Managing Congenital Heart Defects in Elderly: The Platypnea-Orthodeoxia Syndrome in Underestimated Patent Foramen Ovale.

The platypnea-orthodeoxia syndrome (POS) is a rare and often suboptimally managed condition with a complex diagnostic workup, conversely displaying an easy treatment and a good recovery of symptoms, especially if consequent to an intracardiac shunt. However, its identification is challenging, due to the several clinical manifestations, the multiple etiologies, representing often the delayed presentation of a congenital heart disease. We present a case report and review of available literature on patients with the POS secondary to a patent foramen ovale successfully treated with its closure.

A 'shark'-masked electrocardiogram: case report of a Tako-Tsubo syndrome.

BACKGROUND: Triangular ST-segment elevation or 'shark-fin' sign has been described as a specific indicator of acute coronary occlusion and large myocardial ischaemia, translating into poorer prognosis. However, this electrocardiographic presentation has been reported in rare cases of Tako-Tsubo syndrome and associated with more severe physical stressors and neurological involvement. CASE SUMMARY: We present a rare case of a 51-year-old woman presenting with incoming epileptic attacks and concomitant pyometra. Despite controlling epilepsy with phenytoin and the surgical treatment of the infection, she developed sepsis requiring vasopressors, and thereafter sustained ventricular tachycardia and diffuse ST-segment elevation with the 'shark-fin' sign. TTC was confirmed by the documentation of normal coronary arteries and the complete recovery of wall motion abnormalities at discharge. DISCUSSION: Heterogeneous presentation and triggering conditions often challenge the diagnosis of Tako-Tsubo syndrome. The acknowledgement of different electrocardiographic and clinical manifestations can ease the diagnosis and the successful management of these patients, whose prognosis can be extremely severe in the acute phase, if unidentified.

Incidental Diagnosis After a Car Accident: A Rare Case of Asymptomatic Uncorrected Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is a heterogeneous congenital heart disease that is occasionally diagnosed during adulthood. However, although they are often asymptomatic, adult patients with uncorrected TOF often have a poor prognosis. Poor outcomes indicate the importance of the identification and management of these patients, especially in the context of intercurrent disease or noncardiac surgery. We describe a case of clinically silent TOF in a 51-year-old woman. TOF was unmasked during a major noncardiac surgery for a polytrauma and successfully treated with the cooperation of a multidisciplinary team. (Level of Difficulty: Advanced.).

Increased temporal dispersion of myocardial repolarization in myotonic dystrophy type 1: beyond the cardiac conduction system.

BACKGROUND AND OBJECTIVES: The most frequently mechanism underlying sudden cardiac death in myotonic dystrophy type 1 (DM1) is bradyarrhythmias due to cardiac conduction abnormalities. However the risk of ventricular tachyarrhythmias remains a concern in clinical management as well as in its determinant. We therefore assessed autonomic nervous system activity aiming to disclose differences in the QT variability index (QTVI)-a marker of temporal myocardial repolarization lability-between DM1 patients and healthy controls. We also investigated the possible differences within DM1 patients by subdividing them according either to the presence of first degree atrioventricular block (1st AVB) or to the cytosine-thymine-guanine (CTG) repeat expansion size. METHODS: Sixty-two DM1 patients and 20 healthy subjects underwent neurological and cardiological examinations, the latter including ECG, echocardiography and 24-hour Holter monitoring. All underwent a 5-minute ECG recording to assess heart rate variability power spectral components, and the QTVI values. RESULTS: Power spectral data, namely total power, low frequency power and high frequency power, were lower, whereas QTVI values were higher in DM1 patients than in controls (p<.0001). Higher QTVI values were found in DM1 subgroups with 1st AVB (p=.009) and more than 500 CTG repeat (p=.014) with respect to DM1 patients without 1st AVB and CTG<500. Spectral data did not significantly differ. At multivariable analysis, QTVI and age were independently associated with PR interval and CTG repeat. CONCLUSIONS: The increased values of QTVI argue in favour of an important heart involvement extending beyond the conduction system. Whether QTVI could be useful in predicting clinical course of DM1 clearly requires larger prospective studies.