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.

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.

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.

Amplatzer septal occluder closure of secundum atrial septal defects in the presence of persistent left superior vena cava to coronary sinus.

Secundum atrial septal defects (ASDs) are routinely closed using transcatheter devices. In patients with left superior vena cava (LSVC) draining to the coronary sinus (CS), the device must not obstruct CS drainage. We report five cases of successful ASD device closure without obstructing flow from the LSVC or dilated CS.

Growth of an atrial septal defect: missing the window for transcatheter closure.

We describe a patient diagnosed with a small atrial septal defect (3-4 mm) that had grown to 24 mm in diameter over a six-year period. Timing of transcatheter intervention may be affected by growth of the atrial septal defect and this reports the first case of an atrial septal defect outgrowing the possibility of transcatheter closure.

Natural history of growth of secundum atrial septal defects and implications for transcatheter closure.

OBJECTIVE: To investigate the natural history of secundum atrial septal defects (ASDs) over several years using serial echocardiographic studies. METHODS: All patients with isolated secundum ASDs who had serial transthoracic echocardiograms at Texas Children's Hospital, Houston, Texas, from January 1991 to December 1998 were identified. Patients with fenestrated or multiple ASDs, other congenital heart defects, or less than a six month interval between echocardiograms were excluded. There were 104 patients eligible for inclusion in the study. Studies were reviewed by two echocardiographers (blinded) and the maximal diameter was recorded. Defects were defined as small (> 3 mm to < 6 mm), moderate (> or = 6 mm to < 12 mm), or large (> or = 12 mm). ASDs that grew > or = 20 mm were defined as having outgrown transcatheter closure with the device available to the authors' institution. RESULTS: ASD diameter increased in 68 of 104 patients (65%), including 31 patients (30%) with a > 50% increase in diameter. Spontaneous closure occurred in four patients (4%). Thirteen defects (12%) increased to > or = 20 mm. One fifth of the patients studied had an insufficient atrial rim by transthoracic echocardiogram to hold an atrial septal occluder. The only factor associated with significant growth of ASDs was initial size of the defect. ASD growth was independent both of age at diagnosis and when indexed to body surface area. CONCLUSIONS: Two thirds of secundum ASDs may enlarge with time and there is the potential for secundum ASDs to outgrow transcatheter closure with specific devices. Further development in devices and general availability of devices capable of closing larger ASDs should circumvent this problem.

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.

Safety of magnetic resonance imaging immediately following Palmaz stent implant: a report of three cases.

Magnetic resonance imaging (MRI) can provide important information on patients with congenital heart defects. There is some reluctance to perform MRI acutely following intravascular stent implant, due to concerns of distortion or movement of the stent in the magnetic field. We report on three patients who underwent MRI evaluation less than 14 days following Palmaz stent implant in the pulmonary arteries and superior vena cava, with no acute adverse outcome or long-term problems.

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.

A sword for the left hand: an unusual case of left-sided scimitar syndrome.

Scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classically involves the right side. A rare case of left-sided scimitar syndrome is described in an asymptomatic child, with a review of the literature.

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.

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.

Steerable control of the eustachian valve during transcatheter closure of secundum atrial septal defects.

Over the past decade there has been increased use of transcatheter devices for closure of secundum atrial septal defects. The presence of a large eustachian valve complicating transcatheter closure has not been described. We describe four patients with prominent eustachian valves, in three of whom we employed a simple technique to obtain control of the eustachian valve during device placement using transesophageal echo guidance.

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.

18-year experience with transseptal procedures through baffles, conduits, and other intra-atrial patches.

The presence of an intra-atrial patch (IAP) has been considered a relative contraindication to transseptal puncture (TSP). The purpose of this study is to determine the efficacy and safety of the TSP through baffles, conduits, pericardial patches and other prosthetic materials in the intra-atrial septum. We reviewed the records of all pediatric patients with IAP who underwent TSP at Texas Children's Hospital from November 1979 through February 1998. The review included the cardiac diagnoses, indications for TSP, technical difficulties and follow up echocardiograms specifically addressing residual atrial shunts A total of 1958 TSP were performed. Thirty-nine patients had IAP. Cardiac diagnoses in those 39 patients included D-transposition of the great arteries after Mustard (10) or Senning procedure (6), single ventricle variant post-Fontan operation (4), total anomalous venous return repair (4), atrioventricular canal repair (9) and atrial septal defect with patch repair (6). Patients' age ranged from 1-31 years (median 7 years). The duration from the time of surgical repair to TSP ranged from 0.1-21 years (median 5 years). Indications for TSP included diagnostic and therapeutic intervention for pulmonary venous obstruction (12), creation of a baffle fenestration (2), prosthetic mitral valve evaluation (1), left ventricular outflow tract evaluation (1), access the left heart for hemodynamic evaluation (23). The IAP was traversed in 38/39 patients (97.5%), followed by diagnostic or therapeutic prograde left-heart catheterization. No complications were encountered. Follow up echocardiography in 30/38 PTS demonstrated no residual shunting across the atrial septum except for two cases in which the atrial baffle had been intentionally fenestrated. Transseptal puncture through an intra-atrial patch is a safe procedure. This technique is effective in permitting diagnostic and therapeutic left heart catheterization and does not result in residual shunting through the patch.

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.

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.

A technique to prevent newly implanted stent displacement during subsequent catheter and sheath manipulation.

We describe a novel technique to prevent the displacement or migration of a newly implanted stent as a consequence of any subsequent catheter and sheath manipulation during the same catheterization procedure. The technique involves reinflation of the dilation balloon within the stent immediately after implant followed by advancing the long delivery sheath carefully over the balloon as the balloon is slowly deflated within the stent. The technique was used successfully in 78 stents in 30 patients without stent dislodgment or migration.