Synovial sarcoma in the right atrium and right ventricle

A metastatic synovial sarcoma in the right atrium and ventricle is described. A 36-year-old man was admitted to our hospital with generalized fatigue, dyspnoea, and precordial pain. Transthoracic echocardiography demonstrated a metastatic tumour in both the right atrium and right ventricle and revealed obstruction of the inflow tract of the right ventricle caused by a metastatic right atrial tumour. Thoracic computed tomography revealed a pleural-based paravertebral mass in the left intrathoracic cavity and multiple pulmonary nodules in both lungs. Cardiac surgery was performed for palliative treatment due to right cardiac failure and a risk of fatal embolization. The patient died 12 months after the cardiac surgery.     

Rupture of right coronary artery aneurysm into the right atrium

A 63-year-old man presented with acute congestive heart failure and was found to have a continuous murmur. Two years earlier, he had an inferior myocardial infarct, when no murmurs were heard. Angiography showed a right coronary artery aneurysm communicating with the right atrium. The distal vessel was occluded. The aneurysm was resected and the patient remains well. It is proposed that this was a congenital aneurysm which led firstly to the myocardial infarct and finally ruptured into the right atrium.     

Predominant right ventricular dysfunction after right ventricular destruction in the dog

Right and left ventricular function was assessed by observing the response to rapid blood volume expansion before and after extensive cauterization of the right ventricle in open chest dogs. In the control period, left ventricular end-diastolic pressure surpassed right ventricular end-diastolic pressure by an average of 11.5 mm Hg after volume expansion whereas, after destruction of the right ventricle, pressure in this chamber surpassed pressure in the left ventricle by an average of 4 mm Hg. In contrast, after left ventricular damage, left ventricular end-diastolic pressure averaged 25 mm Hg more than right ventricular end-diastolic pressure. Despite extensive damage to the right ventricular free wall, the right ventricle continued to generate a near normal pressure and aortic flow could be increased above control levels by volume expansion. We conclude that, in contrast to previous evidence, damage to the right ventricle produces a syndrome of predominant right ventricular dysfunction. However, cauterization of the entire free wall of the right ventricle does not reproduce the more profound right ventricular dysfunction noted in infarction of the right ventricle in man.     

Hemangio-endotheliosarcoma of the right atrium

In reporting a case of haemangiosarcoma of the right atrium the authors emphasize the problems encountered in the diagnosis of primary tumours of the heart. The patient was a 50-year old woman who complained of digestive disorders and palpitations. Physical examination showed signs of right heart failure associated with a systolic murmur on the right side of the sternum. ECG gave normal results. Echocardiography displayed a pouch with liquid ultrastructure communicating with the right atrium. Angiography confirmed the presence of a cavity with irregular borders communicating with the right atrium. Coronary arteriography showed an abnormal disorderly distribution of the right coronary artery branches with newly formed vessels extending toward the tumour. At exploratory thoracotomy a large liquid tumour was found which bled at the slightest touch and was attached to the mediastinum and the right pericardium. The pleura and the lung contained several nodules of the same venous colour as the mother tumour. Extemporaneous biopsy and pathological analysis were in favour of a haemangiosarcoma. The patient died a few days after the thoracotomy.     

Rupture of right coronary artery aneurysm into the right atrium.

A 63-year-old man presented with acute congestive heart failure and was found to have a continuous murmur. Two years earlier, he had an inferior myocardial infarct, when no murmurs were heard. Angiography showed a right coronary artery aneurysm communicating with the right atrium. The distal vessel was occluded. The aneurysm was resected and the patient remains well. It is proposed that this was a congenital aneurysm which led firstly to the myocardial infarct and finally ruptured into the right atrium.     

Spontaneously acquired fistula from the right coronary artery to the right ventricular cavity.

A 47 year old man developed a fistula from the right ventricular branch of the right coronary artery to the right ventricular cavity in association with distal occlusion of the main trunk of the right coronary artery. There was no clinical or electrocardiographic evidence of acute myocardial infarction.     

Spontaneously acquired fistula from the right coronary artery to the right ventricular cavity.

A 47 year old man developed a fistula from the right ventricular branch of the right coronary artery to the right ventricular cavity in association with distal occlusion of the main trunk of the right coronary artery. There was no clinical or electrocardiographic evidence of acute myocardial infarction.     

Analysis of right atrium function in patients with chronic pressure overload of the right ventricle

Right atrial function was evaluated in 16 patients with and without chronic right ventricular pressure overload. A simultaneous right atrial pressure recording using a catheter-tip-micromanometer and right atrial volume determination using cross-sectional echocardiography were performed. The pressure-volume curve of the right atrium was composed of an a-loop and a v-loop. The ratio of active atrial emptying to right ventricular stroke volume in patients with right ventricular pressure overload was significantly larger than in the control group (36 +/- 6% vs. 23 +/- 5%, p less than 0.04). The right atrial work was also significantly greater in patients with right ventricular pressure overload (6.2 +/- 2.0 mWs) than in normal subjects (4.2 +/- 2.0 mWs, p less than 0.04). The ratio of active atrial emptying to ventricular stroke volume and right atrial work were significantly related in both control group and patients with right ventricular pressure overload (r = 0.83). Right atrial work also showed a significant linear correlation with right atrial work before active atrial emptying (r = 0.92). We conclude that in patients with right ventricular pressure overload the right atrium shows more pronounced active emptying and contributes to better diastolic filling of the right ventricle.     

Transcatheter closure of right coronary artery fistula to the right ventricle

Coronary artery fistula (CAF) is an uncommon anomaly that is usually congenital but can be acquired. Although most patients are asymptomatic, some may present with congestive heart failure, infective endocarditis, myocardial ischemia or rupture. In the past, surgical ligation was the only option in the management of CAF, but since 1983, transcatheter closure of CAF has been increasing as an alternative to surgery. We report a 3-year-old boy, presented in Queen Alia Heart Institute, who underwent successful transcatheter closure of a large fistula communicating the distal part of the right coronary artery to the right ventricle. Our case differs from other CAFs in that the fistula was communicating the right coronary artery itself to the right ventricle.     

Total disconnection of the right ventricular free wall: surgical treatment of right ventricular tachycardia associated with right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia is a myopathy that affects the right ventricular free wall (RVFW) and gives rise to recurrent reentrant ventricular tachycardia (VT). Because the entire right ventricle is potentially arrhythmogenic, ablating a single site of VT may not eliminate the arrhythmia. We developed an operation to confine any arrhythmic activity arising from the right ventricle to that chamber: total disconnection of the RVFW from the left ventricle. We performed RVFW disconnection in two patients with refractory VT associated with arrhythmogenic right ventricular dysplasia. At least two sites or origin of morphologically distinct VT were identified in the RVFW in each patient. RVFW disconnection was carried out under normothermic cardiopulmonary bypass. An encircling incision was made along the attachment of the RVFW to the aortoventricular unit and the tricuspid annulus; the right coronary artery and its RVFW branches were left intact. Electrical activity of the two chambers became dissociated, and VT arising from the RVFW was confined to that chamber. Postoperatively, there was no clinical evidence of hemodynamic impairment (follow-up 4 months and 3 months). Left ventricular function was unchanged and right ventricular flow was maintained by atrial contraction and motion of the septum toward the RVFW during left ventricular systole. One patient had incessant right ventricular tachycardia confined to the RVFW for 3 weeks. We conclude that RVFW disconnection is feasible and applicable to patients with refractory VT originating in the diffusely diseased RVFW.     

Optimal value of filling pressure in the right side of the heart in acute right ventricular infarction.

Haemodynamic monitoring was performed within the first 48 hours after the onset of symptoms in basal conditions, during volume loading, and during infusion of glyceryl trinitrate in 41 patients who fulfilled the diagnostic electrocardiographic and haemodynamic criteria of right ventricular infarction. In most patients an increase of mean right atrial pressure up to 10-14 mm Hg was followed by an increase in right ventricular stroke work index. But raising the mean right atrial pressure above 14 mm Hg was almost always accompanied by a reduction in right ventricular stroke work index. When the mean right atrial pressure was reduced by intravenous glyceryl trinitrate to less than 14 mm Hg the right ventricular stroke index increased. The same response was seen with cardiac and stroke index. The mean (SD) values of optimal right atrial and pulmonary capillary pressures were 11.7 (2.1) and 16.5 (2.7) mm Hg respectively. Thus cardiac and stroke index increased and the right ventricle reached its maximum stroke work index when the filling pressure was 10-14 mm Hg. These values may be regarded as the optimal level of right ventricular filling pressure in patients with right ventricular infarction.     

Embolus to the right atrium simulating myxoma

A space-occupying mass of the right atrium was found by two-dimensional echocardiography and angiography in a 68-year-old woman whose clinical diagnosis indicated multiple pulmonary emboli. Since right heart myxomas frequently cause pulmonary thromboembolism, the patient was initially diagnosed by noninvasive and invasive techniques as having a right atrial myxoma. Surgery, however, revealed the pathologic findings of large thrombi of the right atrium, femoral and iliac veins, and pulmonary arteries. This case vividly demonstrates that deep venous thrombosis may embolize and lodge in the right atrial cavity simulating a right atrial myxoma.     

Diverticulitis of the right colon

The authors' experience with right-sided diverticulitis is reviewed. The symptoms among 18 patients were continuous right lower quadrant abdominal pain and periumbilical pain radiating to the right lower quadrant. Only three patients had nausea and vomiting. Twelve patients (67 per cent) had an abnormally high white blood cell count; three had granulocytosis. Barium enema examination was not a helpful diagnostic aid; and in only two patients was the preoperative diagnosis correct. The operative procedures undertaken were right colectomy with ileotransverse colostomy (15 patients), partial right colectomy with ileoascending colostomy (two patients), and diverticulectomy and appendectomy (one patient). There were no deaths; the average hospital stay was 14.2 days (range 5 to 30 days). The authors conclude that there is no characteristic clinical pattern pointing to this diagnosis; diagnostic maneuvers are usually unrewarding; right hemicolectomy is a safe and expeditious procedure; and open cecotomy is not favored, as recommended in the medical literature, to establish the diagnosis. Read at the meeting of the American Society of Colon and Rectal Surgeons, Colorado Springs, Colorado, June 7 to 11, 1981.     

Technique of echographic examination of right ventricular wall thickness from the right sternal border.

Right ventricular wall motion was readily accessible for echocardiographic recording along the right sternal border in 27 pediatric patients (age: 24 hrs--12 yrs). Right ventricular epicardial and endocardial wall motion could be recorded only at the right sternal border in 10 of these patients, emphasizing the need for a new, alternative examination site. Calculated measurements of right ventricular wall thickness recorded from both right and left sternal borders (7 pts) were similar (range of diff.=0--0.6 mm; mean of diff.=0.26 mm). Autopsy confirmation of right ventricular wall thickness was obtained in 2 patients. Epicardial motion could not be identified at the left sternal border in 10 patients. In these patients, right ventricular wall thickness was estimated by measuring from the inner chest wall to the endocardium. These measurements were compared to right ventricular wall thickness obtained from the right sternal border. Wall thickness obtained from the left sternal border was significantly underestimated in the absence of a definite epicardial interface (range of diff.=0.2--2.6 mm; mean of diff.=1.3 mm).     

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.     

Revascularization of the right coronary artery: Influence thermodilution right ventricular ejection fraction

This study was designed in order to evaluate the influence of right coronary artery (RCA) disease and its revascularization on right heart performance monitored by measuring thermodilution right ventricular ejection fraction (RVEF). Forty patients undergoing elective aortocoronary bypass surgery were divided into two groups: group 1, with RCA revascularization, n = 20; and group 2, without RCA disease or revascularization, n = 20. RVEF was measured using a pulmonary arterial catheter mounted with a fast-response thermistor and a bedside microprocessor ejection fraction computer. The major finding of the study was that myocardial revascularization with extracorporeal circulation was followed by a decrease in RVEF which was significantly more pronounced in group 1 (−13.1%) in comparison to group 2 (−5.0%). RVEF gradually increased after bypass, but did not reach baseline values. By the first postoperative day, RVEF had reached baseline values again in group 1 and had increased beyond baseline values in group 2. Traditionally measured hemodynamic parameters could not be correlated with the course of RVEF, except for cardiac index. The present study further suggests that right-sided events may have clinical effects on left-sided function. Inadequate protection of the right heart, especially in patients with RCA stenosis, may result in depression of right ventricular myocardial performance, which can be monitored serially by measuring RVEF.     

A fistula connecting the right coronary artery to the right atrium: a hitherto undescribed association

A case of double right coronary artery accompanying a fistulous connection is presented. An additional right coronary artery is an extremely rare congenital abnormality. In this case, the second right coronary artery was draining into a cardiac chamber via a fistulous connection. This is the first case in the literature to present these abnormalities coexisting in the same patient.     

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.     

False aneurysm of the right atrium.

A false aneurysm of the right atrium is described. The false aneurysm appeared after open-heart surgery and was probably related to loosening of a right atrial suture. Because of the low pressure in the right atrium, the danger of rupture seemed to be low, and conservative therapy was chosen.     

Stent implantation to the stenosed right coronary artery in a patient whose right and left coronary arteries originate from a single ostium in the right sinus of Valsalva

The authors report a rare case of a patient who had anomalous origin of left and right coronary arteries from a single coronary ostium in the right sinus of Valsalva. The stenosed right coronary artery was successfully dilated with stent implantation.