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Biomedical subjects

J H Moller

Publications and source records attributed to J H Moller.

At least 55 records · Page 3Linked to original sources

Predictive value of lung biopsy in ventricular septal defect: long-term follow-up.

Although the Heath-Edwards classification has been used for more than 25 years to evaluate pulmonary vascular changes, its potential to predict the long-term course after successful ventricular septal defect closure has not been proved. Operative lung biopsy slides obtained at the time of closure from 57 infants and children who had been among the first survivors (between 1954 and 1960) of such surgery were graded in blinded fashion according to the Heath-Edwards classification system, and the resultant biopsy grade of each was compared with the eventual long-term outcome of each child. In 53 (93%) of the 57 cases, the Heath-Edwards system correlated well with long-term clinical or hemodynamic status of the patient. Grade IV changes were predictive of a usually fatal outcome, while grade I and II changes were generally benign. Death from pulmonary vascular disease occurred, however, in four children whose operative lung biopsy had been classified as either grade I or II, indicating that the focal nature of the higher Heath-Edwards grades, or human error, must be considered when this classification system is employed.

Adolescent↗

The first open heart corrections of tetralogy of Fallot. A 26-31 year follow-up of 106 patients.

Tetralogy of Fallot became a correctable malformation on August 31, 1954, and from that data through 1960, 106 patients (ages 4 months-45 years) who underwent open repairs at the University of Minnesota and were discharged, have been followed (99% complete) until death or for 26-31 years (mean: 23.7 years, 2424 patient years). The purposes of this study were to determine survival, morbidity, hemodynamics, educational/employment attainments, and relation of these to surgical technics. Operations were done by cross circulation (6 patients) and bubble oxygenator (100 patients). This group had the first uses of patch ventricular septal defect closure, outflow root, infundibuloplasty, atresia correction, ischemic arrests, and pacemakers among other innovations. Twenty-one (of 105 patients) have died during the followup: eight deaths in the first 10 years, 12 between 10 and 20 years, and 1 greater than 20 years. The causes of death were sudden (5), accidental (4), congestive failure (2), reoperation (2), suicide (2), and other (2). Actuarial survival at 30 years was 77%. Late complications were ten reoperations, five arrhythmias, and one endocarditis. Actuarial freedom from reoperations at 30 years was 91%. Cardiac recatheterizations in 62 patients disclosed only 10 with residual shunts. Peak right ventricular systolic pressures were less than 40 mmHg (34 patients), 41-60 mm (2 patients), 61-70 mm (4 patients), greater than 71 mm (4 patients). Thirty-four patients (32%) completed college, ten of these completed graduate school (5 masters degrees, 2 M.D.'s, 2 Ph.D.'s, 1 lawyer). Fifteen others attended college, and nine received technical school diplomas. Forty patients (18 men, 22 women) had progeny, with 82 (93%) live births and six major cardiac defects (7.3%). In summary, complete repair gave excellent late results in this group cared for very early in the open heart era. Survivors led productive lives without restrictions in education and employment. Many of the deaths/complications that occurred are now easily preventable, which augurs extremely well for this generation.

Adolescent↗

Accessory tissue of the tricuspid valve protruding into the left ventricle through a septal defect.

We report the pathologic findings in 13 cases with accessory tissue originating from the tricuspid valve and protruding into the left ventricular outflow tract through a ventricular septal defect (VSD). In eight cases the accessory tissue formed a pouch, the walls of which were similar to the tissue of the normal tricuspid valve. In five cases, papillarylike masses of young connective tissue formed the accessory tissue. The degree of left ventricular outflow tract obstruction was mild in five cases, intermediate in five, and severe in three. Adhesions to the rims of the VSD causing obstruction of the VSD were seen in ten cases. Associated anomalies were present in all cases. The most frequent associated anomalies other than the tricuspid valvular anomalies and the VSDs were transposition of the great arteries and a variety of vascular anomalies.

Child, Preschool↗

Management of pulmonary atresia with intact ventricular septum.

Infants with pulmonary atresia and intact ventricular septum pose a difficult clinical problem. Pulmonary valvotomy has been widely recommended for relief of the right ventricular obstruction, and most infants also have had an aortopulmonary shunt placed to ensure pulmonary blood flow. We have evolved a different approach that includes placement of a right ventricular outflow tract patch initially and continuation of prostaglandin E1 infusion postoperatively until the need for a shunt can be determined. We report here our management of 15 neonates with this diagnosis and suprasystemic right ventricular pressures. All of the infants were placed on a regimen of prostaglandin E1 before the operation to improve pulmonary blood flow, and all had an outflow patch placed early in life. Satisfactory postoperative right ventricular function, which would allow both outflow patching and ductus ligation, could be confidently predicted in only two of the 15 patients. For nine of the 15 an outflow patch was placed and prostaglandin was infused postoperatively to provide pulmonary blood flow until right ventricular function became adequate. Early in the series, three other infants were judged to need an aortopulmonary shunt in addition to decompression by an outflow patch, and one infant had only a shunt placed. Postoperatively, adequate pulmonary blood flow was present in all, and 11 of the 15 (73%) survived. Three of the deaths (average 2.8 days) after the outflow patch operation probably resulted from premature cessation of the prostaglandin infusion. One neonate with an outflow patch and a shunt died of myocardial ischemia because of coronary artery steal through right ventricular sinusoids. One late death occurred suddenly in the child with only a shunt, presumably because of an arrhythmia. The remaining survivors (10/15, 67%) are alive and have had complete repair. Study of these patients has also revealed that the definition of adequate right ventricular size needs to be more liberal. Five of the 10 surviving patients had a residual atrial septal defect with a right-to-left shunt at the ages of 1 to 3 years, but balloon occlusion of the atrial septal defect during cardiac catheterization revealed that the right ventricle in these patients was functionally adequate. These five children subsequently underwent closure of the atrial septal defect, and in two the aortopulmonary shunt was also taken down. In summary, correction was achieved in all survivors, in contrast to reported studies in which many patients are living with shunts.(ABSTRACT TRUNCATED AT 400 WORDS)

Angiography↗

Rest and exercise hemodynamics in pulmonary stenosis: comparison of children and adults.

To better understand the hemodynamics of pulmonary stenosis (PS), 24 adults and 53 children with similar degrees of PS who had undergone cardiac catheterization at rest and during supine exercise were retrospectively studied. Three groups were defined. Group I consisted of 9 adults and 18 children with a pulmonary valve area of less than 0.5 cm2/m2; group II, 6 adults and 25 children with a pulmonary valve area of 0.5 to 1.0 cm2/m2; and group III, 9 adults and 10 children with pulmonary valve area of more than 1.0 cm2/m2. The mean ages of the adults were 29, 26 and 22 years for groups I, II, and III, respectively. The mean ages of the children were 11, 10 and 9 years for groups I, II and III, respectively. The pertinent data collected from catheterization included oxygen consumption, cardiac rate and index, arterial venous oxygen difference, stroke index, right ventricular (RV) systolic pressure and RV end-diastolic pressure. Adults and children in groups II and III had an appropriate response to exercise. Group I children responded abnormally by increasing their RV end-diastolic pressure and decreasing their stroke index. In group I adults both of these variables increased. Group I adults exhibited a significantly lower cardiac index at rest and exercise secondary to a significantly lower absolute cardiac rate. Long-standing severe PS results in hemodynamic compromise. Hence, early relief of PS is recommended.

Adolescent↗

Small ventricular septal defect associated with severe pulmonary hypertension.

A small ventricular septal defect in an 18 year old man was shown by serial cardiac catheterisation to be associated with a progressive increase in pulmonary arterial pressure. Surgical closure of the defect had no effect on the increased pulmonary arterial pressure. Whether pulmonary hypertension occurred secondary to the haemodynamics of the ventricular septal defect or as an independent event is uncertain.

Adolescent↗

Exercise induced pulmonary vasoconstriction.

Pulmonary vascular resistance normally falls or remains unchanged during exercise. Seven children with pulmonary hypertension were exercised during cardiac catheterisation after operative correction of ventricular septal defect (6) and truncus arteriosus (1). Except for the presence of moderate pulmonary hypertension, resting haemodynamics in these seven children were similar to those of normal children of equal age, but during exercise the postoperative patients showed a rise rather than a fall (+2% vs -18%) in total pulmonary vascular resistance. Two of the seven children had a substantial increase in pulmonary arteriolar resistance during exercise (from 509 to 715 dyne s cm-5 in one patient and from 606 to 828 dyne s cm-5 in the other). These two patients did not differ from normal children in respect of arterial or mixed venous oxygen saturations or of pH with exercise, nor was left atrial pressure related to the rise in pulmonary resistance. These two patients, however, had only a small rise in cardiac output during exercise (6.8% and 43.1%) in spite of a substantial increase in oxygen consumption (121% and 373%). One of the patients with exercise-induced pulmonary vasoconstriction had an 82% increase in resting pulmonary vascular resistance over a five year period subsequent to her first exercise study. Analysis of these data, and those previously reported, suggests that exercise induced pulmonary vasoconstriction may occur in 10 to 25% of patients who survive correction of certain congenital cardiac defects. The vasoconstriction cannot be attributed to abnormal changes in blood gases or left atrial pressure, and may be an early sign of progressive pulmonary hypertension.

Adolescent↗

The pulmonary vascular response to oxygen and its influence on operative results in children with ventricular septal defect.

The ratio of pulmonary vascular resistance to systemic resistance was determined before and after oxygen administration in 25 children with an isolated large ventricular septal defect and elevated pulmonary vascular resistance (pulmonary/systemic resistance ratio, Rp/Rs greater than 0.25). A fall of 30% or more in the Rp/Rs, after 20 minutes of oxygen inhalation (FiO2 greater than 0.90), was considered a positive response to oxygen. In the 6 children with Down's syndrome, the Rp/Rs fell significantly more in oxygen (52%) than it did in the 19 children without Down's syndrome (31%, p less than 0.05). No other clinical or baseline hemodynamic finding was predictive of vascular responsiveness. Seventeen children underwent closure of the ventricular septal defect. Three of 11 children who responded to O2 expired shortly after operation; each of the 6 children who did not respond to O2 survived operation. Two of the deaths (occurring less than 12 hours after closure) were from low cardiac output; a third child died suddenly 5 days postoperatively. Thirteen children, 7 who responded to oxygen and 6 who did not, were restudied by cardiac catheterization an average of 2.0 years following successful closure of the ventricular septal defect. While the Rp/Rs in room air fell following closure of the defect in both groups, the decrease was 22% in responders and 28% in nonresponders. The results of this study, unlike similar studies performed at higher altitudes, suggest that the preoperative responsiveness of the pulmonary vasculature to oxygen does not predict either operative survival or postoperative pulmonary vascular resistance.

Child↗

Residual right-to-left atrial shunt following surgical relief of pulmonary valvar stenosis.

Of 264 patients undergoing pulmonary valvotomy for pulmonary stenosis or atresia at the University of Minnesota, 23 had a right-to-left shunt preoperatively. In none was the atrial communication closed at the time of pulmonary valvotomy. At postoperative cardiac catheterization, no atrial level shunt was found in 19, indicating that effective closure of the communication had occurred. In the remaining 4, a right-to-left shunt persisted despite adequate relief of the stenosis. Each of the 4 has undergone subsequent closure of the atrial septal communication without difficulty.

Adolescent↗

Left-sided anomalies in Ebstein's malformation of the tricuspid valve.

A review of 34 autopsied cases with classical Ebstein's malformation of the tricuspid valves revealed 8 cases with left-sided anomalies. Among these, appearing in 1 case each, were aortic atresia and persistent common atrioventricular canal. The latter 2 conditions were dominant clinically. In the remaining 6 cases the left-sided anomalies were not apparent clinically and probably of no functional significance. These conditions were parachute mitral valve, bicuspid aortic valve, cor triatriatum and pulmonary stenosis, cleft mitral valve, stenosis of individual pulmonary veins, and prolapse of mitral valve.

Adolescent↗

Interruption of the aortic arch with right descending aorta. A rare condition and a cause of bronchial compression.

Four cases of interruption of the aortic arch with the rare association of right ductus arteriosus and right descending aorta are described. Three different variations in subclavian arterial origin are described. Each represents a mirror image of anatomic arrangements previously reported for interruption of the aortic arch with left descending aorta. One of the cases demonstrated massive emphysema of the right lung resulting from compression of the right main bronchus by the enlarged right ductus arteriosus. Another case documented unusually long survival (26 years) of a patient with interruption of the aortic arch and ventricular septal defect.

Adult↗

Congenital cardiac, pulmonary, and vascular malformations in oculoauriculovertebral dysplasia.

Three cases of oculoauriculovertebral dysplasia (OAV) with associated cardiac, pulmonary, and facial malformations are described. One patient had a rare condition: isolation of the left innominate artery. All 3 had pulmonary anomalies ranging from minor (incomplete lobulation) to major (hypoplasia of the left lung). When pulmonary hypoplasia occurs, it is usually on the same side of the body as the facial anomalies. Forty-five reported cases of OAV with associated cardiac, vascular, and pulmonary abnormalities are reviewed. Tetralogy of Fallot and ventricular septal defect account for half of the cardiac malformations. In the remaining cases, a wide variety of cardiovascular malformations are present. It is suggested that the facial malformations in OAV may result from a perfusion abnormality. An isolated left innominate artery (as in one of our cases) could produce abnormal vascular supply to the external carotid artery and the facial structures that it supplies.

Arteriovenous Malformations↗

Multimethod study of clinical judgment.

Expert and novice clinicians judged the likelihood of disease alternatives and gave thinking-aloud protocols as they evaluated simulated cases of congenital heart disease. Specific combinations of cues in the patient data were manipulated to create alternate versions of a single case so that the use of critical cues could be identified. Analyses of variance of subjects' disease judgments revealed differences between expert and novice clinicians in their use of critical cues and cue combinations. Analyses of the thinking-aloud protocols revealed that clinicians with different degrees of expertise employed different interpretations of patient data cues as well as qualitatively distinct "lines of reasoning" in reaching clinical judgments.

Analysis of Variance↗

Angio-pathological appearances of pulmonary valve in pulmonary atresia with intact ventricular septum. Interpretation of nature of right ventricle from pulmonary angiography.

Correlative angiographic-anatomical studies in 19 cases of pulmonary atresia with infarct ventricular septum showed the following relations between the angiographic appearance of the pulmonary valve and the morphology of the right ventricle. (1) Doming of the pulmonary valve was associated with a nearly normal-sized right ventricle and a wide infundibulum patent to the level of the pulmonary valve. (2) A fixed valve was associated either with (a) pronounced hypoplasia of the ventricular changer and stenosis of the infundibulum or (b) less commonly, a massive right ventricle and Ebstein's malformation of the tricuspid valve. (3) An intermediate type valve was associated with a small right ventricle and a small infundibulum which was, however, patent to the level of the pulmonary valve. It is suggested that the configuration of the pulmonary valve is a result of haemodynamic stresses placed upon it. These stresses, in turn, are determined by the morphological nature of the right ventricle. Thus, the nature of the pulmonary valve as seen angiographically may ve used as an index of right ventricular morphology.

Heart Defects, Congenital↗