[A strange case of acute respiratory failure: the platypnea-orthodeoxia syndrome].

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.

The noncompaction of the left ventricular myocardium: our paediatric experience.

Objectives: The noncompaction of the left ventricular myocardium is a rare congenital heart disease, characterized by an excessive prominence of trabecular meshwork, spaced out by deep intertrabecular recesses, consequent to the arrest of the normal myocardium embryogenesis. Although there are numerous descriptions, the physiopathological effects of the structural alterations, just like the clinical spectrum and the evolution of the disease, are not totally clarified. In the present study, we have evaluated the natural history of the disease, the familial incidence and the alterations of the systolic and diastolic function.

Evaluation of diastolic function by the Tissue doppler in children affected by non-compaction.

The Tissue doppler analysis is a new echocardiographic approach to evaluate the diastolic function. In this report we performed a Tissue doppler analysis in a series of 15 children affected by non-compaction. The bidimensional echo-cardiogram showed a systolic function which was reduced only in 8 patients.