Oculocutaneous albinism associated with multiple malformations and psychomotor retardation

Abstract: We present a case of oculocutaneous albinism in a child associating multiple malformations (preaxial polydactyly, small penis, cardiac malformation) and psychomotor retardation. To our knowledge, this association has not been previously described.     

Abnormal Atrioventricular Node Conduction and Atrioventricular Nodal Reentrant Tachycardia in Patients Older Versus Younger Than 65 Years of Age

Study Objective: We examined the possible role of atrioventricular node (AVN) conduction abnormalities as a cause of AVN reentrant tachycardia (RT) in patients >65 years of age.
Study Population: Slow pathway radiofrequency catheter ablation (RFCA) was performed in 104 patients. Patients in group 1 (n = 14) were >65 years of age and had AV conduction abnormalities associated with structural heart disease. Patients in group 2 (n = 90) were <65 years of age and had lone AVNRT.
Results: Patients in group 1 versus group 2 (66% vs. 46% men) had a first episode of tachycardia at an older age than in group 2 (68 ± 16.8 vs 32.5 ± 18.8 years, P = 0.007). The history of arrhythmia was shorter in group 1 (5.4 ± 3.8 vs 17.5 ± 14, P = 0.05) and was associated with a higher proportion of patients with underlying heart disease than in group 2 (79% vs 3%, P < 0.001). The electrophysiological measurements were significantly shorter in group 2: atrial-His interval (74 ± 17 vs 144 ± 44 ms, P = 0.005), His-ventricular (HV) interval (41 ± 5 vs 57 ± 7 ms, P = 0.001), Wenckebach cycle length (329 ± 38 vs 436 ± 90 ms, P = 0.001), slow pathway effective refractory period (268 ± 7 vs 344 ± 94 ms, P = 0.005), and tachycardia cycle length (332 ± 53 vs 426 ± 56 ms, P = 0.001). The ventriculoatrial block cycle length was similar in both groups. The immediate procedural success rate was 100% in both groups, and no complication was observed in either group. One patient in group 2 had recurrence of AVNRT. One patient with a 98-ms HV interval underwent permanent VVI pacemaker implantation before RFCA procedure.
Conclusion: In patients undergoing RFCA for AVNRT at >65 years of age had a shorter history of tachycardia-related symptoms than patients with lone AVNRT. The longer AVN conduction intervals and refractory period might explain the late development of AVNRT in group 1.     

Nonfluoroscopic Catheter Ablation of Cardiac Arrhythmias in Adults: Feasibility,Safety, and Efficacy

Nonfluoroscopic Catheter Ablation of Cardiac Arrhythmias in Adults Background: Catheter ablations are traditionally performed using fluoroscopic guidance, exposing both patients and medical staff to the risks of radiation. Nonfluoroscopic catheter ablation has been used successfully to treat limited types of arrhythmias in children, but whether this approach has broad application in adults is uncertain. The purpose of this study was to evaluate the feasibility, safety, and efficacy of fluoroless catheter ablation in adults being treated for a range of arrhythmias. Methods and Results: Retrospective analysis was performed in 2 patient groups (both n = 60): (1) the nonfluoroscopy (NF) group consisting of consecutive adult patients, in which catheter positioning was accomplished exclusively with intracardiac electrograms (IE), electroanatomic mapping (EAM), and intracardiac echocardiography (ICE); and (2) the fluoroscopy (F) group, in which catheter positioning was additionally guided by fluoroscopy. The patients in the F group were selected to match the types of arrhythmias in the NF group. All ablation procedures were performed by one operator. The total procedure time did not differ between groups for any specific type of arrhythmia ablated. Acute procedural success was similar in both groups (NF, 59/60 [98%] and F, 60/60 [100%]). The complications were limited to a groin pseudoaneurysm in the NF group, and pericardial effusion and groin hematoma in the F group. Conclusion: Catheter ablations were efficiently and effectively performed in adults with a variety of arrhythmias using only IE, EAM, and ICE for catheter guidance. This nonfluoroscopic technique was feasible, posed no additional safety concerns, and should be readily implementable in most electrophysiology laboratories. (J Cardiovasc Electrophysiol, Vol. 23 pp. 1078‐1086, October 2012)