Fig. 7.1
(a) Amplatzer Septal Occluder and (b) Occlutech Figulla Flex II
7.4 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 [16].
In patients with secundum septal defect, two crucial parameters, those 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, which should be assessed to select patients for procedure. The maximum defect diameter must be less than 38 mm. Most of ASDs have ellipsoidal shape, and it 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 undergone the procedure without balloon sizing or multiple defects. Transcatheter closure of large ASD with a maximal native diameter of >30 mm is still challenging, and alternative special techniques for deployment of the device are usually required. In regard to the 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 is <5 mm (Fig. 7.2) [17].
Fig. 7.2
The assessment of surrounding rims using two-dimensional (2D) TEE. Surrounding tissue rims and ASD diameter should be measured in the phase of ventricular end systole with multiple cross-sectional TEE plane. Distances from ASD to aorta (superoanterior rim; 2D TEE view at 0°–30°), superior vena cava (superoposterior rim; 2D TEE view at 90°–120°), right upper pulmonary vein (posterior rim; 2D TEE view at 110°–120°), inferior vena cava (inferoposterior rim; 2D TEE view at 60°–90°), coronary sinus (2D TEE view at 100°–120°), and atrioventricular valve (inferoanterior rim; 2D TEE view at 135°) are assessed. RUPV right upper pulmonary vein, CS coronary sinus
7.5 Cardiac Erosion and Its Mechanism
In patients with superoanterior rim deficiency, the risk of serious complication, so-called cardiac erosion may increase after the device implantation. Although the definite mechanism of “cardiac erosion” has not been established completely, previous clinical experiences suggested that an aortic rim deficiency and oversized occlusion device might be at highly related with cardiac erosion [18, 19]. However, a large number of cases with an aortic rim deficiency resulted in a successful deployment without complicating cardiac erosion. Morphological factors additional to an aortic rim deficiency should be considered in cases with cardiac erosion.
Atrial septal malalignment is a morphological characteristic frequently encountered in cases with a deficient aortic rim. Surfaces arising from septum primum and septum secundum are different in a defect with malaligned atrial septum resulting in vertical displacement and tight impingement of the right atrial disk toward the right atrium. As the device is deployed against the atrial septum from the left atrium side, the right atrium disk moved toward to the left atrium side after the release. Atrial septal malalignment causes a change in the device axis angle against the aortic root and may be a risk for cardiac erosion in catheter closure of ASD using Amplatzer Septal Occluder (Fig. 7.2). Although the observation of the both side of the disks before and after the release of the device is important, assessment of this situation before the device deployment is difficult. Device deployment against an ASD complicated with a deficient aortic rim can result in a splay of the disks across the aortic root with impingement of the device. Atrial septal malalignment can swell the tight impingement of the device, especially after releasing the cable. Thus, atrial septal malalignment may be a potential risk factor for cardiac erosion [20].
Recently, the instruction for user (IFU) of Amplatzer Septal occluder has been updated especially for avoiding cardiac erosion [19]. That is, the contraindication for defect margins less than 5 mm has been updated to include the inferior vena cava rim, because such defect character tend to be caused of oversized device selection.
7.6 Imaging Modality for Transcatheter ASD Closure
Two dimensional (2D) and color Doppler transthoracic echocardiography (TTE) can demonstrate the presence of ASDs, chamber dilatation, estimated pulmonary artery pressure, shunt ratio, and other coexisting heart disease with high sensitivity and specificity in real time. And the advent of tissue Doppler imaging could facilitate the understanding of cardiac diastolic function in which impaired cardiac function before ASD closure may lead to the development of congestive heart failure after ASD closure especially in elderly patients [20]. However, in terms of accurate assessment of ASD morphology including measurements of maximal diameter and surrounding rims, 2D TTE has sometimes limited ability to visualize ASDs in detail clearly especially in adult patients; thus precise evaluation using transesophageal echocardiography (TEE) are necessary in most ASD patients.
Three-dimensional (3D) echocardiography provides better spatial visualization, and 3D TEE can delineate the 3D structure with high-resolution image. As the result, 3D echocardiography offers the ability to improve display and our understanding of complex lesions such as valvular and congenital heart disease [19–24]. In addition, although 3D echocardiography was initially based on reconstructed image from serial 2D images, which required cumbersome acquisition and time-consuming offline analysis, recently real-time 3D echocardiography using matrix array transducer has been available in TTE as well as TEE. 3D TTE is a promising modality to provide comprehensible en face image of ASD because of its noninvasiveness, low cost, portability, and wide availability (Fig. 7.3). In terms of patient selection for transcatheter ASD closure, 3D TTE has a potential to provide accurate information of ASD morphology including location, size, and surrounding rims for the treatment both in children and adults. However, there are several limitations of 3D TTE at present such as dependence on the skill of the operator, restrictive echo window especially in elderly patients, and echo dropout in the region of the mid portion which can lead to false diagnoses of large defects and both lower temporal and spatial resolutions compared to 2D TTE. On the other hand, ASD morphology can be recognized with high-quality en face image using 3D TEE. Real-time 3D TEE allows for the evaluation of various shape of ASD especially in patients with complex-shaped ASD like multiple ASDs (Fig. 7.4) [19, 22, 23]. Nowadays, intracardiac echocardiography is also available for acceptable echocardiographic guidance for this procedure, especially in simple and small defect (Fig. 7.5).
Fig. 7.3
Various shape of ASD visualized by 3D TEE (left atrial en face view). (a) Sufficient rim, (b) deficient superoanterior rim, (c) deficient inferoposterior rim, and (d) deficient both superoanterior and inferoposterior rim. Arrow head indicates the portion of rim deficiency. RUPV right upper pulmonary vein, Ao aorta
Fig. 7.4
ICE-guided transcatheter ASD closure. (a) Short axis view of phased-array ICE demonstrating the anterosuperior rim deficiency before closure. (b) Balloon sizing ASD diameter was 22 mm. (c) ASD was closed with 22 mm device. LA left atrium, RA right atrium
Fig. 7.5
Catheter closure of multiple ASDs. (a) 3D-TEE imaging shows isolated two defects; large defect was 24 mm, and small defect was 7 mm. (b) Balloon sizing was performed simultaneously both defects. (c) A 28 mm and 10 mm devices were deployed
7.7 Various Closure Technique for Difficult ASD
Various technical modifications were reported for transcatheter ASD closure. These are the modification of delivery sheath, deployment position, or additional material to hold the left atrial disk inside the left atrium, avoiding the left atrial disk slipping into the right atrial side [24].
Rotation of the delivery sheath within the heart, or increasing the curvature of the sheath by remolding outside the body, has been described to improve alignment [25]. Some have advocated deployment of the left atrial disk in the right or left upper pulmonary vein followed by pulling of the sheath into the right atrium to improve the approach to the atrial septum in large defects [26]. Concerns exist in this technique causing injury of the pulmonary vein. A specially designed sheath (Hausdorf sheath, Cook, Bloomington, IN) can be used to help align the left atrial disk parallel to the atrial septum [26–28]. Favorable changes in the approach to the interatrial septum and increased ability to deliver the device have been reported with the Hausdorf sheath [28]. Kutty and colleagues developed a method to modify a Mullins transseptal sheath to enhance delivery [29]. The resulting sheath is straight and has an exit orifice essential in the side of the distal portion of the sheath-a straight, side-hole delivery sheath [29]. The use of a balloon catheter [30] or a long dilator [31] to support the left atrial disk and prevent its prolapse into the right atrium during deployment has been described. Although this technique requires additional venous access, the procedure, especially balloon-assist technique, is relatively simple and less traumatic. Even in the large ASD, high procedure success can be expected. Operators are not required to be familiarized all of these techniques; however, operators should master one or two of different techniques to achieve the procedure successfully for difficult patients or defect anatomy.
7.8 Hemodynamic Features of Difficult Transcatheter ASD Closure
7.8.1 Pulmonary Arterial Hypertension and ASD
Not only morphologically, hemodynamic features have influences on difficulties of transcatheter ASD closure. Candidates for ASD closures have a hemodynamically significant atrial shunt or the presence of right ventricular volume overload and/or clinical symptoms of dyspnea, reduced exercise capacity, or paradoxical embolism. Pulmonary vascular resistance < 5 Wood units.m2 and the peak pulmonary artery pressure ≤70% of the systemic blood pressure are also important conditions for ASD closure. Although the most of pediatric patients with ASD fulfill these hemodynamic criteria, the incidence of pulmonary artery hypertension significantly increased in adult population. It is well known that the natural course in patients with ASD and pulmonary hypertension is significantly worse than patients without pulmonary hypertension. Thus, closure of ASD in patients with pulmonary hypertension was considered as a high-risk procedure, especially by the surgical closure. However, once transcatheter closure without cardiopulmonary bypass can be performed even in such high-risk patients, several clinical studies have demonstrated the efficacy and safety of ASD closure. Importantly, the more significant reduction of pulmonary artery pressure can be achieved even in severe pulmonary hypertension [32, 33].
Furthermore, the expansion of therapeutic indication is considered under the combination of new pulmonary hypertension-specific medical treatment, such as prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors. Even in the initial hemodynamic parameter seems to be untreatable (or contraindication) for ASD closure, catheter closure of ASD may be performed if such pulmonary vascular resistance can be considered as responder for pulmonary artery-specific vasodilators [34, 35]. Long-term follow-up is mandatory especially these high-risk populations (Fig. 7.6).
Fig. 7.6
Clinical efficacy of treat and repair strategies in ASD patients with severe pulmonary hypertension: even in patients required PAH-specific medication, long-term event-free ratio is equivalent compared with patients without PAH-specific medication
7.8.2 Management of Elderly Patients with ASD
In the past, surgical correction of ASD in geriatric population has not been indicated positively, because of difficulties of perioperative management and high incidence of comorbidities. Additionally, geriatric patients by themselves did not wish open heart surgery, even if hemodynamic benefit can be expected. However, after the introduction of the transcatheter closure of ASD, the experiences of older or geriatric patients are significantly increasing [11, 14].
In the elderly patients with ASD, hemodynamic features are significantly different from those in children and young adults. Elderly patients with ASD frequently present with hemodynamic abnormalities such as pulmonary hypertension, atrial arrhythmias, and valvular regurgitation, which causes congestive heart failure [14]. The incidence of pulmonary hypertension significantly increase by age in ASD patients; the decision of ASD closure is sometimes difficult especially in patients with severe pulmonary hypertension. Moreover, various comorbidities, such as systemic hypertension, chronic obstructive pulmonary disease, coronary artery disease, chronic kidney disease, and left ventricular diastolic dysfunction often complicate the clinical features in this population (Fig. 7.7). Left ventricular diastolic dysfunction, which is also seen as part of normal aging and frequently occurs in elderly individuals with hypertension or increased arterial stiffness, may cause acute congestive heart failure after ASD closure [13]
Fig. 7.7
Catheter closure of an 82-year-old patient with large ASD and permanent atrial fibrillation. (a) PCW was continuously monitored during the device deployment. In this case, ICE was used as imaging guidance. (b) 32 mm device was deployed, and no significant elevation of PCW pressure was observed
In our institution, approximately 10% of cases of transcatheter closure of ASD were patients whose aged older than 70 years. Most of these patients had at least one major comorbidity, including systemic hypertension, stroke, coronary artery disease, and atrial fibrillation. More than half of the patients were being treated with a diuretic for congestive heart failure, and 30% of the patients had a history of hospitalization due to heart failure. Majority of patients complicated with symptoms of heart failure are classified as NYHA functional class more than class II [14].