Differences in right ventricular-pulmonary vascular coupling and clinical indices between repaired standard tetralogy of Fallot and repaired tetralogy of Fallot with pulmonary atresia.

PURPOSE: The purpose of this study was to compare ventricular vascular coupling ratio (VVCR) between patients with repaired standard tetralogy of Fallot (TOF) and those with repaired TOF-pulmonary atresia (TOF-PA) using cardiovascular magnetic resonance (CMR). MATERIALS AND METHODS: Patients with repaired TOF aged>6 years were prospectively enrolled for same day CMR, echocardiography, and exercise stress test following a standardized protocol. Sanz's method was used to calculate VVCR as right ventricle (RV) end-systolic volume/pulmonary artery stroke volume. Regression analysis was used to examine associations with exercise test parameters, New York Heart Association (NYHA) class, RV size and biventricular systolic function. RESULTS: A total of 248 subjects were included; of these 222 had repaired TOF (group I, 129 males; mean age, 15.9±4.7 [SD] years [range: 8-29 years]) and 26 had repaired TOF-PA (group II, 14 males; mean age, 17.0±6.3 [SD] years [range: 8-29 years]). Mean VVCR for all subjects was 1.54±0.64 [SD] (range: 0.43-3.80). Mean VVCR was significantly greater in the TOF-PA group (1.81±0.75 [SD]; range: 0.78-3.20) than in the standard TOF group (1.51±0.72 [SD]; range: 0.43-3.80) (P=0.03). VVCR was greater in the 68 NYHA class II subjects (1.79±0.66 [SD]; range: 0.75-3.26) compared to the 179 NYHA class I subjects (1.46±0.61 [SD]; range: 0.43-3.80) (P<0.001). CONCLUSION: Non-invasive determination of VVCR using CMR is feasible in children and adolescents. VVCR showed association with NYHA class, and was worse in subjects with repaired TOF-PA compared to those with repaired standard TOF. VVCR shows promise as an indicator of pulmonary artery compliance and cardiovascular performance in this cohort.

Multicentre reference values for cardiac magnetic resonance imaging derived ventricular size and function for children aged 0-18 years.

AIMS: Cardiovascular magnetic resonance (CMR) imaging is an important tool in the assessment of paediatric cardiac disease. Reported reference values of ventricular volumes and masses in the paediatric population are based on small cohorts and several methodologic differences between studies exist. We sought to create steady-state free precession (SSFP) CMR reference values for biventricular volumes and mass by combining data of previously published studies and re-analysing these data in a standardized manner. METHODS AND RESULTS: A total of 141 healthy children (68 boys) from three European centres underwent cine-SSFP CMR imaging. Cardiac structures were manually contoured for end-diastolic and end-systolic phases in the short-axis orientation according to current standardized CMR post-processing guidelines. Volumes and masses were derived from these contours. Age-related reference curves were constructed using the lambda mu sigma method. Median age was 12.7 years (range 0.6-18.5). We report biventricular volumes and masses, unindexed and indexed for body surface area, stratified by age groups. In general, boys had approximately 15% higher biventricular volumes and masses compared with girls. Only in children aged <6 years old no gender differences could be observed. Left ventricle ejection fraction was slightly higher in boys in this study population (median 67% vs. 65%, P = 0.016). Age-related reference curves showed non-linear relations between age and cardiac parameters. CONCLUSION: We report volumetric SSFP CMR imaging reference values for children aged 0-18 years old in a relatively large multi-centre cohort. These references can be used in the follow-up of paediatric cardiac disease and for research purposes.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

[Consensus recommendations of the German Radiology Society (DRG), the German Cardiac Society (DGK) and the German Society for Pediatric Cardiology (DGPK) on the use of cardiac imaging with computed tomography and magnetic resonance imaging]. / Konsensusempfehlungen der DRG/DGK/DGPK zum Einsatz der Herzbildgebung mit Computertomografie und Magnetresonanztomografie.

Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) have been developed rapidly in the last decade. Technical improvements and broad availability of modern CT and MRI scanners have led to an increasing and regular use of both diagnostic methods in clinical routine. Therefore, this German consensus document has been developed in collaboration by the German Cardiac Society, German Radiology Society, and the German Society for Pediatric Cardiology. It is not oriented on modalities and methods, but rather on disease entities. This consensus document deals with coronary artery disease, cardiomyopathies, arrhythmias, valvular diseases, pericardial diseases and structural changes, as well as with congenital heart defects. For different clinical scenarios both imaging modalities CT and MRI are compared and evaluated in the specific context.

[Preoperative and postoperative imaging in patients with transposition of the great arteries]. / Prä- und Postoperative Bildgebung bei Patienten mit Transposition der großen Gefäße.

Transposition of the great arteries (TGA) is a rare disease representing not more than 3-5% of all congenital heart diseases. TGA is a cardiac anomaly in which the aorta arises entirely or largely from the morphological right ventricle and the pulmonary artery from the morphological left ventricle. This is called a ventriculo-arterial discordant connection and when accompanied by an atrio-ventricular concordant connection it is called a complete or D-transposition (D-TGA). The terms congenitally corrected TGA (ccTGA) or L-TGA describe an atrio-ventricular discordant connection. In D-TGA survival can only be achieved if additional shunting is simultaneously present, which possibly has to be created post-natal by the so-called Rashkind maneuver.Nowadays, an early anatomic correction using the arterial switch operation is the treatment of choice. Up to the 1980s, an atrial switch operation according to Senning/Mustard was performed. Apart from echocardiography the imaging modality of choice is usually MRI to assess the complex postoperative anatomy, viability of the myocardium and to perform a volumetric and functional assessment, including MR flow measurements. Multidetector computed tomography (MDCT) is used if there are contraindications to MRI or if an assessment of cardiac and especially coronary anatomy is the main interest.

[Organization of clinical research: in a large scale department for cardiothoracic surgery]. / Organisation der klinischen Forschung: In der Herzchirurgie an einer grossen chirurgischen Klinik.

Translation of basic research results into routine patient care is delayed in parts by lack of institutionalization in clinical research. In this article the research structure and organization of our Department of Cardiac, Thoracic, Transplantation and Vascular Surgery are described.Basic research, separately directed, is accomplished in the Leibniz Research Laboratories for Biotechnology and Artificial Organs (LEBAO) and within the scope of the Excellence cluster "REBIRTH--from Regenerative Biology to Reconstructive Therapy".Clinical research is directed by heads of the subdepartments of our institution (valve and coronary surgery, aortic surgery, surgical electrophysiology, vascular surgery, thoracic surgery, cardiac assist systems, thoracic transplantation, intensive care and pediatric heart surgery).A separate subdepartment for clinical research is responsible for study coordination and accompanies clinical studies from study design and patient screening to publication. This subdepartment also serves as a constant contact to sponsors and superordinated research organizations within the Hannover Medical School.

Bovine jugular veins in the pulmonary position in adults -- 5 years' experience with 64 implantations.

OBJECTIVE: The midterm durability of bovine jugular veins (BJV) in children is comparable to that of homografts. We present the results of 64 bovine jugular vein implantations in adults in a pulmonary position. METHODS: Between August 2003 and July 2008, 60 patients (aged 18 to 65 years) received 64 BJVs. 97 % of them had had previous reconstructions of the right ventricular outflow tract. Diagnoses for treatment included tetralogy of Fallot (n = 49 patients), other congenital malformations (n = 14), and Ross operation (n = 1). Four bovine jugular veins had a diameter of 20 mm, the others had a diameter of 22 mm. RESULTS: Survival after 5 years was 98.4 +/- 1.6 %. Freedom from endocarditis: 84.8 +/- 8.0 % (4 patients required explantation for endocarditis); freedom from explantation for structural valve degeneration: 96.2 +/- 2.6 % (2 patients required explantation); freedom from intervention: 93.2 +/- 2.8 % (3 patients required intervention); moderate insufficiency: 62.9 +/- 15.1 % (8 patients); gradient > or = 50 mmHg: 79.6 +/- 7.4 % (7 patients); degeneration: 56.4 +/- 12.9 % (11); any adverse event: 43.1 +/- 12.8 % (15 patients); calcification or aneurysmal dilatation: 100 %. At any postoperative interval, more than 75 % of the BJVs had neither been explanted nor were they degenerated. CONCLUSION: The BJV might be a promising alternative to homografts, also in adults. Strict antibiotic prophylaxis is mandatory. A prospective randomized multicenter comparison of homografts and BJVs would help to identify the preferable conduit.

Value of postmortem magnetic resonance imaging for fatal neonatal congenital heart disease: a case report.

Autopsies after neonatal death due to suspected or insufficient diagnosed cardiac malformations provide important information to parents and the involved obstetric and pediatric physicians. The autopsy rate, however, is declining. Postmortem magnetic resonance imaging may be an alternative option for selected cases, providing detailed morphologic information about the cardiovascular system. This case report demonstrates the beneficial use of postmortem magnetic resonance imaging in a newborn with a suspected complex cardiac malformation. The magnetic resonance imaging was able to complete the diagnosis of situs inversus totalis, with transposition of great arteries, and to provide valuable information about the underlying physiology.

Terbutaline-induced downregulation of beta 2-adrenoceptors without Gi-protein alterations in human lymphocytes.

Recent evidence suggests that agonist-induced desensitization of Gs protein-coupled beta-adrenoceptors is accompanied by sensitization of Gi protein-coupled receptors and/or an increase in Gi protein. To find out whether such "cross-regulation" between Gs protein- and Gi protein-coupled receptors can be also demonstrated in vivo in humans, we studied the effects of a 2 week treatment of eight male volunteers with the beta 2-adrenoceptor agonist terbutaline (3 x 5 mg/day) on beta 2-adrenoceptor density and Gi-protein content in lymphocytes and on alpha 2-adrenoceptor density (Gi-coupled receptors) in platelets. Terbutaline decreased the lymphocyte beta 2-adrenoceptor density by about 30%, but had no significant influence on lymphocyte Gi-protein levels (assessed by pertussis toxin-catalyzed [32P]ADP ribosylation) or on platelet alpha 2-adrenoceptor density. We conclude that circulating blood cells are not suitable to demonstrate in humans in vivo a "cross-regulation" between Gs- and Gi-coupled beta- and alpha-adrenoceptors.