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Pulmonary veno-occlusive disease (PVOD) is rare condition which can lead to severe pulmonary hypertension, right ventricular dysfunction, and cardiopulmonary failure. The diagnosis of PVOD can be challenging due to its nonspecific symptoms and its similarity to idiopathic pulmonary arterial hypertension and interstitial lung disease in terms of diagnostic findings. This case describes a 57 year old female patient who presented with a 5-month history of progressive dyspnea on exertion and nonproductive cough. Workup at another hospital was nonspecific and the patient underwent surgical lung biopsy due to concern for interstitial lung disease. She subsequently became hemodynamically unstable and was transferred to our hospital where she presented with severe hypoxemia, hypotension, and suprasystemic pulmonary artery pressures. Preliminary lung biopsy results suggested idiopathic pulmonary arterial hypertension and the patient was started on vasodilating agents, including continuous epoprostenol infusion. Pulmonary artery pressures decreased but remained suprasystemic and the patient did not improve. Final review of the biopsy by a specialized laboratory revealed a diagnosis of PVOD after which vasodilating therapy was immediately weaned off. Evaluation for dual heart-lung transplantation was begun. The patient's hospital course was complicated by hypotension requiring vasopressors, worsening right ventricular dysfunction, and acute kidney injury. During the transplantation evaluation, the patient decided that she did not want to undergo continued attempts at stabilization of her progressive multi-organ dysfunction and she was transitioned to comfort care. She expired hours after removing inotropic support.

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Background Patients (pts) with acromegaly (A) require long term follow up, as up to 15% will develop recurrence. Current guidelines for MRI surveillance recommend 12 week post-operative (postop) imaging for all pts and yearly if on pegvisomant (PEG). Many pts with residual tumor postop undergo repetitive imaging even when controlled with pituitary (PIT) directed therapies. However, gadolinium retention and healthcare costs are of increased concern. Aim Assess tumor growth postop and necessity of serial MRI in medically treated A pts. Methods Retrospective, IRB-approved, data analysis of pathology-proven A pts. Included were pts with at least 1 MRI at ≥1 year postop. Initial tumor size, invasion status, pathology, postop remission, MRIs, radiation and medical therapy data were collected. Biochemical (biochem) remission = normal IGF-1 and GH <1 at 3 mo postop. For pts with radiation, data was only collected up to radiation. Stats: t-test, chi-square. Results 83 pts were included; mean age 46±16 years, 45% female, mean follow up 7.9±5.3 years. 55 pts were on PIT-directed therapy (50 on somatostatin receptor ligands (SRL) alone, 1 on cabergoline (Cab) alone, 4 on SRL/Cab), 12 on PEG > 1 year (9 on PEG alone.) 11/83 (13.25%) had tumor growth at median 3.5 years (range 1-11). Tumors that grew were larger at diagnosis (25.21±10.93 mm vs 17.45±8.37 mm, p=0.004), had larger residuals postop (23.83±5.0 mm vs 11.86±7.47 mm, p=0.0003), and tended to be invasive (77.78% (7/9) vs 53.03% (35/66), p=NS). 7/11 were sparsely granulated and 4 mixed GH-PRL. Of 11 that grew, 8 had postop residual tumor, 3 in remission, 4 with discrepant IGF-1/GH, 2 uncontrolled and 2 with no data at 3 months postop. At the time of growth, 9/11 pts were untreated (6 had active A, 1 with discrepant IGF-1/GH and 2 with no IGF-1/GH data), 1 was controlled on pasireotide and one in biochem remission. Only 1/50 (2%) pts on pasireotide had growth and no pts on PEG >1 year. Discussion 86.75% of pts with A did not have tumor growth after surgery. Only one pt on PIT-targeted medications and none on PEG experienced tumor growth. Almost all pts who had growth had large invasive adenomas, majority were sparsely granulated, residual tumor postop, were biochemically uncontrolled and not on medication at the time of growth. A previous metanalysis of SRLs in A showed that tumor increase occurs in 1.4% (follow up 3-36 months). In our study pt follow up was longer and 1.82% (1/55) of pts who were on SRL/Cab had growth. Conclusion We recommend less frequent MRI monitoring for pts treated with PIT-targeted medications. Conversely, pts with residual adenoma not on medical therapy should be closely monitored biochemically and by serial MRIs. Further studies are needed to identify appropriate imaging interval for pts on medications and based on characteristics of aggression (such as sparsely granulated, large residual tumors, lack of biochemical control despite medications).