Balloon dilation and stenting of hypoplastic pulmonary arteries: a new area of cooperation between interventional pediatric cardiologists and cardiac surgeons.
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Biomedical subjects
Publications and source records attributed to C E Mullins.
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Between february and december 1990, we attempted percutaneous closure of patent ductus arteriosus (PDA) in 26 patients and in one ostium secundum atrial septal defect (ASD). One of the PDA was right sided with right aortic arch and two were post surgical leaks. We used the "USCI-Rashkind PDA double disc occluder" and the newly designed "Lock Clamshell" occluder for the ASD. Twenty seven patients (20 females and 7 males), aged 1.3 to 16 years (M = 5.6) and weighing 9.5 to 56 kg (M = 21.3), were studied. Diagnosis was made clinically with the aid of Doppler colour flow echocardiography, with follow-up studies at 24 hours, 6 months and 1 year after the procedure. Diameter of the ductus varied from 1.6 to 8.2 mm (M = 2.6). Successful implant of the device was achieved in 25 cases (95%), of which 18 (72%) were totally occluded at 24 hours and 21 (84%) after 6 months, remaining small residual leaks in four (16%). In only one patient total occlusion was demonstrated 1 year after device implantation. We achieved 22 (88%) total occlusions. One case was not possible to occlude, due to its large size. Angiocardiography demonstrated adequate anatomy in 18, and difficult in 8 patients. Twelve and 17 mm devices were used in 16 and 9 patients, respectively. Successful second implant with subsequent total occlusion was achieved in one patient with a previous moderate residual leak. The ASD was central and single, with a diameter of 20 mm. Immediate total occlusion was achieved. We conclude that percutaneous PDA closure is a safe and effective procedure.(ABSTRACT TRUNCATED AT 250 WORDS)
UNLABELLED: A hyperdynamic left ventricle can lead to post-dilation mid-cavitary obstruction in the absence of any preballoon mid-cavitary obstruction in children with aortic valve stenosis. The purpose of this study was to define the incidence and course of post-dilation mid-cavitary obstruction. We reviewed the catheterization and echocardiographic data of 35 patients, ages 3 days to 18 years (mean = 7.9 years), with isolated aortic valve stenosis who underwent balloon dilation with a fall in aortic valve gradient to less than 20 mm Hg. Post-dilation mid-cavitary obstruction was suspected from post-dilation left ventricular angiogram and was quantitated by echocardiographic and Doppler examinations performed immediately after dilation and in a 1-3 month follow-up period. Continuous wave Doppler directed by color flow Doppler was used for evaluation and differentiation of post-dilation mid-cavitary obstruction and residual aortic valve stenosis. Three children (3/35 [9%]) all under 2 years of age developed post-dilation mid-cavitary obstruction immediately after dilation. The obstruction within the cavity resolved spontaneously in all three patients. CONCLUSIONS: 1) Post-dilation left ventricular mid-cavitary obstruction can occur in children under 2 years of age after balloon dilation of severe aortic valve stenosis, and 2) total regression of post-dilation mid-cavitary obstruction occurs spontaneously in all patients.
We have performed 50 blade and balloon atrial septostomies in 46 patients with diagnoses of transposition of the great arteries--32 patients; mitral atresia or stenosis--10 patients; total anomalous pulmonary venous drainage--2 patients; tricuspid atresia--1 patient; and pulmonary valve atresia with hypoplastic right ventricle--1 patient. The patients' age ranged from 1 day to 72 months (median = 8 months) and weights ranged from 2.7 to 14.5 kg. In patients with transposition the systemic saturation increased from an average of 62% to 74.6% (p less than 0.001) and the inter-atrial mean pressure gradient was reduced from 7.74 +/- 5.3 to 1.4 +/- 2.04 mm Hg. Patients with mitral atresia had no significant increase in systemic arterial saturation but a significant decrease in the mean inter-atrial gradient from 19.6 +/- 12.4 to 3.8 +/- 5.3 mm Hg. In three patients the blade septostomy was unsuccessful for technical reasons and the condition of the patient. Complications included one death due to atrial laceration, blood loss requiring transfusion in 5 patients, transient CVA in one patient, and failure of the blade to close in one patient. We have found the palliative use of the blade catheter in conjunction with balloon atrial septostomy to be an effective and safe procedure.
Fourteen patients with pulmonary vascular disease, either primary pulmonary hypertension or the result of cardiac defects, underwent balloon atrial septostomy (BAS) over the period of July 1981 to June 1988 because of symptoms of syncope, fatigue, right heart failure, and cardiac arrest. Ages ranged from 4 mo to 50 yr. Two moribund patients died within 24 h of the septostomy due to severe hypoxemia and unrelieved low cardiac output; three others died 2 wk to 35 mo later; the remaining 9 patients have improved symptomatically and are alive 11 to 96 mo after septostomy. One received a heart and lung transplant 19 mo later. We conclude that, in patients with symptomatic cor pulmonale secondary to pulmonary vascular disease, atrial septostomy can improve symptoms and may serve as a palliative bridge to heart and/or lung transplantation.
BACKGROUND: Balloon expandable intravascular stents have been used to support vessel walls in coronary and peripheral arteries in adults. The purpose of this study was to examine the efficacy and safety of these stents in the treatment of congenital heart disease. METHODS AND RESULTS: Forty-five stents were placed in 30 patients, who were 0.2-30.2 years old (weight, 3.5-76 kg). Patients with areas of stenosis that were difficult to approach surgically were chosen. Stents were mounted over balloons and placed by standard catheterization techniques. Twenty-three patients had branch pulmonary artery stenosis. Thirty-six stents were placed successfully and had reduced pressure gradients from 50.6 +/- 24 to 15.9 +/- 13.4 mm Hg. Five patients had stents placed after atrial surgery: three in obstructed Fontan repairs, one at the superior vena cava-right atrial junction after sinus venous defect repair, and one at the site of a Glenn shunt. Atrial stents reduced pressure gradients from 9.8 +/- 8.2 to 2.0 +/- 2.6 mm Hg. One patient had a stent placed in the descending aorta after coarctation dilation, and the pressure gradient was reduced from 50 to 25 mm Hg. One patient had pulmonary vein dilation with stent placement. Two stents migrated at the time of placement; one required surgical removal, and one was anchored in place by balloon dilation. One patient died within 24 hours of catheterization because of thrombus obstruction of the Fontan repair. Nine patients have undergone recatheterization. All stented vessels have remained at the same caliber as at original stent placement. CONCLUSIONS: We conclude that balloon expandable stents are useful in selected postoperative stenoses in congenital heart disease.
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Data on 111 lesions, in addition to pulmonary valve, aortic valve, branch pulmonary stenosis, native coarctation and recoarctation, were submitted to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Because there was a very heterogenous group of lesions both in types of lesions and techniques of dilation, only minimal data can be offered concerning the efficacy of the dilation of any or all of these lesions. The registry data do, however, demonstrate the safety of these dilations compared to alternative surgical therapy.
Eight-six children (aged 20 days to 14 years, 20 under age 1 year) underwent 94 percutaneous balloon valvuloplasty for pulmonary valve stenosis. The patients were divided into three groups: typical pulmonary valve stenosis (71), pulmonary valve dysplasia (9), and residual stenosis after surgical valvotomy for pulmonary atresia with intact ventricular septum (PAIS) (6). Each of the three groups was divided into two subgroups. In the early cases, balloon catheters with diameter 10-20% exceeding pulmonary valve annulus were used and the drop of the gradient was 39.5%. In the later cases, balloon diameters 30-40% greater than the valve anulus or double balloons were used and a drop of 66.7% in the RV-PA pressure gradient was achieved. The dilation in patients with dysplastic valve and residual stenosis after surgical valvotomy for PAIS was less effective. Doppler echocardiography was the technique used to evaluate residual gradient. Six months to 4 years follow-up demonstrated a persistent decrease of the valve gradient.
Catheter closure of the patent ductus arteriosus is now a reality. The purpose of this study was to establish the prevalence of associated cardiovascular defects and the accuracy of echocardiography in patients referred for transvenous ductal closure. This study reviewed 146 patients seen from 1981 to 1988: 126 with only a patent ductus arteriosus (Group I) and 20 with additional cardiovascular anomalies (Group II). Groups I and II did not differ significantly in age, gender or physical examination except for the presence of a continuous murmur (Group I 100% versus Group II 80%, p less than 0.001). A left patent ductus arteriosus was visualized by two-dimensional echocardiography in 96% of patients and was evident by Doppler study in 100%. A patent ductus arteriosus was not seen in six patients including a patient who was found to have only a collateral network from the aorta to the main pulmonary artery. The 12 patients with noncardiovascular abnormalities such as Down's syndrome were more likely than the overall group to have additional cardiovascular anomalies (6 of 12, p = 0.001). The cardiovascular anomalies encountered were varied. Eight of the 20 patients with such anomalies had only a restrictive ventricular septal defect in addition to the patent ductus arteriosus. Significant anomalies found at catheterization included two thoracic arteriovenous malformations and an isolated right carotid artery draining into the right pulmonary artery by way of a right ductus arteriosus. This study indicates that echocardiography is an effective diagnostic technique in this patient group. A thorough cardiac catheterization with angiography should be performed before implantation of a ductal device.
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Since the first clinical application of transcatheter closure technique for patent ductus arteriosus in children and infants in 1977 by Rashkind and Coll., this technique is routinely performed only in a few major Pediatric Cardiology Centers. We report our successful series which is the first of its kind both in Italy and in Southern Europe. The series included 11 children aged 1.6 to 10 years (mean age 4.5 years) all affected with patent ductus arteriosus (PDA). Subaortic stenosis (1) and ventricular septal defect with pulmonary stenosis (1) were associated anomalies. In all of the cases, after the standard percutaneous cardiac catheterization, the Mullins method was attempted to advance and deliver the Rashkind PDA double-disk occluder. Successful closure was accomplished in 10 while almost complete closure was achieved in the 11th. Normal Doppler flow pattern after the procedure confirmed the successful results. No complications occurred. Although our experience is limited, non-surgical PDA closure provided an excellent alternative to surgical procedure.
The double-disk occluding device suitable for use in infants and children with patent ductus arteriosus, developed by Rashkind and Mullins, has been available to this institution since December 1987 on an investigational basis. Results of 43 nonsurgical ductal closure attempts using this device in children and young adults between December 1987 and October 1988 are presented. Successful implantation was achieved in 42 patients (98%). In 25 patients a residual shunt was seen on angiography immediately after implantation. In 12 patients a residual ductal shunt was detected by Doppler 24 hours after implantation; 6-week follow-up studies showed a small residual shunt in only 3 patients. After a second occluder device was implanted in 2 of these patients, complete closure of the patient ductus arteriosus was achieved. In 1 patient, early in the series, the occluder device embolized to the left pulmonary artery, necessitating surgical removal at the time of ductus division. This event was the only serious complication; there were no deaths.
The patent ductus arteriosus occlusion device (Rashkind-USCI) was employed to occlude a residual ventricular septal defect after Fontan procedure in a patient with hypoplastic right ventricle, ventricular septal defect, and pulmonary stenosis. There was significant right-to-left shunting across the ventricular septal defect, with cyanosis exacerbated by exercise. After placement of the 17 mm occlusion device, the right-to-left shunt was markedly diminished, and the cyanosis resolved. Occlusion of ventricular septal defects in patients with complex congenital heart disease may be performed with the patent ductus arteriosus occluder in selected instances.
Over a 5 yr period, from October 1983 to September 1988, transseptal left heart catheterisation, using Mullins long-sheath technique, was performed in 217 infants, children, and young adults, with various forms of congenital or acquired heart disease. The mean age was 50 +/- 45 months, (range 1 mo to 21 yr). The procedure was successful in 215 (99%) patients. Pericardial puncture occurred in three patients (1.4%), though without tamponade or sequelae. There was no mortality or morbidity. Transseptal left heart catheterisation is a safe procedure for studying the left side of the heart. In addition, and of increasing importance, it allows the performance of interventional procedures on the left side of the heart and avoids the use of retrograde catheterisation through the femoral arteries.
To study the late hemodynamic results after infant orthotopic heart transplantation, five late survivors, as of October 1988, underwent right and left cardiac catheterization and endomyocardial biopsy. Mean age at transplantation was 1.4 +/- 1.5 months. Mean post-transplantation period was 17 +/- 5.6 months. All patients were on maintenance immunotherapy. The systemic and mixed venous saturations were normal, with no intracardiac shunt. The atrial and pulmonary artery anastomoses were widely patent. Three patients had coarctation of the aorta with a respective gradient of 24, 26, and 30 mm Hg; two were successfully treated by balloon angioplasty. The right-side hemodynamics were normal in all patients. Left ventricular pressures were elevated only in the patients with coarctation. The ventricular ejection fraction was normal. The right ventricular endomyocardial biopsy revealed no evidence of rejection. The mean cardiac index was 4.7 liter/min/m2 (range, 3.68-5.9). These data indicate excellent hemodynamic results and support orthotopic heart transplantation as therapy for selected neonates and infants with incurable heart disease.
A new technique of percutaneous gradational balloon pulmonary valvuloplasty was used successfully in 17 infants less than 1 year of age, including four neonates, with critical congenital pulmonary valve stenosis, who would otherwise have required surgical intervention. This technique uses a sequential series of balloon catheters of gradually increasing size as a means of eventually crossing the very small valve orifice with a balloon catheter of adequate size to achieve successful pulmonary valvuloplasty. The initial dilatations were achieved with balloon catheters 2, 3, or 4 mm in diameter. Immediately after successful balloon valvuloplasty, the mean pulmonary systolic pressure gradient was reduced from 105 +/- 11 to 20 +/- 5 mm Hg. Percutaneous balloon pulmonary valvuloplasty can be performed safely with good results despite critical congenital pulmonary valve obstruction in severely ill infants.