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Recently diagnosed idiopathic dilated cardiomyopathy: incidence of myocarditis and efficacy of prednisone therapy.

Fifty-two patients with recently diagnosed idiopathic dilated cardiomyopathy were studied to determine the incidence of myocarditis; patients were randomly assigned to receive either conventional therapy alone or conventional therapy plus prednisone to assess possible therapeutic efficacy with regard to survival. Inflammatory criteria were present in 23% of the population studied with 13% having overt myocarditis according to the Dallas criteria. The addition of prednisone to conventional therapy did not improve survival in a homogeneous population with new-onset dilated cardiomyopathy. Furthermore, the diagnosis of myocarditis by endomyocardial biopsy did not influence 2-year survival once dilated cardiomyopathy had developed. Biopsy-documented myocarditis resolved in all patients, according to results of 3-month follow-up endomyocardial biopsies, regardless of treatment group. There was a trend for patients with a left ventricular ejection fraction less than 20% to show reduced survival at 2 years compared to the group with a higher ejection fraction (p = 0.07). Right ventricular dysfunction determined at catheterization was present in 20 of 52 patients and was the most significant predictor of survival. Patients with preserved right ventricular function had a 95% 24-month survival rate compared to 47% for patients with right ventricular diastolic dysfunction (right ventricular end-diastolic pressure greater than or equal to 11 mm Hg) (p = 0.005).

Adult↗

Effects of atrial cardioplegia on the ischemic right ventricle after acute coronary artery occlusion and reperfusion.

Right atrial cardioplegia has been advocated as a simple method of delivering retrograde cardioplegia. Passive distention of the right heart inherent with right atrial cardioplegia has been shown to impair right ventricular function in a canine model of global ischemia. This study was designed to compare right ventricular performance after right atrial cardioplegia administered intermittently (n = 5) and continuously (n = 5) with coronary sinus retrograde cardioplegia (n = 5) and aortic root cardioplegia (n = 8) in a canine model of acute right ventricular ischemia and reperfusion. Right ventricular performance was assessed using the load-independent relationship of end-systolic pressure versus dimension (myocardial fiber length). Right ventricular performance was well preserved after reperfusion in those dogs protected with intermittent right atrial cardioplegia (95% of control). Results with continuous right atrial cardioplegia (66% of control) and coronary sinus retrograde cardioplegia (40% of control) demonstrated diminished postreperfusion right ventricular performance. Right ventricular performance in the group protected with aortic root cardioplegia was significantly impaired after reperfusion when compared with all retrograde groups (34% of control, p less than 0.05). In this model, postreperfusion right ventricular performance was preserved in the right atrial cardioplegia groups despite passive ventricular distention. All methods of retrograde cardioplegia resulted in superior preservation of right ventricular performance when compared with standard aortic root cardioplegia.

Animals↗

Different effects of prolonged exercise on the right and left ventricles.

To examine the functional consequences of the greater increase in right ventricular work with exercise, the effects of prolonged exercise on the right and left heart chambers were compared in 41 athletes before, at the finish (13 min) and after recovery (28 h) from the Hawaii Ironman Triathlon (3.9 km swim, 180.2 km bike ride, 42.2 km run). Two-dimensional and Doppler echocardiograms were analyzed for left and right atrial and ventricular areas at end-diastole and end-systole, right and left ventricular inflow velocities and mitral and tricuspid regurgitation. After exercise, left ventricular and left and right atrial sizes were reduced, whereas right ventricular size increased (diastole: 21.4 to 24.2 cm2; systole: 15.8 to 18.2 cm2; p less than 0.01). The emptying fraction of all chambers was unchanged. Left but not right ventricular inflow showed an increase in peak velocity of rapid filling, whereas both atrial systolic velocities increased (26 to 38 cm/s tricuspid; 38 to 54 cm/s mitral; both p less than 0.01). Overall, the right ventricular early to atrial velocity ratio was reduced after exercise (1.56 to 1.17; p less than 0.05) and the left ventricular pattern was unchanged. The prevalence of tricuspid regurgitation was statistically unchanged (86% to 52%), although that of mitral regurgitation was greatly reduced (76% to 0%). Changes in all variables returned toward prerace values during recovery. Thus, in highly trained athletes, prolonged exercise causes differing responses of the right and left ventricles. These differences may be due to changes in right ventricular function, shape or compliance.

Adult↗

Surgical treatment of double-outlet left ventricle in situs inversus [I,D,D].

A case of double-outlet left ventricle [I,D,D] with a subaortic ventricular septal defect and pulmonary stenosis is reported. The diagnosis was made at operation and was reconfirmed postoperatively by two-dimensional echocardiography. Repair was achieved by patch closure of the ventricular septal defect and enlargement of the functional right ventricular outflow tract using a valved patch. The postoperative course was uneventful except for a transient complete heart block. To our knowledge, this represents the second instance of surgical treatment of double-outlet left ventricle [I,D,D] reported in the literature.

Child↗

Right ventricular targeted gene transfer of a beta-adrenergic receptor kinase inhibitor improves ventricular performance after pulmonary artery banding.

OBJECTIVE: Abrupt increases in right ventricular afterload occur after cardiac transplantation and pulmonary artery banding, which can result in right ventricular hypertrophy and dilatation. Right ventricular dysfunction is also accompanied by beta-adrenergic receptor desensitization. We sought to determine whether selective right ventricular expression of a transgene encoding a beta-adrenergic receptor kinase inhibitor can improve right ventricular remodeling early after pulmonary artery banding. METHODS: Rabbits underwent pulmonary artery banding 3 days after percutaneous right coronary artery injection of empty adenovirus (n = 19), a control adenovirus containing the beta-galactosidase transgene (n = 10), or an adenovirus containing the beta-adrenergic receptor kinase inhibitor transgene (n = 14). Sham-operated animals (n = 7) underwent instrumentation without deployment of the pulmonary artery band. Right ventricular function was assessed in each rabbit before and 7 days after pulmonary artery banding. Right ventricular mass and dimensions (surface area and volume) were obtained, and biochemical analysis was performed to confirm transgene expression and to characterize beta-adrenergic receptor signaling. RESULTS: Right ventricular mass was increased in animals treated with adenovirus containing the beta-adrenergic receptor kinase inhibitor transgene, adenovirus containing the beta-galactosidase transgene, and empty adenovirus after banding when compared with results in sham-operated animals. However, right ventricular volume and surface area, as measures of dilatation, were significantly lower in pulmonary artery banded rabbits pretreated with adenovirus containing the beta-adrenergic receptor kinase inhibitor transgene when compared with those treated with empty adenovirus or adenovirus containing the beta-galactosidase transgene. Right ventricular contractility and defective beta-adrenergic receptor signaling were significantly enhanced in rabbits expressing the beta-adrenergic receptor kinase inhibitor after pulmonary artery banding. CONCLUSIONS: Right ventricular preconditioning with the beta-adrenergic receptor kinase inhibitor transgene can attenuate the early right ventricular dilatation and dysfunction associated with pulmonary artery banding. Thus beta-adrenergic receptor kinase inhibition might represent a novel target for limiting ventricular remodeling after increased right ventricular afterload.

Adenoviridae↗

Management of pulmonary hypertension resulting from interstitial lung disease.

Pulmonary hypertension affects right ventricular function by imposing an afterload on the right ventricle and, therefore, as pulmonary hypertension worsens, it ultimately reduces cardiac output. It is clear that in interstitial lung disease, progression of the underlying process with the loss of lung parenchyma contributes substantially to outcome. However, the additional burden of pulmonary hypertension often results in rapid deterioration. Pulmonary hypertension may be treatable and thus may enable the patient to survive until other therapy is effective. Until recently, part of the hesitancy in treating pulmonary hypertension in patients with secondary pulmonary hypertension, has been the limited availability of oral or intravenous drugs that affect the pulmonary circuit without causing excessive systemic vasodilatation. The current drugs available for the treatment of pulmonary hypertension, including prostacyclin and endothelin receptor blockers, have been limited by their high costs and, in the case of prostacyclin, the drug delivery systems. Nitric oxide, which has been used both acutely and chronically for pulmonary hypertension in a variety of diseases, also has limited availability. The role of sildenafil and inhaled prostacyclin remains to be evaluated. The use of these agents in the setting of interstitial lung disease needs to be studied, including combination therapy and early initiation of therapy. Their effect on tissue damage, fibrosis, and inflammation needs to be assessed as well as their effect on the arteriopathy and their potential to cause ventilation-perfusion (V/Q) mismatching. There now appears to be evidence to support treating pulmonary hypertension in patients with interstitial lung disease, with a potential for clinical benefit and a molecular basis for doing it.

Antihypertensive Agents↗

Alterations in cardiac performance in experimentally-induced diabetes.

Responses in cardiac tissue isolated from streptozotocin (STZ)-treated and age-matched control rats to isoproterenol (ISO) and calcium were studied. 4 weeks after STZ, atrial function was found to be depressed, whereas right ventricular function was enhanced. Neither tissue exhibited an alteration in sensitivity to ISO. In contrast, while sensitivity to calcium was found to be unaltered in atrial tissue, supersensitivity to calcium was observed in ventricles after STZ treatment. beta-Adrenoceptor number determination revealed a 36% decrease in Bmax with no change in affinity after STZ treatment. These results suggest that 4 weeks after STZ treatment pacemaker function is depressed, while enhancement in ventricular function is associated with an alteration in calcium utilization.

Animals↗

Gated blood-pool imaging in mechanical left ventricular assistance following myocardial infarction in dogs.

Gated blood-pool scanning has been used to assess the physiological effects of left ventricular assistance following acute myocardial infarction in dogs. Both left atrial-aortic and left ventricular-aortic bypass improve survival up to 6 hr after acute coronary occlusion, compared with control animals. The initial measurement of left ventricular ejection fraction (LVEF) following occlusion appears predictive of the need for ventricular assistance. Assisted animals maintain their level of left ventricular function until 6 hr in contrast with control animals, whose function steadily deteriorates over this period. There was no demonstrable recovery in regional ventricular function in the infarcted territory over this period, and in all assisted animals the right ventricular function deteriorated more rapidly than in controls. These results demonstrate the efficacy of left ventricular assistance in terms of improved survival and preserved left ventricular function. Gated blood-pool ventriculography has also been shown to be a suitable technique for monitoring the physiological changes during left ventricular assistance.

Animals↗

Transfer function analysis of the ventricular function: a new method for calculating right ventricular ejection fraction.

The relationship between the ventricular transfer function and ejection fraction has been investigated by the routine procedure of first-pass radionuclide angiocardiography. Ejection fraction has been shown to equal 1-e(-b), where b is the ratio of the R-R interval over the mean transit time difference between ventricular and atrial time-activity curves. To evaluate the effect of region of interest (ROI) on the right ventricular ejection fraction (RVEF), the results of the transfer function analysis (TFA) technique using precise ROI, TFA using rectangular ROI, and the routine method were compared. Regression analyses among RVEFs obtained from the above ROI methods yielded good correlations. Reliable RVEFs have been obtained even in the case of an improper bolus injection. Thus, the TFA technique is a new, simple, and reliable method for calculating RVEF without needing to outline the right ventricle precisely.

Heart↗

The relationship between "normal" transesophageal color-flow Doppler-defined tricuspid regurgitation and thermodilution right ventricular ejection fraction measurements.

Twenty coronary artery revascularization patients, aged 58 +/- 15 years, were studied intraoperatively to define the impact of Doppler-defined tricuspid regurgitation on measurement of thermodilution right ventricular ejection fraction (50 msec response pulmonary artery catheter). Right ventricular function was also estimated using a measurement technique independent of flow patterns across the tricuspid valve (transesophageal two-dimensional echocardiographic 5.0 MHz phased-array transducer). Measurements included transverse plane long- and short-axis planimetered area ratio, respectively, and tricuspid annular plane systolic excursion ratio (ratio = end-diastolic minus end-systolic value divided by end-diastolic value). Data were expressed as thermodilution-echocardiographic gradients, ie, thermodilution ejection fraction minus long-axis planimetered area ratio, short-axis planimetered area ratio, and tricuspid annular plane systolic excursion ratio, respectively. Tricuspid regurgitation was quantified by color-flow Doppler perimetry of maximal regurgitation jet area and analysis of transduced right atrial pressure waveform. Doppler estimates were expressed as absolute values and as a function of corresponding atrial area (tricuspid regurgitation index = planimetered jet area divided by right atrial area). Data were obtained following endotracheal intubation, sternotomy, pericardiotomy, cardiopulmonary bypass, and chest closure. Data were evaluated by regression analysis, with separate analyses performed for each time period. Profiles were unassociated with right atrial pressure waveform abnormalities. There was no significant relationship between thermodilution ejection fraction variance values and tricuspid regurgitation jet area or regurgitation index, respectively. In each measurement period, thermodilution-echocardiographic gradients were also unrelated to the tricuspid regurgitation estimates.(ABSTRACT TRUNCATED AT 250 WORDS)

Catheterization, Swan-Ganz↗

Glycine application and right heart function in a porcine heart transplantation model.

Glycine reduces ischemia-reperfusion injury after experimental liver transplantation. We hypothesized that glycine might also protect right heart function in an isovolumic cardiac transplantation model. In six domestic donor pigs 150 ml of a 300 mmol L-glycine solution were administered intravenously. The hearts were then arrested with histidine-tryptophan-ketoglutarate solution. Animals without prior glycine infusion served as controls (n = 6). After 4 h of ischemia, hearts were transplanted into recipients. An isovolumic model was used in which the right ventricular (RV) volume was controlled in vivo using an intracavitary high-compliance balloon. After 1 and 2 h of reperfusion the RV balloon volume was gradually increased and the developed pressures were recorded (P(developed) = P(systolic) - P(diastolic)). Right ventricular failure was defined as a decrease in developed intracavitary pressure. Glycine hearts could be loaded with a significantly increased volume after 1 h (glycine: 53 +/- 13.7 ml vs. control: 32 +/- 11.7 ml; P = 0.015) and after 2 h (67 +/- 18.6 ml vs. 43 +/- 8.2 ml; P = 0.018). Maximal RV developed pressures were not significantly different between groups. Postischemic RV end-diastolic compliance was significantly higher in glycine-treated animals (P = 0.04). Glycine protects early postischemic RV compliance, but has no important influence on maximal developed pressures.

Animals↗

Effects of long-term blood pressure control on left and right ventricular structure and function in hypertensive patients.

The functional and anatomical abnormalities of the right ventricle may occur in hypertensive patients with left ventricular hypertrophy (LVH). The present study was designed to assess the functional and structural changes in both left and right ventricles induced by chronic antihypertensive therapy. Doppler and standard echocardiography were performed in 10 hypertensive patients with LVH before and after 1 year of treatment with captopril alone (5 patients) or captopril plus nifedipine (5 patients). We found that the left ventricular mass index and right ventricular thickness were significantly reduced in all patients when compared with pretreatment values. Furthermore, the Doppler-derived diastolic filling indexes show a significant improvement of both ventricular chambers. Our data suggest that anatomical and functional changes induced by therapy in hypertensive patients are not limited to the left ventricle but also involve the right ventricle.

Blood Pressure↗

[CINE-MRT for the study of the effects of regional left ventricular wall motion disorders on global heart function after a myocardial infarct and revascularization].

PURPOSE: To determine changes in global cardiac function and mass caused by infarct-associated regional wall motion abnormalities and to compare the changes after revascularization in patients with and without improvement of regional contractility. MATERIALS AND METHODS: 21 patients with regional left ventricular wall motion abnormalities and associated coronary artery stenoses requiring revascularization were examined with a Cine FLASH-2D sequence 26 +/- 12 days after their first myocardial infarction and re-examined three months after revascularization. Regional contractility and volumes and masses of both ventricles were determined. RESULTS: After revascularization, regional wall motion improvement led to decreased left ventricular volumes and improved ejection fractions, whereas patients with persisting wall motion defects showed unchanged left ventricular functional parameters. Comparing both groups of patients, the patients with improvement of regional contractility revealed lower end-systolic volumes and higher ejection fractions at follow-up. Cardiac masses and right ventricular parameters were not different, patients with a depressed right ventricular ejection fraction showed improvement at follow-up. DISCUSSION: After myocardial infarction, revascularization of the infarct-related coronary artery leads to an improvement of left ventricular function only if there is also an improvement of regional contractility. An effect on right ventricular function was not observed three months after the first small- or middle-sized myocardial infarction.

Adult↗

Left ventricular systolic and diastolic function after two-stage anatomic correction of transposition of the great arteries.

Anatomic repair of transposition of the great arteries (TGA) has been developed because of concerns about right ventricular function after atrial repair by the Mustard or the Senning technique. This study assessed left ventricular systolic and diastolic function in three patients after two-stage anatomic repair. Two patients had a ventricular septal defect (one with coarctation), and the third patient had right ventricular dysfunction precluding atrial repair. All had pulmonary artery banding. The mean ages at the time of repair and catheterization were 2.75 and 4.9 years, respectively. The control group included 10 patients with insignificant or no cardiac disease. At cardiac catheterization the group with TGA had a higher mean end-diastolic volume index (110.9 +/- 4.74 ml/m2) compared to normal subjects (79.1 +/- 14.55; p less than 0.001), mean end-systolic volume index (37.3 +/- 3.69 vs 22.7 +/- 4.42; p less than 0.001), mass index (101.0 +/- 16.9 vs 68.2 +/- 12.34; p = 0.038), and stroke volume index (73.6 +/- 3.52 vs 56.5 +/- 12.1; p = 0.0027). The ejection fractions, end-diastolic and peak systolic pressures, and stresses were not different. There was no difference in the relationship between the mean rate-corrected velocity of circumferential fiber shortening and end-systolic stress for the group with TGA, but myocardial stiffness was markedly elevated (29.5 +/- 1.84 vs 10.8 +/- 2.20; p less than 0.001). Thus, this study found abnormalities of left ventricular size after two-stage anatomic repair of TGA in this group of patients with TGA.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗

DA1-receptor stimulation by fenoldopam in the treatment of postcardiac surgical hypertension.

BACKGROUND: Besides adequate analgesia, sedation and ventilation, postcardiac surgical hypertension has to be treated frequently with vasoactive drugs to avoid possible complications. In this study the hemodynamic effects of the DA1-receptor agonist fenoldopam (F) are compared to those of the Ca-channel antagonist nifedipine (N). METHODS: Postoperatively, 64 CABG-patients with a mean arterial pressure (MAP) of more than 105 mmHg over 10 min were investigated. Patients with compromised ventricular function, insufficient surgical repair, arrhythmia or an ECG unable to detect myocardial ischemia were excluded. The study drugs (initial dosage: F: 0.8; N: 0.3 micrograms.kg-1.min-1) were given continuously via a central venous catheter to reduce and to maintain the MAP between 80 and 95 mmHg. Hemodynamic parameters were determined using thermodilution technique. RESULTS: A significant reduction of the MAP (F: from 121 +/- 11 to 83 +/- 4, N: from 119 +/- 8 to 82 +/- 9 mmHg) and of the calculated systemic vascular resistance (SVR) (F: 2110 +/- 500 to 970 +/- 200, N:1980 +/- 660 to 1020 +/- 300 dyn.s.cm-5) were noted in both groups, whereby in the F group the therapeutic goal could be achieved more quickly with the dosage regimen chosen. As a result, a marked increase of heart rate, cardiac index and stroke volume index could be observed, which was more pronounced due to the initially stronger decrease of SVR with F. There was also a stronger decrease of pulmonary vascular resistance in the F group, but the indices of right ventricular function did not differ between the groups. CONCLUSION: Fenoldopam seems to be an efficient alternative to nifedipine, especially because of its more rapid onset of action.

Antihypertensive Agents↗

Right and left ventricular function at rest and with exercise after the Mustard operation for transposition of the great arteries.

Right and left ventricular function was assessed in 25 children (mean age at study 12.2 years and at operation 2.6 years) after a Mustard repair for transposition of the great arteries. Gated first pass and gated equilibrium radionuclide ventriculography was performed on all patients at rest and during supine bicycle exercise. The mean right ventricular ejection fraction did not increase with exercise by either technique. Individual results for right ventricular ejection fraction showed that with the gated equilibrium technique 71% had an abnormal exercise response (normal response being an increase in ejection fraction greater than 5%) and with the gated first pass technique 61% had abnormal results. Although the mean left ventricular ejection fraction increased significantly with exercise, 35% of patients had an abnormal exercise response with the equilibrium technique and 41% with the first pass technique. There was no evidence of a predictive relation between ventricular function and any clinical or haemodynamic variable examined. Covert right and left ventricular dysfunction can frequently be detected by exercise radionuclide ventriculography in long term survivors of repair for transposition of the great arteries. The prognostic consequences of these findings are unclear at present.

Adolescent↗

Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies.

OBJECTIVE: To describe aspects of the natural history and pathophysiology of giant low pressure pulmonary artery aneurysms and to propose potential surgical strategies. DESIGN: Cross sectional retrospective review. SETTING: Supraregional tertiary referral centre. PATIENTS: All adult patients referred for assessment of giant pulmonary artery aneurysm retrospectively identified from the Mayo Adult Congenital Heart Disease Clinic database. METHODS: Patient data were reviewed from hospital records, including echocardiograms, magnetic resonance images, radiographs, and histology slides. RESULTS: Four patients were identified with a median age of 52 years (range 37-64 years). Presenting symptoms were effort related dyspnoea, chest discomfort, and hoarseness in one patient. All patients had pulmonary regurgitation and clinical evidence of right ventricular enlargement in association with a pulsatile mass at the upper left sternal edge. Transthoracic echocardiography showed the giant pulmonary artery aneurysm involving the main pulmonary artery and proximal branches, and confirmed severe pulmonary regurgitation in all patients. None of the patients had intimal tearing, medial dissection, or pulmonary arterial rupture. The pulmonary valve was replaced to relieve symptoms and preserve right ventricular function. Pulmonary arterial histology showed medial degeneration of elastic fibres with accumulation of basophilic ground substance. CONCLUSIONS: Rupture or dissection of these low pressure aneurysms is rare. The timing of surgical intervention should be determined by changes in right ventricular size and function resulting from pulmonary regurgitation or pulmonary stenosis, and not the size of the aneurysm.

Adult↗

Prolonged cardiac recovery from exercise in asymptomatic adults late after atrial correction of transposition of the great arteries: evaluation with magnetic resonance flow mapping.

After atrial correction of transposition of the great arteries (TGA), dysfunction of the systemic right ventricle at rest and during exercise has been reported. Information on changes in systemic right ventricular function during recovery from exercise is lacking. This study evaluates cardiac recovery from supine exercise using magnetic resonance (MR) imaging in patients with asymptomatic TGA after atrial correction. Flow in the ascending aorta, representing stroke volume of the systemic ventricle, was assessed with MR flow mapping in 10 asymptomatic patients with atrially corrected TGA and in 12 controls at rest during exercise and an 8-minute recovery period. In response to exercise, the patients had a smaller increase in heart rate, stroke volume, and cardiac output than did controls. After exercise, no significant difference in halftime of heart rate recovery was observed (patients, 48 +/- 7 seconds; controls, 39 +/- 4 seconds [p >0.05]). In the patients, the time course of stroke volume recovery was significantly different (p <0.001). Stroke volume in the patients, as a percent difference from rest, remained significantly elevated, from 2.5 minutes (+16 +/- 5% vs +7 +/- 6%; p <0.05) to 8 minutes (+4 +/- 7% vs -3 +/- 5%; p <0.05) after exercise. Subsequently, cardiac output remained significantly elevated, from 4.5 minutes (+27 +/- 13% vs +15 +/- 11%; p <0.05) to 7 minutes (+22 +/- 11% vs +12 +/- 12%; p <0.05) after exercise. We conclude that heart rate recovery is within normal limits in patients with atrially corrected TGA. Furthermore, cardiac recovery from exercise, assessed with MR flow mapping, is prolonged in patients with asymptomatic TGA after atrial correction. Abnormal recovery may reflect dysfunction of the systemic right ventricle and an altered metabolic response to exercise.

Adolescent↗