J INVASIVE CARDIOL 2010;22:E122–E124
Key words: atrioventricular block, congenital heart diseases The incidence of isolated ventricular septal defects is 20% of all congenital heart disease and perimembranous ventricular septal defects comprise the majority (80%) of these.1 The traditional treatment is surgical repair and is considered to be the gold standard, but it is associated with morbidity and mortality, patient discomfort, sternotomy, and skin scar. Since the first ventricular septal defect was closed by Lock et al using a transcatheter approach,2 various devices and techniques have been used. Closure of the perimembranous ventricular septal defect was first reported using the button device. The Rashkind device that were not originally designed for the perimembranous ventricular septal defect.3 We report the first pediatric case of a perimembranous ventricular septal defect closed successfully with an atrial septal occluder. Case Report. A 13-year-old female presented to us with a history of recurrent respiratory tract infections. She had normal growth parameters, intellect and appearance. Her pulse rate was 88 per minute, blood pressure 106/72 mmHg, oxygen saturation 100% in room air. She had left ventricular apical impulse and was in sinus rhythm. Cardiac auscultation revealed normal heart sounds with a 3/6 decrescendo systolic murmur in the lower left sternal border. Her chest radiograph showed cardiomegaly (cardiothoracic ratio 0.6) and increased pulmonary vascularity and her electrocardiogram was suggestive of left ventricular volume overload. Transthoracic echocardiography revealed a perimembranous ventricular septal defect with muscular extension measuring 6 mm, shunting left-to-right with a gradient of 50 mmHg across and evidence of mild pulmonary hypertension. The left atrium and left ventricle were mildly dilated. The subaortic rim was 4 mm. Her oximetry and catheterization data showed a hemodynamically significant ventricular septal defect in form of 13% step-up at the right ventricle, Qp:Qs of 2.1:1 and pulmonary artery systolic pressure of 45 mmHg (mean 28 mmHg). We decided to close the ventricular septal defect with a perimembranous device. The procedure was done using conscious sedation and anticoagulation with intravenous unfractionated heparin 100 units/ kg administered just prior to procedure. Femoral arteriovenous puncture was done and a 5 Fr NIH catheter was introduced through the inferior caval vein into the right atrium, right ventricle and pulmonary artery. A 5 Fr pigtail catheter was introduced through the aorta into the left ventricle. Ventricular septal defect was 6 mm on left ventriculogram and 4 mm from the aortic valve. A 5 Fr left internal mammary artery (LIMA) catheter was used to cross the ventricular septal defect from aorta→ left ventricle→ ventricular septal defect→ right ventricle→ right atrium→inferior caval vein. Exchange wire was advanced out, snared and drawn out through the venous site. Then sheath and dilator were used, followed by a 7 Fr delivery system loaded with 8 millimetres Amplatzer perimembranous device (AGA Medical Corp.) was inserted from venous end and used to cross the ventricular septal defect (inferior caval vein → right atrium → right ventricle→ ventricular septal defect→ left ventricle →aorta). The system was gently pulled towards the left ventricle and confirming the position on transthoracic echocardiography, the left ventricular disc of the device was opened on the left ventricular side and the right ventricular disc was opened on right ventricular side. However, the device slipped into the right ventricle which was detected by echocardiography. We then tried to deploy a 10 mm Amplatzer perimembranous device, but it also slipped before we could release it. We then decided to go ahead with an atrial septal occluder. An 8 Fr delivery system was loaded with a 10 mm Amplatzer Atrial Septal Occluder (AGA Medical Corp.) and was introduced in the same way with the left atrial disc of the device opened on the left ventricular side [Figure 1A] and the right atrial disc on the right ventricular side [Figure 1B)] using transthoracic echocardiographic guidance. The continuous electrocardiographic monitoring did not show any evidence of atrioventricular block. An aortic root angiogram was done to confirm the absence of aortic regurgitation (Figure 2A) before the device was released. A left ventriculogram done showed small residual ventricular septal defect (Figure 2B) which disappeared on subsequent day echocardiogram. The patient was put on oral clopidogrel for 6 months. After 12 months of regular follow up there is no evidence of residual shunt, valvar regurgitation, atrioventricular block or any other complication. Discussion. Transcatheter ventricular septal defect closure with conventionally available devices is reported to be associated with complications, especially high degree atrioventricular block.3 Recently, some trials using the Amplatzer membranous ventricular septal occluder, which has been specifically designed for the perimembranous ventricular septal defect, have reported high success rate and less incidence of complications like atrioventricular block (2–3%).4 Perimembranous ventricular septal defect is usually located in an area quite near the aortic and tricuspid valves as well as atrioventricular conduction system. Encroaching any of these structures would cause severe complications. The location and orientation of the defect make it difficult to access by catheters or guidewires. In addition, because the membranous septum is very thin, it is difficult to feel any resistance during device pullback from the left ventricle to the septum. As a result, the device might be misplaced in the right ventricle. Therefore, echocardiography and angiography are crucial in identifying correct device deployment.3 There are very few reports on the use of atrial septal occluder devices to close ventricular septal defects, and these only involved adults with post myocardial infarction ventricular septal defect or traumatic ventricular septal defect; no successful case has been reported in a child with perimembranous ventricular septal defect.5–8 We believe this is the first report of a perimembranous ventricular septal defect closed successfully with an atrial septal occluder in a pediatric patient. There is currently no ideal device. Device selection and deployment techniques vary and may have to be modified with each patient. Atrial septal occluders can also be used for closure of perimembranous ventricular septal defects in selected patients. From the Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, India. The authors report no financial relationships or conflicts of interest regarding the content herein. Manuscript submitted November 6, 2009, provisional acceptance given December 9, 2009, final version accepted December 21, 2009. Address for correspondence: Dr. Anurakti Srivastava, Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bannerghatta Road, Bangalore, Karnataka, India. E-mail: firstname.lastname@example.org References
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