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Abstract(Please copy/paste the abstract send to the congress) : |
1-Objective: There are several reports of complicated PDA device closure in patients with genetic syndromes. We searched our database of over 200 PDA transcatheter closures to investigate safety and feasibility of this procedure in syndromic patients.
Methods: We found 20 attempted device closures in 16 patients with Down syndrome, 2 with Marfan syndrome, and one with each of Russell-Silver and Rubinstein-Taybi syndromes.
Results: Device closure was successful in 18 patients (90%). In a patient with Down syndrome and pulmonary hypertension, an appropriate size Amplatzer occluder was pulled trough the duct and a larger device was not suitable due to the risk of coarctation. In a patient with Marfan syndrome, residual shunt remained more than 6 months after the procedure. In another patient with Down syndrome and pulmonary hypertension, an appropriate size coil was pulled through the duct when attempted to implant from the pulmonary side but it was successfully implanted from the aortic side.
Conclusions: There are several reports of device embolization or pull through in patients with Down syndrome and pulmonary hypertension, even when the duct is small. Therefore, Down syndrome patients with pulmonary hypertension may need oversize devices. There are reports of aneurysm formation and increasing residual shunts after PDA closure in Marfan syndrome. Closer follow up for these patients to detect possible aneurysm formation and residual shunt is recommended. In general, transcatheter PDA closure is safe and feasible in syndromic patients.
2-Except for transcatheter pulmonary valve replacement or right ventricular to pulmonary artery stenosis, right ventricular outflow tract (RVOT) stenting is not a standard method of therapy. It was used in a limited number of neonates with Tetralogy of Fallot (TOF). However, it can be an acceptable alternative to other therapies in certain situations. Disadvantages include stent fracture, incomplete coverage of the stenosed area, free pulmonary regurgitation, and difficulty in stent removal during corrective surgery. Since June, 2011, we used this method in 3 patients.
Case presentation 1: A 17-month-old girl with a history of pulmonary valvotomy for pulmonary valve atresia/intact ventricular septum was presented with progressive severe pulmonary valvar and supravalvar stenosis (PG= 77 mmHg). Pulmonary balloon valvoplasty was ineffective in relieving the stenosis, so RVOT stenting by a Palmaz Genesis XD stent 2910 on an Opta Pro 10×30 balloon was performed, decreasing the stenosis to 18 mmHg. Recently, the stent was redilated by an Opta Pro 12×30 balloon due to increasing restenosis (45 mmHg). The gradient decreased to 10 mmHg.
Case presentation 2: A 2-month-old infant with TOF and severe pulmonary branch hypoplasia (3 mm) was presented with severe cyanosis (oxygen saturation 55% at room air). Due to small sizes of the pulmonary artery branches and risk of their distortion by a surgical shunt, we decided to stent RVOT. We implanted a Palmaz Blue stent 5×15 premounted on Aviator Plus balloon successfully. Oxygen saturation increased to 75%. Seven months after the procedure, she experienced worsening cyanosis due to pneumonia. Angiography showed growth of the pulmonary arteries, during which the stent was redilated by a 7 mm balloon catheter.
Case presentation 3: A 9-year-old neglected patient with TOF was presented with severe cyanosis (oxygen saturation 55% at room air). His pulmonary artery branches were hypoplastic (8 mm the right, 10 mm the left) and the ascending aorta dilated (37 mm at the sinuses of Valsalva). Due to the potential role of a surgical shunt on aortic dilation, we decide to stent RVOT. We implanted a Palmaz Blue stent 6×24 premounted on Aviator Plus balloon successfully. Oxygen saturation increased to 70%. Six months after the procedure, he did well with Oxygen saturation at the same range.
Case presentation 4: A 3-month-old patient with TOF presented with frequent hypercyanotic attacks and moderate cyanosis at rest (70%). Although her pulmonary branches were acceptable, her body size was not enough for our surgeons to repair the cardiac defect. Acceptable results of the past patients and high load of the operating room encouraged us to stent her RVOT by a Palmaz Blue stent 6×15 premounted on Aviator Plus balloon. Oxygen saturation increased to 87% and hypercyanotic attacks abolished.
Discussion: We treated 4 different patients with RVOT stenting successfully. Patient 1 needed a transannular patch during the surgery, in fact. The surgeon recommended RVOT stenting due to the risk of reoperation and the need for several surgeries for homograft implantations. Patient 2 was a small patient with TOF and hypoplastic pulmonary artery branches. Patient 3 was a neglected TOF patient with hypoplastic pulmonary artery branches and aortic dilatation. Patient 4 needed an urgent remedy for increasing pulmonary flow. However, RVOT stenting was successful in short- to midterm in relieving trans-RVOT gradient and increasing oxygen saturation in all of them. |
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Keywords of your Abstract : |
1-Patent Ductus Aarteriosus, Down syndrome, Marfan syndrome, Russell-Silver syndrome, Rubinstein-Taybi syndrome
2-Stent, Right Ventricular Outflow Tract, Stent Redilation |