Prevalence and predictors of chronic thromboembolic pulmonary hypertension following severe forms of acute pulmonary embolism.

INTRODUCTION AND OBJECTIVES: The true prevalence of chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) in the Portuguese population remains unknown. We aimed to assess the prevalence and predictors of CTEPH two years after a symptomatic high- (HR) or intermediate-high risk (IHR) PE. METHODS: We conducted a retrospective cohort study of patients admitted with PE between 2014 and 2019 to a Portuguese referral center for pulmonary hypertension. RESULTS: In this single-center registry of 969 patients admitted with PE (annual incidence of 46/100000 population), 194 had HR (5.4%) and IHR (14.7%) PE. After excluding patients who died or had no follow-up in the first three months, 129 patients were included in the analysis. The overall prevalence of suspected CTEPH by clinical assessment, Doppler echocardiography and V/Q lung scan was 6.2% (eight patients). CTEPH was confirmed by right heart catheterization in four of these (3.1%). Increased pulmonary artery systolic pressure (PASP) at admission (OR 1.12; 95% CI 1.04-1.22; p=0.005) and the presence of varicose veins in the lower limbs (OR 7.47; 95% CI 1.53-36.41; p=0.013) were predictors of CTEPH. PASP >60 mmHg at admission identified patients with CTEPH at follow-up with sensitivity and specificity of 83.3% and 76.3%, respectively. All patients diagnosed with CTEPH had at least two radiological findings suggestive of CTEPH at the index event. CONCLUSIONS: In our cohort, the prevalence of CTEPH in survivors of severe forms of acute PE was 6.2%. PASP above 60 mmHg and supporting radiological findings on the index computed tomography scan are highly suggestive of acute-on-chronic CTEPH.

Pelvic Desmoid Tumor: A Rare Case with Difficult Diagnosis and Treatment.

Desmoid tumors are rare benign neoplasms, with locally aggressive characteristics. Ongoing or previous pregnancy, antecedent trauma, and familial adenomatous polyposis are known risk factors. Still, the majority of cases are sporadic and its etiology is still unknown. These tumors may occur in any body site, but retroperitoneal and pelvic desmoid tumors are extremely rare. Nonspecific clinical and radiological findings lead to erroneous diagnosis in 50% of patients before surgery. We present a case of a young multiparous female with a deep infiltrative lesion adherent to the right pelvic sidewall leading to severe right hydroureteronephrosis and ipsilateral loss of renal function. Although deep endometriosis was suspected, malignancy features could not be excluded by imaging studies. The patient underwent an exploratory laparotomy for definite diagnosis and treatment, which led to right nephrectomy, hysterectomy, and right oophorectomy because of deep infiltration and difficult dissection. Definite histologic diagnosis revealed the presence of a pelvic desmoid tumor. Positive margins were encountered but, until this moment, no disease relapse occurred.

[Pulmonary metastasis in a cardiac angiosarcoma - case report and discussion]. / Metastização pulmonar na apresentação de angiossarcoma cardíaco - Caso clínico e discussão.

We present a case report of a 35 year-old male without any relevant former pathology admitted to the emergency room with atypical chest pain, cough and sputum with ECG changes suggesting pericarditis. He was initially admitted to the cardiology ward and experienced clinical improvement after initiating anti- inflammatory treatment. As intercurrence he had bacterial origin pneumonia complicated by pleural effusion (PE). After discharge patient was referred to a pulmonology appointment where aetiological investigation of the PE was instigated. Investigation revealed changes in the cardiac cavities and multiple lung nodules, suggesting subacute endocarditis with septic pulmonary embolism. Admitted to the pulmonology unit patient underwent videothoracsopy which diagnosed cardiac angiosarcoma with pulmonary metastisation. Within a few days there was a rapid evolution of the clinical picture, with cardiac failure and death of the patient without radiotherapy or adjuvant chemotherapy being started.

Imunoterapia específica - avaliação dinâmica e cinética in vivo do extrato terapêutico em doentes alérgicos / Specific immunotherapy - in vivo dinamic and kinetic evaluation of a therapeutic extract in allergic patients

Introdução: A imunoterapia específica (IT) é uma forma comum de tratamento da doença alérgica. O mecanismo preciso desta terapêutica não é conhecido, embora a eficácia clínica esteja plenamente documentada. O objetivo deste estudo foi avaliar a cinética da IT , aplicando uma técnica de Medicina Nuclear, marcação de leucócitos com 99mTc-HMPAO, em doentes em fase de manutenção e com excelente eficácia clínica à terapêutica.Pacientes e métodos: Foram estudados 14 doentes alérgicos agrupados de acordo com o tipo de extrato e via de administração: extratos aquosos subcutâneos (látex= 4 doentes; veneno de abelha= 2 doentes), extrato depot subcutâneo (ácaros= 2 doentes; gramíneas= 2 doentes); extrato modificado subcutâneo (gramíneas= 1 doente; Parietária= 1 doente) extrato sublingual (ácaros= 2 doentes). O grupo controle foi constituído por dois doentes alérgicos a ácaros, submetidos respectivamente a: injecção subcutânea de soluto salino e injecção de extrato bacteriano por via subcutânea (controle positivo).Simultaneamente à administração do extrato alergênico terapêutico, procedeu-se à re-injecção de leucócitos marcados com 99mTc-HMPAO, em veia periférica contralateral. A aquisição dinâmica decorreu durante 60 minutos, com matriz de 64x64, 2 imagens/ minuto em projeção torácica anterior. As aquisições estáticas, com matrizes 256x256, durante 5 minutos cada foram adquiridas aos 60, 90, 120, 180, 240, 300 e 360 minutos, em projeção torácica (anterior e posterior) e abdominal (anterior).Resultados: A atividade inflamatória no ocal de administração da IT para os extratos subcutâneos aquosos e depot iniciou-se na primeira hora e manteve um aumento ao longo do tempo de estudo. Para os extratos sublinguais a atividade inflamatória foi observada logo nos primeiros minutos. Todos os estratos subcutâneos condicionaram dreanagem linfática ascendente para áreas axilares homolaterais nos primeiros minutos após a adminstração do extrato e, posteriormente, para tecido linfóide do mediastino superior e anterior, e áreas cervicais. As focalizações torácicas estiveram presentes em todos os doentes estudados, o mesmo não acontecendo para a atividade de intestinal. A via sublingual não induziu focalizações axilares ou instetinais, mesmo tendo havido deglutição do alérgeno. Foram calculados coeficientes corrigidos de captação em áreas individualizadas (ROIs- region of interest) em relação aos coeficientes de captação das áreas de background.