Publications by authors named "Hammerer I"

Background: In patients with Marfan syndrome, progressive aortic dilation implicates a still-unpredictable risk of life-threatening aortic dissection and rupture. We sought to quantify aortic wall dysfunction noninvasively, determine the diagnostic power of various aortic parameters, and establish a diagnostic model for the early detection of aortic abnormalities associated with Marfan syndrome.

Methods: In 19 patients with Marfan syndrome (age, 17.

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The Williams-Beuren syndrome (WBS) is a complex developmental disorder with multisystemic manifestations including supravalvular aortic stenosis (SVAS), a so-called elfin face, a hoarse voice, and a specific cognitive phenotype. Most WBS patients have a >1 Mb deletion on one of their chromosomes 7 in q11 but except for elastin, whose haploinsufficiency causes the cardiovascular malformations, it is unknown which genes in the deletion area contribute to the phenotype. We have investigated a family with a cytogenetically balanced translocation t(7;16)(q11.

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Objectives: We undertook this study to assess the immediate and long-term outcome of balloon angioplasty performed for recurrent or residual coarctation of the aorta, and to assess the changes in the vessel wall caused by this procedure.

Methods: Clinical, echocardiographic, angiographic and hemodynamic data from 71 patients who underwent balloon angioplasty for recoarctation between January 1987 and January 1998 were analysed retrospectively.

Results: Angioplasty was performed after a median of 82.

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Objective: We conducted a prospective study to assess the hemodynamic effects of conventional mechanical ventilation (CMV) compared with high-frequency oscillation (HFO) in newborn babies with respiratory distress syndrome.

Methods: A total of 18 consecutive term and preterm infants were examined by two-dimensional M-mode and pulsed Doppler echocardiography.

Results: Five patients had to be excluded, three of them because of increasing cardiovascular support after initiation of HFO.

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A 13-year-old boy with hypertrophic obstructive cardiomyopathy was treated with dual-chamber pacing after severe progression of left ventricular outflow tract obstruction and of clinical symptoms despite drug therapy. Rapid intrinsic atrioventricular conduction was overcome and complete preexcitation of the septum achieved by omitting atrial sensing and programming constant atrial pacing with a short atrioventricular delay of 70 msec. After 8 weeks of therapy, a reduction of the left ventricular outflow tract gradient from 125 to 16 mmHg and remodeling of the left ventricle were demonstrated.

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Objective: To assess whether the change in cardiac output after volume replacement is due to elevation of stroke volume or heart rate and to determine the effect of mechanical ventilation on the hemodynamic situation.

Design: Prospective study.

Setting: A ten-bed neonatal intensive care unit (level III) at a university hospital.

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PS is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and courses between trachea and esophagus to the hilus of the left lung causing compression of the right mainstem bronchus and trachea. In about half of all cases this vessel anomaly is associated with tracheo-bronchial and/or cardiovascular malformations. We present 6 patients with PS concentrating on the accompanying malformations of the tracheobronchial tree.

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Anthracyclines (doxorubicin, daunorubicin, and derivatives) are among the most effective antineoplastic drugs for pediatric cancer with dose-limiting acute and long-term cardiotoxicity. The exact mechanism of the development of cardiomyopathy is still not clear. Anthracyclines may induce subclinical acute myocardial injury leading to lysis of a limited number of myocytes.

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Objective: To establish criteria for the accurate diagnosis of different forms of left sided pericardial defects on magnetic resonance imaging. Early detection of a partial apical defect is essential as it is potentially fatal.

Design: Examination of four children with congenital pericardial defects by magnetic resonance imaging, the results being compared with the features on conventional chest radiography and echocardiography and with published data.

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Pulmonary sling (PS) is a congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery (RPA), forming a sling around the trachea causing tracheal compression. The incidence is not so rare as initially thought. Symptoms of severe airway obstruction often begin in the newborn or young infant.

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From July 1987 to December 1992, 52 patients underwent balloon-angioplasty of aortic coarctation at three units of pediatric cardiology in Austria (Graz = 35 patients, Innsbruck = 15 patients, and Vienna = 2 patients). 35 patients had postoperative and 17 had native coarctation. Mean age at intervention was 7 10/12 years with 2 patients under 1 year and 5 patients over 18 years old.

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In this study, we investigated whether capillary plasma catecholamines can be used as a suitable substitute for arterial catecholamines. Analysis was done radioenzymatically. Catecholamine concentrations were not different in arterial and simultaneously collected "arterialized" (warmed foot) capillary plasma obtained by heel-prick from 18 neonatal intensive care patients as assessed by linear regression analysis (correlation coefficient: 0.

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We report a 3-year-old girl with the cardio-facio-cutaneous (CFC) syndrome. She presented the typical combination of mild developmental delay, postnatal onset short stature with relative macrocephaly, a wide and prominent forehead with posteriorly rotated ears and down-slanting palpebral fissures, an atrial septal defect, and ectodermal abnormalities. All cases reported to date occurred sporadically.

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The congenital heart disease incidence study was designed to collect valid data with respect to new diagnostic methods and to an improved system of preventive health care in order to compare the results with former studies. In the years 1979-1983 there were 41,725 live births in the Tyrol. Among these, 341 cases of congenital heart disease were recognised giving an incidence of 8.

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We report a child with a rare cardiac involvement in GM1 gangliosidosis. At the age of 9 months a secondary dilatative cardiomyopathy due to the metabolic disorder was diagnosed. During 2 years the functional echocardiographic signs became normal, though the ECG still was not normal.

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An anomalous left pulmonary artery obstructs the right main bronchus and/or the trachea. Depending on the severity of the obstruction, symptoms range from a more or less disturbing stridor over attacks of shortness of breath to a life-threatening or even lethal apnoea. An 18-month-old girl suffering from a mild form of this anomaly is presented.

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We report on a patient, now 17 year old, in whom lactic acidosis was detected at the age of 7 while attempting to diagnose the causes of increasing weakness. The laboratory examinations revealed elevated pyruvate, alanine and oxaloacetate levels in serum and also a lowered citrate level. This led us to suspect a disturbance of the pyruvate dehydrogenase complex.

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Pleuro-pericardial defects have been found incidentally during post mortems or chest surgery but, because of their usual lack of symptoms, are rarely found otherwise. If suspicion of such a malformation arises, it should be clarified because of possible herniation of the atrium or ventricle. The necessary diagnostic steps are discussed in relation to two patients and the value of chest x-rays is stressed.

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A congenital arteriovenous fistula involving the inferior epigastric arteries and the umbilical vein, simulating a congestive heart failure, occurred in a newborn infant. The embryology of this condition is explained. The involved vessels were ligated and excised.

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Midodrin, a new alpha-mimetic drug, has been studied with respect to its therapeutic effectiveness in children suffering from orthostatic hypotension. The study was carried out as a double blind trial against placebo. A statistically significant improvement was found only in relation to the following symptoms: "tendency to collapse", "dizziness", "headache", "change of colour".

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Agenesis of the Ductus arteriosus Botalli is a rare anomaly, which is known to occur in combination with truncus arteriosus, tetralogy of Fallot with pulmonary valve agenesis and also in simple tetralogy. The authors report on a case, where neither a ductus nor any remnant of it could be found in a complex heart malformation consisting of premature closure of the Foramen ovale, mitral atresia, hypoplastic left ventricle, transposition of the great arteries, pulmonary valve atresia and hypoplastic pulmonary artery. Speculations as to the pathogenesis of this anomaly presume slowing and eventual arrest of the ductal blood flow as a consequence of closure of the Foramen ovale in early fetal life.

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The authors report on a male infant displaying all the clinical features of the mucocutaneous lymph node syndrome (MLNS) as described by Kawasaki in 1967. In the same patient, diffuse dilatation of the whole arterial tree--as known in infantile periarteriitis nodosa but so far not shown in MLNS--could be demonstrated angiographically during the acute phase of the disease. As the vascular findings at postmortem examination in MLNS and periarteriitis nodosa were the same too, the authors believe that separation of these diseases is not justified.

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Report on two girls with an obstruction within the right ventricle beneath the infundibulum. A muscular band - probabaly the abnormally situated moderator band - emerges at right angles from the ventricular septum, crosses the lumen and ends at the anterior wall of the right ventricle, thus separating a high-pressure inflow chamber from a low-pressure out-flow chamber. The latter compartment is distinctly larger than the infundibular chamber in Fallot's tetralogy.

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