Atrial Mass in Pregnancy: A Rare Case of Intracardiac Ectopic Liver.

Although rare, an ectopic liver should be considered in the differential diagnosis for right-sided intracardiac mass. Initial workup includes TTE to localize and measure the size of the mass and to identify additional structural abnormalities. TEE provides a more precise view of the involvement of surrounding structures. Cardiac magnetic resonance (CMR) imaging may aid in better delineating tissue characteristics. TEE or CMR would allow one to determine whether the mass is thrombus or tumor with firm intracardiac attachment to guide the choice for the optimal treatment modality, for example, catheter thrombectomy vs surgical resection. Both TEE and CMR could also be used for operative planning to better characterize the mass and its relationship to the intrahepatic IVC, interatrial septum, tricuspid valve, or coronary sinus. For example, if the mass location compromises drainage on cardiopulmonary bypass, alternative cannulation strategies or hypothermic circulatory arrest may be necessary. 7 Depending on the extent of IVC involvement and need for reconstruction, a hepatobiliary surgeon may be necessary. 5 Several cases of intracardiac ectopic liver were reported in women of childbearing age with obesity. Doshi and coworkers 2 described a 46-year-old woman with morbid obesity, hypertension, and hyperlipidemia presenting with atrial fibrillation who was found to have a right atrial mass that was subsequently diagnosed as an ectopic liver following resection. Izzo and coworkers 3 described a 43-year-old woman with prediabetes presenting with syncope, found to have an ectopic liver at the RA-IVC junction. Trocciola and colleagues 4 presented a case of an obese 42-year-old woman presenting with palpitations who had an ectopic liver at the RA-IVC junction. Giritharan and colleagues 5 described a 30-year-old woman with endometriosis, obesity, and history of cholecystectomy presenting with chest pain and found to have a right atrial ectopic liver. These reported cases of ectopic intracardiac liver all appeared to occur in female patients with metabolic derangements, which have led prior authors to speculate about a potential hormonal contribution in the growth of ectopic intracardiac liver. With this mass presenting during pregnancy, this led us to inquire whether the hormonal changes of pregnancy influence the growth of heterotopic liver tissue. It is known that estrogen and progesterone levels rise during pregnancy with a substantial upregulation during the third trimester. These hormones are known to increase hepatic synthetic function during pregnancy. In addition, prolonged exposure to estrogen and androgens has been shown to induce regenerative and dysplastic hepatocellular changes. 8 Although data are limited, our case report again suggests that the hormonal changes of pregnancy may play a role in the development of ectopic liver. Although intracardiac ectopic liver tissue is extremely rare, it should be considered in the differential diagnosis of RA masses. Initial workup should begin with TTE, followed by TEE or CMR to better characterize the mass and to determine the extent of anatomic involvement to guide the optimal strategy for intervention. Ectopic liver is usually diagnosed on microscopic examination after resection. Surgical resection is also indicated, given the potential for malignant transformation, obstruction, and embolization.