Successful Embolization of Coronary Arteriovenous Fistula Using an Interlocking Detachable Coil

Although the efficacy and feasibility of coil embolization of coronary arteriovenous fistulas have been reported, the procedure may be complicated by migration of the coil into peripheral vessels or pulmonary arteries. We report two cases of successful coil embolization of such lesions using an interlocking detachable coil. This system can provide safer and more effective coil embolization in patients with coronary arteriovenous fistula.     

Retrograde Transcatheter Coil Embolization of Congenital Coronary Artery Fistulas in Infants and Young Children

We report the success of transcatheter coil embolization of congenital coronary artery fistulas in infants and young children, primarily using a retrograde coaxial delivery system. Transcatheter coil embolization of coronary artery fistulas has been used successfully in the adult population with limited reports in the pediatric population, especially in infants. Ten patients underwent transcatheter coil embolization of congenital coronary artery fistulas between January 1993 and August 1999. The median age was 4.5 years (ranging from 5 weeks to 9.8 years). Coils were delivered exclusively using a retrograde arterial approach in 8 of 10 patients. A coaxial system was used in 8 patients. Angiography immediately after placement of coils revealed no residual shunt in any patient. Recent follow-up evaluation is available for 9 patients (median duration of follow-up, 7.9 months). Echocardiographic and clinical evaluations show complete occlusion in 8 of 9 patients. All patients are asymptomatic with normalization of chamber dimensions and coronary artery caliber. Transcatheter coil embolization of congenital coronary artery fistulas can be performed safely and successfully in infants and young children. The use of a retrograde approach and coaxial system is safe and effective and may provide better accuracy, stability and precision for placement of coils. Current address: Texas Childrens Hospital, Baylor College of Medicine, 6621 Fannin MC 19345-C, Houston, TX 77030, USA     

Evaluation of Myocardial Perfusion Using Positron Emission Tomography in Infants Following a Neonatal Arterial Switch Operation

This study was performed to examine the use of positron emission tomography (PET) as a method of evaluating myocardial perfusion after the arterial switch operation for correction of transposition of the great arteries. Eleven asymptomatic patients (median age 2.3 years, range 1.3–4.3 years) post successful neonatal arterial switch repair for transposition underwent cardiac PET scanning using N¹³ ammonia before and after dipyridamole infusion. Reconstructed data from static scans were analyzed for regional perfusion defects before and after pharmacological stress. Simultaneous assessment of coronary flow before and after stress was performed using a Patlak graphical analysis of data from dynamic scans. Results obtained from PET scanning were correlated with patterns of coronary artery anatomy, electrocardiogram (ECG) recordings, and echocardiographic evaluation. PET scanning demonstrated normal distribution of myocardial perfusion before and after stress in all but one patient, who was found to have a discrete inferior transmural perfusion defect. The defect was well correlated with perioperative ECG changes and a complicated postoperative course. Myocardial blood flow before dipyridamole (0.690 ml/min/g) was similar to reported adult rest values. There was a small but significant (p < 0.002) increase in myocardial blood flow after dipyridamole stress with a mean coronary flow reserve of 1.19 (±0.103). Echocardiographic evaluation failed to demonstrate significant wall motion abnormalities in any of the patients. Cardiac PET scanning is a reliable noninvasive method for evaluation of myocardial perfusion in small children. In this study, the incidence of myocardial perfusion defects after the arterial switch operation is lower than previously reported. The data obtained concerning coronary flow and coronary flow reserve after the arterial switch need to be interpreted with caution because normal data in children are not available.     

Transcatheter embolization of coronary artery fistula with controlled release coils

ABSTRACT The treatment for large congenital coronary cameral fistulas has been surgical but with advances in interventional catheterization techniques transcatheter embolization of these fistulas with coils or detachable balloons is now possible. This report describes occlusion of a congenital coronary arteriovenous fistula in a 6-year-old girl.     

Development of Superior Vena Cava to Pulmonary Vein Fistulae Following Modified Fontan Operation: Case Report of a Rare Anomaly and Embolization Therapy

A patient with double inlet single ventricle, L-transposition of the great arteries, and atrioventricular valve regurgitation developed progressive heart failure necessitating modified Fontan operation at 16 months of age. The procedure included replacement of the atrioventricular valve and pacemaker insertion. Eight years following the operation, the patient developed progressive cyanosis. Catheterization confirmed the presence of fistulae from the superior vena cava and innominate vein to the pulmonary veins as the cause of cyanosis. Coil embolization of several fistulae was performed successfully at catheterization. Contributing factors for fistula formation in Fontan patients are discussed, and therapy is reviewed.     

Transcatheter Occlusion of Residual Patent Ductus Arteriosus After Surgical Ligation

The reported frequency of residual leaks after surgical ligation of patent ductus arteriosus (PDA) varies from 6% to 23%. Reports on percutaneous closure of PDA also involve patients with residual PDA after ligation, but specific data regarding this type of PDA are rare. Our objective was to assess retrospectively the characteristics of residual PDA relevant to transcatheter closure and occlusion results using three types of occluders. Twelve consecutive patients underwent transcatheter occlusion of residual PDA after surgical ligation at a median age of 4.6 years (range 3.2–44.6 years) and median weight 16.5 kg (range 13–62 kg). Three types of occluder were used: Gianturco coils, detachable Cook PDA coils, and the new Amplatzer duct occluder. The median diameter of residual PDA after ligation was 1.5 mm (range 0.9–4.2 mm). All PDAs were of type A morphology. Thirteen devices were successfully placed in the 12 patients, without embolization. There were no complications. At 1 month and 1 year follow-up all residual shunts were completely closed. Coils are particularly suitable for complete closure of residual leaks after surgical ligation of PDA. A 100% closure rate was achieved with a low number of implanted coils.     

Coronary Blood Flow Assessed by Transthoracic Echocardiography in Neonates

Coronary flow measurement has provided useful clinical and physiologic information. However, there is little information about values for coronary flow in normal neonates, much less neonates with congenital heart disease. The aim of this study was to assess coronary blood flow in normal neonates and to compare the results with those in infants with ventricular septal defect. The study groups consisted of 12 normal neonates and 9 infants with simple ventricular septal defect associated with pulmonary hypertension. Left ventricular dimension, left ventricular mass, and the diameter of the coronary vessel were measured by standard M-mode and two-dimensional echocardiography. Peak flow velocities, flow velocity integrals, and flow volumes in the left anterior descending and circumflex coronary arteries were measured. The flow signals from the left anterior descending and circumflex coronary arteries were recorded in 84% (10/12) and 17% (2/12), respectively, in the normal neonates and 78% (7/9) and 11% (1/9), respectively, in the patients. The left ventricular end diastolic diameter and mass were significantly lower in normal infants than in the infants with ventricular septal defect (1.56 ± 0.11 vs 1.84 ± 0.09 cm and 5.4 ± 1.6 vs 8.8 ± 0.8 g, respectively, p < 0.01). The mean peak diastolic velocity and the flow velocity time integral in the left anterior descending coronary artery were significantly lower in the normal neonates than in the patients (15 ± 4 vs 28 ± 6 cm/sec and 2.3 ± 0.6 vs 5.9 ± 1.5 cm, respectively, p < 0.01). The coronary flow volume was significantly lower in the normal neonates than in the patients (3.1 ± 1.4 vs 7.9 ± 4.7 ml/min, p < 0.05). However, the flow volume of the left anterior descending coronary artery/left ventricular mass did not show any significant difference between the two groups. Our study demonstrated in neonates that it is feasible to detect noninvasively and to evaluate the flow of the left anterior descending coronary artery under physiologic conditions and abnormal hemodynamic situations. Increased flow volume in the left anterior descending coronary artery in patients with ventricular septal defect may be a compensated mechanism for the increase in oxygen demand of hypertrophic myocardium of the left ventricle.     

Coil Embolization Therapy in Congenital Coronary Arterial Fistulas

Three pediatric patients (8&frac12; years, 3 years, and 1 month) presented with congenital coronary arterial fistulas. In all cases the fistulas entered into the right side of the heart (main pulmonary artery, n = 1; right ventricle, n = 2). In the first patient, the fistula and an open ductus arteriosus were closed during the same intervention. The second patient presented with a single left coronary ostium and residual shunt from the coronary artery system to the right ventricle after surgery. The third child had pulmonary atresia with intact ventricular septum and a fistula from the left coronary artery to the right ventricle. The fistulas in all patients were managed with coil occlusion. Fistula occlusion was documented with angiocardiography.     

Noninvasive evaluation of systolic pressures of pulmonary artery and right ventricle using contrast-enhanced doppler echocardiography: Comparative study using sonicated albumin or glucose solution

Evaluation of pulmonary arterial pressure is essential for the diagnosis and management of patients with congenital heart disease; it is usually done by cardiac catheterization. An alternative, noninvasive method may be clinically more useful. The purpose of this study was to assess the usefulness of the noninvasive determination of systolic pressure of the pulmonary artery and right ventricle by contrast-enhanced Doppler echocardiography. We selected 30 pediatric patients (28 with trivial or nonsignificant tricuspid regurgitant Doppler signals and 2 with significant tricuspid regurgitant Doppler signals) aged 2 months to 21 years. The flow velocity of tricuspid regurgitation was measured with continuous-wave Doppler of the right ventricular inflow view or left parasternal or apical four-chamber view before and after injection of two types of contrast medium (hand-agitated 5% glucose or sonicated albumin). The systolic pressure of the pulmonary artery was assessed as the estimated right ventricular systolic pressure (albumin method) minus the peak pressure gradient across the pulmonary valve (nonenhanced Doppler method). After injection of hand-agitated 5% glucose and sonicated albumin, trivial tricuspid regurgitation signals were enhanced in 25 of 28 patients (89%). In two patients, spectral envelopes were well defined enough to obtain the peak systolic velocity of the tricuspid regurgitation jet without contrast medium injection. Peak velocity was not altered by injection of contrast medium in these patients. There was significant correlation between the estimation by contrast-enhanced Doppler using hand-agitated 5% glucose and the cardiac catheterization measurement of the transtricuspid pressure gradient (r= 0.88). The transtricuspid pressure gradients obtained by continuous-wave Doppler during sonicated albumin enhancement corresponded closely to those measured by cardiac catheterization (r = 0.95). Pulmonary arterial and right ventricular systolic pressures measured by Doppler using sonicated albumin and those obtained by cardiac catheterization were highly correlated (right ventricle, r = 0.96; pulmonary artery, r = 0.95). In conclusion, this technique may be a valuable noninvasive method for determining accurate right ventricular and pulmonary arterial systolic pressures.     

Early Presentation of a Congenital Coronary Artery Fistula in a Neonate

A neonate developed severe congestive heart failure secondary to a congenital coronary artery fistula requiring emergent surgery. Intraoperative transesophageal echocardiogaphy helped guide successful emergent closure of the fistula without complications.     

Myocardial Infarction in a 14-Year-Old Girl, Ten Years After Surgical Correction of Congenital Coronary Artery Fistula

Acute myocardial infarction developed in a 14-year-old girl, ten years after surgical repair of a coronary artery fistula. Angiography revealed fresh thrombus in the left anterior descending branch of the left coronary artery. The thrombus probably developed in the residual cul-de-sac of the occluded fistula. A procedure to abolish the cul-de-sac was then performed.     

Pathological Study of Postcoronary Arteritis in Adolescents and Young Adults: With Reference to the Relationship Between Sequelae of Kawasaki Disease and Atherosclerosis

To clarify whether the cardiac sequelae of Kawasaki disease (KD), postcoronary arteritis lesions can become a risk factor for atherosclerosis of the coronary arteries, six autopsy cases of patients older than 15 years of age with coronary arterial lesions caused by arteritis in childhood were examined histologically. Twenty-four arteries were inspected: 10 had no evidence of aneurysm formation, 7 arteries manifested simple dilatation of the lumen, and in 7 arteries there were aneurysms with recanalization. In the group in which there were no aneurysms, ``new intimal thickening' was observed in addition to the preexisting intimal thickening which had been caused by arteritis in the acute phase of KD. In the second group with aneurysmal arteries whose lumen remained dilated, thrombotic occlusion occurred in 4 of 6 aneurysms. In addition, advanced atherosclerotic changes (i.e., complicated lesions) were found in a 39-year-old patient. Finally, in the third group of arteries which manifested recanalized lumens after thrombotic occlusion of the aneurysms, new intimal thickening was seen on the internal side and some of them were occluded. The findings in this study suggest that aneurysms present in coronary arteries in individuals with a history of KD constitute a risk factor for atherosclerosis later in life.     

Radiofrequency Catheter Ablation of Tachycardia in Patients with Congenital Heart Disease

Patients with anomalies of the heart frequently suffer from arrhythmias that either are associated with a congenital heart defect or result from the course of the disease. For most of the bradyarrhythmias, appropriate timing of the initiation of treatment is more challenging than its eventual execution. In the case of tachycardias, technical aspects of treatment require more attention because the often imperative impact such tachycardias have on quality of life, morbidity, and mortality determine intervention timing. Increasingly, interventional electrophysiology is turned to as a potentially definitive and substrate-related treatment because of antiarrhythmic drug therapy's failure to prevent arrhythmia recurrences and the potential detrimental side effects from drug therapy seen in this particular patient population. Using the experience gained during the past 10 years in the treatment of patients with arrhythmias but without associated structural heart disease, several groups reported their results and difficulties with the application of such therapy to patients with congenital heart defects. In this report, we summarize our hospital's experience with transcatheter radiofrequency current application for treatment of various types of tachyarrhythmias in 139 children and adults with congenital heart defects, emphasizing the current limitations of such therapy and addressing the potential benefits expected from future technology. Patient ages ranged from 5 months to 76 years (mean 25.3 ± 17.7 years), including 56 children and adolescents less than 16 years of age. At least one attempt at surgical palliation or correction was made in 93 patients; the remaining 46 patients had no surgical intervention attempts. A total of 225 different tachycardias were found, 93 of which were based on a congenital arrhythmogenic substrate (e.g., an accessory pathway). Acquired substrates (e.g., scars or myocardial fibrosis) gave rise to the remaining 132 tachycardias. Radiofrequency current ablation (183 sessions) successfully treated 121 of 139 patients. Within a follow-up period of 21 months a recurrence of the intrinsically treated tachycardia was seen in 24 patients (10.7%); 13 of the 24 underwent a successful repeat session. There were no significant procedure-related complications. Young and adult patients with congenital heart disease can be safely and successfully treated for tachycardias with the use of radiofrequency current ablation. Because such treatment meets the specific needs of this patient group, early consideration for this therapy is recommended.     

Atypical Bland–White–Garland Syndrome with Stenosis of the Origin of the Left Coronary Artery: Catheter Intervention After Mammary Artery Bypass Stenosis and Residual Fistula to the Pulmonary Trunk

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland–White–Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.     

Pediatric Cardiovascular Embolization Therapy

Transcatheter embolization of superfluous vascular structures has assumed an important role in pediatric interventional cardiology. A variety of devices and materials are being used to treat an increasing number of unwanted arterial, venous, and surgically created vascular connections. In general, the occlusion techniques are simple, the results are good, and the complication rates are low. The current indications, devices, materials, methods, applications, and results of pediatric cardiovascular embolization therapy are described.     

Congenital Subclavian Artery to Subclavian Vein Fistula Presenting with Congestive Heart Failure in an Infant

Contrary to acquired arteriovenous fistulas, the congenital malformations are very rare. Here we report a case of congenital arteriovenous fistula forming a communication between the right subclavian artery to subclavian vein. To our knowledge, there is no similar case in which an infant has been successfully operated on for cardiac failure.     

Mechanical Occlusion of the Patent Ductus Arteriosus with Jackson Coils

The effectiveness and safety of a protocol for transcatheter patent ductus arteriosus (PDA) closure was assessed. Our goal is complete mechanical occlusion of the PDA in the catheterization laboratory by adding coils until it is no longer possible to cross the PDA with a guidewire. Detachable coil closure of a PDA with a narrowest diameter of 2.4 ± 0.1 mm was attempted in 83 patients with a median age of 2.8 years (0.7 to 27.8 years) and whose median weight was 14.5 kg (6 to 61.6 kg). Coils were successfully implanted in 82 of 83 patients, and in 1 patient a large Rashkind double umbrella was used instead. Complete closure was obtained in 80 (97.6%) patients, 48 of those (59%) received more than one coil. Reintervention for residual shunting was required in only 1 patient and another patient has a trivial residual shunt. Device embolization occurred in three cases. Despite the use of multiple coils there was no evidence of significant left pulmonary artery stenosis. The fluoroscopy time increased from 14.0 ± 2.0 minutes for a single coil to 25.3 ± 2.9 minutes for multiple coils (p < 0.01). Attempting to obtain complete mechanical occlusion of the PDA during the implant procedure by adding extra coils reduces the need for reintervention for residual or recurrent shunting.     

Measurement of Atrial Septal Defect Size: A Comparative Study Between Three-Dimensional Transesophageal Echocardiography and the Standard Balloon Sizing Methods

Atrial septal defect (ASD) size measurement is of paramount importance for the successful deployment of a transcatheter septal occluder. The stretched balloon diameter (SBD) has long been regarded as the gold standard for selection of the size of any device. Three-dimensional (3-D) transesophageal echocardiography (TEE) can visualize the overall structure of the atrial septum, therefore rendering an accurate size of the ASD. In this study we aimed to validate the accuracy of ASD size measurement by 3-D TEE and to elucidate the reason for the difference between balloon sizing and 3-D measurement. Forty-one consecutive patients were enrolled in this protocol for ASD device closure using the Amplatzer septal occluder. Thirty-nine patients were diagnosed by 2-D transthoracic echocardiography as secundum ASD and 2 patients were diagnosed as patent foramen ovale. Two measurements of the balloon size were sequentially obtained by 2-D TEE after the balloon was fully inflated in the left atrium. First, no residual shunt across the septum could be seen while the balloon was pulled back against the septum. This measurement was called the balloon occlusive diameter (BOD). Second, with balloon deflation, a slight deformity of the balloon was seen just prior to its popping through the septum. This measurement was called the stretched balloon diameter (SBD). Three-dimensional TEE was performed in all patients at the beginning of the procedure before device deployment and within 15 minutes after device release. Three-dimensional TEE provided superior views of the ASDs, showing the spatial relationship between the ASD and the neighboring structures. For maximal ASD size measurement, balloon sizing was larger than 3-D TEE examination, whereas 2-D was smaller than the other two methods. The best correlation was found between 3-D TEE measurements and the BOD (r= 0.98, p < 0.0001). Three-dimensional TEE provides en face view of ASD; thus, it can accurately measure the size of ASD. Three-dimensional TEE measurement of ASD can be used instead of balloon sizing for the selection of transcatheter ASD occluder size.     

Percutaneous transcatheter occlusion of coronary artery fistulas using detachable balloons

Three pediatric patients underwent successful transcatheter coronary artery fistula occlusion using the Debrun system. This latex balloon system offers several advantages over other occlusion systems. First, the balloon delivery and release is controlled. Second, “test occlusions” can be performed that allow simultaneous balloon inflation, coronary cineangiography, and electrocardiographic monitoring. Third, because the balloons are flow-directed, they are easily positioned in properly chosen locations. Finally, the balloons can be constructed to suit the size of the fistula. In this study, two patients received only one balloon; in the other patient two balloons were placed in the same fistula. All fistulas drained into either the right atrium or ventricle and were successfully occluded. After a follow-up period of up to 3 years, no local or systemic reactions to the balloons were recognized. We conclude that detachable balloon occlusion of coronary artery fistulas is a safe, effective alternative to surgical ligation in selected pediatric patients.     

Increase in Pulmonary Arterial Diameter Under Prostaglandin E1 Therapy in Infants with Cyanotic Congenital Heart Disease

We studied the change in pulmonary arterial diameter under prostaglandin E₁ (PGE₁) therapy in infants with ductus-dependent cyanotic heart disease (pulmonary atresia). Ten infants undergoing administration of PGE₁ for more than 2 weeks were selected for this study. A classic Blalock–Taussig (BT) shunt was performed in seven patients and a modified BT shunt in three. The shunt was successful in all patients. The internal diameters of the right and left pulmonary arteries were measured before and after the start of PGE₁ therapy using two-dimensional echocardiography. The pulmonary arterial index (PA index) was calculated to correct the diameters for body surface area. Both pulmonary arteries enlarged during the first week of PGE₁ therapy in all 10 patients. Further increases in the two diameters were observed even after the first week of treatment in nine patients. Both the mean diameters at 2 weeks after the start of PGE₁ were about 50% larger than the initial diameters (right; increased from 3.1 to 4.7 mm; left; increased from 3.0 to 4.4 mm). Of the eight patients given PGE₁ for more than 3 weeks, four showed no changes in pulmonary arterial diameters after the first 2 weeks and the remaining four showed a slight increase. PA indexes also showed a rapid increase during the first 2 weeks and no significant change thereafter. We suggest that, in infants with pulmonary atresia and small pulmonary arteries, the optimal pulmonary artery size for BT shunt insertion is achieved after 2 weeks of PGE₁ infusion, with no further significant increase in size being observed after this time.