Pediatr Cardiol
DOI 10.1007/s00246-016-1530-4
CASE REPORT
Atrial Septal Defect Closure with OcclutechÒ ASD Fenestrated
Device in a Child with Severe Pulmonary Hypertension
Francisco Gonzalez-Barlatay1 • Anne Fournier1 • Marie-Josée Raboisson1
Nagib Dahdah1
Received: 8 September 2016 / Accepted: 12 November 2016
Ó Springer Science+Business Media New York 2016
Abstract We report a 5-year-old patient with severe pul-
monary hypertension and a large secundum atrial septal
defect who benefited from a percutaneous closure of the
defect with an OcclutechÒ custom-made fenestrated
device. Whereas the closure is technically identical to
standard atrial defect closure, the immediate and midterm
beneficial results are presented.
Keywords Pulmonary hypertension
Atrial septal defect

Partial defect closure
Fenestrated device
Introduction
Closure of an atrial septal defect (ASD) in patients with
severe pulmonary artery hypertension (PAH) is discour-
aged, otherwise a critical rise in pulmonary pressure could
be fatal. Recent advances in PAH medical treatment have
given the possibility to close defects in patients previously
considered inoperable [1]. The instauration of a medical
treatment prior to closure has led to a better outcome after
the procedure. In this context, the fenestrated closure of
ASDs is a tool that allows closing the defect leaving a
controlled residual shunt as a decompression mechanism
with a potential right-to-left shunt, in order to maintain the
cardiac output in case a critical increase in pulmonary
pressure occurs. Given that percutaneous closure is the
treatment of choice in the most part of ASDs, some
& Nagib Dahdah
nagib.dahdah.hsj@ssss.gouv.qc.ca
1 diastolic rectification of the ventricular septum in the short-
Division of Pediatric Cardiology, CHU Sainte-Justine,
University of Montréal, 3175, Côte Sainte-Catherine,
Montréal, QC H3T 1C5, Canada
•
attempts have been reported using handmade fenestrations
of standard devices [2]. Patency of handmade fenestrations
is unpredictable, and they usually have an undesired
spontaneous closure despite anticoagulant therapy [3, 4].
We report our experience of an ASD closure in a patient
with severe PAH using the Occlutech device. A custom-
made 6-mm fenestration was used after parental consent
was obtained. The use of the device was granted approval
from the Health Canada Special Access Programme.
Case Report
A 15.5-kg 5-year-old boy with Noonan syndrome was
diagnosed in his first year of life with ostium secundum
ASD and PAH. Initial cardiac catheterization at 2 years
eliminated structural lesions, and primary PAH was
retained. Due to increasing PAH parameters, the patient
was started on sildenafil, a phosphodiesterase-5 inhibitor at
age 2, to which bosentan, an endothelin receptor antago-
nist, was added. Aminosalisilate anti-aggregation dose and
permanent nasal oxygen delivery were also initiated at that
time.
The patient was recently considered for partial closure
of the ASD given the persistent severe PAH, large ASD,
and worsening tolerance to exercise and daily activities
(NYHA class II; quantifiable example of exertion follow-
ing 5–10-min street play). Oxygen saturation at rest was
92% in room air and 85–90% with exercise. He was
evaluated at age 5 by echocardiography confirming the
large ASD with bidirectional shunt (predominantly left to
right), right chambers dilatation, and both systolic and
axis view. Pulmonary pressures could not be quantitatively
estimated due to the lack of significant tricuspid
123

Table 1 Hemodynamic
parameters in baseline and after
vasodilator challenge PAP (mmHg)
SAP (mmHg)
PWP (mmHg)
Qp (l/min/m2)
Qp/Qs
2
PVR (Woods units 9 m )
SVR (Woods units 9 m2)
PVR/SVR
PAP pulmonary arterial pressure, SAP systolic arterial pressure, PWP pulmonary wedge pressure, Qp
pulmonary flow, Qs systemic flow, PVR pulmonary vascular resistances, SVR systemic vascular resistances
regurgitation. Cardiac MRI delineated an ASD of
22 mm 9 15 mm. Accordingly, three devices of different
sizes (24, 27 and 30 mm) were custom made (Occlutech
International AB, Helsingborg, Sweden) with a 6-mm
fenestration before the scheduled cardiac catheterization.
A diagnostic catheter procedure including oxygen and
nitric oxide challenge was performed under general anes-
thesia with a transesopageal echocardiography guidance.
The ASD measured 19.6 mm with deficient retroaortic rim
and a small inferior vena cava rim of 3.6 mm. All other
rims were sufficient (7.7-mm atrio-ventricular rim and
8-mm superior vena cava rim). Severe PAH was confirmed
with high pulmonary vascular resistances, and satisfactory
33% reduction with a combination of oxygen (100% FiO2)
and nitric oxide (40 ppm) (Table 1). Stop flow balloon
sizing of the defect was 21.5 mm by echo and 21.1 mm on
fluoroscopy. A 24-mm OcclutechÒ fenestrated ASD
occluder (Fig. 1) was then inserted by standard ASD clo-
sure technique. The device was easily deployed at the first
attempt. A slight contact between the left disc and the
mitral valve was seen, but pre-existing mitral regurgitation
remained trivial. The left disc remained mildly convex
initially which improved to full flattening after un-an-
choring of the device. The bidirectional shunt through the
fenestration was documented by echo.
The patient was discharged on day 4 following initiation
of anticoagulation therapy, initially with low molecular
weight heparin in transition to warfarin, maintaining
international normalized ratio between 2 and 2.5 [5]. At the
first follow-up, 9 days after the procedure, the pulmonary
pressure estimated by Doppler ultrasound was 70% of the
systemic pressure via a reliable trivial tricuspid regurgita-
tion. There was no mitral or aortic regurgitation. A bidi-
rectional shunt through the device fenestration was
confirmed. Subsequently, diurnal nasal oxygen supplement
was stopped. At the last follow-up, 5 months after closure,
the patient was reported by the parents to be ‘‘much more
energetic’’ with a significant improvement in exercise
capacity (NYHA class I; up to 40 min tolerance for street
123
Pediatr Cardiol
FiO2 21% FiO2 100% FiO2 100% ? NO 40 PPM
74/42 (57) 62/31 (46) 56/25 (40)
97/61 (80) 96/54 (75) 76/45 (58)
7 7 6
2.87 3.13 2.93
0.81 0.99 1.13
17.27 12.45 11.59
20.57 21.47 20.45
0.84 0.58 0.57
soccer). Although a 6-min walk test could have been
desirable in this patient, in practice this was challenging to
properly perform at this young age. A treadmill or
ergometer exercise stress testing would have been even
more challenging. The oxygen saturation at room air was
between 98 and 100% at rest and 94% with exercise. The
echocardiography showed patency of the fenestration with
bidirectional shunt, predominantly left to right (Fig. 2), and
an estimated pulmonary artery pressure also near 70% of
the systemic pressure. The patient remains on oral antico-
agulation, bosentan, sildenafil, and nocturnal oxygen
delivery.
Discussion
Congenital heart defects, including ASDs, are at risk of
PAH even when corrected at an early age [6]. One would
expect that those PAH in early childhood are at an even
higher risk if lesions with significant left-to-right shunts are
not repaired. In general, in patients with mild PAH sec-
ondary to excessive pulmonary flow, full closure of the
ASD can be achieved with expected normalization of
pulmonary pressure. For those with high degrees of PAH,
anti-PAH therapy could be successful in preparation for
defect closure as reported in a young adult with large ASD
[1]. Otherwise, partial defect closure with a fenestrated
device limits the left-to-right shunt in general and allows a
right-to-left shunt during episodes of transient rises of
pulmonary pressure [7]. The handmade fenestrations using
sheath dilators and balloon stretching of standard
AmplatzerÒ devices closed spontaneously 4 months after
implantation in a 3.4- and a 3.9-year-old children [3].
Whereas these two patients were not reported to be main-
tained on anticoagulation therapy, a 4-mm fenestration
with suturing of the margins of the created hole remained
patent 12 months after implantation in a 66-year-old
patient who was maintained on oral anticoagulation ther-
apy. In another report, a 4-mm fenestration using balloon
Pediatr Cardiol

Fig. 1 Actual Occlutech ASD fenestrated device compared to fluoroscopy and 3D ultrasound imaging

Fig. 2 Sub-costal echocardiography view demonstrating a left-to-right (left panel, red color Doppler signal) and right-to-left (right panel, blue
color Doppler signal) shunt across the fenestration 5 months following the intervention

dilatation on an Amplatzer occluder allowed only small
shunt at 4.5 and 6 months after the procedure in two
elderly patients, and a near-complete closure at 18 months
in a third patient despite oral anticoagulation [4]. An
approach to maintain the patency of the created defect used
a 5-mm bare metal coronary stent within the septal
occluder by securing a coronary guide wire along the
insertion of the device [2]. Patency was confirmed by
echocardiography 7 months post-procedure while under
oral anticoagulant and then under aspirin alone after
11 months from the procedure. Whereas this was particular
for a partial closure of a large ASD with pulmonary
hypertension, a similar technique was described in the
previous report where a bare metal stent was used to
maintain partial shunt across an Amplatzer vascular plug in
order to restrict a fenestration in a Fontan circuit [3]. In the
latter case, a persistent fenestration across the stent was
confirmed for at least 39 months after the intervention
while the patient was maintained on oral anticoagulation.
In our case, a 6-mm fenestration was opted for to insure
proper permeability following expected endothelialization
which would cause a 1–2 mm reduction in diameter.
Accordingly, a permanent communication of 4–5 mm
between the right and the left atria would be secured.

123

There are patients, however, who need a patent fenes-
tration for longer periods, such as our patient, because of
the significant PAH. We therefore elected to use the
Occlutech ASD device for three reasons. First, the feasi-
bility and safety in small children under 2 years old (mean
weight 7.4 kg) have been previously reported in the first
generation of the device [8]. Second, the improved
anchoring mechanism of the device since that publication
offers higher flexibility for better positioning and adapt-
ability to the septum, thus preventing undue angulation and
tension while still attached. Third, unlike other manufac-
turers, a bench-tested controlled fenestration was possible.
In our case, we elected for anticoagulation therapy and
aspirin to facilitate and maintain the patency of fenestration
on the one hand. On the other hand, and in the presence of
bidirectional shunt, potential paradoxical emboli could be
better prevented. Finally, we are hopeful that the PAH will
subside at some point. Should this occur, one could con-
sider closing the fenestration in the future with a small
ASD device. Anticoagulation could be discontinued
afterward.
Compliance with Ethical Standards
Conflict of interest The authors have no conflicts of interest to
declare.
Ethical Standards The use of the custom device for this patient was
approved by Health Canada Special Access Programme for Custom-
Made Devices and Medical Devices.
Informed Consent A written informed consent was obtained from
the parents prior to submitting this report.
123
Pediatr Cardiol
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