A Contemporary 20-Year Cleveland Clinic Experience of Nonbacterial Thrombotic Endocarditis: Etiology,Echocardiographic Imaging,Management, and Outcomes

BackgroundNonbacterial thrombotic endocarditis, or marantic endocarditis, is rare. Contemporary data on the etiology, echocardiographic evaluation, and management of nonbacterial thrombotic endocarditis are limited.MethodsA single-center retrospective cohort study was performed. Electronic medical records and echocardiographic records were searched for patients ages ≥18 years with a confirmed diagnosis of nonbacterial thrombotic endocarditis between January 1999 and November 2019. Demographic, echocardiographic, and management data were collected.ResultsOf 600,577 transthoracic echocardiograms (TTEs) and 89,264 transesophageal echocardiograms (TEEs), 42 patients had nonbacterial thrombotic endocarditis (mean age: 54 ± 14.5 years; 66.7% were female). The median duration of follow-up was 8.2 (interquartile range 3.3-24.4) months. Seventeen patients (40.5%) had malignancy, 33.3% had systemic lupus erythematosus, and 35.7% had antiphospholipid antibody syndrome. Stroke was the most common presentation (59.5%).TTE enabled the diagnosis in 19 cases (45.2%), compared with TEE, which identified the condition in 33 of 34 (97.1%) cases in which it was utilized. Three-dimensional echocardiography was performed in 17 TEEs. The most common valves involved were mitral (61.9%), and aortic (23.8%) valves. Thirty-two patients were managed with anticoagulation. Ten patients underwent surgery. Sixteen (38.1%) patients died, most of whom had a diagnosis of advanced malignancy.ConclusionIn a contemporary 20-year cohort, TTE and TEE played important roles in diagnosis, with superior diagnostic performance of TEE for nonbacterial thrombotic endocarditis. Mortality was high, and advanced malignancy portended a worse prognosis. Management in most cases was therapeutic anticoagulation. In select cases, surgery provided favorable outcomes.     

Congenital stenosis of the pulmonic and tricuspid valves. Clinical, hemodynamic and angiographic observations in a 20 year old woman

Patients with congenital stenosis of the tricuspid and pulmonic valves are rare, indeed, and are expected to have underdeveloped right ventricles. The three obstructions in series, namely, tricuspid orifice, right ventricular inflow and right ventricular outflow typically result in symptomatic, cyanotic infants or young children with right to left shunts at atrial level. We present unique clinical, hemodynamic and angiographic information on a 20 year old acynotic, virtually asymptomatic woman with normally formed right ventricle and congenital stenosis of the tricuspid and pulmonic valves.     

Thrombotic thrombocytopenic purpura in a previously splenectomized patient

Idiopathic thrombocytopenic purpura was diagnosed in a 26 year old man who had rectal bleeding and marked thrombocytopenia (10,000 platelets/mm³). Complete recovery followed treatment with steroids and splenectomy. There was no clinical, laboratory or histopathologic evidence of thrombotic thrombocytopenic purpura. Several months later typical thrombotic thrombocytopenic purpura developed; recovery followed treatment with steroids, aspirin and dipyridamole. The presence of Howell-Jolly bodies and a negative scan indicated that an accessory spleen was not present. Since thrombotic thrombocytopenic purpura developed in the absence of a spleen in this case, it may be that in some, if not all, instances of thrombotic thrombocytopenic purpura the spleen is not importantly related to the pathogenesis of thrombotic thrombocytopenic purpura; this is in contrast to the situation in idiopathic thrombocytopenic purpura. Benefits attributed to splenectomy in thrombotic thrombocytopenic purpura, therefore, may in fact be due to the platelet inhibitory properties of common anesthetic agents, or to some other factor in the surgical procedure rather than to removal of the spleen per se.     

Experience with pindolol, a betareceptor blocker, in the treatment of hypertension.

Ten patients, mean age 48 years, with essential hypertension of stage I and II according to the WHO classification, were studied at rest and during work before and after an average of two and 16 months of oral treatment with the beta-adrenergic blocking agent, pindolol. The pindolol treatment caused a significant decrease in the systemic systolic and diastolic blood pressure, both at rest and during work. Three mechanisms seem to be involved in the antihypertensive effect of pindolol: (1) a negative chronotropic effect on the heart, (2) a decrease in peripheral vascular resistance, and (3) an increase in venous capacitance affecting the venous return. However, the significance of these mechanisms seems to differ when the situations after two months of treatment are compared with those after 16 months of treatment. In the beginning, a decrease in cardiac output seems to be the main cause of the lowering of the blood pressure; later, a decrease in systemic vascular resistance might be of greater importance.     

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.     

Lead poisoning from a gunshot wound. Report of a case and review of the literature.

A man was hospitalized on three occasions for symptoms of lead intoxication 20 to 25 years after a gunshot wound that resulted in retention of a lead bullet in his hip joint. The potential for lead toxicity as a complication of a lead missile injury appears to be related to (1) the surface area of lead exposed for dissolution, (2) the location of the lead projectile, and (3) the length of time during which body tissues are exposed to absorbable lead. Cases of lead poisoning of immediate onset resulting from lead shot have been reported in Europe, but all documented cases of ammunition-related plumbism reported in the United States have involved synovial fluid dissolution of a single lead bullet over many years. The solvent characteristics of synovial fluid and associated local arthritis are apparently important factors in the dissolution and absorption of lead from projectiles located in joints. Awareness that lead intoxication can be a complication of retained lead projectiles should allow rapid institution of appropriate diagnostic and therapeutic modalities when such a clinical situation arises.     

Nonbacterial thrombotic endocarditis in patients with malignant neoplastic diseases

Seventy-five patients with malignant neoplastic disease and nonbacterial thrombotic endocarditis (NBTE) were studied. The over-all frequency of NBTE was double that observed in other reported autopsy series not limited to patients with cancer. The incidence in autopsy patients with bronchiolar and adenocarcinoma of the lung was twice that of patients with pancreatic and prostatic adenocarcinoma and seven times that of patients with breast cancer. The development of NBTE could not be ascribed to duration of illness or nutritional state. Since 14 patients died of cerebral infarcts and five had major myocardial infarcts resulting from thromboemboli, these and other complications of NBTE should be anticipated particularly in patients with those cancers most often associated with vegetative endocarditis.     

Hemodynamic and endocrine changes associated with captopril in diuretic-resistant hypertensive patients

To evaluate the therapeutic efficacy of oral angiotensin-converting enzyme inhibition with low-dose (average 30 mg/day) captopril in diuretic-resistant hypertension, its long-term cardiocirculatory action was determined by dye-dilution method and venous-occlusion forearm plethysmography in 11 uncontrolled patients taking a thiazide diuretic. Significant declines in mean blood pressure (average 12.4 ± 1.4 percent) and systemic vascular resistance (28.7 ± 3.2 percent) accompanied an increase in cardiac output (24.8 ± 4.1 percent). Forearm vascular resistance (16.0 ± 2.7 percent) decreased considerably, but the decrease in limb vascular resistance did not parallel the fall in systemic vascular resistance in magnitude (p < 0.01), indicating that arteriolar dilatation occurred on a selective basis. Plasma renin activity increased after therapy as plasma aldosterone levels consistently fell, while plasma norepinephrine concentrations were not changed. There was a direct correlation between pretreatment plasma renin activity and the magnitude of the decline in systemic vascular resistance (p < 0.05).These findings suggest that the Inhibition of angiotensin-converting enzyme with captopril in diuretic-resistant hypertensive patients improves cardiocirculatory function through selective dilatation. The reordering of regional blood flow, which appears to result from release of angiotensin-mediated vasoconstriction as well as the suppression of aldosterone, may underlie the prolonged benefit observed in these patients. This oral vasodilator in very low dose appears to represent an effective adjunct for the treatment of hypertension refractory to diuretics.     

Usefulness and limitations of saralasin, a partial competitive agonist of angioten II, for evaluating the renin and sodium factors in hypertensive patients.

Saralasin was infused into 52 untreated hypertensive patients. Immediate, transient pressor responses occurred in 94 per cent followed by a more gradual sustained change in blood pressure reaching an apogee in about 20 minutes. Most (86 per cent) patients with high renin values had sustained depressor responses irrespective of sodium balance. In contrast, during a normal sodium intake, the drug produced a neutral (45 per cent) or mildly pressor (50 per cent) response in patients with normal renin and pressor responses in patients with low renin values. Sodium depletion abolished pressor responses and resulted in depressor responses in 64 per cent of the patients with normal renin values. The pretreatment angiotensin level appeared to determine direction and amplitude of the response to saralasin, since increases and decreases in diastolic pressure exhibited a highly significant relationship to the control renin lever (r = 0.80, p less than 0.001). Above a neutral range of control renin values, from 2 to 7 ng Al/ml/hour, depressor responses were the rule, and below it pressor responses were consistent. Sodium balance also appeared to determine the amplitude of the response. In a subset of patients with similar renin values (range 1.4 to 2.2 ngAl/ml/hour), the induced pressor responses correlated directly with the 24-hour sodium excretion (p less than 0.05). For all patients, the induced pressure change also was related to the rate of sodium excretion (r = 0.53, p less than 0.001). The data suggest that saralasin behaves as a partial competitive agonist of angiotensin II. For this reason, saralasin testing provided only a rough physiologic validation for renin profiling. Thus, depressor responses expose most patients with high renin values. Neutral responses occur in many patients with normal renin and intermediate renin values. But pressor responses occur in subjects with either low or normal renin levels and they may reflect sodium and volume excess associated with a partial or relative absence of renin. Accordingly, due to its partial agonism, saralasin testing under-estimates the renin factor. Hence, the drug cannot be used to identify or exclude renin involvement in the blood pressure in the large majority of hypertensive patients who do not exhibit depressor responses. For them an agent devoid of agonism is required. Moreover, prior sodium depletion as a device to increase the frequency of depressor responses to saralasin does not measure intrinsic renin dependency of the blood pressure but rather the reactivity of the system to sodium depletion.