Search

BackgroundCardiovascular involvement is a rare but severe complication of Epstein–Barr virus (EBV) infections. Patients with chronic active EBV (CAEBV) are at increased risk of developing cardiovascular complications and have a poor prognosis. Here, we report the rare case of a pediatric patient with CAEBV and EBV- hemophagocytic lymphohistiocytosis (HLH) complicated with a giant coronary artery aneurysm (CAA) and thrombosis, a giant Valsalva sinus aneurysm, and ascending aorta dilation seven years after the disease onset.Case presentationA previously healthy 3-year-old girl was initially misdiagnosed as presenting incomplete Kawasaki disease complicated by coronary artery lesions (CALs) for which she received intravenous immunoglobulin and aspirin therapy. Subsequently, she was transferred to our hospital, where we diagnosed her as having a primary EBV infection. After acyclovir therapy, her clinical symptoms resolved with negative EBV-DNA, and she was discharged home with aspirin treatment for the remaining CALs. However, she did not have regular follow-ups after that. Seven years later, the 10-year-old girl developed a prolonged fever and fatigue, and she was diagnosed as presenting CAEBV and EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) due to the presence of a high EBV-DNA load, prolonged fever, splenomegaly, bicytopenia, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis, low NK-cell activity, and increased levels of ferritin and soluble CD25. The echocardiography images showed giant left and right coronary artery aneurysms, a giant Valsalva sinus aneurysm, and ascending aorta dilation. Her parents agreed to a therapy with intravenous immunoglobulin, methylprednisolone, antiplatelet, and anticoagulant, but not to the standard therapy of EBV-HLH. However, the cardiovascular complications, including CAAs and thrombosis, Valsalva sinus aneurysm, and aorta lesions, did not resolve. Three weeks later, the patient was finally discharged home asymptomatic. Unfortunately, one month after discharge, the fever recurred the girl. The guardian had refused treatment and took the patient home due to economic difficulties. During our subsequent follow-up visit, the girl subsequently passed away.ConclusionsWe reported the case of a pediatric patient with EBV infection who developed rare and fatal cardiovascular complications (CAAs and thrombosis, Valsalva sinus aneurysm, and aortic lesions) seven years after the onset of the infection. Clinicians should be aware of these complications during the long-term follow-up of patients with EBV infection, especially in patients with CAEBV and/or EBV-HLH.

BackgroundLymphocytic-variant hypereosinophilic syndrome (L-HES) is a form of reactive hypereosinophilia, most commonly associated with interleukin-5 over-production by clonal, most commonly CD3−CD4+CD2hiCD5hiCD45RO+ T-cells. Patients often present with predominant cutaneous and soft-tissue manifestations, while cardiovascular involvement is uncommon.MethodsWe reviewed the medical files of two L-HES patients followed in our center who developed serious vascular complications and performed a literature review for similar cases.ResultsPatient 1, a 52-year-old female, presented with an ischemic stroke secondary to left middle cerebral artery dissection after 10 years of indolent L-HES. Blood eosinophilia was controlled with oral corticosteroids (OCS), but OCS-tapering attempts with hydroxyurea and pegylated interferon failed, prompting the introduction of mepolizumab with rapid normalization. Patient 2, a 62-year-old female, had been asymptomatic for 10 years without treatment when a NSTEMI occurred, due to coronary artery occlusion secondary to a large cauliflower-aneurysm of the proximal aorta and aneurysmal dilatation of several coronary arteries, requiring semi-urgent surgical management. Aortic wall staining for eosinophil major basic protein showed eosinophils in the adventitia. Blood eosinophilia was controlled with OCS.ConclusionsPatients with apparently clinically benign L-HES may develop arterial complications, consisting in dissection and/or aneurysm dilatation of medium-to-large vessels with serious consequences. The value of performing regular vascular imaging and monitoring during follow-up has yet to be determined.

Resumen: Se describe la forma de presentación, el diagnóstico y la resolución quirúrgica de un caso poco frecuente de aneurisma de seno de Valsalva. Se presenta el caso de una paciente femenina de 64 años con antecedentes de dos cirugías, una por ductus arterioso a los 12 años y la otra por una ventana aortopulmonar a los 16 años. Se encontraba asintomática. En un control de ecocardiografía, se observa válvula aórtica trivalva, insuficiencia leve y dilatación aneurismática del seno de Valsalva izquierdo. Realizamos una angiotomografía, la cual informó de un gran aneurisma de 88,9 mm saliendo de la coronaria izquierda de dicho seno. El cateterismo mostró un aneurisma de seno de Valsalva izquierdo, sin lesiones angiográficas. Decidimos realizar una intervención quirúrgica con circulación extracorpórea (canulación aórtica, cava y seno coronario). La paciente presentaba un aneurisma de gran tamaño con hematoma en el seno de Valsalva sobre el ostium de la coronaria izquierda, se extrajo el hematoma y se observó tejido sanioso en vía de fistulizarse hacia la aurícula izquierda. Se realizó la técnica de Yacoub con preservación valvular y un reimplante coronario izquierdo, así como la exclusión del aneurisma. El ecocardiograma intraoperatorio mostró buena competencia valvular e insuficiencia leve. La mujer presentó buena evolución, por lo cual se le dio el alta al séptimo día. Fue un caso complejo debido a la patología en sí y por ser una reoperación cardiaca. Existen varias formas de resolución, todos los componentes del complejo de la raíz aórtica deben considerarse y la técnica debe adaptarse a cada paciente. Aunque el abordaje quirúrgico óptimo sigue siendo controvertido, la competencia a largo plazo de la válvula aórtica parece seguir siendo la principal preocupación de los aneurismas de seno de Valsalva. Abstract: We describe the diagnostic and surgical resolution of a rare case of Valsalva sinus aneurysm. Sixty four (64) year old female patient with history of two previous surgery, one due to ductus arteriosus at age of 12 and an aortopulmonary window at age 16. Echocardiography control shows a severe dilatation of the left Valsalva sinus in a trivalve aortic valve with trivial insufficiency. Angiotomography reported a large aneurysm of left Valsalva sinus (88.9 mm) with coronary left main involved. Coronariography showed the same as TC without angiographic coronary lesions. Heart team dessision was surgical resolution with extracorporeal circulation (aortic cannulation, cava and coronary sinus). Patient presented a large aneurysm with great amount of thrombus in the left Valsalva sinus. The hematoma and thrombus was removed. Yacoub technique was performed with valvular preservation and left coronary reimplantation and exclusion of aneurysm. The intraprocedure trans esofagic echocardiogram showed trivial aortic insufficiency. Patient was discharged at seven days after procedure. There are several techniques for this pathology resolution. All components of the aortic root complex must be considered, and the technique must be adapted to each specific case. Although the optimal surgical approach remains controversial, long-term competence of the aortic valve appears to remain the main concern of Valsalva sinus aneurysms surgical treatment.

Rupture of Valsalva sinus remains a very rare and deadly complication of Valsalva sinus aneurysm with a high mortality rate. We report here the case of a 47-year-old man who presented to the emergency department with acute exercise-induced dyspnea, chest pain, and fever. Transthoracic (TTE) and transesophageal echocardiography (TEE) highlighted a rupture of the right Valsalva sinus in the right atrium due to infective endocarditis. After stabilization of the patient, a successful surgical repair with double pericardial patches was performed.

Aorto-cardiac fistula (ACF) is a rare condition that is often complicated by perivalvular extension of infective endocarditis. Here we report the case of 43-year-old woman with aorto-left ventricle fistula in aortic valve endocarditis, whose echocardiographic manifestations mimicked a ruptured Valsalva sinus aneurysm. The patient was treated successfully with aortic valve replacement and repair of aorto-left ventricle fistula. Based on this rare case, we analyze the factors of misdiagnosis and offer suggestions to improve the diagnostic accuracy of ACF.

Rupture of Valsalva sinus remains a very rare and deadly complication of Valsalva sinus aneurysm with a high mortality rate. We report here the case of a 47-year-old man who presented to the emergency department with acute exercise-induced dyspnea, chest pain, and fever. Transthoracic (TTE) and transesophageal echocardiography (TEE) highlighted a rupture of the right Valsalva sinus in the right atrium due to infective endocarditis. After stabilization of the patient, a successful surgical repair with double pericardial patches was performed. [ABSTRACT FROM AUTHOR]

This report describes a 58 -year -old patient with a giant unruptured asymptomatic aneurysm of right valsava sinus, as well as the significant contribution of magnetic resonance imaging in diagnosis of this cardiovascular disorder and identification of its relationship with surrounding thoracic organs. [Copyright &y& Elsevier]

The article discusses the use of an angioscopic view of a three-dimensional (3D) computed tomographic angiography (CTA) to detect an aneurysm arising from the left sinus of Valsalva in a man who presented with exertional chest pain and a mediastinal mass. It mentions the CTA angioscopic image's confirmation that the aneurysm did not involve the aortic valve or the left coronary ostium. The role of CTA angioscopic images in surgical planning for complex aortic root pathology is cited.