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- Multidimensional CT approach to predict hemodynamics in pulmonary hypertensionPublication . Brito, Joana; Alves da Silva, Pedro; Cazeiro, Daniel; Azaredo Raposo, Miguel; Lousada, Nuno; Inácio, João R.; Guimarães, Tatiana; Almeida, Ana G.; Pinto, Fausto J.; Plácido, RuiAim: Computed tomographic pulmonary angiography (CTPA) allows an excellent visualization of heart chambers and vessels, which may be associated with hemodynamic status in pulmonary hypertension, obviating the need for repetitive right heart catheterization (RHC). In this study, we aimed to evaluate the capacity of CTPA to predict severe hemodynamics and to correlate with clinical status and events. Material and methods: Retrospective study with 51 patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in whom a CTPA and RHC were performed within 6 months. The primary endpoint was to evaluate the CTPA performance to predict severe hemodynamics, defined as the best mPAP threshold with clinical impact. Secondary endpoints were the correlation of CTPA measurements with clinical outcomes. Results: The right ventricle (RV) and right atrium (RA) areas, RV-free wall thickness, septal angle and pulmonary artery diameter assessed by CTPA revealed a good capacity to predict severe hemodynamic status. A CTPA model, incorporating both an RV area above 23 cm2 and an RA area above 21 cm2, increased the prediction capacity to detect severe hemodynamic status. The presence of both parameters above the threshold predicted severe PH with a 100% specificity and a 52% sensitivity and conveyed a 5-fold increased risk of mortality during follow-up. CTPA-altered parameters were directly associated with higher NT-proBNP levels and worse WHO-FC at baseline and follow-up. Conclusion: In this pilot study, a CTPA model was able to predict severe PH hemodynamic status and worse clinical events during follow-up.
- Chronic thromboembolic pulmonary hypertension: a comprehensive review of pathogenesis, diagnosis, and treatment strategiesPublication . Cazeiro, Daniel; Azaredo Raposo, Miguel; Guimarães, Tatiana; Lousada, Nuno; Jenkins, David; Inácio, João R.; Moreira, Susana; Mineiro, Ana; Freitas, Céline; Martins, Susana; Ferreira, Ricardo; Luís, Rita; Cardim, Nuno; Pinto, Fausto J.; Plácido, RuiChronic thromboembolic pulmonary hypertension (CTEPH) is part of group 4 of the pulmonary hypertension (PH) classification and generally affects more than a third of patients referred to PH centers. It is a three-compartment disease involving proximal (lobar-to-segmental) and distal (subsegmental) pulmonary arteries that are obstructed by persistent fibrothrombotic material, and precapillary pulmonary arteries that can be affected as in pulmonary arterial hypertension. It is a rare complication of pulmonary embolism (PE), with an incidence of around 3% in PE survivors. The observed incidence of CTEPH in the general population is around six cases per million but could be three times higher than this, as estimated from PE incidence. However, a previous venous thromboembolic episode is not always documented. With advances in multimodality imaging and therapeutic management, survival for CTEPH has improved for both operable and inoperable patients. Advanced imaging with pulmonary angiography helps distinguish proximal from distal obstructive disease. However, right heart catheterization is of utmost importance to establish the diagnosis and hemodynamic severity of PH. The therapeutic strategy relies on a stepwise approach, starting with an operability assessment. Pulmonary endarterectomy (PEA), also known as pulmonary thromboendarterectomy, is the first-line treatment for operable patients. Growing experience and advances in surgical technique have enabled expansion of the distal limits of PEA and significant improvements in perioperative and mid- to long-term mortality. In patients who are inoperable or who have persistent/recurrent PH after PEA, medical therapy and/or balloon pulmonary angioplasty (BPA) are effective treatment options with favorable outcomes that are increasingly used. All treatment decisions should be made with a multidisciplinary team that includes a PEA surgeon, a BPA expert, and a chest radiologist.
- Pulmonary arterial hypertension with features of venous involvement: a detective's taskPublication . Cazeiro, Daniel; Plácido, Rui; Azaredo Raposo, Miguel; Brito, Joana; Borba, Alexandra; Guimarães, Tatiana; Pinto, Eugénia; Freitas, Pedro; Pinto, Fausto J.Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.