Cardiomyopathy and myocarditis in children with ventricular ectopic rhythm.

OBJECTIVE: The objective of this study was to evaluate the histologic features of the myocardium in children with abnormal ventricular ectopic rhythm but a structurally normal heart. BACKGROUND: Abnormal ventricular ectopic rhythm in children with a structurally normal heart is an uncommon but serious condition. Previous studies in adults with these findings have shown that approximately 10% have "primary electrical disease" and that 40% to 100% of these have abnormal histologic findings. METHODS: Endomyocardial biopsy samples were obtained prospectively in 33 subjects presenting with ventricular ectopic rhythm but a structurally normal heart by physical examination and noninvasive studies. Biopsy specimens were evaluated for histologic changes consistent with dilated cardiomyopathy or myocarditis and these results were compared with noninvasive and invasive clinical findings. RESULTS: Of the 33 subjects, 16 (48%) had normal myocardial histologic features (Group A), 14 (42%) had changes similar to the histologic features seen with idiopathic dilated cardiomyopathy (Group B) and 3 (9%) had lymphocytic myocarditis (Group C). Presenting clinical symptoms, surface electrocardiograms (ECGs), exercise stress testing and electrophysiologic stimulation tests failed to predict the biopsy results. Twenty-four-hour ambulatory ECGs showed a statistical difference between sustained and nonsustained ventricular tachycardia in Group A versus Group B (p less than 0.007), with Group A having more sustained ventricular tachycardia. Left ventricular function measured by fractional shortening on echocardiography did not differ between groups, but left ventricular end-diastolic dimension was greater in the subjects with abnormal histologic findings (Group B) (p less than 0.03). CONCLUSIONS: These results provide evidence that approximately 50% of children with abnormal ventricular ectopic rhythm but a structurally normal heart may have subclinical cardiomyopathy or unsuspected myocarditis.

Extraintestinal amebiasis in infancy: report of three patients and epidemiologic investigations of their families.

Three infants (aged 6 weeks, 7 weeks, and 10 months) had severe Entamoeba histolytica infections characterized by colitis, hepatic abscesses, and peritonitis. The two younger children died after fulminant illnesses while the third recovered. Diagnosis was delayed in all three children by a low index of suspicion and negative stool examinations for parasites. Epidemiologic investigations of the infants' families revealed a high prevalence of amebic infections and elevated antibody titers to E histolytica; however, most family members were asymptomatic. The original source of the infections could not be identified but person-to-person spread within the families was implicated.

Fetal cerebrohepatorenal (Zellweger) syndrome: dysmorphic, radiologic, biochemical, and pathologic findings in four affected fetuses.

Four fetuses with positive family histories for cerebrohepatorenal (Zellweger) syndrome (CHRS) underwent diagnostic amniocentesis or chorionic villus biopsy. Cultured amniocytes or fibroblasts from all of the fetuses displayed abnormal fatty acid ratios, and the parents elected therapeutic abortions. Dysmorphic features in one fetus consisted of micrognathia, proximal implantation of toes, and bilateral talipes equinovarus. Radiologic examination of the fetus confirmed the dysmorphic features and revealed foci of mineralization in the patellae. Biochemical analysis of three of the fetuses demonstrated markedly increased levels of very-long-chain fatty acids, both saturated and monounsaturated, in liver, kidney, adrenal, and brain. Pathologic findings consisted of premature mineralization of patellae; renal cystic tubular dilations; striated cells in adrenal fetal zone and testicular interstitium; dysplastic alterations of inferior olivary nuclei, dentate nuclei, and cerebral cortex; equivocal increases in portal fibrous tissue; and abnormal cytosomes in fetal zone adrenocortical cells, testicular and renal interstitial cells, and brain macrophages. Iron deposition, probably physiologic, was observed only in liver tissue. Distributions of immunoreactive catalase were identical in the fetuses with CHRS and age-matched control subjects. These findings document the accuracy of the prenatal diagnostic test and provide insights into the morphogenesis and pathogenesis of CHRS.

Atrial wound healing with polyglycolic acid and polypropylene sutures.

The suitability of absorbable suture for atrial repair was tested in 25 rabbits in which atrial wounds were repaired with either polyglycolic acid or polypropylene suture. Tensile strength and wound thickness were measured at several time intervals up to 8 weeks after wounding. With each animal used as its own control, the ratio of wound tensile strength to that of unwounded atrium and of wound thickness to thickness of unwounded atrium were compared at several time intervals. There was no difference between polyglycolic acid and polypropylene suture lines in tensile strength index, but the wall thickness index was significantly lower for polyglycolic acid sutures. The latter finding was probably due to a less intense chronic inflammatory response in the polyglycolic acid group. Because of the necessity for maximal size and future growth of atrial anastomoses after repair of some congenital cardiac malformations, polyglycolic acid suture appears to be a reasonable alternative to nonabsorbable suture for atrial repair in children.

Supernumerary mitral valve producing subaortic stenosis.

A ten-year-old girl with severe subaortic stenosis was found to have relatively mature valvular endocardial cushion tissue (fibromyxomatous sheets with a chorda tendinea attached to a left ventricular papillary muscle) immediately beneath the aortic valve. This structure behaved like a valve mechanism, obstructing the left ventricular outflow tract during ventricular systole. This anomaly is an extreme on the spectrum of obstructive endocardial cushion malformations.

Asplenia syndrome. A study of congenital anomalies in 16 cases.

Sixteen new cases of the asplenia syndrome with autopsy findings had a female preponderance of 2:1. Racial incidence of splenic agenesis was nine whites and seven blacks. The average life span of the infants born alive was approximately 38 days. All infants with the asplenia syndrome had recorded weights and lengths below normal standard values. Surgical palliation of the cardiovascular anomalies in the asplenia syndrome was attempted in two cases. The incidence of the asplenia syndrome from the large autopsy series at the Medical University of South Carolina is one case per 2,000 autopsies.

Polysplenic syndrome in association with a rudimentary spleen.

A case report is presented which contains a constellation of congenital anomalies often found associated in the polysplenia syndrome. A single ectopic rudimentary spleen was found instead of the typical polysplenic malformation. The case is discussed in relation to the variability of both the asplenia and polysplenia syndromes.

Effect of prenatal ethanol administration on the urogenital system of mice.

The purpose of the present series of studies was to determine whether an obstruction in the urogenital system or vesicoureteral reflux (reflux, the retrograde passage of urine from the bladder into the kidney) existed in mice prenatally exposed to ethanol which might account for the high incidence of hydronephrosis and hydroureter observed. In order to examine these possibilities, indigo carmine was injected into the bladder of 19-day fetuses previously exposed to ethanol on Day 10 of gestation and the presence of hydronephrosis and/or reflux determined. As expected, we found a greatly increased incidence of hydronephrosis and hydroureter. In addition, there was a significant increase in reflux in the ethanol-treated mice. The incidence of reflux appeared to be related to the severity of the hydronephrosis observed, though cases of hydronephrosis without reflux and reflux without hydronephrosis were found. These data suggest both hypotheses may be salient and that a multiplicity of urogenital abnormalities are found following prenatal ethanol exposure.

Maintaining patency of the ductus-arteriosus for palliation of cyanotic congenital cardiac malformations. The use of prostaglandin E1 and formaldehyde infiltration of the ductal wall.

We have used two methods to maintain ductal ppatency in 13 newborns during surgery for congenital cardiac malformations: prostaglandin E1 (PGE1) infusion for the short-term and formaldehyde infiltration of the ductus arteriosuos (FID) for the longer term. PGE1 increased the arterial oxygen saturation, leading to stable intraoperative hemodynamics in the six infants in whom it was used. FID was used in all 13 patients. Four of these patients died in the hospital, all with the ductus open. Of the nine early survivors, all required an additional shunt procedure. The five long-term survivors had the second palliative operation immediately, three hours, three days, two and one-half months, and four and one-half months after FID. We continue to use PGE1 to maintain ductal atency through operation, but use aortopulmonary anastomosis in the newborn period rather than FID.

Proliferative function of cadaveric bone marrow cells.

The proliferative function of human and murine cadaveric bone marrow was studied using methylcellulose clonal cell culture assays and the murine spleen colony technique. The study revealed persistence of hemopoietic functions for as long as 19 postmortem hours in cadaveric marrows of some patients. Studies of murine cadaveric marrows corresponded with those of human marrows. These results strongly suggest that human pluripotent hemopoietic stem cells survive in cadaveric marrows.

A comparison by HPLC of ferritin subunit types in human tissues.

Ferritin was isolated from human liver and spleen. Reversed phase high performance liquid chromatography of the ferritin subunits from each tissue yielded the same three chromatographic fractions. Physical and chemical characterization of the three fractions indicated that they represented at least two, perhaps three, chemically distinct subunits.

Fatal suture embolism to the left anterior descending coronary artery. A case report and review of the literature.

A variety of emboli may be encountered at autopsy. While pulmonary thromboemboli are a relatively common cause of sudden "natural" death, the medical examiner may discover more exotic emboli during the postmortem examination. Other endogenous sources of emboli include the atheromatous debris, bone marrow, fat, liver, brain, trophoblast, and amniotic fluid. Exogenous ("foreign body") emboli may be introduced during medical/surgical procedures or following penetrating trauma. Fatal intravenous air and gas emboli are also well documented. This report details the first case of a fatal suture embolism to the left anterior descending coronary artery following mitral valve replacement. The role of the medical examiner in the investigation of iatrogenic fatalities is discussed.

Myocardial necrosis in a newborn after long-term maternal subcutaneous terbutaline infusion for suppression of preterm labor.

We report a case of myocardial necrosis in a newborn after treatment of the mother with long-term subcutaneous terbutaline. No such serious side effects in the fetus have previously been reported. We speculate that this myocardial damage was due to beta-sympathomimetic therapy.