Noninvasive diagnosis of left coronary arteriovenous fistula communicating with the right ventricle

In a 20 year old man, the diagnosis of a left coronary arteriovenous fistula communicating with the right ventricle was made by noninvasive techniques using M mode, two dimensional and Doppler echocardiography. The diagnosis was confirmed by coronary angiography.     

Sulfonylureas and platelet function

Intercellular vascular smooth muscle calcium results in vasoconstriction and is therefore a potentially adverse mechanism of increased aflerload in chronic congestive heart failure. Therefore, an evaluation was made of supine and tilt hemodynamic data, sympathetic reflexes, and the hormonal response to calcium channel antagonism after administration of nifedlpine in nine patients with severe chronic congestive heart failure. After a 10 mg oral dose, the peak hemodynamic response occurred at 30 minutes and was characterized primarily by afterload reduction, improvement of systemic flow, and reduction of pulmonary hypertension. Despite reduction of supine blood pressure, there was no orthostatic hypotension during head-up tilt at the same time of peak response. Reflex responses to sympathetic stimulation (cold presser test) were improved but still attenuated when compared with normal responses. Plasma renin activity increased significantly, but a dissociation of the aldosterone response was observed. Plasma catecholamine levels were not significantly altered. In summary, calcium antagonism resulted in significant afterload reduction and hemodynamic improvement in chronic congestive heart failure. This was associated with improved reflex responsiveness and, potentially, altered other vasoconstrictor hormones independently of the hemodynamic response. Calcium antagonism may provide a means to further understand vasoconstrictor mechanisms in heart failure and enhance therapy in appropriate patient subsets.     

Outbreak of toxoplasmosis in a family and documentation of acquired retinochoroiditis

An outbreak of toxoplasmosis in one household is described. It demonstrates the potential for a common source infection with Toxoplasma gondii to cause multiple cases. Six of seven members of a household investigated for toxoplasmosis demonstrated high antibody titers consistent with recent infection; five of these members (83%) were symptomatic. The most common manifestations were fever and lymphadenopathy, which developed from seven to 18 days (mean 11 days) after a common source ingestion of infected meat. Since inadequately cooked lamb, pork and beef are probably the most common sources of infection in the United States, outbreaks of multiple cases may occur more frequently than is generally appreciated. As more outbreaks of febrile illnesses are examined, especially in families and closed communities, it is likely that more common source epidemics of toxoplasmosis will be recognized. Retinochoroiditis is an unusual manifestation of adult acquired toxoplasmosis. In the index case in this epidemic the patient manifested vision threatening retinochoroiditis 129 days after infection with toxoplasmosis. He represents the ninth well-documented case of toxoplasma retinochoroiditis associated with adult acquired disease.     

Role of circulatory congestion in the cardiorespiratory failure of obesity.

The role of circulatory congestion in the cardiorespiratory dysfunction of massive obesity was investigated in 18 patients. They were hypervolemic and had increased cardiac outputs proportionate to their weight. The average resting left ventricular filling pressure was within the upper limits of normal, but it increased to abnormally high levels with increased venous return of passive leg raising, and further during exercise. The elevations in pressure were associated with high resting central blood volumes which increased significantly with exertion. These findings are consistent with reduced distensibility of the central circulation in these congested patients. Weight reduction was accompanied by a decrease in central blood volumes and restoration of a normal left ventricular response in three of four patients and a return toward normal in one. The improvement in ventricular function with relief of edema and dyspnea. In 14 patients with normal or only minimal alveolar hypoventilation, there were no significant transpulmonary diastolic pressure gradients despite a marked increase in left ventricular end-diastolic pressures. One patient, after regaining weight, subsequently had an abnormal gas exchange and an increased pulmonary vascular resistance. He and two others with severe alveolar hypoventilation demonstrated cor pulmonale on a background of left ventricular dysfunction and congestion of the circulation. Two other patients, the least obese of the group, had hypoventilation and cor pulmonale with normal left ventricular pressures. Hypervolemia and a hyperdynamic state are common features of the obese patients. High cardiac output is maintained despite marked circulatory congestion which may result in generalized anasarca and increased ventricular filling pressures. This clinical syndrome may be present in obese patients without intrinsic heart disease and may be reversible with weight reduction. The central circulatory congestion may contribute to the development of the alveolar hypoventilation syndrome in certain obese patients.     

Fistule coronaro-camérale acquise compliquée d’un pseudo-anévrysme ventriculaire

Coronary-cameral fistulas are usually congenital, rarely acquired; the complication of this anomaly with ventricular pseudoaneurysm is exceptional. We report a new case of acquired coronary-cameral fistula, occurred in a patient who had received a bypass graft and who had suffered from angina 1 year after the surgery. On computed tomography coronary angiography, the fistula seems to communicate the first diagonal to a left ventricle pseudoaneurysm. Embolization of the fistula and filling of the pseudoaneurysm by neurocoil were successfully performed. The clinical and angiographic control after 3 months showed symptoms improvement and absence of recanalization of the fistula.     

Sympathetic responsiveness and plasma norepinephrine during therapy of chronic congestive heart failure with captopril

The interaction of cardiac function and sympathetic tone in severe chronic heart failure was evaluated in 24 patients by assessing the cardiac index/plasma norepinephrine relationship. Potential changes were assessed during first-dose and long-term captopril therapy including sympathetic responsiveness to the gravitational stress of head-up tilt. Baseline cardiac index and norepinephrine levels demonstrated a significant inverse correlation (r = ?0.640, p < 0.001). Norepinephrine decreased from 803 ± 116 to 635 ± 76 pg/ml following first-dose captopril therapy (p < 0.02), with overall hemodynamic improvement. However significant first-dose correlations were not observed. During long-term therapy, norepinephrine decreased from 694 ± 118 to 457 ± 106 pg/ml, associated with improvement of symptoms and exercise tolerance. The extent of cardiac index increase was matched by norepinephrine reduction, so that their correlation was maintained (r = ?0.540, p < 0.02). First-dose and long-term therapy were associated with improved responsiveness of sympathetic tone to the reduction of cardiac index induced by the gravitational stress of tilt. In summary, sympathetic tone was increased in severe heart failure, correlating inversely with cardiac function. Although there was improvement of cardiac function with first-dose captopril therapy, significant correlations of supine improvement with reduction of sympathetic tone were noted primarily with long-term therapy. Responsiveness of sympathetic tone to the stress of tilt however, was evident during first-dose and long-term therapy.     

Evaluation of a long-acting converting enzyme inhibitor (enalapril) for the treatment of chronic congestive heart failure

Converting enzyme inhibition of the renin-angiotensin system has proved a valuable therapeutic approach in patients with severe chronic congestive heart failure. In the present study, a new long-acting converting enzyme inhibitor (enalapril) was evaluated with acute single dose testing (10, 20 or 40 mg) in nine patients with severe chronic congestive heart failure. Four hours after administration, there was a significant reduction of systemic vascular resistance (-19%) and pulmonary wedge pressure (-19%); in addition, there were related increases of cardiac index (+16%) and stroke index (+19%) (probability [p] less than or equal to 0.05 for all changes). This was associated with an increase of plasma renin activity (9 +/- 3 to 35 +/- 11 ng/ml per hour) and a decrease of plasma aldosterone (19 +/- 4 to 9 +/- 2 ng/100 ml) (p less than 0.02 for both). With long-term therapy (1 month), there was improvement of exercise tolerance time and lessening of symptoms based on the New York Heart Association classification. Hemodynamic improvement was maintained in most, but not all, patients. There was no orthostatic hypotension during head-up tilt and hemodynamic values in the upright position were associated with normalization of intracardiac pressures. Long-term converting enzyme inhibition was indicated by a persistent increase of plasma renin activity (16 +/- 2 ng/ml per hour) and a decrease of plasma aldosterone (8 +/- 3 ng/100 ml). In addition, relative angiotensin II receptor occupancy was decreased as judged by the pharmacodynamic response to infusion of the angiotensin II analog saralasin. In conclusion, the long-acting converting enzyme inhibitor, enalapril, was effective in patients with chronic congestive heart failure; however, additional studies will be necessary to further delineate the optimal dose range and identify those patients who are most likely to respond to the drug.     

Effect of chlorpropamide treatment on insulin secretion in diabetics: its relationship to the hypoglycemic effect

We have studied the effect of chronic chlorpropamide treatment on insulin secretion in response to intravenous glucose. With treatment, the mean insulin level 5 min after glucose infusion increased significantly in 1 wk and reached its highest level in 1 mo. The increment over the fasting level increased similarly. The mean levels 20, 30, and 60 min after infusion did not change with treatment. All the diabetics, except one, with a fasting blood glucose level greater than $\text{160}\text{mg}\text{100}\text{ml}$ had little or no insulin secretion 5 min after glucose infusion either before or during chlorpropamide treatment. The mean blood glucose levels decreased during treatment more in those with higher initial glucose levels. The increase in early (5 min) insulin secretion was not related to the hypoglycemic effect. These data indicate that with chlorpropamide treatment early insulin secretion usually improves. Since a decrease in blood glucose may occur without any change in insulin secretion in some patients, it is suggested that the improvement in early insulin secretion may be secondary to a general improvement in carbohydrate metabolism.     

Studies in myasthenia gravis: Early thymectomy: Electrophysiologic and pathologic correlations

Indications for thymectomy in myasthenia gravis have been recently expanded to include all cases with extraocular symptoms as a result of the minimal morbidity and negligible mortality of the transcervical approach.As increasing numbers of patients with myasthenia gravis, covering the entire spectrum of generalized disease, have been added to the thymectomy population, a more accurate evaluation of the effects of the operation is possible.Our experience with 353 patients who have undergone thymectomy indicates that early thymectomy, particularly in patients who do not have thymic germinal centers, is followed by early remission of the disease. Delayed remission after thymectomy is related to the duration and severity of the disease, and to presence of thymic germinal centers. Germinal centers were found more frequently in patients with long duration of the disease and in patients in whom the disease had progressed to respiratory involvement. Marked improvement in electromyographic findings immediately after thymectomy was observed in the majority of patients who had had the disease for 1 year or less and where germinal centers were absent. The percentage of malignant thymomas was higher in patients who underwent thymectomy 1 year or more after the onset of symptoms of myasthenia gravis.These data indicate the importance of early thymectomy while the disease is still in the mild stages. Transcervical thymectomy is the treatment of choice as it is followed by a higher percentage of remissions and by less morbidity than other forms of treatment.     

The contributions of sympathetic tone and the renin-angiotensin system to severe chronic congestive heart failure: Response to specific inhibitors (prazosin and captopril)

The contribution of sympathetic tone and the renin-angiotensin system to the pathogenesis of chronic heart failure was evaluated. In 20 paired studies of the same 10 patients, the baseline hemodynamic and humoral correlates of congestive heart failure, and the response to alpha adrenergic blockade (prazosin) and angiotensin converting enzyme inhibition (captopril) were assessed. Despite the extent of failure, baseline plasma renin activity ranged from normal to very high. In contrast, baseline plasma catecholamlne levels were always elevated. Baseline plasma norepinephrine reflected the severity of heart failure, correlating inversely with baseline cardiac index before administration of both drugs. Comparable improvement in left ventricular function was noted after acute therapy. Baseline renin and norepinephrine did not predict the response to prazosin, but baseline renin did predict the response to captopril: pulmonary wedge pressure (r = ?0.776, p < 0.01), stroke index (r = 0.752, p < 0.02), systemic vascular resistance (r = ?0.673, p < 0.05).In summary, elevated levels of plasma norepinephrine were inversely correlated with baseline cardiac function, but norepinephrine levels did not change despite improved hemodynamics with specific prazosin therapy. The renin-angiotensin system exhibited a wide spectrum of activity and hemodynamic improvement with captopril was related to this activity. Absence of a correlation between plasma norepinephrine and plasma renin activity suggested that their contributions to vasoconstriction were not interdependent. Increased sympathetic tone was consistent in severe heart failure, whereas renin-angiotensin activity differed widely. The response to captopril can be used to identify a subset of patients with severe heart failure and adverse angiotensin-mediated vasoconstriction.     

Constrictive pericarditis in procainamide-induced lupus erythematosus syndrome

A rare case of constrictive pericarditis in procainamide-induced lupus erythematosus syndrome is reported. After 6 months of procainamide therapy fever, pleuritic chest pain, arthralgia and muscle soreness developed in a 47 year old man. These symptoms were soon followed by the onset of acute pericarditis and rapidly accumulating massive pericardial effusion. After withdrawal of procainamide therapy and administration of corticosteroids in large doses, there was marked subjective improvement and rapid reduction in pericardial effusion. However, constrictive pericarditis with massive leg edema and ascites developed 6 weeks after admission as corticosteroid therapy was gradually discontinued. These manifestations subsided after pericardiectomy was performed.     

Trends in cardiovascular surgery (1961 to 1977): review of the New York City and State experience.

Statistical data on surgery for congenital and acquired heart disease in New York City in the 17 years from 1961 through 1977 and in New York State in 1977 document trends in volume and types of operations. The ratio of open heart surgery for congenital versus acquired heart disease of 2:1 in 1961 was reversed to 1:6 by 1977 as the number of procedures for valvular and coronary heart disease increased. The case load for surgery for congenital heart disease using the open heart technique gradually increased until 1974 and then decreased. The number of closed heart operations was relatively constant. Observations suggest a finite number of open and closed heart operations for congenital cardiac malformations; the total of 1,381 cardiac operations in New York State in 1977 may continue to diminish if the decreasing birth rate persists.The number of operations for valvular heart disease has stabilized, but the number of operations for coronary artery disease has increased 7-fold since 1970. In 1977, coronary arterial bypass grafting in New York State accounted for 63 percent of operations for acquired heart disease (6,407) and the mortality rate was less than 3 percent.     

Lepromatous leprosy masquerading as disseminated tuberculosis.

A patients with disseminated leprosy is described. A 57 year old man from Cuba presented with fever and pancytopenia. Bone marrow aspirate showed numerous acid-fast bacilli and a liver biopsy specimen contained multiple granulomas. The patient was considered to have tuberculosis and was treated with isoniazid and rifampin, with initial clinical improvement, only to have the fever recur and to show deterioration in hematologic and hepatic function. Failure to grow M. tuberculosis suggested a diagnosis of leprosy which was proved by skin biopsy. How lepromatous leprosy can masquerade as disseminated tuberculosis is discussed.     

Transient functional asplenism in sickle cell-C disease

During a painful crisis, the spleen of a patient with sickle cell-C disease failed to visualize with 99^m Tc-sulfur colloid although it was significantly enlarged by palpation. At that time, the peripheral blood smear showed numerous sickle cells, normoblasts and erythrocytes containing Howell-Jolly bodies. After therapy with hypotonic saline solution and with clinical improvement, the enlarged spleen was completely visualized on repeat scanning. The normoblasts and erythrocytes containing Howell-Jolly bodies also decreased in number. This case of transient asplenism is similar to the cases previously reported only in children with homozygous sickle cell disease. The significance of this phenomenon with respect to increased susceptibility to infection is discussed.     

Analysis of T cell subsets in cancer patients treated with interferon

T cell subsets were analyzed in 33 patients with advanced cancer who were treated with either of two interferon preparations: a partially purified human leukocyte interferon (HulFN-alpha (Le] and a highly purified recombinant interferon (lFLrA). Included in the lFLrA-treated group were eight patients with immunodeficiency and Kaposi's sarcoma. The OKT4+/OKT8+ ratio was used to define the balance between helper/inducer and suppressor/cytotoxic T cell subsets. With both interferon preparations, the mean OKT4+/OKT8+ ratio decreased 24 hours after the first interferon dose. Within the HulFN-alpha (Le) group, the decrease in ratio was related to an increase in OKT8+ cells; in the lFLrA group, it was accompanied by a small decrease in the proportion of OKT4+ cells that was greater than the decrease in OKT8+ cells. Patients treated with lFLrA were followed for the first three weeks of therapy. Most patients treated with lFLrA at all dose levels, ranging from 1 X 10(6) to 54 X 10(6) units per day, had a decrease in OKT4+/OKT8+ ratio on Day 1. No substantial change in the ratio was observed on Days 7, 14, and 22. Patients with immunodeficiency and Kaposi's sarcoma had responses similar to those of patients with other cancers treated with lFLrA. In conclusion, although both HulFN-alpha (Le) and lFLrA induce immediate decreases in the OKT4+/OKT8+ ratio, the T cell subset(s) primarily responsible for the decrease varies with the source of interferon.     

Parasitic diseases in immunocompromised hosts

In patients with compromised host defenses, diseases caused by protozoans and nematodes appeared, a few years ago, to be declining in importance. However, the outbreak of the acquired immunodeficiency syndrome (AIDS) among homosexual men, parenteral drug abusers, and other groups has made it necessary for physicians to familiarize themselves again with the manifestations of these diseases in abnormal hosts. The groups of patients at greatest risk, the usual clinical syndromes in abnormal hosts, and current approaches to diagnosis, treatment, and prevention of infections due to Pneumocystis carinii, Toxoplasma gondii, Strongyloides stercoralis, and organisms of the genus Cryptosporidium are reviewed. Because so many cases of these infections have recently occurred among patients with AIDS, recent experience with these diseases in AIDS patients at Memorial Sloan-Kettering Cancer Center is summarized.     

Release of nucleosides from canine and human hearts as an index of prior ischemia

During ischemia, myocardial adenosine triphosphate is degraded to adenosine, inosine and hypoxanthine. These nucleosides are released into coronary venous blood and may provide an index of ischemia; adenosine may also participate in the autoregulation of coronary flow. In dogs, the temporal relations between reactive hyperemic flow and nucleoside concentrations in regional venous blood were correlated after brief occlusions of a segmental coronary artery. Reactive hyperemia and adenosine release peaked together in 10 seconds, persisted for 10 to 30 seconds and then decreased in a pattern consistent with the hypothesis that they are related. During initial reflow after 45 seconds of ischemia, mean concentrations of adenosine, inosine and hypoxanthine increased, respectively, to 52, 67 and 114 nmol/100 ml plasma; after 5 minutes of ischemia, the respective levels increased to 58, 1,570 and 1,134 nmol and fell quickly.In nine patients there was a similar release of nucleosides into coronary sinus blood during reperfusion after 59 to 80 minutes of ischemic arrest during cardiac surgery. With initial reflow, adenosine, inosine and hypoxanthine levels reached 65, 655 and 917 nmol/100 ml of blood, respectively. Inosine and hypoxanthine concentrations remained high for 5 to 10 minutes after cardiac beating resumed, often when production of lactate had decreased.The results indicate that postischemic release of nucleosides reaches significant levels in man as well as animals, is parallel with the duration of ischemia, is temporary and may be a useful supplement to measurement of lactate as an index of prior myocardial ischemia.     

Outbreak of Kaposi's sarcoma with cytomegalovirus infection in young homosexual men

Kaposi's sarcoma, a multicentric malignant neoplasm, occurs in certain geographic areas in the world. It is most common in Equatorial Africa and Eastern Europe. The annual incidence of Kaposi's sarcoma in the United States is between 0.021 and 0.061 per 100,000 persons. The appearance of an outbreak of Kaposi's sarcoma in young homosexual men in New York and California is a new and unique phenomenon. Certain differences are already recognized between the disease in these young men and the ordinary Kaposi's sarcoma. Herein we report our observations of the first 10 cases of Kaposi's sarcoma in young homosexual men. In these patients, the disease follows an aggressive clinical course characterized by widespread skin lesions with early involvement of the lymph nodes. In some of these patients, the result was death in a short period of time after initial diagnosis. In addition, cytomegalovirus infections were seen in these patients, which suggests at least a possible association between this viral and the disease.     

Thrombolytic therapy in acute myocardial infarction: Review of clinical trials

Intracoronary infusion of streptokinase is associated with recanalization rates of 60 to 90% immediately after the procedure. Mortality data in published trials are conflicting. In 125 registry patients who had paired contrast ventriculograms before streptokinase infusion and hospital discharge, improvement in ejection fraction correlated with incomplete coronary obstruction before angiography, the presence of collateral vessels to the infarct area and recanalization of complete obstruction. In assessing the risk/benefit ratio of intracoronary streptokinase infusion, the risks of angiography in the setting of acute myocardial infarction, reocclusion, bleeding and such secondary interventions as angioplasty or bypass surgery must be considered. Intravenous infusion of conventional doses of streptokinase was associated with improved survival in some trials in which therapy began within 12 hours after the onset of infarction. Immediate recanalization rates in patients who received large doses of intravenous streptokinase were lower than those associated with intracoronary streptokinase infusion. The risks and benefits of high-dose intravenous streptokinase administration must still be assessed.     

Iodine and lithium-induced hypothyroidism. Documentation of synergism

Signs and symptoms of hypothyroidism were noted in a patient receiving lithium carbonate for 2 years as prophylaxis against recurrence of mania and depression. When lithium therapy was stopped, the hypothyroidism resolved; but it recurred with the administration of potassium iodide. When potassium iodide therapy was discontinued, the patient became euthyroid. In a second patient chemical evidence of hypothyroidism (fall in circulating thyroid hormone levels, elevation of serum thyrotropin) was noted after he had received 3 weeks of lithium therapy. With the addition of potassium iodide, laboratory evidence of hypothyroidism became more pronounced, and clinical signs of hypothyroidism developed. All abnormalities resolved promptly following the withdrawal of lithium and iodine. These observations suggest that lithium and iodine can act synergistically to produce hypothyroidism and that the combined use of these agents should be avoided.