Alexis F. Hartmann

Recurrent coarctation of the aorta after successful repair in infancy

Abstract Twenty unselected infants who survived repair of coarctation of the aorta were restudied an average of 6.2 years postoperatively. Eight patients (40 percent) showed no significant difference in blood pressures between the arms and legs and had normal pressures in their arms. These patients have been followed up for an average of 5.1 years postoperatively. Another 8 patients (40 percent) had moderate blood pressure differences between the upper and lower limbs. These patients have been followed up for an average of 6.6 years postoperatively. Further follow-up of this group will be required to determine how many will need reoperation. Four patients (20 percent), although asymptomatic, had recurrent severe coarctation of the aorta that will require reoperation. These patients have been followed up for an average of 6.9 years postoperatively. The mechanism of the recurrent coarctation is not clear, but there is evidence that the coarctation is incompletely relieved in some and that it develops with growth in others. All infants surviving repair of coarctation of the aorta must be closely followed up for possible recurrent coarctation until they reach adult size.

Spontaneous closure of secundum atrial septal defect in infants and young children

The records of 264 pediatric patients with uncomplicated ostium secundum atrial septal defect (ASD) were reviewed. Eighty-seven patients were younger than age 4 years at the time of cardiac catheterization. Subnormal weight gain, frequent pneumonia, cyanosis or tachypnea were present in 26 patients (30%). Of the 36 infants at catheterization, 17 (48%) had the previously described symptoms, including 12 (33%) who had congestive heart failure. Eight of the 36 infants were found to have closed their defect at a subsequent catheterization. Six of 18 patients who underwent cardiac catheterization between 1 and 2 years of age also had spontaneously closed their ASD at subsequent study. Statistical analysis of hemodynamic data revealed no difference (except a smaller shunt size) between ASDs that closed and those that did not in patients who were less than 4 years at initial catheterization. Analysis of hemodynamic data revealed no statistical differences between groups of patients with an ASD who were younger than and those older than 4 years at time of diagnostic study. Patients with ASDs that closed were significantly different from patients with atrial level shunting thought to be secondary to a valve-incompetent foramen ovale with respect to age at initial study (11 versus 2 months, p less than 0.001), mean left atrial pressure (7.7 versus 12.3 mm Hg, p less than 0.02) and difference between mean right and left atrial pressures (1.0 versus 4.2 mm Hg, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

A Simple, Safe, and Rapid Technique for the Management of Recurrent Coarctation of the Aorta

Abstract Six patients treated in infancy for coarctation of the aorta developed severe stenosis of the end-to-end aortic anastomosis that produced marked collateral circulation and upper extremity hypertension. Operation relieved the anastomotic obstruction in all 6 patients. A resection of the stenotic anastomosis with end-to-end aortic anastomosis was performed in 2 patients, bypass grafting between the transverse aortic arch and the distal aorta was done in 3 patients, and bypass grafting between the enlarged left subclavian artery and the distal aorta was performed in 1 patient. Interposed graft segments were short and had a diameter equal to or greater than the diameter of the aorta above or below the stenosis. Insertion of such short graft segments over an anastomotic obstruction provides an easy, rapid, and safe method for relieving hypertension and restoring distal pulsatile blood flow. Bypass grafting should be reserved for patients in whom anatomical considerations present an unusual hazard for a second aortic resection.

Evaluation of pulmonary arterial morphology in cyanotic congenital heart disease by magnetic resonance imaging

Before and after surgical therapy, the anatomy of the pulmonary arteries in cyanotic congenital heart disease is often distorted. Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI) and angiography in 20 patients, ages 3 months to 20 years, with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r = 0.96; right pulmonary artery, r = 0.93; left pulmonary artery, r = 0.96). A similar excellent correlation (kappa = 0.83) was found in the assessment of the presence and severity of proximal pulmonary arterial stenoses. However, stenoses in the peripheral pulmonary arteries visualized with angiography were missed with MRI. MRI and angiography showed complete agreement in determining the patency of 11 surgical shunts. MRI did not demonstrate all of the systemic collateral vessels present with angiography, and the distal connections of collaterals were not detected with MRI. MRI is comparable to angiography in the evaluation of central pulmonary arterial anatomy over a wide range of ages. These findings suggest an important role for noninvasive MRI in the serial evaluation of pulmonary artery morphology in patients with cyanotic congenital heart disease before and after surgical repair.

Salicylate intoxication in infants and children

Summary 1. Five cases of salicylate intoxication are presented. 2. In four of the five cases the intoxication occurred during therapeuticadministration of the salicyl compound. 3. Unusually large quantities of alkali may be required to correct the acidosis of salicylate intoxication because of the intense and persistent ketosis. 4. The importance of glycogen depletion specifically associated with the increased metabolic activity due to salicylates in the development of ketosis and acidosis is suggested, and the effect of this on prevention and treatment of the intoxication is discussed.

Diagnosis of pulmonary arteriovenous fistula by contrast echocardiography

That the anatomic substrate of the Wolff-ParkinsonWhite syndrome can be inherited without the ECG evidence is illustrated by Family 1. It is likely that the other three families also represent examples of this phenomenon. These findings explain why previous genetic studies have been inconclusive. It was assumed, erroneously, that many subjects did not have Kent bundles when, in fact, they did. It will be difficult to overcome this problem since at present, cardiac catheterization is necessary to find Kent bundles which conduct only in the retrograde direction. If all subjects with PSVT are assumed to have Kent bundles, then false positives may be included since some PSVT might be due to other mechanisms. The mechanism of inheritance of Kent bundles seems to most closely follow that of autosomal dominant with variable expression. This paper has identified one mechanism of the variability. Another might be a patient with a retrograde conducting bundle of Kent who never has PSVT. Orinious et al e reported results which support ours in his 20-year follow-up of 150 members of two families with WPW. Although he found only two additional patients with WPW, he found 17 new examples of SVT, eight of Which were in parent-child combinations. In a patient with PSVT without WPW who has a family member with WPW, a concealed bundle of Kent should be suspected. This finding has obvious therapeutic implications, since patients With concealed bundles of Kent may respond to different medical treatment or may be cured by surgical division of the anomalous pathway. r

Relationship between clinical status and behavior test performance in a newborn group with histories suggesting anoxia

Summary A battery of five behavioral tests designed and standardized for newborn infants was administered to a group of sixty “anoxic” infants, including those with observable postnatal anoxia and those with possible prenatal anoxia, and to a group of sixty-two normal infants. The five tests consisted of a pain threshold test, a maturation scale, a vision scale, an irritability rating, and a muscle tension rating. The degree of impairment in test performance reflected the seriousness of the clinical condition, both when the clinical history was evaluated by two experienced pediatricians and when it was estimated by a method of weighting multiple factors in the medical history. The behavioral tests thus appear to provide an objective, quantitative, and sensitive method of assessing the clinical status of a newborn infant.

Diagnosis of congenital absence of left pericardium by MR imaging.

Congenital absence of the pericardium, whether partial or total, is a rare abnormality. It can be very difficult to diagnose clinically as well as to confirm radiographically. Plain film fluoroscopy and CT have been shown to be of value in differentiating this entity from others with similar findings. We report a case in which magnetic resonance was utilized to confirm the diagnosis and the findings of this new technique are described.

Anoxia as a significant perinatal experience: A critique

Summary In this paper we have reviewed major findings from several studies designed to test the hypothesis that anoxia is a significant perinatal experience. Several design factors which may help to account for the discordant conclusions arrived at by various investigators were pointed out. These were: approach to the problem (prospective or retrospective), criteria of later impairment, use of control groups, and criterion of early anoxia. It is the factor of definition of early anoxia with which we have been especially concerned in this and earlier studies of neonates. On the basis of these studies, several conclusions and suggestions emerged. First, blood oxygen saturation appears to reflect very transitory levels of oxygenation. Since it is not necessarily closely related to even the immediate neonatal condition, it would not be surprising if there were no relationship with more remote events. It seems necessary, consequently, to employ a less exact but more meaningful clinical criterion of anoxia. Second, even though a clinical criterion is used, it should permit some ordering as to degree in addition to satisfying the usual scientific demands for objectivity and reliability. Third, there is considerable variability in the postnatal rate of recovery of anoxic infants. Measurement of these differences provides a way of identifying those infants who may be most likely to show residual effects. Fourth, events believed to lead to fetal anoxia were associated, in our subjects, with relatively greater impairment in performance on Newborn Behavior Tests than either postnatal anoxia or nonmechanical delivery complications. This finding highlights the necessity of controlling for fetal factors in any research designed to assay the effects of anoxia.

Lactate metabolism: Studies of a child with a serious congenital deviation

After a short summary of experiences with sodium lactate in the treatment of metabolic acidosis over a 30 year period, we present clinical and metabolic findings in a child born with a serious defect in the metabolism of lactate. The consequences were a constant threat of severe and perhaps fatal acidosis and tetany-like episodes leading to severe respiratory obstruction on the basis of bronchiolar muscle spasm and sometimes laryngeal spasm which would superimpose severe respiratory acidosis on an already severe metabolic acidosis. It is our opinion that no such case has previously been described.