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Single-lung transplantation for pulmonary hypertension. Three-month hemodynamic follow-up.

BACKGROUND: Shorter waiting times, relative technical simplicity, and satisfactory application to a broad spectrum of patients has made single-lung transplantation an attractive option in the treatment of patients with end-stage pulmonary hypertension. METHODS AND RESULTS: Seven patients with pulmonary hypertension underwent single-lung transplantation. Simultaneous closure of associated atrial septal defects was accomplished in two patients. Despite severely compromised pretransplant right ventricular function in all patients, there was no early or late mortality. Right ventricular functional recovery as characterized by hemodynamic assessment before and at a mean of 13 weeks posttransplant was nearly uniform and characterized by a drop in 1) pulmonary arterial systolic pressure from 92 +/- 7 mm Hg to 29 +/- 6 mm Hg (p = 0.001), 2) central venous pressure from 10 +/- 6 mm Hg to 1 +/- 2 mm Hg (p = 0.02), and 3) pulmonary vascular resistance index from 1,924 +/- 663 to 232 +/- 73 dyne.sec.cm-9 (p = 0.001). Radionuclide ventriculography before and at a mean of 17 weeks posttransplant documented a significant (p = 0.006) increase in right ventricular ejection fraction from 22 +/- 15% to 51 +/- 11%. Quantitative pulmonary perfusion scintigraphy at a mean of 17 weeks posttransplant demonstrated a significant (p = 0.001) increase in perfusion to the transplanted lung from 56 +/- 6% to 89 +/- 7%. There was a concomitant, slight but significant (p = 0.004) decrease in ventilation to the transplanted side from 56 +/- 6% to 49 +/- 8%. After transplantation, all patients returned to New York Heart Association functional class I or II from their preoperative levels of class III or IV. CONCLUSIONS: These early follow-up data cautiously support the option of single-lung transplantation in patients with pulmonary hypertension, although long-term durability of these hemodynamic changes deserves documentation before widespread application.

Follow-Up Studies↗

Regulation of human heart contractility by essential myosin light chain isoforms.

Most of the patients with congenital heart diseases express the atrial myosin light chain 1 (ALC-1) in the right ventricle. We investigated the functional consequences of ALC-1 expression on the myosin cycling kinetics in the intact sarcomeric structure using multicellular demembranated fibers ("skinned fibers") from the right ventricular infundibulum of patients with Tetralogy of Fallot (TOF), double outlet right ventricle (DORV), and infundibular pulmonary stenosis (IPS), Force-velocity relation was analyzed by the constant-load technique at maximal Ca2+ activation (pCa 4.5). Half-time of tension development (t1/2) was investigated by monitoring contraction initiation upon photolytic release of ATP from caged-ATP in rigor. The patients investigated here expressed between 0 and 27% ALC-1. There was a statistically significant correlation between ALC-l and maximal shortening velocity (Vmax) which rose 1.87-fold from 1.2 muscle length per second (ML/s) to 2.25 ML/s in a normal (0% ALC-1) and diseased (19.9% ALC-1) ventricle. Half-time of tension development decreased 1.85-fold with increasing ALC-1 expression (t1/2) was 0.252 s and 0.136 s at 2 and 18.4% ALC-1, respectively). We conclude that the expression of ALC-1 in the human heart modulates cross-bridge cycling kinetics accelerating shortening velocity and isometric tension production.

Adenosine Triphosphate↗

Use of a valved conduit from right atrium to pulmonary artery for "correction" of single ventricle.

Nine patients with single ventricle and pulmonary stenosis were treated by using a valved conduit from right atrium to the main pulmonary artery. The patient's own mobilized pulmonary valve was used in one patient and fresh, adult-sized aortic homograft in the remaining eight. Two additional unstented homografts were inserted at the entrance of both superior and inferior venae cavae in four patients. A pericardial baffle was used to direct pulmonary venous blood to both atrioventricular valves in three, and the tricuspid valve was oversewn in six. L-transposition of the great arteries was present in five patients and D-transposition in one. Tricuspid valve hypoplasia was present in one and mitral hypoplasia in one. The age at operation was 4 months to 36 years. All patients were severely incapacitated with arterial oxygen saturation varying from 28% to 76%. Four patients died within the first week after operation, the remaining five patients derived excellent symptomatic improvement. Repeat cardiac catheterization, performed in five patients, showed a mean right atrial pressure varying from 8 to 17 mm Hg and a prominent right atrial A wave, measuring 10 to 25 mm Hg, with evidence of good function of the homografts at the entrance of the venae cavae. The arterial oxygen saturation varied from 88% to 97%.

Adolescent↗

The relationship between cardiac function and neuropsychological status among heart transplant candidates.

BACKGROUND: Cognitive deficits among heart transplant candidates have been well documented. This study was designed to examine the hypothesis that impaired cognitive test performance among heart transplant candidates may be attributed, in part, to decreased cerebral perfusion secondary to poor cardiac function. METHODS AND RESULTS: Sixty-two patients participated in the study who underwent heart catheterization within 1 day of completing a battery of cognitive tests. Multiple demographic and patient characteristics were examined for their potential moderating role in the relationship between measures of cardiac function and cognitive performance including age, education, race, gender, psychiatric history, medication usage, cardiac surgical history, and self-reported symptoms of depression and anxiety. Only age and education were significantly related to cognitive performance (P < .01). Thus, partial correlation analyses controlling for age and education were used to examine the relationship between cardiac function and cognitive performance. In general, increasing hemodynamic pressure variables (ie, pulmonary artery pressure and right atrial pressure), and to a lesser extent cardiac output and cardiac index, were related (r = - .32 to - .43; P < .01) to decreased performance on cognitive tasks that assessed simple attention, speed of mental processing, and mental flexibility (Digit Span-Forward, Trail Making Test-Part B, Symbol Digits Modalities Test, and Stroop Neuropsychological Screening Test). Left ventricular ejection fraction, systemic and pulmonary vascular resistance, and mean arterial pressure were largely unrelated to cognitive performance in this sample of patients with end stage cardiac disease. CONCLUSIONS: Hemodynamic pressure variables seem to be most consistently related (ie, inversely) to cognitive functioning among heart transplant candidates.

Cognition Disorders↗

New observations on decremental atriofascicular and nodofascicular fibers: implications for catheter ablation.

INTRODUCTION: The purpose of this study was to characterize the anatomy and physiology of accessory pathways that exhibit anterograde decremental conduction. RESULTS: Among 100 consecutive patients with an accessory pathway undergoing electrophysiological study, six individuals with decremental anterograde accessory pathway conduction were identified. Anterograde accessory pathway effective refractory periods and conduction curves were assessed by atrial extrastimulus testing. Atrial pace mapping and ventricular activation sequence mapping were used to define accessory pathway origin and insertion. Surgical ablation (N = 1) or radiofrequency catheter ablation (N = 3) was performed based on accessory pathway anatomy as determined during electrophysiological study. Four of 6 patients had gaps in anterograde accessory pathway conduction. Two patients had evidence of functional longitudinal dissociation in the accessory pathway. Five of 6 patients had atriofascicular fibers with an atrial rather than AV nodal site of origin of their decrementally conducting accessory pathway and with distal insertions in the right bundle branch. Among these five patients, a right posterior atrial origin was nearly as common as a right anterior atrial origin. One patient had a true nodofascicular fiber that arose from the AV node, inserting distally into the left bundle branch. CONCLUSION: Most accessory pathways with anterograde decremental conduction arise from the right anterior or right posterior atrium, not the AV node. A gap in anterograde accessory pathway conduction and functional longitudinal dissociation are common in such accessory pathways. Surgical or catheter ablation of such pathways is effective when directed at the atrial origin of the accessory pathway. True nodofascicular fibers arising from the AV node are rare. These may insert distally in the left ventricle. Catheter ablation of the proximal origin of such fibers is likely to result in complete AV block.

Adult↗

Some aspects of cardiac heterogeneity.

It is remarkable that the heart, which obviously functions as a homogenous pump, shows such a high degree of heterogeneity. There are often contradictory or controversial results as far as transmural gradients are concerned (78). These discrepancies or inconsistencies, however, can at least in part be explained by species differences. Of particular interest is the heterogeneity that is due to the separation into two hearts. In view of the differences in the morphology and function of the left and right heart, it is surprising that the whole organ works in a rather homogenous fashion. In the past, the right heart has received not as much attention as the left heart. This is in particular true for small laboratory animals. It becomes more and more evident that compared to the left ventricle, the right ventricle reacts to various stimuli in a quantitatively different manner. More research on the right heart is therefore needed. Collectively, the data summarized in this article may be interpreted in that the heart not only works as a homogeneous unit, but may even benefit from the heterogeneity of its different parts or subunits under normal as well as under various pathophysiological conditions. Analysis of the processes involved in creating cardiac heterogeneity can provide new insights into the mechanism of adaptation of the heart.

Animals↗

Plasma levels of atrial natriuretic peptide and hemodynamic function in cardiac disease.

Plasma concentrations of atrial natriuretic peptide were measured in eight patients with cardiac disease but normal resting right atrial pressure, during cardiac catheterization. No patient had clinical evidence of overt heart failure. An increase in peptide concentrations was observed between the aorta or the peripheral vein and the pulmonary artery. A linear relation was found between peripheral vein and pulmonary artery peptide concentration. Mean pulmonary artery and capillary wedge pressure also correlated with the peptide levels. No correlation was observed between mean right atrial pressure and peptide concentration. These findings demonstrate that atrial natriuretic peptide release, even in the absence of cardiac failure, seems at least partly regulated by left atrial pressure. Finally, peripheral levels reflect the central concentrations of atrial natriuretic peptide.

Adult↗

Effect of therapeutic-dose irradiation on left ventricular function in conscious dogs.

Every week, 8 conscious, chronically instrumented dogs underwent left ventricular (LV) function studies before, during, and after cardiac irradiation with cobalt 60 (myocardial dose of 5,000 rads at 200 rads per day through a 5 X 5 cm port). During the weekly LV function studies, left atrial pressures were raised by rapid infusion of balanced saline solution. Heart rate, aortic pressures, left and right atrial pressures, LV pressure, left ventricular end-diastolic pressure (LVEDP), and maximum rate of rise of LV pressure were recorded. Electrocardiograms were made. Cardiac outputs were obtained by thermodilution. Stroke volume, LV stroke work, and LV minute work were calculated. LV function curves were constructed each week. All dogs lost weight and became irritable after approximately 800 rads. The electrocardiograms showed signs of myocardial injury after 1,200 rads. All variables were slightly depressed during the first 8 weeks following irradiation. At the eleventh week, both left atrial pressure and LVEDP increased significantly and LV function declined. There was also clinical evidence of LV failure at rest and after volume loading. This study documents that external cardiac irradiation, in a therapeutic dose and schedule range, causes depression of LV function. These functional changes were partially reversed when the follow-up study was continued to six months after irradiation.

Animals↗

Patent foramen ovale associated with platypnea and orthodeoxia.

A case of positionally symptomatic right-to-left shunting across a patent foramen ovale with both platypnea and orthodeoxia despite normal pulmonary arterial pressures and normal right ventricular function is documented. When the patient was in a supine position, the calculated right-to-left shunt was 12.8 percent, and when seated 25 percent. Surgical closure of the patent foramen ovale relieved the symptoms and positionally induced shunting. The prevalence and associations of permanent and intermittent patency of the foramen ovale are discussed. It is recommended that those at risk of thromboembolism be screened for patency by contrast ultrasound or color flow techniques. If present, surgical closure needs to be considered to prevent paradoximal embolism and stroke.

Aged↗

Severe coronary sinus dilatation after a modification of the classic Fontan operation: successful resolution with a revision Fontan operation.

Two patients with functional single ventricle and a left superior vena cava, which drained to the coronary sinus, are presented. Each had undergone a Fontan modification that incorporated the coronary sinus into the Fontan pathway. Each developed severe right atrial and coronary sinus dilatation. Revision of a Fontan with an intraatrial lateral tunnel and an extracardiac conduit resulted in improved functional capacity of the patients.

Adolescent↗

Echocardiographic assessment of the right ventricle in Ebstein's anomaly: relation to clinical outcome.

Two-dimensional echocardiography was performed in 16 patients with Ebstein's anomaly to assess right ventricular anatomy and function in relation to clinical features and prognosis. Measurements of right ventricular anatomy and function were established in 10 normal subjects for comparison. Ten patients were in New York Heart Association functional class I, four in class II and one each in classes III and IV. Right ventricular morphology and the three tricuspid valve leaflets were assessed from right ventricular inflow tract and apical four chamber views. The anterior tricuspid leaflet was abnormal but not displaced in all patients; the septal and posterior leaflets were displaced in 14 (88%) and 11 (69%) patients respectively. The posterior leaflet was best visualized from the right ventricular inflow tract, and in two patients this view was required for the echocardiographic diagnosis of Ebstein's anomaly, based on displacement of the septal tricuspid valve leaflet. An index of right ventricular function, the fractional area contraction, was defined as the difference between the end-diastolic and the end-systolic area, normalized to the end-diastolic area. This index was calculated for both the proximal (atrialized) right ventricle and the total right ventricle. Total right ventricular end-diastolic area and fractional area contraction exceeded 95% confidence limits when compared with values in the normal group. During a median follow-up period of 4 years three patients died. They had severe right heart morphologic or functional abnormalities; two were in functional class III or IV and one was asymptomatic. None of the survivors had severe symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Ostium secundum atrial septal defect: report of a case with atypical auscultatory findings (author's transl)].

The wide and persistent splitting of S2 is commonly considered the auscultatory hallmark of uncomplicated ostium secundum atrial septal defect (ASD). We have recently observed a 31-year-old female with a variable splitting of S2 and a 2/6 systolic murmur at the second left intercostal space, who showed incomplete right bundle branch block on the ECG and normal heart shadow with increased pulmonary vascular markings on the chest film. Cardiac catheterization revealed an isolated ostium secundum atrial septal defect with significant left-to-right shunt, normal pulmonary pressures and normal ventricular function. Subsequently the patient underwent closure with patch of the ASD without complications. We feel our case is of interest since it demonstrates that the absence of a wide and persistent splitting of S2 in adults does not exclude the diagnosis of uncomplicated "ostium secundum" ASD, when ECG and x-ray findings suggest the disease.

Adult↗

The effect of pericardial restraint, atrial pacing, and increased heart rate on left ventricular systolic and diastolic function in patients undergoing cardiac surgery.

UNLABELLED: Baseline measurements of systolic and diastolic function performed after the induction of anesthesia may be compared with subsequent measurements acquired under different physical conditions such as open pericardium and different heart rate or rhythm. We acquired data from 21 patients undergoing coronary artery surgery. Combined echocardiographic and pulmonary artery catheter measurements were performed before and after pericardial opening, atrial pacing at the native rate, and atrial pacing 30 bpm faster. Indices of systolic function included fractional area change, afterload corrected fractional area change, and myocardial performance index; diastolic function included mitral inflow and pulmonary vein Doppler profiles, color M-Mode Doppler flow propagation velocity, instantaneous end-diastolic stiffness, and isovolumetric relaxation time. Hemodynamic indices included cardiac index, mean arterial, right atrial, and pulmonary capillary wedge pressures, and systemic vascular resistance index. There were no changes in measurements after opening of the pericardium or with institution of atrial pacing. With increased heart rate, there were no changes in systolic function, but instantaneous end-diastolic stiffness increased. Propagation velocity showed a paradoxical improvement with increased heart rate opposite to other trends. Beat fusion occurs with increasing heart rate for mitral inflow Doppler. We recommend that serial measurements are performed at a similar heart rate. IMPLICATIONS: Pericardial restraint or the institution of atrial pacing do not alter left ventricular function, as assessed by pulmonary artery catheter and transesophageal echocardiography measurements. Diastolic (but not systolic) measurements showed inconsistency with increased heart rate.

Aged↗

Quantitative echocardiographic assessment of the performance of the functionally single right ventricle after the Fontan operation.

BACKGROUND: Performance of the functionally single right ventricle may deteriorate over time. Quantitative assessment of this chamber, however, is complicated by its asymmetric geometry. Automatic detection of borders, and the Doppler-derived index of myocardial performance, are echocardiographic techniques that allow for quantitative assessment regardless of ventricular shape. We sought to evaluate the mechanics of contraction and relaxation in the functionally single right ventricle using these parameters. METHODS: We evaluated systemic ventricular function in 35 asymptomatic patients with functionally single right ventricle, having a mean age of 7.8+/-3.1 years, who had undergone the Fontan procedure. We compared them with 32 age-matched normal controls using both automatic detection of borders and Doppler indexes. RESULTS: When compared with the controls, the group with a functionally single right ventricle demonstrated diminished systolic function as evidenced by a lower fractional change in area (42.7+/-10.1% vs. 54.6+/-10.5%, p = 0.001), and diminished diastolic function, as demonstrated by a greater reliance on atrial contraction to achieve ventricular filling (32.0+/-4.4% vs. 22.2+/-4.1%, p = 0.001). The mean index of myocardial performance in those with functionally single right ventricles was also greater than in controls (0.41+/-0.12 vs. 0.30+/-0.05, p = 0.001), and the indexed ejection time was shorter (0.35+/-0.05 vs. 0.39+/-0.05, p = 0.01), suggesting less efficient ventricular mechanics. CONCLUSIONS: These data demonstrate that the systolic and diastolic properties of the functionally single right ventricle differ significantly from those of the normal systemic left ventricle. Use of the echocardiographic techniques provide insight into ventricular mechanics in patients with functionally single ventricles, and may be valuable tools for serial quantitative follow-up.

Adolescent↗

The isolated human pectinate muscle: a reliable preparation of human cardiac tissue.

We have developed an anatomically and functionally intact preparation of isolated pectinate muscles from readily-available surgical specimens of human right atrial appendage. Individual pectinate muscles (2-3 per specimen) are dissected free and mounted in a tissue bath. Field stimulation is used for electrical pacing, and isometric contractions are recorded. The pectinate muscle develops a stable and large force of contraction and hence is superior to strips cut from atrial appendage specimens. Isolated pectinate muscles develop spontaneous beating or can be induced to beat spontaneously by brief periods of electrical pacing or transient exposure to epinephrine or histamine. Spontaneously-beating muscles increase their rate and force of contraction in response to drugs which have positive chronotropic and inotropic effects in the whole heart. Because the force of contraction of the pectinate muscle is a function of the rate of beating, inotropic effects of agents should also be evaluated in preparations which are electrically paced at a constant rate.

Atrial Function↗

Atrial natriuretic peptide and N-terminal atrial natriuretic peptide in plasma reflect right ventricular volumes following coronary artery surgery.

BACKGROUND: Atrial natriuretic peptide (ANP) and the more stable N-terminal fragment (N-ANP) of prohormone are peptides, released in equimolar amounts from cardiac myocytes in response to atrial stretch or ventricular overload and myocardial ischaemia. Protection of the right ventricular (RV) myocardium during ischaemia in cardiac surgery is difficult, especially in patients with severe right coronary artery (RCA) disease. This prospective study was designed to ascertain a possible relationship between changes in plasma ANP/N-ANP concentration and RV function in RCA-diseased patients. METHODS: Plasma ANP and N-ANP concentrations and RV function, measured by fast-response thermodilution, were determined serially in 15 patients with total RCA stenosis and in another 15 with no significant RCA disease (controls) before, during and after coronary surgery. RESULTS: The RV ejection fraction was lower and the RV end-systolic volume index higher in the RCA-diseased patients than in the controls (P < 0.05) on the second postoperative day, and both ANP and N-ANP were higher in the RCA patients (P < 0.05) from 6 h after cardiopulmonary bypass till the second postoperative day. At the same time the changes in N-ANP concentrations from the levels before induction of anaesthesia correlated with RV ejection fraction and RV volume indexes, but not with heart rate or parameters indirectly reflecting left-sided loading. Right atrial pressure did not differ between the groups nor did it increase significantly during the study. CONCLUSIONS: The relationships found between N-ANP and RV volume indexes and RV ejection fraction suggest ventricular expression of ANP: ANP release may be stimulated by RV distension, the more so the poorer the RV function.

Aged↗

Tricuspid annular motion.

Triscupid annular motion is related to right ventricular systolic function in the same way mitral annulus motion is related to left ventricular function. Tricuspid annular excursion reflects the longitudinal motion of the right ventricle, and the systolic descent of the anulus correlates with systolic venous inflow to the right atrium. However, it has not been shown clearly how to reproducibly quantify this motion. Therefore we describe a method to measure triscuspid annular motion using two-dimensional oriented M-mode echocardiography from the apical view. We studied a group of 10 normal subjects (mean age, 28.7 years; range, 25 to 38 years) and a group of 29 patients (mean age, 57.2 years; range, 20 to 84 years) with disease of the left side of the heart but no evidence of involvement of the right side of the heart. In each subject, tricuspid and mitral annular motion were measured respectively at their lateral, septal or medial, anterior, and posterior margin points. The total tricuspid annular motion for normal subjects was, as follows: lateral, 24.9 +/- 3.5 mm; medial, 20.1 +/- 2.5 mm; anterior, 21.6 +/- 3.8 mm; and posterior, 22.3 +/- 2.3 mm. Interobserver and intraobserver variability was low, with a coefficient of variance for the different annular points ranging from 6.19% to 11.56% between observers and from 4.10% to 7.26% within observer. We conclude that it is possible to measure tricuspid annular motion with this method in a reproducible way and to use it as a diagnostic tool in evaluating function of the right side of the heart.

Adult↗