Jerome Liebman

Cardiac Rhythm after the Mustard Operation for Complete Transposition of the Great Arteries

The Mustard operation corrects the effects of congenital transposition of the great arteries by creating an intraarterial baffle to direct pulmonary venous blood to the tricuspid orifice and systemic venous blood to the mitral orifice. To identify the long-term effects of this procedure, we followed 372 patients with complete transposition of the great arteries who survived the Mustard operation for at least three months. The mean follow-up period was 4.5 years (range, 0.4 to 15.9); the mean age at operation was 2.0 years. Mean resting heart rates were consistently lower than those for age-matched normal children. Seventy-six per cent of the patients had sinus rhythm during the year of operation--a figure that decreased to 57 per cent by the end of the eighth postoperative year. Twenty-five patients died during the follow-up period, nine suddenly. Life-table analysis revealed a cumulative survival rate of 91 per cent for 11 years and 71 per cent for 15 years after the operation. No strong risk factor for sudden unexpected death identified. This study demonstrates that extended survival among patients with transposition can be expected after the Mustard operation. However, over time there is a decreasing prevalence of normal sinus rhythm in survivors, as well as a small risk of sudden death.

Natural History of Transposition of the Great Arteries Anatomy and Birth and Death Characteristics

The natural history of transposition of the great arteries between 1957 and 1964 is presented based on a total of 742 cases. The area of study was the State of California and included 290 cases from 14 selected hospital centers. It is clear that in this era of more successful surgery, no such study will ever again be possible.Of major importance has been a compilation of a life table so that present day cardiologists and surgeons, when evaluating whether surgery should be undertaken in a particular patient, can weigh the risk of the operative procedure against the expected number of years of life remaining if surgery is not done.For the whole group the age of death was as follows: by 1 week, 28.7%; by 1 month, 51.6%; and by 1 year, 89.3%.The average life expectancy at birth was 0.65 years; at 1 week of age, 0.87 years; at 1 month, 1.12 years; and at 1 year, 3.92 years.Associated lesions had a marked effect on prognosis. The life tables presented tell a more complete story, and the pertinent data are presented in detail. (See longer summary at end of paper for further information.)

Long-Term Prognosis of Rheumatic Fever Patients Receiving Regular Intramuscular Benzathine Penicillin

The prognosis for 115 rheumatic fever patients subsequently receiving regular intramuscular penicillin prophylaxis for at least 5 years was studied. Average follow-up was 9.3 years, and 57 patients were followed for 10 years or longer. Of the 79 patients with acute mitral regurgitation, 70% lost their murmur from 4 days to 8.5 years after it was first heard. This is in contrast to those with acute aortic regurgitation which persisted in 73%. No patient developed mitral or aortic stenosis: this suggests that regular prophylaxis may prevent the development of stenosis. Congestive failure, cardiomegaly, and arrhythmia correlated closely with persistence of murmurs, but increased P-R interval, acute QRS or T-voltage variation or both, and ST-T abnormalities did not.

Infective endocarditis in infants and children during the past 10 years: A decade of change

Abstract Infective endocarditis (IE) is closely associated with congenital heart diseases. 1–9 In major series done over several decades up to the early 1970s 2,6 and in a recent review 8 both the changing pattern of etiologic agents and the increasing incidence of IE overal has been stressed. In the last decade, the practice of pediatric cardiology has changed greatly. New diagnostic methods such as M-mode and two-dimensional echocardiography (2DE) have been developed. The rate of survival, particularly that of infants and of children with complex heart diseases, has greatly increased, coincident with improved surgery and intensive care of severely ill infants and children. Advances have also occurred in the antibiotic armamentarium. With these multiple recent developments it was questioned whether a change might have also taken place in the pattern of IE. Few reviews, however, dealing with the pattern of pediatric IE over the last decade are available. 8–10 This report is a review of our experience in diagnosis and treatment of pediatric IE during the last decade (1972 to 1982).

Assessment of the pulmonary vascular bed by echocardiographic right ventricular systolic time intervals.

SUMMARY Echocardiography was used to measure right ventricular systolic time intervals (RVSTI) in 85 normal children (group I) and in 229 patients undergoing cardiac catheterization (group II). Corrected right ventricular pre-ejection period (RPEPC) and right ventricular ejection time (RVETC) (based on regression analysis of group I) and RPEP/RVET were each correlated with pulmonary artery (PA) diastolic and mean pressures and pulmonary vascular resistance (PVR). The best correlation (0.83) was between a second degree polynomial of the RPEP/RVET and PA diastolic pressure. The RPEP/RVET allowed prediction of PA diastolic pressure within 10 mm Hg in 85% of the patients. The utility of RPEP/RVET was confirmed in sequential data of 22 patients, in whom alteration in RPEP/RVET accurately reflected the changing PA diastolic pressure. The RPEP/RVET could not be used to assess PA pressure in six patients with congestive cardiomyopathy nor in 18 patients with complete right bundle branch block (CRBBB).

The association of congenital heart disease with down's syndrome (mongolism)

Abstract Between 1957 and 1964 in California there were 7,625 known deaths due to congenital heart disease; in 367 of these cases the subjects had Downs syndrome. Autopsy records from 14 hospital centers allowed more specific anatomic diagnoses in 139 of the 367 cases. Five types of defects, with some overlap, accounted for 91.4 per cent of the cases: ventricular septal defect in 55 cases (32.4 per cent), atrioventricularis communis in 34 (24.5 per cent), atrial septal defect in 28 (20.1 per cent), patent ductus arteriosus in 16 (11.5 per cent) and tetralogy of Fallot in 15 (10.8 per cent). It was not possible to ascertain how many of the atrial septal defects were of the ostium primum variety, nor how many of the ventricular septal defects were of the atrioventricular canal type. The 139 cases are compared with 172 documented anatomic findings from four other studies. The major difference in this, the largest single study, is the larger number with ventricular septal defect as opposed to atrioventricularis communis. Many of the ventricular septal defects were associated with other defects, however.

Electrocardiographic body surface potential mapping in the Wolff-Parkinson-White syndrome. Noninvasive determination of the ventricular insertion sites of accessory atrioventricular connections.

BackgroundA reliable, noninvasive procedure to determine the location of accessory atrioventricular connections in patients with Wolff-Parkinson-White syndrome would add an important diagnostic tool to the clinical armamentarium. Methods and ResultsBody surface potential mapping (BSPM) using 180 electrodes in varioussized vests and displayed as a calibrated color map was used to determine the ventricular insertion site of the accessory atrioventricular (AV) connections in 34 patients with Wolff-Parkinson-White syndrome. Attempts were made to determine the 17 ventricular insertion sites described by Guiraudon et al. All 34 patients had an electrophysiologic study (EPS) at cardiac catheterization, and 18 had surgery so the ventricular insertion sites could be accurately located using EPS at surgery. A number of physiologic observations were also made with BSPM. ConclusionsThe following conclusions were drawn: 1) BSPM using QRS analysis accurately predicts the ventricular insertion site of accessory AV connections in the presence of a delta wave in the electrocardiogram; 2) the ventricular insertion sites of accessory AV connections determined by BSPM and by EPS at surgery were identical or within one mapping site (1.5 cm or less) in all but four of 18 cases; three of the four exceptions had more than one accessory AV connection, and the other had a very broad ventricular insertion; 3) BSPM and EPS locations of the accessory AV connections correlated very well in the 34 cases despite the fact that BSPM determines the ventricular insertion site and EPS determines the atrial insertion site of the accessory AV connection; 4) as suggested by the three cases of multiple accessory AV connections, EPS and BSPM may be complementary since BSPM identified one pathway and EPS identified the other (in the case with a broad ventricular insertion, BSPM and EPS demonstrated different portions of that insertion); 5) BSPM using ST-T analysis is very much less accurate in predicting the ventricular insertion site of accessory AV connections unless there is marked preexcitation; 6) standard electrocardiography using the Gallagher grid methodology (but with no attempt at stimulating maximal preexcitation) was not as accurate as QRS analysis ofBSPM in predicting the ventricular insertion site of the accessory AV connection; however, exact comparison is hampered by the different number and size of the Gallagher and Guiraudon insertion sites; 7) BSPM using QRS analysis appears to be very accurate in predicting right ventricular versus left ventricular posteroseptal accessory AV connections; 8) typical epicardial right ventricular breakthrough, indicative of conduction via the specialized AV conduction system, occurs in all patients with left ventricular free wall accessory AV connections; 9) epicardial right ventricular breakthrough was not observed in cases with right ventricular free wall or anteroseptal accessory AV connections; 10) epicardial right ventricular breakthrough can occur in the presence of posteroseptal accessory AV connections, whether right or left ventricular; and 11) the delay in epicardial right ventricular breakthrough in cases with left ventricular insertion may provide a marker to estimate the degree of ventricular preexcitation. (Circulation 1991;83:886–901)

Electrocardiographic body surface potential maps of the QRS of normal children

Electrocardiographic body surface potential maps (BSPM), utilizing 180 active dry electrodes imbedded in an inflatable vest, were obtained in 40 normal children, ages 8 to 18. The potential levels of the maps are displayed as different colors. A qualitative analysis indicated that the onset of right ventricular breakthrough could always be recognized in the upstroke of the QRS by a pseudopod from a right shoulder minimum extending into an anterior maximum, at an average of 24.4 +/- 4.2 msec., for an average QRS duration of 75.0 +/- 7.1 msec. However there was considerable normal variation, particularly in the mid and late QRS. At the time of depolarization of the free walls of the ventricles, the maximum often remained anterior, with an extension posterior, even through the Frank system vectorcardiogram invariably was posterior. Most remarkable was the terminal QRS of the BSPM, where the terminal maximum may be right superior anterior, anterior superior, or right posterior, presumably reflecting the right ventricular outflow tract, the superior septum, or the posterior basal left ventricle.

Persistence of fetal circulation syndrome: an echocardiographic study.

Serial echocardiograms were performed on 17 infants with persistence of fetal circulation syndrome to measure right ventricular systolic time intervals from pulmonic valve echograms and left ventricular systolic time intervals from aortic valve echograms. Right ventricular pre-ejection period/right ventricular ejection time ratio was prolonged in PFCS when compared to that in normal newborn infants, and diminished with clinical improvement. Left ventricular pre-ejection period/left ventricular ejection time ratio was prolonged in infants with PFCS. Echographic RPEP/RVET was consistent with the elevated pulmonary artery pressure and pulmonary vascular resistance of PFCS; elevated LPEP/LVET suggested left ventricular dysfunction.

The effect of high lung conductivity on electrocardiographic potentials. Results from human subjects undergoing bronchopulmonary lavage.

The effect of increased lung conductivity on ECG potentials was studied in human subjects undergoing pulmonary lavage of a whole lung. In this procedure, the air in the lung is replaced by physiologic saline solution, which is a highly conductive fluid. The same situation was simulated theoretically with an eccentric spherical model of the heart and torso. Both the experimental results and theoretical simulations show a decrease in body-surface potentials as the lung conductivity increases. In particular, a large decrease was observed in the posterior vector and the scalar Z lead both experimentally and theoretically. The model simulation shows that the scalar Z lead is maximal at a conductivity value that is very close to the typical normal lung conductivity, so that low voltages are predicted for low lung conductivities as well.