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Biomedical subjects

C E Mullins

Publications and source records attributed to C E Mullins.

At least 127 records · Page 7Linked to original sources

Transseptal left heart catheterization in infants and children.

Transseptal left heart catheterization was performed in 80 infants and children with various forms of congenital heart disease. The majority had left heart obstructive lesions. Forty percent were under 5 years of age and less than 20 kg in weight. Uncomplicated cardiac perforation occurred in two patients. The technique is described in detail with emphasis on measures which increase the safety of the procedure for the patient. We conclude that this is a useful technique and in selected patients may be the preferred approach to the left heart.

Adolescent↗

Anomalous origin of circumflex coronary artery from right pulmonary artery: report of a rare anomaly.

A heretofore unreported anomaly of the coronary arteries in which a large circumflex artery arises from the pulmonary artery is described. The circumflex coronary artery in an 8-year-old girl was filled in a retrograde fashion via collaterals from a dominant left anterior descending coronary artery, and the right coronary artery was small and nondominant. Successful surgical correction of this defect was performed by use of total cardiopulmonary bypass and cardioplegic arrest. The orifice of the anomalous vessel and detached from the right pulmonary artery and implanted into the posterolateral aspect of the ascending aorta. Cardiopulmonary bypass with cardioplegic arrest is recommended for reconstruction of coronary arterial anomalies with abnormal sites of origin.

Aorta↗

Pulmonary artery wedge pressures in congenital heart disease.

The bilateral measurement of pulmonary artery wedge pressure is essential in the cardiac catheterization evaluation of all patients with pulmonary artery hypertension. Five cases of pulmonary venous obstruction are presented, 4 of whom had additional intracardiac defects. The pulmonary artery hypertension in these 4 patients was initially attributed to the associated cardiac anomalies and because the left atrium had been entered directly with the catheter in each case, pulmonary artery wedge to the left atrium pressure gradients were initially either not obtained or were discounted as being artificial. Pulmonary venous obstruction was recognized in all 5 cases on the basis of an elevated pulmonary artery wedge pressure, and the anatomic site of the obstruction was successfully documented. Unless bilateral pulmonary artery wedge pressures are measured in all patients with pulmonary artery hypertension regardless of the presence of additional cardiac anomalies, a surgically correctable cause of pulmonary hypertension may be overlooked.

Blood Pressure↗

Late development of pulmonary venous obstruction following Mustard's operation using a dacron baffle.

Three patients developed late pulmonary vein obstruction (PVO) following Mustard's operation for transposition of the great arteries. In all three the absence of PVO had been documented by an earlier postoperative cardiac catheterization. At reoperation shrinkage and kinking of the dacron baffle was evident. After Mustard's operation, patients should be observed closely for symptoms and signs of PVO since it can occur insidiously despite previously proven absence of this complication.

Cardiac Catheterization↗

Sudden death after repair of tetralogy of Fallot. Electrocardiographic and electrophysiologic abnormalities.

In order to try to determine the mechanism of sudded death in patients after surgical repair of tetralogy of Fallot, electrocardiographic, intracardiac electrophysiologic, and clinical data of 51 children who had postoperative intracardiac electrophysiologic studies were reviewed. Ninety-four percent had developed right bundle branch block (RBBB) and 16 percent had additional left anterior hemiblock (LAH). Two had had transient complete A-V block (CAVB) and one had permanent CAVB. Six had a first degree A-V block and nine had premature ventricular contractions (PVC). Nine patients were found to have prolonged intra-atrial conduction times, four prolonged A-V nodal conduction, four prolonged His-Purkinje conduction, and five prolonged corrected sinus node recovery times. Patients with first degree A-V block or LAH did not have an increased incidence of abnormalities on electrophysiologic study. No patient with RBBB and LAH developed complete A-V block or died. Three of the nine patients with PVCs died, one of intractable ventricular fibrillation and two suddenly, presumably of dysrhythmia. All three had significant congestive heart failure. Although late complete A-V block occurs and should be watched for, ventricular dysrhythmias in patients with PVCs may be the cause of most sudden deaths after tetralogy repair. We currently are treating all of our postoperative tetralogy patients who have PVCs with quinidine or propranolol.

Adolescent↗

Significance of pacemaker recovery time after the Mustard operation for transposition of the great arteries.

Pacemaker recovery time corrected for cycle length was measured in 24 patients 6 months to 3 years after the Mustard operation for transposition of the great arteries. This index of sinoatrial (S-A) nodal function was obtained by subtracting the control R-R interval from the first R-R interval recorded after 3 minutes of rapid atrial pacing at the superior vena caval-right atrial junction. Recovery times were compared with those of a control group of 54 children with various forms of congenital heart disease who had not had cardiac surgery and whose electrocardiogram revealed normal sinus rhythm. The longest corrected pacemaker recovery time in the control group was 250 msec. Recovery time was normal (mean 115 msec) in 13 of the 24 patients who had undergone the Mustard procedure and prolonged (mean 472 msec) in the other 11. In 4 of these 11 a junctional escape complex was recorded before recovery of the S-A node. The configuration of the P wave and the development of dysrhythmias in the postoperative follow-up period were compared in patients with a normal and a prolonged corrected pacemaker recovery time. The incidence of dysrhythmias was much smaller in the former group. Normal sinus rhythm after the first postoperative week was noted in 10 (76 percent) of patients with a normal corrected pacemaker recovery time but in only 2 (18 percent) of those with an abnormal recovery time. One patient with an abnormal recovery time died suddenly 26 months after operation in spite of good hemodynamic repair. A prolonged pacemaker recovery time after the Mustard operation for transposition of the great arteries indicated that rhythm abnormalities were likely to develop, but a normal recovery time did not exclude abnormal sinus nodal function.

Adolescent↗

Transient hypertrophic subaortic stenosis in infants of diabetic mothers.

Three newborn infants with congestive heart failure had hemodynamic, angiographic, and echocardiographic features of hypertrophic subaortic stenosis (hypertrophic obstructive cardiomyopathy). Treatment with digitalis and diuretic drugs was ineffective, but improvement occurred when these agents were withheld in one patient, and when treatment with propranolol was begun in two patients. Echocardiography was helpful in establishing the diagnosis in two patients and showed resolution of the condition during the first six months of life. Serial cardiac catheterizations confirmed resolution of the outflow obstruction in the third patient. Family studies revealed no evidence of familial cardiomyopathy, but the mothers of two infants had insulin-dependent diabetes mellitus and the mother of the third was presumed to be prediabetic.

Adult↗

Protection of the sinus node in Mustard's operation.

The occurrence of dysrhythmias after the Mustard operation for transposition of the great arteries was compared in 70 patients operated upon before and 58 patients operated upon after January 1972 when surgical modifications aimed at preserving the sino-atrial node and its arterial supply were initiated. The surgical modifications included changing the site of the superior vena cava (SVC) cannulation away from the SVC-right atrial junction, incision into the right atrial wall anterior to the sulcus terminalis, and sewing of the superior part of the baffle patch away from the sino-atrial node area. A significant decrease in the incidence of dysrhythmias occurred in the group of patients operated upon after the surgical modifications. The modifications in surgical technique have reduced but not avoided dysrhythmias.

Arrhythmia, Sinus↗

Aneurysm of right-sided cervical arch: surgical removal and graft replacement.

Right-sided cervial aortic arch is a rare congenital anomaly which may cause respiratory symptoms or dysphagia. In the past, surgical correction of the cervical arch has not been attempted except in one patient in whom an erroeous diagnosis of aneurysm of the innominate artery led to an unsuccessful operation. A case is reported of a 39-year-old woman with an extensive fusiform aneurysm of a right-sided cervical arch. The arch aneurysm was successfully removed surgically and normal circulation was restored to the arch vessels. At the same operation, a ventricular septal defect was repaired with the aid of temporary cardiopulmonary bypass.

Adult↗

Intracardiac electrography in children and young adults.

The interpretation of IE recorded in children has been hampered by a lack of agreement regarding normal values. We recorded IE in 158 children and young adults (ages, three days to 33 years) to define the various conduction intervals in normal and disease states. The HBP was recorded in 156 subjects. In 85 subjects with normal conduction indicated by surface ECG, including 19 subjects with normal hearts, there were no statistically significant age-related differences in internodal, A-V nodal, or His-Purkinje conduction intervals. Therapeutic levels of digitalis did not alter the conduction intervals. In 11 subjects with first degree A-V block and in five subjects with congenital complete A-V block, the site of block as determined by IE could not be predicted from the surface ECG. No abnormalities in conduction intervals were found in 18 subjects with right bundle branch block (surgically induced in 17 cases). Intracardiac electrography with recording of the HBP was found to be a safe, informative technique for electrophysiologic investigations in children and young adults.

Adolescent↗

Concealed his bundle extrasystoles in congenital heart disease.

His bundle electrograms were recorded in two children with cardiac conduction disturbances. In the surface electrocardiograms of one patient His bundle extrasystoles mimicked atrial nonconducted bigeminal rhythms; in the other, they simulated second degree atrioventricular (A-V) block and conducted junctional extrasystoles. In both instances the conduction disturbance was a result of concealed conduction of the His bundle extrasystole into the A-V junction. We conclude that the surface electrocardiogram is suggestive but not definitive in diagnosing His bundle extrasystoles and that His bundle recordings are indispensable for correct diagnosis and treatment.

Bundle of His↗

Congenital and surgical atrioventricular block within the His bundle.

In three patients with congenital heart disease the site of atrioventricular (A-V) block was localized within the His bundle with the aid of His bundle electrograms. In one patient with first degree A-V block and normal QRS configuration, electrophysiologic studies revealed "split" His potentials. The other two patients had complete A-V block, and their His bundle electrograms revealed His spikes both proximal and distal to the site of block. One of the two patients, who had a pattern of left bundle branch block in the electrocardiogram, had surgically induced complete A-V block after repair of an ostium primum atrial septal defect. The other patient with congenital A-V block had a narrow QRS complex and, in addition to complete block within the His bundle, prolonged A-V nodal conduction time but no associated cardiac anomaly. Both patients with complete heart block required pacemaker insertion. The natural history of intra-H-is bundle block is not known, and it is difficult to recommend appropriate therapy. More electrophysiologic studies are needed in patients with A-V block to determine the prognostic significance of such block or conduction delay in the His bundle.

Adult↗

Unilateral pulmonary vein atresia: Diagnosis and treatment.

The clinical, hemodynamic, angiographic and pathologic features of unilateral pulmonary vein atresia in a 16 month old boy are described. The relevant findings were symptoms and signs of influenza, roentgenographic evidence of unilateral diffuse interstitial edema, prominent interlobar fissures and pleural effusion, absence of abnormal cardiac structure and prominent unilateral bronchial circulation. Angiocardiography was necessary for a definitive diagnosis and revealed the lack of a site of entry for venous blood from the right lung into the circulation. The patient was treated successfully by a right pneumonectomy. The histologic hallmark in this case was extensive intimal fibrosis of the pulmonary veins.

Congenital Abnormalities↗

Variation in the phenotypic expression of beta-glucuronidase deficiency.

Defiency of beta-glucuronidase was demonstrated in serum, leukocytes, and cultured skin fibroblasts of two unrelated patients. One patient died at 2 9/12 years with a phenotype consistent with severe mucopolysaccharidosis; the other is 14 years of age and well, except for hypertension and obstructive lesions of large blood vessels. Analysis of urinary mucopolysaccharides revealed impaired degradation of dermatan sulfate and, to a lesser extent, of heparan sulfate. Cultured skin fibroblasts accumulated excess glycosaminoglycans (the term glycosaminoglycans is synonymous with acid mucopolysaccharides) as indicated by 35-SO-4 uptake.

Adolescent↗

Hemodynamic and angiographic changes after Mustard operation for transposition of the great arteries.

After Mustard operation for transposition of the great arteries, hemodynamic and angiocardiographic changes were evaluated in 25 patients. In 19 patients, postoperative studies were done electively and, in 6 patients, they were required to investigate symptoms of heart failure, these symptoms were temporary in 4 patients and progressive in 2. Both of the latter had pulmonary venous obstruction which was later relieved successfully by reoperation. After operation, systemic arterial oxygen saturation and blood pressure increased and polycythemia disappeared in every patient. However, several complications-some of them unsuspected clinically-were identified by cardiac catheterization: (a) patch detachment in 5 patients; (b) obstruction of superior vena caval return in 10 patients; (c) obstruction of inferior vena return in 1 patient; and (d) pulmonary venous obstruction in 2 patients. The incidence of pulmonary or systemic venous obstruction was higher in patients who had a Dacron intraatrial baffle (8 of 19 patients). Comparison of 21 sets of preoperative and postoperative right ventricular angiograms demonstrated an increase in right ventricular trabeculations in each patient, poorer right ventricular contractility in 12 patients, and development of tricuspid insufficiency in nine patients. None of the patients with poor right ventricular contractility had had surgical ventriculotomy. Although Mustard operation for transposition of the great arteries is effective in relieving cyanosis, it may be followed by obstruction to systemic or pulmonary venous return, intraatrial patch detachment, tricuspid insufficiency and angiocardiographic evidences of poor right ventricular contractility.

Adolescent↗