Herbert Ulmer - Academia.edu (original) (raw)
Papers by Herbert Ulmer
Langenbecks Archiv für Chirurgie, Dec 1, 1983
The Journal of Pediatrics, May 1, 1977
Cardiology in The Young, Nov 1, 1999
Journal of The American Society of Echocardiography, Nov 1, 2007
Zeitschrift Fur Kardiologie, Mar 1, 2004
Zeitschrift Für Herz-,thorax- Und Gefäßchirurgie, May 14, 2020
Herzschrittmachertherapie Und Elektrophysiologie, Sep 1, 2002
During the last decade, the understanding of the long QT-syndrome (LQTS) as an inherited arrhythm... more During the last decade, the understanding of the long QT-syndrome (LQTS) as an inherited arrhythmogenic disease has dramatically increased. The LQTS has been recognized to be a heterogeneous family of ion-channel disorders caused by numerous mutations in at least six distinct gene loci, thus, explaining the prolongation of the myocardial repolarization. Consecutive studies of the LQTS advanced our knowledge of pathophysiology, clinical course and possible therapeutic impact. As a genetically determined disorder the clinical manifestation of the LQTS naturally starts in childhood. In 34% of the children, syncope or cardiac arrest was found to occur before the age of 15 years. In addition, 54% of all LQTS patients who died from sudden cardiac death were less than 20years old. Most cases in children are identified by the detection of a prolonged QT interval while evaluating unexplained cases of syncope or by family investigations of an index patient. In children more than in adults, however, normal values of QT interval duration are dependent from age, gender, heart rate and circadian variations. Therefore, the moreover applied correction formulas for the QT interval to heart rate ratio have to be used with caution in the pediatric setting. A useful and reliable tool for the analysis of QT duration, QT patterns, and its circadian variation is multilead digital Holter recordings. The determination of the diagnosis is based on clinical findings according to the criteria of the "International LQTS Registry". Genetic investigations, however, are actually diagnostic in about 50% of the patients, but are not effective as a clinical screening tool. The genetically determined LQTS types (LQT1-LQT6) differ significantly in terms of reaction to triggering stimuli that may induce life-threatening arrhythmias and their response to treatment. In LQT1, physical stress will more likely induce Torsades de Pointes than in the LQT3, which is more sensitive to emotional stress. The typical LQTS treatment is life-long medication with beta blocking agents; however, in LQT3 patients with mutations in the cardiac sodium channel gene, treatment with mexiletine may have advantages. In order to prevent bradycardia or by short-long sequences inducible torsades, the use of implantable pacemakers is recommended. Stellectomy to minimize cardiac adrenergic susceptibility has proven to be less effective in children. Recently, the rapid technical improvement of implantable defibrillators led to a more frequent use of these devices in children. In order to sufficiently manage pediatric LQTS patients in the future and to reduce the risk of sudden cardiac death due to inherited arrhythmias, a national and international multicenter approach is necessary.
Monatsschrift Kinderheilkunde, 2003
Neonatal Intensive Care, 1981
According to extensive studies, the incidence of congenital heart disease is about 8.8 per 1000 l... more According to extensive studies, the incidence of congenital heart disease is about 8.8 per 1000 livebirths [12]. Life-threatening cardiac symptoms develop in 2.3% of all liveborns during the neonatal period. The overall mortality associated with congenital heart disease in the first month of life is reported to be 1.2 per 1000 live births. Infants with congenital heart disease therefore still account for about 30% of all neonatal mortality [6]. The frequency of these cardiac disorders and the early, life-threatening symptoms explain why these infants represent a relatively high percentage (12%–15%) of cases in neonatal intensive care units. The most common congenital heart diseases with life-threatening symptoms, together with their relative diagnostic frequency, are listed in Table 42.
Kliniktaschenbücher, 1978
Die Haufigkeit angeborener Herzerkrankungen ist nach umfangreichen Untersuchungen mit 7,5 auf 100... more Die Haufigkeit angeborener Herzerkrankungen ist nach umfangreichen Untersuchungen mit 7,5 auf 1000 Lebendgeborene anzunehmen [1]. Bei 2,3% aller Lebendgeborenen entwickelt sich innerhalb der Neugeborenenperiode eine lebensbedrohliche kardiale Symptomatik. Die Mortalitat aufgrund angeborener Herzerkrankungen innerhalb des ersten Lebensmonats wird mit 1,2 auf 1000 Lebendgeborene angegeben. Damit entfallen noch immer 33% aller Todesfalle innerhalb der Neugeborenenperiode auf Sauglinge mit angeborenen Herzfehlern [2]. Die Haufigkeit dieser Erkrankungen und die fruhe, bedrohliche Symptomatik erklaren den mit 12–15% relativ hohen Anteil herzkranker Neugeborener an der Belegung einer Neugeborenen-Intensivpflegestation [3].
Pädiatrische Kardiologie, 1998
Die Fallot-Tetralogie wurde erstmals im Jahre 1571 von Nils Stensen und danach 1671 von Nicolaus ... more Die Fallot-Tetralogie wurde erstmals im Jahre 1571 von Nils Stensen und danach 1671 von Nicolaus Steno, 1777 von Sandifort, 1784 von Hunter und 1866 von Peacock pathologischanatomisch beschrieben H. H. Fallot wies 1888 auf die vier anatomischen Befunde hin, die der Tetralogie zugrunde liegen [18]. Aber erst 1944 konnte den ersten Patienten durch eine Palliativoperation geholfen werden [12].
Virchows Archiv, 2005
Fibrillar collagens I and III, nonfibrillar collagen IV, and the glycoproteins fibronectin and la... more Fibrillar collagens I and III, nonfibrillar collagen IV, and the glycoproteins fibronectin and laminin, are elements of the myocardial extracellular matrix (ECM). Alterations in the normal concentrations and ratios of these elements may reflect remodeling in response to physiologic stress. In the case of patients' post-heart transplantation (HTx), specific patterns of alteration may herald myocardial dysfunction. Right ventricular biopsies were taken from the same 28 HTx patients before implantation and 1 week, 2 weeks, and 1, 2, and 3 years after HTx. The above-noted five ECM proteins, six matrix metalloproteinases (MMPs) and two of their tissue inhibitors (TIMPs) were detected by immunohistochemistry and scored as cells per square millimeter or semiquantitatively. The total connective tissue fibers were detected by connective tissue stain and morphometry. Variations in these ECM components were followed in the same patient cohort over 3 years. In summary, during the first 2 weeks after HTx, a predominant increase in connective tissue occurred. Increases in MMP-8 and MMP-9 were found. By 3 years after transplantation, there was a decrease of connective tissue fibers and a significant reduction of all ECM components and an increase in MMPs and TIMPs. These findings may reflect a pattern of remodeling specific to the transplanted heart.
The Thoracic and Cardiovascular Surgeon, 2008
Objective: Timing of the operation for exchange of RV to PA conduits is a matter of considerable ... more Objective: Timing of the operation for exchange of RV to PA conduits is a matter of considerable debate. We aimed to study the course of RV dimension in patients undergoing conduit exchange. Methods: We retrospectively studied patients who underwent implantation and or replacement of RV/PA conduits during the period between 1990 and 2005. Clinical and echocardiographic data were recorded as obtained at follow-up visits. Results: A total of 229 patients underwent surgery for implantation and or replacement of RV/PA conduits during the study period. Patients were assigned to three age groups including 37 infants, 125 children aged 1–10 years and 67 patients more than 10 years of age. 185 pulmonary (81%) and 44 aortic homografts (19%) were implanted. Fifty-eight of these patients (25%) required exchange of conduits after a median time of 6.4 [8 months –12 years]. The follow-up was 7.55 [0.1–17] years. The survival of the patients after homograft change was 98%. Age at conduit exchange (coefficient: –4.917; p<0.001) and RV end-diastolic dimension (RVDD) before conduit exchange (coefficient: 8.255; p<0.001) were related to RVDD as measured by M-mode echocardiography at follow-up. RVDD decreased in 48/58 patients, remained unchanged in 8/58 and increased in 2/59 patients. Conclusions: Reoperation for exchange of degenerated conduits should be performed early to prevent the development of irreversible structural myocardial changes and persistence of right ventricular dilatation.
The Thoracic and Cardiovascular Surgeon, 2005
Journal of the American Society of Echocardiography, 2007
Langenbecks Archiv für Chirurgie, Dec 1, 1983
The Journal of Pediatrics, May 1, 1977
Cardiology in The Young, Nov 1, 1999
Journal of The American Society of Echocardiography, Nov 1, 2007
Zeitschrift Fur Kardiologie, Mar 1, 2004
Zeitschrift Für Herz-,thorax- Und Gefäßchirurgie, May 14, 2020
Herzschrittmachertherapie Und Elektrophysiologie, Sep 1, 2002
During the last decade, the understanding of the long QT-syndrome (LQTS) as an inherited arrhythm... more During the last decade, the understanding of the long QT-syndrome (LQTS) as an inherited arrhythmogenic disease has dramatically increased. The LQTS has been recognized to be a heterogeneous family of ion-channel disorders caused by numerous mutations in at least six distinct gene loci, thus, explaining the prolongation of the myocardial repolarization. Consecutive studies of the LQTS advanced our knowledge of pathophysiology, clinical course and possible therapeutic impact. As a genetically determined disorder the clinical manifestation of the LQTS naturally starts in childhood. In 34% of the children, syncope or cardiac arrest was found to occur before the age of 15 years. In addition, 54% of all LQTS patients who died from sudden cardiac death were less than 20years old. Most cases in children are identified by the detection of a prolonged QT interval while evaluating unexplained cases of syncope or by family investigations of an index patient. In children more than in adults, however, normal values of QT interval duration are dependent from age, gender, heart rate and circadian variations. Therefore, the moreover applied correction formulas for the QT interval to heart rate ratio have to be used with caution in the pediatric setting. A useful and reliable tool for the analysis of QT duration, QT patterns, and its circadian variation is multilead digital Holter recordings. The determination of the diagnosis is based on clinical findings according to the criteria of the "International LQTS Registry". Genetic investigations, however, are actually diagnostic in about 50% of the patients, but are not effective as a clinical screening tool. The genetically determined LQTS types (LQT1-LQT6) differ significantly in terms of reaction to triggering stimuli that may induce life-threatening arrhythmias and their response to treatment. In LQT1, physical stress will more likely induce Torsades de Pointes than in the LQT3, which is more sensitive to emotional stress. The typical LQTS treatment is life-long medication with beta blocking agents; however, in LQT3 patients with mutations in the cardiac sodium channel gene, treatment with mexiletine may have advantages. In order to prevent bradycardia or by short-long sequences inducible torsades, the use of implantable pacemakers is recommended. Stellectomy to minimize cardiac adrenergic susceptibility has proven to be less effective in children. Recently, the rapid technical improvement of implantable defibrillators led to a more frequent use of these devices in children. In order to sufficiently manage pediatric LQTS patients in the future and to reduce the risk of sudden cardiac death due to inherited arrhythmias, a national and international multicenter approach is necessary.
Monatsschrift Kinderheilkunde, 2003
Neonatal Intensive Care, 1981
According to extensive studies, the incidence of congenital heart disease is about 8.8 per 1000 l... more According to extensive studies, the incidence of congenital heart disease is about 8.8 per 1000 livebirths [12]. Life-threatening cardiac symptoms develop in 2.3% of all liveborns during the neonatal period. The overall mortality associated with congenital heart disease in the first month of life is reported to be 1.2 per 1000 live births. Infants with congenital heart disease therefore still account for about 30% of all neonatal mortality [6]. The frequency of these cardiac disorders and the early, life-threatening symptoms explain why these infants represent a relatively high percentage (12%–15%) of cases in neonatal intensive care units. The most common congenital heart diseases with life-threatening symptoms, together with their relative diagnostic frequency, are listed in Table 42.
Kliniktaschenbücher, 1978
Die Haufigkeit angeborener Herzerkrankungen ist nach umfangreichen Untersuchungen mit 7,5 auf 100... more Die Haufigkeit angeborener Herzerkrankungen ist nach umfangreichen Untersuchungen mit 7,5 auf 1000 Lebendgeborene anzunehmen [1]. Bei 2,3% aller Lebendgeborenen entwickelt sich innerhalb der Neugeborenenperiode eine lebensbedrohliche kardiale Symptomatik. Die Mortalitat aufgrund angeborener Herzerkrankungen innerhalb des ersten Lebensmonats wird mit 1,2 auf 1000 Lebendgeborene angegeben. Damit entfallen noch immer 33% aller Todesfalle innerhalb der Neugeborenenperiode auf Sauglinge mit angeborenen Herzfehlern [2]. Die Haufigkeit dieser Erkrankungen und die fruhe, bedrohliche Symptomatik erklaren den mit 12–15% relativ hohen Anteil herzkranker Neugeborener an der Belegung einer Neugeborenen-Intensivpflegestation [3].
Pädiatrische Kardiologie, 1998
Die Fallot-Tetralogie wurde erstmals im Jahre 1571 von Nils Stensen und danach 1671 von Nicolaus ... more Die Fallot-Tetralogie wurde erstmals im Jahre 1571 von Nils Stensen und danach 1671 von Nicolaus Steno, 1777 von Sandifort, 1784 von Hunter und 1866 von Peacock pathologischanatomisch beschrieben H. H. Fallot wies 1888 auf die vier anatomischen Befunde hin, die der Tetralogie zugrunde liegen [18]. Aber erst 1944 konnte den ersten Patienten durch eine Palliativoperation geholfen werden [12].
Virchows Archiv, 2005
Fibrillar collagens I and III, nonfibrillar collagen IV, and the glycoproteins fibronectin and la... more Fibrillar collagens I and III, nonfibrillar collagen IV, and the glycoproteins fibronectin and laminin, are elements of the myocardial extracellular matrix (ECM). Alterations in the normal concentrations and ratios of these elements may reflect remodeling in response to physiologic stress. In the case of patients' post-heart transplantation (HTx), specific patterns of alteration may herald myocardial dysfunction. Right ventricular biopsies were taken from the same 28 HTx patients before implantation and 1 week, 2 weeks, and 1, 2, and 3 years after HTx. The above-noted five ECM proteins, six matrix metalloproteinases (MMPs) and two of their tissue inhibitors (TIMPs) were detected by immunohistochemistry and scored as cells per square millimeter or semiquantitatively. The total connective tissue fibers were detected by connective tissue stain and morphometry. Variations in these ECM components were followed in the same patient cohort over 3 years. In summary, during the first 2 weeks after HTx, a predominant increase in connective tissue occurred. Increases in MMP-8 and MMP-9 were found. By 3 years after transplantation, there was a decrease of connective tissue fibers and a significant reduction of all ECM components and an increase in MMPs and TIMPs. These findings may reflect a pattern of remodeling specific to the transplanted heart.
The Thoracic and Cardiovascular Surgeon, 2008
Objective: Timing of the operation for exchange of RV to PA conduits is a matter of considerable ... more Objective: Timing of the operation for exchange of RV to PA conduits is a matter of considerable debate. We aimed to study the course of RV dimension in patients undergoing conduit exchange. Methods: We retrospectively studied patients who underwent implantation and or replacement of RV/PA conduits during the period between 1990 and 2005. Clinical and echocardiographic data were recorded as obtained at follow-up visits. Results: A total of 229 patients underwent surgery for implantation and or replacement of RV/PA conduits during the study period. Patients were assigned to three age groups including 37 infants, 125 children aged 1–10 years and 67 patients more than 10 years of age. 185 pulmonary (81%) and 44 aortic homografts (19%) were implanted. Fifty-eight of these patients (25%) required exchange of conduits after a median time of 6.4 [8 months –12 years]. The follow-up was 7.55 [0.1–17] years. The survival of the patients after homograft change was 98%. Age at conduit exchange (coefficient: –4.917; p<0.001) and RV end-diastolic dimension (RVDD) before conduit exchange (coefficient: 8.255; p<0.001) were related to RVDD as measured by M-mode echocardiography at follow-up. RVDD decreased in 48/58 patients, remained unchanged in 8/58 and increased in 2/59 patients. Conclusions: Reoperation for exchange of degenerated conduits should be performed early to prevent the development of irreversible structural myocardial changes and persistence of right ventricular dilatation.
The Thoracic and Cardiovascular Surgeon, 2005
Journal of the American Society of Echocardiography, 2007