February 23rd 2018

Percutaneus Atrial Septal Defect Closure in Severe Pulmonary Hypertension with Fenestrated Device

Category: Structural, Congenital,



Yovi Kurniawati National Cardiovascular Center Harapan Kita



Radityo Prakoso National Cardiovascular Center Harapan Kita

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.

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.


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

Video 1. The transthoracal echocardiography pre 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.

Video 2. Deploy of ASD fenestrated device

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.


Video 3. The transesophageal echocardiography post procedure


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



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.

what the experts say

Poppy S Roebiono
National Cardiovascular Center Harapan Kita

Most children with atrial septal defect (ASD) are asymptomatic. If the defect is large and untreated, pulmonary arterial hypertension (PAH) and symptoms begin to develop in adults of 30s and 40s due to less RV and LV complaints. Therefore, diagnosis of ASD sometimes may not be recognized in children, and later these patients come to seek medical treatment after develop symptoms and severe PAH.

           In the era of transcatheter closure of secundum ASDs well established, almost all this type of ASDs can be closed by a device, unless lack adequate rim for a device to lean on or the defect is too large for the available device. In elderly patients, the prompt recovery following transcatheter ASD closure makes this alternative approach to surgery more attractive in view of prolonged convalescence and an increased rate of significant complications in the older population due to comorbidities. Even patients with PAH and high pulmonary arterial resistance, the defect can be closed by the device, as long as it still reversible by pulmonary vasodilator testing. Fenestrated ASD device is the effective option for closing the defect in ASD patients with severe PAH. Partial defect closure will decrease the left-to-right shunting through the defect and also decreasing the pulmonary hypertension crisis due to restrictive LV physiology.

           Congratulations to dr. Radityo Prakoso, dr. Yovi Kurniawati and all team from National Cardiovascular Center Harapan Kita, Jakarta for the successful trancatheter closure of a large secundum ASD with severe PAH. Hopefully, all these kind of patients can be treated well and will reduce the morbidity and mortality in Indonesia.