Transvenous permanent pacemaker implantation in dextrocardia: technique, challenges, outcome, and a brief review of literature.

AIMS: Dextrocardia is a rare congenital anomaly. Pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy and associated anomalies. The literature regarding implantation of pacemaker in dextrocardia is scarce. METHODS AND RESULTS: The study involved retrospective analysis of records of patients with dextrocardia who had undergone pacemaker implantation between January 2006 and July 2013 from a single centre. Six patients with dextrocardia (five males and one female) underwent permanent pacemaker implantation (PPI) between January 2006 and July 2013. Of them, three had situs solitus dextrocardia and three situs inversus dextrocardia. All three patients with situs solitus dextrocardia had associated corrected transposition of great arteries. The indication for pacemaker implantation was symptomatic complete atrioventricular (AV) block in four, high-grade AV block in one, and sinus node dysfunction in one patient. A favourable outcome was noted during a mean follow-up of 3.9 years (4 months to 7 years) with one patient needing a pulse generator replacement. CONCLUSION: Permanent pacemaker implantation in dextrocardia can be challenging because of the distorted anatomy. Use of a technique employing angiography to delineate chamber anatomy and relationship can assist the operator during such difficult PPIs. The medium- and long-term survival after a successful pacemaker implantation in dextrocardia is favourable.

Takayasu arteritis coexisting with scalp necrosis, alopecia, and sterile osteomyelitis.

A twelve-year-old girl with classical features of Takayasu arteritis presented with scalp ulceration and osteomyelitis. Her computed tomography (CT) of the head revealed an extensive ulcerated lesion over the left high parietal region with lytic destruction of the outer and inner tables of the skull. Because of full-thickness calvarial bone involvement, chronic osteomyelitis, and ulcerated scalp lesion, she underwent debridement of involved bone along with the margin of normal skin. During surgery, underlying dura was found to be not involved, and a transposition flap was done for reconstruction. Histopathology did not reveal any evidence of bacterial infection or granulomas. Sterile osteomyelitis of the skull associated with alopecia and scalp necrosis has not been reported with typical Takayasu disease. Family physicians should be vigilant to keep this as a differential diagnosis in nonhealing osteomyelitis, not responding to antibiotics, or showing any evidence of infection.

Isolated congenital left-sided pulmonary vein stenosis in an adult patient misdiagnosed as primary pulmonary hypertension.

Pulmonary vein stenosis (PVS) is a rare condition, seen usually in association with congenital heart disease or secondary to various acquired causes. Isolated PVS, in adults, especially in absence of congenital heart disease is extremely uncommon. We report PVS of left sided pulmonary veins in an 18-year-old male, who had been till then diagnosed as primary pulmonary hypertension (PPH).

Catheter ablation of complex atrial tachyarrhythmias in adult patients with cor triatriatum.

PURPOSE: Reports concerning clinical characteristics of cor triatriatum and approaches for catheter ablation of complex atrial tachyarrhythmias remain limited. Here, we describe successful catheter ablation treatments for complex atrial tachyarrhythmias in patients with cor triatriatum and address the clinical caveats. METHODS: Demographic characteristics, electrophysiologic findings, and ablation results in four patients with cor triatriatum were described. RESULTS: Catheter ablation was performed in four patients with cor triatriatum (three sinister and one dexter) and complex atrial arrhythmias (three with persistent atrial fibrillation (AF) and one with atypical left atrial flutter). A transseptal puncture was selectively directed into the accessory compartment containing the pulmonary veins. A comprehensive preview involving transthoracic echocardiography, transesophageal echocardiography, and computed tomography of the pulmonary veins was critical for proper positioning of ablation catheters. The pulmonary veins remain the major triggers or initiators for AF, and four pulmonary vein isolation procedures were sufficient to achieve successful results with negative inducibility test in the patients with AF. Heterogeneous conduction and complex fractionated signals were observed on the fibromuscular membrane. Atypical flutter was terminated during ablation over the connection between membrane and left atrial roof. The procedure was successfully performed on all patients without complications. No acute recurrences of atrial tachyarrhythmias were observed in any of the patients during short-term follow-up. CONCLUSIONS: Catheter ablation is a feasible and efficient therapeutic strategy for treating complex atrial tachyarrhythmias in patients with cor triatriatum. Atrial remodeling due to anatomical obstruction or heterogeneous conduction of the fibromuscular membrane may serve as an arrhythmic substrate.