[A strange case of acute respiratory failure: the platypnea-orthodeoxia syndrome]. / Un insolito caso di insufficienza respiratoria acuta: la sindrome platipnea-ortodeossia.

Platypnea-orthodeoxia syndrome is a rare disorder characterized by dyspnea and oxygen desaturation in upright position, with improvement of symptoms and blood oxygenation in supine position. Basically a right-to-left shunt (intracardiac or pulmonary) or a ventilation/perfusion mismatch are necessary to develop platypnea-orthodeoxia syndrome. Atrial septal defects, including patent foramen ovale (PFO), are known to be a frequent cause of platypnea-orthodeoxia syndrome. We describe herein the case of a 79-year-old woman with platypnea-orthodeoxia syndrome and PFO who developed a refractory worsening respiratory failure. Only the "rescue" emergency closure of PFO allowed the patient a clinical turning point with immediate and sustained respiratory improvement.

Comparison of right ventricular longitudinal strain imaging, tricuspid annular plane systolic excursion, and cardiac biomarkers for early diagnosis of cardiac involvement and risk stratification in primary systematic (AL) amyloidosis: a 5-year cohort study.

AIMS: To determine the role of assessing right ventricular (RV) function, using standard echocardiography and Doppler myocardial imaging (DMI), in the early diagnosis of cardiac amyloidosis and in the prediction of mortality. METHODS AND RESULTS: Patients with primary systemic (AL) amyloidosis seen at our institution from 1 February 2004 through 31 October 2005 (N=249) were categorized by left ventricular thickness and E' velocity and compared with 38 age- and sex-matched controls. Standard echocardiographic and DMI examination were used to measure echocardiographic parameters of RV function: systolic tissue velocity, strain rate, and strain were determined for basal and middle RV free wall segments. Patients were followed up for the endpoint of mortality. RV tricuspid annular plane systolic excursion (TAPSE) and all DMI measurements were lower in patients with AL amyloidosis and normal echocardiography results (AL-normal-echo group) than controls. A bivariate model including strain of the basal segment of the RV free wall and TAPSE was the best for distinguishing AL-normal-echo patients from controls. Male sex [hazard ratio (HR), 2.2; P=0.005], brain natriuretic peptide levels (HR 1.4; P=0.003), troponin T levels (HR 1.6; P=0.01), pleural effusion (HR 3.6; P<0.001), E/A ratio (HR 1.3; P=0.006), RV systolic pressure (HR 1.02; P=0.01), and RV strain rate of the middle segment (HR 1.3; P=0.02) were independent predictors of death. CONCLUSION: DMI measures of the RV can identify early impairment of cardiac function or stratify risk of death in patients with AL amyloidosis. Further studies with longer follow-up are warranted to confirm these results.