Emilio R. Giuliani

The natural history of idiopathic dilated cardiomyopathy

Between 1960 and 1973, a total of 104 patients at the Mayo Clinic had a diagnosis of idiopathic dilated cardiomyopathy on the basis of clinical and angiographic criteria; these patients were followed up for 6 to 20 years. Twenty-one percent of the patients had a history of excessive consumption of alcohol, 20 percent had had a severe influenza-like syndrome within 60 days before the appearance of cardiac manifestations and 8 percent had had rheumatic fever without involvement of cardiac valves several years before; thus, possible etiologic risk factors of infectious-immunologic type may be important. Eighty patients (77 percent) had an accelerated course to death, with two thirds of the deaths occurring within the first 2 years. Twenty-four patients (23 percent) survived, and 18 of them had clinical improvement and a normal or reduced heart size. Univariate analysis at the time of diagnosis revealed three factors that were highly predictive (p less than 0.01) of the clinical course: age, cardiothoracic ratio on chest roentgenography and cardiac index. Systemic emboli occurred in 18 percent of the patients who did not receive anticoagulant therapy and in none of those who did; thus, anticoagulant agents should probably be prescribed unless their use is contraindicated.

Emboli in Infective Endocarditis: The Prognostic Value of Echocardiography

OBJECTIVE To determine whether vegetations visualized on two-dimensional echocardiography are an independent risk factor for the development of subsequent emboli in patients with infective endocarditis and to assess the timing of emboli relative to the initiation of antimicrobial therapy. DESIGN Investigator-blinded, retrospective incidence cohort study. SETTING Tertiary referral center. PATIENTS Patients with left-sided native valve infective endocarditis who had two-dimensional echocardiography within 72 hours of beginning antimicrobial therapy. MEASUREMENTS AND MAIN RESULTS The crude incidence rate of first embolic events in patients receiving antimicrobial therapy was 6.2 per 1000 patient-days (95% CI, 4.2 to 9.2). The rates in patients with and without vegetations were 7.1 and 4.9 per 1000 patient-days, respectively (incidence rate ratio, 1.4; 95% CI, 0.6 to 3.3). The relation between vegetations and risk for emboli was microorganism-dependent: Stratified incidence rate ratios were 6.9 (95% CI, 1.1 to 42.5; P less than 0.05) and 1.0 (95% CI, 0.2 to 3.9) for viridans streptococcal and Staphylococcus aureus endocarditis, respectively. The rate of first embolic events diminished over time (P less than 0.001), falling from 13 per 1000 patient-days during the first week of therapy to less than 1.2 per 1000 patient-days after completion of the second week of therapy. CONCLUSIONS Overall, the presence of vegetations on echocardiography was not associated with a significantly higher risk for embolus in patients with left-sided native valve infective endocarditis. The relative risk for embolic events associated with echocardiographically visualized vegetations may be microorganism-dependent, with a significantly increased risk seen only in patients with viridans streptococcal infection. The rate of embolic events declines with time after initiation of antimicrobial treatment.

M-mode and two-dimensional echocardiographic features in cardiac amyloidosis.

SUMMARY Twenty-eight patients with cardiac amyloidosis were studied by echocardiography — 26 by Mmode and 13 by two-dimensional (2D) studies. All had heart failure and biopsy-proved amyloidosis. M-mode features included (1) normal left ventricular (LV) dimension in all; (2) thickened ventricular septum (88%), LV posterior wall (77%), and right ventricular (RV) anterior wall (79%); (3) decreased thickening of ventricular septum (96%) and of LV posterior wall (65%) and reduced LV global function (62%); (4) left atrial enlargement (50%); and (5) pericardial effusion (58%). Two-dimensional echocardiography provided additional features: (1) thickened papillary muscles (five of 13); (2) thickened valves (four of 13); (3) better appreciation of thickened RV wall; and (4) a characteristic “granular sparkling” appearance of thickened cardiac walls — presumably secondary to the amyloid deposit — which was noted in 12 of 13 patients. Thus, M-mode echocardiography is helpful in the recognition of cardiac amyloidosis. However, the better appreciation with 2D echocardiography of thickened cardiac walls with a “granular sparkling” appearance in patients with unexplained cardiac failure is virtually diagnostic of cardiac amyloidosis.

Prosthetic Valve Endocarditis

Prosthetic valve endocarditis is an infrequent but serious complication of cardiac valve replacement. The overall frequency of prosthetic valve endocarditis is approximately 2%. The frequency of early-onset and late-onset infections is 0.78% and 1.1%, respectively. Staphylococci are the most common isolate from patients with early-onset infection, accounting for 47.5% of the total number of isolates. Staphylococcus epidermidis causes 27% of these staphylococcal infections. Among patients with late-onset infection, streptococci are the predominant microorganism, constituting 42% of the total number of isolates from patients in this group. The overall mortality among patients with prosthetic valve endocarditis is high--59%; the mortality among patients with early- or late-onset infections is 77% and 46%, respectively. Most patients with staphylococcal prosthetic valve endocarditis should undergo cardiac valve replacement in addition to antimicrobial therapy. Closely monitored anticoagulant therapy should be cautiously continued in patients with prosthetic valve endocarditis.

Prognosis in rupture of the ventricular septum after acute myocardial infarction and role of early surgical intervention

Since 1944, 91 patients (50 men and 41 women, mean age 68 years [range 39 to 86]) with ventricular septal rupture after acute myocardial infarction were seen at the Mayo Clinic. Patients were divided into 4 groups according to therapy and timing of surgical intervention. Fourteen patients seen before 1965, when surgery was not performed for such a complication or not readily available, were excluded from the analysis. Group 1 (n = 22) had surgery within 48 hours of septal rupture, group 2 (n = 6) underwent operation between 2 and 14 days, group 3 (n = 24) had surgery after 14 days, and group 4 (n = 25) only received medical treatment. Short-term (30 days) survivors (45%, 35 of 77 patients) were compared with nonsurvivors. Using logistic regression, by univariate analysis, 3 variables were significantly associated with outcome: age (p less than 0.01), cardiogenic shock (p less than 0.00001), and long delay between ventricular septal rupture and surgical intervention (p less than 0.004). By multivariate analysis, however, only cardiogenic shock (p less than 0.00001) and age (p less than 0.007) correlated with an adverse outcome. In patients with cardiogenic shock after septal rupture, the prognosis was uniformly fatal unless patients undergo early surgery. None of the 23 patients in groups 2, 3 or 4 survived, whereas 5 of 13 patients (38%) who had surgery within 48 hours of septal rupture survived.(ABSTRACT TRUNCATED AT 250 WORDS)

Spectrum of echocardiographic findings in bacterial endocarditis.

Forty-seven echocardiograms were obtained in 32 patients with bacterial endocarditis. Preexistent abnormalities were found in 14 patients. In five of them thought to have bacterial endocarditis on normal valves, echocardiography showed mitral stenosis (one), bicuspid aortic valve (two), and prolapse of mitral valve (two). Definite vegetations were seen in 22 patients--on the aortic valve in seven, the mitral valve in 12, and both valves in three. Ten patients had milder changes suggestive but not diagnostic of vegetations. In 12 patients, surgery confirmed the echocardiographic findings. Fourteen had systemic embolic episodes and all had echocardiographic evidence of vegetations. Abnormalities secondary to bacterial endocarditis, other than vegetations, were common. Twenty-one patients had left ventricular volume overload. Ten had a flail posterior leaflet of the mitral valve, three of which were confirmed surgically. Eight had abnormal coarsely fluttering echoes in the left ventricular outflow tract consistent with a prolapsing aortic valve or underlying aortic vegetations; four were confirmed by surgery. Five had signs of severe aortic regurgitation of recent onset (premature mitral valve closure) and all had confirmation by surgery. Echocardiographic abnormalities persisted after successful medical treatment. We conclude that echocardiography is helpful in patients with bacterial endocarditis. It permits recognition of unsuspected preexistent lesions and the characteristic vegetations, as well as the extent and nature of valvular damage secondary to bacterial endocarditis. However, echocardiography does not differentiate between active and healed lesions.

Postinfarction Ventricular Septal Rupture Surgical Considerations and Results

Twenty-two patients had ventricular septal rupture complicating acute myocardial infarction. Sixteen of the 22 patients underwent surgical repair. The clinical findings, catheterization data, and operative results suggest that closure of the rupture should be delayed when possible from three to six weeks after the infarction to allow firm fibrous healing of the region. When surgery is thus delayed, the operative risks are smaller and the long-term results are good.

The Association of Unexplained Gastrointestinal Bleeding with Calcific Aortic Stenosis

The association of chronic gastrointestinal bleeding and aortic stenosis remains problematical. The cases of 91 patients (age 38 to 80 years) with these disorders who were examined between 1955 and 1975 were reviewed to address this controversy. All patients underwent upper and lower gastrointestinal radiography, small bowel series, and proctoscopy. Other studies were endoscopy in 84 patients, colonoscopy in 61, and visceral angiography in 16. Of the 37 patients who underwent abdominal exploration, 35 (95%) continued to bleed postoperatively, including 8 of 10 patients who had bowel resection for angiodysplasia. Forty patients did not have an abdominal operation, and all have continued to bleed. Sixteen patients (2 of whom had had an abdominal procedure) underwent aortic valve replacement for aortic stenosis. There were 2 intraoperative deaths among these 16 patients. At follow-up, which ranged from 8 to 12 years, only 1 patient who underwent aortic valve replacement had recurrent bleeding secondary to excessive anticoagulation. Thus, overall, gastrointestinal operation was successful in only 5% of patients, but aortic valve replacement was effective in 93%. For unexplained gastrointestinal bleeding associated with aortic stenosis, aortic valve replacement should be considered because of the likelihood of cure.

Computerized M-mode echocardiographic analysis of left ventricular dysfunction in cardiac amyloid.

We assessed left ventricular (LV) function in cardiac amyloid using computer-assisted analisis of M-mode echocardiograms from 20 patients with biopsy-proved amyloid and compared them with similar data from 20 normal subjects. Patients with cardiac amyloid had a consistent and characteristic set of quantitative echocardiographic findings: (1) LV cavitv size was normal or small. (2) The peak rate of diastolic cavity filling was decreased (p < 0.01). (3) Isovolumic relaxation was prolonged (p < 0.01). (4) Fractional shortening and peak Vcf were decreased (p < 0.01). (5) Peak rates of both systolic thickening and diastolic thinning of the septum and posterior LV wall were decreased (p < 0.01). LV function in patients with cardiac amyloid was compared with that in patients with aortic stenosis and normal coronary arteries, who were used as a model of similar wall thickness and cavitx size. There was significantly greater impairment of regional and global LV function in amyloid, that is, more than could be accounted for by increased wall thickness alone, indicating that the further abnormalities of LV function were caused by an intramyocardial restriction secondary to amyloid deposition per se. LV function was also compared in amyloid and in patients with nonobstructive hypertrophic cardiomvopathv, as these two groups of patients may be confused both clinically and echocardiographically. The technique we used differentiated between these two disorders in terms of cavity and regional LV dynamics when patients were considered as a group, but with less certaintv when patients were considered individuallv due to overlap between the two groups. The severity and consistency of the echocardiographic abnormalities in cardiac amyloid are of value in establishing the diagnosis, which can be confirmed directlv bv tissue biopsv.

Hypertrophic obstructive cardiomyopathy: Ten- to 21-year follow-up after partial septal myectomy

This study reviews the outcome in 36 consecutive patients who survived partial septal myectomy for hypertrophic obstructive cardiomyopathy operated on between 1960 and 1972. All patients were followed up until death or until June 1981 (mean 13.4 years). Of the 26 survivors, 17 had been more than mildly symptomatic preoperatively, but only 1 remained so postoperatively. The operation was effective in relieving the obstruction (peak systolic pressure gradient reduced from 79 to 8 mm Hg [p less than 0.001]), and mitral regurgitation was relieved. No survivors symptoms worsened, but 10 died late--4 suddenly, 5 from congestive heart failure, and 1 from a malignancy. The 10-year survival rate was 77%. No correlation with outcome was found with respect to age, surgical approach, preoperative functional class, pressure gradient, left ventricular end-diastolic pressure, or presence of atrial fibrillation, but atrial fibrillation occurring late postoperatively (12 patients) was associated with an increased frequency of late death (7 of 10 late deaths) or continuing New York Heart Association functional class III status. Early or late postoperative complete heart block occurred in 1 patient each. Thus, these results suggest a favorable effect of operation and support continued surgical intervention for appropriate patients.