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Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P =0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P =0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Direct hepatic veins-to-hemiazygos connection offers the balanced distribution of hepatic venous blood to both lungs, not requiring anticoagulation. We report a 13-year follow-up after this type of off-pump Fontan completion. Patient’s hepatic veins-to-hemiazygos confluence increased with growth to allow for unobstructed flow. This unique technique can be recommended in heterotaxy patients, if atrial hepatic venous drainage and hemiazygos vein are in close proximity.

A 25-year-old patient with signs of cirrhosis on ultrasound and CT presented with portal vein thrombosis on routine follow-up examinations; retrograde hepatic wedge angiography demonstrated only the right-sided portal vein branch. Development of a portosystemic collateral vessel to the left-sided renal vein prevented signs of hypersplenism. This unique complication of portal vein thrombosis should be considered during long-term surveillance.

In der Kommentierung unserer Arbeit aus 1992 uber die chirurgische Behandlung univentrikularer Herzen zeigen wir die vorhandenen Moglichkeiten der 1980er-Jahre und die Entwicklungen auf, die sich daraus ergeben haben. Wahrend sich zwischenzeitlich der Fortschritt auf dem chirurgisch technischen Gebiet nivelliert hat, stehen heute die Langzeitprobleme der Fontan-Zirkulation und Uberlegungen zu ihrer Vermeidung im Vordergrund.

Objectives Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. Study design Our study population consisted of all patients included in the PAN study (Pravalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. Results There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures—most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. Conclusion Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

ObjectiveChronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease.Patients and methodsThe Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.ResultsGreater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child’s health-related quality of life.ConclusionsBeyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.

This chapter describes the pathology and surgical treatment of the cardiac diseases which among other parts of the heart also affect the right ventricular outflow tract. According to Richard Van Praagh, among the six distinct entities dealt with, three reveal a similar intracardiac anatomy; we consider them variants of tetralogy of Fallot (TOF): TOF/pulmonary stenosis (classical TOF), TOF/pulmonary atresia (PA), and TOF/absent pulmonary valve syndrome. In a continuous spectrum of ventricular overriding of the aorta, starting with TOF/PA on one end, passing classical TOF, double outlet right ventricle (DORV) could be considered being the other end of this anatomical spectrum of intracardiac TOF anatomy, making it number 4 TOF variant. In itself DORV may feature the whole spectrum of subpulmonary pathology or unobstructed anatomy seen in other forms of TOF. The fifth entity is pulmonary atresia with intact ventricular septum (PA/IVS) and critical neonatal pulmonary stenosis, associated with intact ventricular septum, is nowadays an interventional cardiology domain. Therefore, we do not have a separate surgical chapter. Neonatal failures of catheter intervention may go the surgical track for PA/IVS. The double-chambered right ventricle (DCRV), in simple functional terms a subpulmonary stenosis, deserves its own paragraph due to its distinct anatomical and clinical features. As for membranous subaortic stenosis, DCRV is supposed to be a congenital heart defect; however, its clinical course resembles an acquired disease.

The congenital anomalies of the great vessels are anomalies of the aorta, resulting in aortic hypoplasia/stenosis or causing compression of neighboring organs; anomalies of the pulmonary artery, resulting in pulmonary artery hypoplasia/stenosis or causing compression of neighboring organs; and abnormal congenital shortcuts involving major intrathoracic vessels, leading to a left to right shunt or a right to left shunt.

The management concepts for the pediatric postoperative cardiac surgery patient now require an interdisciplinary approach including pediatric and adult congenital cardiac surgery, cardiac anesthesia, and neonatal, pediatric, and also adult cardiac intensive care. New management strategies have been developed for postoperative complications. Hybrid techniques for primary treatment and various postoperative cardiac catheterization techniques are becoming more important.

Moderate persistent elevation of the γ-glutamyltransferase (γGT) level is a frequent finding during long-term follow-up of patients with total cavopulmonary connection (TCPC) for palliation of functionally univentricular hearts. Serial intraindividual data revealed a significant increase in the γGT level within a minimum 4-year interval in more than 80 % of cases. The level of γGT elevation showed a significant correlation to hemodynamic parameters such as systemic ventricular end diastolic pressure and mean pulmonary artery pressure, but did not strongly correlate with duration of follow-up or other liver function parameters, which were less frequent and less impressively deranged. None of the patients had signs of synthetic dysfunction. With increasing postoperative follow-up, abnormalities of sonographic hepatic texture including increased echogenicity, inhomogeneity, or liver surface nodularity were found. All 17 patients with liver surface nodularity had a follow-up period of over 10 years. Structural abnormalities did not correlate with biochemical or hemodynamic parameters. Doppler evaluation revealed inspiratory dependence of hepatic vein flow in more than 90 % as a relevant finding after TCPC; a decrease in portal vein flow velocity was observed in many patients. Since long-term survivors after Fontan procedure are at an increased risk of cardiac hepatopathy and cirrhosis, detailed routine investigation and monitoring of hepatic morphology are needed.

Heart diseases are often associated with residual injuries, persisting functional restrictions, and long-term sequelae for psychosocial development. Currently, there are no disease-specific instruments to assess the health-related quality of life (HrQoL) of pre-school children. The aims of this study were to develop a parent proxy instrument to measure the HrQoL of children aged 3–7 years with a heart disease and to confirm its validity and reliability. Items from the Preschool Pediatric Cardiac Quality of Life Inventory (P-PCQLI) were generated through focus groups of caregivers. In a pilot study, comprehensibility and feasibility were tested. Five subdimensions were defined theoretically. Psychometric properties were analysed within a multicentre study with 167 parental caregivers. The final 52-item instrument contains a total score covering five moderately inter-correlated dimensions. The total score of the questionnaire showed a very high internal consistency (Cronbachs’ α = 0.95). Test–retest correlation was at r tt = 0.96. External validity was indicated by higher correlations (r = 0.24–0.68) with a generic paediatric quality of life questionnaire (KINDL) compared to the Strengths and Difficulties Questionnaire (r = 0.17 to 0.59). Low P-PCQLI total scores were significantly associated with inpatient as opposed to outpatient treatment (t = 6.04, p

Transplantation of cryopreserved heart valves (allografts) is limited by immune responses, inflammation, subsequent structural deterioration and an expensive infrastructure. In previous studies we demonstrated that conventional frozen cryopreservation (FC) is accompanied by serious alterations of extracellular matrix (ECM) structures. As the main culprit of the observed damages ice crystal formation was identified. Objective of this study was the application principles of cryoprotection as observed in nature, occurring in animals or plants, for ice-free cryopreservation (IFC) of heart valves. Using IFC, valves were processed and stored above the glass transition temperature of the cryoprotectant formulation (-124 degrees C) at -80 degrees C to avoid any ice formation, tissue-glass cracking and preserving ECM. After implantation in the orthotopic pulmonary position in sheep, we demonstrate that IFC resulted in cell free matrices, while maintaining crucial ECM-components such as elastin and collagen, translating into superior hemodynamics. In contrast, we reveal that FC valves showed ECM damage that was not restored in vivo, and T-cell inflammation of the stroma with significant leaflet thickening. Compared to currently applied FC practice IFC also reduced infrastructural needs for preservation, storage and shipping. These results have important implications for clinical valve transplantation including the promise of better long-term function and lower costs.

Background Tetralogy of Fallot (ToF), the most frequent cyanotic congenital heart disease, is associated with a wide range of intra- and extracardiac phenotypes. In order to get further insight into genotype–phenotype correlation, a large cohort of 230 unselected patients with ToF was comprehensively investigated. Methods and results 230 patients with ToF were studied by karyotyping, comprehensive 22q11.2 deletion testing and sequencing of TBX1, NKX2.5 and JAG1 , as well as molecular karyotyping in selected patients. Pathogenic genetic aberrations were found in 42 patients (18%), with 22q11.2 deletion as the most common diagnosis (7.4%), followed by trisomy 21 (5.2%) and other chromosomal aberrations or submicroscopic copy number changes (3%). Mutations in JAG1 were detected in three patients with Alagille syndrome (1.3%), while NKX2.5 mutations were seen in two patients with non-syndromic ToF (0.9%). One patient showed a recurrent polyalanine stretch elongation within TBX1 which represents a true mutation resulting in loss of transcriptional activity due to cytoplasmatic protein aggregation. Conclusion This study shows that 22q11.2 deletion represents the most common known cause of ToF, and that the associated cardiac phenotype is distinct for obstruction of the proximal pulmonary artery, hypoplastic central pulmonary arteries and subclavian artery anomalies. Atrioventricular septal defect associated with ToF is very suggestive of trisomy 21 and almost excludes 22q11.2 deletion. We report a further patient with a recurrent polyalanine stretch elongation within TBX1 and for the first time link TBX1 cytoplasmatic protein aggregation to congenital heart defects.

Purpose Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC. Methods Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients). Results TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type ofTAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery. Conclusion Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC. The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.

UNLABELLED PURPOSE/MATERIALS AND METHODS: The aorto-pulmonary window is a rare congenital cardiac malformation consisting of a connection between the ascending aorta and the main pulmonary artery. Based on 5 consecutive patients who were treated in our tertiary referral centre between 7/2000 and 11/2005, we report the echocardiographic features as well the limitations of this diagnostic method in the assessment of this rare malformation. RESULTS Dilation of the left atrium and ventricle due to the large left-to-right-shunt was the characteristic echocardiographic feature in all patients with aorto-pulmonary window. In all patients we were able to visualise the window by 2D-echocardiography. Pulsed- and continuous-wave Doppler showed diastolic negative flow in the descending aorta and in systemic arteries due to left-to-right shunting in diastole via the aorto-pulmonary window. All 5 patients had significant associated cardiovascular malformations. They were accurately diagnosed by echocardiography. Coronary anomalies could be excluded noninvasively in all patients. CONCLUSION Echocardiography is the method of choice for diagnosis of aortopulmonary window. Detection of aortopulmonary window, however, requires a systematic and careful investigation of all cardiovascular anatomic details, since this anomaly is frequently associated with substantial additional cardiac anomalies, which would by themselves be sufficient to explain the haemodynamic abnormalities of the patient. Cardiac catheterisation is required only in cases with inadequate information about coronary artery anatomy or in older patients with pulmonary hypertension, to determine the patient's suitability for surgical correction.

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.

Objective To assess the sensitivity and specificity of echocardiography in the detection of anomalies of the subclavian artery (cervical origin, aberrant origin from the descending aorta or isolation) in patients with monosomy 22q11. Method From 1/1998 until 03/2002 we examined 57 patients with conotruncal cardiac malformations. 30 patients had pulmonary atresia and ventricular septal defect (53 %), 13 interrupted aortic arch (23 %), 9 tetralogy of Fallot (16 %) and 5 common truncus arteriosus (9 %). Echocardiographic examination included identification of the laterality of the aortic arch as well as examination of the origin of the brachiocephalic vessels. The results were compared with angiographic and intraoperative findings. Median age at echocardiographic investigation was 74 days (range 1 d - 33.4 yrs.). Results Laterality of the aortic arch was assessed correctly in all patients. 20/57 patients had a right-sided aortic arch (35 %). Echocardiography detected all anomalies of the subclavian artery (14/57 patients, 25 %, sensitivity 100 %) and excluded these anomalies correctly in 43 patients (specificity 100 %). Exact classification of the anomaly of the subclavian artery was possible in 6/6 patients with a cervical origin of the artery and in 7/8 patients with aberrant origin from the descending aorta. Monosomy 22q11 was diagnosed in 21 patients (37 %). Conclusion Echocardiography achieves a high sensitivity in the detection of anomalies of the subclavian artery. The diagnosis of cervical origin of the artery, in particular, can be easily established. As this anomaly appears to be a specific marker for monosomy 22q11, echocardiography facilitates reliable identification of these patients in clinical practice.

Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures. At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies. The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.

We report on an asymptomatic 2 1/4 year old girl. Echocardiography revealed cor triatriatum with a membrane separating the left atrium. Transesophageal echocardiography demonstrated 3 large perforations of the intraatrial membrane. Using Doppler sonography, no pressure gradients across these orifices were identified. Obstruction of the intraatrial membrane and pulmonary hypertension were excluded by cardiac catheterization. We therefore decided against surgical resection of the membrane and to follow the patient noninvasively by echocardiography. Follow-up over 18 months revealed no development of an obstruction across the intraatrial membrane. Our case shows that immediate surgical correction is not necessary in all patients with cor triatriatum. Conservative management of these patients requires full evaluation of the hemodynamics and careful follow-up examinations by echocardiography.

Aim/method The aorto-left ventricular tunnel (ALVT) is a rare congenital cardiac malformation with clinical findings of severe aortic insufficiency. We report the echocardiographic differentiation of different anatomical variants of ALVT in two infants. Results Echocardiography in both patients demonstrated severe enlargement of the ascending aorta and the left ventricle. In the first patient the ALVT originated from the ascending aorta above the right coronary sinus and entered the left ventricle just below the aortic valve. In the second patient the ALVT originated above the left coronary sinus and took a lateral course to the left ventricle. Colour-Doppler-sonography in both patients confirmed a systolic-diastolic flow across the tunnel. Many patients have associated cardiac defects. Exact determination of the morphology of the aortic valve and coronary arteries is mandatory for surgical repair. Postoperative follow-up studies focus on the function of the aortic valve and the left ventricle. Conclusion Differentiation of different anatomical variants of ALVT is possible trough echocardiography. Cardiac catheterization is required only in cases with inadequate information about coronary artery anatomy.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare anomaly which is almost always associated with hypertension in the contralateral pulmonary artery originating from the right ventricle. We report echocardiographic evaluation of an infant with tetralogy of Fallot and aortic origin of the right pulmonary artery. Since all relevant information regarding anatomy and hemodynamics could be obtained by echocardiography, cardiac catheterization and angiography were unnecessary. Surgical correction including transatrial patch closure of the ventricular septal defect, resection of the infundibular stenosis and direct reimplantation of the right pulmonary artery was performed successfully at the age of 7 weeks. The postoperative course was uneventful and persistent pulmonary hypertension was excluded by echocardiography. Our case shows that complete echocardiographic evaluation is possible in infants with this complex cardiac malformation. We recommend reserving cardiac catheterization for those patients with echocardiographic evidence of significant elevation of pulmonary vascular resistance. In order to avoid irreversible pulmonary vascular changes early surgical correction within the first 3 months of life should be performed in all children with aortic origin of a pulmonary artery including those with associated tetralogy of Fallot.

Data on mid- and long-term follow-up for the recent devices for closure of secundum-type atrial septal defects are limited. The purpose of our retrospective study was to report the effectiveness of transcatheter closure in patients with various morphological types of atrial septal defect other than centrally located defects within the oval fossa using the CardioSEAL and CardioSEAL-Starflex occluder. A total of 91 patients (age 1.5-71 years, median 6 years) underwent transcatheter closure. On the transesophageal echocardiogram, defect size varied from 6 to 18 mm with an estimated stretched diameter of 11 to 24 mm, median 15 mm; the ratio of the stretched diameter to septal length ranged from 0.28 to 0.68. Mean follow-up was 28.7 +/- 11,9 months (range 3-46 months). Isolated secundum-type defects were present in 59 patients (65%), multiple septal defects including patients with perforated atrial septal aneurysms and defects with deficient atrial rim in 32 patients (35%). Occlusion rate using device diameters from 23 to 40 mm increased from 66% (60/91 patients) immediately after implantation to 86% (48/56 patients) 24 months after implantation. Patients with isolated secundum-type defects presented with a significantly higher primary closure rate (45/59 patients, 76%) compared to patients with various defect morphology. Closure rate did not depend on the type of implanted device modification. No thrombus formation, sustained atrial arrhythmia or infective endocarditis occurred. Serial transthoracic echocardiographic findings revealed protrusion of one left-sided arm onto the right atrial aspect in 5 patients; malposition of one right-sided superior arm of the device was observed in 7 patients. Fluoroscopy showed single fatigue fracture in 7 patients (7.7%) within the first 6 months after implantation. These results demonstrate that transcatheter closure with the double umbrella device was effective and safe on medium-term follow-up and could be extended to atrial septal defects of various morphology.

Myxoma in neonatal life are extremely rare. We report a case of a neonate with a pedunculated cardiac tumour arising from the anterolateral left ventricular wall protruding across the left ventricular outflow tract and continuously extending into the distal aortic arch. Surgical removal at 14 days of age via combined transaortic approach and apical ventriculotomy was indicated because of the risk of further compromise of aortic valve function and aortic arch obstruction. Histopathologic examination was consistent with a myxoma.

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°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.

Hintergrund: AV-Knoten-Reentry-Tachykardien stellen die zweithaufigste Form supraventrikularer Tachykardien im Kindesalter dar.¶ Patienten und Methoden: Bei 41 jungen Patienten mit einem mittleren Alter von 9,6 (3,7–16) Jahren wurde aufgrund medikamentos refraktarer AV-Knoten-Reentry-Tachykardien (n=38) bzw. rezidivierender Synkopen (n=3) eine elektrophysiologische Untersuchung durchgefuhrt. Hier konnte bei allen Patienten durch programmierte Vorhofstimulation eine duale AV-Knoten-Physiologie nachgewiesen und die typische Tachykardie (slow-fast-Typ, Kammerfrequenz im Mittel 220/min) induziert werden. Ein steuerbarer 7 F Ablationskatheter wurde zunachst in der inferoparaseptalen Region nahe am Ostium des Koronarvenensinus am Trikuspidalklappenanulus plaziert. Ziel war die Registrierung eines spaten und fraktionierten lokalen atrialen Elektrogramms wahrend Sinusrhythmus. Von dieser Stelle ausgehend wurde Hochfrequenzstrom (500 kHz) mit der Zieltemperatur von 70°C zur Ablation des langsam leitenden Schenkels appliziert. Sofern wahrend der Energieabgabe ein langsam beschleunigter junktionaler Rhythmus (

Bei Vitien, die mit einer Aortenatresie einhergehen, erfolgt die Koronarperfusion in der Regel durch retrograden Blutfluss in der Aorta ascendens. Wir berichten uber zwei Patienten mit antegradem Blutfluss in der Aorta ascendens trotz Aortenatresie: Bei einem Patienten mit hypoplastischen Linksherzsyndrom (Aortenatresie, hochgradiger Mitralstenose) wurde ein intaktes interatriales Septum (vorzeitiger Verschluss des Foramen ovale) gefunden. Wahrend sich echokardiographisch und angiographisch keine anderen Wege einer linksatrialen oder -ventrikularen Dekompression fanden, zeigten sich bei der Autopsie linksventrikulare, koronare Sinusoide als alleinige pulmonalvenos-aortale Verbindung zur Oxygenation des Korperkreislaufes. Bei einem zweiten Patienten mit komplexer kongenitaler Herzerkrankung einschlieslich Aortenatresie fand sich ein antegrader Fluss in der Aorta ascendens durch eine Koronarfistel als Shuntfluss aus der Pulmonalarterie.¶ Hamodynamisch findet sich bei beiden Fallen eine Perfusion des Korperkreislaufs, die von einer retrograden Koronarperfusion bei koronararerio-venoser Fistel und myokardialen Sinusoiden abhangt und zu dem Phanomen eines antegraden Flusses in der Aorta ascendens trotz Aortenatresie fuhrt.

Objective—To report initial findings from a selected group of patients with morphological variations of the atrial septal defect who underwent transcatheter closure with a second generation redesigned double umbrella device. Patients—Two patients with abnormal location of the oval fossa and partial deficiency of the septal rim, three patients with multiple defects, and two patients with a multiperforated aneurysm of the interatrial septum (age range, 3.6-25.5 years). Methods—Defects were closed with the double umbrella device (CardioSEAL) consisting of two sets of flexible arms (with central and two mid-arm hinges) covered with sewn Dacron patches. The implantation procedure was monitored by transoesophageal echocardiography. Results—The diameter of the defect measured during transoesophageal echocardiography ranged from 7-18 mm and the balloon stretched diameter ranged from 13-21 mm. The size of the devices varied from 28-33 mm and the ratio of device size to defect size varied from 1.6-2.1. Two devices (23 and 28 mm) were chosen in a patient with two separated defects. No complications or serious arrhythmias were observed during implantation or follow up (median, 1.8 months). Residual shunting was trivial in three patients and mild in one patient (inferiorly located additional defect). Conclusions—To extend the selection critera of an isolated central interatrial defect for transcatheter closure, some modifications of the implantation technique are needed. Using the redesigned double umbrella device, effective closure in patients with multiple or irregularly shaped atrial septal defects was achieved, indicating a broadening of the spectrum of transcatheter closure. Keywords: atrial septal defects; transcatheter closure; congenital heart disorders; double umbrella device; CardioSEAL

To assess the effect of a modified Fontan operation on systemic venous blood flow and the hepatic circulation, we compared 11 patients having an atriopulmonary connection and 35 with total cavopulmonary anastomosis. The Doppler echocardiographic study of the caval venous, hepatic venous and portal venous flow was performed so as to calculate the pulsatility ratio and the variation of flow with respiration. All patients had undergone cardiac catheterization. In addition, we included specific laboratory investigations to assess function of various organs.Significantly lower maximum velocities of flow at inspiration (0.31±0.12 rn/sec vs 0.45±0.14 m/sec) and expiration (0.23±0.09 rn/sec vs 0.32±0.11 m/sec), less pulsatility (0.43 vs 0.16) and a lower ratio of systolic to diastolic velocity (1.22 vs 1.85) were found in the patients having a cavopulmonary as compared to an atriopulmonary anastomosis. Peak velocities of hepatic venous flow during inspiration and expiration were significantly lower in those with a cavopulmonary anastornosis (p=O.OOl and pThe dependence of hepatic venous flow on respiration in the presence of a chronically elevated systemic venous pressure in patients after the total cavopulmonary anastornosis may influence hepatic function in the postoperative period.

Choreoathetotische Bewegungsstorungen gehoren zu den seltenen neurologischen Komplikationen nach der Operation angeborener Herzfehler am hypothermen kardiopulmonalen Bypass. Wir berichten uber 3 praoperativ neurologisch unauffallige Patienten, bei denen fruhpostoperativ (3. bis 6. Tag) ausgepragte extrapyramidale Hyperkinesien mit Muskelhypotonie auftraten. CCT und/oder NMR schlossen eine Embolie oder Blutung aus. Wegen der starken Bewegungsunruhe wurde bei Patient 1 und 2 eine Therapie mit Tiaprid eingeleitet. Wahrend sich bei Patient 2 eine rasche Ruckbildungstendenz abzeichnete, waren bei Patient 1 und 3 extrapyramidale Bewegungsstorungen noch 48 bzw. 14 Monate postoperativ residuell nachweisbar. Diskussion: Die akute postoperative Choreoathetose tritt in unterschiedlichem Schweregrad und zumeist transient auf. Sie wird – wie bei den geschilderten 3 Fallen – im Zusammenhang mit Operationen am hypothermen Bypass und im hypothermen Kreislaufstillstand beschrieben. Bei geringer Inzidenz scheinen jedoch weitere schadigende Faktoren hinzutreten zu mussen, wie durch die zitierte Literatur dokumentiert wird.

OBJECTIVE: To investigate the specific sequelae of the Fontan operation, and particularly the potential sequelae of chronically elevated systemic venous pressure. DESIGN: A retrospective analysis of clinical and haemodynamic data and evaluation of organ function in 80 surviving patients undergoing modified Fontan operation for various forms of underlying functionally univentricular hearts. PATIENTS: 65 patients (81%) who had undergone a total cavopulmonary anastomosis and 15 an atriopulmonary anastomosis. Follow up ranged from 12 to 106 months (mean 54 (SD 23) months). RESULTS: 62 patients underwent postoperative cardiac catheterisation (mean systemic venous pressure 10.5 (2.5) mm Hg and cardiac index 3.1 (0.7) l/min/m2). Older age at operation was significantly correlated with both higher systemic venous pressure and lower cardiac index. Atrial arrhythmia was documented on Holter electrocardiogram in 17%. Protein losing enteropathy (with abnormal alpha 1-antitrypsin clearance) was found in 2/80 patients (2.5%). Ten patients had hypoproteinaemia, with a significantly higher incidence in patients after total cavopulmonary anastomosis and young age at operation. Liver function tests reflecting liver synthesis and metabolism were normal in all, whereas mild cholestasis was found in nearly 30%-predominantly in patients with a cardiac index of < 3 l/min/m2 (P = 0.045). Five patients (6.2%) developed atrial thrombosis. Coagulation factor analysis in 44 patients showed protein C deficiency in 11 (25%); laboratory signs of activation of the coagulation system were found in four of these (9%). None of the abnormal laboratory indices was significantly related to underlying cardiac malformation, postoperative systemic venous pressure, or follow up interval. CONCLUSIONS: A high proportion of clinically asymptomatic patients had abnormal laboratory findings on mid-term follow up. Detailed evaluation of organ function is necessary to detect the need for further diagnostic procedures before clinical symptoms develop.

The modified Fontan operation for definitive palliation has been performed in an increasing number of patients with various forms of a functionally univentricular heart. To eliminate the influence of different surgical techniques only patients after total cavopulmonary anastomosis (TCPA) were included in this retrospective analysis of preoperative risk factors. The age at operation in 72 patients varied from 7 to 219 months (median 54 months); 29 patients had been younger than 4 years. Forty-nine patients presented with a complex form of a functionally univentricular heart; associated systemic- or pulmonary venous anomalies were found in 22 patients, AV valve abnormalities in 42 patients with AV valve incompetence in 21 patients. Preoperative hemodynamic data revealed an elevated mean pulmonary artery pressure (> 15 mm Hg), increased pulmonary arteriolar resistance (> 3 U.m2) or end-diastolic ventricular pressure (> 12 mm Hg) in 23 patients. The overall mortality was 9.7% (7/72 patients). Variables with significant influence on postoperative mortality were associated systemic- and pulmonary venous anomalies. AV-valve incompetence and prolonged cardio-pulmonary bypass time. Age at operation, preoperative pulmonary arteriolar resistance, systemic- and pulmonary blood flow did not influence postoperative mortality. Even in patients with a complex form of a univentricular heart with associated anomalies and borderline preoperative hemodynamics TCPA can be performed with an acceptable risk.

Thrombosis of the right side of the heart has previously been a rarely reported early or late postoperetive complication after a modified Fontan-procedure.In 4(5.5%) of 72 surviving patients who underwent a total cavopulmonary connection for various forms of univentricular atrioventricular connection or other complex lesion a thrombus within the right atrium was diagnosed by transthoracic echocardiogaphy either early (in 3 cases) or late (in one cases) postoperatively. Predisposing haemodynamic abnormalities could be identified in all four patients, but no patient was found to have severe abnormalities of coagulation. Two patients presented with acute clinical symptoms on the third and twelfth day after initial surgery. They underwent emergency reoperation with thrombectomy followed by prophylactic treatment with coumadin or aspirin after initial heparinization. In the other 2 patients, routine postoperative echocardiography revealed a right atrial thrombus without specific clinical symptoms 25 days or 3 months postoperatively. Thrombolytic therapy with an initial bolus of tissue plasminogen activator (0.1 mg/kg) followed by continuous infusion (1 mg/kg/day) combined with heparinization yielded complete resolution of the thrombus after 27 and 40 hours, respectively, as demonstrated on follow-up echocardiograms. Our own experience, coupled with a review of the literature, indicate that prophylactic anticoagulation therapy with coumadin should continue for at least 4 to 6 months for patients with an increased risk for postoperative thrombosis. For infants and young children with a low risk for postoperative thrombosis, aspirin should be given routinely for 6 months postoperatively.

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 >20mm 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 30mm 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.

Long-term follow-up studies after intraatrial repair for complete transposition have shown postoperative morphologic and hemodynamic abnormalities in many patients which often require reevaluation and therapeutic intervention. The purpose of this study was to assess, using magnetic resonance imaging, the presence and extent of late postoperative complications in a large cohort of 44 patients undergoing repair with the Mustard procedure. Of these 34 had an intact ventricular septum with or without obstruction of the left ventricular outflow tract, while 10 had an associated ventricular septal defect. Transverse spin-echo and gradient-echo images were acquired of the entire heart from the diaphragm to the bifurcation of the pulmonary trunk. Additional oblique images were acquired for better visualization of venous connections. Abnormal findings were diagnosed by visual inspection of spin-echo and gradient-echo images, and diagnoses were compared to previous findings at cardiac catheterization. On magnetic resonance imaging, a baffle leak was seen in eight patients, two had pulmonary venous obstruction, four had obstruction at the caval veins, 23 had obstruction of the left ventricular outflow tract and 18 had tricuspid regurgitation. Cardiac catheterization showed a leak across the baffle in 16, pulmonary venous obstruction in two, obstruction at the caval veins in four, obstruction of the left ventricular outflow tract in nine, and tricuspid regurgitation in nine. We conclude that combined spin-echo and gradient-echo magnetic resonance imaging provides extensive noninvasive assessment of postoperative sequels and residuals in patients after the Mustard procedure for complete transposition.

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.

In the majority of patients, secundum atrial septal defects (ASDs) are treated interventionally or surgically, before the onset of clinical symptoms, between 3 and 6 years of age. Because right-ventricular dimensions usually normalize after ASD closure, it has been assumed that cardiac function and exercise performance also normalize at long-term follow-up. The aim of our study was to determine cardiac index (CI) at rest and during exercise at medium-term follow-up of children who had undergone surgical or interventional closure of ASD because no such reports have been published thus far. Seventeen patients (age range 8.8–17.3 years) who underwent surgical correction were included together with 17 subjects who received an interventional procedure with Amplatzer and Helex occluders (age range 12.2–17.3 years). The study was performed after a median interval of 8.6 years (range 6.5–11.6) after the procedure. Twelve healthy children of comparable age served as controls. CI measurements were performed based on the inert gas-rebreathing method with the Innocor system. For exercise testing, the standard treadmill protocol of the German Society of Pediatric Cardiology was used. CI, stroke volume (SV), and heart rate (HR) were determined at rest and at two standardized submaximal exercise levels (levels 3 and 6). CI increased in all subjects under exercise conditions. Neither SV nor HR displayed significant differences between the three groups either at rest or under exercise conditions. Although HR increased continuously, no increase of indexed SV occurred beyond level 3. Noninvasive determination of CI at rest and during exercise with the IGR method is feasible in the pediatric age group. At medium-term follow-up, we found no significant differences between patients who underwent surgical or interventional ASD closure compared with normal controls.

Objective To obtain information on health and quality of life in adults with Noonan syndrome. Study design From a cohort of 144 children with the diagnosis of Noonan syndrome whose height data had been published 23 years ago, 103 pediatric files providing adequate data were identified. Participants were sent questionnaires and asked to provide saliva for DNA analysis and to return for physical examination. Results Ten of 103 individuals had died, 3 of them suddenly (standardized mortality ratio, 3.00; 95% CI, 1.44-5.52). Eighty-one individuals could be contacted by mail, with a positive response from 45. Genotyping in 36 of 45 participants revealed characteristic mutations in 61%. Median age at follow-up was 42.8 years. Mean adult heights were 169.2 cm (men) and 154.4 cm (women). In comparison with the general population, participants had lower educational status and lived more frequently without any partner. According to the response to the Short Form-36 questionnaire, quality of life was not impaired. Conclusions Individuals with Noonan syndrome have higher mortality, lower education, and rarely partnership. Quality of life according to self-reported Short Form-36 was good. Men grew taller than previously reported from this cohort.

Cardiotocography and echocardiography are currently standard for fetal heart monitoring. However, both do not provide adequate temporal resolution to measure fetal cardiac time intervals and detect arrhythmias, which can occur during normal sinus rhythm. Fetal magnetocardiography (fMCG) is a non-invasive technique measuring magnetic signals generated by fetal heart activity. Most fMCG devices are installed in research institutions limiting the implementation of this method in a clinical setting. Several institutions made a step forward by installing devices, in particular for fetal investigations, in hospital sites to evaluate the clinical benefit. Based on instrumentation differences which can affect signal quality, there is still no established reference database for fetal cardiac time intervals. A new magnetograph dedicated to fetal recordings was implemented with improved patient comfort. The setting was optimized to establish a standard. A total of 103 healthy fetuses starting as early as possible after the first trimester were recorded and fMCG values of cardiac time intervals were compared to former studies. Data allowed high and reliable detection for all fMCG components starting at 17 weeks. The data were comparable to fMCG multicenter studies, fetal electrocardiography and neonatal ECG results and could serve as a database of norm values for further investigation of fetal arrhythmias.

The purpose of this study was evaluation of an ice-free cryopreservation method for heart valves in an allogeneic juvenile pulmonary sheep implant model and comparison with traditionally frozen cryopreserved valves. Hearts of 15 crossbred Whiteface sheep were procured in Minnesota. The valves were processed in South Carolina and the pulmonary valves implanted orthotopically in 12 black faced Heidschnucke sheep in Germany. The ice-free cryopreserved valves were cryopreserved in 12.6 mol/l cryoprotectant (4.65, 4.65, and 3.31 mol/l of dimethylsulfoxide, formamide and 1,2-propanediol) and stored at −80°C. Frozen valves were cryopreserved by controlled slow rate freezing in 1.4 mol/l dimethylsulfoxide and stored in vapor-phase nitrogen. Aortic valve tissues were used to evaluate the impact of preservation without implantation. Multiphoton microscopy revealed reduced but not significantly damaged extracellular matrix before implantation in frozen valves compared with ice-free tissues. Viability assessment revealed significantly less metabolic activity in the ice-free valve leaflets and artery samples compared with frozen tissues (P