[Arrhythmogenic right ventricular dysplasia/cardiomyopathy - diagnosis in childhood]. / Arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie. Diagnostik im Kindesalter.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare, but important cause for sudden death in adolescents and young adults. Part of the patients affected show the pattern of autosomal-dominant inheritance. Two pediatric patients with ARVD/C are presented who may reflect the spectrum of clinical presentation of ARVD/C in childhood resulting in difficulties or even delay to establish the correct diagnosis. One patient with a sporadic form of ARVD/C presented with a syncope and spontaneous as well as inducible ventricular tachycardia. On the ECG, an epsilon wave could be identified. An automatic cardioverter defibrillator was implanted. The second patient had a familiar form of ARVD/C with no symptoms. There was a history of frequent sudden deaths in this family. Biopsies of the right ventricular myocardium showed fibrosis with deposition of fatty tissue. There was clear evidence of ARVD/C in the necropsy of the patient's aunt. Therapy with propanolol was started in this patient.

Atrial reentrant tachycardia after surgery for congenital heart disease: endocardial mapping and radiofrequency catheter ablation using a novel, noncontact mapping system.

BACKGROUND: The purpose of the present study was to determine the role of a novel, noncontact mapping system for assessing a variety of atrial reentrant tachycardias (ART) in patients after the surgical correction of congenital heart disease. METHODS AND RESULTS: In 14 patients, an electrophysiological study using the Ensite 3000 system was performed to assess ARTs resistant to medical treatment. Sixteen different forms of ART were inducible in the 14 patients studied. The reentrant circuit of all ARTs could be characterized and localized with respect to anatomic landmarks such as atriotomy scars, intraatrial patches/baffles, and cardiac structures. In 15 of the 16 ARTs (in 13 of the 14 patients), a target area of the reentrant circuit for radiofrequency current application (ie, an area of conduction between 2 anatomical obstacles such as surgical barriers and cardiac structures of electrical isolation) could be localized within the systemic venous atrium. Nine patients exhibited macroreentry, and 4 showed microreentry. In 12 patients, ART could be terminated by creating linear radiofrequency current lesions (75 degrees C, 180 to 390 s). Completeness of linear lesions after radiofrequency current delivery was proven by analyzing color-coded isopotential maps of atrial activation while applying atrial pacing techniques. The mean duration of the procedures was 286 minutes (range, 130 to 435 minutes); fluoroscopy time ranged from 7 to 33.8 minutes (mean, 17.4 minutes). CONCLUSIONS: In patients with ART after the surgical correction of congenital heart disease, the use of the noncontact mapping system allows for characterization of the tachycardia and guidance for effective radiofrequency current delivery.

Abbreviated combined anatomical/electrophysiological approach for catheter ablation of atrioventricular nodal reentrant tachycardia in children.

Atrioventricular nodal reentrant tachycardia was proven during electrophysiologic study in 41 children, aged from 3.7 to 16 years, who were referred for catheter ablation of symptomatic supraventricular tachycardia. Using an abbreviated combined anatomical and electrogram-guided approach for selective ablation of the slow pathway, a steerable ablation catheter was placed at the inferior region of the vestibule of the tricuspid valve close to the orifice of the coronary sinus, with the intention of recording a multicomponent local atrial electrogramm during sinus rhythm. If application of radiofrequency current of 500 kHz at 70 degrees C at this site did not result in a slowly accelerated junctional rhythm, at a rate of less than 120 beats per minute, the catheter was stepwise advanced up to a position midway towards the apex of the triangle of Koch for additional applications of energy. Ablation was achieved in 35 of the patients. In 6 patients, the slow pathway was modulated such that the tachycardia could no longer be induced. The number of applications of energy ranged from 1 to 19, with a median of 6 applications. The mean period of fluoroscopy was reduced to 15.6 (4.3 to 39.8) minutes, while the overall duration of the catheterization procedures ranged from 88 to 280 (mean 173.2) minutes. In none of the patients did we observe permanent high grade atrioventricular block. During follow-up over a mean of 4.1 years, two patients had recurrence of tachycardia, corresponding to a 95% rate of success in the midterm. We conclude that selective radiofrequency ablation of the slow pathway using the abbreviated anatomical and electrophysiological approach is a safe and curative therapeutic approach in children with atrioventricular nodal reentrant tachycardia. Periods required for fluoroscopy can be significantly reduced, and mid-term results are excellent.

[Atrioventricular nodal reentry tachycardia in children: curative treatment by high frequency catheter ablation]. / AV-Knoten-Reentry-Tachykardien im Kindesalter: Kurative Behandlung durch Hochfrequenzstromablation.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is one of the most common forms of supraventricular tachycardia in the pediatric population. PATIENTS AND METHODS: 41 children with a mean age of 9.6 (3.7-16) years with recurrent atrioventricular nodal reentrant tachycardia (AVNRT) refractory to medical treatment (n = 38) and recurrent syncope (n = 3) underwent electrophysiologic (EP) study. In all patients dual AV-nodal physiology could be demonstrated during EP study and typical form of AVNRT (mean heart rate 220/min) could be induced by programmed atrial stimulation. A steerable 7 F ablation catheter was placed at the inferoparaseptal region of the tricuspid valve annulus close to the orifice of the coronary sinus with the intention to record a late fractionated local atrial electrogram during sinus rhythm. Starting at this point radiofrequency current (500 kHz) with a target temperature of 70 degrees C was delivered with the intention to ablate the slow pathway. If a slowly accelerated junctional rhythm (< 120/min) occurred during energy discharge, programmed atrial stimulation was repeated. Otherwise radiofrequency current was delivered step by step up to a septal position next to the tricuspid valve annulus. Slow pathway ablation was defined as lack of evidence of dual AV nodal pathways during repeated atrial stimulation. Slow pathway modulation was defined as maximal one atrial echoimpulse after ablation. RESULTS: The number of energy applications ranged from 1-19 (median 6). In 35/41 patients slow pathway ablation could be achieved; in six patients the slow pathway was modulated. In none of the patients permanent high grade AV block was observed. During follow-up (mean 4.1 years) two patients had a recurrent episode of AVNRT after slow pathway modulation. All other patients are still free of AVNRT without medical treatment. CONCLUSION: Selective radiofrequency current ablation/modulation of the slow pathway is a safe and curative treatment of AVNRT in young patients.

[Supraventricular tachycardia in infants, children and adolescents: diagnosis, drug and interventional therapy]. / Supraventrikuläre Tachykardien bei Säuglingen, Kindern und Jugendlichen: Diagnostik--Medikamentöse und interventionelle Therapie.

Supraventricular tachycardias are the most frequent forms of symptomatic tachyarrhythmias in infants, children and adolescents. Clinical symptoms depend on age and underlying cardiac anatomy. Newborn babies and infants with paroxysmal atrioventricular reentrant tachycardias usually present with signs of congestive heart failure due to rapid heart rate. In older children and adolescents, palpitations are the leading symptom. Patients with chronic-permanent tachycardias (i.e., atrial ectopic tachycardia, permanent form of junctional reciprocating tachycardia) often develop a secondary form of dilated cardiomyopathy, the so-called "tachymyopathy". Adenosine has evolved as the drug of choice in any age group for the termination of atrioventricular reentrant tachycardia of any origin. In addition, it serves as a diagnostic tool in primary atrial tachycardias. Long-term management of atrioventricular reentrant tachycardia in infancy and childhood is age dependent. In newborn babies and infants, pharmacological therapy is advised due to the high spontaneous cessation rate of those tachycardias at the end of the first year of life. In contrast to this, the probability of spontaneous cessation of tachycardia in children > 1 year of age is very low. Therefore, radiofrequency catheter ablation of the anatomical substrate of the tachycardia is a rational alternative to long-lasting antiarrhythmic therapy. Results in children with a structurally normal heart are comparable to those achieved in adults. In patients with congenital heart disease and supraventricular tachycardias, catheter ablation during preoperative cardiac catheterization is recommended. Atrial reentrant tachycardias have been identified as one major risk factor for late postoperative morbidity and mortality in young patients. Pharmacological therapy is often not sufficient to control the tachycardia. In addition, underlying sinus node dysfunction may be aggravated in a considerable portion of the patients affected. Catheter ablation based on conventional endocardial mapping techniques by multipolar electrode catheters with the aim of identifying the critical region of the reentrant circuit is associated with an impaired success rate and a considerable recurrence rate. It may be assumed that, using the modern mapping techniques currently available (electroanatomical mapping and non-contact mapping), results of radiofrequency catheter ablation of atrial reentrant tachycardias after surgical correction of congenital heart disease will be significantly improved within the next few years.

Application of noninvasive and invasive tests for risk assessment in patients with ventricular arrhythmias.

Sudden cardiac death remains a major public health problem in western society. Because most patients who experience cardiac arrest are not successfully resuscitated, primary prevention of sudden death remains an important challenge. A number of noninvasive risk stratification techniques have been suggested as providing useful information in patients with underlying structural heart defects. Unfortunately, the positive predictive value of most of these techniques has been limited. Left ventricular ejection fraction, the presence of nonsustained ventricular tachycardia on Holter monitoring, and inducible sustained ventricular tachycardia at electrophysiologic testing in patients with coronary artery disease remain the best established prognostic test. However, with the exception of two ICD studies using the combination of these markers, prospective studies have not yet completely validated the use of these and other prognostic markers. Further understanding of the pathophysiology of ventricular fibrillation and other risk stratification techniques will be necessary before a clear algorithm can be developed for application to patients at risk for sudden death.

Effects of intracardiac radiofrequency current application on coronary artery vessels in young pigs.

INTRODUCTION: Radiofrequency current is widely used in children to ablate accessory AV pathways. Previous data in a pig model demonstrated coronary artery obstruction adjacent to radiofrequency current lesions 48 hours and 6 months after energy delivery. In the present study, the long-term effects (>6 months) of radiofrequency current application on coronary artery vessels in young pigs are assessed. METHODS AND RESULTS: Radiofrequency current (500 kHz) was delivered over 30 seconds in ten piglets (mean body weight 12.8 kg) using a steerable 6-French catheter with a 4-mm thermistor tip electrode (target temperature 75 degrees C). Lesions were created under fluoroscopic and electrocardiographic guidance at the lateral right atrial (RA) wall above the tricuspid valve orifice, and at the lateral left atrial and left ventricular wall adjacent to the mitral valve orifice. Selective coronary angiography and intravascular ultrasound (IVUS) studies were performed 3, 6, 9, and 12 months after energy application. After 12 months, the lesions were studied pathohistologically. All lesions consisted of compact fibrous tissue. RA lesions extended to the adjacent right coronary artery and led to coronary artery involvement with increased fibrous tissue in the adventitia and media and intimal thickening in three animals. Coronary arterial narrowing was documented by IVUS during follow-up in all three cases 9 months after energy application. Angiography failed to demonstrate coronary pathology in any of the three animals. CONCLUSION: The risk of late coronary artery lesions must be considered when catheter ablation at the RA wall is planned in children with free-wall accessory AV pathways.

[Early postoperative dysfunction of a modified Blalock-Taussig shunt: successful therapy with angioplasty and stent implantation in the subclavian artery]. / Frühe postoperative Dysfunktion eines modifizierten Blalock-Taussig-Shunts: Erfolgreiche Therapie mit Angioplastie und Stentimplantation in die Arteria subclavia.

A newborn with pulmonary atresia with ventricular septal defect and transposition of the great arteries was palliated with a modified Blalock-Taussig shunt at 5 days of age. Three days postoperatively arterial oxygen saturations dropped significantly. Thrombosis of the shunt was suspected. Angiography revealed stenosis of the proximal subclavian artery related to the creation of the shunt. The patient was successfully treated with angioplasty and stent implantation into the stenotic subclavian artery. Follow-up angiography 3 months after stent deployment showed a completely patent subclavian artery and unobstructed blood flow across the modified Blalock-Taussig shunt.

[Interventional therapy for hemangioendothelioma of the liver in a newborn infant using a central venous approach]. / Interventionelle Therapie eines Hämangioendothelioms der Leber bei einem Neugeborenen über einen venösen Zugang.

We report on a newborn (birth weight 3600 g) with high-output cardiac failure due to a large hepatic hemangioendothelioma requiring mechanical ventilation. One day post-partum therapy with steroids, furosemide, and digitalis was initiated. Despite this, the clinical condition deteriorated. Therefore, selective coil-embolization of the arterial vessels supplying the hemangioendothelioma was performed using a venous approach with passage to the arterial side via the ductus arteriosus. After the embolization the patient improved quickly and he was extubated 4 days postinterventionally. Follow-up sonography 4 months postinterventionally showed dramatic regression of the hepatic tumor. We conclude that the coil-occlusion of hepatic hemangioendothelioma should be performed early in symptomatic newborns as arterial puncture and its associated complications can be avoided by using a venous approach with passage to the arterial side via the ductus arteriosus. In addition, in this age group, access to the portal system can be gained through the umbilical vein to occlude portal venous supplies to the hepatic hemangioendothelioma.

Radiofrequency current application on immature porcine atrial myocardium: no evidence of areas of slow conduction after 12-month follow-up.

INTRODUCTION: Radiofrequency current (RFC) application is a widely used procedure for treatment of supraventricular arrhythmias. The purpose of this study was to investigate late electrophysiologic sequelae of RFC lesions at immature atrial myocardium in pigs, as they have not yet been systematically investigated in vitro. METHODS AND RESULTS: RFC application (temperature guided) was performed in seven piglets (mean age 6 weeks) by a steerable 6-French electrode catheter positioned at the lateral aspect of the tricuspid valve annulus. After 12 months, hearts were removed, and lesions with surrounding tissue were isolated. The viable tissue at the border of the specimen was paced with a cycle length of 500 and 600 msec. One hundred fifty impalements were performed on each specimen using capillary microelectrodes to record action potential characteristics from the lesion's surface and the surrounding tissue. In all seven specimens, no transmembrane action potentials from the fibrotic surface of each of the lesions could be recorded. The surrounding viable tissue was sharply demarcated electrically. No areas of slow conduction were detected. Action potential characteristics as mean maximum diastolic transmembrane potential, mean action potential duration at 90% repolarization, and upstroke velocity of phase 0 of the action potential were all normal. CONCLUSION: No evidence of areas of slow conduction 12 months after RFC application at immature atrial myocardium suggests that this technique is safe regarding occurrence of late atrial tachyarrhythmias after the procedure.

[Mechanical recanalization of venous thrombosis and pulmonary embolism with the Clotbuster thrombectomy system in a 12-year-old boy]. / Mechanische Rekanalisation einer Venenthrombose und Lungenembolie mit dem Clotbuster-Thrombektomie-System bei einem 12 Jahre alten Jungen.

The treatment of patients with deep vein thrombosis and pulmonary embolism with contraindications for a thrombolytic therapy is a therapeutic challenge. We report on a 12 year old patient who was treated for large cell lymphoma according to NHL-BFM 95: Block AA protocol. During his therapy, he developed a thrombosis of his right femoral vein and pulmonary embolism affecting the left segments 4, 5, 8, and 9. Because of cerebral metastasis a fibrinolytic therapy was contraindicated. Therefore, we performed a mechanical thrombectomy using the Amplatz thrombectomy device. The postinterventional scintigraphy showed a markedly improved pulmonary perfusion; dopplersonography 4 months postinterventionally showed a patent right femoral vein.

[Severe stenosis of the right coronary artery in a 15-year-old girl with type IIa hypercholesterolemia: successful treatment with stent implantation]. / Hochgradige Stenosierung der rechten Koronararterie bei einem 15jährigen Mädchen mit Hypercholesterinämie Typ IIa: erfolgreiche Behandlung durch Stentimplantation.

Coronary artery stenosis with need for therapy is rarely seen in childhood. A 15-year-old girl with hypercholesterinaemia type II a was undergoing lipid aphereses therapy (once or twice a week) since she was 6 years old. The girl was seen in our hospital with stenocardia and depression of the ST-segment in the inferior ECG leads at rest. Myocardial scintigraphy with technetium 99 showed an ischemia of the infero-lateral left ventricular myocardium. During selective coronary angiography a 90% stenosis of the proximal right coronary artery over a distance of approximately 5 mm close to the ostium was found. Post stenotic dilatation of the vessel was obvious. In addition a diffuse 10% stenosis in the proximal and middle part of the right coronary artery was found. The left coronary artery appeared angiographically normal. After balloon dilatation, stent implantation was performed without complications with a 6 mm microstent. Reduction of the stenosis from 90% to 40% could be achieved. Ticlopidin 2 x 250 mg was started for thrombocyte aggregation inhibition. During the following 12 months the patient has been free of symptoms. Lipidaphareses has been continued as before. Stent implantation seems to be a successful treatment for coronary artery stenosis also in young patients.