Longitudinal study of cardiac function in thalassemia major.
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Biomedical subjects
Publications and source records attributed to M A Engle.
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Postoperative fever and pericardial-pleural reaction, designated postpericardiotomy syndrome (PPS), is a common complication of cardiac surgery involving entry into the pericardium. To determine whether the etiology of PPS is viral or immunologic, we undertook a prospective, triple-blind study of consecutive long-term survivors of intrapericardial surgery in the pediatric age group. We evaluated clinical evidence of syndrome and concurrent appearance of antiheart antibody (AHA) by indirect immunofluorescence and antiviral antibody (AVA) by complement fixation in sera preoperatively and serially postoperatively. Incidence of PPS was 27% overall in 400 subjects, but only 3.5% in infants younger than 2 years of age. AHA in high titer appeared in all patients with PPS. A fourfold or greater rise in titer to AVA was found in 70% of these but in only 5% of those with negative AHA and no PPS. AVA rise, tested in 280 consecutive patients, was to no single one of the eight viruses studied (adenovirus, cytomegalovirus, and coxsackievirus B 1-6). Instead, the rise and fall, consistent with antiviral response to a recent infection, was exhibited usually to one but occasionally to two or more viruses, and the viral prevalence changed from year to year, as did that in the community. The study suggests that concurrent fresh or reactivated viral illness plays a role in triggering the immunologic response that characterizes the PPS.
A 14-year-old boy had a protracted illness characterized by bilateral tenderness and weakness of the extremities; the illness was considered to be a connective tissue disease similar to polymyositis. Not until nine months later, when the patient had new cardiac murmurs, pulmonary edema, and then a cerebrovascular accident, was the true nature of his illness suspected. The diagnosis of left atrial myxoma with the triad of constitutional, obstructive, and embolic effects was confirmed by echocardiographic studies and cardiac catheterization. The tumor was successfully removed.
Two serial cardiac catheterizations before the age of four months and another at six years confirmed spontaneous closure of a large atrial septal defect which caused congestive heart failure in infancy.
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The postpericardiotomy syndrome is a febrile illness with pericardial and pleural reaction that either persists or appears beyond the 1st postoperative week. We believe that it begins in the 1st week after intrapericardial cardiac surgery, and that clinical signs of illness correlate with appearance of AHA and with significant rise in titer to AVA. Our present working hypothesis is that myocardial damage with bleeding into the pericardial sac at the time of surgery combines with concurrently acquired or reactivated viral illness to set the stage for the syndrome. The immune response is triggered by viral invasion of traumatized myocardium and an immune response is mounted, not against autologous myocardium per se but against the neo-antigen, the virus-infected myocardium. The illness is self-limited. It sometimes recurs but it seems to leave no sequelae other than the bad memory of a painful postoperative complication that prolonged hospitalization and delayed the realization of the full benefits of that heart operation.
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One hundred thirty-seven courses of furosemide therapy were given to 106 hospitalized pediatric patients with salt and water retention associated with cardiac or renal disease. The diuretic was effective and safe in the pediatric age group when administered acutely as a parenteral medication and over a long-term course by the oral route in the doses and at the time intervals used in this study. On the basis of each kilogram of body weight, the infants with edema as a result of cardiac failure and the children with edema secondary to renal disease responded equally well to furosemide therapy.
A recent surgical experience with the spectrum of atrioventricular (A-V) canal is reviewed. Twenty-five patients underwent surgery for the partial and complete from of this defect in the 4 years from 1971 through 1974. Sixteen had a partial defect, two a transitional defect and seven complete A-V canal. The characteristic murmurs accompanied by cardiac enlargement, pulmonay overcirculation and left axis deviation in the electrocardiogram were sufficient for diagnosis in most cases. Cardiac catherization was performed in all patients preoperatively and in 11 postoperatively. The operative approach, including a double patch modification of the usual repair for complete canal, is considered. Definitive repair, rather than pulmonary arterial banding, is advocated regardless of the patient's age. The operative mortality rate is low in patients with the ostium primum type of defect but is related to associated intracardiac anomalies in those with the complete form of the defect. Residual mitral insufficiency is a common finding after surgical repair of both partial (75 percent) and complete (100 percent) A-V canal. Although no patient in the series died of florid mitral regurgitation, the long-range effects of this complication may lead to mitral valve replacement.
His bundle electrograms and atrial pacing are reported in a 10 1/2-year-old boy and a 12-year-old girl, both of whom underwent Mustard repair for complete d-transposition of the great arteries. Each patient had progressive evidence of sick sinus syndrome culminating in syncope. Improvement occurred after insertion of a demand pacemaker.
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Presented is a case of aortico-left ventricular tunnel in a 1-day-old girl who was in shock and severe cardiac failure. The clinical, auscultatory, hemodynamic and angiocardiographic, surgical, and pathologic findings in this case and from the 21 others reported in the literature are described. The diagnosis should be considered in an infant or child with signs of aortic insufficiency. Aortography is diagnostic when a localized supravascular aortic channel communicating with the left ventricle is opacified. Definitive treatment is surgical, though residual aortic insufficiency is common.
His bundle electrograms were recorded in 348 patients aged 2 months to 24 years following routine diagnostic cardiac catheterization. Among 41 children found to be free of anatomic or hemodynamic abnormality the following mean values were obtained: P-H interval, 93.4 +/- 15.3 msec; P-A time, 21.2 +/- 7.1 msec; A-H interval, 72.2 +/- 15.9 msec; and H-V interval, 39.8 +/- 5.2 msec. The remaining 307 patients were analyzed by diagnosis of congenital heart disease. The mean P-H interval was found to be significantly increased in both ostium primum and secundum atrial septal defect (ASD) with A-H prolongation in primum ASD and P-A prolongation in secundum ASD. The mean H-V interval was significantly prolonged in ostium primum ASD and in patients with severe aortic stenosis, aortic insufficiency. and mitral regurgitation. Grouping of the patients physiologically revealed that patients with moderate-to-severe right ventricular volume overload had P-H prolongation, and patients with severe left ventricular volume or pressure overload had H-V prolongation. The clinical implications of these findings are discussed.
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Six children (five of them girls) each initially had a large patent ductus arteriosus with auscultatory, radiologic, and electrocardiographic findings typical of that lesion. After surgery for the lesion, an ejection-type basal systolic murmur led to detection of discrete membranous subaortic stenosis, which became worse in late childhood. The murmur became louder, the systolic pressure gradient increased from slight to significant, and hypertrophic subaortic stenosis or aortic insufficiency (or both) developed in the older children. Surgical excision of the membrane afforded improvement, except in one patient with the most severe involvement. Because of the unexpected finding of discrete membranous subaortic stenosis in these infants and young children who had undergone surgery for a large patent ductus arteriosus and because of the treacherous worsening of the effects of the discrete membranous subaortic stenosis as childhood progressed, it is important that those patients with a persistent systolic murmur after ductal ligation not be discharged from cardiac follow-up as cured. Serial cardiac catheterization during the growing years appears to be the most accurate way of detecting worsening discrete membranous subaortic stenosis, so that the membrane can be excised before severe complications occur.