Spotty pigmentation and atrial myxoma: a case report of Carney's complex.

A case of atrial myxoma that was associated with marked spotty mucocutaneous pigmentation is described. The combination of these two conditions has been described as an easily-recognized syndrome which is familial. Other features which may be associated with the syndrome are cutaneous myxomas and endocrine tumours.

Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis.

The effects of peripheral cold exposure on myocardial perfusion and function were studied in 13 patients with scleroderma without clinically evident myocardial disease. Ten patients had at least one transient, cold-induced, myocardial perfusion defect visualized by thallium-201 scintigraphy, and 12 had reversible, cold-induced, segmental left ventricular hypokinesis by two-dimensional echocardiography. The 10 patients with transient perfusion defects all had anatomically corresponding ventricular wall motion abnormalities. No one in either of two control groups (9 normal volunteers and 7 patients with chest pain and normal coronary arteriograms) had cold-induced abnormalities. This study is the first to show the simultaneous occurrence of cold-induced abnormalities in myocardial perfusion and function in patients with scleroderma. The results suggest that cold exposure in such patients may elicit transient reflex coronary vasoconstriction resulting in reversible myocardial ischemia and dysfunction. Chronic recurrent episodes of coronary spasm may lead to focal myocardial fibrosis.

Clinical recognition of giant left ventricular aneurysm.

The noninvasive diagnosis of left ventricular aneurysm has markedly improved with gated blood pool scintigraphy. However, in patients with giant anterior ventricular aneurysms, the gated blood pool scintigram performed in two standard views (anterior and 40 degree left anterior oblique) may incorrectly suggest ischemic cardiomyopathy. We retrospectively identified five patients who underwent resection of a ventricular aneurysm over a 2 1/2 year period and who had preoperative scintigraphic studies that appeared to show severe diffuse left ventricular dysfunction. contrast ventriculography demonstrated preserved wall motion in septal, inferior and lateral segments not seen by gated blood pool scintigraphy and showed extraordinarily large anterior aneurysms. M-mode or two-dimensional echocardiograms showed intact posterior wall function in all patients, suggesting severe regional myocardial disease rather than global dysfunction. Two-dimensional echocardiography showed additional segments with preserved function as well as discrete aneurysms in all patients. We conclude that gated blood pool scintigraphy, when performed in two standard views, may fail to correctly diagnose some patients with very large anterior wall aneurysms. M-mode echocardiography, two-dimensional echocardiography and additional scintigraphic views that visualize the posterior portions of the left ventricle improve noninvasive diagnosis of patients with resectable giant left ventricular aneurysms.

The echocardiographic assessment of cardiomyopathy: diagnosis, classification and problems.

Two-hundred-and-sixty-three patients with cardiomyopathy were studied by M-mode echocardiography. Measurements of left ventricular cavity size, wall thickness and myocardial contraction were used to classify cardiomyopathy into "congestive" (212 patients) and "hypertrophic" (50 patients) groups; the "hypertrophic" group was further divided into asymmetric septal hypertrophy (37) and symmetric (concentric) mural thickening (13). Using clinical and electrocardiographic information as well as echocardiographic data, the latter group could then be further classified into "concentric infiltrative cardiomyopathy" (9) and "concentric hypertrophic cardiomyopathy" (4). The former either presented with signs of restriction or were known to have systemic amyloidosis; the electrocardiograph showed low voltage and myocardial contraction was impaired in advanced cases. The latter had evidence of severe left ventricular hypertrophy and resembled asymmetric septal hypertrophy clinically. Problems encountered with the echocardiographic diagnosis of congestive cardiography were mainly concerned with proper clinical interpretation of the echocardiographic data whilst technical difficulties in recording the echocardiogram and in interpretation of tracings were a common problem in hypertrophic cardiomyopathy.

Monitoring cardiac function with nuclear techniques.

Noninvasive imaging with radioactive tracers has become widely used since its introduction in the early 1970s. Improvements continue to be made in the techniques and the clinical applications. Much of the information provided by these techniques is new. The first transit studies are used mainly in the evaluation of pulmonary transit time, detection of intracardiac shunting, evaluation of right ventricular function, measurement of ejection fraction and detection of wall motion abnormalities at rest and after exercise. The gated blood pool study is found to be most useful in assessment of global left ventricular function, regional wall motion, valve regurgitation and right ventricular function. The techniques of nuclear cardiac imaging are noninvasive, simple, successfully performed in almost 100 percent of cases. They are easy to interpret, able to be quantified and able to be almost totally automated. Their use is likely to become more widespread in the future.

Genetic influence on cadmium-induced hypertension.

Dahl hypertension-resistant (R) and hypertension-sensitive (S) rats were used to determine whether cadmium-induced hypertension is dependent on genetic predisposition. In experiment I, 16 wk-old R and S rats of both sexes were injected with two doses of cadmium (1 and 2 mg/kg body wt, ip), whereas the controls received the same volumes of saline. Hypertension and renal vascular changes were observed in cadmium-injected S rats but not in R rats. The S females appeared more sensitive than S males to the hypertensinogenic effect of cadmium. In experiment II, groups of weanling female R and S rats were given 0, 1, 2.5, 5, or 10 mg cadmium/liter drinking water and fed either a low-salt (0.4% NaCl) or a high-salt (4% NaCl) diet for 28 wk. Cadmium produced cardiac hypertrophy (1 mg cadmium/liter) and hypertension associated with renal vascular changes (1--5 mg cadmium/ liter), and it enhanced proteinuria (1-10 mg cadmium/liter) in S rats on a low-salt diet. Also, the development of salt-induced hypertension was accelerated in cadmium-fed (1 and 2.5 mg/liter) S rats. These adverse effects of cadmium were not detected in R rats on either salt diet. In experiments I and II, cadmium concentrations in the kidneys and liver of S rats were higher (P less than 0.001) than in those of R rats. These data indicate that genetic differences influence the pathogenesis of cadmium-induced hypertension.

Influence of dietary potassium and sodium/potassium molar ratios on the development of salt hypertension.

Among genetically hypertension-prone rats, dietary sodium (chloride) was demonstrably hypertensinogenic and potassium (chloride) antihypertensinogenic. On diets containing the same NaCl but different KCl concentrations, mean blood pressure was greater in rats receiving less dietary potassium, i.e., diets with a higher Na/K molar ratio. On diets with different absolute concentrations of NaCl and KCl, but the same Na/K molar ratios, rats on the higher absolute NaCl intakes had the higher blood pressures. On diets with different absolute concentrations of NaCl and KCl, and different Na/K molar ratios, a group on a lower absolute NaCl intake but with a higher Na/K ratio could have more hypertension than a group on a higher absolute NaCl intake but with a lower Na/K ratio. At equivalent molar ratios, the respective effects of these two ions on blood pressure were dominated by that of sodium. It was concluded that the dietary Na/K molar ratio can be an important determinant for the severity, or even development, of salt-induced hypertension. The mechanism of the moderating effect of potassium on sodium-induced hypertension was unclear.

Effects of chronic excess salt ingestion. Inheritance of hypertension in the rat.

TWO STRAINS OF RAT HAVE BEEN DEVELOPED BY SELECTIVE BREEDING: one strain (R rats) is resistant to salt hypertension, the other strain (S rats) is highly susceptible. The inheritance of these traits has been explored in the first (F(1)) and second (F(2)) generation of crossbred rats and in backcrosses between parent and first filial (F(1) x R, F(1) x S) generations. Male F(1) rats had an average blood pressure close to the mid-parental (R and S) values, and the average of F(2) males was equivalent to that of F(1). Male offspring of F(1) with R, or F(1) with S also showed averages close to the respective mid-parental values. Female offspring showed deviations from this linear relationship, indicating a significant dominance in the female for the genes of normal blood pressure. A model of two autosomal, nonlinked diallelic loci, with a dominance deviation at one locus in the female, gave predictions with a reasonable agreement to the observed values. The same model also appeared compatible with human data if we assume a gene frequency of 0.13 for the hypertensinogenic allele on both loci. Random fluctuations in blood pressure, and incomplete homogeneity of parental strains permit several alternative models. The major conclusions are: that more than one locus is needed to explain the findings though as few as two loci may possibly suffice; the allelic effect seems additive in males, but there is a sex-determined influence on the expression in females; there is no consistent evidence for sex-linked inheritance. Furthermore, this model developed from the study of rats may provide a framework for analysis of human data.

Genetic influence on the development of renoprival hypertension in parabiotic rats. Evidence that a humoral hypertensinogenic factor is produced in kidney tissue of hypertension-prone rats.

Rats from two strains with opposite constitutional predisposition to hypertension were joined in parabiosis and one partner was nephrectomized. The influence of genetic factors and of diet on the blood pressures of the two classes of parabionts, operated and intact, indicated that renoprival hypertension occurred with equal frequency in rats from both strains; that the development of renoprival hypertension depended on the influence from an intact S partner, or on a high salt intake, or on both. A nephrectomized S rat developing renoprival hypertension did not induce high blood pressure in its intact R partner. In this respect renoprival hypertension differs from salt and renal hypertension. The findings are interpreted to mean that the hypertensinogenic agent specific for S rats is produced by S kidneys.

Genetic influence on the development of renal hypertension in parabiotic rats. Evidence for a humoral factor.

The effects of several renal manipulations including uninephrectomy, unilateral renal artery constriction, and a combination of these two (Goldblatt procedure) were studied in two strains of rats with opposite constitutional predispositions to experimental hypertension. The protective value of intact renal tissue to protect against hypertension was shown to be genetically determined. The Goldblatt procedure carried out on only one member of a parabiotic pair induced hypertension in this operated rat but significant hypertension developed in the intact partner only when the operated animal belonged to the strain predisposed to hypertension. It was speculated that there were qualitative differences in the pressor signals of the two strains of rats. In the strain genetically predisposed to hypertension there are at least two pressor principles: (a) one which is common to both strains, not transmittable via the parabiosis junction and presumably related to the renin-angiotensin system; and (b) a second which is specific for the hypertension-prone strain and can be transmitted through the parabiosis junction. This transmittable agent is probably identical with the factor that produces salt hypertension and is associated with the salt-excreting mechanism.

Effects of chronic excess salt ingestion. Genetic influence on the development of salt hypertension in parabiotic rats: evidence for a humoral factor.

Parabiosis has been found to modify the expected blood pressure response of rats from two strains with opposite genetic propensities for experimental hypertension. When a member from one strain was united in parabiosis with a member from the other and both were maintained on high NaCl diet, the rat from the strain ordinarily resistant to it rapidly developed hypertension, in contrast to appropriate controls from this strain. The development of hypertension in this resistant animal preceded that in its mate from the strain highly sensitive to hypertension. In the latter, both the level of hypertension and mortality were significantly less than in its control. It seems likely that the hypertension observed is the resistant parabiont was initiated in its partner from the sensitive strain. This modification in blood pressures was not observed in the absence of a high NaCl diet. Parabiosis between animals from the same strain did not alter their response. Thus, as in earlier experiences (1-4) the interaction of a nongenetic factor (NaCl) with the appropriate genetic substrate appeared to be necessary for the development of hypertension. The findings are interpreted as evidence that a transmittable humoral influence plays an important role in the pathogenesis of rat hypertension. The presence of this agent is genetically determined but, under the conditions of these experiments, it took the added stimulus of dietary NaCl to demonstrate its existence.