[Stenosis of the right ventricle caused by excentric hypertrophy of the left ventricle or ventricular septum (Bernheim's syndrome)].
Explore the source record for details and available documents.
SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Numerous technologic advances have greatly facilitated the noninvasive analysis of right ventricular function. Nevertheless, important clues continue to be available to the astute clinician by physical examination. The chest x-ray is of rather limited utility. The electrocardiogram can show evidence of right atrial enlargement or right ventricular hypertrophy. Unfortunately, both sensitivity and specificity are deficient. Echocardiography is a widely available and potentially very accurate source of information concerning right ventricular dysfunction. Careful temporal analysis of the M-mode echocardiogram can give information beyond chamber size and wall thickness. Two-dimensional echocardiography allows more accurate determination of chamber size and wall thickness and also permits analysis of segmental wall motion and chamber contour. Doppler echocardiography allows measurement of pressure differences and flow kinetics. Preliminary data indicate that one can accurately assess pulmonary artery pressure and possibly right ventricular diastolic function. Color-flow mapping allows for accurate determination of valvular regurgitation and enhances the accuracy of standard Doppler echocardiographic techniques. Radionuclide analysis of the right ventricle by blood-pool imaging allows accurate determination of ejection fraction and wall motion. In addition, it may be possible to estimate pulmonary artery pressure. Use of short-acting radionuclides allows for serial imaging of the right ventricle after pharmacologic intervention or exercise. Perfusion scanning can show evidence of exercise-induced ischemia, although applicability to the right ventricle is somewhat limited. Avid scanning allows localizing of myocardial injury to the right ventricle. CT scanning of the heart is of limited clinical utility, because cardiac motion occurs too rapidly for accurate imaging. The advent of the cine-CT may overcome this problem and allow evaluation of right ventricular volumes and wall motion. Digital subtraction imaging allows for accurate video densitometric calculation of ejection fractions, but offers no advantage over other currently available techniques. Magnetic resonance imaging may prove to be the methodology of choice for analysis of right ventricular function, because it can give accurate measurement of right ventricular wall motion, ejection fraction, and (similar to Doppler flow studies) some indication of flow within the right-sided chambers. It will soon be possible to generate information concerning the biochemical content of the right ventricular myocardium, perhaps providing early evidence of hypertrophy or myopathy.(ABSTRACT TRUNCATED AT 400 WORDS)
Combined heart-lung transplantation has been used for end-stage primary pulmonary hypertension. Experience with single-lung transplantation for other conditions suggested that associated severe right ventricular dysfunction resulting from increased afterload would recover after placement of a satisfactory lung allograft. Early experience with the application of single-lung transplantation for pulmonary hypertension supports this contention. We devised a reversible canine model of chronic progressive pressure-overloaded right heart failure by pulmonary artery banding to study the echocardiographic, hemodynamic, and pathological reversibility of the failing right heart. Clinical right heart failure was defined as the development of ascites and pleural effusions. Right heart failure developed in 23 dogs 67 to 348 days after banding, and they were divided into two groups to determine its early and long-term effects. Group 1 dogs (n = 11) were either sacrificed immediately after the onset of right heart failure (n = 5) or unbanded (n = 6); group 2 dogs (n = 12) were maintained in right heart failure for 3 months and then either sacrificed (n = 6) or unbanded. Unbanded dogs in both groups were observed for 4 additional months before sacrifice. A control group of 6 normal dogs was sacrificed for pathological comparisons. After unbanding, the right ventricular systolic pressure fell from 97 +/- 17 mm Hg (group 1) and 88 +/- 31 mm Hg (group 2) to 44 +/- 11 mm Hg and 47 +/- 13 mm Hg, respectively. Despite this persistent gradient across the pulmonary artery, echocardiographic and hemodynamic measures of right ventricular function returned to normal, albeit more slowly in the group 2 dogs.(ABSTRACT TRUNCATED AT 250 WORDS)
There are an increasing number of patients with moderate to severe pulmonary regurgitation who develop right ventricle dysfunction, with associated symptoms of fatigue and exercise intolerance, after surgical repair of tetralogy of Fallot. Critical longitudinal follow-up of these patients is needed, with assessment by clinical and laboratory studies of right ventricular size, function, and exercise capability. Pulmonary valve replacement is recommended before significant right ventricular dysfunction occurs. The search for an ideal valve to use in this condition continues.
The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.
Radionuclide ventriculography allows non-invasive assessment of right ventricular performance. This study has confirmed that there is a modest reduction in right ventricular ejection fraction (RVEF) in patients with chronic obstructive pulmonary disease (COPD), as compared to normal subjects. However, occult right ventricular dysfunction also becomes apparent in these patients during exercise. The change in RVEF during exercise is related to the corresponding fall in arterial oxygen saturation in patients with COPD. Oxygen improves the response of the right ventricle to exercise, although the mechanism remains unclear. Long-term oxygen therapy, in patients with respiratory failure, does not appear to have any significant effect on RVEF.
OBJECTIVES: This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation. BACKGROUND: Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented. METHODS: Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined. RESULTS: Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1. Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work. CONCLUSIONS: The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.
BACKGROUND: Postbypass refractory right ventricle (RRV) may develop due to right ventricular (RV) ischemia or infarction. In cases with RV infarction, recovery is often prolonged and salvage rate is extremely poor. In this retrospective study, we have examined the role of right ventricular exclusion (RVE), as a possible option to conventional weaning or bridging to heart transplant (B-HTX), in patients who were unsuitable for heart transplant. METHODS: During last 5 years, cumulative incidence of postbypass refractory circulatory failure (RCF) in our adult patients was 0.39% (26/6542). This problem was caused by a RRV in 17 (65%) patients. After CABG, these patients developed a grossly distended and poorly contracting RV (RVEDV: 330-400 ml, RVEF: 0-10%), high central venous pressure (> or =18 mmHg) and an inadequate aortic pressure for weaning off cardiopulmonary bypass. Three patients, who were unacceptable for HTX under UNOS program (age >65 years), were weaned off bypass after RVE, and remaining patients with RVAD (n=3) or BiVAD support, depending upon their concomitant moderate or poor left ventricular performance. RESULTS: The significant predictors of RRV by univariate analysis were; 2nd or 3rd redo CABG for a recent myocardial infarction, and failed graft angioplasty. Hospital mortality (14-60 days) was 0/3, 3/3 and 3/11 for the patients weaned off with RVE, RVAD and BiVAD respectively. At 3 years, overall salvage rate was 9/17 (RVE: 3/3, BiVAD B-HTX 4/6 + 2 weaned with BiVAD support). CONCLUSION: Right ventricular exclusion is a possible option to conventional B-HTX with mechanical support, in patients who develop postinfarct RRV and are unsuitable for transplant.
OBJECTIVE: The indications for the use of thrombolytic agents in massive pulmonary embolism (MPE) remain controversial and it has been suggested that transthoracic echocardiographic (TTE) examination, which is able to detect an associated right ventricular dysfunction, may cast light on this question. The goal of this study was to examine the incidence of acute cor pulmonale (ACP) in MPE, diagnosed on the basis of TTE criteria, its clinical implications and its resolution rate. DESIGN: Ten-year retrospective clinical study. SETTING: A medical and a coronary intensive care unit, university hospital. PATIENTS: One hundred sixty-one patients with proven MPE. INTERVENTIONS: Acute cor pulmonale was defined as right ventricular end-diastolic area / left ventricular end-diastolic area (RVEDA/LVEDA) ratio in the long axis greater than 0.6 associated with septal dyskinesia in the short axis. ACP patients were divided into three groups according to circulatory status: 32 patients without circulatory failure constituted group 1, 32 patients with circulatory failure requiring inotropic support, but free of metabolic acidosis, constituted group 2 and 34 patients in whom circulatory failure was associated with metabolic acidosis (defined by a base deficit >5 mEq/l) constituted group 3. RESULTS: Acute cor pulmonale was present in 61% of patients with MPE and carried a 23% mortality, but this mortality was very different in stable patients (groups 1 and 2, 64 patients, 3% mortality) and in unstable patients (group 3, 34 patients, 59% mortality). A multivariate logistic regression analysis showed that the TTE results were not predictive of the risk of death. Conversely, the same analysis showed that the presence of metabolic acidosis was a powerful predictor of death. CONCLUSION: Because none of the TTE measurements in ACP could be used to stratify the severity of MPE, TTE was of no help in deciding on medical thrombolysis. However, depending on its severity, metabolic acidosis could justify a large cooperative study to assess the impact of thrombolytic therapy on mortality rate in this specific group.
Recurrent pulmonary emboli ultimately may produce respiratory insufficiency, severe hypoxemia, and progressive pulmonary hypertension. In many patients this syndrome is silent in its initial phases, and when thrombophlebitis is present it is often unresponsive to anticoagulant therapy. Unless pulmonary embolectomy is undertaken, most of these patients characteristically succumb with severe respiratory insufficiency. Twenty-five patients with this syndrome have been evaluated at the Duke University Medical Center, and 14 were selected for elective pulmonary embolectomy for relief of severe and incapacitating pulmonary insufficiency. In each patient preoperative pulmonary scans and arteriography demonstrated a high degree of vascular occlusion. The obstructing lesions affected both lungs in the majority of patients. Bronchial arteriography was found to be a very valuable method for demonstrating patency of the pulmonary arteries distal to occluding lesions by retrograde filling through collateral vessels joining the bronchial and pulmonary circulations. Preoperatively radionuclide angiocardiography revealed severe right ventricular dysfunction with significantly depressed ejection fractions at rest and during exercise. Retrograde pulmonary arterial flow as shown by selective bronchial arteriography was excellent in ten patients, fair in three, and absent in one. Long term follow-up indicated a clear relationship between the magnitude of arterial backflow at the time of embolectomy and the degree of clinical improvement. There were two perioperative deaths, one from massive reperfusion pulmonary hemorrhage and another from intractable right ventricular failure. Eleven patients with this syndrome were unsuitable candidates for embolectomy and of these, nine had severe distal emboli diffusely spread in the small pulmonary arteries and not amenable to direct removal. One patient had severe right ventricular failure with extreme pulmonary hypertension (145/45 mmHg) and another was massively obese with severe congestive heart failure and expired in the hospital a week later. In this group of 11 patients, three succumbed and most of the others are currently totally debilitated at rest (NYHA Class IV). Long-term follow-up of the surgically managed patients (1 to 15 years) shows that ten patients improved from NYHA functional Class IV to either I or II, another patient from Class III to Class I, and a final patient was only minimally improved.(ABSTRACT TRUNCATED AT 400 WORDS)
OBJECTIVES: The purpose of this study was to verify in a long-term follow-up whether frequent monomorphic right ventricle extrasystoles may progress to arrhythmogenic right ventricular dysplasia (ARVD). BACKGROUND: Frequent monomorphic right ventricle extrasystoles are generally considered benign. However, in patients with this pattern, cardiac magnetic resonance (MR) has recently shown anatomical and functional abnormalities of the right ventricle. METHODS: Sixty-one patients who had been classified by noninvasive examinations as having frequent idiopathic right ventricle ectopy were contacted after 15 +/- 2 years (12 to 20) and submitted to clinical examination, electrocardiogram (ECG), Holter monitoring, stress test, signal averaged ECG, echocardiography and, in 11 patients, cardiac MR. The primary end point was to ascertain the presence of cases of sudden death or progression to ARVD. RESULTS: At the end of the follow-up, 55 patients were alive; six died, none of sudden death; eight stated to be well but refused further examinations. The 47 patients examined had normal ECG; in 24 patients (51%), extrasystoles were no longer present at Holter monitoring; late potentials were present in up to 15% of the patients; the right ventricle was normal at echocardiography. In 8 of 11 patients (73%), cardiac MR showed focal fatty replacement and other abnormalities of the right ventricle. CONCLUSIONS: In this long-term follow-up study, no patient died of sudden death nor developed ARVD; two-thirds of the patients were asymptomatic, and, in half of the patients, ectopy had disappeared. Focal fatty replacement in the right ventricle was present in most.
Explore the source record for details and available documents.
BACKGROUND: Many patients with congenital heart disease develop right ventricular (RV) failure due to anatomy and prior therapy. RV problems may include right bundle-branch block (RBBB), volume loading, and chamber enlargement. Because the failing RV may have regional dyskinesis, we hypothesized that resynchronization therapy might augment its performance. METHODS AND RESULTS: We studied 7 patients with RV dysfunction and RBBB, using a predefined pacing protocol. QRS duration, cardiac index (CI), and RV dP/dt were measured in 4 different pacing states. Atrioventricular pacing improved CI and RV dP/dtmax and decreased QRS duration as compared with atrial pacing or sinus rhythm. CONCLUSIONS: Atrioventricular pacing in patients with RBBB and RV dysfunction augments RV and systemic performance. RV resynchronization is a promising novel therapy for patients with RV failure.
We report the case of a 42-year-old female, affected by mitral valve prolapse and ventricular arrhythmias, who died suddenly from ventricular fibrillation recorded during Holter monitoring. The lethal arrhythmia initiated with late diastolic couplets followed by a ventricular tachycardia that eventually degenerated into ventricular fibrillation. The patient had experienced four orthostatic and stress-related syncopal episodes, associated with nausea and diaphoresis, and a positive tilt test. Holter monitoring documented ventricular arrhythmias, consisting of both isolated monomorphic and sporadic repetitive beats. Her standard ECG and exercise test were normal, but signal-averaged study findings were significantly positive. QT prolongation in the absence of arrhythmias was observed during the Valsalva manoeuvre and isoproterenol infusion. 2D echo showed a remarkable mitral valve prolapse without regurgitation and localized structural abnormalities of the right ventricle. Postmortem study confirmed mitral valve prolapse, and also disclosed pulmonary infundibulum dilatation, massive adipose infiltration of the right ventricular free wall, patchy fibrosis and scattered myocardial inflammatory infiltrates in the left ventricle; these features are all consistent with arrhythmogenic right ventricular cardiomyopathy.
BACKGROUND: Systemic right ventricular dysfunction is a major concern in the follow-up of patients who underwent an atrial redirection procedure for transposition (concordant atrio-ventricular and discordant ventriculo-arterial connections). No good non-invasive method is currently available for quantifying right ventricular function. AIMS: We have used ultrasonically based imaging of strain rate and strain to quantify regional deformation in the right ventricle after the Senning operation, comparing properties of regional deformation of the right ventricle with right ventricular ejection fraction as measured using magnetic resonance imaging. METHODS: In 20 asymptomatic patients who had undergone the Senning procedure, we measured peak systolic strain rate and systolic strain values in the right ventricular free wall, the septum and the left ventricular lateral wall using colour Doppler myocardial imaging, comparing the data with findings obtained in 30 healthy subjects. Global right ventricular ejection fraction was assessed using magnetic resonance imaging. RESULTS: Properties of deformation of the right ventricular free wall were reduced and homogeneous after the Senning procedure compared to normals, with significantly lower values for peak systolic strain rate and systolic strain (-1.1 +/- 0.4 vs. -2.5 +/- 0.9 s(-1); p < 0.05 and -16 +/- 7% vs. -38 +/- 13%; p < 0.05, respectively). There was a significant correlation between regional longitudinal right ventricular systolic strain values and right ventricular ejection fraction (r = -0.87, p < 0.001). In the septum, peak systolic strain rate was again reduced and homogeneous (-1.2 +/- 0.4 vs. -1.8 +/- 0.5 s(-1); p < 0.05 vs. normals). Also in the left ventricle, the lateral wall peak systolic strain rate and systolic strain values were reduced (-1.5 +/- 0.5 vs. -2.1 +/- 0.9 s(-1); p < 0.05 and -20 +/- 6% vs.-25 +/- 9%; p < 0.05, vs. normals, respectively). CONCLUSIONS: Properties of regional longitudinal deformation of the systemic right ventricle are reduced after the Senning procedure compared to normal controls, and correlate well with global right ventricular performance. These findings suggest that ultrasonic strain rate and strain imaging could be used in the non-invasive follow-up of ventricular function in these patients.
BACKGROUND: Right heart dysfunction is a major cause for early morbidity and mortality after heart transplantation. Experiments were designed to evaluate the influence of the calcium-desensitizing drug 2,3-butanedione 2-monoxime (BDM) on right heart function in a porcine model of heart transplantation. METHODS: Donor hearts of domestic pigs were arrested with BDM in Krebs solution (n = 7) and with BDM in Bretschneider's histidine-tryptophan-ketoglutarate (HTK) solution (n = 6). There were 2 control groups: University of Wisconsin (UW, n = 6) and HTK (n = 6). An isovolumic model was used in which the right ventricular volume was precisely controlled in vivo with an intracavitary high-compliance balloon. After 4 hours of ischemia, hearts were transplanted into recipients. After 1 and 2 hours of reperfusion, the right ventricular balloon volume was increased in 10-mL increments until right ventricular failure occurred and the developed pressures were recorded. RESULTS: Maximal right ventricular developed pressures were significantly different after 2 hours of reperfusion (UW: 35 +/- 13 mm Hg; HTK: 47 +/- 8 mm Hg; Krebs+BDM: 49 +/- 9 mm Hg; HTK+BDM: 50 +/- 6 mm Hg; P =.04). Hearts subjected to BDM could be loaded with a significantly increased volume after 1 hour and after 2 hours (UW: 57 +/- 10 mL vs HTK: 43 +/- 8 mL vs Krebs+BDM: 70 +/- 10 mL vs HTK+BDM: 67 +/- 15 mL; P =.002). Postischemic right ventricular enddiastolic compliance was significantly increased in groups treated with BDM after 1 hour (P =.02) and after 2 hours (P =.039). CONCLUSIONS: The drug BDM significantly improves right ventricular function in a heart transplantation model. The increase in volume load and developed right ventricular pressure achieved by BDM application would translate into a decreased risk of right ventricular failure after clinical transplantation.
Targeted deletion of actinin-associated LIM protein (ALP) in mice leads to right ventricular (RV) dysplasia and a mild RV cardiomyopathy. Although the phenotype has been thoroughly characterized, the mechanisms leading from the cytoskeletal defect to the disease are unclear. We hypothesized that ALP deficiency may be associated with (1) changes in regional systolic dysfunction and (2) regional dysregulation of hypertrophic growth, in accordance with the restricted expression of ALP in the outflow tract of the RV. We examined RV regional epicardial systolic strains with respect to end-diastole in ALP knockout (ALPKO) mice and wild-type controls using an open-chest preparation. Strain components were consistently lower in the ALPKO mice than wild-type controls (second principal strain E2: p = 0.05). RV pressure was slightly but not significantly lower in ALPKO mice as well. To assess regional growth, geometric remodeling was analyzed in ALPKO and wild-type mice after 4 weeks of chronic hypoxia (11% oxygen). The average amount of RV wall thickening in response to hypoxia was reduced to 11% in the ALPKO mice compared with 44% in the wild-type controls. In summary, the results are consistent with the view that disruption of ALP is associated with diminished RV contractile function as well as altered hypertrophic remodeling.
The Mustard operation in infancy and childhood has successfully palliated many patients with transposition of the great arteries who have now survived to adulthood. Right ventricular dysfunction and tricuspid regurgitation are important determinants of late morbidity and mortality. The value of noninvasive magnetic resonance imaging (MRI) in the assessment of cardiac function and anatomy 9 to 20 years after this procedure has been investigated, and compared with findings on echocardiography, radionuclide ventriculography and angiography in 17 adult patients. Ejection fractions measured by MRI were higher compared with radionuclide ventriculography. The correlation for the left ventricle was closer (r = 0.75) than for the right ventricle (r = 0.49). Tricuspid regurgitation was assessed by Doppler echocardiography and by MRI using the right/left ventricular stroke volume ratio. The mean stroke volume ratio in those with Doppler evidence of tricuspid regurgitation was 1.6:1 compared to 1.1:1 in those without, and this difference reached significance (p less than 0.01). The anatomy of the great arteries was clearly visible in all patients. Five patients had a residual ventricular septal defect which, with the exception of 1 small defect, was easily visualized. The intraatrial baffle was best seen in transverse slices, and the systemic venous connection showed as a relatively narrow channel lying in the posterior part of the cavity. In general, baffle anatomy was easier to assess on 2-dimensional echocardiography.(ABSTRACT TRUNCATED AT 250 WORDS)