ArticleClinical research in cardiology : official journal of the German Cardiac Society2026
Right-sided cardiogenic shock from acute pulmonary tumor thrombotic microangiopathy: a rare but deadly cardio-oncologic and metabolic emergency.
Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundPulmonary tumor thrombotic microangiopathy (PTTM) is a fatal but treatable condition characterized by the rapid development of pulmonary hypertension (PH) in patients with possibly unknown adenocarcinoma. PTTM is mostly diagnosed post-mortem and considered a rare disease since its acute onset and misdiagnosis provides significant diagnostic and therapeutic challenges.
methodsWe conducted a retrospective analysis of patients who presented with unclear sudden cardiac death and acute right heart failure that had an incidental very recent or unknown malignant cancer, identified eight patients with PTTM and reported the results. Patients were considered from 2009 to 2024 and analyzed at Ulm University Heart Center, Germany with the aim to describe the fatal consequences of unknown acute PTTM with right heart failure and discuss diagnostic and therapeutic strategies.
resultsThe median age was 47 years (41-84 years); gender was equally distributed. The latest median body mass index (BMI) was elevated with 28.4 kg/m
conclusionOur data suggest that the combination of elevated hsTnT, NT-proBNP, d-dimer, and HbA1c values in patients with unexplained acute right heart failure may indicate PTTM. Our findings also emphasize the diagnostic challenge posed by PTTM, and imply that targeted therapy, enabled by a timely diagnosis, may improve survival. Therefore, acute and fatal right heart failure in the adult in absence of coronary artery disease, pulmonary embolism, or any other apparent cause, especially in patients with uncontrolled metabolic syndrome, should prompt an urgent diagnostic work-up to rule out unknown cancer with treatable pulmonary tumor embolism, beginning with more extensive imaging (e.g., computed tomography (CT) and magnetic resonance tomography (MRI)), as well as laboratory diagnostics (e.g., tumor markers). In still inconclusive cases, lung biopsy and right heart catheterization should be considered eventually, if possible.
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