Case Discussion
In present study, we described a middle aged woman with DOE from 8
months ago and echocardiographic finding of ruptured sinus Valsalva to
RA and PFO. After, trans-catheter device closure the orthostatic
hypoxemia (platypnea) was more evident. Hence, she underwent PFO closure
afterwards due to platypnea-orthodeoxia syndrome (POS).
The aneurysm of sinus Valsalva may be congenital or acquired after
infective endocarditis, atherosclerosis or dissection[4]. This rare
lesion, may originate from RCC (65%–85%), the non-coronary sinus
(10%–30%), and, rarely, the left coronary sinus (1%–5%)[5].
This pathology is more often seen in Asian male and is frequently
asymptomatic. Cases with un-ruptured aneurysm and symptoms of RV outflow
obstruction or cerebrovascular emboli have been reported[6].
Ruptured sinus Valsalva aneurysm (RSVA), however, is related to
significant cardiac malfunction. Rupture of RCC to RA causes significant
left to right shunt, tricuspid regurgitation, RV failure and finally
biventricular heart failure may happen[1]. If being uncorrected it
will result in deterioration of heart function, hence, early invasive
management is advised. Trans-catheter approach is preferred over open
surgery in cases with RSVA.
Symptoms in patients with RSVA are mostly acute or subacute. However,
the disease course of our patient was chronic. The one explanation may
be the presence of PFO decreases a proportion of left to right shunt.
High RA pressure because of massive regurgitated flow from aorta caused
right to left shunt and decreased RV inflow. This patient did not
experience acute RV failure and the size and function of RV were within
normal range.
POS is a rare disorder. Both dyspnea and O2 saturation deteriorates in
upright position compared to supine state[7]. Occurrence of this
syndrome requires two things; one, intra-cardiac interatrial septal
defect or intrapulmonary shunt and two, a functional component during
upright standing; interatrial septum deformation. Atrial septum will be
deformed while standing and may allow easy streaming of blood from
inferior vena cava[8]. The relation between aortic pathologies and
POS has been reported in two previous cases. One, in a patient with
thoracic aortic aneurysm and another one after Transcatheter Aortic
Valve Implantation[9, 10].
As far as we know this is the first case of POS after trans-catheter
device closure of RSVA. The patient had dyspnea on exertion from 8
months ago, however, during many visits; no one carefully auscultated
the heart. Even after COVID-19 infection she did not undergo
echocardiography to evaluate the other causes of dyspnea.
After successful RSVA closure, the symptoms of the patient were
carefully noticed and POS was diagnosed by basic physical examinations.
This syndrome is one of the indications of PFO closure[10].