Kent Ellis

Myocardial blood flow in congestive and hypertrophic cardiomyopathy: relationship to peak wall stress and mean velocity of circumferential fiber shortening.

SUMMARY Myocardial blood flow/unit mass (MBF) and the determinants of myocardial oxygen consumption were measured in seven control subjects (group I) and 15 patients (pts) with cardiomyopathy (CM), group II (group Ila-congestive CM: 10 pts; group Ilb-hypertrophic CM: 5 pts). In group I left ventricular (LV) MBF was 64±8 (SD) ml/10g - min; it was significantly lower in Ila (45 ± 15 ml/100g. min, P < 0.01) and IIb (39 ± 7 ml/100g min, P < 0.01). However, calculated total LV flow (LV mass X MBF) was increased in the two CM groups. In nine CM pts, LV MBF increased in response to atrial pacing from 41±7 to 63±13 ml/100g * min.In group Ila, calculated peak wall stress was normal (4.39±0.77 dynes/cm2 × 106) but mean velocity of circumferential fiber shortening (MVcf) was significantly reduced (0.53±0.18 vs 1.26±0.12 circum/ sec, P < 0.01). In llb, MVcf was normal but peak stress was significantly reduced (2.80±0.75 vs 4.51±1.10 dynes/cm2 × 106, P < 0.05). Multiple regression analysis based on all pts yielded, MBF = 16.9 MVcf + 9.30 Stress + 0.26 Heart Rate − 26.4, (r = 0.79). The data indicate that MBF is reduced in CM patients and the regression analysis suggests that MBF in these 22 pts with normal coronary arteriograms was determined largely by heart rate, peak stress, and ventricular performance.

Spontaneous complete closure of a congenital coronary artery fistula

The first documentation is reported of spontaneous closure of a coronary artery to right ventricle fistula that was demonstrated initially in a 14 month old boy. Over a 4 year period after diagnosis, the characteristic continuous murmur gradually disappeared. When the patient was 5 1/2 years of age, selective coronary arteriography showed normal coronary vessels and circulation. Six other cases of coronary fistula observed during the past 10 years are also reviewed. This study supports the rationale for clinical follow-up rather than obligatory surgical intervention in asymptomatic patients with a small shunt who have no evidence of myocardial dysfunction.

Reduced left ventricular myocardial blood flow per unit mass in aortic stenosis.

SUMMARYMyocardial blood flow (MBF) per unit mass was measured in 10 patients (pts) with severe aortic stenosis (AS) and no significant aortic Insufficiency, normal ejection fractions, and normal coronary arteriograms, using xenon-133 and a multiple crystal scintillation camera. MBF per unit mass was reduced in AS (53 i 13 ml/lOOg - min) in comparison to a group of seven normal control patients (69 ± 12 ml/lOOg - min) (P < 0.05). When normalized for heart rate, MBF remained depressed in aortic stenosis (0.65 i 0.11 ml/ lOOg - beat). MBF/beat was strongly related to peak left ventricular wall stress in both groups (r- 0.97). Individual values of MBF/beat were normalized for peak stress using an analysis of covariance; the adjusted mean values were 0.62 ± 0.03 mllOOg - beat for the AS patients and 0.84 ± 0.03 ml/lOOg- beat for the control patients. There was no overlap between groups in adjusted MBF per beat. Values of MBF per beat and peak stress for a group of ten cardiomyopathy patients with depressed contractility were observed to fall close to the regression line for AS patients. The results suggest that variability in resting MBF in these AS patients is due primarily to differences in LV stress and that reduction in MBF per beat in this group may be due to reduced contractility.

Muscular ventricular septal defects repaired with left ventriculotomy

Between 1974 and 1979 nine patients, aged 10 months to 4 years, underwent left ventriculotomy for closure of single or multiple defects in the muscular ventricular septum. The vertical incision paralleled the anterior descending branch of the left coronary artery near the apex of the left ventricle and ranged from 2.5 to 3.5 cm in length. Four patients also had a right ventriculotomy with closure of a high perimembranous ventricular defect in two. Serial electrocardiograms indicated changes of myocardial ischemia or necrosis. Left bundle branch block did not develop in any patient. Three patients died in the early postoperative period. The six surviving patients are living and well 2 to 7 years later. There is apparent complete closure of the ventricular defects, which was documented by cardiac catheterization in four cases. Two patients had cardiomegaly and left ventricular dysfunction as assessed with echocardiographic and angiographic study, whereas four displayed good cardiac function. In three of the latter patients, cardioplegia or deep hypothermia techniques were utilized intraoperatively. The observations indicate that left ventriculotomy of limited size is an acceptable approach to the difficult problem of repair of muscular ventricular defects but may involve some risk of compromise of the coronary circulation.

Congenitally corrected transposition of the great vessels.

Congenitally corrected transposition of the great vessels is an important basic anomaly of the heart which has only recently received wide clinical attention. First described by von Rokitansky in 1875 (33), the anomaly remained a clinical curiosity until modern surgical advances in the treatment of congenital heart disease made accurate diagnosis important. Less than 150 cases are described in the literature, but the condition cannot be extremely rare, since we have encountered more than 30 cases, and similar large series have been reported from other institutions (17, 27). Among our cases is the first example of congenitally corrected transposition without associated anomalies diagnosed on the basis of chest films, the second such case to be recognized in life. Basic Anatomy of Corrected Transposition of the Great Vessels The aorta and pulmonary artery are transposed so that the aorta arises on the left and ventrad, in the usual position of the pulmonary artery, while that vessel arises on the right and ...

Pulmonary atresia and intact ventricular septum complicating corrected transposition of the great vessels

Abstract A three and one-half month old infant with corrected transposition of the great vessels, pulmonary atresia, and intact ventricular septum is presented. Cardiac catheterization and angiocardiography disclosed a small blind right-sided pulmonary (anatomic left) ventricle with systolic pressure exceeding systemic levels. The chamber communicates with the coronary arterial system via transmyocardial sinusoids. To our knowledge, this is the first report of this syndrome complex diagnosed during life.

VENTRICULAR SEPTAL DEFECT WITH SHUNT FROM LEFT VENTRICLE TO RIGHT ATRIUM BACTERIAL ENDOCARDITIS AS A COMPLICATION

During a two-year period we have seen six patients with malformations of the ventricular septum resulting in the shunting of blood between the left ventricle and the right atrium. Surgical closure of the defect has been successful in all. A history of bacterial endocarditis was established in two patients and strongly suspected in two others before operation. The surprisingly large number of patients with a malformation previously considered rare and the unusually high incidence of endocarditis as a complication prompted a review of the anatomical and clinical features of this anomaly. There have been 26 patients reported with left ventricular to right atrial shuntst on whom relatively complete clinical studies are available (Perry, Burchell, and Edwards, 1949; Stahlman et al., 1955; Kirby, Johnson, and Zinsser, 1957; Ferencz, 1957; Lynch et al., 1958; Gerbode et al., 1958; Kjellberg et al., 1959; Braunwald and Morrow, 1960; Levy and Lillehei, 1962). Symptoms were directly related to the amount of left-to-right shunting of blood with pulmonary vascular engorgement. These consisted of poor feeding in infancy and easy fatigue; tachypncea, pneumonia, and congestive heart failure were often noted. The usual findings on examination were a precordial thrill and loud systolic murmur along the lower left sternal border; a mid-diastolic apical rumble was heard in some patients. Chest radiography generally demonstrated cardiomegaly, pulmonary vascular engorgement, and in 15 of the 26 patients specific enlargement of the right atrium. The electrocardiogram was not diagnostic; an abnormal P wave was present in a few of the tracings. Except for one patient with a mean electrical axis of -90°, there were no reports of the axis being to the left or superiorly oriented. Cardiac catheterization demonstrated a left-to-right shunt at the level of the right atrium in all but 2 patients. The right atrial pressure was raised in 5 of the 9 patients in whom it was recorded. Descriptions of the cardiac defects at operation or necropsy are available in 24 patients: in 6 there was a defect high in the membranous ventricular septum with an opening in the floor of the right atrium just above the insertion of the septal leaflet of the tricuspid valve. One of these also had an abnormal mitral valve with obstruction to the left ventricular outflow tract (Ferencz, 1957). In the remaining patients a defect in the membranous ventricular septum occurred below the level of the tricuspid ring and was associated with a defect in the tricuspid valve. In 11 of these it appears that

Postoperative evaluation of mitral valve function in ostium primum defect with cleft mitral valve (partial form of atrioventricular canal).

Postoperative cardiovascular studies were carried out in eight patients with ostium primum defect and cleft mitral valve. Apical systolic murmurs were present in all patients, but in seven of them no mitral regurgitation was detected by pulmonary wedge pressure and left ventricular angiography. Surgical repair of the cleft anterior leaflet without resection of subjacent chordae tendineae, as performed in these patients, usually results in a competent mitral valve. Postoperative angiograms showed persistence of the characteristic preoperative deformity of the medial border of the left ventricular outflow tract especially in systole. As illustrated in one of these patients, subaortic obstruction occasionally complicates abnormalities of the atrioventricular valves.

Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms.

SUMMARY The clinical, hemodynamic, and angiographic observations, as well as the surgical approach used for repair in three patients with congenitally corrected transposition of the great arteries and ventricular membranous septal aneurysms, are presented. In two of the three patients the membranous septal aneurysm caused subpulmonary obstruction, with 94 and 125 mm Hg systolic gradients. In each patient the aneurysm was demonstrated by angiocardiography, which also showed differences in size and shape with cardiac systole and diastole.Review of the previously described reports indicates that patients with congenitally corrected transposition often display various forms of pulmonary outflow obstruction and when a ventricular membranous septal aneurysm exists, a significant subpulmonary obstruction is present in most patients. The unique anatomic relationship between the pulmonary artery and a ventricular membranous septal aneurysm in patients with transposition of the great arteries with and without atrioventricular discordance explains why subpulmonary obstruction sometimes develops.

Ventricular septal defect: Survival in adult life

Abstract The clinical course of thirty-five patients, seventeen years of age and over, with ventricular septal defect observed during the past thirty years is reviewed. Thirty-one patients were studied by right heart catheterization since 1946; ten were recatheterized five to thirteen years after the initial investigation. The remaining four were examined at necropsy before cardiac catheterization technics were available. A wide spectrum of hemodynamic findings was noted at all ages in adult life. The two largest groups were those with normal pulmonary artery pressure (group i) and those with pulmonary artery pressure at approximately the systemic level (group iv). In the first group the patients were essentially asymptomatic and showed no change in pulmonary artery pressure with advancing age. In contrast, most of the patients with severe pulmonary hypertension were symptomatic in infancy or early childhood and probably always had elevated pulmonary artery pressure. They manifested progressive pulmonary vascular disease with the development of cyanosis and ultimately right-sided heart failure. Bacterial endocarditis was the cause of death in three of the four patients examined at necropsy before 1946. This complication was infrequent among the patients in recent years and was never a terminal event. In this series only five patients were forty years of age or over, the oldest was forty-nine years of age. The explanation for lack of patients beyond fifty years is not clear, but shortened survival time is suggested.