The medical therapy of cardioinhibitory syncope in pediatric patients.

A small percentage of pediatric patients with neurally mediated syncope will have an asystolic response during upright tilt table testing. The purpose of this study is to evaluate the incidence of asystole during tilt table testing, and to assess the outcome of medical management of such patients. Of 398 patients undergoing evaluation for recurrent syncope between January 1989 and 1994, 18 (4.5%) experienced asystole lasting > or = 5 seconds during baseline tilt test. Patients had experienced a mean of four episodes of syncope, with a mean age at the time of tilt test of 11.1 +/- 4.0 years. The median duration of asystole was 10 seconds (range 5-40 s). Treatment was individualized to increased fluids and salt intake (3 patients), metoprolol (8 patients), pseudoephedrine (4 patients), disopyramide (1 patient), or combination therapy with fludrohydrocortisone (2 patients). During a median duration of follow-up of 31 months, no additional syncope was experienced by 78% of patients. Recurrent syncope in 4 patients was associated with either noncompliance or discontinuation of therapy in 3 patients; in 1 patient, increasing the dose of metoprolol was effective in preventing recurrences. We conclude that young patients with recurrent syncope and asystole during tilt test may be safely and effectively managed with pharmacological therapy, without resorting to pacemaker implantation.

Verapamil-induced "primary" polydipsia.

An 11-month-old male infant with recurrent supraventricular tachycardia (SVT) was treated with oral verapamil. Shortly thereafter he developed marked changes in behavior including lethargy, intensely increased thirst and urination, and irritability when denied fluids. "Primary" polydipsia was diagnosed following an evaluation which showed no evidence of adrenal insufficiency, diabetes insipidus, diabetes mellitus, hypercalcemia, hyperosmolality, or renal disease. The symptoms resolved 1 week after verapamil was discontinued.

Nephrocalcinosis and nephrolithiasis in infants with congestive heart failure treated with furosemide.

Nephrocalcinosis and nephrolithiasis developed in five children after furosemide therapy for congestive heart failure. In four children renal calcifications were detected by ultrasonography and in one by autopsy. Discontinuation of the loop diuretic in three children resulted in resolution of the calcifications in two of the patients. Residual renal morbidity included reduced creatinine clearance, microscopic hematuria, and hypercalciuria. The phenomenon of renal calcifications associated with furosemide treatment is more frequent than previously recognized.

Chest pain in otherwise healthy children and adolescents is frequently caused by exercise-induced asthma.

Chest pain in children and adolescents, unlike in adults, is rarely of cardiac origin and its etiology is frequently unknown. In this age group, chest pain can limit normal activity and sports participation. The reported incidence of exercise-induced asthma in children with chest pain is less than 20%. For this study, 88 otherwise healthy children and adolescents with chest pain followed a treadmill protocol without a warm-up period designed to obtain a target heart rate of 180 or greater during the first several minutes of exercise. Patients maintained this workload for 6 to 8 minutes. Pulmonary function tests performed prior to exercise and at 2, 5, 10, 15, 20, and 25 minutes revealed a decrease in forced expiratory volume in 1 second or peak expiratory flow rate of greater than or equal to 15% in 64 (72.7%) children. Inhaled albuterol resulted in subjective improvement in 97% (35/36) and objective improvement in 70% (25/36) of patients. In otherwise healthy children and adolescents with chest pain, the incidence of exercise-induced asthma seems greater than previously reported. Treatment with bronchodilators may help these patients lead a more active life-style.

A clinical study of the dynamics of parasystole.

A mathematical model for pure parasystole and modulated parasystole leads to a number of quantitative predictions. The predictive power of the model is examined by confronting it with data obtained from a 16-year-old symptomatic male born with a ventricular septal defect that was surgically closed at 5 years of age. A diagnosis of ventricular parasystole and inducible ventricular tachycardia was made following a syncopal episode. The physiological variables required by the model to make specific predictions are the sinus and ectopic cycle lengths and the ventricular refractory period. From these three variables, a two-dimensional parameter space is constructed consisting of the ratio of the refractory period to the sinus cycle length and the ratio of the ectopic to sinus cycle length. For any set of parameters, predictions are made concerning the number of sinus beats between ectopic beats. The different behaviors exhibited in the electrocardiographic (ECG) data agree with theoretical predictions.

Ventricular rhythm with intermediate rate in the neonate without heart disease.

Nonsustained, sustained, or incessant AVR, a form of VRIR, although infrequent, can present in the neonatal period. It must be considered in the differential diagnosis of wide QRS tachycardia. It seems to be different from VI, and it is asymptomatic and self-limited. It has good long-term prognosis and requires no treatment. These patients should not undergo invasive diagnostic evaluation unless symptoms develop.

Bradycardia with traumatic hyphema in children.

Eleven patients, aged 4 to 18 years, who were admitted to the hospital with the diagnosis of traumatic hyphema were placed on 24-hour cardiac (Holter) monitors. Ten of the 11 patients had episodes of bradycardia and bradyarrhythmias. Blood pressures monitored during the same period showed that the bradycardia was not hemodynamically significant. Cardiac monitoring was repeated on six patients 3 months later, and nocturnal cardiac rhythms were compared; the minimum heart rates immediately following the eye injury were significantly lower (p = .03) than those measured 3 months later. While these data do not establish a relationship between bradyarrhythmias and the frequently-observed occurrence of somnolence, it does appear that episodes of bradyarrhythmias represent a common physiologic response to traumatic hyphema, and they can be expected to resolve spontaneously.

Permanent cardiac pacemaker implant in the fetal lamb.

To investigate the feasibility of permanent cardiac pacing in the fetal lamb, we attempted five implants in lambs between 100 and 115 days of gestation. The lambs were approached by a left thoracotomy done through a transverse hysterotomy in the ewe. An epicardial lead was fixed to the left ventricle. Pacing parameters were measured and the lead was connected to an Enertrax implantable pulse generator. A pouch was created for the generator deep to the latissimus dorsi. Documentation of pacing was obtained electrocardiographically; pacing faster than the fetal intrinsic rate. The muscle and the fetal skin were closed. The pacemaker was programmed to a rate of 70 in the ventricular inhibited (VVI) mode and the uterine and abdominal wall incisions were then closed. One lamb was successfully delivered by Cesarean section at term. Pacing parameters were measured at birth and one month later. There were small changes in the pacing lead function parameters as the implant progressed into the chronic phase. They were within the capacity of the generator to pace with a margin of safety. Four lambs died in utero and were delivered 5 to 26 days post-implant. We conclude that permanent cardiac pacemaker implantation is feasible in the fetal lamb and with further development, it may eventually be used in humans in the treatment of fetal congestive heart failure due to bradyarrhythmias.

Electrophysiologic drug testing in symptomatic ventricular arrhythmias after repair of tetralogy of Fallot.

Nine patients with symptomatic ventricular arrhythmias were evaluated a mean interval of 16 years after surgical repair of tetralogy of Fallot. The clinical arrhythmia was sustained ventricular tachycardia (VT) in 4 patients (group I) and premature ventricular contractions in 5 (group II). All patients underwent cardiac catheterization and electrophysiologic studies. Ventricular tachycardia was induced at electrophysiologic study in all patients in group I and in 3 patients in group II. Six patients with inducible sustained monomorphic VT underwent chronic drug testing based on electrophysiologic study. A mean of 3.3 drugs per patient was tested. Patients with right ventricular systolic hypertension did not respond to any drug tested, and underwent surgery. Five patients received drug treatment based on the results of electrophysiologic study. During a mean follow-up period of 2.2 years, no patient in either group had recurrent episodes of VT or syncope. In the postoperative patient with tetralogy of Fallot with symptomatic ventricular arrhythmias, it is concluded that electrophysiologic study is useful in reproducing clinical episodes of VT and in selecting effective antiarrhythmic medication; a small number of patients with ventricular premature complexes alone will have inducible sustained VT during electrophysiologic study; prognosis of these patients may be improved by treatment that results in prevention of VT induction; and in patients with right ventricular hypertension, VT is likely to be refractory to drug treatment.

Arrhythmias in the tiny, premature infant.

In contrast to the older infant or child in whom arrhythmias are usually secondary to congenital heart disease, arrhythmias in the small, preterm infant are most commonly secondary to central nervous system immaturity or extrinsic causes. Bradycardia is the most common arrhythmia observed in this population. The staff involved in the care of these extremely fragile and usually complicated patients have to be aware of the possibility of other rhythm disturbances occurring in such patients; this will help recognition and appropriate management of the individual cases.

Ventricular tachycardia in a young population without overt heart disease.

Since 1974, 24 young patients presenting with ventricular tachycardia and without clinical evidence of heart disease were evaluated and followed. Sixteen patients (67%) were symptomatic. Clinical episodes of ventricular tachycardia were sustained in 18, incessant in four, and nonsustained in two patients. The rate of tachycardia ranged from 130 to 300 beats/min (mean = 200 beats/min). Subtle abnormalities of cardiac size or function were present at cardiac catheterization in 16 of 23 patients (70%). During electrophysiologic studies, spontaneous ventricular tachycardia was present in six patients. The clinical ventricular tachycardia was inducible by programmed stimulation in 13 of 18 patients. The site of origin of tachycardia based on endocardial mapping in 17 patients was the right ventricle in 14, the ventricular septum in one, and indeterminate in two patients. Seventeen patients were treated based on results of short-term drug testing. During a mean follow-up period of 7.5 years, three patients died suddenly; none of these patients were receiving antiarrhythmic medication at the time of death. We conclude that in a young population without clinical evidence of heart disease, ventricular tachycardia may be the first manifestation of cardiomyopathy, since at least two-thirds of these patients have abnormalities at cardiac catheterization. Without treatment mortality in this population may be as high as 13% over an 8 year period. Presently we recommend treatment of ventricular tachycardia in any symptomatic patient, with therapy guided by electrophysiologic and treadmill testing. In addition, we recommend treatment for any asymptomatic patient with exercise-related tachycardia, since this group appears to be at increased risk for sudden death.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital aneurysm of the left sinus of Valsalva with an aortopulmonary tunnel.

Aneurysm of the left sinus of Valsalva is rare, and there is only one previous report of rupture into the pulmonary artery. This report describes a patient with valvular pulmonary atresia and ventricular septal defect in whom a portion of his pulmonary blood flow was supplied by an aortopulmonary tunnel arising from a left sinus of Valsalva aneurysm. The surgical implications of precise definition of the type of aortopulmonary communication are discussed.

Pharmacological observations in patients with nodoventricular pathways.

Three patients with accessory nodoventricular pathways and re-entry tachycardia are reported. In all three patients the accessory nodoventricular pathway formed the anterograde limb of the re-entry circuit while the His-Purkinje-atrioventricular node axis formed the retrograde limb of the tachycardia in two of the patients and a concealed accessory pathway formed the retrograde limb in the remaining patient. All three patients also manifested dual anterograde atrioventricular nodal pathways with conduction through the accessory nodoventricular pathways being associated with the atrioventricular nodal fast pathway. Type I antiarrhythmic drugs, especially disopyramide and quinidine, were effective for the treatment of the re-entry tachycardia because of their depressive action on the nodoventricular pathway. Beta blockers were also effective because of their action on the atrioventricular nodal portion of the re-entry circuit in one patient and most probably due to atypical (atrioventricular nodal like) properties of a retrogradely conducting accessory pathway in a second patient.

Inducible polymorphous ventricular tachycardia following Mustard operation for transposition of the great arteries.

A 22-year-old woman with chronic atrial tachycardia following Mustard's operation for transposition of the great arteries presented with dizziness and ventricular tachycardia documented with dynamic 24-h electrocardiogram. During intracardiac electrophysiology study, programmed ventricular extrastimulation induced polymorphous ventricular tachycardia (torsades de pointes). This was prevented by intravenous administration of procainamide. We postulate that polymorphous ventricular tachycardia is a possible cause of death in patients with Mustard's operation. Postoperative electrophysiologic study may define those patients at risk to develop this potentially fatal arrhythmia.

Efficacy of pulmonary artery banding in infants with complete atrioventricular canal.

Total correction in infants less than 1 year old with complete atrioventricular (AV) canal carries a significant operative mortality. However, past reports suggest that the alternative palliative procedure, pulmonary artery banding (PAB), may be contraindicated in the presence of severe mitral insufficiency and/or a large left ventricular to right atrial shunt. Contrary to these previous reports, we report the results in 21 consecutive patients with congestive heart failure who underwent PAB at a mean age of 3.9 +/- 2.8 months and at a weight of 3.6 +/- 0.9 kg. (17/21 less than 6 months). Regardless of mitral valve competency, PAB was performed in conjunction with ligation of a patent ductus arteriosus (11 patients) and coarctation repair (two patients) with one death secondary to gastrointestinal bleeding (4.7% in-hospital mortality); one patient required early band readjustment because of hypoxemia. Repeat cardiac catheterization in 10 patients performed 4 to 41 months after PAB showed significant reduction in pulmonary hypertension and flow with no change in pulmonary vascular resistance. All infants were symptomatically improved after PAB and four have undergone successful total correction. Previous reports since 1977 indicate a significantly higher risk for total repair of complete A V canal before 1 year of age (36/147, 24%) than the risk for PAB in this series (p less than .05). Therefore, we believe that PAB is a rational alternative to total repair as the initial surgical treatment for symptomatic infants with complete A V canal, particularly when anatomic variants known to increase operative risk are recognized before cardiotomy.

Electrophysiologic testing of bretylium tosylate in sustained ventricular tachycardia.

We used programmed ventricular stimulation to test intravenous bretylium tosylate in 10 consecutive patients with inducible sustained ventricular tachycardia (usually refractory to type I antiarrhythmic agents). These 10 patients had previously documented sustained ventricular tachycardia and/or ventricular fibrillation complicating stable heart disease. Following control inductions of sustained ventricular tachycardia, bretylium 10 mg/kg was infused over 30 minutes. Thirty minutes after this infusion, sustained ventricular tachycardia could be induced in 9 of the 10 patients (one of these nine patients also had bretylium-potentiated spontaneous ventricular tachycardia). Tachycardia induced in the nine patients after bretylium was similar to control tachycardia with respect to morphology and cycle length (333 +/- 16 msec after bretylium versus 330 +/- 16 msec during control). However, five of the nine patients tolerated induced tachycardia less well after bretylium (exacerbated hypotension). In one patient, ventricular tachycardia could not be induced after intravenous bretylium.

Electrophysiologic drug testing in prophylaxis of sporadic paroxysmal atrial fibrillation: technique, application, and efficacy in severely symptomatic preexcitation patients.

Electrophysiologic drug testing was performed in nine patients with severely symptomatic sporadic (2 to 13 [mean 4.2] attacks/24 months) paroxysmal atrial fibrillation (PAF). All patients had control inductions of sustained (greater than 30 seconds) AF by high right atrial stimulation, and attempted inductions following serial administration of drugs. Drugs tested were intravenous procainamide (1.0 to 1.5 gm) (five patients), intravenous propranolol (0.1 mg/kg) (three patients), oral quinidine (1.6 to 2.4 gm/day) six patients), oral disopyramide (1.2 to 1.6 gm/day) (four patients), and oral aprindine (100 to 250 mg/day) (four patients). In all patients, one or more drugs prevented induction of sustained AF: procainamide (one patient), quinidine (five patients), disopyramide (four patients), and aprindine (four patients). All patients were treated with drugs which prevented induction of sustained AF and followed for 8 to 40 (mean 24) months. Seven patients tolerated their drugs: six had no AF and one had several short nonsustained attacks. Two patients did not tolerate their drugs: one had paroxysmal palpitation (on decreased aprindine dosage), and one had AF (while off of aprindine). In conclusion, electrophysiologic drug testing is feasible in patients with sporadic PAF. Inability to induce sustained AF following drug administration suggests successful prophylaxis of spontaneous PAF with the same drug.