Journey from Right Atrial Pseudoaneurysm to Cardiac Angiosarcoma: A Case Report

6113 

Cardiothoracic Resident Case Report Competition 

Salman Zaheer (1), Alice Zhou (2), John Gross (3), Ahmet Kilic (4)

(1) Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland, (2) Johns Hopkins University School of Medicine, Baltimore, Maryland, (3) Bone and Soft Tissue Pathology, The Johns Hopkins Hospital, Baltimore, MD, (4) Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, United States

Salman Zaheer    -  Contact Me
Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital

Alice Zhou    -  Contact Me
Johns Hopkins University School of Medicine

John Gross    -  Contact Me
Bone and Soft Tissue Pathology, The Johns Hopkins Hospital

*Ahmet Kilic    -  Contact Me
Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital

Salman Zaheer    -  Contact Me
N/A

We present the case of a 46-year-old male who presented with chest pain for nine days, intermittent fevers, and dyspnea. The patient underwent computed tomography (CT) angiography of his chest that showed a moderate sized pericardial effusion with high density concerning for hemopericardium, along with an outpouching on the right atrial wall with a narrow neck concerning for a pseudoaneurysm. Transesophageal echocardiogram (TEE) redemonstrated the pericardial effusion and right atrial pseudoaneurysm. Given the concern for rupture into the pericardial space, the patient was taken to the operating room for resection of the pseudoaneurysm. The operation was performed via median sternotomy with cardiopulmonary bypass. After meticulous lysis of dense pericardial adhesions, central cannulation was performed, and the aorta and right atrium were cannulated. Right atrial pseudoaneurysm was identified and excised with the surrounding atrial wall. The defect in the atrial wall was closed using a bovine pericardial patch. The patient's post-operative course was unremarkable, and he was discharged home on post operative day 4. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma. The patient is currently undergoing chemotherapy for metastatic cardiac angiosarcoma.

Cardiac angiosarcomas are a rare but aggressive form of malignancy. They most commonly arise from the right atrium, and patients often present with nonspecific symptoms such as chest pain, dyspnea, and weight loss. Over half of patients present with metastatic disease, with spread to the lungs or pleura being most common. CT scan often shows a large multilobular mass that comprises the majority of the right atrium. Transthoracic echocardiography (TTE) often shows a large echogenic mass with poorly defined borders. Without surgical resection, average survival is about 4 months. With surgical resection, median post-operative survival is 14 months. A multidisciplinary treatment approach involving local resection, systemic chemotherapy, and radiation therapy is the most favorable strategy.

Our case represented a diagnostic challenge and initially presented on imaging as a right atrial pseudoaneurysm with a narrow neck, as opposed to a large multilobular mass with broad-based attachment. Cardiac angiosarcoma presenting initially on imaging as a right atrial pseudoaneurysm has only been reported in a handful of cases. Diagnosis was further delayed by the challenging histologic presentation in the context of low clinical suspicion, with malignant cells being obscured by extensive fibrin, granulation tissue, and organizing thrombus. Given these difficulties, diagnosis was not made until after distant metastatic spread. The unique presentation of this case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium. With an aggressive disease course, early diagnosis is vital and allows for multidisciplinary care with medical and radiation oncology to offer patients the best chances of survival.

Adult Cardiac