Efficacy and safety of propafenone in congenital junctional ectopic tachycardia.

OBJECTIVES: Class IC antiarrhythmic drugs have recently been shown to be effective in some patients with atrial ectopic tachycardia. We therefore studied the efficacy of these drugs in patients with congenital junctional ectopic tachycardia. BACKGROUND: Follow-up data on patients with junctional ectopic tachycardia suggest that this potentially lethal arrhythmia may cease spontaneously in a significant proportion of affected children. Therefore, temporary antiarrhythmic treatment appears to be the therapy of choice. METHODS: The efficacy of propafenone was prospectively assessed in four infants with junctional ectopic tachycardia (ventricular rate 180, 185, 210 and 320 beats/min, respectively). The diagnosis of junctional ectopic tachycardia was established before the age of 2 months in all four infants. Propafenone was given orally in a mean dose of 350 (300 to 500) mg/m2 body surface area per day. Success of therapy was determined by serial electrocardiograms (ECGs) and Holter ambulatory ECG monitoring. RESULTS: In two patients, junctional ectopic tachycardia was completely suppressed. In the remaining two patients, the tachycardia rate decreased to less than 150 beats/min. Serum propafenone levels did not correlate with efficacy of treatment. The mean duration of therapy was 18 months (range 3 to 36). No chemical or clinical side effects were noted. CONCLUSIONS: Because of its effectiveness, safety and lack of side effects, propafenone appears to be a valuable drug in the treatment of junctional ectopic tachycardia in newborns and infants.

Efficacy and proarrhythmia of oral sotalol in pediatric patients.

OBJECTIVES: This study sought to assess the efficacy of oral sotalol for various arrhythmias in pediatric patients and to evaluate the incidence of proarrhythmia and systemic side effects. BACKGROUND: Sotalol is a beta-adrenergic blocking agent with additional class III antiarrhythmic properties. Experience in pediatric patients is limited. Data concerning the incidence of proarrhythmia in children are lacking. METHODS: Seventy-one pediatric patients (mean age 7.3 years) with various supraventricular and ventricular tachyarrhythmias were treated with oral sotalol. All the patients were admitted to the hospital for initiation of sotalol therapy. Antiarrhythmic and proarrhythmic effects of sotalol were assessed by daily surface electrocardiograms (ECGs) during the in-hospital phase and by serial Holter monitoring. RESULTS: Sotalol was either completely (27 [66%] of 41 patients) or partially effective (11 [27%] of 41) in 38 (93%) of 41 patients with supraventricular reentrant tachycardias. In patients with atrial flutter predominantly after operation for congenital heart disease, sotalol was effective in 84% of patients (completely in 9 of 19 and partially in 7 of 19). Ventricular tachycardia was completely (3 of 11) or partially (4 of 11) controlled in 64% of children. Proarrhythmia occurred in seven patients (10%) and consisted of symptomatic bradycardia from sinoatrial block and high grade atrioventricular (AV) block, respectively, in two children; asymptomatic high grade AV block in one; torsade de pointes in one; and relevant increased ventricular ectopic activity in three. Proarrhythmia required drug discontinuation in four patients. Mean duration of treatment for all patients was 18 months (range 1 to 40). CONCLUSIONS: Sotalol was an effective antiarrhythmic drug for a wide range of pediatric tachyarrhythmias. The considerable number of patients with proarrhythmic effects indicates the need for initiation of treatment on an inpatient basis and close monitoring by serial Holter electrocardiography.

Efficacy and safety of intravenous and oral propafenone in pediatric cardiac dysrhythmias.

Propafenone was administered to 58 patients with a mean age of 3.2 years (range 0.1 to 16). Mean intravenous dose was 1.2 mg/kg body weight (range 0.3 to 1.5 mg). The final mean oral maintenance dose was 308 mg/m2 body surface area (range 200 to 600 mg/m2, 16.8 mg/kg body weight). After intravenous application, propafenone was effective in 21 of 36 patients; atrial flutter was converted in 1 of 5 patients, and reentry supraventricular tachycardia was controlled in 15 of 25 patients. Propafenone was partially or completely effective in 3 of 4 patients with chaotic atrial tachycardia. Junctional ectopic tachycardia was suppressed in 2 infants. Thirty-seven patients had oral treatment with a mean follow-up of 2.2 years. Propafenone was effective in 33 of 37 patients (89%); atrial flutter was controlled in 2 patients, ventricular ectopy was suppressed in 1 of 2 patients. In reentry supraventricular tachycardia, propafenone was effective in 25 of 28 patients. Chaotic atrial tachycardia (n = 3) and junctional ectopic tachycardia (n = 2) were controlled after successful intravenous therapy. Systemic side effects were rare. Two patients developed a proarrhythmic effect, and 1 patient with ventricular ectopy after repair of tetralogy of Fallot died suddenly during propafenone maintenance therapy.

Usefulness of propafenone for supraventricular arrhythmias in infants and children.

The relation between propafenone dose, serum level, electrocardiographic parameters, antiarrhythmic drug efficacy and adverse effects was studied in 47 children with symptomatic supraventricular arrhythmias aged 1 day to 10.3 years (median 2.2 months) with a mean follow-up of 14.3 months. Propafenone trough serum levels were measured using gas chromatography. Oral propafenone (mean dose 353 mg/m2/day) was effective in 41 of the 47 patients (87.2%). Serum levels did not differ between patients responding and not responding to propafenone (0.45 +/- 0.40 vs 0.36 +/- 0.41 mg/liter). PR interval and QRS complex duration increased more significantly with propafenone dose increments (p < 0.001), than with propafenone serum levels (p < 0.05). At successful treatment PR interval and QRS complex were prolonged by a mean of 19.2 and 20.5% compared with pretreatment status. Five patients exhibited unexpected marked QRS complex prolongation (50 to 200%) despite low propafenone dosage (< 300 mg/m2/day) and level ranging from 0.05 to 1.33 mg/liter. Three patients (6.1%) were suspected of being "poor" metabolizers of propafenone. Mild chronic elevation of serum liver enzymes was observed in 5 patients treated with a larger dose (mean 448 mg/m2/day, p < 0.001). No proarrhythmia was noted on serial Holter monitors. One patient with Wolff-Parkinson-White syndrome and a normal heart had cardiac arrest after aspiration. Serial monitoring of PR interval and QRS complex duration was more useful for proper dosage adjustment than propafenone serum levels. Serum liver enzymes should be closely monitored when using higher propafenone doses. Malignant proarrhythmia could not be excluded in the 1 patient with cardiac arrest.

Acrofacial dysplasia resembling geleophysic dysplasia.

We report on a 12-year-old girl with acrofacial dysplasia, growth retardation, joint contractures, mitral valve incompetence and focal hepatic storage of material reacting histochemically as glycoprotein. The patient's phenotype resembles that of patients with geleophysic dysplasia but differs with respect to facial appearance, milder changes of hand bones and normal capital femoral epiphyses. It is undecided if her disorder is part of a wider phenotypic spectrum of geleophysic dysplasia or a different entity.

Modified Fontan operation in functionally univentricular hearts: preoperative risk factors and intermediate results.

BACKGROUND: The modified Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy. To eliminate the influence of different surgical strategies, only patients undergoing total cavopulmonary anastomosis were included in this retrospective study. METHODS: Seventy-two patients had been operated on at ages ranging from 7 to 219 months, with 29 patients younger than 4 years. Twenty-three patients had mean pulmonary artery pressures higher than 15 mm Hg, elevated pulmonary arteriolar resistances (> 3 U.m2), or elevated end-diastolic ventricular pressure (> 12 mm Hg). Associated systemic or pulmonary venous anomalies were present in 22 patients (30%), and atrioventricular valve incompetence was present in 21 patients (29%). RESULTS: The overall mortality rate was 9.7% (7/72). Variables with significant effects on postoperative mortality were associated systemic or pulmonary venous anomalies, atrioventricular valve incompetence, mean pulmonary artery pressure greater than 15 mm Hg, and prolonged cardiopulmonary bypass time. Postoperative morbidity resulted mainly from atrial arrhythmia (20%). Clinical signs of protein-losing enteropathy or atrial thrombi were rare (3% and 6%, respectively). Postoperative hemodynamic data from 48 surviving patients (74%) revealed a mean transpulmonary gradient of 6.3 mm Hg, systemic venous pressure greater than 12 mm Hg in only 10 patients, and cardiac index less than 3 L.min-1.m-2 in 18 patients. CONCLUSION: In a high proportion of hearts with complex univentricular anatomy, associated anomalies, and borderline hemodynamics, the Fontan operation can be performed as a total cavopulmonary anastomosis with acceptable intermediate postoperative morbidity and hemodynamic results.

Successful management of junctional tachycardia by hypothermia after cardiac operations in infants.

BACKGROUND: Junctional ectopic tachycardia is an early postoperative complication after intracardiac repair of congenital heart disease, especially in infants. Because of the high ventricular rate and the usually poor response to antiarrhythmic drugs, this condition is associated with a high morbidity and mortality. The purpose of this study was to assess the safety and efficacy of moderate body surface hypothermia in the treatment of postoperative junctional ectopic tachycardia in infants. METHODS: Six consecutive infants with postoperative junctional ectopic tachycardia (mean age at operation, 14 weeks) were treated with surface cooling. The decision to start treatment was based on the definition of a critical heart rate (180 to 200 beats/min) in the presence of junctional ectopic tachycardia diagnosed according to established criteria. Moderate hypothermia (rectal temperature between 32 degrees and 34 degrees C) was achieved by placing ice bags on the child's body surface. The patients were sedated, mechanically ventilated, and paralyzed. RESULTS: Mean interval between diagnosis of tachycardia and initiation of hypothermia was 4 hours. Rectal temperature was rapidly (within 1 hour) lowered to 32 degrees to 34 degrees C in all 6 patients. This significantly lowered the tachycardia rate from 219 +/- 27 beats/min to 165 +/- 25 beats/min (mean +/- standard deviation; p < 0.001). Three patients with signs of low cardiac output had restoration of stable hemodynamics once the tachycardia rate had been decreased by hypothermia. Cooling was maintained for a period of 24 to 88 hours (mean, 59 hours). No serious side effects were observed. CONCLUSIONS: Early institution of moderate hypothermia by body surface cooling was a safe and efficient measure to control ventricular rate in infants with postoperative junctional ectopic tachycardia.

Surgical treatment of double-outlet left ventricle in 2 patients with D-position and L-position of the aorta.

Two cases of double-outlet left ventricle (DOLV) are described, 1 with D-position and 1 with L-position of the aorta. Both patients had situs solitus, atrioventricular concordance with D-relationship of the two ventricles, a ventricular septal defect, and subpulmonary stenosis. The patient with D-position of the aorta was operated on using a technique similar to that for repair of Fallot's tetralogy but including emergency implantation of a bioprosthesis into the pulmonary artery position on the eighth postoperative day. Surgical correction in the patient with L-position of the aorta required the implantation of a composite valved conduit between the right ventricle and the pulmonary artery. The various surgical techniques for correction of DOLV are described, and the literature is reviewed. Postoperative right heart failure in both patients is explained by the impaired function of the right ventricle being exposed to gross pulmonary incompetence or by the persistence of elevated right ventricular pressure.

Total cavopulmonary anastomosis in patients less than three years of age.

BACKGROUND: As young age at modified Fontan operation was thought to be a preoperative risk factor for poorer survival, we studied early and intermediate outcome in our young patient group. METHODS: Results in children less than 3 years of age (group I; n = 26; age range, 7 to 35 months) were compared with those in older patients (group II; n = 46; age range, 36 to 219 months). For both groups there was no significant difference with regard to preoperative pathology and hemodynamics. RESULTS: With an overall mortality of 9.7% (7/72) there was no significant difference for both groups. Group I (n = 23) and group II (n = 42) survivors did not differ with respect to early postoperative incidence of atrial dysrhythmias, duration and volume of pleural effusion, or incidence of reoperation. Results on intermediate follow-up (group I, 31 +/- 14 months; group II, 44 +/- 20 months) demonstrated a relatively low incidence of late atrial dysrhythmias (12.3%; 8/65). Late atrioventricular valve insufficiency was significantly more frequent in group I patients. CONCLUSIONS: With similar preoperative anatomic and hemodynamic parameters including 68% of patients with complex univentricular heart, total cavopulmonary anastomosis could be performed in patients less than 3 years of age with good clinical and hemodynamic results, as achieved in older patients.

Complete correction of total anomalous pulmonary venous drainage: experience with 53 patients.

From January, 1973, to August, 1984, 53 infants with total anomalous pulmonary venous drainage (TAPVD) underwent a corrective operation in our unit. TAPVD was of the supracardiac type in 41% of the patients, cardiac in 17%, infracardiac in 36%, and mixed in 6%. Overall operative mortality was 23%; it was highest at 42% in the infracardiac group. Factors determining the outcome were the anatomical type of the lesion, the degree of pulmonary venous obstruction, the severity of pulmonary hypertension, and the young age of the patients. In addition, surgical experience appears to be an important factor in determining the outcome. During the study, hospital mortality decreased considerably to 11%. A corrective procedure offers the only chance of survival for patients with TAPVD. With some experience, excellent results can be obtained.

Isolated aortic coarctation in neonates and infants: results of resection and end-to-end anastomosis.

BACKGROUND: Operative resection is the treatment of choice for native aortic coarctation in most institutions. The ideal timing for elective repair is still a matter of debate. This study evaluated one institution's results with resection and end-to-end anastomosis in the first year of life. METHODS: Between January 1987 and December 1993, 46 neonates and infants with functionally isolated aortic coarctation underwent operative resection and end-to-end anastomosis. For the patients included in the study, all hospital records, catheterization data, and operative protocols were evaluated for retrospective analysis. To obtain valid follow-up information, all patients were systematically seen on an outpatient basis during 1994. RESULTS: After a mean follow-up of 49 +/- 24 months (range, 13 to 95 months), recoarctation (arm-leg blood pressure gradient > 20 mm Hg) occurred in 5 of 26 patients with neonatal operations (19%) and in 1 of 20 patients with operations in infancy (5%, p = not significant). Four of these 6 children with recoarctation needed reintervention. The other 2 patients had only mild recoarctation (gradients of 22 and 30 mm Hg, respectively) and were not treated. In all 6 patients, recoarctation was diagnosed within the first 6 months postoperatively. During the whole follow-up period, right arm systolic blood pressures slightly above the 90th percentile of normal developed in 11 of the patients (24%) (7 in the group with neonatal operation and 4 after operation in infancy; p = not significant). CONCLUSIONS: Resection with end-to-end anastomosis was shown to be an adequate therapeutic strategy for isolated aortic coarctation in neonates and infants. The results indicate that already beyond the neonatal age, there is a relatively low incidence of recoarctation.

Right-sided heart with mirror-image atrial arrangement, complete transposition, ventricular septal defect and subpulmonary stenosis.

An infant is described with complete transposition in the setting of a right-sided heart, and mirror-image atrial arrangement. A ventricular septal defect and subpulmonary stenosis were also present. We discuss the anatomic features of this patient, undescribed prior to this case, and their implications for surgical repair.

Truncus arteriosus type A3: complex repair with cryopreserved pulmonary homograft.

A 6-year-old girl with truncus arteriosus type A3, one of the rarest anatomic types, underwent corrective surgery using a cryopreserved valved pulmonary homograft. The special anatomic features in this case were individual origin of the pulmonary arteries. The right pulmonary artery came directly off the truncal vessel, whereas the left pulmonary artery was connected to the aortic arch via a stenotic ductus or ductus-like vessel. The preoperative pulmonary blood flow distribution was 94% on the right versus 6% on the left side. The underperfusion of the left lung may have been related to pulmonary hypoplasia due to long lasting stenosis at the left pulmonary artery take off. The postoperative pulmonary blood flow distribution was 67% on the right versus 33% on the left side. The operative and postoperative course has been uneventful; presently, 6 months after the operation, the patient is in improved clinical condition.

Coarctation of the aorta in complex congenital heart disease: simultaneous repair via sternotomy.

Coarctation of the aorta (CoA) is often associated with complex congenital heart disease. Patients with such a combination may not benefit from coarctectomy alone. Eight children who presented with complex malformations of the heart underwent simultaneous repair of CoA and intracardiac surgery via sternotomy. After extensive mobilization of the aortic arch, cardiopulmonary bypass was established. During the cooling phase for deep hypothermic circulatory arrest (six cases), a persistent temperature gradient between the upper and lower half of the body confirmed the significance of CoA. One child was operated upon in deep hypothermia with low flow and one underwent valve repair on cardiopulmonary bypass. Mobilization of the descending aorta enabled CoA resection and end-to-end anastomosis with a running absorbable suture. The average descending aortic cross-clamping time was 15 min. By this time, the patient had been cooled sufficiently for the intracardiac procedure. There were two operative deaths not related to coarctectomy. The remaining children showed no arm-to-leg pressure gradient. Five were discharged from hospital and one patient died late from septicaemia. In our hands, this technique has served to accomplish simultaneous relief of CoA and repair of the intracardiac lesion thus sparing critically ill infants the hazards of repeated procedures.

Early and late atrial dysrhythmias after modified Fontan operation.

UNLABELLED: Occurrence of supraventricular tachycardia and sinus node dysfunction was investigated pre- and postoperatively by serial ECG and Holter monitors in 63 consecutive patients with univentricular circulation after modified Fontan operation (total cavopulmonary connection 39 patients, atriopulmonary connection 24 patients). Mean age at operation was 7.2 (0.1-20.3) years. Of the 63 patients, 14 (22%) had early (< 14 d) supraventricular tachycardia or sinus node dysfunction, which was not related to the type of operation. None of 9 patients with a preoperative mean right atrial pressure < or = 2.5 mm Hg had early supraventricular tachycardia or sinus node dysfunction in contrast to 16/54 patients (30%) with a preoperative mean right atrial pressure > 2.5 mm Hg. 6/63 patients died during the early (< 14 d) postoperative period. In only 1 child, death was related to a dysrhythmia (junctional ectopic tachycardia). During a mean follow-up of 2.5 years, 15/57 long-term survivors (21%) had late supraventricular tachycardia or sinus node dysfunction. Early supraventricular tachycardia/sinus node dysfunction was a predictor or late atrial dysrhythmias, as it occurred in 8 of the surviving 14 patients with early dysrhythmias in contrast to 4 children without early atrial dysrhythmias (p < or = 0.001). After creation of an atriopulmonary connection, 10/22 patients (45%) had late supraventricular tachycardia/sinus node dysfunction, but only 2/35 patients (6%) with a total cavopulmonary connection had late atrial dysrhythmias (p < 0.001). CONCLUSIONS: Early atrial dysrhythmias after the Fontan operation were related to preoperative hemodynamics. Early supraventricular tachycardia/sinus node dysfunction and the atriopulmonary type of Fontan connection were significant risk factors for late atrial dysrhythmias.

Congenital heart disease and cardiac surgery in children.

In the period reviewed, more than 150 papers were published on congenital heart disease in English, Dutch, Spanish, French, Italian, and German, the majority dealing with specific operative procedures, general surgical management, indications for and results of palliative and corrective operations, and mid- and long-term postoperative outcome. New aspects of cardiological interventions are noteworthy, especially the dilation of valves and vessels and the use of occluding devices in hitherto untouched regions. A great deal of new information has been published with respect to specific lesions, such as tetralogy of Fallot, transposition of the great arteries, pulmonary atresia, and the univentricular circulation. This will certainly change our management of these patients in the future. Several large centers again showed how fruitful the close collaboration between cardiac surgeons and "interventionists" can be.

[Unusual combination of absence of the left pulmonary artery and homolateral malformation of the kidney and ureter (author's transl)]. / Ungewöhnliche Kombination einer linksseitigen Pulmonalarterienagenesie mit einer homolateralen Fehlbildung der Niere und des Ureters

In an one year old asymptomatic girl with a slight levoposition of the heart on chest X-ray and mild right ventricular hypertrophy in the ECG further cardiological investigation disclosed several anomalies: Absence of the left pulmonary artery, collateral vessels to the left lung both from the abdominal aorta and from a left brachiocephalic trunc, hypoplasia of the left lung, right sided aortic arch. There was no malformation of the heart itself. This is supposed to be the first reported case, in which the aforementioned anomalies were associated with hypoplasia and hydronephrosis of the left kidney and stenosis of the ostium on the left ureter. Considering the normal and pathologic development of the human embryo it may be assumed, that there is no immediate relationship between the malformation of the left lung and its vessels on one hand and the defects of the left kidney and ureter on the other hand. The hypothesis is put forward, that the damage of the embryo occurred limited to one side during the period of teratogenetic determination common to both of the organ systems involved.

[Solitary pulmonary arteriovenous fistula in infancy and childhood (author's transl)]. / Solitäre arteriovenöse Lungenfistel im Säuglings- und Kindesalter

Pulmonary arteriovenous fistula is not a rare congenital malformation, but obviously most often it is a very late diagnosis. Moreover the disease is associated with a high risk of serious complications, namely cerebrovascular accidents. Two patients, an one month old infant and an 8 years old boy with typical symptoms and signs are presented. Both of them were operated upon successfully. It is concluded, that the diagnosis of solitary pulmonary arteriovenous fistula should lead to immediate operation, which is technically easy and of low risk only, even in infancy. Conservative surgery is recommended without sacrifice of healthy lung tissue, i.e. local excision or segmental resection in subpleural lesions and lobectomy for deep-seated fistulas.