Charles E. Mullins

Use of endovascular stents in congenital heart disease.

BackgroundBalloon 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 ResultsForty-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. ConclusionsWe conclude that balloon expandable stents are useful in selected postoperative stenoses in congenital heart disease. (Circulation 1991;83:1923—1939)

Implantation and intermediate-term follow-up of stents in congenital heart disease.

BackgroundBalloon-expandable stents (Johnson and Johnson Interventional Systems) have been in use for congenital heart disease since late 1989. They have made possible treatment in previously untreatable branch pulmonary artery stenoses and systemic venous stenosis. The purpose of this report is to detail the results and intermediate-term follow-up of stents used for treatment of congenital heart disease. Methods and ResultsEighty-five patients underwent placement of 121 stents in Houston and Boston. Fifty-eight patients had stents put in pulmonary arteries, nine had stents in conduits or outflow tracts, and 21 had stents in venous stenoses or narrowed Fontan anastomoses. (Three patients had stents in two locations.) These stent procedures resulted in gradient reduction from 55.2 ± 33.3 to 14.2+13.5 mm Hg in pulmonary arteries, from 41.4 ± 26.0 to 20.7 ± 17.0 mm Hg in conduits or outflow tracts, and from 9.8 ± 6.9 to 2.4+3.1 mm Hg in venous stenoses or Fontan anastomoses. Diameter of narrowings increased from 4.6 ± 23 to 113 ± 3.2 mm in the pulmonary artery, from 8.8+3.6 to 12.7 ± 2.6 in conduits, and from 3.8 ± 2.9 to 113+2.8 in venous stenoses. Follow-up has shown stent fracture in one patient, restenosis in one, and sudden death in one. Recatheterization has been done in 38 patients an average of 8.6 months after stent installation. Compared with immediately postimplant data, there was no significant change in luminal diameter or pressure gradient. Redilation was performed in 14 patients (17 stents) 1 week to 24 months after implantation (mean, 10.2 months), with a small but significant increase in stenosis diameter. ConclusionWe conclude that stent treatment of vascular stenoses in congenital heart disease retains efficacy at medium-term follow-up and offers a much-improved outlook for patients with these lesions.

Implantation of balloon-expandable intravascular grafts by catheterization in pulmonary arteries and systemic veins.

The purpose of this investigation was to evaluate the efficacy and safety of implanting expandable intravascular stents in pulmonary arteries and systemic veins. Twenty-seven balloon-expandable grafts were placed in 13 mongrel dogs under anesthesia. A long sheath was introduced over a wire and catheter or dilator into the pulmonary artery or target vein. A collapsed stainless steel expandable mesh stent was placed over the balloon of an angioplasty catheter. The catheter with the mounted stent was advanced through the sheath. The stent expanded to the diameter of the balloon as the balloon was inflated, and remained expanded as the balloon was deflated. The stent was expanded further with a larger balloon in 11 instances. Eleven stents were placed successfully in pulmonary arteries (out of thirteen attempted), and 11 of 14 were installed in tributaries of the precava or postcava. Three inadvertent embolizations of the devices occurred. All three devices that embolized lodged in the pulmonary arteries and did not obstruct flow. Seven dogs were recatheterized at intervals ranging from 56 to 278 days. Twelve stents were patent and nonobstructive, and two were malpositioned, one of which was obstructed. Three animals were killed 2 months (two dogs) and 9 months (one dog) after the implantations. The stents (four in the pulmonary arteries and two in veins) were completely covered with neointima and were patent, without thrombosis. These stents hold promise for definitive dilation of congenital or postoperative vessel stenoses.

Intravascular Stents in Congenital Heart Disease: Short- and Long-Term Results From a Large Single-Center Experience☆☆☆

OBJECTIVES This report describes the results of the Food and Drug Administrations phase 1 and 2 clinical trials of intravascular stents at Texas Childrens Hospital. BACKGROUND Since the late 1980s, intravascular stent implantation for the treatment of arterial and venous stenoses in congenital heart disease has been highly successful. METHODS Stents were placed in postoperative pulmonary artery (PA) stenoses, congenital PA stenoses or stenoses of systemic veins/venous anastomoses. Prospective collection of data according to protocol was done before intervention, after stent implantation and at follow-up catheterization. RESULTS At stent implantation, pressure gradients decreased significantly in all three groups (mean +/- SD): from 46 +/- 25 to 10 +/- 13 mm Hg in postoperative PA stenoses (p < 0.001); from 71 +/- 45 to 15 +/- 21 mm Hg in congenital PA stenoses (p < 0.001); and from 7 +/- 6 to 1 +/- 2 mm Hg in stenoses of systemic veins/venous anastomoses stenoses (p < 0.001). Vessel diameters markedly increased: from 6 +/- 3 to 12 +/- 3 mm in postoperative PA stenoses (p < 0.001); from 3 + 1 to 9 + 1 mm in congenital PA stenoses (p < 0.001); and from 3 +/- 4 to 12 +/- 4 mm in stenoses of systemic veins/venous anastomoses (p < 0.001). In the postoperative and congenital PA stenoses groups, right ventricular pressure decreased (right ventricular pressure indexed to femoral artery pressure ratio): from 0.63 +/- 0.2 to 0.41 +/- 0.02 (p < 0.001) and from 0.71 +/- 0.3 to 0.55 +/- 0.35 (p = 0.04), respectively. Perfusion to a single affected lung increased from 31 +/- 17% to 46 +/- 14% (p < 0.001). On recatheterization (mean 14 months), results varied minimally. Repeat angioplasty of residual stent stenoses was safe and effective. Complications included four early patients with stent migration, three with stent thrombosis and two deaths. There were no late complications. Significant restenosis occurred in only three patients. CONCLUSIONS Intravascular stents for the treatment of vascular stenoses in congenital heart disease provide excellent immediate and long-term results.

Balloon angioplasty of native coarctation of the aorta : midterm follow-up and prognostic factors

UNLABELLED OBJECTIVES, This retrospective analysis was designed to examine the intermediate and long-term effects of balloon angioplasty for native coarctation of the aorta and to determine whether any factors are predictive of outcome. BACKGROUND Balloon angioplasty for native coarctation of the aorta is controversial. Concerns exist over lack of long-term follow-up and possible formation of aneurysms. The role of transverse arch and isthmus hypoplasia after balloon dilation is unknown. METHODS Included in the study were all patients 3 days to 29 years old (mean age 4.6 years) referred for possible balloon dilation to the pediatric cardiac catheterization laboratory with evidence of a discrete coarctation of the aorta. The hemodynamic data, angiograms and clinical records of 102 patients were examined, with follow-up data from 2 to 117 months (median 36.2) available in 92 patients. RESULTS Immediate success with balloon angioplasty was achieved in 93 (91.2%) of the 102 patients. Seventy-one patients (77.2%) with intermediate follow-up data (range 12 to 117 months) available are asymptomatic and normotensive, with insignificant arm to leg blood pressure gradients (< or = 20 mm Hg). Twenty-one patients (22.8%) with an initial successful result developed an increase in gradient 2 at 86 months after angioplasty, requiring reintervention in 18. Follow-up > 72 months is available in 17 patients, 16 of whom are normotensive and have not required additional intervention. No additional intervention was needed in 88.4% of older children and infants > 7 months old. Ten of the 13 surviving neonates who initially had a successful dilation required reangioplasty or operation 14 days to 10 months (median 4.6 months) after angioplasty. Transverse arch hypoplasia had minimal effect on follow-up blood pressure gradient, whereas isthmic hypoplasia was associated with reintervention in 50%. A small aneurysm was noted in 2 (1.9%) of 102 patients. CONCLUSIONS Balloon angioplasty of native aortic coarctation is effective in infants and older children. In neonates, balloon angioplasty provides effective palliation only. Aneurysm formation is rare; however, lifetime follow-up is warranted.

Congenital Stenosis of Individual Pulmonary Veins: Clinical Spectrum and Unsuccessful Treatment by Transvenous Balloon Dilation

Congenital stenosis of the pulmonary veins is a rare but frequently lethal congenital cardiac abnormality. Eight patients with this malformation were diagnosed, evaluated and treated. All eight patients had associated congenital cardiac defects. Two of the eight died, one of sepsis and one after operative pulmonary venoplasty. In three patients who underwent transvenous balloon catheter dilation of the stenosis the procedure provided immediate but transient relief of the stenosis. The prognosis for symptomatic infants with pulmonary vein stenosis is poor and its treatment an enigma.

Sudden death after repair of tetralogy of Fallot. Electrocardiographic and electrophysiologic abnormalities.

In order to try to determine the mechanism of sudden death in patients after surgical repair of tetralogy of Fallot, electrocardiographic, intracardiac electrophysiologic, and clinical data of 51 children who had postoperative intracardiac electrophysiologic studies were reviewed. Ninety-four percent had developed right bundle branch block (RBBB) and 16 percent had additional left anterior hemiblock (LAH). Two had had transient complete A-V block (CAVB) and one had permanent CAVB. Six had a first degree A-V block and nine had premature ventricular contractions (PVC). Nine patients were found to have prolonged intra-atrial conduction times, four prolonged A-V nodal conduction, four prolonged His- recovery times. Patients with first degree A-V block or LAH did not have an increased incidence of abnormalities on electrophysiologic study. No patient with RBBB and LAH developed complete A-V block or died. Three of the nine patients with PVCs died, one of intractable ventricular fibrillation and two suddenly, presumably of dysrhythmia. All three had significant congestive heart failure. Although late complete A-V block occurs and should be watched for, ventricular dysrhythmias in patients with PVCs may be the cause of most sudden deaths after tetralogy repair. We currently are treating all of our postoperative tetralogy patients who have PVCs with quindine or propranolol

Dysrhythmias after mustard's operation for transposition of the great arteries☆

Abstract Dysrhythmias after Mustards operation for transposition of the great arteries were studied in 60 patients who survived the operation. Only 3 patients consistently had sinus rhythm after the operation. In 54 patients, low voltage atrial waves having the configuration of sinus P waves were seen at one time or another. The incidence of passive dysrhythmias remained nearly the same during the follow-up period. Eight patients showed at various times a specific pattern designated junctional rhythm with right inferior P axis. The mean P axis was directed downward, to the right and anteriorly, occurring either before or after the QRS complex with fixed PR or RP intervals. The characteristics of the junctional rhythm with right inferior P axis remained during subsequent attacks of supraventricular tachycardias. Whereas the incidence of sinus rhythm gradually decreased, the incidence of active dysrhythmias increased during follow-up study. There were no instances of second or third degree atrioventricular (A-V) block. Wolff-Parkinson-White syndrome, type A, developed and persisted in 1 patient. In 5 patients who died, presumably from rhythm disturbances, the histologie features of the sinoatrial (S-A) nodal area were examined. In a control group of normal hearts and 1 specimen with unoperated upon transposition, the S-A node and artery were readily identified. In the necropsy material from patients with transposition who had recently undergone Mustards operation, S-A nodal tissue was identified but fresh hemorrhage and acute inflammation were seen in and about the node. The S-A nodal artery in the 3 patients who died 4 months or more after operation was nearly or completely obliterated by intimai sclerosis and medial hypertrophy. The S-A node was replaced by dense connective tissue and fatty degeneration.

Repeat Dilation of Intravascular Stents in Congenital Heart Defects

BACKGROUND Intravascular (Palmaz) stents have been successfully implanted in patients with congenital and acquired branch pulmonary stenosis. Early results are excellent; however, there is no information on restenosis and repeat dilation in patients with congenital heart disease. The purpose of this study is to review the incidence of restenosis and demonstrate the safety and efficacy of repeat dilation of stents in this group of patients. METHODS AND RESULTS Of 94 patients with 163 implanted stents in this single-center study, 43 patients with 73 implanted stents underwent recatheterization. Only 2 of 73 restudied stents (3%) developed significant restenosis. In 20 patients, 30 stents were redilated. At stent implantation, the mean age of this subgroup was 14.2 years, the mean intraluminal diameter increased from 4.9 to 10.7 mm (P = .0001), and the systolic gradient (mean) across the stent decreased from 52 to 11 mm Hg (P = .0001). At recatheterization (mean, 13 months), all stents were patent. The mean diameter decreased by 1.2 mm (P = .0001), but the increase in the gradient (mean, 3 mm Hg) was not significant (P = .11). After repeat dilation, the diameter increased from 9.5 to 12.2 mm (P = .0001), and the gradient decreased from 14 to 8 mm Hg (P = .0003). The 2 stents with restenosis were redilated successfully. Two patients underwent a successful second redilation of 3 stents at 18 and 26 months. There were no complications. CONCLUSIONS All stents remained patent. The occurrence of significant restenosis is low (3%), and these restenoses can be redilated and/or restented. Repeat dilation of the Palmaz stent implanted in branch pulmonary artery stenosis can be performed with safety and efficacy (94% success rate) up to 3 years after stent implantation.

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patients ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.