Isolated non-A, non-B dissection of the aortic arch.
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Biomedical subjects
Publications and source records attributed to R Hetzer.
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Anti-beta1-adrenoceptor antibodies may play a harmful role, and the elimination of these antibodies could have beneficial effects for some patients with dilated cardiomyopathy. In vitro experiments showed that the antibody was able to influence the function of cultured cardiomyocytes. In these experiments, the antibody prevented the down-regulation of the beta-adrenoceptor-mediated chronotropic response. This lack of desensitization, which resulted in permanent stimulation, could also influence the Ca2+ homeostasis of cardiomyocytes. However, in longer-term (72 hours)-treated cells, the antibodies were able to decrease subtype-specific expression of the beta1 adrenoceptor. In animal experiments, it was shown that long-term immunization with a peptide corresponding to the second extracellular loop of the beta1 adrenoceptor induced a failing heart similar to that in dilated cardiomyopathy. In humans, we observed a remarkable correlation between disappearance of the antibodies and improvement of heart function. Furthermore, in anti-beta1-adrenoceptor-positive patients with dilated cardiomyopathy treated with the immunoadsorption technique, removal of the antibodies also led to improvement of cardiac function and quality of life. This finding indicates that autoimmune processes may be involved in some patients with dilated cardiomyopathy.
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Despite its importance for the regulation of heart function, little is known about the isoform expression of the multifunctional Ca2+/calmodulin-dependent protein kinase (CaMKII) in human myocardium. In this study, we investigated the spectrum of CaMKII isoforms delta2, delta3, delta4, delta8, and delta9 in human striated muscle tissue. Isoform delta3 is characteristically expressed in cardiac muscle. In skeletal muscle, specific expression of a new isoform termed delta11 is demonstrated. Complete sequencing of human delta2 cDNA, representing all common features of the investigated CaMKII subclass, revealed its high homology to the corresponding rat cDNA. Comparative semiquantitative reverse transcription-polymerase chain reaction analyses from left ventricular tissues of normal hearts and from patients suffering from dilated cardiomyopathy showed a significant increase in transcript levels of isoform delta3 relative to the expression of glyceraldehyde-3-phosphate dehydrogenase in diseased hearts (101. 6+/-11.0% versus 64.9+/-9.9% in the nonfailing group; P<0.05, n=6). Transcript levels of the other investigated cardiac CaMKII isoforms remained unchanged. At the protein level, by using a subclass-specific antibody, we observed a similar increase of a delta-CaMKII-specific signal (7.2+/-1.0 versus 3.8+/-0.7 optical density units in the nonfailing group; P<0.05, n=4 through 6). The diseased state of the failing hearts was confirmed by a significant increase in transcript levels for atrial natriuretic peptide (292. 9+/-76.4% versus 40.1+/-3.2% in the nonfailing group; P<0.05, n=3 through 6). Our data characterize for the first time the delta-CaMKII isoform expression pattern in human hearts and demonstrate changes in this expression pattern in heart failure.
Acute decompensation leading to progressive pump failure is a main cause of death in patients with congestive heart failure. To find possible metabolic defects associated with the onset of this fatal occurrence, we measured myocardial adenine nucleotides, glycogen, and Na,K-ATPase in patients with end-stage idiopathic dilated cardiomyopathy. The biopsy specimens were obtained from the right ventricle of beating hearts during implantation of a biventricular assistance device in 23 patients (group I) suffering from irreversible cardiogenic shock and during heart transplantation in 20 patients (group II) in compensated heart failure. Left ventricular ejection fraction (LVEF) was determined preoperatively by echocardiography. Left ventricular function in group I was more severely impaired than in group II (LVEF 16.8%+/-4.6% vs 22.1%+/-5.1 %; p <0.01). Myocardial adenosine triphosphate (ATP) in group I was significantly reduced in comparison with group II (119.4+/-10.2 vs 27.7+/-7.4 nmol/mg noncollagen protein; p <0.01). There was no difference in glycogen levels. Na,K-ATPase concentration in group I (n = 8) was lower than that of group II (n = 20) (425+/-80 vs 498+/-75 pmol/g wet weight; p <0.05). Linear regression analyses showed a significant correlation between adenosine triphosphate (ATP) and LVEF (r = 0.41, p <0.01) and between Na,K-ATPase and LVEF (r = 0.55, p <0.01). These results indicate that loss of myocardial ATP and Na,K-ATPase could partially contribute to the development of spontaneous deterioration of the chronically overloaded heart.
The activity of the cardiac renin-angiotensin system is altered in human heart failure, but the regulatory mechanisms are unknown. We analyzed whether angiotensin-converting enzyme (ACE) mRNA expression in heart failure is altered in the atrial myocardium, and whether a correlation exists between atrial ACE mRNA expression and the parameters of left ventricular function. We also investigated whether the use of ACE inhibitors or the ACE I/D genotype modulates the atrial ACE mRNA content. For this purpose patients who were to undergo routine cardiac surgery were selected in a prospective manner according to left ventricular function and ACE inhibitor therapy. Samples of atrial myocardium were taken, and ACE mRNA expression was determined by internally standardized reverse transcription polymerase chain reaction. Atrial ACE mRNA expression did not differ in patients with left ventricular ejection fraction higher than 55% (2423+/-199 copies/ng RNA) and those with a value less than 45% (2661+/-143 copies/ng RNA, n.s.). ACE mRNA expression also did not differ in patients using ACE inhibitors (2585+/-175 copies/ng RNA) and those not using ACE inhibitors (2476+/-185 copies/ng RNA). Furthermore, atrial ACE mRNA expression was not affected by the ACE genotype (DD 2573+/-203, ID 2472+/-215, II 2563+/-249 copies/ng RNA). We conclude that the regulation of atrial ACE mRNA expression occurs predominantly by local mechanical or para- or autocrine factors.
We report about a 17 year old male patient with a cardiomyopathy secondary to type IV glycogenosis (Andersens disease) and class II immunoglobulin deficiency who underwent cardiac transplantation. The patient first developed symptoms of heart failure at the age of twelve. The histologic diagnosis was cardiomyopathy secondary to glycogenosis. In addition, the patient suffered recurrent pulmonary infections and developed bronchiectases in the left lower lobe. This region was atelectatic since he was eleven. The patient did have two younger brothers who died of congestive heart failure at the age of nine and ten. Neither his parents nor anybody else of his relatives had a history of heart failure or glycogenosis. Since the patient suffered recurrent cardiac decompensations with the need for catecholamines he was accepted for cardiac transplantation although several relative contraindications to transplantation such as cachexia, myopathy, immunglobulin deficiency and bronchiectases had been present. The patient was transplanted successfully. The postoperative weaning from the respirator was markedly prolonged and complicated by pulmonary infection. Furthermore, mobilization was retarded. One year after transplantation, he is in a good condition without pulmonary or systemic infection. Right ventricular endomyocardial biopsies did not show recurrence of glycogenosis in the donor organ.
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BACKGROUND: The maze circuit lengthens the period of atrial depolarization and may lead to small or absent P waves, which may not be readily apparent in a standard 12-lead electrocardiogram. In this prospective study, we investigate if the right precordial leads V3R through V6R can improve detection of P waves and identification of atrial electrical activity. METHODS: Standard 12-lead electrocardiograms (with leads I through III, aVR, aVL, aVF, and V1 through V6) and right precordial electrocardiograms (V3R through V6R) were recorded in 30 consecutive patients during the first 5 postoperative days and 1, 3, 6, 12, and 24 months after combined Cox-maze III procedure and mitral valve surgery. The P wave identification as an indication of atrial electrical activity and atrial contraction was proved by transesophageal echocardiographic identification of atrial contractions. RESULTS: Despite echocardiographically identifiable atrial contractions, the P waves were not visible on standard 12-lead electrocardiograms in 7 (23%) patients after 1 and 3 months, and in 4 patients after 6 months. In contrast, right precordial electrocardiograms showed P waves in all of these patients that were best seen in lead V4R. Twelve and 24 months postoperatively, only 2 patients had no visible P waves in the standard leads that were still present in the right precordial leads. However, if P waves were easily identifiable in standard 12-lead electrocardiograms (23 patients at 1 and 3 months after surgery), the right precordial leads showed P waves only in 15 patients. CONCLUSIONS: Right precordial electrocardiogram with leads V3R through V6R is a helpful tool for visualizing P waves after the Cox-maze procedure. Standard 12-lead electrocardiogram should be combined with right precordial electrocardiogram in all patients after the Cox-maze procedure.
BACKGROUND: Arterial pressures are described as an important factor in the development of graft degeneration and in reduced patency rate in vein bypass grafts. Sheathing of the graft with a pressure resistant mesh tubing might slow down this development. METHODS: Saphenous vein grafts were implanted into the carotid arteries of five pigs in order to evaluate the influence on myointimal hyperplasia of a compliant Phynox mesh tubing (a wrought Cobalt-Chromium-Nickel-Molybdenum-Iron Alloy), which surrounded autologous vein grafts that were exposed to arterial pressure. Each pig was operated on using a sheathed vein graft (biocompound-graft, a hybrid vascular prosthesis) on one side and an untreated saphenous vein on the other. RESULTS: After 4 weeks intimal hyperplastic changes were found in all histological sections. The wall thickness (medial and intimal layer) varied from 351 microm to 432 microm in the biocompound-graft and from 391 microm to 1196 microm in the native vein grafts (p < 0.05, n = 5). Severe myocytial and fibroblast proliferation was only found in the control grafts. Cellularity of the medial layer differed at sites of maximal cellular density and ranged from 11 to 12 cells in the biocompound-graft and from 17 to 18 cells per counting field in the native vein grafts (p < 0.05, n = 5). CONCLUSIONS: External support of vein grafts reduces intimal and medial layer proliferation. The findings of this study are in accordance with the results reported by other research groups.
BACKGROUND: There is controversy about the benefit of surgical repair for atrial septal defect in adults, especially its effect on the incidence of supraventricular dysrhythmias, atrial flutter and fibrillation. We studied their incidence before and after operation. METHODS: We examined surface and 24-hour Holter electrocardiograms before, early (between 3 and 7 days), and late (more than 6 months) after operation, performed at age 42.2 years (range, 18.5 to 74.9 years), in 211 adults with atrial septal defect. Patients were arbitrarily divided into three groups: age 18 to 40 years (n = 101), age 40 to 60 years (n = 83), and age more than 60 years (n = 27). All consecutive patients operated on between January 1988 and December 1996 and having a pulmonary to systemic flow ratio of 1.5:1 or greater were included in this study. RESULTS: The age of patients without arrhythmias before or after atrial septal defect closure (39+/-13 years) was significantly lower than that of patients with flutter (54+/-12 years) or fibrillation (59+/-8 years). The incidence of atrial flutter was influenced by surgical repair as atrial flutter converted to sinus rhythm late after operation in 10 of 18 patients. However, there was no change in the incidence of atrial fibrillation before (n = 28) and after (n = 21) operation. CONCLUSIONS: Our data show that surgical correction of atrial septal defect leads to regression of the incidence of atrial flutter but not fibrillation. Thus, surgical repair of atrial septal defect to abolish supraventricular tachyarrhythmias in adults is warranted, but in patients with fibrillation, it may have to be combined with a Maze operation in the future.
BACKGROUND: Lasting recovery from intractable end-stage dilated cardiomyopathy, which occurs with ventricular unloading, has recently been demonstrated in 5 patients. Here our extended clinical experience with the "weaning" concept is presented. METHODS: In 19 patients (23 to 65 years) with intractable end-stage dilated cardiomyopathy, ventricular assist devices were explanted after support periods of up to 26 months, when repeat off-pump studies had shown either restoration of cardiac function (left ventricular ejection fraction, > 45%) and dimensions (left ventricular internal diameter in diastole, < 55 mm) or partial recovery (left ventricular ejection fraction between 35% and 40%) with serious complications on the device. At the time of device placement left ventricular ejection fraction was below 20% and left ventricular internal diameter in diastole more than 64 mm and bridge-to-transplantation had been planned. RESULTS: Seven patients with persistently restored cardiac function for more than 8 months and 5 patients for less than 5 months after weaning were studied. Five patients with recurrent heart failure died within 4 to 8 months after explantation. Four patients had to be transplanted and 2 died for reasons unrelated to cardiac function. An individual optimal left ventricular ejection fraction and left ventricular internal diameter in diastole was reached before pump removal was actually conducted in all patients. These parameters gradually deteriorated until pump removal. CONCLUSIONS: Lasting recovery can be reached by ventricular unloading in a subset of patients with intractable end-stage dilated cardiomyopathy. Obviously, there is an individual optimum of recovery that cannot be further improved by prolonged unloading.
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OBJECTIVE: The Cox maze III procedure includes isolation of the pulmonary veins and multiple incisions in both atria in what corresponds to partial autotransplantation and partial denervation of the heart. The aim of this prospective longitudinal study was to identify physiologic effects of reinnervation on changes in heart rate at rest and in response to various stimulations and on atrial function after the Cox maze III procedure. PATIENTS AND METHODS: Power spectral analysis of heart rate variability, exercise testing, 24-hour Holter monitoring, electrocardiography, and transthoracic and transesophageal echocardiography were performed in 30 adult patients after the combined Cox maze III procedure and mitral valve surgery (maze group). They were prospectively followed up at 1, 3, 6, and 12 months after the operation. The results were compared with those of 15 heart transplant recipients (transplant group) and normal probands (healthy adults, n = 12). RESULTS: The physiologic effects of denervation with no differences in cardiac autonomic activity between the groups were seen early after the operation. Later, evidence of autonomic reinnervation was observed only in the maze group but not in the transplant group. Inappropriate heart rate responses during physical exercise were clearly evident in both groups after 1 and 3 months, with progressive improvement seen between 6 and 12 months only in the maze group. Left atrial function after the Cox maze procedure improved parallel to the recovery of sinus node function. CONCLUSION: Progressive improvement of sinus node function and atrial contractions with significant functional normalization 1 year after the Cox maze procedure corresponded to functional reinnervation and recovery of the autonomic nervous system.
This report describes three neonates who were supported with extracorporeal membrane oxygenation before surgical correction of total anomalous pulmonary venous connection. Extracorporeal membrane oxygenation was initially used to treat preoperative end-organ failure and suspected persistent pulmonary hypertension. All patients underwent surgical correction of total anomalous pulmonary venous connection after 8, 4 and 4 days of preoperative support, respectively. Two of these patients required extracorporeal membrane oxygenation after surgery; one died from bleeding while the other was weaned from extracorporeal membrane oxygenation on day 8 and discharged from the hospital. These results show that veno-arterial extracorporeal membrane oxygenation represents a life-saving perioperative means for supporting moribund neonates with total anomalous pulmonary venous connection and is effective in improving preoperative patient's condition.
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An infant with complete atrioventricular canal, total anomalous pulmonary venous drainage into the left superior vena cava and an unroofed coronary sinus successfully underwent total correction. A homograft vein was used to connect the proximal left superior vena cava to the right atrium and can be recommended when other methods of correction of unroofed coronary sinus cannot be used.
OBJECTIVES: Endoscopic transthoracic sympathicotomy (ETS) is a minimal invasive procedure of thoracic sympathetic blockage. The purpose of this study was to evaluate cardiac autonomic nervous activity after ETS in order to confirm the reliability and safety of ETS. METHODS: A series of electrophysiological studies were performed before and 1 week after bilateral 2nd and 3rd thoracic sympathicotomy in 13 patients with primary palmar hyperhydrosis. Palmar perspiration was measured under sympathetic stress, and body surface mapping was recorded in a supine position. In the head-up tilt test of 0, 30, 60 and 90 degrees, corrected QT interval (QTc) and T wave amplitude (Twa) were assessed. The power spectral analysis of heart rate variability was processed to attain power values of the low-frequency (0.04-0.15 Hz), the high-frequency (0.15-0.40 Hz) and the low/high frequency ratio. RESULTS: In all patients, the perspiration response on the palm to sympathetic stimulation was completely inhibited after ETS. Isointegral mapping revealed that ETS altered electroactivity on the heart. In the head-up tilt study, R-R intervals significantly increased after the surgery in the head-up tilt positions (P < 0.05), although there was no significant difference in the supine position. There is no significant difference in QTc and Twa before and after the surgery, both in the supine and the head-up tilt positions. There was no significant difference in the LF or HF before and after surgery, either in the supine position or the head-up tilt positions. In the LF/HF, there was no significant difference before and after surgery in the supine position. However, the LF/HF in the head-up tilt positions was significantly decreased after surgery (P < 0.05). Sympathetic suppression of ETS was recognized more obviously under the steeper head-up tilt positions. CONCLUSIONS: The influences on the cardiac autonomic nerve system of the ETS of upper thoracic sympathetic nerve were seen to be of a lesser degree at rest. However, the response to sympathetic stimulation was suppressed after the surgery.