Lorin E. Ainger

Congenital rubella syndrome: New clinical aspects with recovery of virus from affected infants

Seventeen newborn infants with stigmas of the congenital rubella syndrome were studied clinically and virologically. These data are presented with postmortem findings in 2 of the infants. Myocardial necrosis, interstitial pneumonitis, and regurgitative jaundice associated with hepatosplenomegaly are reported for the first time. Cataracts, glaucoma, cardiac anomalies, thrombocytopenic purpura, and a variation of a previously reported bone lesion were also observed. Rubella virus was isolated in one or more specimens from each infant, from necropsied viscera, biopsied liver, and from spinal fluid. The congenital rubella syndrome is a chronic viral infection of the neonate which is acquired in utero.

ACUTE ASEPTIC MYOCARDITIS. CORTICOSTEROID THERAPY.

Acute aseptic myocarditis associated with serologic evidence of recent infection with various types and stains of influenza virus occurred in 8 infants and children. The clinical picture was quite constant and consisted of an antecedent influenzal illness, septic fever, myalgia, leukocytosis, transient rash, and the sudden onset of severe congestive heart failure, intractable to conventional therapy. The addition of corticosteroids or their synthetic analogues to the therapeutic regimen proved a valuable life-saving adjunct.

The Frank Vectorcardiogram and the Electrocardiogram in Duchenne Progressive Muscular Dystrophy

Electrocardiograms and Frank vectorcardiograms from 44 patients with classical childhood progressive muscular dystrophy (Duchenne type) were analyzed. Seventy-seven per cent had definite electrocardiographic abnormalities. All had one and usually several vectorcardiographic abnormalities. Abnormalities in orientation, magnitude, or both, of the planar and spatial ventricular gradients were the most consistent of the vectorcardiographic abnormalities and were observed in 95% of the patients. This finding is of significance if this vector theoretically reflects diffuse myocardial disease at the cellular level. QRS changes, in general, reflected the relatively greater loss of posterior ventricular force with consequent exaggeration of anterior ventricular force. There was no demonstrable relationship between the electrocardiographic and vectorcardiographic abnormalities with the age of the patient and the severity and rapidity of clinical progression of the disease in patients less than 16 years of age. This study suggests that the vectorcardiogram may be a useful diagnostic adjunct to the standard electrocardiogram in the study of the cardiomyopathy of progressive muscular dystrophy in childhood.

OSTIUM SECUNDUM ATRIAL SEPTAL DEFECTS AND CONGESTIVE HEART FAILURE IN INFANCY.

Abstract Foramen secundum defects of the interatrial septum with large left to right shunts may cause congestive heart failure during infancy. In three infants with this lesion, varying in age from 5 days to 2 years, intractable congestive heart failure developed. Case 1 presented with signs of congestive heart failure in the newborn nursery. She failed to respond to the usual anticongestive measures. At 6 weeks of age her defect was reduced in size by the external closure technic of Sondergaard. She had an excellent postoperative course. Case 2 presented in severe congestive heart failure at 3 months of age. Clinical improvement followed ligation and division of a large patent ductus arteriosus. Signs of cardiac decompensation reappeared at 6 months. Closure of a secundum atrial septal defect on cardiopulmonary bypass was successful. The oldest child, a 2 year old female, presented with severe congestive heart failure of unknown duration. An attempt was made to close her atrial septal defect with cardiopulmonary bypass. Her death following surgery was apparently due to almost complete pneumonic consolidation of her lungs. Each of these female infants had distinctive physical findings which led to the correct clinical diagnosis of their lesion. In Case 2 the physical findings appeared after ligation and division of the patent ductus arteriosus. These physical signs were: a right ventricular systolic thrust along the lower left sternal margin, a harsh systolic ejection murmur along the left sternal border associated with a palpable systolic thrill, and a wide and fixed split of the second heart sound at the cardiac base. In addition to the physical findings, the electrocardiogram showed right atrial enlargement and right ventricular hypertrophy. Chest roentgenograms demonstrated moderate to marked heart enlargement with increased pulmonary blood floW. Our experience with these infants demonstrates again that foramen secundum interatrial septal defects may be lethal lesions and may be responsible for considerable morb dity during infancy. The gratifying postoperative course following partial closure of the defect of the atrial septum by the Sondergaard technic in the small infant suggests that this technic should be evaluated further as a palliative procedure for small infants in congestive heart failure caused by this lesion.

Double-outlet right ventricle: intact ventricular septum, mitral stenosis, and blind left ventricle

Abstract The clinical course, physical findings, and postmortem observations have been described in a child with double-outlet right ventricle complicated by congenital mitral stenosis and a hypoplastic and nonfunctioning left ventricle with an intact ventricular septum. A continuous murmur was considered to be due to the continuous flow of blood across a small foramen secundum atrial septal defect.

Rupture of a sinus of Valsalva aneurysm in an infant: Surgical correction∗

Abstract A newborn infant with rupture of an aneurysm of the right sinus of Valsalva had clinical and electrocardiographic evidence of this condition at birth. Only a diastolic murmur was present, and incorrect timing of this murmur, correlated with suggestive angiocardiographic findings, led to an erroneous diagnosis of severe congenital aortic stenosis. Surgical exploration revealed the true nature of the anomaly, and successful surgical correction was accomplished when the infant was 6 weeks of age. During subsequent follow-up, it was obvious that the patient had physical stigmata of Marfans syndrome and aortic insufficiency frequently described in association with this syndrome.

Prosthetic mitral valve replacement in children

Nine children with severe mitral valvular disease of varied etiologies had replacement oftheir valve with a Starr-Edwards ball-cage prosthesis. There was one death in the immediate postoperative period due to clot formation within the prosthesis, and one late death of unknown cause. The survivors have had dramatic symptomatic and objective evidence of postoperative improvement. The causes of mitral valvular disease in childhood are discussed, and the hazards of artificial valve implantation are weighed against the benefits of the procedure.

Digital Computer Analysis of the Vectorcardiogram of the Newborn Infant Quantitative and Comparative Measurement of Three Orthogonal Lead Systems

Data derived from digital computer analysis of three corrected lead recordings for each of 13 term, normal newborn infants obtained on the first day of life are reported. The Frank, Schmitt SVEC-III, and McFee axial systems recorded the time sequence of ventricular depolarization force orientation in an almost identical manner. The following electrocardiographic measurements are reported for each of the three lead systems: heart rate, P-R interval, QRS and S-T intervals, planar and spatial mean P vectors, successive planar mean instantaneous QRS vectors, maximum QRS spatial vector, maximal rightward and leftward QRS spatial vectors, mean and maximum planar and spatial T vectors, and planar and spatial ventricular gradients. In general, vector quantities during this period of life are of lesser magnitude and more variable in orientation than for older infants and children. Although newborn infant ventricular depolarization forces for the majority are directed anteriorly, to the right, and inferiorly, posterior and superior orientation is not uncommon.

Vectorcardiographic studies in infants and children. I. Comparative orthogonal lead studies in the neonatal period.

Abstract Planar vectorcardiographic QRS loops of 33 normal, term infants were recorded by the Grishman cube, Frank and McFee-Parungao axial lead-placement systems on the first through the third days of life. No changes occurred in direction of loop inscription or in magnitude of the mean and maximal planar and spatial QRS vectors that might reflect changing ventricular loading patterns during the period of transition from the fetal to adult circulation. In this group of infants the orthogonal systems performed quite similarly; the major differences observed among recordings were in the magnitudes of the recorded X, Y and Z deflections, which were greatest for the axial system and smallest for the Frank system. On successive days of life, mean and maximal QRS vectors were of a similar, but not identical, order of magnitude and orientation for a given lead system, but in individual instances wide deviations were observed. In general, in most infants QRS vector directions were right, inferior and anterior, thus reflecting the expected electrical and anatomic preponderance of the right ventricle at this age. The cube system, an unweighted lead system based on geometric placement of skin electrodes, performed in approximation to the orthogonal systems. It was, in general, a technically unsatisfactory system, even though electrode placement seemed to offer the advantage of fewer electrodes. Tracings in general were small, with considerable noise level produced by body tremor; this factor, together with the small signal, produced a small signal to noise ratio. These data will be expanded to encompass measurements from a much larger series of infants, with more detailed, extensive analysis made possible through the use of digital computer technics.

Spatial QRS curves of the newborn infant. Spatial magnitude, velocity, and orientation (Frank system).

Abstract Spatial curves, derived by digital computational procedures from Frank lead system recordings on the first day of life in thirteen term newborn infants have been presented. Although these data are not considered definitive, they do illustrate some interesting aspects of the spatial characteristics of electrocardiograms recorded at this age and the curves reflect the unique anatomic characteristics of the heart for this period of life. Of interest also is the great normal variability in time course of force orientation characteristic of this age.