Familial Occurrence of Accessory Atrioventricular Pathways (Preexcitation Syndrome)

Abstract

Accessory atrioventricular pathways, the anatomical structures responsible for the preexcitation syndromes, may result from a developmental failure to eradicate the remnants of the atrioventricular connections present during cardiogenesis. To study whether preexcitation syndromes could also be transmitted genetically, we determined the prevalence of preexcitation in the first-degree relatives of 383 of 456 consecutive patients (84 percent) with electrophysiologically proved accessory pathways. We compared the observed prevalence of preexcitation among the 2343 first-degree relatives with the frequency reported in the general population (0.15 percent). For 13 of the 383 index patients (3.4 percent), accessory pathways were documented in one or more first-degree relatives. At least 13 of the 2343 relatives identified (0.55 percent) had preexcitation; this prevalence was significantly higher than that in the general population (P less than 0.0001). Identification of affected relatives may have been incomplete because clinical information was obtained only about symptomatic relatives. Patients with familial preexcitation have a higher incidence of multiple accessory pathways and possibly an increased risk of sudden cardiac death. Our data suggest a hereditary contribution to the development of accessory pathways in humans. The pattern of inheritance appears to be autosomal dominant.

Familial Hypertrophic cardiomyopathy with Wolff-Parkinson-White syndrome maps to a locus on chromosome 7q3.

“…Wolff-Parkinson-White syndrome (WPW)' is one of the most common congenital cardiac abnormalities with a prevalence of 0.15-3 per 1,000 (1). WPW results from an anomalous atrioventricular (AV) conduction pathway that can produce ventricular preexcitation and paroxysmal reentrant tachycardias.…”

Evaluation of fetal heart rate artifacts, hemodynamics and digoxin treatment in fetal tachyarrhythmia by Doppler measurement of fetal blood flow — case report of a pre-excitation syndrome

“…The nonhydropic male infant weighed 4150 g and demonstrated minimal signs of neonatal distress (Apgar score 5 and 1 at 1 and 5 min respectively) with delayed onset of respiration and bradycardia, although the pH of umbilical cord blood was normal (art: 7.32, ven: 7.39). Postpartum echocardiography confirmed normal heart anatomy and ECG showed pre-exitation with delta-waves [14], but no signs of digitalis intoxication. Thus a Wolff-Parkinson-White syndrome was diagnosed and continuation of digoxin treatment was indicated [6,12].…”

Radiofrequency Catheter Ablation in Familial Paroxysmal Supraventricular Tachycardia Due to Accessory Atrioventricular Pathways

“…8 The results showed that the prevalence of manifest AP in first-degree relatives was significantly higher than that of the general population (0.55% vs 0.15%). Also, they examined the pedigrees of patients with first-degree relatives with AP and proposed autosomal dominance as a mode of transmission of familial WPW syndrome.…”

“…Compared with sporadic WPW syndrome, familial WPW syndrome is characterized by a high incidence of multiple pathways, lack of male dominance, high risk of sudden death, and low association of structural heart disease. 8 Concealed APs that conduct impulse only in the retrograde direction can also be an anatomic substrate for AVRT. However, the existence of a concealed AP cannot be suspected until the EPS-confirmed PSVT develops.…”