Richard R. Liberthson

Task force 1: the changing profile of congenital heart disease in adult life.

The extraordinary advances in cardiac surgery, intensive care, and noninvasive diagnosis over the last 50 years have led to an enormous growth in the U.S. and throughout the world in the number of adults with congenital heart disease (CHD). Approximately 85% of babies born with cardiovascular

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.

Prehospital ventricular defibrillation. Prognosis and follow-up course.

Abstract The subsequent course of 301 subjects with prehospital ventricular fibrillation detected by rescue units was analyzed. Defibrillation was successful in 199. Of these patients, 98 died before admission; of 101 who were hospitalized, 59 died in the hospital, and 42 were discharged alive. Initially rapid post-defibrillation heart rates and atrial fibrillation or sinus tachycardia were associated with good survival in contrast to slow rates and idioventricular or junctional rhythms. Among hospitalized patients ventricular fibrillation or ventricular tachycardia recurred in 57 per cent and in most within 24 hours. Acute myocardial infarction was diagnosed in 35 per cent, and ischemia alone (predominantly of the anterior wall) in 32 per cent. In 17 per cent no acute myocardial changes evolved. Congestive heart failure occurred in 63 per cent, cardiogenic shock in 25 per cent, and pulmonary complications in 42 per cent. Of the 42 who were discharged, 60 per cent returned to pre-arrest status; 28 per cen...

Pathophysiologic observations in prehospital ventricular fibrillation and sudden cardiac death.

In order to better understand the problem of prehospital sudden cardiac death (SCD) two groups of individuals were studied. One group was monitored by rescue squads during attempted rescue. These subjects were defibrillated from prehospital ventricular fibrillation (VF) and hospitalized if they survived or autopsied if they could not be resuscitated. The second group were SCDs which were witnessed and described by observers. Detailed past histories of both groups were collected, and either clinical or autopsy diagnoses were obtained.On the day of death or VF, one quarter reported new symptoms (primarily chest pain and dyspnea) preceding collapse by more than 30 minutes, one quarter reported symptoms lasting from 1 to 30 minutes, and one half collapsed instantaneously or within 1 minute of acute symptoms. A history of old myocardial infarction (MI) was present in 41% and of angina pectoris in 54%, and 27% reported new or changing symptoms within four weeks.In defibrillated survivors, “would-be SCDs,’ electrocardiographic (ECG) changes of acute myocardial infarction (AMI) or ischemia were nearly three times more frequent than changes detected histologically in SCDs, and in the former involved predominantly the anterior wall in contrast to the inferior wall in most autopsied deaths. This disparity implicates acute myocardial lesions, particularly of the anterior wall, in the majority of SCDs.Acute coronary lesions were found in 58% of SCDs autopsied. Most of these were ruptured plaques although almost one fifth of all autopsied SCDs had thromboses without intimal rupture. Severe chronic multivessel stenosis was present in most subjects, although 15% had only disease of a single vessel and in these, the left anterior descending (LAD) or left main coronary artery were involved in three quarters.The SCD population may be subgrouped into those with recent MI, those with only myocardial ischemia, and those with no detectable myocardial change. When rescuers were able to monitor prehospital SCDs, VF was found in the majority; however, 28% did have other terminal rhythms.

Aberrant Coronary Artery Origin From the Aorta Diagnosis and Clinical Significance

Twenty-one patients with anomalous coronary artery origin from the aorta are discussed, and the cases reported in the literature are reviewed. The left anterior descending (LAD) and left circumflex (Cx) arteries arose aberrantly from the right sinus of Valsalva of the aorta (RSV) in six patients. In four of these patients the connecting branch from the anomalous origin passed anterior to the aorta and the right ventricular in-fundibulum (RVinf), and in two patients, this branch passed between the aorta and RVinf. In 11 patients only the Cx was aberrant, and arose either from the RSV directly or from the right coronary artery (RCA), and passed posterior to the aorta and RVinf. In four patients the RCA arose aberrantly — in three from the left sinus of Valsalva of the aorta passing anteriorly, and in one from the left Cx passing posteriorly.Aberrant coronary artery origin from the aorta had clinical consequences only when the branch connecting the LAD and Cx to the RSV passed between the aorta and RVinf. Both our findings and those reported in the literature associate this variant with exertional sudden death in young persons. Anomalous coronary patterns can be delineated readily by selective coronary cineangiography, and as illustrated, the right anterior oblique projection can readily distinguish those prone to sudden death from the clinically insignificant variants. The former can be corrected with coronary artery bypass surgery.

Interventricular septal configuration as a predictor of right ventricular systolic hypertension in children: a cross-sectional echocardiographic study.

Abnormal interventricular septal position and motion have been noted in patients with right ventricular pressure overload. The quantitative relationship between this alteration in septal configuration and the severity of right ventricular systolic hypertension has not been previously reported. We used cross-sectional echocardiography to assess the radius of septal curvature at end-diastole, midsystole, and end-systole in 20 normal children and 29 children (ages 2 weeks to 20 years) undergoing cardiac catheterization for a variety of congenital cardiac disorders. The measured septal radius of curvature (r) was normalized by the ideal radius (ri) for the left ventricular cavity area and then expressed as normalized septal curvature [l/(r/ri)]. A slight leftward shift and flattening of the interventricular septum occurred in the course of normal systolic contraction (mean +/- SEM normalized curvature at end-diastole 0.92 +/- 0.03 and at end-systole 0.85 +/- 0.02; p less than .05). Marked exaggeration of this...

Coarctation of the aorta: Review of 234 patients and clarification of management problems

Abstract Two hundred thirty-four patients with coarctation of the aorta who were managed between 1948 and 1978 were reviewed. Their ages ranged from 1 day to 72 years. Only 6 percent of the patients who had surgical correction between ages 1 and 5 years had residual hypertension. In contrast, 21 percent of those operated on before age 1 year (all with residual or recurrent coarctation), 30 percent of those operated on between ages 6 and 18 years, 47 percent of those operated on between ages 19 and 40 years and 50 percent of patients over 40 age years at the time of operation had residual postoperative hypertension. These data suggest that the optimal time for elective surgical correction of coarctation is between the ages of 1 and 5 years. Preoperative congestive heart failure was present in 67 percent of patients under age 1 year, in 67 percent of those over age 40 years and in only 4 percent of those aged 1 to 40 years. Eight of the 29 infants with preoperative heart failure had residual postoperative failure related in most to associated congenital cardiac anomalies. Six of the 16 patients with preoperative heart failure who were over age 40 years at correction had residual failure, although even in these patients heart failure was improved. Bacterial endocarditis, cerebral vascular accident, myocardial infarction and aortic dissection were present in 2, 6, 3 and 2 percent of patients, respectively; these complications occurred more frequently with advancing age and occurred in 13 patients who had had prior coarctation repair. Additional congenital heart lesions were present in 34 percent of the patients. Of those under age 1 year, 67 percent had a clinically significant patent ductus arteriosus with or without ventricular septal defect or aortic stenosis. After age 1 year, 27 percent of the patients had associated cardiac lesions, and no patient had a clinically significant patent ductus or ventricular septal defect. Aortic stenosis or insufficiency was present in 33 patients. When identified during infancy, aortic stenosis was often clinically significant. Thereafter, only eight patients had clinically significant aortic valve disease at the time of this review, and only two of these patients were younger than age 40 years.

Discrete membranous subaortic stenosis. Report of 31 patients, review of the literature, and delineation of management.

The presentation, management, and follow-up of 31 patients with discrete membranous subaortic stenosis (DMSS) is presented. DMSS comprised 16%percnt; 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 < 90 mm Hg. Bacterial endocarditis was found in 13%percnt;, and in 13%percnt; 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.

Ischemie heart disease in systemic lupus erythematosus in the young patient: Report of six cases

Abstract 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 ischemie 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 patients symptoms Irrespective of the prior history of lupus and, if indicated, cardiac surgery for symptomatic relief can be safely performed.

Single (Primitive) Ventricle

Single ventricle is that condition in which both atrioventricular (A-V) orifices enter a common ventricular sinus, from which the arterial trunks emerge with their coni. The following types of single ventricle were found: (1) with regular (noninverted) transposition in levocardia; (2) with inverted transposition in levocardia; (3) with normal position of arterial trunks in levocardia; (4) in dextrocardia, and (5) in mesocardia. This entity is differentiated from common ventricle which is basically a heart with a large ventricular septal defect. Single ventricle does not include mitral and tricuspid atresia by definition, nor does it include straddling tricuspid orifice, in which two distinct ventricles are present. The concept of single ventricle herein defined differs from de la Cruz and Millers concept of double-inlet left ventricle which includes straddling tricuspid orifice. Embryologically in single ventricle we are dealing with persistence of the primitive state of the bulboventricular loop, due to a lack of expansion of the atrial canal to the right during the process of absorption of the bulbus.