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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.
- Phenotype of BTK‐lacking myeloid cells during prolonged COVID‐19 and upon convalescent plasmaPublication . Gomes, André; Farias, Guilherme B.; Trombetta, Amelia Chiara; Godinho-Santos, Ana; Parreira, Inês; Gonçalves, Hélder Diogo; Lessa Simões, Mariana; Aguiar, Patrício; Deveza, Maria Manuel; Inácio, João R.; Sousa, Ana E.; Silva, SusanaXLA patient with 7-month course of COVID-19 with persistent plasma SARS-CoV-2 load revealed a sustained non-inflammatory profile of myeloid cells in association with contained severity of disease, arguing in favor of the use of BTK inhibitors in SARS-COV-2 infection.
- Epipericardial fat necrosis in chest CT and MRI: a case report of an unusual cause of chest pain associated with the initial diagnosis of undifferentiated connective tissue diseasePublication . Barreto, Inês; Oliveira, Francisca Godinho; Barreira, Sofia; Inácio, João R.Background: Epipericardial fat necrosis (EFN) is a benign and self-limited condition of unknown cause with a good prognosis, usually affecting otherwise healthy patients. Clinically, it presents with severe acute left pleuritic chest pain, often leading the patient to the Emergency Room (ER). Case presentation: A 23-year-old male, smoker (5 pack-years), was evaluated in the ER due to left pleuritic chest pain, worsening with deep breathing and Valsalva maneuver. It was not associated with trauma and did not present other symptoms. The physical examination was unremarkable. The arterial blood gases while breathing room air and the laboratory tests, including D-dimers and high-sensitivity cardiac Troponin T, were normal. The chest radiograph, electrocardiogram, and transthoracic echocardiogram showed no abnormalities. A computed tomography (CT) pulmonary angiogram showed no signs of pulmonary embolism but depicted at the left cardiophrenic angle a focal 3 cm ovoid-shaped fat lesion with stranding and thin soft tissue margins, consistent with necrosis of the epicardial fat, which was confirmed by magnetic resonance (MRI) of the chest. The patient was medicated with ibuprofen and pantoprazole, with clinical improvement in four weeks. At a two-month follow-up, he was asymptomatic and presented radiologic resolution of the inflammatory changes of the epicardial fat of the left cardiophrenic angle on chest CT. Laboratory tests revealed positive antinuclear antibodies, positive anti-RNP antibody, and positive lupus anticoagulant. The patient complained of biphasic Raynaud's phenomenon initiated five years ago, and a diagnosis of undifferentiated connective tissue disease (UCTD) was made. Conclusions: This case report highlights the diagnosis of EFN as a rare and frequently unknown clinical condition, which should be considered in the differential diagnosis of acute chest pain. It can mimic emergent conditions such as pulmonary embolism, acute coronary syndrome, or acute pericarditis. The diagnosis is confirmed by CT of the thorax or MRI. The treatment is supportive and usually includes non-steroidal anti-inflammatory drugs. The association of EFN with UCTD has not been previously described in the medical literature.