Carcinoid heart disease from ovarian primary presenting with acute pericarditis and biventricular failure

A case is described of a 54 year old woman who had acute pericarditis with large exudative effusion accompanied by severe right and left ventricular failure. The patient was finally diagnosed with carcinoid heart disease from an ovarian carcinoid teratoma. She was treated with octreotide--a somatostatin analogue--followed by radical surgical resection of the neoplasm. At one year follow up only mild carcinoid tricuspid regurgitation remained. Only 16 cases of carcinoid heart disease from an ovarian primary have been described in literature. Moreover clinically manifest acute, nonmetastatic pericarditis and left heart failure are not considered as possible presentations of carcinoid heart disease, whatever the origin. In a recent series a small pericardial effusion was considered an infrequent and unexpected echocardiographic finding in carcinoid heart patients. One case of "carcinoid pericarditis" has previously been described as a consequence of pericardial metastasis. Left sided heart involvement is usually caused by bronchial carcinoids or patency of foramen ovale; both were excluded in the case presented.     

Cardiac Sarcoidosis Presenting as Constrictive Pericarditis

In patients with cardiac sarcoidosis, the sarcoid granulomas usually involve the myocardium or endocardium. The disease typically presents as heart failure with ventricular arrhythmias, conduction disturbances, or both. Constrictive pericarditis has rarely been described in patients with sarcoidosis: we found only 2 reports of this association.We report the case of a 57-year-old man who presented with clinical and hemodynamic features of constrictive pericarditis, of unclear cause. He was admitted for treatment of recurrent pleural effusion. After a complicated hospital course, he underwent pericardiectomy. His clinical and hemodynamic conditions improved substantially, and he was discharged from the hospital in good condition. The pathologic findings, the patient''s clinical course, and his response to pericardiectomy led to our diagnosis of cardiac sarcoidosis presenting as constrictive pericarditis. In addition to the patient''s case, we discuss the nature and diagnostic challenges of cardiac sarcoidosis. Increased awareness of this disease is necessary for its early detection, appropriate management, and potential cure.     

LEFT HEART INVOLVEMENT WITH CARDIAC SHUNT COMPLICATING CARCINOID HEART DISEASE

The manifestations of carcinoid heart disease are usually right heart failure due to pulmonary and tricuspid valve disease and sometimes high output failure due to peripheral vasodilation. Recently there has been one case report of progressive central cyanosis due to right to left shunting through a patent foramen ovale.¹ We present a similar case where such a shunt developed and was also complicated by significant valvular disease affecting the left side of the heart.     

Left Atrial Myxoma Causing Coronary Steal: An Atypical Cause of Angina

Cardiac myxomas are rare primary cardiac tumors that usually present with dyspnea or manifestations of systemic embolization. Coronary steal is a rare phenomenon of unbalanced blood flow that is seen primarily in patients who have undergone coronary artery bypass grafting and have subclavian artery stenosis.We report the case of a 72-year-old woman who presented with fatigue, weakness, and exertional chest heaviness and had abnormal results on a cardiac stress test. The results of coronary angiography showed no obstructive coronary artery disease but revealed a large intracardiac left atrial mass that was supplied by 2 anomalous coronary arteries. The patient underwent successful ligation of the anomalous coronary arteries and resection of the mass, which was histologically an atrial myxoma. The patient''s symptoms resolved, and results of a repeat cardiac stress test were normal.To our knowledge, this is the first report of a highly vascularized atrial myxoma that caused coronary steal with objective evidence of ischemia, and with subsequent resolution after resection of the mass and ligation of the anomalous coronary arteries.     

The clinical presentation and management of carcinoid heart disease

Carcinoid heart disease is a major cause of morbidity and mortality in patients with metastatic neuroendocrine tumours (NETs). Although cases of carcinoid syndrome and severe carcinoid heart disease requiring urgent intervention are well described, many patients with significant carcinoid heart disease may have insidious symptoms or even be asymptomatic. As haemodynamically significant carcinoid heart disease may be clinically silent, specific and individualised considerations must be made as to the most appropriate clinical criteria and time point at which surgical valve replacement should be undertaken in patients with carcinoid heart disease.     

Benign Metastasizing Leiomyoma in the Heart of a 45-Year-Old Woman

We report a rare case of benign metastasizing leiomyoma in the heart of a 45-year-old woman 2 years after a uterine leiomyoma had been discovered during hysterectomy. Computed tomograms at presentation showed a large mixed cystic mass in the pelvis and bilateral lung nodules suggestive of metastatic disease. A large cardiac mass, attached to the chordae of the tricuspid valve and later shown to be histopathologically consistent with uterine leiomyoma, was successfully resected through a right atriotomy. This case suggests that benign metastasizing leiomyoma should be considered in the differential diagnosis of right-sided cardiac tumors.     

Recurrent Primary Cardiac Lymphoma on Aortic Valve Allograft: Implications for Therapy

Primary malignant cardiac lymphomas associated with grafts are extremely rare: to our knowledge, only 6 cases of prosthesis-associated B-cell lymphoma have been reported. Ours is the first report of recurrent diffuse large B-cell lymphoma associated with aortic valve allografts.We treated a 60-year-old man who presented in early 2007 with aortic valve endocarditis. He underwent aortic valve replacement with an allograft; the resected native valve showed active endocarditis without tumor. In January 2011, the patient underwent repeat aortic valve replacement because of symptomatic aortic regurgitation. The explanted valve specimen displayed diffuse large B-cell lymphoma. In September 2011, the patient presented with fever and a mass around the aortic valve. He died in January 2012. On autopsy, the explanted replacement valve displayed recurrent diffuse large B-cell lymphoma. The recurrent lymphoma on a new graft leads us to believe that this tumor is more aggressive than had been thought. We propose early systemic chemotherapy, in addition to tumor resection, for the possibility of a better prognosis. We discuss our patient''s case and review the relevant medical literature.     

Large Lipomatous Hypertrophy of the Interventricular Septum

We present the case of a 58-year-old woman who had large lipomatous hypertrophy of the interventricular septum, a condition that is reported very infrequently. Preoperative cardiac magnetic resonance images revealed an inhomogeneous, infiltrating mass that was suppressed in fat-suppression mode. The extensive mass was causing right ventricular dysfunction, so we excised it through a right ventricular approach. The findings on histologic analysis of the mass were consistent with lipomatous hypertrophy. The patient died of septic shock on the 28th postoperative day. In addition to the patient''s case, we discuss the characteristics and diagnosis of this rare entity.     

Percutaneous balloon valvuloplasty in carcinoid pulmonary valve stenosis

More than half of patients with carcinoid syndrome develop carcinoid valve disease. Both the tricuspid and pulmonary valve are often involved. Symptoms of carcinoid syndrome with flushing, diarrhea, and bronchospasm often precedes cardiac symptoms. We report a case of carcinoid initially presenting with rapid development of right heart failure due to severe pulmonary valve stenosis. In untreated carcinoid, there is a risk of carcinoid crisis with anesthesia and surgery. In local anesthesia, we performed a sub‐acute balloon pulmonary valvuloplasty. The procedure was successful without any residual pulmonary valve stenosis and with immediately relief of dyspnea. The final diagnostic workup for the underlying malignancy continued the day after valvuloplasty. © 2015 Wiley Periodicals, Inc.     

Fractured Inferior Vena Cava Filter Strut Presenting with ST-Segment Elevation and Cardiac Tamponade

The fracture of an inferior vena cava filter strut and its migration to the heart is a rare sequela of implanted inferior vena cava filters. Perforation through the right ventricle into the pericardium with resultant cardiopulmonary compromise is even less frequent. We report the case of a 53-year-old man who presented with chest pain and hypotension consequent to cardiac tamponade. A fractured inferior vena cava filter strut had migrated and perforated his right ventricle. The fractured strut was successfully removed by means of cardiac surgery. Inferior vena cava filters should be placed when necessary to minimize the risk of pulmonary embolism, and regular radiologic monitoring should be performed; however, the eventual extraction of retrievable filters should be considered. In addition to discussing the patient''s case, we briefly review the relevant medical literature.     

Atypical presentation of a carcinoid syndrome]

A 76-year-old woman presenting with right heart failure is reported. The investigation showed an ileal carcinoid tumour with multiple metastases and tricuspid regurgitation caused by a cardiac carcinoid lesion. Echocardiographic study and histopathological examination revealed typical characteristics of carcinoid heart disease. Nevertheless, except for the cardiac symptomatology, the patient did not show any other clinical manifestation of the carcinoid syndrome. Isolated right heart failure as the first sign of a carcinoid syndrome is rare; only two other cases have been described in the literature.     

An unusual cause of carcinoid heart disease

Carcinoid heart disease (CHD) is a rare form of valvular heart disease characterized by right-sided heart valve dysfunction. Carcinoid heart disease occurs most frequently when carcinoid tumour cells metastasize from a primary site in the gut to the liver, so that vasoactive substances produced by the tumour are able to reach the systemic circulation. By contrast, in ovarian carcinoid tumours carcinoid heart disease develops in the absence of liver metastasis, because vasoactive substances can be released directly into the systemic circulation, bypassing the first-pass metabolism of the liver. There are only a few case reports in the world literature of carcinoid heart disease caused by ovarian carcinoid tumour. We report a case of an 85-year-old woman with carcinoid heart disease caused by a bilateral ovarian carcinoid tumour metastasized from a primary ileal site.     

Carcinoid heart disease. A unique case of postvalvotomy diagnosis

Carcinoid heart disease is a rare entity and has always been hallmarked by its late appearance in the malignant carcinoid syndrome. Most cases are discovered when patients complain of typical symptoms, including flushing and diarrhea, with subsequent heart failure and valvulopathy developing years later. Only a few case reports of successful valve replacements have appeared in the literature, and the cause of the valvulopathy in those patients was known at surgery. I discuss herein a unique case of carcinoid heart disease that was diagnosed postoperatively.     

Carcinoid Heart Disease in Association with a Primary Ovarian Carcinoid Tumor: Diagnostic Role of Echocardiography

We describe a patient with carcinoid heart disease secondary to a primary ovarian carcinoid tumor who initially presented with unexplained right-sided heart failure. The patient did not exhibit any of the typical clinical manifestations of the carcinoid syndrome. Echocardiography demonstrated typical features of carcinoid heart disease and played an important role in the detection and management of a condition that was previously unrecognized.     

Ochronotic Involvement of the Aortic and Mitral Valves in a 72-Year-Old Man

Ochronosis, an autosomal recessive metabolic disorder, causes an excess of homogentisic acid that results in adverse pigmentation, calcification, and inflammation of cartilaginous and other tissues. Cardiovascular abnormalities are less frequently reported than are other manifestations. In rare cases, ochronosis can cause valvular heart disease. We report the case of a 72-year-old man with aortic stenosis and mitral insufficiency who was diagnosed with ochronosis while undergoing surgical aortic and mitral valve replacement. We discuss the history and surgical management of alkaptonuric ochronosis.     

Multidetector Computed Tomographic Imaging of Erdheim-Chester Disease

Erdheim-Chester disease is a rarely reported disease that can affect nearly every organ and chiefly infiltrates the connective, perivascular, and adipose tissue. The disease is a form of non-Langerhans-cell histiocytosis characterized by the proliferation of foamy histiocytes; its cardiovascular complications carry a severe prognosis. We present the case of a 29-year-old woman who was admitted for analysis of her angina. Our evaluation with use of cardiac multidetector computed tomographic angiography revealed large mediastinal soft tissue that compressed the patient''s left anterior descending coronary artery. To our knowledge, this is the first report of the use of low-dose, dual-source, 256-slice multidetector computed tomography to characterize Erdheim-Chester disease that exclusively caused angina and stenosis of a coronary artery in a young adult.     

Carcinoid heart disease: typical findings on echocardiography and cardiac magnetic resonance

Carcinoid syndrome is often complicated by carcinoid heart disease. Deposition of carcinoid plaques on the endocardium of the tricuspid and pulmonary valves causes the typical echocardiographic iconography with thickening of the leaflets and valvular insufficiency and/or stenosis leading to right heart failure. Additionally, the fibrous plaques can be visualized with cardiac magnetic resonance using delayed enhancement sequences. Accurate assessment of the dimensions of the dilated right ventricle by magnetic resonance implicates therapeutic options and defines prognosis. We report the case of a patient with advanced right heart failure as the clue to the diagnosis of carcinoid syndrome. Echocardiography was suggestive. Advanced investigation with magnetic resonance confirmed carcinoid plaque deposition on the tricuspid and the pulmonary valves, and we also had evidence of plaque deposition in the right ventricle. Multimodality imaging is essential in the investigation of this rare disorder.     

Fetal Left Ventricular Apical Aneurysm Progressing to Dilated Cardiomyopathy Due to Glycogen Storage Disease

Fetal dilated cardiomyopathy is a rare anomaly characterized by ventricular dilation and dysfunction. Its causes are diverse, and its outcomes are generally dismal. We describe a rare case of prenatally diagnosed left ventricular apical aneurysm that progressed rapidly to dilated cardiomyopathy. At age 2 months, the infant underwent heart transplantation. Pathologic examination of the explanted heart revealed that the cause of the dilated cardiomyopathy was glycogen storage disease. This case highlights the crucial roles of timely diagnosis, frequent close monitoring, and multidisciplinary care in achieving a successful postnatal outcome.     

Silent Kawasaki Disease Affecting Multiple Coronary Arteries in a 39-Year-Old Egyptian Woman

Kawasaki disease, an acute febrile illness, can cause vasculitis in the coronary arteries. It is the chief acquired cause of myocardial infarction and sudden cardiac death in infants, children, and young adults in developed countries. We report a case of chronic, silent Kawasaki disease complicated by multivessel thrombosis in a 39-year-old Egyptian woman. The patient presented with progressive, unstable angina but was otherwise asymptomatic and at negligible risk of ischemic heart disease. Coronary angiograms showed critical arterial stenosis with multiple aneurysms. During revascularization surgery, the patient''s harvested left internal mammary artery was found to have occlusive lesions and aneurysmal areas that made it unfit for bypass grafting, and subsequent histopathologic examination revealed features characteristic of chronic Kawasaki disease–associated systemic vasculitis.We think that this is only the second report of Kawasaki disease in the Arabian Mediterranean region. In addition to the patient''s case, we discuss the epidemiology and management of Kawasaki disease, in hopes of increasing clinicians'' awareness.     

不同病因所致心力衰竭的预后分析

目的：探讨不同病因所致心力衰竭（心衰）对患者预后的影响。方法：回顾性分析2007年1月~2010年12月兰州大学第一医院心内科收治住院的556例心衰患者的临床资料,并进行电话随访,确认患者是否死亡。根据引起心衰的病因不同将研究对象分为冠心病组、高血压病组、扩张型心肌病组（扩心病组）、心脏瓣膜病组和多病因组。Cox比例风险模型评价不同病因组患者的预后差异,并建立Kaplan Meier生存曲线。结果：共纳入分析患者513人,截止随访结束,死亡231人,占45%;不同病因组患者的年龄、心功能分级、左室射血分数等指标有明显差异（P〈0.05）;多因素Cox比例风险模型分析显示冠心病组和扩心病组的死亡风险分别为心脏瓣膜病组的2.517倍（P〈0.05）和2.480倍（P〈0.05）。结论：心衰患者不同病因的预后不同。