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CASE REPORT
Year : 2014  |  Volume : 27  |  Issue : 1  |  Page : 145-151

Atrial septal defects: clinical presentation and recent approach in its diagnosis and treatment


Cardiology Department, Faculty of Medicine, National Heart Institute, Menoufia University, Giza, Egypt

Date of Submission13-Feb-2013
Date of Acceptance07-May-2013
Date of Web Publication20-May-2014

Correspondence Address:
Mohammed A Alrefaey Atwa
MB BCh, Albakashine, Kafr Shoukr, Alkalubia
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-2098.132788

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  Abstract 

Objective
This study aimed to review the atrial septal defects (ASDs), describing their types, presentations, and different lines of management.
Data summary
ASDs are the second most common congenital lesion in adults (after bicuspid aortic valves). They represent ∼7% of all cardiac anomalies. Transcatheter closure of a secundum ASD is now widely accepted as an alternative to surgical closure. With currently available devices and techniques, ∼80-90% of secundum ASDs can be closed percutaneously. In this review, we summarize the literatures of ASD in terms of its closure.
Conclusion
Closure of secundum ASD percutaneously has become the standard of care in pediatric and adult patients. Patients of all ages experience reduction in pulmonary artery pressure and right ventricular size, and an improvement in functional capacity after percutaneous device closure of ASD, and these improvements appear to be greater if the defect is closed earlier.

Keywords: Atrial septal defect, congenital heart defect, percutaneous device closure


How to cite this article:
Badran HM, Soltan GM, Alrefaey Atwa MA. Atrial septal defects: clinical presentation and recent approach in its diagnosis and treatment. Menoufia Med J 2014;27:145-51

How to cite this URL:
Badran HM, Soltan GM, Alrefaey Atwa MA. Atrial septal defects: clinical presentation and recent approach in its diagnosis and treatment. Menoufia Med J [serial online] 2014 [cited 2020 Feb 27];27:145-51. Available from: http://www.mmj.eg.net/text.asp?2014/27/1/145/132788


  Introduction Top


Atrial septal defects (ASDs) are the second most common congenital lesion in adults (after bicuspid aortic valves). They represent ∼7% of all cardiac anomalies. These defects are often undetected until adulthood because of the lack of prominent clinical symptoms initially. If untreated, an ASD can eventually result in right ventricular (RV) heart failure, pulmonary hypertension (PHTN), atrial arrhythmias, or paradoxical embolization and ischemic cerebral events [1].

Often, atrial tachyarrhythmias may coexist or precede symptoms. A study has shown that the incidence of arrhythmias increases with age as well as an increase in pulmonary pressure. However, it is still unclear whether atrial arrhythmias improve with the closure of the defect, although some trials have shown that atrial flutter may improve with ASD closure as opposed to atrial fibrillation, which usually remains unchanged following closure. As a result, the development of an atrial tachyarrhythmia alone does not constitute an immediate need for ASD closure [2].

Traditionally, as shown in the pediatric literature, patients with a significant pulmonary to systemic blood flow (Qp/Qs) ratio 1.5 or more have experienced the most benefit after ASD closure. One study in adults has shown that asymptomatic and mildly symptomatic patients with a Qp/Qs as low as 1.2 may also benefit from ASD closure [3].

ASD repair with the transcatheter technique has been shown to have a high closure rate. Unfortunately, the anatomy of the defect often limits their use. Currently, transcatheter closure is limited to secundum-type defects that are less than 36 mm in size and there are two devices approved by the FDA in USA: The Amplatzer septal occluder (ASO; AGA Medical, Golden Valley, Minnesota, USA) and the Helex (W.L. Gore and Associates Inc, Flagstaff, AZ, USA) closure device. The benefit of the percutaneous approach has been well demonstrated in the pediatric population [4].

Patients with severe fixed PHTN may actually worsen with ASD closure because of the need for partial right-to-left shunting of blood to decrease right-sided pressures. Early diagnosis and follow-up of ASDs offers the best opportunity to avoid late complications from PHTN, heart failure, arrhythmia, and stroke [5].

Objectives

This study aimed to review the ASDs, describing their types, presentations, and different lines of management.

Data summary

Data source

Data were obtained from previous literatures, reviews, and studies as well as medical websites (PubMed, MD consult) and scientific journals.

Study selection

Selection was performed by supervisors for the study of new advancements in percutaneous transcatheter device closure of ASDs with different approaches.

Data extraction

In this review, data from published studies were manually extracted and summarized.

Data synthesis

In this review, the data indicated that several studies of transcatheter closure for ASDs were included. There are different methods for closure of ASDs with different indications. We obtained our data by studying the different approaches of ASDs closure to determine which is preferred taking into consideration different complications of the approaches.


  Atrial septal defects Top


There are four different types of ASDs, described as following [Figure 1]:

(1) A secundum ASD is the most common cause of an atrial level shunt. Usually, the defect is because of deficiency of septum primum, the valve of the fossa ovalis, but rarely, it results from a deficiency of septum secundum. The defect may be single or multiple, with several fenestrations.
Figure 1:

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(2) A primum ASD is a variant of an incomplete common atrioventricular canal and is the third most common interatrial communication. This defect involves the septum of the atrioventricular canal and is almost always associated with a cleft anterior mitral leaflet.

(3) A sinus venosus septal defect results from deficiency of the sinus venosus septum, which separates the pulmonary veins from the systemic veins and the sinus venosus component of the right atrium (RA). Most commonly, a sinus venosus defect is between the right upper pulmonary vein and the cardiac end of the superior vena cava. Rarely, the defect involves the right lower and/or the middle pulmonary veins and the inferior aspect of the RA at its junction with the inferior vena cava.

(4) A coronary sinus septal defect is a rare type in which the septum between the coronary sinus and the left atrium is either partially or completely unroofed, allowing the right and left atria to communicate through the defect and the coronary sinus orifice. The association of a coronary sinus septal defect and persistent left superior vena cava is termed Raghib's syndrome, and may result in cyanosis [6].

The size of interatrial communication is an important determinant of the magnitude of the shunt [Figure 2]. Because of the lower resistances involved, interatrial communication tends to be larger than a ventricular defect for a shunt of similar magnitude [7].
Figure 2:

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ASDs less than 3 mm in diameter invariably close by 18 months of age and are generally considered as patent foramen ovale. Spontaneous closure has been reported to occur in anywhere between 14 and 66% of ASDs. Factors such as smaller size of defect and earlier age at diagnosis led to early spontaneous closure reporting an increased likelihood of spontaneous closure in those diagnosed before two years of age [8],[9].

Elective surgical closure of moderate-sized to large-sized ASDs is recommended between 4 and 6 years of age. Since the realization of transcatheter closure, first performed in 1976 by King and colleagues, it has become an attractive alternative to surgery, obviating the need for sternotomy and potential complications [10],[11].

An ASD is diagnosed in childhood; the ASD diameter, when untreated, increases in 65% of cases, and 30% will show more than a 50% increase in diameter. Only 4% of ASDs close spontaneously [12].

Without treatment over time, even small ASDs can develop increased left to right shunting because of a progressive increase in left ventricular (LV) diastolic pressure with aging, which causes increased left atrial pressure. In patients who develop PHTN from their ASD, ∼10% will progress to Eisenmenger's syndrome [13].

Atrial septal defects treatment: transcatheter closure versus surgery

In 1999, Berger et al. [15] published a prospective study comparing the results and complications of surgical closure using different approaches [Figure 3] and [Table 1] and Amplatzer device closure of ASDs with a Qp/Qs ratio of 1.5/1 or more. Sixty-one patients underwent surgery at a median age of 20 years and 61 patients underwent defect closure with an Amplatzer device at a median age of 12 years. Hospital stay in surgically treated patients was 8 days versus 3 days in interventionally treated patients. ASDs and shunt sizes were larger in the surgical group; closure rate in the two groups was identical (98%). One patient in the surgical group had a perforated duodenal ulcer that necessitated operation 8 days after closure of the ASD and one had an infected lateral thoracotomy wound necessitating plastic surgery. One patient in the other group had embolization of the device to the left ventricle and vascular surgery was required to extract the device from the femoral artery [15].
Figure 3:

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Table 1: Surgical approaches to atrial septal defects (Argenziano et al. [14])

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In recent years, transcatheter closure has become an alternative to operations for the treatment of ASDs. However, this procedure may be unsuccessful or complicated and requires surgical treatment. A study showed that an operation was required after transcatheter closure of an ASD or a patent foramen ovale in 8% of patients. After device complications, the ASD and the patent foramen ovale can still successfully be closed surgically, with good results and low morbidity. However, serious complications such as cardiac perforation may have a fatal outcome. Residual shunt, dislocation, or vascular complications are the most frequent problems that require surgical interventions [16].

Du et al. [17] published the results of a multicenter, nonrandomized concurrent study that was carried out in 29 pediatric cardiology centers from March 1998 to March 2000. The patients were assigned to either the device or the surgical closure group according to the patients' options. Baseline physical exams and echocardiography were performed before the procedure and at follow-up (6 and 12 months for the device group and 12 months for the surgical group). The early, primary, and secondary efficacy success rates for surgical versus device closure of ASD were not statistically different; however, the complication rate was lower and the length of hospital stay was shorter for the device closure group than for the surgical repair group [17].

Benefit of percutaneous atrial septal defect occlusion versus surgery in adults

King and Mills attempted the first transcatheter closure of secundum ASD in 1976. Over the next three decades, many different devices and techniques have been introduced [Figure 4] and [Table 2]. There are currently two such devices that are FDA approved in USA [Table 3]. The first one is the ASO device [Figure 5], which is a prosthesis that consists of two round nitinol wire disks connected together with a short connecting disk. It can be used in small, moderate, and large ASDs. The second is the Helex septal occluder [Figure 6], which is a low-profile device that has a double disk non-self-centering design.

Percutaneous ASD closure results not only in symptomatic improvement and increase in exercise capacity but also in improvements in cardiac chamber geometry and in cardiac hemodynamics. Majunke et al. [18] studied 650 consecutive adults with a median age of 45 who underwent closure with ASO. The patients had a mean PASP of 33.3 mmHg. Implantation was successful in 98% of patients. Complete closure was achieved in 96% of patients (22 of 25 of the incomplete closures had a very small residual shunt). The mean pulmonary artery systolic pressure (PASP) decreased to 28.3 mmHg. Intraprocedural complications were observed in two patients with device embolization, one patient with transient ST depression. A total of 0.9% needed emergent cardio thoracic surgery [18].
Figure 4:

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Figure 5:

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Figure 6:

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Table 2: Beneficial effects of percutaneous atrial septal defect closure in adults (Majunke et al. [18])

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Table 3: Devices for closure of atrial septal defect (Butera et al. [19])

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Others have shown that ASD closure with an ASO device leads to a significant decrease in RV size (RV end-diastolic diameter 35.3-29.2 mm in first 24 h) and improvement in clinical symptoms, with a very high success rate (112 of 113). As such, the authors suggest that ASD closure device should be the first option for the management of secundum ASDs [20].

Similar encouraging results have been reported in the Helex closure device. Jones et al. [21] compared Helex septal occluder with surgery at 12 months of follow-up and found that closure rate, and major and minor adverse outcomes were not different.

Concerns in terms of the potential development of mitral regurgitation were essentially put to rest when Wilson et al. [4] reported that 2-year follow-up data in 227 adult and children after ASO showed 98.5% success for closure rate, resolution of atrial fibrillation (AF) in half of the patients after the procedure, and unchanged degree of mitral regurge (MR) in 88% of patients (in 1%, MR increased by two grades and in 9% there was an increase of one grade; 7% showed a decrease in MR).

The comparison of the surgical versus the percutaneous approach was reported in a large cohort of 596 pediatric patients in a nonrandomized parallel group study. In this study, 442 patients received ASO device closure and 154 received surgical closure. The success rate was 95.7% in the ASO group and 100% in the surgical group. No mortality was observed in either group; however, the rate of complications was significantly lower in the device group (7.2% ASO vs. 24.0% surgical). The length of stay was shorter in the transcatheter arm (1.0 vs. 3.4 days). A similar rate of success (98%) with an increased length of stay (8 vs. 3 days) was observed by other studies [15],[17].

Indications and contraindications to percutaneous atrial septal defect closure

The ACC/AHA guidelines suggest that secundum ASDs larger than 5 mm should be closed in the following scenarios as long as the pulmonary artery (PA) pressure is less than two-third of systemic pressure, pulmonary vascular resistance is less than two-third of systemic vascular resistance, or when the patient is responsive to either pulmonary vasodilator therapy or test occlusion of the defect [Table 4] and [Table 5]:

  1. Presence of symptoms such as exercise intolerance, shortness of breath, heart failure, or atrial arrhythmias;
  2. Evidence of RV or RA dilatation on echocardiogram or cardiac computed tomography/MRI, with or without symptoms;
  3. Presence of paradoxical embolism irrespective of the size of the defect;
  4. Documentation of orthodeoxia-platypnea.
Table 4: List of recommendations (Baumgartner et al. [22])

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Table 5: Levels of evidence (Baumgartner et al. [22])

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Contraindications are as follows:

  1. Presence of other congenital cardiac deformities such as anomalous pulmonary venous drainage, or ostium primum, sinus venosus, or coronary sinus ASD that would require surgical correction (should be closed surgically).
  2. Severe irreversible PHTN and no evidence of left to right shunt. Most operators agree that the majority of secundum ASDs can be closed percutaneously. When it is not feasible, the surgical approach is recommended. Defect size of more than 40 mm and lack of adequate rims of tissue from the defects to important surrounding structures, atrial thrombus, and contraindication to antiplatelet therapy are included in this category [23].


Changes in cardiac chamber size and hemodynamics after closure

The effect of ASD closure on cardiac chambers following either surgical or percutaneous closure is well documented. In a study examining a group of pediatric and adult patients after successful device closures of ASD, Kaya et al. [24] observed that over a 2-year follow-up period, RV end-diastolic diameter decreased from 36 to 30 mm and LV end-diastolic diameter increased from 33 to 37 mm. Furthermore, there was a significant reduction in PAP on echocardiogram and a clinically significant improvement in exercise capacity [24].

Another analysis of patient groups in the 40-60 years showed that when ASD was diagnosed after the age of 40 years, there was more PHTN, tricuspid regurgitation, and RV dilatation. On follow-up after closure, the older patient population showed a greater reduction in PA pressure (19 vs. 11.3%) [25].


  Discussion Top


ASDs account for 5-10% of all congenital heart defects. These defects should be closed when diagnosed during childhood or adulthood because they lead to right atrial and ventricular volume load, arrhythmias, and paradoxical embolism [26].

Transcatheter closure of ASD has become an important alternative to surgical repair in the management of patients with secundum-type ASD [27].

As a result of ASD closure, the right heart is protected from volume load, leading to reductions in both pulmonary artery pressure and right heart cavity dimensions. Thus, a significant symptomatic improvement with a decrease in arrhythmic events is observed in these patients [28].

Kaya et al. [29] performed ASD transcatheter closure in 117 patients (48 males and 69 females) with secundum-type ASD. The mean age of these patients was 15 ± 12 years (range, 2-65 years). A total of 112 patients had successful device closure (96%). Because of migration of the Amplatzer device into the main pulmonary artery of a 4-year-old male patient and easy displacement of the device during procedural maneuvers in three male patients (ages 7, 31, and 47 years), the procedure was stopped and surgical treatment was recommended and performed by surgeons [29].

Oliveira et al. [30] studied ASD closure over a period of 3 years using the Occulotech device in 120 patients with hemodynamic significant ASD ranging in age from 10 to 76 years (mean, 46 years); 66% were females. All patients showed a significant reduction or normalization of the RV and no residual shunt [30].

Chien et al. [31] studied 40 patients (15 males and 25 females, mean age 11.7 ± 7.8 years) with secundum ASDs who underwent transcatheter closure. In group 1, 30 patients underwent the procedure using both balloon sizing and transthoracic echocardiography sizing. In 10 patients (group 2), transthoracic echocardiography (TTE) sizing was used as the sole tool for selecting device size [31].

Kaya et al. [29] studied patients with a mean ASD diameter measured by transoesophageal echocardiography of 14.0 ± 4.2 mm (range, 5-27 mm) and a mean ASD diameter measured by balloon sizing of 16.6 ± 4.8 mm (range, 6-30 mm). The mean Amplatzer device diameter used in closure of the ASD was 18.6 ± 4.9 mm (range, 5-36 mm). There was a linear association between the ASD diameter measured by transesophageal echocardiography (TEE) and the ASD diameter measured by catheter balloon sizing (r = 0.826) and also between the ASD diameter measured by balloon sizing and the Amplatzer device diameter (r = 0.786; P = 0.001) [29].

Chien et al. [31] showed that transthoracic echocardiographic sizing is a safe and ideal method to measure interatrial defects and choose the occluding device, respectively.

In our review, we reported the techniques in device deployment and decreased right heart volume after ASD closure using transcatheter device closure. Thus, reduction in PAP and right heart cavity dimensions was established. Previous studies have shown a significant cardiac remodeling early after percutaneous ASD closure.


  Conclusion Top


An ASD is the second common congenital heart disease in adults. Transcatheter ASD closure is an important alternative to surgical repair in the management of patients with secundum type. It is a simple, effective technique, with improvement in heart cavity dimensions and avoidance of sternotomy and cardiopulmonary bypass. Residual shunt, dislocation, or vascular complications are the most frequent problems that require a surgical intervention. The efficacy of both procedures is equal, with a lower rate of complications in transcatheter closure and significant cost reduction because of shorter recovery and shorter hospital stay.


  Acknowledgements Top


Conflicts of interest

There are no conflicts of interest.

 
  References Top

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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