Comparison between the safety profile and clinical results of the Cook detachable and Gianturco coils for transcatheter closure of patent ductus arteriosus in 272 patients.

OBJECTIVES: We evaluated the occlusion rate and safety of Cook detachable coils versus Gianturco coils in transcatheter closure of patent ductus arteriosus (PDA). BACKGROUND: The Cook detachable coil recently was introduced in an attempt to improve the safety of transcatheter closure of PDA. METHODS: Between January 1994 and September 1998, 272 patients underwent transcatheter PDA closure. Cook detachable coils were used in 137 patients, with a mean age of 43.9 months and weight of 13.8 kg. In 135 patients, Gianturco coils were used, with a mean age of 56.8 months and weight of 17.8 kg. The mean narrowest diameter of the PDA in the Cook detachable coil group was 2.85 mm versus 2.32 mm for the Gianturco coil group. RESULTS: The Cook detachable coil group was younger and weighed less than the Gianturco group (P < 0.05 and 0.02, respectively). Their narrowest PDA diameter was larger (P < 0.01). Embolization rate was significantly lower in the Cook coil group (9[6.5%] of 137 vs 22 (16.3%) of 135; P = < 0.013). The mean follow-up for the Cook coil group was significantly shorter (0.55 years) than for the Gianturco coil group (1.18 years; P < 0.001). On an intention-to-treat basis, complete occlusion by echocardiography was achieved in 99 (72.3%) of 137 patients in the Cook detachable coil group, which was significantly less than the Gianturco coil group (114 [84.4%] of 135; P = 0.008). CONCLUSION: Cook detachable coils for transcatheter closures of the PDA are safer than Gianturco coils. Hence, children with large ductal can be treated earlier in life. Short-term complete occlusion rate was lower in the Cook detachable coil group. This rate can be explained by a shorter follow-up time, larger ductal diameter, and the different materials used for the detachable coils.

Severe tortuosity and stenosis of the systemic, pulmonary and coronary vessels in 12 patients with similar phenotypic features: a new syndrome?

We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.

Tetralogy of Fallot associated with scimitar syndrome.

We present a case of tetralogy of Fallot associated with Scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported.

Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair.

OBJECTIVE: To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999. DESIGN: The charts of patients with DCRV were studied. SETTING: Tertiary care hospital. PATIENTS AND METHODS: A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed. MAIN RESULTS: An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography. CONCLUSIONS: The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.

Transcatheter closure of single muscular ventricular septal defects using the amplatzer muscular VSD occluder: initial results and technical considerations.

Surgical closure of multiple muscular ventricular septal defects (MVSDs) is associated with mortality and morbidity; therefore, both surgeons and cardiologists welcome a nonsurgical safe approach. We report our initial results of catheter closure of MVSD using the new Amplatzer muscular VSD occluder delivered via the venous or arterial routes. Eight patients with MVSD underwent closure of their VSDs using the Amplatzer VSD occluder under general endotracheal anesthesia. The mean +/- SD of age was 5.4 +/- 3.1 years (2-10 years) and mean weight was 18.4 +/- 6.5 kg (11.5-29 kg). All patients had left ventricular volume overload with mean Qp/Qs ratio of 1.7 +/- 0.6 (1.4-3). The location of the VSD was mid muscular in four, anterior in two, apical in one, and posterior in one. The systolic pulmonary artery pressure ranged from 25 to 85 mm Hg (mean, 39.9 +/- 18.8 mm Hg). The device was implanted successfully in all eight patients. In five patients (four mid muscular and one apical), the deployment of the device was anterograde from the right internal jugular vein and in three patients (two anterior and one posterior VSD), the initial attempt at anterograde deployment was unsuccessful due to kinking in the delivery sheath; therefore, retrograde deployment was attempted successfully. The size of the device used ranged from 6 to 14 mm (the size of the connecting waist). In patients with elevated pulmonary artery pressure, repeat measurements immediately after closure revealed normalization in all. There was immediate complete closure of the defect in two patients and six patients had trivial residual shunt (foaming through the device), which disappeared completely within 24 hr in five and at 6-month follow-up in the sixth patient. The mean fluoroscopy time was 37.1 +/- 13 min (11.7-55 min). Complications encountered included transient junctional rhythm in one patient. No blood transfusion was required. On follow-up evaluation, there has been no episode of endocarditis, thromboembolism, hemolysis, or wire disruption. we conclude that the Amplatzer MVSD occluder is a safe and effective device for closure of MVSDs up to 12 mm in diameter. Further clinical trials with this device are underway. Cathet. Cardiovasc. Intervent. 49:167-172, 2000.

Radiographic characteristics of Cook detachable and Gianturco coils as well as clinical results of transcatheter closure of the patent ductus arteriosus.

PURPOSE: To describe the radiographic appearance of the Gianturco and the Cook detachable coils and present the clinical results in patients who underwent transcatheter closure of patent ductus arteriosus. MATERIALS AND METHODS: Between January 1994 and June 1997, eighty-two patients underwent closure of patent ductus arteriosus (PDA) using either Gianturco or Cook detachable coils. The chest x-ray and echocardiography of all patients were reviewed and the following parameters were evaluated: 1) the size of the heart (cardiothoracic ratio), 2) the position and the type of the coils in the postero-anterior and the lateral projection, 3) the number of coils used, 4) the existence of residual ductal flow, 5) Doppler velocity in the left pulmonary artery. RESULTS: Complete occlusion was achieved in 94%, and cardio-thoracic ratio regressed from 0.57 to 0.53 (p < 0.01), after a mean follow-up of 1.2 years. The identification of the different coils on the chest radiograph was successful in only 47% of cases, difficulties arising especially, when multiple coils were used. In 55 patients (67%) the coil position was judged to be optimal, in 27 patients (33%) suboptimal. The latter correlates with the presence of residual shunt. Multiple coils correlated more with a left pulmonary artery flow velocity exceeding 1.5 m/s. CONCLUSION: Coil-occlusion of patent ductus arteriosus is effective and leads to reduced cardio-thoracic ratio. Radiographic coil identification is possible but may be difficult if multiple coils are deployed. Suboptimal coil position led more often to residual PDA shunt. Multiple coils are more commonly associated with increased LPA velocities, but hemodynamic significant obstruction to flow is rare.

The long QT syndrome with impaired atrioventricular conduction: a malignant variant in infants.

INTRODUCTION: The long QT syndrome (LQTS) is occasionally complicated by impaired AV conduction, mostly 2:1 AV block. This form of LQTS can manifest before birth or during neonatal life, and it is more sporadic than familial. It is usually an isolated disorder, although it can be accompanied by a variety of cardiovascular and other anomalies. In spite of different treatment modes, mortality is high. METHODS AND RESULTS: The reported case presented not only with 2:1 AV conduction, but also with Wenckebach episodes with impaired right and left bundle branch conduction, and decremental conduction in the His-Purkinje axis. We also observed sinus pauses and accelerated AV junctional escape beats. CONCLUSION: Our findings, and similar observations by others, suggest involvement of the sinus node and the distal conduction system in this form of the LQTS. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed. Development of an internal cardiac defibrillator for this young age group is necessary.

Pulmonary artery augmentation with autologous aortic tissue.

OBJECTIVE: To assess durability and viability of autologous aortic tissue used to augment severe branch pulmonary artery stenosis with a novice surgical technique. PATIENTS AND METHODS: Seven patients underwent corrective surgery for complex cyanotic congenital heart disease. Their age ranged from 3-6 years, and their weight 11-17.4 kg. All had concomitant branch pulmonary artery stenosis repaired utilizing an autologous patch, harvested from the patient's own aorta by excising a ring and opening it to form the patch. The aorta is reconstructed directly by end to end anastomosis. RESULTS: One patient died in hospital. Another patient died at 18 months at home. The surviving five patients have remained well in the follow up period of mean 31 months (range 10-52). All patients were restudied by follow up echocardiography and remain with no evidence of the aortic autograft tissue calcification or stenosis. The reconstructed aorta showed no stenosis at the site of anastomosis. CONCLUSION: The intermediate term results of this novice surgical technique appear encouraging and justify the technique. However, longer follow up will be required to confirm the continued growth of this patch material.

Extracardiac right atrium-to-right ventricle homograft for uncorrectable tricuspid valve disease.

Surgically uncorrectable tricuspid valve disease in children is rare. However, when it happens the surgical options are very limited. Tricuspid valve replacement using a mechanical valve or stented bioprosthesis is impractical. Use of homografts in the "anatomic position" has its limitations. We report here the use of an extracardiac homograft connection between the right atrium and right ventricle in a 16-month-old boy in whom severe tricuspid valve stenosis developed after surgical repair of a complex ventricular septal defect associated with dextrocardia and anomalous systemic venous drainage. The patient remains well receiving no cardiac medication 12 months after the procedure.

Follow-up results of balloon aortic valvuloplasty in children with special reference to causes of late aortic insufficiency.

The purpose of this study was to report on 3- to 9-year follow-up data after balloon aortic valvuloplasty in children and to investigate causes of aortic insufficiency at late follow-up. Although the immediate and short-term results of balloon aortic valvuloplasty have been well documented, little information is available on long-term follow-up results. During a 7.3-year period ending December 1992, 26 young patients, aged 6 weeks to 20 years, underwent balloon aortic valvuloplasty with resultant reduction of peak-to-peak aortic valvar gradient from 71 +/- 20 (mean +/- SD) to 25 +/- 12 mm Hg (p < 0.001). None required immediate surgical intervention. At intermediate-term follow-up, 6 (23%) of 26 had restenosis develop and underwent surgical (4 patients) or repeat balloon valvuloplasty (2 patients). Clinical and echo-Doppler data 3 to 9 years (median 6 years) after balloon valvuloplasty revealed residual peak instantaneous Doppler gradients of 26 +/- 13 mm Hg (p < 0.001), without restenosis beyond what was observed at intermediate-term follow-up. Aortic insufficiency progressed in seven patients. However, none required intervention. Actuarial intervention-free rates at 1, 2, 5, and 9 years were 80%, 76%, 76%, and 76%, respectively. Logistic regression analysis suggested that the degree of Doppler-quantitated aortic insufficiency 1 day after valvuloplasty predicts persistent aortic insufficiency at late follow-up. These data indicate that immediately successful balloon aortic valvuloplasty in children yields a residual gradient of < or = 36 mm Hg at a median of 6 years of follow-up in most patients and an intervention-free rate at 9 years of 76%. Restenosis occurs but can be treated with a repeat intervention with good results. Aortic insufficiency remains stable and does not appear to require intervention, at least during the first decade after balloon dilatation.

The role of surgical ligation of patent ductus arteriosus in the era of the Rashkind device.

BACKGROUND: The role of surgery in managing patent ductus arteriosus (PDA) was studied in the era of the Rashkind double-umbrella device. METHODS: All 354 patients with PDA referred to our center in a 5-year period were included in this report. Of the 354 patients, 236 underwent cardiac catheterization with the intent of transcatheter PDA closure, and 118 had surgical intervention. RESULTS: In 46 (19.5%) of the 236 patients having cardiac catheterization, the procedure either was abandoned or failed. Color Doppler echocardiography demonstrated total occlusion of the ductus after 24 hours in 97 patients (41%) in the cardiac catheterization group. An additional 20 patients had no residual leaks at follow-up. Twenty other patients underwent reocclusion because of a residual shunt. Thus, of the 236 patients, 137 (58%) had successful complete closure of the PDA. Surgical PDA ligation was performed in 118 patients as the initial procedure and in 26 of the 46 patients in whom transcatheter closure was abandoned. If the remaining 20 patients in whom transcatheter closure failed are added to the 144 patients who underwent PDA ligation, the percentage having surgical intervention versus transcatheter occlusion is higher than 46%. CONCLUSIONS: Our data suggest that surgery plays a major role in the management of patients with PDA despite the advent of new interventional catheterization techniques.

Clinical results and radiographic appearance of the Rashkind double umbrella device in patients with occlusion of the ductus arteriosus.

BACKGROUND: The Rashkind double umbrella device for patent arterial duct occlusion has been used in many patients. Its radiographic appearance has not been sufficiently described. OBJECTIVE: To present the varying radiographic appearances of the Rashkind double umbrella device on the chest X-ray. MATERIALS AND METHODS: The chest radiographs of 69 patients (median age 60 months; median weight 17 kg), who underwent closure of their patent arterial duct between March 1990 and August 1994, were reviewed. The following parameters were evaluated: 1) the size of the heart (cardio-thoracic ratio) and pulmonary vessels, 2) the position of the device in AP/PA and lateral projections. The results of occlusion of the patent arterial duct were also reviewed. RESULTS: Sixty-two of 69 (90 %) patients had complete occlusion after a follow-up between 2 months and 3(1)/2 years. The cardio-thoracic ratio showed significant reduction at follow-up (P < 0.001). The two different size devices could be well differentiated in the AP and the lateral projection. In 14 patients (20 %) the device was in an asymmetrical position. There was no significant correlation between position of the device and success of occlusion in our material. CONCLUSION: Complete occlusion of the arterial duct using Rashkind double umbrella devices can be achieved in 90 % of our population. In 20 % the device will have an asymmetrical position. There is no correlation between asymmetrical position of the device in the chest radiograph and residual shunting.

Closure of residual leak after umbrella occlusion of the patent arterial duct, using Gianturco coils.

Residual leak after transcatheter occlusion of the patent arterial duct (PDA) using the Rashkind double umbrella technique is a well documented problem. At our institution, there is a 15% incidence of persistent residual leak after 12 months. Since September 1994, 15 patients have undergone successful occlusion of the residual leak using single or multiple Gianturco coils. The median age of the patients was 48 months (range: 12-354 months). The median fluoroscopy time was 11 min (range: 4-14). There were no complications and no embolization of the coils. Eleven patients had total occlusion demonstrated on angiography 5-10 min after coil occlusion. A further three patients were demonstrated on colour Doppler echocardiography to have no residual leak before discharge the same day-despite a small leak on angiography. Only one of 15 patients had a small residual leak at the time of hospital discharge and this was finally occluded 1 year later with another coil. Coil occlusion of a residual leak on the umbrella device is effective and a safe and reliable procedure which appears to be an improvement on using a second umbrella device.

Thrombosis on the Rashkind double umbrella device: a complication of PDA occlusion.

A 3-year-old child with a patent arterial duct underwent percutaneous transcatheter occlusion using Rashkind's "double umbrella" technique. The procedure, using a 17 mm device, was uncomplicated. An echocardiogram done 6 hr later showed a mobile 5 x 3 mm thrombus on the pulmonary aspect of the device. The thrombus resolved after 24 hr of intravenous heparin.

Anterograde transcatheter occlusion of the patent ductus arteriosus with Gianturco coils--a new, effective and inexpensive technique.

OBJECTIVE: To report the use of Gianturco coils in non-surgical closure of patent ductus arteriosus (PDA). DESIGN: Retrospective review of patient data. SETTING: Two specialist paediatric cardiology units in Riyadh, Saudi Arabia, and Cape Town, South Africa. PATIENTS: Thirty children (median age 36 months, weight 12.5 kg) and one adult (24 years old) with PDA underwent attempted transcatheter closure of the duct between August 1994 and February 1995. OUTCOME MEASURES: Assessment of duct closure as measured angiographically or with colour flow Doppler techniques after insertion of a Gianturco coil or coils. RESULTS: Total occlusion was achieved in 90% of patients. In 2 patients accidental embolisation of the coil occurred during the procedure. The coils were easily retrieved with a snare retrieval device. The median diameter of PDA occluded was 2.5 mm. Between 1 and 6 coils were used to achieve occlusion. The median procedure time was 79 minutes. Seventy-nine per cent of patients in Riyadh were handled as day cases. CONCLUSION: Anterograde transcatheter occlusion of a small to moderate PDA with Gianturco coils is safe and effective. The technique is considerably less expensive than the Rashkind double umbrella.

Stent implantation for relief of pulmonary artery stenosis: immediate and short-term results.

Our objective was to assess the immediate and short-term results of stent implantation to relieve pulmonary artery stenosis (PAS). Thirty-seven patients underwent an attempt at stent implantation at a median age of 7.0 years (range, 0.8-31.4 years) and a median weight of 20.5 kg (range, 7.4-85 kg). Twenty-two patients had previous tetralogy of Fallot repair. A total of 55 stents were implanted successfully in 36 patients. The peak systolic gradient across the stenotic segment decreased from a mean of 43 +/- 20.4 mmHg prestent to 13 +/- 13.9 mmHg (P < 0.001) poststent. The diameter of the narrowest segment increased from a mean of 4.8 +/- 1.6 mm to 10.5 +/- 2.6 mm (P < 0.001). The right ventricular-to-aortic mean systolic pressure ratio decreased from 0.74 +/- 0.2 to 0.52 +/- 0.19 (P < 0.001). Complications included balloon rupture prior to full stent expansion in 4 patients (in 2 patients the stent was positioned in the superior vena cava, and in 2 in the inferior vena cava), distal migration of a stent which was successfully retrieved at surgery 1 mo later in 1 patient, and tethering of the stent to the balloon requiring surgical removal in 1 patient. One patient died several hours after stent placement. Sixteen patients underwent repeat catheterization at a mean follow-up interval of 0.9 +/- 0.5 years (range, 0.2-2.0 years). The mean gradient across the stent for these 16 patients was 26.7 +/- 19.8 mmHg, and there was no change in the mean diameter (9.4 +/- 3.2 mm). Two patients developed stenosis related to neointimal proliferation at the stent site which was redilated successfully. In conclusion, stent implantation is generally safe and effective in relieving PAS.

Transcatheter closure of the patent ductus arteriosus: comparison between the Rashkind occluder device and the anterograde Gianturco coils technique.

Thirty-five patients (mean age 43.7 months, mean weight 13 kg) underwent anterograde patent ductus arteriosus (PDA) occlusion with Gianturco coils (coil group). These patients were compared with 35 age- and weight-matched patients who underwent closure of their PDA with the Rashkind umbrella device (device group). The mean PDA diameter at its narrowest point was 2.8 mm in the coil group and 2.7 mm in the device group. There was immediate closure angiographically in 20 (57%) of 35 in the coil group compared with 9 (26%) of 35 for the device group. Color flow mapping before discharge revealed complete closure in 30 (86%) of 35 in the coil group compared with 18 (51%) of 35 in the device group (chi square = 9.5455, p < 0.005). Mean fluoroscopy time was 18.5 minutes (median 13.5 minutes) and 14.7 minutes (median 13 minutes) for the coil and device, respectively. Four coils and one device embolized down the pulmonary artery; all were successfully retrieved in the catheterization lab. Coil closure of the small to moderate PDA is safe and effective. It is more effective in achieving immediate closure than the Rashkind device. More clinical trials with the coil technique are warranted to establish the long-term results of this technique.

Transcatheter closure of residual patent ductus arteriosus shunting after the Rashkind occluder device using single or multiple Gianturco coils.

Transcatheter closure of a patent ductus arteriosus (PDA) using the Rashkind occluder or the buttoned device is accompanied with a 7-38% incidence of residual shunt. Closure of the residual shunt with a second device is expensive, associated with possible morbidity, can be technically challenging, and occasionally does not completely eliminate the shunt. Our objective was to assess the immediate and short-term results of transcatheter closure of residual PDA after the Rashkind device using single or multiple Gianturco coils. Five patients (one male/four female) underwent transcatheter closure of residual PDA at a median age of 8.4 yr (range 2-10.1 yr) and median weight of 23 kg (range 11.8-32 kg). A 4F catheter was used for delivery of the coils in all patients. Three patients underwent multiple coil and two single coil closure with complete elimination of the shunt. There was complete elimination of the shunt as documented by repeat angiography immediately after the coil closure. The median fluoroscopy time was 24 min (range, 22-55 min). All patients were discharged home on the same day. There were no complications Follow-up evaluation was performed with color flow mapping of the main pulmonary artery within 4 hr after closure and 6 weeks later with echocardiography and chest radiography. We conclude that transcatheter closure is an effective therapy for patients with residual PDA after the Rashkind device using the single or multiple coil technique. This technique can be performed on an out-patient basis without the need for general anesthesia and at a lower expense than a repeat occluder device or surgery.