF at a mean age of 4.6 d(range 1-16 d) were enrolled in this study.A helical scan of 64-row MDCT was performed at the 64 mm×0.625 mm collimation.EA and TEF were reconstructed with MPVR and TL-VR,respectively.Initial diagnosis of EA was made by chest radiography showing the inserted catheter in the proximal blind-ended esophageal pouch.Manifestations of MDCT images were compared with the findings at surgery.RESULTS:MDCT showed the proximal and distal esophageal pouches in 20 cases.No significant difference was observed in gaps between the proximal and distal esophageal pouches detected by MPVR and TLVR.The lengths of gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR correlated well with the findings at surgery(R=0.87,P<0.001).The images of MPVR revealed the orifice of TEF in 13 cases,while TL-VR images showed the orifice of TEF in 4 cases.CONCLUSION:EA and distal TEF can be reconstructed using MPVR and TL-VR of 64-row MDCT,which is a noninvasive technique to demonstrate the distal esophageal pouches and inter-pouch distance in neonates with EA and distal TEF.

Congenital coronary artery flstnlae in adults: diagnosis and surgical treatment

Objective To summarize the clinical characteristics, diagnosis and surgical treatment results of congenital coronary artery fistulas (CAF) in adults. Methods Fourteen patients (8 men,6 women) ,aged from 18 to 60 years with a mean of 32 ±12 years, underwent surgical correction of CAF between March 1985 and April 2002. Eleven of the 14 patients (78. 57% ) were symptomatic. The diagnosis of CAF was made by echocardiography or angiocardioraphy preoperatiely. The fistulae originated from the right, left and double coronary arteries in 10 (71%),3(21%) and 1(7%) patient (s),respectively. The fistulae drained into the right ventricle (8 patients), left ventricle (4),right atrium (1) and pulmonary artery (1), respectively. The diameter of fistulae ranged from 0.30 tol.80 cm with a mean of (1.16±0.49)cm.There were 6 CAF patients associated with coronary artery aneurysms and 4 CAF patients with other coexisting cardiac defects. The distal fistulae were closed in 10 patients with cardiopulmonary bypass (CPB)

Congenital heart disease

950283 Surgical treatment of congenital coronaryartery fistula.CAO Qingheng(曹庆亨),et al.DeptCardiovasc Surg,Shanghai Chest Hosp,Shanghai,200030.Shanghai Med J 1995;18(1):10-12.From October 1957 through December 1990,twenty-five patients with congenital coronary artery fistula(CCAF),including 3 cases complicated with giantcoronary artery aneurysms,underwent surgical repair.The ages ranged from 4 to 47 years (mean 19.8years).CCAF originated from the right coronaryartery in 17 cases (68.0%) and terminated into RA,RV,pulmonary artery (PA) or LV,in 8 cases (32.

Circumflex Coronary Artery Fistulae Draining into Right Atrium: A Case Report

Coronary artery fistula (CAF) is an anomaly making a coronary artery communicate with a heart cavity or a great vessel, thus bypassing the myocardial capillary bed. CAF is frequently diagnosed as an incidental finding. Herein, we present the case of a 4-year-old boy. He was referred for a pediatric cardiology assessment due to a continuous murmur at the middle sternal border. Echocardiogram showed dilated left coronary artery and an abnormal diastolic flow in the right atrium. The right-sided chambers were slightly enlarged, but ventricular contractility was normal. CAF was suspected. Angiography and CT confirmed the diagnosis of coronary fistula from the circumflex coronary artery to the right atrium. Successful transcatheter closure with an Amplatzer PiccoloOccluder was performed with complete occlusion.

儿童梨状窝瘘合并瘘管内异物致颈部反复感染1例

1临床资料患儿,女,10岁,因左颈部反复红肿8年就诊于河北省儿童医院。患儿8年前以“颈部脓肿”于当地行颈部脓肿切开引流术,术后愈合良好。2年前再次因颈部红肿于外院行颈部脓肿切开引流术,术后颈部切口一直未愈。2021-07-29就诊我科,行喉镜及颈部CT检查考虑梨状窝瘘不除外,门诊以“梨状窝瘘继发感染”收治入院。查体:左侧胸锁乳突肌前缘平甲状软骨下缘可见一约0.3 cm×0.3 cm大小瘘口,瘘口内可见肉芽组织增生,瘘口周围瘢痕增生。辅助检查:颈部超声(图1A)、颈部瘘管造影及MRI检查,均提示梨状窝瘘继发感染不除外。

超声心动图对婴幼儿先天性冠状动脉瘘的应用价值

目的探讨超声心动图对先天性冠状动脉瘘(CAF)的应用价值。方法选择2015年3月至2022年7月在深圳市儿童医院收治的19例先天性CAF患儿,其中男性7例,女性12例;年龄1天~3岁1个月,平均年龄10.56个月;病程0.5~37.3个月,平均病程3.89个月;就诊原因,1例因体检发现,1例因生长缓慢就诊,1例因胸痛就诊,9例因发现心脏杂音而就诊,3例因气促而就诊,2例因胎儿期超声检出CAF而就诊,2例因既往检查发现先天性心脏病(CHD)而就诊。全部患儿行超声心动图检查,观察左右心房室大小、各瓣膜启闭情况和冠状动脉起源、内径及走行、瘘口位置、瘘口大小及术后残余瘘,比较手术前后冠状动脉和心腔的变化。观察手术前后的超声心动图结果,并与手术结果进行对比分析。结果19例先天性CAF患儿手术前后均行超声心动图检查。术前17例患儿超声心动图及手术均发现位置及数目相同的病变,1例术前超声心动图漏诊CAF,1例术前超声心动图显示1个瘘口术中发现2个瘘口。术前超声诊断准确率达89.4%。19例中右CAF 14例(73.7%),左CAF 5例(26.3%)。19例中合并房间隔缺损6例,室间隔缺损3例,法洛四联症1例。冠状动脉不同程度增宽迂曲,内径为2.4~16.0 mm,瘘口直径为1.8~5.0 mm。术后3例超声心动图显示瘘口有残余瘘,8例受累心腔大小恢复正常,8例受累扩张的冠状动脉内径变小。结论超声心动图在先天性CAF的术前诊断、术后随访中具有重要的临床应用价值。