Platypnea orthodeoxia syndrome is a rare disorder which includes dyspnea associated with standing up and concomitant hypoxia. The following is a case report of platypnea orthodeoxia in an elderly patient with patent foramen ovale (PFO) and severe tricuspid regurgitation (TR), which was successfully treated with percutaneous closure of the PFO with a Cardioseal device .
J.N. is an 85 years old white male with medical history significant for hypertension and chronic atrial fibrillation. He was active and fully functional when he presented to the ER complaining of weakness and exertional dyspnea, which had worsened over the prior 3 months. At the time of presentation, he had dyspnea with minimal effort which was promptly relieved by resting in bed. The patient denied chest pain, orthopnea or palpitations and reported no fever or cough. The blood pressure in the ER was 138/87 mm Hg and the oxygen saturation on nasal oxygen (4 L) was 98%. The pulse was irregularly irregular, and cardiac examination was notable for a widely fixed and split second heart sound with 2/6 holosystolic murmur along the left sternal border. Breath sounds were distant but clear. Mild acral cyanosis was present while lying down, and this significantly worsened while standing up. Chest roentgenogram on admission showed cardiomegaly. The electrocardiogram revealed atrial fibrillation and right bundle branch block. Routine blood work revealed mild microcytic anemia and mild renal insufficiency with serum creatinine of 1.2 mg/dl. Other blood tests including liver function tests and coagulation panel were within normal limits. Room air pulse-oximetry performed while supine, standing up and walking 10 steps showed saturation trend of 93%, 88% and 78%, respectively. Echocardiogram showed normal left ventricular function, severe right atrial (RA) and right ventricular (RV) dilatation and severe tricuspid insufficiency with pulmonary artery (PA) systolic pressure of 40 mm Hg. Trans-thoracic echocardiogram done in supine and standing position with contrast using agitated saline (‘bubble injection‘) showed minimal shunting while supine, and significant worsening of right to left heart shunting while upright (Figure 1). Right heart catheterization revealed: mean RA pressure 5 mmHg, RV pressure 39/4 mmHg, PA pressure 39/12 mmHg and wedge pressure of 10 mmHg. There was no step-up in oxygen saturation. Intervention was done percutaneously under general anesthesia. Closure of the PFO was successfully performed using 33mm Cardioseal device. Final trans-esophageal echocardiogram showed no evidence of shunting. In contrast to presentation, post-procedural oxygen saturation remained 98% both in supine and standing positions. Patient noted significant symptom relief and improvement in functional status.
Platypnea orthodeoxia is an unusual syndrome which has most commonly been described post-pneumonectomy and in patients with atrial septal defect / PFO and elevated right heart pressures. There have been very few cases describing patients with platypnea orthodeoxia in the setting of PFO and normal right heart pressures. Platypnea orthodeoxia is postulated to occur because of stretching of the inter-atrial septum in the upright position, resulting in relative increase in defect size. The decreased RV compliance associated with advancing age might explain increase in prevalence of this syndrome in the elderly. Symptoms in our patient were thought to occur due to severe TR and presence of uncoiled aorta which might have caused anatomical distortion causing the TR jet to be directed towards the PFO, resulting in significant right to left heart shunting with cyanosis when standing up.
Trans-catheter closure in this case was the preferred treatment, given that a surgical approach potentially carried higher peri-procedural risk. After the procedure, patient‘s symptoms promptly resolved and there were no residual signs of oxygen desaturation associated with standing upright.
Maja Zaric, None.