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.

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)

Anomalous origin of the left anterior descending coronary artery from the pulmonary artery.

Three children were identified as having anomalous origin of the left anterior descending coronary artery (LAD) from the pulmonary artery (PA). Two had had congestive heart failure in infancy with clinical diagnosis of endocardial fibroelastosis and all had abnormal ECGs. The correct diagnosis was delayed in each case, and two patients required selective coronary angiography. Surgery was accomplished in the three children although ECG abnormalities have persisted and one child has dyskinesis of the left ventricular apex. Because this diagnosis may be difficult to make when intercoronary anastomoses are inadequate to outline the left anterior descending coronary flow into the PA, patients with clinical findings suggestive of anomalous coronary artery may require selective coronary studies to exclude this anomaly.

M-mode echocardiographic findings in children with idiopathic restrictive cardiomyopathy.

The M-mode echocardiographic findings in five pediatric patients, ages 4-15 years, with primary idiopathic restrictive cardiomyopathy, diagnosed by cardiac catheterization, and of 12 normal children (control group) are presented. The M-mode echocardiographic findings in patients with restrictive cardiomyopathy were (1) normal left and right ventricular end-diastolic dimension, (2) normal left ventricular posterior wall and interventricular septal thickness (three patients) or mild concentric hypertrophy (two patients), (3) normal opening and closing velocity of the mitral valve, (4) consistently enlarged left atrium (more than 40 mm) in all, and (5) right ventricular systolic time intervals compatible with pulmonary artery hypertension. The left ventricular ejection phase parameters (systolic time intervals, shortening fraction, and mean velocity of circumferential fiber shortening) were normal. Left ventricular relaxation phase parameters (diastolic function) were abnormal. The isovolumic relaxation time index was prolonged, 68 +/- 40 ms (+/- SD), in the study group as compared with 11 +/- 6 ms (+/- SD) in the control group (P less than 0.001). Percent relaxation of left ventricular posterior wall endocardium at 50% of diastole was decreased, 58 +/- 4% (+/- SD), in the study group as compared with 85 +/- 6% (+/- SD) in the control group (P less than 0.005). We conclude that M-mode echocardiography provides a relatively useful and specific noninvasive method for the diagnosis of primary restrictive cardiomyopathy in pediatric patients.

Single coronary artery originating from the right pulmonary artery.

An infant with a single coronary artery originating from the right pulmonary artery branch is the subject of this report. He survived to the age of one month possibly because of the association of two ventricular septal defects. While the pulmonary vascular resistance remained high, perfusion of the heart muscle was accomplished due to the pulmonary hypertension. The electrocardiogram did not show typical signs of infarction, but poor left ventricular contractility was observed by echocardiogram. The diagnosis was made postmortem. This specific coronary malformation has not been previously described and needs to be included in the classification of congenital coronary arteries anomalies.

A method of maintaining thermal homeostasis during cardiac catheterization in newborns.

This device has been designed to maintain thermal homeostasis in newborns undergoing cardiac catheterization. It is easily constructed from materials available in any hospital, and it is safe and effective. It consists of a warming coil and a delivery unit made from Plexiglas which encloses the patient. In addition to maintaining a neutral thermal environment, it allows for the selection of any desired oxygen concentration by simply blending the gas mixture before warming and delivery. Since using this device we have had no problems or complications related to thermal stress during catheterization of the neonate.

Arrival of excitation at the left ventricular apical endocardium in Wolff-Parkinson-White syndrome type B.

Electrograms were recorded from the His bundle area, right ventricular apex, right ventricular inflow tract, and left ventricular apical endocardium in four patients (aged, 1, 1, 1.5, and 16 years) with Wolff-Parkinson-White syndrome type B. In beats without preexcitation: (a) delayed activation of the right ventricular inflow tract reflected the occurrence of a conduction disturbance through the "distal" or "peripheral" ramifications of the right bundle branch; and (b) the slightly earlier activation of the left ventricular apical endocardium (in reference to the right ventricular apex) may have been due to an earlier emergence from the divisions of the left bundle branch, presumably due to the greater length of the right bundle branch. In beats with preexcitation: (a) the "incomplete" right bundle branch block pattern was concealed because the right ventricular inflow tract was activated before the right ventricular apex; (b) the delta-right ventricular apical intervals were shorter than those of adults with Wolff-Parkinson-White type B; and (c) arrival of excitation at the left ventricular endocardium was a function, either of the impulse emerging from the left bundle branch, or of that propagating from the preexcited site. Therefore, the delta-left ventricular apical endocardial intervals were considered to have represented conduction time from preexcited region to endocardium of left ventricle only when it could be proven that the conduction time (from atrial site of origin to left ventricular apical endocardium) was shorter through the right sided accessory pathway than through the normal pathway. This study suggests that some beats, which may be interpreted as representing "pure" Wolff-Parkinson-White type B complexes from epicardial maps, may in reality be "fusion" complexes.

Right ventricular apical activation time in children. Reference standards for clinical use.

To differentiate proximal from peripheral right bundle-branch block, several investigators have used the right ventricular apical (RVA) activation time, but there is a lack of reference standards for infants and other children. Using intracardiac electrography, His bundle and RVA electrograms were recorded in 123 children before surgery for various types of congenital cardiac malformations. None had evidence of conduction defects on their surface ECG. The average RVA activation time was 15 +/- 7 msec (+/- SD) linearly increasing with age from infancy to adolescence. The values found in this large population may be useful as reference standards for right bundle-branch conduction times in other infants and children.

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.

Congenital systemic arteriovenous fistula between the descending aorta, azygos vein, and superior vena cava.

Various congenital systemic arteriovenous fistulas have been described. An unusual case of congenital systemic arteriovenous fistula arising from the descending aorta and draining separately into the superior vena cava, the azygos vein, and the innominate vein is reported. Clinically, this case simulated a patent ductus arteriosus. To our knowledge, this particular anomaly has not been described before.

Determinants of ventricular refractory periods in children with congenital heart disease: effects of cycle length and age.

Ventricular effective refractory periods (ERP) and functional refractory periods (FRP) were determined by programmed ventricular extrastimulation in 53 pediatric patients with a spectrum of congenital heart disorders. Of these 63 children (ages 8 months to 18 years), 38 were preoperative, 17 had repair of their cardiac lesion via right ventriculotomy, and eight were postoperative without a ventriculotomy. We demonstrated that there was a linear relationship between the cycle length and ventricular refractory periods. The regression equations 73 + 0.29 x cycle length (msec) for the ventricular ERP (msec) and 80 + 0.30 x cycle length (msec) for ventricular FRP (msec) were found to define the determined properties of ventricular refractory periods (VRP) in children. These VRP characteristics were independent of age in children less than 13 years of age.

His-Purkinje responses and refractory periods during atrial extrastimulation in children with heart defects.

During atrial extrastimulation, split His potentials, prolonged His-to-ventricular (H2V2) intervals and block distal to the His bundle deflection were observed in both preoperative and postoperative children with heart defects. His-Purkinje responses and refractoriness were identified in 31 of 78 (40%) pediatric patients (20 of 51 preoperative and 11 of 27 postoperative) during atrial extrastimulation coupled to sinus and/or paced cycle lengths. Split His potentials were found in 14 patients (eight preoperative and six postoperative) and His bundle relative refractory periods ranged from 250--490 msec. Prolonged H2V2 intervals were found in these and in an additional 16 patients (11 preoperative and five postoperative) and the relative refractory period of the His-Purkinje system ranged from 230--500 msec. Block distal to the His deflection occurred in seven patients (five preoperative and two postoperative) and the effective refractory period ranged from 230--510 msec. Split His potentials, long H2V2 intervals and block distal to the His bundle deflection produced by atrial extrastimulation were found in peroperative as well as postoperative children. These responses probably represent functional electrophysiologic characteristics of the pediatric cardiac conduction system.

Echocardiographic assessment of right bundle branch injury after repair of tetralogy of Fallot.

Nineteen patients, ages 3 1/2-18 years, with electrocardiographic evidence of right bundle branch injury after intracardiac repair of tetralogy of Fallot, underwent invasive intracardiac electrophysiologic evaluation 1-13 years (mean 4.4 years) postoperatively. Categorization of the site of right bundle branch injury as proximal or distal was made by determining the V-RVA interval. In 11 of the patients, the V RVA interval was prolonged (> 35 msec), indicating proximal right bundle branch injury and in the other eight it was normal (< 35 msec), indicating distal bundle branch injury. Within 24 hours of the study, all patients were studied by M-mode echocardiography. Measurements were made of the tricuspid valve closure, mitral valve closure and the difference between the two, or the delta value. All but one patient with distal bundle branch injury had delta values of less than 40 msec (range 8-38 msec), while 10 or 11 patients with proximal bundle branch injury had delta values greater than 40 msec (range 41-116 msec). there was a significant positive correlation (r = 0.74, p < 0.001) between V-RVA and the delta value. We conclude that the delta value is an indicator of relative activation delay of the right ventricle, and therefore, in most cases, proximal vs distal bundle branch injury can be diagnosed noninvasively.

Age-related differences in the protein binding of quinidine.

The in vitro serum protein binding of quinidine was determined in 26 pediatric patients. Group I consisted of 6 cord blood samples obtained at the time of delivery, group II of 8 infants aged 8-18 months, and group III of 12 children over the age of 2 years. The percentage of free quinidine in group I was 39.2 +/- 10.8, group II 24.4 +/- 10.6 and group III 16.6 +/- 6.5, demonstrating a larger proportion of unbound quinidine in the newborn group and an increase in protein binding with age (p less than 0.0001). Therefore, the protein binding of quinidine is diminished in the neonate and young infant and may result in enhanced quinidine activity.

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.

Congenital bilateral subclavian steal: ductus-dependent symptoms in interrupted aortic arch associated with ventricular septal defect.

There are two types of congenital bilateral subclavian steal. The first type is accompanied by severe coarctation or interruption of the aorta in which both the right and left subclavian arteries have their origin distal to the obstruction in the aorta. In most cases with complete interruption of the aorta there is a large ventricular septal defect. The second, less common type of steal is associated with isolated atresia of the proximal segment of the subclavian arteries. In the first type of steal, the anatomic predisposition for the steal is present from birth but clinical symptoms frequently do not appear until childhood or adolescence. In the newborn the presence of a widely patent duclus arteriosus and its capacity to transmit the flow and pressure from a hypertensive pulmonary artery to the descending aorta and thus to the subclavian arteries prevents cerebral symptoms and angiographic signs, but in the older infant and child, the internal diameter of the ductus has usually decreased, leading to symptoms of the subclavian steal syndrome.

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.