Supravalvular aortic and peripheral pulmonary arterial stenoses. A report of eight cases in two generations.

Peripheral pulmonary arterial stenosis, either alone or in combination with supravalvular aortic stenosis, is described in two generations of one family. The last child born in the first generation ws the only fatal case and showed severe narrowing and thrombosis of the pulmonary arteries and significant narrowing of the descending aorta. Physical and mental development were normal in the seven surviving patients. Five had slight dyspnea on effort. Hemodynamic and angiocardiogrphic studies showed multiple peripheral pulmonary stenosis in six patients and supravalvular aortic stenosis or aortic hypoplasia in the last three of the first generation and in one of the second generation. The younger children were more severely affected. A marked systolodiastolic caliber variation of the main pulmonary arteries was noted angiographically in all those studied. We suggest that this finding can be used as an indirect sign of the presence of peripheral pulmonary arterial stenosis.

Exercise tests in patients with severe angina pectoris: an angiographic correlation.

Graded submaximal ergometric tests were peformed on 60 patients who suffered from clinically severe angina pectoris, and the results were correlated with their coronary angiograms. The test was positive in 44, negative in 9, and undetermined in 7 patients (defined as failure to reach the target heart rate without ischemic ST changes). Among patients with positive tests, 42 (95%) had obstruction of one to three coronary vessels. Among patients with negative tests, only 3 had significant coronary disease (sensitivity 93%). While all patients suffered clinically from severe "angina pectoris," 8 (15%) had insignificant CAD, and among them 6 had a negative and 2 a false-positive exercise response (specificity 75%). Although ST depression was a good indicator of CAD, its degree did not parallel the severity of the lesions. The peak heart rate on exercise of patients with ischemic ST changes was lower than their target heart rate, suggesting that the heart rate at which ST changes occur constitutes in itself a good indicator of severity. Among the 7 patients with undetermined tests, CAD was found in 6. In these patients the absence of ST changes may be ascribed to extensive myocardial fibrosis, and the only clue to CAD resides in the negative chronotropic response to exercise. Although exercise testing does not always distinguish between normal and CAD patients, it nevertheless constitutes a valuable noninvasive technique for the detection of the high-risk patients.

Holter monitoring in dizziness and syncope.

Holter monitoring was used to detect the underlying mechanism among 53 patients referred for dizziness, fainting and/or syncope. The complaints were unexplained on clinical grounds in 38, suggestive of SSS in 11, and of pacemaker dysfunction in 4 patients who underwent pacemaker implantation for symptomatic A-V block. Occult dysrhythmias were revealed in 24 of 38 (61%) of the first group; the clinical impression of SSS was confirmed in 8 of 11 (72%) in the second, and ineffective pacing confirmed in 2 of 4 in the third group. Thus, the diagnosis was clarified in 34 of 53 (64%) of patients. It is concluded that Holter monitoring is most useful for detecting the underlying mechanism in the above mentioned conditions, especially in elderly subjects whose syncopal attacks remained unexplained despite routine cardiological and neurological examination. Holter monitoring should be carried out for at least 36 hours before ruling out dysrhythmias as a cause of dizziness and/or syncopal attacks.

"Coronary" T waves in the presence of complete left bundle-branch block: a normal variant?

The case report of a patient with an ECG pattern of intermittent left bundle-branch block, presenting inverted and symmetric ("coronary") T waves in leads V1 to V3 during periods of normal intraventricular conduction, is presented. The patient was followed up for eight years during which time no relevant symptoms appeared while extensive noninvasive investigations repeatedly failed to reveal any organic basis for the ECG changes. It is stressed that inverted, symmetric T waves in right-sided chest leads may be encountered in young, otherwise healthy subjects with intermittent left bundle-branch block during periods of normal intraventricular conduction. Knowledge of this fact is important in order to avoid iatrogenic cardiac invalidism in such cases.

Arteriosclerotic aneurysm of the coronary artery.

The findings in a patient with an angiographically proven aneurysm of the coronary artery are described. The case is reviewed in the light of 115 similar cases reported in the literature. The patient had had numerous episodes of variant angina, a feature not previously described in coronary arterial aneurysms, which may be related to embolic showers originating from the aneurysm.

Chordal rupture: a common complication of myxomatous degeneration of the mitral valve.

In two patients with a clinical picture of acute mitral insufficiency, the presence of chordal rupture secondary to myxomatous degeneration of the mitral valve was disclosed during surgery. There was no evidence of previous rheumatic valvulitis, subacute bacterial endocarditis or other etiologies. It appears from the literature and from the cases described that ruptured chorda tendinea is a not uncommon complication of myxomatous transformation of the mitral valve. While this fact has been mentioned in anatomophological reports, clinicians are less aware of the association. In the absence of supportive evidence for a rheumatic or arteriosclerotic etiology, a clinical picture of acute mitral insufficiency should suggest ruptured chorda tendinea secondary to myxomatous degeneration of the mitral apparatus. This is particularly true in older patients, especially among those followed for a midsystolic click-late systolic murmur or an apical pansystolic murmur of pure mitral regurgitation.

Pulmonary hydatid embolization. Report on 2 operated cases and review of published reports.

Two patients with pulmonary hydatid embolization are described and commented upon in the light of 43 similar published cases. The diagnosis was strongly suspected from the medical history and the chest x-ray films and supported by angiocardiography. The angiocardiographic features of this condition have not been described previously in detail. They include amputation and filling defects of pulmonary artery branches, which are typically located proximal to a rounded tumour-like opacity seen on the plain x-ray film. Both patients underwent successful embolectomy.

Left ventricular aneurysm of unusual aetiology: report of two cases.

Two young patients aged 14 and 25 with left ventricular aneurysms are described. In both, a clinical picture initiated by attacks of supraventricular tachycardia led to the discovery of the condition. In the first patient the clinical picture clearly suggested a traumatic aetiology. In the second, the angiographic and histological findings strongly favoured a congenital origin. Both patients underwent successful aneurysmectomy. The literature on the incidence and aetiology of traumatic or congenital ventricular aneurysms is discussed.

Ebstein's anomaly of tricuspid valve: critical review of roentgenological features and additional angiographic signs.

The plain roentgenographic and angiocardiographic features of 15 patients with Ebstein's anomaly of the tricuspid valve were reviewed. Of note on the plain roentgenogram was the fact that the "posterior bulge" seen on the lateral view was demonstrated to be due to either a markedly enlarged right atrium or the posterior displacement of normal left heart structures. The angiocardiographic features described in the literature were reviewed. Two main features seen were the "sail-like" appearance of the anterior leaflet of the tricuspid valve, which sometimes produces a tumor like picture, and the 2 notches seen when the ventricle contracts. One notch corresponds to the true tricuspid annulus. The other represents the site of attachment of the displaced tricuspid valve. The previously undescribed features were noted: (a) all three leaflets could sometimes be identified; (b) fenestrations in the anterior leaflet were demonstrated in 5cases; and (c) the sinus portion of the ventricle was sometimes filled in a retrograde fashion from the infundibular chamber.

Wenckebach A-V block: a frequent feature following heavy physical training.

Among 126 top Israeli athletes, in whom an ECG was obtained during a random survey, 11 had first-degree heart block (P-R greater than or equal 0.21 sec.) and in three of them Wenckebach's phenomenon was found. The latter could be demonstrated only after 15 minutes' rest in a recumbent position and was abolished by sitting, standing, and the administration of atropine. This subjects with Wenckebach's phenomenon were followed for 6 years. The heart block was found to be present only during seasons of intensive training and could not be demonstrated a few weeks after the training was reduced in intensity or stopped. No heart disease or diminution of performance developed during 6 years of follow-up. Transient second-degree heart block in top athletes is probably much more frequent than hitherto suspected, but it can be demonstrated only if the athlete is examined during rest and in the recumbent position. It is assumed to be a physiological phenomenon related to heavy physical training.

Nonrheumatic mitral incompetence.

This review clearly indicates that a clinical picture of pure, isolated mitral insufficiency constitutes an interesting diagnostic challenge. In adult patients especially, this common valvular lesion is often nonrheumatic and may be found in a variety of cardiac conditions. The following provides a general orientation for their differential diagnosis. The first clue to the presence of papillary muscle dysfunction, a "spontaneous" chordal rupture, or a congenital or traumatic lesion which may account for the mitral insufficiency, may be derived from the patient's case history. A history suggesting systemic manifestations raises the possibility of atrial myxoma. When a familial incidence is reported, various syndromes or a cardiomyopathy should be considered as the etiology of the mitral incompetence. The auscultatory findings are typical in the mid-late systolic click and murmur syndrome, but recognition of this condition may require careful examination of the patient in different postures. The possibility of obstructive cardiomyopathy may be confirmed by the characteristic carotid pressure tracing. ECG findings of acute or chronic coronary heart disease favor the possibility of papillary muscle dysfunction. In addition, the ECG may support the clinical impression of a cardiomyopathy. Fluoroscopy may show calcification of the coronary arteries and/or dyskinetic left ventricular contractions in papillary muscle dysfunction, intracardiac calcifications in atrial space-occupying lesions, or calcification of a mitral annulus. Chest X-rays may contribute to the diagnosis of acute mitral insufficiency by showing a relatively small left atrium and ventricle in the presence of severe congestive failure. While echocardiography is invaluable as a noninvasive procedure and readily demonstrates the presence of a flail mitral leaflet from chordal rupture, or aids in the recognition of obstructive cardiomyopathy, an atrial space-occupying lesion, or of a billowing mitral leaflet, left ventriculography and coronary angiography constitute the procedure of choice for the fine anatomic diagnosis and functional evaluation of most cases. The accurate diagnosis of the anatomic disruption of the mitral valvular apparatus, as well as of the state of the myocardium and of the coronary arteries, is of particular importance in symptomatic patients, in order to determine the prognosis and to plan the surgical approach.