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.

This study aimed to evaluate the feasibility of a noninvasive operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH) based on multidetector computed tomographic angiography (MCTA). Up to 176 patients were evaluated from January 2016 to April 2018. Throughout the first phase, the initial surgical decision was made based on MCTA with further analysis of pulmonary angiography (PA) in order to evaluate in which cases the initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or those whose assessment was not possible. Patients deemed operable (50%) based on MCTA along the first phase had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase. Regarding operated patients, the decision of operability was based solely on MCTA in 94% of those with level I disease, in 75% with level II, and 54% with level III. This approach enabled shorter periods of time to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures, and survival rates at 1 year after surgery were comparable in both phases. Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizes surgical candidacy evaluation.

Background – The development of an optimized assessment of operability in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is crucial. This study aimed to evaluate the feasibility of a noninvasive operability assessment of CTEPH based on multidetector computed tomographic angiography (MCTA). Methods –176 patients with CTEPH were evaluated from January 2016 to April 2018. Throughout the first phase, operability was assessed with MCTA and pulmonary angiography (PA): initial surgical decision was made based on MCTA with further analysis of PA to evaluate which cases initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or in those whose assessment was not possible. Results – Patients deemed operable (50%) based on MCTAalong the first phase, had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase, in which decision of operability was based on MCTA in 49% of the patients. Regarding operated patients, decision of operability had been based solely on MCTA in 94% of those with level I disease regarding intraoperative CTEPH classification; in 75% with level II disease and 54% with level III. This approach enabled shorter time periods to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures and survival rates at 1 year after surgerywere comparable in both phases. Conclusions - Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizessurgical candidacy evaluation.