Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties
Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantatio...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-01-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/11/1/141 |
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| author | Marta Pérez Núñez Sergio Alonso Charterina Carmen Pérez-Olivares Yolanda Revilla Ostolaza Rafael Morales Ruiz Ana Belén Enguita Valls Jair Antonio Tenorio Natalia Gallego Zazo Alicia De Pablo Gafas Pablo Lapunzina Adriana Rodríguez Chaverri Pilar Escribano Subías |
| author_facet | Marta Pérez Núñez Sergio Alonso Charterina Carmen Pérez-Olivares Yolanda Revilla Ostolaza Rafael Morales Ruiz Ana Belén Enguita Valls Jair Antonio Tenorio Natalia Gallego Zazo Alicia De Pablo Gafas Pablo Lapunzina Adriana Rodríguez Chaverri Pilar Escribano Subías |
| author_sort | Marta Pérez Núñez |
| collection | DOAJ |
| description | Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease. |
| first_indexed | 2024-03-09T04:20:14Z |
| format | Article |
| id | doaj.art-9ad2e25d7eb74ce9bf1e464ab15e78e0 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T04:20:14Z |
| publishDate | 2021-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-9ad2e25d7eb74ce9bf1e464ab15e78e02023-12-03T13:49:02ZengMDPI AGDiagnostics2075-44182021-01-0111114110.3390/diagnostics11010141Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and UncertaintiesMarta Pérez Núñez0Sergio Alonso Charterina1Carmen Pérez-Olivares2Yolanda Revilla Ostolaza3Rafael Morales Ruiz4Ana Belén Enguita Valls5Jair Antonio Tenorio6Natalia Gallego Zazo7Alicia De Pablo Gafas8Pablo Lapunzina9Adriana Rodríguez Chaverri10Pilar Escribano Subías11Servicio de Radiología, Unidad Multidisciplinar de Hipertensión Pulmonar, Hospital Universitario Doce de Octubre, 28041 Madrid, SpainServicio de Radiología, Unidad Multidisciplinar de Hipertensión Pulmonar, Hospital Universitario Doce de Octubre, 28041 Madrid, SpainCentro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, SpainServicio de Radiología, Unidad Multidisciplinar de Hipertensión Pulmonar, Hospital Universitario Doce de Octubre, 28041 Madrid, SpainServicio de Radiología, Unidad Multidisciplinar de Hipertensión Pulmonar, Hospital Universitario Doce de Octubre, 28041 Madrid, SpainCentro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, SpainInstitute of Medical and Molecular Genetics (INGEMM)-IdiPAZ, Hospital Universitario La Paz-UAM, Paseo de La Castellana, 261, 28046 Madrid, SpainInstitute of Medical and Molecular Genetics (INGEMM)-IdiPAZ, Hospital Universitario La Paz-UAM, Paseo de La Castellana, 261, 28046 Madrid, SpainCentro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, SpainInstitute of Medical and Molecular Genetics (INGEMM)-IdiPAZ, Hospital Universitario La Paz-UAM, Paseo de La Castellana, 261, 28046 Madrid, SpainCentro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, SpainCentro de Referencia Nacional de Hipertensión Pulmonar Compleja, Spain and ERN-Lung-Pulmonary Hypertension) Referal Center, 28041 Madrid, SpainPulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease.https://www.mdpi.com/2075-4418/11/1/141pulmonary veno-occlusive diseasepulmonary hypertensionmultidetector computed tomography (MDCT) |
| spellingShingle | Marta Pérez Núñez Sergio Alonso Charterina Carmen Pérez-Olivares Yolanda Revilla Ostolaza Rafael Morales Ruiz Ana Belén Enguita Valls Jair Antonio Tenorio Natalia Gallego Zazo Alicia De Pablo Gafas Pablo Lapunzina Adriana Rodríguez Chaverri Pilar Escribano Subías Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties Diagnostics pulmonary veno-occlusive disease pulmonary hypertension multidetector computed tomography (MDCT) |
| title | Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties |
| title_full | Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties |
| title_fullStr | Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties |
| title_full_unstemmed | Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties |
| title_short | Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties |
| title_sort | radiological findings in multidetector computed tomography mdct of hereditary and sporadic pulmonary veno occlusive disease certainties and uncertainties |
| topic | pulmonary veno-occlusive disease pulmonary hypertension multidetector computed tomography (MDCT) |
| url | https://www.mdpi.com/2075-4418/11/1/141 |
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