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Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia.

BACKGROUND: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation. PATIENTS AND METHODS: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5). RESULTS: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed. CONCLUSIONS: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.

Aorta↗

Rest and exercise right and left ventricular function late after the Mustard operation: assessment by radionuclide ventriculography.

To evaluate ventricular function late after atrial repair of transposition of the great arteries (TGA), 26 asymptomatic patients had rest and exercise radionuclide ventriculography performed a mean of 9 years (range 5 to 15) after undergoing the Mustard operation. The mean resting right (systemic) ventricular (RV) ejection fraction (EF) was 0.50 +/- 0.10 (+/- 1 standard deviation); the RVEF was less than 0.45 in 8 patients. With exercise the RVEF increased in 9 patients and either failed to increase or decreased in 15 (including all 8 patients with resting values less than 0.45). The weight-adjusted work load performed was a first predictor of RV exercise response (sensitivity 87%, specificity 92%); patients whose RVEF increased did more work. The mean resting left (pulmonary) ventricular (LV) EF was 0.58 +/- 0.09; the LVEF was less than 0.50 in 3 patients. With exercise the LVEF increased in 14 patients and did not increase in 10 (including all 3 with resting values less than 0.50). The presence of complex ventricular arrhythmia documented on Holter monitoring was a first predictor of failure of the LVEF to increase with exercise (sensitivity 84%, specificity 71%). The patient's age, operative age, postoperative interval, residual arterial desaturation, preoperative large ventricular septal defect or pulmonary stenosis, postoperative pulmonary stenosis or superior vena caval obstruction, or performance of a second open-heart procedure was predictive of the rest or exercise EF of either ventricle.

Adolescent↗

The incidence of atrial arrhythmias during inferior wall myocardial infarction with and without right ventricular involvement.

The atrial arrhythmia profile during inferior wall acute myocardial infarction (AMI) has not been systematically examined with respect to right ventricular (RV) involvement. To this end, 62 consecutive patients with first inferior wall AMI and no other conditions known to increase susceptibility for rhythm disturbances were studied by 24-hour Holter monitoring during the first and tenth day of infarction. Based on radionuclear ventriculography performed on day 2 of infarction, patients were allocated to two groups: group A--36 patients (58%) with right ventricular ejection fraction (RVEF) less than 40% (mean +/- SD, 31 +/- 6%) and group B--26 patients (42%) with normal (greater than 40%) RVEF (mean +/- SD, 47 +/- 5%). There were no significant differences between the two groups with respect to age, sex, or left ventricular (LV) function. In the group as a whole, ectopic activity in the different categories of atrial arrhythmias was significantly higher during the first day than on the tenth day of infarction. Comparing the two groups, 33 patients (92%) in group A had a mean hourly frequency of one or more atrial premature contractions (APCs) during the first day of infarction compared with 18 patients (69%) in group B (p less than 0.001). Atrial and supraventricular tachycardia were recorded more frequently in group A patients (16 of 36 [44%] versus 8 of 26 [31%]) as well as atrial fibrillation (AF) (7 of 36 [19%] versus 1 of 26 [4%]). Quantitative analysis showed a similar trend for a higher rate of ectopic events in group A patients. Ectopic activity was neither influenced by LVEF nor by age or sex.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrial Fibrillation↗

Sinus arrest in diaphragmatic myocardial infarction: treatment of power failure with atrial pacing.

Sinus arrest occurred in a patient with acute diaphragmatic myocardial infarction associated with right ventricular infarction. Cardiac output fell dramatically despite maintenance of a junctional rate of 72. Ventricular pacing at rate 82 and dopamine administration resulted in only slight hemodynamic improvement. Atrial pacing at rate 84 restored normal cardiac output until resumption of sinus node activity. These results suggest that atrial contraction is important for the maintenance of ventricular function in some patients with acute infarction, and may be of particular importance in the presence of right ventricular infarction. Temporary atrial or atrioventricular sequential pacing may be of great hemodynamic benefit in selected patients with conduction defects complicating myocardial infarction.

Cardiac Pacing, Artificial↗

Prucalopride is a partial agonist through human and porcine atrial 5-HT4 receptors: comparison with recombinant human 5-HT4 splice variants.

Prucalopride is a gastrointestinal prokinetic drug that acts through 5-HT4 receptors, but its potential effects on cardiac atrial function are unknown. We investigated the effects of prucalopride on human right atrium, piglet left atrium, and piglet sinoatrial node. The effects of prucalopride on 5-HT4 receptor splice variants a, b, g and i, known to be expressed in human atrium, were studied for comparison. Prucalopride was an inotropic partial agonist, compared with 5-HT, on paced human atrial trabeculae (-logEC50M=7.4) and porcine left atria (-logEC50M=7.2), with intrinsic activity of 0.77 and 0.63 respectively. Prucalopride (1 microM) surmountably antagonized the positive inotropic effects of 5-HT on human (pK(P)=7.2) and porcine (pK(P)=7.1) atrium. Prucalopride was also a chronotropic partial agonist (-logEC50M=7.4, intrinsic activity=0.72 with respect to 5-HT) on spontaneously beating piglet atria. The cardiostimulant effects of prucalopride were prevented by GR113808 (1 microM), consistent with mediation through 5-HT4 receptors. Prucalopride bound to recombinant 5-HT4(a), 5-HT4(b), 5-HT4(g), and 5-HT4(i) receptors, labeled by [3H]GR113808, with pKi values of 7.6, 7.5, 7.4, and 7.8 respectively. Prucalopride stimulated adenylyl cyclase as a partial agonist on 5-HT4(a), 5-HT4(b), and 5-HT4(i) receptors with intrinsic activities of 0.82, 0.86, and 0.78 and -logEC50 values of 7.2, 7.3, and 7.2 respectively. At the 5-HT4(g) receptor prucalopride acted as a full agonist (-logEC50M=8.0) compared with 5-HT in the cell line tested, which was probably due to high receptor expression levels. We conclude that prucalopride is a cardiostimulatory partial agonist through human and porcine 5-HT4 receptors. Since prucalopride acts similarly through 5-HT4(a), 5-HT4(b), 5-HT4(g), and 5-HT4(i) receptors, any of these variants could be involved in the mediation of cardiostimulation.

Adenylyl Cyclases↗

The effect of renal transplantation on plasma atrial natriuretic peptide.

The changes in plasma atrial natriuretic peptide (ANP) were studied in four adult patients after cadaveric renal transplantation. In three patients who achieved good renal function, the correction of volume overload, as reflected by reduction in weight and right atrial pressure, was associated with a steady fall in plasma ANP and a parallel decrease in both fractional excretion of sodium and plasma cyclic guanosine monophosphate. The fourth patient, with severe acute rejection, developed severe peripheral oedema, and fractional sodium excretion remained low despite high values of ANP.

Adult↗

Radionuclide analysis of right and left ventricular response to exercise in patients with atrial and ventricular septal defects.

In patients with ventricular or atrial septal defect, the ventricle which is chronically volume overloaded might not appropriately respond to increased demand for an augmentation in output and thereby might limit total cardiac function. In this study we simultaneously measured right and left ventricular response to exercise in 10 normal individuals, 10 patients with ventricular septal defect (VSD), and 10 patients with atrial septal defect (ASD). The normal subjects increased both right and left ventricular ejection fraction, end-diastolic volume, and stroke volume to achieve a higher cardiac output during exercise. Patients with VSD failed to increase right ventricular ejection fraction, but increased right ventricular end-diastolic volume and stroke volume. Left ventricular end-diastolic volume did not increase in these patients but ejection fraction, stroke volume, and forward left ventricular output achieved during exercise were comparable to the response observed in healthy subjects. In the patients with ASD, no rest-to-exercise change occurred in either right ventricular ejection fraction, end-diastolic volume, or stroke volume. In addition, left ventricular end-diastolic volume failed to increase, and despite an increase in ejection fraction, left ventricular stroke volume remained unchanged from rest to exercise. Therefore, cardiac output was augmented only by the heart rate increase in these patients. Right ventricular function appeared to be the major determinant of total cardiac output during exercise in patients with cardiac septal defects and left-to-right shunt.

Adolescent↗

Right atrial thrombus in an asymptomatic hemodialysis patient with malfunctioning catheter and patent foramen ovale.

The creation of an accurate functioning arteriovenous fistula has been a long-lasting problem in the hemodialysis setting. In spite of recent guidelines and largely because of the old age of the current dialysis population and a high incidence of diabetes mellitus, atherosclerosis, and related vascular problems, it is not always possible to create an adequate fistula. In that case, long-term tunneled indwelling central vein catheters are a frequently used alternative. Of the many possible complications related to venous access in hemodialysis patients, catheter dysfunction is the most prevalent. We report a 23-year-old female hemodialysis patient in whom such malfunctioning was followed by echocardiography that revealed a large right atrial thrombus (RAT) in close contact to the tip of a long-term indwelling catheter in the presence of a patent foramen ovale. Although RAT is a rare complication in hemodialysis patients, it has very specific therapeutic implications. The present patient underwent a successful surgical atrial thrombectomy. Our experience underscores that in cases of malfunctioning catheter, echocardiographic screening is mandatory.

Adult↗

Atrial and ventricular function after cardioversion of atrial fibrillation.

OBJECTIVE: Previous studies on atrial recovery after cardioversion of atrial fibrillation have not taken into account new knowledge about the pathophysiology of transmitral and transtricuspid flow velocity patterns. It is possible to shed further light on this problem if atrioventricular inflow velocity, venous filling pattern, and atrioventricular annulus motion are recorded and interpreted together. DESIGN: Prospective examinations of mitral and tricuspid transvalvar flow velocities, superior caval and pulmonary venous filling, and mitral and tricuspid annulus motion were recorded using Doppler echocardiography. Examinations were performed before and 24 hours, 1 month, and 20 months after cardioversion. SETTING: Tertiary referral centre for cardiac disease with facilities for invasive and non-invasive investigation. PATIENTS: 16 patients undergoing cardioversion of atrial fibrillation in whom sinus rhythm had persisted for 24 hours or more. RESULTS: Before conversion there was no identifiable A wave in transvalvar flow recordings. The total motion of the tricuspid and mitral annulus was subnormal and there was no identifiable atrial component. Venous flow patterns in general showed a low systolic velocity. After conversion, A waves and atrial components were seen in all patients and increased significantly (P < 0.01) with time. There was a similar time course for the amplitude of annulus atrial components, an increased systolic component of venous inflow, an increased A wave velocity, and a decreased E/A ratio of the transvalvar velocity curves. The ventricular component of annulus motion was unchanged. Changes in general occurred earlier on the right side than the left. CONCLUSIONS: This study indicates that, in addition to the previously known electromechanical dissociation of atrial recovery that exists after cardioversion of atrial fibrillation, there may also be a transient deterioration of ventricular function modulating the transvalvar inflow velocity recordings. Function on the right side generally becomes normal earlier than on the left. Integration of information from transvalvar inflow curves, annulus motion, and venous filling patterns gives additional insight into cardiac function.

Aged↗

Impact of atrial fibrillation on clinical status, atrial size and hemodynamics in patients after mitral valve replacement.

BACKGROUND AND AIM OF THE STUDY: The association between mitral valve disease and atrial fibrillation (AF) is well known, but few data exist regarding the impact of AF after mitral valve replacement (MVR) on NYHA functional class, atrial size and hemodynamic parameters. The present study was conducted to evaluate these issues. METHODS: Eighty-six patients (26 men, 60 women) who underwent MVR were evaluated by transthoracic echocardiography. Fifty-nine patients had chronic AF (AF group), and 27 were in sinus rhythm (sinus group). Variables analyzed included end-systolic left atrial and right atrial areas, tricuspid regurgitation, and presence and duration of AF. Peak and mean transprosthetic mitral valve gradients and pulmonary pressure were estimated by Doppler echocardiography. RESULTS: Groups were matched for age, sex and time from MVR (mean 6.6 years). Sixty-four patients (77%) had rheumatic heart disease, 18 (21%) had mitral valve disease, and two (2%) had mitral valve prolapse. Mean duration of AF was 11+/-12 years (range: 8-50 years). Preoperatively, AF patients had a worse NYHA class than sinus patients (2.8+/-0.8 versus 1.1+/-0.7, p = 0.001), but both had similar fractional shortening of the left ventricle and preserved prosthetic mitral valve function. Multivariate analysis identified AF as a single predictor of NYHA class after MVR. Although left and right atrial areas were larger in AF patients (47+/-25 versus 27+/-7 cm2, p = 0.0001 and 30+/-12 versus 17+/-5 cm2, p = 0.0001, respectively), the left:right atrial size ratio was not significantly different between groups. Multivariate analysis identified mean transmitral gradient and duration of AF as independent predictors of left atrial size after MVR (p = 0.01 and p = 0.0001, respectively). Tricuspid regurgitation and duration of AF were independent predictors of right atrial size (p = 0.003 and p = 0.0001, respectively). CONCLUSION: The presence of AF after MVR is associated with a worse NYHA functional class, increased transmitral gradients, and larger areas of both atria, when compared with sinus rhythm. Hence, a special effort should be made to correct arrhythmia during surgery, and in case of paroxysmal arrhythmia, earlier surgery should be considered before the condition becomes chronic.

Aged↗

In vitro comparison of central venous catheters for aspiration of venous air embolism: effect of catheter type, catheter tip position, and cardiac inclination.

STUDY OBJECTIVE: To test the relative efficiency of balloon-tipped and plain catheters for aspiration of venous air embolism. DESIGN: The following four single-lumen central venous catheters were studied in a silastic model of the right atrium, tricuspid valve, and vena cavae: (1) the 16-gauge single-orifice catheter; (2) the 14-gauge Bunegin-Albin multiorifice catheter; (3) the 7-Fr pulmonary angiography catheter with balloon distal to orifices; (4) the 7-Fr pulmonary angiography catheter with balloon proximal to orifices. A 10% glycerol-water solution was circulated at 3.7 to 4.0 L/min. Catheter tips were positioned at 1 cm increments from -3 to +3 cm around the superior vena cava-right atrial junction with cardiac inclinations of 65 degrees and 80 degrees. Air (10 ml) was infused over 30 seconds; aspiration from the test catheter began 5 seconds later at 40 ml/min for 75 seconds. The balloon catheters were evaluated with the balloons inflated and deflated. SETTING: Experimental laboratory of a university-affiliated hospital. MEASUREMENTS AND MAIN RESULTS: Amounts of air aspirated were compared by analysis of variance and Tukey's multiple comparison, p less than 0.05, for all combinations. The 16-gauge single-orifice catheter tested best at a 65 degrees cardiac inclination with 86% of the venous air embolism recovered, while the 14-gauge Bunegin-Albin multiorifice catheter tested best at an 80 degrees cardiac inclination with 62% of the venous air embolism recovered. Both catheters functioned most efficiently at or above the superior vena cava-right atrial junction. CONCLUSIONS: This study demonstrated that efficacy of air recovery depends on catheter type, catheter tip position, and cardiac inclination. No benefit was derived from positioning the catheter tip inside the atrium or from using balloon-tipped catheters.

Catheterization, Central Venous↗

[Problems in patients with use of a ventricular assist device].

The purpose of the present study was to review the results obtained in patients with a ventricular assist devices (VAD) in our hospital, and to discuss various problems concerning a VAD use, such as indications, right ventricular failure, and evaluation of cardiac function. Fourteen VADs were applied to 11 patients for left ventricular assist, including two for right ventricular assist and for one as biventricular assist with a VAD in the left and a biopump in the right. The clinical diagnoses of the patients were as follows: 10 ischemic heart disease, two valvular disease, one acute aortic dissection, and one corrected transposition of the great arteries. VADs were indicated in 11 patients because of difficulty in weaning from cardiopulmonary bypass (CPB), and in three patients because of cardiogenic shock after discontinuing CPB. Among the 14 patients, 11 had an effective VAD, six were successfully weaned from a VAD, and two survived. The VAD was ineffective due to uncontrollable bleeding and improper indications for the device, as in applying a one-sided heart assist when a biventricular assist was necessary. In spite of an effective VAD, five patients could not be weaned from VAD because of brain damage, sepsis, and hypoxia. After removing a VAD, four patients died; one due to mediastinitis, two due to respiratory failure, and one due to low output syndrome. All the four patients had renal failure followed by multi-organ failure finally, because of prolonged CPB time. The CPB time was shorter among the long survivors than in others. Cardiac function during assist and the weaning probability from a VAD were evaluated not only by the so-called on-off test, but also by transesophageal Doppler echocardiography. Ventricular wall motion and pulmonary venous flow pattern were analyzed by transesophageal Doppler echocardiography. The pattern of monophasic forward flow in the pulmonary vein was associated with reduced wall motion during deteriorated cardiac function, while the flow pattern became biphasic as cardiac function recovered. From these results, we concluded as follows: 1. Early decisions as to whether VAD is indicated are important. 2. A right VAD should be considered in cases with biventricular failure, during left ventricular assist, if right atrial pressures elevated more than 18 mmHg constantly. 3. The evaluation of cardiac function by transesophageal Doppler echocardiography is useful for making decisions as to wean patients from a VAD.

Adolescent↗

Filling and arterial pressures as determinants of RV stroke volume in the sheep fetus.

Right ventricular (RV) function was investigated in nine fetal lambs (125-130 days gestation) that were instrumented with pulmonary artery electromagnetic flow sensors and vascular catheters. Control arterial CO2 and O2 tension, pH, and hematocrit values were 46.1 +/- 1.6 (SD) Torr, 20.6 +/- 1.8 Torr, 7.39 +/- 0.02, and 31 +/- 5.3%, respectively. Control values for right ventricular output (247 +/- 75 ml X min-1 X kg-1), stroke volume (SV, 1.5 +/- 0.4 ml X kg-1), right atrial pressure (3.7 +/- 1.2 mmHg), heart rate (166 +/- 18 beats X min-1), and arterial pressure (AP, 43 +/- 4 mmHg) were unchanged by administration of atropine and propranolol. Withdrawal and infusion of fetal blood with or without concomitant infusion of nitroprusside or phenylephrine produced RV function curves at low, normal, and high arterial pressures. All function curves had a steep ascending limb and a plateau. The breakpoint joining the limbs of the control curve was right atrial pressure 3.4 +/- 1.2 mmHg and SV 1.5 +/- 0.4 ml X kg-1. Increased AP shifted the breakpoint downward. Linear regression of SV on AP from 15 to 95 mmHg at right atrial pressure greater than breakpoint was SV = -0.016 ml X kg-1 mmHg-1 X AP + 2.25 ml X kg-1.

Animals↗

Effects of long-term right ventricular apical pacing on left ventricular perfusion, innervation, function and histology.

OBJECTIVES: The purpose of this study was to better understand the effects of long-term right ventricular pacing on left ventricular perfusion, innervation, function and histology. BACKGROUND: Long-term right ventricular apical pacing is associated with increased congestive heart failure and mortality compared with atrial pacing. The exact mechanism for these changes is unknown. In this study, left ventricular perfusion, sympathetic innervation, function and histologic appearance after long-term pacing were studied in dogs in an attempt to see whether basic changes might be present that might ultimately be associated with the adverse clinical outcome. METHODS: A total of 24 dogs were studied. Sixteen underwent radiofrequency ablation of the atrioventricular (AV) junction to produce complete AV block. Seven of these underwent long-term pacing from the right ventricular apex (ventricular paced group), and nine had atrial and right ventricular apical pacing with AV synchrony (dual-chamber paced group). A control group of eight dogs had sham ablations with normal AV conduction. These dogs had atrial pacing only. Regional perfusion and sympathetic innervation were studied in all dogs by imaging with thallium-201 and [I123]metaiodobenzylguanidine, respectively. The degree of innervation was also determined by assay of tissue norepinephrine levels. Left ventricular function was assessed by radionuclide ventriculography. Cardiac histology was studied with both light and electron microscopy. RESULTS: Mismatching of perfusion and innervation in the ventricular paced group was noted, with perfusion abnormalities of both the septum and free wall. Regional [I123]metaiodobenzylguanidine distribution was homogeneous. Tissue norepinephrine levels were elevated in both the ventricular and dual-chamber paced groups compared with the control group. No light or electron microscopic findings were noted in any groups. In the dual-chamber paced group, diastolic dysfunction was noted, with normal systolic function. CONCLUSIONS: Ventricular pacing resulted in regional changes in tissue perfusion and heterogeneity between perfusion and sympathetic innervation. Both ventricular and dual-chamber pacing were associated with an increase in tissue catecholamine activity. The abnormal activation of the ventricles via right ventricular apical pacing may result in multiple abnormalities of cardiac function, which may ultimately affect clinical outcome.

3-Iodobenzylguanidine↗

Right ventricular size and function as assessed by echocardiography and angiography in patients with different volume load.

A comparative study of right ventricular (RV) function, assessed by echocardiography and angiography, undertaken in 20 patients, 10 of whom had atrial septal defects (ASDs) and 10 had various other heart diseases. All of the measured echocardiographic variables of RV size, apart from RV length, were larger in the patients with ASD. When assessed by multiple regression analysis, the RV M-mode dimension was an independent variable of RV angiographic end-diastolic volume (EDV) in patients without RV volume load (R = 0.92, R2 = 0.85, p < 0.001). In the patients with ASD, echocardiographic RV end-diastolic area was an independent variable of angiographic RVEDV (R = 0.75, R2 = 0.55, p < 0.05), whereas M-mode dimension had a weaker correlation (r = 0.29). The agreement between RV ejection fraction (RVEF) obtained by echocardiography and angiography was moderate in both patient groups. However, fractional area change and fractional length change could not estimate RVEF better. Thus care should be taken to use single measurements and derivatives as the only parameters of RV size and function.

Adolescent↗