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Biomedical subjects

J H Moller

Publications and source records attributed to J H Moller.

At least 19 recordsLinked to original sources

1,000 consecutive children with a cardiac malformation with 26- to 37-year follow-up.

The current status of 997 of 1,000 consecutive children with a cardiac malformation initially evaluated between 1952 and 1963 was determined. Of the 1000, 285 have died and the survivors have been followed up for periods of 26 to 37 years. Six hundred thirty-two are in excellent or good clinical condition, being asymptomatic and without planned need for further treatment. The other 80 have significant abnormalities, although 63 of these have few symptoms. Infective endocarditis occurred in 12 of 10,000 susceptible patient-years, with a lower rate in the past decade. Only 22 of the survivors are currently receiving cardiac medication. These data are derived from a group of patients initially seen during a period of time that cardiac surgery was being developed for congenital heart disease. Thus, the outlook should be even better for children who are currently undergoing treatment.

Arrhythmias, Cardiac

Pulmonary circulation in pulmonary atresia associated with the asplenia cardiac syndrome.

OBJECTIVE: The goal of this study was to determine the patterns of the pulmonary circulation in patients with pulmonary atresia and asplenia. BACKGROUND: The asplenic cardiac syndromes characteristically have complex cardiac anomalies including pulmonary stenosis or atresia. Definition of the pulmonary artery circulation and pulmonary venous connections is needed for consideration of surgical procedures. METHODS: In 35 patients, the sources of pulmonary blood flow, anatomic features of pulmonary arteries and pulmonary venous connections were determined from angiograms or autopsy specimens. RESULTS: The main pulmonary artery was absent or hypoplastic in 91% of patients; most had a ductus arteriosus. The right and left pulmonary arteries were confluent in 90% and usually of normal size (right 71%, left 63%). Total anomalous pulmonary venous connections were present in 38%. CONCLUSIONS: The anatomic features of the pulmonary arteries in pulmonary atresia associated with the asplenic cardiac syndrome are usually favorable for palliative surgical procedures. Total anomalous pulmonary venous connection may exist as a complicating factor.

Child

Late results (30 to 35 years) after operative closure of isolated ventricular septal defect from 1954 to 1960.

This study was designed to determine the clinical status, cause of death, and effects of pulmonary vascular disease and conduction abnormalities 30 to 35 years after surgery in 296 consecutive surviving patients of closure of ventricular septal defect. Of the 296 patients, current status was determined by contact with patient and physician in 290 cases, with 6 (2%) lost to follow-up (7,912 patient years are included). Cardiac catheterization after surgery in 168 patients showed complete closure of the defect in 80%. Death occurred in 59 patients (20%), with higher mortality rates in those operated on after the age of 5 years, those with pulmonary vascular resistance greater than 7 units (51%), and those with complete heart block (78%). Of 37 patients with transient heart block after surgery, 8 (22%) have died (3 pulmonary vascular disease, 2 sudden death, 2 unknown causes and 1 complete heart block). Twenty other patients had a dysarrhythmia after surgery, and none of these died. Nine episodes of endocarditis occurred (11.4/10,000 patient years). Nine of 296 (3%) offspring had cardiac malformation. Most patients are in New York Heart Association class I, 57% attended college and 15% received an advanced degree. The data show good results for this group of patients operated on during an early era (1954 to 1960) of open cardiac surgery. They support the current trend toward operation in patients with ventricular septal defects at an early age and with low pulmonary vascular resistance.

Adult

Forty years of cardiac disease in children. Progress and problems--first of three parts.

We review the major advances that have occurred in the diagnosis and treatment of cardiac diseases in children during the last four decades. During this period, most forms of congenital heart disease have become treatable by operation, and diagnosis has become possible through echocardiography alone. Operative mortality has dropped, and long-term follow-up shows excellent results in most patients. The incidence of rheumatic fever has declined, although it has increased recently in several areas of the United States. The occurrence of infective endocarditis and of myopericarditis has remained stable, while the recently described Kawasaki disease can affect the coronary arteries in children and appears to represent a new condition.

Adolescent

The Pediatric Cardiac Care Consortium: a physician-managed clinical review program.

A voluntary physician-initiated and physician-managed clinical review program for pediatric cardiology uses clinical practice data and expected outcome to influence physicians to change their clinical behavior. The program offers a model for clinical review in situations where disease incidence is relatively low and physician performance is difficult to assess and evaluate. The program's major accomplishment is a collective pooling of data across cardiac centers that allows for statistical analysis and comparison not routinely possible at a single center because of sample size; a forum for comparison of different practice styles; and feedback to participants of actual, in contrast to anecdotal, results. The principles, structure, and format of the program can be applied to other specialty areas.

Cardiac Care Facilities

Pediatric cardiac care consortium. Demonstrated value of a physician-directed quality assessment system.

A quality assessment system for analyzing the results of inpatient care of children with cardiac abnormalities is described. Three Minnesota institutions (the Mayo Clinic, Minneapolis Children's Medical Center, and the University of Minnesota) participate with 15 other similar centers elsewhere in the United States in this system. Patient data are analyzed and adjusted for case mix to allow for differences in patient populations and referral patterns. The statistical methods used are shown for two conditions: ventricular septal defect and aortic valvotomy. Data presented show reduction in length of stay for cardiac catheterization. This change occurred as physicians annually reviewed and discussed the data and, as a result, instituted changes. This physician-directed program seeks improvement in patient care through physician education.

Aortic Valve

Cardiac malformations in relatives of children with truncus arteriosus or interruption of the aortic arch.

Familial recurrence risks for congenital cardiac malformations have been estimated at 1 to 4%, but little is known of recurrence risks for uncommon congenital cardiac malformations. This study determined the incidence of congenital cardiac malformations in relatives of patients with either truncus arteriosus (TA) or interruption of the aortic arch (IAA) seen between 1955 and 1985. Prior to this study, only 2 cases of IAA in siblings had been reported and the recurrence risk of TA had been estimated at 1.2%. In the families of 36 index cases of IAA, there were 98 siblings, 2 with congenital cardiac malformations (2.1%). All recurrences were in IAA type B families. Single cases of congenital cardiac malformations were found in second- and third-degree relatives and 2 cases in more distant relatives. In the families of 49 index cases of TA, there were 106 siblings, 7 with congenital cardiac malformations (6.6%). Two had TA, 3 had other conotruncal anomalies and 2 had other congenital cardiac malformations. Two parents had congenital cardiac malformations, 1 with TA and 1 with conotruncal malformation. Among second- and third-degree relatives, 7 had congenital cardiac malformations (5 were conotruncal, including 1 half sibling). Among greater than third-degree relatives, 3 had congenital cardiac malformations. Thus, in IAA, the recurrence rate of congenital cardiac malformations (2.1%) was higher than expected and could be related to the etiology of IAA type B as a conotruncal malformation. In TA, the recurrence rate of congenital cardiac malformations was 6.6%, higher than previously reported. When TA was complex the recurrence risk was higher (13.6%). These findings must be taken into consideration for satisfactory genetic counseling.

Adult

15- to 30-year follow-up of patients undergoing repair of ruptured congenital aneurysms of the sinus of Valsalva.

Rupture of a congenital aneurysm of the sinus of Valsalva is a rare congenital cardiac malformation. Between 1956 and 1971, we operated on 14 patients aged 9 to 36 years (median, 20 years) for repair of a ruptured aneurysm of the sinus of Valsalva, and have followed each patient to the present. Two operative and 4 late deaths occurred, 3 following a second cardiac operation and the other from dysrhythmia. Late complications have included development of complete heart block in 2 patients (necessitating permanent pacemaker insertion 11 and 24 years after initial repair), progression of aortic regurgitation in 2 (necessitating valve replacement 9 and 13 years after initial repair), and endocarditis in 1 patient 20 years after repair. Four of the 8 remaining long-term survivors (followed for 15 to 30 years [mean, 23.6 years]) are in New York Heart Association (NYHA) Class I, and the other 4 are in NYHA Class II. Three of the 5 patients with suture closure (no pledgets or adjacent ventricular septal defect repair) of the ruptured aneurysm of the sinus of Valsalva sustained recurrent rupture and required repeat closure. Whether the lack of prosthetic material to bolster the repair or inadequate resection of redundant aneurysmal fibrous tissue was responsible for these recurrences cannot be stated. Operative management of patients with ruptured congenital aneurysms of the sinus of Valsalva is discussed.

Adolescent

Comparison of late clinical status between patients with different hemodynamic findings after repair of tetralogy of Fallot.

Long-term results and postoperative hemodynamic findings were reviewed in 144 cases of tetralogy of Fallot. A correlation was found between the late clinical status of patients and the postoperative hemodynamic findings. The mortality in our series of patients followed for 10 years was 6.25%. Each of the deaths occurred in the group with unsatisfactory postoperative hemodynamic findings. An ideal late clinical result can be anticipated in most patients with excellent or satisfactory postoperative hemodynamic findings. The greater the deviation from normal hemodynamic findings, the worse the prognosis. From the excellent group to the unsatisfactory group, the incidence of late ideal result decreased from 95% to 61.1%, while the incidence of poor result increased from 0% to 27.8%. Among various factors in patients in the unsatisfactory hemodynamic group, right ventricular outflow tract obstruction seems to have the worst late result. A large right ventricular-pulmonary artery pressure gradient was noted in three of four who died suddenly. The late result of patients with a small shunt (pulmonary blood flow/systemic blood flow less than 2) seems acceptable; the decision to reoperate in these patients should be made very cautiously.

Child

Long-term evaluation of aortic valvotomy for congenital aortic stenosis.

Aortic valvotomy (AV) for relief of congenital aortic stenosis (AS) is a palliative operation, and the purpose of this study was to determine the long-term benefit. After analyzing the symptoms at time of operation, 177 patients were separated by age at AV: newborns (1-14 days) 26, infants (2 weeks-1 year) 19, and children (greater than or equal to 1 year) 132. The newborn with critical AS typically presents with severe cardiac failure and the infant with moderate failure, whereas children may be asymptomatic. In the newborn group, final diagnosis has been made exclusively by noninvasive cardiac echogram since 1984. The presence of a hypoplastic left ventricle (HLV) and/or endocardial fibroelastosis (EFE) are the most important determinants of mortality. Operative survival was 11 of 13 (85%) in the newborn group if HLV/EFE were not present. No operative deaths for AV occurred after 1965 in children. When present, a preoperative electrocardiographic strain pattern resolved in 80% of patients. Of 33 reoperations (21%), four were required 1.0 (0.5-2) years later for newborns, five were required for infants 7.2 (1.5-20) years later, and 24 were required in children 11.0 (1.5-25) years later. These included 10 repeat valvotomies and 23 aortic valve replacements (AVRs) with a 91% survival rate. In summary, although valves of patients with congenital AS are morphologically abnormal, valvotomy is beneficial. It was found that (1) the operative survival rate has been 100% over the past 20 years for children and currently is greater than 80% in newborns in the absence of HLV/EFE; (2) reoperation rate is higher in newborns and infants; (3) benefit was shown by a sharp decrease in obstruction initially and improved ECG later; (4) long-term follow-up revealed a subset of patients who are asymptomatic and have only mild residual obstruction over 20 years after AV.

Actuarial Analysis

Congenital unilateral pulmonary venous atresia with pulmonary veno-occlusive disease in contralateral lung: an unusual association.

A patient presenting with the rare association of congenital unilateral pulmonary venous atresia in one lung and pulmonary veno-occlusive disease in the other is described. The patient first presented at the age of 3 1/2 years with anemia, hemoptysis, and pulmonary hypertension. After cardiac catheterization and angiocardiography, a diagnosis of atresia of the left pulmonary veins was made for which left pneumonectomy was done. Four years later the patient presented with right-sided congestive failure and radiologic evidence of right-sided pulmonary edema from which death resulted. At autopsy, the right lung showed changes of pulmonary veno-occlusive disease, while the major veins were not involved.

Child

Single papillary muscle of the left ventricle associated with persistent common atrioventricular canal: variant of parachute mitral valve.

Six cases of single papillary muscle of the left ventricle in persistent common atrioventricular canal (AVC) are described. Except for one case with double outlet right ventricle and AVC, in the other five cases AVC occurred with normally related great vessels. Down's syndrome was present in two cases. The mitral valvular condition in AVC is not the classic parachute mitral valve since the separation between the common anterior and the common posterior leaflet leaves a space (cleft) oriented toward the ventricular septum. This mitral valve in AVC may or may not be obstructive. The common anterior leaflet fixed to the ventricular septum may result in obstruction. Clinically, single left ventricular papillary muscle should be suspected if the AVC is associated with coarctation of the aorta or tubular hypoplasia of the aortic arch. This type of mitral valve in AVC may also be associated with double outlet right ventricle. Preoperative recognition of the single papillary muscle by echocardiogram or left ventriculogram will prevent postoperative mitral obstruction following closure of the mitral cleft.

Aortic Coarctation

Pediatric cardiology manpower in the 1980s.

The allocation of professional time of board-certified pediatric cardiologists in 1980 to 1981 was compared with that of pediatric cardiologists in 1967, by use of a similar questionnaire. At both times, the majority of pediatric cardiologists were in a full-time academic or hospital setting. In 1980 to 1981, the number of hours devoted to professional activities was significantly less than during the earlier period, with the reduction being principally in amount of time devoted to research, either clinical or basic, and teaching. This change has implications for manpower and the scientific aspects of pediatric cardiology. An average of 40 physicians enter pediatric cardiology yearly. With the current number of certified pediatric cardiologists, and a modest number of pediatric cardiologists who will leave the field in the next half decade, we project 900 pediatric cardiologists in 1990. A number of factors are discussed that will influence the needs for pediatric cardiologists.

Cardiology

The first open-heart repairs of ventricular septal defect, atrioventricular communis, and tetralogy of Fallot using extracorporeal circulation by cross-circulation: a 30-year follow-up.

From March 26, 1954, to July 19, 1955, 45 patients with major cardiac malformations not previously correctable underwent open repair utilizing cross-circulation between patient and donor without donor deaths. All operations were carried out at normothermia with lowered flow rates based on azygos flow studies. Twenty-seven patients, more than half of them infants, had ventricular septal defects closed. There were 8 hospital deaths, and there have been only 2 late deaths in 30 years. Fourteen (87.5%) of 16 who underwent recatheterization have closed defects. The 17 30-year-survivors are all in New York Heart Association Functional Class I. Five patients 4 months to 10 years old were operated on for atrioventricular canal (complete form). All had intractable failure, and 4 had pulmonary hypertension. Two of the 3 hospital deaths were due to heart block. The long-term survivor, a 15-month-old infant at the time of operation (severe pulmonary hypertension, 90/50 mm Hg), underwent repair 31 years ago and is now married with 3 children. Recatheterization disclosed normal pulmonary pressure (20/4 mm Hg), no shunts, and mild mitral regurgitation. Ten cyanotic tetrads 13 months to 14 years old were operated on with 5 hospital deaths. Of the 3 late deaths, 1 was accidental at 17 years, 1 occurred suddenly at home 13 years after operation in infancy for atresia, and the third occurred at reoperation 10 years later. The 2 remaining patients (1 the first patient operated on) are in excellent health. The surgical methods used and the physiological advantages of cross-circulation (temporary placenta) that made these results possible at a time when surgical knowledge was primitive are described.

Cardiac Catheterization

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