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Atrial Septal Defect Associated Pulmonary Arterial Hypertension: Outcomes of Closure with a Fenestrated Device

Poster · January 2018

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13 authors, including:

Joseph J Vettukattil Vishal Kaley

Helen DeVos Children's Hospital Spectrum Health
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Amal El Sisi Nikolaus Alexander Haas

Cairo University Herz- und Diabeteszentrum Nordrhein-Westfalen
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Atrial Septal Defect Associated Pulmonary
Arterial Hypertension: Outcomes of
Closure with a Fenestrated Device
Joseph Vettukattil1,2 MBBS, MD, DNB, CCST, FRCPCH, FRSM, FRCP, Vishal Kaley1 MBBS, MD, Nagib Dahdah3 MD, Amal El-
Sisi4 MD, Jochen Grohman5 MD, Nikolaus Haas6 MD, PhD, Nicholas Hayes7 BSc, MBChB, MRCPCH, Gareth Morgan8 MPhil,
MRCPCH, Eustaquio Onorato9 MD, Supaporn Roymanee10 MD, Anselm Uebing11 MD, PhD, Walter Wiebe12 MD, Ziyad M.
Hijazi13,14 MD, MPH, FACC, MSCAI
1Congenital Heart Center, Spectrum Health Helen DeVos Children’s Hospital, Grand Rapids, MI, USA
2Pediatrics and Human Development, Michigan State University College of Human Medicine, Grand Rapids, MI, USA

Background  MPAP decreased significantly from 47 to 43

 Unlike other intracardiac shunts, there is no mmHg (p=0.0002), right atrial pressure had a
known linear relationship between ostium trivial decrease from mean of 11.43 to 10.24
secundum atrial septal defects (ASD) and mmHg immediately after deployment (Table
development of pulmonary arterial 1).
hypertension (PAH).
Figure 1.
Occlutech®  During follow-up, one patient had

Fenestrated ASD
However, PH is observed in 8-10% of all device
spontaneous occlusion of the fenestration
ASDs of which 85% are females. ASD requiring emergency stenting.
associated PAH (ASD-PAH) is usually
Conclusion
independent of the degree of shunting, and
defect size. Complete closure in severe PH  FASD device implantation results in significant
is not recommended due to the risk for symptomatic improvement in patients with
increase in mean pulmonary arterial ASDPH.
pressure (MPAP). Figure 2. Post  However, further studies are required in a

deployment 3DTEE
Fenestrated ASD (FASD) closure allows for shows FASD device larger cohort of patients to determine long-
controlled residual shunt providing term prognosis.
adequate cardiac output with a mechanism Figure 3. Oxygen saturation (N=12)

for decompression in the event of critical

Collaborators
increase in MPAP.
100
98  3Division of Pediatric Cardiology, CHU Ste-
96
Justine, University of Montreal, Montreal, Qc,
 The lack of an ASD closure device with a
94
92
90 CANADA
predefined fenestration led to the

88
86
4Department of Pediatrics, Division of Pediatric
development of the Occlutech FASD device 84
82 Cardiology, Cairo University Children's Hospital,
(Figures 1 and 2). 80
Baseline Short term Follow Long term follow Cairo, EGYPT
 We describe outcomes of FASD up up
 5Department of Congenital Heart
implantation in patients with ASD-PAH. Defects/Pediatric Cardiology, Heart Center,
Figure 4. NYHA Class (N=12)
Mathildenstrasse, Freiburg, GERMANY
Methods
 6Pediatric Cardiology Department, Klinikum
 Patients meeting echocardiographic
100%

80%
2
4
I Universität München, München, GERMANY
inclusion criteria were selected for 8

compassionate use of the FASD device at

60%
10
II
 7Department of Paediatric Cardiology,
40% 7 III
Southampton General Hospital, Southampton,
11 international centers. 20%
3 IV
UK

1 1
Physician implanters provided outcomes 0%
Baseline Short term Long term  8Evelina London Children’s Hospital, Guys and
data for analysis via electronic survey. follow up follow up
St Thomas’ NHS Foundation Trust, London, UK
Results  9Cardiovascular Department, Humanitas

Gavazzeni Institute, Bergamo, ITALY


Forty-two patients underwent FASD device
implantation. Follow-up data was available
Figure 5. Change in 6 minute walk test (N=5)
700
 10Division of Cardiology, Department of

600
Pediatrics, Faculty of Medicine, Prince of
for 24 patients (females n=17) with mean Patient 1
Songkla University, Hat-Yai, Songkhla,
500 Patient 2
age of 52 years (range 5 to 80 years). Patient 3
THAILAND
400


Patient 4
Oxygen saturations improved significantly 300 Patient 5
 11Adult Congenital Heart Centre and National
from baseline mean of 93% (81-100%, 200
Centre for Pulmonary Hypertension, Royal
n=24) to 96% on short term follow-up (90- 100
Brompton Hospital, Imperial College London,
0
100%, n=21, p=0.0147) and was Baseline Short term follow Long
up term follow up London, UK
maintained at 96% on long-term follow-up
Table 1. Change in hemodynamics after FASD
 12Department of Cardiology, Deutsches
(n=12) (Figure 3). implantation (N=16) Kinderherzzentrum Sankt Augustin, Sankt
 Symptomatic improvement was observed Baseline Short-term
follow-up
P-value Augustin, GERMANY
with NYHA Class III symptoms noted in Right atrial 11.43±3.04 10.24±3.04 0.052  13Pediatrics, Weill Cornell Medicine,

81% at baseline, decreased down to 8% at pressure

(mmHg) New York, USA
long term follow-up (n=12), with Mean PA 46.78±19.53 43.00±16.83 0.0002  14Department of Pediatrics, Sidra
improvement in average 6MWT distance pressure
Cardiac Program, Sidra Medical and
(mmHg)
from 210 to 456 meters (Figure 5).
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