Carolyn M. McCue

Congenital heart block in newborns of mothers with connective tissue disease.

Of 22 children with congenital complete heart block (CCHB) available for study, 14 (63.6%percnt;) were born to 11 mothers with clinical or laboratory evidence of connective tissue disease, primarily lupus erythematosus (LE). Seven mothers had both clinical and laboratory evidence of disease while four had only positive laboratory studies including fluorescent antinuclear antibody, rheumatoid factor, and depressed complement levels. In adults with systemic LE, pathologic changes in the collagen surrounding the conduction system have led to fibrosis and death from heart block. Antinuclear antibodies of the IgG class cross the placental barrier and newborn infants have been reported with transient skin lesions of lupus. Placental transmission of such antibodies may affect the fetal cardiac conduction system, surrounding collagen, and myocardium, leading in some cases to CCHB. This is probably one important etiologic factor in CCHB even though the mother is asymptomatic during her pregnancy.

Natural history of Wolff-Parkinson-White syndrome discovered in infancy.

Abstract The prognosis of 20 infants with a diagnosis of Wolff-Parkinson-White syndrome under 1 year of age is presented. Children were followed up from 1 to 19 years (mean 9 years). Seven patients had associated congenital heart disease. Analysis of the associated arrhythmias revealed episodes of supraventricular tachycardia in 18 (90 percent). During paroxysmal supraventricular tachycardia the QRS complex was normal in seven and wide in nine children. Categorizing patients into groups according to the width of the QRS complex and the presence or absence of a delta wave during tachycardia helps in understanding and planning therapy. Eighteen patients had a stable or improved course with growth but two patients with tetralogy of Fallot died postoperatively with resistant supraventricular tachycardia. The high prevalence rate of supraventricular tachycardia during infancy which later decreases or disappears could be explained by the different electrophysiologic responses between normal and accessory pathways during the fast heart rate characteristic of this young age. Invasive electrophysiologic studies are indicated in children older than 1 year of age who remain symptomatic and in all children being conconsidered for cardiac surgery regardless of the effectiveness of the preoperatlve therapy.

Correction of platelet dysfunction and bleeding in cyanotic congenital heart disease by simple red cell volume reduction

Red cell volume reduction corrected the platelet aggregation abnormality and bleeding tendency in four boys, aged 5 to 16 years, with severe cyanotic congenital heart disease and polycythemia. Red cell volume was reduced by replacing 15 to 20 ml/kg body weight of the patients blood with plasma in 50 ml increments over a 1 to 2 hour period. Within 3 days, platelet aggregation was restored essentially to normal, and previous bleeding symptoms disappeared. Platelet aggregation remained normal during 3 weeks of follow-up study and hematocrit values remained at palliative levels. The procedure was safe and simple, and it had beneficial effects not only on bleeding but also on other symptoms related to polycythemia. These preliminary observations suggest that red cell volume reduction may be useful preoperatively in patients with cyanotic heart disease and defects in platelet function to lessen the risk of serious bleeding during the early postoperative period. Palliation in severely cyanotic children whose condition is inoperable is another possible indication for this procedure.

Pulmonary artery coarctations

Twenty cases of local peripheral pulmonary artery coarctations are presented and classified by anatomic types. The clinical history, physical examination, electrocardiographic, radiologic, and catheterization data are described. Selective angiocardiographic illustrations are given to show the preferred method of diagnosis. The literature was extensively reviewed and 319 cases are tabulated according to type and associated defects. Two thirds were associated with other forms of congenital heart disease. The pathologic lesions are briefly described and possible etiologic factors are discussed.

Congenital Coronary Arteriovenous Fistula Associated with Large Patent Ductus

An unusual case of congenital coronary arteriovenous fistula is reported, bringing the total number of reported cases to 39. This is the second case in which the left coronary artery system communicated directly with the right atrium, and also the second case associated with a patent ductus. Our case is well documented by cardiac catheterization data, retrograde aortography, and postmortem findings. Although surgical correction of both the patent ductus and coronary arteriovenous fistula was accomplished without great difficulty, the patient died 30 hours postoperatively, presumably from the effects of severe pulmonary hypertension.

Lack of connection between the atria and the peripheral conduction system in a case of corrected transposition with congenital atrioventricular block

This is the first documented histologic study of the heart of a patient with corrected transposition of the great vessels and congenital atrioventricular (A-V) block with no connection between the atria and an anterior type of peripheral conduction system. Musculature in the superior (anterior) walls of both atria was absent, as was the anterior A-V node. The peripheral conduction system began with the bundle of His. In place of the absent atrial musculature, fibrosis and calcification were present. The relation of laboratory evidence of connective tissue dyscrasia in the mother to the congenital A-V block in the child is discussed.

Observations on the natural history of pulmonary artery coarctations.

Seventeen of a series of 70 patients with coarctation of the pulmonary artery underwent serial cardiac catheterizations from 2.0 to 12.4 years apart. Main pulmonary artery pressures were measured in all patients, and the values from each study were compared. A change in pressure in the main pulmonary artery was considered to reflect a change in the distal pulmonary artery obstruction. Seven patients with no other associated cardiac defects, four with associated pulmonary valve disease, and six with associated left-to-right shunts all demonstrated little change in main pulmonary artery pressures between the two studies. These data substantiate the observation that pulmonary artery coarctations represent a nonprogressive form of congenital heart disease in which the severity of obstruction to pulmonary blood flow does not increase with age.

Cardiac failure in infancy

C A R D I A Cl f a i l u r e in a n i n f a n t is a r e l a t ive ly common pediatric emergency. This study records the findings in 115 consecutive cases of cardiac failure in infants under 2 years of age seen on the pediatric service of the Medical College of Virginia Hospitals between January, 1953, and January, 1960. Detailed studies of cardiac failure in children of all ages have been presented by Keith, 1 who recorded a mortality rate of 85 per cent in 304 children; most of the deaths occurred in the first years of life. Nadas and associates 2 have been concerned with the therapeutic aspects of cardiac failure. More recently Blumenthal and Anderson 3 reported a mortality rate of 67 per cent in children under 1 year of age, and Simpson 4 in England, one of 48 per cent. Catherine Neills excellent monographs 5, s emphasize the necessity of exact diagnosis of the underlying cardiac lesion as soon as emergency measures for cardiac failure have been effective. In the child with a congenital heart lesion, the occurrence of cardiac failure often is an indication for as prompt surgical repair as possible.

A preliminary report on rheumatic fever in virginia

Summary 1. Of a total of 1,205 patients referred by physicians to the Medical Collegeof Virginia Rheumatic Fever Clinic from April, 1940, to June, 1947, as having probable rheumatic fever or heart disease, 504 had the diagnosis of rheumatic fever confirmed, while 203 were being followed as possible and/or potential heart disease, and 253 were still being listed as deferred. 2. Two hundred and twenty-five, or 44 per cent of the 504 patients, were the subjects of special study since they had been followed for at least three years or until death. 3. The study of this group of 225 patients brought out the following facts: The average duration of the disease was 5.95 years as followed thus far. The sexes were about evenly divided. Race incidence closely paralleled the percentage distribution in population A family history of rheumatic fever was obtained in 17.3 per cent. The average age of onset of initial manifestations was 7.97 years. January was the peak month for onset in both the initial and recurrent episodes. A history of preceding streptococcal infection was obtained in about 50 percent of the children. Only 20 per cent had had tonsillectomies prior to the onset of the disease. Joint pains led the list in frequency of symptoms occurring with initial and secondary attacks. Fever and malaise ran close seconds. Laboratoryfindings as to elevation of sedimentation rate, leucocytosis, andanemia were somewhat helpful but far from conclusive. Sixty-one per cent had one or more recurrent episodes while under observation. As nearly as could be estimated, the mean duration of the first attack was 6.4 months, and that of the second was slightly under eight months. Cardiac involvement was demonstrable in 83 per cent of all patients during the active stages of the disease. Only 49 per cent showed demonstrable residual cardiac damage, but the rest are still under observation as having possible and/or potential heart disease. Seven and one-tenth per cent (sixteen patients) died during the period ofobservation. The average duration of life after onset of the disease in these children was 40.8 months.

Steroid therapy for rheumatic fever

Summary Ninety-four patients with acute rheumatic fever have been studied and treatment at the Medical College of Virginia Hospitals with varying doses of cortisone and prednisone in the past six years. About one-half of the patients on any of the large-dosage schedules treated within 28 days had no heart disease at the end of therapy and 73.8 per cent either had no heart disease or were much improved at the end of therapy (Table IX). There have been eight deaths while on treatment. The drug was considered lifesaving in eleven cases. Better results were obtained when the drug was started earlier in the course of the disease and in large doses.