Severe stomatitis complicating immune-suppressive switch after cardiac transplantation.

Everolimus is a recently developed immunosuppressive drug for patients following solid organ transplantation. Its mechanism of action, independent of calcineurin, is different from that of ciclosporin and tacrolimus and because of its lack of nephrotoxicity, it is a good alternative for calcineurin inhibitors in patients with renal dysfunction. In this paper we describe the case report of a 66-year-old caucasian female who underwent heart transplantation in December 2006. After induction with rabbit anti-thymocytic globulin, her immunosuppressive therapy comprised the combination of tacrolimus, mycophenolate mofetil (MMF) and steroids. Because of renal dysfunction, tacrolimus was changed for everolimus after 6 months. Unfortunately our patient developed severe stomatitis with aphthous ulcerations, shortly after the switch. Despite oral therapy (local anaesthetics), severe pain and malnourishment prompted interruption of everolimus and MMF and therapy was changed to ciclosporin and azathioprine. In addition, thalidomide was added. During the following weeks, there was progressive healing of the ulcerations. MMF was re-introduced and thalidomide was stopped after 6 weeks, without recurrent lesions after 4 months of follow-up.

Screening for solid organ malignancies prior to heart transplantation.

BACKGROUND: Prognosis of solid organ cancer in immunosuppressed hosts is generally dismal. Therefore, every effort to identify patients with asymptomatic carcinomas before transplantation should be encouraged. METHODS: Sixty-seven patients referred for heart transplantation were examined adhering to the scheme proposed at the 24th Bethesda Conference. To increase the sensitivity of this work-up, the following items were added: tumor marker assays (prostate-specific antigen in males, carcino embryogenic antigen), abdominal ultrasound, CT scan of the abdomen and the thorax, mammography/echography of the breasts, PAP smear, colonoscopy if carcino embryogenic antigen abnormal or occult blood in stool, prostate echography if prostate-specific antigen abnormal or prostate hypertrophy. RESULTS: Carcinoma was detected in 10 of the 67 patients; for 8 patients of this cancer group, transplantation was denied. Importantly, 9 of the 10 malignancies were detected by means of the diagnostic items that were added to the standard screening protocol. There were no significant differences between the cancer and the non-cancer group regarding mean age, sex, etiology of heart failure, and smoking history. Stratifying patients in younger (i.e., < or =54 years) and older (i.e., > or =55 years) age groups showed a significantly greater proportion of older patients in the cancer group (8/10=80%) compared to the non-cancer group (25/57=44%), P=0.04. After a mean follow-up of 34 months, 5 of the 36 transplanted patients developed a malignancy (4 skin carcinomas, 1 non-Hodgkin lymphoma). There have been no malignancy-related deaths until now. CONCLUSION: The importance of a thorough screening program in the triage of candidates with preexisting malignancies, especially in an older patient population, is illustrated in this report.

Paradoxic pulmonary vasoconstriction in response to acetylcholine in patients with primary pulmonary hypertension.

Pulmonary vascular reactivity was assessed during diagnostic heart catheterization in two patients with pulmonary hypertension unexplained by pulmonary or cardiac disease and in five patients with atypical chest pain and normal coronary arteriograms. Acetylcholine, an endothelium-dependent vasodilator that also has a direct contracting effect on vascular smooth muscle cells, was infused in the right atrium in a step-wise increasing dose in order to obtain final blood concentrations in the pulmonary circulation ranging from 10(-6) mol/L to 10(-4) mol/L. In the five control patients, acetylcholine induced a dose-related decrease of pulmonary vascular resistance (-52 percent +/- 9 percent). In the patients with primary pulmonary arterial hypertension, however, acetylcholine caused a paradoxic increase of pulmonary arterial pressure and of pulmonary vascular resistance. Thus, it appears that endothelium-dependent vasodilation is impaired in the pulmonary circulation of patients with primary pulmonary arterial hypertension. Endothelial dysfunction in the pulmonary circulation may play a role in the pathophysiology of this disease.

Ultrastructurally normal and motile spermatozoa in a fertile man with Kartagener's syndrome.

The findings in a 40-year-old man with Kartagener's triad (sinusitis, bronchiectasis, and situs inversus) and corrected transposition of the great vessels are presented. Electron microscopy revealed normal ultrastructure of the axoneme in both respiratory cilia and sperm tails. Light microscopic evaluation of the spermatozoa showed 50 percent motility, suggesting normal fertility. This assumption is confirmed, as the patient has two children. We suggest that an abnormal, uncoordinated motility pattern of the ultrastructurally normal respiratory cilia results in improper mucociliary clearance. This coordination is not needed in swimming spermatozoa, which could explain the apparent paradox between bronchopulmonary symptoms and normal fertility in our patient.

Spontaneous dissection of three major coronary arteries subsequent to cystic medial necrosis.

This case report describes the devastating consequences of spontaneous coronary dissection in a 36-year-old female patient. Surgical revascularization was attempted, but diffuse myocardial infarction developed. The patient was bridged to heart transplantation but died secondary to multiple organ failure. To our knowledge, this is the only reported case of spontaneous dissection of the three main coronary arteries due to severe cystic medial necrosis.

Coronary artery spasm complicating anaphylaxis secondary to skin disinfectant.

We report a patient in whom presumed vasospasm of an angiographically normal coronary artery led to severe transmural myocardial ischemia. To our knowledge, this is the first case in which an allergic reaction to locally applied chlorhexidine caused such a severe reaction.

Primary cardiac allograft failure after donor carbon monoxide poisoning treated with biventricular assist device.

Because of the current donor shortage, organ selection criteria are being progressively liberalized. We present a case of carbon monoxide poisoning in a multiorgan donor that led to primary cardiac allograft failure. A biventricular assist device was used as a bridge to recovery.

Isolated non-compaction of the left ventricle: a rare indication for transplantation.

This report describes the diagnostic difficulty encountered in a young female patient presenting with neurologic symptoms, atrial fibrillation and severe left ventricular systolic dysfunction, eventually leading to cardiac transplantation. The scrutiny used in the evaluation of the particular aspect of the left ventricle, and the integration of the information obtained from echocardiography, angiography and magnetic resonance imaging, led to the diagnosis of a rare and mostly unknown cause of cardiac failure. The correct identification of this entity is mandatory because enhanced risk of thromboembolism and malignant arrhythmia should be anticipated. A review of the literature revealed only 6 patients in whom isolated non-compaction of the left ventricle was treated by heart transplantation.

Semi-automatic external defibrillation and implanted cardiac pacemakers: understanding the interactions during resuscitation.

Many emergency medical service (EMS) systems are currently implementing semi-automatic external defibrillation (AED) by emergency medical technicians. Surprisingly little information is available on the possible interactions between AEDs and implanted cardiac pacemakers. Therefore, at present there are no clear guidelines for the use of AEDs on patients having a cardiac pacemaker. During resuscitation, multiple interactions between pacemakers and AEDs are possible. External defibrillation can cause damage to several functions of the pacemaker. On the other hand, the presence of pacemaker spikes during cardiac arrest might prohibit recognition of the ventricular fibrillation by the AED. We report on two resuscitation attempts in which the interaction between the ventricular fibrillation, an implanted dual chamber pacemaker and the AED was decisive for the defibrillation success. A clear understanding of these possible interactions is necessary for the further refining of diagnostic algorithms and clinical strategies of prehospital defibrillation.

Apical hypertrophic cardiomyopathy.

Illustrated by a case report, which was rather suggestive of coronary ischemic heart disease, the principle characteristics of apical hypertrophic cardiomyopathy are outlined. Outside East Asia, it remains a very uncommon variant of hypertrophic cardiomyopathy. The electrocardiogram in basal conditions showed giant negative T-waves (-14 mm) in the precordial leads, while the ventriculogram in RAO projection revealed the typical, so called "ace of spades" configuration. Based on the present literature and the follow-up of these patients a review of apical hypertrophic cardiomyopathy is presented. One of the most important consequences of the identification of this variant seems to be the rather good prognosis when compared to other forms of hypertrophic cardiomyopathy.

Blurred vision, left bundle-branch block and cardiac failure.

We cared for a patient with progressive renal impairment who presented with blurred vision, QRS broadening and cardiac failure due to chronic cibenzoline intoxication. Treatment consisted of catecholamines and repetitive infusions of Ringer lactate. Cardiac function and symptoms recovered completely.

Sudden death in hypertrophic cardiomyopathy: risk stratification and prevention.

Hypertrophic cardiomyopathy is an important cause of sudden death in young and asymptomatic patients. Young athletes and patients with unexplained sudden death, including their relatives, should be screened for its presence. Risk stratification identifies a high-risk group, in which preventive measures should be taken to reduce the risk for sudden death: moderate to heavy physical activity has to be avoided and arrhythmias with haemodynamic impact should be rigorously prevented.

Cardiac graft vasculopathy: aetiologic factors and therapeutic approaches.

Heart transplantation has become an accepted and successful treatment for end-stage heart disease with survival rates of 80-90% after one year and 70% after 5 years. During the early posttransplant period (6-12 months), patients are subject to two major complications: rejection and infection. Fortunately, a certain degree of graft tolerance develops, allowing the immunosuppressive therapy to be progressively tapered off with a concomitant decrease in infectious problems. Long-term survival, however, is largely dependent on the development of an accelerated atherosclerosis in the graft, which is believed to represent some kind of chronic rejection. This transplant vasculopathy is documented by means of coronary angiography with an incidence of approximately 5 to 10% per year. It is not surprising therefore that major interest has risen concerning risk factors promoting this pathology.

Clinical outcome of patients with an uncomplicated myocardial infarction: effect of revascularization.

In 80 patients (pts) with an uncomplicated myocardial infarction (MI) the rate of major cardiac events (MACE) including cardiac death, non-fatal myocardial infarction and recurrent ischemia requiring hospitalization was prospectively assessed over a mean follow-up period of 17 +/- 9 months and related to clinical, angiographic and scintigraphic findings, the latter obtained from adenosine Tc-99m sestamibi SPECT imaging. Decision for revascularization was mainly based on angiographic data and was carried out in a total of 50 patients (angioplasty in 34 pts and cardiac surgery in 16 pts). The overall MACE rate was 24% with a mortality and myocardial infarction rate of 4% and 5%, respectively. Early (< 2 months) revascularization seemed to have a beneficial effect on clinical outcome as was suggested by the following findings: 1) Cardiac events (MACE) were not significantly different in patients with versus without revascularization (MACE 24% versus 23%) although the former constituted a subgroup at higher risk for ischemic events because of a more extensive coronary artery disease state. 2) In the subset of patients with at least one significant coronary artery stenosis the clinical outcome was significantly better in those who were revascularized than in those who underwent no revascularization (MACE 24% vs 47%, p < 0.05. Among a variety of factors, including the scintigraphic and angiographic extent of coronary artery disease and post-MI treatment strategy, multivariate analysis selected hypercholesterolemia (> 240 mg%) as the only independent predictor of MACE with a more than fourfold increase in risk for development of MACE. These data suggest that the natural history, especially the rate of recurrent ischemic events, can be favourably changed by an elective and early revascularization, strategically oriented by the results of the angio-graphic study. Furthermore, our data emphasized the deleterious role of hypercholesterolemia on clinical outcome in patients with a recent MI.

Primary pulmonary hypertension in a patient with HIV infection.

Several case-reports and small series suggest a causal relationship between human immunodeficiency virus (HIV) infection and pulmonary hypertension. We report on a HIV seropositive man with a high and stable CD4 lymphocyte count (+/- 600/mm3) who developed severe pulmonary hypertension, not attributable to other known causes. This case report underscores the fact that the degree of immunosuppression secondary to the HIV-infection seems to be of little relevance in the pathophysiology of the syndrome. HIV-infected patients with dyspnoea, not related to pulmonary infection, with exercise intolerance, syncope or precordial pain should receive an electrocardiogram and echocardiographic assessment. The exact pathogenetic mechanism of this rapidly progressive disease and whether anti-viral therapy should be promoted is still under investigation.

Heart transplantation: how to select patients? What are the alternatives?

The gap between the number of potential recipients of a cardiac graft and the availability of donor hearts is still growing. A proper selection of heart transplant candidates is mandatory to ensure that patients in critical need, who are likely to benefit from this procedure, are identified. The work-up of a patient with terminal chronic heart failure includes a comprehensive cardiac and systemic evaluation. Critical in the decision is the exclusion of irreversible pulmonary hypertension. Furthermore, underlying diseases that could compromise short- and long-term prognosis after transplantation should be carefully assessed. The mortality on the waiting list and the growing pool of patients with chronic heart failure that is excluded from transplantation has stimulated the search for alternative treatment modalities. Besides a pharmacologic approach, the last few years have witnessed a tremendous evolution in so-called mechanical devices, designed to improve both morbidity and mortality of these patients. Although several of these devices have only just entered the clinical phase, internal cardioverter defibrillators, left ventricular assist devices and biventricular pacemakers can no longer be viewed as experimental tools.

Emergency ventricular assist device: better survival rates in non-post cardiotomy-related cardiogenic shock.

OBJECTIVE: The Abiomed BVS 5000 ventricular assist device (VAD) has been approved in Belgium for emergency cardiac support in patients with postcardiotomy failure with the aim of native heart function recovery. Other indications have emerged from world wide experience, but the indication and usefulness of emergency implantation of assist devices is often debated. METHODS: To decide which patients benefit most from emergency ventricular assist device implantation, we retrospectively reviewed our results of mechanical circulatory support with Abiomed in 20 patients over a 4-year period. Fifteen patients with mean age 58 +/- 6 years experienced postcardiotomy failure and underwent biventricular assist device (BVAD) implantation (group A), after elective (n = 9) or after emergency coronary artery bypass grafting (CABG) (n = 6). Five patients (group B), with mean age 35 +/- 19 years, had an implantation for other underlying conditions: hypertrophic cardiomyopathy (n = 3), myocarditis (n = 1) and primary cardiac allograft failure (n = 1). RESULTS: Of these two groups, eight and two patients respectively needed cardiopulmonary resuscitation before VAD implantation. The mean duration of support in both groups was 5.8 (range 12 h-13 days) and 4.4 days (range 2 h-9 days) respectively. Six and two patients could be weaned from the device and nine and one patients respectively, died on the device. Two patients in group B underwent successful heart transplantation and four patients in group A died after weaning. Two patients in the postcardiotomy group and four patients in group B survived (13% and 80%) with an overall survival and discharge rate of 30%. CONCLUSION: Although sample sizes are small, better survival rates with emergency Abiomed BVS 5000 implantation were obtained in the non postcardiotomy group (group B). For patients in the postcardiotomy group, outcome was negatively influenced by cardiac arrest and resuscitation before urgent CABG. Since death is the only alternative for these patients in cardiogenic shock and organ recovery cannot be predicted, we continue to consider emergency VAD implantation in this patient population.

Importance of characteristics and modalities of physical activity and exercise in the management of cardiovascular health in individuals with cardiovascular risk factors: recommendations from the EACPR. Part II.

In a previous paper, as the first of a series of three on the importance of characteristics and modalities of physical activity (PA) and exercise in the management of cardiovascular health within the general population, we concluded that, in the population at large, PA and aerobic exercise capacity clearly are inversely associated with increased cardiovascular disease risk and all-cause and cardiovascular mortality and that a dose­response curve on cardiovascular outcome has been demonstrated in most studies. More and more evidence is accumulated that engaging in regular PA and exercise interventions are essential components for reducing the severity of cardiovascular risk factors, such as obesity and abdominal fat, high BP, metabolic risk factors, and systemic inflammation. However, it is less clear whether and which type of PA and exercise intervention (aerobic exercise, dynamic resistive exercise, or both) or characteristic of exercise (frequency, intensity, time or duration, and volume) would yield more benefit for each separate risk factor. The present paper, therefore, will review and make recommendations for PA and exercise training in the management of cardiovascular health in individuals with cardiovascular risk factors. The guidance offered in this series of papers is aimed at medical doctors, health practitioners, kinesiologists, physiotherapists and exercise physiologists, politicians, public health policy makers, and individual members of the public. Based on previous and the current literature overviews, recommendations from the European Association on Cardiovascular Prevention and Rehabilitation are formulated regarding type, volume, and intensity of PA and regarding appropriate risk evaluation during exercise in individuals with cardiovascular risk factors.

ST-segment analysis: a useful marker for reperfusion after thrombolysis with APSAC? The Belgian EMS Study Group.

The European Multicentre Study (EMS) assessed coronary reperfusion, functional outcome and safety of anistreplase (anisoylated plasminogen streptokinase activator complex = APSAC) compared to a control group treated with heparin alone in patients with a clinical diagnosis of acute myocardial infarction (AMI). In the Belgian subset of data (n = 103) the reperfusion results, based on non-invasive clinical parameters, were significantly better in the APSAC group: 66% versus 18% in the heparin group (P less than 0.0001). No significant difference was found between the two treatment groups with respect to left ventricular function and adverse events. The purpose of the present study was to analyse the indicative value of ST-segment changes as a possible predictor of reperfusion in threatened myocardial tissue. Two scoring systems were used: the first was based on the combined evaluation of three clinical non-invasive parameters (course of chest pain, ECG evaluation of ST segment changes, reperfusion arrhythmias); the second was based on the changes of the sum of the ST-segment (sigma-ST) evaluations on consecutive 12-lead ECGs. There was a good correlation between the combined clinical scoring system and the residual stenosis on coronary angiography (P less than 0.05) and early CK peak (P less than 0.0001). Analysis of the ECG data revealed that a decrease of greater than or equal to 50% of the sum of ST-elevations (sigma-ST) at 2 h post-treatment in both limb and precordial leads is a fairly useful predictor of reperfusion (sensitivity = 73%, specificity = 63%, predictive value = 88%).