Case presentation
Fifty years old woman with chief complaint of dyspnea of exertion
functional class III from 8 months ago and suspicious intra-cardiac
shunt referred to Tehran Heart Center, a referral educational hospital
in Tehran, Iran, for further evaluation. Ehocardiography showed normal
left ventricle function (Ejection Fraction 55%), mildly dilated right
ventricle (RV) and moderate tricuspid regurgitation (TR). An important
finding was diastolic turbulent flow from right coronary cusp (RCC) of
aortic valve to right atrium (RA) at the level of tricuspid valve septal
leaflet, suggestive for rupture of sinus Valsalva.
At the time of admission her physical exams were as follows: blood
pressure 128/68 mmHg, heart rate 93 bpm and respiratory rate 19. Her O2
saturation in ambient air was 94% with no sign of peripheral or central
cyanosis. In heart & lung examination, continuous murmur was
auscultated simply in left parasternal border. No rales or crackle was
heard.
Past medical history was unremarkable except hypertension and recent
COVID-19. COVID-19 was diagnosed 3 months ago and was treated in home
with supportive therapy.
Based on these findings she was planned to undergo transesophageal
echocardiography (TEE) and cardiac catheterization in order to decide
the possibility of percutaneous closure of RCC to RA rupture. In-cath
lab TEE showed ruptured RCC with continuous flow shunt to RA (Fig. 1;
A). The aortic and RA orifices were 8 and 6 mm, respectively. The
distance between aortic valve to right coronary artery (RCA) was 12 mm.
Patent foramen oval (PFO) was also visible. Cardiac catheterization
showed significant flow from aorta to RA in aortic root injection (Fig.
1; B). The Qp/Qs ratio was 2.3 in favor of significant left to right
shunt (aorta to RA). She was planned for device closure at the next
session with patent ductus arteriosus (PDA) device.
After aortic root injection, wiring (0.035 wire) was done through sinus
rupture to pulmonary artery (PA) and was snared and externalized via
right femoral vein. Delivery sheath was inserted through rupture and
Occlutech PDA occluder 12-15 was deployed to close the rupture (Fig 1;
C). Afterwards, aortic root injection showed mild residual shunt and TEE
confirmed it.
The patient was transferred to the critical care unit (CCU) and
monitored closely. However, she had still dyspnea on exertion. The
important new finding was the different O2 saturation in supine and
upright position. In supine position the O2 saturation was 86% which
fell down to 79% while the patient was upright. Another finding was the
resistance of hypoxia to O2 therapy; even 100% O2 did not increase the
level of O2 saturation. All were in favor of intra-cardiac shunt. This
platypnea-orthdeoxia (POS), orthostatic hypoxia, was considered to be
related to PFO. She was then, planned to undergo PFO device closure.
The next day, she transferred to the cath-lab. The procedure was guided
with intra-cardaic-echocardiography (ICE) under fluoroscopy. The right
to left shunt was visible in ICE (Fig. 2; A). Cardiac oximetry was done
before device closure. The O2 saturation in pulmonary vein was 97%
however significant step-down was seen in the left atrium with O2
saturation of 88%. In addition balloon closure of PFO resulted in rapid
increase of systemic saturation up to 95%. Hence, PFO with significant
right to left shunt was responsible for patient’s symptoms.
At first, PFO closure device FigullaFlex 23-25 mm
(Occlutech GmbH, Jena, Germany) was used but retrieved due to
significant residual shunt. Then the Amplatzer ASD occluder (AGA Medical
Corp, Golden Valley, Minn) 14, was successfully deployed without any
significant residual shunt in contrast injection (Fig 2; B). The
oximetry after wards showed immediate improvement (89% to 94%).
The patient was discharged in good condition afterwards.