Unconventional uses of septal occluder devices: Our experience reviewed

Abstract

Device closure is now an accepted modality of treatment for cardiac septal defects such as fossa ovalis Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD) and Patent Ductus Arteriosus (PDA) and have well-accepted indication and long term results. Devices used for these defects have been specifically designed for use in closing these defects. In this manuscript, we are reporting the efficacy of closure of nonseptal defects with devices conventionally used for septal cardiac defects although they have not been prototyped for use in such conditions.

Aim

To study use of occluder devices in nonseptal defects/malformation.

Material & methods

39 patients, in the age group 2–67 yrs, were treated percutaneously with occluder devices for various conditions. These included: coronary arteriovenous (CAV) fistula (n = 6), pulmonary AV fistula (n = 4), systemic AV fistula (vascular plug; n = 1), closure of AP window (duct occluder; n = 3), closure of ascending aorta perforation (septal occluder; n = 2), ruptured sinus of Valsalva (RSOV) (duct occluder; n = 13), Fontan fenestration closure (ASD septal occluder, patent foramen ovale device, vascular plug n = 3,1 each), splenic artery (duct occluder; n = 1), Balock Taussig shunt (duct occlude; n = 1)and closure of mitral paravalvular leak (n = 3; duct occlude devices = 2, VSD device: n = 1) and aortic paravalvular leak n = 2 (duct occluder; n = 2 additional vascular plug = 2).

Results

Procedural success: Successful closure as signified by no residual shunt was achieved in all coronary AV fistula (immediately n = 2, at 3 months in all), ruptured sinus of Valsalva (immediate in all), fenestrated Fontan (immediately in all), and ascending aorta perforations (immediate), mitral paravalvular leak (immediate in none, and late in 2/3). The aortic paravalvular leak closed at 3 months follow-up in one and small residual persisted after 1 month in another. Complications: Local site Hematoma was observed in 4 patients. 2 of them required post procedure transfusion for the same. Hematuria was observed in 2 of the 4 patients of mitral paravalvular leak and 2 patients of RSOV device closure. Hematuria subsided with conservative management before discharge from hospital in all the 4 cases. One patient with residual mitral regurgitation required surgical management for continuing hematuria, anemia and hyperbilirubenemia. There was one mortality observed on table during the attempted closure of a very large RSOV who presented to us in severe congestive heart failure and shock. On follow up ranging from 2 months to 6 years, all the patients are asymptomatic. There was no late complication related to device in any patient.

Conclusion

It is feasible in selected nonseptal defects, which traditionally have been subjected to surgical interventions, to treat successfully, non surgically with the use of non prototype occluder devices without significant complications. Conventionally these devices have not been recommended for closure of nonseptal defects but show good early outcome. Adequate sample size with good follow up data is necessary before concluding that it can be safe alternative to surgery on long term.

1. Introduction

Transcatheter closure of septal defects (fossa ovalis ASD, VSD, PDA) is a recognized modality for treatment of cardiac defects. With increasing experience, unconventional use of these devices in defects other than septal defects is now increasing. Here we are reporting our experience of these devices in cardiac and extracardiac defects/malformation and review the available literature for their usage in the current era.

2. Methods

2.1. Patient population

From June 2001 to April 2013 39 patients were included.

2.2. Exclusions

Unconventional methods of device deployment at conventional sites like hybrid approaches for VSD devices have been excluded from the manuscript. Excluded from the study were venous channels closed with vascular plugs, major aortopulmonary collaterals (MAPCA), PDA who underwent coil or vascular plug closures have also been excluded. An informed written consent was taken from all the patients or their parents.

2.3. The device

The devices used included, Amplatzer Septal Occluder and delivery system (AGA Medical, Golden Valley, Minnesota), Lifetech (Heart R) device systems and delivery system (ASD, VSD, PDA). Amplatzer vascular plug (generation 1,2 and 4). All the devices have been described in detail in other reports (2,6). There are well documented standardized protocol for the deployment of the respective devices with multiple series describing the efficacy and safety of the device at conventional sites.

2.4. Patient's selection for transcatheter closure

Selection of patients suitable for device closure was based on measurement of maximal defect diameter done using various investigational modalities like echocardiography, Computerized Tomography (CT) angiography, Magnetic resonance imaging (MRI) and angiography. It was also based on morphological characteristics like location and size of the defect, relationship with the adjoining structures and rims of the defect.

2.5. Protocol for all patients

Detailed clinical and physical examinations, a standard 12-lead electrocardiogram (ECG), chest radiograph, transthoracic echocardiography (TTE) were performed in all patients. In patients with poor echo windows (adolescents and adults) or whenever it was needed to define the morphological characteristics of the defect, transesophageal echocardiography (TEE), CT angiography or MRI and angiography was also done if necessary for anatomical definition.

2.6. Implantation procedure

The procedure was done in catheterization laboratory under general anesthesia/local anesthesia in most of the patients with or without echocardiography and fluoroscopy guidance (Table 1). After obtaining the venous and arterial access, 100 units/kg of unfractionated Heparin is given to all the patients. Right heart hemodynamic data is obtained in all the patients prior to the procedure.

Table 1.

Showing the summarization of cases with respect to the type of cases, their imaging modalities and the sedation used for the same (n = 39).

	Site	Device	Sedation	Imaging
1	Coronary AV fistula	6	LA	TTE, Floro
2	Systemic & pulmonary	5	LA	TTE (1), Floro
3	RSOV	13	GA and LA	TEE, Floro
4	Fenestration Fontan	3	GA	TTE, Floro
5	Paravalvular leak (mitral + aortic)	3 + 2	GA	TEE, Floro
6	AP Window	3	GA	TTE, Floro
7	Ascending aortic perf	2	GA	Floro
8	Splenic artery occlusion	1	GA	Floro
9	BT shunt	1	GA	Floro

(TTE: transthoracic echocardiography, TEE: Transesophageal echocardiography, Floro: Floroscopy and angiography, PDA: Patent ductus arteriosus).

2.7. Follow up protocol

Detailed echocardiography examination was performed immediately after device closure in all patients to check for device position, stability, encroachment of device on adjoining structures. Flow through device fabrics was common at the time of device deployment and was not taken as residual shunt while any additional jet of shunt was taken as residual shunt. At 24 h Chest X-ray (Deep penetrating, frontal and lateral views), and transthoracic echocardiography were performed. On TTE, device position, stability, any evidence of encroachment over adjoining structures was checked. Bacterial endocarditis prophylaxis was advised for 6 months post procedure. Thereafter, follow up was done at 1 month, 3 months, 1 year and then annually with clinical evaluation, Electrocardiogram (ECG), TTE and TEE if any doubt on TTE or symptomatic evaluation.

3. Results

A total of 39 patients were included. The details of the individual sites have been describes as below:

3.1. Fistulae: coronary, pulmonary and systemic

3.1.1. Coronary AV fistulae

7patients underwent coronary AV fistulae using interventional techniques (in cath lab). 3 patients underwent the closure with PDA ductal occluder device, 4 patients underwent coil closure of the coronary AV fistula. 2 of the latter patients had vascular plug occlusion of the defect followed by the additional coil closure of the same in view of the residual flow. Demographic profile is summarized in Table 2. Mean follow up is 3.6 years (ranging from 1 to 10 years). One 22 year old young girl had the fistulae closure in multiple sittings as there were additional openings of the coronary fistulae, which became apparent only after the closure of the fistulaous tracts. Case highlighted a proper evaluation preferably with a CT reconstruction to define the course of the fistulae. All patients underwent complete closure of the defect with no residual shunt. There was no associated ECG changes or vascular complications.

Table 2.

Coronary AV fistulae: table showing our experience with Coronary AV fistulae devices.

	Age (yrs)	Sex	Device size	Device type	Follow up
1	53	F	.018 × 3 × 3	Coil	1 yrs
2	57	F	10/12 mm	PDA device HRT R	5 yrs
3	2	M	0.38, 5 × 5	GT COOK COIL	10 yrs
4	1 yrs 7 months	M	8 mm vascular plug (additional 3 coils)	Vascular plug	1 yrs
5	45	M	10/12	PDA	2 yrs
6	45	F	0.38 × 5 × 5	Coil	3 yr
7	20	M	10/12	PDA	1 yr
8	22	F		PDA devices; 2, Vascular plug 1	1 yr

(M: male, F: female, PDA: patent ductus arteriosus, HRT R: Lifetech heart – R device).

3.1.2. Systemic and pulmonary AV fistulae

A total of 5 patients were included in the group. Of these 2 underwent systemic AV fistula closure while 3 patients underwent pulmonary AV fistulae closure. Complete closure was achieved in all the patients (Table 3). A 4 month old boy (case1, Table 3) had abnormal fistulous connection between the proximal right subclavian artery to upper end of SVC, a rare communication. Amplatzer Vascular plug (6 mm) occlusion which was deployed from right subclavian artery under fluoroscopic guidance with complete occlusion of the abnormal fistulous tract was achieved. He also required associated SVC stenting because of the constriction in SVC at the site of fistulous insertion.¹Of all the patients of pulmonary AV fistulae (n = 8)who underwent transcatheter closure, four had closure of the fistulae using the ductal occluder (detailed in Table 3), using the ductal occluder 18 × 16 mm (Heart R, Lifetech) in all the cases. It was done successfully with no residual shunt. Patient had mean systemic saturation of 75% which increased to mean systemic saturations of 97% in the catheterization laboratory immediately after procedure. Patients are doing well with no complications and saturations are maintained at follow up An interesting case of a 20 year old girl (Case 2) who was deeply cyanosed was evaluated to have multiple pulmonary av fistulae which were closed by 2 ductal occluders and additional third fistulae was closed by a vascular plug. It is thus important to evaluate the presence of additional AV fistulae in any case of pulmonary AV fistulae.

Table 3.

Pulmonary And Systemic AV Fistula: table showing our experience with devices.

	Age	Sex	Primary diagnosis	Device size	Device type
1	4 months	Male	av fistula, rsca to SVC	6 mm	Vascular plug
2	20 yrs	Male	PAVF	18 × 16 mm, 8 × 10, Plug size 10 mm	PDA device, PDA device Hert R, Vascular Plug: Amplatzer
3	28 yrs	Female	SAVF: left shoulder joint (brachial artery, axillary artery.)	0.35 × 4 × 3	Cook coil, PDA
4	20	F	PAVF	0.38 × 5 × 5, 18 × 16	Cook Coil, PDA
5	21	M	PAVF	18 × 16	Cook coil, PDA

(RSCA: right subclavian artery, SVC: superior vena cava, SAVF: systemic arteriovenous fistulae, AV: arteriovenous, PAVF: pulmonary AV fistulae).

3.2. RSOV

13 patients (11 males and 2 female) with RSOV underwent transcatheter device closure during the study period. The mean age was 41 years (21–59 years). All the patients had shortness of breath (NYHA class II – eight patients, class III – two patients) at the time of presentation. Two patients (25.0%) presented with acute exacerbation of symptoms and cardiac failure. The sites of origin and exit of RSOV are summarized in Table 4. Noncoronary cusps were sites of origin in 10/13 patients while it was from right coronary sinus in 3 patients. The site of exit was into the right atrium in 10/13 patients and right ventricular outflow tract (3/13). The PDA devices used for RSOV closure were 16/14 (9/13), 12/14 (1/13), 10/12 (1/13) and 8/10 (1/13). The mean procedural time was 45.3 ± 5.4 min, while the fluoroscopic time was 26.5 ± 6.9 min. All patients undergoing device closure were extubated electively after 4–6 h following the procedure. Complete closure was achieved in all the patients.

Table 4.

RSOV: table showing details of patient who underwent successful RSOV device closure.

	Age (yr)	Sex	RSOV to	Sinus	Device size	Device type	Follow up
1	40	M	RA	NCC	14/16 mm	PDA (Ductal occluder)	2
2	21	M	RA	RCC	14/16 mm	PDA	3
3	23	M	RA	NCC	14/12 mm	PDA	3
4	38	M	RA	NCC	14/16 mm	PDA	5
5	57	M	RVOT	?NCC	14/16 mm	PDA	1
6	59	F	ra	NCC	14/16 mm	PDA	1
7	52	M	ra	NCC	14/16 mm	PDA	7
8	46	M	ra	NCC	14/16	PDA	1
9	40	M	RA	NCC	14/16	PDA	1
10	35	F	RA	NCC	14/16	PDA	6 months
11	25	M	RVOT	RCC	10/12	PDA	1 month
12	24	M	RVOT	RCC	8/10	PDA	1 month

(RSOV: ruptured sinus of valsalve, NCC: non coronary cusp, RCC: right coronary cusp, PDA: patent ductus arteriosus, ra: right atrium, RVOT: right ventricular outflow tract).

One patient with biventricular dysfunction (case1 not included in Table 4), acute renal failure, and thrombocytopenia had cardiac arrest in an attempted crossing of the RSOV. Follow up is available for all patients with mean follow up of 2.5 years (1 months–7 years). At the time of the most recent follow-up, all the device-closure patients were in NYHA symptom class I. Clinical evaluation revealed stable hemodynamics with absence of murmur in all. Their ECGs did not reveal any ischemia, conduction abnormalities, or rhythm disturbances. Two-dimensional echocardiography revealed the device to be in appropriate position with no evidence of residual shunt, in all the patients. There was no neo-AR.

Three of our patients had an RSOV to right ventricular outflow tract (RVOT). There was kinking of the sheath at the point of crossing of the sheath from right ventricle (RV) to aorta. We overcome the difficulty by using “Kissing technique” (where in a multipurpose A2 (MPA2) catheter was advanced from the arterial end into the sheath). As the device was advanced the MPA2 catheter was withdrawn simultaneously in order to overcome the kink (case 5). Another modification we did for the case was passing a buddy wire (0.018 coronary wire) across which was passed prior to the deployment of the device, across the sheath. This not only gave support while deploying the device, it was also a useful access to the site even after the device sheath assembly has been withdrawn (Buddy wire technique) (case 5) (case 1).

3.3. FONTAN fenestration

Fontan fenestration was closed successfully in three patients. The devices used were Patent foramen ovale (PFO) device (18 mm), ASD septal occluder (Lifetech) device (6 mm) and Vascular plug (4 mm) one each. The device was successfully delivered in all 3 patients attempted with no major complication. Table 5 summarizes patient procedure detailed and hemodynamic data. Arterial oxygen saturation and the ratio of pulmonary to systemic blood flow increased significantly in all patients.

Table 5.

Fontan fenestration closure (Demographic data).

	Age (yrs)	Sex	Wt (kg)	Primary diagnosis	Previous surgery	Device size	Device used	Pressures rise	Spo2 rise
1	5	M	17.5	DILV, VSD, PS	Primary Fontan ? 4 yrs	6 mm	ASD device	no	5%
2	25	F	75	Univentricular AV connection, PS		25 mm	PFO device	no	5%
3	16	F	70	Single outflow pulmonary atresia hypoplastic, RV	BT shunt (6 months) Fontan (4 yrs)	4 mm	Vascular plug 4	no	4%

(DILV: double inlet left ventricle, VSD: ventricular septal defect, PS: pulmonary stenosis, ASD: atrial septal defect, BT: Ballock Taussing shunt, RV: right ventricle).

3.4. Paravavular leak closure

Device closure of paravalvular leak was attempted in 5 patients (Table 6). 3 patients underwent closure of mitral paravalvular leak, Two patients had aortic paravalvular leak. In case of mitral paravalvular leak, 2 cases were crossed from the left atrium after trans-septal puncture, the third case it was crossed from the left ventricle and snared from left atrium after trans-septal puncture. PDA device was used in 2 and VSD septal occluder in 1 patient. The device was deployed successfully in all the 3 patients. Immediately post device deployment the patients were extubated within 8 h. 2/3 patients of mitral paravalvular leak had hematuria following the procedure which was conservatively managed. It recovered in one patient while in another it required re-operative intervention, 2 months later for persistent hematuria and residual paravalvular leak. In two cases of the aortic paravalvular leak it was crossed retrogradely. In case 5 in a 56 year old man it required the usage of 2 additional vascular plug to occlude the leak. Mild paravalvular leak persisted in this patient after closure, even in follow up at 2 months.

Table 6.

Paravalvular Device closure: Table showing our experience with paravalvular devices.

	Age (yrs)	Sex	Valve	Device type	Device size	Follow up
1	65	M	Mitral	VSD muscular device	10 mm	4 yrs
2	46	M	Mitral	PDA device Amplatzer	8/6 mm	9 yrs
3	55	F	Mitral	PDA device Amplatzer	8/6 mm	6 months
4	45	M	Aortic	PDA device Amplatzer	8/6 mm	6 years
5	56	M	Aortic (status post DVR)	PDA Heart R 8/10, 2 vascular plugs	8/10	1 month

3.5. AP window closure (Table 7)

Table 7.

AP window device closure.

	Age (yrs)	Sex	Device type	Device size	Follow up
1	3 yrs	M	PDA device Lifetech	4/6	4 yrs
2	6 yrs	M	PDA device Amplatzer	8/6	9 yrs
3	3 yrs	F	PDA device Amplatzer	8/6	8 months
4	4 yrs	M	Abondend	No

3 patients underwent successful transcatheter closure of AP window, Proximal type. It was attempted in 4 patients, while in one 3 year old patient the procedure needed to be abandoned in view of in ability of the PDA device deployment; patient successfully underwent direct ligation of the restricted AP window. PDA ductal occluder was used in all the cases.6/8 mm in 2 cases and 4/6 mm in 1patient. Complete closure was achieved in the catheterization lab in all the 3 successful attempted cases.

3.6. Ascending aortic perforation closure

A 70 year old male who had undergone surgery (CABG) was noticed to tachycardia, but stable hemodynamics. He was observed to have mediastinal widening on chest x-ray. CT done showed perforation of the ascending aorta. A retrograde crossing of the perforation was done using right Judkins catheter and termo wire. This was exchanged with an appropriate sized sheath and thereafter successful device closure using 8 mm ASD device, retrograde through aorta. Patient was discharged successfully in an asymptomatic state but no follow up data is available for the same. Another 50 year old post Bentall patient presented with pseudoaneurysm of the ascending aorta which was closed successfully using ASD device.

3.7. BT shunt occlusion with the device

A newborn 12 days old male child case of valvular pulmonary atresia with intact ventricular septum had surgical valvotomy and right BT shunt surgery successfully. Child was extubated and discharged after 12th postoperative day. He was regularly followed up and electively admitted at 18 months of age where in he underwent Balloon valvuloplasty, closure of the BT shunt using 4/6 ductal occluder device. Child was on regular follow up showing adequate growth parameters.

3.8. Splenic artery occlusion with a device

A 12 year old male case of hypersplenism with depressed cell lines (pancytopenia) as a result of splenic sequestration. The splenic artery was selectively profiled using an RCA catheter. A supporting extra stiff wire was placed across the catheter into the distal spleen. This was exchanged with the Mullins sheath. A 10/12 ductal occluder device was deployed distally in the splenic artery. The patient underwent successful occlusion of the splenic artery and showed significant improvement in the cell lines. Subsequently splenectomy was done successfully for the patient.

4. Follow-up

Follow-up data are summarized in individual tables. Mean duration of follow up was 2.4 years (1 week–7 years). It is available for 37/39 patients who were doing well clinically. Those who are lost to follow up include: 1 patient who underwent ascending aortic perforation closure, and 1 patient who underwent splenic sequestration. There were no shunts detectable through or around the devices by transthoracic echocardiography. There was also no evidence of thrombosis, baffle (Fontan) obstruction, or new atrioventricular valve stenosis or insufficiency.

1 patient with mitral paravalvular device closure with residual flow was readmitted 2 months following the procedure with anemia. She improved symptomatically with blood transfusion. There was no evidence of hemolysis in peripheral smear, with no evidence of hematuria. Transthoracic echocardiography was done in all patients on follow up. Device was in situ with no residual flow in all except one with small residual flow. Ventricular function was normal on follow up. There was no evidence of new appearance of valve leak (mitral, tricuspid or aortic), cardiac perforation, erosion of aorta by device, device embolization or device distortion/fracture. There was one procedure-related mortality in our study group as elaborated under RSOV and no other major complications were noted.

5. Discussion

Devices utilized for ASD, VSD or PDA closure have now generated a long term data showing their efficacy as a recognized tool for closure of these defects. Encouraged by their conventional usage these devices have now been used for various other cardiac and extracardiac defects (unconventional usage) especially in areas for which surgery was traditionally considered as the only therapeutic option. Unconventional uses of devices may be divided into two groups:1) Use of the devices for the lesions for which there are no customized available devices (our group1, Table 1), 2) Use of the devices for lesions which have customized devices which necessitated the use of alternative strategy or devices. This review attempts to review the available literature for the same and to supplement the data with our own experience for group1. Use of the devices for the lesions for which there are no customized available devices includes: A) Coronary arteriovenous fistula, B) Pulmonary arteriovenous fistula, C) Systemic arteriovenous fistula, D) Venovenous collateral, E) Hepatic venovenous collateral, F) Ruptured sinus of Valsalva, G) Aortic aneurysm, H) Pulmonary artery aneurysm, I) SCA aneurysm closure, J) Occlusion of the artery, J1) Subclavian artery occlusion, J2) Persistent LSVC occlusion, J3) MAPCA occlusion (with Device), J4) Internal Iliac artery occlusion, K) AP window, M) LV aneurysm, L) Paravalvular leak, M) Fontan fenestration, N) Possible closure of aorta to LV tunnel, O) Bronchopleural fistula closure.

5.1. Congenital coronary arteriovenous fistula

Non surgical closure of these fistulous communications is now the accepted mode of therapy with numerous case reports.^1–5 The aim of the procedure should be to achieve complete occlusion at as distal a location as possible, avoiding any possibility of occluding branches to normal myocardium, the largest device that fits in the smallest guiding catheter or sheath is chosen. All varieties of coils, devices have been used in the studies available. Although the uses of coil outnumber the usage of devices in the literature. In our own cases we have used PDA occluder devices, Gianturco coils and Vascular plugs to close these fistulae. In two of our patients we used both the coils and PDA device for the closure of the fistulae. Our technique of the procedure, success rate and long term follow up are similar to that available in the literature.

5.2. Pulmonary arteriovenous fistulae, or malformations (PAVMs)

A variety of percutaneous embolization techniques have been successfully employed to address these lesions in the literatue.^6,7 The aim of the procedure is to occlude the artery immediately before it enters the aneurysmal part. Coils, coil bags, detachable balloons, double umbrellas and Amplatzer devices have been utilized with different degrees of success to occlude such fistulae over the last two decades and hence is a well described enteity^6,7 our experience was similar to that observed in the literature. We used PDA occluder devices in 4 of the 8 patients who underwent closure of PAVM at our institute with similar results for systemic AV fistulae.^8–10Amplatzer device has been used interestingly as a buttress to avoid embolization of coils. We used the same technique in closing a large coronary artery fistula where device was deployed followed by placement of coils.

5.3. Rupture of aneurysm of the sinus of Valsalva (RSOV)

RSOV closure is a controversial area although initial reports of small series indicate good immediate results.^11–21The objection to the method of treatment is mainly related to the underlying pathology. RSOV represents the weakness in the wall of the sinuses leading to aneurysmal dilatation and subsequent rupture into any of the cardiac chambers viz. right atrium, right ventricle, inter-ventricular septum or rarely left ventricle. The surgical technique consists of closing the defect and reinforcing the weak sinus wall. By Percutaneous technique although closure of the fistulous tract is achieved, no attempts are made to correct the underlying pathology. There is an anecdotal case of re-appearance of the flow after initial successful closure of the same, emphasizing the theory that long term effects need to be seen of the Percutaneous closure of defects and that a close follow up of the patients who have undergone the procedure is warranted. Very large defects should not be considered for percutaneous intervention as was exemplified in one of our cases who had a fatal outcome.

5.4. Occlusion of the vessel

The occlusion of vessels such as subclavian artery occlusion, splenic artery occlusion and closure of aortopulmonary collaterals are isolated cases and hence do not merit discussions. Use of percutaneous closure avoided possible surgical intervention in these cases.

5.5. AP window (APW)

The device selection plays a vital role in the selection of the patient for suitability of a ductal occluder. The optimum device would depend upon the size of the defect as well as relationship to surrounding structures especially coronary arteries. The ductal occluder device has several desirable characteristic for the APW such as ability to recapture and reposition the device, high complete closure rates and relatively smaller introducer sheaths.^22–26 For a large hypertensive AP window muscular VSD or ADO2 may be useful. We had used PDA devices for the same in all our patients.

5.6. Paraprosthetic leak

Symptomatic paraprosthetic leak can be treated with percutaneous transcatheter closure as an alternative to cardiac surgery. Dedicated devices are not yet available, but once the morphology of the leakage has been defined, it is possible to choose among the existing devices that are currently used for other percutaneous procedures.^27–31 The availability of 3D echocardiography has been observed by us to be particularly useful in defining the morphology of the defect and hence in selection of the device for the occlusion. In the aortic position: The sizing of the defect have been by TEE although few have used balloon sizing also. The commonest used device is PDA occluder. Its distal disk remains in the left ventricle while the proximal part is positioned inside the defect. Muscular VSD device with its two disc have been used for larger defects as we used it for one of our cases. In the mitral position: for small leaks Gianturco coil or Jackson coils (Cook, Inc) have been used.²⁹ For larger defects VSD or ASD device may be used. They remain in place because the two disks assume an oval rather than a circular position around the defect.³¹ In one case we had used the vascular plug II and IV with stable position.

5.7. Fenestrated fontan

Circuit may prevent excessive increase in venous pressure in the early post-operative phase, especially in high risk patients. Many of these close spontaneously, however some remain open causing persistent arterial desaturation and are potential cause of paradoxical embolism. Subsequent closure of the fenestration using different transcatheter devices such as ASD device and PDA device has been described. Percutaneous device were easy to use, is easily retrieved prior to release, and can be implanted into Fontan fenestrations with acceptable procedure length and radiation time. The profile of the device does not appear to alter blood flow through the baffle significantly. These results were well shown in a number of case reports.^32–35

Conflicts of interest

All authors have none to declare.