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Selective right ventricular angiography in apparently idiopathic ventricular fibrillation.

The definition of underlying heart disease in apparently idiopathic ventricular fibrillation seems to be important in regard to prognosis and choice of therapy. From October 1989, until August 1993, cardiac arrest due to the documented ventricular fibrillation occurred in eight consecutive patients with normal results on clinical examination, normal echocardiography, and normal or apparently nonspecific electrocardiogram (ECG) findings. Complete invasive investigations, including selective right ventricular angiography, were done; regional hypokinesia and segmental bulging of the right ventricle were found in seven patients (88%). Arrhythmogenic right ventricular dysplasia was suspected in these patients, although endomyocardial biopsy was not performed. After the finding of localized right precordial QRS prolongation of more than 110 ms in November 1993 in five patients, a retrospective, a more precise approach to QRS duration in standard ECG supported this diagnosis. Selective right ventricular angiography is of great help in identifying underlying heart disease in patients with apparently idiopathic ventricular fibrillation, and confirms ECG findings.

Adolescent↗

Anatomic correction of atrioventricular discordance.

Between June 1989 and September 1991, 11 patients underwent anatomic correction of atrioventricular discordance. Their ages at operation ranged from 1 to 11 years (mean 6.7 years) and their weights ranged from 7.1 to 31.8 kg (mean 19.1 kg). Atrial situs was solitus in nine and inversus in two patients. Ventriculoarterial connection was discordant in five and was double-outlet right ventricle in six patients. Associated congenital heart defects were seen in all patients, including 10 with ventricular septal defect, eight with atrial septal defect, nine with pulmonary stenosis or pulmonary atresia, seven with tricuspid regurgitation, and four with mitral regurgitation. Five patients had prior Blalock-Taussig shunts. One patient with an intact ventricular septum had repeated pulmonary banding. Anatomic correction consisted of the Senning and Rastelli procedures in three, the Mustard and Rastelli procedures in five, the Senning and arterial switch operations in two, and the Mustard and arterial switch operations in one patient. In addition, mitral valvuloplasty or valvular annuloplasty was performed in three patients. We did not encounter kinking or torsion of the translocated coronary arteries in our three patients with the arterial switch operation. There was one surgical death. The other patients pursued satisfactory postoperative courses (mean follow-up period of 12.6 months). We recommend that anatomic correction for atrioventricular discordance should be indicated, especially in patients with any sign of systemic right ventricular dysfunction.

Cardiac Surgical Procedures↗

[ECG-gated myocardial SPECT with 99mTc-MIBI in patients with right ventricular infarction].

Although 99mTc-pyrophosphate (PYP) myocardial scintigraphy has so far been widely used for the diagnosis of right ventricular infarction, PYP accumulation disappears within one week or so. To evaluate the myocardial condition of the right ventricle alternatively, myocardial SPECT with 99mTc-MIBI was performed in 16 patients with acute inferior left ventricular infarction, and ECG-gated myocardial SPECT data acquisition was accompanied in 14 of 16 patients. Right ventricular perfusion defect was observed in 4 of 16 patients (RVI (+) group), and the remains were negative (RVI (-) group). Then, right ventricular count increase rate (RV %WT) of MIBI from end-diastole to end-systole was calculated using an automated method which was developed for quantification of wall thickening in our laboratory. The RV %WT was conceived to be an objective index representing right ventricular contractility. RVI (+) group (n = 3) as compared with RVI (-) group (n = 11) had significant lower RV %WT (26.7 +/- 3.2 vs. 49.6 +/- 14.2; p < 0.01). In conclusion, ECG-gated myocardial SPECT with MIBI was considered to be useful for assessment of myocardial perfusion and contractility of right ventricle.

Aged↗

Transcatheter reconstruction of the right heart.

We report the interventional procedures performed on a 12-year-old child with obstruction of the right ventricular outflow tract, pulmonary valvar insufficiency, pulmonary arterial stenosis, and an atrial septal defect. A staged repair of all anomalies was performed successfully using transcatheter techniques.

Cardiac Catheterization↗

Levels of brain natriuretic peptide in children with right ventricular overload due to congenital cardiac disease.

OBJECTIVE: To evaluate the role of the concentration of brain natriuretic peptide in the plasma, and its correlation with haemodynamic right ventricular parameters, in children with overload of the right ventricle due to congenital cardiac disease. METHODS: We studied 31 children, with a mean age of 4.8 years, with volume or pressure overload of the right ventricle caused by congenital cardiac disease. Of the patients, 19 had undergone surgical biventricular correction of tetralogy of Fallot, 11 with pulmonary stenosis and 8 with pulmonary atresia, and 12 patients were studied prior to operations, 7 with atrial septal defects and 5 with anomalous pulmonary venous connections. We measured brain natriuretic peptide using Triage(R), from Biosite, United States of America. We determined end-diastolic pressures of the right ventricle, and the peak ratio of right to left ventricular pressures, by cardiac catheterization and correlated them with concentrations of brain natriuretic peptide in the plasma. RESULTS: The mean concentrations of brain natriuretic peptide were 87.7, with a range from 5 to 316, picograms per millilitre. Mean end-diastolic pressure in the right ventricle was 5.6, with a range from 2 to 10, millimetres of mercury, and the mean ratio of right to left ventricular pressure was 0.56, with a range from 0.24 to 1.03. There was a positive correlation between the concentrations of brain natriuretic peptide and the ratio of right to left ventricular pressure (r equal to 0.7844, p less than 0.0001) in all patients. These positive correlations remained when the children with tetralogy of Fallot, and those with atrial septal defects or anomalous pulmonary venous connection, were analysed as separate groups. We also found a weak correlation was shown between end-diastolic right ventricular pressure and concentrations of brain natriuretic peptide in the plasma (r equal to 0.5947, p equal to 0.0004). CONCLUSION: There is a significant correlation between right ventricular haemodynamic parameters and concentrations of brain natriuretic peptide in the plasma of children with right ventricular overload due to different types of congenital cardiac disease. The monitoring of brain natriuretic peptide may provide a non-invasive and safe quantitative follow up of the right ventricular pressure and volume overload in these patients.

Adolescent↗

[An accurate formula for assessing the volume of the right ventricular outflow tract].

Practical application of formulae for separate assessment of the volumes of inlet and outlet sections of the right ventricle derived previously by the authors has shown that the formula for right ventricular outlet section determining it as a curved truncated cone with nonparallel bases does not take into account a narrowing in the middle third of the outlet portion during the right ventricular systole and/or supraventricular crest hypertrophy. In the specified formula right ventricular outlet portion volume is determined as a sum of the volumes of two truncated cones with a borderline in the middle of the outlet portion long axis. The most reliable difference from the main formula was obtained in the control group where the borderline of outlet portion division coincided with the area of the maximal stenosis of outlet portion stenosis in the systole. In other groups (patients with some congenital heart diseases) the maximal stenosis zone is located lower. Hence, the specified formula helps reduce the overvaluing of the right ventricular outlet portion volume; to make the estimation more accurate, the borderline between the cones should be carried out at the site of this portion maximal stenosis.

Cardiac Volume↗

Elevated right ventricular pressures are not a contraindication to liver transplantation in Alagille syndrome.

BACKGROUND: Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome. METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive. All patients had pulmonary artery stenosis. In 10 patients considered to have elevated RV pressure on ECG and/or Doppler-echocardiography, a cardiac catheterization was performed before liver transplantation. Mean RV systolic pressure was 55 mmHg (median 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic pressure ratio 0.53 (median 0.53, range 0.29-0.78) with a ratio above 0.5 in 6 patients (median 0.66, range 0.5-0.8). All patients underwent successful liver transplantation. Five patients died 1 to 9 months after transplantation from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in one and 0.37 in the second. In the three others, echocardiography before transplantation estimated RV pressures below 0.5 systemic pressures. At follow-up (median 6 years, range 1.5-15 years), liver tests were normal in all, none complained of pruritus and body weight was normalized in 70%. None of the patients presented cardiac symptoms, arrhythmias, or worsening of their cardiac status. CONCLUSIONS: Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.

Adolescent↗

N-terminal pro-brain natriuretic peptide in patients with acute pulmonary embolism.

Plasma brain natriuretic peptide (BNP), released from myocytes of ventricles upon stretch, has been reported to differentiate pulmonary from cardiac dyspnoea. Limited data have shown elevated plasma BNP levels in acute pulmonary embolism (APE), frequently accompanied by dyspnoea and right ventricular (RV) dysfunction. The aim of this study was to assess plasma N-terminal proBNP (NT-proBNP) in APE, and to establish whether it reflects the severity of RV overload and if it can be used to predict adverse clinical outcome. On admission, NT-proBNP and echocardiography for RV overload were performed in 79 APE patients (29 males), aged 63 +/- 16 yrs. Plasma NT-proBNP was elevated in 66 patients (83.5%) and was higher in patients with (median 4,650 pg x mL(-1) (range 61-60,958)) than without RV strain (363 pg x mL(-1) (16-16,329)). RV-to-left ventricular ratio and inferior vena cava dimension correlated with NT-proBNP. All 15 in-hospital deaths and 24 serious adverse events occurred in the group with elevated NT-proBNP, while all 13 (16.5%) patients with normal values had an uncomplicated clinical course. Plasma NT-proBNP predicted in-hospital mortality. Plasma N-terminal pro-brain natriuretic peptide is elevated in the majority of cases of pulmonary embolism resulting in right ventricular overload. Plasma levels reflect the degree of right ventricular overload and may help to predict short-term outcome. Acute pulmonary embolism should be considered in the differential diagnosis of patients with dyspnoea and abnormal levels of brain natriuretic peptide.

Acute Disease↗

Cardiac dysfunction in beta-thalassemic children.

The biventricular systolic function was evaluated by M mode and Doppler echocardiography in 16 patients with homozygous beta-thalassemia and beta-thalassemia Hb E disease, aged 5-14 yrs (9.9 +/- 2.4 yr). The left ventricular end-diastolic dimension and left ventricular mass were increased in 88 per cent of the patients. Left ventricular fractional shortening and cardiac index were normal in all but one patient. Fifteen patients had evidence of pulmonary artery hypertension manifested by abnormality in the ratio of right ventricular acceleration time to ejection time (AT/ET). There was good correlation between platelets count and AT/ET (r = -0.70, P = 0.002). Thrombocytosis was noted in 4 patients. Our findings indicated that in beta-thalassemic children, right ventricular dysfunction was detected earlier than left ventricular dysfunction and platelets may play a role in the pathogenesis of pulmonary artery hypertension.

Adolescent↗

Ventricular interdependence: significant left ventricular contributions to right ventricular systolic function.

This article reviews diastolic and systolic ventricular interaction, and clinical pathophysiological conditions involving ventricular interaction. Diastolic ventricular interdependence is present on a moment-to-moment, beat-to-beat basis, and the interactions are large enough to be of physiological and pathophysiological importance. Although always present, ventricular interdependence is most apparent with sudden postural and respiratory changes in ventricular volume. Left ventricular function significantly affects right ventricular systolic function. Experimental studies have shown that about 20% to 40% of the right ventricular systolic pressure and volume outflow result from left ventricular contraction. This dependency of the right ventricle on the left ventricle helps to explain the right ventricular response to volume overload, pressure overload, and myocardial ischemia. The septum and its position are not the sole mechanism for ventricular interdependence. Ventricular interdependence causes overall ventricular deformation, and is probably best explained by the balance of forces at the interventricular sulcus, the material properties, and cardiac dimensions.

Animals↗

Tetralogy of Fallot in adults--107 cases.

Clinical and haemodynamic profile of 107 adult patients above the age of 15 years with TOF was analysed. Cardiac catherization and selective cine-angiography were performed in all cases. Infundibular pulmonary stenosis, mal-alignment type of ventricular septal defect, mitral-aortic fibrous continuity and equal systolic pressures in both the ventricles and aorta were considered mandatory for the diagnosis of Tetralogy of Fallot. Aortic regurgitation was seen in 26 cases (24%), tricuspid regurgitation in 22 cases (21%), absent pulmonary valve in 3 cases (3%), branch pulmonary artery stenosis in 9 case (8.4%), major aortopulmonary collaterals in 15 cases (14%), right atrial pressure was more than 10 mmHg in 10 cases (11%) and right ventricular end diastolic pressure more than 9 mmHg in 73 cases (68%). The left ventricular end diastolic pressure was above 13 mmHg in 58 cases (54%).

Adolescent↗

Lack of effect of synthetic pericardial substitute on right ventricular function after coronary artery bypass surgery. An echocardiographic and magnetic resonance imaging study.

Abnormal right heart function after cardiac surgery is a well-known finding. Inadequate preservation during the operation and restricted cardiac motion due to pericardial adhesions have been proposed as underlying mechanisms. This study focuses on the impact of a pericardial substitute implantation on right ventricular function, using echocardiography and magnetic resonance imaging. A test group of six patients (mean age 54 years) was examined before surgery, and 4-15 days and 5-9 months after coronary artery bypass surgery, where the pericardium was closed with a biodegradable pericardial patch. A group of 11 patients (mean age 63 years) in whom the pericardium was left open served as controls. Tricuspid annulus motion was markedly decreased, abnormal septal motion was present and decreased systolic to diastolic ratio in the vena cava superior flow was present in all patients in both groups one week after surgery. At the late follow-up, all patients still had decreased tricuspid annulus motion, while 17% of the patients in the test group and 22% of the patients in the control group (ns) demonstrated normal septal motion. We conclude that closing the pericardium with a biodegradable patch does not affect the postoperative changes in right heart function normally seen after open-heart surgery.

Aged↗

The remarkable right ventricle of patients with Eisenmenger syndrome.

Patients with Eisenmenger syndrome do much better than patients with all other etiologies of severe pulmonary hypertension. The primary reason, and a major focus of this review, is the right ventricle, a remarkable chamber with preserved systolic function despite a lifetime of systemic level pulmonary hypertension.

Adaptation, Physiological↗

Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow obstruction.

Sinus of Valsalva aneurysms rarely present until rupture occurs. We describe the case of a patient who presented with acute right heart failure and upon investigation was found to have an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction; there was an associated subaortic ventricular septal defect. To the best of our knowledge, only 1 other case with these features has been reported in the medical literature. The diagnosis was made by transthoracic echocardiography and cardiac catheterization. Through an aortic and right ventricular approach, we successfully excised the aneurysmal right coronary sinus, closed the ventricular septal defect, and replaced the aortic valve.

Adult↗

[Changes in the ECG in chronic heart failure and after transplantation].

UNLABELLED: ECG examination belongs to basic procedures in the care of patients with heart failure. The Euroheart Survey study followed the data and therapy of hospitalized patients with chronic heart failure (CHF) with participation of 116 hospitals from 25 European countries and surveyed documentation of 45,993 patients dismissed from internal wards. A new atrial fibrillation or supraventricular tachycardia affected 25.3% of patients, chronic fibrillation was encountered in 23%. Bradyarrhythmia occurred in 10.8% and pacemaker was applied in 8.5% of patients. Ventricular arrhythmias were present in 8.4%, implantable cardiovertor-defibrillator in 1.5%. Syncope was reported in 15% and the arrhythmic death was described in 1.83%. The patients with heart failure suffer from a high incidence of ventricular arrhythmias. About 40-50% of death events in patients with CHF are estimated to be associated with a sudden death or arrhythmia. Large clinical studies investigate the occurrence of and influence upon the sudden death as indices of therapy. The disorders of rhythm as well defects of ventricular conduction may be the risk factors of survival in patients with CHF. The Italian registry of heart failure includes examinations of 5 517 patients. A complete blockade of the left bundle branch block (LBBB) was present in 25.2% patients, whereas a complete blockade of the right bundle branch block (RBBB) occurred in 6.1%. The patients differed in the CHF cause: the group with LBBB suffered more frequently from dilatative cardiomyopathy, whereas IHD more present more frequently in patients without the blockade. The groups also differed in the severity of the disease. The patients with LBBB had a higher prevalence of heart weakness NYHA III and IV (32.8% and 26.4%, respectively, P < 0.001), lower systolic blood pressure, more frequently third heart sound (34.2% versus 22.2%, P < 0.001), cardiomegaly classified as a cardiothoracic index higher that 0.55 (63.2% versus 55%, P < 0.04). In the course of one-year observation 11.9% of patients died, death being evaluated as sudden in 46%. The one-year mortality in patients those with LBBB was 16.1% in contrast to 10.5% in those who had not suffered from the blockade. A sudden death affected 5.5% of patients in the whole cohort, the frequency being again in LBBB (7.3% vs. 4.9%). The risk further increased with a simultaneous atrial fibrillation. After heart transplantation, ECG is also of importance for revealing an acute rejection. In the bilateral operation technique a direct alteration of the sinus node could be the cause of dysfunction. Various bradyarrhythmias developed in later periods of time. A modification of the operation technique has been used in the last 10 years. The application of bicaval method eliminated brachycardiac complications after heart transplantation. A cardiostimulator proved to be necessary in 5 to 15% of patients when biatrial technique was used, whereas it was virtually not required with the bicaval technique. The blockade the left bundle branch block is an unfavorable prognostic factor in the same way as in CHF, whereas of the right bundle branch block is critical in the heart transplantation. It is supposed to be caused by surgical or thermal damage or it is also associated with different degree of the right ventricular dysfunction and a higher number of rejections. The blockade is also associated with worse one-year survival (74% vs. 92%, P < 0.03). CONCLUSION: ECG retains its position even in modern times not only in the diagnosis of conditions responsible for heart failure, in the diagnosis of disorder of rhythm and conduction, but it is also of prognostic value. It also retains its position in the same areas as in heart failure, i.e. in the diagnosis and prognosis in heart transplantation.

Aged↗

[Prevention of negative hemodynamic effects of protamine sulfate by simultaneous infusion of microdoses of adrenaline].

Two groups of coronary patients subjected to revascularization of the myocardium were examined in order to detect the negative hemodynamic effects of protamine sulfate (PS) and the possibility of their correction by simultaneous infusion of adrenaline microdoses. In group 1 (27 pts.) heparin was neutralized by infusion of PS alone (6 mg/kg) and in group 2 (27 pts.) by simultaneous infusion of PS and adrenaline (15 ng/kg/min). The functions of the right and left ventricles were evaluated by catheterization of the pulmonary artery by a Swan-Ganz catheter and transesophageal Doppler echocardiography. These methods provided volume and velocity characteristics of the right and left compartments of the heart. PS deteriorated the systolic function of the right ventricle, particularly in patients with initial dysfunction of the right heart. Infusion of adrenaline simultaneously with PS leveled its negative effects, thus preventing myocardial dysfunction.

Adrenergic Agonists↗

Right ventricular failure--insights provided by a new model of chronic pulmonary hypertension.

This study was designed to examine the effects of both nitric oxide and milrinone on pulmonary hemodynamics and right ventricular function using a newly established model of monocrotaline pyrrole-induced chronic pulmonary hypertension. Sixteen mongrel dogs (23-25 kg) were used. All animals underwent percutanous pulmonary artery catheterization to measure right heart hemodynamics prior to and 8 weeks after a right atrial injection of either monocrotaline pyrrole (MCTP, n=8) or placebo (CTL, n=8). Eight weeks postinjection, all hearts were instrumented with a pulmonary artery flow probe and intracavitary micromanometers. Data were collected at baseline as well as following both nitric oxide and milrinone administration. There was no significant difference in the baseline hemodynamic measurements between the two groups. Eight weeks postinjection, significant increases in the pulmonary artery pressure and pulmonary vascular resistance were observed in MCTP compared with CTL. Both nitric oxide and milrinone resulted in significant improvements in pulmonary vascular resistance, pulmonary blood flow, and right ventricular contractility. In addition, nitric oxide caused a significant improvement in pulmonary artery pressure and transpulmonary efficiency, while milrinone led to a significant increase in right ventricular hydraulic power. This study demonstrates the well-known clinical effects of nitric oxide and milrinone in improving pulmonary hypertension, which were also associated with an increase in pulmonary blood flow, transpulmonary efficiency, and right ventricular hydraulic power in the setting of monocrotaline pyrrole-induced chronic pulmonary hypertension.

Administration, Inhalation↗