Rare Cause Of Pulmonary Hypertension Research Articles

PULMOEAST: A Comprehensive Analysis of Pulmonary Hypertension in Eastern India.

Background Pulmonary hypertension (PH) is a debilitating cardiovascular disorder characterized by abnormally elevated blood pressure within the lungs. The diverse range of causes and varied clinical presentations contribute to the complexity of its diagnosis and management. In eastern India and surrounding areas, awareness of PH remains limited, and resources for its management are scarce. This study aims to address this knowledge gap by investigatingclinical characteristics and treatment approaches adopted for PH patients in eastern India. Methods This retrospective-prospective cohort study included patients diagnosed with PH, defined by a pulmonary artery systolic pressure (PASP) > 50 mmHgor amean pulmonary artery pressure (mPAP)>20 mmHg, between July 2015 and October 2023. Data retrieved from hospital records formed the retrospective cohort, while the prospective cohort comprised patients directly recruited for the study. Results The PULMOEAST study enrolled 93 patients with confirmed PH, divided into prospective (59 patients) and retrospective (34 patients) cohorts. The most prevalent cause of PH was congenital heart disease (CHD), with shunt lesions (59.13%), followed by complex CHD (13.97%) and idiopathic PH (20.43%). Six additional patients presented with rare causes of PH, and three experienced transient PH following atrial septal defectdevice closure. Geographic distribution revealed that 72.04% of patients originated from eastern India, while 18.27% hail from other eastern states and 8.6% from neighboring countries. Patients exhibited varying functional classes (FC), with 57 classified as FC-II and 31 classified as FC-III. Treatment strategies primarily involve supportive medications and pulmonary vasodilators. Monotherapy was administered to 26 patients (27.95%), dual therapy to 50 patients (53.76%), and triple therapy to one patient. Notably, 16 patients did not receive any vasodilator therapy as they were waiting for further evaluation. Among the vasodilator regimen, two patients received Selexipag. Three patients underwent intervention for shunt lesion closure, including one patient who received a fenestrated atrial septal occluder implant. Additionally, one patient underwent clot removal for pulmonary thromboembolism. Despite the overall positive response to treatment, the study recorded eight fatalities (8.6%) during the observation period. However, most patients exhibited significant improvement, including a decrease in functional class, during a mean follow-up duration of 14.31 months. Conclusion The PULMOEAST study undertook a comprehensive exploration of PH in eastern India and surrounding regions, revealing a stark dominance of CHD as the primary culprit. The study confirmed the pivotal role of echocardiography as a readily available and effective tool for both initial and follow-up evaluations in resource-scarce settings. It painted a hopeful picture by showcasing significant clinical improvement in most treated patients, with supportive medications and pulmonary vasodilators playing a crucial role. However, the diverse etiologies, limited access to PH-specific resources, and lack of widespread awareness within the region continue to pose substantial challenges for patients. The study underscores the need for refined diagnostic approaches, cost-effective management strategies, collaborative care initiatives, and enhanced patient education to optimize PH care and improve outcomes in eastern India.

Retrospective analysis of confirmed cases of pulmonary veno-occlusive disease: a single tertiary referral center study

Abstract Background Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that primarily results in intimal fibrosis of the pulmonary venules and veins, the venous lesions are often associated with changes in interlobular septa, lung parenchyma, and pulmonary arterioles and arteries. Purpose We sought to study the clinical characteristics of patients with confirmed PVOD at a single large referral center. Methods We retrospectively reviewed data from a total of 675 patients with pulmonary hypertension (identified by ICD codes or search of text) from 2017 and 2022 from a database obtained from electronic medical records at our institution. Patients with group 2 pulmonary hypertension were excluded. Only 29 cases were confirmed by histology or genetic testing. Results Out of the 29 patients confirmed with PVOD, 28 (96.5%) had a characteristic histology (lung biopsy (55.1%), explanted lung for transplant (27.5%) and autopsy (13.7%)) and 1 (3.4%) had a positive genetic test for EIF2AK4 biallelic mutation. The age at diagnosis was 53±16 years, 14 (48.2%) were men, 2 (6.8%) had systemic scleroderma, 2 (6.8%) had psoriasis, 2 (6.8%) were exposed to cytotoxic agents, 16 (55.1%) were smokers and 2 (6.8%) had a family history of pulmonary hypertension. All patients had computed tomography of the chest but only 12 (41.3%) had all 3 typical features found in PVOD (ground glass opacities, interlobular septal thickening, and lymphadenopathy). The most common feature was interlobular septal thickening in 26 (89.6%), followed by ground glass opacities present in 25 (86.2%) and lymphadenopathy in 12 (41.3%) of the patients. At diagnosis, mean pulmonary artery pressure was 45±15 mmHg and pulmonary vascular resistance was 9±6 WU. The median DLCO was 41 (34-58) %. On echocardiogram, 14 (48.2%) had severely enlarged right ventricle (RV), RV function was ≥moderately reduced in 17 (58.6%), RV systolic pressure was 63±20 mmHg and 6 (20.6%) had pericardial effusion. Out of 21 (72.4%) patients who received pulmonary vasodilators, 4 (13.7%) received single agent. 10 (34.4%) received 2 agents, 6 (20.6%) 3 agents, and 1 (3.4%) received 4 agents. 35.7% of these treated patients reported an improvement or initial worsening of symptoms with subsequent improvement afterwards. 18 (62%) patients with PVOD died, 12 (41.3%) had a lung transplant and 6 (20.6%) were still alive without the transplant until the day data was collected. Mean duration from diagnosis to death and lung transplantation was 446 ± 405 days and 274±479 days respectively. Conclusion PVOD is a rare and lethal cause of pulmonary hypertension. When suspected, a trial of medical therapy should be initiated carefully. The evaluation for lung transplant should be considered early given its high mortality.

Pulmonary veno-occlusive disease with vanished pulmonary consolidation.

Pulmonary veno-occlusive disease (PVOD) is an extremely rare cause of pulmonary hypertension. Previously reported computed tomography (CT) findings of PVOD included centrilobular ground-glass opacities, a mosaic pattern, and septal lines; however, chest CT revealing pulmonary consolidation disappearance with repositioning has not been reported.

A rare cause of pulmonary hypertension in an elderly woman.

A rare cause of pulmonary hypertension in an elderly woman.

A Case of Fulminant Right Heart Failure Owing to Tumoral Pulmonary Hypertension

Tumoral pulmonary hypertension is a rare cause of pulmonary hypertension. We report a patient who was thought to have idiopathic pulmonary arterial hypertension, but later developed fulminant right heart failure ultimately leading to death. Autopsy revealed substantial pulmonary tumor embolism burden originating from liver adenocarcinoma. (Level of Difficulty: Advanced.)

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

Vascular anomaly diagnosis by central venous catheter misplacement: a case report

BackgroundCongenital heart diseases rarely have a primary manifestation in adulthood. They are a rare cause of pulmonary hypertension in adults.Case presentationA 70-year-old woman of Eurasian descent underwent emergency surgery for bowel ischemia. Her history of mild pulmonary hypertension likely correlates with a peculiar diagnosis of an anatomic anomaly on the postoperative x-ray and computed tomography scan. The central venous catheter was misplaced. Initial management consisted of removal of the catheter. The diagnosis, partial anomalous pulmonary venous return, may pose a clinical therapeutic dilemma.ConclusionsPartial anomalous pulmonary venous return is a potentially treatable cause of pulmonary hypertension. With the current trend toward more medical imaging, we expect this diagnosis to be made more often in the future.

Lung Transplant Outcomes in Patients with Pulmonary Veno-Occlusive Disease

<h3>Purpose</h3> Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Prognosis of PVOD is worse than PAH. Most patients need lung transplantation. Previous study showed that PVOD patients had higher waitlist mortality compared to PAH patients. To date, there are no study report outcomes of lung transplant in this population. We would like to study the outcome and compare with IPAH patients. <h3>Methods</h3> This is a retrospective observational study. Patients with a diagnosis of PVOD and PAH underwent lung transplant between 2005 and 2018 were identified from SRTR database. Numerical data were reported in mean and standard deviation. Categorical data were reported in count and percent. T-test and Chi-square test were performed to compare variables between 2 groups. Survival was compared using Kaplan-Meier (K-M) method. <h3>Results</h3> 61 PVOD patients and 970 PAH patients underwent lung transplant were identified. There was no significant difference in age and BMI. Patients with PVOD had significant lower post-transplant systolic, diastolic and mean PA pressure and higher cardiac output. LAS at matching were not significantly different. PVOD patients had shorter time on waiting list (152.7±263.34 vs 337.56±511.14, p<0.01). Proportion of patients requiring ECMO post lung transplant were higher in PVOD group (13.11% vs 4.23%, p<0.01). Proportion of patients developing primary graft dysfunction (PGD) were lower in PVOD group (37.5% vs 61.93%, p=0.056). There was no difference of length of stay, FEV1, FVC, PCO2 post lung transplant and acute rejection between 2 groups. Survivals were not different between 2 groups using K-M method (p=0.33). <h3>Conclusion</h3> We found that PVOD patients who underwent lung transplant had shorter waiting time compared to PAH patients which suggest that disease severity of PVOD is higher than PAH. Despite that more PVOD patients required ECMO support post-operative, patient survival and other long-term outcomes such as lung function or rejection were not different between from PAH patients.

Pulmonary Capillary Hemangiomatosis and Refractory Hypoxemia During PH Therapy

<h3>Introduction</h3> Pulmonary capillary hemangiomatosis (PCH), a rare cause of pulmonary hypertension (PH), presents clinically with right heart dysfunction and may be indistinguishable from typical pulmonary arterial hypertension. Vasodilator therapy exacerbates hypoxemia in PCH despite improvement in heart failure symptoms. Lung transplant is the only definitive therapy. We present the case of a patient succumbing to progressive hypoxemia on PH therapy during lung transplant evaluation. Autopsy revealed PCH. <h3>Case Report</h3> A 42-year-old female with PH due to undifferentiated connective tissue disease presented with acute respiratory distress. She was diagnosed 2 years prior and initially treated with macitentan, tadalafil and diuretics with clinical improvement; however, worsening shortness of breath and new finding of hypoxemia developed after 1 year. Imaging revealed faint patchy nodular opacities initially attributed to UCTD-associated pneumonitis. Steroids and mycophenolate failed to achieve radiographic improvement. Repeat right heart catheterization showed continued elevation in pulmonary artery pressure and stably reduced cardiac output. Subcutaneous infusion of Treprostinil was initiated 2 months prior to admission, which worsened hypoxemia. Clinical diagnosis of pulmonary veno-occlusive disease (PVOD) vs PCH was suspected and lung transplant evaluation initiated. On admission, patient presented with syncope and was found to have refractory hypoxemia despite high-flow oxygen supplementation. Cardiogenic shock and hypoxemic respiratory failure ensued, resulting in hepatic and renal failure, leading to cardiac arrest and unsuccessful resuscitation. Autopsy showed PCH. <h3>Summary</h3> Refractory hypoxemia during up-titration of vasodilator therapy for PH should prompt clinical suspicion for atypical causes of PH such as PCH. Early lung transplant referral is essential if PCH/PVOD is suspected given its poor prognosis and potentially harmful responses associated with traditional PH therapy.

Difficult diagnostics of a rare cause of pulmonary hypertension: a case report

Pulmonary hypertension is a syndrome difficult for differential diagnosis, which is the outcome of various pathological conditions. With the exclusion of the most common causes of pulmonary hypertension (left heart disorders and pulmonary embolism), further search for cause often becomes an insoluble problem. Sezary syndrome is classified as a rare type of cutaneous T-cell lymphomas. Early diagnosis of this syndrome is important for the initiation of adequate therapy, since cases of complete recovery or long-term remission in patients with Sezary syndrome are very rare. A case report of Sezary syndrome verification in cardiology practice is described.

Une maladie veino-occlusive pulmonaire familiale avec mutation biallélique hétérozygote composite d’EIF2AK4

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Heritable and sporadic forms have been distinguished. Hypoxemia, profound reduction in the diffusion of carbon monoxide and haemodynamic confirmation of pre-capillary pulmonary hypertension are the major diagnostic criteria. Thoracic CT scanning and a response to pharmaceutical therapy provide additional information to confirm the diagnosis. A 52-year-old patient, three of whose siblings had pulmonary hypertension, was admitted with dyspnoea, malaise and palpitations. Right heart catheterisation confirmed pre-capillary pulmonary hypertension. A search for an EIF2AK4 mutation was carried out, and this showed a composite biallelic heterozygous mutation compatible with the diagnosis of familial PVOD, identical to that showed in one of his brothers. Given the signs of severity of the disease and the diagnosis of PVOD, whose response to pharmaceutical therapy is often poor, the patient was placed on a waiting list for lung transplantation. Despite a similar diagnosis in 3 brothers and follow-up proposed 11 years before the diagnosis, pulmonary hypertension appeared within a few weeks and led immediately to a severe clinical situation. Annual clinical and echocardiographic monitoring had been strongly advised to the patient, but had not allowed diagnosis at a mild or moderate stage of the disease. This clinical case shows that the identification of factors predicting the development of heritable PVOD at a pre-symptomatic stage is an important issue for clinical research.

PULMONARY CAPILLARY HEMANGIOMATOSIS: A RARE CASE OF STABLE DISEASE WITHOUT TREATMENT

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension caused by extensive proliferation of pulmonary capillaries within parenchymal, vascular, and bronchial tissue. Typically, PCH progresses rapidly with a median time to lung transplantation of two years post diagnosis. To our knowledge, we present the longest case of biopsy proven PCH that has been stable without treatment for seven years. CASE PRESENTATION: A healthy 23-year old non-smoking male presented to the emergency department (ED) with a 2-week history of hemoptysis associated with intermittent left-sided pleuritic chest pain, fatigue, and dyspnea. His physical exam, routine lab work, and chest X-ray were unremarkable. He was discharged home and prescribed clarithromycin for suspected bronchitis. He returned to the ED months later with ongoing symptoms and was referred for urgent outpatient consultation to pulmonology. Bronchoscopy was performed with no blood on bronchoalveolar lavage analysis. Further serum rheumatologic and vasculitis panels were negative. A non-contrast computed tomography (CT) scan of his chest revealed subtle bilateral upper lung predominant centrilobular ground-glass opacities. (Figure 1) With worsening hemoptysis, he returned to the ED six months later. A CT Chest angiogram was performed and showed progressive bilateral airspace opacities. (Figure 2) The patient was referred to Thoracic Surgery for lung biopsy. This demonstrated septal proliferation of capillaries consistent with PCH without evidence of vasculitis, venous occlusion or microorganisms. Immunofluorescence staining was also negative. (Figure 3) The patient remains stable with no signs of progression or pulmonary hypertension on echocardiogram. His enhanced CT and pulmonary function tests remain normal seven years from his initial presentation. DISCUSSION: The typical presentation of PCH includes progressive dyspnea, cough with or without hemoptysis, chest pain, and fatigue. (1) Chest X-ray can be normal or progress to demonstrate bilateral opacities in keeping with diffuse alveolar hemorrhage (DAH). CT chest often shows diffuse centrilobular ground-glass opacities. (1) Bronchoscopy may not reveal erythrocytes if the patient is not actively bleeding. Diagnosis of PCH is essential, as vasodilators used in pulmonary hypertension can cause hemodynamic instability in PCH. (2) Interestingly, an autopsy case series found incidental cases of PCH in patients who were asymptomatic during life. (3) This raises the possibility that subclinical cases of PCH may exist which do not require urgent referral for lung transplantation. CONCLUSIONS: This case represents the longest active case of PCH after confirmation with biopsy without treatment. Re-evaluating the expected prognosis for individuals diagnosed with PCH is important, as median survival may be longer than currently reported in the literature. Reference #1: O'keefe MC, and Post MD. Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension. Archives of Pathology & Laboratory Medicine, 2015;139(2):274–277. Reference #2: Almagro P, Julia J, Sanjuame M, Gonalez G, Casalots J, Heredia JL, et al. Pulmonary Capillary Hemangiomatosis Associated with Primary Pulmonary Hypertension. Medicine. 2002;81(6):417–24. Reference #3: Havlik DM, Massey LW, Williams WL, and Crooks LA. Pulmonary Capillary Hemangiomatosis- like foci: an autopsy study of 8 cases. Anatomic Pathology. 2000;113(5):665-662. DISCLOSURES: No relevant relationships by Meredith Chiasson, source=Web Response No relevant relationships by Jasper Johar, source=Web Response No relevant relationships by Daria Manos, source=Web Response No relevant relationships by Matthew Nunn, source=Web Response

Eating Cheerios on ECMO: The Use of VA ECMO as a Bridge to Lung Transplant in an Infant

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. In PVOD the pulmonary venous vessels are obliterated resulting in obstructive pulmonary vascular disease, increase in pulmonary vascular resistance, right heart failure and death. Pulmonary hypertension is the most common reason for lung transplant in infants. Lung transplant is the only therapy for PVOD. Prolonged intubation, deconditioning and extra corporeal life support are associated with poor outcomes after lung transplant. We present a case of PVOD in an infant who had successful lung transplant using a novel veno-arterial extracorporeal membrane oxygenation (VA ECMO) configuration. A previously healthy 15-month-old male presented with cough and tachypnea. Cardiomegaly and pulmonary edema were seen on chest x-ray. Echocardiogram revealed severe pulmonary hypertension. Cardiac catheterization study and chest computed tomography revealed PVOD. He was unable to be extubated after cardiac catheterization and was transferred to our institution for lung transplant evaluation. He had severely depressed right ventricular function, requiring vasopressors to maintain hemodynamic stability. Respiratory status was tenuous, requiring sedation and paralysis. He was evaluated and listed for lung transplant. The patient was centrally cannulated for VA ECMO using cannulae for a ventricular assist device (Berlin ExCor) that were exteriorized on the abdomen. "Venous" inflow cannula was placed in the main pulmonary artery to provide afterload reduction for the right ventricle. After starting VA ECMO, vasopressor support, respiratory support, paralysis and sedation were weaned. He was woken up, extubated; and intensive physical and occupational rehabilitation began. He regained strength, was able to sit, stand and take steps. He also regained his milestones, was able to babble, wave and eat simple foods. The patient remained on VA ECMO for 61 days until he successfully underwent lung transplantation. Our case highlights the successful use of a novel VA ECMO configuration as a bridge to lung transplant in an infant with PVOD. To our knowledge this is the youngest patient to undergo awake VA ECMO reported. The use of a unique cannulation allowed for rehabilitation while on VA ECMO. Though challenging, VA ECMO remains an option as a bridge to lung transplant in infants with PVOD.

More than a rare cause of pulmonary hypertension in the elderly

© 2020 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espana, S.L.U. This is an open access article under the CC-BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

More than a rare cause of pulmonary hypertension in the elderly

More than a rare cause of pulmonary hypertension in the elderly

PARTIAL ANOMALOUS PULMONARY VENOUS RETURN WITH INTACT ATRIAL SEPTUM: A RARE CAUSE OF PULMONARY ARTERIAL HYPERTENSION

Partial anomalous pulmonary venous return (PAPVR) is a rare cause of pulmonary hypertension (PH) with a prevalence of 0.1 - 0.2% in the adult population. Most PAPVR is associated with atrial septal defect but it can occur in isolation. A 65-year-old woman presented with gradual-onset exertional

P1719 A rare complication of homozygous sickle cell disease and high output cardiac failure

Abstract Introduction/Background Sickle cell disease (SCD) is an autosomal recessive blood disorder characterised by chronic anaemia and abnormally shaped red blood cells. SCD impacts multiple organ systems causing effects on morbidity and mortality, including cardiovascular (CV). Long term SCD can have a significant impact on the heart through multiple pathways, including chronic anaemia, microvascular dysfunction and the development of hypertension and chronic kidney disease, leading to the development of a high-output state and adverse cardiac remodelling. We report a rare case of homozygous SCD with the development of high output cardiac failure complicated by the development of associated pulmonary hypertension. Case summary A 58 yr old female patient (Height 158cm, weight 51.25kg, cardiac index 4.01 L/min/m²) with known homozygous SCD (HbSS) undergoing regular transfusions for chronic anaemia was admitted following a routine transthoracic echocardiogram (Figure 1a &amp; 1b) due to the presence of a moderate sized global pericardial effusion, severe tricuspid regurgitation, dilated right heart with impaired longitudinal function, and good left ventricular (LV) systolic function (LVEF Simpson’s biplane = 62 +/- 5%). In the months preceding this, the patient described progressive dyspnoea associated with decreasing workload and orthopnoea. On clinical examination there was evidence of right sided heart failure. Blood results demonstrated a longstanding anaemia (Hb 57 g/L) with MCV 109.6. NT-proBNP was 10233 ng/L. Cardiac magnetic resonance imaging demonstrated LV dilatation with hyperdynamic function, severe LV hypertrophy, and impaired longitudinal function (global longitudinal strain -9.7%). Cardiac output was elevated at 6.5 L/min. There was no late gadolinium enhancement and T2* mapping did not demonstrate cardiac iron loading (29.6ms). Right heart catheterisation confirmed pulmonary hypertension (pulmonary artery pressure 55/33, mean 46mmHg) due to left heart disease (PC wedge pressure (24/30, mean 24mmHg). The pericardial effusion was drained and the aspirate demonstrated a cellular infiltrate reflecting pericardial inflammation (moderate pus cells, no growth after 48hrs, total protein = 56 g/L, no histological evidence of malignancy). Additionally, the patient underwent intravenous diuresis and blood transfusion with improvement in clinical status. Discussion We report a rare cause of pulmonary hypertension due to elevated left ventricular end-diastolic pressure as a result of long-term high cardiac output in a patient who has established homozygous SCD. Long-term adverse cardiac remodelling in SCD is well described. Pulmonary hypertension is a rare multifactorial complication of this process and the precise mechanisms remain unclear. It confers a poor prognosis and the only current treatment is the management of the underlying condition. Pulmonary hypertension should always be considered in SCD patients with clinical and cardiac imaging evidence. Abstract P1719 Figure 1

Pulmonary Artery Stenosis in a Patient with Prior Histoplasmosis and the Discovery of Complications

Pulmonary hypertension (PH) results from varied etiologies, leading to progressive symptoms and limiting physical activity and quality of life, with associated morbidity and mortality. External compression of the pulmonary artery (PA) is a rare cause of PH and may give the clinician cause to investigate compression of nearby structures. In this E-Challenge, the authors present a case of PA stenosis in a patient with prior histoplasmosis scheduled for left PA stenting. However, because the pulmonary veins were not well-visualized on chest computed tomography, the anesthesia team performed a perioperative transesophageal echocardiogram (TEE) to help differentiate the causes of PH. TEE revealed external compression of the pulmonary veins. This case highlights the value of pathophysiologic understanding, preoperative planning, and the effect of echocardiography on clinical management and patient safety. In this case, TEE prevented possible morbidity and mortality.

Chronic Thromboembolic Pulmonary Hypertension: Endovascular Treatment.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare cause of pulmonary hypertension; less than 5% of pulmonary hypertension is caused by recurrent pulmonary thromboembolism (PTE). By definition, CTEPH happens within the first two years after symptomatic PTE; however, cases are often diagnosed without a history of acute PTE. Because of the poor functional status and chronicity of this disease, the classic and curative strategy of open pulmonary endarterectomy cannot be applied in some patients with lesions that involve the distal subsegmental pulmonary artery. Bridging therapy is needed for cases that are technically operable but have an unacceptable risk-benefit assessment or residual symptomatic pulmonary hypertension following surgical removal. Groups in Europe and Japan recently introduced balloon pulmonary angioplasty or percutaneous transluminal pulmonary angioplasty, which has led to significant improvement in functional and hemodynamic parameters in patients with CTEPH. This article introduces recent updates in patient selection and interventional procedures for this chronic and devastating disease.

ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA AS A CAUSE OF PERSISTENT PULMONARY HYPERTENSION

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is rare, accounting for 15-20% of peripheral T-cell lymphomas. Diagnosis can be challenging, as AITL may present with symptoms such as rash and arthralgias more suggestive of an autoimmune disorder (1). There are very few published reports of lymphoma-associated pulmonary hypertension (PH), most involving intravascular B-cell lymphomas (2). Here we present a case of AITL-associated PH that did not resolve with lymphoma remission. CASE PRESENTATION: A previously healthy 63-year-old woman presented after weeks of fevers, arthralgias, rashes, and dyspnea on exertion. Pulmonary function testing and CT chest were initially unremarkable. After an equivocal work-up she was provisionally diagnosed with Still’s disease, but saw no improvement on colchicine or anakinra. FDG-PET obtained a year after presentation revealed new pleural and pericardial effusions, splenomegaly, and diffuse lymphadenopathy, but no abnormal uptake in the lungs. Echocardiogram revealed mild RV enlargement with reduced function and an estimated RVSP of 73. A lymph node biopsy was consistent with AITL, a diagnosis further supported by the presence of a TCR gene rearrangement. She was started on chemotherapy with CEOP and had complete response after 2 cycles. Despite her excellent response to chemotherapy, her dyspnea rapidly progressed and repeat PFTs showed interval development of a moderately reduced diffusion capacity. Repeat CT chest was again negative for pulmonary infiltrates and V/Q scan did not suggest chronic embolism. On physical exam she now had JVD, 2+ pitting edema to her knees, and cool extremities. Right heart catheterization confirmed severe PH with mPAP 46 mmHg, normal PAWP, and PVR 13 WU. A thorough evaluation for other causes of PH was unrevealing. The patient’s symptoms improved on PDE5i and ERA therapy; HRCT did not reveal signs of PVOD nor did she develop hypoxia on treatment. DISCUSSION: Lymphoma is a rare cause of PH, and to our knowledge this is only the second reported case of PH due to AITL (3). In that case, as well as in other cases of lymphoma-associated PH, the PH improved or resolved with lymphoma remission. Our patient’s PH continued even after complete response to chemotherapy, and we hypothesize that the delay in diagnosis allowed time for fixed remodeling of the pulmonary arterial endothelium. CONCLUSIONS: Lymphoma may be an underappreciated cause of PH, and should be considered in patients with unexplained PH. AITL, in particular, should be considered in patients with PAH and symptoms suggestive of a systemic rheumatologic disorder. Imaging devoid of interstitial changes does not preclude this diagnosis, particularly once PE, PVOD, and pulmonary capillary hemangiomatosis have been considered. Treatment with PAH therapy was safe and effective in this case. Reference #1: de Leval et al. Br J Haematol. 2010 Mar; 148(5): 673-89 Reference #2: Nelson et al. J Thorac Oncol. 2012 Dec; 7(12): e34-5 Reference #3: Hua J et al. J Hematol Malig. 2012; 2(3): 26. DISCLOSURES: No relevant relationships by Anna Bracewell, source=Web Response No relevant relationships by Sachin Gupta, source=Web Response