The clinical course of primary myocardial disease in infants and children.

A total of 161 infants and children, ranging in age from 1 day to 17 years at initial encounter (mean, 3.7 years), was seen over a 30 year period with primary myocardial disease (idiopathic myocarditis, nonobstructive cardiomyopathy, endocardial fibroelastosis, and an anatomically unknown category). These patients were observed from 1 hour to 23 years after initial encounter and cardiac disease has resolved in 27 per cent, resulted in death in 35 per cent, and continues in 38 per cent. The majority were first referred to us with congestive heart failure; all exhibited ST-T changes and cardiomegaly, 67 of 150 had left ventricular hypertrophy, 23 of 151 arrhythmias, and 55 of 153 pulmonary vascular congestion. Initial ventricular depolarization abnormalities were very frequent. Significant clinical predictors of fatal outcome included pulmonary vascular congestion, "northwest" axis deviation, and a cardiac index less than three L./min./M.2. Death occurred during the first year after initial encounter in 44 of 57 who died, and in all 13 with proved myocarditis. Primary myocardial disease is a serious disease of infancy and childhood, resulting in death or residual cardiac disease in three fourths of those affected.     

Ascending aorta-pulmonary artery anastomosis for cyanotic congenital heart disease.

The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age at operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.     

Systolic time intervals in pregnancy and the postpartum period

Systolic time intervals in the supine and upright positions were obtained in 16 antepartum subjects at maximal expansion of blood volume (27 to 33 weeks) and six weeks postpartum. Isovolumic contraction time (IVCT) was determined by 2 methods: (1) first heart sound to carotid upstroke interval (I_m-CAR_u) and minus pulse transmission time (I_m-CAR_u-PTT), and by (2) upstroke of apexcardiogram to upstroke of carotid (ACG_u-CAR_u) and minus pulse transmission time (ACG_u-CAR_u-PTT). The IVCT was short in the antepartum supine subject (I_m-CAR_u was 60.5, SD 4.3 msec.) and 31.7 msec. when corrected for PTT) and became longer in the upright position (63.7 msec. [SD 6.1] and 35.6 msec., respectively).In the postpartum state the supine IVCT was significantly longer than in the antepartum state (72.2 msec. [SD 9.3] and 42.2 msec. when corrected for PTT) (P < 0.001), and became longer in the sitting postpartum subject (85.4 msec. [SD 8.8] and 56.8 msec., respectively.)The left ventricular ejection time index was 425.1 msec. (SD 13.5) in the antepartum supine subject and demonstrated corresponding shortening in the erect position (412.8 msec., SD 14.2).In the postpartum period the LVETI was shorter (407.0 msec. [SD 17.4]) and shortest in the upright position (392.9 msec. [SD 11.6]).     

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)     

Acute bacterial percarditis in children: Report of 25 cases

Twenty-five patients, aged 5 months to 14 years, with acute bacterial pericarditis are reported. Thirteen (52 per cent) of the patients died. The presenting symptoms, associated illness and physical findings, bacteriology, and response to therapy are reviewed. Optimum therapy consists of intravenous administration of specific antibiotics combined with surgical drainage; 90 per cent of our patients treated in this fashion survived. Antibiotic therapy alone is usually inadequate, especially in the presence of significant effusion, and among our patients only three of 10 patients so treated survived. One patient developed constrictive pericarditis 1 month after the initial attack with meningococcal pericarditis and required pericardectomy.     

Exercise-induced ventricular ectopy in children and young adults with complete heart block

Twenty-five children and young adults with complete heart block and 50 normal control subjects were maximally exercised on a treadmill and their electrocardiograms were analyzed for the presence and degree of ventricular ectopy activity. Sixty-eight % (17 of 25) of the heart block patients had significant ventricular ectopy (frequent unifocal ectopy or worse) on exercise compared to 2% (one of 50) of the controls (p = <0.01). In the patients with block there was a significant trend toward more frequent and more severe ectopy with increasing age (p = <0.05) independent of heart rate.More severe ventricular ectopy was also seen in those patients with QRS prolongation (p = <0.05). No significant differences were found between the 14 patients with isolated “congenital” heart block, the seven with associated defects, and the four with surgically acquired block. We believe that severe ventricular ectopy is common at exercise in patients with complete heart block and they may have an increased risk for sudden death.     

The use of noninvasive methods in the evaluation of left ventricular performance in coronary artery disease. I. Relation of systolic time intervals to angiographic assessment of coronary artery disease severity.

Determination of left ventricular performance by external STI was evaluated in 113 patients with possible coronary artery disease undergoing selective coronary artery disease undergoing selective coronary arteriography and left ventriculography. Angiographically determined significant coronary artery disease was considered as 70 per cent obstruction of a coronary vessel. PEPI and PEP/LVET increased with increasing severity of coronary artery disease. LVETI decreased with increasing coronary artery involvement. Presence of prior myocardial infarction or clinically apparent congestive heart failure did not significantly alter mean STI values when groups were compared according to severity of coronary artery disease. LVETI was significantly less for patients with three-vessel coronary artery disease than for those with no significant disease; PEPI and PEP/LVET were significantly greater in those with two- or three-vessel disease than in those without significant disease. Angiographically determined LVEF correlated directly with LVETI and inversely with PEPI and PEP/LVET. Abnormal left ventricular wall motion was associated with decreased LVETI and increased PEPI and PEP/LVET. LVEDP was not significantly different in any of the groups. These findings indicate that externally determined systolic time intervals reflect abnormalities in left ventricular performance which in turn appear more pronounced with increasingly extensive coronary artery disease.     

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.     

The significance of right aortic arch in D-transposition of the great arteries

A review of 195 cases of d-TGA disclosed the incidence of right aortic arch to be 8 per cent. In cases associated with VSD and PS the incidence was higher (16 per cent). Fifty per cent of the cases with right arch had other severe cardiac malformation.     

Systolic and diastolic time intervals in pulsus alternans; significance of alternating isovolumic relaxation

Systolic and diastolic time intervals in 14 cardiac patients with pulsus alternans revealed significant alternation of PEP, IVCT, LVET, ETI, $\text{PEP}\text{LVET}$, and carotid $\text{dD}\text{dt}$, with better functional values in the strong beats. Cycle length, duration of electromechanical systole (EMS), and total diastole (TD) did not alternate. A new observation, alternation of the components of total diastole—i.e., isovolumic relaxation period (IRP) and diastolic filling period (DFP) occurred in 7 out of 8 patients. These diastolic intervals alternated reciprocally such that the IRP of the strong beats encroached upon the DFP of the next (weak) beats.     

Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations.

Total anomalous pulmonary venous connection (TAPVC) is failure of development of the common pulmonary vein, with consequent ersistence and enlargement of embryonic collaterals between the lungs and the systemic veins. In the great majority (91 of 93 autopsied cases, 97 per cent, no remnant of the common pulmonary vein was found, supporting the concept of agenesis or involution of the common pulmonary vein. Occasionally, a cordlike strand was found in the location of the common pulmonary vein (in three of 93 cases, 3 per cent), supporting the concept of atresia of the common pulmonry vein. The order of frequency of the various types of TAPVC was: left innominate vein ("snowman"), 26 perent cent; subdiaphragmatic, 24 per cent; coronary sinus, 18 per cent; right superior vena cava, 15 per cent; right atrium, 8 percent; mixed, 5 per cent; azygos, 2 per cent; and left supeior vena cava, 2 per cent...     

Primary benign intramural ventricular tumors in children: Pre- and postoperative electrocardiographic,echocardiographic, and angiocardiographic evaluation

The clinical, ECG, echocardiographic, and angiographic data, as well as the operative findings and postoperative courses of three children with intramural ventricular tumors are presented. In all three children, the specific diagnosis of intramural tumor was unsuspected. The ECGs showed a superior axis in all patients. M-mode echocardiography missed the mass in one patients and was nonspecific in the other two; however, two-dimensional echo (2DE) was able to accurately delineate the tumors preoperatively in all three. Angiographic findings were nonspecific, only demonstrating the presence of space-occupying lesions. All three patients underwent surgery and the tumors were removed successfully. This investigation emphasizes the clinical picture that led to the diagnostic evaluations, and demonstrates the usefulness of 2DE in accurately diagnosing the intramural ventricular tumors. In addition, the sequential ECGs and 2DEs after removal of the tumors are delineated as well as showing the resolution of the tumor craters by 2DE examinations.