Journal of Cardiology 65 (2015) 17–25
Contents lists available at ScienceDirect
Journal of Cardiology journal homepage: www.elsevier.com/locate/jjcc
Review
Current concept of transcatheter closure of atrial septal defect in adults Teiji Akagi (MD, PhD, FJCC, FACC, FSCAI, FAHA)* Adult Congenital Heart Disease Center, Okayama University Hospital, Okayama, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 24 August 2014 Accepted 25 August 2014 Available online 11 October 2014
After the introduction of catheter intervention for atrial septal defect (ASD) in the pediatric population, therapeutic advantages of this less invasive procedure were focused on adult through geriatric populations. The most valuable clinical benefits of this procedure are the significant improvement of symptoms and daily activities, which result from the closure of left to right shunt without thoracotomy and cardiopulmonary bypass surgery. These benefits contribute to increase the number of adult patients of this condition who have hesitated over surgical closure. In terms of technical point of view for catheter closure of ASD, the difficulties still exist in some morphological features of defect, or hemodynamic features in the adult population. Morphological features of difficult ASD closure are (1) large (30 mm) ASD, (2) wide rim deficiency, and (3) multiple defects. Hemodynamic features of difficult ASD are (1) severe pulmonary hypertension, (2) ventricular dysfunction, and (3) restrictive left ventricular compliance (diastolic dysfunction) after ASD closure. To complete the catheter ASD closure under these difficult conditions, various procedural techniques have been introduced. These are new imaging modalities such as real-time three-dimensional imaging, new technical modifications, and new concepts for hemodynamic evaluation. Especially, real-time three-dimensional transesophageal echocardiography can provide the high quality imaging for anatomical evaluation including maximum defect size, surrounding rim morphology, and the relationship between device and septal rim. In adult patients, optimal management for their comorbidities is an important issue, which includes cardiac function, atrial arrhythmias, respiratory function, and renal function. Management of atrial arrhythmias is a key issue for the long-term outcome in adult patients. Because the interventional procedures are not complication-free techniques, the establishment of a surgical back-up system is essential for the safe achievement of the procedure. Finally, the establishment of a team approach including pediatric and adult cardiologists, cardiac surgeons, and anesthesiologists is the most important factor for a good therapeutic outcome. ß 2014 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
Keywords: Atrial septal defect Three-dimensional imaging Atrial arrhythmia Congestive heart failure
Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Clinical feature of ASD in various aged populations . . . . . . . . . Transcatheter closure of ASD . . . . . . . . . . . . . . . . . . . . . . . . . . . Morphological features of difficult transcatheter ASD closure. . Cardiac erosion and its mechanism . . . . . . . . . . . . . . . . . . . . . . Imaging modality for transcatheter ASD closure . . . . . . . . . . . . Various closure techniques for difficult ASD . . . . . . . . . . . . . . . Hemodynamic features of difficult transcatheter ASD closure . Pulmonary arterial hypertension and ASD. . . . . . . . . . . . Specific issues for management of elderly patients with Future direction of transcatheter intervention for ASD . . . . . . . Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
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* Correspondence to: Cardiac Intensive Care Unit, Okayama University Hospital, 2-5-1, Shikata-cho, Okayama, Okayama 700-8558, Japan. Tel.: +81 86 235 7357; fax: +81 86 235 7683. E-mail address: [email protected] http://dx.doi.org/10.1016/j.jjcc.2014.09.002 0914-5087/ß 2014 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
18 18 18 18 19 20 20 22 22 22 24 25 25
[(Fig._2)TD$IG]
18
T. Akagi / Journal of Cardiology 65 (2015) 17–25
Trigger of ASD diagnosis
Introduction
2% Atrial septal defect (ASD) accounts for 7% of all congenital heart diseases. The most common ASD is a secundum defect, versus defects located in the septum primum, sinus venosus defects, or unroofed coronary sinus [1]. If left untreated, these defects may result in right-sided heart failure, arrhythmia, and pulmonary hypertension. Although surgical closure of ASD is safe, effective, and time-tested, it requires open heart surgery and hospitalization [2]. Even in the current surgical procedure, complications which are related to surgery or cardiopulmonary bypass have not disappeared.
heart murmur
5%
symptoms
23%
17%
arrhythmia heart failure examinaon
11%
11%
23%
8%
cerebral infarcon cerebral abscess pulmonary embolism other disease unknown
Clinical feature of ASD in various aged populations Clinical features of ASD are widely varied from pediatric population through adults [3–6]. The majority of these children are asymptomatic and diagnosed by school physical examination, heart murmur detected by primary care pediatrician, and cardiac echocardiographic screening in the newborn period. If the defect is smaller than 6 mm, spontaneous closure can be expected during newborn to pediatric periods [7]. Operation is scheduled based on children’s body size, usually before the elementary school with low incidence of mortality rate. On the contrary, adult patients with ASD are usually symptomatic. Their heart disease may be discovered as a result of palpitation, arrhythmia, or progress of congestive heart failure. In our recent series of adult patients with ASD, they were diagnosed within 3 years before the procedure, meaning that the majority of adult patients could not be diagnosed during the pediatric period under the current medical screening system (Fig. 1) (unpublished data). In 1990 Murphy and colleagues reported the natural course of ASD [2]. In this study, survival rate was significantly deteriorated compared to the general population, if patients did not have surgical closure until age of 24 years and/or there were complications with pulmonary hypertension. However, our current patients’ backgrounds are significantly different compared to such studies, rather numerous adult patients are asymptomatic and occasionally diagnosed at the time of detailed cardiovascular evaluation for atrial arrhythmia, congestive heart failure, or stroke (Fig. 2). Although the catheter closure of pediatric or young adults with ASD population has attracted research interest over the past two decades, that of the elderly ASD population has yet to be characterized [8]. Extrapolation of studies on younger patients is not appropriate in the geriatric patients. First, elder patients with ASD acquire comorbid conditions include arrhythmia, hypertension, [(Fig._1)TD$IG]respiratory distress, kidney disease, etc. that always play a
Interval from Diagnosis to Intervenon
16% within 3 years within 3 5 years
12%
over 5years
72%
Fig. 1. Interval from the diagnosis of atrial septal defect to intervention in adult patients age older than 40 years. More than 80% of patients were diagnosed within 5 years before the intervention.
Fig. 2. Trigger of diagnosis of adult patients with atrial septal defect (ASD) age older than 40 years. Symptoms (palpitation, shortness of breath, etc.) and routine physical examination are the most common factors for trigger of diagnosis.
significant role in their heart conditions [4,9]. Second, elder ASD patients may have inherently superior resilience, milder disease, or balanced physiology in contrast to those not surviving to an advanced age. Therefore, elder or geriatric patients with ASD represent a distinct population for which focused studies are needed. Transcatheter closure of ASD Transcatheter closure of ASD is associated with low complication rates, short anesthetic times, and short hospitalizations [5]. When conditions are favorable, transcatheter ASD closure has become the treatment of choice rather than surgery in many institutions. Echocardiography, either transesophageal (TEE) or intracardiac, plays a significant role in the guidance of these procedures and in the assessment of the final result. Research efforts are ongoing to examine other imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI), as a means of three-dimensional (3D) imaging prior to transcatheter ASD closure. To date, Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA) is widely used in the world. This device is suitable for all subtypes of ASD and has successfully closed defects as large as 38 mm in diameter. Much larger sized devices are available, such as Occlutech device (Helsingborg, Sweden) [10] or Lifetech Cera devices (Shenzhen, China) [11]. However, procedural difficulties still exist due to morphological features of septal defect or from a hemodynamic stand point. Morphological features of difficult transcatheter ASD closure It is well known that morphological variations of ASD are frequent and appropriate patient selection for transcatheter ASD closure is crucial for successful procedure. ASDs are grouped into four major categories: ostium primum, ostium secundum, sinus venosus, and coronary sinus septal defect. Secundum defect is the most common type of ASDs in which the defect involves the region of fossa ovale, and this type is indicated for transcatheter ASD closure. Coronary sinus septal defect is a rare type, in which a communication occurs between the coronary sinus and the left atrium as a result of unroofed coronary sinus. Primum septal defect and sinus venosus defect are indicated for surgical repair. Regarding coronary sinus septal defect, although surgical repair is the standard treatment for this type of ASD, there are some case reports in which transcatheter closure was successful without any conduction disturbance [12]. In patients with secundum septal defect, two crucial parameters, which are the maximal ASD diameter in order to select a device with the appropriate size and the surrounding rim dimensions to optimize the placement of the device, should be
T. Akagi / Journal of Cardiology 65 (2015) 17–25
assessed to select patients for procedure. The maximum defect diameter must be less than 38 mm. Most ASDs have ellipsoidal shape and that varies during cardiac cycle. The major axis diameter of the defect measured in the phase of ventricular end systole is mandatory for selecting the optimal device size, especially in patients undergoing the procedure without balloon sizing or multiple defects. Transcatheter closure of large ASD with a maximal native diameter >30 mm is challenging, and alternative special techniques for deployment of the device are usually required. In regard to classification of surrounding rims, although there are some differences among studies, distances from ASD to aorta (superoanterior rim), superior vena cava (superoposterior rim), right upper pulmonary vein (posterior rim), inferior vena cava (inferoposterior rim), coronary sinus, and atrioventricular valve (inferoanterior rim) are assessed. The definition of rim deficiency varies among different studies; any rim was considered deficient if its length was 20 mmHg, we judge such ASD closure could lead to the development of pulmonary edema and the procedure should be abandoned (Fig. 10). Especially in patients who had a history of heart failure, we considered to be hemodynamically high-risk patients. Creation of a fenestration hall in the device may avoid the abrupt hemodynamic change after the transcatheter closure of ASD. However, the optimal fenestration size has not been evaluated, and the experiences are still limited [36]. Although the majority of elder patients were complicated with various comorbidities, a high procedural success rate can be expected even in this aged population. Also, significant improvement in NYHA functional class was observed after closure even though about 30% of the patients in this study had a history of hospitalization for congestive heart failure. No patient required additional hospitalization for congestive heart failure during the follow-up period. Atrial arrhythmias are the most commonly seen comorbidities in adult patients with ASD. In our experience, approximately one third of patients aged over 60 years, or half of patients aged over 70 years, are complicated with atrial fibrillations. Previously, we reported the clinical benefits of catheter closure of ASD in patients with permanent atrial fibrillation [37]. Even in patients complicated with atrial fibrillation, the resolution of left to right shunt contributes significant improvement of clinical symptoms of these patients. Although, strict anticoagulation treatment is required even after the ASD closure, transcatheter closure of ASD should be considered even in patients with permanent atrial fibrillation without age limitation (Fig. 10). In patients complicated with symptomatic paroxysmal or persistent atrial fibrillation, catheter ablation such as pulmonary vein isolation should be indicated prior to the catheter closure of ASD. In our institution, pulmonary vein isolation is indicated in patients aged younger than 75 years, symptomatic paroxysmal or persisted atrial arrhythmias, and left atrial dimension 3 months after the ablation. Although the experiences are still limited, the collaborations
Fig. 10. Catheter closure in an 82-year-old patient with large atrial septal defect and permanent atrial fibrillation. (a) Pulmonary capillary wedge pressure (PCW) was continuously monitored during the device deployment. (b) 32-mm device was deployed and no significant elevation of PCW pressure was observed.
between electrophysiological and interventional procedures can contribute to new therapeutic strategies in patients with ASD and atrial arrhythmias. Future direction of transcatheter intervention for ASD Transcatheter closure of ASD using currently available devices has been established as safe and effective medical treatment for most patients with ASD. Although some specific anatomical features are not suitable for this procedure, the remaining patients are probably treatable by cardiac surgery. On the other side, complications due to this procedure, especially cardiac erosion, are still observed in a certain number patients. The mechanism of cardiac erosion has not been clarified completely, some erosion occurred even in the long-term after the procedure. Thus, longitudinal follow-up after this procedure is essential. Other important concerns, such as device-induced late atrial arrhythmias, atrioventricular valve regurgitation, and reaction of myocardial tissue should be checked in the future. The target of this procedure has been expanded from the pediatric population to adults, including the geriatric population. In this regard, the collaboration between adult and pediatric cardiologists has to be conducted as total therapeutic management
T. Akagi / Journal of Cardiology 65 (2015) 17–25
in this procedure. If possible, the establishment of an adult congenital heart disease specialist in each institution, which involved these interventional procedures, is the most desirable direction for optimal patient care. Acknowledgments The author is deeply thankful to Drs Yasufumi Kijima, Koji Nakagawa, Yoichi Takaya, Manabu Taniguchi, and Nobuhisa Watanabe for their devoted contributions for patient care and research work. References [1] Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–900. [2] Murphy JG, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, McGoon DC, Puga FJ, Kirklin JW, Danielson GK. Long-term outcome after the surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med 1990;323:1645–50. [3] Masura J, Gavora P, Formanek A, Hijazi ZM. Transcatheter closure of secundum atrial septal defects using the new self-centering Amplatzer septal occluder: initial human experience. Catheter Cardiovasc Diagn 1997;42:388–93. [4] Nakagawa K, Akagi T, Taniguchi M, Kijima Y, Kusano K, Itoh H, Sano S. Transcatheter closure of atrial septal defect in a geriatric population. Catheter Cardiovasc Interv 2012;80:84–90. [5] Du ZD, Koenig P, Cao QL, Waight D, Heitschmidt M, Hijazi ZM. Comparison of transcatheter closure of secundum atrial septal defect using the Amplatzer septal occluder associated with deficient versus sufficient rims. Am J Cardiol 2002;90:865–9. [6] Oho S, Ishizawa A, Akagi T, Dodo H, Kato H. Transcatheter closure of atrial septal defects with the Amplatzer septal occluder – a Japanese clinical trial. Circ J 2002;66:791–4. [7] Radzik D, Davignon A, van Doesburg N, Fournier A, Marchand T, Ducharme G. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol 1993;22:851–3. [8] Schubert S, Peters B, Abdul-Khaliq H, Nagdyman N, Lange PE, Ewert P. Left ventricular conditioning in the elderly patient to prevent congestive heart failure after transcatheter closure of atrial septal defect. Catheter Cardiovasc Interv 2005;64:333–7. [9] Ho¨rer J, Eicken A, Mu¨ller S, Schreiber C, Cleuziou J, Prodan Z, Holper K, Lange R. Risk factors for prolonged intensive care treatment following atrial septal defect closure in adults. Int J Cardiol 2008;125:57–61. ¨ zkutlu S, Canpolat U, Kaya EB, Yorgun H, S¸ahiner L, Sunman [10] Aytemir K, Oto A, O H, Ates¸ AH, Kabakc¸ı G. Transcatheter interatrial septal defect closure in a large cohort: midterm follow-up results. Congenit Heart Dis 2013;8:418–27. [11] Hayes N, Rosenthal E. Tulip malformation of the left atrial disc in the Lifetech Cera ASD device: a novel complication of percutaneous ASD closure. Catheter Cardiovasc Interv 2012;79:675–7. [12] Kijima Y, Taniguchi M, Akagi T. Catheter closure of coronary sinus atrial septal defect using Amplatzer septal occluder. Cardiol Young 2012;22:223–6. [13] Amin Z, Hijazi Z, Bass J, Cheatham JP, Hellenbrand WE, Kleinman CS. Erosion of Amplatzer septal occluder device after closure of secundum atrial septal defects: review of registry of complications and recommendations to minimize future risk. Catheter Cardiovasc Interv 2004;63:496–502. [14] Kijima Y, Akagi T, Nakagawa K, Promphan W, Toh N, Nakamura K, Sano S, Ito H. Cardiac erosion after catheter closure of atrial septal defect: septal malalignment may be a novel risk factor for erosion. JC Cases 2014;9:134–7. [15] Amin Z. Echocardiographic predictors of cardiac erosion after Amplatzer septal occluder placement. Catheter Cardiovasc Interv 2014;83:84–92. [16] Ewert P, Berger F, Nagdyman N, Kretschmar O, Dittrich S, Abdul-Khaliq H, Lange P. Masked left ventricular restriction in elderly patients with atrial septal defects: a contraindication for closure? Catheter Cardiovasc Interv 2001;52:177–80.
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[17] Jategaonkar S, Scholtz W, Schmidt H, Horstkotte D. Percutaneous closure of atrial septal defects: echocardiographic and functional results in patients older than 60 years. Circ Cardiovasc Interv 2009;2:85–9. [18] Taniguchi M, Akagi T. Real-time imaging for transcatheter closure of atrial septal defects. Interv Cardiol 2011;3:679–94. [19] Taniguchi M, Akagi T, Watanabe N, Okamoto Y, Nakagawa K, Kijima Y, Toh N, Ohtsuki S, Kusano K, Sano S. Application of real-time three-dimensional transesophageal echocardiography using a matrix array probe for transcatheter closure of atrial septal defect. J Am Soc Echocardiogr 2009;10:1114–20. [20] Baker GH, Shirali G, Ringewald JM, Hsia TY, Bandisode V. Usefulness of live three-dimensional transesophageal echocardiography in a congenital heart disease center. Am J Cardiol 2009;103:1025–8. [21] van den Bosch AE, Ten Harkel DJ, McGhie JS, Roos-Hesselink JW, Simoons ML, Bogers AJ, Meijboom FJ. Characterization of atrial septal defect assessed by real-time 3-dimensional echocardiography. J Am Soc Echocardiogr 2006;19: 815–21. [22] Roberson DA, Cui W, Patel D, Tsang W, Sugeng L, Weinert L, Bharati S, Lang RM. Three-dimensional transesophageal echocardiography of atrial septal defect: a qualitative and quantitative anatomic study. J Am Soc Echocardiogr 2011;24:600–10. [23] Cao Q, Radtke W, Berger F, Zhu W, Hijazi ZM. Transcatheter closure of multiple atrial septal defects. Initial results and value of two- and three-dimensional transesophageal echocardiography. Eur Heart J 2000;21:941–7. [24] Watanabe N, Taniguchi M, Akagi T, Tanabe Y, Toh N, Kusano K, Ito H, Koide N, Sano S. Usefulness of the right parasternal approach to evaluate the morphology of atrial septal defect for transcatheter closure using two-dimensional and three-dimensional transthoracic echocardiography. J Am Soc Echocardiogr 2012;376–82. [25] Harper RW, Mottram PM, McGaw DJ. Closure of secundum atrial septal defects with the Amplatzer septal occluder device: techniques and problems. Catheter Cardiovasc Interv 2002;57:508–24. [26] Varma C, Benson LN, Silversides C, Yip J, Warr MR, Webb G, Siu SC, McLaughlin PR. Outcomes and alternative techniques for device closure of the large secundum atrial septal defect. Catheter Cardiovasc Interv 2004;61:131–9. [27] Pedra CAC, Pedra SRF, Esteves CA, Cassar R, Pontes Jr SC, Braga SLN, Fontes VF. Transcatheter closure of secundum atrial septal defects with complex anatomy. J Invasive Cardiol 2004;16:117–22. [28] Nagm AM, Rao PS. Percutaneous occlusion of complex atrial septal defects. J Invasive Cardiol 2004;16:123–5 [commentary]. [29] Staniloae CS, El-Khally Z, Ibrahim R, Dore A, de Guise P, Mercier L. Percutaneous closure of secundum atrial septal defects in adults – a single center experience with the Amplatzer septal occluder. J Invasive Cardiol 2003;15: 393–7. [30] Kutty S, Asnes JD, Srinath G, Preminger TJ, Prieto LR, Latson LA. Use of a straight, side-hole delivery sheath for improved delivery of Amplatzer ASD occluder. Catheter Cardiovasc Interv 2007;69:15–20. [31] Dalvi BV, Pinto RJ, Gupta A. New technique for device closure of large atrial septal defects. Catheter Cardiovasc Interv 2005;64:102–7. [32] Wahab HA, Bairam AR, Cao QL, Hijazi ZM. Novel technique to prevent prolapse of the Amplatzer septal occluder through large atrial septal defect. Catheter Cardiovasc Interv 2003;60:543–5. [33] Khan AA, Tan JL, Li W, Dimopoulos K, Spence MS, Chow P, Mullen MJ. The impact of transcatheter atrial septal defect closure in the older population: a prospective study. JACC Cardiovasc Interv 2010;3:276–81. [34] Yong G, Khairy P, De Guise P, Dore A, Marcotte F, Mercier LA, Noble S, Ibrahim R. Pulmonary arterial hypertension in patients with transcatheter closure of secundum atrial septal defects: a longitudinal study. Circ Cardiovasc Interv 2009;2:455–62. [35] Hirabayashi A, Miyaji K, Akagi T. Continuous epoprostenol therapy and septal defect closure in a patient with severe pulmonary hypertension. Catheter Cardiovasc Interv 2009;73:688–91. [36] Holzer R, Cao QL, Hijazi ZM. Closure of a moderately large atrial septal defect with a self-fabricated fenestrated Amplatzer septal occluder in an 85-year-old patient with reduced diastolic elasticity of the left ventricle. Catheter Cardiovasc Interv 2005;64:513–8. [37] Taniguchi M, Akagi T, Ohtsuki S, Okamoto Y, Tanabe Y, Watanabe N, Nakagawa K, Toh N, Kusano K, Sano S. Transcatheter closure of atrial septal defect in elderly patients with permanent atrial fibrillation. Catheter Cardiovasc Interv 2009;73:682–6.
Contents lists available at ScienceDirect
Journal of Cardiology journal homepage: www.elsevier.com/locate/jjcc
Review
Current concept of transcatheter closure of atrial septal defect in adults Teiji Akagi (MD, PhD, FJCC, FACC, FSCAI, FAHA)* Adult Congenital Heart Disease Center, Okayama University Hospital, Okayama, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 24 August 2014 Accepted 25 August 2014 Available online 11 October 2014
After the introduction of catheter intervention for atrial septal defect (ASD) in the pediatric population, therapeutic advantages of this less invasive procedure were focused on adult through geriatric populations. The most valuable clinical benefits of this procedure are the significant improvement of symptoms and daily activities, which result from the closure of left to right shunt without thoracotomy and cardiopulmonary bypass surgery. These benefits contribute to increase the number of adult patients of this condition who have hesitated over surgical closure. In terms of technical point of view for catheter closure of ASD, the difficulties still exist in some morphological features of defect, or hemodynamic features in the adult population. Morphological features of difficult ASD closure are (1) large (30 mm) ASD, (2) wide rim deficiency, and (3) multiple defects. Hemodynamic features of difficult ASD are (1) severe pulmonary hypertension, (2) ventricular dysfunction, and (3) restrictive left ventricular compliance (diastolic dysfunction) after ASD closure. To complete the catheter ASD closure under these difficult conditions, various procedural techniques have been introduced. These are new imaging modalities such as real-time three-dimensional imaging, new technical modifications, and new concepts for hemodynamic evaluation. Especially, real-time three-dimensional transesophageal echocardiography can provide the high quality imaging for anatomical evaluation including maximum defect size, surrounding rim morphology, and the relationship between device and septal rim. In adult patients, optimal management for their comorbidities is an important issue, which includes cardiac function, atrial arrhythmias, respiratory function, and renal function. Management of atrial arrhythmias is a key issue for the long-term outcome in adult patients. Because the interventional procedures are not complication-free techniques, the establishment of a surgical back-up system is essential for the safe achievement of the procedure. Finally, the establishment of a team approach including pediatric and adult cardiologists, cardiac surgeons, and anesthesiologists is the most important factor for a good therapeutic outcome. ß 2014 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
Keywords: Atrial septal defect Three-dimensional imaging Atrial arrhythmia Congestive heart failure
Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Clinical feature of ASD in various aged populations . . . . . . . . . Transcatheter closure of ASD . . . . . . . . . . . . . . . . . . . . . . . . . . . Morphological features of difficult transcatheter ASD closure. . Cardiac erosion and its mechanism . . . . . . . . . . . . . . . . . . . . . . Imaging modality for transcatheter ASD closure . . . . . . . . . . . . Various closure techniques for difficult ASD . . . . . . . . . . . . . . . Hemodynamic features of difficult transcatheter ASD closure . Pulmonary arterial hypertension and ASD. . . . . . . . . . . . Specific issues for management of elderly patients with Future direction of transcatheter intervention for ASD . . . . . . . Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
.... .... .... .... .... .... .... .... .... ASD .... .... ....
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* Correspondence to: Cardiac Intensive Care Unit, Okayama University Hospital, 2-5-1, Shikata-cho, Okayama, Okayama 700-8558, Japan. Tel.: +81 86 235 7357; fax: +81 86 235 7683. E-mail address: [email protected] http://dx.doi.org/10.1016/j.jjcc.2014.09.002 0914-5087/ß 2014 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.
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[(Fig._2)TD$IG]
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T. Akagi / Journal of Cardiology 65 (2015) 17–25
Trigger of ASD diagnosis
Introduction
2% Atrial septal defect (ASD) accounts for 7% of all congenital heart diseases. The most common ASD is a secundum defect, versus defects located in the septum primum, sinus venosus defects, or unroofed coronary sinus [1]. If left untreated, these defects may result in right-sided heart failure, arrhythmia, and pulmonary hypertension. Although surgical closure of ASD is safe, effective, and time-tested, it requires open heart surgery and hospitalization [2]. Even in the current surgical procedure, complications which are related to surgery or cardiopulmonary bypass have not disappeared.
heart murmur
5%
symptoms
23%
17%
arrhythmia heart failure examinaon
11%
11%
23%
8%
cerebral infarcon cerebral abscess pulmonary embolism other disease unknown
Clinical feature of ASD in various aged populations Clinical features of ASD are widely varied from pediatric population through adults [3–6]. The majority of these children are asymptomatic and diagnosed by school physical examination, heart murmur detected by primary care pediatrician, and cardiac echocardiographic screening in the newborn period. If the defect is smaller than 6 mm, spontaneous closure can be expected during newborn to pediatric periods [7]. Operation is scheduled based on children’s body size, usually before the elementary school with low incidence of mortality rate. On the contrary, adult patients with ASD are usually symptomatic. Their heart disease may be discovered as a result of palpitation, arrhythmia, or progress of congestive heart failure. In our recent series of adult patients with ASD, they were diagnosed within 3 years before the procedure, meaning that the majority of adult patients could not be diagnosed during the pediatric period under the current medical screening system (Fig. 1) (unpublished data). In 1990 Murphy and colleagues reported the natural course of ASD [2]. In this study, survival rate was significantly deteriorated compared to the general population, if patients did not have surgical closure until age of 24 years and/or there were complications with pulmonary hypertension. However, our current patients’ backgrounds are significantly different compared to such studies, rather numerous adult patients are asymptomatic and occasionally diagnosed at the time of detailed cardiovascular evaluation for atrial arrhythmia, congestive heart failure, or stroke (Fig. 2). Although the catheter closure of pediatric or young adults with ASD population has attracted research interest over the past two decades, that of the elderly ASD population has yet to be characterized [8]. Extrapolation of studies on younger patients is not appropriate in the geriatric patients. First, elder patients with ASD acquire comorbid conditions include arrhythmia, hypertension, [(Fig._1)TD$IG]respiratory distress, kidney disease, etc. that always play a
Interval from Diagnosis to Intervenon
16% within 3 years within 3 5 years
12%
over 5years
72%
Fig. 1. Interval from the diagnosis of atrial septal defect to intervention in adult patients age older than 40 years. More than 80% of patients were diagnosed within 5 years before the intervention.
Fig. 2. Trigger of diagnosis of adult patients with atrial septal defect (ASD) age older than 40 years. Symptoms (palpitation, shortness of breath, etc.) and routine physical examination are the most common factors for trigger of diagnosis.
significant role in their heart conditions [4,9]. Second, elder ASD patients may have inherently superior resilience, milder disease, or balanced physiology in contrast to those not surviving to an advanced age. Therefore, elder or geriatric patients with ASD represent a distinct population for which focused studies are needed. Transcatheter closure of ASD Transcatheter closure of ASD is associated with low complication rates, short anesthetic times, and short hospitalizations [5]. When conditions are favorable, transcatheter ASD closure has become the treatment of choice rather than surgery in many institutions. Echocardiography, either transesophageal (TEE) or intracardiac, plays a significant role in the guidance of these procedures and in the assessment of the final result. Research efforts are ongoing to examine other imaging modalities, such as computed tomography (CT) or magnetic resonance imaging (MRI), as a means of three-dimensional (3D) imaging prior to transcatheter ASD closure. To date, Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA) is widely used in the world. This device is suitable for all subtypes of ASD and has successfully closed defects as large as 38 mm in diameter. Much larger sized devices are available, such as Occlutech device (Helsingborg, Sweden) [10] or Lifetech Cera devices (Shenzhen, China) [11]. However, procedural difficulties still exist due to morphological features of septal defect or from a hemodynamic stand point. Morphological features of difficult transcatheter ASD closure It is well known that morphological variations of ASD are frequent and appropriate patient selection for transcatheter ASD closure is crucial for successful procedure. ASDs are grouped into four major categories: ostium primum, ostium secundum, sinus venosus, and coronary sinus septal defect. Secundum defect is the most common type of ASDs in which the defect involves the region of fossa ovale, and this type is indicated for transcatheter ASD closure. Coronary sinus septal defect is a rare type, in which a communication occurs between the coronary sinus and the left atrium as a result of unroofed coronary sinus. Primum septal defect and sinus venosus defect are indicated for surgical repair. Regarding coronary sinus septal defect, although surgical repair is the standard treatment for this type of ASD, there are some case reports in which transcatheter closure was successful without any conduction disturbance [12]. In patients with secundum septal defect, two crucial parameters, which are the maximal ASD diameter in order to select a device with the appropriate size and the surrounding rim dimensions to optimize the placement of the device, should be
T. Akagi / Journal of Cardiology 65 (2015) 17–25
assessed to select patients for procedure. The maximum defect diameter must be less than 38 mm. Most ASDs have ellipsoidal shape and that varies during cardiac cycle. The major axis diameter of the defect measured in the phase of ventricular end systole is mandatory for selecting the optimal device size, especially in patients undergoing the procedure without balloon sizing or multiple defects. Transcatheter closure of large ASD with a maximal native diameter >30 mm is challenging, and alternative special techniques for deployment of the device are usually required. In regard to classification of surrounding rims, although there are some differences among studies, distances from ASD to aorta (superoanterior rim), superior vena cava (superoposterior rim), right upper pulmonary vein (posterior rim), inferior vena cava (inferoposterior rim), coronary sinus, and atrioventricular valve (inferoanterior rim) are assessed. The definition of rim deficiency varies among different studies; any rim was considered deficient if its length was 20 mmHg, we judge such ASD closure could lead to the development of pulmonary edema and the procedure should be abandoned (Fig. 10). Especially in patients who had a history of heart failure, we considered to be hemodynamically high-risk patients. Creation of a fenestration hall in the device may avoid the abrupt hemodynamic change after the transcatheter closure of ASD. However, the optimal fenestration size has not been evaluated, and the experiences are still limited [36]. Although the majority of elder patients were complicated with various comorbidities, a high procedural success rate can be expected even in this aged population. Also, significant improvement in NYHA functional class was observed after closure even though about 30% of the patients in this study had a history of hospitalization for congestive heart failure. No patient required additional hospitalization for congestive heart failure during the follow-up period. Atrial arrhythmias are the most commonly seen comorbidities in adult patients with ASD. In our experience, approximately one third of patients aged over 60 years, or half of patients aged over 70 years, are complicated with atrial fibrillations. Previously, we reported the clinical benefits of catheter closure of ASD in patients with permanent atrial fibrillation [37]. Even in patients complicated with atrial fibrillation, the resolution of left to right shunt contributes significant improvement of clinical symptoms of these patients. Although, strict anticoagulation treatment is required even after the ASD closure, transcatheter closure of ASD should be considered even in patients with permanent atrial fibrillation without age limitation (Fig. 10). In patients complicated with symptomatic paroxysmal or persistent atrial fibrillation, catheter ablation such as pulmonary vein isolation should be indicated prior to the catheter closure of ASD. In our institution, pulmonary vein isolation is indicated in patients aged younger than 75 years, symptomatic paroxysmal or persisted atrial arrhythmias, and left atrial dimension 3 months after the ablation. Although the experiences are still limited, the collaborations
Fig. 10. Catheter closure in an 82-year-old patient with large atrial septal defect and permanent atrial fibrillation. (a) Pulmonary capillary wedge pressure (PCW) was continuously monitored during the device deployment. (b) 32-mm device was deployed and no significant elevation of PCW pressure was observed.
between electrophysiological and interventional procedures can contribute to new therapeutic strategies in patients with ASD and atrial arrhythmias. Future direction of transcatheter intervention for ASD Transcatheter closure of ASD using currently available devices has been established as safe and effective medical treatment for most patients with ASD. Although some specific anatomical features are not suitable for this procedure, the remaining patients are probably treatable by cardiac surgery. On the other side, complications due to this procedure, especially cardiac erosion, are still observed in a certain number patients. The mechanism of cardiac erosion has not been clarified completely, some erosion occurred even in the long-term after the procedure. Thus, longitudinal follow-up after this procedure is essential. Other important concerns, such as device-induced late atrial arrhythmias, atrioventricular valve regurgitation, and reaction of myocardial tissue should be checked in the future. The target of this procedure has been expanded from the pediatric population to adults, including the geriatric population. In this regard, the collaboration between adult and pediatric cardiologists has to be conducted as total therapeutic management
T. Akagi / Journal of Cardiology 65 (2015) 17–25
in this procedure. If possible, the establishment of an adult congenital heart disease specialist in each institution, which involved these interventional procedures, is the most desirable direction for optimal patient care. Acknowledgments The author is deeply thankful to Drs Yasufumi Kijima, Koji Nakagawa, Yoichi Takaya, Manabu Taniguchi, and Nobuhisa Watanabe for their devoted contributions for patient care and research work. References [1] Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–900. [2] Murphy JG, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, McGoon DC, Puga FJ, Kirklin JW, Danielson GK. Long-term outcome after the surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med 1990;323:1645–50. [3] Masura J, Gavora P, Formanek A, Hijazi ZM. Transcatheter closure of secundum atrial septal defects using the new self-centering Amplatzer septal occluder: initial human experience. Catheter Cardiovasc Diagn 1997;42:388–93. [4] Nakagawa K, Akagi T, Taniguchi M, Kijima Y, Kusano K, Itoh H, Sano S. Transcatheter closure of atrial septal defect in a geriatric population. Catheter Cardiovasc Interv 2012;80:84–90. [5] Du ZD, Koenig P, Cao QL, Waight D, Heitschmidt M, Hijazi ZM. Comparison of transcatheter closure of secundum atrial septal defect using the Amplatzer septal occluder associated with deficient versus sufficient rims. Am J Cardiol 2002;90:865–9. [6] Oho S, Ishizawa A, Akagi T, Dodo H, Kato H. Transcatheter closure of atrial septal defects with the Amplatzer septal occluder – a Japanese clinical trial. Circ J 2002;66:791–4. [7] Radzik D, Davignon A, van Doesburg N, Fournier A, Marchand T, Ducharme G. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol 1993;22:851–3. [8] Schubert S, Peters B, Abdul-Khaliq H, Nagdyman N, Lange PE, Ewert P. Left ventricular conditioning in the elderly patient to prevent congestive heart failure after transcatheter closure of atrial septal defect. Catheter Cardiovasc Interv 2005;64:333–7. [9] Ho¨rer J, Eicken A, Mu¨ller S, Schreiber C, Cleuziou J, Prodan Z, Holper K, Lange R. Risk factors for prolonged intensive care treatment following atrial septal defect closure in adults. Int J Cardiol 2008;125:57–61. ¨ zkutlu S, Canpolat U, Kaya EB, Yorgun H, S¸ahiner L, Sunman [10] Aytemir K, Oto A, O H, Ates¸ AH, Kabakc¸ı G. Transcatheter interatrial septal defect closure in a large cohort: midterm follow-up results. Congenit Heart Dis 2013;8:418–27. [11] Hayes N, Rosenthal E. Tulip malformation of the left atrial disc in the Lifetech Cera ASD device: a novel complication of percutaneous ASD closure. Catheter Cardiovasc Interv 2012;79:675–7. [12] Kijima Y, Taniguchi M, Akagi T. Catheter closure of coronary sinus atrial septal defect using Amplatzer septal occluder. Cardiol Young 2012;22:223–6. [13] Amin Z, Hijazi Z, Bass J, Cheatham JP, Hellenbrand WE, Kleinman CS. Erosion of Amplatzer septal occluder device after closure of secundum atrial septal defects: review of registry of complications and recommendations to minimize future risk. Catheter Cardiovasc Interv 2004;63:496–502. [14] Kijima Y, Akagi T, Nakagawa K, Promphan W, Toh N, Nakamura K, Sano S, Ito H. Cardiac erosion after catheter closure of atrial septal defect: septal malalignment may be a novel risk factor for erosion. JC Cases 2014;9:134–7. [15] Amin Z. Echocardiographic predictors of cardiac erosion after Amplatzer septal occluder placement. Catheter Cardiovasc Interv 2014;83:84–92. [16] Ewert P, Berger F, Nagdyman N, Kretschmar O, Dittrich S, Abdul-Khaliq H, Lange P. Masked left ventricular restriction in elderly patients with atrial septal defects: a contraindication for closure? Catheter Cardiovasc Interv 2001;52:177–80.
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