Primary lymphoma of the heart: case report and literature review

Primary cardiac lymphoma (PCL) is a rare and usually fatal neoplasma. A case of PCL in a 78-year-old man who complained of exertional dyspnea and peripheral edema is presented. Echocardiography revealed a mass in the right atrium and a diagnosis of low-grade B-cell lymphoma was obtained with the surgically resected tumor. The lesion appeared to have originated in the right atrium and involved the right ventricle. The patient died of bronchopneumonia 8 months after the initial consultation. The present case and 39 patients with PCL reported between 1995 and 2002 were reviewed. Forty patients showed various and non-specific symptoms such as dyspnea, edema, arrhythmia and pericardial effusion. Primary cardiac lymphoma occurred slightly more often in male patients (M : F = 23:17) and in the elderly in general (mean age, 67 years), with lesions found in the following locations, listed in order of frequency: right atrium, pericardium, right ventricle, left atrium, left ventricle, and other sites. Antemortem diagnosis was obtained in 37 of the 40 patients. Thirty-seven cases were of B-cell lineage and two cases were of T-cell lineage. Complete remission was obtained in only 15 of the 40 patients. Although PCL antemortem diagnoses have been made in the majority of recent cases, the prognosis still remains poor.     

Hemangiomas of the heart. A clinicopathologic study of ten cases

The clinical and histopathologic features of 10 cardiac hemangiomas are presented. The patients' ages ranged from 2 weeks to 65 years (mean: 32 years). Eight patients had no other cardiovascular abnormalities, one was associated with intra-atrial lipoma, and one was associated with an anomalous origin of the left coronary artery. One patient had other hemangiomas of the oral cavity and small intestine. The tumors presented as pericardial effusion (2), sudden death (2), shortness of breath (1), incidental finding on chest X-ray (1), asymptomatic murmur (1), syncope (1), and incidental finding at autopsy (2). Location of the tumors included the right ventricle (4), left ventricle (3), atrial septum (2), and right atrium (1). All six tumors presenting in life were resected successfully, one requiring placement of a synthetic graft. Cardiac hemangiomas are usually sporadic; presenting symptoms depend on location in the heart; arrhythmias or sudden death can occur if the tumor is present in the basal region of the ventricular septum; surgical resection is feasible in other locations.     

Clinical experiences of cardiac myxoma

Although cardiac myxoma is rare, it is the most common primary cardiac tumor. Seventy-four cases of cardiac myxoma that were surgically treated in our center between August 1980 and February 2005 were retrospectively reviewed. The mean patient age was 50.4 +/- 15.0 (range 7-80) years, and 53 patients (71.6%) were female. The most common preoperative symptom, occurring in 44 patients, was dyspnea. The interval from onset of symptoms to surgery was 9 months. Seventy cases were located in the left atrium, 3 in the right atrium and 1 in the right ventricle. The myxoma in the right ventricle could not be resected completely, due to severe infiltration. Cardiopulmonary bypass and aortic cross clamp times were 100.4 +/- 37.1 and 64.8 +/- 29.8 minutes, respectively. There were no hospital deaths, and 7 patients suffered from postoperative complications including atrial fibrillation in 2 cases. During the follow up period (mean 105.7 +/- 73.6 months), there was no tumor recurrence and 6 late deaths that were not related to the underlying tumor. There was no evidence of tumor growth in the cases with incomplete resection during the 14-month follow-up. In conclusion, in this study there was no recurrence of tumors after complete resection and surgical resection is considered to be the curative method of treatment for cardiac myxoma.     

Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience

Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor.     

Iatrogenic cardiac papillary fibroelastoma: a study of 12 cases (1990 to 2000)

Cardiac papillary fibroelastoma (PFE) is a rare, benign, slow-growing tumor of the endocardium. Whether it represents a reactive tumoral lesion or a true neoplasm remains a matter of debate. However, an anecdotal association of PFEs with previous cardiac surgery has been reported. The current study was undertaken to determine the frequency and nature of iatrogenic events associated with PFEs and to provide a comprehensive review of the topic. The study group comprised 12 cases seen between 1990 and 2000, with specimens from 7 women and 5 men. Six developed postoperatively and 6 developed after thoracic irradiation. The 9 Mayo cases represented 18% of all surgically excised PFEs during the study period. The mean age at operation was 54 years (range, 29 to 79 years). The mean interval between the iatrogenic event and excision of the tumor was 18 years (range, 9 to 31 years). In 58% of cases, the presence of multiple tumors was either confirmed pathologically (41.7%) or strongly suggested by echocardiography (16.6%). Among patients who had undergone previous cardiac surgery, PFEs were found in the chamber closest to the procedure. Similarly, in patients who had received radiation therapy, tumors developed in the left atrium, in the right ventricle and atrium, and on the tricuspid valve within the radiation field. In conclusion, iatrogenic PFEs may be relatively common among all such tumors, are frequently multiple, and often involve nonvalvular endocardial surfaces.     

RHABDOMYOSARCOMA OF THE RIGHT VENTRICLE

A case of a 66-year-old male with rhabdomyosarcoma of the right ventricle who presented first with a pleuritic chest pain and later developed congestive heart failure with A-V block is described. The total period of the disease was about 9 months. At autopsy the right ventricle and wall were found to be replaced by tumor tissue which was diagnosed histologically as rhabdomyosarcoma of the alveolar type. We described one case and 52 rhabdomyosarcomas of the heart from the literature; the ages ranged from 3 months to 92 years with 29 men and 23 women (unknown 1). The primary lesions were right ventricle 29%, right atrium 26%, left atrium 24%, left ventricle 6%, and others 15%.     

Right ventricular protection with coronary sinus retrograde cardioplegia

Fifteen consecutive patients having open heart surgery using retrograde cardioplegia were studied to demonstrate that important venous collateralization exists between the coronary sinus (CS) and its left ventricular branches and the right ventricle (RV). The venous collateralization makes possible RV myocardial protection during retrograde cardioplegia. Right ventricular venous drainage principally occurs via anterior cardiac veins, which drain into the right atrium, and thebesian veins, which drain into both the RV and the atrium, generally without connection to the CS. Retrograde cardioplegia used during open heart surgery should, therefore, give inadequate myocardial protection to the RV. Two RV temperature probes used as markers for RV perfusion were monitored continuously during cardiac arrest. Systemic temperature while on cardiopulmonary bypass was 25°C, and the retrograde perfusate solution temperature was 4°C. Coronary sinus pressure during the bypass procedure was maintained between 20 torr and 50 torr. Mean temperatures at the two probe sites were 16.1°C and 14.5°C. We conclude that a complex network of venous collaterals between the coronary sinus and left ventricle and the right ventricle allow excellent myocardial protection during retrograde cardioplegia. © 1994 Wiley-Liss, Inc.     

Right atrial myxoma: an unusual cause of intracardiac right-to-left shunt

A case of a right-to-left intracardiac shunt caused by a right atrial myxoma is reported. Sudden hypoxaemia was the unique symptom. Measurement of Pao2 while the patient breathed pure oxygen demonstrated the presence of a shunt. Right heart catheterization showed a patent foramen ovale and a pressure gradient between the right atrium and the right ventricle, and between the right and the left atria, explaining the shunt. Cineangiography demonstrated a right atrial myxoma, which was surgically removed. This case report illustrates how a step-by-step diagnostic procedure, based on pathophysiology, may lead to such unexpected findings.     

人工智能云技术在乳腺癌患者心脏亚结构自动勾画中的应用

目的:评估人工智能云技术勾画平台（AI Contour）在乳腺癌患者心脏亚结构自动勾画中的准确性和可行性。方法:选取10例进行乳腺癌放射治疗患者的血管增强CT作为研究对象。在AI Contour上分别采用手动勾画、自动勾画和自动勾画后手动修改模式来完成10例患者的心脏亚结构勾画,包括左心房、右心房、左心室、右心室。比较Dice相似性系数（DSC）、Jaccard系数（JC）、Hausdorf距离（HD）、质心偏差（CMD）、包容性系数（IncI）、敏感性指数（SI）、勾画时间。结果:以手动勾画为金标准,自动勾画与手动勾画各心脏亚结构的DSC>0.8,JC>0.6,HD<9 mm,CMD<5 mm,IncI>0.8,SI>0.7。自动勾画后手动修改进一步提高了勾画精度,其中JC>0.8。自动勾画时间与手动勾画时间为（85.50±6.06） s vs （1 160.30±74.31） s,差异具有统计学意义（P<0.05）。自动勾画后手动修改总时间与手动勾画时间为（558.70±33.40） s vs （1 160.30±74.31） s,差异具有统计学意义（P<0.05）。结论:通过比较发现自动勾画技术能以较高的精度完成乳腺癌患者左心房、右心房、左心室、右心室的勾画,节省了大量时间,自动勾画后手动修改能进一步提高各心脏亚结构的勾画精度,同时云勾画平台具有远程协作的优势,值得推广运用。     

Aneurysm of fossa ovalis in adults: a pathologic study.

BACKGROUND: Aneurysm of the fossa ovalis is an out pouching, space-occupying, interatrial septal structure. The anatomic morphology and characteristics of this aneurysm are of interest for pathologists and cardiologists alike. METHODS: We identified 33 specimens of adult hearts with a large size aneurysm of the fossa ovalis (length equal to or more than 10 mm from the plane of the atrial septum) in a registry of cardiovascular disease. Anatomic-morphologic features of these aneurysms were examined by macroscopic and histopathologic studies. RESULTS: Nineteen aneurysms were from females (57%) and 14 were from males (43%). Most aneurysms had a dome shape with maximal length of the aneurysmal excursion into an atrium varying from 10 to 35 mm (mean 16+/-5 mm) and width varying from 16 to 40 mm (mean 24+/-6 mm). Twenty-four aneurysms (73%) protruded into the right atrium while only nine (27%) penetrated into the left atrium. In 24 patients the interatrial ostium II was patent, and in 22 (91%) of them, abnormal increased intracardiac pressure was deemed responsible for the formation of the aneurysm. Among nine patients in whom the foramen ovale was closed, eight (89%) had an aneurysm protruding into the right atrium, and only one aneurysm penetrated into the left atrium. In six patients, the aneurysm further stretched an already patent foramen ovale resulting in creation of an atrial septal defect so that bidirectional shunting could occur, and in three cases, the aneurysm narrowed the inferior vena caval orifice. In three hearts, the aneurysm wall had endocardial fibrosis, and in three other specimens, a focal mural thrombus was present on the aneurysmal surface. Most common associated cardiac conditions in this series included atherosclerotic coronary artery disease (51%), aortic valvular disease (21%) and mitral valve disease (24%). CONCLUSIONS: Aneurysm of the fossa ovalis is a space-occupying, redundant structure, most commonly with a dome shape. In a majority of cases, the formation of this aneurysm relates to the effect of extrinsic mechanisms, which create abnormally elevated intracardiac pressures. The aneurysm protruded into the right atrium in 73% of cases. Endocardial fibrosis and a focal mural thrombus were present in several cases on the aneurysmal wall. Bidirectional shunt via stretched patent foramen ovale and distal embolization can contribute to complications related to the aneurysm.     

Coenzyme Q10 content in different parts of the normal human heart

Coenzyme Q10 (CoQ10) and citrate synthase (CS) activities were analysed in the myocardium of brain-dead organ donors (14-40 years). Different parts of the heart were studied: right and left auricular appendage, right and left atrium, right ventricle (septum and free wall) and left ventricle (septum, free wall, and papillary muscle). Freeze-dried, dissected myocardial samples were analysed for CoQ10 content by HPLC and CS activity by fluorometric technique. CoQ10 content in the normal human myocardium was lowest in auricular appendages and atria (0.25 +/- 0.06 mg X g-1 dry muscle), intermediate in right ventricle (0.37 +/- 0.05 mg X g-1 dm) and highest in left ventricle (0.42 +/- 0.07 mg X g-1 dm). CS activity showed the same relationship between these locations as CoQ10. The results suggest that there exist differences in CoQ10 content between different parts of the normal human myocardium. These differences were closely related to the differences in CS activity between corresponding parts. The differences between different parts of the heart may be related to divergent work demand, and the constant relationship between CS and CoQ10 may be related to their coupling to the mitochondrial oxidative metabolism.     

正常引流肺静脉解剖变异的多层螺旋CT血管成像研究

目的 探讨正常引流肺静脉的解剖变异率及其变异形式。方法 回顾性分析2013年5月—2014年7月中山大学附属孙逸仙纪念医院220例两侧肺静脉均引流至左心房患者的胸部64层螺旋CT血管成像(MSCTA)资料,对肺静脉进行多平面重建(MPR)、最大强度投影(MIP)及VR重建,观察段以上肺静脉引流区域,以及双侧肺静脉与左心房连接模式。两侧肺静脉分别以上、下肺静脉独立开口于左房,右中叶肺静脉回流至上肺静脉者为正常肺静脉引流模式;一侧单支或多于两支肺静脉引流、跨叶引流者为肺静脉解剖变异。结果 220例正常引流肺静脉中,左右肺静脉总变异发生率22.7%(50/220)。右肺静脉解剖变异38例(17.3%,38/220),共见8种变异模式,分别为:(1)上、下叶肺静脉分别汇入左心房,中叶静脉汇入下叶肺静脉 4例(1.8%,4/220);(2)上、下叶肺静脉分别汇入左心房,上叶后段汇入下叶肺静脉2例(0.9%,2/220);(3)上、中、下叶静脉分别汇入左心房16例(7.3%,16/220);(4)上叶后段、尖前段、下叶肺静脉分别汇入左心房,中叶静脉汇入尖前段肺静脉4例(1.8%,4/220);(5)上叶、下叶背段、下叶基底段肺静脉分别汇入左心房,中叶静脉汇入上叶肺静脉2例(0.9%,2/220);(6)上叶、 中叶内段、中叶外段、下叶肺分别静脉汇入左心房6例(2.7%,6/220);(7)上叶后段、上叶尖前段、中叶、下叶肺静脉分别汇入左心房2例(0.9%,2/220);(8)上叶、中叶、下叶背段、下叶基底段肺静脉分别汇入左心房2例(0.9%,2/220)。左肺静脉变异12例(5.5%,12/220),共见2种变异模式,即上、下叶肺静脉组成共干汇入左心房8例(3.6%,8/220),上叶、舌叶、下叶肺静脉分别汇入左心房4例(1.8%,4/220)。220例患者中,左右肺静脉解剖变异率的差异有统计学意义(χ²＝13.533, P<0.01)。结论 MSCTA上正常引流肺静脉解剖变异常见,右肺静脉解剖变异发生率显著高于左肺静脉,且变异模式多样。     

Transesophageal echocardiography guided patent ductus arteriosus occlusion in adults with severe pulmonary hypertension through a parasternal approach

Between April 2010 and April 2014, 39 consective adult patients (> 18 years) with PDA associated severe pulmonary hypertension underwent transesophageal echocardiography guided patent ductus arteriosus occlusion through a parasternal minimally invasive approach. Among 39 patients, the procedure was successful in 32 cases (82.1%) and failed in 7 cases (17.9%). In the failed cases, 3 cases had a large residual shunt and 4 cases had persistent pulmonary hypertension. The mean minimum miameter of the successfully closed PDAs was 15.2 ± 2.1 mm (range 9 to 24), and the mean diameter of the mushroom-shaped occluder was 17.5 ± 2.5 mm (range 11 to 26). The pulmonary artery pressure decreased significantly after occlusion (P < 0.05), but there were no significant differences in the aortic pressure and blood oxygen saturation before and after occlusion (P > 0.05). Echocardiography performed on the first postoperative day showed decreased volume within the left atrium, left ventricle, and pulmonary artery in 23 cases, decreased volume within the left atrium and left ventricle in 4 cases, and no change in the volume of the atrium and ventricle in 3 cases. A minor residual shunt was observed in 6 cases. The posteroanterior chest X-ray showed improved pulmonary congestion in all cases and significantly reduced cardiothoracic ratio in 25 cases. Patients were followed-up at least for 1 year. No symptoms including palpitation, dyspnoea, or chest tightness were observed. The heart function ranged from NYHA class I to II. A minor residual shunt was observed only in one case. There were varying degrees of decrease in volume within the atrium and ventricle. In conclusion, transesophageal echocardiography guided patent ductus arteriosus occlusion through a parasternal minimally invasive approach is a feasible and effective method for the treatment of PDA in adults with severe pulmonary hypertension.     

肺血栓栓塞症超声心动图诊断

目的 肺血栓栓塞症是心血管急症，应提高对其认识和超声心动图诊断水平；方法 对12例以超声心动图首先诊断的肺血栓栓塞症进行回顾性总结，6例手术或尸检证实；结果 肺血栓栓塞症临床表现多样化，超声心动图直接依据是肺动脉主干及左右分支内血栓回声；间接征象为右房室腔扩大及肺动脉高压；深静脉血栓是栓子来源常见病因，血管彩色多普勒血流显像对深静脉血栓有确诊价值；结论 超声心动图对肺血栓栓塞症具有重要诊断价值，并有助于临床与急性心肌梗塞、主动脉夹层动脉瘤和心包填塞进行鉴别。     

AN AUTOPSY CASE OF IDIOPATHIC ENLARGEMENT OF THE RIGHT ATRIUM, AND A REVIEW OF THE LITERATURE

A 69-year-old man in whom idiopathic enlargement of the right atrium was revealed at autopsy is described. The patient had had cardiomegaly of at least 19 years'duration prior to his death, even though cardiac symptoms were absent. Cause of death was pancreatic carcinoma. Postmortem examination revealed marked and diffuse dilatation of the right atrium and moderate dilatation of the left atrium. Measurement of the cardiac chambers showed that the right and left atria were 7.6 and 4.7 times as large as those of normal hearts, respectively. The volume of either ventricle was about twice the normal value. Histologically, widespread cardiac muscular degeneration and necrosis, diffuse fibrosis, and focal lymphocytic infiltration were found in the right atrium and also, to a lesser degree, in the left atrium. Such pathologic changes were not found in either of the ventricles. The etiology of these muscular changes, which might have been related to atrial enlargement, was unclear. The present case was thought to be consistent with idiopathic enlargement of the right atrium, and a brief review of the literature is given.     

Double inlet right ventricle

Summary A case of double inlet right ventricle in which both atrioventricular valves opened completely into the right ventricle is described. The left atrium communicated with the right ventricle by way of an opening low in the atrial septum and there was a large ventricular septal defect. The pulmonary trunk and aorta took their origin from the right and hypoplastic left ventricles respectively. The literature on this rare cardiac anomaly is reviewed and the morphological findings in this case are compared with those of the recorded cases. A short discussion of the possible embryogenesis is given.     

Right atrial thrombi complicating use of central venous catheters in hemodialysis

BACKGROUND: There has been an increase in the use of central venous catheters for temporary hemodialysis access. In this report, we describe a case series of 12 patients on hemodialysis who developed intracardiac clots associated with the use of central venous catheters, their outcomes and review the literature on this syndrome. METHODS: Retrospective, single-center case series of 12 patients with right atrial thrombi associated with central venous catheter use for dialysis vascular access. These patients were treated between June 1, 2001 and June 30th 2002 at the three University of Rochester affiliated dialysis clinics. The medical records of these patients were reviewed to obtain information concerning demographics, dialysis history, dates of catheter insertion, catheter complications, identification and dimensions of intracardiac thrombi, echocardiographic data, and outcome of anticoagulant therapy. RESULTS: Right heart thrombi were identified in 12 patients in our hemodialysis population over the study interval. Anticoagulation for 6 months led to clot resolution in more than 50% of our patients. Bacteremia recurred in 6 patients (50%), in spite of catheter replacement. There was zero mortality related to presence of clots in the short term. One patient with non-bacteremic right atrial thrombus suffered sudden cardiac arrest, but was successfully resuscitated. CONCLUSION: Right atrial thrombi may occur in hemodialysis patients who use central venous catheters for dialysis access. These clots are frequently found in the right atrium or right atrial-superior vena caval junction. Ten of 12 patients (83%), had catheter-associated bacteremia, but the precise relationship between bacteremia and intracardiac clots is unclear. Chronic anticoagulation for 6 months lead to resolution of these clots in more than 50% of patients in our series.     

An autopsy case of idiopathic enlargement of the right atrium, and a review of the literature

A 69-year-old man in whom idiopathic enlargement of the right atrium was revealed at autopsy is described. The patient had had cardiomegaly of at least 19 years' duration prior to his death, even though cardiac symptoms were absent. Cause of death was pancreatic carcinoma. Postmortem examination revealed marked and diffuse dilatation of the right atrium and moderate dilatation of the left atrium. Measurement of the cardiac chambers showed that the right and left atria were 7.6 and 4.7 times as large as those of normal hearts, respectively. The volume of either ventricle was about twice the normal value. Histologically, widespread cardiac muscular degeneration and necrosis, diffuse fibrosis, and focal lymphocytic infiltration were found in the right atrium and also, to a lesser degree, in the left atrium. Such pathologic changes were not found in either of the ventricles. The etiology of these muscular changes, which might have been related to atrial enlargement, was unclear. The present case was thought to be consistent with idiopathic enlargement of the right atrium, and a brief review of the literature is given.     

Biventricular support with the Jarvik 2000 axial flow pump: a feasibility study

Patients with congestive heart failure who are supported with a left ventricular assist device (LVAD) may experience right ventricular dysfunction or failure that requires support with a right ventricular assist device (RVAD). To determine the feasibility of using a clinically available axial flow ventricular assist device as an RVAD, we implanted Jarvik 2000 pumps in the left ventricle and right atrium of two Corriente crossbred calves (approximately 100 kg each) by way of a left thoracotomy and then analyzed the hemodynamic effects in the mechanically fibrillated heart at various LVAD and RVAD speeds. Right atrial implantation of the device required no modification of either the device or the surgical technique used for left ventricular implantation. Satisfactory biventricular support was achieved during fibrillation as evidenced by an increase in mean aortic pressure from 34 mm Hg with the pumps off to 78 mm Hg with the pumps generating a flow rate of 4.8 L/min. These results indicate that the Jarvik 2000 pump, which can provide chronic circulatory support and can be powered by external batteries, is a feasible option for right ventricular support after LVAD implantation and is capable of completely supporting the circulation in patients with global heart failure.     

Isolated right ventricular hypertrophic obstructive cardiomyopathy

Two cases of isolated hypertrophic cardiomyopathy of right ventricle without any involvement of interventricular septum or left ventricle are reported. Two cases reported in literature are also reviewed. In these cases symptoms are those of left ventricular hypertrophic obstructive cardiomyopathy. Right ventricular dominance on clinical examination and electrocardiogram and inspiratory increase in intensity of murmur are helpful clues. 2-Dimensional echocardiography with Doppler evaluation can confirm the diagnosis noninvasively.