Transcatheter creation of an atrial septal defect using radiofrequency perforation.

Transseptal perforation using radiofrequency energy was performed successfully in a patient with congenital heart disease and a thickened interatrial septum. This was followed by balloon dilatation of the atrial septal defect. Radiofrequency is presented as a alternative to standard transseptal needle puncture. Cathet Cardiovasc Intervent 2001;54:83-87.

Outcomes of transcatheter balloon angioplasty of obstruction in the neo-aortic arch after the Norwood operation.

Obstruction of the reconstructed aortic arch, or the neoaortic arch, is now known to be an important factor increasing mortality after the Norwood operation for hypoplastic left heart syndrome. Transcatheter balloon angioplasty has been shown to provide effective relief of both native aortic coarctation and obstructions of the aortic arch occurring subsequent to therapeutic intervention. We sought to determine the outcomes of balloon angioplasty used as an initial treatment for obstruction of the neoaortic arch occurring after the Norwood operation. We gathered the characteristics of 58 patients with such obstruction from 8 institutions, noting procedural factors and outcomes of initial balloon dilation. Obstruction occurred at a median interval of 4 months, with a range from 1.5 months to 6.3 years, after a Norwood operation. Ventricular dysfunction was present before dilation in 13 patients. Mean peak to peak systolic pressure gradients were acutely reduced from 31+/-20 mm Hg to 6+/-9 mmHg (p<0.001), with outcome subjectively judged to be successful in 89%. Three patients with pre-existing ventricular dysfunction died within 48 hours of dilation. There were 10 additional deaths during the period of follow-up, with Kaplan Meier estimates of survival after intervention of 87% at 1 month, 77% at 12 months, and 72% after 15 months. In addition, 9 patients required re-intervention during the period of follow-up, with Kaplan Meier estimates of freedom from re-intervention after dilation of 87% at 6 months, 78% at 12 months and 74% after 18 months. Although transcatheter dilation of neoaortic arch obstructions after Norwood operation is successful, there is a high risk of re-intervention and ongoing mortality in this subgroup of patients. Close follow-up is recommended.

Comparison of two transcatheter closure methods of persistently patent arterial duct.

A randomized trial of arterial duct occlusion with a double umbrella (DU) or wire coil (WC) was undertaken for patients <18 years of age, weighing >10 kg with isolated ducts < or = 3 mm in diameter. Baseline, procedural, and outcome characteristics were compared in an intention-to-treat analysis according to randomization group. From 40 consecutively screened patients, 2 were not enrolled due to a ductal diameter of >3 mm on initial aortography, 38 patients were randomized to either the DU (n = 20) or WC (n = 18) groups. The groups did not differ significantly with respect to age, weight, gender, duct size, type, or branch pulmonary artery diameters. Crossover occurred only in the DU group, where 4 patients (20%) had a ductal diameter of < or = 1 mm and could not be entered for umbrella placement. All remaining DU group patients had ductal diameters of > or = 1.3 mm (p <0.0001). There were no embolizations or secondary implants in the DU group, but in the WC group there was 1 early and 1 late embolization, with 6 patients (33%) with > or = 2 coils. Mean times for the procedure (DU 68+/-19 minutes; WC 65+/-27 minutes; p = 0.70) and fluoroscopy (DU 14+/-4 minutes; WC 11+/-6 minutes; p = 0.22) did not differ significantly. Angiographic duct closure was documented in 4 of 13 patients (31%) of the DU group and 4 of 18 patients (22%) of the WC group (p = 0.69). Combined with an echocardiogram, closure in 11 of 17 patients with DU (65%) and 13 of 18 patients with WC (72%) (p = 0.64) was documented before hospital discharge. One WC group patient received thrombolytic therapy for a femoral artery thrombus. Follow-up at a median of 6.5 months (range 3.2 to 37) showed closure by Doppler echocardiography in 15 of 19 patients with DU (79%) versus 14 of 18 patients with WC (78%) (p = 1.0). Thus, with a tendency toward similar procedural characteristics and outcomes, the higher cost of the DU system compared with coil implants favors the use of coils for closure of the small arterial duct.

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.

Transcatheter closure of atrial septal defects using the Cardio-Seal implant.

OBJECTIVE: To review the outcomes of transcatheter closure of atrial septal defects using the Cardio-Seal implant. DESIGN: A prospective interventional study. SETTING: Tertiary referral centre. PATIENTS: The first 50 patients (median age 9.7 years) who underwent attempted percutaneous occlusion. INTERVENTIONS: Procedures were done under general anaesthesia and transoesophageal guidance between December 1996 and July 1998. MAIN OUTCOME MEASURES: Success of deployment, complications, and assessment of right ventricular end diastolic diameter, septal wall motion, and occlusion status by echocardiography. RESULTS: The median balloon stretched diameter was 14 mm. Multiple atrial septal defects were present in 11 patients (22%) and a deficient atrial rim (< 4 mm) in 19 (38%). In four patients (8%), a second device was implanted after removal of an initially malpositioned first implant. There were no significant immediate complications. All patients except one were discharged within 24 hours. At the latest follow up (mean 9.9 months) a small shunt was present in 23 patients (46%), although right ventricular end diastolic dimensions (mean (SD)) corrected for age decreased from 137 (29)% to 105 (17)% of normal, and septal motion abnormalities normalised in all but one patient. No predictors for a residual shunt were identified. Supporting arm fractures were detected in seven patients (14%) and protrusion of one arm through the defect in 16 (32%), the latter being more common in those with smaller anterosuperior rims. No untoward effects resulted from arm fractures or protrusion. There were no complications during follow up, although five patients (10%) experienced transient headaches. CONCLUSIONS: The implantation of the Cardio-Seal device corrects the haemodynamic disturbances secondary to the right ventricular volume overload, with good early outcome.

Antegrade transcatheter closure of coronary artery fistulae using vascular occlusion devices.

Two children (a 9 year old boy and a 2.5 year old girl) with coronary artery fistulae communicating with the right ventricle underwent successful transcatheter occlusion using an antegrade technique. A Rashkind double umbrella device was used in one case and an Amplatzer duct occluder in the other.

Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections.

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn.

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 +/- 0.6 days and weight 3.4 +/- 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0-13.0 mm) and Z-value -0.85 (range: -4.5-1.0). The mean right ventricular systolic pressure fell from 117 +/- 16 to 55 +/- 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 +/- 0.33 to 0.82 +/- 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3-23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4-32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair.

Unraveling of a Gianturco coil during reocclusion of a patent ductus arteriosus.

The Gianturco spring coil has been employed in the settling of the small arterial duct and for reocclusion of residual shunts; the ability to manipulate the coil is advantageous for optimal results. This case study reports an associated complication of unraveling of the coil. Careful attention to coil appearance during manipulation and the use of 0.038-inch coils may reduce this risk.

Clinical impact of transcatheter closure of secundum atrial septal defects with the double umbrella device.

The clinical impact of transcatheter closure of the isolated secundum atrial septal defect was reviewed. Closure by echocardiographic evaluation was 23 +/- 14% at 6 months, 49 +/- 16% at 2 years, and 64 +/- 16% at 4 years, and right ventricular end-diastolic dimensions in patients without residual shunts did not differ significantly from those with residual shunts.

Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up.

OBJECTIVES: This study sought to evaluate ventricular and valvular morphologic changes, hemodynamic consequences and clinical outcomes of pulmonary balloon valvotomy performed in the neonatal period. BACKGROUND: Previous studies support percutaneous balloon valvotomy as the management option of choice for infants and children with valvular pulmonary stenosis. Less information is available to define the impact of valvotomy on the clinical course when performed in the neonatal period. METHODS: Patient records, catheterization data, cineangiograms and selected echocardiograms (initial and most recent studies) of 37 consecutive neonates undergoing attempted balloon dilation were reviewed. RESULTS: Dilation was accomplished in 35 (94%) of 37 attempts. Immediately after dilation, the transvalvular peak to peak systolic gradient decreased from 60 +/- 22 mm Hg (mean +/- SD, range 20 to 100) to 11 +/- 10 mm Hg (range 0 to 45) (p < 0.0001), and the right ventricular/aortic systolic pressure ratio decreased from 1.25 +/- 0.43 (range 0.5 to 2.6) to 0.66 +/- 0.22 (range 0.2 to 1) (p < 0.0001). Oxygen saturation measured by percutaneous oximetry increased from 80 +/- 7% to 92 +/- 4% (p < 0.0001). Three patients died (8%), and two required repeat balloon dilation. At the follow-up visit (median 31 months, range 6 months to 8 years), the estimated peak instantaneous Doppler gradient was 15 +/- 9 mm Hg (range 6 to 36). Thickening of valve leaflets, initially present in 93% of patients, was found in only 4%, and leaflet mobility improved in all. Hypoplasia of the right ventricle, initially present in 31%, was found in only 4% at the latest evaluation. Pulmonary annulus diameter Z score increased from -3 +/- 1.0 to 0 +/- 0.1 (p < 0.0001). Freedom from reintervention was 90%, 84% and 84% at 1, 2 and 8 years, respectively. CONCLUSIONS: These data support the application of balloon valvotomy as the initial intervention in the treatment algorithm for neonates with critical pulmonary valve stenosis. Medium-term follow-up observations demonstrate sustained hemodynamic relief and support maturation of the right ventricle and pulmonary valve annulus, with the expectation of a good long-term outcome.

Endovascular stents in children under 1 year of age: acute impact and late results.

OBJECTIVES: To review efficacy and safety of endovascular stent implants in children < 1 year of age with congenital heart lesions. DESIGN: Retrospective study of patients in a tertiary care setting. PATIENTS: 26 children (median age of 4.7 months, range 2 days to 1 year) with various vascular obstructive lesions. INTERVENTION: Percutaneous or intraoperative implantation of balloon expandable endovascular stents. RESULTS: Optimal stent placement was obtained in 31 of the 37 deployed implants. Complications resulted primarily from stent malpositioning and one episode of bleeding at a puncture site. Stent implantation in three patients with a restrictive arterial duct allowed for patency and five patients with conduit stenosis had mean (SD) right ventricule to systemic artery pressure ratios falling from 0.99 (0.20) to 0.52 (0.18) (P < 0.05). In 10 patients with pulmonary artery stenosis, the mean vessel diameter increased from 2.8 (0.9) mm to 5.8 (1.4) mm (P << 0.001). No clinical improvement was seen in two patients because of diffuse hypoplasia of the pulmonary vessels. Nine of 10 patients with miscellaneous obstructive lesions improved clinically. Recatheterisation was performed in 19 patients (median 8 months, range 12 days to 28 months) and 11 patients required redilatation (17 stents). CONCLUSIONS: Stent implantation is technically feasible in infants and under specific circumstances may provide an alternative to surgical palliation or avoid reoperation. The long term impact on clinical course, however, involves further interventions directed at stent management.

Experience with repair of congenital heart defects using adjunctive endovascular devices.

The use of endovascular devices as an adjunct to repair of congenital heart anomalies represents a novel but unproven therapeutic approach. Intraoperative implantation of pulmonary arterial stents (5 to 15 mm diameter) was done in 11 patients with pulmonary atresia with ventricular septal defect (n = 4), classic tetralogy of Fallot (n = 2), truncus arteriosus (n = 1), hypoplastic left heart syndrome (stage II [n = 1] and stage III [n = 1] Norwood procedure), and miscellaneous pulmonary arterial stenoses (n = 3), as well as in patients with congenital (n = 1) and postoperative (n = 3) pulmonary venous obstruction and in 1 patient with combined pulmonary arterial and venous obstruction. The stents were effective at achieving immediate patency in all patients. There were two early deaths, one related to acute thrombosis of a small-diameter left pulmonary artery stent. Reintervention because of stent-related pulmonary arterial stenosis was frequently necessary. In five of seven patients who survived more than 1 month after implantation of stent size 8 mm or smaller severe stent-related pulmonary arterial obstruction developed. In four of the five patients with pulmonary vein stent implantation intractable obstruction developed, resulting in death in all three patients who had bilateral pulmonary vein stent implantation. Intraoperative occlusion of apical muscular ventricular septal defect with use of a clamshell device inserted from the right atrial approach was accomplished in four patients. One patient who underwent associated aortic arch reconstruction died as a result of left ventricular hypoplasia. The results in the remaining three patients were favorable on the basis of absence of significant late residual intraventricular shunting, left ventricular dysfunction, or arrhythmia. We conclude that recurrent intraluminal obstruction as a result of neointimal hyperplasia appears to be an eventual certainty in currently designed small-diameter endovascular stents. For this reason, we would recommend standard surgical techniques for repair of obstructive lesions of the pulmonary arterial confluence to maximize growth potential. Device occlusion of muscular ventricular septal defects is feasible but probably only indicated for complex cases of ventricular septal deficiency that otherwise necessitate a left ventriculotomy.

Right to left interatrial communications after the modified Fontan procedure: identification and management with transcatheter occlusion.

OBJECTIVE: To describe unusual venous communications from the right to the left atrium resulting in cyanosis after the modified Fontan procedure, and their management with transcatheter occlusion. METHODS: Between September 1992 and November 1994, eight patients were assessed for persistent cyanosis after a modified Fontan procedure. Desaturation was found to be caused by unusual venous shunts originating at atrial level, and transcatheter occlusion with either a double umbrella or coil was attempted. RESULTS: Three types of venous channels were identified. The first type of communication consisted of thin long tortuous channels originating from the right atrial wall, and draining into the left atrium through a capillary network. The second type of communication was in the superior anterior portion of the atrial baffle, incorporating the pectinate muscles of the right atrium, draining into the neoleft atrium. These channels were shorter and often fanned out into small vessels toward the right atrial appendage. In each instance, the shunts were in the superior suture line of a lateral tunnel modification of the Fontan procedure. The third type of communication originated from the inferior vena cava, connecting inferior phrenic veins to pericardial veins and subsequently to the left atrium, at or close to the ostium of the left pulmonary veins. Before device occlusion, the room air aortic oxygen saturation was 88(SD 4)% (range 84% to 94%), and increased to 95(3)% (range 91% to 100%) following occlusion (PL << 0.001). The mean right atrial pressure was 14(4)mm Hg and remained unchanged after occlusion. In six patients there was complete shunt obliteration, while in two both occluded with umbrella devices, a small residual leak persisted. No complication occurred during or immediately after catheterisation. CONCLUSIONS: Unusual venous communications can evolve after the Fontan procedure, resulting in the development or persistence of cyanosis. Some of these communications may be present preoperatively as normal veins draining into the right atrium, enlarging with the increased atrial pressure after surgery. These observations affect long term function after the Fontan procedure. Transcatheter occlusion of these communications is technically feasible and effective, although recurrence may occur.

Transcatheter patent ductus arteriosus occlusion: application in the small child.

OBJECTIVES: This study intended to evaluate application of transcatheter occlusion of the patent ductus arteriosus in children < 10 kg body weight. BACKGROUND: Transcatheter occlusion of the patent ductus arteriosus in the child weighing > 10 kg has been proved safe and effective. METHODS: We reviewed 74 consecutive patients weighing < 10 kg (median 8.1 kg, range 3.98 to 10) and aged 4 to 30 months (median 13 months) who underwent patent ductus arteriosus occlusion between June 1986 and November 1992. A modification of the delivery system to facilitate application in small children is described. RESULTS: A 12-mm device was implanted in 50 patients and a 17-mm device in 24. Three device embolizations occurred early in the experience, and one required removal because of hemolysis associated with a moderate residual shunt. One 17-mm device was removed at catheterization because of acute compromise to left pulmonary artery flow after implantation. Prevalence of residual shunting was 33% at 6 months, 20% at 12 to 18 months and 17% at 2-year follow-up and was not related to device, age, weight or size of the patent ductus. Altered flow to the left pulmonary artery was noted in seven patients (9.9%), with more significant compromise associated with the 17-mm device. CONCLUSIONS: Transcatheter patent ductus arteriosus occlusion is feasible in the small child < 10 kg, particularly with the use of a modified delivery system. However, implantation of a 17-mm device in this patient population may impair flow to the left lung and should be reserved for the symptomatic child.

Transcatheter occlusion of the patent ductus arteriosus in the presence of mild isthmal hypoplasia.

Between January 1990 and February 1992, 5 asymptomatic patients were identified with hypoplasia of the aortic isthmus at the time of cardiac catheterization for closure of a small patent ductus arteriosus. Isthmal diameters measured from lateral aortograms were 62 +/- 11% of age-matched controls. All patients had a small communication occluded with a 12 mm device and remained asymptomatic at 6-30 months follow up. Residual shunting was demonstrated by color Doppler in 2 patients. No child has developed an arm-leg systolic blood pressure gradient or systemic hypertension. Following device placement, Doppler interrogation across the isthmus in 2 patients demonstrated a velocity shift of 2.1 m/s. Transcatheter occlusion of the patient ductus arteriosus associated with mild isthmal hypoplasia of the aortic arch appears safe and effective and does not result in a clinically significant impairment to isthmal flow in short-term follow-up.

Retrobronchial vertical vein in totally anomalous pulmonary venous connection to the innominate vein and its specific occurrence in right isomerism.

Recently, a case of right isomerism with totally anomalous pulmonary venous connection (TAPVC) to the innominate vein through a vertical vein that ascended in the mediastinum posterior to the left-sided main bronchus was encountered. This retrobronchial vertical vein that has not been described previously in patients with TAPVC to the innominate vein prompted the performance of a retrospective analysis of 44 cases with TAPVC to the innominate vein in which angiography had been performed. The 44 cases consisted of 36 patients with situs solitus, 7 with right isomerism, and 1 with left isomerism. The spatial relation of the vertical vein to the adjacent pulmonary artery and main bronchus could be defined in 41 cases. The vertical vein was retrobronchial in 4 of 5 cases with right isomerism in which the location of the vertical vein could be defined with certainty. No case with situs solitus or left isomerism was associated with a retrobronchial vertical vein. In all 4 patients with a retrobronchial vertical vein, angiographic evidence of obstruction of pulmonary venous drainage was present, and was due to an extrinsic bronchial compression in 2 and to an intrinsic narrowing of the vertical vein in 2.