Acute complications of electrophysiology and pacing procedures: identification and management.

Non-pharmacologic interventional techniques for treatment and management of almost all cardiac arrhythmias have greatly expanded over the past decade. These newer interventional electrophysiologic techniques continue to demonstrate increasing success at achieving their targeted goals, and enhancing the patient's quality of life. However, like all interventional procedures, complications may result. In this article we provide the reader with an overview of the more common and significant adverse events that may follow electrophysiologic and pacing procedures, and how best to recognize and manage these complications. After providing the reader with an overview of the complications inherent to all electrophysiologic procedures, we will detail the adverse events intrinsic to specific therapeutic electrophysiologic interventions (DC cardioversion, pharmacologic-based cardioversion, antitachycardia pacing, and ablation of specific arrhythmias). In the last part of the review, we will delineate complications associated with pacing procedures (pacemaker and defibrillator implantation, biventricular pacing and pacing lead extraction).

Congenital heart disease caused by mutations in the transcription factor NKX2-5.

Mutations in the gene encoding the homeobox transcription factor NKX2-5 were found to cause nonsyndromic, human congenital heart disease. A dominant disease locus associated with cardiac malformations and atrioventricular conduction abnormalities was mapped to chromosome 5q35, where NKX2-5, a Drosophila tinman homolog, is located. Three different NKX2-5 mutations were identified. Two are predicted to impair binding of NKX2-5 to target DNA, resulting in haploinsufficiency, and a third potentially augments target-DNA binding. These data indicate that NKX2-5 is important for regulation of septation during cardiac morphogenesis and for maturation and maintenance of atrioventricular node function throughout life.

Clinical course of idiopathic dilated cardiomyopathy in children.

Previous studies in adults with dilated cardiomyopathy suggest that the presence of arrhythmia, especially ventricular tachycardia, correlates with increased mortality. We performed a retrospective analysis of 63 children with idiopathic dilated cardiomyopathy to determine the prognostic significance of arrhythmias and other findings with respect to mortality. The mean age at diagnosis of the cardiomyopathy was 4.96 +/- 5.3 years. The overall mortality rate was 16% over a 10 year follow-up period. Persistent congestive heart failure and ST-T wave changes correlated with increased mortality (p less than 0.05). No other variables affected outcome. Arrhythmias were found in 46% of the patients; of the arrhythmias, 48% were atrial arrhythmias. Ventricular tachycardia was present in six patients. Death occurred in 4 (14%) of 29 patients with known arrhythmia; 1 of the 5 died suddenly. The remaining 6 deaths in the series occurred in the 34 patients without a documented arrhythmia. It is concluded that 1) arrhythmias are frequently seen in children with dilated cardiomyopathy but are not predictive of outcome; 2) sudden death in children with this disease is rare; and 3) persistent congestive heart failure portends a poor prognosis.

Mexiletine: an effective antiarrhythmic drug for treatment of ventricular arrhythmias in congenital heart disease.

The use of antiarrhythmic drugs to suppress ventricular arrhythmias in pediatric patients with a structurally or hemodynamically abnormal heart appears to improve long-term prognosis. The previously successful use of phenytoin to treat serious ventricular arrhythmias led to the investigation for an alternative antiarrhythmic agent, in the same antiarrhythmic drug class, for those patients who develop side effects or become intolerant to phenytoin's antiarrhythmic effect. Forty-two children and young adults (age range 5 months to 34 years, mean 15.5 years) were treated with mexiletine. Arrhythmias treated were ventricular tachycardia (25), ventricular couplets (8), multiform ventricular premature beats (4) and frequent uniform ventricular premature beats (5). Anatomic diagnoses included congenital heart disease (postoperative in 26, unoperated in 2), cardiomyopathy (7), no heart disease (4) and other (3). Thirty-three patients had been previously treated with 1 to 5 (mean 1.6) antiarrhythmic drugs. In the short term, ventricular arrhythmias were effectively suppressed in 30 (71%) of all 42 patients treated. During follow-up (ranging to 42 months, median 10.6), 18 (60%) of the 30 acute responders continued to have excellent control. Early suppression of ventricular arrhythmias was more effective in patients with congenital heart disease (89%) than in those with cardiomyopathy (29%) or no heart disease (43%) (p less than 0.01). Initial complexity of ventricular ectopic activity had no effect on treatment results. Of 25 patients previously treated with phenytoin, in whom alternative antiarrhythmic therapy was required, 40% had long-term arrhythmia control when treated with mexiletine. Mexiletine therapy was terminated for side effects in only five patients (12%). Mexiletine is recommended for young patients with congenital heart disease and serious ventricular arrhythmias.

Neurally mediated cardiac syncope: autonomic modulation after normal saline infusion.

OBJECTIVES: This study assessed the heart variability response to orthostatic stress during tilt table testing before and after normal saline administration. BACKGROUND: The efficacy of sodium chloride and mineralocortoid in the treatment of neurally mediated cardiac syncope is attributed to intravascular volume expansion; however, their modulation of autonomic nervous system activity has not been evaluated. METHODS: Heart rate variability analysis was performed on 12 adolescents with a history of syncope or presyncope (mean age 15.2+/-0.7 years) during tilt table testing. Subjects were upright 80 degrees for 30 min or until syncope. After normal saline administration, the patient was returned upright for 30 min. Heart rate variability analysis data were analyzed by an autoregression model (Burg method). RESULTS: All subjects reproducibly developed syncope during control tilt table testing; median time to syncope was 9.4+/-2.1 min. After normal saline infusion, none of the subjects developed syncope after 30 min upright. In the control tilt, there was an initial increase followed by a progressive decrease in low frequency power until syncope. Repeat tilt after normal saline administration demonstrates that low frequency power increased but the magnitude of initial change was blunted when compared with control. In addition, low frequency power increased during normal saline tilt sequence compared with the control tilt, during which it decreased. CONCLUSIONS: Normal saline blunted low frequency power stimulation and prevented paradoxical low frequency power (sympathetic) withdrawal. Increasing intravascular volume with normal saline alters autonomic responses that may trigger neurally mediated syncope reflexes.

Persistence of ventricular arrhythmia after resolution of occult myocarditis in children and young adults.

OBJECTIVES: We sought to examine whether resolution of occult myocarditis in children with associated ventricular arrhythmia correlated with the presence of arrhythmia at late follow-up. BACKGROUND: Complex ventricular arrhythmias have been documented in children with myocarditis. Therapy is aimed at controlling the arrhythmia and any associated ventricular dysfunction. However, no reported studies have documented whether resolution of myocarditis in children is associated with resolution of the associated arrhythmias. METHODS: We performed a retrospective analysis of 12 patients (mean age 12 years) with myocarditis. Ambulatory electrocardiographic (Holter) monitors were reviewed for ventricular arrhythmias at presentation and follow-up. Patients were assigned to Group I if they received corticosteroids in addition to any antiarrhythmic agents and to Group II if they did not receive steroids. Follow-up endomyocardial biopsy was performed in some patients, and results were analyzed in relation to the presence of arrhythmias at follow-up. RESULTS: Eleven patients had ventricular tachycardia, and one had multiform couplets. Corticosteroids were given to seven patients (Group I). Follow-up biopsy was performed in seven patients (six received steroids), with resolution of inflammation in all; four of the seven still had ventricular arrhythmias but with improved control. Of the five patients without follow-up biopsy, three had persistent arrhythmia. Absence of inflammation at follow-up biopsy did not correlate with loss of ventricular arrhythmias, and there was no difference between Group I and II patients with respect to resolution of arrhythmia (Fisher exact test, p = 0.70, power 11%). CONCLUSIONS: Complex ventricular arrhythmias persist after apparent resolution of occult myocarditis in children. Although these arrhythmias are easier to control after such resolution, the patients may require long-term antiarrhythmic therapy.

Ventricular arrhythmias and sudden death in children.

In children, sudden death related to ventricular arrhythmias occurs virtually always in a patient with an abnormal heart. Therefore, children with ventricular tachycardia should be thoroughly investigated by anatomic cardiac catheterization and possibly electrophysiologic study. Sudden death may occur in a patient who had been relatively asymptomatic. This especially occurs in patients after repair of congenital heart disease. The patient may also never have had documented ventricular tachycardia, although most have had at least premature ventricular complexes on a Holter monitor recording. Finally, sudden death related to ventricular arrhythmias can often be prevented with vigorous medical and surgical therapy.

Incessant ventricular tachycardia in infants: myocardial hamartomas and surgical cure.

Infants with incessant ventricular tachycardia (occurring greater than 10% of the day) have generally been described in pathologic studies. This report describes 21 patients with incessant ventricular tachycardia present greater than 90% of the day and night; the age at diagnosis ranged from birth to 30 months (mean 10.5 months). The most common clinical presentation was cardiac arrest (11 patients, in 5 after digitalis for presumed supraventricular tachycardia); another 6 patients had congestive heart failure and 4 were asymptomatic. Three patients had coexisting Wolff-Parkinson-White syndrome. The rate of incessant ventricular tachycardia ranged from 167 to 440 (mean 260 beats/min) and the QRS duration from 0.06 to 0.11 second. The most common electrocardiographic (ECG) pattern (10 of 21) was right bundle branch block with left axis deviation, but other right and left bundle branch block patterns were observed. Conventional and investigational antiarrhythmic agents (nine patients received amiodarone) failed to eliminate incessant ventricular tachycardia in all. Electrophysiologic studies localized incessant ventricular tachycardia to the left ventricle in 17 (to the apex in 2, the free wall in 9 and the septum in 6) and to the right ventricular septum in 4. No structural abnormalities were found on the echocardiogram or angiocardiogram. All 21 patients had surgery at an age of 3.5 to 31 months (mean 16). In 15 a tumor was found: 13 myocardial hamartomas (9 discrete, 4 diffuse throughout both ventricles) and 2 rhabdomyomas (1 multiple). Myocarditis was found in one patient (the oldest). In four, only myocardial fibrosis was found; results of one biopsy were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Prevention of sudden death after repair of tetralogy of Fallot: treatment of ventricular arrhythmias.

The majority of sudden deaths after repair of tetralogy of Fallot have been presumed to be due to ventricular arrhythmia; however, it remains to be demonstrated that antiarrhythmic medication reduces the incidence of sudden death. Since 1978, ventricular arrhythmias have been treated aggressively; these include any ventricular arrhythmia on routine electrocardiogram and more than 10 uniform premature ventricular complexes per hour on 24 hour electrocardiogram. A review was undertaken of 488 patients followed up for more than 1 month after repair of tetralogy of Fallot (mean follow-up time 6.1 years); 13.5% had ventricular arrhythmia on routine electrocardiogram. Ventricular arrhythmia appeared from 2 months to 21 years postoperatively (mean 7.3 years). Ventricular arrhythmias were significantly (p less than 0.01) related to: longer follow-up duration, older age at follow-up, older age at operation and higher postoperative right ventricular systolic and end-diastolic pressures. Ventricular arrhythmia on routine electrocardiogram occurred in 100% of those who later died suddenly compared with 12% of those who did not die (p less than 0.01). Treatment for ventricular arrhythmia was given to 46 patients and considered "successful" if there were fewer than 10 uniform premature ventricular complexes per hour on 24 hour electrocardiogram. A successful drug was found in 44 of the 46: 30 of 34 given phenytoin, 6 of 9 given propranolol, 1 of 7 given quinidine, 1 of 2 given disopyramide, 8 of 9 given mexiletine and 4 of 5 given amiodarone.(ABSTRACT TRUNCATED AT 250 WORDS)

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

OBJECTIVE: We report 16 infants with complete congenital heart block (CHB) who developed late-onset dilated cardiomyopathy despite early institution of cardiac pacing. BACKGROUND: Isolated 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. METHODS: A 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. RESULTS: Congenital 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%). CONCLUSIONS: Despite 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.

Progressive atrioventricular valvular regurgitation in single ventricle.

The hemodynamic status of the patient with single ventricle is not static. This study describes the rapid development of mechanical malfunction of the atrioventricular (AV) valve in a subset of patients with single ventricle. Eighty patients with single ventricle were reviewed. Eight patients with single ventricle and single AV valve were observed to have moderate to severe AV valvular regurgitation. Patient age when AV regurgitation was first detected was 22 months to 35 years (median 15 years). Clinically, all patients were symptomatic and had severe congestive heart failure. All patients underwent cardiac catheterization, which revealed moderate to severe AV regurgitation; 7 of 8 patients had well preserved ventricular function. Earlier catheterization revealed no significant AV regurgitation. Four patients underwent surgery: 2 had AV valve replacement with the Fontan procedure and 2 had Carpentier ring placement (1 with the Fontan procedure). The 3 survivors were in improved clinical condition. At surgery 1 patient had an abnormal AV valve with 4 leaflets, and the other 3 had myxomatous degeneration of the AV valve. Thus, AV regurgitation is a significant and specific complication that occurs in patients with single ventricle and single AV valve. If recognized before development of eventual myocardial dysfunction, surgical management should be considered.

Mortality following radiofrequency catheter ablation (from the Pediatric Radiofrequency Ablation Registry). Participating members of the Pediatric Electrophysiology Society.

Deaths have been reported following radiofrequency catheter ablation (RFCA), but the mortality rate in children has not been defined. This study sought to analyze the incidence and the factors associated with mortality related to RFCA. Ten of 4,651 cases (0.22%) reported to the Pediatric RFCA Registry resulting in death were reviewed and compared with a matched control group (n = 18). Death occurred in 5 of 4,092 children (0.12%, ages 0.1 to 13.3 years) with structurally normal hearts. Death was related to traumatic injury, myocardial perforation and hemopericardium, coronary or cerebral thromboembolism, and ventricular arrhythmia. All cases were left-sided (p = 0.019 vs right or septal) supraventricular arrhythmias with radiofrequency applications in the systemic atrium and/or ventricle, and all procedures were successful. Mortality occurred in 5 of 559 children (0.89%, p = 0.001 vs normals, ages 1.5 to 17.4 years) with structural heart disease. No new pathology except the mural radiofrequency lesions was seen at autopsy. Those with structurally normal hearts who died were smaller (32.7 vs 55.6 kg, p = 0.023) and had more radiofrequency applications (26.3 vs 8.7, p = 0.019) than those who survived. No differences were demonstrated for those with abnormal hearts. Operator experience was not different (deaths 103 +/- 106 vs controls 117 +/- 125, p = 0.41). Mortality associated with pediatric RFCA is rare, but is more frequent when there is underlying heart disease, lower patient weight, greater number of radiofrequency energy applications, and left-sided procedures. Operator experience does not appear to be a factor leading to mortality.

Encainide for resistant supraventricular tachycardia in children: follow-up report.

Forty-one children (26 weeks gestational age to 20 years) with drug-resistant supraventricular tachycardia were treated with oral encainide, and 29 were followed for 3 to 34 months (mean 15). Diagnoses obtained by electrocardiographic criteria (23 patients) or electrophysiologic testing (18 patients) included permanent junctional reciprocating tachycardia in 15 children, paroxysmal atrioventricular reciprocating tachycardias (AVRT) in 13, atrial ectopic tachycardia in 4, atrial flutter in 1, chaotic atrial tachycardia in 5 and junctional ectopic tachycardia in 3. Encainide was completely effective in 54% (22 of 41 study patients) and partially effective in an additional 24% (10 of 41 patients), when combined with propranolol or verapamil. Within 1 month, 13 (32%) discontinued encainide for inefficacy or intolerance. Encainide was most effective in the treatment of permanent junctional reciprocating tachycardia (60% effective) and AVRT (69% effective). It controlled only 40% of primary atrial tachycardias. Encainide was well tolerated on a long-term basis in patients not experiencing symptoms during initiation. In study infants younger than age 6 months, encainide was associated with excessive QRS aberrancy during initiation in 4 of 13 (31%), compared with 3 of 28 (11%) in older children. Ventricular proarrhythmia occurred in 2 children and 1 died suddenly. Mean effective encainide dose was 3.5 mg/kg/day or 86 mg/m2/day. In 4 children who had nonextensive metabolism of encainide, the drug was ineffective. Encainide is effective in the treatment of some resistant forms of permanent junctional reciprocating tachycardia and AVRT in otherwise healthy children. Children younger than age 6 months and those with either previous proarrhythmic events or severe cardiac dysfunction appear to have a high incidence of adverse effects.

Encainide for refractory supraventricular tachycardia in children.

Fifteen children, aged 5 days to 19 years (mean 4.7 years), with medically refractory supraventricular tachycardia were given oral encainide. In 10 of 15 children with "incessant" tachycardia (greater than 10% of the day), encainide alone controlled supraventricular tachycardia in 5 children; in combination with other antiarrhythmic agents, it partially controlled supraventricular tachycardia in 4 and was ineffective in 1. In 5 children with accessory atrioventricular connections, encainide eliminated supraventricular tachycardia in 3 and was ineffective in 2. Therapeutic encainide dosages ranged from 60 to 120 mg/m2/day (mean 90) (2.0 to 5.7 mg/kg/day). Encainide caused prolongation of the PR interval by 35%, RP interval by 17%, QRS interval by 44% and corrected QT interval by 10%. In 5 children with depressed left ventricular function administration of encainide, by controlling the arrhythmia, increased echocardiographic left ventricular shortening fraction from a mean of 24% to a mean of 36%. Three patients developed excessive QRS aberrancy, which was associated with wide QRS tachycardia in 2. No adverse reactions were noted in the absence of QRS aberration. Side effects were minor and noted in only 1 of 9 patients continuing to take the drug in 9 months of follow-up. Encainide was effective, or partially effective, in the control of resistant or incessant supraventricular tachycardia in 80% of children treated. Encainide allowed rapid resolution of arrhythmia-induced cardiomyopathy by controlling chronic supraventricular tachycardia.

Ethmozine (moricizine HCl): a promising drug for "automatic" atrial ectopic tachycardia.

Atrial ectopic tachycardia is an uncommon life-threatening supraventricular tachycardia in children and is resistant to usual antiarrhythmic drugs. Whereas the cellular mechanism of atrial ectopic tachycardia is unknown, atrial ectopic tachycardia may be due to a form of automaticity. Moricizine HCl has been used primarily for ventricular rhythm disturbances; the drug depresses abnormal automaticity and delayed after-depolarizations but has little effect on normal automaticity. Because of this property, we have used moricizine HCl in 4 patients with atrial ectopic tachycardia. As evidenced by continuous 24-hour Holter monitoring, moricizine HCl was successful in suppressing atrial ectopic tachycardia in each patient. During a limited follow-up (6 months) no side effects have occurred. Moricizine HCl is a promising primary drug for atrial ectopic tachycardia.

Late ventricular arrhythmia and sudden death following direct-current catheter ablation of the atrioventricular junction.

Early reports of direct-current catheter ablation (DCCA) of the atrioventricular (AV) junction for resistant AV tachycardias documented efficacy of DCCA with little morbidity. Nine patients underwent DCCA at our institution 4 to 9 years ago: 3 patients had DCCA in the coronary sinus for permanent junctional reciprocating tachycardia, 2 patients had His ablation, 2 had coronary sinus and His ablation for permanent junctional reciprocating tachycardia, and 2 had DCCA for congenital tachycardia, and 2 had DCCA for congenital junctional ectopic tachycardia. Shocks (total 1 to 5) ranged from 12.5 to 400 J. Five patients had pacemaker implant at the time of DCCA. During follow-up, 3 patients developed clinical ventricular tachycardia: all 3 had DCCA of the His bundle. One asymptomatic patient with ventricular tachycardia, who had DCCA of the bundle of His, died suddenly 6 years later with ventricular fibrillation. Autopsy revealed 2 ventricular scars: 1 extending from the AV junction and 1 in the outflow tract. No patient with DCCA limited to the coronary sinus developed ventricular tachycardia. DCCA of the His bundle can result in late ventricular arrhythmias, possibly a result of extension of the DCCA lesion into the ventricle. These late findings should be considered in evaluating the safety and efficacy and follow-up for patients undergoing radiofrequency ablation.

Invasive electrophysiologic studies in children.

Although the technique of performing invasive electrophysiologic studies is similar in children and adults, the indications, interpretation, and management based on the studies are different. The major indications in children are determination of the specific diagnosis of an unknown arrhythmia and mapping prior to surgical treatment; chronic drug studies are performed for severely symptomatic patients, but technical considerations in the child limit the routine use of repeated drug trials. The greatest potential indication for invasive studies is in prognostication of symptomatic arrhythmias; this has not been possible in sinus node dysfunction and AV block, and the role in ventricular arrhythmias is under investigation. In the area of therapeutics, although drugs may have a similar effect in adults and children, the mechanism for the underlying arrhythmias may differ. The ventricular arrhythmias after congenital heart disease may have as their underlying cause the hypertrophic ventricle, not the pediatric substrate. Thus, some of the investigations originally performed in children eventually may contribute to a better understanding of arrhythmias in patients of all ages.

Surgical treatment of arrhythmias in children.

Primary surgical treatment of many tachyarrhythmias in children is now possible. In those with life-threatening arrhythmias not responsive to any form of medical treatment, the choice for surgery is clear. These arrhythmias include atrial fibrillation with the Wolff-Parkinson-White syndrome, PJRT, or atrial ectopic tachycardia with severe congestive cardiomyopathy, incessant ventricular tachycardia in infancy, and recurrent sustained ventricular tachycardia in postoperative congenital heart disease. In the majority of patients, however, surgical treatment remains an option to be weighed carefully against chronic medical treatment. Surgery is now possible with very low mortality for infants and children with Kent bundles, atrial ectopic tachycardia, and the permanent form of junctional reciprocating tachycardia. The mortality, morbidity, and likelihood of eventual resolution of the arrhythmia with each type of management plan should be considered. With possible direct surgical ablation of atrial flutter and newer forms of catheter treatment of arrhythmias, the future looks promising.

Case report: spontaneous atriofascicular pathway automaticity simulating ventricular tachycardia.

Atriofascicular pathways most commonly present electrocardiographically as an antidromic reciprocating AV reentrant tachycardia. We report the case of a child who presented in infancy with a wide QRS complex tachycardia thought to be supraventricular tachycardia with aberrant conduction, associated with tachycardia-induced cardiomyopathy. Later in life the same patient represented with episodes of palpitations secondary to a wide QRS complex tachycardia, thought to be ventricular tachycardia. Electrophysiologic mapping demonstrated the origin of the wide QRS complex tachycardia was from automatic activity originating from a right anterolateral atriofascicular pathway, which also participated in a reentrant antidromic AV reciprocating tachycardia. Radiofrequency ablation of the atriofascicular pathway successfully eliminated both arrhythmias. The mechanism of the wide QRS complex tachycardia appeared to result from spontaneous automaticity of the atriofascicular pathway.

Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation.

Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation.