The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature.

The major cause of morbidity and mortality in patients with the hypereosinophilic syndrome is cardiac dysfunction. A review of 65 cases from the literature (historic series) revealed the following cardiovascular manifestations to be most common: dyspnea (60 per cent), signs of congestive heart failure (75 per cent), murmur of mitral regurgitation (49 per cent), cardiomegaly (37 per cent), T wave inversions on electrocardiogram (37 per cent) and pathologic findings of endocardial fibrosis, myocardial inflammation and mural thrombus formation (57 per cent). We have prospectively followed 26 patients with the hypereosinophilic syndrome for up to nine years (average follow-up prospectively was 3.3 years, retrospectively 5.7 years). Common cardiac findings in our 26 patients were dyspnea (42 per cent), chest pain (27 per cent), signs of congestive heart failure (38 per cent), murmur of mitral regurgitation (42 per cent), cardiomegaly (35 per cent) and T wave inversions (35 per cent). Thus, these patients demonstrated cardiovascular manifestations similar to those in the historic series, although the literature review showed a higher incidence of overt congestive heart failure.Of 22 patients having echocardiograms, 55 per cent demonstrated some clinical, roentgenographic or electrocardiographic evidence of cardiac involvement, but 82 per cent had echocardiographic abnormalities. This suggests that the echocardiogram is a sensitive and perhaps early indicator of cardiac involvement in this disease. Common echocardiographic findings included increased left ventricular wall thickness (68 per cent), left ventricular mass (73 per cent) and left atrial size (37 per cent). Prospective echocardiographic follow-up of 18 patients (for up to four and a half years) revealed that seven of eight untreated or inadequately treated patients had increases in left ventricular wall thickness, whereas all 10 adequately treated patients had decreases (eight of 10) or no change (two of 10) in left ventricular wall thickness. This suggests that adequate antihypereosinophilic therapy (with prednisone and/or hydroxyurea) may stabilize and, in some cases, reverse the cardiac manifestations of the hypereosinophilic syndrome.In previous studies, congestive heart failure due to eosinophilic cardiomyopathy has been reported to be very resistant to therapy. In our patients with congestive heart failure, treatment has been almost invariably effective when digitalis and diuretics were combined with adequate antihypereosinophilic therapy.     

Echocardiographic identification of cardiac abnormality in scleroderma and related disorders.

Although pathologic examination may readily disclose cardiac abnormality in patients with scleroderma, clinical identification of primary heart involvement can be difficult. In order to assess left ventricular systolic function, chamber size and wall thickness, and to determine whether pericardial effusion is present, echocardiograms were obtained in 11 patients with progressive systemic sclerosis (PSS) and in 13 patients with forms of scleroderma in which visceral involvement has been considered rare or absent: three with CREST syndrome, three with morphea, three with diffuse fasciitis with eosinophilia, and four with mixed connective tissue disease. Increased left ventricular wall thickness was noted in 13 of 23 (57 per cent) patients who could be evaluated, including six (46 per cent) from subgroups other than PSS. Left atrial dimension was increased in 12 patients (52 per cent) whereas the left ventricular end-diastolic dimension was increased in only three (13 per cent). Mitral valve closure velocity, an index of left ventricular compliance, was diminished in 10 (42 per cent) patients. However, left ventricular systolic contractile performance was normal in all. Pericardial effusion was detected in five patients (21 per cent), including one patient each with morphea, mixed connective tissue disease and diffuse fasciitis. Cardiac abnormalities were evident even in patients with PSS and no renal or severe pulmonary involvement. Thus, primary cardiac involvement, characterized by left ventricular wall thickening, decreased left ventricular compliance, left atrial enlargement and pericardial effusion, may be common in patients with scleroderma. These abnormalities occur in patients with PSS as well as in those with “nonsystemic” forms of scleroderma and are readily detected by echocardiography.     

Correlation of heart size with clinical and hemodynamic findings in patients with coronary artery disease.

The clinical, hemodynamic, and angiographic findings were correlated with the heart size in 207 patients with proved coronary artery disease. Cardiomegaly was noted in 34 patients and normal heart size in 173. In these two groups, the patients' age range, duration of disease, and history of myocardial infarction were similar. There was no statistical difference in incidence of shortness of breath, hypertension, left ventricular hypertrophy, or abnormal glucose tolerance. Patients with cardiomegaly had a significantly higher incidence of congestive heart failure (26 per cent) as compared to patients with normal heart size (2.9 per cent) (P less than 0.001). Patients with enlarged heart presented a high incidence of anterior wall or multiple myocardial infarction (73 per cent) (P less than 0.001). The cardiomegaly group had a high incidence of elevated end-diastolic volumes, elevated end-diastolic pressures, and diminished ejection fractions when compared to patients with normal heart size (P less than 0.01). Double and triple coronary artery disease was more frequent in patients with cardiomegaly and total coronary score was also higher in this group (P less than 0.005). Asynergy was present in 55 per cent of patients with normal heart size but in 82 per cent of those with enlarged hearts (P less than 0.01). The group of patients with cardiomegaly and documented congestive heart failure had ejection fractions less than 0.30. Cardiac catheterization is probably not advisable in these patients in the absence of associated significant mitral regurgitation, ventricular septal defect, or ventricular aneurysm.     

Pericardial tamponade in systemic sclerosis (scleroderma).

The frequency of pericardial disease in scleroderma found at necropsy in high. The clinical recognition of pericarditis with or without effusion is rare and tamponade with haemodynamic impairment is exceptional. Three patients with scleroderma presented with an acute syndrome of dyspnoea, chest pain, and cardiomegaly requiring pericardiocentesis for relief of pericardial tamponade. One patient died. The mechanism of the pericardial effusion remains unknown. The haemodynamic data recorded from one patient suggested that pericardial fibrosis in scleroderma may predispose to pericardial tamponade.     

Factors predicting development of renal involvement in progressive systemic sclerosis

Renal involvement or "scleroderma renal crisis" developed in 60 patients with progressive systemic sclerosis evaluated at the University of Pittsburgh during the period from 1972 to 1982. Forty-seven of these patients had progressive systemic sclerosis with diffuse scleroderma, representing 18 percent of persons with progressive systemic sclerosis and diffuse scleroderma evaluated during this time period. Ten additional patients did not have truncal scleroderma but were suspected of having incompletely developed diffuse scleroderma. Only three patients were classified as having progressive systemic sclerosis with the CREST syndrome. Renal crisis was observed early in the course of the illness, a mean of 3.2 years after onset. During May and June, this complication developed in fewer patients than expected. Thirty-six patients who had diffuse scleroderma and renal involvement after their initial Pittsburgh evaluation were compared with 212 who had diffuse scleroderma without renal involvement during follow-up. The patients with renal involvement had a shorter mean disease duration at the time of their first evaluation (2.4 versus 4.2 years, p less than 0.05) and less frequently had digital pitting scars (29 versus 54 percent), but no other significant clinical, laboratory, or serologic differences were noted. Data available for 31 patients with renal involvement during the six months preceding the onset of renal disease were analyzed. Blood pressure, serum creatinine, urine protein and red blood cells, and plasma renin levels were similar in these patients and the 212 patients without renal involvement. More patients with renal involvement had anemia or clinical evidence of cardiac involvement during this period compared with the patients without renal involvement. During the 12-month period prior to renal involvement, seven of 16 (44 percent) patients with such involvement had an impressive increase in skin thickening on physical examination compared with only 23 of 180 (14 percent) patients without renal involvement at any time during their course. Thus, the subset of patients with diffuse scleroderma who show rapid progression of their skin thickening early in the illness with development of anemia, pericardial effusion, or congestive heart failure have a high risk of "scleroderma renal crisis."     

Pericardial windows or pericardiocentesis for pericardial effusions

The hospital records of 20 patients admitted to Parkland Memorial Hospital in Dallas with pericardial effusion during the four-year period of 1966 to 1969, and who underwent pericardiocentesis and percutaneous open pericardial windows, were reviewed. The etiologies of the effusions were as follows: purulent pericarditis (5), hypertensive and ischemic heart disease with congestive heart failure (4), and chronic idiopathic effusion (4). Specific etiologic diagnoses were made from the pericardial biopsy in only two cases (10 per cent), while 13 (65 per cent) had at least one serious complication in the postoperative period with eight (40 per cent) developing secondary infection. Twenty-one patients underwent pericardiocenteses without complications and four etiologic diagnoses (20 per cent) were made. Suggestions for indications for these procedures are presented.     

Pericardial and pleural effusions in decompensated chronic heart failure

BACKGROUND: In decompensated chronic heart failure, there is controversy regarding the incidence and amount of pericardial fluid. Moreover, the relation of pericardial effusion to pleural effusion has not yet been clarified. The current study examined the incidence and amount of pericardial effusion in patients with decompensated chronic heart failure as a function of the volume of pleural effusion. METHODS AND RESULTS: The study subjects were 60 consecutive patients with chronic heart failure requiring diuresis to improve the symptoms and signs of congestion. Pericardial effusion was semiquantified on the basis of M-mode echocardiographic findings and the volume of thoracic effusion drawn from computed tomographic images of the chest with Simpson's method. Causes of decompensated chronic heart failure in the 60 patients included cardiac valve disease (n = 26), arterial hypertension (n = 12), chronic ischemic heart disease (n = 9), and others (n = 13). As many as 52 (87%) of the 60 patients had pleural effusion; of these, 45 had bilateral effusion, 5 had right-sided effusion only, and 2 had left-sided effusion only. In contrast, only 12 (20%) patients had small (n = 9) or moderate (n = 3) pericardial effusion. There was no significant association between the amount of pleural effusion and the semiquantified pericardial effusion (chi-square 3.27, P =.775). CONCLUSIONS: In this series of patients with congestive heart failure, small pericardial effusion was sometimes observed, but moderate to large effusion was uncommon, and there was no significant association between a given amount of pleural effusion and the volume of pericardial effusion.     

Two cases of primary cardiac lymphoma presenting with pericardial effusion and cardiac tamponade

The heart as the primary site of lesion in malignant lymphoma is extremely rare. We experienced 2 cases of malignant lymphoma whose initial presentation was massive pericardial effusion with cardiac tamponade. The first case was a 75-year-old man who had shortness of breath for 1 week. Chest X-ray showed cardiomegaly (CTR 65%), and his condition was diagnosed as congestive heart failure at first and thereafter echocardiogram revealed pericardial effusion. The second case was a 76-year-old man who complained of exertional dyspnea which worsened over 2 weeks. His condition was diagnosed as congestive heart failure at first and echocardiogram revealed pericardial effusion. Pericardial drainage tapped bloody fluid and cytological examination revealed malignant lymphoma. After treatment, the first case lived for eleven months with no recurrence of pericardial effusion. The second case has lived for 4 years. We present these 2 cases can be defined as primary cardiac lymphoma according to McALLISTER and FENOGLIO, i.e., a lymphoma that involves only the heart and pericardium. More cases of primary cardiac lymphoma will be found in the future because of the ease with which the echocardiogram can detect pericardial effusion.     

Contribution of M-mode echocardiography to cardiac diagnosis: An assessment in 1,000 successive patients

The contribution of M-mode echocardiography to cardiac diagnosis was evaluated in a series of 1,000 successive patients. Among subjects in whom a presumptive clinical diagnosis had been made, echocardiography demonstrated totally unexpected findings in 10 per cent, supported the clinical diagnosis in 50 per cent and was entirely within normal limits in 19 per cent. Among patients with evidence of heart disease but no firm clinical diagnosis, echocardiography established the diagnosis in 23 per cent, including 20 per cent of all patients referred for evaluation of chest pain or arrhythmia of unclear etiology. “Missed” clinical diagnosis frequently involved patients with mitral valve prolapse, congestive cardiomyopathy, pericardial disease or asymmetrical septal hypertrophy of the heart.This study quantifies the amount of independent information contributed by echocardiography to cardiac diagnosis and demonstrates that this technic provides data of important clinical relevance in a surprisingly large number of cardiac patients.     

Primary effusion lymphoma complicating cardiac tamponade: a case report

A 76-year-old woman was admitted to our hospital because of exertional dyspnea and leg edema during the previous month. Her systolic blood pressure on admission was 80 mmHg with 12 mmHg of pulsus paradoxous, and her pulse rate was 110 beats/min. Chest radiography revealed marked cardiomegaly and echocardiography showed massive pericardial effusion mainly behind the left ventricle and collapse of the right ventricle. The initial diagnosis was pericardial tamponade. Pericardiocentesis and pericardial drainage revealed bloody pericardial effusion. After drainage, her vital signs improved and her symptoms immediately disappeared. The cytological analysis of the pericardial effusion revealed numerous lymphoma cells. Computed tomography of the neck, chest and abdomen showed no evidence of tumor masses, lymph node enlargement, or hepatosplenomegaly. Infectious disease, collagen disease and aortic dissection were excluded. The final diagnosis was primary effusion lymphoma. The prognosis of primary effusion lymphoma is generally unfavorable because it is frequently accompanied by immunodeficiency disease. However, there was no human immunodeficiency virus infection in this patient. Fortunately, the effect of chemotherapy was excellent and the patient is doing well 1 year after the diagnosis.     

Pericardial involvement in end-stage renal disease

Pericardial involvement in end-stage renal disease (ESRD) is manifested most commonly as acute uremic or dialysis pericarditis and infrequently as chronic constrictive pericarditis. The causes of uremic and dialysis pericarditis remain uncertain. The clinical and laboratory manifestations of acute pericarditis, pericardial effusion, cardiac tamponade, and constrictive pericarditis in patients with chronic renal failure are similar to those observed in nonuremic patients with similar pericardial involvement, except that chest pain occurs less frequently in those with ESRD. Therapeutic interventions for acute uremic or dialysis pericarditis with or without pericardial effusion include intensive hemodialysis, pericardiocentesis (infrequently used), pericardiostomy with or without instillation of intrapericardial glucocorticoids, pericardial window, and pericardiectomy. Chronic constrictive pericarditis is treated with pericardiectomy.     

Echocardiographic features of progressive systemic sclerosis (PSS). Correlation with hemodynamic and postmortem studies.

Echocardiographic abnormalities were noted in 37 (69 per cent) of 54 patients with progressive systemic sclerosis (PSS). Thirty-one of the 54 patients also underwent right heart catheterization. Eight of the 11 patients who died underwent postmortem examination. Pericardial effusion was noted in 22 subjects (41 per cent), although it was suspected on clinical grounds in only seven. Echocardiography appeared to be an excellent tool for evaluating and quantitating pericardial effusions. The presence of a small pericardial effusion (<50 ml) did not affect prognosis, but the presence of a large effusion (>200 ml) was associated with a poor prognosis. The finding on echocardiogram of right ventricular dilatation, left atrial dilatation, asymmetrical septal hypertrophy, paradoxic motion of the interventricular septum or signs of pulmonary hypertension were seen predominantly or exclusively in patients who had pulmonary artery hypertension at cardiac catheterization. Pericardial effusions and abnormal mitral valve diastolic slope on echocardiogram did not correlate with pulmonary hypertension.The present study confirms clinically the frequent involvement of myocardium and pericardium that has been reported in autopsy series. At the same time, however, it suggests that the usefulness of the echocardiogram in clinical practice may really be limited to evaluating pericardial effusion and cardiomegaly, and to substantiating pulmonary artery hypertension in a safe, noninvasive manner.     

Scleroderma pericardial disease presented with a large pericardial effusion--a case report

Scleroderma pericardial disease is usually silent and benign. The incidence of pericardial involvement in scleroderma is about 50% according to autopsy results, but symptomatic pericarditis manifests in about 16% of patients with diffuse scleroderma and in about 30% of patients with limited scleroderma. The clinically evident pericardial effusion is rare in scleroderma, although it can be detected in about 41% of patients with echocardiography. In majority of the patients, the pericardial effusion is small and not associated with symptoms. The pericardial effusion manifests usually after the manifestation of the other clinical and serologic features of scleroderma. A case of scleroderma is reported that presented with a large pericardial effusion, which antedated the other clinical and serologic features of scleroderma. The pericardial involvement in scleroderma is reviewed.     

Correct diagnosis and successful treatment for pericardial effusion due to toothpick injury： A case report and literature review

We reported a 55-year-old man who suffered from chest pain and dyspnea on exertion for two weeks associated with night sweating, general malaise, poor appetite, and body weight loss. Physical examination revealed friction rub with distant heart sound, bilateral clear breathing sound, no abdomen tenderness, and normal bowel sound. Subsequent chest X-ray revealed cardiomegaly and cardiac echo showed massive pericardial and pleural effusion with normal left ventricular function. Constrictive pericarditis was diagnosed based on clinical information. Tuberculosis (TB), malignancy, autoimmune disease, infection, hypothyroidism, and idiopathic could be the causes but excluded by further study. High-resolution lung CT scan after reconstruction revealed a moderate amount pericardial effusion with possible superimposed infection. Thickness of pericardium and left lobe liver abscess were found. A straight tubular structure about 6 cm in length transverses the lateral segment of liver to pericardial space and unknown foreign body was suspected. Laparotomy was performed, 6.5 cm toothpick was found through the liver into pericardium. Post-operative course was uneventful and he discharged one week later. The patient could not remember swallowing the toothpick before. He had no chest pain and dyspnea on exertion during a 6-mo follow-up period.     

Pericardial effusions in patients with end-stage renal disease.

Echocardiography has greatly increased the accurate recognition of pericardial effusion. Echocardiograms were performed prospectively on the total group of 35 stable asymptomatic patients on chronic haemodialysis to determine the incidence of pericardial effusion. Effusions were shown in 11 per cent (4/35); only 6 per cent (2/35) were estimated as greater than 100 ml. For comparison, records were reviewed retrospectively from 41 haemodialysis patients referred during a 27-month period for echocardiographic assessment of suspected pericardial effusion. These 41 patients came from a total group of 108 patients treated with chronic dialysis over this interval. Of 41 examined, 21 (51%) or 21 of 108 (19%) of the population at risk had an effusion. Of 21 with echocardiographic effusions, 15 (71%), or 15 of 41 (37%) of those with clinically suspected effusion, had more than 100 ml fluid. Gross (greater than 100 ml) pericardial effusions are infrequent in stable, asymptomatic patients with end-stage renal disease. When clinical findings suggest pericardial disease, the echocardiographic demonstration of over 100 ml pericardial fluid is indicative of new effusion, rather than coincidental pre-existing effusion.     

Pericardial effusions in patients with end-stage renal disease

Echocardiography has greatly increased the accurate recognition of pericardial effusion. Echocardiograms were performed prospectively on the total group of 35 stable asymptomatic patients on chronic haemodialysis to determine the incidence of pericardial effusion. Effusions were shown in 11 per cent (4/35); only 6 per cent (2/35) were estimated as greater than 100 ml. For comparison, records were reviewed retrospectively from 41 haemodialysis patients referred during a 27-month period for echocardiographic assessment of suspected pericardial effusion. These 41 patients came from a total group of 108 patients treated with chronic dialysis over this interval. Of 41 examined, 21 (51%) or 21 of 108 (19%) of the population at risk had an effusion. Of 21 with echocardiographic effusions, 15 (71%), or 15 of 41 (37%) of those with clinically suspected effusion, had more than 100 ml fluid. Gross (greater than 100 ml) pericardial effusions are infrequent in stable, asymptomatic patients with end-stage renal disease. When clinical findings suggest pericardial disease, the echocardiographic demonstration of over 100 ml pericardial fluid is indicative of new effusion, rather than coincidental pre-existing effusion.     

Coronary artery disease in patients dying from cardiogenic shock or congestive heart failure in the setting of acute myocardial infarction.

Pathological findings in the heart and particularly in the coronary arteries are reported from 70 patients dying from pump failure after acute myocardial infarction. Fifty of the patients had died in cardiogenic shock, the remainder from refractory congestive heart failure. Three-vessel disease (greater than or equal to 75% occlusion) was present in 68 per cent of the group with cardiogenic shock but in only 35 per cent of those with fatal congestive heart failure (P less than 0-02). In both groups there was an almost equal incidence (84% for cardiogenic shock and 80% for congestive heart failure) of severe disease (greater than or equal to 75% occlusion) over a long segment of the left anterior descending artery. However, there were differences between the two groups regarding the involvement of the other coronary arteries. Whereas patients with cardiogenic shock generally showed severe disease over a long segment in all coronary arteries, in 60 per cent of those with congestive heart failure there was only local severe narrowing of the right coronary artery with little or no narrowing of the peripheral part. Similarly, 60 per cent of those with congestive heart failure had less than 75 per cent narrowing in the left circumflex artery. These anatomical findings may be of relevance with regard to desirability of acute coronary bypass surgery in patients with pump failure after acute myocardial infarction.     

The demonstration of vegetations by echocardiography in bacterial endocarditis: An indication for early surgical intervention

The visualization of vegetations by M-mode echocardiography in patients with infective endocarditis has been suggested to imply a poor prognosis regarding the development of major systemic emboli, congestive heart failure and the need for early surgical intervention. The question of using the finding of vegetations by echocardiography as an indication for surgery is controversial. To answer this question, 30 patients with the clinical diagnosis of endocarditis were studied by echocardiography. In 17 of the 30 (57 per cent) vegetations were present (aortic eight, mitral four, both mitral and aortic five), whereas in 13 (43 per cent) no vegetations were visualized. Infecting organisms were similar in each group; Streptococcus viridans being the most common. The patients with echocardiographically demonstrable vegetations had a higher incidence of congestive heart failure compared to the patients without (14 of 17 versus six of 13, p < 0.05), major emboli (eight of 17 versus two of 13, p = NS) and need for valve surgery (17 of 17 versus two of 13, p < 0.001). Mortality was not significantly different in the two groups (six of 17 versus three of 13, p = NS). Urgent or emergency surgery was required in 16 of 17 patients with vegetations. Thus, the demonstration of vegetations by echocardiography i dentified a subset of patients with more severe disease in whom early operative intervention was required.     

Heart disease caused by Coxsackie virus B infection.

A study of 55 patients with heart disease suspected of being viral in origin was carried out a Medical College Hospital, Nagpur, over a period of 2 years. Virus studies as well as other routine tests were carried out on all patients. In 19 patients a virus aetiology of the heart disease was proved by isolation of one of the subtypes of Coxsackie B virus and/or on the basis of fourfold rise in neutralizing antibody titre in paired sera. Of these patients, 5 had acute myocarditis and 5 had acute myopericarditis; 3 had acute pericarditis; 3 had congestive cardiac failure of obscure aetiology; 2 had pleuropericarditis, and the remaining 1 developed post-partum heart failure with cardiogenic shock. All had electrocardiographic abnormalities. Thirteen had cardiomegaly; 1 had a right-sided pleural effusion and 2 had pericardial effusion. Virus could not be isolated from pericardial fluid or pleural fluid in these 3 patients. Follow-up studies up to 10 weeks from discharge revealed that 8 patients were clinically normal but 4 of these 8 had persisting ST-T wave changes, and in 4 the electrocardiogram had returned to normal. Of the remaining 11 patients, 3 had persistent chronic heart failure, 3 had vague symptoms of praecordial pain but no abnormal signs, and 5 patients were lost to follow-up.     

Heart disease caused by Coxsackie virus B infection.

A study of 55 patients with heart disease suspected of being viral in origin was carried out a Medical College Hospital, Nagpur, over a period of 2 years. Virus studies as well as other routine tests were carried out on all patients. In 19 patients a virus aetiology of the heart disease was proved by isolation of one of the subtypes of Coxsackie B virus and/or on the basis of fourfold rise in neutralizing antibody titre in paired sera. Of these patients, 5 had acute myocarditis and 5 had acute myopericarditis; 3 had acute pericarditis; 3 had congestive cardiac failure of obscure aetiology; 2 had pleuropericarditis, and the remaining 1 developed post-partum heart failure with cardiogenic shock. All had electrocardiographic abnormalities. Thirteen had cardiomegaly; 1 had a right-sided pleural effusion and 2 had pericardial effusion. Virus could not be isolated from pericardial fluid or pleural fluid in these 3 patients. Follow-up studies up to 10 weeks from discharge revealed that 8 patients were clinically normal but 4 of these 8 had persisting ST-T wave changes, and in 4 the electrocardiogram had returned to normal. Of the remaining 11 patients, 3 had persistent chronic heart failure, 3 had vague symptoms of praecordial pain but no abnormal signs, and 5 patients were lost to follow-up.