Atrial pacing-induced alternating Wenckebach periodicity and multilevel conduction block in children.

Multilevel block within the atrioventricular (AV) node has not been previously described in children. Six children with atrial pacing-induced repetitive block are presented. The conduction patterns satisfy the requisites for alternating Wenckebach periodicity or multilevel AV block. In 2 patients the block is documented in the AV node and infra-His region. In 4 patients multilevel block within the AV node is postulated by deductive reasoning. In this study, 2 patterns of alternating Wenckebach periodicity are reported for the first time: sequences of 3:1 block with progressive prolongation of the conducted impulses terminating in 4:1 block; and sequences of 2:1 block with progressive prolongation of the conducted impulses terminating in 2 series of 3:1 block, in which the first conducted impulse following the first 2 blocked beats is not the shortest one, whereas that following the second 2 blocked beats is the shortest.

Wolff-Parkinson-White syndrome in the neonate.

Of 16 infants who presented with paroxysmal supraventricular tachycardia in the neonatal period, 50 percent had an electrocardiographic pattern consistent with Wolff-Parkinson-White conduction, type A. It is suggested that infants have bypass pathways similar to or identical with a Kent pathway as part of normal maturation. Infants with paroxysmal supraventricular tachycardia have electrically active bypass tracts but these are documented in only about one half of the patients because of the short duration of recordings or because of concealment (the bypass tract conducts only in retrograde fashion). The activity of these pathways is enhanced by the predominant cholinergic innervation of the neonatal heart. Resolution of the arrhythmias and the Wolff-Parkinson-White pattern in most patients occurs because of anatomic maturation of the conduction tissue, development of adrenergic innervation and a decrease in cholinergic dominance. In some children, maturation is incomplete and the bypass fibers remain quiescent or become active under certain circumstances such as those associated with increased autonomic discharge. Extended surveillance is recommended for all infants who present with paroxysmal supraventricular tachycardia and the Wolff-Parkinson-White pattern.

Application of the Rosenblueth hypothesis to assess atrioventricular nodal behavior.

Based on the Rosenblueth hypothesis, atrioventricular nodal behavior is evaluated using a method involving the coupling interval at the level of the atrioventricular nodal step delay. If the sum of the coupling interval at the level of step delay and the resultant step delay is greater than the atrial coupling interval, Wenckebach periodicity results; if the sum is less than the atrial coupling interval, the reverse Wenckebach phenomenon occurs; and if both are equal, steady state conduction is reached.

Application of the Rosenblueth hypothesis to assess cycle length effects on the refractoriness of the atrioventricular node.

In adults, the effective refractory period of the atrioventricular (AV) node is lengthened, whereas that of the atrium, His-Purkinje system or ventricular myocardium is shortened with a shorter atrial pacing cycle length. However, in children, the effective refractory period of the AV node at shorter cycle lengths is also shortened. Based on Rosenblueth's 1-step delay hypothesis, an index of refractoriness within the AV node is defined as the longest coupling interval at the level of step delay within the AV node of an impulse that cannot be conducted to the His bundle. The slopes relating cycle length and refractoriness of the AV node are determined by both the conventional and revised methods in 9 pediatric patients with heart disease. The slope is positive for all patients using the revised method. The difference in values between the 2 methods in older children is striking because the slope is converted from a negative to a positive value. It is concluded that the AV node has the same positive slope relating cycle length and refractoriness as other cardiac tissues.

Reverse alternating Wenckebach periodicity.

An atrial pacing-induced reverse conduction pattern of the alternating Wenckebach periodicity was observed in 5 of 42 children (12%) during electrophysiologic study. This conduction pattern is a reverse of the usual alternating Wenckebach periodicity: During an underlying 2:1 atrioventricular conduction block there is progressive shortening of the conduction time of the conducted impulses with termination in a lower degree of block. This reverse alternating Wenckebach periodicity may be caused by a mechanism similar to that in other Wenckebach phenomena.

Similar time-dependent recovery property of fast and slow atrioventricular nodal pathways.

The purpose of this study was to determine whether fast and slow atrioventricular (AV) nodal pathways have the same recovery property. AV nodal recovery property is studied by delivering atrial extrastimuli coupled to atrial beats and plotting nodal coupling intervals against nodal conduction time. In patients with dual pathways the resultant curves will include a fast to fast (F-F) and a fast to slow (F-S) pathway coupled curves. Although fast pathway recovery property can be represented by the former, slow pathway recovery property requires further assessment by studying slow to slow (S-S) pathways coupled curve. In 9 patients with dual pathways F-F, F-S, S-F, and S-S curves were obtained by pacing protocols. In 8 patients (control) without dual pathways, F-F curve and atrial extrastimuli coupled to a preceding slowly conducted fast pathway beat (also designated as S-F curve) were obtained. (1) The S-S curve had a similar time constant as the F-F curve. (2) Although the S-S curve was markedly shifted upward and leftward from the F-F curve, the degree of leftward and upward shifts of the S-S curve from the F-F curve were both close to the difference of the basic fast and slow pathway conduction time (a constant). (3) Although the effective refractory period of the fast pathway in dual pathway patients was longer than that of the control patients, the slow pathway effective refractory period when corrected was close to that of fast pathway in control patients. These results suggest that the fast and slow AV nodal pathways have a similar time-dependent recovery property.

Electrophysiologic cardiac function before and after surgery in children with atrioventricular canal.

Thirty-two children with atrioventricular (AV) canal underwent electrophysiologic studies: 18 underwent preoperative studies at a median age of 3 years (range 6 months to 16 years); 14 underwent postoperative studies at a median age of 4 years (range 2 to 19); and 2 underwent both preoperative and postoperative matched studies. In the preoperative group the following abnormalities were observed: first-degree AV block in 5 patients (due to internodal conduction delay in 1, AV nodal conduction delay in 2 and normal intracardiac intervals in 2); internodal conduction delay but normal PR interval in 4; and disease of the sinus node in only 1. In the postoperative group the following abnormalities were observed: first-degree AV block in 9 (due to AV nodal conduction delay in 2, His-Purkinje system conduction delay in 1, upper normal intracardiac intervals in 3 and unidentified in 3); prolongation of the right ventricular apical activation time in 11 of 13 with right bundle branch block; abnormal sinus node function in 3; and abnormal AV nodal function in 4 (1 of whom had associated sinus node disease). Atrial and ventricular functions were normal in all preoperative and postoperative patients. Electrophysiologic dysfunction is rare in preoperative patients with AV canal; in postoperative patients electrophysiologic abnormalities occur in 38% and involve the sinus and AV nodes in 19 and 25%, respectively.

Developmental changes of atrioventricular nodal recovery properties.

Atrioventricular (AV) nodal recovery properties can be studied by a periodic premature stimulation protocol performed at a slow basic rate. Developmental aspects of these properties have not been determined. The purpose of this study was to determine the developmental changes of AV nodal recovery properties. Forty-three children and young adults (male:female ratio 25:18) without AV nodal disease (aged 3.3 to 21.9 years) were studied by delivering premature atrial extrastimuli coupled to basic driven atrial beats. The individual recovery curve was fitted to the equation: A2H2 = A0H0 + exp(alpha -H1A2/tau) for H1A2 > or =theta, where A0H0 is the minimum AH interval, H1A2 is any recovery interval that exceeds the nodal effective refractory period, A2H2 is the corresponding nodal conduction time at any given H1A2, alpha is a constant, tau is the recovery time constant, and theta is the nodal effective refractory period. We found that: (1) A0H0 and alpha constant did not change significantly with age; (2) both tau (r = 0.324; p <0.05) and theta (r = 0.401; p <0.05) had a positive correlation with age; and (3) the maximum change in A2H2 with a 10-ms decrement in H1A2 was 32 ms and did not change significantly with age. Our results suggest that AV nodal recovery properties are age-dependent and both the recovery time constant and effective refractory period lengthen with age.

Site of conduction delay and electrophysiologic significance of first-degree atrioventricular block in children with heart disease.

Associated electrophysiologic abnormalities and site of delay were studied in 20 patients, aged 1.5 to 16.5 years, with congenital heart disease and first-degree atrioventricular (AV) block (PR interval above the 98th percentile for age and heart rate). Eight of the 20 patients with first-degree AV block were studied after 1 or more cardiovascular operations. Refractory periods of the atrium, AV node, His-Purkinje system and ventricle were determined. As a further test for AV nodal integrity, rapid atrial pacing was performed and the cycle at which Wenckebach periodicity occurred was noted. Four groups were identified. Group I included 4 patients (20%) with intraatrial conduction delay (long PA interval). Three patients had depressed sinus nodal function and 1 had depressed AV nodal function. Group II included 7 patients (35%) with AV nodal delay (long AH interval). One patient had sinus nodal depression and 2 had AV nodal depression (prolonged AV nodal refractory period or Wenckebach at a long paced cycle length). Group III included 3 patients (15%) with His-Purkinje delay (long HV interval). Measured functions were normal in all patients. Group IV included 6 patients (30%) with normal or high normal intracardiac intervals with long PR. One patient had sinus nodal dysfunction, 2 patients had long atrial refractory periods, 1 had AV nodal depression; 2 had long refractory period of the His-Purkinje system, and 1 had long ventricular refractory period. Atrial flutter was induced in 1 patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Dual atrioventricular nodal pathways: a benign finding in arrhythmia-free children with heart disease.

The incidence and significance of dual atrioventricular (A-V) nodal pathways are described in 78 children with associated congenital or acquired heart disease. None of these patients had clinical or electrocardiographic evidence of arrhythmia. Dual A-V nodal pathways were observed in 35 percent of the preoperative group and in 33 percent of the postoperative group. Despite this substrate for A-V nodal reentry, supraventricular tachycardia was neither induced during electrophysiologic evaluation nor did it develop clinically over a follow-up period of 1 month to 15 years. It is concluded that dual A-V nodal pathways are common and may be a benign finding in arrhythmia-free children with heart disease.

The fast-slow form of atrioventricular nodal reentrant tachycardia in children.

An unusual form of atrioventricular (A-V) nodal reentry is described as the underlying mechanism for incessant tachycardia in two children. During tachycardia a fast pathway was utilized for anterograde conduction and a slow pathway for retrograde conduction. This is the reverse of the usual form of A-V nodal reentrant tachycardia, in which the slow pathway is utilized for anterograde conduction and the fast pathway for retrograde conduction. One patient had a smooth ventriculoatrial (V-A) conduction curve demonstrating exclusive utilization of the slow pathway for retrograde conduction. The other had a discontinuous V-A conduction curve demonstrating failure of retrograde fast pathway conduction with resultant slow pathway conduction. In both cases the retrograde effective refractory period of the fast pathway was longer than that of the slow pathway, resulting in the establishment of this unusual reentry circuit. Both patients had a superior P axis with a P-R interval shorter than the R-P interval during tachycardia, features described in a significant number of children with incessant tachycardia. This unusual form of reentrant tachycardia can be suggested by its electrocardiographic pattern and is another mechanism for reentrant tachycardia not previously documented in children.

Hemodynamics and intracardiac conduction after operative repair of tetralogy of Fallot.

Electrophysiologic studies were performed in 47 children aged 3 to 18 years, 15 of whom had cardiac arrhythmias 1 to 15 years after repair of tetralogy of Fallot. Six exhibited sinus or atrioventricular nodal dysfunction, 8 had ventricular extrasystoles, and 1 had supraventricular tachycardia. Hemodynamic and electrophysiologic data were obtained at postoperative catheterization. Although electrophysiologic responses were abnormal in a proportion of both the children with and those without arrhythmia, hemodynamic values were similar. Three of 6 children with impaired sinus impulse generation or atrioventricular nodal conduction had a prolonged A-H interval, and in 3 Wenckebach heart block developed at low pacing rates. Ventricular ectopic rhythm was not associated with any particular abnormality of basic intracardiac conduction intervals. Thus, arrhythmias and conduction abnormalities are not consistently related to residual right ventricular hypertension. Abnormalities in electrophysiologic function are common after repair of tetralogy of Fallot in patients with sinus rhythm and may have prognostic implications for these patients.

Left isomerism and complete atrioventricular block: a report of six cases.

Six children, aged 12 days to 13 years, with left isomerism and complete atrioventricular (A-V) block are presented. In all six patients the diagnosis of left isomerism was suggested by an interrupted inferior vena cava found during cardiac catheterization and angiocardiography; four patients had complex heart disease consisting of endocardial cushion defect, five had a common atrium, three had pulmonary stenosis, three had patient ductus arteriosus and two had dextrocardia. Further anatomic abnormalities included situs inversus of the viscera (four patients) as well as partial malrotation of the bowel. Of the six patients, four had congenital complete A-V block, whereas the remaining two had A-V conduction disturbances documented during early infancy that progressed to complete A-V block later in life. All six patients required pacemaker implantation and five of the six patients died. This report discusses the clinical presentation of complete AV block and left isomerism and reviews the literature.

Ventricular arrhythmias in postoperative tetralogy of Fallot.

Ventricular arrhythmias in patients after total surgical repair of tetralogy of Fallot have been associated with late sudden death. In this large multicenter retrospective study of 359 patients with postoperative tetralogy of Fallot, spontaneous ventricular premature complexes (VPCs) on 24-hour ambulatory electrocardiographic monitoring and laboratory-induced ventricular tachycardia (VT) by electrophysiologic stimulation were analyzed. The mean age at surgical repair was 5 years and the mean follow-up duration after repair was 7 years. Spontaneous VPCs on ambulatory monitoring were found in 48% and induced VT on electrophysiologic stimulation was found in 17% of patients. Both spontaneous VPCs and induced VT were significantly related to delayed age at repair, longer follow-up interval, symptoms of syncope or presyncope and right ventricular systolic hypertension (greater than 60 mm Hg) (p less than 0.05), but not to right ventricular diastolic pressure greater than 8 mm Hg. The VPCs on ambulatory monitoring were more complex with increasing age at repair and follow-up duration. Induction of VT on electrophysiologic stimulation correlated with spontaneous VPCs including VT on 24-hour ambulatory electrocardiographic monitoring. The electrophysiologic stimulation protocol varied and the induction of VT increased with a more aggressive stimulation protocol. While induced sustained monomorphic VT was related to all forms of spontaneous VPCs, induced nonsustained polymorphic VT was related to more complex forms of VPCs on ambulatory monitoring. VT was not induced in asymptomatic patients who had normal 24-hour ambulatory electrocardiographic monitoring and normal right ventricular systolic pressure. (ABSTRACT TRUNCATED AT 250 WORDS)

Sodium channel abnormalities are infrequent in patients with long QT syndrome: identification of two novel SCN5A mutations.

Long QT syndrome (LQTS) is a heterogeneous disorder caused by mutations of at least five different loci. Three of these, LQT1, LQT2, and LQT5, encode potassium channel subunits. LQT3 encodes the cardiac-specific sodium channel, SCN5A. Previously reported LQTS-associated mutations of SCN5A include a recurring three amino acid deletion (DeltaKPQ1505-1507) in four different families, and four different missense mutations. We have examined the SCN5A gene in 88 index cases with LQTS, including four with Jervell and Lange-Nielsen syndrome and the remainder with Romano-Ward syndrome. Screening portions of DIII-DIV, where mutations have previously been found, showed that none of these patients has the three amino acid deletion, DeltaKPQ1505-1507, or the other four known mutations. We identified a novel missense mutation, T1645M, in the DIV; S4 voltage sensor immediately adjacent to the previously reported mutation R1644H. We also examined all of the additional pore-forming regions and voltage-sensing regions and discovered another novel mutation, T1304M, at the voltage-sensing region DIII; S4. Neither T1645M nor T1304M were seen in a panel of unaffected control individuals. Five of six T1304M gene carriers were symptomatic. In contrast to previous studies, QT(onset-c) was not a sensitive indicator of SCN5A-associated LQTS, at least in this family. These data suggest that mutations of SCN5A are responsible for only a small proportion of LQTS cases.

Sinus and atrioventricular nodal function: Preoperative and early postoperative assessment in children.

Ninety-three rapid atrial pacing studies were performed in 38 children to compare preoperative and early postoperative function of the sinus and atrioventricular (AV) nodes. The interval between the preoperative and postoperative studies was under 6 months in the majority of patients. Postoperative studies were performed within 48 hours of operation and between 4 and 8 days after operation. Sinus nodal function as measured by sinus nodal recovery time (SNRT) was an unreliable index in determining depression since the number who improved postoperatively (10/55) was nearly equal to the number that worsened (12/55). The majority who had abnormal function postoperatively demonstrated a junctional rather than sinus recovery focus. This finding appears a more definitive and more reproducible indicator of sinus node depression in the postoperative patient. Postoperative AV nodal function was decreased (as measured by the cycle length [CL] at which Wenckebach periodicity occurred) in 15 of 55 studies (27%) of the entire group. There was nearly an equal chance for improvement (24%) in function. This also applied to those patients who had sequential studies. Therefore, this method of assessment for AV nodal function was unreliable, or else the operation did not affect the node significantly. The latter is unlikely in view of late postoperative data. The greatest utility of this test was to determine the capability for AV conduction in certain patients with slow escape rhythms in the absence of surface P waves, and to differentiate complete heart block from AV dissociation when atrial activity was absent. Despite the variability of effects on the sinus and AV nodes in these patients, those who demonstrated depression had a significantly higher incidence of dysrhythmias (80% of patients with sinus nodal depression and 100% of patients with AV nodal depression).

Intraoperative identification of the conduction system in repair of endocardial cushion defect.

Electrophysiological identification intraoperatively of the His-Purkinje system eliminates the complication of complete heart block consequent upon repair of endocardial cushion defect (ECD). Ten patients, 6 with complete ECD and 4 with incomplete ECD, underwent repair of the defect. None of the patients developed heart block. Slight variations were noted in the location of the His bundle in 9 patients, and a major deviation was found in 1 patient with incomplete ECD. The data support the use of intraoperative recordings as a necessary acconpaniment to the operative repair of ECD.

Cardiologic assessment in participants of Pop Warner Junior League football.

Physical examinations and electrocardiograms were performed on 50 young men in preseason training for Pop Warner Football. Functional murmurs were found in 52%. The majority of electrocardiographic findings were within the accepted normal range for nonathletes but some were consistent with findings in trained athletes. Awareness of these normal variations is important for proper assessment of prospective and participating athletes.