Thalidomide-induced symptomatic third-degree atrioventricular block.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M Hinterseer.
Explore the source record for details and available documents.
We report a case of recurrent episodes of Torsades de Pointes arrhythmia in the setting of transiently impaired left ventricular ejection fraction, acute respiratory distress syndrome, transient hypokalaemia and QT-prolonging drugs, in a previously healthy 25-yr-old female patient. In the course of the clinical and genetic work-up this patient was newly diagnosed with a mutation in KCNH2 encoding the alpha-subunit of the human repolarizing potassium channel I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.
A 46-year old woman experienced an episode of arterial desaturation despite administration of 100% oxygen during anesthetization for an elective laparoscopy. Further evaluation revealed a giant pulmonary arteriovenous malformation (PAVM) with right-left shunt associated with previously undiagnosed hereditary hemorrhagic telangiectasia (HHT, Morbus Osler- Weber-Rendu). The PAVM was treated interventionally with an Amplatzer duct occluder. Transcatheter embolization of the PAVM was well tolerated with symptomatic and hemodynamic improvement. CT scan after six months demonstrated correct position of the duct occluder in the left pulmonary artery with nearly complete occlusion of the feeding vessel.PAVMs are rare direct communications between pulmonary arteries and veins, associated with HHT in the majority of cases and often presenting with dyspnea or major neurological complications due to paradoxic embolism. In this case report, we present a rational and stepwise diagnostic workup for this rare medical condition and show that transcatheter embolization is an appropriate treatment for larger malformations.
There is increased awareness of the extent to which cardiac function is influenced by gender. One of the most dramatic and potentially lethal differences is that seen in cardiac repolarization reflected in the QT interval of the surface ECG. Gender differences in QT and QTc intervals have been observed to change during the lifetime in the general population. These differences can be explained to a large extent by sex hormone driven differences in gene expression of myocardial ion channels. Numerous studies have shown that women's risk to suffer arrhythmias in the context of QT prolonging drugs is doubled compared to men. For familial long QT syndrome there is no conclusive evidence for gender effects with respect to disease onset or mortality. Only subgroup analysis by genotype demonstrated a higher risk in female patients carrying mutations in the LQT2 locus. Special attention should be given to drug-induced QT prolongation in women.