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Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia.

ABSTRACT: Unusual forms of pulmonary hypertension include pulmonary hypertension related to mediastinal fibrosis and the use of serotonergic drugs. Here, we describe a patient with diffuse mediastinal fibrosis and pulmonary hypertension while she was on dopamine agonist therapy. A young woman, who was treated with cabergoline and bromocriptine for hyperprolactinemia, presented with progressive dyspnea over several months. Based on the clinical investigation results, in particular, elevated pulmonary arterial pressures and significant perfusion defects on computed tomography (CT) pulmonary angiography and ventilation/perfusion (V/Q) scintigraphy, chronic thromboembolic pulmonary hypertension (CTEPH) was initially considered the most plausible diagnosis. However, during an attempted pulmonary endarterectomy, loose fibrous tissues were observed in the mediastinum and cryosection of the right pulmonary artery showed fibrosis and chronic inflammation. Subsequent investigations revealed that diffuse mediastinal fibrosis with concurrent pulmonary hypertension, and not CTEPH, was the most likely diagnosis and cabergoline and bromocriptine may have triggered the fibrotic changes. Both drugs are ergot-derived dopamine agonists, which are known to cause cardiac valve fibrosis and less frequently, non-cardiac fibrotic changes. The underlying mechanism is attributed to their interactions with serotonin receptors. There is much evidence that serotonin, a potent vasoconstrictor and mitogen, is involved in the pathogenesis of pulmonary hypertension. In conclusion, as CT and V/Q scintigraphy findings can occasionally be deceptive, physicians should be particularly aware of differential diagnoses in patients without obvious history of venous thromboembolism that are suspected of having chronic thromboembolic pulmonary hypertension.

SUBMITTER: Su J

PROVIDER: S-EPMC5517450 | biostudies-other | 2017

REPOSITORIES: biostudies-other

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Project description:Abstract Celiac artery (CA) occlusion or stenosis is identified in up to almost half of all patients undergoing abdominal angiography, and the resulting increased collateral blood flow from the superior mesenteric artery to the pancreaticoduodenal artery (PDA) may cause PDA aneurysms (PDAAs). PDAAs are rare but could be fatal if they rupture. However, treatment of the PDAA could block this important collateral blood flow pathway, leading to ischemic organ damage. Treatment of such aneurysms is therefore difficult, especially in patients with multiple PDAAs. Successful treatment of PDAAs requires establishing blood flow in the CA region and selecting which aneurysm(s) to treat. We present four patients who underwent surgery for unruptured PDAAs caused by CA obstruction. Blood flow in the CA region was established by bypassing the splenic artery and by anastomosing it either directly to the left renal artery (n = 1) or to the abdominal aorta using a graft (saphenous vein: n = 1; artificial vessel: n = 2). Three patients had multiple PDAAs: all PDAAs were treated in one patient with PDAAs of similar size and shape, but only the largest PDAA with the highest risk of rupture was treated in the other two patients to simplify the procedure. The median observation period was 19.5 months (range: 11‐28 months), and all patients were alive without recurrence at the time of writing. Surgical treatment including splenic artery bypass may thus be a viable option for treating patients with unruptured PDAAs. Since celiac artery occlusion represents a common attributable aetiology for these aneurysms, restoration of blood flow to the regions supplied by the celiac artery should also be taken into account when designing a treatment plan. Here, we present 4 cases of PDA aneurysms coexisting with coeliac artery occlusion, all of which were managed with bypass surgery using the splenic artery. Furthermore, in this study, we showed that preserving either arcade of the PDA without treating a small aneurysm may provide a secondary pathway and avoid complications in case of bypass problems.

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Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia.

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