Benjamin E. Victorica

Atrial natriuretic peptide as a marker for doxorubicin‐induced cardiotoxic effects

Doxorubicin is an effective antineoplastic agent, but it frequently causes dose‐related cardiotoxic effects. Because the atrial natriuretic peptide (ANP) level is elevated in children with heart defects, the authors measured the ANP levels in children to determine whether ANP might serve as a simple diagnostic indicator of cardiotoxic effects. Sixteen patients, 5 to 19 years of age, who were being treated with doxorubicin (45 mg/m2 body surface area) for various malignancies had ANP levels measured in plasma. There was a group of six children, with a significant peak of plasma ANP (pANP) levels 3 weeks after the administration of the drug. Of these six patients, five had received high cumulative doses of doxorubicin (160 to 370 mg/m2), and two of them went into congestive heart failure without a previous decline in left ventricular ejection fraction, a standard technique for monitoring cardiac function during treatment with doxorubicin. The other ten patients had normal ANP levels throughout the study, and signs of cardiac dysfunction did not develop. None of the patients in the control group who had cancer and were not treated with doxorubicin and none of the healthy volunteers had elevated ANP levels. These preliminary results suggest that pANP may be useful as an early and sensitive indicator for doxorubicin‐related myocardial damage. Cancer 1992; 69:1492‐1497.

Ostium secundum atrial septal defect associated with balloon mitral valve in children

Abstract Eight children with an ostium secundum atrial septal defect and balloon mitral valve are described. Leftward-superior QRS forces, abnormal T waves and suggestive apical auscultatory findings (midsystolic click or a late systolic murmur of mitral insufficiency) were frequently observed and led to the erroneous diagnosis of ostium primum atrial septal defect in six cases. Lack of any or all of these manifestations did not preclude the presence of a balloon mitral valve. The prognostic importance of this valvular lesion indicates the need to perform left ventriculography in all patients with an atrial septal defect.

Congenital complete heart block associated with maternal mixed connective tissue disease

adrenal tumour in the mother, Acta Endocrinol 65:627, 1970. 7 . Harrison KA, and Lister UG: Successful pregnancy in a patient with adrenogenital syndrome, J Obstet Gynaecol Br Comm 73:493, 1966: 8. Riddick DH, and Hammond CB: Adrenal virilism due to 21-hydroxylase deficiency in the postmenarchial female, Obstet Gynecol 45:21, 1975. 9. Leichter SB, and Jacobs LS: Normal gestation and dimin10. ished androgen responsiveness in an untreated patient with 21-hydroxylase deficiency, J Clin Endocrinol Metab 42:575, 1976. Klingensmith GJ, Garcia SC, Jones HW, and Migeon CJ: Linear growth, age of menarche, and pregnancy rates in females with steroid-treated cor/genital adrenal hyperplasia at the Johns Hopkins Hospital, in Lee PA, Plotnick LP, Kowarski AA, and Migeon CJ, editors: Congenital adrenal hyperplasia, Baltimore, 1977, University Park Press.

Percutaneous transcatheter occlusion of coronary artery fistulas using detachable balloons

Three pediatric patients underwent successful transcatheter coronary artery fistula occlusion using the Debrun system. This latex balloon system offers several advantages over other occlusion systems. First, the balloon delivery and release is controlled. Second, “test occlusions” can be performed that allow simultaneous balloon inflation, coronary cineangiography, and electrocardiographic monitoring. Third, because the balloons are flow-directed, they are easily positioned in properly chosen locations. Finally, the balloons can be constructed to suit the size of the fistula. In this study, two patients received only one balloon; in the other patient two balloons were placed in the same fistula. All fistulas drained into either the right atrium or ventricle and were successfully occluded. After a follow-up period of up to 3 years, no local or systemic reactions to the balloons were recognized. We conclude that detachable balloon occlusion of coronary artery fistulas is a safe, effective alternative to surgical ligation in selected pediatric patients.

Differences in Antibody Response to Streptococcal Antigens in Children with Rheumatic and Non-rheumatic Mitral Valve Disease

The usefulness of streptococcal antibody tests — anti-streptolysin O (ASO), anti-desoxyribonuclease B (anti-DNAse B), and anti-group A carbohydrate (A-antibody) — in differentiating rheumatic from non-rheumatic mitral disease was explored. Sera from 154 children with mitral disease were studied. Fifteen of 17 patients with chronic mitral insufficiency (MI) following acute rheumatic fever manifested persistence of elevated A-antibody levels following the decline of ASO and anti-DNAse B titers to normal 18-36 months after their acute illness. This antibody pattern was not observed among sera from 35 patients with congenital mitral valve deformities. Serologic study of 71 children with mitral disease of obscure etiology identified three groups: subgroup A includes those 12 who demonstrated the antibody pattern associated with rheumatic MI, while subgroup B is comprised of 39 children with normal A-antibody levels. Twenty patients (subgroup C) manifested evidence of recent streptococcal infection. These data suggest that the use of the A-antibody test in conjunction with other streptococcal antibody tests is of potential value in differentiating rheumatic from non-rheumatic mitral disease.

Persistent truncus arteriosus in infancy: A study of 14 cases

Abstract Infants with PTA (Types I and II) usually have growth retardation and the physical findings of a large left-to-right shunt. Cyanosis is almost always either absent or minimal. Characteristically, a loud systolic murmur along the left sternal border is present. It is preceded by a prominent constant ejection click and ends before the second sound. A diastolic flow murmur is frequently present at the apex. Continuous or truncal valve insufficiency murmurs are rare. “Splitting” of the second sound is not uncommon. This auscultatory finding may be explained because, in some patients, the second sound is “wide” being composed of several closely approximated, or indeed, 2 discrete components. The ECG usually shows atrial enlargement, a mean QRS axis between +55 and +110 degrees, combined ventricular hypertrophy, and abnormal T waves in V 6 . Isolated right or left ventricular hypertrophy is rare. A high incidence of extracardiac anomalies is found especially in patients with a left aortic arch. The typical roentgen findings are prominent vascular markings of the shunt type, in a patient with right aortic arch (50 per cent of our cases). The plain film findings in patients with left aortic arch are similar to those with complete transposition of the great vessels except that they have a straighter upper left heart border. Selective angiocardiography either with the catheter in the root of the trunk or in the right ventricle is the best diagnostic procedure to delineate the anatomic abnormalities. Absence of the right ventricular infundibulum with a single arterial trunk arising from both ventricles are the diagnostic features. At cardiac catheterization, the systemic arterial pulse pressure is generally wide reflecting the “aortic” run-off lesion. Systemic arterial oxygen saturation approaches normal in many of these infants. Differences in oxygen saturations between the pulmonary arteries and the “ascending aorta” are most likely due to preferential streaming.

Interatrial groove tear: An unusual complication of balloon atrial septostomy

SummaryThe authors present an unusual complication of the balloon atrial septostomy procedure performed in a neonate with D-transposition of the great arteries. Cardiac tamponade developed shortly after the balloon atrial septostomy procedure and the infant was found to have a tear in the superior aspect of the left atrium, parallel to the interatrial groove. The tear was successfully sutured and may have been caused by the greater pull-back force needed if the catheter balloon is maximally distended for the first pull-back.

Tricuspid Atresia and Annular Hypoplasia: Report of a Familial Occurrence

SummaryOccurrence of a similar cardiac malformation in multiple family members has been reported for many lesions. Neither tricuspid atresia nor tricuspid annular hypoplasia and tricuspid atresia and one case of tricuspid annular hypoplasia with an atrial septal defect in siblings. The findings in this family suggest an autosomal recessive pattern of inheritance for abnormal tricuspid valve morphogenesis.

Electrocardiogram and vectorcardiogram in ventricular inversion (corrected transposition)

Abstract The vectorcardiographic (VCG) and electrocardiographic (ECG) features in nine cases of ventricular inversion with two functioning ventricles (congenital corrected transposition of the great arteries) and associated cardiac malformations were correlated related with their hemodynamic states. All three cases with low venous ventricular pressure showed a similar VCG pattern with a posterior and completely counter-clockwise horizontal QRS loop. The ECG showed a leftward QRS axis, QS complexes in V 1 , and RS complexes in V 6 . These findings are normal for ventricular inversion without major associated defects. Three patients with isolated pressure or volume overload of the venous ventricle showed large leftward and posterior initial QRS forces, followed by a predominantly clockwise and rightward horizontal QRS loop. The ECG showed right axis deviation, qR complexes in V 1 , and rS complexes in V 6 . These findings are considered to represent venous ventricular hypertrophy. Three patients with hemodynamic situations which would be expected to result in biventricular overload demonstrated varying degrees of anterior displacement of the horizontal QRS loop, two with counter-clockwise inscription and one predominantly clockwise. The initial QRS forces, although leftward and superior as in the other groups, were anterior in each case. The ECG demonstrated normal or rightward QRS axis, no precordial q waves, and large biphasic RS complexes in the midprecordial leads. These findings are considered to represent biventricular hypertrophy. One patient had serial tracings which revealed progressive ECG-VCG changes due to increasing venous ventricular hypertension secondary to increasing pulmonic stenosis. These findings indicate that the ECG-VCG accurately reflect the anatomy and hemodynamics in ventricular inversion.

Ascending Aorta-to-Pulmonary Artery Anastomosis for Cyanotic Congenital Heart Disease

Abstract A total of 101 infants and children with cyanotic congenital heart disease and inadequate pulmonary blood flow underwent an ascending aorta-to-pulmonary artery anastomosis at the University of Florida from January, 1964, to December, 1972. The operative mortality was 37%. The 64 surviving patients have experienced improved oxygenation of the blood and disappearance of hypoxic episodes for up to 8 years postoperatively. Two of 4 late deaths were related to inadequate pulmonary blood flow. Cardiac catheterization was performed in 16 patients 1 week to 6 years after the operation. Eight patients later underwent complete cardiac repair and discontinuance of the shunt.