Seshadri Balaji

Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.

OBJECTIVEnWe report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing.nnnBACKGROUNDnIsolated CHB has an excellent prognosis following pacemaker implantation. Most early deaths result from delayed initiation of pacing therapy or hemodynamic abnormalities associated with congenital heart defects.nnnMETHODSnA multi-institutional study was performed to identify common clinical features and possible risk factors associated with late-onset dilated cardiomyopathy in patients born with congenital CHB.nnnRESULTSnCongenital heart block was diagnosed in utero in 12 patients and at birth in four patients. Ten of 16 patients had serologic findings consistent with neonatal lupus syndrome (NLS). A pericardial effusion was evident on fetal ultrasound in six patients. In utero determination of left ventricular (LV) function was normal in all. Following birth, one infant exhibited a rash consistent with NLS and two had elevated hepatic transaminases and transient thrombocytopenia. In the early postnatal period, LV function was normal in 15 patients (shortening fraction [SF] = 34 +/- 7%) and was decreased in one (SF = 20%). A cardiac pacemaker was implanted during the first two weeks of life in 15 patients and at seven months in one patient. Left ventricular function significantly decreased during follow-up (14 days to 9.3 years, SF = 9% +/- 5%). Twelve of 16 patients developed congestive heart failure before age 24 months. Myocardial biopsy revealed hypertrophy in 11 patients, interstitial fibrosis in 11 patients, and myocyte degeneration in two patients. Clinical status during follow-up was guarded: four patients died from congestive heart failure; seven required cardiac transplantation; one was awaiting cardiac transplantation; and four exhibited recovery of SF (31 +/- 2%).nnnCONCLUSIONSnDespite early institution of cardiac pacing, some infants with CHB develop LV cardiomyopathy. Patients with CHB require close follow-up not only of their cardiac rate and rhythm, but also ventricular function.

Management of atrial flutter after the Fontan procedure.

OBJECTIVESnThe purpose of this study was to review the management of atrial flutter occurring after the Fontan procedure.nnnBACKGROUNDnAtrial flutter occurs frequently after the Fontan procedure and is often hemodynamically poorly tolerated.nnnMETHODSnThe patients charts were reviewed for relevant information.nnnRESULTSnBetween 1984 and 1992, 18 patients had atrial flutter after the Fontan procedure. The underlying heart defect was tricuspid atresia in nine, mitral atresia in six and double inlet left ventricle in three. All but three patients had undergone previous palliative surgery. The time interval from Fontan operation to atrial flutter was < 1 day to 16 years (mean 3.7 years). Seven had early atrial flutter before leaving the hospital. Electrophysiologic study in 15 showed sinus node dysfunction in 12. Atrial flutter was inducible in all patients, and 13 had > 1 flutter configuration. Digoxin and a variety of other antiarrhythmic agents (mean 2.7 drugs/patient) were tried with poor results. Only digoxin, amiodarone, flecainide and propafenone showed some benefit when used alone or in combination. Antitachycardia pacemakers were implanted in 16 patients (endocardial 14, epicardial 2) and, with drugs, were useful in 8 (50%). Because atrial flutter was resistant to treatment, right atriectomy was performed in three patients (with benefit in two, one death), successful radiofrequency catheter His bundle ablation in one patient and catheter ablation of atrial flutter in three patients (two failed, one partial success). One patient underwent heart transplantation, and two died suddenly. Another died of complications after an elective epicardial pacemaker replacement procedure.nnnCONCLUSIONSnAtrial flutter after the Fontan procedure is difficult to control. Aggressive drug and antitachycardia pacemaker therapy help about half of the patients. When these measures fail, other options, such as atriectomy, His bundle ablation or catheter ablation of atrial flutter, need consideration. The risk of sudden death justifies the use of such aggressive treatment methods.

QRS prolongation is associated with inducible ventricular tachycardia after repair of tetralogy of Fallot

Prolonged QRS duration on the electrocardiogram has been found to predict adverse arrhythmic events in patients late after repair of tetralogy of Fallot. Whether QRS duration can also predict inducible ventricular tachycardia (VT) at electrophysiologic study is unknown. Between 1984 and 1995 we studied 135 survivors of tetralogy of Fallot surgery whose age at surgery was 34 days to 37 years (3.7 +/- 3.9, median 2.5) and age at electrophysiologic study was 1.4 to 43 years (9.7 +/- 8.2, median 6.7). QRS duration was 80 to 240 ms (137 +/- 29) and > or = 180 ms in 9 patients. Sustained VT was induced in 22 patients (monomorphic in 17). Induced sustained monomorphic VT was related to QRS duration, right ventricular dimension, H-V interval, and presence of symptoms. QRS duration was also related to induced sustained monomorphic VT by multivariate analysis. QRS duration > or = 180 ms was 35% sensitive and 97% specific for induced sustained monomorphic VT. QRS duration was related to induced sustained monomorphic VT even when only asymptomatic patients were analyzed. A QRS duration > or = 180 ms was 100% sensitive and 96% specific for detecting clinical VT. Prolonged QRS duration on the electrocardiogram is associated with induced sustained monomorphic VT on electrophysiologic study. The finding of prolonged QRS duration should suggest the need for further testing to determine the risk of adverse arrhythmic events in patients after repair of tetralogy of Fallot, even if they are asymptomatic.

Neurocardiogenic syncope in children with a normal heart

OBJECTIVESnThe purpose of this study was to review the results of investigation and management of children with syncope and a structurally normal heart.nnnBACKGROUNDnSyncope is a common clinical problem and has many etiologies. Autonomic testing and, in particular, the tilt/orthostatic test have helped to positively diagnose neurocardiogenic syncope in a high proportion of such patients.nnnMETHODSnPatient case notes and autonomic test charts were reviewed in 162 children aged 1 to 20 years (mean age 12.8 years) with syncope. The autonomic test consisted of orthostatic maneuver, carotid sinus massage, diving reflex, Valsalva maneuver and dose response to intravenous boluses of isoproterenol and phenylephrine. Serum levels of epinephrine and norepinephrine were drawn during the orthostatic test. After confirmation of neurocardiogenic syncope, treatment was begun with fludrocortisone and salt, and beta-adrenergic blocking agents were used as a second line of therapy when indicated.nnnRESULTSnThe orthostatic test was positive for neurocardiogenic syncope in 100 patients (62%) and negative in 62 (38%). Patients in the former group were older, were more often female and had a diminished response to carotid sinus massage, a higher Valsalva ratio and a higher supine epinephrine level. Both groups showed an increase in epinephrine and norepinephrine levels at 5 min of standing. In the orthostatic positive group at the time of syncope, norepinephrine levels decreased, whereas epinephrine levels increased. Patients in this group were also more sensitive to the vasodilating effect of isoproterenol but not to its chronotropic effects. Eleven patients had cardioinhibitory syncope (asystole > or = 3 s). Of these, three had pacemaker implantation. Fludrocortisone and salt used in 84 patients in the orthostatic positive group produced resolution of symptoms in 55 patients (65%) and improvement in 14 (17%). Ten patients received beta-blockers, with resolution in four and improvement in four.nnnCONCLUSIONSnPatients with orthostatic test-proved neurocardiogenic syncope show evidence of autonomic dysfunction. They also show beta-adrenergic hypersensitivity. Treatment initiated on the basis of the protocol was associated with amelioration of symptoms in the majority of patients.

Immunosuppressive treatment for myocarditis and borderline myocarditis in children with ventricular ectopic rhythm.

OBJECTIVES--To ascertain the responsiveness to immunosuppressive treatment of myocarditis and borderline myocarditis in children with ventricular ectopic rhythm (that is, all ventricular arrhythmia except benign premature ventricular contractions). To determine the impact of the diagnostic information provided by an endomyocardial biopsy specimen in these patients. BACKGROUND--The therapeutic value of performing an endomyocardial biopsy in children with ventricular ectopic rhythm is not established. In turn, the treatment of myocarditis with immunosuppressive drugs is also controversial. METHODS--The case notes and endomyocardial biopsy findings of all children with ventricular ectopic rhythm and a biopsy diagnosis of myocarditis were reviewed. RESULTS--Ten (14%) of 69 patients with ventricular ectopic rhythm and an anatomically normal heart had histological evidence of myocarditis or borderline myocarditis. Eight patients received corticosteroids and efficacy was judged by regular 24 hour Holter monitoring. Total resolution of arrhythmia was seen in four, improvement in two, and no change in two patients. At follow up (8-39 months, mean 22 months), arrhythmia recurrence was seen in the two patients who showed an improvement but not resolution during treatment. Both received azathioprine with further reduction in ectopy rates. Patients who responded to treatment were symptomatic (six of six patients) at presentation compared with those who did not respond to treatment (none of two patients) who were not symptomatic. Five patients had a repeat biopsy specimen taken which confirmed histological improvement. CONCLUSIONS--Steroid treatment seems to benefit a subset of children with ventricular ectopic rhythm and a biopsy diagnosis of myocarditis or borderline myocarditis. Because it can identify a treatable cause for the ventricular arrhythmia, endomyocardial biopsy is a valuable investigation in these patients.

Radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in children.

Radiofrequency (RF) catheter ablation has been widely used in the treatment of cardiac arrhythmias. In atrioventricular nodal reentrant tachycardia (AVNRT), the experience has been predominantly in adults. The cardiac electrophysiological records of 18 consecutive children undergoing RF catheter AV node modification for AVNRT were reviewed. The patients (10 females, 8 males) were 8.2–17.9 years of age (mean 13.6 ± 3.0), weight 15.2–88.1 kg (mean 52.2 ± 20.8), and height 103–190 cm (mean 157.1 ± 21.7). Thirteen were on antiarrhythmic medications (1–3, average 1.5 drugs/day). All drugs were discontinued 48 hours prior to the ablations. The procedures were performed under sedation and local anesthesia. Pre‐ and post‐AV node modification electrophysiological studies were performed in all procedures. The 18 patients underwent a total of 25 procedures (1.39 ± 0.61 per patient): the anterior approach aimed at the antegrade fast pathway in the first four patients and the posterior approach aimed at the slow pathway in the remainder. Thenumber of energy applications was 8–54 (19.8 ± 10.7) per procedure. The maximum energy used in each procedure was 30–50 watts (33.8 ± 8.4). The average energy was 24–50 watts (33.0 ± 6.8). The fluoroscopy time was 7.1–73.4 minutes (29.9 ± 20.0) per procedure, for a total catheterization time of 228–480 minutes (300.3 ± 59.1). Preablation spontaneous or induced AVNRT (cycle length 310.4 ± 55.0 msec) was seen in all except one who had the arrhythmia (cycle length 270 msec) on surface ECG. In 22 of 25 studies, the AH interval measured 67.4 ± 13.2 msec pre‐ and 98.7 ± 58.4 msec post‐AV node modification (P < 0.02). Procedures were initially successful in 16 (89%) of 18 patients. One patient developed complete AV block requiring DDD pacemaker and has since recovered normal AV conduction. Transient third‐ or second‐degree block was seen in four. Other complications included airway obstruction in one and excessive emesis in another. In follow‐up of 2–26 months (13.0 ± 7.3), one patient underwent surgical ablation for failed initial RF catheter ablation, and two underwent successful RF procedures for recurrences. RF catheter AV node modification for AVNRT in children is a useful technique. Under ideal circumstances, it is safe and efficacious. Follow‐up to determine the potential long‐term complications is necessary.

Effect of heart rate on QT interval in children and adolescents

OBJECTIVE: To study the effect of sympathetic stimulation and increase in heart rate on the QT and QTc intervals. DESIGN: Prospective non-randomised study of eight consecutive patients. SETTING: Electrophysiology laboratory at a tertiary centre. PATIENTS: Eight patients aged 10-20 years (median 12.5) undergoing repeat electrophysiological study after previously successful catheter ablation (n = 6) or presumed supraventricular tachycardia (n = 2) with negative studies. INTERVENTIONS: Electrocardiograms were obtained (a) at baseline, (b) during atrial pacing at 450 ms cycle length, (c) during isoprenaline infusion at 0.025 microgram/kg/min, (d) adding atrial pacing (450 ms cycle length) to isoprenaline at 0.025 microgram/kg/min, and (e) isoprenaline at 0.05 microgram/kg/min. MAIN OUTCOME MEASURES: QT and QTc intervals at each of the above mentioned stages. RESULTS: The QT interval was reduced from a mean value of 350 ms to around 315-325 ms by each of the above manoeuvres. Correspondingly, the QTc increased from a mean of 407 ms to around 445-470 ms. Pacing was as effective as isoprenaline in shortening the QT interval and prolonging the QTc intervals. CONCLUSIONS: Heart rate directly influences QT and QTc intervals in children and adolescents. The QT is shortened, but QTc is prolonged. Hence, reliance on the QTc alone could lead to mistaken diagnosis of long QT syndrome.

Arrhythmias and electrocardiographic changes after the hemi-Fontan procedure

T he hemi-Fontan procedure has been proposed as an alternative to the bidirectional Glenn procedure in the first stage of the Fontan operation.1*2 In the hemiFontan operation, an atriopulmonary patch directs superior vena caval blood flow into both pulmonary arteries and the inferior vena caval flow into the ventricle. The hemi-Fontan procedure involves atria1 suture lines, which can potentially affect atria1 conduction and cause arrhythmias. Our surgical experience with the hemiFontan has been reported.2 Here, we report the early effects of the hemi-Fontan on the electrocardiogram and arrhythmias after the hemi-Fontan. Between August 1989 and May 1992,21 children (11 boys and 10 girls, aged 4 months to 5 years [median 11 months]), underwent the hemi-Fontan procedure at our hospital. The underlying cardiac defect was double-inlet left ventricle in 6, hypoplastic left heart syndrome in 5, pulmonary valve atresia with intact ventricular septum in 4, mitral atresia in 3 and complex double-outlet right ventricle in 3. All but 3 had had previous palliative procedures that were modified Blalock-Taussig shunt in 6, pulmonary artery banding in 5, stage 1 Norwood operation in 5, central shunt (a tube graft between the ascending aorta and the pulmonary trunk) in 3, coarctation of the aorta repair in 3, pulmonary artery reconstruction in 2, Blalock-Hanlon atria1 septectomy in 1 and Damus-Kaye-Stansel operation in 1. Before hemi-Fontan, no patient had clinical or electrocardiographic features of arrhythmia. After surgery 2 patients died, both of hemodynamic reasons. Atria1 JEutter occurred in the early postoperative period in 1 patient and recurred later in the same patient. Therapy for this patient was begun with amiodarone and is well controlled. Another had an episode of sustained ventricular tachycardia in the early postoperative period that responded to intravenous lidocaine, and did not reCUK The electrocardiographic results are summarized in Table I. All electrocardiograms showed sinus rhythm. The P-wave axis and PR interval were not sigr

QT Dispersion in Children With Ventricular Arrhythmia and a Structurally Normal Heart

cantly di

Inducible lethal ventricular arrhythmias in swine with pacing-induced heart failure

erent when compared between preoperative, early postoperative (~30 days) and late postoperative (followup of 5 to 33 months, mean 20) electrocardiograms. Pwave amplitude, however, decreased signijcantly in the early postoperative period. The late postoperative electrocardiogram showed some increase in P-wave amplitude compared with the early postoperative electrocardiogram but was still diminished compared to the preoperative electrocardiogram. No patient had fragmented P waves (defined as >2 upward dejections in