James A. Manning

Respiratory Syncytial Viral Infection in Infants with Congenital Heart Disease

Occasional reports have suggested that infants with congenital heart disease may have an increased risk of severe illness from respiratory syncytial virus (RSV) infection. We prospectively studied 699 infants hospitalized during the winters of 1976 through 1980, when RSV was prevalent in the community; 229 of these infants had proved RSV infections acquired either before admission or during hospitalization; 27 had both congenital heart disease and RSV infection, and 46 had congenital heart disease without RSV infection. Infected infants with congenital heart disease had significantly more severe illness than those without congenital heart disease, as judged by the requirement for intensive care and assisted ventilation and by the mortality rate (37 per cent vs. 1.5 per cent, P less than 0.01). The infection was acquired nosocomially by 21 per cent of infected infants; the mortality rate from nosocomial infection was also higher in infants with congenital heart disease (44 per cent vs. 5 per cent, P less than 0.01). Pulmonary hypertension was the one condition particularly associated with severe RSV illness. Eight of the 11 infants (73 per cent) with congenital heart disease and pulmonary hypertension died during their RSV illness. The courses in infants with congenital heart disease with and without RSV infection were also compared. Their ages, types of cardiac lesions, and incidence of pulmonary hypertension were similar, but the infants with RSV infection had a higher mortality rate (37 per cent vs. 6.5 per cent, P less than 0.1).

Early closure of the patent ductus arteriosus in very low-birth-weight infants: A controlled trial

A controlled clinical trial comparing early closure (mean = 48.8 hours) of the patent ductus arteriosus using indomethacin to conventional medical management, with intervention only after cardiopulmonary decompensation (mean = 167.4 hours), was undertaken in 24 preterm infants with severe respiratory distress syndrome and evidence of PDA. An interval analysis of one-half the projected sample revealed that infants undergoing early closure of the PDA had significantly reduced occurrence of BPD or mortality by 6 months of age. A comparison of birth weight, Apgar scores, gestational age, age of initial PDA diagnosis, and fluid therapy during the first seven days of life showed no significant differences between early intervention and control groups. At the time of the interval analysis, there were no differences between the groups in duration of intermittent mandatory ventilation or oxygen exposure. Studies will be required to determine whether these and other variables can be altered by early closure of the PDA.

Echocardiographic Recognition of the Congenital Bicuspid Aortic Valve

Twenty-one patients (age four to 25 years) with bicuspid aortic valves proved by surgery and angiography (12) or angiography alone (9) were studied by echocardiography. Seventeen patients had aortic valve disease (11 stenosis, 6 incompetence) and four had coarctation of the aorta with normally functioning aortic valves. A comparison group of 16 patients (9 with aortic valve disease and 7 without aortic valve disease) who had proven tricuspid aortic valves was also studied. Echocardiograms in tricuspid aortic valve patients showed the closed position of the cusps near the middle of the aortic lumen. In contrast marked eccentricity of the aortic valve cusp echoes in diastole could be demonstrated in all with bicuspid aortic valves. The Eccentricity Index (½ aortic lumen diameter/minimum distance of the diastolic cusp echo from the nearest aortic margin) was low (range 1.0-1.25) with tricuspid aortic valves and high (range 1.5-5.6) with bicuspid aortic valves (P < 0.001). This index was not significantly affected following successful valvotomy. Bicuspid aortic valve cusps showed normal systolic motion pattern resulting in marked asymmetry of leaflet images. About half of patients with bicuspid aortic valves also showed multilayered echoes in diastole in the absence of fluoroscopic evidence of valvular calcification. Echocardiography appears to be specific in the recognition of the bicuspid aortic valve.

A collaborative study of infective endocarditis in the 1970s. Emphasis on infections in patients who have undergone cardiovascular surgery.

Twenty-six major cardiovascular centers participated in a cooperative study of all cases of infective endocarditis occurring during a single calendar year to obtain an overview of infective endocarditis. The study was designed to learn which patients appear to be at highest risk to develop this infection after palliative or reparative cardiovascular surgery. Of 278 patients developing infective endocarditis during the year at these medical centers, 63 (23%) had had previous cardiovascular surgery and 215 had not. Seventy percent of the 278 patients had recognized congenital or acquired heart disease before developing the infection. Rheumatic heart disease accounted for over half of the patients with underlying structural heart disease. A majority (55%) of the 63 patients who had been operated on before developing endocarditis had prosthetic valves inserted. Of those who did not require prosthetic valves, the majority had congenital heart disease with systemic artery-to-pulmonary artery shunts. Although these data were obtained from a selected group of patients, they confirm a significant risk of endocarditis in patients with prosthetic valves and suggest that in postoperative patients with non-valvular congenital heart disease, the highest risk appears to be in cyanotic patients with palliative pulmonary artery-tosystemic artery shunts

Trisomy 13–15 in a male infant*

Summary 1. A male infant with trisomy 13–15 is described. The congenital malformations present are similar to those of previous cases, lending support to the view that they constitute a specific syndrome. 2. The present case is the first described in a male, and the first described in a non-Caucasian individual. 3. In addition to the congenital anomalies usually associated with this trisomy, others are described which have not previously been reported.

Echocardiographic Diagnosis of Transposition of the GreatVesselsl

Experience in the echocardiographic diagnosis of dextro-transposition of the great vessels in 11 cases is presented. Transposition can be recognized by the altered spatial relationship of the great vessels characteristic of this condition. In the normal subject, the posterior out-flow vessel (aorta) is detected by medial transducer angulation while the anterior vessel (pulmonary artery) requires lateral angulation. In dextro-transposition of the great vessels, this relationship is reversed. This safe noninvasive technique is recommended in newborns when the cause of cyanosis is uncertain or when other causes of cyanosis may confuse the diagnosis.

Trisomy 18 (16–18) associated with congenital glaucoma and optic atrophy

A case of trisomy 18 in a female infant is reported. Many abnormalities characteristic of this syndrome were present, including failure to thrive, mental retardation, hypertonicity, low-set ears with partial absence of antihelices, prominent occiput, flexion abnormality of the hands, syndactyly of the toes, interventricular septal defect, unilateral kidney, hypertrichosis of the forehead, and corneal opacities. In addition, the patient had bilateral congenital glaucoma and optic atrophy which had not been reported previously. The child was amaurotic and deaf. Hypogammaglobulinemia was also noted but tentatively attributed to the failure to thrive. The opportunity for chromosomal markers that may be afforded by autosomal trisomies is briefly mentioned.

IMPAIRED CALCIUM HOMEOSTASIS IN THE INFANTILE HYPERCALCEMIC SYNDROME

The etiology of the “severe form” of the infantile hypercalcemic syndrome (a rare condition which includes supravalvular aortic stenosis, mental retardation, and an “elfin facies”) is not known, nor has the origin of the hypercalcemia been established. Current hypotheses include vitamin D hypersensitivity or excess, an abnormality of cholesterol metabolism, and delayed turnover or degradation of vitamin D. Although a review of the literature led Seelig (28) to conclude that these patients have an increased response to vitamin D, Fraser et al. (14) are of the opinion that none of these hypotheses have been proven. The hypercalcemia usually disappears during childhood but the other features remain. Although large oral doses of vitamin D will intensify the hypercalcemia in infants with this syndrome (28), modest doses do not alter serum calcium in older children (5, 13, 33). Barr & Forfar (6) and Dormandy & Begum (11) have reported that an oral calcium load resulted in higher and more sustained levels

Echocardiographic features of subpulmonic obstruction in dextro-transposition of the great vessels.

Eleven patients with transposition of the great vessels (TGV) and subpulmonic (left ventricular outflow) obstruction documented by cardiac catheterization/ angiography were studied by echocardiography. Ten showed prolonged mitral-septal apposition in diastole indicative of a narrow left ventricular outflow tract. In addition, 5 patients showed systolic anterior movements (SAMs) of the anterior mitral leaflet resembling those observed in hypertrophic subaortic stenosis. The SAMs were large in 3 patients and may have accentuated the outflow obstruction. Additional evidence for this was provided by the tendency of the pulmonic valve towards closure with the onset of mitral SAMs. A comparison group of 21 patients with TGV and no outflow obstruction did not show these features. Echocardiography appears to be useful in the diagnosis of subpulmonic muscular obstruction in TGV. Demonstration of definite mitral SAMs in some patients suggests the presence of associated functional obstruction of the type seen in hypertrophic subaortic stenosis.

Two-dimensional echocardiographic identification of pulmonary artery branch stenosis.

Pulmonary artery branch stenosis is a not uncommon congenital lesion, noted in approximately 4% of children at the time of cardiac catheterization. Real-time 2-dimensional echocardiography was used to examine 10 patients with angiographically documented pulmonary branch stenosis. Five cases of pulmonary artery branch narrowing were identified by echocardiography without prior knowledge of angiographic findings. In 5 other patients whose angiographic results were known, the narrowings were identified in 4 of 5 cases. These results demonstrate the feasibility of evaluating pulmonary branch stenosis with the nonionizing, noninvasive method of 2-dimensional echocardiography.