Relation of angina pectoris to coronary artery disease in aortic valve stenosis.

One hundred three patients with isolated, severe aortic stenosis (AS) were retrospectively analyzed to determine the relation of angina pectoris to angiographically significant coronary artery disease (CAD). All patients underwent coronary angiography regardless of the presence or absence of angina. Angina was significantly associated with CAD (p less than 0.002), with a sensitivity of 78% and a specificity of 53%. However, 25% of the patients without angina had angiographically significant CAD, and in these patients there was a 70% prevalence of 1-vessel disease. Patients with isolated, severe AS should undergo coronary angiography to identify coexistent CAD accurately. The absence of angina does not reliably exclude angiographically significant CAD.

Idiopathic hypertrophic subaortic stenosis: evaluation of anginal symptoms with thallium-201 myocardial imaging.

The evaluation of angina pectoris in patients with idiopathic hypertrophic subaortic stenosis is difficult in those in the age group prone to coronary artery disease. Ten patients with angina pectoris, normal coronary angiograms and idiopathic hypertrophic subaortic stenosis were studied with thallium-201 myocardial imaging performed in conjunction with submaximal treadmill exercise testing. The resting electrocardiogram demonstrated left ventricular hypertrophy with S-T segment abnormalities in seven patients, thereby vitiating the further increase in S-T segment abnormalities that developed in these patients during exercise or in the postexercise period. Of the three patients with a normal resting electrocardiogram, one had significant exercise-induced S-T segment depression. Thallium-201 myocardial imaging revealed no significant perfusion defects in 9 of the 10 patients (90 percent). In one patient with severe left ventricular hypertrophy significant perfusion defects developed after exercise that were not present at rest. Stress thallium-201 myocardial perfusion imaging is a useful noninvasive technique that assists in ruling out the presence of significant coronary artery disease in patients with idiopathic hypertrophic subaortic stenosis.

Frequency of angiographically significant coronary arterial narrowing in mitral stenosis.

Ninety-six consecutive patients older than 40 years with severe mitral stenosis were retrospectively analyzed to determine the relation of angina pectoris (AP) and coexistent coronary artery disease (CAD). Of the 96 patients, 27 (28%) had angiographically significant CAD, 10 (37%) with AP and 17 (63%) without AP. Of the 96 patients, 21 had AP, 10 (48%) with angiographically significant CAD and 11 (52%) without (CAD). Of 75 patients without AP, 17 (23%) had angiographically significant CAD AP had a specificity of 84% and a sensitivity of 37% in its ability to detect significant CAD. The pulmonary artery systolic, diastolic and mean pressures and the pulmonary vascular resistance did not differ between patients with and those without AP (p greater than 0.05). It is concluded that coexistent CAD is commonly found in patients older than age 40 with severe MS, and is usually clinically silent.

Angina pectoris and coronary artery disease in isolated, severe aortic regurgitation.

Seventy-eight patients with isolated, severe aortic regurgitation (AR) were studied retrospectively to determine the prevalence of angiographically significant coronary artery disease (CAD) and its relation to angina pectoris (AP). Angiographically, significant CAD was present in 29 of 78 patients (37%), and 36 patients (46%) had AP. Twenty-one of 36 patients (58%) with AP and 8 of 42 patients (19%) without AP had angiographically significant CAD. AP as a predictor of significant CAD had a sensitivity of 73%, specificity of 69% and a risk ratio of 3:1. The predictive accuracy of detecting CAD in the absence of AP was 81%. The benefit from concomitant coronary artery bypass grafting at the time of aortic valve replacement for AR has not been clearly demonstrated; therefore, routine coronary angiography is still recommended for all AR patients older than 40 years undergoing aortic valve replacement.

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome.

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.

Clinical and echocardiographic features of isolated severe pure mitral regurgitation.

Recent studies have shown that rheumatic heart disease is no longer the main cause of isolated severe pure mitral regurgitation. In this study, we evaluated various clinical and echocardiographic features found in the syndrome of mitral regurgitation. Our data is consistent with recent reports that mitral valve prolapse and coronary artery disease are now the predominant causes of mitral regurgitation and that rheumatic heart disease is a much less common etiology. In addition, our data suggest that clinical evaluation alone is usually very accurate in identifying the correct etiology. Various clinical and echocardiographic features found in the subsets of acute and chronic mitral regurgitation are described. Specifically, patients with acute mitral regurgitation were more likely to have echocardiographic evidence of segmental left ventricular dysfunction and flail mitral valve leaflet. In chronic mitral regurgitation, atrial fibrillation and left atrial dilatation were more commonly present. Echocardiography was found to be more useful in the detection of the complications of coronary artery disease rather than in identifying its presence. Patients with a New York Heart Association classification of IV and those with echocardiographic evidence of an increased left ventricular endsystolic dimension or left ventricular hypertrophy had a worse prognosis.

Heritable anomalies among the inhabitants of regions of normal and high background radiation in Kerala: results of a cohort study, 1988-1994.

In a genetic epidemiological and fertility survey among 70,000 inhabitants in a high-background radiation region (HBRR) and normal radiation region (NRR) in Kerala, India, 985 persons were found to have heritable anomalies. Suggested etiologies for the anomalies were chromosomal and Mendelian, 15 percent; multifactorial, 60 percent; and congenital, 25 percent. There was a statistically significant increase of Down syndrome, autosomal dominant anomalies, and multifactorial diseases and an insignificant increase of autosomal recessive and X-linked recessive anomalies in the HBRR. The total fertility rate was 3.85 per couple; 9 percent of live-born children were reported dead. The rate of untoward pregnancy outcome--death of the offspring or presence of an anomaly in a living child--was 6.4 percent among the unrelated couples in the NRR, with one spouse born outside the area of current residence ("migrant"). Considering this as the base, the excess relative risks in the other groups are: "NRR-nonmigrant," 35 percent; "HBRR-nonmigrant," 69 percent; "NRR-consanguineous," 76 percent; and "NBRR-consanguineous," 157 percent. Ionizing radiation, consanguinity, and nearness of birthplace of the spouse are risk factors for the death of offspring and for anomalies. The higher risk among the "nonmigrant" couples may be due to geographic inbreeding. The findings are suggestive of an autosomal recessive etiology for the majority of the multifactorial anomalies.

Submaximal treadmill exercise testing of patients with coronary artery disease.

The submaximal treadmill exercise test is a valuable noninvasive tool for the diagnosis of overt or latent coronary artery disease (CAD). When submaximal heart rates of 80% to 90% of the predicted maximal rates are attained and when ST-segment depression of at least 1 mm is taken as a criterion of ischemia, testing by any of the various exercise protocols with continuous ECG monitoring affords reasonable specificity and sensitivity. The objectives of testing are to (1) diagnose and determine the severity of CAD, (2) assess functional capacity, (3) observe the natural history of disease, (4) evaluate the effects of medical and surgical treatment, and (5) evaluate responses to physical conditioning or to programs directed toward prevention of CAD. Proper precautions and safety standards minimize the risk of exercise testing.

Left atrial size, pressure, and V wave height in patients with isolated, severe, pure mitral regurgitation.

In recent years, some concepts regarding the etiology and hemodynamics present in the syndrome of mitral regurgitation have changed. Coronary artery disease and mitral valve prolapse have replaced rheumatic heart disease as the most frequent cause of mitral regurgitation. Hemodynamic studies have shown that tall V waves in the pulmonary capillary wedge tracings are neither specific nor sensitive in detecting the presence of mitral regurgitation. In this study, we evaluated the role of various clinical, echocardiographic, and hemodynamic findings with regard to left atrial (LA) size, pressure, and V wave height. We found that the mean pulmonary capillary wedge pressure (PCW) and V wave height for the subset of patients with acute mitral regurgitation (PCW = 24.1 +/- 10.9; V = 41.2 +/- 20.7 mm Hg) was similar to the subset with chronic mitral regurgitation (PCW = 17.9 +/- 7.5; V = 32.0 +/- 18.2 mm Hg). In addition, we found that there was a significant logarithmic relationship between the LA size and the duration of the mitral regurgitation (y = 1.404 [log X] + 3.948; R = 0.678; p less than 0.0005). Lastly, we found that LA size, compliance, regurgitant volume, and regurgitant valve orifice area all increase with time.

Familial occurrence of sinus bradycardia, short PR interval, intraventricular conduction defects, recurrent supraventricular tachycardia, and cardiomegaly.

Four members of a family presenting with sinus bradycardia, a short P-R interval, intraventricular conduction defects, recurrent supraventricular tachycardia (SVT), syncope, and cardiomegaly had His bundle studies and were found to have markedly shortened A-H intervals (30 to 55 msec.) with normal H-V times (35 to 50 msec.). Right atrial pacing at rates as high as 170 to 215 per minute failed to increase the A-H or H-V intervals significantly. The data are compatible with the presence of an A-V nodal bypass tract (James bundle) or even complete absence of an A-V node. Ventricular pacing and spontaneous ventricular premature beats resulted in a short ventriculoatrial conduction time (110 msec.) suggesting that if A-V nodal bypass tracts exist, they are utilized in an antegrade and retrograde fashion. None of the features of WPW syndrome was present. The mechanism of syncope in the mother and daughter was intermittent third-degree heart block. Both went on to develop permanent complete heart block despite electrophysiologic studies demonstrating 1:1 A-V conduction at extremely rapid atrial pacing rates and both required implantation of permanent pacemakers. The mechanism of syncope in the two brothers was possibly marked sinus bradycardia, but transient complete heart block has not been ruled out. Permanent pacemaker therapy was recommended for both. The nature of the cardiomegaly, which was mild in three patients, is not known. Although not well documented, several maternal relatives have had enlarged hearts, SVT, complete heart block, and syncope.