General anesthesia in a child with urticaria pigmentosa.
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Biomedical subjects
Publications and source records attributed to R R Liberthson.
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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.
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.
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.
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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.
Three cases of total unilateral pulmonary venous connection of the left lung with intact atrial septum are described. All patients were acyanotic and had findings of volume overload of the right side of the heart. An anomalous vertical vein in the superior mediastinum and large pulmonary arteries were visible on the plain chest radiograph, similar to what is seen in cases of total anomalous pulmonary venous connection. Selective pulmonary angiography delineated the anomalous connection of the left pulmonary veins and documented the absence of an atrial septal defect.
To evaluate the role of QT interval prolongation in the genesis of the sudden infant death syndrome (SIDS), the post-resuscitation electrocardiograms of 21 aborted SIDS infants were reviewed. The infants had been found apneic, cyanotic, limp and unresponsive during sleep and required vigorous physical stimulation and mouth-to-mouth resuscitation. Three subsequently experienced repeat similar episodes from which they could not be resuscitated. Extensive studies eliminated all "known" etiologies for death. The QT intervals of these infants were compared to age and sex matched normal infants as well as to established normal values in the literature; in both the aborted and the subsequent actual SIDS infants, the QT intervals were not significantly different from those of the normal population. Thus, we conclude that QT intervals prolongation does not play a major role in the genesis of the aborted SIDS.
The presentation, management, and follow-up of 31 patients with discrete membranous subaortic stenosis (DMSS) is presented. DMSS comprised 16% of 185 patients with congenital left ventricular (LV) obstruction. Only one patient was older than 40 years. The rarity of DMSS in older patients in both our population and in the literature is noted, and possible explanations are discussed. One-quarter of these patients had dyspnea, chest pain, or syncope combined with electrocardiographic left ventricular hypertrophy (LVH) and strain, and these all had peak LV outflow gradients (PSG) greater than 85 mm Hg. One-quarter had neither symptoms nor electrocardiographic abnormalities and all had PSG less than 90 mm Hg. Bacterial endocarditis was found in 13%, and in 13% an immediate family member also had congenital LV obstruction. Following surgical resection (25 patients), 18 were asymptomatic, two had residual fibromuscular obstruction, and four developed new fibromuscular obstruction after from one to six years (leading in one to late sudden death). Thus, even after resection, these patients require continued re-evaluation for residual or new LV obstruction.
Causative factors, clinical consequences and treatment of atrial tachyarrhythmias were reviewed in 917 monitored patients with definite acute myocardial infarction. Significant atrial tachyarrhythmias were found in 104 (11 per cent) of them and included atrial fibrillation in 67, atrial flutter in 29 and paroxysmal atrial tachycardia in 33. These episodes were single in 79 patients and multiple in 25, and began within the first four days of acute myocardial infarction in 90 per cent of the patients. Fifty per cent of these atrial tachyarrhythmias were heralded by premature atrial contractions. The incidence of atrial tachyarrhythmia was not related to the location of the acute myocardial infarction or to the presence or degree of power failure; however, atrial tachyarrhythmias were significantly more frequent in patients with pericarditis. Atrial tachyarrhythmias were well tolerated in almost one fifth of the patients, caused marginal compromise in almost two thirds and led to severe clinical deterioration in one fifth. Paroxysmal atrial tachycardia rarely required specific treatment, atrial fibrillation was best managed with intravenous administration of digoxin except when associated with severe clinical compromise, and atrial flutter generally required cardioversion or rapid intravenous therapy and usually caused severe clinical deterioration. Over-all, atrial tachyarrhythmia was not associated with a significantly increased mortality, and in those who died, death was not related specifically to the atrial tachyarrhythmia but rather to the severity of the underlying acute myocardial infarction. However, persisting atrial tachyarrhythmias, particularly atrial flutter which tends to be refractory to both heart rate control and cardioversion, may contribute indirectly to morbidity and mortality.
Seven patients with pulmonary regurgitation (PR), normal pulmonary artery (PA) pressures and large left-to-right atrial shunts are reported. Six had secundum atrial septal defects (ASD) and one had anomalous pulmonary venous drainage. These comprised 4% of 180 patients with atrial shunts and normal PA pressures. Pulmonary regurgitation was diagnosed clinically by mid-frequency diastolic decrescendo murmurs beginning after the pulmonic component of the second heart sound, and diagnoses were confirmed by catheterization. In two patients who had serial preoperative catheterizations over 8 and 16 years, PR progressed in one and was present only on the second study in the other. All patients underwent shunt correction, at which time the pulmonic anulus and artery appeared dilated, but the pulmonic valves were normal and did not require revision. In all patients the PR murmur disappeared after shunt correction alone, and on chest X-ray both PA and overall heart size decreased. Although it is known that pulmonary regurgitation occurs with atrial septal defects and pulmonary hypertension, the present study demonstrates that it also occurs with high flow atrial shunts, in which setting it has different implications and is reversible with shunt correction alone.
We investigated the function of the conus arteriosus in the bullfrog Rana catesbeiana using a combination of anatomical and physiological techniques. Although there is a normal delay in ventriculoconal conduction and we could induce a spectrum of ventriculoconal conduction disturbances by manipulating the region of the ventriculoconal junction, we found no histological evidence of specialized conducting myocardial tissue in this region. The performance of the conus arteriosus was explored during various disturbances of ventriculoconal conduction and also during hemodynamic disturbances produced by hemorrhage and afterloading. The conus was found to contribute little to forward flow under ordinary circumstances, but its contribution increased greatly during bleeding or partial occlusion of the truncus. In contrast to the conclusion of others, no evidence could be adduced to support the idea that the conus serves as a depulsating chamber. Disparities in previous reports concerning the operation of the conus as a booster pump are attributed to special experimental circumstances.
Prehospital ventricular fibrillation can be treated successfully by rescue (paramedic) personnel. By retrospective history, one-half of prehospital sudden death victims experienced no prior warning and collapsed either instantaneously or within one minute of symptoms. The follow-up course was analyzed. Of 301 such subjects, 199 were defibrillated successfully, but only 101 survived long enough to be hospitalized, and only 42 were discharged alive. Predictors for successful outcome were initially rapid post-defibrillation heart rates and atrial fibrillation or sinus tachycardia rhythms. In the hospitalized group, 57% suffered repeat ventricular fibrillation and/or ventricular tachycardia usually during the first 24 hours. Among those discharged from the hospital, mean survival was 13 months with 28% dying suddenly regardless of presence of antiarrhythmic therapy. Intensive antiarrhythmic therapy and monitoring of this survivor population seems indicated.
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