Introduction
Closure of Atrial Septal Defect (ASD) in patient with severe pulmonary arterial hypertension (PAH) is discouraged. Recent advences in PAH medical treatment  and percutaneous procedure in ASD closure have given the possibility to close defect in patients with previously considered inoperable. We report our first experiences of an ASD transcatheter closure in a patient with severe PAH using a fenestrated ASD device manufactured by Lifetech . To the best of our knowledge, this is the first case of successful transcatheter closure of ASD using a fenestrated device in the setting of severe pulmonary hypertension in Indonesia.
 

History
A 27-year-old female presented with worsening dyspnea and activity intolerance since 4 months ago. She was diagnosed with ASD secundum with pulmonary hypertension, and already taken sildenafil, a phosphodiesterase-5 inhibitor therapy for 1 year . The cardiac catheterization followed by transcatheter closure of ASD was scheduled.

 

Findings
Cardiac auscultation revealed that SI normal, S2 wide fixed split with accentuated P2. We performed the echocardiography for further evaluation and the result was secundum atrial septal defect with pulmonary hypertension, mitral regurgitation mild caused by AML prolapse, mild tricuspid regurgitation, and mild pulmonary regurgitation (Figure 1, Video 1).

Figure 1. Diameter of ASD and TVG from TTE

Procedure
A diagnostic catheterization procedure and oxygen vasoreactivity test was performed under general anesthesia with a transesophageal echocardiography guidance. The hemodynamics data revealed that systolic pressure of pulmonary arteries was high (more than 2/3 of systemic pressure) with Qp:Qs 0.8, PARi 7.9 WU/m², and PVR/SVR 0.8. And after oxygen vaso-reactivity test showed Qp:Qs 1.6, PARi 3.6 WU/m², and PVR/SVR 0.4. (Figure 2)

 

Figure 2. The hemodynamics data pre and post oxygen vaso-reactivity test

 

Due to the value of PVR/SVR > 0.33, we decided to perform the transcatheter closure of ASD with fenestrated device. The transesophageal echocardiography was done intra-procedure and we obtained the diameter of ASD was 24-28 mm with favourable rims for device closure. We planned to close the ASD with fenestrated device Cera (Lifetech) No. 36 mm. (Figure 3)

 

Figure 3. An ASD fenestrated occlude device Cera (Lifetech)

 

We inserted a 5F MP catheter through FEVL into the LPA to monitor the PA pressure continuously.  While the 6F MP catheter from RA entered LA through ASD and was positioned in upper left pulmonary vein (ULPV). An Amplatzer exchange guidewire 0.035’’ was advanced to ULPV. The sheath and a 6F MP catheter was pulled out. The 14F delivery sheath was inserted along with the Amplatzer wire into the ULPV. The ASD fenestrated device closure Cera (Lifetech) No. 36 mm entered the 14F delivery sheath into the ULPV. Left atrium side disc of device was inflated in LA, pulled into RA until the right atrium side disc was inflated maximally in RA.

Suddenly, the ECG changed to atrial fibrillation and SVT, HR 140 bpm. For a moment, the pulmonary pressure exceed the systemic pressure (PH crisis). However, after a while, the ECG autoconvert into the baseline (sinus rhythm) with HR 80 bpm and the systemic pressure uprise, exceed the pulmonary pressure. We observed the pressure for around 5 minutes until decided to detach the device. We underwent the right and left heart catheterization once again. The result was PARi 5.7 WU.m² and PVR/SVR 0.39. The transesophageal echocardiography post procedural documented the device stowed in place with the bidirectional shunt through the fenestration. A slight contact between the left disc and the mitral valve was seen, but pre-exixting mitral regurgitation remained trivial.

 

Discussion

Closure of an atrial septal defect in patients with severe pulmonary artery hypertension (PAH) remains controversial since a critical rise in pulmonary pressure (PH crisis) could be fatal.1 General consensus agreed that patients with PAH and PVR index of <6WU.m2 or a PVR of<3 WU or a PVR/SVR <0.3 could undergo defect closure.2-3 However, guidelines for evaluation of operability in CHD with pulmonary hypertension do not offer clear cutoff values. Schwerzmann et al reported recommendations of PAH experts regarding ASD closure. According to them, patients with PVR index >9WU.m2 or PVR >5WU or PVR/SVR >0.5 should not undergo shunt closure.4 Borderline hemodynamics data in patients with PAH (PVR index of 6–9 WU.m2 or a PVR of 3–5 WU or a PVR/SVR <0.5) should be discussed on an individual base and may considered to perform an acute vasodilator testing for such cases.4 Recently, transcatheter closure in atrial septal defect has become an preferable method compared to surgical closure. Some advances in medical treatment (especially occluder devices) have given the possibility to close defects in patients previously considered inoperable.1,5 A customized handmade fenestrations of standard device has been made to solve problems regarding closure of defect in PAH patients. Partial defect closure with a fenestrated device limits the left-to-right shunt in general and allows as right-to-left shunt during episodes of transient rises of pulmonary pressure.1,5 Some attempts have been reported using these handmade fenestrations of standard devices and showed good results.5-6 A close follow up shoud be made to evaluate clinical complain, functional status, and echocardiography evaluation since there is a report of partial or complete closure of the fenestration. 5

 

References:

1. Barlatay FG, Fournier A, Raboisson MJ, et al. Atrial septal defect closure with Occlutech ASD fenestrated device in a child with severe pulmonary hypertension. Pediatr Cardiol; 2016: 1-4.

2. Lopes AA, O’Leary PW. Measurement, interpretation and use of haemodynamic parameters in pulmonary hypertension associated with congenital cardiac disease. Cardiol Young. 2009; 19(5): 431-5.

3. Baumgartner H, Bonhoeffer P,
De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010; 31(23): 2915-57.

4. Schwerzmann M, Pfammatter JP. Approaching atrial septal defects in pulmonary hypertension. Expert Rev Cardiovasc Ther. 2015; 13(6): 693–701.

5. Dell’Avvocata F, Rigatelli G, Cardaioli P, Giordan M. Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension. J Geriatr Cardiol. 2011; 8(2): 127–9.