related to orthodeoxia caused by true vascular lung shunts," The New England Journal of Medicine, vol.
The most evident symptom of HPS is dyspnea; however, other clinical manifestations, more commonly seen in this syndrome, despite not specific for it, are platypnea
(increased shortness of breath while sitting up from prone position), orthodeoxia (decrease in arterial saturation by more than 5% or 4 mmHg in erect position), spider nevi, and clubbing .
Platypnea orthodeoxia, defined as dyspnea and hypoxemia when adopting an upright position and resolving during decubitus, has been attributed to PAVMs .
Vital signs showed orthostatic tachycardia and severe orthodeoxia; however, platypnea was not described (Table 1).
Patients were further evaluated for presence of platypnea
, cyanosis, clubbing and angiomas; the typical associations of hepatopulmonary syndrome.
One unique patient in our study presented with platypnea
(dyspnea on standing) and orthodeoxia (hypoxemia on standing) because of pulmonary hypertension from tumor emboli in combination with a patent foramen ovale that created a right-to-left intracardiac shunt and the reduced venous return to the heart on standing left an insufficient amount of unshunted blood to keep him from deoxygenation.
Most common pulmonary symptom is dyspnea, which may accompany platypnea
, and/or orthodeoxia.
Part II is entitled "Common Presentations in the Outpatient Setting" and includes the following chapters: Chronic Cough, Recurrent Episodes of Purulent Phlegm, Progressive Exertional Dyspnea, Chronic Exertional Dyspnea, Fatigue-Associated Daytime Sleepiness, Solitary Pulmonary Nodule, Hemoptysis, Digital Clubbing, Bilateral Pleural; Effusion, Unilateral Right-Sided Pleural Effusion, Platypnea
, Chronic Hypercapnia, Community-Acquired Pneumonia, Pleuritic Chest Pain, Non-Pleuritic Chest Pain, Upper Lobe Pulmonary Infiltrate, Wheezing, Stridor, Cavitary Pulmonary Infiltrate, and Bilateral Hilar Adenopathy.
orthodeoxia (PO) is an uncommon presentation of an underlying disorder consisting of hypoxemia and dyspnea upon assuming an upright position and is relieved by decubitus .
In our study, dyspnoea was present in 14 patients (65%), clubbing was present in 8 patients (20%), spider naevi was present in 13 patients (32.5%), cyanosis was present in 2 patients (5%) and platypnea
in 6 patients (15%).
This syndrome should be suspected in any patient with underlying liver disease presenting with platypnea