Echocardiographic features of free floating thrombus mimicking right ventricular myxoma.

M-mode and cross-sectional echocardiography performed in a patient with acute pulmonary embolism showed a sausage shaped, mobile mass in the right ventricular cavity highly suggestive of a right ventricular myxoma. Emergency thoracotomy 24 hours later showed the right ventricle to be free of tumour but both pulmonary arteries contained embolised venous thrombi, one or more of which were thought to have given rise to the false echocardiographic diagnosis of a right ventricular tumour.     

Large thymic carcinoma presenting with right ventricular failure: a case report

Thymic carcinoma is an overall rare tumour with variable clinical manifestations. Right ventricular failure remains an uncommon occurrence and has not been reported in literature so far. A 40-year-old lady presented with the complaints of progressively worsening retrosternal chest pain, shortness of breath, easy fatigability and cough since 1 year. Computed tomography scan of the thorax revealed a mass measuring 12 x 10 cm in the anterior mediastinum. This mass appeared to be adherent to both lungs and pericardium and was impinging on the right atrium and right ventricle. It appeared to be infiltrating the ascending aorta, pulmonary arteries and superior vena cava. Ultrasound of the abdomen showed hepatomegaly and moderate ascites. Echocardiography showed evidence of right ventricular dysfunction as well as elevated right ventricular systolic pressures secondary to extrinsic compression. Percutaneous biopsy of the thymus was performed showing a malignant thymoma. Radical thymectomy with resection of pericardium was planned. Intra-operatively, the tumour was separated from the right and left lungs, pulmonary artery and aortic arch. Morphologically, immunochemically and clinically, the features were consistent with those seen in Masoka stage III thymic carcinoma. She also received six cycles of chemotherapy (PAC regimen) including cisplatin (50 mg/m2), doxorubicin (50 mg/m2) and cyclophosphamide (500 mg/m2). Radiation therapy in the adjuvant setting was planned but the patient was lost to follow-up after 4 months. Although right ventricular failure is a very rare presentation of thymic carcinoma, clinicians should be aware of this presentation to appreciate the complete clinical spectrum of presentation of this neoplasm.     

Right ventricular fibroma. Value of different methods of invasive and noninvasive exploration

The authors report the case of a 20 year old man with a primary cardiac tumour. The relative usefulness of invasive (catheterisation and angiography), and non-invasive investigations (echocardiography, computerised axial tomography, myocardial scintigraphy and digitalised angiography) in determining operability and the benign or malignant nature of the tumour was evaluated. The patient was admitted to hospital for severe incapacitating effort dyspnoea. Cardiac auscultation was suggestive of pulmonary stenosis associated with tricuspid regurgitation. M mode and 2D echocardiography demonstrated a large mass within the right ventricular cavity and also its size shape, mobility and its relationship to the interventricular septum, tricuspid valve and the main pulmonary artery. Echo contrast studies confirmed tricuspid regurgitation and also demonstrated a patent foramen ovale. The cardiac CAT scan confirmed the preceding data. Myocardial scintigraphy demonstrated the vascular character of the tumour. Digitalised angiography showed the presence of a tumour in the right ventricle and the rest of the morphological information was identical to that obtained by conventional angiography. Cardiac catheterisation demonstrated an obstruction to right ventricular ejection and abnormal filling of both ventricles. It was the association of 2D echocardiography and Technetium 99 myocardial scintigraphy which provided the most information. The results of the other investigations were not essential in deciding the operative indications. A 230 g tumour was excised at surgery and the tricuspid valve replaced by a Hancock n 33 bioprosthesis. Anatomopathological examination showed the tumour to be a fibroma. The finding of a tricuspid diastolic rumble led to control catheter and angiographic studies 13 days after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary right ventricular osteosarcoma

A 41-year-old man suffering from dyspnea on exertion was found to have a mass located on the right ventricle. Resection of the tumour required reconstruction of the right ventricular outflow tract and replacement of the pulmonary valve. Histopathology confirmed the diagnosis of high-grade osteosarcoma.     

Echocardiographic features of right ventricular outflow tumor prolapsing into the pulmonary artery

The echocardiographic features of a tumor in the right ventricular outflow tract that prolapsed into the pulmonary artery during systole are described. The patient was a 19 year old woman who presented clinically with bacterial endocarditis involving a mildly stenotic pulmonary valve. An echocardiogram, obtained to evaluate the pulmonary valve for bacterial vegetations, showed abnormal echoes throughout the cardiac cycle; they suggested a tumor mass in the right ventricular outflow tract in front of the pulmonary valve with possible extension into the pulmonary artery during systole. Additional tumor echoes confined to diastole were recorded in front of the aortic root and the tricuspid valve. These features were further elucidated with a computer-generated two dimensional cineechocardiogram that clearly showed a portion of the tumor mass passing beyond the position of the pulmonary valve into the main pulmonary artery in systole and returning into the right ventricular outflow in diastole. At surgery, a large myxoma was found in the right ventricular outflow tract with a polypoid extension that projected into the pulmonary artery in systole and contained a fibrinous vegetation at its tip. The left cusp of the pulmonary valve was normal, but the other two leaflets showed evidence of endocarditis.     

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.     

Investigation of the relation of impedance rheopneumogram with pulmonary hemodynamics and right ventricular function in patients with chronic obstructive pulmonary disease

In order to investigate the relation of impedance rheopneumogram with pulmonary hemodynamics and right ventricular function in patients with chronic obstructive pulmonary disease (COPD), we measured impedance rheopneumogram and did right heart catheterization in 150 COPD patients simultaneously both at rest and after exercise. The results showed that impedance rheopneumogram was mainly influenced by right ventricular after-load, while the influences of right ventricular pre-load, right ventricular contraction, right ventricular stroke work index and cardiac output were rather small, suggesting that in COPD patients impedance rheopneumogram is valuable in predicting pulmonary arterial pressure noninvasively, but not so use in judging the right ventricular function.     

先心病患者脉冲多普勒肺动脉血流频谱时间间期的影响因素分析

对２８例先心病患者进行了脉冲多普勒超声心动图，右心导管及右室造影检查，探讨了影响脉冲多普勒肺动脉血流频谱时间间期的因素．结果表明，右室射血前期．加速时间，加速时间／右室射血前期及加速时间／右室射血期主要受右室后负荷的影响；右室射血期受右室后负荷及每搏量影响；右室射血前期／右室射血期不仅受右室后负荷影响，而且受右室收缩功能状态的影响．     

Arrhythmogenic right ventricular cardiomyopathy with pulmonary embolism: a case report

A 50-year-old man complained of dyspnea on exertion. Electrocardiography showed frequent premature ventricular contraction and inverted T wave in leads V1-V6. Echocardiography and right ventriculography showed dilation and systolic dysfunction of the right ventricle. The diagnosis was arrhythmogenic right ventricular cardiomyopathy. In addition, thrombus was also observed in the pulmonary artery on chest computed tomography and pulmonary artery angiography. The final diagnosis was arrhythmogenic right ventricular cardiomyopathy complicated by pulmonary embolism. Pulmonary embolism should be considered in patients with arrhythmogenic right ventricular cardiomyopathy with dyspnea.     

Importance of right ventricular outflow tract angiography in distinguishing critical pulmonary stenosis from pulmonary atresia.

OBJECTIVE: To investigate the spectrum of pulmonary atresia and critical pulmonary stenosis using right ventricular outflow tract angiography and explore its implications for catheter interventional treatment. DESIGN: Prospective clinical study. SETTING: Two paediatric cardiology centres. SUBJECTS: 11 neonates or infants (aged 1 day to 8 months; weighing 2.3 to 7.8 kg) with pulmonary atresia or where the differentiation of pulmonary atresia from critical pulmonary stenosis was unclear on either echocardiography or angiography. METHODS: Right ventricular outflow tract angiography was performed on all patients to distinguish pulmonary atresia from critical pulmonary stenosis before opening the right ventricular outflow tract. RESULTS: Right ventricular outflow tract angiography showed that three of seven patients diagnosed as pulmonary atresia by echocardiography had pin hole jets across the pulmonary valve; another had a probe patent valve that appeared imperforate on both echocardiography and right ventricular outflow tract angiography. Three of the four patients diagnosed by echocardiography as critical pulmonary stenosis were found on right ventricular outflow tract angiography to have pulmonary atresia. The remaining patient had such a tiny orifice that a second orifice had to be created with a radiofrequency catheter. The right ventricular outflow tract was opened successfully in 10 of the 11 patients, six of whom required application of radiofrequency energy. The right ventricular to aortic systolic pressure ratio fell from 1.4 (0.9 to 1.9) to 0.6 (0.2 to 1.1) (P < 0.05). All 11 patients were alive and well with transcutaneous oxygen saturations ranging from 84% to 95% at a median follow up duration of nine months. CONCLUSIONS: Critical pulmonary stenosis and pulmonary atresia cannot always be accurately distinguished by echocardiography. Right ventricular outflow tract angiography helps to distinguish the two groups. In most cases the right ventricular outflow tract can be opened without mortality and with short to medium term survival.     

Comparison of the Tei index with invasive measurements of right ventricular function

The Doppler-derived Tei index has been reported to be clinically useful in assessing global right ventricular function. It could increase in response to combinations of increased pulmonary artery pressure and/or ventricular dysfunction. We compared the Tei index with invasive measurements of right ventricular function during acute pulmonary hypertension. Right and left ventricular pressures, pulmonary and aortic pressures, pulmonary flow and right ventricular volume by sonomicrometry were measured in six anaesthetized sheep. Graded pulmonary arterial hypertension was induced by a mechanical occlusion maneuver. Pressure-volume loops were generated during preload reduction through caval occlusion. Epicardial echocardiograms were also performed. Invasive indexes including preload recruitable stroke work, ventricular diastolic time constant and stiffness constant, and cardiac output were assessed, as were noninvasive echocardiographic indexes including Tei index and E/A ratio. The right ventricular pressure-volume loop became rectangular, with well-defined isovolumic phases. The slope of preload recruitable stroke work was increased significantly during pulmonary pressure of 30 mm Hg. The ventricular time constant showed a significant increase with no change of chamber stiffness during pulmonary pressure of 35 mm Hg. Concomitantly, the Tei index increased significantly from 0.06+/-0.03 to 0.31+/-0.06, together with the shortening of the ejection time and a decrease of the E/A ratio (P<0.05). Thus, the right ventricular Tei index was noted to be affected by acute graded afterload increase. The alteration in invasive measurements of systolic and diastolic function makes the Tei index a sensitive indicator of right ventricular dysfunction in the settings of acute pulmonary hypertension.     

Leiomyosarcoma of the right ventricle. Report of a case and review of the literature]

Primary cardiac leiomyosarcoma is a very rare condition. Only 15 cases have been reported in the literature. We report the case of a 27 year old man admitted to hospital for chest pain in March 1985. Echocardiography showed a right ventricular tumour which was completely resected at surgery. The diagnosis of leiomyosarcoma was confirmed by histological examination. After 22 months follow-up, the patient was still alive despite pulmonary metastases. The diagnosis of cardiac tumour used to be made post-mortem but since the introduction of new methods of investigation, especially 2D echocardiography, the diagnosis can be made at an early stage and allows rapid surgical resection, the only means of obtaining the histological diagnosis and of completely curing benign tumours.     

Right ventricular dysfunction and the exercise limitation of chronic obstructive pulmonary disease

This study examined right ventricular function during exercise in patients with chronic obstructive pulmonary disease to answer the following questions: Is there a significant correlation between oxygen consumption at maximal exercise and exercise right ventricular ejection fraction? Does the right ventricular ejection fraction response to exercise correlate with exercise changes in pulmonary artery pressure, total pulmonary resistance or pulmonary vascular resistance? Which combinations of cardiac, ventilatory and blood gas variables are the best predictors of oxygen consumption at maximal exercise? Twenty-six patients with stable chronic obstructive pulmonary disease performed symptom-limited supine bicycle exercise with simultaneous hemodynamic and radionuclide ventriculographic measurements. The oxygen consumption at maximal exercise correlated with the exercise right ventricular ejection fraction (n = 21, r = 0.66; p less than 0.005), exercise stroke volume (r = 0.68; p less than 0.001), exercise cardiac output (r = 0.77; p less than 0.00005) and exercise ventilation (r = 0.85; p less than 0.00001). The change in right ventricular ejection fraction from rest to exercise correlated inversely with the change from rest to exercise in total pulmonary resistance (r = -0.51; p less than 0.05) but not with the change in mean pulmonary pressure (r = -0.37) or in pulmonary vascular resistance (r = 0.09). Multivariate analysis showed that the variables giving the highest combined correlation with oxygen consumption were ventilation and right ventricular ejection fraction (r = 0.95, adjusted r2 = 0.88). These results suggest that exercise oxygen consumption of patients with chronic obstructive pulmonary disease is related to right ventricular systolic function, exercise right ventricular dysfunction is related, in part, to abnormal exercise total pulmonary resistance, and exercise limitation in chronic obstructive pulmonary disease occurs as a result of the dynamic interaction between disordered right heart function and ventilation.     

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.     

Natural history of supravalvular aortic stenosis and pulmonary artery stenosis

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.     

Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report

A rare case of pulmonary hypertension due to glycogen storage disease type II (Pompe's disease) was reported. An 18-year-old girl was admitted to Kawasaki Medical School Hospital because of cyanosis, dyspnea on exertion and amenorrhea. She was 149 cm in height and 29 kg in body weight. Clinical examination revealed that pulmonary artery pulse and right ventricular heave were palpable over the precordium. On auscultation, an accentuated pulmonic second heart sound, pulmonic ejection sound and diastolic decrescendo murmur (Levine III/VI) were heard in the second intercostal space at the right sternal border. Her skeletal muscles, especially her intercostal muscles were generally weak and atrophic. Her electrocardiogram showed a pulmonary P-wave and right ventricular hypertrophy. The chest X-ray revealed right ventricular enlargement and a dilated pulmonary trunk. On echocardiography, the right ventricle and the main pulmonary artery were dilated, and a systolic notch of the pulmonary valve was found. Swan-Ganz catheterization disclosed that pulmonary artery pressure, right ventricular pressure and mean pulmonary capillary wedge pressure were 76/35 (50) mmHg, 76/12 mmHg and 10 mmHg, respectively. Respiratory function tests showed severe restrictive ventilatory impairment with hypercapnea and hypoxemia. On biopsy of the left quadriceps femoris muscle, the most striking finding was numerous intracytoplasmic vacuoles. The small vacuoles were stained with PAS and acid phosphatase. Electron microscopy showed massive glycogen accumulation in the sarcoplasm and membrane bound vacuoles (glycogenosome). Alpha-1, 4-glucosidase activity in the peripheral lymphocytes was definitely decreased. Her pulmonary hypertension resulted from respiratory muscular atrophy and alveolar hypoventilation caused by Pompe's disease.     

ILL型右室双出口(附一例报告)

<正> 全内脏转位合并镜面右位心是心脏原发位置异常,多无其他心脏畸形,但在先天性心脏畸形中有全内脏转位的发生率可达0.8%。最常见的合并畸形是纠正型大动脉转位。右室双出口是大动脉转位的一种,即主动脉及肺动脉均发自右心室,较为少见。全内脏转位、镜面右位心合并右心室双出口更为少见,本院曾收治一例,介绍如下: 临床资料王×(住院号21821)女、13岁,自幼发现心脏杂音,经检查为室间隔缺损、肺动脉高压,合并内脏转位、右位心,转来本院。患儿生后易患感冒、肺炎。近两年来有劳累后心     

Right-sided endomyocardial fibrosis with recurrent pulmonary emboli leading to irreversible pulmonary hypertension.

A 26 year old Saudi man with features of both Loeffer's endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli. Echocardiographic examination showed obliteration of the right ventricular apex by an attached mass. The results of haemodynamic studies were somewhat abnormal and medical treatment was started. Despite anticoagulation with warfarin the patient's condition deteriorated rapidly over a four month period after a further episode of pulmonary embolism and the development of pulmonary hypertension. Two haemodynamic studies performed four months apart were typical of pulmonary hypertension and later right ventricular failure; they showed none of the characteristics of restriction. Pulmonary embolectomy was attempted but there was no cleavage plane between the organised thrombi and the endothelium of the pulmonary artery. The patient died of severe pulmonary hypertension and right ventricular failure several days after operation. Surgical intervention in the early stages of right-sided endomyocardial fibrosis might have prevented the development of pulmonary embolism and pulmonary hypertension.     

Late Presentation of Pulmonary Valve Stenosis Confirmed by Cardiovascular Magnetic Resonance

We describe the case of a 70‐year‐old man who presented with increasing exertional dyspnea. He was found to have an ejection systolic murmur and evidence of right ventricular outflow tract obstruction, with a peak velocity of 4.5 m/s recorded by transthoracic Doppler echocardiography. Cardiovascular magnetic resonance showed right ventricular hypertrophy, pulmonary valve stenosis, peak recorded velocity 4.2 m/s, with thickened pulmonary valve leaflets of reduced mobility, and poststenotic dilatation of the main pulmonary artery. The case illustrates that severe pulmonary valve stenosis can present late in life and that cardiovascular magnetic resonance can be useful in clarifying nature and level of right ventricular outflow tract obstruction in an adult.     

Myxoma of the right ventricle. Apropos of 2 cases

Two cases of primary cardiac tumour are reported. The first was that of a 16 year old girl investigated after the detection of a systolic murmur on routine examination. The clinical signs suggested a diagnosis of pulmonary stenosis. The second case was that of a 36 year old man admitted to hospital for right ventricular failure. Echocardiography revealed right ventricular tumours in both cases and also indicated their size, form, mobility and site of implantation. The outcome of the first case, a fibromyxoma, was favourable with no detectable recurrence after one year. In the second case, the patient unfortunately died of cardiogenic shock in the immediate preoperative period. These two cases emphasise the value of echocardiography in the diagnosis of isolated right ventricular tumours, especially considering the difficulty of clinical diagnosis and the risks of angiocardiography.