Transcatheter Closure of Secundum Atrial Septal Defectin a Patient With Dextrocardia Using the Amplatzer

Septal Occluder

Fakhri Hakim, 1 MD, Awni Madani, 1 MD, Yousef Samara, 1 MD, Ibrahim Abu Ata, 1 MD,Aktham Hiari, 1 MD, Yousef Goussous, 1 MD, FSCAI, and Ziyad M. Hijazi, 2* MD, MPH, FSCAI

Transcatheter closure of secundum atrial septal defects (ASD) in patients with levocardiais performed routinely using various investigational devices. A 6-yr-old child withdextrocardia, situs inversus, and secundum ASD measuring 13 mm by TEE underwentsuccessful transcatheter closure using a 15 mm Amplatzer Septal Occluder with completeclosure of the defect. Cathet. Cardiovasc. Diagn. 43:291–294, 1998. r 1998 Wiley-Liss, Inc.

Key words: catheter closure; ASD; dextrocardia

INTRODUCTION

Atrial septal defect (ASD) is a common form ofcongenital heart disease accounting for,7% of alldefects [1]. The secundum type is the most common andis amenable for transcatheter closure techniques. The eraof transcatheter closure of ASD began in 1976 when Kinget al. [2] reported the first human application of a doubleumbrella device. However, because of the large deliverycatheter (23F) needed to introduce the umbrella, thisdevice was not adopted by many cardiologists. Rashkinddeveloped a single, self-expandable umbrella with hooksto close ASDs. This device underwent limited clinicaltrials that were stopped because of the low success rate ofimplantation [3]. Subsequently, few other devices (clam-shell, buttoned, Angle Wings, ASDOS, and Amplatzer)have been evaluated in humans with levocardia andsecundum ASD with variable degrees of successfuldeployment and closure [4–9].

Dextrocardia with situs inversus is the third mostcommon type of dextrocardia in a series of autopsiedcases [10]. The most common subtype of this group is thesitus inversus of the viscera and atria (I), L-loop ven-tricles (L), and normally related great arteries of theinverted type (I), i.e.,5I,L,I 6. This kind of rightsided heartcan be structurally normal; however, congenital heartdisease is the rule occurring in 5/7 autopsies [10].Associated cardiac anomalies include tetralogy of Fallotof the inverted type, ventricular septal defect, and atrialseptal defect. To our knowledge, there are no reports ofASD device closure in patients with dextrocardia. There-fore, here we report on a case of transcatheter closure of asecundum ASD in a patient with dextrocardia and situsinversus and normally related great vessels5I,L,I 6.

CASE REPORT

M.K. is a 6-year-old male child who was diagnosed atthe age of 1 yr to have dextrocardia, situs inversus of theviscera, normally related great vessels5I,L,I 6 and moder-ate size secundum ASD. Of note, the patient’s father hassitus inversus totalis with no cardiac involvement; themother is healthy and normal; one of three brothers hassitus inversus totalis with no cardiac involvement; andthree sisters are healthy normal. At the age of 6 yr,physical examination revealed a normal first heart sound,fixed splitting of the second heart sound with grade II/VIejection systolic murmur at the right upper sternal border.An electrocardiogram revealed right axis deviation andintraventricular conduction delay. Chest X-ray revealedthe presence of dextrocardia with situs inversus (Fig. 1A),cardiothoracic ratio of 55%, and mild increase in pulmo-nary vascular markings. Two-dimensional echocardiogra-phy revealed moderate size secundum ASD measuring 10mm with left-to-right shunt and volume overloaded rightatrium and ventricle.

The patient underwent routine right and left heartcatheterization with the intent to close the defect with a

1Queen Alia Heart Institute, King Hussein Medical Center, Am-man, Jordan2Division of Cardiology, Department of Pediatrics, Floating Hospi-tal for Children, New England Medical Center, Tufts UniversitySchool of Medicine, Boston, Massachusetts

*Correspondence to: Ziyad M. Hijazi, M.D., Division of Cardiology,Department of Pediatrics, Floating Hospital for Children, New EnglandMedical Center, 750 Washington Street, Box 313, Boston, MA 02111.E-mail: ziyad.hijazi@es.nemc.org

Received 18 July 1997; Revision accepted 7 October 1997

Catheterization and Cardiovascular Diagnosis 43:291–294 (1998)

r 1998 Wiley-Liss, Inc.

Fig. 1. Chest radiograph in the frontalview pre (A) and post device closure (B).Note: The heart position on the right andthe improved vascular markings post clo-sure; the device is marked by the arrow-heads in B.

A

B

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device. Transesophageal echocardiogram (TEE) undergeneral endotracheal anesthesia confirmed the diagnosisof a 13 mm ASD in the fossa ovalis with good rimssurrounding the defect. Oxygen saturation in the superiorvena cava was 73%, right atrial saturation 77%, and thefinal left pulmonary artery saturation was 82%. The aortawas fully saturated at 96%, and right heart pressures werenormal.

Following a previously reported protocol [9], an angio-gram was performed in the left atrium in the right anterior

oblique view with cranial angulation to profile the atrialseptum (Fig. 2). The stretched diameter of the defect was14 mm. A 15 mm Amplatzer Septal Occluder (AGAMedical Corporation, Golden Valley, MN) was success-fully deployed across the defect with complete closuredocumented by angiography and TEE (Fig. 2). Thepatient recovered and the following day repeat chestX-ray revealed the device to be in stable position withnormal pulmonary vascular markings (Fig. 1B). Transtho-racic echocardiogram also revealed complete closure and

Fig. 2. A: Left atrial angiogram in theright anterior oblique view with cranialangulation preclosure demonstrating themoderate size atrial septal defect (be-tween the black and white arrows). B:Cine image of the device along the atrialseptum demonstrating the radio-opacityof the device. C: Angiogram in the pulmo-nary artery with pulmonary levophasepostdevice closure using the AmplatzerSeptal Occluder demonstrating no re-sidual shunt.

Catheter Closure of ASD in Dextrocardia 293

the size of the right ventricle to be smaller than preclo-sure.

DISCUSSION

Transcatheter closure of secundum ASD in patientswith levocardia is easily done under fluoroscopic andTEE guidance using different investigational devices [11,12]. This case illustrates the use of a new device (theAmplatzery Septal occluder) in closing a secundum ASDin a child with dextrocardia. The main advantages ofusing this device for catheter closure of ASD’s are: (1)small introducer size needed (7Fr) for device implanta-tion, (2) user friendly delivery system with the ability toretrieve or reposition the device prior to its release fromthe cable without distortion of the device, and (3)self-centering mechanism rendering complete closurerates higher than closure rates with other devices. Hence,this device should be considered for closure of unusualtypes of ASDs or ASDs in unusual anatomy.

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