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Isolated right ventricular cardiomyopathy in a dog.

Right-sided heart failure was diagnosed in a young dog with clinical and pathologic features of isolated right ventricular cardiomyopathy. This disorder, also referred to as right ventricular dysplasia, is an infrequent cause of heart failure and sudden death in people. Echocardiography of this dog revealed right ventricular and right atrial dilatation and severe right ventricular systolic dysfunction. Left ventricular size and function were normal. Electrocardiography revealed ventricular bigeminy. The configuration of the ectopic beats was similar to that seen with left bundle branch block. The dog died suddenly, and histologic examination of the heart revealed fibroadipose replacement of right atrial and right ventricular myocardium.

Animals↗

Right and left ventricular dysfunction in patients with dilated cardiomyopathy: a study with equilibrium radionuclide ventriculography.

Right and left ventricular function was evaluated in 28 patients with dilated cardiomyopathy to determine whether both ventricular functions were equally impaired in each patient. The ejection fractions of both ventricles were measured using equilibrium radionuclide ventriculography. In 13 patients the left ventricular ejection fraction (LVEF) was lower than the right ventricular ejection fraction (RVEF) by 6% or more, their mean values being 24 +/- 8% (mean +/- SD) and 37 +/- 7%, respectively (group 1). The difference between the LVEF and RVEF was less than 6% in nine patients; LVEF 29 +/- 8% and RVEF 30 +/- 7% (group 2). In six patients the RVEF was lower than the LVEF by 6% or more, and their mean values were 21 +/- 6% and 37 +/- 10%, respectively (group 3). The frequency of ventricular tachycardia, which was determined by the Holter ECG, was significantly higher in group 3 (100%, 6/6) than in the others (group 1 + 2; 41%, 9/22), p less than 0.05. The NYHA functional class correlated well with the LVEF, but not with the RVEF. It was concluded that the left and right ventricular functions are not necessarily equally impaired in patients with dilated cardiomyopathy, and that the difference may correlate with their clinical features.

Adult↗

Regional left ventricular motion during early filling phase in patients with right ventricular pressure overload.

Global left ventricular (LV) diastolic function has been reported to be disturbed under conditions of right ventricular pressure overload (RVPO). However, from the standpoint of regional wall motion, only a little information related to the mechanism of LV diastolic dysfunction is available. Eight patients with RVPO and 7 healthy volunteers were investigated using tagged cine magnetic resonance imaging. Regional diastolic fraction (RDF) was determined in 4 segments (anterior, lateral, inferior, and septal) in the mid-ventricular short axis section and in 2 segments (septal and lateral) in the 4-chamber section. A heterogeneity index was obtained from the RDFs of the short axis section. In the RVPO group, in both short axis and 4-chamber sections, the RDF of the septal segment was depressed, and it showed an inverse correlation with the right-to-left ventricular systolic pressure (RV/LV) ratio (r = -0.74, p < 0.05) in the short axis section. In the 4-chamber section, the RDF was lower in the septal segment than in the lateral segment (p < 0.05). The heterogeneity index in the RVPO group was greater than that in the control group (p < 0.01). The index correlated positively with the RV/LV ratio (r = 0.77, p < 0.05). The altered regional diastolic motion results in increased heterogeneity in regional diastolic motion.

Adolescent↗

Submassive and massive pulmonary embolism: a target for thrombolytic therapy?

Thrombolytic agents have been consistently demonstrated to dissolve pulmonary thrombi much more rapidly and effectively than heparin alone. Rapid resolution of pulmonary embolism (PE) is accompanied by a significant decrease in pulmonary artery pressure and an improvement in right ventricular function. However, it is no longer than 7 days until the findings of patients treated with heparin improve to a similar extent. Previous studies were not designed to determine whether this short-lasting difference in favor of thrombolysis can indeed affect the prognosis of patients with PE and thus justify the 1% (or even higher) risk of cerebral or fatal bleeding. Recently, two large registries demonstrated the importance of right ventricular dysfunction assessed by echocardiography as an independent predictor of mortality. Thrombolytic treatment was shown in one of these registries to be associated with a 50% reduction of death risk in clinically stable patients with right ventricular enlargement. It was thus possible to identify a group of patients with massive PE who are most likely to benefit from early thrombolysis. These findings now have to be confirmed by a prospective randomized trial which will compare thrombolysis with heparin alone in this high-risk patient population, focusing on clinical end points such as overall and event-free survival in the acute phase of PE.

Animals↗

The pathophysiology and management of perioperative pulmonary hypertension with specific emphasis on the period following cardiac surgery.

Pulmonary hypertension, right ventricular dysfunction, and RV failure are common occurrences following CPB. The endothelium is now recognized as an important organ and is central to the pathophysiology and management of this condition. Current progress has arisen from the development of newer methods and the refinement of older methods attempting to protect the endothelium. Modern methods attempting to preserve endothelial function (thereby preventing pulmonary vasoconstriction and RV dysfunction) include new pharmacological inhibitors of the inflammatory response to CPB, improved myocardial protection using substrate enhanced cardioplegia, improved cardioplegia delivery techniques combined with monitoring by myocardial contrast TEE, and new minimally invasive surgical techniques not requiring extracorporeal support. Progress in therapeutic methods attempting to reverse pulmonary hypertension, RV dysfunction, and support of the failing right ventricle include the application of nitric oxide, prostacyclin, other pulmonary selective vasodilators, and improved ventricular assist devices that provide support to the right ventricle until recovery of the myocardium occurs or serve as a bridge to transplantation.

Cardiopulmonary Bypass↗

Myocardial revascularization as a therapeutic strategy in the patient with advanced ventricular dysfunction.

The number of patients with severe ventricular dysfunction from coronary artery disease is constantly increasing. Although the medical management of these patients with angiotensin-converting enzyme inhibitors and beta-blockers has favorable impact on the morbidity and mortality the overall prognosis is still poor. Historically many of these patients have been referred for transplantation. In the past few years there has been an increasing amount of information about the utility of surgical revascularization in patients with low ejection fraction. Careful patient selection and optimal perioperative management is of critical importance for good outcome. Coronary artery bypass grafting (CABG) can be performed relatively safely despite the advanced level of left ventricular dysfunction. Quality of life is improved by CABG with elimination of angina and enhanced functional capacity. Improvement in the ejection fraction and increased survival after the operation has been objectively demonstrated. However patients with advanced right ventricular dysfunction, pulmonary hypertension, redo bypass and ungraftable coronaries should be considered for heart transplantation. In this review we describe our experience and focus on pertinent issues in patient selection, perioperative management and long term outcome after coronary artery bypass grafting.

Aged↗

[The effect of continuous positive pressure in the nasal airway on the right ventricular function in obstructive apnea sleep syndrome].

BACKGROUND: The aim of this study was to evaluate the right ventricular function in the obstructive apnea sleep syndrome (OSAS) and to determine the effect of the continuous use of a continuous positive pressure nasal device on the airway (CPSPn) produces on this aspect of the disease. METHODS: Forty patients were diagnosed of OSAS by study of spontaneous night sleep. A functional respiratory study was performed in all the patients as was a calculation of the index of body mass (IBM) and isotopic ventriculography for the calculation of the right and left ventricular ejection fractions (RVEF and LVEF). Twenty-six patients were followed after 8.4 +/- 3.3 months of home treatment with CPAPn in which these studies were repeated. RESULTS: Twenty-four of the 40 patients (60%) had RVEF lower than 0.45. These 24 patients had paO2 in vigil state (69.9 +/- 13.6 mmHg) than those with a normal RVEF (80.1 +/- 8.7 mmHg) (p < 0.05). After treatment with CPAPn an elevation was observed in the RVEF in the group which was followed. This increase was significant in the subgroup sharing low RVEF (n = 16) prior to starting treatment upon passing from 0.39 (+/- 0.02) to 0.45 (+/- 0.04) (p < 0.001). CONCLUSIONS: Right ventricular dysfunction in frequent in patients with the obstructive apnea sleep syndrome and is more frequent in patients maintaining hypoxemia in vigil. The continual use of continuous positive pressure in the nasal airway produces improvement in right ventricular function particularly in those in whom this was most disturbed.

Adult↗

Evaluation of pulmonary vascular response to inhaled iloprost in heart transplant candidates with pulmonary venous hypertension.

OBJECTIVE: Chronic left-heart failure is often associated with the development of pulmonary venous hypertension. In heart transplant candidates this is of great significance because the healthy donor heart has to compensate the increased right-ventricular afterload. Right-ventricular dysfunction is still responsible for 19% of all early deaths after orthotopic heart transplantation. Careful preoperative assessment of pulmonary vascular resistance by right-heart catheterization is essential. Reversibility testing is generally carried out to clarify therapeutic options for the post-transplant period. The objective of this case series is to report our institutional experience with inhaled iloprost compared to the common used oxygen/nitroglycerin method for reversibility testing. METHODS: Right-heart catheterization was performed in 23 patients with severely impaired left-ventricular function (EF < or = 25%, pVO2 < or = 14 ml/kg/min, NYHA III or IV) with combined pulmonary venous hypertension (TPG > 12 mm Hg and or PVR > 250 dyn x s x cm(-5)). An intraindividual comparison was performed between of the hemodynamic effect with oxygen/nitroglycerin s.l. and inhaled iloprost. RESULTS: The transpulmonary gradient fell significantly from an initial 16 mm Hg to 13 mm Hg on oxygen/nitroglycerin s.l. compared to 10 mm Hg on inhaled iloprost. Pulmonary vascular resistance fell significantly from an initial 344 dyn x s x cm(-5) to 270 dyn x s x cm(-5) on oxygen/nitroglycerin s.l. compared to 209 dyn x s x cm(-5) on inhaled iloprost. On inhaled iloprost a moderate systemic effect was noticed. CONCLUSION: In heart transplant candidates with pulmonary venous hypertension reversibility testing with inhalation of iloprost is a save method and significantly more effective than the combination of inhaled oxygen plus nitroglycerin s.l.

Administration, Inhalation↗

Echocardiographic findings in myocarditis.

This study analyzes morphologic and functional alterations detected by M-mode and 2-dimensional echocardiography in 41 patients with histologically proven myocarditis and different clinical presentations: congestive heart failure (63%), atrioventricular block (17%), chest pain (15%) and supraventricular arrhythmias (5%). Left ventricular dysfunction was common (69%), particularly in patients with congestive heart failure (88%), often without or with minor cavity dilatation. Patients with atrioventricular block or chest pain had usually preserved ventricular function. Right ventricular dysfunction was present in 23%. Additional findings included asynergic ventricular areas (64%), left ventricular "hypertrophy" sometimes reversible (20%), hyperrefractile myocardial areas (23%), ventricular thrombi (15%) and "restrictive" ventricular filling (7%). It is concluded that echocardiographic features of myocarditis are polymorphous and nonspecific. The echocardiographic pattern can simulate alternatively dilated, hypertrophic, restrictive or "right" ventricular cardiomyopathy, as well as coronary artery disease. In an appropriate clinical context, echocardiography can be helpful in the diagnosis of myocarditis and in the selection of patients for endomyocardial biopsy.

Adult↗

Pulmonary hypertension and cor pulmonale in COPD.

Pulmonary artery hypertension (PAH) is the primary cardiovascular complication encountered in chronic obstructive pulmonary disease (COPD). Cor pulmonale can range clinically from mild changes in right ventricular function to frank right heart failure. The prevalence of PAH increases as COPD worsens, and the development of PAH and cor pulmonale appears to affect survival of patients with COPD. Potential causes proposed to explain the development of PAH in COPD include gas exchange abnormalities, destruction of the pulmonary vascular bed, alterations in respiratory mechanics, changes in intrinsic pulmonary vessel tone, and increased blood viscosity. Standard clinical evaluation, including history, physical examination, spirometry, electrocardiography, and chest radiography, is generally inadequate in identifying right ventricular dysfunction. Noninvasive techniques, such as echocardiography, radionuclide ventriculography, and magnetic resonance imaging, have largely replaced invasive pulmonary artery catheterization in the initial assessment of cor pulmonale. The goals of therapy consist of attenuation of PAH, enhancement of right ventricular function, alleviation of clinical symptoms, and improvement in survival. The agents that have been most extensively evaluated for these purposes include oxygen, vasodilators, theophylline, and inotropic medications.

Journal Article↗

Tricuspid regurgitation in patients with beta-thalassemia major.

Although cardiac complications remain the main causes of death in thalassemic patients, right heart dysfunction has been little studied and the mechanism is still unclear. Echocardiography was performed in 39 patients with beta-thalassemia major and 35 aged-matched controls. The gender, age, heart rate, blood pressure, left ventricular ejection fraction (LVEF), acceleration time (AcT) of right ventricular outflow and right ventricular ejection time (RVET), AcT/RVET, and the presence of tricuspid regurgitation (TR) were compared between the two groups. We also compared the gender, age, age at first blood transfusion, serum ferritin level, alanine aminotransferase (ALT), the presence of antibodies to hepatitis C virus, liver fibrosis, splenectomy, platelet counts, diabetes mellitus, arrhythmia, cardiomegaly, LVEF, AcT, RVET, AcT/RVET, and signal intensity ratio (SIR) of myocardial magnetic resonance imaging (MRI) between thalassemic patients with and without TR. The incidence of TR in thalassemic patients was significantly higher than that in the control group (30.8 vs 11.4%, p=0.03). The incidences of splenectomy (p=0.03), platelet counts (p=0.01), and SIR of myocardial MRI (p=0.03) in thalassemic patients with TR were significantly higher than in those without TR. The AcT was shorter and the AcT/RVET ratio was smaller, suggesting higher pulmonary pressure in the thalassemic patients with TR. Occurrence of TR in patients with beta-thalassemia major may be a consequence of cardiac iron deposit, thrombocytosis, splenectomy, or pulmonary hypertension.

Adolescent↗

Abnormal oxygen uptake responses to exercise in patients with mild pulmonary sarcoidosis.

Exercise intolerance and exertional dyspnea are common complaints in patients with sarcoidosis. Although in many cases these complaints are attributable to restrictive or obstructive lung mechanics or inefficiency of pulmonary gas exchange, other processes also may contribute to impairment in exercise function and may not be readily detected or distinguished from problems of lung mechanics on the basis of symptoms or routine laboratory testing. To identify the frequency and etiology of impaired exercise capacity in sarcoidosis patients with mild lung disease, integrative cardiopulmonary exercise testing was performed in 23 patients. Breath-by-breath measurements were made of gas exchange, ventilation, and heart rate. In 9 of 20 evaluable patients, the oxygen uptake (VO2) at the anaerobic threshold was low, and/or the rate of increase of VO2 was abnormal relative to work rate or heart rate, suggesting a defect in cardiocirculatory function. Resting and exercise echocardiography revealed normal left ventricular ejection fractions and wall motion in all nine of these patients, but findings suggestive of right ventricular hypertrophy and/or right ventricular dysfunction were present in five. Abnormal responses of VO2 during exercise are common in patients with sarcoidosis and may be due to subclinical impairment of right-sided cardiac function.

Adult↗

[Congestive heart insufficiency. Evaluation using isotopic methods].

Radioisotopic cardiac studies became, on the last decade, one of the more useful non-invasive methods for the diagnostic evaluation and follow-up of patients with heart disease. Such a contribution of nuclear cardiology is specially noticeable on ischemic heart disease: radionuclide angiography (RNA) allows quantification of left and right ventricular dysfunction, and therefore stands as an ideal technique for the prognostic approach of congestive heart failure. As to diagnosis, RNA is useful in the analysis of regional wall motion abnormalities of ischemic heart disease and detection of ventricular aneurysms. Clinical examples are given of these specific situations. In dilated cardiomyopathies RNA gives not only a measure of ventricular dysfunction, but also a diagnostic information, often definitive, of the diffuse involvement of all left ventricle, and commonly of the right ventricle. Ischemic heart disease, on the other hand, displays different degrees of segmental wall motion impairment together with areas of normal contractility. These aspects are documented with cases of our Registry. Also discussed is the inotropic stimulation technique for the evaluation of the functional reserve of left ventricle in patients with severe rest L.V. dysfunction. Focus is given to the clinical value of isotopic studies for the quantification of valvular regurgitations and pre-operative serial studies in order to define an optimum surgical timing. An attempt is done to grade the practical importance of the different exams of Nuclear Cardiology on the diagnostic and prognostic evaluation on heart disease patients, with a cost benefit relation perspective and comparing with other methods, classic of recent ones, available for the management of patients with heart failure.

Cardiomyopathy, Dilated↗

Atrial baffle procedures for complete transposition of the great arteries: natural course of sinus node dysfunction and risk factors for dysrhythmias and sudden death.

359 patients after the Mustard (275) or Senning (84) operations for transposition of the great arteries were followed-up for a mean of 103.7 (range 0.4 to 204) months. 259 patients had postoperative 24-h Holter recordings. In 129 Mustard children serial (mean 4.4) postoperative Holter recordings were available for evaluation. Criteria based on Holter and scalar electrocardiograms at normal sinus node functions were used for rhythm analysis. Postoperative dysrhythmias appeared in 70% of the patients: sinus node dysfunction in 62.8%, second or third degree atrioventricular block in 3.2%, sustained atrial tachycardia or atrial flutter in 4.5%, and significant ventricular arrhythmia (Lown 2-5) in 21.4%. The prevalence of sinus node dysfunction increased slightly from 50.8% during the first 2 postoperative years to 64.4% in patients more than 10 years postoperatively. Fifteen patients (4.2%) died suddenly during follow-up. By multivariate analysis severe tricuspid regurgitation and/or right ventricular dysfunction and uncontrolled supraventricular tachydysrhythmias were identified as the two significant risk factors for sudden death.

Adolescent↗

Pulsed Doppler tissue imaging of the velocity of tricuspid annular systolic motion; a new, rapid, and non-invasive method of evaluating right ventricular systolic function.

AIMS: Rapid, accurate, and widely available non-invasive evaluation of right ventricular function still presents a problem. The purpose of the study was to determine whether the parameters derived from Doppler tissue imaging of tricuspid annular motion could be used as indexes of right ventricular function in patients with heart failure. METHODS: Standard and pulsed Doppler tissue echocardiography were obtained in 44 patients with heart failure (mean left ventricular ejection fraction 24 +/- 7%) and in 30 age- and sex-matched healthy volunteers. The tricuspid annular systolic and diastolic velocities were acquired in apical four-chamber views at the junction of the right ventricular free wall and the anterior leaflet of the tricuspid valve using Doppler tissue imaging. Within 2 h of Doppler tissue imaging, the first-pass radionuclide ventriculogram, determining right ventricular ejection fraction and equilibrium gated radionuclide ventriculography single photon emission computed tomography, were performed in all patients. RESULTS: In patients with heart failure, the peak systolic annular velocity was significantly lower and the time from the onset of the electrocardiographic QRS complex to the peak of systolic annular velocity was significantly greater than the corresponding values in healthy subjects (10.3 +/- 2.6 cm. s(-1) vs 15.5 +/- 2.6 cm.s(-1), P < 0.001, and 198 +/- 34ms vs 171 +/- 29 ms, P < 0.01, respectively). There was a good correlation between systolic annular velocity and right ventricular ejection fraction (r = 0.648, P <0.001). A systolic annular velocity < 11.5 cm.s(-1)predicted right ventricular dysfunction (ejection fraction < 45%) with a sensitivity of 90% and a specificity of 85%. CONCLUSION: We conclude that the evaluation of peak systolic tricuspid annular velocity using Doppler tissue imaging provides a simple, rapid, and non-invasive tool for assessing right ventricular systolic function in patients with heart failure.

Adolescent↗