We report a successful percutaneous neonatal closure of haemodynamically significant patent arterial duct. The neonate was suffering multiorgan failure, and had complete atrioventricular block. Closure of the patent arterial duct, either with surgery or inhibitors of cyclooxygenase, was contraindicated. We inserted the Amplatzer Duct Occluder II, showing that percutaneous closure of a patent arterial duct is now feasible in critically ill neonates.

Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.

A 41-day-old boy was transferred to our department with severe congestive heart failure. Digital subtraction counter current aortography, and antegrade aortography, revealed coarctation of a persistent right fifth aortic arch, stenosis of the origin of an aberrant left subclavian artery, and sequestration of the lower lobe of the right lung.

As his heart failure seemed to be caused not only by pressure overload to the left ventricle following coarctation, but also by the volume load associated with the shunting effect of the pulmonary sequestration, we performed transcatheter balloon dilation of the coarctation and the origin of the aberrant subclavian artery, together with embolization of the aberrant pulmonary artery. His heart failure responded dramatically to these procedures.

Device closure of oval fossa atrial scptal defects with the Amplatzer Septal Occluder was performed in 26 patients ranging in age from 0.89 to 60.44 years. In eight additional patients no device implant was performed because of the presence of multiple defects or because the defect was of a size unsuitable for closure with the devices currently available. The strectched diameter of the defects that were closed ranged from 4 to 23 mm (mean 14±5.4 mm) and device sizes ranged from 4 to 24 mm. Two devices were unstable, of which one embolized to the right atrium after release. Both devices were retrived at the same procedure. One of these parients subsequently underwent a successful device closure of his defect using a larger (24-mm) device. Three patients had multiple defects, which were successfully closed with a single device. At 1-month follow-up 23/26 (88%) and at 3-month follow-up 22/24 (92%) patients had complete closure of their defects, while two had residual shunts. One further patient who had complete closure of his defect at 1-month post-implant had his device removed and his atrial septal defect patched surgically 8 weeks after device closure. This was done as a result of the development of a vegetation affecting the device after an episode of septicaemia, which was not relate to the cardiac problems. There was no procedure-related morbidty or martality and all patients remain well at the present time.

Transcatheter closure of ventricular septal defects with the Amplatzer® Membranous VSD Occluder has yielded promising initial results, but disturbances of conduction, including complete heart block, have been reported. We report our experience with the Amplatzer occluder in 35 patients with a median age 4.5 years, the defects being sized angiographically at 4.4 plus or minus 1.1 millimetres, with a range from 3 to 8 millimetres, and the size of the occluder varying from 4 to 12 millimetres. Over a median follow-up of 2.5 years, the rate of complete closure was 87% and 91%, at 1 and 2 years respectively, while 2 patients required surgical closure of the defect subsequent to the insertion of the device. Persistent regurgitation across the tricuspid valve related to the occluder was observed in 3 patients, and in 6 patients across the aortic valve. Abnormalities of conduction related to the procedure were noted in 7 patients, one-fifth of the cohort. The disturbances were transient in 1 patient, but permanent in 6, in one of the latter progressing after 6 months from left bundle branch block to intermittent Mobitz II second-degree atrioventricular block in association with expansion of the occluder. We conclude that transcatheter closure of perimembranous ventricular septal defects with the Amplatzer occluder is effective with limited complications, but the incidence of immediate and progressive disturbances of conduction related to the proximity of conduction tissues to the rims of the occluder stress the importance of larger and longer studies to assess the safety of this procedure.

Unrecognized pseudoaneurysm or rupture of a pulmonary artery is a rare but potentially catastrophic complication of pulmonary arterial catheterization. Herein, we describe a teenage patient with a pulmonary arterial pseudoaneurysm, probably iatrogenic, who presented with haemoptysis following catheterization of the right heart. The pseudoaneurysm was successfully embolized using coils inserted by catheter. Increased awareness of this lesion, its rapid recognition, and prompt therapy are the keys to a successful outcome.

Supraventricular tachycardia is a documented feature of Marfan syndrome. The safety and efficacy of radiofrequency ablation in this population of patients, however, has not been reported. We report on the successful use of radiofrequency ablation utilizing a trans-septal approach for the treatment of supraventricular tachycardia produced by an accessory muscular atrioventricular connection in a patient with Marfan syndrome.

Balloon angioplasty is now the elective technique for relief of aortic recoarctation, since it has low mortality, low morbidity, and good results at follow-up. Some concern exists concerning the possible increased risk in young children. To investigate such age-related aspects, we examined 58 children undergoing consecutive balloon angioplasty for postsurgical recoarctation. Of the children, 30 were younger and 28 older than 1 year. Recoarctation was more frequent with ventricular septal defect or other cardiac anomalies (p < 0.001). Systolic hypertension was present in 70% of children aged less than 1 year, but in only 32% of those older than 1 year (p < 0.001). The initial gradient was inversely related with the age at dilation (r = −0.28, p < 0.05), and correlated with systolic blood pressure (r = 0.81, p < 0.01). The procedure was successful in 87% of children older than, and 86% of those under 1 year. Age was not related with residual gradient, nor with the percentage increase of diameter of the site of stenosis. After balloon angioplasty, systolic hypertension was still present in 37% of children younger than 1 year, and in 25% of those older than 1 year (p < 0.05). Three complications occurred in children younger than 1 year, and 6 in those older (p < 0.05). Our results show, first, that recoarctation occurs earlier in the setting of complex disease, second that children suffering recoarctation at a younger age are more likely to be hypertensive, either before or after balloon angioplasty, third, that balloon angioplasty has the same rate of success in children below or above the age of one year, although the procedure still carries a not negligible risk. Finally, the procedure does not carry a higher risk for those children below the age of 1 year.

Patients with complex congenital cardiac malformations who have been converted to the Fontan circulation with partial exclusion of the hepatic veins may develop progressive cyanosis because of formation of intrahepatic veno-venous malformations. We describe transcatheter closure of a major intrahepatic fistula in such a setting using an Amplatzer septal occluder delivered by the left jugular venous approach in a 5 year old boy.

Background: From January, 1997, as part of an international multicentric trial, we have been closing small-to-moderate atrial septal defects within the oval fossa using the Amplatzer Septal Occluder (ASO, AGA Medical). Methods: All patients with defects within the oval fossa deemed potentially suitable for transcatheter closure were investigated by transesophageal echocardiography with the aim of gaining extra information that might alter the decision to use the device to close the defect. Views were obtained in transverse and longitudinal planes, permitting measurements of the diameter of the defect, and its distance from the atrioventricular valves, coronary sinus, and pulmonary veins. Additionally, we sought to identify multiple defects, and to exclude sinus venosus defects. Results: Of 56 patients with left-to-right shunts, 41 (73.2%) were deemed suitable for closure with the Amplatzer Septal Occluder. All underwent the procedure successfully, with no complications. This includes 5 patients with multiple small defects that were sufficiently close to the main defect to be closed with a single device. Only two of these had been detected on the transthoracic study. In the remaining 15 of 56 patients, transcatheter closure was deemed unsuitable. In 9 patients, this was due to the limitation of the size of the device available during the period of study, this representing a relative contraindication. In the remaining 6 (10.7%), transcatheter closure was not performed because multiple defects were too far apart to be closed with a single device in 3 patients, two patients were noted to have a sinus venosus defect, and another was noted to have anomalous connection of the right upper pulmonary vein to the right atrium. Excluding patients contraindicated due to the size of the defect alone, transesophageal echocardiography provided extra information in one-tenth of our patients, which altered the decision regarding management. Conclusion: Transesophageal echocardiography is indispensable in the evaluation of patients undergoing transcatheter closure of atrial septal defect.

Background: We sought to determine if the acute gains in pulmonary arterial diameter achieved by using Cutting Balloons to dilate stenoses in small arteries are maintained at follow-up. Methods: A search of our database identified all patients who underwent dilation with Cutting Balloons for pulmonary arterial stenosis between June 2001 and September 2003. We reviewed the procedural and angiographic data obtained at the initial and follow-up procedures. Results: Over the period of study, 29 patients with a median age of 3.7 years, and a range from 0.4 to 46.6, underwent treatment with Cutting Balloons at 41 procedures for one or more pulmonary arterial obstructions resistant to conventional angioplasty. At the initial procedure, we enlarged 79 vessels, with an initial minimal luminal diameter of 1.5 plus or minus 0.8 millimetres, to a diameter of 3.0 plus or minus 1.1 millimetres (p smaller than 0.001). Angiographic data indicated that 49% of the vessels showed evidence of vascular damage not requiring intervention, while stents were placed in 9 vessels. Follow-up angiography was available for 39 of the vessels, evaluated at a median of 6 months, with a range from 3 to 24 months, after the initial procedure. In these vessels, there was a mean loss of 10 plus or minus 25% in luminal diameter, p not significant. Of the 39 vessels, 8 had returned to a diameter within half of the initial diameter, giving a failure rate at follow-up of 21%, with 95% confidence intervals from 9% to 36%. All complications were identified within 24 hours of the catheterization procedure, and there were no late complications or deaths during the period of follow-up. Placement of stents produced both greater increases in diameter at the initial procedure (p smaller than 0.001), and greater losses at follow-up (p equal to 0.01). Conclusions: Despite the frequent identification of intravascular trauma, initial gains in luminal diameter after use of Cutting Balloons to dilate stenotic pulmonary arteries are maintained at follow-up.

Objectives: A retrospective study was undertaken to determine the degree of pulmonary regurgitation following percutaneous balloon valvoplasty for isolated pulmonary valvar stenosis. Background: Percutaneous balloon valvoplasty is the recognised treatment of choice in pulmonary valvar stenosis with effective relief of gradient. Few studies have reviewed the degree of pulmonary regurgitation after the balloon valvoplasty. Methods: We reviewed all patients with isolated pulmonary valvar stenosis undergoing percutaneous balloon valvoplasty at a tertiary centre between December 1984 and August 1996. Those with an associated haemodynamically insignificant atrial septal defect or patent oval foramen were also included. Their medical records, echocardiograms and angiograms were studied. Colour flow Doppler was used as a semi-quantitative way of assessing the pulmonary incompetence. Results: Over the period of review, 57 procedures had been performed in 49 patients. The median age at the time of the procedure was 1.08 years, with a range from 0.01 to 13.3 years. The median period of follow-up was 5.64 years, with a range from 3.00 to 14.26 years. Immediately following the dilation of the pulmonary valve, the peak-to-peak instantaneous systolic pressure gradient was significantly reduced. Seven patients required a second dilation of the valve. Their median age at the first valvoplasty, at 0.49 year, was significantly lower than those who required only one procedure, at a median of 1.50 years. Following the valvoplasty, mild pulmonary incompetence was noted in 26 out of 42 patients the day after the procedure, but only 7% had moderate incompetence. On follow-up, there was an increase in the number of patients with moderate to severe pulmonary incompetence, from 7% to 29%. Those patients in whom the procedure was performed at a younger age had more significant pulmonary incompetence. Neither the initial gradient across the pulmonary valve, nor the size of the balloon used, were related with statistical significance to the subsequent development of pulmonary incompetence. Conclusions: The majority of the patients with congenital isolated pulmonary valvar stenosis had only a mild increase in the degree of pulmonary incompetence following a single pulmonary valvoplasty. Patients who required the procedure early in life were more likely to develop significant pulmonary incompetence. The ratio of the size of the balloon to the diameter of the valve did not significantly affect the outcome. We suggest that those patients who had more severe stenosis because of a more abnormal pulmonary valve, and hence required early intervention, were more likely to develop greater pulmonary incompetence after the valvoplasty.

Objectives: This study was designed to identify predictors of residual defects following deployment of the Amplatzer device to close septal defects within the oval fossa. Methods: Between February 1997 and February 2000, we used the Amplatzer device to close defects in the oval fossa in 89 patients. Of these patients, 18 (20%) had residual defects. At 6 or 12 months following placement of the device, 13 defects (14.6%) had persisted. We evaluated several variables derived from clinical features, transesophageal echocardiography and catheterization to establish predictors for residual shunting. Results: Multivariate analysis identified a shorter superior rim of less than 8 mm (Odds ratio = 10.1; 95% confidence intervals = 2.64–38.72; p = 0.001), and a smaller interatrial septum in the 30-degree transesophageal echocardiographic plane of less than 30 mm (Odds ratio = 5.5; 95% confidence intervals = 1.17–26.14; p = 0.03) as independent predictors of residual defects. When the analysis was repeated defining only those 13 patients with persisting residual defects at 6 or 12 months as failures, a short superior rim (p = 0.004) remained a predictor for residual shunting. Conclusions: Defects with a short superior rim and smaller interatrial septum in the 30-degree transesophageal echocardiographic plane independently and additively predict an increased probability of residual shunting following closure of defects in the oval fossa using the Amplatzer device.

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.

A young woman with hypertrophic cardiomyopathy presented with intractable chest pain due to a myocardial bridge over the left anterior descending artery, producing severe compression during systole. Percutaneous intracoronary stenting was performed. The patient, however, developed severe and diffuse restenosis within 30 days of the procedure. Myotomy may provide a more effective treatment option for such patients with symptomatic myocardial bridging.

The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.

Objectives: We evaluated the efficacy of percutaneous transluminal coronary angioplasty for anastomotic stenosis after coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease. Subjects and Methods: From July 1997 to April 2000, four boys and one girl underwent percutaneous transluminal coronary angioplasty for 6 anastomotic lesions following coronary arterial bypass grafting using the left or right internal thoracic artery. Progressive severe stenosis of the grafts in the follow-up angiograms after grafting, and evidence of ischemia, were regarded as indications for percutaneous transluminal coronary angioplasty. Age at coronary angioplasty ranged from 4.2 to 16.7 years, with a median of 6.9 years, while the interval from operation ranged from 0.3 to 3.0 years, with a median of 1.1 years. The diameter of the balloon catheter employed varied from 1.5 to 2.5 mm, and the pressure of inflation ranged from 8 to 16 atmospheres. Results: The degree of stenosis decreased from 63 to 99%, with a median of 88%, to 0 to 40%, with a median of 17% immediately after angioplasty. A follow-up angiogram either 3 months or 1 year later revealed no restenosis in any patient. Conclusion: Percutaneous transluminal coronary angioplasty is a feasible and useful procedure for treating anastomotic stenosis following coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease.

I have previously reported the successful use of the 0.9 millimetre excimer laser catheter in 2 neonates with pulmonary valvar atresia and intact ventricular septum. In this report, I describe the expanded role of the excimer laser in a premature infant weighing 1.8 kilograms who presented with an occluded right pulmonary artery secondary to an organized thrombus. Successful reconstruction of the right pulmonary artery was performed by sequential use of lasers of 0.9, 1.4, and 2 millimetres diameter, followed by conventional balloon angioplasty. As is now the situation in adults, laser catheter intervention should now be an integral part of the armamentarium of the paediatric cardiac interventionalist.

Objectives: To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older. Background: Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes. Methods: We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years. Results: The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 ± 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 ± 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation. Conclusion: Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.