FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3148402286> ?p ?o ?g. }

• W3148402286 endingPage "e260" @default.
• W3148402286 startingPage "e257" @default.
• W3148402286 abstract "Case PresentationA 26-year-old woman with no significant medical history was referred for 5 months of dry cough, dyspnea, presyncope and chest pressure, and nausea with exertion. The family history was notable for thromboembolic disease in the setting of malignancy and autoimmune disease. She was not on any medications. She is a never smoker and did not use recreational drugs. She had no work-related exposures. Her BP was 95/67 mm Hg; her heart rate was 93 beats per minute, and oxygen saturation was 98% on room air. Lung fields were clear to auscultation. She had a prominent P2 heart sound. There was no jugular venous distension or edema. There was no clubbing, rash, or synovitis. A 26-year-old woman with no significant medical history was referred for 5 months of dry cough, dyspnea, presyncope and chest pressure, and nausea with exertion. The family history was notable for thromboembolic disease in the setting of malignancy and autoimmune disease. She was not on any medications. She is a never smoker and did not use recreational drugs. She had no work-related exposures. Her BP was 95/67 mm Hg; her heart rate was 93 beats per minute, and oxygen saturation was 98% on room air. Lung fields were clear to auscultation. She had a prominent P2 heart sound. There was no jugular venous distension or edema. There was no clubbing, rash, or synovitis. Autoimmune serologies that included anti-nuclear antibody, anti-topoisomerase 1, anti-centromere, anti-double stranded DNA, rheumatoid factor, anti-citrullinated protein antibody, anti-ribonucleoprotein, and anti-Sjögren's-syndrome-related antigens A and B were negative. D-dimer was elevated at 1,138 μg/mL. Pulmonary embolism protocolled CT scan was negative for pulmonary embolism, but the read noted shotty mediastinal lymphadenopathy and centrilobular emphysema. A nuclear stress test was negative for ischemia. Echocardiogram revealed a severely enlarged and moderately dysfunctional right ventricle with septal flattening. The right ventricular systolic pressure was 70 mm Hg. The right atrium was moderately enlarged, and there was a small pericardial effusion. A ventilation perfusion scan was low probability. Right-sided heart catheterization revealed a right atrium pressure of 3 mm Hg, a right ventricle pressure of 79/1 mm Hg, a wedge pressure of 8 mm Hg, a pulmonary artery pressure of 85/33/53 mm Hg, and a Fick cardiac output of 4.4 L/min with an index of 2.5 L/min/m2. Pulmonary vascular resistance was 10.2 Wood units. There was no step up in saturations. A vasodilator challenge was negative (mean pulmonary artery pressure was 57 mm Hg after administration of adenosine). Pulmonary function testing was not available. She was hospitalized for the initiation of subcutaneous treprostinil for idiopathic pulmonary arterial hypertension (IPAH). She initially felt better, but her dyspnea progressively worsened despite aggressive prostacyclin up titration. Within 6 months, she was hospitalized three times for up titration and diuresis. A right heart catheterization was repeated at 5 months on 74 ng/kg/min of treprostinil that revealed a right atrium pressure of 9 mm Hg, a right ventricle pressure of 82/1 mm Hg, a wedge pressure of 10 mm Hg, a pulmonary artery pressure of 87/38/54 mm Hg, and a Fick cardiac output of 3.5 L/min with an index of 2 L/min/m2. Pulmonary vascular resistance was 12 Wood units. Treprostinil was increased to 113 ng/kg/min, but she experienced hypoxemia, and a repeat CT scan to evaluate for pulmonary emboli revealed diffuse ground glass opacities, smooth interlobular septal thickening and a small right pleural effusion (Fig 1). What is the diagnosis and what should be done next? Diagnosis: Although she had responded initially to prostacyclin therapy, the rapid progression of her disease and the development of pulmonary edema was concerning for pulmonary venoocclusive disease (PVOD). Confirmation would require a lung biopsy that was contraindicated due to the risk of life-threatening complications. Because she was in refractory right ventricle failure due to high pulmonary vascular resistance, the next step was to list her emergently for bilateral lung transplantation. In the interim, she did require brief venoarterial extracorporeal membrane oxygenation support. PVOD was later confirmed on pathologic examination of her explanted lungs (Fig 2). PVOD is a very rare disease within the World Health Organization group 1 classification of pulmonary hypertension. The true incidence is likely underestimated because it is almost indistinguishable from and often misclassified as IPAH. Patients with PVOD have the same symptoms of progressive dyspnea on exertion and signs of right ventricular failure. Findings on echocardiography and right-sided heart catheterization are also identical. Although seemingly counterintuitive, the wedge pressure does remain normal because the larger pulmonary veins are not affected and will transmit the left atrial pressure to the transducer in the static-wedged position.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar The only way to definitively establish a diagnosis of PVOD is by lung biopsy, but this is usually contraindicated due to the risk of bleeding and/or hemodynamic collapse. As a result, most diagnoses are made after death or after examination of the explanted lungs from a lung allograft recipient.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar Despite its similarities with IPAH and the previously mentioned diagnostic challenges, there are some important reasons to be wary of a diagnosis of PVOD. First of all, patients with PVOD typically have a more aggressive course and a poorer prognosis, so urgent referral for lung transplantation is key. There are no clinical trials for pulmonary vasodilators in PVOD, although it is hypothesized that the antiproliferative effects of vasodilator therapy may be beneficial.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar Experts in the field do favor a cautious trial of vasodilator therapy as a bridge to lung transplantation.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar,2Luo Q. Jin Q. Zhao Z. et al.Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?.BMC Pulm Med. 2019; 19: 257Crossref PubMed Scopus (1) Google Scholar Up to 25% to 45% of patients will experience the development of pulmonary edema at any point during up titration or sequential addition of vasodilators; this is one of the most profound clinical clues to the diagnosis. It is recommended therefore to initiate monotherapy with slow titration and an aggressive diuretic regimen.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar, 2Luo Q. Jin Q. Zhao Z. et al.Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?.BMC Pulm Med. 2019; 19: 257Crossref PubMed Scopus (1) Google Scholar, 3Montani D. Achouh L. Dorfmüller P. et al.Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology.Medicine (Baltimore). 2008; 87: 220-233Crossref PubMed Scopus (251) Google Scholar Of note, pulmonary capillary hemangiomatosis is considered to be on the spectrum of the same disease as PVOD. Sporadic and familial cases of PVOD/pulmonary capillary hemangiomatosis have been associated with bi-allelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene. PVOD can also occur after hematopoietic stem-cell transplantation and in association with connective tissue disease, especially systemic sclerosis. Exposure to chemotherapeutics and organic solvents has also been implicated.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar,4Eyries M. Montani D. Girerd B. et al.EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension.Nat Genet. 2014; 46: 65-69Crossref PubMed Scopus (252) Google Scholar,5Montani D. Lau E.M. Descatha A. et al.Occupational exposure to organic solvents: a risk factor for pulmonary veno-occlusive disease.Eur Respir J. 2015; 46: 1721Crossref PubMed Scopus (55) Google Scholar Although PVOD cannot be distinguished from IPAH based on hemodynamics, certain findings on chest CT imaging can be suggestive of the diagnosis. These findings include smooth interlobular septal thickening, ground glass opacities, and mediastinal lymphadenopathy. The key to the pathogenesis of this imaging pattern is the post capillary location of the lesions, which results in capillary and lymphatic congestion.6Frazier A.A. Franks T.J. Mohammed T.H. Ozbudak I.H. Galvin J.R. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis.RadioGraphics. 2007; 27: 867-882Crossref PubMed Scopus (119) Google Scholar In retrospect, the pattern of centrilobular emphysema noted on the patient’s initial CT scan was actually due to subtle ground glass opacities that resulted in prominence of less congested and better aerated areas. PVOD is characterized by a vasculopathy of the small pulmonary venules and septal veins that lead to intimal fibrosis, narrowing, and eventual obliteration. As a result, there is engorgement and dilation of pulmonary capillaries and lymphatics. Occasionally, there can be frank capillary angioproliferation, as can be seen in the pulmonary capillary hemangiomatosis phenotype. Elastin stains can help highlight pulmonary veins. Interestingly, there is often some degree of coexisting arteriopathy that may explain the initial/partial response to pulmonary vasodilator therapy. Nevertheless, venous vasculopathy is the predominant histologic feature of PVOD.1Montani D. Lau E.M. Dorfmüller P. et al.Pulmonary veno-occlusive disease.Eur Respir J. 2016; 47: 1518Crossref PubMed Scopus (127) Google Scholar •PVOD is a rare cause of pulmonary hypertension that is characterized by a vasculopathy of the pulmonary veins.•PVOD can be difficult to distinguish from idiopathic pulmonary hypertension, and biopsies are usually contraindicated.•Development of pulmonary edema with vasodilator therapy and findings of smooth interlobular septal thickening, ground glass opacities, and mediastinal lymphadenopathy on chest imaging are suggestive of the diagnosis.•Urgent referral for evaluation for lung transplantation is recommended if there is any suspicion that a patient may have PVOD." @default.
• W3148402286 created "2021-04-13" @default.
• W3148402286 creator A5018604266 @default.
• W3148402286 creator A5081340273 @default.
• W3148402286 date "2021-04-01" @default.
• W3148402286 modified "2023-09-26" @default.
• W3148402286 title "A 26-Year-Old Woman With Dyspnea on Exertion" @default.
• W3148402286 cites W1978752282 @default.
• W3148402286 cites W2014916402 @default.
• W3148402286 cites W2024407185 @default.
• W3148402286 cites W2129894155 @default.
• W3148402286 cites W2258142925 @default.
• W3148402286 cites W2995140996 @default.
• W3148402286 doi "https://doi.org/10.1016/j.chest.2020.10.044" @default.
• W3148402286 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/34022029" @default.
• W3148402286 hasPublicationYear "2021" @default.
• W3148402286 type Work @default.
• W3148402286 sameAs 3148402286 @default.
• W3148402286 citedByCount "0" @default.
• W3148402286 crossrefType "journal-article" @default.
• W3148402286 hasAuthorship W3148402286A5018604266 @default.
• W3148402286 hasAuthorship W3148402286A5081340273 @default.
• W3148402286 hasBestOaLocation W31484022861 @default.
• W3148402286 hasConcept C126322002 @default.
• W3148402286 hasConcept C164705383 @default.
• W3148402286 hasConcept C2777953023 @default.
• W3148402286 hasConcept C2778122182 @default.
• W3148402286 hasConcept C2778310968 @default.
• W3148402286 hasConcept C42219234 @default.
• W3148402286 hasConcept C71924100 @default.
• W3148402286 hasConcept C84393581 @default.
• W3148402286 hasConceptScore W3148402286C126322002 @default.
• W3148402286 hasConceptScore W3148402286C164705383 @default.
• W3148402286 hasConceptScore W3148402286C2777953023 @default.
• W3148402286 hasConceptScore W3148402286C2778122182 @default.
• W3148402286 hasConceptScore W3148402286C2778310968 @default.
• W3148402286 hasConceptScore W3148402286C42219234 @default.
• W3148402286 hasConceptScore W3148402286C71924100 @default.
• W3148402286 hasConceptScore W3148402286C84393581 @default.
• W3148402286 hasFunder F4320309555 @default.
• W3148402286 hasFunder F4320315730 @default.
• W3148402286 hasIssue "4" @default.
• W3148402286 hasLocation W31484022861 @default.
• W3148402286 hasOpenAccess W3148402286 @default.
• W3148402286 hasPrimaryLocation W31484022861 @default.
• W3148402286 hasRelatedWork W13581831 @default.
• W3148402286 hasRelatedWork W13763128 @default.
• W3148402286 hasRelatedWork W1384134 @default.
• W3148402286 hasRelatedWork W16767194 @default.
• W3148402286 hasRelatedWork W19072478 @default.
• W3148402286 hasRelatedWork W21141899 @default.
• W3148402286 hasRelatedWork W6603077 @default.
• W3148402286 hasRelatedWork W9354387 @default.
• W3148402286 hasRelatedWork W9759441 @default.
• W3148402286 hasRelatedWork W9868683 @default.
• W3148402286 hasVolume "159" @default.
• W3148402286 isParatext "false" @default.
• W3148402286 isRetracted "false" @default.
• W3148402286 magId "3148402286" @default.
• W3148402286 workType "article" @default.