1205-262 - TUMORAL PULMONARY HYPERTENSION SECONDARY TO GI MALIGNANCY

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Author Block: Rachel Wester, Lindsay M. Castle, OhioHealth, Columbus, OH, USA
Background: Tumoral pulmonary hypertension (PH) as a result of an underlying malignancy is a rare disease that is underdiagnosed but associated with high mortality. Pulmonary tumor thrombotic microangiopathy (PTTM) is a broadly defined category of PH that encompasses microvascular tumor emboli, tumor thrombotic microangiopathy, and macrovascular tumor obstruction within the pulmonary circulation. There have been scarce case reports that describe tumor microemboli from breast and pulmonary malignancy with most in patients with advanced age. Literature review shows rare incidence of tumoral PH from GI malignancy, especially in young patients. We report a rare case of a 28 yo patient with likely tumoral PH from presumed GI malignancy.
Case: A 28 yo female with no past medical history presented to the emergency department with complaints of 3 syncopal events over one month. She appeared acutely ill with hypoxia. A CT of her chest, abdomen and pelvis showed dilation of the main pulmonary artery, marked dilation of the right atrium and right ventricle. This was confirmed on TTE. This study also showed mediastinal and retroperitoneal adenopathy. She was taken for emergent right heart catherization that showed low cardiac index of 1.14 and PA pressure of 68/13, PVR of 11 and a PCWP of 4.
Decision‐making: Unfortunately, providers felt that her thrombocytopenia prohibited use of VA-ECMO. She was started on milrinone and inhaled epoprostenol and transitioned to IV treprostinil and oral sildenafil. The typical workup for PH was unremarkable. Her CA 19-9 was markedly elevated. Lymph node biopsy showed adenocarcinoma with signet ring features and GI malignancy workup was recommended. However prior to this, she experienced a PEA arrest and ROSC was unable to be obtained.
Conclusion: The precise mechanism of PH in patients with malignancy remains to be explored. Histopathological diagnosis with tissue sampling is the gold standard. The optimal management of PTTM is unclear but prior case reports discuss supportive care, chemotherapy, steroids, oxygen and PAH medications. PTTM is an often overlooked cause of PH and clinicians should exercise a high index of suspicion to facilitate prompt diagnosis and treatment.