Paul R. Lurie

Endocardial fibroelastosis is not a disease

T he article in this issue on endocardial fibroelastosis (EFE) from the Hospital for Sick Children in Toronto’ provides me with the opportunity to argue here for a change in terminology. In my opinion, it is unrealistic to continue to write and talk about EFE as if it were a specific disease rather than the pathologic result of many different diseases. Because the former usage, which I believe is now incorrect, is still accepted by most current textbooks of medicine, pediatrics, cardiology and pathology and was recently unchallenged by the new World Health Organization2 classification, this protest is overdue. Let us look briefly at the history of thought about EFE.3 The striking pathologic finding of diffuse thickening of the endocardium with layering of collagen and elastic fibers in patients, mostly infants and small children who died either suddenly or after protracted congestive heart failure, was recognized over a century ago. There were even then conflicting concepts. Some deemed it a disease of the endocardium, a relic of “fetal endocarditis” then favored as a mechanism of production of all congenital cardiac anomalies. Others took note of the associated hypertrophy of the myocardium, usually without apparent explanation, calling it “idiopathic hypertrophy of the heart.” Because some patients with idiopathic hypertrophy had an abnormal endocardium and others did not, it was postulated that the thick endocardium was a reaction to myocardial abnormality. In 1943 the term “EFE” was first used to describe the endocardial findings. It was quickly accepted, although other terms continue to be used (endocardial sclerosis, endomyocardial fibroelastosis, etc.). Shortly thereafter the distinction was drawn between EFE “secondary” to congenital malformations of the heart and “primary” EFE where no malformation was present. Explanations for finding a grossly “normal” myocardium with a thick endocardium included intrinsic developmental anomaly, chronic hypoxia and lymphatic obstruction. These theories tended to deemphasize the myocardial findings of dilatation and hypertrophy, some even suggesting they were secondary to splinting of the myocardium by the

Hypertonicity Following Selective Angiocardiography

Plasma osmolality and serum sodium concentrations were studied in 30 children with a variety of cardiac abnormalities who underwent selective angiocardiography. A significant increase of osmolality occurred, but there were no significant changes in sodium concentration. The pathologic effects of hypertonic solutions including contrast media are discussed and it is concluded that hypertonicity is a preventable factor in serious reactions seen after angiocardiography.

Clinical and hemodynamic effects of pulmonary artery banding

Abstract Experience with a large number of pulmonary artery banding procedures performed on diverse groups of patients is presented. A high mortality rate has been observed in patients under 3 months of age, in patients with conotruncal anomalies plus ventricular left to right shunt and in patients with complete atrioventricular canal. The operative mortality rate in patients with ventricular septal defect has improved dramatically in the last few years probably as a result of the combination of later timing of banding and concerted efforts to achieve stable anesthesia, minimal surgical trauma and accurate monitoring of pulmonary and systemic arterial pressures during the procedure. Benefits of pulmonary artery banding, such as improved growth rate, freedom from respiratory infections and improvement in chest roentgenograms and hemodynamic findings, bear a fair correlation with the degree of pulmonary artery constriction (by pressure). Finally, the pulmonary vascular resistance progressively declines in some patients following the pulmonary artery banding, although this response cannot be predicted before surgery. Fibrosis around the pulmonary artery and pericardium is a definite disadvantage inherent in the present technic of banding.

Postural Effects in Tetralogy of Fallot

Abstract The postural effects upon cyanosis and dyspnea in tetralogy of Fallot, including the beneficial effects of squatting, the knee-chest and other positions, and the disadvantageous effect of standing are shown to be directly due to alterations in volume of venous return with consequent change in the oxygen saturation of mixed venous blood. Speculations are adduced to explain the peculiarly marked effect of altered venous return in malformations when there is venoarterial shunt, reduced pulmonary blood flow coexisting with high systemic flow and absence of a tendency to go into congestive heart failure. Diagnostically, these postural effects are important when they can be demonstrated in the form of simple clinical tests. A patient demonstrating them will respond well to systemic-pulmonary anastomosis. Therapeutically, they are of real importance in carrying a patient through the period of waiting for definitive surgery.

Revision of pediatric endomyocardial biopsy technique.

A transvascular endomyocardial biopsy technique specifically designed for infants and small children was described in this journal in 1978.l Since then, 2 reportG3 and many personal communications indicate acceptance of the method. Improvements in the technique now warrant publication of a revision. The method consists of passage of a soft forceps through a guiding catheter preshaped for the specific course in the specific patient.

Hemodynamic Effects of Valvulotomy in Pulmonic Stenosis

Seven patients with mild to moderate pulmonic stenosis with intact ventricular septum were subjected to cardiac catheterization before and from 4 to 18 months after pulmonary valvulotomy. Despite uniform clinical improvement, remarkably slight changes were observed, following surgery, in right ventricular pressure, pulmonary artery pressure, pulmonary artery flow, and pulmonary valve area, when measured at rest. When present preoperatively, venoarterial shunt was reduced or eliminated after operation. It was speculated that further studies including the effect of exercise might show that valvulotomy increases the mobility of the valve under stress even when it does not increase valve area at rest.

Further Observations on the Closure of Atrial Septal Defects

Though patients with small atrial septal defects may do well, in certain individuals with large defects operative closure is highly desirable because of the resultant cardiac dysfunction and the poor prognosis without treatment. Extensive efforts in the experimental laboratory have led to the development of a number of methods for the surgical closure of these defects, a number of which have had clinical trial. An earlier method, in which a pericardial pocket was affixed to an incision in the atrial wall and invaginated into the atrial cavity so that its posterior wall could be sutured to the rim of the defect, proved safe and reliable in experimental animals. One case demonstrated, however, that in large human defects the pericardium may not become sufficiently rapidly vascularized to permit its survival. The present experiments have shown that the same technic modified by substitution of a plastic nylon pocket appears entirely satisfactory. In one clinical case it was applied with success. It is suggested that when simpler methods are not applicable this procedure may prove safer and more reliable than certain other methods which have been used.

The physical examination in pediatric cardiology.

The proper approach to pediatric cardiovascular examination involves patience, timing, consideration of the babys needs, and use of diagnostic implements of correct size. The most important general considerations are those concerned with color and respiration. Careful inspection and palpation of the chest is most instructive, and percussion and auscultation reveal valuable information. The author prefers the auscultatory method of determining blood pressure, which is an essential part of evaluating the circulatory system.