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].