Multifenestrated Atrial Septal Defects closed with Single ASO Device – Planning and Strategies

7 Years old girl presented with incidental detection of murmur. She was born full term via normal vaginal delivery with birth weight of 2.9 kg. She had uneventful neonatal and infantile period otherwise and did not require hospitalization so far for recurrent respiratory infection. On further evaluation she underwent echocardiography which revealed Two fossa ovalis defect (Measuring 10 x 6 mm and small fenestration near IVC rim) with Floppy aneurysmal Septum primum with moderate size defect (16 x 12 mm) with left to right shunt. There was an evident volume overload on right side of the heart.

On examination wise there was an ejection systolic murmur with fixed split at Pulmonary area with unremarkable other systemic examinations. Her vitals were within normal limits with saturation of 98% and Heart rate of 84/ min. Her Chest X ray was suggestive of Cardiothoracic ratio of 0.6. She was counselled for need of ASD closure and provided with the available options (Surgical closure V/S Percutaneous Device closure (Multiple Device V/S Single Device).

Transcatheter device closure of secundum atrial septal defects (ASDs) has become a safe and effective alternative for surgical closure in recent decades (1). With increasing experience in device closure s and improved imaging techniques, Varying complex defect morphologies are considered for closure in the catheterization laboratory by the interventional cardiologists. Such complex defects include large defects exceeding 35 mm, defect with deficient rims, Multiple defects, and multiple defects or Multifenestrated defects (2).

In case of multiple defects that are close to each other with intervening tissue smaller than 5–7 mm, they may be amenable for closure with a single self-centering Amplatzer septal occluder ASO (Abbott Medical, Santa Clara, CA)(3,4). If the satellite defects that are located more than 7 mm away from the main defect are insignificant measuring <3–4 mm, they may be ignored as the shunt through these small defects may be clinically irrelevant. If the satellite defects merit additional closure, then they require multiple devices in the atrial septum (5,6,7).

Increasing experience accumulated over the past 2 decades of safe use of ASO for closure of secundum ASD has encouraged operators to include patients with multiple atrial defects after careful evaluation of the number of defects, their size, the length and sturdiness of the intervening tissue between the defects and age of the patient (8,9). The most common strategy uses a single ASO through the largest and most central of the defects if the surrounding satellite defects are small and close to the main orifice. n some instances, this is facilitated by a balloon enlargement of the central orifice to accommodate a larger device (5,6,7,10). While nonself-centering cribriform occluders are applicable in relatively smaller orifices of multifenestrated defects, larger multiple defects need multiple devices.

After proper work up and documenting blood parameters to be in normal limits she was taken up for transcatheter device closure keeping in mind that she might require multiple devices to close the different defects. We took two femoral venous access to cross the two defects separately. From first access we crossed major defect located Antero- Superiorly and parked the guide wire in Left upper pulmonary vein while second defect located posterior inferiorly was crossed from the contralateral femoral vein and guidewire was parked in Right upper pulmonary vein and used a support. Our initial plan was to cover the major defect with slightly oversized device to cover the additional defect located postero-inferior defect with single device while leaving behind the tiny fenestration near the IVC rim as it is as it was not significant hemodynamically. We were ready with the multiple devices if it required after closing major defect with single large device where significant residual shunt would have remained through the posteriorly located defect. Ideally in such cases Balloon interrogation of the two different defects form each side should be dome to look for displacement of the intervening tissue and free movement where free movement of tissue with closing the smaller defect through balloon interrogation across major defect would ideally need single device whereas residual shunt thorough additional defect and inability to mover intervening tissue would require two separate defects

In our case we crossed the major defect and deployed the 22 mm Amplatzer Septal Occluder (ASO) keeping 5F MP1 in Right upper pulmonary vein. Slightly oversized device stretched out the septum primum and closed the two major defects with the single while additional tiny defect near IVC rims remained uncovered which was not significant hemodynamically. Crossing and Placing the second catheter across second defect provided us the support and would have helped in case we would have required the two separate devices as it would be difficult to cross another defect after deploying the device across major defect. Thus, we could avoid the surgical ASD closure and its associated complications and its cosmetic implications. Recent advancement in techniques could allow us to proceed ahead and complete the procedures. She was discharged in hemodynamically stable condition with the single anti-platelet medication to be continued for 6 References:

Figures:

(a)           Multiple fossa ovalis defect with intervening tissue,

(b)          Multiple fossa ovalis defect with intervening tissue located postero-inferiorly,

(c)           Floppy septum primum with large defects,

(d)          ASD device covered both (Anterior superior defect across  floppy septum primum and postero inferiorly located major defect

(e)          Post device additional tiny residual defect near IVC rim

Figures:

(f)           Crossing two different defects via tow separate femoral venous accesses and parking of MPA1 catheter in LUPV and RUPV.

(g)           Crossing major defect with 22 mm ASO

(h)          deploying the device across Antero-superiorly located major defect with the support of another catheter placed through second defects.

References:

1 ) Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K Amplatzer Investigators. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: Results of a multicenter nonrandomized trial. J Am Coll Cardiol. 2002;39:1836–44.

2) Podnar T, Martanovic P, Gavora P, Masura J. Morphological variations of secundum-type atrial septal defects: Feasibility for percutaneous closure using Amplatzer septal occluders. Catheter Cardiovasc Interv. 2001;53:386–91.

3) Zanchetta M, Rigatelli G, Pedon L, Zennaro M, Carrozza A, Onorato E. Catheter closure of perforated secundum atrial septal defect under intracardiac echocardiographic guidance using a single amplatzer device: Feasibility of a new method. J Invasive Cardiol. 2005;17:262–5.

4) Szkutnik M, Masura J, Bialkowski J, Gavora P, Banaszak P, Kusa J, et al. Transcatheter closure of double atrial septal defects with a single Amplatzer device. Catheter Cardiovasc Interv. 2004;61:237–41.

5) Cao Q, Radtke W, Berger F, Zhu W, Hijazi ZM. Transcatheter closure of multiple atrial septal defects. Initial results and value of two- and three-dimensional transoesophageal echocardiography. Eur Heart J. 2000;21:941–7.

6) Awad SM, Garay FF, Cao QL, Hijazi ZM. Multiple Amplatzer septal occluder devices for multiple atrial communications: Immediate and long-term follow-up results. Catheter Cardiovasc Interv. 2007;70:265–73.

7) Mahadevan VS, Gomperts N, Haberer K, Silversides C, Benson LN, McLaughlin PR, et al. Transcatheter closure of atrial septal defects with multiple devices in adults: Procedural and clinical outcomes. Int J Cardiol. 2009;133:359–63.

8) Yang Y, Xu Z, Jiang S, Zhao S, Zhang G, Jin J, et al. Simultaneous transcatheter closure of multiple atrial septal defects using dual Amplatzer septal occluder devices. Am J Med Sci. 2016;352:245–51.

9) Pedra CA, Fontes-Pedra SR, Esteves CA, Assef J, Fontes VF, Hijazi ZM. Multiple atrial septal defects and patent ductus arteriosus: Successful outcome using two Amplatzer septal occluders and Gianturco coils. Cathet Cardiovasc Diagn. 1998;45:257–9.

10) Awad SM, Garay FF, Cao QL, Hijazi ZM. Multiple Amplatzer septal occluder devices for multiple atrial communications: Immediate and long-term follow-up results. Catheter Cardiovasc Interv. 2007;70:265–73..