Biomedical subjects
Publications and source records attributed to E Oechslin.
The publisher regrets that this was an accidental duplication of an article that has already been published in Eur. J. Echocardiogr., 4 (2003) 223-225, . The duplicate article has therefore been withdrawn.
OBJECTIVE: To investigate the predictors for adverse clinical outcome in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) during long term follow up. METHODS: 61 patients with ARVD/C were studied to assess the impact of family history, clinical findings, surface ECG parameters, echocardiographic findings, and electrophysiological findings on clinical outcome. The prevalence of these risk factors were compared in two patient groups: group A (patients with adverse clinical outcome: sudden cardiac death, death from heart failure, or heart transplant) and group B (survivors excluding patients who received a heart transplant). RESULTS: Mean age at first diagnosis was 44 (14) years. The mean follow up duration was 55 (47) months. Ten patients (16%) died during follow up. The cause of death of eight of these patients was probably arrhythmic. Two patients died of advanced heart failure. Five patients underwent heart transplantation because of terminal heart failure. Risk factors significantly associated with adverse outcome were history of congestive heart failure (p < 0.001), the presence of left ventricular involvement on echocardiography (p < 0.001), left atrial dilatation (p < 0.05), prolonged PR duration (p < 0.01), prolonged QRS in V1 (p < 0.05), and bundle branch block (p < 0.05). In multivariate analysis, history of congestive heart failure and presence of left ventricular involvement were identified as independent risk predictors for an adverse outcome. CONCLUSIONS: Congestive heart failure and left ventricular involvement are independently associated with adverse outcome in patients with ARVD/C during long term follow up.
While considering long-term oral anticoagulation one should assess benefit (i.e., reduction in thromboembolic events) and risks (i.e., bleeding complications) associated with therapy for each individual patient. The classic cardiac indications for oral anticoagulation include chronic atrial fibrillation, prosthetic heart valves, and left ventricular thrombus formation following anterior myocardial infarction. The value of anticoagulation in patients with impaired left ventricular function in stable sinus rhythm and in secondary prevention of coronary artery disease remains controversial. For decades warfarin has been the only compound available. Currently, promising results have been achieved with the oral thrombin inhibitor ximelagatran. In the future, oral anticoagulants, which are administered in fixed dose with no need for monitoring of the anticoagulation level, may replace warfarin. Safety and efficacy of double antiplatelet therapy (aspirin and clopidogrel) in the secondary prevention of thromboembolic events in patients with atrial fibrillation are currently being addressed in large-scale clinical trials.
Intractable ventricular tachycardia was investigated in a 51-year-old man with isolated left ventricular non-compaction during implantation of an automated internal cardioverter-defibrillator. Favorable bridging to cardiac transplantation was achieved with the DeBakey left ventricular assist device (LVAD).
OBJECTIVE: The MicroMed DeBakey left ventricular assist device (LVAD) axial blood flow pump was used as bridge to heart transplantation (HTx) in patients with terminal heart failure. The aim was to evaluate this novel mechanical circulatory support system in regard to overall outcome. METHODS: Prospective study in 15 HTx candidates (mean age 40+/-7 years) with terminal heart failure and maximal medical treatment due to ischemic cardiomyopathy (CMP, n=5), dilated CMP (n=3), restrictive CMP (n=2), unclassified CMP (n=1), metabolic CMP (n=1), valvular CMP (n=1) and congenital CMP (n=2). All patients were implanted with a MicroMed DeBakey LVAD. A rescue procedure was necessary in eight critical patients, while seven underwent elective LVAD implantation. Procedures were performed via median sternotomy, in normotherm femoro-femoral CPB (mean duration 59+/-1 min). Oral Marcoumar (INR 2.0-3.0) and Aspirin (100 mg daily) were started as soon as possible. Patients were discharged into a specialized rehabilitation clinic from which it was possible to release them home after a few weeks. RESULTS: Successful implantation and discharge from ICU (mean stay 10+/-7 days) was possible in 11 patients. Seven were transplanted (mean support 50.7 days) and one is awaiting HTx (support >310 days) in the comfort of his home (NYHA I). Survival was 100% among the transplanted patients. Of the seven elective implants, five, and of the eight rescue procedures three patients underwent successful HTx. Four patients died early, while three patients died late on pump support due to intracranial hemorrhage (n=2, 73 and 76 days) and chest infection (n=1, 124 days). All survivors were discharged from hospital, with significant decrease in NYHA class (mean 3.8-2.4 (n=11)). Treadmill testing showed increased exercise tolerance, from 35 to 71W (n=4). Plasma BNP values (mean 950-162 ng/l (n=4)) and pulmonary resistance (mean 316-194.5 dyne s/cm(5) (n=3)) decreased significantly during LVAD support. CONCLUSIONS: The MicroMed DeBakey LVAD is simple to implant; outpatient treatment is safe and efficient. Patients' condition and pulmonary resistances normalize within 6 weeks, making previously considered inoperable patients amenable for HTx. HTx can be performed in low-risk situation, allowing better donor-recipient matching and improving overall outcome.
Diverticula and aneurysms are rare congenital anomalies of the right atrium. Here, we report a case of a giant congenital diverticulum of the right atrium in a 27-year-old female and discuss the morphological characteristics distinguishing diverticula and aneurysms.
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A 77 year old female patient was admitted to our hospital because of a rapid onset left-sided visual loss. Occlusion of the left central retinal artery was confirmed by the consultant ophthalmologist. The patient was further evaluated to exclude a thromboembolic event. Chest x-ray was consistent with pulmonary hypertension. Doppler echocardiography revealed a secundum atrial septal defect with a bi-directional shunt. In this 77 year old patient, arterio-arterial embolism from atherosclerotic plaques is the most probable cause of her central retinal artery occlusion. Yet, paradoxical embolism might be another reason for acute visual loss in this setting, which, however, is difficult to confirm definitively.
BACKGROUND: Recommendations for treatment of mechanical prosthetic heart valve thrombosis (PVT) include systemic thrombolysis and/or reoperation. Data on complications and outcome are limited. METHODS: Clinical and echocardiographic findings of 17 patients with mechanical PVT were reviewed. Complications and outcome of surgery and/or thrombolysis were analysed. Prospective follow-up was obtained. RESULTS: Symptomatic PVT occurred 8.4 +/- 7.2 years after mechanical valve replacement at mean age 55 +/- 15 years. Thrombosis involved the mitral valve in 12 patients (71%), the aortic valve in 4 (24%) and the tricuspid valve in one (6%). The reason for PVT was inadequate anticoagulation in 11 patients (65%), endomyocardial fibrosis in 2 (12%) and unknown in 4 (24%). Prior to diagnosis, systemic emboli occurred in 6 patients (35%). Thirteen patients (76%) presented in functional class NYHA IV. Haemodynamic valve obstruction was documented by echocardiography in 15 patients (88%). Treatment included primary reoperation in 12 patients (71%), thrombolysis with urokinase in 3 (18%) (with reoperation in 1), reinstitution of adequate anticoagulation in one (6%); death occurred before treatment in one (6%). Intraoperatively, both pannus and thrombus were found in 5 of 13 patients (38%). Treatment-related emboli occurred in 5 patients (29%), to the brain in 3, to the legs in one and to a coronary artery in one. Five patients died (mortality 29%) within 30 days due to multiorgan failure/septicaemia (3 patients), congestive heart failure (1), or cerebral emboli (1). Follow-up after 28 +/- 28 months in the 12 surviving patients was unremarkable. CONCLUSIONS: The most common aetiology for obstructive PVT is thrombus formation due to inadequate anticoagulation. PVT remains a serious complication with high morbidity and mortality despite aggressive treatment by thrombolysis and/or surgery. Surgery is often needed due to the frequent presence of pannus and/or large thrombi. However, long-term prognosis after successful treatment of PVT is excellent.
A role of the potent and long-acting vasoconstrictor peptide endothelin-1 and the pathophysiology of chronic human heart failure has been postulated based upon indirect evidence such as elevated plasma endothelin-1 levels and their with the degree of hemodynamic impairment. The advent of specific of endothelin-1 receptor antagonists has provided the opportunity not only to directly evaluate its pathophysiological role but also to assess its potential role as a new approach to heart failure therapy. This brief review summarizes the evidence linking endothelin-1 to the pathophysiology of chronic heart failure and the clinical results obtained in patients during acute, intravenous and more prolonged, oral administration with bosentan, a mixed ET(A)/ET(B)-receptor antagonist. Bosentan acutely and during short-term oral therapy markedly improved hemodynamics in patients in addition to standard heart failure therapy, including an ACE-inhibitor. These effects were associated with a reduced responsiveness of the renin-angiotensin system to diuretic therapy and reduced basal plasma aldosterone levels. Although the hemodynamic and neurohumoral profile of short-term bosentan therapy looks promising for the treatment of patients with chronic heart failure appropriate trials will have to be performed to document clinical benefit during long-term therapy. Finally, the question remains open whether mixed endothelin-1 receptor antagonists like bosentan will have similar effects as compared to antagonists which block the ET(A) receptor only.
A growing, heterogeneous group of children with congenital heart disease is surviving into adulthood due to advances in medicine. These patients including those with simple and complex congenital heart disease and operated on during childhood are facing long-term complications. Superspecialist care and expertise are required during their life to deal with their unique problems the most common being ventricular failure, arrhythmias, valve and conduit longevity. Teenagers and adolescents disappear from both medical and parental care because of the lack of transition programs. Transition of care from pediatric to adult cardiologists must be organized in each country and must reflect regional history, regional politics and realities. Transition of care requires goodwill from parents, adolescents, pediatric and adult cardiologists. Transition clinics being held jointly by pediatric and adult cardiologists between the age of 16 and 18 years are essential to encourage the adolescents to take charge of their own life and health issues. Adequate information about their heart defect, their operations and their residual lesions may help them understand the implications for the future and improve their compliance. A transition program must include counseling on education, career, endocarditis prophylaxis, insurance and lifestyle issues such as sexuality and reproduction (including anticonception, pregnancy), cardiovascular risk factors and sports activities. Medical reports including operative reports and heart catheterization reports must be transferred to the adult cardiologists. In Switzerland, care of adults with congenital heart disease is based on three levels: 1) primary caregivers including general practiioners, internists and community cardiologists; 2) cardiologists with special commitment and expertise to patients with congenital heart disease who organize regional outpatient clinics; 3) supraregional referral centers with cardiologists trained in pediatric and adult congenital heart disease and experienced in the special needs, problems and management of this unique population. A close collaboration between pediatric and adult cardiologists participating jointly in the care of congenital heart disease patients is very important. An interdisciplinary team offers all non-invasive and invasive facilities for diagnostic and therapeutic procedures in a supraregional referral center. A close collaboration among all physicians is crucial for optimal care and management. Integration of adolescents with congenital heart disease into an adult medical system improves both compliance and quality of care and supports the patient's esteem because many medical aspects and lifestyle issues are completely different from those during childhood.
BACKGROUND: Patients with prosthetic heart valves have an increased risk of thromboembolic events, and transcranial Doppler sonography reveals microembolic signals. Whereas microembolic signals were initially assumed to be of particulate matter, recent studies suggest that they are partially gaseous in origin. If this is true, alteration of environmental pressure should change microembolic signal counts. We undertook this study to evaluate the influence of hyperbaric exposure on microembolic signal counts in persons with prosthetic heart valves. METHODS AND RESULTS: Microembolic signal counts were monitored by transcranial Doppler sonography of both middle cerebral arteries under normobaria (normobaria 1), 2 subsequent periods of hyperbaria (2.5 and 1.75 bar), and a second period of normobaria (normobaria 2) in 15 patients with prosthetic heart valves. Each monitoring period lasted 30 minutes. Compression and decompression rates were 0.1 bar/min. Microembolic signal counts increased from 20 (12-78) at normobaria 1 to 79 (30-165) at 2.5 bar (P <.01 vs normobaria 1 and 2), decreased to 44 (18-128) at 1.75 bar (P <.01 vs normobaria 1 and 2.5 bar; P <.001 vs normobaria 2), and returned to 20 (8-96) at normobaria 2 (values are medians and 95% confidence intervals). CONCLUSIONS: Our results strongly suggest that gaseous bubbles are underlying material for part of the microembolic signals detected in patients with prosthetic heart valves.
A role of the potent and long-acting vasoconstrictor peptide endothelin (ET)- I in the pathophysiology of chronic human heart failure has been postulated, based upon indirect evidence such as elevated plasma ET-1 levels and their relationship to the degree of haemodynamic impairment. Acute heart failure shares many features of chronic heart failure, albeit in an exaggerated fashion. As both the mixed ETA/ETB-receptor antagonist bosentan and the selective ETA receptor antagonist BQ 123 acutely improved the haemodynamics of chronic heart failure patients, there seems to be good reason to believe that ET-1 receptor antagonism may also be of benefit in the setting of acute heart failure. However, appropriate trials will have to be performed to document the clinical benefit of such an approach. Finally, the question remains open as to whether mixed ET-1 receptor antagonists like bosentan will prove better, worse or equal to antagonists that block the ETA, receptor only.
AIM: To determine clear cut echocardiographic criteria for isolated ventricular non-compaction (IVNC), a cardiomyopathy as yet "unclassified" by the World Health Organization. The disease is not widely known and its diagnosis mostly missed. METHODS AND RESULTS: In seven out of a series of 34 patients with IVNC the in vivo echocardiographic characteristics were validated against the anatomical examination of the heart removed after death in four and due to heart transplantation in three patients. Four morphological criteria diagnostic for IVNC were found. (1) Coexisting cardiac abnormalities were absent (by definition). (2) A two layer structure was seen, with a compacted thin epicardial band and a much thicker non-compacted endocardial layer of trabecular meshwork with deep endomyocardial spaces. A maximal end systolic ratio of non-compacted to compacted layers of > 2 is diagnostic. (3) The predominant localisation of the pathology was to mid-lateral (seven of seven patients), apical (six), and mid-inferior (seven) areas. The pathological preparations confirmed the echocardiographic findings. Concomitant regional hypokinesia was not confined to the non-compacted segments. (4) There was colour Doppler evidence of deep perfused intertrabecular recesses. CONCLUSIONS: Four clear cut echocardiographic diagnostic criteria were established. It is suggested that the WHO classification of cardiomyopathies be reconsidered to include IVNC as a distinct cardiomyopathy.
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The transplantation of a heart restores the quality of life and productivity to patients who have experienced severe loss of contractility of heart muscle during an infection or as a result of long-lasting chronic heart disease. Monitoring for allograft rejection requires expensive procedures whereby, with catheterisation of the right heart, endomyocardial biopsies are retrieved. Evaluation by the pathologist uses a standardised diagnostic scheme which is in use worldwide. We have tested the interobserver agreement in a very heterogeneous group of examining pathologists and found moderate agreement with a kappa value of 0.52. The development of other methods to improve and even to replace invasive procedures for monitoring acceptance/rejection of a cardiac allograft requires interdisciplinary cooperation.
Heart transplantation has been established as therapy in patients with terminal heart failure who remain severely symptomatic (NYHA III-IV) despite optimal drug therapy and surgical interventions other than transplantation. In addition to symptoms, various objective criteria are used to determine the patients most suitable for transplantation. Of these, peak oxygen consumption below 12-14 ml/kg/min, no irreversibly elevated pulmonary resistance, and no major concomitant disease are most important. However, conventional therapy of advanced heart failure has considerably improved over the last decade. Thus, heart transplantation may be avoided or at least postponed in many patients. Nevertheless, continuous treatment at a heart failure/heart transplantation clinic in collaboration with the general practitioner is essential in these patients. This may allow to closely monitor these severely ill patients and to select the optimal point of time for transplantation. Prognosis after heart transplantation is relatively good with a 10-year survival of 63%. However, graft coronary artery disease and lymphomas are still unresolved problems which limit the success of heart transplantation. Competent clinical monitoring, aggressive therapy of conventional risk factors and good co-operation of patients and doctors are the basis for a successful outcome.