CONGRESS ABSTRACT

A 53-year-old female with Crohn’s disease (CD) presented with suspected pulmonary hypertension (PH) and an infiltrative phenotype cardiomyopathy.  The patient was initially treated with infliximab, but due to intolerance, this was suspended and ustekinumab was introduced. Subsequently, mesalazine was administered. Abdominal ultrasound revealed liver cirrhosis, while cardiological assessment showed HFpEF with ground glass interventricular septum. Right heart catheterization confirmed combined post- and pre-capillary PH (CpcPH) (mPAP 45 mmHg, PVR 5 W.U., PCWP 22 mmHg). Key findings included evidence of normal coronary arteries on angiographic study, negative serum amyloid A assay (SAA), monoclonal protein on electrophoresis, and inconclusive cardiac MRI. Bone tracer scintigraphy ruled out transthyretin amyloidosis, while V/Q scintigraphy confirmed chronic thromboembolism. Chest CT identified interstitial lung disease (ILD). Porto-Pulmonary Hypertension (PoPH) was ruled out according to endoscopy findings that excluded portal hypertension (Humbert et al., 2022). Chronic thromboembolism, which is very frequent due to the pathophysiology of CD (Sugiyama et al., 2019) couuld explain the precapillary component of PH (Humbert et al., 2022). Beside ILD could be an extraintestinal manifestation or a a side effect of mesalazine treatment. Increased PCWP could be explained by HFpEF, indeed, individuals with IBD have a higher risk of hospitalization for HF compared to the general population (Kristensen et al., 2014; Sun et al., 2024). HFpEF and CD share mechanisms such as inflammation, endothelial dysfunction, resulting in myocardial fibrosis, and impaired cardiac relaxation (Pugliese et al., 2022) even if data on the association between CD and HFpEF are inconsistent (Sun et al., 2024). Therefore, another hypothesis investigated to correlate CD with the increased PCWP was the AA Amyloidosis, hypothesis supported by echocardiographic findings, even if SAA assay was negative. Indeed, it may be negative when the disease is in a quiescent phase (Georgin-Lavialle et al., 2023). Further diagnostic evaluations, including abdominal fat biopsy and bone biopsy, are underway to confirm diagnosis. Conclusion: in this case a different potential link between heart, lung and gut could justify the emodinamic condition of CpcPH. Even if several extraintestinal CD manifestations, data on the association between CD and HFpEF are inconsistent.