Atrial septal defect is the most common type of congenital heart diseases. Interventional closure is currently the best treatment
for atrial septal defect. However, atrial septal defect combined with severe pulmonary hypertension remains a clinical problem. We
applied a perforated occlude to the clinical treatment of atrial septal defect with severe pulmonary hypertension and achieved good
results. As the pulmonary pressure gradually went down, the reserved hole of the occlude can be blocked latterly
Closure of an atrial septal defect (ASD) with severe pulmonary
artery hypertension (PAH) is discouraged, since a serious rise in
pulmonary pressure will be fatal. In patients with ASD and severe
pulmonary hypertension, it is critically important to identify
whether the PHT is reversible before considering transcatheter
or surgical closure. As to the reversible PHT, manual fenestrated
device closure will be effective. Here we reported a patient with
ASD and severe PHT who firstly received percutaneous closure of
the defect with a manual fenestrated device.
A 26 years old female patient, with no known cardiovascular
history, was admitted to hospital for a heart murmur at the
second intercostal space on the left sternal border. She can do
light to moderate physical activity without developing dyspnea.
The transthoracic echocardiography indicated atrial septal defect
(Type II, diameter 25mm); left-to-right bidirectional shunt, severe
pulmonary hypertension (PASP: 114mmHg); right atrium and
ventricular dilatation with tricuspid severe regurgitation (Figure
1). Blood gas analysis showed: PH: 7.36, PaO2: 77.2 mmHg, PaCO2:
36.1mmHg. The electrocardiogram was normal. Chest radiograph
demonstrated pulmonary artery segment bulging suggesting sever
PTH. Right heart catheter verified sever PTH (pulmonary artery
systolic pressure was 112mmHg). Therefore, repair or closure of
the ASD would be a high risk for a probable surge of pulmonary
artery pressure post the procedure. After serious consideration,
we decided to treat the girl by a manual fenestrated closure device.
Under general anesthesia and transesophageal echocardiography
(TEE) guidance, the patient was inserting a handmade fenestrated
closure device (reserved hole 5mm, Figure 2) through the right
femoral vein, occlusion of the occluder in advance. After the device
was released, TEE showed a slight left-to-right shunt. After the
procedure, the patient’s oxygen saturation was 100%. On the second
day post procedure, the girl was discharged. Echocardiography was
performed at the 2nd month and 6th month post operation, and the
PASP was 68mmhg and 34mmhg respectively. Meanwhile, tricuspid
regurgitation was reduced to mild reflux. Right atrium and right
ventricle size also decreased when compared with preoperative.
Therefore, the patient’s PHT was reversible since the pulmonary
artery pressure decreased significantly after the procedure. Thus,
after careful evaluation, we take the second stage of procedure
to re-plug the 5mm reserved hole in the ASD closure device to
eliminate the shunt (see Figure 3). The operation was successful,
and the patient was discharged the next day.
Atrial septal defect is the most common congenital heart
disease. Although considered to be a benign disease, it can still
cause serious complications and fatalities with pathophysiological
changes [1-2]. Interventional sealing has become the most effective
way to treat secondary atrial septal defects. However, for atrial septal
defect with severe pulmonary hypertension, whether the operation
under direct vision or percutaneous closure is high risky. In this case, we performed two stage procedure to treat the ASD. Firstly,
we closed the ASD with a handmade fenestrated closure device.
Such procedure has been proved effective because the occlusion
can reduce interatrial shunt, meanwhile the handmade reserved
small hole will prevent the surge of postoperative pulmonary
hypertension . Postoperative follow-up also confirmed that
pulmonary artery pressure decreased significantly, tricuspid
regurgitation also reduced, and the heart was remodeled. When the
patient’s pulmonary arterial pressure reduced to a safe range, we
completely blocked the reserved hole through the femoral venous
esophageal ultrasound guidance.
In summary, the occluder with reserved holes has a good effect
on the treatment of patients with secondary atrial septal defect with
severe pulmonary hypertension. According to the follow-up results
after surgery, the reserved holes can be re-plugged if necessary.
However, large samples of clinical data are still needed for further
study and validation.
Bruch L, Winkelmann A, Sonntag S (2010) Fenestrated occluders for
treatment of ASD in elderly patients with pulmonary hypertension and/
or right heart failure. Journal of Interventional Cardiology 21(1): 44-49.