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Biomedical subjects

R J Hall

Publications and source records attributed to R J Hall.

At least 109 records · Page 6Linked to original sources

The surgery of "correctable" biliary atresia.

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.

Bile Ducts↗

Surgical implications of bronchopulmonary dysplasia.

Twenty of 30 patients with bronchopulmonary dysplasia (BPD) had major tracheobronchial abnormalities, which in 18 could be incriminated as contributing to their symptoms. There were 15 examples of tracheobronchial stenosis, 13 of tracheomalacia, nine of bronchomalacia, and one of tracheal web. Sixteen patients had operations. Tracheostomy was successful as a temporizing measure in ten patients although there was significant morbidity. Balloon dilatation of tracheobronchial stenosis produced temporary (2) or long-term (2) improvement in four patients. Electroresection of tracheobronchial stenosis was successful in the three instances it was employed. Lobectomy for lobar emphysema was curative in both patients. Aortopexy for tracheomalacia improved all three patients. We conclude that (1) symptomatic major airway lesions are not uncommon manifestations of BPD, (2) many of the lesions are amenable to surgical therapy, and (3) bronchoscopic evaluation should be considered early in the course of infants with BPD.

Bronchopulmonary Dysplasia↗

Subjective analysis of cross-sectional echocardiograms: reproducibility and sources of variability.

Subjective analysis of left ventricular segmental wall motion is widely used to assess regional left ventricular function and the effects of intervention in patients with ischaemic heart disease. To determine its reproducibility we studied 20 consecutive men with proven ischaemic heart disease and 10 age-matched control subjects. Standard parasternal, apical and subcostal views were attempted in all subjects by two echocardiographers, and echoes were reported by two analysers, each on two occasions. The left ventricle was subdivided into 5 segments, and segmental wall motion scored as normal, hypokinetic, akinetic, dyskinetic or inadequately visualised. The different sources of variability were evaluated. The echocardiographers contributed little variability, the frequency and reproducibility of reporting being the same for echoes produced by each echocardiographer. The only exception was for subcostal views in which one echocardiographer produced significantly more reportable segments than the other (42% vs 27%; P less than 0.001). Within and between analyser variability was large. Replicate analysis of the same segment showed within analyser agreement of 92% for one analyser but only 80% for the other. Between analyser agreement was 89% for some segments but only 63% for others and was best in those segments where both analysers infrequently reported abnormal wall motion. Reporting bias is thus an important variable. The subjective assessment of regional left ventricular function thus has major limitations for general applications particularly when being used in longitudinal studies.

Coronary Disease↗

Electrophysiologic testing: predictive of amiodarone efficacy in recurrent sustained ventricular tachycardia?

The role of programmed ventricular stimulation (PVS) was evaluated in 12 patients with recurrent sustained ventricular tachycardia (VT) who were treated with amiodarone as the sole antiarrhythmic agent. At control PVS, sustained VT was induced in 11 patients and nonsustained VT was induced in one patient, as compared with late PVS (mean, 8.6 weeks) when sustained VT was induced in six patients and nonsustained VT was induced in five. Amiodarone significantly prolonged the patients' RR, PR, QRS, and QTc intervals, VT cycle length, and right ventricular effective refractory period. During a mean follow-up of 16 +/- 13.6 months, two patients had recurrent clinical VT. In the patients in whom amiodarone therapy failed (1) sustained VT was induced during late PVS, (2) VT cycle length and symptoms during late PVS and during recurrent clinical VT were similar, and (3) the QTc failed to be prolonged significantly (32.5 +/- 1.6 ms in amiodarone failure vs. 84.1 +/- 27.1 ms in amiodarone success, P<0.05). It is concluded that (1) amiodarone in high-risk patients is clinically effective (88.3%), (2) patients with noninducible VT or nonsustained VT during late PVS did not have recurrent clinical VT, (3) late PVS is probably predictive of electrophysiologic and hemodynamic consequences in patients with recurrent spontaneous VT, and (4) failure of the QTc interval to be prolonged substantially is probably predictive of clinical recurrence of VT.

Journal Article↗

Chloroquine-induced cardiomyopathy.

Biventricular hypertrophy and failure developed in two patients during treatment of systemic lupus erythematosus with chloroquine phosphate. In both patients, morphologic analysis of the myocardium, obtained by a right ventricular endomyocardial biopsy in one patient and at autopsy in the other, revealed accumulations of electron-dense concentric and parallel lamellae and curvilinear bodies within cardiac myocytes. These deposits were similar to those reported in chloroquine-induced skeletal myopathy and were considered to represent evidence of chloroquine-induced cardiotoxicity rather than a cardiovascular manifestation of the underlying disease. Clinical awareness and an endomyocardial biopsy specimen are necessary for the appropriate diagnosis of chloroquine-induced cardiomyopathy.

Adult↗

Biliary atresia in the newborn.

A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.

Biliary Atresia↗

Hemodynamic compromise (tricuspid stenosis and insufficiency) caused by an unruptured aneurysm of the sinus of Valsalva.

A patient is described with an unruptured aneurysm of the noncoronary sinus of Valsalva that occupied the right ventricular inflow tract and caused dynamic tricuspid stenosis and insufficiency. Results of two-dimensional echocardiography delineated the anatomy of the aneurysm and pulsed Doppler examination provided evidence that the aneurysm was unruptured. The unruptured aneurysm was resected successfully.

Adult↗

Post-infarction exercise testing in patients under 55 years. Relation between ischaemic abnormalities and the extent of coronary artery disease.

Previous studies have suggested that the early post-infarction exercise test is useful in predicting the extent of coronary artery disease. The results of a heart rate limited exercise test three weeks after infarction and a symptom limited exercise test six weeks after infarction obtained by both standard lead electrocardiograms and 16 lead precordial maps were compared in 100 consecutive survivors of acute myocardial infarction under 55 years of age. Exercise tests were defined as being positive on the basis of angina, ST segment depression greater than or equal to 1 mm in any electrocardiogram lead, or exertional hypotension. Multivessel disease, that is two or three vessel disease, was present in 60 patients, and three vessel disease in 22 patients. The sensitivity, specificity, and predictive value for multivessel disease of the three week test were 38%, 83%, and 76% respectively; and results for the six week test were 55%, 75%, and 77% respectively. Only 32% of patients with three vessel disease were identified at the three week test, and 59% at the six week test. Significantly more patients with multivessel and three vessel disease were identified by the symptom limited six week test. Precordial mapping offered no advantages over the standard 12 lead electrocardiogram in either the identification of patients with multivessel disease or the prediction of the distribution of coronary artery disease. Angina pectoris during the exercise test at six weeks was the single most useful predictor of multivessel disease. Multivessel disease was found in 27 (87%) of the 31 patients with angina with or without ST depression during the test at six weeks compared with 33 (48%) of the 69 patients who did not have angina during the test at six weeks. Exercise testing in the early post-infarction period in patients under 55 years of age is of limited value in predicting the extent of coronary artery disease. It is, therefore, unreasonable to use such exercise tests to select patients for coronary arteriography after myocardial infarction. None the less angina pectoris occurring during a symptom limited exercise test six weeks after infarction is a strong predictor of multivessel disease, and coronary arteriography is recommended in these patients.

Adult↗

The echocardiographic spectrum of atrial myxoma: a ten-year experience.

We analyzed the echocardiograms of 28 patients with a left atrial myxoma and two with a right atrial myxoma. Our purpose was to evaluate the value of echocardiography for the diagnosis of these cardiac masses. Only 59% of the m-mode echocardiograms in patients with a left atrial myxoma showed the characteristic findings of multiple diastolic echoes within the mitral orifice as well as abnormal systolic echoes within the left atrium. M-mode echocardiograms were atypical for left atrial myxoma in the remaining patients, and a definitive diagnosis could not be established on the basis of this procedure in six of the patients (22%). Two-dimensional echocardiography showed the presence of a left atrial mass in all 16 patients who had the procedure, and aided in understanding the atypical m-mode recordings. There was a close relationship (r =.82) between two-dimensional echocardiographic measurements of the myxomas' size and pathologic measurements. Both right atrial myxomas could be identified on the m-mode echocardiogram. Our experience indicates that two-dimensional echocardiography is superior to the m-mode technique for the diagnosis and characterization of left atrial myxomas.

Journal Article↗

The QS2/QT ratio as an index of appropriate left ventricular response to autonomic and inotropic stimuli.

Dissociation between duration of electrical and mechanical systole has been seen with increase of myocardial shortening velocity or adrenergic activity. We found a decrease of the QS2/QT ratio after exercise in 10 pts with semisitting bicycle maximal exercise test and a normal radionuclide angiogram. No change was seen in 9 patients with a normal study with beta blockade, and in pts with a abnormal radionuclide test, without beta blockade (11 pts). In 19 normal active individuals, a significant QS2/QT decrease (p less than 0.001) was seen after a submaximal exercise treadmill test, which was inhibited by beta blockade. We believe that QS2/QT diminution is suggestive of an increase of the inotropic state of the left ventricle with exercise, produced through adrenergic stimulation. It is not seen either when an ischemic left ventricle cannot adequately respond to exercise or after beta blockade.

Adult↗

Collateral circulation in coronary artery disease.

The coronary arteriograms and left ventriculograms of 202 consecutive patients were reviewed. All had at least 75% diameter reduction of 1 or more major coronary arteries. In 127 patients (63%), at least 1 major branch was totally occluded. Collateral circulation was seen in 125 of these 127 patients (190 of 192 totally occluded arteries). Of the 75 patients without total occlusion, only 2 with 99% (or near-total) occlusion had demonstrable collateral circulation (2 of 208 arteries). In no patient with 75 to 98% diameter narrowing was collateral circulation demonstrated (0 of 164 arteries). An analysis was made of the relation between left ventricular (LV) segmental wall motion and the quality of collateral circulation in 190 totally occluded arteries among 125 patients. Of 126 arteries with good collateral circulation, LV contraction was normal in 21%, hypokinetic in 48% and akinetic/dyskinetic in 29%. Of 64 arteries with poor collateral circulation, LV contraction was normal in 23%, hypokinetic in 55% and akinetic/dyskinetic in 20%. There was no statistically significant difference between the effect of good or poor collateral circulation on LV function. These data indicate that collateral circulation cannot be seen angiographically unless there is total or near-total occlusion, and that the presence of collateral circulation does not correlate with LV wall motion abnormalities, i.e., akinetic area, despite good collateral flow or normal wall motion despite absent or poor collateral flow.

Collateral Circulation↗

Correlations of systolic time intervals and radionuclide angiography at rest and during exercise.

Systolic time intervals (STI) were correlated with radionuclide angiography studies (RAS) in 57 patients at rest, during maximal semisitting bicycle exercise, and at 4 minutes following the cessation of exercise. Eleven were judged as being free of coronary artery disease (group 1), while 14 had coronary artery disease without (group 2A), and 27 (group 2B) with a previous transmural myocardial infarction. For RAS, resting radionuclide ejection fraction (REF), the changes in REF and end-systolic volume, and the development of a wall motion abnormality at peak exercise were each highly correlated with the presence of coronary disease (p less than 0.001). The accuracy of STI parameters in predicting the presence of coronary disease was poor (less than 60%). Changes in end-diastolic volume (EDV) correlated significantly with PEP/LVET and LVET1 changes following exercise. Moreover, patients with an abnormal (greater than 25%) increase in EDV at peak exercise had a greater increase in LVET1 in the postexercise period (p less than 0.01). We conclude that STI is not accurate enough a predictor of coronary disease or left ventricular function to serve as a useful screening test. Changes in STI parameters appear to be more related to changes in ventricular volume than to ventricular function.

Adult↗

Auscultatory and echocardiographic features of mobile left atrial thrombus.

Free floating ball thrombus of the heart is a rare disorder that usually involves the left atrium, particularly in association with mitral stenosis and atrial fibrillation. The diagnosis of ball thrombus is suspected clinically when there are auscultatory changes in the intensity of the murmur of mitral stenosis. Two-dimensional echocardiography is especially useful in the diagnosis of this condition. By combining phonocardiographic recordings with both M-mode and two-dimensional echocardiography, attenuation and disappearance of the murmur were demonstrated as the mobile thrombus randomly drifted into the mitral valve orifice. Reappearance of the cardiac murmur was noted as the thrombus was expelled from the orifice. This study, combining simultaneous phonocardiography and two-dimensional echocardiography, identifies for the first time the relation between thrombus movement into the mitral aperture and changes of the murmur of mitral stenosis.

Echocardiography↗