Cardiac rhabdomyomas with atrial septal defect and tricuspid insufficiency: A case report

Primary cardiac tumors are very rare and generally benign. The most common type, cardiac rhabdomyoma, comprises 45% to 75% of primary cardiac tumors. Cardiac rhabdomyoma is a rare benign tumor that commonly presents with tuberous sclerosis. We present a case of an infant with multifocal cardiac rhabdomyomas with an atrial septal defect and tricuspid insufficiency and no sign of tuberous sclerosis. She was successfully treated with an operation, the treatment plan included mass resection, tricuspid annuloplasty, and closure of the patent foramen ovale. The right atrial lesion was resected entirely, while the lobulated lesion in the right ventricle was resected as two pieces. There was no evidence of recurrence 1 year after the surgery.     

Successful surgical resection and reconstruction for a huge primary cardiac lymphoma filling the right heart

Primary cardiac lymphoma (PCL) is rare, with a frequency of 1.0%–1.6% among cardiac malignant tumors. Chemotherapy is often selected as a first‐line treatment for PCL. However, when the tumor causes heart failure or life‐threatening hemodynamic collapse, antecedent urgent surgery is required. We herein report a successful case of complete tumor resection and reconstruction of the right atrium and right ventricle using a bovine pericardial patch combined with tricuspid valve replacement in a patient with a huge PCL filling the right heart that manifested as tricuspid valve stenosis and subsequent heart failure.     

A case report of isolated tricuspid valve endocarditis in a non-addicted adult person without underlying cardiac disease]

We report a rare case of isolated tricuspid valve endocarditis. A 46-year-old man who was non-addicted, non-alcoholic and had no underlying cardiac disease was admitted to our hospital for persistent pyrexia. Appropriate intravenous antibiotics therapy was unable to control the repeated infection and destruction of his tricuspid valve progressed. The patient was referred to our surgical division where on intraoperative inspection, tricuspid valve endocarditis was found to involve the entire anterior leaflet and part of the posterior leaflet. Following complete debridement of the infectious lesion, tricuspid valvuloplasty was not considered to be possible. So we performed tricuspid valve replacement using a mechanical valve (CARBOMEDICS 27 mm). His postoperative course was uneventful and he had no ventricular arrhythmia. After prophylactic antibiotic administration for 3 weeks, he was afebrile for 2 weeks without any medication. Thereafter he was discharged and has been free from any complications for over 1 year.     

原发性感染性心内膜炎瓣膜损害的外科治疗

目的总结２２例原发性感染性心内膜炎（ＰＩＥ）致瓣膜损害的外科治疗经验。方法心脏瓣膜置换术２０例，主动脉瓣和肺动脉瓣病灶清除及瓣膜成形术１例，三尖瓣病灶清除及瓣膜成形术１例。结果手术死亡率１３．６％，早期人工瓣膜心内膜炎１例（４．５％）。随访１５例，随访率６８．２％，随访时间２～８９个月，平均３５．６个月；发生晚期人工瓣膜心内膜炎１例（６．６％），再次手术出院后半月猝死，死因不明。其余１４例情况良好。结论ＰＩＥ致瓣膜损害时应积极手术治疗。只要手术方法正确，抗菌素应用合理，则手术疗效满意     

Debulking and three‐dimensional reconstruction of a rare case of right atrium‐ventricular angiosarcoma

A 48‐year‐old man presented with chest pain and ischemic manifestations according to an electrocardiogram due to coronary artery compression from a cardiac mass and was admitted to the emergency room and underwent extensive debulking followed by right atrium and ventricular three‐dimensional reconstruction with concomitant tricuspid valve remodeling. He recovered a normal sinus rhythm and was discharged from the hospital a week later with a diagnosis of cardiac malignant angiosarcoma according to the pathological examination. He survived and had a normal cardiac structure and function performance, but vertebral metastasis was suspected after more than 4 months of follow‐up after the procedure.     

Unique case of primary malignant fibrous histiocytoma of the right ventricle with moderator band involvement

Malignant primary tumors of the heart are very rare and in most cases are located in the left side of the heart, but involvement of the moderator band of the right heart has not yet been described in the literature. We report the case of a 22-year-old woman who presented after an episode of transient sudden loss of consciousness. The transthoracic echocardiography (TTE) and transesophageal echocardiography evaluations showed an echogenic mass that originated in the right ventricle and protruded into the tricuspid valve during systole. While operating on the patient, we found a sessile tumor that originated exclusively from the moderator band of the right ventricle. The tumor was completely resected through the tricuspid valve. An immunohistochemistry examination of the tumor confirmed the diagnosis of a malignant fibrous histiocytoma (MFH) (undifferentiated pleomorphic sarcoma). To our knowledge, this case is the first of moderator band involvement among the 50 cases of primary MFH of the heart that have been reported in the literature. After a year and a half, TTE, computed tomography (CT), and positron emission tomography CT (PET-CT) showed the absence of cardiac tumor, a normal tricuspid function, and no metastasis. The completeness of the surgical resection and the absence of local recurrence >1 year after surgery are encouraging signs for the patient's outcome.     

Right ventricular myxoma: Report of a case

We herein report a 36-year-old man who underwent surgical resection for myxoma. Preoperative two-dimensional echocardiography demonstrated a mass in the right ventricle. Intraoperatively, the tumor was found to derive from an anterior papillary muscle of the tricuspid valve. The tumor was successfully excised and the tricuspid valve was repaired with chordoplasty and annuloplasty. A histopathological examination revealed myxoma and a 2-year follow-up has shown no evidence of recurrence or tricuspid valve regurgitation.     

Surgery for atrial fibrillation using radiofrequency catheter ablation

OBJECTIVE: We present the results obtained in 40 patients with chronic atrial fibrillation using direct intraoperative radiofrequency to perform atrial fibrillation surgery. METHODS: Between April 2001 and June 2002, 40 patients underwent surgery for atrial fibrillation using radiofrequency ablation and cardiac surgery at the Department of Cardiovascular Surgery of the University of Bologna [corrected]. There were 8 men and 32 women with a mean age of 62 +/- 11.6 years (range: 20 to 80 years). RESULTS: Concomitant surgical procedures were: mitral valve replacement (n = 13), mitral valve replacement plus tricuspid valvuloplasty (n = 11), combined mitral and aortic valve replacement (n = 8), and combined mitral and aortic valve replacement plus tricuspid valvuloplasty (n = 5). Moreover, 1 patient underwent tricuspid valvuloplasty plus atrial septal defect repair, another required aortic valve replacement plus coronary artery bypass graft, and a third underwent aortic valve replacement. After the mean follow-up time of 16.5 +/- 2.5 months survival was 92.8% and the overall cumulative rate of sinus rhythm was 88.5%. CONCLUSIONS: We conclude that the radiofrequency ablation procedure is a safe and effective means of curing atrial fibrillation with negligible technical and time requirements, allowing recovery of the sinus rhythm and atrial function in the great majority of patients with atrial fibrillation who underwent cardiac surgery (88.5% of our study population).     

Septic coronary embolism in a deceptive case of presumed isolated tricuspid valve endocarditis

A 46‐year‐old female presented with native tricuspid valve endocarditis complicated by a stroke with a hemorrhagic component. There was no evidence of intracardiac shunt nor left‐sided valve involvement. Delayed surgery was planned to allow neurologic recovery, however, the patient developed an ST‐elevation myocardial infarction and cardiac arrest from an occluded right posterior ventricular branch of the right coronary artery from a septic embolism. Repeat imaging demonstrated new aortic valve vegetation involving the right coronary cusp. This case highlights a unique sequence of events in a patient initially presenting with presumed isolated tricuspid valve vegetation.     

A successfully operated case of isolated tricuspid insufficiency due to traumatic laceration of anterior leaflet of tricuspid valve

A case of isolated traumatic tricuspid insufficiency is presented. The patient was a 26-year-old designer with chief complaints of palpitation and dyspnea on exertion, who had the traffic accident three years and four months ago. The cardiac catheterization and two-dimensional echocardiography showed isolated insufficiency, and revealed that the basic damage was laceration of anterior leaflet of tricuspid valve itself. Tricuspid valve was replaced with SJM M-31 valve. His postoperative course was satisfactory. Until present to our knowledge, 13 cases of traumatic tricuspid insufficiency have been reported in Japan including our case. In three instances the papillary muscles ruptured. In nine patients the chordae tendineae were torn and in this case, there was primary laceration of anterior leaflet.     

Papillary fibroelastoma of the tricuspid valve

A 72-year-old male patient was admitted with chest oppression. Echocardiography disclosed a mobile tumor which was located on the atrial side of the tricuspid valve. Neither tricuspid obstruction nor regurgitation was observed. The mobility and the size, 20 mm in diameter, of the tumor indicated the need to perform surgical treatment. Through a right atriotomy, the tumor with multiple papillary fronds was found on the basal zone of the anterior leaflet. A resection of the tumor and tricuspid valvuloplasty with a partial annular reconstruction were performed. A pathological examination confirmed papillary fibroelastoma. He had an uneventful recovery, and postoperative echocardiography detected neither any residual tumor nor tricuspid regurgitation. In conclusion, it is reasonable to state that echocardiography is useful for detecting cardiac tumors, and a surgical resection is indicated for a mobile or large papillary fibroelastoma even when it is located on the right side of the heart.     

Surgical treatment of infective endocarditis in childhood--report of 3 cases

This is the case report of 3 surgical patients with infective endocarditis in childhood. Case 1: A 8 year-old boy was admitted with high fever and congestive heart failure. Aureus staphylococci were identified by blood culture. Echocardiogram showed a vegetating mass on the posterior mitral leaflet. This patient was cured by emergency mitral valve replacement during the active phase of infection. Case 2: A 3 year-old girl was admitted with infective endocarditis related to VSD. Viridans streptococci were identified by blood culture. Echocardiogram showed a vegetating mass on the septal tricuspid leaflet. After successful antibiotic therapy, this patient underwent the closure of VSD and tricuspid valvuloplasty. Case 3: A 7 year-old boy was admitted with infective endocarditis related to VSD. Aureus staphylococci were identified with blood culture. Echocardiogram showed a vegetating mass on the anterior tricuspid leaflet and moderate tricuspid regurgitation. After successful antibiotic therapy, this patient underwent the closure of VSD and tricuspid valvuloplasty. We believe that echocardiography plays an important role in the diagnosis and management of infective endocarditis and that tricuspid valvuloplasty is the method of first choice in treatment of the patient with regional tricuspid infective endocarditis.     

Concomitant mitral valvuloplasty and fenestrated total cavopulmonary connection: a case report

A seven-year-old boy with tricuspid atresia successfully underwent a fenestrated total cavopulmonary connection and mitral valvuloplasty. Preoperative cardiac catheterization showed a mean pulmonary artery pressure of 16 mmHg. Pulmonary arteriography showed poor development of the branches (PA index: 180). Echocardiography revealed mild to moderate mitral valve incompetence due to prolapse of anterior leaflet. Mitral valve was exposed through the trans-septal approach. The excess chorda length was tucked into a longitudinal split in the top of the posterior papillary muscle. Then wedge resection of the redundant segment of the anterior leaflet and bilateral annuloplasty were performed. Finally, a total extracardiac cavopulmonary anastomosis with a 6 mm fenestration was completed. Postoperative clinical course was uneventful, and he is doing well with no recurrence of mitral incompetence 1 year after the operation.     

Endocardial Fibroelastosis of the Right Ventricle and Tricuspid Valve in a Young Adult with Behcet's Disease

Abstract Endocardial fibroelastosis is characterized by massive proliferation of collagenous and elastic tissue, in which the pathological process is restricted to the endocardium. In this report, we present the case of a 20‐year‐old man with Behcet's disease and endocardial fibroelastosis of the right ventricle involving tricuspid valve resulting in a tumor mass that was resected along with tricuspid valve replacement. The clinical and pathological features of this rare entity are reviewed . (J Card Surg 2010;25:347‐349)     

Long-term follow-up of resection of primary benign right ventricular tumours: a 10-year surgical experience

IntroductionPrimary benign right ventricular tumours are rare. They can cause significant mortality without appropriate and timely treatment. We investigated surgical treatment and survival characteristics for right ventricular tumours.Materials and methodsFrom 2007 to 2017, 21 patients with primary benign right ventricular tumours who underwent tumour resection were retrospectively reviewed. Clinical findings and follow-up results were analysed.ResultsThirteen men and eight women were enrolled, with a mean age of 42.3 ± 15.3 years. The most frequent histotypes were myxoma, haemangioma and papillary fibroelastoma. Eight patients underwent concomitant tricuspid valvuloplasty and one had tricuspid valve replacement. No major adverse events or death occurred during the perioperative period. One patient with haemangioma underwent partial tumour resection; however, the tumour regressed gradually during follow-up. Within the 10-year follow-up period (mean 4.8 ± 2.6 years), the recurrence-free and overall survival rates were 81.0% and 85.7%, respectively.ConclusionsTumour resection for primary benign right ventricular tumours is safe and effective, and has a good prognosis. Tricuspid valvuloplasty or tricuspid valve replacement may be necessary for the resection of right ventricular tumours to improve the haemodynamics. Haemangiomas naturally undergo spontaneous regression.     

小儿原发性心脏肿瘤27例分析

目的 探讨原发性心脏肿瘤的治疗经验.方法 回顾性分析1999年5月至2009年5月27例经心脏超声检查确诊为心脏肿瘤患者的临床资料.男性20例,女性7例;年龄24 d～12.6岁,＜1岁者16例,占59.2%.22例为单发心脏肿瘤,5例为多发.其中22例因心律失常、心包积液、呼吸困难、晕厥等症状进行手术切除,5例未施行手术.根据肿瘤的具体位置选择不同的切口径路,14例完整切除肿瘤,8例部分切除;5例行二尖瓣整形,2例行三尖瓣整形.结果 术后肿瘤组织学结果:纤维瘤5例,横纹肌瘤8例,黏液瘤4例,毛细血管瘤3例,纤维肉瘤和卵黄囊瘤各1例.16例术后循环稳定;2例出现低心排血量,经治疗后心功能恢复;4例因严重低心排血量并发多器官功能衰竭死亡.18例术后随访1～10年,2例横纹肌瘤残余肿块缩小,1例消失;1例纤维肉瘤、1例血管瘤残余肿块未增大;黏液瘤术后无复发或远处种植转移.5例未手术者随访1～3年,其中2例无明显症状;1例左心室游离壁肿瘤因心律失常死亡,另1例仍存活;1例多发性心脏肿瘤伴低心排血量患者因心力衰竭死亡.结论 小儿原发性心脏肿瘤应采用个体化治疗原则,对有明显症状者应行手术治疗,对无明显症状者要密切随访.手术的目的 不是完整切除肿瘤,而是要恢复正常的血流动力学状态.

Abstract：

Objective To analyze the experience of treatment strategies for pediatric patients with primary cardiac tumors. Methods The clinical data of 27 patients with primary cardiac tumors which detected by echocardiography from May 1999 to May 2009 was analyzed retrospectively. There were 20 male and 7 female patients, aged from 24 d to 12. 6 years. There were 59. 2% less than 1 year old at the time of diagnosis. A single tumor were present in 22 cases and multiple in 5 cases. Surgery was performed for 22 patients due to the varied significant symptoms such as arrhythmia, pericardial effusion, swoon and congestive heart failure with dyspnoea. Five patients were discharged hospital without surgical treatment. The surgical approachs were adopted according to tumor location. Complete surgical resection was performed in 14 patients and partial resection in 8 patients. Seven patients were underwent valve reconstruction,5 involving the mitral valve and 2 involving the tricuspid valve. Results Histologic examination of the surgically resected tumors showed rhabdomyomas in 8 cases, fibromas in 5 cases, hemangiomas 3 cases,myxomas in 4 cases, fibrosarcoma in 1 case and yolk sac sarcoma in 1 case. Sixteen cases revealed stable haemodynamic status postoperative. Two cases occurred apparent symptoms of low cardiac output and significant arrhythmias, finally recovery after comprehensive treatment of restoration the heart function. There was a total of 4 patients in-hospital death following surgery due to multiorgan system failure. Of the 18 patients who survived after the surgery were followed up from 1 to 10 years, echocardiography showed the residual mass of the tumor with partial resection, rhabdomyoma diminishing in 2 patients and almost vanishing in 1 patient. The residual mass of one fibrosarcoma patient and one hemangioma patient were not increased. Patients with myxomas had no recur or systemic embolisation after the initial surgery. Five nonsurgical patients were followed up from 1 to 3 years, 2 patients without haemodynamic alterations,1 patients with giant tumor of left ventricular free wall was died of arrhythmia, the other one was alive; the patient of multiple cardiac tumor with low cardiac output was died of heart failure. Conclusions Despite the benign histology of most paediatric primary cardiac tumours, there may be significant associated with morbidity and occasional mortality. Therapy strategies should be individualised: surgery is indicated in cases with significant clinical symptoms and close follow-up is necessary for asymptomatic patients. Total resection is not the only therapeutic aim. Most important is the restoration of the normal haemodynamic heart function.     

A case of two-chambered right ventricle complicating infective endocarditis and tricuspid regurgitation

A 31-year-old male of two-chambered right ventricle with ventricular septal defect, complicating infective endocarditis and tricuspid regurgitation, was presented. Two-dimensional echocardiographic study demonstrated tricuspid vegetations and a hypertrophied, anomalous muscle bundle in the right ventricle. Cardiac catheterization revealed 58 mmHg pressure gradient between inflow chamber and outflow chamber of the right ventricle. It seems that tricuspid regurgitation was resulted from infective endocarditis. He underwent resection of anomalous muscle bundle, repair of ventricular septal defect, and tricuspid valve replacement with satisfactory result. It has not been reported in Japan so far that tricuspid valve replacement was performed for the treatment of tricuspid regurgitation due to infective endocarditis in the patient with two-chambered right ventricle. In our case, cardiac catheterization was performed after subsidence of infective endocarditis. As echocardiography can detect vegetations and anomalous muscle bundle precisely, surgical intervention would be performed without cardiac catheterization in the case of infective endocarditis intractable to medical therapy.     

Surgical treatment of early acute thrombosis of mechanical mitral prosthesis

Prosthetic valve thrombosis is a rare but life threatening complication of mechanical heart valve prosthesis. A 44-year-old woman diagnosed with rheumatic heart disease with severe mitral valve stenosis, moderate tricuspid valve insufficiency, and atrial fibrillation underwent transseptal mitral valve replacement and tricuspid valvuloplasty in our department. Heparin and warfarin were routinely used postoperatively. Although the international normalized ratio (INR), activated partial thromboplastin time ratio, and platelet count were satisfactory, the patient presented with severe dyspnea suddenly 10 days after discharge; echocardiogram showed that the prosthetic posterior leaflet was immobile. The patient suffered cardiac arrest suddenly during the examination and cardiopulmonary resuscitation was carried out successfully. Emergent surgery was performed, confirming the prosthetic valve thrombosis. The prosthetic valve was replaced with another mechanical prosthesis. The patient recovered smoothly and was discharged 14 days later with atrial fibrillation. During the 12-months follow-up period, her prosthetic valve and heart function were normal with INR around 3.0. This case highlights the need for awareness among clinicians for the possibility of valve thrombosis in the early postoperative period.     

Lymphangioma of the hepatoduodenal ligament

Abdominal lymphangiomas are extremely rare in the adult population. We report an unusual case of a cystic lymphangiomas arising from the hepatoduodenal ligament. A 45‐year‐old man was admitted to our hospital with discontinuous upper abdominal pain. The preoperative CT diagnosis was neurinoma. Exploratory laparotomy revealed a cystic mass originating from the hepatoduodenal ligament and then it was resected successfully. The pathological sections proved it to be lymphangioma. The patient remains well with no evidence of recurrence two years post resection.