Page 94 - BH-2-2

Brain & Heart Lipomatous hypertrophy of the septum secundum associated with PFO

necessitating the use of devices designed for the occlusion elevated (systolic/diastolic/mean: 20/10/15 mmHg).
of atrial septal defect. This case report details that the Intraprocedural 2D contrast-TEE color Doppler confirmed
anatomical and functional aspects of LHSS are detailed, a significant RLS, a permanent leftward displacement
with a focus on the technical features of percutaneous of the hypermobile septum primum, and a prominent
interventions. LHSS with a thickness of 25 mm (Figure 1). After an
uncomplicated septal crossing, a 0.035” ×260 cm exchange
2. Case report stiff wire was positioned in the left upper pulmonary
A 76-year-old male patient suffering from arterial vein. A 25 × 45 mm balloon (Occlutech sizing balloon,
hypertension, chronic obstructive pulmonary disease OSB, Abbott, USA) revealed a 12 mm waist due to LHSS,
(COPD), and severe kyphosis of the thoracic spine was which did not completely disappear with further balloon
admitted for a sudden onset of dysarthria, dysphagia, inflation above its nominal level (Figure 2). Test occlusion
and moderate left facial weakness occurring 12 h before of the tunnel was carried out for 15 min, ensuring the
admission. Brain magnetic resonance imaging confirmed a patency of pulmonary veins or the mitral valve’s orifice.
right parietal lobe infarction. Paroxysmal atrial fibrillation Subsequent hemodynamic measurements revealed a
was observed during electrocardiogram (ECG) monitoring, remarkable increase in O saturation up to 98%, with no
2
leading to the initiation of apixaban 5 mg twice daily. changes in pulmonary and systemic arterial pressures.
Contrast-transcranial Doppler revealed a severe right-to- A third-generation 15 mm Figulla Flex II atrial septal
left shunt (RLS) through PFO under basal conditions. The defect device (FSO, Occlutech GmbH, Germany), with
patient demonstrated neurological improvement following distal and proximal discs measuring 30 mm and 26 mm,
treatment. On discharge, long-term anticoagulation respectively, featuring a very flexible double-disc design
therapy with warfarin was prescribed. However, he was with adjustable waist length, hubless left disk, and unique
readmitted weeks later for pneumonia associated with fever ball-connection between pusher and occluder was selected
and exacerbation of COPD, manifesting severe shortness (with adjustments in 3 mm increments of the waist size
of breath and hypoxemia that necessitated oxygen support. as per FSO Technology). It was successfully implanted,
ECG at admission indicated sinus rhythm, leftward QRS with the discs splayed appropriately, aligned correctly with
axis deviation, and poor R wave progression in the right the interatrial septum, and anchored appropriately to the
precordial leads. Chest X-ray revealed signs of increased LHSS rims, attributed to the unique discs and connecting
pulmonary flow and right heart chamber enlargement. waist flexibility, resulting in no residual shunt (Figure 3
One-week treatment with antibacterial drugs, short-acting and Video A1). Post-procedure hemodynamic parameters
beta-agonists (SABAs), and systemic glucocorticosteroids remained stable, with O saturation at 99%. The patient,
2
resulted in normalization of temperature, improvement who demonstrated improved clinical condition the
of wheezing, and reduction of acute inflammatory following day, was discharged home on a medication
markers. However, severe hypoxemia (O saturation of regimen including clopidogrel 75 mg daily, apixaban 5 mg
2
86–88% on room air) persisted in the upright position, twice daily, atorvastatin 20 mg daily, and bisoprolol 2.5 mg
improving with recumbency, confirming the diagnosis of daily. At the 12-month follow-up, 2D TTE color Doppler
POS. Poor acoustic echocardiographic window hindered confirmed correct device positioning with no residual
the assessment of cardiac structures and function using shunt (Figure 4). In addition, there was a significant
two-dimensional (2D) transthoracic echocardiography clinical improvement, with O saturation ranging from
2
(TTE). 2D transesophageal echocardiography (TEE) color 94–96% on room air. The patient became more active, and
Doppler provided better visualization and identified a a course of physical rehabilitation was started. To date, no
giant LHSS with a fat tissue thickness of 25 mm, a floppy complications such as device embolism, endocarditis, or
septum primum convex to the left, preserved left ventricle significant RLS have occurred.
ejection fraction (60 %).
3. Discussion
After heart team discussion, the decision to proceed
with catheter-based treatment was confirmed based on Lipomatous hypertrophy of the septum secundum,
the presence of POS exacerbated by kyphosis progression. first described by Prior in 1964 during a post-mortem
3
Written informed consent was obtained from the patient. examination, is defined as fatty infiltration exceeding
4
The procedure was performed under general anesthesia, 20 mm in thickness within the atrial septum. This benign
with continuous 2D/three-dimensional (3D) TEE and condition entails an accumulation of excessive mature
fluoroscopic guidance. Pulmonary artery pressure adipose tissue and brown fetal adipose tissue deposition
was within the normal range (systolic/diastolic/mean: in the septum secundum, excluding the fossa ovalis
30/10/20 mmHg), while the right atrial pressure was (septum primum), giving it a pathognomonic dumbbell

Volume 2 Issue 2 (2024) 2 doi: 10.36922/bh.2190