Abstract
TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: The Fontan procedure is a palliative surgical procedure performed in children ages 2-4 years old with hypoplastic right heart syndrome by relocating the inferior vena cava from the right atrium to the right pulmonary artery to complete the Fontan circuit[1][2]. This circuit allows for normalization of arterial saturation and abolishment of chronic volume overload at the cost of decreased cardiac output.[1] This adjustment allows for almost all venous blood to return to heart through the right pulmonary artery[3]. We present a case below in a patient with hypoplastic right heart treated with the Fontan procedure who was later diagnosed with testicular choriocarcinoma with hematogenous metastasis through the right pulmonary artery. CASE PRESENTATION: An 18-year-old male with a past medical history of heterotaxia, hypoplastic right heart treated with Fontan palliation and Kawashima surgery initially presented for persistent, worsening dyspnea. On further questioning the patient reported an active lifestyle but over the past several weeks had begun to develop dyspnea with limited ambulation and persistent cough. On presentation, the patient was found to be in acute hypoxic respiratory failure. Computed tomography (CT) imaging of the chest, abdomen and pelvis showed a new large right retroperitoneal mass causing hydronephrosis as well as innumerable right sided pulmonary nodules concerning for metastatic disease. Biopsy of the retroperitoneal mass revealed germ cell tumor, with choriocarcinoma heavily present in the tissue. The patient underwent MRI of the brain for staging, which subsequently revealed numerous embolic strokes. He was started on a chemotherapy regimen of etoposide, ifosfamide, and cisplatin; however, his course was complicated by COVID-19 infection which caused further deterioration in his respiratory status. He unfortunately passed away from respiratory failure. DISCUSSION: In this case, the Fontan procedure provided the patient with excellent oxygenation as the patient was able to exercise and live relatively unbound by his complex cardiovascular history. However, this procedure served as the conduit that allowed his testicular choriocarcinoma to hematogenously spread and predominately seed his right lung. Hospitalization was further complicated by active COVID-19 infection and in concert with his poor lung status, the patient passed. CONCLUSIONS: This case highlights the unique anatomy and physiology of patients treated with the Fontan circulation. This life saving surgery performed in early childhood unfortunately served as the conduit for hematogenously spread of choriocarcinoma. As a consequence of the surgery, the right pulmonary artery receives all the venous return which then allowed the malignancy to spread predominately to the right lung. REFERENCE #1: Gewillig M. The Fontan Circulation. Heart. 2005;91:839-846 REFERENCE #2: Kutty S, Jacobs M, Thompson R, Danford D. Fontan Circulation of Next Generation: Why It's Necessary, What It May Look Like. JAHA. 2020;9:e01 3691 REFERENCE #3: Cowgill LD. The Fontan Procedure: A Historical Review. Ann Thoracic Surgery. 1991;51:1026-30 DISCLOSURES: No relevant relationships by Arjan Ahluwalia, source=Web Response No relevant relationships by Martin Delatorre, source=Web Response No relevant relationships by Kaitlyn Musco, source=Web Response No relevant relationships by Alina Zhu, source=Web Response
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