Leonard C. Harris

Severe Pulsus Alternans Associated with Primary Myocardial Disease in Children Observations on Clinical Features, Hemodynamic Findings, Mechanism, and Prognosis

Five children, including two siblings, with clinically similar primary myocardial disease, were studied. One of the siblings expired 3 years after the onset of symptoms, and autopsy showed features of “cardiovascular collagenosis (endocardiomyopathy).” Moderate-to-severe pulsus alternans was present in all cases and persisted even after control of cardiac failure and return to moderate physical activity. No electrical alternans or bigeminy was observed.Cardiac catheterizations were performed on all patients. Left ventricular volume was radiographically determined in the siblings. Left ventricular end-diastolic volume was calculated as greater, and end-systolic volume smaller, for large beats as compared with small beats. Though alternating, end-diastolic volume was abnormally large, preceding both small and large beats. End-diastolic pressure alternated in the same way. Beat to beat (R-R) interval, alternating by as much as 0.09 second, was equalized by pacing in the right atrium and right ventricle, with persistence of pulsus alternans to a lesser degree. Isovolumetric contraction time was prolonged preceding small beats in spite of the large preceding end-diastolic volumes.The data appear to be consistent with Starlings law but suggest that impaired contractility, possibly due to alternate deletion of contractile elements, is an additional etiological factor.

Abnormal Communication between the Aorta and Left Ventricle Aortico-Left Ventricular Tunnel

A case is presented of a 16-month-old white boy with a communication or tunnel between the right sinus of Valsalva and the outflow tract of the left ventricle. The presence of a murmur at birth and the subsequent clinical course suggest that the tunnel was congenital. The physical, electrocardiographic, phonocardiographic, and aortographic findings were quite similar to those of “aortico-left ventricular tunnel” as described by Levy et al.1 The tunnel was occluded surgically, but failure to appreciate the presence of severe aortic stenosis contributed to a fatal outcome.Postmortem study was carried out, and the relationship of the findings of aortico-left ventricular tunnel to aneurysm of the sinuses of Valsalva and the Marfan syndrome is briefly discussed.

Congenital absence or hypoplasia of the radius with ventricular septal defect: Ventriculo-radial dysplasia

Holt and Oram had previously described the combination of atrial septal defectscoexisting with characteristic congenital deformities of the thumb. Ventricular septal defects tend to occur in conjunction with defective formation or absence of the radius and sometimes absence of the thumb. Pulmonary hypertension and even the Eisenmenger complex may be present in such instances. Intermediate between these two syndromes is a third, miscellaneous group of anomalies with some characteristics of both the Holt-Oram syndrome and ventriculo-radial dysplasia, including severe pulmonary hypertension in some cases.

Idiopathic, nonobstructive cardiomyopathy in children

Abstract Eight cases of idiopathic, nonobstructive cardiomyopathy in children have been studied; 7 were of the congestive (pulsus alternans) type and 1 of the restrictive type. The congestive cases were characterized by moderate to marked cardiac enlargement, pulsus alternans and, in 3, cerebral emboli. The electrocardiograms indicated left ventricular hypertrophy in 5, anterolateral myocardial necrosis in 1, and suggested anterior myocardial necrosis in 2. The case of the restrictive type had impaired right and left ventricular filling due to marked biventricular hypertrophy. The atria were greatly enlarged and the ventricular cavities small. Of the 8 patients, 4 died. When last seen, 1 in whom the heart size had returned to normal and 1 with slight cardiac enlargement were asymptomatic and 1 other was moderately symptomatic, with cardiac enlargement. One was lost to follow-up study. Autopsies were performed in 3 cases, and right ventricular biopsy in 1. Interstitial and endocardial fibrosis and mild, focal myocardial fiber degeneration were characteristic. Left ventricular thrombi were observed at autopsy in 2 cases. The clinical picture seems sufficiently characteristic to enable a clinical diagnosis of cardiomyopathy to be made, and the congestive and restrictive forms to be distinguished.

Cardiomyopathies in infants and children

I N THE PAST FEW YEARS increased attention has been devoted to the subject of myocardial disease in the pediatric age group. Pediatric cardiomyopathies are characterized by a high degree of morbidity and mortality. There have been different approaches to the definition and nomenclature of the different myocardial diseases constituting the cardiomyopathies. A physiologic classification as proposed by Goodwin’ does not commit one to a system that is dependent on an understanding of the etiology. It avoids the pitfall of using the term “chronic myocarditis” when referring to myocardial disease of obscure etiology. The authors have found it convenient to modify Goodwin’s definition because of the different spectrum of myocardial disease in children when compared with adults. In this communication the term “cardiomyopathy” is used to designate an intrinsic disease of the myocardium, which is not caused by shunts or valvular disease. Excluded from the cardiomyopathies are cases of known congenital morphologic anomalies. The term “primary cardiomyopathy” is used to indicate disorders not known to be secondary to systemic disease, to disease in other organs or other systems. Usually the etiology is obscure. The term “secondary cardiomyopathy” designates an intrinsic myocardial disease that is secondary to or associated with systemic disease, diseases of other organs or in other systems. Where myocardial disease coexists with skeletal muscle disease as in muscular dystrophies, the cardiomyopathy has been regarded as secondary, since the presenting and main clinical features usually are those of the skeletal myopathy. Also, the existing terminology of muscular dystrophy is well established. The course of the disease in the secondary cardiomyopathies usually is acute or subacute, rather than subacute or chronic as in the primary pediatric cardiomyopathies. For the classification of pediatric cardiomj*opathies, see Table 1.

Congenital idiopathic hypertrophic subaortic stenosis associated with a phenotypic Turner's syndrome☆

Abstract A case of idiopathic hypertrophic subaortic stenosis in a girl with phenotypic Turners syndrome is reported. The coincidence of these rare anomalies, reported only once before, is unlikely to occur by chance. Essential clinical features leading to early diagnosis of idiopathic hypertrophic subaortic stenosis in infancy are presented. Surgical treatment when the child weighed 15.9 kg gave satisfactory results at 18-month follow-up study.

Idiopathic hypercalcemia of infancy with interruption of the aortic arch

Supravalvar aortic stenosis is known to occur with idiopathic hypercalcemia of infancy. Interruption of the aortic arch is now reported as the second major aortic anomaly, occurring in a well-documented case of the severe form of idiopathic hypercalcemia of infancy. Since both conditions are rare, the possibility of a chance relationship is slight. In idiopathic hypercalcemia of infancy, the arterial system of the embryo may be affected at a number of different sites.

Spontaneous closure of a ventricular septal defect after pulmonary artery banding

Summary A case is reported of spontaneous anatomic closure of a ventricular septal defect after surgical banding of the pulmonary artery. Diagnosis of this occurrence by cardiac catheterization and angiocardiography prior to the removal of the band avoided the necessity of performing a ventriculotomy. The importance is stressed of cardiac catheterization immediately prior to the banding procedure and again before removal of the band in order to assess the status of the ventricular septal defect. Before recommending pulmonary artery banding for moderate-sized ventricular septal defects, the possibility of spontaneous closure should be considered.

Cardiac Volume in Normal Children and Adolescents Its Application to Patients with Rheumatic Mitral Insufficiency

Cardiac volume was determined by a simplified Rohrer-Kahlstorf method in 305 healthy children and adolescents. The ages ranged from birth to 19 years. Height and weight were comparable with normal growth standards. Race, sex, age, height, weight, and body surface area were studied for their value in predicting cardiac volume. Cardiac volume was different in males and females (p < 0.005) and was more closely predicted by weight than by body surface area. In this study body weight has been used as a single predictor of cardiac volume for both sexes. Normal values are presented and the limitations of the technique discussed.Serial cardiac volume plotted against weight and cardiothoracic ratio plotted against time were correlated with the clinical course in 27 children with pure rheumatic mitral insufficiency. Cardiac volume was found to be superior to the cardiothoracic ratio in reflecting the severity and prognosis of mitral insufficiency. After the cardiac volume had reached a level of 1,100 to 1,300 ml, the course was progressively downhill in 10 adolescents in the absence of evidence of rheumatic activity in most cases.That the course of rheumatic mitral insufficiency is essentially volume-dependent is a phenomenon of great interest which may be used to assess the severity of mitral regurgitation and its future course.