Case Reports: Case Report
Platypnea-orthodeoxia is a rare syndrome, defined clinically by dyspnea and decreased oxygenation while sitting or standing. We describe a case of platypnea-orthodeoxia after open repair of a contained rupture of an abdominal aortic aneurysm, which was managed successfully with patent foramen ovale (PFO) closure.
Informed consent was obtained from the patient for publication of this report.
A 67-year-old man with a history of essential hypertension and diabetes mellitus presented in our emergency department with a contained rupture of an abdominal aortic aneurysm. After hemodynamic stabilization and computed tomography, the patient was immediately transferred to the operating room, where a tubular Dacron graft (Silver Graft, B. Braun, Melsungen, Germany) was inserted via a transperitoneal approach. Suprarenal aortic cross-clamping was necessary for 15 minutes. After an intraoperative massive transfusion, hemodynamic stability was achieved (arterial blood pressure 135/85 mm Hg, heart rate 85/min, hemoglobin 13.4 g/dL), and the patient was brought to the intensive care unit (ICU). Fibrinogen and fresh frozen plasma were transfused to further stabilize coagulation. Because of intraabdominal bleeding with significant abdominal distension, a second laparotomy was performed 7 hours later, and venous bleeding from the pancreas was identified and corrected. Again, a massive transfusion was necessary during surgery to stabilize his hemodynamics. On the second postoperative day, acute renal failure was apparent and continuous veno-venous hemodialysis with regional citrate anticoagulation was started. Due to prolonged and difficult separation from the respirator, tracheostomy was performed on the 11th postoperative day. During the following ICU course, there was continued abdominal distension because of recurrent intraabdominal hematomas, and in contrast to normal conditions, his arterial hemoglobin oxygen saturation (SpO2) dramatically decreased from 96% to approximately 78%, when his position was changed from supine to upright despite unchanged fraction of inspired oxygen (FIO2 = 0.50). Chest radiograph did not reveal any relevant intrapulmonary pathology (Fig. 1).
Because a right-to-left intracardiac shunt was suspected, transesophageal echocardiography (TEE) was performed, and after injection of agitated saline via a central venous catheter, a PFO with increased right-to-left flow while the patient was in the upright position was demonstrated (Fig. 2; see Video 1, Supplemental Digital Content 1, http://links.lww.com/AACR/A8 and see Video 2, Supplemental Digital Content 2, http://links.lww.com/AACR/A9). Except for a minor tricuspid insufficiency, no other pathologic results were diagnosed. Blood gas analyses obtained during his TEE examination while supine and upright are provided in Table 1. At the time of diagnosis, the patient was already receiving intermittent dialysis, and no significant orthostatic changes in arterial blood pressure or central venous pressure were observed during mobilization. Furthermore, intravascular normovolemia was maintained as well as controlled regularly via transthoracic echocardiography during the whole ICU course.
Right heart catheterization was performed to evaluate intracardiac pressures. Because these were within normal limits (right ventricle systolic/diastolic pressure 23/4 mm Hg; right atrial pressure 4 mm Hg), the diagnosis of a platypnea-orthodeoxia syndrome was confirmed. Subsequently, a PFO occluder (Cardia Ultrasept® 25 mm; Cardia Inc., Eagan, MN ) was implanted successfully after which the patient was easily separated from the respirator (SpO2 at 95% breathing room air) and discharged from our hospital 45 days after admission.
Platypnea-orthodeoxia is a rare syndrome, defined clinically by dyspnea and decreased arterial oxygenation while in a sitting or standing position.1 Three conditions need to coexist in a patient with classical platypnea-orthodeoxia syndrome: an anatomic component such as an interatrial communication, normal intracardiac pressures, and a condition that changes the shape of the atrial septum and redirects the venous blood flow from inferior vena cava directly through the PFO.1–3
Although PFO is a common cardiac condition found in nearly 25% of the healthy population, approximately only 150 cases of platypnea-orthodeoxia syndrome are described in the literature. Due to difficulty in establishing the diagnosis, the incidence of this syndrome may be underestimated. Different causes could have been responsible for platypnea-orthodeoxia syndrome in our patient. First, the ruptured abdominal aortic aneurysm and subsequent extensive intraabdominal surgeries with recurrent hematomas may have increased intraabdominal pressure and influenced the anatomical axis of the heart. Although this exact combination of events is not yet described in the literature, several case reports described the coincidence of a thoracic aortic aneurysm and platypnea-orthodeoxia syndrome.4–6
Moreover, an enlarged aortic root may lead to increased mobility and direct compression of the atrial septum, resulting in opening the foramen ovale, and simultaneous right-to-left-shunting by the entry of inferior vena caval flow.2 In our case, the patient had a borderline aortic root diameter (40 mm) that may have contributed to platypnea-orthodeoxia syndrome. Cases of platypnea-orthodeoxia syndrome are, among others, also described after pneumonectomy,7 blunt chest trauma,8 and in the presence of a pericardial effusion,9 which supports the notion that a change in thoracic anatomy is the principal initiating factor for the development of platypnea-orthodeoxia syndrome. Because our patient never complained about dyspnea while in a sitting or standing position before his present illness, we assume this pathomechanism to be responsible in our case as well.
We speculate that in most cases of platypnea-orthodeoxia, the upright posture allows gravity to stretch the PFO, resulting in an increased right-to-left shunt.10 However, this could not be demonstrated in our TEE examination. For diagnosis, TEE is recommended11 while the patient, in whom a PFO has been demonstrated, is in an upright position after injection of agitated saline. An additional Doppler examination is able to classify its hemodynamic relevance. Furthermore, TEE is mandatory before interventional closure to measure the exact dimensions of the PFO.
In conclusion, further investigations are needed to explain the precise mechanism causing a right-to-left shunt despite normal intracardiac pressures. In light of the increasing trend in ICUs toward very early mobilization of even mechanically ventilated patients and patients receiving extracorporeal membrane oxygenation,12 we wish to raise awareness among anesthesiologists for this rare and potentially underdocumented complication in which early diagnosis can alter the outcome.
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