Abstract
BackgroundPulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of pulmonary hypertension that is associated with malignancies and is marked by the presence of non-occlusive tumor emboli and fibrocellular intimal proliferation of small pulmonary arteries leading to increased pulmonary vascular resistance and right heart failure. The diagnosis of PTTM is challenging to make pre-mortem and guidelines on treatment are lacking.Case presentationA 45-year-old woman with advanced squamous cell carcinoma of the cervix developed symptoms of dyspnea and evidence of right heart failure during a phase I clinical trial with cediranib and durvalumab. After an extensive evaluation, pre-capillary pulmonary hypertension was confirmed by right heart catheterization. Vasodilator therapy was initiated but resulted in the development of symptomatic hypoxemia and was discontinued. Despite continued supportive care, she continued to decline and was transitioned to hospice care. At autopsy, the cause of her right heart failure was found to be due to PTTM with features of pulmonary veno-occlusive disease (PVOD).ConclusionPTTM and PVOD are important diagnoses to consider in patients with a malignancy and the development of right heart failure and may be manifestations of a spectrum of similar disease processes.
Highlights
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of pulmonary hypertension that is associated with malignancies and is marked by the presence of non-occlusive tumor emboli and fibrocellular intimal proliferation of small pulmonary arteries leading to increased pulmonary vascular resistance and right heart failure
PTTM and pulmonary veno-occlusive disease (PVOD) are important diagnoses to consider in patients with a malignancy and the development of right heart failure and may be manifestations of a spectrum of similar disease processes
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare condition characterized by microscopic tumor cell emboli, which cause proliferative changes in the pulmonary microvasculature leading to a syndrome of hypoxemia, pulmonary hypertension, right heart failure and death [1]
Summary
In the setting of malignancy, PTTM should be included in the differential diagnosis of a patient that presents with subacute to chronic pulmonary hypertension. Serial echocardiography may be useful for identifying evidence of pulmonary hypertension or right ventricular dysfunction prior to the onset of severe symptoms. These findings are non-specific and there are no established criteria for screening patients who are at higher risk for developing PTTM. This case report illustrates that a pre-mortem diagnosis of PTTM is difficult to confirm, treatment guidelines are lacking and the prognosis is poor.
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