Matthias Gass

DDD pacemaker implantation after Fontan-type operations.

HEINEMANN, M.K., et al.: DDD Pacemaker Implantation After Fontan‐Type Operations. Bradyarrhythmias developing after Fontan‐type operations impair the function of the univentricular heart causing fatigue, headaches, ascites, and protein‐losing enteropathy (PLE). Transvenous inaccessibility, requiring epicardial implantation, accounts for the reluctance to implant a pacemaker (PM). Between 1997 and 2000, 24 patients (mean age 9.5 years, range 6 months to 19 years) with Fontan‐type operations received DDD pacing systems with atrial steroid‐eluting stitch‐on electrodes (mean capture threshold 1.9 V/0.5 ms, range 0.4–3.5 V) and ventricular screw‐in electrodes (mean capture threshold 1.7 V/0.5 ms, range 0.1–3 V). The systems were implanted at the time of conversion from atrio‐ to cavopulmonary connections in 5 patients, at the time of a total cavopulmonary Fontan operation in 6, and 1–50 months thereafter (mean = 18) in 13 patients. A right ventricular anatomy was present in 13 (54%) of 24 of PM recipients, versus 35% of the overall population. After a mean follow‐up of 3.5 years, the PM were functioning in DDD mode in 23 of the 24 patients. Length of hospital stay in the ten patients who underwent repeat sternotomy was 5 days, without procedure related complications. In three children a repeat sternotomy was avoided by implanting the atrial electrodes during the Fontan operation. All patients improved clinically, including resolution of PLE in four patients. Bradyarrhythmias may lead to significant morbidity after Fontan‐type operations. Electrophysiological evaluation is advised at follow‐up. The indication for implantation of a DDD pacemaker system should be liberal. Placing atrial electrodes during the Fontan operation, especially in the presence of a right ventricular anatomy, avoids repeat sternotomy. (PACE 2003; 26[Pt. II]:492–495)

Use of the implantable loop recorder in children and adolescents.

INTRODUCTION Recurrent but infrequent syncopes represent a diagnostic challenge, since they frequently remain unexplained despite extensive investigations. This applies specifically for patients who carry an increased risk of potentially lifethreatening arrhythmias, either due to congenital cardiac disease or primary electrical disorders. Implantable loop recorders permit long-term electrocardiographic monitoring. Experience with these devices is still limited in children. PATIENTS AND METHODS Between January 1999 and August 2005, 12 patients underwent implantation of a loop recorder in our tertiary referral centre. The mean age was 10.9 years, with a range from 2 to 17 years. Of the patients, 6 had structural disease, 3 had primary electrical abnormalities, and 3 had no cardiovascular disease. RESULTS Resyncope occured in 9 of the 12 patients. Arrhythmic origin of the syncope was diagnosed in 4 of these patients. The events recorded were ventricular fibrillation in 2, intermittent asystole in 1, and pacemaker-syndrome in the other patient. Malignant arrhythmia was ruled out in the remaining 5 patients. There were no complications related to implantation of the loop recorder, and the mean duration until explantation was 8.3 months. CONCLUSIONS Based on our experience, we suggest that implantation of a loop recorder represents an additional tool for a selected group of children. Due to its invasive nature, it should be restricted to patients at high risk, or those in which there is substantial clinical suspicion of the likelihood of serious arrhythmias when conventional testing has been inconclusive. In this cohort, implantation of the loop recorder either helps to establish the correct diagnosis, or to exclude an arrhythmic event, thus avoiding unnecessary escalation of therapy and providing reassurance for the family.

The diagnostic yield from implantable loop recorders in children and young adults.

BackgroundSyncope and palpitations occur frequently in young patients. Noninvasive diagnostic testing may be inconclusive.AimTo assess the diagnostic yield of implantable loop recorders in young patients.Patients and methodsThirty-three young patients underwent implantation of a loop recorder for long-term monitoring of cardiac rhythm, to establish symptom–rhythm correlation. They belonged to one of three subgroups: those with structurally normal heart, normal electrocardiogram at rest, and negative family history (n = 16); patients with structural heart disease and previous surgical repair (n = 11), and patients with proven or suspected primary electrical disease (n = 6). A combination of automatic and patient-activated recordings was used to monitor cardiac rhythm during symptomatic episodes.ResultsThere were no procedural complications. Diagnostic electrograms could be obtained in all patients. A high degree of symptom–rhythm correlation was established. In 8/33 patients, no recurrence of symptoms was observed either until end of battery life of the device (n = 4) or until last follow-up (n = 2). Specific cardiac therapy was required, based on rhythms recorded by the device in 15 patients (until last follow-up). This consisted of catheter ablation of a tachyarrhythmia (n = 7), pacemaker implantation or upgrade (n = 5) or ICD implantation (n = 5). In the remaining patients (n = 10), recurrence of symptoms was associated with a normal electrocardiogram, and in two of these patients a non-cardiac diagnosis was made.ConclusionsIn selected patients, the implantable loop recorder provides valuable diagnostic information to guide further therapy.

Anomalous origin of left coronary artery in young athletes with syncope

Possible causes of exercise induced syncope or sudden death in young athletes include undetected cardiomyopathies, myocarditis, arrhythmias (especially inherited arrhythmia syndromes—such as long QT syndrome, Brugada syndrome), coronary artery abnormalities and disease, and aortic rupture.1 2 3 Coronary artery anomalies are of special interest as they are potentially treatable lesions.4 5 The most frequent coronary artery anomaly is anomalous origin of the left coronary or the left anterior descending artery from the right coronary sinus of the aorta. According to a recent north Italian study, anomalous origin of left or right coronary artery from the opposite aortic sinus is the third most frequent cardiovascular cause of deaths associated with exercise among athletes aged 12-35 years.2 Fig 1 Coronary artery anatomy in case 1 as shown by echocardiography from the parasternal short axis: both the right and left coronary arteries originate from the right aortic sinus. The main left coronary artery courses between the aorta and pulmonary artery and divides into left anterior descending artery and left circumflex branch We report on two adolescents who presented with a history of recurrent exertional pain and syncope during exercise. The results of previous cardiological evaluation—including 12 lead electrocardiography, exercise testing, and echocardiography—had been normal. Both patients were admitted to our hospital after life threatening events of ventricular fibrillation. The diagnosis of the coronary artery anomaly was detected in both patients by transthoracic echocardiography. These two cases show that this potentially fatal anomaly cannot be excluded by a negative exercise test.1 4 ### Case 1 This 15 year old boy was a talented, competitive soccer player with a history of recurrent syncope during soccer training at the age of 6, 7, and 11. Previous …

Surgical correction of scoliosis in children with congenital heart failure (Fontan circulation): case report and literature review

Patients with Fontan circulation represent a significant cardiorespiratory risk during spinal surgery. We report about two patients with severe scoliosis and Fontan circulation and their successful operative treatment. The case report will be compared with the national and international literature.

Radiofrequency ablation of right ventricular outflow tract tachycardia using a magnetic resonance 3D model for interactive catheter guidance

Stephan Miller, MD Department of Diagnostic Radiology, University of Tuebingen, Tuebingen, Germany Sirs: Radiofrequency catheter ablation is an accepted therapy for idiopathic ventricular tachycardia nonresponsive to medical management [1–4]. The Ensite 3000 non-contact mapping system (Endocardial Solutions Inc., St. Paul, MN) has been used previously in the management of various arrhythmias [1, 5–7]. In this report the initial experience with the noncontact mapping system in combination with a high resolution three-dimensional surface rendered magnetic resonance (3D MR) model for clear definition of origin and course of a superior right ventricular outflow tract tachycardia is presented.

Recanalisation of bilateral superior vena cava after total cavopulmonary connection

Systemvenöse Kollateralgefäße werden häufig nach Glenn- oder modifizierter Fontan-Operation beobachtet. Therapie der Wahl zum Verschluss kleinkalibriger Gefäße ist die Embolisation mit Hilfe von replatzierbaren Metallspiralen. Etwas problematischer ist die Situation bei Patienten mit großen Kollateralen wie z. B. einer rekanalisierten bilateralen oberen Hohlvene. Wir berichten über zwei Patienten, bei denen mehrere Jahre nach Fontan-Operation bei zunehmender Zyanose eine rekanalisierte linke bzw. rechte obere Hohlvene diagnostiziert wurde. Bei beiden Patienten gelang der interventionelle Verschluss dieser großlumigen Venen mittels 8 mm Amplatzer®-Muscular-VSD-Occluder, die arteriellen Sauerstoffsättigungen stiegen bei beiden Patienten postinterventionell an. Der Verschluss rekanalisierter oberer Hohlvenen nach Fontan’scher Kreislauftrennung ist mittels Amplatzer®-Muscular-VSD-Occluder technisch ohne Probleme möglich. Um die erneute Formation von venovenösen Kollateralen zu vermeiden muss darauf geachtet werden, dass der Okkluder in die V. cava knapp unterhalb der Mündung der V. azygos/hemiazygos platziert wird. Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalised bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer® muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. The closure of recanalised bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer® muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

Recanalisation of bilateral superior vena cava after total cavopulmonary connection. Interventional occlusion with the Amplatzer VSD Occluder.

Systemvenöse Kollateralgefäße werden häufig nach Glenn- oder modifizierter Fontan-Operation beobachtet. Therapie der Wahl zum Verschluss kleinkalibriger Gefäße ist die Embolisation mit Hilfe von replatzierbaren Metallspiralen. Etwas problematischer ist die Situation bei Patienten mit großen Kollateralen wie z. B. einer rekanalisierten bilateralen oberen Hohlvene. Wir berichten über zwei Patienten, bei denen mehrere Jahre nach Fontan-Operation bei zunehmender Zyanose eine rekanalisierte linke bzw. rechte obere Hohlvene diagnostiziert wurde. Bei beiden Patienten gelang der interventionelle Verschluss dieser großlumigen Venen mittels 8 mm Amplatzer®-Muscular-VSD-Occluder, die arteriellen Sauerstoffsättigungen stiegen bei beiden Patienten postinterventionell an. Der Verschluss rekanalisierter oberer Hohlvenen nach Fontan’scher Kreislauftrennung ist mittels Amplatzer®-Muscular-VSD-Occluder technisch ohne Probleme möglich. Um die erneute Formation von venovenösen Kollateralen zu vermeiden muss darauf geachtet werden, dass der Okkluder in die V. cava knapp unterhalb der Mündung der V. azygos/hemiazygos platziert wird. Formation of systemic to pulmonary venous or systemic venous left atrial collaterals frequently occurs in patients after Glenn or Fontan-type operations. Embolization with detachable metal coils is the therapy of choice for the closure of small vessels. These devices however are not appropriate for the occlusion of large collaterals, e. g. recanalised bilateral caval veins. We report two patients who presented late after Fontan-type operations with a gradual decrease in oxygen saturation due to recanalisation of bilateral caval veins. Interventional closure of these large veins was carried out successfully with the use of 8 mm Amplatzer® muscular VSD Occluders, resulting in an increase of arterial oxygen saturations. The closure of recanalised bilateral superior caval veins after Fontan procedures is possible without technical problems by means of the Amplatzer® muscular VSD Occluder. In order to avoid future formation of venous collaterals via the azygos or hemiazgos system, the occluder should be placed in the vena cava below the orifice of the azygos/ hemiazygos vein.

Atriale Tachykardie, Vorhofflattern, Vorhofflimmern: Kurative Therapie durch fokale Ablation in einer Pulmonalvene

Wir beschreiben den Fall einer 50-jährigen Patientin mit der klinischen Diagnose einer Kardiomyopathie bei medikamentös therapierefraktärer Tachyarrhythmia absoluta. Deren elektrokardiographisches Korrelat bestand aus Episoden mit atrialer Tachykardie, Vorhofflattern und Vorhofflimmern. Die Symptome und Befunde ließen sich allesamt ursächlich auf eine fokale Tachykardie mit Ursprung in der linken oberen Pulmonalvene zurückführen. Ihre erfolgreiche Ablation stellte eine kurative antiarrhythmische Therapie dar, die auch zur Normalisierung der Ventrikel-Funktion führte. Eine AV-Knoten-Ablation mit Schrittmacher-Implantation konnte so vermieden werden. We describe the case of a 50-year-old woman with the clinical diagnosis of cardiomyopathy associated with supraventricular tachycardia refractory to pharmacological treatment. The totally irregular tachyarrhythmia was the result of different episodes of atrial tachycardia, atrial flutter and atrial fibrillation that could be identified in the surface ECG. These findings and the patient’s symptoms were all caused by a single focal tachycardia originating from the left upper pulmonary vein. Ablation of this focus represented a curative antiarrhythmic therapy also restoring a normalized ventricular function. Thus, an ablation of the AV node with consecutive pacemaker implantation could be prevented.

Babybodyplethysmography in infants and children with congenital heart disease and pulmonary hypertension

Abstract We investigated pulmonary mechanics in 46 children (27 males, 19 females) with congenital heart defects with left-to-right shunt and pulmonary hypertension. Patients ranged in age from 6 weeks to 4 years. Babybodyplethysmography studies were performed before corrective heart surgery, and again at 2 weeks and 6 months after surgery. Preoperative pulmonary function data were compared to hemodynamic data obtained during cardiac catheterization: pulmonary artery pressure, oxygen saturation, and ratios of pulmonary to systemic blood flow (Qp/Qs) and vascular resistance (Rp/Rs). Airway resistance was elevated before surgery to 148% of predicted and it dropped significantly to normal levels, 104% of predicted, only 6 months after surgery. Functional residual capacity (FRCpleth) was increased to 127% of predicted before surgery and normalized significantly to 101% of predicted within the same time period. On the other hand, breathing frequency and minute volume, indexed to body weight, decreased significantly in the 2 weeks after surgery. Statistical analysis of the hemodynamic data and lung function tests showed only a poor linear correlation between functional residual capacity and oxygen saturation in the pulmonary artery ( r =0.26–0.37) and the Rp/Rs ratio ( r =0.38). Airway resistance also correlated poorly with the Qp/Qs and Rp/Rs ratios (both r =0.29). In young children with heart defects with increased pulmonary blood flow and pulmonary hypertension, lung mechanics are abnormal leading to bronchial obstruction and hyperinflation. Additional studies of dynamic lung compliance and elastance will be needed to look for interstitial lung alterations in these patients.