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Michael Hofbeck

Publications and source records attributed to Michael Hofbeck.

At least 19 recordsLinked to original sources
PubMed2006

Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT.

BACKGROUND: Precise visualization of the pulmonary vasculature is mandatory for adequate treatment of patients with pulmonary atresia and ventricular septal defect (PA-VSD). Aortopulmonary collateral arteries (APCs) can be visualized by selective injections of contrast agent in the catheterization laboratory. OBJECTIVE: To evaluate multidetector CT (MDCT) and different image postprocessing methods for analysis of complex pulmonary blood supply in patients with PA-VSD. MATERIALS AND METHODS: Eight patients (6 weeks to 27.8 years of age) with PA-VSD and APCs underwent MDCT and cardiac catheterization. Using multiplanar reformatting, volume rendering and semiautomatic segmentation algorithms, the aorta, pulmonary arteries and APCs were displayed. MDCT and cardiac catheterization were analyzed by two independent observers. RESULTS: MDCT accurately imaged central pulmonary arteries (n=8), aortopulmonary shunts (n=2), right ventricular to pulmonary artery conduits (n=2) and origin, course and intrapulmonary connections of APCs (n=25), compared to X-ray angiography. A high correlation was found between the MDCT vessel diameter measurements by two independent observers (n=70, r=0.96, P<0.01) and between MDCT and angiographic vessel diameter measurements (n=68, r=0.96, P<0.01). CONCLUSIONS: Using three-dimensional imaging software, a complex pulmonary blood supply can be non-invasively and accurately imaged with high-resolution MDCT. This technique may help to reduce the number of cardiac catheterizations or guide interventional or surgical therapy.

Adolescent↗
PubMed2006

Transcatheter occlusion of a large intercostal side-branch of left internal mammary artery bypass with detachable platinum coils.

Intercostal branches of the internal mammary artery (IMA) are usually tied off during IMA-bypass surgery. Some side-branches may be missed, however, due to anatomical variants or during minimal invasive procedures with limitation of the surgeon's ability to ligate proximal branches. There are a number of reports in the literature describing interventional closure of side-branches using Gianturco coils. Following embolization or malposition, however, these coils may be extremely difficult to retrieve from coronary arteries. We report about interventional embolization of a IMA side-branch with detachable micro-coils in a patient with symptomatic coronary steal. Detachable coils are safer than Gianturco coils and are an effective method to abolish symptomatic coronary steal due to unligated intercostal branches of the IMA graft.

Cardiac Catheterization↗
PubMed2006

Recombinant human deoxyribonuclease shortens ventilation time in young, mechanically ventilated children.

Recombinant human deoxyribonuclease I (dornase alfa) is currently used as an inhaled mucoactive agent in the treatment of cystic fibrosis. In a randomized, placebo-controlled, double-blind clinical study in 100 infants, we investigated whether the therapeutic use of dornase alfa can be extended to ventilated, fluid-restricted children to reduce reintubation rate, ventilation duration, pediatric intensive care unit (PICU) stay, and ventilation complications. While reintubation rates were similar for dornase alfa 7% vs. placebo 9% (odds ratio, 0.77; confidence interval, 0.11-4.9), the incidence of atelectasis (6 vs. 17, respectively; P-value 0.051), median ventilation time (2.2 vs. 3.4 days, respectively; P-value 0.043), median length of PICU stay (7 vs. 8 days, respectively; P-value 0.051), and mean costs (4,830 vs. 6,320, respectively) were lower in the dornase alfa group. No adverse effects were observed, even in critically ill patients. We found that dornase alfa was beneficial and safe. Our findings also indicate that dornase alfa is possibly of value from the first day of mechanical ventilation onward, particularly when longer ventilation (>3 days) is expected in fluid-restricted children after cardiac surgery.

Administration, Inhalation↗
PubMed2006

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.

Adolescent↗
PubMed2005

Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis).

OBJECTIVES: Thrombotic events have been reported as a major cause of morbidity after the Fontan procedure. There is no consensus concerning the postoperative mode and duration of anticoagulation prophylaxis. In a retrospective study, we evaluated the results of a prophylactic regimen on the basis of the surgical technique, potentially predisposing risk factors, and specific sequelae. METHODS: We evaluated 142 surviving patients after total cavopulmonary anastomosis (mean follow-up was 91.1 +/- 43.9 months). Prophylactic antithrombotic treatment was initiated in 86 patients with partial prosthetic venous pathway with acetylsalicylic acid; 45 patients with complete autologous tissue venous pathway or partial prosthetic venous pathway received no anticoagulation, and 11 patients received warfarin sodium (Coumadin). During long-term follow-up, 22 patients (12 after acetylsalicylic acid medication) crossed over to warfarin. RESULTS: Thrombotic events occurred in 10 patients (7%), with systemic venous thrombus formation in 8 (5.6%), stroke in 2 (1.4%), and a peak incidence during the first postoperative year. Eight of 10 patients were receiving heparin therapy mainly for prolonged postoperative immobilization. During follow-up, none of the 74 patients receiving acetylsalicylic acid and 1 of 40 patients without medication presented with thrombus formation. Under warfarin medication, 1 of 28 patients had an asymptomatic thrombus. Expected freedom from a thromboembolic event was 92% at 5 years and 79% at 10 years. There was no association with coagulation factor abnormalities. Protein-losing enteropathy was present in 4 of 10 patients. CONCLUSION: A prophylactic anticoagulation strategy that considers the surgical technique and potential predisposing circumstances proved effective in the prevention of late thrombotic complications after total cavopulmonary anastomosis. There is no need for routine anticoagulation during long-term follow-up after Fontan-type surgery in pediatric patients.

Anticoagulants↗
PubMed2005

Hypoplasia of the left heart with discordant ventriculoarterial connections.

We report the rare combination of a severely hypoplastic left ventricle and discordant ventriculoarterial connections, with associated subpulmonary obstruction of the left ventricular outflow tract. The long tortuous arterial duct originated at an acute angle from the aorta, suggesting that the subpulmonary obstruction developed early in fetal life. Residual flow via the hypoplastic left ventricle to the pulmonary circulation after insertion of an aortopulmonary shunt resulted in haemodynamic deterioration, which was instantly reversed by closing the pulmonary trunk.

Coronary Vessel Anomalies↗
PubMed2004

Laterality of the aortic arch and anomalies of the subclavian artery-reliable indicators for 22q11.2 deletion syndromes?

UNLABELLED: A variety of cardiac defects, encompassing truncus arteriosus, tetralogy of Fallot, pulmonary atresia with ventricular septal defect and interrupted aortic arch, are generally summarised as conotruncal malformations. Patients with these cardiac defects were frequently found to have a common microdeletion on chromosome 22, the so-called monosomy 22q11.2. The aim of our study was to determine whether the laterality of the aortic arch or the presence of subclavian artery anomalies (SAA) represent markers for monosomy 22q11.2 in these patients. 170 patients with these cardiac anomalies were recruited at presentation in the paediatric cardiology units of two tertiary referral centres from 1994 until 2003. Of the 170 children and young adults, 33 had interrupted aortic arch, 35 tetralogy of Fallot, 31 truncus arteriosus communis and 71 pulmonary atresia with ventricular septal defect. All were screened for monosomy 22q11.2 and the results were correlated with the laterality of the aortic arch and the presence of SAA contralateral to the aortic arch (aberrant origin from the descending aorta, isolation, distal ductal origin from the pulmonary artery and cervical origin of the right subclavian artery). Monosomy 22q11.2 was present in 59/170 patients (35%). A left aortic arch (LAA) was found in 118 (69%), a right aortic arch (RAA) in 52 (31%) patients. Almost 50% of the patients with RAA (46%), but only 30% of the patients with LAA had monosomy 22q11.2 ( P=0.054). A total of 47 patients (28%) had an anomaly of the subclavian artery, 81% of whom had monosomy 22q11.2. This deletion was found in decreasing percentage in patients with LAA+SAA (85%) >RAA+SAA (75%) >RAA without SAA (28%) >LAA without SAA (13%). CONCLUSION: In patients with conotruncal malformations, anomalies of the subclavian arteries are the most important anatomical marker for the presence of monosomy 22q11.2, independent of the laterality of the aortic arch. Therefore, we recommend cytogenetic testing for this microdeletion in all patients with subclavian artery anomalies and conotruncal malformations.

Adolescent↗
PubMed2004

Search for somatic 22q11.2 deletions in patients with conotruncal heart defects.

A wide range of clinical variability in patients with 22q11.2 deletions has been demonstrated in numerous studies. Nevertheless, it is still an open question if major genetic factors contribute to clinical expression. Therefore one aim of this study was to investigate, if patients with 22q11.2 deletion and conotruncal heart defects show a "second hit" somatic 22q11.2 deletion in tissue from the conotruncus, heart vessels or thymus. The second aim was to analyse patients with conotruncal heart defects without 22q11.2 deletion in blood cells for somatic deletion mosaicism. We were able to study tissue samples from heart surgery from 23 patients, 9 of whom had 22q11 deletions by FISH analysis on metaphase spreads from peripheral lymphocytes. Analysis of 18 polymorphic markers from the 22q11.2 region in DNA prepared from thymus and/or heart vessels and/or conotruncus tissue and peripheral lymphocytes in each patient did not show any allelic loss. Thus somatic 22q11.2 deletions apparently do not play a major role in conotruncal heart defects in patients with or without germ line 22q11.2 deletion.

Child↗
PubMed2004

[Atrial isomerism and visceral heterotaxy].

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.

Abnormalities, Multiple↗
PubMed2004

Genotype-phenotype correlations in Noonan syndrome.

OBJECTIVE: To study genotype-phenotype correlations in a cohort of clinically well-characterized pediatric patients with Noonan syndrome (NS). Study design Fifty-seven unrelated patients with the clinical diagnosis of NS ascertained according to standardized inclusion criteria were prospectively enrolled. Mutational analysis was performed by direct sequencing of the entire coding sequence of the PTPN11 gene. RESULTS: Sixteen known and 3 novel PTPN11 mutations could be detected in 60% of index patients, in all familial and in 52% of the sporadic cases. Presence of pulmonic stenosis, short stature, easy bruising, and thorax deformities was significantly associated with a PTPN11 mutation, whereas cardiomyopathy was more common in patients without a mutation. On average, PTPN11 mutation-negative probands fulfilled fewer clinical criteria of NS, but more than half-among them all with cardiomyopathy-had the full clinical picture of NS indistinguishable from typical cases with PTPN11 mutation. CONCLUSIONS: The phenotype of NS due to PTPN11 mutations is clinically unambiguous in the majority of patients and represents a highly penetrant trait. Individuals with the clinical diagnosis of NS but without a PTPN11 mutation presumably represent a heterogeneous group in which patients with cardiomyopathy appear to constitute an interesting subgroup for future research.

Adolescent↗
PubMed2003

[Two- and three-dimensional echocardiographic analysis of congenital heart disease in the fetus].

INTRODUCTION: With increasing experience of obstetric sonographers, a higher proportion of cardiac malformations is found antenatally. However, undiagnosed fetal cardiac defects still result in a significant pre- and postnatal morbidity and mortality. The purpose of two- and three-dimensional echocardiographic imaging in the fetus is to provide clear representations of the underlying cardiac and vascular anatomy. Studies on pre- and postnatal echocardiography have shown these techniques to provide an adequate form of image display for comprehensive assessment of most cases with congenital heart disease. METHODS: To date, two different methods are used for three-dimensional echocardiography in the fetus. The technique currently employed at numerous institutions derives from a complex assembly of sequentially acquired and reconstructed two-dimensional images and is analogous to the 3-D technology assessed in studies on neonates, children, and adults. Although an electromagnetic location device is used to register transducer position during data acquisition, this technique has important limitations due to fetal movement artifacts and difficulties in cardiac gating. This often results in inadequate image quality when compared with 2-D echocardiography. Recent progress in the design and fabrication of higher-frequency real-time volumetric transducers has greatly improved 3-D echocardiographic imaging resolution and allows more immediate three-dimensional "on-line" analysis of cardiac anatomy. CONCLUSIONS: Advantages of 3-D fetal echocardiography include the ability to slice the acquired 3-D volume data into an infinite number of two-dimensional cross sections, and the ability to reconstruct unique three-dimensional views not seen with two-dimensional imaging. However, considering the current limitations and the time needed for 3-D image processing, its practical clinical relevance in the antenatal situation is not yet clear.

Echocardiography↗