clinicAl cArdiology: cAse rePorT
Patent ductus arteriosus closure using an AmplatzerTM ventricular septal defect closure device Rajeev Fernando MD1, Ketan Koranne MD1, Pranav Loyalka MD2, Biswajit Kar MD2, Igor Gregoric MD2
r Fernando, K Koranne, P Loyalka, B Kar, i Gregoric. Patent ductus arteriosus closure using an amplatzertM ventricular septal defect closure device. exp Clin Cardiol 2013;18(1):e50-e54. The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids
T
he ductus arteriosus is a physiological fetal structure that connects the descending aorta to the pulmonary artery and spontaneously occludes after birth. Patency of the ductus arteriosus is associated with a variety of complications including left ventricular failure, endarteritis and arrhythmias. These may manifest early in childhood or in adulthood, depending on the size and flow through the ductus. While medical management can improve symptoms and prevent complications, mechanical closure remains the definitive therapy. Since 1967, transcatheter closure of patent ductus arteriosus (PDA) has been constantly evolving, both in terms of the technique and the closure device
Figure 1) Cardiac magnetic resonance image. Coronal section demonstrating patent ductus arteriosus (arrow)
sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs. Key Words: AmplatzerTM ventricular septal defect closure device; Patent ductus arteriosus; Transcatheter closure
used. Multiple occluder devices have been studied and used for PDAs of different shapes, sizes and orientations, but there is currently little evidence regarding the use of Amplatzer (St Jude Medical, USA) muscular ventricular septal defect (mVSD) closure devices for PDA closure. We present an interesting case involving a 35-year-old woman with a large PDA that was successfully occluded with an Amplatzer mVSD occluder.
Case Presentation
A 35-year-old woman presented with a two-month history of progressive dyspnea, bilateral lower extremity swelling and abdominal distension. A physical examination revealed a displaced point of maximum impulse, a high-pitched, continuous murmur, ascites and peripheral edema. Her chest x-ray showed cardiomegaly and interstitial pulmonary edema, and an electrocardiogram revealed sinus tachycardia, inferolateral T wave abnormality and frequent premature ventricular complexes. A transthoracic echocardiogram revealed a PDA with leftto-right shunt (pulmonary flow [Qp]/systemic flow [Qs] ratio of 3.33) and a left ventricular ejection fraction of 45% to 50%. Cardiac magnetic resonance imaging (MRI) revealed a PDA connecting the aortic isthmus (immediately distal and inferior to the left subclavian takeoff) to the main pulmonary artery and excluded coexistent congenital abnormalities (Figures 1, 2 and 3). The patient underwent cardiac catheterization with an aortic angiogram, PDA angiogram, intravascular ultrasound (IVUS) of the PDA and right heart catheterization. A 7 Fr sheath was inserted into the right femoral vein, followed by a Swan Ganz monitor catheter, and right-sided pressures were recorded. The Swan Ganz catheter was removed and a 6 Fr, 4.0 cm Judkins left catheter was inserted in the right femoral artery; left-sided pressures were then recorded. A Magic Torque 0.35 inch × 260 cm guidewire (Boston Scientific, USA) was then inserted via the right femoral artery. The right femoral vein sheath was exchanged for a 9 Fr, 11 cm standard sheath and a multipurpose 6 Fr, 100 cm AL-1 guide catheter (Boston Scientific, USA) was inserted through it. An Amplatzer GooseNeck Snare Kit
1University
of Texas Health Science Center at Houston; 2Center for Advanced Heart Failure, Houston, Texas, USA Correspondence: Dr Rajeev Fernando, University of Texas Health Science Center at Houston, 6411 Fannin Street, Houston, Texas 77030, USA. Telephone 713-876-6115, fax 713-500-6556, e-mail [email protected]
e50
©2013 Pulsus Group Inc. All rights reserved
Exp Clin Cardiol Vol 18 No 1 2013
Amplatzer VSD closure devices for the treatment of PDA
Figure 2) Cardiac magnetic resonance image. Transverse section demonstrating patent ductus arteriosus (arrow) (Covidien, USA) was inserted into the right femoral vein, where it met with the Magic Torque wire and pulled it. IVUS was then performed and images were recorded. The patient was found to have a large 10.5 mm × 3 mm PDA with 2.4:1 Qp:Qs preclosure shunt. Right heart catheterization demonstrated a pulmonary arterial pressure of 24 mmHg/6 mmHg (mean 16 mmHg) with a pulmonary capillary wedge pressure of 9 mmHg/13 mmHg (mean 6 mmHg). A 10 mm mVSD Amplatzer occluder was then advanced to the site of the PDA through a 7 Fr torque delivery system. Its subsequent deployment closed the PDA (Figures 4 and 5). Postprocedure pressures were recorded (Table 1). The patient later underwent placement of a single-chamber implantable cardioverter defibrillator (Boston Scientific, USA) due to symptomatic premature ventricular complexes, with subsequent resolution of symptoms. At her eight-month follow-up, she denied exertional dyspnea, angina or lower extremity edema. Significant variability in the size, shape and orientation of PDAs poses a challenge in selecting the ideal closure device. The Amplatzer mVSD closure device was selected because it has a broader waist (up to 18 mm) and dual discs that ensure stability and reduce the risk of embolization, even with high pulmonary artery pressures.
Figure 3) Cardiac magnetic resonance image. Sagittal section demonstrating patent ductus arteriosus (arrow)
DisCussion
The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch, connecting the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery near the origin of the left pulmonary artery (1,2). It spontaneously occludes 24 h to 48 h after birth through the contraction of the medial smooth muscle in the vessel wall. This is followed by endothelial cell adhesion and replacement of the muscle fibres with connective tissue, resulting in ligamentosum arteriosum within three weeks. Persistence of the duct beyond 48 h after birth is abnormal and results in PDA. It is rare in adults because it is usually detected and treated in childhood. PDAs usually fall into one of three categories, depending on their size and hemodynamic effects: small PDA without apparent hemodynamic significance; moderate-to-large PDA with left heart volume overload and without increased pulmonary vascular resistance; and large PDA with evidence of increased pulmonary vascular resistance (Table 2) (3). Krichenko et al (4) classified isolated PDAs based on anatomical and topological variations. These were identified by their angiographic appearances, which could influence the technique of catheter occlusion. The authors identified five groups using the narrowest segment of the ductus arteriosus as a landmark (Table 3).
Exp Clin Cardiol Vol 18 No 1 2013
Figure 4) Transcatheter closure of patent ductus arteriosus (arrow) using an Amplatzer muscular ventricular septal defect closure device (St Jude Medical, USA) A PDA of moderate size may remain asymptomatic during infancy, but can manifest as fatigue, dyspnea or palpitations during childhood and adult life (5,6). While left ventricular failure is the complication most commonly associated with particularly large PDAs, these patients are also at risk for endarteritis, infective endocarditis, ductal aneurysm and rupture (5,7,8). Eventually, if closure is not performed, pulmonary vascular obstruction may develop with subsequent Eisenmengerization (5,9). Heart failure, pulmonary hypertension and endarteritis are the most common causes of death in patients with PDA without closure (6). The mortality rate of untreated PDAs is estimated to be 1.8% per year.
e51
Fernando et al
TAble 2 Patent ductus arteriosus (PDA) classification based on size and hemodynamic significance Type
Description
Silent PDA
Usually 1.7. While precise anatomical delineation of the PDA is possible with off-axis or orthogonal MRI planes, the anatomical relationship of the aorta, pulmonary artery and PDA can be well defined with magnetic resonance angiography volume-rendered three-dimensional models. It is, therefore, an excellent noninvasive tool to determine both the need for shunt closure and the type of closure device to be used (10). The location, size, shape, presence and extent of calcification, and spatial relationship of PDAs to their surrounding structures can also be
e52
Type
Anatomy
Topology
A
Conical
Narrowest segment at the pulmonary artery end with well-defined aortic ampulla
B
Window
Short duct with narrowing at the aortic insertion
C
Tubular
No constrictions present
D
Complex
Multiple constrictions present
E
Elongated conical Long ductus with constriction at the pulmonary artery end, remote from the anterior tracheal edge
Adapted from Krichenko et al (4)
clearly visualized using retrospectively electrocardiography-gated multidetector computed tomography (MDCT). Volume-rendered three-dimensional reconstruction of the duct classifies that duct into angiographic subgroups while establishing the relationship of the pulmonary insertion of the PDA with the trachea; this can guide therapy (11). IVUS can detect calcification, endarteritis and infection in the PDA and correlates well with angiography (12). Patients with symptomatic PDA may experience improvement with medical management, although the definitive therapy remains surgical closure. It is indicated in all symptomatic patients with a leftto-right shunt and in asymptomatic patients with evidence of left heart enlargement to minimize complications. Yamaki et al (13) suggested that a lung biopsy should be performed to determine operability in patients with pulmonary vascular resistance >8 U/m2. Histological characteristics based on Heath and Edward’s classification (14), such as fibrosis of the arterial and arteriolar media and intima with dilation (grade 5) and necrotizing arteritis (grade 6), may suggest irreversible pulmonary hypertension. A reduction in pulmonary vascular pressures on test occlusion and testing for response to pulmonary vasodilators are additional strategies that may be used to assure reversibility before closure. transcatheter PDa closure Although open thoracotomy for PDA ligation and/or division can be safely performed in children without recurrence, the procedure can be challenging in adults. Calcification of the ductus, aneurysmal changes (morphological types D and E), fragile aortic walls due to atheromatous lesions, the presence of friable tissue in the surgical site, pulmonary hypertension associated with aging and endarteritis may increase the operative risk (15). Transcatheter closure avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. Therefore, this remains the procedure of choice for PDA closure in adults. A percutaneous catheter technique for persistent PDA was first described by Portsmann et al (16), who used a conical Ivalon plug in 1967, followed by Rashkind and Cuaso (17), who used an umbrellatype device in 1979. Transcatheter techniques have since evolved and many new devices are commercially available. While many devices have either been used in an off-label manner or have
Exp Clin Cardiol Vol 18 No 1 2013
Amplatzer VSD closure devices for the treatment of PDA
TAble 4 Considerations for device selection based on patent ductus arteriosus (PDA) size Size
Device
Silent PDA
Closure is usually not recommended
TAble 5 Considerations for device selection based on patent ductus arteriosus (PDA) shape (18) Device
Very small PDAs These can be closed with conventional 0.038 inch Gianturco coils Small PDAs
Moderate to large PDAs
May require larger (0.052 inch) Gianturco coils. Often, multiple coils may be necessary to overcome the possibility of residual shunting. Amplatzer ductal occluders (St Jude Medical, USA) have been successfully used Amplatzer duct occluder can be used. A device size at least 2 mm larger than the minimal ductal diameter should be selected
Adapted from Rao (18)
I
For very small or small PDAs, the shape does not influence the choice of the device significantly
II
For moderate to large PDAs, an ADO can be used for most of the angiographical PDA types
III
For long and tubular PDAs, an Amplatzer vascular plug may be more efficacious than an ADO. An ADO II may also be selected in such cases
IV
For ducts that are very short or have an aortico-pulmonary window-like appearance, an Amplatzer atrial septal occluding device may be used. A ventricular septal occluding device may be used when pulmonary arterial pressures are elevated
ADO Amplatzer ductal occluder (St Jude Medical, USA)
undergone clinical trials, only five devices are approved by the Food and Drug Association: the Gianturco coil (1992); the Cook detachable coil (1994); the Flipper detachable coil (1996); the GianturcoGrifka sac (1996); and the Amplatzer duct occluder (ADO) I (1996) (18). The safety and efficacy of the ADO I (AGA Medical Corporation, USA) has been established (19). It is constructed from nitinol wire mesh shaped into a cylindrical plug, with a flared collar to secure the occluder in place. Observed limitations of the ADO I include restrictions in the shape and size of the PDA amenable to transcatheter occlusion, size of the delivery sheath/system and associated complications. In cases in which the PDA makes an acute angle with the aorta (20), the disc of the ADO I may protrude into the aorta and cause obstruction and hemodynamic compromise. An angled ADO with a 320˚ angle at the aortic end, a swivel-disc device and a plug occluder were some of the modifications implemented to address this issue. While the risk of embolization persists in ADO I given its single retention disc, the use of vascular plugs was associated with large residual shunting (21). The ADO II (2009) has two retention discs on either end to secure it to the PDA and to prevent embolization. It has a flexible waist and low-profile discs that adapt to the lumen and orientation of the PDA, thereby avoiding protrusion and obstruction of the aorta or pulmonary artery (22). Both feasibility and efficacy (96% to 100%) of the ADO II to occlude PDA with a minimum diameter >2 mm have been established (22,23). However, the limitation of the ADO II remains the inability to use it with large PDAs because the maximum available waist diameter is 6 mm. Occasionally, Amplatzer vascular plugs and septal occluders have been used in an off-label manner for PDA closure due to the large variation in the shape and size of PDAs (3). The Amplatzer mVSD occluder device has been approved by the Food and Drug Association for transcatheter VSD closure, and has two concentric discs and a wide waist to accommodate the thicker portion of the ventricular septum. mVSD occluders have a higher waist-to-disc ratio (0.44 for the smallest to 0.69 for the largest) compared with the ADO II (0.33 for the smallest to 0.5 for the largest). This, along with
a larger maximum waist diameter (18 mm in the mVSD occluder compared with 6 mm in ADO II), makes it more appropriate for use in large PDAs such as that observed in our patient. use of amplatzer VsD and asD devices for the closure of PDa In cases in which the PDA diameter is large and its length is short, the PDA may be considered to be similar to an aorticopulmonary window for closure purposes. In such instances, a VSD or an ASD closure device may be more appropriate. While the ADO II occluder has a narrower waist, the septal occluders provide a better approximation to the PDA wall with their wider waists. Because 50% of the occlusive effect of these devices originates from the waist and only 25% from each of the discs, selection of the appropriate waist size is crucial to achieving complete occlusion. For the same reason, the ADO II may have resulted in a residual shunt when used in a patient similar to ours, given the short length and larger diameter of PDA. Furthermore, double discs reduce the risk of embolization and have been shown to be effective in patients with elevated pulmonary pressures. Trehan et al (24) reported the successful use of a duct occluder, mVSD occluder and perimembranous VSD occluder in three patients with aorticopulmonary windows. Successful PDA closures using a VSD occluder in two patients was reported by Atiq et al (19) in a trial to assess the efficacy of newer PDA closure devices. While effective closure of PDAs with VSD closure devices has been reported in the literature, more trials assessing its use are needed. The wider waists in these VSD closure devices may be may be important factors to consider in the future development of devices
for the occlusion of large PDAs in adult patients. We have described certain considerations for device selection based on PDA size and shape in Tables 4 and 5, respectively. We also performed a Medline search for reported cases of transcatheter PDA closure using Amplatzer VSD occluder devices (Table 6). DisCLosures: The authors have no financial disclosures or conflicts of interest to declare.
TAble 6 Patent ductus arteriosus (PDA) closure with Amplatzer muscular ventricular septal defect (mVSD) occluder Case number (reference) 1 (25)
Age, years
Sex
Type of PDA
15
Female
Large tubular Type C hypertensive PDA
Female 14 mm large nonrestrictive PDA
Presence of pulmonary hypertension
Device used
Yes
16 mm mVSD occluder
Complications Residual shunt None
None
2 (26)
35
Yes
16 mm mVSD occluder
None
None
3 (27)
35
Male
Large PDA
Yes
16 mm mVSD occluder
None
None
4 (28)
30
Male
Large 18 mm tubular PDA
Yes
26 mm mVSD occluder
None
None
Exp Clin Cardiol Vol 18 No 1 2013
e53
Fernando et al
reFerenCes
1. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med 2000;342:256-63. 2. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873-82. 3. Meadows J, Landzberg MJ. Advances in transcatheter interventions in adults with congenital heart disease. Prog Cardiovasc Dis 2011;53:265-73. 4. Krichenko A, Benson LN, Burrows P, et al. Angiographic classification of the isolated, persistently patent ductus arteriosus and implications for percutaneous catheter occlusion. Am J Cardiol 1989;63:877-80. 5. Campbell M. Patent ductus arteriosus: Some notes on prognosis and on pulmonary hypertension. Br Heart J 1955;17:511-33. 6. Campbell M. Natural history of persistent ductus arteriosus. Br Heart J 1968;30:4-13. 7. Ohtsuka S, Kakihana M, Ishikawa T, et al. Aneurysm of patent ductus arteriosus in an adult case: Findings of cardiac catheterization, angiography, and pathology. Clin Cardiol 1987;10:537-40. 8. Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus: A review of the literature and the surgical implications. Eur J Cardiothorac Surg 1991;5:566-70. 9. Espino-Vela J, Cardenas N, Cruz R. Patent ductus arteriosus: With special reference to patients with pulmonary hypertension. Circulation 1968;38(Suppl 1):45-60. 10. Goitein O, Fuhrman CR, Lacomis JM. Incidental finding on MDCT of patent ductus arteriosus: Use of CT and MRI to assess clinical importance. Am J Roentgenol 2005;184:1924-31. 11. Morgan-Jughes GJ, Marshall AJ, Roobottome C. Morphologic assessment of patent ductus arteriosus in adults using retrospectively ECG-gated multidetector CT. Am J Roentgenol 2003;181:749-54. 12. Hijazi ZM, Ahmad WH, Geggel RL, Marx GR. Intravascular ultrasound during transcatheter coil closure of patent ductus arteriosus: Comparison with angiography. J Invasive Cardiol 1998;10:251-4. 13. Yamaki S, Mohri H, Haneda K, et al. Indications for surgery based on lung biopsy in cases of ventricular septal defect and/or patent ductus arteriosus with severe pulmonary hypertension. Chest 1989;96:31-9. 14. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease: A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958;18:533-47.
e54
15. Bell-Thomson J, Jewell E, Ellis FH Jr, Schwaber JR. Surgical technique in the management of patent ductus arteriosus in the elderly patient. Ann Thorac Surg 1980;30:80-3. 16. Portsmann W, Wierny L, Warnke H. Closure of persistent ductus arteriosus without thoracotomy. Ger Med Monthly 1967;12:259-61. 17. Rashkind WJ, Cuaso CC. Transcatheter closure of a patent ductus arteriosus: Successful use in a 3.5-kg infant. Pediatr Cardiol 1979;1:3-7. 18. Rao PS. Percutaneous closure of patent ductus arteriosus – current status. J Invasive Cardiol 2011;23:517-20. 19. Atiq M, Aslam N, Kazmi KA. Transcatheter closure of small-tolarge patent ductus arteriosus with different devices: Queries and challenges. J Invasive Cardiol 2007;19:295-8. 20. Thanopoulos BD, Hakim FA, Hiari A, et al. Patent ductus arteriosus equipment and technique. Amplatzer duct occluder: Intermediate-term follow-up and technical considerations. J Interv Cardiol 2001;14:247-54 21. Javois AJ, Husayni TS, Thoele D, et al. Inadvertent stenting of patent ductus arteriosus with Amplatzer vascular plug. Cathet Cardiovasc Interv 2006;67:485-9 22. Thanopoulos B, Eleftherakis N, Tzannos K, Stefanadis C. Transcatheter closure of the patent ductus arteriosus using the new Amplatzer duct occluder: Initial clinical applications in children. Am Heart J 2008;156:917e1-917e6. 23. Dua J, Chessa M, Piazza L, et al. Initial experience with the new Amplatzer Duct Occluder II. J Invasive Cardiol 2009;21:401-5. 24. Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv 2008;71:405-11. 25. Demkow M, Ruzyllo W, Siudalska H, Kepka C. Transcatheter closure of a 16 mm hypertensive patent ductus arteriosus with the Amplatzer muscular VSD occluder. Catheter Cardiovasc Interv 2001;52:359-62. 26. Eicken A, Balling G, Gildein HP, et al. Transcatheter closure of a non-restrictive patent ductus arteriosus with an Amplatzer muscular ventricular septal defect occluder. Int J Cardiol 2007;117:e40-2. 27. Hokanson JS, Gimelli G, Bass JL. Percutaneous closure of a large PDA in a 35-year-old man with elevated pulmonary vascular resistance. Congenit Heart Dis 2008;3:149-54. 28. Zhou T, Shen XQ, Zhou SH, et al. Percutaneous closure of huge patent ductus arterious associated with anomalous inferior vein cava drainage and dextrocardia with muscular ventricular septal defect occluder. Chin Med J (Engl) 2006;119:69-72.
Exp Clin Cardiol Vol 18 No 1 2013
Patent ductus arteriosus closure using an AmplatzerTM ventricular septal defect closure device Rajeev Fernando MD1, Ketan Koranne MD1, Pranav Loyalka MD2, Biswajit Kar MD2, Igor Gregoric MD2
r Fernando, K Koranne, P Loyalka, B Kar, i Gregoric. Patent ductus arteriosus closure using an amplatzertM ventricular septal defect closure device. exp Clin Cardiol 2013;18(1):e50-e54. The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids
T
he ductus arteriosus is a physiological fetal structure that connects the descending aorta to the pulmonary artery and spontaneously occludes after birth. Patency of the ductus arteriosus is associated with a variety of complications including left ventricular failure, endarteritis and arrhythmias. These may manifest early in childhood or in adulthood, depending on the size and flow through the ductus. While medical management can improve symptoms and prevent complications, mechanical closure remains the definitive therapy. Since 1967, transcatheter closure of patent ductus arteriosus (PDA) has been constantly evolving, both in terms of the technique and the closure device
Figure 1) Cardiac magnetic resonance image. Coronal section demonstrating patent ductus arteriosus (arrow)
sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs. Key Words: AmplatzerTM ventricular septal defect closure device; Patent ductus arteriosus; Transcatheter closure
used. Multiple occluder devices have been studied and used for PDAs of different shapes, sizes and orientations, but there is currently little evidence regarding the use of Amplatzer (St Jude Medical, USA) muscular ventricular septal defect (mVSD) closure devices for PDA closure. We present an interesting case involving a 35-year-old woman with a large PDA that was successfully occluded with an Amplatzer mVSD occluder.
Case Presentation
A 35-year-old woman presented with a two-month history of progressive dyspnea, bilateral lower extremity swelling and abdominal distension. A physical examination revealed a displaced point of maximum impulse, a high-pitched, continuous murmur, ascites and peripheral edema. Her chest x-ray showed cardiomegaly and interstitial pulmonary edema, and an electrocardiogram revealed sinus tachycardia, inferolateral T wave abnormality and frequent premature ventricular complexes. A transthoracic echocardiogram revealed a PDA with leftto-right shunt (pulmonary flow [Qp]/systemic flow [Qs] ratio of 3.33) and a left ventricular ejection fraction of 45% to 50%. Cardiac magnetic resonance imaging (MRI) revealed a PDA connecting the aortic isthmus (immediately distal and inferior to the left subclavian takeoff) to the main pulmonary artery and excluded coexistent congenital abnormalities (Figures 1, 2 and 3). The patient underwent cardiac catheterization with an aortic angiogram, PDA angiogram, intravascular ultrasound (IVUS) of the PDA and right heart catheterization. A 7 Fr sheath was inserted into the right femoral vein, followed by a Swan Ganz monitor catheter, and right-sided pressures were recorded. The Swan Ganz catheter was removed and a 6 Fr, 4.0 cm Judkins left catheter was inserted in the right femoral artery; left-sided pressures were then recorded. A Magic Torque 0.35 inch × 260 cm guidewire (Boston Scientific, USA) was then inserted via the right femoral artery. The right femoral vein sheath was exchanged for a 9 Fr, 11 cm standard sheath and a multipurpose 6 Fr, 100 cm AL-1 guide catheter (Boston Scientific, USA) was inserted through it. An Amplatzer GooseNeck Snare Kit
1University
of Texas Health Science Center at Houston; 2Center for Advanced Heart Failure, Houston, Texas, USA Correspondence: Dr Rajeev Fernando, University of Texas Health Science Center at Houston, 6411 Fannin Street, Houston, Texas 77030, USA. Telephone 713-876-6115, fax 713-500-6556, e-mail [email protected]
e50
©2013 Pulsus Group Inc. All rights reserved
Exp Clin Cardiol Vol 18 No 1 2013
Amplatzer VSD closure devices for the treatment of PDA
Figure 2) Cardiac magnetic resonance image. Transverse section demonstrating patent ductus arteriosus (arrow) (Covidien, USA) was inserted into the right femoral vein, where it met with the Magic Torque wire and pulled it. IVUS was then performed and images were recorded. The patient was found to have a large 10.5 mm × 3 mm PDA with 2.4:1 Qp:Qs preclosure shunt. Right heart catheterization demonstrated a pulmonary arterial pressure of 24 mmHg/6 mmHg (mean 16 mmHg) with a pulmonary capillary wedge pressure of 9 mmHg/13 mmHg (mean 6 mmHg). A 10 mm mVSD Amplatzer occluder was then advanced to the site of the PDA through a 7 Fr torque delivery system. Its subsequent deployment closed the PDA (Figures 4 and 5). Postprocedure pressures were recorded (Table 1). The patient later underwent placement of a single-chamber implantable cardioverter defibrillator (Boston Scientific, USA) due to symptomatic premature ventricular complexes, with subsequent resolution of symptoms. At her eight-month follow-up, she denied exertional dyspnea, angina or lower extremity edema. Significant variability in the size, shape and orientation of PDAs poses a challenge in selecting the ideal closure device. The Amplatzer mVSD closure device was selected because it has a broader waist (up to 18 mm) and dual discs that ensure stability and reduce the risk of embolization, even with high pulmonary artery pressures.
Figure 3) Cardiac magnetic resonance image. Sagittal section demonstrating patent ductus arteriosus (arrow)
DisCussion
The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch, connecting the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery near the origin of the left pulmonary artery (1,2). It spontaneously occludes 24 h to 48 h after birth through the contraction of the medial smooth muscle in the vessel wall. This is followed by endothelial cell adhesion and replacement of the muscle fibres with connective tissue, resulting in ligamentosum arteriosum within three weeks. Persistence of the duct beyond 48 h after birth is abnormal and results in PDA. It is rare in adults because it is usually detected and treated in childhood. PDAs usually fall into one of three categories, depending on their size and hemodynamic effects: small PDA without apparent hemodynamic significance; moderate-to-large PDA with left heart volume overload and without increased pulmonary vascular resistance; and large PDA with evidence of increased pulmonary vascular resistance (Table 2) (3). Krichenko et al (4) classified isolated PDAs based on anatomical and topological variations. These were identified by their angiographic appearances, which could influence the technique of catheter occlusion. The authors identified five groups using the narrowest segment of the ductus arteriosus as a landmark (Table 3).
Exp Clin Cardiol Vol 18 No 1 2013
Figure 4) Transcatheter closure of patent ductus arteriosus (arrow) using an Amplatzer muscular ventricular septal defect closure device (St Jude Medical, USA) A PDA of moderate size may remain asymptomatic during infancy, but can manifest as fatigue, dyspnea or palpitations during childhood and adult life (5,6). While left ventricular failure is the complication most commonly associated with particularly large PDAs, these patients are also at risk for endarteritis, infective endocarditis, ductal aneurysm and rupture (5,7,8). Eventually, if closure is not performed, pulmonary vascular obstruction may develop with subsequent Eisenmengerization (5,9). Heart failure, pulmonary hypertension and endarteritis are the most common causes of death in patients with PDA without closure (6). The mortality rate of untreated PDAs is estimated to be 1.8% per year.
e51
Fernando et al
TAble 2 Patent ductus arteriosus (PDA) classification based on size and hemodynamic significance Type
Description
Silent PDA
Usually 1.7. While precise anatomical delineation of the PDA is possible with off-axis or orthogonal MRI planes, the anatomical relationship of the aorta, pulmonary artery and PDA can be well defined with magnetic resonance angiography volume-rendered three-dimensional models. It is, therefore, an excellent noninvasive tool to determine both the need for shunt closure and the type of closure device to be used (10). The location, size, shape, presence and extent of calcification, and spatial relationship of PDAs to their surrounding structures can also be
e52
Type
Anatomy
Topology
A
Conical
Narrowest segment at the pulmonary artery end with well-defined aortic ampulla
B
Window
Short duct with narrowing at the aortic insertion
C
Tubular
No constrictions present
D
Complex
Multiple constrictions present
E
Elongated conical Long ductus with constriction at the pulmonary artery end, remote from the anterior tracheal edge
Adapted from Krichenko et al (4)
clearly visualized using retrospectively electrocardiography-gated multidetector computed tomography (MDCT). Volume-rendered three-dimensional reconstruction of the duct classifies that duct into angiographic subgroups while establishing the relationship of the pulmonary insertion of the PDA with the trachea; this can guide therapy (11). IVUS can detect calcification, endarteritis and infection in the PDA and correlates well with angiography (12). Patients with symptomatic PDA may experience improvement with medical management, although the definitive therapy remains surgical closure. It is indicated in all symptomatic patients with a leftto-right shunt and in asymptomatic patients with evidence of left heart enlargement to minimize complications. Yamaki et al (13) suggested that a lung biopsy should be performed to determine operability in patients with pulmonary vascular resistance >8 U/m2. Histological characteristics based on Heath and Edward’s classification (14), such as fibrosis of the arterial and arteriolar media and intima with dilation (grade 5) and necrotizing arteritis (grade 6), may suggest irreversible pulmonary hypertension. A reduction in pulmonary vascular pressures on test occlusion and testing for response to pulmonary vasodilators are additional strategies that may be used to assure reversibility before closure. transcatheter PDa closure Although open thoracotomy for PDA ligation and/or division can be safely performed in children without recurrence, the procedure can be challenging in adults. Calcification of the ductus, aneurysmal changes (morphological types D and E), fragile aortic walls due to atheromatous lesions, the presence of friable tissue in the surgical site, pulmonary hypertension associated with aging and endarteritis may increase the operative risk (15). Transcatheter closure avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. Therefore, this remains the procedure of choice for PDA closure in adults. A percutaneous catheter technique for persistent PDA was first described by Portsmann et al (16), who used a conical Ivalon plug in 1967, followed by Rashkind and Cuaso (17), who used an umbrellatype device in 1979. Transcatheter techniques have since evolved and many new devices are commercially available. While many devices have either been used in an off-label manner or have
Exp Clin Cardiol Vol 18 No 1 2013
Amplatzer VSD closure devices for the treatment of PDA
TAble 4 Considerations for device selection based on patent ductus arteriosus (PDA) size Size
Device
Silent PDA
Closure is usually not recommended
TAble 5 Considerations for device selection based on patent ductus arteriosus (PDA) shape (18) Device
Very small PDAs These can be closed with conventional 0.038 inch Gianturco coils Small PDAs
Moderate to large PDAs
May require larger (0.052 inch) Gianturco coils. Often, multiple coils may be necessary to overcome the possibility of residual shunting. Amplatzer ductal occluders (St Jude Medical, USA) have been successfully used Amplatzer duct occluder can be used. A device size at least 2 mm larger than the minimal ductal diameter should be selected
Adapted from Rao (18)
I
For very small or small PDAs, the shape does not influence the choice of the device significantly
II
For moderate to large PDAs, an ADO can be used for most of the angiographical PDA types
III
For long and tubular PDAs, an Amplatzer vascular plug may be more efficacious than an ADO. An ADO II may also be selected in such cases
IV
For ducts that are very short or have an aortico-pulmonary window-like appearance, an Amplatzer atrial septal occluding device may be used. A ventricular septal occluding device may be used when pulmonary arterial pressures are elevated
ADO Amplatzer ductal occluder (St Jude Medical, USA)
undergone clinical trials, only five devices are approved by the Food and Drug Association: the Gianturco coil (1992); the Cook detachable coil (1994); the Flipper detachable coil (1996); the GianturcoGrifka sac (1996); and the Amplatzer duct occluder (ADO) I (1996) (18). The safety and efficacy of the ADO I (AGA Medical Corporation, USA) has been established (19). It is constructed from nitinol wire mesh shaped into a cylindrical plug, with a flared collar to secure the occluder in place. Observed limitations of the ADO I include restrictions in the shape and size of the PDA amenable to transcatheter occlusion, size of the delivery sheath/system and associated complications. In cases in which the PDA makes an acute angle with the aorta (20), the disc of the ADO I may protrude into the aorta and cause obstruction and hemodynamic compromise. An angled ADO with a 320˚ angle at the aortic end, a swivel-disc device and a plug occluder were some of the modifications implemented to address this issue. While the risk of embolization persists in ADO I given its single retention disc, the use of vascular plugs was associated with large residual shunting (21). The ADO II (2009) has two retention discs on either end to secure it to the PDA and to prevent embolization. It has a flexible waist and low-profile discs that adapt to the lumen and orientation of the PDA, thereby avoiding protrusion and obstruction of the aorta or pulmonary artery (22). Both feasibility and efficacy (96% to 100%) of the ADO II to occlude PDA with a minimum diameter >2 mm have been established (22,23). However, the limitation of the ADO II remains the inability to use it with large PDAs because the maximum available waist diameter is 6 mm. Occasionally, Amplatzer vascular plugs and septal occluders have been used in an off-label manner for PDA closure due to the large variation in the shape and size of PDAs (3). The Amplatzer mVSD occluder device has been approved by the Food and Drug Association for transcatheter VSD closure, and has two concentric discs and a wide waist to accommodate the thicker portion of the ventricular septum. mVSD occluders have a higher waist-to-disc ratio (0.44 for the smallest to 0.69 for the largest) compared with the ADO II (0.33 for the smallest to 0.5 for the largest). This, along with
a larger maximum waist diameter (18 mm in the mVSD occluder compared with 6 mm in ADO II), makes it more appropriate for use in large PDAs such as that observed in our patient. use of amplatzer VsD and asD devices for the closure of PDa In cases in which the PDA diameter is large and its length is short, the PDA may be considered to be similar to an aorticopulmonary window for closure purposes. In such instances, a VSD or an ASD closure device may be more appropriate. While the ADO II occluder has a narrower waist, the septal occluders provide a better approximation to the PDA wall with their wider waists. Because 50% of the occlusive effect of these devices originates from the waist and only 25% from each of the discs, selection of the appropriate waist size is crucial to achieving complete occlusion. For the same reason, the ADO II may have resulted in a residual shunt when used in a patient similar to ours, given the short length and larger diameter of PDA. Furthermore, double discs reduce the risk of embolization and have been shown to be effective in patients with elevated pulmonary pressures. Trehan et al (24) reported the successful use of a duct occluder, mVSD occluder and perimembranous VSD occluder in three patients with aorticopulmonary windows. Successful PDA closures using a VSD occluder in two patients was reported by Atiq et al (19) in a trial to assess the efficacy of newer PDA closure devices. While effective closure of PDAs with VSD closure devices has been reported in the literature, more trials assessing its use are needed. The wider waists in these VSD closure devices may be may be important factors to consider in the future development of devices
for the occlusion of large PDAs in adult patients. We have described certain considerations for device selection based on PDA size and shape in Tables 4 and 5, respectively. We also performed a Medline search for reported cases of transcatheter PDA closure using Amplatzer VSD occluder devices (Table 6). DisCLosures: The authors have no financial disclosures or conflicts of interest to declare.
TAble 6 Patent ductus arteriosus (PDA) closure with Amplatzer muscular ventricular septal defect (mVSD) occluder Case number (reference) 1 (25)
Age, years
Sex
Type of PDA
15
Female
Large tubular Type C hypertensive PDA
Female 14 mm large nonrestrictive PDA
Presence of pulmonary hypertension
Device used
Yes
16 mm mVSD occluder
Complications Residual shunt None
None
2 (26)
35
Yes
16 mm mVSD occluder
None
None
3 (27)
35
Male
Large PDA
Yes
16 mm mVSD occluder
None
None
4 (28)
30
Male
Large 18 mm tubular PDA
Yes
26 mm mVSD occluder
None
None
Exp Clin Cardiol Vol 18 No 1 2013
e53
Fernando et al
reFerenCes
1. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med 2000;342:256-63. 2. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873-82. 3. Meadows J, Landzberg MJ. Advances in transcatheter interventions in adults with congenital heart disease. Prog Cardiovasc Dis 2011;53:265-73. 4. Krichenko A, Benson LN, Burrows P, et al. Angiographic classification of the isolated, persistently patent ductus arteriosus and implications for percutaneous catheter occlusion. Am J Cardiol 1989;63:877-80. 5. Campbell M. Patent ductus arteriosus: Some notes on prognosis and on pulmonary hypertension. Br Heart J 1955;17:511-33. 6. Campbell M. Natural history of persistent ductus arteriosus. Br Heart J 1968;30:4-13. 7. Ohtsuka S, Kakihana M, Ishikawa T, et al. Aneurysm of patent ductus arteriosus in an adult case: Findings of cardiac catheterization, angiography, and pathology. Clin Cardiol 1987;10:537-40. 8. Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus: A review of the literature and the surgical implications. Eur J Cardiothorac Surg 1991;5:566-70. 9. Espino-Vela J, Cardenas N, Cruz R. Patent ductus arteriosus: With special reference to patients with pulmonary hypertension. Circulation 1968;38(Suppl 1):45-60. 10. Goitein O, Fuhrman CR, Lacomis JM. Incidental finding on MDCT of patent ductus arteriosus: Use of CT and MRI to assess clinical importance. Am J Roentgenol 2005;184:1924-31. 11. Morgan-Jughes GJ, Marshall AJ, Roobottome C. Morphologic assessment of patent ductus arteriosus in adults using retrospectively ECG-gated multidetector CT. Am J Roentgenol 2003;181:749-54. 12. Hijazi ZM, Ahmad WH, Geggel RL, Marx GR. Intravascular ultrasound during transcatheter coil closure of patent ductus arteriosus: Comparison with angiography. J Invasive Cardiol 1998;10:251-4. 13. Yamaki S, Mohri H, Haneda K, et al. Indications for surgery based on lung biopsy in cases of ventricular septal defect and/or patent ductus arteriosus with severe pulmonary hypertension. Chest 1989;96:31-9. 14. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease: A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958;18:533-47.
e54
15. Bell-Thomson J, Jewell E, Ellis FH Jr, Schwaber JR. Surgical technique in the management of patent ductus arteriosus in the elderly patient. Ann Thorac Surg 1980;30:80-3. 16. Portsmann W, Wierny L, Warnke H. Closure of persistent ductus arteriosus without thoracotomy. Ger Med Monthly 1967;12:259-61. 17. Rashkind WJ, Cuaso CC. Transcatheter closure of a patent ductus arteriosus: Successful use in a 3.5-kg infant. Pediatr Cardiol 1979;1:3-7. 18. Rao PS. Percutaneous closure of patent ductus arteriosus – current status. J Invasive Cardiol 2011;23:517-20. 19. Atiq M, Aslam N, Kazmi KA. Transcatheter closure of small-tolarge patent ductus arteriosus with different devices: Queries and challenges. J Invasive Cardiol 2007;19:295-8. 20. Thanopoulos BD, Hakim FA, Hiari A, et al. Patent ductus arteriosus equipment and technique. Amplatzer duct occluder: Intermediate-term follow-up and technical considerations. J Interv Cardiol 2001;14:247-54 21. Javois AJ, Husayni TS, Thoele D, et al. Inadvertent stenting of patent ductus arteriosus with Amplatzer vascular plug. Cathet Cardiovasc Interv 2006;67:485-9 22. Thanopoulos B, Eleftherakis N, Tzannos K, Stefanadis C. Transcatheter closure of the patent ductus arteriosus using the new Amplatzer duct occluder: Initial clinical applications in children. Am Heart J 2008;156:917e1-917e6. 23. Dua J, Chessa M, Piazza L, et al. Initial experience with the new Amplatzer Duct Occluder II. J Invasive Cardiol 2009;21:401-5. 24. Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv 2008;71:405-11. 25. Demkow M, Ruzyllo W, Siudalska H, Kepka C. Transcatheter closure of a 16 mm hypertensive patent ductus arteriosus with the Amplatzer muscular VSD occluder. Catheter Cardiovasc Interv 2001;52:359-62. 26. Eicken A, Balling G, Gildein HP, et al. Transcatheter closure of a non-restrictive patent ductus arteriosus with an Amplatzer muscular ventricular septal defect occluder. Int J Cardiol 2007;117:e40-2. 27. Hokanson JS, Gimelli G, Bass JL. Percutaneous closure of a large PDA in a 35-year-old man with elevated pulmonary vascular resistance. Congenit Heart Dis 2008;3:149-54. 28. Zhou T, Shen XQ, Zhou SH, et al. Percutaneous closure of huge patent ductus arterious associated with anomalous inferior vein cava drainage and dextrocardia with muscular ventricular septal defect occluder. Chin Med J (Engl) 2006;119:69-72.
Exp Clin Cardiol Vol 18 No 1 2013
