Congenital heart disease affects local gyrification in 22q11.2 deletion syndrome

22q11.2 deletion syndrome (22q11.2DS) is a common genetic condition associated with cognitive and learning impairments. In this study, we applied a three-dimensional method for quantifying gyrification at thousands of points over the cortical surface to imaging data from 44 children, adolescents, and young adults with 22q11.2DS (17 males, 27 females; mean age 17y 2mo [SD 9y 1mo], range 6–37y), and 53 healthy participants (21 males, 32 females; mean age 15y 4mo [SD 8y 6mo]; range 6–40y). Several clusters of reduced gyrification were observed, further substantiating the pattern of cerebral alterations presented by children with the syndrome. Comparisons within 22q11.2DS demonstrated an effect of congenital heart disease (CHD) on cortical gyrification, with reduced gyrification at the parieto-temporo-occipital junction in patients with CHD, as compared with patients without CHD. Reductions in gyrification can resemble mild polymicrogyria, suggesting early abnormal neuronal proliferation or migration and providing support for an effect of hemodynamic factors on brain development in 22q11.2DS. The results also shed light on the pathophysiology of acquired brain injury in other populations with CHD.     

Sotalol: From "Just Another Beta Blocker" to "The Prototype of Class III Antidysrhythmic Compound"

Sotalol is a beta-blocking drug devoid of membrane stabilizing properties, as well as intrinsic sympathomimetic actions, or cardioselectivity. In addition, sotalol prolongs atrial and ventricular repolarization (Class III antiarrhythmic activity). It appears to have less myocardial depressant effect than other beta-blocking agents. Given orally, bioavailability of the drug reaches 100%. Sotalol's plasma half-life is 15 hours (range 7–18) and is dependent only on renal function. In clinical practice, it has been found effective in the suppression of nearly all supraventricular and ventricular dysrrhythmias except those related to prolonged ventricular repolarization. Most common adverse effects are dyspnea, bradycardia, and fatigue, which results in drug termination in 16% of the cases. Torsades de pointes usually associated with bradycardia and drug induced QTc prolongation has been reported in 1.9%–3.5% of the patients receiving sotalol. This complication may be reduced by limiting the dose (< 640 mg/day) especially in patients with impaired renal function. In addition hypokalemia must be avoided. To sum up, the combination of Class II and Class III effects may carry additional benefits. However, further studies are required to test such hypotheses.     

Electronic Article Surveillance: A Possible Danger for Pacemaker Patients

In order to evaluate if antitheft devices commonly designed as electronic article surveillance (EAS) systems can be dangerous for pacemaker patients, in vitro and in vivo studies were made in close cooperation between a pacemaker center and an EAS designer. Three types of EAS radiation including radiofrequency, magnetic, and pulsed electromagnetic fields were applied to various pacemakers. The in vitro study consisted of exposing to the EAS fields 28 pacemakers connected to unipolar leads. Radiofrequency fields and pulsed electromagnetic fieids evoked minor effects and no prolonged inhibitions. When exposed to magnetic fields, most of the pacemakers switched to "fixed rate" pacing, but inhibitions were observed in 13 pacemakers exposed to 300 Hz, and in 14 pacemakers exposed to a 10-kHz magnetic field when they were moved at cardiac frequencies within the fields. The in vivo study was made on 32 volunteers treated by 26 different pacemakers: 22 single chamber and ten dual chamber. All patients had been monitored in the pacemaker clinic and pacemakers were working well. Radiofrequency and pulsed electromagnetic fields did not affect the pacemaker function. Magnetic interference evoked prolonged inhibition of seven out of the ten dual chamber pacemakers, causing brief asystole in patients being continually paced. None of the dual chamber pacemakers incorporated "safety stimulation intervals" after ventricular bianking. The EAS artifact was sensed after the ventricular blanking causing a cross-talk ECG pattern. No reprogramming was induced by the electromagnetic fields. This experience demonstrates that certain EAS may be dangerous for pacemaker patients. Following this cooperative study a pacemaker safe EAS circuit delivering short bursts of magnetic fields has been designed.     

Primary cardiogenic shock during acute myocardial infarction: results of emergency cardiac transplantation

Fifteen patients with acute myocardial infarction and cardiogenicshock underwent emergency cardiac transplantation after medicaltreatment failed to improve their haemodynamic status. Theirmean age was 49 ± 7 years. The infarction was anteriorin 12 cases, inferoposterior in two cases, and septal in one.Shock occurred within 3 days after the onset of chest pain innine patients, and during the first day in six of them. Mechanicalcirculatory assistance was used in six patients as a bridgeto transplantation when their haemodynamic status could notbe stabilized pharmacologically. Orthotopic cardiac transplantationwas performed an average of 15.6 ± 14 days after onsetof infarction. Three patients died during the early post-operativeperiod. Another died 7 months after transplantation. Duringthe mean follow-up period of 30.6 ± 20.3 months, therewere three acute rejections, all successfully treated, and onechronic rejection. The survival rate for this series is 70%.Thus, emergency cardiac transplantation may be the best optionfor selected patients with acute myocardial infarction and cardiogenicshock refractory to conventional therapy.     

Symptomatic Pericardial Disease Associated with Patch Electrodes of the Automatic Implantable Cardioverter Defibrillator: An Underestimated Complication?

Constrictive pericarditis can be associated with ICD patch electrodes. During a mean follow-up of 24 months, in a population of 35 patients who received ICDs with a patch electrodes configuration, we identified three patients with clinical and hemodynamic signs compatible with this event. Patient 1, a 35-year-old male, underwent implantation of an ICD because of a primary electrical disease complicated by cardiac arrest. Fourteen months later he complained ofexertional dyspnea without any signs of heart failure. Right heart catheter- ization showed high filling pressures and diastolic dip and plateau in pressure curves. Thoracotomy and pericardia! exploration were performed. Three months after removal of the patches and insertion of an endocardial lead system, the patient had normal respiration. Patients 2 and 3, who suffered from coronary heart disease without heart failure, exhibited a hemodynamic profile suggestive of constrictive pericarditis: in one patient, 10 months after ICD implantation, associated with right heart failure; and in the other, 18 months after ICD implantation with left heart failure. Patch electrodes were removed in these two patients and replaced by endocardial lead electrodes with subsequent clinical improvement. It is concluded that constrictive pericarditis related to epicardial patch is not an uncommon occurrence during ICD therapy and should be considered in patients who show clinical signs of cardiac decompensation.