Clinical (short stature, macrocephaly, constipation) and biochemical (low free T4/free T3 ratio, subnormal rT3) findings that are congruent with previous cases and newly recognized features (epilepsy) in this adult female with defective TRα define a shared phenotype in TRα-mediated resistance to thyroid hormone, with differential tissue responses to T4 treatment.

In a controlled study, the cardiac involvement and arrhythmia profile of 32 patients with acromegaly were correlated with endocrine parameters (somatomedine C, growth hormone), clinical score and duration of the disease. Data were compared with those of 50 controls free of cardiac disease. Stress ECG, 24 h Holter monitoring and echocardiography were performed. Supraventricular premature complexes occurred no more often in acromegalics than in controls. Both prevalence and severity of ventricular arrhythmia, however, were significantly higher in patients compared to controls (P less than 0.01). 15/32 (48%) acromegalic patients had complex ventricular arrhythmias (Lown III-IV) as compared with 6/50 (12%) normal subjects (P less than 0.01). Repetitive ventricular arrhythmias (Lown IV a/b) occurred in 10/32 (31%) patients, but only in 4/50 (8%) controls (P less than 0.01). Furthermore, the frequency of ventricular premature complexes increased with duration of acromegaly (P less than 0.01). No correlation was found between the severity of ventricular arrhythmia and hormone levels. Left ventricular muscle mass was significantly increased (285 +/- 139 g, P less than 0.02) due to concentric hypertrophy. Severity of ventricular arrhythmias correlated with left ventricular mass and with clinical activity score (P less than 0.01). Thus, compared to controls, acromegalic patients show more frequent and complex ventricular arrhythmias and left ventricular hypertrophy. Duration of the disease rather than hormone levels seems to be relevant for these pathological changes.

Infective endocarditis is associated with significant morbidity and mortality, with valvular destruction and congestive heart failure being more common in patients with echocardiographically discernible vegetations. The transoesophageal approach affords consistently high quality images with excellent structural resolution. Two-hundred and eighty-one patients with clinically suspected infective endocarditis were studied, to evaluate the prognostic value of ascertaining the site of vegetations. Among them were 118 patients with vegetations attached to the aortic or mitral valve. These patients were followed for a mean period of 14 months. Mitral valve vegetations were associated with a significantly higher incidence of embolic events than vegetations on aortic valves (25% vs 9.7%). The incidence of abscess formation was higher in aortic than in mitral valve endocarditis (6% vs 0%), as were the need for surgical intervention (11% vs 5.5%) and mortality (1.6% vs 0%) respectively). Bivalvular endocarditis was associated with an increased rate of complications: embolism (50%), abscess formation (15%), surgery (35%) and mortality (10%). By multivariate analysis, echocardiographically accessible risk factors for subsequent embolism were a vegetation size of more than 10 mm and mitral valve involvement. Risk factors associated with in-hospital fatality were embolism, a vegetation size of more than 10 mm, and Staphylococcus aureus infection. Our data suggest that the site influences both the rate and the type of complications. Precise echocardiographic visualization of vegetations helps to stratify patients into a high-risk sub-group, perhaps warranting early prophylactic surgical intervention. Transoesophageal echocardiography may play an important role in assessing the clinical outcome for these patients.

No abstract

The object of the study was to follow patients with endocarditis-associated abscesses in order to evaluate the clinical outcome with and without surgical intervention. Transesophageal echocardiography successfully displayed the location and extent of abscess cavities in 14 patients (group A) with aortic valve endocarditis. The infective process was limited to the perivalvular tissue in two, extended into the ascending aorta in six, and included the interventricular septum, the right ventricular outflow tract, interatrial septum, and/or mitral valve annulus in six patients. The complication rate was significantly higher in group A than in group B, which consisted of 27 patients with proven signs of endocarditis but without endocarditis-associated abscesses. The complication rates were embolic events 64.3% in group A vs 29.6% in group B, need for surgery in 64.3% vs 18.5%, and death in 50.0% vs 3.7%, respectively. The duration of fever--as a marker of an active infective process--before diagnosis and the onset of adequate treatment was significantly higher in group A than in group B (46.7 +/- 8.4 days vs 7.7 +/- 2.6 days). Organisms were isolated in 71.4% in group A and in all patients of group B. Streptococcal infections were noted in A in 54.5% vs 44.4% in B., staphylococcal in 27.3% vs 40.7%. Initial surgical repair in 9 of 14 patients in A (64.3%) included nine aortic valve and one mitral valve prosthesis implantations, two aortic valve-annulus reconstructive procedures, one dacron patch closure, and three partial resections of the aorta ascendens with end-to-end anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)