Isolated right ventricular aneurysm following right ventricular infarction

A 78-year-old man with atherosclerotic heart disease developed extensive right ventricular infarction fibrosis with aneurysm formation following right coronary artery occlusion. No symptoms of right-sided heart failure were present. Postmortem examination revealed that 40% of the right ventricle, 11% of the septum and 7% of the left ventricular free wall were infarcted due to right coronary artery occlusion. This is the first documented case of isolated aneurysm of the right ventricle following infarction and it demonstrates that even extensive right ventricular destruction may be present without symptoms.     

Traumatic rupture of the right anterior sinus of Valsalva into the right ventricle combined with aortic regurgitation

We report the case of a 14-year old boy who was stabbed with a knife and sustained a chest wound responsible for clinical tamponade. Surgery was performed as an extreme emergency, so that lesions could not be assessed preoperatively. During the operation, beside the pericardial blood collection a wound of the pulmonary infundibulum was discovered and sutured (closed heart surgery). One year later the patient was readmitted for global heart failure. Auscultation of the heart revealed a continuous left latero-sternal murmur. Two-dimensional echocardiography showed discontinuity between the right anterior sinus of Valsalva and the right ventricular outflow tract. Contrast echocardiography displayed a negative jet image in the right ventricular outflow tract. At that level, pulsed doppler ultrasound recorded continuous turbulence as well as diastolic turbulence in the left ventricular outflow tract. Tiered oxymetry showed a left-to-right shunt in the right ventricle with a pulmonary/systemic flow ratio of 1.9. The diagnosis was confirmed by angiography which demonstrated a fistula between the right anterior sinus of Valsalva and the right ventricular outflow tract, and aortic regurgitation. In a second operation, performed under extracorporeal circulation, the traumatic lesions were repaired, and the patient thereafter recovered. This unusual case highlights the value of combined two-dimensional echocardiography, pulsed doppler ultrasound and contrast echocardiography in the diagnosis of traumatic ventriculo-aortic lesions.     

Usefulness of measuring right ventricular anterior wall thickness in patients with right ventricular overload using M-mode echocardiography

Right ventricular anterior wall thickness measured by M-mode echocardiography and right ventricular systolic pressure obtained by right heart catheterization were correlated in 62 patients with chronic right ventricular overload including congenital heart disease and primary pulmonary hypertension. The patients were divided into two groups; one, with right ventricular systolic pressures of 39 mmHg or less; the other, 40 mmHg or more. The following results were obtained. 1. The correlation coefficient for right ventricular anterior wall thickness and right ventricular systolic pressure was r = 0.90 (p less than 0.001), and the regression equation was y = 13.2x-1.3. 2. Right ventricular end-diastolic dimension increased significantly in both groups, but no statistically significant differences were detected between the two. Right ventricular anterior wall thickness increased significantly in the group with higher right ventricular pressures (7.1 +/- 0.5 mm vs 3.1 +/- 0.5 mm). 3. When right ventricular anterior wall thickness was more than 4.0 mm, pulmonary hypertension was detected, with a sensitivity of 97.5% and a specificity of 90.9%. In conclusion, measurements of right ventricular anterior wall thickness by M-mode echocardiography via the anterior chest wall proved to be potentially useful in predicting right ventricular systolic pressures in patients with chronic right ventricular overloads.     

A case of arrhythmogenic right ventricular cardiomyopathy

The present report describes a 40-year-old woman with a long history of monomorphic ventricular tachycardia and left bundle branch block. She was treated with various antiarrhythmic agents; ventricular tachycardia ablation was attempted and an automatic implantable cardioverter defibrillator was implanted. Three-dimensional echocardiography clearly demonstrated features of arrhythmogenic right ventricular cardiomyopathy, including marked right ventricular (RV) dilation, decreased RV systolic function and thinning of the RV free wall. Other RV morphological abnormalities included excessive trabeculations and a localized apical aneurysm. Two years later, the patient developed symptoms of congestive heart failure. Despite maximal medical therapy, her clinical condition continued to deteriorate and she was referred for heart transplantation. Results of the pathology of her explanted heart confirmed this rare diagnosis. She presented with an unusual clinical course for arrhythmogenic right ventricular cardiomyopathy, which was complicated by progressive congestive heart failure and ultimately required heart transplantation. Three-dimensional echocardiography identified the structural abnormalities related to this rare disease.     

Spontaneous resolution of severe right ventricular dysfunction in right ventricular infarction: documentation by radionuclide studies

A case of inferior myocardial infarction complicated by severe arrhythmias and right heart failure is presented. Radionuclide studies performed near the acute phase and one month later illustrate the reversibility of right heart dysfunction caused by infarction. Signs of right ventricular involvement in acute inferior myocardial infarction are noted in about 40% of cardiac blood-pool studies: right ventricular dilatation with a significantly decreased ejection fraction, and ventricular wall motion abnormalities. Follow-up studies in the recovery period show good recovery of right ventricular function.     

Evaluation of suspected right ventricular pathology in the athlete

Rigorous training remodels the heart of elite endurance athletes to produce the phenotype of the "athlete's heart." This remodeling, which advantages cardiac performance, creates challenges in the diagnosis of cardiac disorders within this population. This is particularly so for right ventricular pathologies because of the limited number of studies documenting the impact of training on right ventricular remodeling. Although arrhythmogenic right ventricular cardiomyopathy is the focus of this review, several other pathologies that may mimic arrhythmogenic right ventricular cardiomyopathy, including right ventricular outflow tract tachycardia, Wolff-Parkinson-White syndrome, Brugada syndrome, pulmonary embolism, cardiac sarcoidosis, myocarditis, and right ventricular infarction, are also included. In particular, the electrocardiographic findings for each condition are highlighted because this is the most informative and easily accessible diagnostic clinical tool.     

Extensive thrombus formation in the right ventricle due to a rare combination of arrhythmogenic right ventricular cardiomyopathy and heterozygous prothrombin gene mutation G20210 A

Arrhythmogenic right ventricular cardiomyopathy is a rare heart muscle disease characterized by right and often left ventricular myocardial atrophy and fibrofatty replacement. Heart failure, arrhythmias and sudden death are characteristic complications. We observed a female in whom arrhythmogenic right ventricular cardiomyopathy was diagnosed due to presyncopes and dyspnea on exertion. A left ventricular thrombus was found echocardiographically, which disappeared with oral anticoagulation. Subsequently, however, extensive thrombus formation in the dilated akinetic right ventricle occurred which was resistant to combined treatment with heparin and oral anticoagulation. Thrombophilia screening showed a mutant prothrombin 20210A allele which is an inherited coagulopathy associated with increased plasma levels of prothrombin and increased risks of mainly venous thrombosis. The patient developed endstage biventricular heart failure and underwent heart transplantation within 3 months after thrombus formation in the right ventricle was diagnosed. In the explanted heart, the thrombus in the right ventricle was impressively large and calcified. In patients with unusual thrombus formation in the heart, coagulopathy may be associated and should be excluded.     

Noninvasive recognition of the parchment right ventricle (Uhl's anomaly arrhythmogenic right ventricular dysplasia) syndrome

The case of a 53-year-old man with right heart failure, selective enlargement of the right-sided cardiac chambers, and recurrent sustained ventricular tachycardia is presented. Echocardiographic, radionuclide ventriculographic, and angiographic studies were typical of the right ventricular abnormalities in Uhl's anomaly; electrocardiographic and electrophysiologic findings were those of arrhythmogenic right ventricular dysplasia. Features of this unusual cause of cardiac failure and ventricular arrhythmias in the adult are reviewed, and compared to previous reports of both Uhl's anomaly and arrhythmogenic right ventricular dysplasia. We postulate that these two syndromes are manifestations of a single, presumably congenital, pathophysiologic process—the “parchment right ventricle” syndrome.     

Characterization of the human right ventricular pressure-volume relation: effect of dobutamine and right coronary artery stenosis

Right ventricular function was assessed in 15 patients using right ventricular pressure-volume loops. Right ventricular pressure using a micromanometer-tipped catheter, thermodilution cardiac output and gated blood pool scintigrams were simultaneously obtained. To help isolate the right ventricle, a slant hole collimator was used. The measurements were repeated during dobutamine infusion, which was titrated so there was minimal change in systemic pressure and heart rate. The right ventricular pressure-volume loop resembles the usual left ventricular loop except that the isovolumic contraction phase is often not as distinct, and right-sided ejection may continue well beyond right ventricular peak systolic pressure. Systolic but not diastolic function improved with dobutamine administration. There was no significant difference in right ventricular systolic function (ejection fraction, stroke work index, stroke volume index and cardiac index) or in end-diastolic volume index between patients without (Group I) and with (Group II) significant right coronary artery stenosis. However, there was a small but significant difference in right ventricular end-diastolic pressure (5.3 +/- 2.5 and 8.1 +/- 1.8 mm Hg [p less than 0.05]) for Group I and II, respectively. Thus, the right ventricular pressure-volume loop can be used to graphically display right ventricular function and improvement in contractility with dobutamine. The right ventricular isovolumic contraction phase and ejection phase differ from those in the usual left ventricular loop. Although there was a small difference in right ventricular end-diastolic pressure in patients with and without right coronary artery stenosis, the right ventricular pressure-volume loop did not provide additional discriminatory information between these two groups of patients.     

右心室流入道间隔部起搏血流动力学研究

目的本文旨在对右心室流入道间隔部起搏的血流动力学进行分析,以确立右心室流入道间隔部起搏的临床地位。方法本研究通过射频消融房室结建立Ⅲ°房室传导阻滞模型,结合影像学及心电图定位方法于右心室流入道间隔部置入螺旋电极导线,并分别比较右心室心尖部、右心室流出道及右心室流入道间隔部起搏后急性血流动力学指标变化,并随访右心室流入道间隔部起搏2周后的血流动力学指标。结果即刻血流动力学研究结果显示,右心室流入道间隔部较心尖部和右心室流出道起搏心排血量高(P<0.05),左心室舒张末期压力较低(P<0.05),而右心室流入道间隔部起搏前后各项血流动力学无显著变化。结论右心室流入道间隔部起搏具有良好的血流动力学效应,可作为右心室心尖部起搏的替代起搏部位。     

Pure right ventricular infarction

The case is reported of a patient with chronic emphysema andcor pulmonale who developed the signs of acute myocardial infarctionwhich were initially interpreted as being localized in the postero-inferiorwall of the left ventricle. The patient rapidly developed thefeatures of massive right heart failure in the absence of pulmonarycongestion, a condition thought to be due to pulmonary emboli.The autopsy, however, revealed a pure right ventricular infarct,which covered more than half of the pre-existent right ventricularmyocardium, in the setting of marked right ventricular hypertrophyinfraction. The case furthermore shows that the possibilityof pure right ventricular infarction should be evaluated inall patients with puzzling right heart failure, in the courseof what initially is thought to be an acute left ventricularinfraction, is important since therapy with plasma volume expandersshould be used with great caution in such patients     

Hemodynamic correlates of right ventricular ejection fraction measured with gated radionuclide angiography

Right ventricular function was studied in 60 patients with equilibrium gated radionuclide angiography. The mean (± standard deviation) right ventricular ejection fraction in 20 normal subjects was 53 ± 6 percent, a value in agreement with previous data from both radionuclide and contrast angiographie studies. This value was similar (55 ± 7 percent) in 11 patients with coronary artery disease but normal left ventricular function.Radionuclide measurements of right ventricular ejection fraction were correlated with right heart hemodynamics. There was a significant negative linear correlation between right ventricular ejection fraction and mean pulmonary arterial pressure (r = ?0.82) and between right ventricular ejection fraction and right ventricular end-diastolic pressure (r = ?0.67). Furthermore, patients with elevated right ventricular enddiastolic pressure and mean pulmonary arterial pressure had a more severely depressed ejection fraction than did those with an elevated mean pulmonary arterial pressure alone.Thus, an abnormal value for right ventricular ejection fraction by gated radionuclide angiography in the absence of primary right ventricular volume overload suggests abnormal right heart pressures, whereas a normal value excludes severe pulmonary arterial hypertension or an elevated right ventricular end-diastolic pressure.     

Performance of the failing and nonfailing right ventricle of patients with pulmonary hypertension.

Hemodynamic performance of the right ventricle was measured in 34 patients: 17 with pulmonary hypertension, 9 with pulmonary hypertension and right ventricular failure and 8 control subjects. Among the patients with pulmonary hypertension who did not have right ventricular failure, right ventricular maximal isovolumic rate of development of ventricular pressure (dP/dt) was significantly elevated (P less than 0.001), whereas maximal 1/P dP/dt and maximal velocity of contractile element shortening (Vmax) were comparable with values observed in control subjects. The patients with pulmonary hypertension who had right ventricular failure also showed an augmented right ventricular maximal dP/dt (P less than 0.001) and normal 1/P dP/dt and Vmax. These observations indicate that in pulmonary hypertensive heart disease, even when the right ventricle failed in a clinical sense, the contractile effort was normal. Consequently, right ventricular failure may develop in patients with pulmonary hypertensive heart disease even though the cardiac muscle performs normally as a contractile tissue.     

Mechanism of pressure-overload right ventricular hypertrophy in infant rabbits

Although pressure-overload right ventricular hypertrophy is a long-term risk in some congenital heart diseases such as tetralogy of Fallot, how it develops is unclear. The aim of this study was to investigate the mechanism of development of this right ventricular heart failure.Pulmonary artery banding in 10-day-old rabbits induced pressure-overload right ventricular hypertrophy as they grew. Comparisons were made with age-matched sham controls (n = 24 per group). In weekly serial echocardiography, the right ventricular contraction and diastolic function decreased from 3 weeks after surgery (P < 0.01), and the right ventricle became hypertrophic from 4 weeks after (P < 0.05). Pressure-overload increased cardiomyocyte apoptosis from 4 weeks postoperatively (TUNEL staining and Western blotting analysis, P < 0.05); and fibrosis occurred in the right ventricular cardiomyocytes at 8 weeks after operation (Masson's trichrome stain, P < 0.01). In our model, pressure-overload to the right ventricle caused the right ventricular disorder, hypertrophy, and fibrosis. Apoptosis of right ventricular cardiomyocytes was involved in progression. We have shown for the first time the mechanism whereby pressure-overload right ventricular hypertrophy develops in an infant rabbit model.     

Clinical use of ultrashort-lived radionuclide krypton-81m for noninvasive analysis of right ventricular performance in normal subjects and patients with right ventricular dysfunction

The ultrashort-lived radionuclide krypton-81m, eluted in 5% dextrose from a bedside rubidium-81m generator, was intravenously infused for rapid imaging of the right-sided heart chambers in the right anterior oblique projection adjusted for optimal right atrioventricular separation. Left-sided heart and lung background was minimized by rapid decay and efficient exhalation of krypton-81m, requiring no algorithm for background correction. A double region of interest method decreased the variability in the assessment of ejection fraction to 5%. In 10 normal subjects, 11 patients with pulmonary hypertension, 4 patients with right ventricular outflow tract obstruction and 4 patients with right ventricular infarction, right ventricular ejection fraction determined by krypton-81m equilibrium blood pool imaging ranged from 14 to 76%. The correlation between these values and those determined by cineangiography according to Simpson's rule was close: r = 0.93 for all data points (p less than 0.001), r = 0.92 for studies at rest (p less than 0.001) and r = 0.93 for exercise studies (p less than 0.001). Exercise-related changes in right ventricular function revealed a disturbed functional reserve with pulmonary hypertension and right ventricular infarction, whereas in compensated right ventricular outflow tract obstruction there was a physiologic increase in ejection fraction with exercise (p less than 0.001). Thus, equilibrium-gated right ventricular imaging using ultrashort-lived krypton-81m is a simple, accurate and reproducible method with potential for serial assessment of right ventricular ejection fraction in a variety of right ventricular anatomic and functional abnormalities, both at rest and during exercise. Advantages of this method include an extremely low radiation dose to patients and clear right atrioventricular separation without the need to correct for background activity.     

Two autopsied cases of arrhythmogenic right ventricular dysplasia

Autopsy studies of arrhythmogenic right ventricular dysplasia (ARVD) have rarely been reported, and its etiology remains unknown. The present report describes a detailed histopathological study of two autopsied cases of ARVD. Case 1: This 21-year-old man experienced palpitation accompanied by syncope. He died after ventricular tachycardia of right ventricular origin. The heart weighed 365 g and the right ventricular cavity was markedly dilated. The distribution of fatty tissue was roughly limited to the middle layer of the free wall, replacing the myocardium with fatty degeneration. Medial hyperplasia of the small arteries within the fatty tissue was also observed. Fibroelastosis was observed in the left ventricular endocardium. In the conduction system, fatty tissue was found in the sinus node. In addition, medial hypoplasia was observed in the pulmonary arteries. Case 2: This 32-year-old man who had had an arrhythmia for 10 years died of ventricular tachycardia of right ventricular origin. His older brother also died of heart disease. His heart weighed 515 g and both the right and left ventricles were dilated. Fatty tissue, unlike that in Case 1, was shown to markedly infiltrate from the epicardium into both the right and left ventricular walls. In the right ventricular wall, muscle layers disappeared in some portions. In the conduction system, fatty tissue was observed in the sinus node. Although ARVD may be considered a syndromic entity, individual cases are different in terms of pathological morphology, with possible variations in the etiology and pathogenesis.     

Two-dimensional echocardiographic assessment of the right ventricle in patients with chronic obstructive lung disease

A consecutive series of 40 patients (39 men, one woman; mean age 54.1 +/- 8.2 years) with chronic obstructive lung disease (COLD) underwent right heart catheterization and 2-D echocardiographic examination within 72 hr. An adequate 2-D echocardiographic study was obtained in 32 patients (80 percent) using the apical and/or subcostal views. Mean pulmonary artery pressure at rest and during exercise were correlated with right ventricular diameters and areas in end-systole and end-diastole (r = 0.63 to 0.73). Correlations between right ventricular free wall thickness and pulmonary artery pressure were weak (r = 0.51 and 0.57). Right ventricular end-diastolic pressure was also weakly correlated with right ventricular dimensions (r = 0.45 to 0.51), whereas right ventricular area fractional shortening was not correlated with hemodynamic parameters. Patients with previous episode of right heart failure had larger right ventricles than those without such episodes. Thus, 2-D echocardiography appears useful to study the right heart chambers in patients with COLD.     

Initial report of percutaneous right ventricular assist for right ventricular shock secondary to right ventricular infarction.

A 57-year-old female suffered an acute inferior ST segment elevation myocardial infarction. The patient failed thrombolysis and was urgently transferred for rescue percutaneous coronary intervention of the right coronary artery. She decompensated after reperfusion of the occluded RCA and developed cardiogenic shock from severe right heart failure refractory to IABP support and maximal pressors. A percutaneous right ventricular assist device was successfully implanted, which improved mean arterial pressure to a viable range and allowed withdrawal of inotropic medications.Right ventricular failure after infarction remains difficult to manage and has a high mortality. Intraaortic balloon pump and LVAD support have not proven beneficial in cardiogenic shock secondary to RV infarction. This is a report of the first insertion of a percutaneous right ventricular assist device for right ventricular support in a human. Further evaluation is warranted to evaluate the potential benefits of such a device as well as optimal timing of initiation of RV support.