Improvement of acute RFCA outcomes was contemporaneous with introduction of novel technologies. Intra-atrial re-entrant tachycardia recurrence was common, and no effect on mortality was discerned, but most patients had effective palliation of symptoms. Chronic outcome predictors included the underlying disease severity, application of novel technologies and successful ablation of all targeted arrhythmia circuits.

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The crista terminalis, atriotomy, and right atrial scars can be identified in patients with repaired congenital heart disease by electroanatomic mapping in sinus/atrially paced rhythm. These conduction barriers frequently function as the central obstacle for IART. Demonstration of such features may help focus investigational mapping without reliance on spontaneous initiation of the tachycardia.

Background-Catheter ablation of left-sided atrial arrhythmias generally is performed using a transfemoral venous approach through the inferior vena cava (IVC). In this report, we assessed the feasibility of a percutaneous transhepatic approach to ablation of left-sided atrial arrhythmias in 2 patients with interruption of the IVC. Methods and Results-Patient 1 had atrial flutter in the setting of complex congenital heart disease and prior Fontan for univentricular physiology and a single atrium. Patient 2 had atrial fibrillation. Percutaneous hepatic vein access was obtained with ultrasound and fluoroscopic guidance. Transseptal catheterization was performed in patient 2. After the procedure, the hepatic tract in patient 1 was cauterized using a bipolar radiofrequency catheter, and an Amplatzer vascular plug was used in patient 2 to obtain hemostasis. Percutaneous hepatic vein access was achieved without complications. After electroanatomical mapping, a linear lesion was placed between the single atrioventricular valve and the confluence of the hepatic veins in patient 1; this terminated the flutter, and bidirectional block was achieved. In patient 2, the pulmonary veins were electrically isolated using an extraostial approach, isolating the ipsilateral veins in pairs. Additionally, ablation of right-side atrial flutter was achieved by obtaining bidirectional block across a linear lesion between the tricuspid valve and confluence of the hepatic veins. Hemostasis of the transhepatic tract was attained in both patients. Conclusions-In

Background-Identification of infants at risk for sudden arrhythmic death remains one of the leading challenges of modern medicine. We present a family in which a common polymorphism (single nucleotide polymorphism) inherited from the father, combined with a stop codon mutation inherited from the mother (both asymptomatic), led to 2 cases of sudden infant death. Methods and Results-KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, CACNA1c, CACNB2b, and KCNJ2 genes were amplified and analyzed by direct sequencing. Functional electrophysiological studies were performed with the single nucleotide polymorphism and mutation expressed singly and in combination in Chinese ovary (CHO-K1) and COS-1 cells. An asymptomatic woman presenting after the death of her 2-day-old infant and spontaneous abortion of a second baby in the first trimester was referred for genetic analysis. The newborn infant had nearly incessant ventricular tachycardia while in utero and a prolonged QTc (560 ms). The mother was asymptomatic but displayed a prolonged QTc. Genetic screening of the mother revealed a heterozygous nonsense mutation (P926AfsX14) in KCNH2, predicting a stop codon. The father was asymptomatic with a normal QTc but had a heterozygous polymorphism (K897T) in KCNH2. The baby who died at 2 days of age and the aborted fetus inherited both K897T and P926AfsX14. Heterologous coexpression of K897T and P926AfsX14 led to loss of function of HERG current much greater than expression of K897T or P926AfsX14 alone. Conclusions-Our data suggest that a common polymorphism (K897T) can markedly accentuate the loss of function of mildly defective HERG channels, leading to long-QT syndrome-mediated arrhythmias and sudden infant death.