Jameel Al-Ata

The efficacy and safety of the Amplatzer ductal occluder in young children and infants.

BACKGROUND We have used the Amplatzer ductal occluder for transcatheter closure of large persistently patent arterial ducts, and used our experience to assess the safety and efficacy of the device in young children and infants. METHODS AND PATIENTS We used the Amplatzer ductal occluder prospectively in 43 patients with large patent arterial ducts, reviewing our experience to identify any problems or complications. RESULTS The procedure proved successful in 42 of the patients. We achieved complete occlusion of the duct in 33 (78.5 per cent) of the patients on the day of insertion. In 6 additional patients, complete occlusion occurred 1 week to 6 months after the procedure. Trivial leaks persisted in 2 patients, while one had a significant residual leak. Problems were encountered in 7 patients. The procedure failed in one, a device was wasted in 2, pulled through in 3, while we experienced kinking of the long Mullins sheath, being unable to retrieve the device, in one patient. Minor complications occurred in 6 patients, finding flow at a peak velocity of 2.2 metres per second in the descending aorta in 2 patients, and at 2.5 metres per second in 2 further patients, and flow at 2.5 metres per second in the pulmonary arteries of two patients. One patient experienced a major complication due to excessive bleeding. Out of the 14 patients suffering adverse events, 13 weighed less than 10 kilograms. This rate of problems and complication in these patients weighing less than 10 kilograms was significantly higher than in the patients weighing more than 10 kilograms. CONCLUSION Transcatheter occlusion of moderate to large patent arterial ducts with the Amplatzer ductal occluder device is safe and effective, with a high rate of complete occlusion. Problems and minor complications may be encountered in children weighing less than 10 kilograms. If the device is to be deployed completely in the ductal ampulla, and to avoid descending aortic obstruction, the size of the retention flanges of the occluder should not exceed the largest diameter of the patent arterial duct.

Midterm Outcome of Stent Dilatation of Patent Ductus Arteriosus in Ductal‐dependent Pulmonary Circulation

OBJECTIVE We sought to assess the outcome of transcatheter ductus arteriosus stenting in newborns with ductal-dependent pulmonary circulation. BACKGROUND Better results of ductal stenting have been reported using stents with better scaffolding and ensuring stenting of the entire length of the ductus arteriosus. METHODS Twenty-one patients with ductal-dependent pulmonary circulation were brought to the catheterization laboratory for ductal stenting. Five patients did not qualify because of a complex tortuous ductus arteriosus or branch pulmonary artery stenosis. Stent implantation was successful in 14 patients. The duct was accessed with a 0.014-inch guidewire. A low profile premounted coronary stent was implanted in the duct without using a long delivery sheath. Attempts were made to cover the entire length of the ductus arteriosus. RESULTS The mean age of the patients at the time of stent implantation was 24 +/- 17.5 days. The mean body weight was 2.9 +/- 0.35 kg. The ductus was mildly tortuous in four, moderately tortuous in four, conical in four, and vertical in four patients. Ductal stenting was successful in 14 patients. The mean ductal diameter was 3.9 +/- 0.5 mm. The mean diameter and the length of the stent implanted were 3.9 +/- 0.72 and 15.4 +/- 3.16 mm, respectively. Mean fluoroscopy and procedure times were 22.16 +/- 12.5 and 107.9 +/- 34.5 minutes, respectively. Out of the 14 successful stent implantations, the ductus arteriosus was not completely covered with the stent in five patients at the time of primary procedure, four of them required re-stenting for significant desaturation. One of these patients died despite successful re-stenting. Another patient died due to aspiration pneumonia. Of the 12 survivors, five underwent Glenn shunt (two deaths related to pulmonary hypertension), two underwent biventricular repair, one patient was lost to follow-up, and the remaining four are doing well with a mean oxygen saturation of 85% at a mean follow-up of 13 +/- 6 months. CONCLUSION Stenting of ductus arteriosus, including moderately tortuous ducts, is a safe palliation for patients with ductal-dependent pulmonary circulation. Incomplete stenting of the duct invariably results in ductal stenosis and compromised pulmonary flow.

Left ventricular dysfunction after closure of large patent ductus arteriosus.

Changes in left ventricular dimensions and performance were studied in 43 patients after transcatheter occlusion or surgical ligation of patent ductus arteriosus. The patients were assigned to 2 groups based on their ductal diameter: ≥ 3.1 mm to group A (n = 27) and ≤ 3 mm to group B (n = 16). The mean age and weight of the groups were comparable. Before intervention, group A had a significantly larger mean left ventricular end-diastolic diameter than group B, while all patients had normal shortening fraction and ejection fraction. Within 1 month after intervention, left ventricular end-diastolic diameter showed a trend towards regression while shortening fraction and ejection fraction decreased significantly in group A. There were no significant changes in these parameters in group B. Between 1 and 6 months after intervention, left ventricular performance improved in most of the group A patients who were followed up. We conclude that closure of large ductus arteriosus in children leads to significant immediate deterioration of left ventricular performance, which appears to recover within a few months. Echocardiographic study before hospital discharge is recommended in these patients. Serious deterioration of ventricular performance after closure may warrant the use of angiotensin converting enzyme inhibitors.

Stent Angioplasty: An Effective Alternative in Selected Infants with Critical Native Aortic Coarctation

Management of native aortic coarctation during early months of life poses therapeutic challenges, and there is no consensus among medical professionals regarding a management plan. Much can be argued about the benefits, limitations, and/or complications of transcatheter versus surgical intervention in such cases. Occasionally, the complexity of the lesions limits management options. Therefore, each patient requires individual management decisions because there is no one therapeutic plan that satisfies all patients. In this report, four critically ill infants who had complex native coarctation are presented. Surgical repair was not possible because of relative contraindications. The patients underwent transcatheter stent implantation (six procedures and seven stents) as a nondefinitive procedure with acceptable results. Three patients improved. One patient did not survive, mainly due to other major complications. Multiple reexpansions of the stents were carried out when indicated. After a mean follow-up of 45 months (range, 41–49), the three survivors were doing fine and had gained an average weight of 9.7 kg (range, 6.6–13.3). At the time of reporting, the relative contraindications no longer exist and the final surgical repair can be carried out. Our experience suggests that in certain situations and in critically ill infants with complex form of coarctation, stent angioplasty can be used as a life-saving palliative procedure. Further reexpansions can be done when required. This may serve as a bridge to major surgical repair in the future.

Transcatheter Occlusion of a Large Left Coronary Artery to Right Superior Vena Cava Fistula Using the Amplatzer Duct Occluder Device

Transcatheter embolization has become the therapy of choice for most coronary artery fistula. We report a 5.9-kg infant with a coronary artery fistula from the left coronary artery to the right superior vena cava with significant congestive heart failure. The infant underwent successful complete occlusion using the Amplatzer duct occluder.

Comparative Outcome of Bidirectional Glenn Shunt in Patients With Pulmonary Vascular Resistance ≥ 3.5 Woods Units Versus < 3.5 Woods Units

Moderate to severe pulmonary hypertension is considered to be an absolute contraindication to the performance of bidirectional Glenn (BDG) shunting. However, BDG shunting has been performed in young children with pulmonary hypertension associated with unrestricted pulmonary blood flow. In this study, the medical records of patients who underwent BDG starting from October 2000 to March 2004 were reviewed. Patients were divided into 2 groups on the basis of indexed pulmonary vascular resistance (PVRI) measured in room air: a high-risk group (n = 12) with PVRI > or = 3.5 Woods units (WU)/m(2) and a low-risk group (n = 28) with PVRI <3.5 WU/m(2) in room air. The 2 groups were comparable with respect to age, weight, ventricular morphology, pulmonary arterial anatomy, and atrioventricular valve function. Mean pulmonary arterial pressure and PVRI were significantly higher in the high-risk group compared with the low-risk group (39.2 +/- 20.7 vs 15.1 +/- 6.25 mm Hg, p <0.002, and 6.0 +/- 2.5 vs 1.6 +/- 0.82 WU/m(2), p <0.0005, respectively). The ratio of pulmonary flow to systemic flow was 1.45 +/- 0.76 in the high-risk group and 1.24 +/- 1.2 in the low-risk group. In the high-risk group, mean PVRI decreased to 2.0 +/- 1.0 WU/m(2) on 100% oxygen (p <0.0005). A contraindication to Glenn shunting was PVRI >3.5 WU/m(2) on 100% oxygen. Hospital mortality was 17% (2 of 12) in the high-risk group and 4% (1 of 28) in the low-risk group. Of 10 survivors in the high-risk group, 1 had undergone a Kawashima procedure, 7 had undergone Fontan procedures (with 1 death), and 2 were awaiting the completion of Fontan procedures as of this writing. In conclusion, these preliminary data suggest that in young children with increased pulmonary flow, BDG shunting can be safely performed, despite the apparent elevation of pulmonary arterial pressure to inoperable levels, provided PVRI decreases to < or = 3.5 WU/m(2) on 100% oxygen.

Stent Implantation to Maintain Patency of a Stenosed Blalock Taussig Shunt

A 14-year-old female with complex congenital heart disease underwent a left-sided classical Blalock Taussig (BT) shunt 15 days after birth. Ten years after the operation her oxygen saturation had decreased significantly. An angiography revealed a severely stenosed BT shunt. Balloon dilation including implantation of a 6 × 13 mm stent was performed successfully. Immediately after intervention, oxygen saturation rose from 55% to 80 84% in room air. Follow-up at a year and a half later showed the classical BT shunt was still patent.

Safety and efficacy of transcatheter closure of atrial septal defect type II under transthoracic echocardiographic guidance: A case control study

Background Transcatheter closure of secundum atrial septal defect is routinely performed under general anesthesia and transesophageal echocardiography guidance. If patients have good echo windows, the procedure could be performed under transthoracic echo guidance. Aim of study To evaluate safety and efficacy of the intervention using fluoroscopy and echo guidance. Methods In a case control study design, 180 patients underwent atrial septal defect closure between January 2010 and December 2016. In 32 patients, the intervention was performed under fluoroscopy and transthoracic echo guidance. Our study group consisted of 22 out of 32 patients (<13 years old). For the other 10 patients, we could not find a matching pair. The data of the study group were compared with an age, weight, and height matched group (controls), who underwent the procedure under transesophageal echocardiography guidance. Results The diameter of the atrial septal defect, septal length, and most of the rims were comparable. The superior rim and inferior rims were longer in the study group. The devices chosen for the cases were larger than the control group. Procedure time and fluoroscopy times were shorter in the study group. Success rate was comparable. On follow-up, both groups had almost no or minimal incidence of residual shunt. Conclusion We conclude that transcatheter closure of atrial septal defect under fluoroscopy and transthoracic echo guidance is safe and successful in selected patients who have single central atrial septal defect with adequate septal lengths and adequate septal rims, with high incidence of complete occlusion rate.

Isolated right superior vena cava draining into the left atrium in a child with vein of Galen aneurysmal malformation—case report

BackgroundIsolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described.Case presentationWe describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully.ConclusionsThe superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

The Spectrum of Congenital Heart Diseases in Robinow Syndrome@@@مدى الأمراض القلبية الخلقية في متلازمة روبينو

We present the case of a newborn infant with classical signs of Robinow Syndrome and major congenital cardiac malformations consisting of double outlet right ventricle with subaortic VSD, infundibular and valvular stenosis, and patent ductus arteriosus. Similar patients of Robinow Syndrome with cardiac involvement are briefly reviewed from the literature and the importance of recognizing heart defects in early life is stressed.