A case report of a right ventricular mass in a patient with Behçet's disease: Myxoma or thrombus?

INTRODUCTION: Behçet's disease (BD) is a multi-system, chronic and relapsing disorder classified as "vasculitic syndrome". It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet's disease. METHODS: The authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet's disease. RESULTS: A 19-year-old boy was admitted to our hospital for the investigation of "fever of unknown origin", weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as "an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells". Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet's disease. The medical management included immunosuppressant and anticoagulation. CONCLUSION: Behçet's disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.

Ischemic gall bladder perforation: a complication of type A aortic dissection.

Malperfusion of end organs occurs in 20% to 40% patients with acute type A aortic dissection. Because irreversible ischemia is a time-dependent event, expedient diagnosis and treatment are necessary. We herein report successful surgical management of a patient with acute type A aortic dissection causing transient gut ischemia and a rare gall bladder perforation. We implemented one-stage surgical and laparoscopic management approach for the diagnosis and treatment. Increased awareness of this complication and appropriate use of available diagnostic tools may improve the outcome in similar patients. Patients with aortic dissection complicated by visceral ischemia require a prompt sequential and rational multidisciplinary approach for successful management.

Tenecteplase in prosthetic mitral valve thrombosis.

In the few reported cases of prosthetic mitral valve thrombosis, where surgical intervention was considered as high risk, fibrinolytic therapy had proved life saving. The authors present clinical, laboratory, and imaging data from such a patient, with prosthetic mitral valve thrombosis and its successful management with tenecteplase. The use of tenecteplase as a viable fibrinolytic agent for the first time was justified, due to the lack of immunogenicity concerns compared to streptokinase.

Papillary fibroelastoma of the aortic valve--a case report and literature review.

The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

Inflammatory myofibroblastic tumor of the right atrium.

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity and is associated with distinct clinical, pathological and molecular features. The clinical behavior, natural history, biological potential, management and prognosis of such tumors are unclear. We present herewith an adolescent girl who presented with similar entity involving the junction of the right atrium and the inferior vena cava (IVC) in association with thrombocytosis and IVC thrombosis leading to obstruction of blood flow. Diagnostic tools included imaging and immuno-histopathology studies. Surgical management included resection of the tumor and thrombo-embolectomy of the IVC under cardiopulmonary bypass. This case is unique due to association of complete obstruction of IVC caused by the strategic location of the tumor, thrombosis of vena cava and association of thrombocytosis. These features have not been reported yet in relation to the cardiac IMT. This report will help in better understanding and management of similar cases in terms of planning cannulation of femoral veins or application of total hypothermic circulatory arrest during cardiopulmonary bypass and prompt us to look for recurrence or metastasis during follow up using echocardiography and laboratory investigations. The possibility of IMT should be kept in the differential diagnosis of cardiac tumors especially in children and adolescents.

Dislocation of a stent-graft into the aortic arch during endovascular repair of a descending thoracic aortic aneurysm.

A 59-year-old man showed a saccular aneurysm due to a penetrating atherosclerotic ulcer, as well as a small type B aortic dissection located in the proximal descending aorta. The lesion was treated by the implantation of a stent-graft. On release, the stent-graft dislocated into the aortic arch. Intraoperative angiogram showed free perfusion of the brachiocephalic trunk and left common carotid artery; however, an overstenting of the carotid artery was apparent. Computed tomographic scan exhibited a complete covering of the supra-aortic vessels, and conventional, open aortic arch surgery was inevitable. A partial resection of the proximal part of the stent-graft was performed.

Implantation of a prosthesis mounted inside a self-expandable stent in the pulmonary valvar area without use of cardiopulmonary bypass.

Patients with right ventricular outflow tract reconstruction often require redo operations with time. Unique surgical problems exist in this group of patients. We report an innovative method of implantation of a pulmonary valve without the use of cardiopulmonary bypass.

Absent long-term benefit of patch versus linear reconstruction in left ventricular aneurysm surgery.

BACKGROUND: Endoventricular patch reconstruction of the left ventricle is considered the gold standard in surgery for left ventricular aneurysms, because of improved preservation of ventricular geometry. However, the superiority over conventional linear closure has not been demonstrated, as assessed by the long-term outcome. METHODS: Two hundred patients (66%) underwent linear closure (group L) and 105 patients (34%) had endoventricular patch reconstruction (group D) using the Dor technique. Linear closure has been performed since 1974 and from 1985 on the Dor technique has been applied as an alternative procedure. Both patient groups differed regarding age, sex distribution, site of infarction, and indication for surgery. Prior to the operation, 71% of the patients were in New York Heart Association (NYHA) class III or IV and mean ejection fraction was 34% +/- 12%. Follow-up extends up to 25 years, with a cumulative total of 2,605 patient years. RESULTS: Early mortality was 6.5% in group L vs 5.7% in group D (not significant [NS]). Actuarial survival after 10 years was 56 +/- 3.2%, with no difference between groups. Freedom from reoperation after 10 years was 95.6% in group L vs 95.2% in group D (NS). Preoperative risk factors for late mortality were age, left ventricular enddiastolic volume index and concomitant mitral valve surgery. The type of procedure and the date of operation had no influence on mortality. To date, 63% of the survivors are in NYHA class I and II. CONCLUSIONS: In regard to long-term survival, rate of reoperation, and postoperative NYHA functional class, no benefit could be demonstrated when linear closure was compared with ventricular patch reconstruction for LV aneurysm repair. Hence, the technique of ventricular reconstruction may not be as important as previously thought, and at least for small aneurysms the simple and time sparing technique of linear closure may still be considered.

Cardiac malignant tumor as a rare cause of acute myocardial infarction.

Acute myocardial infarction is predominantly caused by coronary artery atherosclerotic plaque rupture and subsequent occlusive thrombus formation. The recognition of less common causes of acute myocardial infarction is important because they may require a different treatment strategy. We report a patient with acute myocardial infarction without any angiographic evidence of coronary atherosclerosis and a left atrial mass detected on echocardiography. Therefore, coronary embolism from intracardiac thrombus or tumor was suspected. No additional manifestations of a potential tumor were found on thoracic, abdominal and cranial computed tomography. During subsequent cardiac surgery, a large tumor could be in toto resected and was diagnosed as a highly malignant leiomyosarcoma on histopathological evaluation.

[Rare case of enterovirus myocarditis with consecutive acute heart failure and subsequent "rescue therapy" with biventricular assist device]. / Seltene Form der Myocarditis mit akutem Herzversagen und erfolgreicher "Rescue-Tehrapie" mit biventikulärem Unterstützingssystem.

PATIENT HISTORY AND FINDINGS: A 37-year-old woman was admitted in cardiogenic shock and multiorgan failure. On echocardiography, left ventricular function was reduced, at that time, to 35%. The patient had been suffering from a cold for 1 week prior to admission. Within 24 h, left ventricular function dramatically decreased to 7%. Examinations included left-and right-heart catheter evaluation with removal of right ventricular biopsies. Histology and molecular pathology revealed the diagnosis of an enteroviral myocarditis. THERAPY: Imminently, a biventricular assist device (BVAD -Berlin Heart) was implanted. POSTOPERATIVE COURSE: Postoperatively, only low-dose inotropic support was required. Under the requested anticoagulation, recurrent bleeding necessitated three rethoracotomies. Both renal and liver function normalized over time. On postoperative day 25, the BVAD was explanted without the need for cardiopulmonary bypass. Global ventricular function had normalized. The implantation of BVAD proved to be an efficient rescue therapy.

Modified bypass procedure and apicoaortic conduit. Management of coronary artery disease, aortic valve stenosis and porcelain aorta.

BACKGROUND: In case of severely calcified ascending aorta, modified operative strategies are required in order to avoid manipulations of the aorta and minimize subsequent cerebral vascular accidents. CASE REPORT: A 73-year-old woman, with a coronary two-vessel disease and aortic stenosis was scheduled for coronary artery bypass grafting and aortic valve replacement. Due to severed calcification of the ascending aorta including the transverse arch, neither cannulation, clamping nor incision of the aorta or its replacement was feasible. Therefore bypass operation was performed using a modified approach. After 1 month, implantation of a valved conduit between the left ventricular apex and the descending aorta through a lateral thoracotomy followed. CONCLUSION: Only in few cases the surgical treatment of a coronary artery disease in combination with left ventricular outflow tract obstruction and heavily calcified ascending aorta has been described. Undoubtedly, creation of an apicoaortic connection is today only indicated in the adult population in a small collective with multiple previous operations or porcelain aorta.