Trans catheter closure of large ductus arteriosus in patients with severe pulmonary hypertension: safety and efficacy of test occlusion Sulafa KM Ali, FRCPCH, FACC Sudan Heart Institute, Department of Pediatric Cardiology and University of Khartoum, Faculty of Medicine, Department of Pediatrics. قفل القناة الشريانية عن طريق القسطرة في حاالت ارتفاع الضغط الرئوي: سالمة و فعالية القفل التجريبي د.سالفة خالد محمد علي السودان معھد القلب قسم أمراض القلب لألطفال وجامعة الخرطوم كلية الطب قسم طب األطفال القناة الشريانية من العيوب الخلقيه الھامة التي قد تؤدي الي ارتفاع الضغط الرئوي الثابت. مؤخرا أصبح استعمال السدادات عن طريق القسطرة ھو العالج األمثل بدال من الجراحة. يعتبر القفل التجريبي خاصة في حاالت ارتفاع الضغط الرئوي-من ميزات استعمال السدادات. وصفنا في ھذا التقرير 3 حاالت لديھم قناة شريانية كبيرة و ارتفاع شديد بالضغط الرئوي تم عالجھم بنجاح باستعمال السدادات و القفل التجريبي. Summary Patent ductus arteriosus (PDA) is a serious congenital heart disease that can lead to irreversible pulmonary hypertension. Catheter closure has largely replaced surgical ligation and has the advantage of feasibility of test occlusion in patients with borderline operability. We present 3 cases of large PDA with severe pulmonary hypertension where test occlusion helped to verify operability and all cases had successful closure. Keywords: Patent ductus arteriosus, cardiac catheterization, pulmonary hypertension Introduction Patent ductus arteriosus (PDA) accounts for approximately 10% of all congenital heart diseases, with an incidence of 2-4 per 1000 term births. Like many other congenital heart disease, PDA can lead to irreversible pulmonary hypertension (PHT) if left untreated (1). Transcatheter closure of PDA is well established with excellent short and long term results both in children as well as in adults (2). In patients with severe PHT closing the PDA may lead to fatal complications and therefore critical peri- Corresponding author Sulafa KM Ali Email: sulafakhalid2000@yahoo.com operative evaluation is needed by cardiac catheterization for the pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). We report 3 sporadic patients (2 adults and 1 infant) with severe PHT who had successful PDA catheter closure, after test occlusion, using different devices. Patients were seen at the Sudan Heart Institute in 2010 and 2011. Clinical examination, chest X-ray and electrocardiograms were done. Complete 2-dimensional echocardiogram was done using Esaote (MyLab 50) machine. A standard right heart cardiac catheterization study was performed to measure PAP, left to right shunt (QP/QS) and PVR in air then repeated after giving 100% for 10 minutes. The PDA was measured at the narrowest point from a lateral aortogram. In patient 1 (65 years old), a valvuloplasty balloon 10x20 mm was introduced through the femoral vein and inflated at the PDA; aortogram was repeated to ascertain complete PDA occlusion. In the other 2 patients (40 years old & 8 months old), test occlusion was done using the PDA device. The device was introduced using a long sheath through the femoral vein to pulmonary artery across the PDA to the descending aorta. In all patients test, occlusion was done for 10 minutes then the device was deployed. 147
During this time the patient s vital signs and saturation were continuously monitored. The clinical and echocardiographic data of 3 patients who had PDA and PHT are shown in Table 1: Clinical and Echocardiographic Findings Patient Age Sex Clinical Features 1 65 years Female Heart failure, New Atrial fibrillation York Heart Association (NYHA) class IV, Soft ejection systolic murmur 2 40 years Female Exertional dyspnea, NYHA class II. Ejection systolic murmur 3 8 months Male Recurrent chest infections. Oxygen saturation 80%, Ejection systolic murmur Table 1 and cardiac catheterization findings and outcome are shown in Table 2. Patient 1 had a coronary angiogram which revealed normal coronaries. ECG CXR Echocardiography Left ventricle enlargement Right ventricle enlargement. No left ventricle enlargement. Pulmonary edema, left ventricle and atrial enlargement (Fig 1A) Pulmonary edema. left ventricle and atrial enlargement Mild cardiomegaly with right ventricle enlargement. Normal lung parynchyma. PDA 11 mm with bidirectional shunt, dilated left ventricle and atrium. PDA 12 mm with bidirectional shunt. dilated left ventricle and atrium PDA 4 mm with bidirectional shunt. Dilated right ventricle, no dilatation of the left side. Table 2: Cardiac Catheterization Findings and Outcome Patient Oxygen saturati on in QP/QS in PAP PVR in (Woods Units) Procedure and PDA Devise Outcome 1 92/98 0.7:1/1.5:1 99/47/5 8 2 95/98 1.2:1/1.4:1 95/50/6 2 15/13 Test occlusion using Numed Balloon 10/20 mm for 10 minutes. Blood pressure and saturation (O 2 ) were maintained then closed using 14/16 (Ballmedic-China) PDA device. 13/11 PDA was 14 mm from the angiogram. (Fig 2A)Test occlusion using an atrial septal defect occluder 16mm (Amplatzer-AGA-USA). Blood pressure and O 2 saturation were maintained. Device deployed in good position (Fig 2B). Immediate improvement of symptoms (NYHA class improved from IV to I). Oxygen saturation remained 95%. Pulmonary edema Improved (Fig 1B). Normal PAP on follow-up at 12 months. Still in atrial fibrillation. Improvement of symptoms (NYHA improved from class II to I) and chest X-ray Normal PAP on follow-up at 6 months. 3 85/99 1.2:1/16:1 16/5 Test occlusion using PDA device 8/6mm (Starway-China). Blood pressure and O 2 saturation were maintained. Device deployed in a good position. Immediate improvement of symptoms. (No signs of respiratory distress, normal O 2 saturation) Normal PAP on follow-up at 8 months 148
Fig 1A: Chest X-ray of a 65 year old lady with a large PDA showing left ventricle and atrial enlargement and pulmonary edema. Fig 2A: Lateral aortogram showing the PDA. Fig 1B: Chest X-ray of the same patient 6 hours after PDA closure showing remarkable improvement in left side dilatation and pulmonary edema. Fig 2B: Lateral fluoroscopic view showing the catheter in the aorta and the occluding device well fitted into the PDA. Discussion The outlook for patients with untreated large PDA is poor with prompt development of severe irreversible PHT (Eisenmenger s syndrome (ES) with advancing age; therefore, efforts should be to treat such patients as early as possible. The timing of development of ES 149 is variable, patients with large congenital left to right shunts can develop irreversible PHT as early as 2 months of age (3). On the other hand, we do encounter adults with large left to right shunts who still have reversible PHT late in their lives.
All patients with such shunts should be evaluated early and transcatheter/surgical closure should be considered. Catheter closure using coils or occluders has largely replaced surgical PDA ligation. In our experience as well as others, the short and long term outcomes are excellent (2,4). The advantages of catheter closure include the avoidance of surgical scar, the short hospital admission (12 hours for catheter versus 3-5 days for surgical closure) and avoiding the complications of thoracotomy like vocal cord, phrenic nerve and lung injury. In fact, the morbidity in adult patients undergoing surgical PDA ligation is still significant with 32% complication rate and occasionally the need for cardiopulmonary bypass compared with a complication rate of 4.7% in large series of those undergoing PDA catheter closure (5,2). In patients with clinical, electrocardiographic and/or echocardiographic signs of shunt reversal, cardiac catheterization should be done to evaluate the PAP and PVR. Measuring PAP alone, a commonly encountered practice does not give enough information to assess operability; therefore PVR should always be calculated. PVR of less than 10 Woods Units after giving 100% (or nitric oxide inhalation) is usually taken as a cut-off point for operability. In those with borderline PVR careful evaluation of signs of left to right shunt clinically, by electrocardiograms and/ or by echocardiography should be performed. These signs include the presence of left sided heart failure (as in patient 1), an saturation References 1. Engelfriet PM, Duffels MG, Möller T, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682-7. 2. Faella HJ, Hijazi ZM. Closure of the patent ductus arteriosus with the amplatzer above 90% and signs of left side enlargement. Patients with such signs, (as in patients 1 & 2) benefit from the use of test occlusion to verify reversibility (6,7). In patient 3, the PVR showed a favorable response to with drop of PVR to a safe level; however, we performed the test occlusion because of the extremely high PVR of 16 Woods Units in air. Test occlusion is done by closing the PDA for 10-15 minutes using a balloon catheter or the PDA device itself and assessing the patient s hemodynamics, if the patient remained stable, the device closure is undertaken. Assessment of the response to test occlusion by the arterial blood pressure and saturation allowed us to confidently proceed to device deployment with good results in the short term and on follow-up. Test occlusion has also been performed successfully in patients with atrial septal defect where reduction of the PAP by 25% relative to the basal level, without a fall in systemic pressure is taken as a favorable response (8). There are few reported cases of catheter closure of PDA at an advanced age (more than 60 years) (9). PDA catheter closure using other devices like atrial septal and ventricular septal defect occluder has been occasionally reported (10). In conclusion, physicians should refer patients with congenital heart disease early in order to avoid the development of ES. Cardiologists need to carefully examine such patients and review investigations to decide operability. Transcatheter test occlusion is safe and effective for patients with borderline PHT. PDA device: immediate results of the international clinical trial. Catheter Cardiovasc Interv 2000;51:50-4. 3. Alt B, Shikes RH. Pulmonary hypertension in congenital heart disease: irreversible vascular changes in young infants. Pediatr Pathol 1983;1:423-34. 150
4. Sulafa KM Ali Interventional catheterization for congenital heart disease in Sudan. Sudanese Journal of Paediatrics 2010;10:24-7. 5. Jatene MB, Abuchaim DC, Tiveron MG, et al. Surgical treatment of patent ductus arteriosus in adults.m Rev Bras Cir Cardiovasc 2011;26:93-7. 6. Roy A, Juneja R, Saxena A. Use of amplatzer duct occluder to close severely hypertensive ducts: utility of transient balloon occlusion. Indian Heart J 2005;57:332-6. 7. Yan C, Zhao S, Jiang S, et altranscatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults. Heart 2007;93:514-8. 8. Sánchez-Recalde A, Oliver JM, Galeote G, et al. Atrial septal defect with severe pulmonary hypertension in elderly patients: usefulness of transient balloon occlusion Rev Esp Cardiol 2010;63:860-4. 9. Cassese S, Losi MA, Rapacciuolo A. Transradial approach for percutaneous closure of patent ductus arteriosus with the amplatzer duct occluder II: a case report. Catheter Cardiovasc Interv 2011;77:103-7. 10. Zanjani KS. Closure of a short patent ductus arteriosus using an atrial septal occluder. Chin Med J 2010;123:1220-1. 151