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Large bilateral pulmonary artery aneurysms in Behçet's disease: rupture of the contralateral lesion after aneurysmorrhaphy.

An 18-year-old man presented with Behçet's disease and two large bilateral pulmonary artery aneurysms. The day following aneurysmorrhaphy of the right aneurysm, a massive hemoptysis led to death. Postmortem examination disclosed rupture of the left aneurysm in a segmental bronchus. The aneurysms had inflammatory walls and were associated with organized pulmonary arterial thromboses and endomyocardial fibrosis of the right ventricle. Our case report highlights the surgical risk of rupture of associated arterial lesions, certainly related to per- and postoperative pulmonary arterial pressure variations.

Adolescent↗

Echocardiographic diagnosis of heart disease in Uganda.

Five hundred consecutive cardiological patients were reviewed and evaluated by echocardiography in Mulago Hospital as a referral service. All diagnostic problems in cardiology in the hospital and to a certain degree from other institutions were reviewed. The commonest diagnosis was endomyocardial fibrosis (EMF) 19.8%, followed by congenital heart disease (CHD) 15% and rheumatic heart disease (RHD) 11%. The anatomical distribution of fibrotic lesions in EMF (left, right or biventricular) correlated with that found in previous autopsy series from Mulago Hospital thus demonstrating the accuracy of echocardiography. We were able to quantify the contribution of echocardiography to a correct diagnosis which varied between 13% in RHD to 90% in mitral valve prolapse (MVP). Echocardiography was able to confirm the clinical diagnosis of subacute bacterial endocarditis (SBE) in 69% of cases. The obtained data suggests that EMF may be the most common heart disease in Uganda contrary to the pattern of heart disease outlined in earlier works. The favourable cost/benefit ratio of echocardiography (i.e. its easy availability and maintenance), as well as the absence of consumable materials in comparison to other cardiac diagnostic tools such as catheterization, chest X-ray and ECG is emphasized, together with the multipurpose use of the machine.

Adolescent↗

Cerium depresses endocardial endothelial cell-mediated proliferation of cardiac fibroblasts.

Cerium has been implicated in the pathogenesis of cardiac disorders such as acute myocardial infarction and endomyocardial fibrosis (EMF). A geochemical hypothesis for the causation of EMF linked the cardiac lesions to magnesium deficiency consequent to malnutrition and increased cardiac levels of cerium derived from monazite soils in the coastal regions of the tropics. We tested the hypothesis that the stimulus for fibroblast proliferation and enhanced collagen synthesis in EMF is derived from cardiac endothelial cells activated or injured by cerium. We explored whether endocardial endothelial cells exposed to cerium secrete factors responsible for the increased proliferation and collagen synthesis in cardiac fibroblasts. Our results suggest that the growth response of cardiac fibroblasts to cerium is not mediated through growth factors secreted by endocardial endothelium and that the cardiac lesions in EMF result from direct stimulation of subendocardial fibroblasts by cerium.

Animals↗

Myocardial late enhancement in contrast-enhanced cardiac MRI: distinction between infarction scar and non-infarction-related disease.

OBJECTIVE: Our objective was to assess and compare the patterns of late enhancement (LE) in contrast-enhanced cardiac MRI caused by myocardial infarction and different myocardial diseases that are not related to ischemic infarction. MATERIALS AND METHODS: A total of 811 consecutive contrast-enhanced cardiac MRI studies performed for different indications were reviewed for left ventricular myocardial LE after gadopentetate dimeglumine administration. MRI studies were performed on a 1.5-T scanner using an inversion recovery turbo FLASH sequence (TR/TE, 8/4 msec; flip angle, 25 degrees). The LE pattern of ischemic infarction scar was compared with that in nonischemic myocardial disease. RESULTS: LE was found in 421 (52%) patients. In all patients with myocardial infarction, LE included the subendocardial layer. Nineteen patients without history of myocardial infarction and angiographically excluded coronary artery disease showed different patterns of LE caused by myocarditis, sarcoidosis, arrhythmogenic right ventricular dysplasia, cardiomyopathy, endomyocardial fibrosis, and iatrogenic scars after biopsy, ablation of septal hypertrophy, and myocardial laser revascularization. CONCLUSION: LE in contrast-enhanced cardiac MRI is not specific for ischemic infarction. LE in ischemic infarction always involves the subendocardial layer, whereas it does not necessarily do so in other myocardial diseases. Therefore, if LE omit the subendocardial layer, different nonischemic myocardial diseases have to be considered. The pattern of LE might be helpful for the differential diagnosis of myocardial disease and in distinguishing it from ischemic disease.

Adult↗

Symptomatic mechanical heart valve thrombosis: high morbidity and mortality despite successful treatment options.

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.

Adult↗

[Congenital atrioventricular block].

Congenital atrioventricular block is defined (Yater) as a documented conduction defect in a young subject with unquestionable bradycardia in the absence of a history of infection which might have caused the condition after birth. It is a rare condition (1 out of 20,000 births) and may be isolated or associated with another congenital malformation. Four cardiac malformations are classically associated: endomyocardial fibrosis, morphological abnormalities close to the conduction system: corrected transposition, left isomerism; patent ductus arteriosus and atrial septal defect apparently unrelated but the most common; the association of mitral regurgitation in adults, although the significance is not very clear. Isolated block is often observed in patients with mothers suffering from autoimmune disease, often clinically latent. Anti Ro/SS-A and La/SS-B antibodies cross into the foetal circulation and cause inflammation of the conduction tissues but the causal mechanism is not known. The diagnosis of the conduction defect is sometimes made during foetal life by echocardiography. After birth, the diagnosis is made by electrocardiography but the block is not always complete or permanent, its degree often increasing with time. In addition, in advanced degrees of block, the escape rhythm tends to slow down. Long-term follow-up studies have revised the previously considered good prognosis of isolated congenital atrioventricular block but advances in cardiac pacing provide satisfactory treatment. In the foetus, isolated atrioventricular block is usually associated with an escape rhythm > 60/min and enables normal vaginal delivery; a low heart rate < 55/min and anasarca carry a poor prognosis. In the neonate, pacing is indicated in babies with cardiac failure and a heart rate < 55/min. Follow-up by Holter monitoring, exercise testing and echocardiography is justified in children and adolescents; the patients may become symptomatic at any age. Pacing is essential in symptomatic cases (malaise, ventricular dysfunction) and useful in cases with long QT intervals, frequent ventricular extrasystoles and wide ventriculogrammes. Pacing is not always easy in children. Epicardial pacing by thoracotomy or an epigastric approach is possible but endocavitary pacing is to be preferred using thin pacing catheters introduced via the subclavian vein and small pacemakers implanted in a sub or prepectoral site. A pacing mode which restores the normal atrioventricular sequence is theoretically superior to single ventricular pacing even if rate responsive frequency.

Adult↗

[Dwarfism and constrictive pericarditis. Apropos of a case of so-called Mulibrey dwarfism with hypereosinophilia].

A child of 10 was admitted to a cardiology unit with adiastole. The marked degree of dwarfism and anatomical features were characteristic of the so-called 'Mulibrey' dwarfism. Surgical intervention confirmed the constrictive pericarditis which is normally found in this condition. Unfortunately, the adiastole persisted which, taking into account the marked eosinophilia, made an associated endomyocardial fibrosis seem likely. The distinctive features of constrictive pericarditis and of the constrictive type of cardiomyopathy are recalled.

Cardiomyopathies↗

[Multiple organ failure in patients with hemoblastosis during long-term follow-up of treatment].

AIM: Investigation of the condition of the liver, gastrointestinal tract, heart in patients at the stage of clinicohematological remission after treatment for hemoblastosis (acute leukemia, Hodgkin's disease, non-Hodgkin's lymphoma). RESULTS: Marked functional-morphological changes were found: endomyocardial fibrosis, cardiopathy, pulmonary hypertension, chronic atrophic gastritis, colon lesions, dysbacteriosis, viral or drug-induced hepatitis. CONCLUSION: The above affections have developed because of the treatment: chemotherapy, radiotherapy, hemotransfusions, antibacterial drugs. They deteriorate life quality and require a special system of rehabilitation measures.

Acute Disease↗

[Cardiovascular manifestations of Behçet's disease].

Vascular involvement in Behçet's disease, recognized since 1946, is peculiar since it occurs in young subjects with no vascular risk factor. Recurrent phlebitis, sometimes associated with fever and biological signs of inflammation, commonly involve the large vessels (superior and inferior vena cava, hepatic veins) and cerebral veins. Arterial involvement was more recently identified and is expressed by thrombosis, stenosis and/or aneurysms diversely associated. Aneurysms, true "arterial aphthae", may be multifocal and can involve all arterial territories with a clear preference for the abdominal aorta and the pulmonary arteries (Hughes-Stovin syndrome). Mortality is significant due to rupture and the risk of recurrence. Cardiac involvement includes coronary artery disease which merits attention since it affects young subjects and is often expressed by myocardial infarction or angina; all three tunics can be involved; mortality is high: 20% in the months or years following diagnosis. A few cases of recurrent pericarditis have been reported. Myocardiopathy can be of inflammatory nature or secondary to coronary artery disease. Endocardiac involvement may be limited to valve disease or spread to the ventricular wall. Endomyocardial fibrosis is exceptional and usually associated with intracavitary thrombus formation. Coagulation disorders have been reported but they cannot explain the different thrombotic manifestations which are probably the consequence of an abnormal response of the vascular endothelial cells.

Behcet Syndrome↗

[Loffler's fibroblastic endocarditis. A report of a case complicating toxocarosis].

The authors report a case of Löffler's fibroblastic endocarditis complicating a toxocarosis infection. Parasitic infestation with toxocara canis is usually asymptomatic, but this was a very rare observation of cerebral involvement associated with symptomatic Löffler's endocarditis. This is an unusual form of restrictive cardiac disease constantly accompanied by prolonged hypereosinophilia. In addition to the classical signs of cardiac failure, an acute febrile illness imitating a connective tissue disease may be observed. Echocardiography helps diagnosis by showing endomyocardial fibrosis and adherent thrombosis at one or both ventricular apices. The management of cardiac failure should include, whenever possible, radical treatment of the hypereosinophilia. At an advanced stage, surgical endocardial decortication is the only means of improving symptoms and the prognosis of these patients.

Coronary Thrombosis↗

Cardiac disease in Malawi.

A prospective clinical study of 114 Malawian patients with cardiac disease seen at Queen Elizabeth Central Hospital, Blantyre, is presented. Rheumatic and hypertensive heart disease, cardiorenal failure and severe anaemia were the most common causes. Pericardial disease, mainly due to tuberculosis, was an important treatable cause of cardiac disease. Endomyocardial fibrosis was not diagnosed with certainty and cardiomyopathy was relatively uncommon. Ischaemic heart disease was not seen.

Adolescent↗

[Sudden death and physical activity and sports].

A growing number of people are involved in recreational physical activity. We examined the clinical and cardiac pathological patterns in 29 persons who died suddenly during a sport activity and were autopsied at the Institute of Forensic Medicine of Paris, 1990-1999. Twenty-seven males ranging in age from 13 to 57 years, mean 30.5, and two females, 8 and 60 years old, died suddenly during a sport activity. The sports included running, 11 cases; cycling, 4; soccer, 3; swimming, 2; basket-ball, 2; rugby, 1, gymnastic exercises, 2; tennis, 1; gockart, 1; skiing, 1; hand-ball, 1 case. The causes of deaths were as follows; coronary artery disease, 9 cases; congenital anatomic anomaly of a coronary artery, 1; hypertrophic cardiomyopathy, 5; right ventricular dysplasia, 3; myocarditis, 2; endomyocardial fibrosis, 1; bridging of the left anterior descending coronary artery, 2; stroke, 2; rupture of aortic aneurysm, 4. Despite severity of lesions, only 4 victims had a known cardiovascular disease. Efforts should be continued to improve sensitivity and specificity of diagnostic tools and screening strategies. Therefore, medico-legal autopsies should be systematically performed for a better knowledge of sport-related mortality.

Adolescent↗

Atrial flutter in fetuses and early childhood: a report of eight cases.

Atrial flutter is an uncommon arrhythmia in children. The presenting features and treatment responses of two fetuses and six children with atrial flutter were presented. Two patients were diagnosed prenatally at 31 and 35 weeks' gestation. One patient was successfully controlled in utero by maternal administration of digoxin and the other by direct current cardioversion after delivery. Two fetuses and 2 of 6 children had normal cardiac structure by echocardiogram. Three patients had right atrial enlargement due to atrial primun septal defect, secundum atrial septal defect with primary pulmonary hypertension, and endomyocardial fibrosis. One patient developed atrial flutter with sinus node dysfunction after surgical correction of total anomalous pulmonary venous connection. Four cases presented as atrial flutter with regular atrioventricular conduction (2:1 or 3:1 conduction) while the remainder presented with irregular atrioventricular conduction. Heart failure was presented in two patients and improved after the arrhythmias were controlled. Successful termination of atrial flutter was accomplished by using DC cardioversion in 4 patients and one patient converted to normal sinus rhythm following combination of digoxin and amiodarone administration. Digoxin was administered for one year as the prophylactic medication in six patients. None had recurrence of atrial flutter during the follow-up period of one to four years. Two patients required chronic digoxin and amiodarone therapy. We concluded that atrial flutter in fetuses and early childhood carries a good prognosis. Acute treatment with direct current cardioversion is sufficient in most patients, combination of digoxin and amiodarone may be an alternative therapy to convert atrial flutter.

Anti-Arrhythmia Agents↗

[Investigation of chronic hypereosinophilia: new perspectives].

In some circumstances and despite investigations, no aetiology is found for hypereosinophilia. This becomes of particular concern when hypereosinophilia is elevated and persistent. While some chronic hypereosinophilias signal the onset of malignant haemopathies, others remain unexplained for up to several years. But all may cause severe complications to the viscera, among which cardiopathies, and in particular endomyocardial fibrosis, are predominant. New data on chronic unexplained hypereosinophilia point to regulation defects in eosinophils or more often in T lymphocytes. Thanks to simple investigations such as flow cytometry, along with other more sophisticated procedures, we should soon be better equipped to classify chronic hypereosinophilia and, in the long term, to define more adequate strategies for treatment.

Antigens, CD↗

Atrial fibrillation, the arrhythmia of the elderly, causes and associated conditions.

Atrial fibrillation (AF) is a common clinical problem, particularly in the elderly, and in patients with organic heart disease. A small percentage of patients, have a potentially reversible cause. Atrial fibrillation is in most patients (approximately 70%) associated with chronic organic heart disease including valvular heart disease, coronary artery disease, hypertension, particularly if left ventricular hypertrophy is present, hypertrophic cardiomyopathy, dilated cardiomyopathy and congenital heart disease and most commonly in adults, atrial septal defect. As in many chronic conditions, determining whether AF is the result or is unrelated to the underlying heart disease, remains unclear. The list of possible etiologies also include cardiac amyloidosis, hemochromatosis and endomyocardial fibrosis. Other heart diseases, such as mitral valve prolapse (with or without mitral regurgitation), calcification of the mitral annulus, atrial myxoma, pheochomocytoma and idiopathic dilated right atrium, present a higher incidence of AF. The relationship between these findings and the arrhythmia are still unclear. Atrial fibrillation may occur in the absence of detectable organic heart disease, the so-called "lone AF", in about 30% of cases. The term "lone AF" or "idiopathic AF" implies the absence of any detectable etiology including hyperthyroidism, chronic obstructive lung disease, overt sinus node dysfunction, and overt or concealed preexcitation (Wolf-Parkinson-White syndrome), only to mention a few of other rare causes of AF. In every instance of recently discovered AF, thyrotoxicosis should be ruled out. The autonomous nervous system may contribute to the occurrence of AF in some patients. Atrial fibrillation occurs commonly in patients with valvular heart disease, particularly when it involves the mitral valve. The occurrence of AF is unrelated to the severity of mitral stenosis but is more common in patients with enlarged left atrium and congestive heart failure. In patients with coronary artery disease, Af occurs predominantly in older patients, males and patients with left ventricular dysfunction. Important predictive factors of AF include hypertension, left ventricular hypertrophy and diabetes. However, the relation between AF and hypertension remains unclear. The risk of the development of AF, in an individual patient, is often difficult to assess but increasing age, presence of valvular heart disease and congestive heart failure, increase the risk of AF.

Age Factors↗

Cardiac pathology of permanent pacemaker implementation.

The cardiac findings in five cases of permanent pacemaker implantation seen at autopsy in a three-year period is reported. Implantation was done for complete heart block in four patients and sick sinus syndrome in one. The periods of implantation ranged from seven days to four years. The common findings were right sided valvar and mural bland thrombus formation. Additional findings included superior vena caval thrombosis, endocardial 'tunnel' formation for the pacing wires, perforation of the tricuspid leaflet and a gross morphology similar to that seen in right sided endomyocardial fibrosis.

Autopsy↗

Feline cardiomyopathy.

Cardiology was diagnosed by means of clinical, radiographic, electrocardiographic phonocardiographic, angiocardiographic, and pathological findings in 271 or 3,745 cats necropsied from January 1962 to April 1974. The affected cats can be divided into three groups on the basis of the gross and microscopic pathological lesions: 1)endocarditis and myocarditis in 20 young cats; 2)endomyocardial fibrosis and left ventricular hypertrophy in 182 cats; and 3)myocardial degeneration and biventricular dilatation in 69 cats. Of 271 affected cats, thromboembolus was observed in the aorta, and in the carotid, femoral, iliac, renal, pulmonary, and hepatic arteries in 104 instances. The important aspects of cardiomyopathy in cats appears to be the reduced diastolic compliance of the thick left ventricle, resulting in poor fillin. Resistance to ventricular inflow raises the diastolic pressure and causes compensatory left atrial enlargement. A pathogenesis for the onset of clinical signs at any stages as the cause of the heart disease is postulated on the basis of stress causing tachycardia and poor left ventricular filling. Acute left-sided failure with pulmonary edema may be precipitated. Approximately one-fourth of the cats have enlargement of all cardiac chambers, typical of congestive cardiomyopathy. On the basis of the close similarily to cardiomyopathy in man, the cat could serve as a suitable animal model for a conservation of time and effort in the attack against this disorder. There is a need for coordinated research programs for utilizing the multiple avenues of approach such as: epidemiological, clinical, biochemical, pathological, ultrastructural, virological, and immunological.

Angiocardiography↗

Tricuspid valve replacement: clinical long-term results for acquired isolated tricuspid valve regurgitation.

BACKGROUND AND AIM OF THE STUDY: Acquired isolated tricuspid valve insufficiency (TVI) is a rare condition, and tricuspid valve replacement (TVR) is reserved for those patients in whom operative repair is not possible. The long-term follow up of patients who underwent isolated TVR at the authors' institution was analyzed. METHODS: All patients (n = 107) who underwent TVR between January 1985 and December 2002 were identified from a clinical database. Among 87 multiple valve replacements, 20 consecutive patients (12 females, eight males; mean age 52.4 years) were encountered who underwent TVR for acquired isolated TVI. Preoperative and perioperative data were recorded retrospectively. The patients and/or their physicians were contacted directly to determine long-term outcome; the follow up was 100% complete. RESULTS: The mean follow up period was 40.8 months (range: 1-211 months). The cause of TVI was endocarditis (n = 6) or trauma (n = 1); two patients suffered from hypertrophic non-obstructive cardiomyopathy, and one patient had endomyocardial fibrosis. TVI was related to constrictive pericarditis (n = 1) and to prior heart transplantation (n = 3). Seven patients were in NYHA functional classes I/II, and 13 were in classes III/IV. TVR was performed with either a mechanical valve (n = 15) or a bioprosthesis (n = 5). One patient (5%) died perioperatively, and five (25%) died during the follow up period. Two patients underwent a redo-operation during follow up, one due to prosthetic endocarditis, and one after thrombosis of a mechanical prosthesis. There was no structural deterioration of biological prostheses, and no bleeding due to anticoagulation with mechanical prostheses. Among the surviving patients, 13 were in NYHA class I, and one was in class II/III at the time of follow up. CONCLUSION: Isolated TVR for acquired TVI is rarely performed. Among the present patients, perioperative mortality and morbidity were lower than previously reported in the literature. The long-term outcome appeared to be largely dependent on the etiology of the regurgitation.

Adult↗