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Biomedical subjects

F J Fricker

Publications and source records attributed to F J Fricker.

At least 73 records · Page 4Linked to original sources
PubMed1985

Ventricular septal defect associated with aneurysm of the membranous septum.

The most common variety of ventricular septal defect, a perimembranous defect, is frequently associated with a so-called aneurysm of the membranous septum. Previous studies have suggested that ventricular septal defects associated with an aneurysm of the membranous septum tend to spontaneously decrease in size or close more than defects without such an aneurysm. To better define the natural history of this entity, clinical and catheterization data from 87 patients with ventricular septal defect and aneurysm of the membranous septum were reviewed. The initial evaluation was made at a median age of 0.3 years (range 0.1 to 11), with the final evaluation at a median age of 10 years (range 1.5 to 20) and a median duration of follow-up of 8.6 years (range 1.2 to 18.8). Approximately 75% of the ventricular septal defects had a small or no left to right shunt at last evaluation. Overall, 48 patients (55%) had no significant change in the size of the defect, and 39 (45%) showed improvement during the period of observation. Only four patients (5%) had spontaneous closure of the defect. Of the 49 patients who presented with a large left to right shunt, with or without congestive heart failure, 23 (47%) had persistence of a shunt large enough to warrant surgery. When spontaneous improvement occurred, it did so by 6 years of age in all but one patient. Therefore, a continued tendency for a ventricular septal defect associated with an aneurysm of the membranous septum to spontaneously decrease in size or close after this age may be less likely than previously suggested.(ABSTRACT TRUNCATED AT 250 WORDS)

Aneurysm↗
PubMed1984

Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients.

Seventeen patients with tetralogy of Fallot (TF) and absent pulmonic valve were seen at Children's Hospital of Pittsburgh from 1958 to 1981. In 7 patients, severe respiratory symptoms developed due to bronchial compression by an aneurysmal pulmonary artery, and 6 died. In 4 patients, moderate respiratory symptoms developed. Six patients remained free of respiratory distress. Medical and surgical management of patients with respiratory distress in infancy has been frustrating and frequently unsuccessful. A 4-month-old infant with respiratory distress underwent complete intracardiac repair with valve insertion and has subsequently survived with relief of symptoms. Considering the high mortality rate in this group of patients, further attempts at aggressive surgical repair in infancy for patients with severe respiratory symptoms is warranted. An anatomic review of pathologic specimens with this anomaly confirmed previous reports of the frequent association of absence of the ductus arteriosus in patients with tetralogy of Fallot and absent pulmonary valve. The exception is noted in patients with discontinuity between the right and left pulmonary arteries in which a ductus arteriosus may be present supplying the isolated left pulmonary artery.

Abnormalities, Multiple↗
PubMed1984

Surgical management of univentricular heart with subaortic obstruction.

In children with a univentricular heart and a rudimentary subaortic chamber, surgical relief of subaortic obstruction caused by a restrictive bulboventricular foramen is associated with high morbidity and mortality. A 6-year-old child with a univentricular heart of the left ventricular type, a rudimentary subaortic chamber, and atresia of the left-sided atrioventricular valve had pulmonary artery banding in infancy. Severe subaortic obstruction subsequently developed. At operation, the pulmonary artery was transected and the stump was anastomosed directly to the posterior aspect of the ascending aorta, diverting left ventricular blood into the aorta through the pulmonary valve. The distal pulmonary artery was anastomosed side-to-side to the ascending aorta to provide pulmonary blood flow. Cardiac catheterization fifteen months after the operation demonstrated an excellent hemodynamic result. When the pulmonary artery is adequate in size, a bypass operation by way of an anastomosis between the ascending aorta and the pulmonary artery is a relatively safe and effective means of relieving the ventricular outflow obstruction caused by a restrictive bulboventricular foramen.

Aortic Valve Stenosis↗
PubMed1984

Ventricular inversion with normal ventriculoarterial connection and left atrial isomerism: correction by the Mustard operation.

A 2 year old boy with ventricular inversion and normal ventriculoarterial connection is described. Associated cardiac lesions included single atrium, absence of the coronary sinus, bilateral superior venae cavae, absence of the hepatic segment of the inferior vena cava with azygos and hemiazygos continuation, right aortic arch, levocardia and left atrial isomerism. At 5 days of age, the patient underwent a Waterston (aortopulmonary) anastomosis because of suspected pulmonary atresia. The correct diagnosis was established at 2 years of age and the patient had a successful Mustard operation (interatrial baffle procedure) and closure of the Waterston anastomosis. Accurate preoperative diagnosis is difficult in this rare cardiac anomaly and the reported mortality rate is high.

Cardiac Catheterization↗
PubMed1983

Hemodynamic function after the Mustard operation for transposition of the great arteries.

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Adolescent↗
PubMed1983

Exercise studies after the Mustard operation in transposition of the great arteries.

Twenty-one postoperative patients with transposition of the great arteries (TGA) underwent graded exercise testing 4 to 15 years (mean 9) after the Mustard operation. No patient had subjective exercise intolerance before testing, although some had symptomatic resting arrhythmias. Correlations were made between cardiac catheterization data and 24-hour ambulatory monitoring. Exercise tolerance was diminished in nearly half of the patients. No statistically significant differences were found in heart rate or blood pressure responses, but maximal oxygen consumption values were lower than the control values (p less than 0.001) in the larger patients. Arrhythmias were present or provoked in most patients during exercise testing. Only 28% remained in normal sinus rhythm during and after exercise. Multifocal premature contractions were the most serious arrhythmias demonstrated. Some long-term survivors of the Mustard operation may have abnormal exercise dynamics, even though they may be asymptomatic and have normal physical activities and endurance. Mean maximal systolic blood pressure, heart rate, oxygen consumption, and maximal treadmill times were consistently in the low-normal range or were statistically lower than normal.

Adolescent↗
PubMed1983

Arrhythmias in transposition of the great arteries after the Mustard operation.

Disorders of rhythm or conduction in patients with transposition of the great arteries (TGA) after the Mustard operation have been widely reported. This study provides a systematic evaluation of the electrophysiologic function of 87 survivors of the Mustard operation at a single institution. Surface electrocardiograms were reviewed in all 87 patients, Holter monitoring data in 26 patients, exercise electrocardiograms in 21 patients, and invasive electrophysiologic data in 61 patients. Surface electrocardiograms showed normal sinus rhythm in 52%, sinus node dysfunction in 27%, and atrioventricular block in 16%. Holter monitoring was obtained in an unselected subgroup of 26 patients who had a mean age of 12 years and a mean interval from operation of 9 years. Sinus node dysfunction was found in 58%, atrioventricular block in 27% ventricular ectopy in 50%, supraventricular ectopy in 27%, and no abnormalities in only 8%. Intracardiac electrophysiologic evaluation showed a high frequency of abnormal sinus node recovery times and suboptimal response of the atrioventricular-conduction system to rapid atrial pacing. When all modalities used in this study were considered, sinus node dysfunction occurred in 47%, ectopy in 34% and atrioventricular block in 23%. Although only 30% of patients had no evidence of arrhythmia, symptoms of rhythm or conduction disturbances were rare.

Adolescent↗
PubMed1983

The anatomical heterogeneity of hearts lacking a patent communication between the left atrium and the ventricular mass ('mitral atresia') in presence of a patent aortic valve.

Thirty-five hearts were studied with no patent communication between the left atrium and the ventricular mass ('mitral atresia'). In ten, an imperforate and hypoplastic membrane separated the left atrium from a hypoplastic left ventricle. The imperforate valve ranged from a tiny membrane with no evidence of tension apparatus, through a larger membrane supported by muscular columns in the ventricular inlet portion, to an aneurysmal imperforate sac attached to a single hypoplastic papillary muscle. The ventriculo-arterial connection was concordant in nine of the ten hearts and double outlet from the right ventricle in one. In one further heart, there was double inlet left ventricle with imperforate left atrioventricular valve, a rudimentary right ventricle and ventriculo-arterial discordance. In the other 24 hearts the muscular floor of the left atrium was completely separated by the atrioventricular sulcus from the ventricular mass (absent left atrioventricular connection). In 11 of these hearts, the right atrium was connected to a right ventricle and there was a posterior and left-sided rudimentary left ventricular chamber which had no connection with the atrial chambers. The left ventricle supported the aorta in five of the 11 hearts, but in the other six it was simply a pouch of left ventricular morphology. In ten cases the right atrium was connected to a dominant left ventricle. An anterior rudimentary right ventricle was present in each but had no connection with the atrial chambers. It was to the left of the left ventricle in seven, directly anterior in one and to the right in two cases. The right ventricle supported the aorta in eight cases, the pulmonary trunk in one and neither great artery in the remaining case. Lastly, there were three cases in which a sole ventricular chamber of indeterminate morphology was present and gave rise to both great arteries.

Aortic Valve↗
PubMed1982

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patient's ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.

Arteries↗
PubMed1980

Coarctation of the aorta in children. Late results after surgery.

From 1960 to 1970, 110 patients underwent operation for coarctation of the aorta. The overall mortality was 23%. However, in infants operated on at less than 6 months of age, the mortality was 63%, whereas there was only a 4% mortality in those operated on after 6 months of age. Late follow-up was available in 87 patients from five to 15 years postoperatively. There was a 14% frequency of recoarctation in the group of long-term survivors. Systemic hypertension, defined as an upper extremity blood pressure greater than 140/90 mm Hg, was found in 23% of the survivors. This study has demonstrated a significant frequency of postcoarctectomy hypertension and residual hemodynamic and angiographic abnormalities in patients without clinical evidence of recoarctation. This indicates the need for continued long-term follow-up of these patients.

Adolescent↗
PubMed1980

Absent right superior vena cava with persistent left superior vena cava: implications and management.

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 and in a 26 day old infant with multiple cogenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals s short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well, the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.

Adolescent↗
PubMed1979

Iliac venous thrombosis in infants and children after cardiac catheterization.

Twenty-two patients developed thrombosis of the lower abdominal portion of the inferior vena cava, iliac or femoral vein (IVT) after cardiac catheterization. All patients has at least one previous study from 1 day to 11 months of age (mean, 2 months) and seven had two catheterizations before discovery of IVT. Transposition of the great arteries was the most common defect associated with IVT (12/22, 55%). From one to seven catheter changes were made during the time of venous cannulation (mean, 105 minutes). No clinical evidence of IVT was present immediately after the preceding cardiac catheterization. The discovery of significant IVT usually necessitated the use of a vein from the upper extremity to complete the cardiac catheterization. IVT was associated with previous balloon atrial septostomy using either a Fogarty or Rashkind septostomy catheter in 13/22 patients (59%). In addition, 86% of the patients had either of these balloon catheters or a Swan-Ganz catheter used during a previous study. The potential development of IVT should be considered especially in cyanotic infants and small children in whom balloon catheters are used.

Cardiac Catheterization↗