C63-27 High Pressure, High Stakes: A Case of Rapidly Progressive Right Ventricular Failure

Abstract Rapidly progressive right ventricular failure is a distinct phenomenon that requires prompt recognition and treatment. While left sided heart failure remains the most common cause, there are numerous other causes that may cause significant morbidity and mortality.We present a case of a 58-year-old female with history of breast cancer presenting from home with six weeks of vaginal bleeding. She was found to be hypoxemic, tachypneic, and hypotensive. CT pulmonary angiogram was negative for pulmonary embolus, but revealed a dilated pulmonary artery as well as innumerable sub-centimeter pulmonary nodules in a random distribution. Echocardiogram showed severe dilation and reduced function of the right ventricle with estimated RVSP 104 mm Hg, and normal left ventricular function with ejection fraction 70%.The patient’s condition deteriorated with progressive shock and respiratory failure. She was started on intravenous epoprostenol at 10 ng/kg/min and inhaled epoprostenol in addition to vasopressor and inotrope support. Right heart catheterization demonstrated pulmonary pressure 86 mm Hg / 49 mm Hg with mean PA 64 mm Hg; pulmonary capillary wedge pressure 16 mm Hg; Fick cardiac index 1.1 L/min/m2, and pulmonary vascular resistance 23.66 Woods unit. Pulmonary artery (PA) aspirate was performed and was negative for malignant cells.The patient died on hospital day 15. Autopsy revealed high grade endometroid adenocarcinoma, with bilateral multiple metastatic tumor nodules (up to 0.8 cm) and intravascular tumor emboli. Cause of death was determined to be right ventricular failure secondary to pulmonary tumor thrombotic microangiopathy (PTTM) from metastatic endometrial carcinoma.PTTM is an ultimately fatal condition associated with migration of metastatic cells to the pulmonary vasculature. Vascular remodeling as well as vasoconstriction can lead to progressive pulmonary hypertension. Antemortem diagnosis is very challenging. While PA aspirate cytology has been noted as a useful diagnostic modality in case reports, our patient had a false negative PA aspirate cytology despite a high pre-test probability. The false negative PA aspirate cytology rate is not known due to the rarity of this condition. Despite aggressive PAH therapies, our patient died from progressive right ventricle failure, highlighting the nearly universal fatal nature of this disease process.To our knowledge, this is the second case report identifying PTTM in a patient with endometrial carcinoma. PTTM remains a rare and likely underdiagnosed cause of progressive PAH in patients with suspected malignancy; PA aspirate cytology may be used but false negative potential must be incorporated into the diagnostic work-up. This abstract is funded by: None