|Year : 2022 | Volume
| Issue : 2 | Page : 71-75
Gerbode defect – Percutaneous closure of three cases and a brief review of literature
Mahua Roy1, Debasree Gangopadhyay1, Siddhartha Saha1, Sushil Sukla2, Pooja Sinha1
1 Department of Pediatric Cardiology, NH RTIICS, Kolkata, West Bengal, India
2 Department of Pediatric Cardiology, Max Super Speciality Hospital, New Delhi, India
|Date of Submission||12-Feb-2021|
|Date of Acceptance||16-Feb-2021|
|Date of Web Publication||21-May-2022|
Dr. Mahua Roy
931 Jawpur Road, Kolkata - 700 074, West Bengal
Source of Support: None, Conflict of Interest: None
Gerbode defect is very rare cardiac abnormality accounting for <1% of cardiac defects. Historically treated by open heart surgery, many authors have now popularized the concept of percutaneous closure of such defects by various devices. We report three such cases and provide a brief review of the literature.
Keywords: Gerbode defect, percutenous closure, open heart surgery
|How to cite this article:|
Roy M, Gangopadhyay D, Saha S, Sukla S, Sinha P. Gerbode defect – Percutaneous closure of three cases and a brief review of literature. J Indian coll cardiol 2022;12:71-5
|How to cite this URL:|
Roy M, Gangopadhyay D, Saha S, Sukla S, Sinha P. Gerbode defect – Percutaneous closure of three cases and a brief review of literature. J Indian coll cardiol [serial online] 2022 [cited 2022 Sep 24];12:71-5. Available from: https://www.joicc.org/text.asp?2022/12/2/71/345628
| Introduction|| |
Gerbode defect is a rare anomaly where there is a communication between the left ventricle (LV) and the right atrium (RA) through a defect in the ventricular septum. This anomaly accounts for 0.08% of intracardiac shunts and <1% of all cardiac defects. Although first described by Thurman in 1838, first surgical correction was described by Gerbode et al. in a series of five patients in 1958. This defect can be congenital or acquired. Congenital variety was divided into two types: Direct and Indirect by Riemenschneider and Moss. Direct defect is located in the membranous septum between LV to RA and the indirect defect presents with a perimembranous ventricular septal defect (VSD) associated tricuspid regurgitation. Further re-classification was done by Sakakibara and Konno into supravalvular, subvalvular, and intermediate type, which is a mixture of the two, based on the location of the defect in respect to the septal leaflet of the tricuspid valve.
Acquired LV to RA communication can be iatrogenic acquired gerbode defect (AGD) or non-itarogenic Acquired noniatrogenic gerbode defect (ANIGD). AGD occurs after cardiac surgeries close to the membranous septum such as, mitral/aortic valve replacement, tricuspid annuloplasty, repair of complete atrio-VSD, percutaneous interventions such as AV node ablation, endomyocardial biopsy etc. The conditions giving rise to ANIGD include infective endocarditis, myocardial infarction in the right coronary artery distribution, and penetrating chest trauma.
The safety of surgical correction is well established in case of congenital defects while transcatheter closure is an acceptable alternative for acquired defects. There are only few reports of transcatheter closure of congenital direct Gerbode defect available in the English literature. We describe transcatheter device closure of two intermediate type defects and one direct defect and discuss choice of device in both situations. To the best of our knowledge, this is the first report of transcatheter device closure of indirect Gerbode defect.
| Case Reports|| |
An 8-year-old girl evaluated for recurrent chest infection, poor growth, and systolic heart murmur. Clinically, there was cardiomegaly and loud pan systolic murmur and echocardiography revealed intermediate type Gerbode defect with two jets of left to right shunts above and below the septal leaflet of TV [Figure 1]. After taking proper consent, the patient was prepared for percutaneous intervention under conscious sedation. The defect was profiled by left ventricular angiogram at left anterior oblique view with cranial tilt (60°–20° and 30°–30°) [Figure 2]. A 0.032” X 260 cm angulated Glide wire (Terumo Medical Corporation, Somerset, New Jersey) and 5F Judkins right (JR) catheter (Cordis de Mexico S. A de C. V) were used to cross the defect from the aortic side. An arterio-venous loop was made by snaring out the wire from the femoral venous end. A 12/10 mm Amplatzer Duct Occluder (AGA Medical Corporation, Golden Valley, MN, USA) was loaded on its delivery cable and was deployed antegradely by a 7F Amplatzer PDA delivery sheath (AGA Medical Corporation, Golden Valley, MN, USA) in the same technique as that for perimembranus VSD. A meticulous assessment of the device was made by echocardiogram and LV angiogram after deployment. The device was placed in such a way that both the direct and indirect shunts were closed by its retention skirts [Figure 3] and [Figure 4]. Some residual left to right blood flow was observed through the device which was expected to close by the fabric of the device after release. Continuous electrocardiogram monitoring was done throughout the procedure, and the patient remained in sinus rhythm. Total fluoroscopy time was 14 min, and procedure time was 50 min. To our dismay, next day, she developed hemoglobinuria due to the residual intra-device leak through the device. This was managed expectantly with blood transfusion and intravenous hydration for the next 3 days. Gradually over next 4 days, her urine color normalized. She was started on oral Aspirin at 5 mg/kg/day dose and was discharged home on the 6th postprocedure day. On all subsequent follow-ups up to next 1 year, the device was noted in the good position without any residual leak or aortic or tricuspid valve complications.
|Figure 1: Color Doppler flow of a 4-chamber view showing systolic flow in to right atrium from the left ventricle (a) directly from the left ventricle to right atrium above the tricuspid valve (b) through the perimembranous ventricular septal defect through right ventricle below the tricuspid valve. A diagnosis of Type III Gerbode Defect (combination of direct and indirect) was made|
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|Figure 2: Left ventricle angiogram confirmed the Gerbode defect of intermediate type|
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|Figure 3: Defect was closed with 12/10 mm Amplazer Duct Occlude-I, angiography showing large intra-device shunt|
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|Figure 4: Cartoon showing how single device completely occluding intermediate Garbode defect|
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An 11-month-old infant was referred for frequent chest infections and poor weight gain. Clinical examination and echocardiogram revealed an intermediate Gerbode defect with shunt both above and below the attachment of septal leaflet of the tricuspid valve measuring 8 mm in its entire length with significant volume overload of right and left atrium and LV.
After obtaining consent, we proceeded for percutaneous closure under conscious sedation. The defect was delineated by LV angiogram. It was crossed from the right ventricle side by 0.035” X 260 cm angulated glide wire and 5F JR catheter. The wire passed from inferior vena cava to RA to LV and up through the ascending aorta and parked in the left subclavian artery. A 7F Mullin's long delivery sheath was taken over the wire and parked into the LV facing its apex [Figure 5]. A Multifunctional occluder size 10/8 mm (Lifetech scientific corporation, Shenzhen, PRC) was loaded on the delivery cable and placed across the defect in such a way that its conical portion remained across the defect while the two discs on either side held the device securely in place [Figure 6]. Device was released following assessment by trans-thoracic echo. The patient remained in sinus rhythm during and after the procedure. Total fluoroscopy time was 12 min, and procedure time was 40 min. He was discharged home on Aspirin on the 3rd post procedure day and has remained well on the last follow-up for 6 months from the procedure.
|Figure 5: Intra-procedure echocardiography showing delivery sheath from right atrium to left ventricle|
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|Figure 6: Post procedure echocardiography showing multifunctional device one disc in left ventricle side another one in right atrium side|
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A 5-year-old boy weighing 13 kg was referred for cardiac murmur. A pan-systolic murmur was appreciated and echo revealed a gap in the atrioventricular septum above the septal leaflet of the TV allowing shunting from LV to RA. A direct Gerbode defect was diagnosed which measured 4 mm [Figure 7]. After proper consent, he was taken to cath laboratory for device closure of the defect under conscious sedation. Both femoral artery and venous accesses were obtained from right groin. After LV angiogram, the defect was crossed with 5FJR and 0.032” X 260 cm angled tip glide wire from the aortic side. The wire was kept within the RA (Ao-LV-RA) and a 5F guiding JR was exchanged over it. A 5/4 ADOII (AGA Medical Corporation, Golden Valley, MN, USA) device was deployed across the defect such a way that one disc remained in LV side and other one on RA side and totally occluded the defect [Figure 8]. The device was steady upon release with a minimal residual shunt. The patient was in sinus rhythm throughout the procedure. Total fluoroscopy time was 7 min, and procedure time was 30 min. He was discharged home on the 3rd day after the procedure.
|Figure 7: Echocardiography two dimensional and colour Doppler showing moderate sized direct Garbode defect above the tricuspid valve with left to right shunt|
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|Figure 8: Echocardiography showing Amplazer duct occlude II device in situ|
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| Discussion|| |
Gerbode defect is a very rare congenital abnormality which accounts for <1% of all cardiac defects. Surgical closure is the conventional and well-established method of treatment although with significant risk of complete heart block (CHB). Reports are available for transcatheter closure of acquired type of defects.,,,,,, Only very few reported series have published experience of transcatheter closure in the congenital form.,, It has been reported that interventional closure is associated with risk of residual shunt while CHB risk is higher in the surgical group.
Various devices which have been used are duct occluders (ADOI and II), muscular VSD occluder, Nit–occlud coil, vascular plug, Ampatzer septal ocluder, etc.,,,,,,, Vijaylxmi et al. used only ADOII in 12 patients with this defect. There were one transient CHB and two patients with junctional rhythm, all responded to conservative therapy with temporary pacing and oral, intravenous steroid. Fortunately for us, we experienced no rhythm problems in our series.
Trans-thoracic echocardiography remains the key diagnostic tool in children or adolescents where obtaining a clear image is much easier compared to adult patients with acquired form of the defect where transesophageal echo is necessary for the diagnosis and treatment. Indirect or intermediate varieties may be misdiagnosed as VSD with pulmonary arterial hypertension unless the defect is visualized by three dimensional imaging.
Previous reports have documented device closure of both direct and indirect defect. Ours is the first report of device closure of the intermediate variety by the transcatheter technique. We visualized the placement of the device in a way so that both the shunts get closed by the retention skirt of the single device.
For the consideration of transcatheter closure of Gerbode defect as like any VSD up to certain size should be considered as suitable candidate and for intermediated Gerbode defects both defects must be close to each other (defect in membranous septum and atrioventricular septum) for closing by single device. Regarding the selection of device, most suitable device would be low profile, single disc, available in wide range of sizes [Table 1]. Single disc device has got advantage of no disc on low pressure RA side, whereas low profile device has got advantage of less chance of compression of nearby structure which could culminate conduction abnormality.
|Table 1: Comparison of different types of devices for closure of Gerbode defect|
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| Conclusion|| |
Transcatheter device closure of congenital Gerbode defect is feasible. It is well-established for direct defects which are relatively small in size. Here, we report the closure of even relatively larger intermediate type defects percutaneously by a single large device with high success rate and very few complications.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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