Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome.
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A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease. (+info)
Late onset postpartum eclampsia without pre-eclamptic prodromi: clinical and neuroradiological presentation in two patients.
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In two patients eclampsia started 9 days postpartum. Headache and visual disturbances preceded seizures but none of the classic pre-eclamptic signs oedema, proteinuria, and hypertension were present until shortly before seizure onset. Brain herniation (patient 1) and status epilepticus (patient 2) necessitated neurointensive care management. Brain MRI initially showed only frontal sulcal effacement in one patient but later showed white matter hyperintensities on T2 weighted images and a previously undescribed pattern of cortical-subcortical postgadolinium enhancement on T1 weighted images in both. Neurological deficits and MRI findings were reversed with therapy in both patients. It is concluded that late postpartum eclampsia can manifest without classic prodromi and that characteristic MRI findings may lag behind clinical manifestation. (+info)
Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death.
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BACKGROUND: Sudden death (SD) in ventricular preexcitation (VP) syndrome is believed to be the result of atrial fibrillation with rapid ventricular response over the accessory pathway. Previous reports are anecdotal and often lack autopsy validation. METHODS AND RESULTS: Prevalence and clinicopathological features of VP were investigated in a series of 273 SDs in children and young adults (aged +info)
Electrophysiologic characterization and postnatal development of ventricular pre-excitation in a mouse model of cardiac hypertrophy and Wolff-Parkinson-White syndrome.
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OBJECTIVES: We sought to characterize an animal model of the Wolff-Parkinson-White (WPW) syndrome to help elucidate the mechanisms of accessory pathway formation. BACKGROUND: Patients with mutations in PRKAG2 manifest cardiac hypertrophy and ventricular pre-excitation; however, the mechanisms underlying the development and conduction of accessory pathways remain unknown. METHODS: We created transgenic mice overexpressing either the Asn488Ile mutant (TG(N488I)) or wild-type (TG(WT)) human PRKAG2 complementary deoxyribonucleic acid under a cardiac-specific promoter. Both groups of transgenic mice underwent intracardiac electrophysiologic, electrocardiographic (ECG), and histologic analyses. RESULTS: On the ECG, approximately 50% of TG(N488I) mice displayed sinus bradycardia and features suggestive of pre-excitation, not seen in TG(WT) mice. The electrophysiologic studies revealed a distinct atrioventricular (AV) connection apart from the AV node, using programmed stimulation. In TG(N488I) mice with pre-excitation, procainamide blocked bypass tract conduction, whereas adenosine infusion caused AV block in TG(WT) mice but not TG(N488I) mice with pre-excitation. Serial ECGs in 16 mice pups revealed no differences at birth. After one week, two of eight TG(N488I) pups had ECG features of pre-excitation, increasing to seven of eight pups by week 4. By nine weeks, one TG(N488I) mouse with WPW syndrome lost this phenotype, whereas TG(WT) pups never developed pre-excitation. Histologic investigation revealed postnatal development of myocardial connections through the annulus fibrosum of the AV valves in young TG(N488I) but not TG(WT) mice. CONCLUSIONS: Transgenic mice overexpressing the Asn488Ile PRKAG2 mutation recapitulate an electrophysiologic phenotype similar to humans with this mutation. This includes procainamide-sensitive, adenosine-resistant accessory pathways induced in postnatal life that may rarely disappear later in life. (+info)
Improvement of the diagnostic accuracy in computer-assisted differential diagnosis for wide QRS premature complexes.
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The differential diagnosis of wide QRS premature contractions is often inaccurate in most ECG machines with automatic computer-assisted diagnosis. The purpose of the present study was to improve the accuracy of the automated differential diagnosis between premature ventricular contractions (PVC) and supraventricular contractions with aberrant conduction (A-PSC). The study investigated 180 consecutive electrocardiograms (ECGs) with wide QRS premature contractions picked up from 3,723 in the ECG database. A new algorithm, Detection of Wide QRS Complex --> Second Derivative --> Absolute Value --> Smoothing --> T Wave Subtraction --> P' Detection, was compared with a conventional QRS morphology algorithm and P' algorithm without T wave subtraction. The rate of false positives was reduced step by step (22.3% in the conventional algorithm, 7.8% in the P' without T subtraction algorithm and 3.0% in the P' with T subtraction algorithm), resulting in a marked increase in diagnostic accuracy for A-PSC from 77.2% to 90.6% and 95.0%, respectively. In a general population with external samples, the newest algorithm showed 77.8% sensitivity, 99% specificity, and 98.9% accuracy for diagnosis of A-PSC. The new algorithm for differential diagnosis of wide QRS complexes is simple, reliable, and easy to apply to most 12-lead ECG machines with computer-assisted automatic diagnosis. (+info)
Main clinical analyses on amniotic fluid.
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BACKGROUND AND AIM OF THE WORK: To suggest a series of clinical analyses performable on amniotic fluid, in different medical complications occurring during pregnancy. METHODS: Various methodology, for different obstetrics situations, were compared among them. RESULTS: The main indication for late amniocentesis are the tests for fetal lung maturity. The most broadly accepted is the L/S ratio, which has become the "gold standard", although this chromatographic technique is labor intensive compared to several other procedures proposed and used. CONCLUSIONS: By amniocentesis, accurate fetal diagnosis has become possible. Clinical diagnostic tests are critical for many obstetric situations including premature rupture of membranes, management of pre-eclampsia, prevention of RDS. More recently, the widespread use of amniocentesis has led to study different aspects of fetal metabolism and/or fetal molecules whose functions remain to be established. (+info)
Real time electrocardiogram QRS detection using combined adaptive threshold.
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BACKGROUND: QRS and ventricular beat detection is a basic procedure for electrocardiogram (ECG) processing and analysis. Large variety of methods have been proposed and used, featuring high percentages of correct detection. Nevertheless, the problem remains open especially with respect to higher detection accuracy in noisy ECGs METHODS: A real-time detection method is proposed, based on comparison between absolute values of summed differentiated electrocardiograms of one of more ECG leads and adaptive threshold. The threshold combines three parameters: an adaptive slew-rate value, a second value which rises when high-frequency noise occurs, and a third one intended to avoid missing of low amplitude beats. Two algorithms were developed: Algorithm 1 detects at the current beat and Algorithm 2 has an RR interval analysis component in addition. The algorithms are self-adjusting to the thresholds and weighting constants, regardless of resolution and sampling frequency used. They operate with any number L of ECG leads, self-synchronize to QRS or beat slopes and adapt to beat-to-beat intervals. RESULTS: The algorithms were tested by an independent expert, thus excluding possible author's influence, using all 48 full-length ECG records of the MIT-BIH arrhythmia database. The results were: sensitivity Se = 99.69 % and specificity Sp = 99.65 % for Algorithm 1 and Se = 99.74 % and Sp = 99.65 % for Algorithm 2. CONCLUSION: The statistical indices are higher than, or comparable to those, cited in the scientific literature. (+info)
False-positive exercise test secondary to Wolff-Parkinson-White syndrome in the absence of manifest preexcitation and disappearance of ST depressions after accessory pathway ablation.
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Wolff-Parkinson-White syndrome with manifest preexcitation is a common cause of false-positive exercise test results. However, false-positive results are extremely rare without manifest preexcitation. We report a case with intermittent Wolff-Parkinson-White syndrome and exercise-induced marked ST depressions in the absence of preexcitation of the QRS complexes. His coronary arteries were normal on angiography and no ST changes were observed in the control exercise test after ablation of the accessory pathway. (+info)