PubMed Health⌕ Search

Biomedical subjects

M Hofbeck

Publications and source records attributed to M Hofbeck.

At least 91 records · Page 5Linked to original sources
PubMed1991

[Presystolic increasing maximal flow velocity in the intracranial vessels in 3 children with heart failure].

The doppler-sonographic examination of flow velocities in intracerebral arteries is of central importance in the diagnostic evaluation of potential neurologic complications in intensive care patients. Furthermore pulsed Doppler-sonography can assess the hemodynamic consequences of several congenital heart defects (e.g. the ductus arteriosus Botalli). Characteristics of the normal flow profile in the intracranial arteries are the systolic and diastolic forward flow caused by the "Windkessel" function of the aorta. Well known pathologic flow profiles (systolically and diastolically increased flow velocities, diastolically decreased flow velocities and diastolic retrograde flow) occur in neurologic and cardiovascular diseases. We have investigated three children after cardiac surgery, who showed a presystolic increase in the flow velocities, which is clearly different from the flow profiles mentioned above. In our opinion, this abnormal flow pattern is due to a decreased ventricular output in combination with an elevated central venous pressure in these patients.

Aortic Valve Stenosis↗
PubMed1991

[Faulty origin of the right subclavian artery from the pulmonary artery: a rare cause of subclavian steal syndrome in childhood].

A congenital subclavian steal syndrome may be caused by coarctation or interruption of the aortic isthmus or by isolation of a subclavian artery. We describe a patient with D-transposition of the great arteries, a left aortic arch, and isolation of the right subclavian artery which originated from the right pulmonary artery via a right ductus arteriosus. A subclavian steal syndrome was demonstrated noninvasively by echocardiography and doppler sonography of the cerebral arteries. We recommend routine cerebral doppler sonography for all infants with congenital heart disease and unilaterally weak brachial pulses. Since the long term outcome of the congenital subclavian steal syndrome is uncertain the aberrant subclavian artery should be reimplanted at the time of corrective cardiac surgery.

Angiocardiography↗
PubMed1990

Prenatal ultrasound diagnosis of multiple cardiac rhabdomyomas.

We report on a neonate with multiple cardiac rhabdomyomas, including a huge rhabdomyoma of the left ventricular posterior wall. Prenatal ultrasonography performed because of supraventricular tachycardia led to the diagnosis in the 28th week of gestation. Postnatal echocardiography confirmed the prenatal diagnosis. The tumor could not be removed surgically and the child died as a result of intractable arrhythmia at the age of 5 days. The diagnosis of multiple cardiac rhabdomyomas was confirmed by autopsy.

Female↗
PubMed1990

Coarctation of the aorta in the syndrome of absent pulmonary valve with ventricular septal defect.

The case of a male child is reported in which the syndrome of absent pulmonary valve and ventricular septal defect was associated with anomalous drainage of the right superior pulmonary vein into the superior vena cava, atrial septal defect, a right aortic arch, and an aortic isthmic coarctation. The combination of aortic coarctation with a high degree of right-ventricular outflow obstruction has not been reported so far in the literature. This case shows that there is no proof of aplasia of the ductus arteriosus in the syndrome of absent pulmonary valve and ventricular septal defect. Most likely, there is an early involution of the ductus arteriosus; persistence of the ductus arteriosus would not be compatible with fetal survival.

Abnormalities, Multiple↗
PubMed1989

Diverticulum of the right ventricle associated with ventricular septal defect.

A true diverticulum of the ventricle is a very rare congenital cardiac lesion which is characterized by a normal myocardial wall and active participation in the ventricular contractions. Diverticula of the right ventricle may originate from the apical or anterosuperior wall. We report three patients with an anterosuperior diverticulum of the right ventricle associated with a perimembranous ventricular septal defect, one of whom additionally had obstruction of the right ventricular outflow tract. These patients presented clinically with symptoms related only to their associated defects. The right ventricular diverticulum was diagnosed by angiography in all, but was also seen on cross-sectional echocardiography in one patient. The ventricular septal defect was repaired through an incision of the diverticulum, which was reduced in size at the same time. Complete resection appears necessary only if severe endocardial fibrosis is present within the diverticulum, because such a diverticulum does not contribute to right ventricular contraction, but rather is at risk of rupture.

Child↗
PubMed1989

[Doppler sonography determination of flow ratios Qp/Qs and resistance ratios Rp/Rs in isolated ventricular septal defect].

We present a model based on an electric analogon that describes the haemodynamic situation of an isolated ventricular septal defect. The model shows a way to estimate the ratio of pulmonary to systemic vascular resistance by Doppler sonographic measurements of pulmonary to systemic flow ratio (Qp/Qs) and the ratio between the size of the defect and the cross-sectional area of the aorta (Av/Aa). The ratio of pulmonary to systemic vascular resistance is theoretically given by: Rp/Rs = Qs/Qp + log (Av/Aa) * (1-Qs/Qp). Systemic to pulmonary flow ratio can be measured by Doppler echocardiography as well as Av/Aa; therefore, Rp/Rs can be estimated noninvasively by the equation given above. The values estimated noninvasively by Doppler echocardiography showed a good correlation with the haemodynamic data obtained at cardiac catheterisation.

Aorta, Thoracic↗
PubMed1989

[Resection of a cardiac rhabdomyoma of the right and left ventricular outflow tract].

Rhabdomyomas are benign cardiac tumors often occurring as multiple lesions. We describe an infant with multiple cardiac rhabdomyomas causing severe right-ventricular and left-ventricular outflow tract obstructions. The intracavitary parts of the rhabdomyomas were successfully resected when the patient was 5 weeks old. Echocardiography showed regression in size of the remaining intramural tumors. Since there is only a low growth potential in cardiac rhabdomyomas, surgical management is indicated only in patients with hemodynamic obstruction or significant arrhythmias. Surgery should be primarily directed to relieve hemodynamic obstruction. If complete resection is not possible without damage to vital structures, resection should be restricted to the intracavitary parts of the tumor.

Echocardiography↗
PubMed1987

[Pulmonary atresia with ventricular septal defect: the significance of collateral lung perfusion for the prognosis of corrective operations].

From 1970 to 1986, 43 patients with pulmonary atresia and ventricular septal defect (PA-VSD) were diagnosed in our hospital. 19 children had a collateral lung perfusion via a ductus botalli (DB), three children had a DB and systemico-pulmonary collateral arteries (SPCA), and 21 children had only SPCA. Patients with a DB alone had significantly larger pulmonary arteries. Children with SPCA always had hypoplastic pulmonary arteries and intrapulmonary arborization anomalies and in a high percentage (19 patients) they showed intrapulmonary stenoses. 26 palliative operations were performed in 24 children. After palliative operations in 15 cases (patients with DB: RVOT-Patch: one patient, aortopulmonary shunt: three patients, Brock procedure: one patient, patients with SPCA: RVOT-Patch: five patients, aortopulmonary shunt: four patients, Brock procedure: one patient) we checked the possibility of corrective surgery with the equation of Alfieri. According to this equation, corrective surgery without excessive right ventricular pressure would have been possible in all post-operatively catheterized patients with DB after primary palliative operations but, due to the pulmonary artery anomalies, only in seven out of nine patients with SPCA (one patient of the latter group was operated twice, Brock procedure and RVOT-Patch). In four cases we could prove the value of the Alfieri equation after corrective operations. There was an excellent correlation between the predicted relation of PRV/PLV and the actually measured pressures. In our opinion, the Alfieri equation forms a valuable aid in the preoperative assessment of children with PA-VSD.

Angiocardiography↗
PubMed1987

[Mitral valve atresia with normal aortic valve and pulmonary atresia. A case with pulmonary collateral vascular perfusion by systemic pulmonary arteries].

A case of mitral atresia with normal aortic valve, situs solitus, d-loop, ASD, VSD, DORV and atresia of the pulmonary valve is presented. Systemic-pulmonary collateral arteries were the single source of collateral lung perfusion. These vessels are often found in patients with tetralogy of Fallot and pulmonary atresia with VSD. The case favours the idea that these vessels are not specific for the above mentioned cardiac anomalies. It supports the theory that they represent true embryonal vessels which can persist in cases of an inadequate or completely missing connection of the sixth aortic arch derivates with the pulmonary vascular bed.

Cardiac Catheterization↗
PubMed1983

[Pulmonary atresia with ventricular septal defect: palliative operations of primarily inoperable forms].

Between 1970 and 1981 we observed 26 children with pulmonary atresia and ventricular septal defect (PA-VSD). 24 patients had two pulmonary arteries with a bifurcation, one patient had only one central pulmonary artery and one patient had none. Patients in whom lung perfusion was carried out by the ductus arteriosus usually had well developed pulmonary arteries. Palliative surgery (aortopulmonary shunts, Brock procedure, right ventricular outflow tract patch without closure of the VSD) was performed in cases with hypoplastic pulmonary arteries. A comparison of pre- and postoperative angiocardiographies showed an improvement of pulmonary perfusion in all patients. In 3 of 8 children there was an increase in pulmonary artery diameter, which made them more suitable candidates for corrective surgery. Intrapulmonary stenoses and lung segments perfused exclusively by large systemicopulmonary collaterals made corrective surgery impossible in the other 5 children.

Adolescent↗
PubMedDate not supplied

Enteral nutritional support by percutaneous endoscopic gastrostomy in children with congenital heart disease.

One of the major problems of children with severe congenital heart disease (CHD) is their poor nutritional status. Among other consequences, it influences the surgical outcome. Retrospectively we present our experience with percutaneous endoscopic gastrostomy (PEG) in 15 children with CHD. This technique allows enteral nutritional support without the disadvantages related to long-term nasogastric tube feeding. Major complications were absent, and minor complications were rare both at PEG insertion, which was performed under deep sedation, and during feeding via PEG tube. In 4 of the 8 children who were followed for at least 6 months the age-matched body weight increased more than one standard deviation. In 2 other patients it increased more than 0.5 standard deviations. In 7 children the tube was removed after 2.5 to 42 months since enteral support was no longer necessary. Apart from initial reservations the parental acceptance of PEG was good. We conclude that the PEG is a safe and reliable technique to support enteral nutrition in children with severe CHD.

Enteral Nutrition↗
PubMedDate not supplied

Balloon valvuloplasty of critical pulmonary valve stenosis in a premature neonate.

Balloon dilatation of critical pulmonary valve stenosis in neonates with a weight of less than 2.5 kg is associated with specific problems, including temperature loss during the procedure, venous access, and problems related to the small size of the cardiac structures. We report our experience with balloon valvuloplasty in a premature newborn weighing 1.22 kg. Venous access was gained with a 4 French sheath, and balloon dilatation was performed with a 3.5 French 7-mm balloon catheter. Temperature loss of the baby was avoided by puncturing the femoral vein prior to the procedure on a neonatal open care system, wrapping the child in cotton, and covering the extremities with aluminium foil. The good result in our patient demonstrates that balloon valvuloplasty is a therapeutic option for treatment of critical pulmonary stenosis in premature infants.

Angiography↗
PubMedDate not supplied

[The use of implantable cardioverter-defibrillators (ICD) in children and adolescents].

BACKGROUND: The compelling safety and efficacy data in numerous large, blinded trials on adult patients, and the progress in device- and leadtechnology have led to increasing use of implantable cardioverter defibrillators in pediatric patients. The purpose of our study was to assess the efficacy and safety of ICD in the pediatric age group of a tertiary referral centre. PATIENTS AND METHODS: Between March 1998 and October 2003 12 patients underwent ICD-implantation. The mean age at implantation was 14,8 years with a range between 10-17 years. The underlying cardiac disorders included long QT-syndrome in 4 patients, ventricular fibrillation in 3 patients, dilated cardiomyopathy in 4 patients, and congenital heart disease in 1 patient (pulmonary atresia with ventricular septal defect after Rastelli repair). All patients received a transvenous ICD-system (VVI-ICD in 4 patients, DDD-ICD in 8 patients). RESULTS: The mean follow up was 35 months (6-68 months). During this period there were no severe complications nor mortality. We haven't seen infections, thromboembolic complications or lead-perforations. 2 patients (17 %) received appropriate DC-shocks, 1 patient (8 %) received an inappropriate DC-shock. 10 patients (83 %) had no malignant ventricular arrhythmia under medical therapy. 2 patients (17 %) required revision because of lead-dysfunction. In 2 patients with DCM the device was explanted during orthotopic heart transplantation. CONCLUSIONS: Our data demonstrate that advances in device- and leadtechnology have resulted in a decrease of severe complications in the pediatric age group. We conclude that ICD-implantation represents a safe and effective therapy for children and adolescents with lifethreatening ventricular dysrhythmias. Since it represents an invasive therapy, indication should be confined to patients with lifethreatening dysrhythmias according to the guidelines of the American Heart Association.

Adolescent↗
PubMedDate not supplied

[Echocardiographic diagnosis of congenital diverticuli of the right and left ventricle].

A congenital diverticulum of the ventricle is a very rare cardiac lesion which is almost always associated with additional cardiovascular anomalies. In the majority of cases a diverticulum of the heart has been diagnosed during cardiac catheterization for evaluation of additional cardiovascular anomalies. We report noninvasive echocardiographic diagnosis of a congenital cardiac diverticulum in two patients. Noninvasive imaging of the diverticulum facilities planning of cardiac catheterization, angiographic imaging and surgical treatment of this rare anomaly.

Angiocardiography↗
PubMedDate not supplied

[Functional tricuspid atresia in a newborn infant with cardiac rhabdomyoma].

Cardiac rhabdomyomas are benign tumours closely associated with tuberous sclerosis. We report on a neonate with a cardiac rhabdomyoma, which simulated tricuspid atresia with duct-depending pulmonary perfusion due to almost complete obliteration of right ventricular cavum. Under infusion of prostaglandin E1 the newborn stabilized and was successfully operated on the 3rd day of life. We conclude that successful tumour resection is possible in neonates with cardiac rhabdomyomas causing relevant hemodynamic obstruction.

Echocardiography↗
PubMedDate not supplied

[Aortic aneurysms in Ullrich-Turner syndrome].

BACKGROUND: Adolescents and young adults with Marfan-syndrome, Ehlers-Danlos-syndrome and with coarctation of the aorta are at risk of developing dissecting aortic aneurysms. The rupture of an aortic aneurysm has a poor prognosis. PATIENTS: We report on two adolescents with Ullrich-Turner-syndrome (UTS) who died from dissection of an aneurysm of the aorta. RESULTS: Including these two case reports we reviewed the literature concerning congenital heart disease and development of aortic aneurysms in UTS. Patients with UTS are at increased risk to develop an aortic aneurysm. Beside the basic chromosomal anomaly additional risk factors like coarctation of the aorta, aortic valve defects or arterial hypertension are frequently present in the affected girls. The rupture of dissecting aortic aneurysms is one major reason for the shorter life expectancy of patients with UTS compared to the normal population. CONCLUSION: Annual echocardiographic surveillance of the diameter of the ascending aorta is necessary in all patients with UTS. A sudden increase of the diameter or a diameter of 5-6 cm are criteria for the elective surgical replacement of the aortic root. Vigorous treatment of arterial hypertension is mandatory. Patients with dilated ascending aorta or coarctation of the aorta should avoid competitive sports and all sports associated with the risk of trauma to the thorax, i.e. boxing or jumping from diving platforms.

Adolescent↗
PubMedDate not supplied

[Extra-cerebral intracranial fluid collections in childhood: differentiation between benign subarachnoid space enlargement and subdural effusion using color-coded duplex ultrasound].

BACKGROUND: Extracerebral fluid collections in infancy are a common diagnostic problem, because by noninvasive imaging studies (including cranial ultrasonography, CT and NMR), no definite differentiation between two distinct pathological conditions can be found until today: An enlargement of the subarachnoid spaces in children with macrocephaly is a frequent observation of mostly unknown etiology but is known to be associated with a good prognosis. If surgery is necessary in these patients, ventricular shunting is required. On the other hand subdural effusions are often of traumatic origin and require frequently neurosurgical intervention (subdural shunting). Most reports on extracerebral fluid collections in infancy have not differentiated between both pathological conditions and therefore reveal confusing results. Recent studies using magnetic resonance imaging have shown that vascular flow phenomena in the arachnoid space can be used to a reliable diagnosis, whereas previous noninvasive neuroimaging attempts including high resolution computerized tomography (CT) have been useless. PATIENTS AND METHODS: We investigated a cohort of 20 patients aged 4 mths to 30 mths (mean 10.5 +/- 6.6 months) 16 with the history of macrocephaly and normal neurological development and 4 patients after head trauma and symptoms of an elevated intracranial pressure. RESULTS: In all 16 patients with the clinical diagnosis of benign subarachnoid space enlargement colour coded Doppler sonography detected archnoid vessels within the fluid collection, furthermore high resolution ultrasound demonstrated the dural border of of the arachnoidea as an echogenic membrane, an observation useful as a further sign of the subarachnoid location of the fluid collection. In the 4 patients with subdural hematoma the fluid collection showed an increased echogenity, no vascular structures and no surrounding border. CONCLUSION: Out of these observations we conclude that high resolution ultrasound and colour-Doppler sonography are able to reliably differentiate between a subdural and a subarachnoid fluid collection. An NMR investigation with its higher risks (sedation, anesthesia) focused on this target only seems therefore to be not necessary in these patients.

Blood Flow Velocity↗