The location of the coronary sinus orifice in endocardial cushion defects

Forty-six human hearts with an ECD malformation were studied, particularly examining the anatomical location of the coronary sinus ostium. The sinus opened in the right atrium in 22 cases, and in the left side in 18 cases. Six cases showed no definite coronary sinus ostium.In most cases with a left-side ostium, the posteroinferior position of the atrial septum was absent. The failure of the union of the posteroinferior primum septum with the left atriovenous fold is considered to result in left-side coronary sinus orifices. The mean frontal electrical axis of ECG's showed no relationship with the location of the coronary sinus orifice.     

Two types of ventricular septal defect in corrected transposition of the great arteries: reference to surgical approaches

Six autopsied cases of corrected transposition of the great arteries with VSD were examined from the surgical exposure. Two anatomic types concerning the relative height of the two semilunar valves and the position of the VSD were recognized. In four cases (Type I), the two semilunar valves are at the same height viewed from the anteroposterior direction, the parietal band is hypoplastic, and the VSD is located immediately below the aortic and pulmonary valves. In the other two cases (Type II), the aortic valvular level is higher than the pulmonary valvular level and the parietal band in these cases is well-developed. The VSD located beneath the pulmonary valve is clearly separated from the aortic valve by a well-developed parietal band. The first type has an excellent exposure viewed from the left-sided ventricle, while the second has an extremely poor exposure. Therefore, right-sided ventriculotomy is recommended in the latter type. Furthermore, these two anatomic types can be recognized and differentiated in angiocardiographic findings by the relative height of the two semilunar valves and the presence of a filling defect formed by the crista supraventricularis.     

Evaluation of mitral regurgitation in endocardial cushion defect by selective left ventriculography with reference to surgical intervention on mitral valvular cleft

New criteria for the evaluation of mitral regurgitation associated with endocardial cushion defect using selective left ventriculography are presented. The severity of the regurgitation is classified into four groups, namely Grades I to IV, by comparing degrees of opacification of both atria and the pulmonary arterial trunk with that of the ascending aorta. Validity of this criteria is substantiated with clinical findings, and operative methods for the mitral cleft are discussed from the viewpoints of the degree of regurgitation and the postoperative follow-up results.Those with more than a Grade III regurgitation show left atrial enlargement in the majority of cases. Those with Grade IV regurgitation have increases in the amplitude of left atrial V-wave, while in patients with less than Grade III regurgitation, no definite relationship is found between the degree of regurgitation and left atrial pressure. The cardiothoracic ratio has a good correlation with an angiocardiographic classification of mitral regurgitation. On auscultation of those cases with Grade III or Grade IV regurgitation, a Grade $\text{3}\text{6}$ to $\text{4}\text{6}$ systolic regurgitant murmur is heard at the apex, whereas in patients with a Grade II or Grade I regurgitation, intensity of the murmur varies from Grade $\text{2}\text{6}$ to $\text{3}\text{6}$.This angiocardiographic classification of mitral regurgitation correlates well with the clinical severity of the patients' condition.Our policy of surgical intervention on the mitral cleft in endocardial cushion defect includes (1) simple closure of the primary defect without direct intervention on the valve, (2) a few interrupted stitches applied near the base of the cleft, (3) complete closure of the left cleft to the free margin of the leaflet, (4) pericardial patch application to close the gap, and (5) prosthetic valve replacement. Although marked clinical improvement is seen in patients with Grade I or Grade II regurgitation repaired with methods 1, 2, or 3, these methods when applied in patients with Grade III or Grade IV regurgitation generally end in less favorable clinical results such as hemolytic anemia or congestive heart failure with persistence of the regurgitation. One case of Grade IV regurgitation repaired with method 4 has a good clinical result.     

Relationship between myocardial infarction and preinfarction angina: a histopathological study of coronary arteries in two sudden death cases employing serial section

Two patients who had previously experienced old myocardial infarction and who died suddenly after an attack of chest pain were examined and discussed. In both cases two of the three main coronary arteries showed severe stenosis with canalization. Ruptured atheromatous plaque was found in the unblocked coronary artery. Fibrin was already formed and surrounded the fractured intimal collagen fiber, foam cells, and cholesterin clefts, but a luminal thrombi had not yet been formed. Fresh occluding thrombi were formed at the site of the ruptured atheromatous plaque. Coronary thrombi containing abscess components such as foam cells, cholesterin clefts, and the fractured intimal collagen fiber were found in our preliminary study. These views support the supposition that this fracture between the lumen and the plaque might precede and be responsible for the formation of the thrombus and the onset of acute myocardial infarction. It was confirmed that the attack of preinfarction angina occurred at the time of the rupture of the atheromatous plaque. The rupture of the atheromatous plaque plays an important part as an initiating factor of peinfarction angina and myocardial infarction. Thus, it is necessary to examine coronary arteries by serial histopathological section method.     

Systematic visualization of coronary arteries by two-dimensional echocardiography in children and infants: evaluation in Kawasaki's disease and coronary arteriovenous fistulas

We describe in our report a systematic approach for visualizing in detail coronary artery anatomy by two-dimensional echocardiography (2DE). The approach provides longitudinal and transverse images of the proximal coronaries, as well as transverse images of the anterior descending and right coronary artery tree. The method is delineated by showing certain cases of Kawasaki's disease (MCLS) and coronary arterial fistula. Using these multiple echo planes, the diagnostic accuracy was improved for both right and left coronary arteries.     

Ventricular septal defect with aortic insufficiency. Angiocardiographic aspects and a new classification

Based on 91 operated cases of VSD with AI and 67 biplane aortographies taken before surgical treatment, we have made a classification of this syndrome and have described our opinions on the aortographic aspects.Type 1 is subpulmonary VSD, which is further divided into subtypes a and b. Type 2 is infracristal VSD which is divided into subtypes a, b, and c. These two types, which are distinguished according to the position of the VSD, have two forms found in the lateral aortogram, as follows: (1) protruding aortic valve and sinus, and (2) nonprotruding aortic valve and sinus. Protruding AI is easily observed in aortography, and the protruding part frequently coincides with the position of the VSD as ascertained by operation. As to nonprotruding AI, however, aortography does not demonstrate the position of the deformed valve and the VSD, but can show only the grade of aortic regurgitation.Infundibular stenosis often accompanies this syndrome. This complication occurs mainly with Type 2 VSD with AI, but also occurs with Type 1 in a small number of cases. Of the cases associated with infundibular stenosis, AI is caused by the protrusion of the aortic valve and the sinus in some cases and by different factors in other cases.     

Left ventricular performance during exercise in patients with left bundle branch block: evaluation by gated radionuclide ventriculography

To investigate changes in left ventricular (LV) function during exercise in patients with left bundle branch block (LBBB), 22 patients without a history or physical findings of previous myocardial infarction or LV dysfunction were studied by gated radionuclide ventriculography (GRNV) at rest and during bicycle exercise. Coronary arteriography demonstrated greater than 75% diameter narrowing of at least one coronary artery in nine patients. Of the remaining 13 patients, GRNV demonstrated wall motion abnormalities in seven patients either at rest or with exercise. During exercise, mean ejection fraction (EF) did not increase in patients without coronary artery disease (CAD). Patients with CAD had a 12-point fall in mean EF with exercise. We conclude that LV reserve, as demonstrated by ability to increase EF with exercise, is impaired in patients with LBBB even in the absence of CAD or other underlying cardiac disease and that standard GRNV criteria to exclude the presence of CAD (a greater than five-point increase in EF with exercise and normal wall motion) are not strictly applicable in screening patients with LBBB.     

M-mode and two-dimensional echocardiographic correlation with findings at catheterization and surgery in patients with mitral stenosis

Thirty-two consecutive patients referred to our institution for evaluation of rheumatic mitral stenosis were studied with M-mode echocardiography (M-mode E), two dimensional echocardiography (2DE), and cardiac catheterization. Twenty-three of these patients underwent mitral valve surgery, 11 requiring mitral valve replacement, and 12 requiring open mitral commissurotomy. Clinical and noninvasive parameters were assessed in order to predict catheterization-determined mitral valve areas as calculated by the Gorlin formula, and to predict the choice of operation in patients selected for surgery. For the prediction of valvular area, 2DE planimetry correlated highly (r = 0.89, p less than 0.01) with Gorlin formula results. The presence or absence of pericardial effusion, the anterior-posterior valve leaflet separation (M-mode E), and the left atrium-aortic index (2DE) correlated poorly with the degree of mitral stenosis as determined by the Gorlin formula. The most useful predictors of type of mitral surgery were age over 50 years, 2DE valve classification, the presence or absence of calcium at fluoroscopy, and degree of anterior leaflet-septal separation (M-mode E).     

Early systolic notch in the apexcardiogram in mitral stenosis

An early systolic notch on the upstroke of the apexcardiogram (ACG) has previously been reported to occur in mobile left atrial myxoma but has not been noted in valvular mitral stenosis. It has therefore been considered a useful diagnostic sign for myxoma. We report four patients with mitral valve stenosis who had an early systolic notch in the ACG. Myxoma was excluded by catheterization and/or surgery. We feel that the notch is not specific for myxoma and that its finding should be interpreted with caution.     

QT interval prolongation and increased plasma catecholamine levels in patients with mitral valve prolapse

The heart rate corrected QT interval (QTc) and plasma catecholamine (CA) and norepinephrine (NE) levels were measured in 15 symptomatic patients with idiopathic mitral valve prolapse (MVP) and in 19 control subjects. MVP patients showed longer mean QTc and were divided into two groups: group A normal QTc (greater than 440 msec) and group B prolonged QTc (less than 440 msec). In supine resting conditions CA levels were as follows: group A 0.420 +/- 0.035 ng/ml and group B 0.619 +/- 0.104 ng/ml (p less than 0.05); both were greater than control values (0.348 +/- 0.017 ng/ml, p less than 0.005). NE levels were as follows: group A 0.350 +/- 0.031 ng/ml and group B 0.376 +/- 0.052 ng/ml (NS); both were greater than control values (0.242 +/- 0.025 ng/ml, (p less than 0.05). When a standing position was assumed, CA and NE levels increased significantly in all groups but this was most marked in group B as compared to control levels (CA: 1.039 +/- 0.123 ng/ml versus 0.625 +/- 0.037 ng/ml; NE: 0.737 +/- 0.076 ng/ml versus 0.504 +/- 0.031 ng/ml) (p less than 0.001 and p less than 0.05, respectively). Thus the longest QTc was observed in patients with MVP who had the highest levels of CA and NE, in both supine and standing positions. These data may account, in part, for the occurrence of severe ventricular arrhythmias in some patients with MVP and may offer a rationale for adrenergic blockade in that subset of patients with MVP and markedly prolonged QTc.     

Maximal exercise in children with aortic regurgitation: an adjunct to noninvasive assessment of disease severity

Twenty-five asymptomatic children with chronic aortic regurgitation were evaluated by graded bicycle exercise testing and standard resting M-mode echocardiogram. These results were compared to those of 35 normal controls matched for age and body surface area. Twenty-one patients underwent cardiac catheterization to rule out associated lesions. Patients fell into two groups based upon the left ventricular end-diastolic volume per body surface area (LVEDVI): group I (n = 10) had LVEDVI less than or equal to 2 SD from the mean of normal; group II (n = 15) had LVEDVI greater than or equal to 4 SD from the mean of normal. All had normal shortening fraction and velocity of circumferential fiber shortening. At maximal exercise, patients in group I were found to have no significant differences from normals in maximal workload, total work, percent maximal oxygen consumption, heart rate, blood pressure, or ST segment depression. However, patients in group II had blunted mean maximal exercise heart rate (p less than 0.001), systolic hypertension (p less than 0.05), and increased frequency and maximal amplitude of ST depression (p less than 0.01, p less than 0.001, respectively) compared to normal controls. Within group II the mean maximal amplitude of ST depression was significantly related to increasing LVEDVI (r = 0.53, p less than 0.05). The mean maximal exercise heart rate, systolic blood pressure, and maximal amplitude of ST segment depression were significantly related to LVEDVI for patient groups and normal controls together (r = -0.384, p less than 0.01; r = 0.28, p less than 0.05; r = 0.70, p less than 0.001, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)     

Significance of ST-segment and T wave changes in the resting electrocardiograms of patients with exertional angina, studied by exercise radionuclide angiocardiograms

In order to investigate the clinical significance of ST-T changes in resting ECG in angina pectoris, symptom-limited ergometer exercise radionuclide angiocardiography with ECG was performed in 60 patients with exertional angina. In those with normal ECG at rest (Group N), left ventricular ejection fraction (EF) did not change during exercise (71 +/- 5% to 71 +/- 6%). In those with only ST change at rest (Group ST) and those with only T change at rest (Group T), EF decreased significantly during exercise (68 +/- 5% to 63 +/- 7%, p less than 0.01; 68 +/- 6% to 61 +/- 7%, p less than 0.001). In those with ST and T changes at rest (Group ST + T), EF was low at rest (58 +/- 11%) and decreased further at exercise (52 +/- 8%, p less than 0.001). In those whose negative or flat T wave became more negative during exercise, EF was low both at rest and at exercise. In conclusion, ST and T changes at rest in patients with exertional angina might suggest a depressed reserve of myocardial function for exercise.     

Severe impairment in adenine metabolism with a partial deficiency of adenine phosphoribosyltransferase

Among three unrelated patients with recurrent 2,8-dihydroxyadenine urolithiasis, two completely lacked adenine phosphoribosyltransferase (APRT) in both erythrocytes and proliferative T cells. The third patient possessed significant enzyme activities in both hemolysates and T-cell extracts at levels comparable to heterozygotes for complete APRT deficiency. Despite significant APRT activities in cell extracts, cultured T cells from the third patient were at least 100-fold more resistant than normal T cells to an adenine analog, 6-methylpurine, whose cytotoxicity is dependent on APRT. These data indicate that APRT activity in T cells from the third patient is positive in cell extracts, but apparently not operating in viable cells. Although the cells from the patients with complete APRT deficiency were as resistant to 6-methylpurine as the cells from the third patient, the cells from the heterozygotes for complete APRT deficiency were almost as sensitive as normal T cells. Therefore, adenine metabolism in the third patient but not in the heterozygotes seems to be as severely impaired as in the patients with complete APRT deficiency, which is quite consistent with the clinical manifestations in these individuals.     

P wave changes during exercise in normal subjects and in patients with hemodynamic evidence of left atrial overload

P wave characteristics at rest and during exercise in normal subjects and patients with hemodynamic evidence of left atrial overload were compared. Three groups were studied, including 48 normal subjects, 15 patients with mitral stenosis, and 24 patients with left ventricular dysfunction. Frank lead electrocardiograms were recorded on magnetic tape at rest sitting and during upright bicycle exercise. Digital computer techniques were used to derive P wave area vectors. The initial 3/8 of the P wave were regarded as representing primarily right atrial, and the terminal 5/8 primarily left atrial activity.The P wave response to exercise in normal subjects include a small but significant (p<0.005) increase of the vertical component of the initial or right atrial vector and a significant (p<0.001) increase of the posterior terminal P vector component from 0.5 to 1.1 μV-sec.Analysis of area vectors in the abnormal groups at rest increased the recognition rate of left atrial overload over that obtained with standard 12-lead criteria, from 38 to 82% (P<0.005) at a specificity of 92%. The recognition rate during exercise was 67%, slightly lower than that at rest, due to increased inter-individual variation and exercise-induced changes in the normal group in the direction of the left atrial overload.     

Progression of coronary artery disease in patients with chest pain and normal or intraluminal disease on arteriography

The incidence of progression of coronary artery disease (CAD) in symptomatic patients was determined from serial coronary arteriograms in 27 patients with normal coronary arteries on initial study, in 17 patients with intraluminal disease (narrowings less than or equal to 20%) (ILD), and in 125 patients with occlusive disease (CAD) on the first arteriogram. Interval between arteriograms ranged from 9 months to 13 years. The normal patients progressed less frequently (14.8%) than did either the ILD patients (58.8%) or CAD patients (60%) (p less than 0.001). The incidence of progression was the same in those with ILD and CAD. These data suggest that patients with normal coronaries rarely progress to CAD and then only rarely in less than 4 years. However, ILD is a serious finding on arteriography and progresses to occlusive disease frequently in symptomatic patients. Repeat coronary arteriography is warranted at short intervals in symptomatic patients with ILD.     

Attempts to reduce arterial thrombosis after cardiac catheterization in children: use of percutaneous technique and aspirin

In an attempt to determine if percutaneous arterial catheterization rather than open arteriotomy or the use of an antiplatelet agent, aspirin, would reduce the incidence of arterial thrombosis after cardiac catheterization, 95 children were studied. After measuring the amplitude of pulsations by oscillometry on the day of admission, the children were randomly divided into two groups. One received aspirin 15 mg. per kilogram of body weight per dose for 5 doses and the other served as a control. Method of arteriotomy—percutaneous or open surgical incision—was left to the discretion of the catheterizer. Repeat oscillometric measurements were obtained before discharge.Percutaneous catheterization was associated with a significantly fewer number of diminished pulses (p = < 0.001). This effect was most significant in the older children. No significant effects on the number of diminished pulses were noted with the use of aspirin.