Macdonald Dick

The long QT syndrome in children. An international study of 287 patients.

BackgroundThe Pediatric Electrophysiology Society studied children with the long QT syndrome (LQTS) to describe the features ofLQTS in patients less than 21 years old, define potential “low-risk” and “high-risk” subpopulations, and determine optimal treatment Methods and ResultsPatients less than 21 years old were included if either QTc was more than 0.44; they had unexplained syncope, seizures, or cardiac arrest preceded by emotion or exercise; or family history of LQTS. We found 287 patients from 26 centers in seven countries. Mean±SD age at presentation was 6.8±5.6; 9%o presented with cardiac arrest, 26% with syncope, and 10%, with seizures. Of those with symptoms, 67% had symptoms related to exercise. Family history was positive for long QT interval in 39% and for sudden death in 31%. Hearing loss was present in 4.5%. A normal QTc was present in 6%, and QT, of more than 0.60 was in 13%. Atrioventricular block occurred in 5%, but 13 of 15 patients had second-degree atrioventricular block (2:1), and only two of 287 had complete atrioventricular block. Ventricular arrhythmias were found on 16% of initial routine ECGs: 4% uniform premature ventricular contractions, 5% multiform premature ventricular contractions, 1% monomorphic ventricular tachycardia, and 6% torsade de pointes. Overall, treatment was effective for symptoms in 76% and for ventricular arrhythmias in 60%. There was no difference between propranolol and other β-blockers in effective treatment. Left stellectomy was performed in nine patients, and defibrillators were implanted in four, no sudden death occurred in these 13 patients. In follow-up (duration, 5.0±4 years; age, 10.9±6.3 years), 5% had cardiac arrest, 4% had syncope, and 1% had seizures. The two multivariate predictors of symptoms at follow-up were symptoms at presentation and propranolol failure. Sudden death occurred in 8%; multivariate predictors of sudden death were length of QTc at presentation of more than 0.60 and medication noncompliance. ConclusionThe appearance of 2:1 atrioventricular block, multiform premature ventricular contractions, and torsade de pointes are relatively more common in children with LQTS than other children and should raise the index of suspicion for LQTS. Because 9% of patients presented with cardiac arrest and no preceding symptoms, perhaps prophylactic treatment in asymptomatic children is indicated. Asymptomatic patients with normal QTc and positive family history may be a low-risk group. Patients with QTc of more than 0.60 are at particularly high risk for sudden death, and if treatment is not effective, consideration should be given to cardiac sympathetic denervation, pacemaker implantation, and perhaps implantation of a defibrillator.

SUDDEN CARDIAC DEATH AND THE USE OF IMPLANTABLE CARDIOVERTER-DEFIBRILLATORS IN PEDIATRIC-PATIENTS

BACKGROUND During the past decade, the implantable cardioverter-defibrillator (ICD) has emerged as the primary therapeutic option for survivors of sudden cardiac death (SCD). Investigation of the clinical efficacy of these devices has primarily assessed outcome in adults with coronary artery disease. The purpose of this cooperative, international study was to evaluate the impact of ICDs on the pediatric population of SCD survivors, based on an analysis of the clinical characteristics and outcomes of young patients who underwent ICD implantation following an episode of life-threatening ventricular tachycardia or resuscitation from SCD. METHODS AND RESULTS An initial data base, established by contacting the manufacturers of the various commercially and investigationally available devices, identified 177 patients who were less than 20 years of age at the time of initial implantation of an ICD. With this data base as a reference, detailed responses were subsequently obtained from physicians involved in the care of 125 (71%) of these patients. The patients ranged in age from 1.9 to 19.9 years (mean, 14.5 +/- 4 years) and weighted 9.7-117 kg (mean, 44.6 +/- 14 kg). Of the 125 patients, 76% were survivors of SCD, 10% had drug refractory ventricular tachycardia, and 10% had syncope with heart disease and inducible sustained ventricular tachyarrhythmias. The most common types of associated cardiovascular disease were hypertrophic and dilated cardiomyopathies (54%), primary electrical diseases (26%), and congenital heart defects (18%). Ventricular function was abnormal in 46% of the patients. During a mean follow-up of 31 +/- 23 months, at least one ICD discharge occurred in 85 of the 125 (68%) patients. Seventy-three patients (59%) received at least one appropriate ICD discharge, and 25 patients (20%) had one or more spurious or indeterminate discharges. Duration of follow-up > 24 months (p = 0.001) and inducibility of a sustained ventricular arrhythmia (p = 0.05) were correlated with appropriate ICD discharges. There were nine deaths during the study period: five sudden, two due to recurrent ventricular arrhythmias, and two related to congestive heart failure. Abnormal ventricular function (p = 0.002) and prior ICD discharge (p = 0.01) were univariate correlates of patient mortality; by multivariate logistic regression, abnormal ventricular function was the only significant correlate of death (p = 0.005). By actuarial analysis, the estimated overall post-ICD implant survival rates at 1, 2, and 5 years were 95%, 93%, and 85%, respectively. The corresponding sudden death-free survival rates were 97%, 95%, and 90%. CONCLUSIONS Pediatric patients resuscitated from SCD appear to remain at risk for recurrence of life-threatening tachyarrhythmias. During a mean follow-up of 31 months, the ICD provided an effective therapy for such arrhythmias in the majority of patients in this study. Following ICD implant, impaired ventricular function was the primary factor correlated with mortality. The patterns of ICD discharge observed in young patients and, thus, inferred risk of recurrent life threatening arrhythmias are similar to those of adult survivors of SCD. Thus, the use of ICDs in pediatric patients, with implant selection criteria similar to adults, appears valid.

Doppler evaluation of left ventricular diastolic filling in children with systemic hypertension

To assess left ventricular (LV) diastolic function in children with systemic hypertension, 11 patients with hypertension (mean blood pressure 99 mm Hg) and 7 normal patients (mean blood pressure 78 mm Hg) underwent M-mode echocardiography and pulsed Doppler examination of the LV inflow. From a digitized trace of the LV endocardium and a simultaneous phonocardiogram, echocardiographic diastolic time intervals, peak rate of increase in LV dimension (dD/dt), and dD/dt normalized for LV end-diastolic dimension (dD/dt/D) were measured. Doppler diastolic time intervals, peak velocities at rapid filling (E velocity) and atrial contraction (A velocity), and the ratio of E and A velocities were measured. The following areas under the Doppler curve and their percent of the total area were determined: first 33% of diastole (0.33 area), first 50% of diastole, triangle under the A velocity (A area), and the triangle under the E velocity (E area). The A velocity (patients with hypertension = 0.68 +/- 0.11 m/s, normal subjects = 0.49 +/- 0.08 m/s), the 0.33 area/total area (patients with hypertension = 0.49 +/- 0.09, normal subjects = 0.58 +/- 0.08), the A area (patients with hypertension = 0.17 +/- 0.05, normal subjects = 0.12 +/- 0.03), and the A area/total area (patients with hypertension = 0.30 +/- 0.11, normal subjects = 0.20 +/- 0.07) were significantly different between groups (p less than 0.05). M-mode and Doppler time intervals, (dD/dt)/D, E velocity, and the remaining Doppler areas were not significantly different between groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Rhythm after the Mustard Operation for Complete Transposition of the Great Arteries

The Mustard operation corrects the effects of congenital transposition of the great arteries by creating an intraarterial baffle to direct pulmonary venous blood to the tricuspid orifice and systemic venous blood to the mitral orifice. To identify the long-term effects of this procedure, we followed 372 patients with complete transposition of the great arteries who survived the Mustard operation for at least three months. The mean follow-up period was 4.5 years (range, 0.4 to 15.9); the mean age at operation was 2.0 years. Mean resting heart rates were consistently lower than those for age-matched normal children. Seventy-six per cent of the patients had sinus rhythm during the year of operation--a figure that decreased to 57 per cent by the end of the eighth postoperative year. Twenty-five patients died during the follow-up period, nine suddenly. Life-table analysis revealed a cumulative survival rate of 91 per cent for 11 years and 71 per cent for 15 years after the operation. No strong risk factor for sudden unexpected death identified. This study demonstrates that extended survival among patients with transposition can be expected after the Mustard operation. However, over time there is a decreasing prevalence of normal sinus rhythm in survivors, as well as a small risk of sudden death.

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)

Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle

The bidirectional Glenn operation may be particularly useful as an intermediate procedure before Fontan correction in high-risk patients. From October 1989 through February 1992, 50 patients 1 to 60 months old (median 12) have undergone a bidirectional Glenn operation. Diagnoses included hypoplastic left heart syndrome in 21 patients, pulmonary atresia with intact ventricular septum in 10, tricuspid valve atresia in 9, other complex univentricular heart defects in 9, and Ebsteins anomaly in 1. Mean pulmonary vascular resistance was 2.2 +/- 0.2 Wood U (range 0.5 to 7.3) and mean pulmonary artery area Nakata index was 318 +/- mm2/m2 (range 80 to 821). Additional procedures were performed in 17 patients, including pulmonary artery reconstruction in 15 (29%) and bilateral caval anastomoses in 5 (10%). There were 4 hospital deaths (8%). Two deaths resulted from myocardial infarction in patients with pulmonary atresia with intact ventricular septum and sinusoids and 1 from severe pulmonary vascular disease in a patient with hypoplastic left heart syndrome. There was 1 late death from pneumonia. Actuarial survival is 92 +/- 4% at 1 month and beyond, with a mean follow-up of 13.4 +/- 1 months. Risk factor analysis showed that pulmonary vascular resistance > 3 Wood U and pulmonary artery distortion were associated with increased mortality. Twelve patients have undergone a Fontan procedure at a mean duration after bidirectional Glenn of 18 months with 1 death (8%). The bidirectional Glenn procedure provides excellent palliation in high-risk patients and appears useful as a staging procedure before Fontan correction.

Randomized comparison of atenolol and fludrocortisone acetate in the treatment of pediatric neurally mediated syncope

Overall, these results indicate that oral treatment of neurally mediated syncope is safe and efficacious. Further randomized trials in children will be required to determine the significance of a placebo effect, as well as potential differences in results related to the mechanism of syncope.

Percutaneous balloon valvuloplasty for treatment of congenital pulmonary valvular stenosis in children

Percutaneous balloon pulmonary valvuloplasty was performed in seven children with moderate to severe valvular pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mm Hg). All patients experienced a decrease in right ventricular peak systolic pressure from 108 +/- 30 to 60 +/- 5.6 mm Hg (p less than 0.001), decrease in right ventricular to pulmonary artery gradient from 90.1 +/- 30 to 38.8 +/- 5 mm Hg (p less than 0.001) and increase in pulmonary valve area from 0.33 +/- 0.06 to 0.55 +/- 0.15 cm2/m2 (p less than 0.001). In the two patients who underwent supine bicycle exercise before and after valvuloplasty, a significant decrease in both the maximal right ventricular peak systolic pressure (212 to 140 and 175 to 125 mm Hg, respectively) and in right ventricular to pulmonary artery peak pressure gradient (185 to 110 and 151 to 85 mm Hg, respectively) occurred. All patients tolerated the procedure well and no serious complications were observed. It is concluded that percutaneous balloon valvuloplasty is a safe and effective method for relief of right ventricular obstruction due to moderate or severe valvular pulmonary stenosis. However, long-term results remain unknown.

Use of radiofrequency current to ablate accessory connections in children.

BackgroundSeveral investigators have recently ablated electrophysiologically mapped accessory connections in the adult human myocardium by using radiofrequency current. To examine the effectiveness and safety of radiofrequency current for ablation of accessory connections in children, 20 consecutive patients (age, 3–18 years) with preexcitation and/or supraventricular tachycardia were evaluated by electrophysiological study. Methods and ResultsNineteen of the 20 patients were completely studied and demonstrated accessory connections. After identification of the earliest retrograde atrial activation site, a steerable 7F catheter (with a 4-mm-long electrode at the distal tip) was placed within the ventricular cavity ipsilateral to the accessory connection and positioned at the atrioventricular valve annulus directly opposite the earliest point of retrograde atrial activation. Radiofrequency current was delivered at 50–65 volts for 10–60 seconds at a frequency of 500 kHz. Radiofrequency pulses were delivered for two to 26 trials. Upon completion of radiofrequency trials, repeat electrophysiological testing was performed. Thirteen of 19 subjects (68%) experienced definite successful ablation of their accessory pathway; an additional patient had probable successful ablation, yielding an overall success rate of 74%. Eighty-seven percent of individuals with a left-sided pathway had permanent ablation and 100% with a manifest left-sided pathway experienced successful ablation. Only 29% of the first seven patients had a successful result; in contrast, 92% of the next 12 patients had successful interruption of their accessory pathways. After ablation, 4-day continuous electrocardiographic telemetry disclosed no significant arrhythmias. CPK enzyme rises peaked at 12–24 hours. The rise was excessive and associated with general anesthesia in five patients. The isoenzyme MB fraction rose mildly in five other patients and returned to normal within 72 hours. No clinical or electrocardiographic evidence of myocardial ischemia was detected. Follow-up for 4–12 months indicates no return of preexcitation or tachycardia in any patient whose accessory connection was successfully ablated. ConclusionsThis experience indicates that radiofrequency current is an effective and safe technique for ablation of accessory connectionsin children.

Percutaneous balloon angioplasty for native coarctation of the aorta

Twenty-six children, aged 5 weeks to 14.7 years, underwent percutaneous balloon angioplasty for a discrete native coarctation of the aorta. The procedure reduced the systolic coarctation gradient acutely in all children. The mean systolic gradient decreased by 75%, from 48.6 +/- 2.4 before to 12.3 +/- 1.9 mm Hg after angioplasty (p less than 0.001). Long-term results were evaluated in 14 children by follow-up catheterization 12 to 26 months (mean 15.3) after angioplasty. At follow-up, the residual gradient averaged 11.7 +/- 3.7 mm Hg (range -5 to 36) and had not changed from that measured immediately after angioplasty (p = 0.64). Compared with preangioplasty values, the systolic pressure in the ascending aorta had improved substantially at follow-up (116.0 +/- 3.2 versus 143.9 +/- 3.1 mm Hg, p less than 0.001). On the basis of follow-up data, two groups of children were identified: Group 1 consisted of nine children with a good result, defined as a residual gradient less than 20 mm Hg and no aneurysm; Group 2 consisted of five children with a poor result, four with a residual gradient greater than 20 mm Hg (range 25 to 36) and one with an aneurysm at the dilation site. There was no statistical difference between the two groups in age at angioplasty, balloon size, ratio of balloon to isthmus diameters, follow-up duration, heart rate or cardiac output. However, of the four children with a residual gradient greater than 20 mm Hg, two were the youngest in the study, and in two the aorta was inadvertently dilated with a balloon 4 to 5 mm smaller than the isthmus diameter.(ABSTRACT TRUNCATED AT 250 WORDS)