Novel arterioplasty of severe left pulmonary artery stenosis using native main pulmonary artery.

There are multiple approaches described for the repair of stenosed branch pulmonary arteries. Regardless of the technique used, restenosis is common. We describe a case of severe left pulmonary artery stenosis repaired with a novel technique using the native main pulmonary artery which was transected and turned down to create a direct anastomosis with the left pulmonary artery. This tension-free tissue to tissue anastomosis resulted in hemodynamically gratifying results with the potential for growth.

One stage combined repair of intracardiac defects with vascular ring employing sternotomy approach.

We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.

Transcatheter closure of elongated and pulmonary hypertensive patent arterial duct in infants using Amplatzer vascular plug II.

OBJECTIVE: To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants. INTRODUCTION: Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems. METHOD: This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II. RESULTS: Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome. CONCLUSIONS: Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

Long-term follow-up of an adult with left pulmonary artery stenting and absent right pulmonary artery: a case report.

BACKGROUND: Unilateral absence of pulmonary artery is a rare congenital abnormality with varied clinical presentations. We present a unique case of congenital absence of right pulmonary artery (RPA) with left pulmonary artery (LPA) origin stenosis without any intracardiac or extracardiac lesion and its long-term follow-up. CASE SUMMARY: A 68-year-old woman underwent successful LPA stenting 23 years back for absent RPA and LPA origin stenosis. She was recently evaluated for breathlessness, almost two decades after the procedure. On evaluation, she was found to have severe pulmonary hypertension (Group 5) without any distortion of the LPA and with normally functioning stent. DISCUSSION: This report discusses the long-term outcome of stenting in the setting of severe stenosis of a single pulmonary artery. Over the years, the patient went on to develop severe segmental pulmonary hypertension (Group 5).

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

OBJECTIVES: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS: The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS: Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results.

Dual pathology causing severe pulmonary hypertension following surgical repair of total anomalous pulmonary venous connection: Successful outcome following serial transcatheter interventions.

Surgical repair of total anomalous pulmonary venous connection (TAPVC) can be complicated by the development of pulmonary venous stenosis later on. In addition, the vertical vein, if left unligated, can remain patent and lead to hemodynamically significant left to right shunting. We report an infant who required transcatheter correction of both these problems after surgical repair of TAPVC.

Transposition of the Great Arteries With Total Anomalous Pulmonary Venous Connection.

Transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is a rare association. Very few such cases have been reported. Among them 1 patient underwent anatomic repair. The rest received repair at the atrial level with either a modified Senning technique or a Mustard technique. We report a rare combination of TGA, TAPVC draining to the coronary sinus, and left juxtaposition of the atrial appendages with a diminutive right atrium. The anomalies were successfully repaired by anatomic correction.

Modified pediatric Bentall procedure: A novel technique in a rare case.

Aneurysms of ascending aorta are rarely seen in pediatric age group. Only few cases with Marfans syndrome have been reported in the literature. Preferred treatment for these children has been the standard Bentall procedure (aortic root replacement with composite graft prosthesis). We report a 4-year-old male child with huge aneurysm of ascending aorta and aortic root dilation with severe aortic regurgitation, having phenotypic features of Loeys-Dietz syndrome type I. He underwent Bentall procedure with a novel modification (medial trap-door technique for coronary reimplantation). Short-term result of this procedure is encouraging and he is asymptomatic for the last 14 months of follow-up.

Spontaneous closure of right pulmonary artery-to-left atrium fistula: a case report.

Direct communication between the right pulmonary artery and the left atrium is rare. We report a case that was first detected in utero and confirmed by a two-dimensional echocardiographic study performed postnatally. The patient remained asymptomatic, and hence was managed conservatively. The fistula gradually decreased in size and closed spontaneously at 15 months of age. The rarity of the case is discussed.

Transcatheter closure of large patent ductus arteriosus with severe pulmonary arterial hypertension: Short and intermediate term results.

OBJECTIVE: To assess the efficacy and safety of transcatheter closure (TCC) of patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PHT). BACKGROUND: TCC of small and moderate-sized PDAs is well established. However, there is a paucity of data on TCC of large PDA with severe PHT. METHODS: This is a retrospective observational study of 76 patients with large PDA and severe PHT who were referred for TCC. Multiple clinical and investigational parameters were evaluated to decide the reversibility of PHT. Following the TCC, patients were serially followed up to assess the efficacy and safety of closure and its impact on PHT. RESULTS: Of 76 patients, nine were found to have PDA with Eisenmenger's syndrome. Of remaining 67, two were thought to have irreversible PHT based on hemodynamic data obtained after balloon occlusion of the duct. Sixty five patients, who eventually underwent TCC of PDA, had a median age of 9.1 years (range 1 month to 40 years). The weight ranged between 2.5 to 62 kg (median 14 kg). The PDA size was 9.1 ± 4.6 mm. The mean systolic pulmonary artery pressure was 66.9 ± 15.3 mm Hg. Duct occluder was used in 63 and muscular ventricular septal defect closure device in 2. The follow up was available in 56 (86%) with a mean follow up period of 65 ± 34 months. All the patients had complete closure of the PDA at 6 months follow up. Mild obstruction of left pulmonary artery (n=3) and aortic isthmus flow (n=6) was noted at the time of discharge. During the follow up, partial or complete resolution of PHT was observed in all the patients in whom Doppler-derived right ventricular systolic pressure was recorded (available in 40 of 56 patients). CONCLUSIONS: TCC of large PDA with severe PHT and significant left to right shunt was found to be effective and safe in the short and intermediate term.

Transcatheter closure of perimembranous ventricular septal defects using amplatzer asymmetric ventricular septal defect occluder: preliminary experience with 18-month follow up.

BACKGROUND: This study reports our experience in the nonsurgical closure of perimembranous ventricular septal defects in children and adolescents with the Amplatzer asymmetric ventricular septal defect occluder and the outcome of an 18-month follow up. METHODS AND RESULTS: Twenty patients (median age:10 years; median weight:32 kg) with perimembranous ventricular septal defect were selected for transcatheter closure with the Amplatzer device. The prosthesis diameter chosen was 1-2 mm larger than the largest measured diameter of the defect on transesophageal echo (TEE). All patients were put on oral aspirin (5 mg/kg/day in children and 150 mg/day in adults) five days prior to and for six months after closure. Follow-up evaluation at 48 hr and 1, 6, 12 and 18 months included clinical examination, electrocardiogram, and a transthoracic echocardiogram. The mean defect diameter on color flow mapping on TEE was 7.1 +/- 2.3 mm. The device diameter ranged from 6-14 mm (median = 8 mm). One patient developed an anaphylactic reaction to contrast. The procedure was successful in 17 out of 19 patients where it was attempted (89.4%). In two patients with associated significant aortic valve prolapse and mild aortic regurgitation the device could not be successfully deployed. A trivial residual shunt observed during postdeployment left ventricular angiogram in 7 of 17 patients (41.2%) completely disappeared at one month follow-up. Three patients had right bundle branch block (2 complete and 1 incomplete) whereas one developed junctional escape rhythm with a right bundle branch block morphology. One patient had clinically silent thromboembolism to the left vertebral artery and another patient had hemolysis which resolved spontaneously within 48 hr. Follow-up at 13.5 +/- 5.3 months (range 1-18 months) revealed no residual shunt. The left ventricular internal dimension in diastole decreased significantly from 45 +/- 6 mm to 40 +/- 6 mm (P < 0.01) at the time of the last follow up. The baseline tricuspid regurgitation (n = 4) and aortic regurgitation (n = 3) remained unchanged during the follow up period. None of the patients developed left ventricular outflow tract obstruction or new aortic or tricuspid regurgitation. There were no other device related complications such as device migration, systemic thromboembolism, infective endocarditis, pericardial effusion or delayed conduction disturbances. CONCLUSIONS: In carefully selected children and young adults, the Amplatzer asymmetric ventricular septal defect occluder is a promising device for transcatheter closure of perimembranous ventricular septal defect with encouraging results on short term follow up.

Transcatheter closure of large secundum atrial septal defects using the 40 mm Amplatzer septal occluder: results of an international registry.

Little is known about the efficacy and safety of the 40 mm Amplatzer septal occluder (ASO). Thirty-three patients (22 female, 11 male) with a large secundum atrial septal defect (ASD) underwent attempted device closure using the 40 mm ASO at a median age of 40 years (range, 14-81 years) and median weight of 65 kg (range, 48-98 kg). The median size of the ASD measured on 2D transesophageal echocardiography (27 patients) or intracardiac echocardiography (6 patients) was 30.5 mm (range, 24-39 mm) and the median balloon-stretched diameter was 37.7 mm (range, 32-43.7 mm). The median Qp:Qs ratio was 3.2:1 (range, 1.4-6.2). The attempt was unsuccessful in five patients; two had device embolization and one had left atrial wall perforation due to the sheath; all three required emergent surgery. The attempt was successful in the 28 remaining patients, resulting in complete immediate closure in 14 and a trivial residual shunt in 14. Fluoroscopy time ranged from 8.6 to 37.8 min (median, 12.2 min). At 24-hr follow-up, 2D transthoracic echocardiography with color flow Doppler revealed complete closure in 23 patients, and 5 had a trivial residual shunt. There were no complications encountered in patients who received the device. On follow-up, all patients are doing well. We conclude that the 40 mm ASO is safe and effective in most patients with a large ASD up to a diameter of 39 mm. However, the use of this device requires careful attention as the procedure may be unsuccessful or the device may embolize.

New technique for device closure of large atrial septal defects.

The objective of this study was to describe a new technique for transcatheter device closure of large atrial septal defects (ASDs) using the Amplatzer septal occluder and our experience with this technique in 14 patients. Transcatheter closure of large (> 25 mm) ASDs is challenging. We have developed a balloon-assisted technique (BAT) to facilitate device closure of large ASDs. The BAT consists of using a balloon catheter to support the left atrial (LA) disk of the Amplatzer septal occluder during device deployment. The balloon support prevents prolapse of the LA disk into the right atrium. Between April 2003 and February 2004, 14 patients with large ASDs (mean age, 25.71 +/- 15.71 years; mean weight, 51.21 +/- 23.78 kg) underwent device closure with the Amplatzer septal occluder using the BAT. The median balloon-stretched diameter of the ASD was 32 (range, 26-40) mm. The median device size used was 33 mm (range, 26-40 mm). All 14 patients had successful deployment of the device using the BAT. The mean follow-up period was 16.5 +/- 11.95 weeks. No major complications were noted during the procedure or on short-term follow-up. The BAT enables predictably successful closure of large ASDs using the Amplatzer septal occluder.