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[Extracardial manifestations in constrictive pericarditis].

Constrictive pericarditis is a slowly progressive disabling disease. The diagnosis is easily overlooked because of the striking extracardial signs and symptoms such as abdominal discomfort, general fatigue, cachexia, ascites and oedema. We describe 7 patients with these symptoms in whom the diagnosis was missed during 0.5-17 years. The decisive clue for correct diagnosis appeared to be the raised central venous pressure. This proves the importance of an accurate physical examination. Other findings were: ascites (7/7), hepatomegaly (7/7), oedema (6/7), narrow pulse pressure (less than or equal to 35 mmHg) (5/7), ECG abnormalities (7/7) and pericardial calcifications on the chest X-ray (5/7). In addition we found slightly raised liver enzymes and a protein-losing enteropathy leading to low serum protein levels. These abnormalities are all explained by the alterations in haemodynamics and lymph flow. The only curative therapy is surgical decortication of the heart.

Adult

Constrictive pericarditis.

Constrictive pericarditis is a complex disorder characterized by abnormal thickening of the pericardium that leads to pathologic changes in cardiac hemodynamic data. The disorder can be suspected by history and physical findings. Data from echocardiography, CT, and MRI offer diagnostic information. The diagnosis cannot generally be established with certainty by noninvasive methods. Additional information from cardiac catheterization may help confirm the diagnosis. Together, these diagnostic modalities aid in the assessment of disease and help to differentiate it from related conditions such as restriction, cardiac tamponade, and right ventricular infarction. Treatment is largely surgical, and new techniques and approaches have made it relatively safe. Early diagnosis and pericardiectomy may lead to cure in most patients.

Cardiac Catheterization

CT in tuberculous constrictive pericarditis.

Constrictive pericarditis (CP) and restrictive cardiomyopathy have the same pathophysiological disorder (impaired diastolic filling) and a similar presentation. To determine the value of a noninvasive technique, CT, in differentiating between the two, we reviewed the CT scans of 212 patients with clinical signs and symptoms of CP and/or restrictive cardiomyopathy, after 26 cases that did not proceed to surgery were excluded. Computed tomography showed pericardial thickening (greater than 3 mm) in 157 patients with proven tuberculous CP. In two children with proven constriction, the pericardium measured between 2 and 3 mm. We determined that pericardial thickening, in the correct clinical setting, is the only CT sign necessary to diagnose CP. Other CT signs, inferior vena cava dilatation (97%), and deviation of the interventricular septum (15%) were confirmatory. Computed tomography accurately differentiates between CP and restrictive myocardial disease, making more invasive modalities such as cardiac catheterization and endomyocardial biopsy, with few exceptions, unnecessary.

Adolescent

Constrictive pericarditis.

Constrictive pericarditis, although still a relatively rare disease, continues to be a clinical problem that most practicing cardiologists may encounter. A major clinical clue to diagnosis is the continued elevation of the central venous pressure after adequate diuresis. The diagnosis is further supported by (1) prominent X and Y descents in the jugular venous pulse, (2) a relatively normal or only slightly enlarged cardiac silhouette in a patient with congestive heart failure, (3) pericardial calcification or significant congestive failure especially when the right sided signs predominate without obvious cause. When the disease is suspected, appropriate investigation should be undertaken using both the noninvasive and the catheterization studies. If the diagnosis is supported, then the choice of therapy at present is based primarily on severity of symptoms with surgical removal of the constricting pericardium being the therapy of choice in patients unable to be managed medically.

Adolescent

Traumatic hemopericardium and chronic constrictive pericarditis.

Chronic constrictive pericarditis following traumatic hemopericardium has been reported in recent years, but it has not been reproduced experimentally in dogs. The present study attempted to produce posttraumatic constrictive pericarditis in 34 experimental animals. Hemopericardium by means of trauma to the epicardium or pericardium was produced by a sharp instrument or by the injection of autologous blood inside the pericardial sac. All animals were killed at intervals between 3 and 31 months. The animals in which hemopericardium was induced by injecting blood into the pericardial cavity showed no changes. The hemopericardium was completely resolved without noticeable residual trace. Animals having hemopericardium as a result of trauma evidenced a well-developed constrictive pericarditis that was documented clinically, hemodynamically, and histologically. These experimental findings indicate that chronic constrictive pericarditis may well be due to traumatic hemopericardium rather than to specific infection.

Animals

[Echocardiography in a case of acute tuberculous pericarditis which progressed to constrictive pericarditis].

A case of acute tuberculous pericarditis with massive pericardial effusion progressed to constrictive pericarditis under echocardiographic observation during one year. This 59-year-old man was hospitalized because of dyspnea. On admission, his physical examination revealed a paradoxical pulse, engorged jugular veins, hepatomegaly, and pitting edema in the pretibial regions. Chest radiography revealed an enlarged cardiac silhouette and a marked left pleural effusion. His echocardiogram showed a massive pericardial effusion. A biatrial echogram recorded by esophageal echocardiography showed a massive pericardial effusion anterior to the right atrial free wall. Echocardiography performed four months after commencing therapy revealed a reduction in the pericardial effusion, and normal motion of the interventricular septum and posterior wall. However, the motion of the interatrial septum was already abnormal and the excursion of the right atrial free wall was markedly reduced. These finding were similar to those in constrictive pericarditis, as previously reported. Eleven months after admission, both conventional and esophageal echograms showed findings typical of constrictive pericarditis. Thus, in this case, the abnormal biatrial dynamics were recognized earlier than the abnormal left ventricular wall motion.

Echocardiography

[Lassa fever associated with effusive constrictive pericarditis and bilateral atrioventricular annular constriction: a case report].

A case of Lassa fever associated with effusive constrictive pericarditis and bilateral atrioventricular annular constriction was reported. A 49-year-old man, who had been diagnosed by indirect fluorescent antibody test as the first case of Lassa fever in Japan, was referred to the Hiroo Hospital because of syncope, progressive hepatomegaly, ascites and pericardial effusion in spite of pericardiocentesis and corticosteroid therapy. On admission, his blood pressure was 92/60 mmHg and he had a paradoxical pulse. Two-dimensional echocardiography revealed a localized pericardial effusion adjacent to the right ventricular wall and behind the left ventricular posterior wall. Bilateral atrioventricular annular constriction was also present. On pulsed Doppler echocardiography, the peak inflow velocities of the right and left ventricles increased during atrial systole. Right heart catheterization revealed a mean diastolic pressure gradient of 8 mmHg across the tricuspid valve. After pericardiectomy, a diastolic dip and plateau pattern became evident in the right ventricular pressure tracing, suggesting the presence of residual constriction. However, the atrioventricular annular constriction was no longer evident on two-dimensional echocardiography. This is considered the first reported case of subacute effusive constrictive pericarditis caused by Lassa fever.

Cardiac Catheterization

[Complications of rheumatoid pericarditis: constriction and tamponade].

Pericarditis is frequent in patients with rheumatoid arthritis. It is usually asymptomatic, but cases of constrictive pericarditis or, more rarely, tamponade have been reported. In such cases, the study of pericardial fluid is of special interest for the aetiological diagnosis. Early medical treatment with or without pericardiocentesis is effective in most cases of tamponade, but constrictive pericarditis demands pericardiectomy. We report here three cases of pericarditis responsible for cardiac compression: two cases of tamponade and one case of constrictive pericarditis.

Adult

[Constrictive pericarditis].

Etiology of constrictive pericarditis is diversified. In recent years the role of viral and iatrogenic factors (X-ray therapy, cardiac surgery) has increased. Clinical manifestations, including subjective complaints, resemble those in congestive heart failure although the mechanism of hemodynamic disorders is different--namely impairment of ventricular filling and diastolic performance. One encounters major difficulties while differentiating constrictive pericarditis from restrictive cardiomyopathy. In some patients conservative treatment is effective, in a majority of them surgical operation, pericardiectomy is the treatment of choice.

Adolescent

Constrictive pericarditis complicating dermatomyositis.

Chronic constrictive pericarditis may complicate both rheumatoid arthritis and systemic lupus erythematosus. To our knowledge, however, this is the first time it has been described in a patient with dermatomyositis. This association should be kept in mind as constrictive pericarditis should be considered in the differential diagnosis of liver cirrhosis and Budd-Chiari syndrome in rheumatic diseases.

Dermatomyositis

Collagen diseases as a cause of constrictive pericarditis.

Seven patients with constrictive pericarditis associated with collagen disease underwent pericardiectomy with good results in all but one. The collagen disease was confirmed as rheumatoid arthritis in five patients, but in two its nature remained obscure. In one case the illness was marked by a persistent eosinophilia and eosinophilic infiltration of the pericardium. The association of constrictive pericarditis with rheumatoid arthritis and other collagenoses is briefly discussed.

Adolescent

[A case of rapidly developed constrictive pericarditis following acute non-specific pericarditis].

A case of constrictive pericarditis which developed within one month after the onset of acute pericarditis was presented. A 3-year-old boy was admitted to our hospital suffering from dyspnea. Pericardectomy was performed 3 weeks after the pericardial drainage. His echocardiograms revealed the progressive thickening of the pericardium, severely restricted ventricular motion in both systole and diastole and paradoxical motion of the interventricular septum. Echocardiography at close intervals is useful for making the diagnosis and decision of the surgical intervention of rapidly developed constrictive pericarditis.

Acute Disease

An echocardiographic study of the interventricular septum in constrictive pericarditis.

Ten patients with constrictive pericarditis were studied echocardiographically with specific reference to inter-ventricular septal dynamics. Abnormal movement of the interventricular septum was present in 8 patients and consisted of flattening in systole and unusual posterior motion in diastole. The aetiology of this type of movement is at present unknown but may be related to restriction of normal cardiac rotational dynamics. The interventricular septum also showed diminished degree of thickening (mean 21-2%). The amplitude of excursion was generally at the upper limit of or greater than normal. Left ventricular posterior wall amplitude of excursion was normal. Flattening of left ventricular posterior wall diastolic movement was seen in 4 patients. Right ventricular end-diastolic dimension was slightly increased (1-2 to 1-7 cm/m2) in 5 of 8 patients with abnormal septal motion, but no haemodynamic evidence of diastolic volume overload was found. Posterior pericardial thickening was noted echocardiographically when posterior calcification was present. We conclude that the most common though non-specific feature of the echocardiogram in patients with constrictive pericarditis is abnormal septal motion. Flattening of left ventricular posterior wall diastolic movement, posterior pericardial thickening, and epicardial-pericardial separation may also occur.

Adult

The etiologic spectrum of constrictive pericarditis.

Ninety-five consecutive patients with constrictive pericarditis that was documented at the time of surgery during 1970 to 1985 were reviewed. The etiologies included idiopathic (42%), postradiotherapy (31%), post-cardiac surgery (11%), postinfective (6%), connective tissue disease-related (4%), neoplastic (3%) uremic (2%), and sarcoidosis (1%). Post-cardiac surgery etiology was seen only after 1980, but constituted 29% of cases during 1980-1985. Postradiotherapy etiology occurred with equal incidence in 1980-1985 and in 1970-1980, but the interval from radiotherapy to presentation with constrictive pericarditis was longer in the more recent period (11 vs 4.75 years). Effusive constrictive pericarditis occurred in 24% overall with similar prevalence in all of the etiologic groups except the postsurgical cases, which were caused by noneffusive fibrous constrictive pericarditis in all instances. Operative mortality was 12% overall: It was lower in the idiopathic group (8%) and higher in the postradiotherapy group (21%). Thus postradiotherapy constrictive pericarditis continues to occur despite technical changes aimed at reducing its likelihood, but recent cases have a longer latent period: and postsurgical constrictive pericarditis has emerged as an important etiology.

Adult

Constrictive pericarditis: assessment of current diagnostic procedures.

Constrictive pericarditis frequently poses a diagnostic challenge because of its varied manifestations. Accurate diagnosis is essential, however, because surgical decortication may yield excellent clinical results. Although new diagnostic procedures have helped the clinician to diagnose constrictive pericarditis, the initial clinical suspicion of this diagnosis must be high for appropriate interpretation of these tests. Echocardiography is useful, primarily for distinguishing various other cardiac abnormalities that may simulate constrictive pericarditis. Computed tomography is a valuable procedure for assessment of pericardial thickening. In addition, evaluation of early diastolic filling by computerized digitization in conjunction with echocardiography, angiography, and invasive hemodynamics shows promise as a diagnostic tool. Even with these new diagnostic aids, distinguishing constrictive pericarditis from restrictive cardiomyopathy may be difficult and, in some cases, may necessitate an exploratory operative procedure.

Aged