Edward I. Curtiss

Physiologic Abnormalities of Cardiac Function in Progressive Systemic Sclerosis with Diffuse Scleroderma

To investigate cardiopulmonary function in progressive systemic sclerosis with diffuse scleroderma, we studied 26 patients with maximal exercise and redistribution thallium scans, rest and exercise radionuclide ventriculography, pulmonary-function testing, and chest roentgenography. Although only 6 patients had clinical evidence of cardiac involvement, 20 had abnormal thallium scans, including 10 with reversible exercise-induced defects and 18 with fixed defects (8 had both). Seven of the 10 patients who had exercise-induced defects and underwent cardiac catheterization had normal coronary angiograms. Mean resting left ventricular ejection fraction and mean resting right ventricular ejection fraction were lower in patients with post-exercise left ventricular thallium defect scores above the median (59 +/- 13 per cent vs. 69 +/- 6 per cent [P less than 0.025], and 36 +/- 12 per cent vs. 47 +/- 7 per cent [P less than 0.025], respectively). We conclude that in progressive systemic sclerosis with diffuse scleroderma, abnormalities of myocardial perfusion are common and appear to be due to a disturbance of the myocardial microcirculation. Both right and left ventricular dysfunction appear to be related to this circulatory disturbance, suggesting ischemically mediated injury.

Cardiac tamponade: hemodynamic observations in man.

SUMMARY Hemodynamic studies were performed before and after pericardiocentesis in 19 patients with pericardial effusion. Right atrial pressure decreased significantly, from 16 ± 4 mm Hg (mean ± SD) to 7 ± 5 mm Hg in 14 patients with cardiac tamponade. This change was accompanied by significant increases in cardiac output (3.87 ± 1.77 to 7 ± 2.2 I/min) and inspiratory systemic arterial pulse pressure (45 ± 29 to 81 ± 23 mm Hg). The remaining five patients did not demonstrate cardiac tamponade, as evidenced by lack of significant change in these hemodynamic parameters.In all patients with tamponade, right ventricular end-diastolic pressure (RVEDP) was elevated and equal to pericardial pressure; equilibration was uniformly absent in patients without tamponade. During gradual fluid withdrawal in the tamponade group, significant hemodynamic improvement was largely confined to the period when right ventricular filling pressure remained equilibrated with pericardial pressure. In 10 patients with tamponade and pulsus paradoxus, pulmonary arterial wedge pressure (PAW) was equal to pericardial pressure except during early inspiration and expiration when it was transiently less and greater, respectively; however, inspiratory right atrial pressure never fell below pericardial pressure. In these 10 patients, PAW decreased significantly following pericardiocentesis (P < 0.001). In the remaining four patients with tamponade but without pulsus paradoxus, all of whom had chronic renal failure, PAW was consistently higher than pericardial pressure or RVEDP and did not decrease after pericardiocentesis.These data tend to confirm the hypothesis that in patients with tamponade, the venous pressure required to maintain any given cardiac volume is determined by pericardial rather than ventricular compliance. When pericardial compliance determines diastolic pressure in both ventricles, relative filling of the ventricles will be competitive and determined by their respective venous pressures (pulmonary vs systemic), which vary with respiration and alternately favor right and left ventricular filling. This results in pulsus paradoxus. However, if pulmonary arterial wedge pressure is markedly elevated before the onset of tamponade, as in patients with chronic renal failure, then pericardial compliance may only determine right ventricular filling pressure. In such cases, pulsus paradoxus may be absent.

The Duration of Holter Monitoring in Patients With Syncope: Is 24 Hours Enough?

To determine the incremental yield of ambulatory monitoring in the evaluation of syncope, three serial 24-hour Holter recordings were obtained in a consecutive series of 95 patients with syncope, the cause of which was not explained by history, physical examination, or 12-lead electrocardiogram. The mean age of patients was 61 years and 41% were men. Major electrocardiographic abnormalities were found in 26 patients (27%), including unsustained ventricular tachycardia (19 patients), pauses of at least 2 seconds (8 patients), profound bradycardia (1 patient), and complete heart block (1 patient). The first 24-hour Holter recording had at least one major abnormality in 14 patients (15%) (95% confidence interval, 8.3% to 23.4%). Of the 81 patients without a major abnormality on the first Holter recording, the second Holter recording had major abnormalities in 9 (11%) (95% confidence interval, 5.1% to 20.0%). Of the 72 patients without a major abnormality on the first two Holter recordings, only 3 patients (4.2%) had a major abnormality on the third Holter recording (95% confidence interval, 0.8% to 11.7%). Four factors were significantly associated with an increased likelihood of a major abnormality on 72 hours of monitoring: age above 65 years (relative risk, 2.2), male gender (relative risk, 2.0), history of heart disease (relative risk, 2.2), and an initial nonsinus rhythm (relative risk, 3.5). These results suggest that 24 hours of Holter monitoring is not enough to identify all potentially important arrhythmias in patients with syncope. Monitoring may need to be extended to 48 hours if the first 24-hour Holter recording is normal.

Spectrum of hemodynamic changes in cardiac tamponade

To investigate the pathophysiology of cardiac tamponade, the hemodynamics of 77 consecutive patients with greater than 150 ml of pericardial effusion were studied. Patients were classified into 3 groups based on the equilibration of intrapericardial with right atrial and pulmonary arterial wedge pressures (mm Hg): group I (n = 16), intrapericardial pressure was less than right atrial and pulmonary arterial wedge pressures; group II (n = 13), intrapericardial pressure was equilibrated with right atrial but not pulmonary arterial wedge pressures; group III (n = 48), intrapericardial pressure was equilibrated with right atrial and pulmonary arterial wedge pressures. Pericardiocentesis produced the following changes: group I--significant (p less than 0.03) decreases in intrapericardial pressure (7 +/- 2 mm Hg), right atrial pressure (3 +/- 2 mm Hg), pulmonary arterial wedge pressure (2 +/- 2 mm Hg), and the inspiratory decrease in arterial systolic pressure (3 +/- 4 mm Hg) but no significant change in cardiac output; group II--significant (p less than 0.02) decreases in intrapericardial pressure (11 +/- 5 mm Hg), right atrial pressure (6 +/- 4 mm Hg), pulmonary arterial wedge pressure (4 +/- 5 mm Hg), and inspiratory decrease in arterial systolic pressure (8 +/- 7 mm Hg), and increase in cardiac output (1.1 +/- 1.2 liters/min); group III--significant (p less than 0.001) decreases in intrapericardial pressure (16 +/- 7 mm Hg), right atrial pressure (9 +/- 4 mm Hg), pulmonary arterial wedge pressure (8 +/- 5 mm Hg), inspiratory decrease in arterial systolic pressure (17 +/- 11 mm Hg), and increase in cardiac output (2.8 +/- 1.5 liters/min). The changes after pericardiocentesis in all parameters were significantly (p less than 0.05) greater in group III than in groups I or II except for the change in right atrial pressure, which was not significantly different in groups II versus III. The changes after pericardiocentesis indicate pericardial effusion caused the greatest abnormalities in group III but also caused significant abnormalities of pressure and flow in group II and of pressure alone in group I.(ABSTRACT TRUNCATED AT 250 WORDS)

The electrocardiogram in systemic sclerosis (scleroderma). Study of 102 consecutive cases with functional correlations and review of the literature

The electrocardiographic findings in 102 consecutive patients with scleroderma were reviewed to determine the frequency and nature of the electrocardiographic abnormalities associated with this disease. Septal infarction pattern unassociated with QRS prolongation was present in 10 percent, compared with none of 96 control subjects (p less than 0.001). Ventricular conduction abnormalities were present in 17 percent. A normal electrocardiogram was obtained in 49 percent. A subset of 48 patients underwent detailed cardiopulmonary evaluation including exercise thallium scintigraphy, rest and exercise radionuclide ventriculography, pulmonary function tests, and chest roentgenography. Functional correlations of the electrocardiographic findings were examined in this subset. Septal infarction pattern (five of 48) and ventricular conduction abnormalities (10 of 48) were both associated with septal or anteroseptal thallium perfusion abnormalities (10 of 15 versus six of 33 of the remainder, p less than 0.005), which were present despite normal coronary angiographic results. Thallium defect scores were greater in patients with septal infarction pattern or ventricular conduction abnormalities compared with the remainder (defect scores 3.0 +/- 2.6 versus 1.4 +/- 2.2, respectively, p less than 0.025). In patients with ventricular conduction abnormalities, both left bundle branch block and right bundle branch block with left anterior fascicular block were associated with abnormal left ventricular function, whereas isolated right bundle branch block or left anterior fascicular block was associated with normal left ventricular function. A normal electrocardiographic finding (19 of 48) was associated with normal left ventricular function at rest (19 of 19). However, 11 of 19 (58 percent) had thallium perfusion defects and four of 19 (21 percent) had an abnormal response to exercise, although in none was the peak ejection fraction less than 50 percent. It is concluded that both septal infarction pattern and ventricular conduction abnormalities are electrocardiographic abnormalities associated with scleroderma heart disease; they appear to be a result of myocardial fibrosis. Some degree of myocardial fibrosis may be present with a normal electrocardiographic result, but significant left ventricular dysfunction is unlikely. Septal infarction pattern and ventricular conduction abnormalities, when present, are indicators of more advanced fibrosis.

Sound Pressure Correlates of the Second Heart Sound An Intracardiac Sound Study

The sound pressure correlates of the second heart sound were studied in 22 patients during diagnostic cardiac catheterization. Simultaneous right ventricular and pulmonary artery pressures were recorded with equisensitive catheter-tip micromanometers together with the external phonocardiogram and ECG. In 12 patients having normal pulmonary vascular resistance (group 1), pulmonic closure sound was coincident with the incisura of the pulmonary artery pressure curve which in turn was separated from the right ventricular pressure trace by an interval denoted hangout. The duration of this interval varied (33-89 msec), was independent of pulmonary artery pressure or resistance and was felt to be primarily a reflection of the capacitance of the pulmonary vascular tree. The absolute value of this interval during inspiration was very similar to the splitting interval and, when subtracted from the Q-P2 interval, the remaining interval (QRV) was almost identical to the Q-A2 interval, indicating that the actual duration of right and left ventricular systole is nearly equal. Awareness of the existence of the hangout interval and its hemodynamic determinants offers a reasonable mechanism to explain the audible expiratory splitting of the second heart sound found in patients with idiopathic dilatation of the pulmonary artery following atrial septal defect repair and in one additional patient studied with mild valvular pulmonic stenosis. In nine patients with elevated pulmonary vascular resistance approaching systemic levels (group 2), the absolute value of the hangout interval was markedly reduced (15-28 msec) consistent with the decrease in capacitance of the pulmonary vascular bed and the increased pulmonary vascular resistance known to occur in pulmonary hypertension. In those patients where the duration of right and left ventricular systole were nearly equal, narrow splitting of the second heart sound was present. In those patients where selective prolongation of right ventricular systole occurred, the narrow hangout interval persisted, but the splitting interval was prolonged proportionate to the increased duration of right ventricular systole.

Alterations of right ventricular systolic time intervals by chronic pressure and volume overloading.

Right ventricular (RV) systolic time intervals and hemodynamic parameters were determined by micromanometric techniques in 13 subjects with normal right ventricles (NRV). These data were compared to those of 16 patients with pulmonary hypertension (PH) or predominant pressure overloading and 13 individuals with uncomplicated secundum atrial septal defects (ASD) or predominant volume overloading.In PH, the QP2 interval tends to remain within the normal range due to reciprocal changes in isovolunmic contraction (ICT) and ejection (RVET) times. Elevations of pulmonary artery diastolic pressure are associated with increases in the mean rate of isovolumic pressure rise (MRIPR) (r = 0.84), but the latter change does not fully compensate for the widened ventriculoarterial diastolic pressure dif-splitference and ICT becomes prolonged (P ⩽ 0.001). Factors other than stroke index depression which may contribute to the decreased duration of RVET (P ⩽ 0.001) include tricuspid regurgitation and elevation of pulmonary vascular impedance.In ASD, QP2 is significantly prolonged (P ⩽ 0.025) due to a significant increase in RVET (P ⩽ 0.005). In contrast to NRV, a linear correlation of RVET and stroke index was not present, which suggested an alteration of ejection dynamics in this group. Despite a high incidence of complete or incomplete right bundle branch block, the interval from QRS onset to rapid RV pressure upstroke was not prolonged. This is most probably the result of peripheral bundle branch block of genesis of the QRS pattern by right ventricular hypertrophy.

Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis: Exercise radionuclide evaluation and comparison with diffuse scleroderma

Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease.

Pulsus paradoxus: definition and relation to the severity of cardiac tamponade.

Based on 101 control patients, the 95% confidence limit for the inspiratory decrease in arterial systolic pressure (delta SYST, mm Hg) and the percentage decrease in arterial systolic pressure (% delta SYST = delta SYST/expiratory systolic pressure) were determined to be 12 mm Hg and 9%, respectively. In 65 patients with pericardial effusion, the severity of cardiac tamponade was estimated on the basis of the percentage increase in cardiac output resulting from pericardiocentesis: absent = less than 20% (n = 24), mild = 20% to 49% (n = 9), and moderate or severe = greater than or equal to 50% (n = 32). The accuracy of a delta SYST greater than 12 mm Hg and % delta SYST greater than 9% in the identification of tamponade was 92% and 97%, respectively. A % delta SYST greater than 15% with relative hypotension (expiratory systolic pressure less than or equal to 120 mm Hg) or a delta SYST greater than 25 mm Hg or inspiratory pulse obliteration was found in 31 of 32 patients with moderate or severe tamponade compared to 2 of the 33 remaining patients. We conclude that the inspiratory decline in arterial systolic pressure can be used to accurately estimate the level of hemodynamic embarrassment resulting from pericardial effusion.

Myocardial ischemia after orthotopic liver transplantation

A hypercoagulable state exists after orthotopic liver transplantation. This hematologic abnormality may predispose patients to coronary thrombosis and unstable angina. The incidence of postoperative myocardial ischemia in such patients is unknown. Suitable electrocardiograms and clinical events of consecutive patients undergoing orthotopic liver transplantation (n = 45) and major intraabdominal surgery (n = 28) during a 3-month period at a major university teaching hospital and transplant center were examined retrospectively. Clinical myocardial ischemia or ischemic electrocardiographic changes, or both, occurred in 6 transplant patients compared with no patient in the nontransplant or comparison group. In 4 of the 6 patients with dramatic electrocardiographic changes and ischemic events, coronary arteriography failed to demonstrate significant obstructive disease. It is concluded that severe myocardial ischemia may occur in patients after orthotopic liver transplantation in the absence of significant coronary disease. A hypercoagulable state may predispose to coronary thrombosis in this setting, providing insight (and a future model for study) into the development of unstable angina.