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.

Reconstruction of stenotic or occluded iliofemoral veins and inferior vena cava using intravascular stents: re-establishing access for future cardiac catheterization and cardiac surgery.

OBJECTIVES: The study evaluated the safety and efficacy of stent reconstruction of stenotic/occluded iliofemoral veins (IFV) and inferior vena cava (IVC). BACKGROUND: Patients with congenital heart defects and stenotic or occluded IFV/IVC may encounter femoral venous access problems during future cardiac surgeries or catheterizations. METHODS: Twenty-four patients (median age 4.9 years) underwent implantation of 85 stents in 22 IFV and 6 IVC. Fifteen vessels were severely stenotic and 13 were completely occluded. Although guide wires were easily passed across the stenotic vessels, occluded vessels required puncture through the thrombosed sites using a stiff wire or transseptal needle. Once traversed, the occluded site was dilated serially prior to stent implantation. RESULTS: Following stent placement, the mean vessel diameter increased from 0.9 +/- 1.6 to 7.4 +/- 2.6 mm (p < 0.05). Twenty-one of 28 vessels had long segment stenosis/occlusion requiring two to seven overlapping stents. Repeat catheterizations were performed in seven patients (9 stented vessels) at mean follow-up of 1.6 years. Seven vessels remained patent with mean diameter of 6.4 +/- 2.0 mm. Two vessels were occluded, but they were easily recanalized and redilated. Echocardiographic follow-up in two patients with IVC stents demonstrated wide patency. In four additional patients, a stented vessel was utilized for vascular access during subsequent cardiac surgery (n = 3) and endomyocardial biopsy (n = 1). Therefore, 13 of 15 stented vessels (87%) remained patent at follow-up thus far. CONCLUSIONS: Stenotic/obstructed IFV and IVC may be reconstructed using stents to re-establish venous access to the heart for future cardiac catheterization and/or surgeries.

Redilation of endovascular stents in congenital heart disease: factors implicated in the development of restenosis and neointimal proliferation.

OBJECTIVES: We sought to determine the incidence of and risk factors for the development of restenosis and neointimal proliferation after endovascular stent implantation for congenital heart disease (CHD). BACKGROUND: Risk factors for the development of restenosis and neointimal proliferation are poorly understood. METHODS: This was a retrospective review of patients who underwent endovascular stent redilation between September 1989 and February 2000. RESULTS: Of 368 patients who had 752 stents implanted, 220 were recatheterized. Of those 220 patients, 103 underwent stent redilation. Patients were classified into three groups: 1) those with pulmonary artery stenosis (n = 94), tetralogy of Fallot/pulmonary atresia (n = 72), congenital branch pulmonary stenosis (n = 9), status post-Fontan operation (n = 6), status post-arterial switch operation (n = 7); 2) those with iliofemoral venous obstruction (n = 6); and 3) those with miscellaneous disorders (n = 3). The patients' median age was 9.9 years (range 0.5 to 39.8); their mean follow-up duration was 3.8 years (range 0.1 to 10). Indications for stent redilation included somatic growth (n = 67), serial dilation (n = 27) and development of neointimal proliferation or restenosis, or both (n = 9). There was a low incidence of neointimal proliferation (1.8%) and restenosis (2%). There were no deaths. Complications included pulmonary edema (n = 1), hemoptysis (n = 1) and contralateral stent compression (n = 2). CONCLUSIONS: Redilation or further dilation of endovascular stents for CHD is effective as late as 10 years. The risk of neointimal proliferation (1.8%) and restenosis (2%) is low and possibly avoidable. Awareness of specific risk factors and modification of the stent implantation technique, including avoidance of minimal stent overlap and sharp angulation of the stent to the vessel wall and avoidance of overdilation, have helped to reduce the incidence of restenosis.

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 Administration's phase 1 and 2 clinical trials of intravascular stents at Texas Children's 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.

Premature closure of the foramen ovale associated with aortic stenosis, left ventricular dilation with thrombus, and early mortality.

Premature foramen ovale (FO) closure has been postulated as a cause of hypoplastic left heart syndrome. We suggest that premature FO closure is also associated with left ventricular (LV) dilation and LV thrombus formation, and that FO closure in patients with aortic stenosis and LV dilation is a secondary event that occurs later in gestation than that seen with the hypoplastic left heart.

Transcatheter patent ductus arteriosus closure in an infant using the Gianturco-Grifka Vascular Occlusion Device.

A 2-month-old child with Down syndrome and a large patent ductus arteriosus underwent transcatheter closure using a new Food and Drug Administration-approved occlusion device. This device is described, along with its usefulness for other vascular defects.

Transcatheter closure of surgical shunts in patients with congenital heart disease.

The aim of this study was to review contemporary techniques, devices, and results of transcatheter occlusion of surgical shunts in 2 pediatric cardiac programs. Closure of superfluous surgical shunts may reduce cardiac work and risk of endocarditis. Previous studies have shown that transcatheter closure of shunts is feasible, but have not demonstrated acceptable efficacy or safety. In addition, the performance of new techniques and devices has not been reviewed. Between 1993 and 1998, 18 patients with congenital heart disease underwent transcatheter closure of 19 Blalock-Taussig shunts. Detachable and standard Gianturco coils and Gianturco-Grifka vascular occlusion devices were employed. All 19 shunts had complete closure. Eight shunts had initial placement of detachable coils. Five shunts had stents placed that bridged the pulmonary end of the shunts. These 5 and 4 additional shunts had closure by standard coils. Two shunts were closed with Gianturco-Grifka devices. There were no complications, no embolizations, and no requirement for surgery precipitated by the procedures. This review of contemporary techniques, devices, and results suggests that transcatheter occlusion of surgical shunts is effective and safe.

Vascular endothelial growth factor and basic fibroblast growth factor in children with cyanotic congenital heart disease.

OBJECTIVE: Vascular endothelial growth factor and basic fibroblast growth factor are potent stimulators of angiogenesis. Children with cyanotic congenital heart disease often experience the development of widespread formation of collateral blood vessels, which may represent a form of abnormal angiogenesis. We undertook the present study to determine whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor and basic fibroblast growth factor. METHODS: Serum was obtained from 22 children with cyanotic congenital heart disease and 19 children with acyanotic heart disease during cardiac catheterization. Samples were taken from the superior vena cava, inferior vena cava, and a systemic artery. Vascular endothelial growth factor and basic fibroblast growth factor levels were measured in the serum from each of these sites by enzyme-linked immunosorbent assay. RESULTS: Vascular endothelial growth factor was significantly elevated in the superior vena cava (P =.04) and systemic artery (P =.02) but not in the inferior vena cava (P =.2) of children with cyanotic congenital heart disease compared to children with acyanotic heart disease. The mean vascular endothelial growth factor level, determined by averaging the means of all 3 sites, was also significantly elevated (P =.03). Basic fibroblast growth factor was only significantly elevated in the systemic artery (P =.02). CONCLUSION: Children with cyanotic congenital heart disease have elevated systemic levels of vascular endothelial growth factor. These findings suggest that the widespread formation of collateral vessels in these children may be mediated by vascular endothelial growth factor.

Intervention in the thorax in children.

The 1970s and 1980s have been a period of significant growth and innovation in interventional radiology. Interventional procedures that were developed and perfected with adult patients are now being used very successfully in pediatric patients. In comparison with surgical management, interventional procedures generally have lower morbidity and mortality, require shorter hospital stays, and are less expensive. Preprocedural imaging and planning, adequate monitoring and sedation during the procedure, and selection of equipment appropriate for pediatric patients maximizes the chance for a successful outcome.

Cyanotic congenital heart disease with increased pulmonary blood flow.

Pediatricians daily encounter children with systemic cyanosis. The numerous reasons for cyanosis in neonates and infants include pulmonary, hematologic, toxic, and cardiac causes. Congenital heart defects may cause cyanosis. Often, an obvious cardiac reason for cyanosis is decreased PBF; however, several congenital heart defects cause systemic cyanosis with increased PBF, such as TGA, truncus arteriosus, and TAPVR. Because neonates are discharged from the hospital soon after birth, this magnifies the importance of each physical examination. Pediatricians need to remain alert for children who have symptoms of increased PBF with or without cyanosis. With advances in the diagnosis and treatment of patients with CHD, corrective procedures can be performed at many ages.

Transcatheter intervention for the treatment of congenital cardiac defects.

Cardiac catheterization has an illustrious history, originating in 1929 when Werner Forsmann, a surgical resident, performed a heart catheterization on himself. Transcatheter interventional procedures have been performed since the 1960s. The 1st intracardiac procedure to become standard therapy was a balloon atrial septostomy. Skeptics attacked this innovative procedure. However, the balloon septostomy procedure soon became the standard emergency procedure for certain congenital heart defects, and was the impetus for other investigators in the field of transcatheter intervention. We will discuss transcatheter treatment for congenital vascular stenoses and vascular occlusion.

Development of advanced pulmonary vascular disease in D-transposition of the great arteries after the neonatal arterial switch operation.

We report the case of a neonate with D-transposition of the great arteries who, after undergoing an uneventful arterial switch operation at the age of 4 days, was found at the age of 42 months to have developed advanced pulmonary vascular disease. Because the arterial switch operation was performed when our patient was only 4 days old, this case challenges the hypothesis that postnatal hemodynamics alone dictate the development of advanced pulmonary vascular disease in infants and children with transposition of the great arteries.

Coronary artery fistula. Management and intermediate-term outcome after transcatheter coil occlusion.

During the last 2 decades, transcatheter occlusion of coronary artery fistulae has developed into a safe and effective therapy for children. This procedure avoids the need for open surgical repair and the attendant complications of cardiopulmonary bypass and median sternotomy. The long-term outcome in patients after transcatheter occlusion remains unknown. We describe the intermediate-term progress of 4 such patients after coil occlusion of coronary artery fistulae. Persistent coronary artery dilatation was present in all patients reviewed, as late as 4 years after occlusion.

Balloon pulmonary valvuloplasty and stent implantation. For peripheral pulmonary artery stenosis in Alagille syndrome.

We report the case of a patient with Alagille syndrome and severe pulmonary valve and bilateral pulmonary artery branch stenosis. In this patient, transcatheter balloon pulmonary valvuloplasty combined with bilateral pulmonary artery angioplasty and stent placement provided excellent immediate results and long-term improvement.

Sizing of atrial septal defects to predict successful closure with transcatheter cardioSEAL device.

We conducted this retrospective study to compare methods for measuring atrial septal defects and to identify factors affecting echocardiographic measurement of such defects before transcatheter closure with the CardioSEAL'Septal Occluder. We reviewed the records of patients considered for device placement at our institution from January 1997 to April 1999. Atrial septal defect size was measured by transthoracic and transesophageal echocardiography; the stretched diameter was measured during catheterization by fluoroscopy and transesophageal echocardiography. The stretched-diameter fluoroscopic measurement was used for device size selection. Analysis of variance was used to calculate the effect of size, age, and size-by-age interaction. Thirty-one patients (3.3 to 72 years of age) underwent transthoracic and transesophageal echocardiography One patient was excluded from catheterization because of a 25-mm septal defect as indicated by transesophageal echocardiography (our maximum diameter, 15 mm). Thirty patients underwent transcatheter stretched-diameter sizing; 5 were excluded from device implantation because of defects >20 mm by stretched-diameter fluoroscopy (4) or septal length insufficient for device support (1). Implantation was successful in 23/25 patients; 2/23 had a residual shunt. In patients with available results (26/30), the stretched diameter was the same whether measured by stretched-diameter fluoroscopy or transesophageal echocardiography (P=0.007 R square=0.963). Compared with stretched-diameter fluoroscopy, precatheterization transthoracic and transesophageal echocardiography underestimated defect size by a mean of 22% and 13.2%, respectively. When data from those same tests were compared in defects of < or =0 mm and > 10 mm, transthoracic and transesophageal echocardiography were reliable predictors (P=0.003 and P=0.05, respectively) of stretched-diameter size in defects < or =0 mm.

Pulmonary venous stenosis as an associated manifestation of left-sided obstructive lesions in an infant with partial atrioventricular canal defect.

Patients with partial atrioventricular canal defect (PAVC) who present with congestive heart failure within the first year of life are usually found to have associated left-sided obstructive lesions. We present a case of a child with PAVC who subsequently developed progressive pulmonary venous stenosis as a manifestation of her disease.

Use of tissue plasminogen activator for femoral artery thrombosis following transcatheter coil occlusion of patent ductus arteriosus.

Femoral artery thrombosis is an uncommon but potentially serious complication following pediatric cardiac catheterization. Management options include heparin infusion, thrombolytic therapy, and surgical thrombectomy. The use of thrombolytic agents following coil occlusion of shunts, collaterals, and patent ductus arteriosus (PDA) may be successful in resolving the femoral arterial thrombosis but may also reopen the device-occluded vessel. We report the successful use of tissue plasminogen activator for management of femoral artery thrombosis in a child following transcatheter PDA coil occlusion in which the PDA remained occluded.

Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response.

Inhaled nitric oxide (NO) has been used in the preoperative evaluation of patients with congenital heart disease and pulmonary hypertension. The purpose of this study was to characterize responses in pulmonary vascular resistance (PVR) to oxygen and increasing doses of NO during cardiac catheterization and to determine if any related factors affect the response of the pulmonary vascular bed to NO. A prospective analysis of 42 patients (median age, 3.0 years) with congenital heart disease and pulmonary hypertension who underwent NO testing was performed. Systemic vascular resistance (SVR) and PVR were assessed in room air, 100% oxygen, and oxygen plus 20, 40, and 80 parts per million (ppm) NO. Changes in pulmonary artery pressure, PVR, and SVR were assessed. The response to NO was then correlated to individual patient's age, gender, type of heart defect, the presence of trisomy 21, and baseline PVR/SVR. There was a greater decrease in PVR and PVR/SVR with 20 ppm NO than with oxygen alone. There was no additional decrease at 40 or 80 ppm NO. There was no correlation between age, gender, type of congenital heart disease, and baseline PVR/SVR ratio with the degree of response to NO. Patients with trisomy 21 had less of a response to NO (p = 0.017) than patients without trisomy 21. There is no difference in determining PVR response with doses of NO beyond 20 ppm during cardiac catheterization. Age, gender, and baseline PVR/SVR ratio are not associated with responsiveness to NO. Patients with trisomy 21 may be less responsive to NO.

Patent foramen ovale morphology and impact on percutaneous device closure.

We sought to evaluate the effects of atrial septal and patent foramen ovale (PFO) morphology on the efficacy of transcatheter closure. We performed a retrospective analysis of all patients who underwent PFO device closure from January 1997 to January 2002. Forty-seven patients underwent percutaneous closure of a PFO with a median age of 45 years (range, 8-75) and weight of 76 kg (range 28-115). The septal morphology was flat in 33 and aneurysmal in 14 patients. The PFO morphology was a simple flap in 20 and complex in 27 patients. Complex morphologies included long-tunnel PFO (n = 15), coexistent small atrial septal defect (ASD) (n = 5), and aneurysmal septum without a tunnel or ASD (n = 7). Nonstretched PFO diameters were significantly smaller than stretched (4.8 +/- 1.1 mm vs 11.6 +/- 3.8 mm, p < 0.01). Median device size: stretched diameter ratio (DS:SD) was 3.7:1 (range, 2.2-9.1). The DS:SD ratio was significantly higher in patients with complex PFO (mean, 3.9:1 vs 2.6:1; p < 0.05). Device placement was successful in all patients. Five patients required transeptal puncture of the foraminal flap in long-tunnel PFOs. Effective closure on follow-up was achieved in 45 patients (95%). Of the 2 patients with residual shunts, 1 had a complex PFO and the other a simple PFO. Two patients (5%) experienced recurrent neurologic symptoms after device closure despite having no residual shunt by echocardiography. Complex PFO did not increase risk of residual shunt or recurrent neurologic symptoms after device closure.