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Biomedical subjects

R R Liberthson

Publications and source records attributed to R R Liberthson.

At least 19 recordsLinked to original sources

Magnetic resonance imaging of thoracic aortic aneurysms: comparison with other imaging methods.

Fifteen patients with thoracic aortic aneurysms had magnetic resonance images (MRI) and at least one additional diagnostic image study: thoracic aortography, computed tomography (CT), or two-dimensional echocardiography. Twenty aneurysms were demonstrated by MRI, 19 by the other studies. One small saccular aneurysm was missed by CT. There was complete agreement between MRI and other studies regarding aneurysm morphology, and good correlation in diameter measurements of the aneurysms and at multiple additional aortic levels.

Adolescent

Perioperative anesthetic risk of noncardiac surgery in hypertrophic obstructive cardiomyopathy.

To determine their perioperative risk, we reviewed the records of 35 patients with hypertrophic cardiomyopathy diagnosed by cardiac ultrasound and/or catheterization who underwent general (52) or spinal (four) anesthesia--a total of 56 major surgical procedures. There were no operative or related perioperative deaths and no significant ventricular tachyarrhythmias. Intraoperative or postoperative complications included: myocardial infarction with heart failure in one patient who also had coronary artery disease and was one of three patients who had spinal anesthesia, arrhythmia requiring therapy in eight, and angina during supraventricular tachycardia in one. We conclude that the risk of general anesthesia and major noncardiac surgery is low in patients with hypertrophic obstructive cardiomyopathy. Spinal anesthesia, which decreases systemic vascular resistance and increases capacitance, may be relatively contraindicated. Concomitant coronary artery disease may increase the risk.

Adolescent

Incidence and significance of primary abnormalities of cardiac rhythm in infants at high risk for sudden infant death syndrome.

The exact relationship between cardiac arrhythmias and sudden infant death syndrome (SIDS) is uncertain. Several reports have implicated both ventricular and supraventricular arrhythmias in isolated cases, but there have been no studies of the incidence or type of arrhythmias that occur in populations at risk for SIDS. Of 1699 infants at high risk for SIDS, 60 (4%) were found to have a primary cardiac arrhythmia (i.e., not associated with disordered respiration or apnea). The incidence of atrial and ventricular premature beats, supraventricular tachycardia, and Wolff-Parkinson-White syndrome was similar to the incidence found in normal infants. Primary bradycardia (defined as a heart rate less than 60 for greater than 10 s not associated with abnormal respiration) was the most common arrhythmia, occurring with a frequency and severity not seen in normal infants. Thirty-two infants experienced periodic bradycardia. In 19 of these latter infants, there were symptoms associated with these bradyarrhythmias that necessitated treatment. Heart rates as low as 20 beats/min were recorded. One infant presented with an episode of ventricular fibrillation and on further evaluation was noted to have recurrent bradyarrhythmias. In no infant was there abnormal prolongation of the QT interval. Primary bradyarrhythmias are seen at an increased incidence in infants at high risk for SIDS and may play a causal role in this syndrome. Most symptomatic infants can be adequately controlled with sympathomimetic or parasympatholytic therapy. Other cardiac arrhythmias occur at a rate similar to that in normal infants and are therefore unlikely to play a major role in SIDS.

Arrhythmias, Cardiac

Exercise testing in asymptomatic or minimally symptomatic aortic regurgitation: relationship of left ventricular ejection fraction to left ventricular filling pressure during exercise.

Exercise radionuclide angiography is being used to evaluate left ventricular function in patients with aortic regurgitation. Ejection fraction is the most common variable analyzed. To better understand the rest and exercise ejection fraction in this setting, 20 patients with asymptomatic or minimally symptomatic severe aortic regurgitation were studied. All underwent simultaneous supine exercise radionuclide angiography and pulmonary gas exchange measurement and underwent rest and exercise measurement of pulmonary artery wedge pressure (PAWP) during cardiac catheterization. Eight patients had a peak exercise PAWP less than 15 mm Hg (group 1) and 12 had a peak exercise PAWP greater than or equal to 15 mm Hg (group 2). Group 1 patients were younger and more were in New York Heart Association class I. Group 1 patients also had a higher mean rest ejection fraction (0.64 +/- 0.08 vs 0.49 +/- 0.13, p less than 0.01, higher exercise ejection fraction (0.63 +/- 0.10 vs 0.40 +/- 0.18, p less than 0.01), lower end-systolic volume (38 +/- 13 vs 79 +/- 36 ml/m2, p less than 0.01) and higher peak oxygen uptake (24.9 +/- 5.1 vs 16.6 +/- 4.9 ml/kg/min, p less than 0.01) than group 2 patients. However, the two groups had similar cardiothoracic ratios, changes in ejection fractions with exercise, and rest and exercise regurgitant indexes. Using multiple regression analysis, the best correlate of the exercise PAWP was peak oxygen uptake (r = -0.78, p less than 0.01). No other measurement added significantly to the regression. When peak oxygen uptake was excluded, rest and exercise ejection fraction also correlated significantly (r = -0.62 and r = -0.60, respectively, p less than 0.01). Patients with asymptomatic or minimally symptomatic severe aortic regurgitation have a wide spectrum of cardiac performance in terms of the PAWP during exercise. The absolute rest and exercise ejection fraction and the level of exercise achieved are noninvasive variables that correlate with exercise PAWP in aortic regurgitation, but the change in ejection fraction with exercise by itself is not.

Adolescent

Ischemic heart disease in systemic lupus erythematosus in the young patient: report of six cases.

To clarify the clinical spectrum of coronary arterial abnormalities in systemic lupus erythematosus, the data were reviewed on six patients who had a diagnosis of lupus at ages 15 to 29 years and who had ischemic heart disease before age 35. Two patients had coronary arteritis diagnosed on postmortem examination. In a third patient alterations in coronary arterial anatomy occurred with angiographic improvement temporally related to the initiation of steroid therapy. The other three patients had severe diffuse atherosclerotic coronary disease that was identified in two at postmortem examination. In the third patient the course of the disease strongly suggested coronary atherosclerosis, and eventually coronary bypass grafting was performed for relief of angina. In summary, clinically important extramural coronary arteritis and atherosclerosis both occur, although rarely, in young patients with lupus. Coronary artery disease may occur with or without coexisting active extracardiac lupus manifestations. Short-term steroid therapy and follow-up angiography for those with angina and in whom coronary arteritis is suspected warrant consideration. When stable coronary arterial anatomy is demonstrated on follow-up angiography, management is determined by the patient's symptoms irrespective of the prior history of lupus and, if indicated, cardiac surgery for symptomatic relief can be safely performed.

Adolescent

Aberrant origin of the left coronary artery from the proximal right coronary artery: diagnostic features and pre- and postoperative course.

In this report, we present the pre and late postoperative course of a patient with severe angina secondary to aberrant origin or the left coronary artery from the proximal righ coronary artery (Fig. 1). We illustrate the noninvasive diagnosis and evaluation of this patient by two-dimensional ultrasound and stress thallium imaging, and the pre and late postoperative angiographic and thallium perfusion findings.

Angina Pectoris

Multifocal or chaotic atrial rhythm: report of nine infants, delineation of clinical course and management, and review of the literature.

Nine infants with episodic or continuous chaotic atrial rhythm (CAR) are presented. In addition to 3 or more different P-wave contours, atrial rates greater than 100 per minute, variable PP, RR, and PR intervals, and a discrete isoelectric baseline, findings included atrial rates that varied from a low of 50 to 120 to a high of 140 to 270 per minute, ventricular rates that varied from a low of 40 to 50 to a high of 180 to 270 per minute, and periodic sinus arrest with junctional escape rhythm. Except for the arrhythmia, all had a normal cardiac examination, ECG, chest x-ray film, and echocardiogram. Six infants were otherwise normal; one had an orbital rhabdomyosarcoma; one had neonatal asphyxia; and one had respiratory distress, bronchopulmonary dysplasia, and an intraventricular cerebral hemorrhage. The CAR persisted from 3 days to 20 months; it spontaneously reverted to normal sinus rhythm in 8 infants and persists in 1 infant at age 7 months. Digoxin (4 patients), propranolol hydrochloride (3 patients), quinidine sulfate (2 patients), and lidocaine (1 patient) did not alter the CAR. No patient had heart failure secondary to the CAR, although three also had episodes of sustained atrial tachycardia, which while present caused heart failure. All patients are functioning normally at home and have normal findings on cardiac examination and have normal ECGs at ages 3 to 38 months. Seven are in normal sinus rhythm, one has rare atrial premature contractions, and one has persistent CAR. We conclude that specific treatment was not necessary in these infants with CAR, except in those with associated sustained atrial tachycardia, which itself may cause heart failure.

Anti-Arrhythmia Agents

Congenital extracardiac shunts with tetralogy of Fallot.

Congenital extracardiac shunts in tetralogy of Fallot may significantly alter the history and clinical findings and also have significant management implications. An unusual variant of severe tetralogy of Fallot in a 54-year-old man enabled long survival with minimal symptoms. The patient had pulmonary valvular and infundibular stenosis, a large ventricular septal defect, an overriding aorta, and large volume systemic-to-pulmonary artery shunting from his right internal mammary and accessory internal mammary arteries to his right pulmonary artery.

Coronary Circulation

Extrahepatic biliary atresia associated with cyanotic congenital heart disease. Three case reports and a review.

Three patients with extrahepatic biliary atresia associated with cyanotic congenital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green-appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.

Bile Ducts

Aberrant coronary artery origin from the aorta. Report of 18 patients, review of literature and delineation of natural history and management.

To clarify the natural history and management of patients with aberrant origin of a coronary artery from the aorta, we reviewed 18 patients whose right (RCA) or left (LCA) coronary artery arose aberrantly and passed between the aorta and right ventricular infundibulum. Nine patients had aberrant LCA. Three young males died suddenly after exertion, each with proximal focal LCA stenosis. None of the six adults with angina (ages 36-70 years) studied angiographically had proximal LCA stenosis. Unlike the young, "sudden death-prone" patients in whom coronary bypass of proximal stenosis may have prophylactic value, our older patients with aberrant LCA did not have proximal stenosis or sudden death, and therefore LCA bypass for sudden death prophylaxis is not warranted. Nine patients (ages 18-60 years) had aberrant origin and course of the RCA. Seven patients studied angiographically because of angina had no focal proximal RCA stenosis, but two patients had hypoplastic RCA ostia. Although a potential concern in these latter patients, sudden death has not been reported with aberrant RCA, therefore in the absence of syncope, RCA bypass for sudden death prophylaxis is not indicated.

Adolescent

Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management.

Thirteen new patients and 174 patients previously reported with coronary arteriovenous fistula (CAVF) were reviewed to delineate the course and management of CAVF and to clarify the role of surgical ligation in the young asymptomatic patient. Patients were grouped according to age: 99 patients (four new and 95 reported) were less than 20 years old and 88 (nine new and 79 reported) were greater than or equal to 20 years old. Of those under 20 years of age, 19% had preoperative symptoms or CAVF-related complications, including congestive heart failure (CHF) in 6%, subacute bacterial endocarditis in 3% and death in one patient. Seventy-six patients younger than 20 years old had CAVF ligation with only one significant complication. In contrast, 63% of the older group and all of our nine older patients had preoperative symptoms or complications, including CHF in 19%, SBE in 4%, myocardial infarction (MI) in 9%, death in 14% and fistula rupture in one patient. Of the 43 ligated older patients, 23% had surgical complications, including MI in three and death in three. Mean pulmonic-to-systemic flow in the entire group was 1.6:1 and did not differ significantly in those with or without symptoms or complications. One of our patients and one previously reported had spontaneous CAVF closure. In summary, early elective ligation of CAVF is indicated in all patients because of the high incidence of late symptoms and complications and the increased morbidity and mortality associated with ligation in older patients.

Adolescent

Secundum atrial septal defect and significant mitral regurgitation: incidence, management and morphologic basis.

To better understand the association between mitral regurgitation and secundum atrial septal defect and to clarify the evaluation and management of these patients, the records of 235 adult patients with atrial septal defect were reviewed. Ten patients (4 percent) had significant mitral regurgitation defined by clinical, hemodynamic and angiographic criteria. Three patients required mitral valve replacement at the time of closure of the atrial septal defect and four patients had closure alone, one of whom required mitral valve replacement after five years. Three patients did not undergo closure of the atrial septal defect or mitral valve replacement because of severe coexisting medical problems. In six patients, the mitral valves were studied pathologically and all had thick, fibrotic leaflets and short, thick, fibrotic chordae tendineae. Three of these valves also had scattered areas of patchy myxomatous degeneration and three had areas of vascular ingrowth suggestive of rheumatic disease. Although both invasive and noninvasive studies have high-lighted the coincidence between atrial septal defect and mitral regurgitation, particularly the frequent association of mitral valve prolapse, our data indicate that this association rarely has clinical significance. Furthermore, the morphologic basis for mitral regurgitation in patients with atrial septal defect consists of leaflet and chordal thickening fibrosis and deformity rather than attenuation and ballooning as would be expected in mitral valve prolapse.

Adult

Long-term antiarrhythmic therapy in survivors of prehospital cardiac arrest. Initial 18 months' experience.

Ambulatory rhythm monitoring and chronic arrhythmia management were studied in 16 patients resuscitated from prehospital cardiac arrest. Asymptomatic complex ventricular arrhythmias (ACVA) occurred in 12 patients (75%) entering long-term follow-up during the first 12 months (average follow-up, 13.25 months). The patients' therapy consisted of a dose-adjusted, membrane-active antiarrhythmic drug regimen monitored by blood levels. While there has been little change in the frequency of ACVAs despite carefully controlled antiarrhythmic management, only one death has occurred during 212 patient-months of postarrest follow-up, a 6% one-year mortality. This compares favorably to our previous experience in survivors of prehospital cardiac arrest not receiving a controlled antiarrhythmic program. Despite the failure to suppress ACVAs, the drug-monitored population is showing a trend toward a decreased frequency of recurrent cardiac arrest.

Ambulatory Care