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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

Echocardiographic screening to assess the severity of congenital aortic valve stenosis in children.

Recent reports suggest that peak left ventricular systolic pressure can be estimated from the echocardiogram. This study compares the accuracy of echocardiographic measurements with clinical estimates of severity of aortic stenosis derived from information of the Joint Natural History Study. Twenty-seven children with isolated aortic valve stensosis were evaluated clinically and with echocardiography and cardiac catheterization. From the echocardiograms, the ratio of systolic left ventricular wall thickness to internal dimension (Ws/Ds) showed a better correlation (r = 0.82) with peak left ventricular systolic pressure than did the diastolic ratio. However, there was significant variation so that a precise estimate of peak left ventricular systolic pressure could not be made from the echocardiogram. Eight of the 27 patients had a normal Ws/Ds ratio (0.55 or less), and their cardiac catheterization data revealed mild aortic stenosis not requiring operation. In contrast, clinical criteria based on the Natural History Study identified only three of the patients with mild aortic stenosis. Prospective studies are needed to confirm the usefulness of echocardiography in noninvasive evaluation of children with aortic valve stenosis.

Adolescent

Pulmonary valve replacement with a porcine aortic heterograft.

Pulmonary valve replacement is an uncommon surgical procedure, yet lifesaving when performed under the appropriate circumstances. The patient reported on here had the classic indications for valve replacement: increased pulmonary vascular resistance and associated pulmonary valve incompetence following repair of a congenital heart defect resulting in right heart failure and secondary tricuspid insufficiency. A review of the literature provides the basis for the decision to replace the pulmonary valve with a glutaraldehyde-preserved porcine heterograft.

Animals

Pulmonary artery banding: long-term results in 63 patients.

Sixty-three patients who underwent banding of the pulmonary artery between 1968 and 1975 were studied. Mortality among patients who underwent the banding procedure was 22%, and among those with ventricular septal defect it was 7%. Thirty-eight of the 49 survivors of the banding procedure were investigated for abnormalities of the pulmonary outflow tract caused by the band. Seventy-one percent of these 38 patients had an identifiable abnormality of the pulmonary valve or artery. These complications occurred more frequently in patients banded at a very young age (less than 2 months old) and in patients in whom the band was in place more than two years. Corrective operations have been performed in 24 of the 49 patients who survived banding. Seventy-six percent of the patients with ventricular septal defect survived corrective operation, while only 29% of the patients with more complex lesions survived.

Age Factors

Postoperative haemodynamics in tetralogy of Fallot. A study of 132 children.

Our study was undertaken to determine the type, incidence, and functional significance of residual anomalies in patients who have undergone corrective repair for tetralogy of Fallot. We reviewed data from cardiac catheterisations performed on 132 survivors. A significant residual ventricular septal defect was present in only 12 patients. Resting right ventricular systolic pressure was less than 80 mmHg in 100 patients and ranged from 80 to 150 mmHg in the other 32 patients. Thirty-five patients were studied both at rest and during supine exercise. In most patients, the relation between oxygen consumption and cardiac output was normal during exercise. The stroke index and right ventricular end-diastolic pressure at rest and on exercise were compared in 34 patients. Seventeen showed a normal response to exercise. In the other 17 patients, right ventricular end-diastolic pressure rose on exercise; in 5 of these the stroke index fell during exercise, indicating abnormal myocardial response. Our studies indicate the frequent occurrence of residual abnormalities, even in patients who appear asymptomatic, after total correction of tetralogy of Fallot.

Adolescent

Prevalence and characteristics of disporportionate ventricular septal thickening in infants with congenital heart disease.

Necropsy studies were performed in 125 infants, 2 years old or less, with a variety of congenital heart malformations. Disproportionate ventricular septal thickening (septal-to-left ventricular free wall thickness ratio greater than or equal to 1.3) was present in 31 (25%) of the 125 patients. In the majority of patients, an abnormal septal-to-free wall ratio did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy, since: 1) marked disorganization of septal myocardium (involving greater than or equal to 5% of the tissue section) was present in only five of the 31 patients with disproportionate septal thickening; and 2) echocardiographic studies in first degree relatives of four other patients with disproportionate septal thickening and normal septal architecture did not show asymmetric septal hypertrophy. The relatively high prevalence of septal-to-free wall ratios of 1.3 or greater in this population of infants with congenital heart disease appeared to be due in part to minor differerences in ventricular wall thicknesses (in patients with relatively thin absolute wall thicknesses) which may produce particularly large deviations from unity in septal-to-free wall ratio. Hence, 1) nongenetically transmitted disproportionate septal thickening is relatively common in infants with congenital heart diseases studied at necropsy: and 2) a septal-to-free wall ratio of greater than or equal to 1.3 cannot be used as the sole criterion for identifying associated hypertrophic cardiomyopathy in infants with other congenital heart diseases, particularly if marked absolute septal thickening is absent.

Cardiomegaly

Pulmonary artery banding in infants with complete atrioventricular canal.

Management of symptomatic atrioventricular canal (AVC) in infancy may be difficult. Between July, 1969, and September, 1977, 31 infants with complete AVC presented in congestive heart failure (CHF) to the University of Minnesota Hospitals. Fifteen of these patients have responded to medical management and have been followed as outpatients. The other 16 patients remained in CHF. Six of them died of persistent heart failure within 4 months. The other 10 infants, aged 3 weeks to 1 year (mean 4 months), underwent pulmonary artery banding and seven survived operation. One of the survivors died with apparent pneumonia 1 month postoperatively. Each of the remaining six patients, who have been followed for 9 months to 9 years, had minimal mitral insufficiency and a large ventricular shunt. The three patients dying after banding had significant mitral insufficiency. We believe that pulmonary artery banding is an effective palliative procedure for infants with complete AVC and CHF who have large ventricular shunts and minimal mitral insufficiency.

Angiocardiography

Echocardiographic features of total anomalous pulmonary venous connection to the coronary sinus.

Six infants with anomalous connection of the pulmonary veins to the coronary sinus were studied with single crystal echocardiography. The posterior left atrial wall echo was identified by its continuity with the left ventricular posterior wall echo during a sweep from the left atrium to the left ventricle. In five of the six infants an echo-free space measuring 7 to 9 mm was identified behind the posterior left atrial wall; this space was thought to represent the common pulmonary vein. In four of these five, and also in the remaining infant, a highly mobile linear echo with a double wave form per cardiac cycle was recorded behind the posterior aortic wall and anterior mitral leaflet echoes. Echocardiographic contrast studies after the injection of saline solution into the coronary sinus disclosed that this additional echo was produced by the anterior wall of the coronary sinus. The anatomic connection of the pulmonary veins were established in each patient with pulmonary cineangiography. In four of the six patients all four pulmonary veins were connected to the coronary sinus by way of a common pulmonary vein; in the remaining two patients three of four pulmonary veins were connected to the coronary sinus, while the left upper lobe pulmonary vein was connected to the left innominate vein. The coronary sinus was greatly enlarged in each patient.

Cardiac Catheterization

The electrocardiogram in aortic valvular atresia.

In a review of the electrocardiograms of 27 patients with aortic valvular atresia, several showed an unusual pattern. In two, the Wolff-Parkinson-White syndrome was present, and in the third, complete right bundle-branch block was seen. The study also confirms that left axis deviation and left ventricular hypertrophy can occur rarely. No patient showed a Q wave in lead V6.

Aortic Valve

Anomalous left coronary arter from pulmonary trunk. Long-term follow-up after ligation.

Long-term follow-up (10 to 13 years) of four patients with anomalous left coronary artery who underwent ligation of the anomalous left coronary artery is presented. Two were operated upon in infancy and the other two at 6 and 7 years of age, respectively. Postoperatively, each is asymptomatic and doing well clinically. The cardiac size on roentgenogram of the chest returned toward normal in each. Abnormalities persist on the electrocardiogram. On treadmill exercise, the electrocardiogram of the two operated upon in infancy showed an abnormal result, whereas the two patients operated upon later in childhood had no electrocardiographic abnormalities.

Adolescent

Late hemodynamic and angiographic findings after ascending aorta--pulmonary artery anastomosis.

Ascending aorta-pulmonary artery (Waterston) anastomosis was performed in 75 children, 51 of whom were younger than 1 month of age and 36 younger than 1 week of age at the time of operation. There were 21 operative and eight late deaths. Operative deaths occurred more frequently in infants with complex cardiac conditions and severe hypoxemia and acidosis preoperatively. The postoperative status of the pulmonary arteries or arterioles could not be correlate with the degree of cardiomegaly, the pulmonary vascular markings, or characteristics of the shunt murmur. Sixty-four percent of the patients catheterized had one or more postoperative structural abnormalities of the pulmonary arteries following the operation, most frequently kinking or narrowing of a pulmonary artery. Occlusion of a pulmonary artery occurred in five of 33 patients studied by angiography. Pulmonary vascular disease may also develop. Therefore, patients should be catheterized 1 year postoperatively to evaluate the level of pulmonary vascular resistance and the pulmonary arteries.

Aorta

Congenital absence of the right superior vena cava: report of two cases.

Absence of the right superior vena cava with persistence of the left superior vena cava is an uncommon cardiac malformation. 2 patients with this anomaly are presented, and the importance of recognizing this condition is emphasized. Although associated with other cardiac malformations, there is no characteristic type.

Cardiac Catheterization

Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease.

Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abonrmal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chanber enlargement, and other abnormalities. Although Fabry's disease is rate, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.

Adolescent