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.