Underuse of reperfusion therapy with systemic thrombolysis in high-risk acute pulmonary embolism in a Portuguese center.

INTRODUCTION: Reperfusion therapy is generally recommended in acute high-risk pulmonary embolism (HR-PE), but several population-based studies report that it is underused. Data on epidemiology, management and outcomes of HR-PE in Portugal are scarce. OBJECTIVE: To determine the reperfusion rate in HR-PE patients, the reasons for non-reperfusion, and how it influences outcomes. METHODS: In this retrospective cohort study of consecutive HR-PE patients admitted to a thromboembolic disease referral center between 2008 and 2018, independent predictors for non-reperfusion were assessed by multivariate logistic regression. PE-related mortality and long-term MACE (cardiovascular mortality, PE recurrence and chronic thromboembolic disease) were calculated according to the Kaplan-Meier method. Differences stratified by reperfusion were assessed using the log-rank test. RESULTS: Of 1955 acute PE patients, 3.8% presented with hemodynamic instability. The overall reperfusion rate was 50%: 35 patients underwent systemic thrombolysis, one received first-line percutaneous embolectomy and one rescue endovascular treatment. Independent predictors of non-reperfusion were: age, with >75 years representing 12 times the risk of non-treatment (OR 11.9, 95% CI 2.7-52.3, p=0.001); absolute contraindication for thrombolysis (31.1%), with recent major surgery and central nervous system disease as the most common reasons (OR 16.7, 95% CI 3.2-87.0, p<0.001); and being hospitalized (OR 7.7, 95% CI 1.4-42.9, p=0.020). At a mean follow-up of 2.5±3.3 years, the survival rate was 33.8%. Although not reaching statistical significance for hospital mortality, mortality in the reperfusion group was significantly lower at 30 days, 12 months and during follow-up (relative risk reduction of death of 64% at 12 months, p=0.013). Similar results were found for MACE. CONCLUSIONS: In this population, the recommended reperfusion therapy was performed in only 50% of patients, with advanced age and absolute contraindications to fibrinolysis being the main predictors of non-reperfusion. In this study, thrombolysis underuse was associated with a significant increase in short- and long-term mortality and events.

A Rare Case of a Chylous Pleural Effusion as the Initial Manifestation of Synchronous Tumors.

Multiple primary malignancies (MPMs) are defined as two or more histopathologically distinct malignancies in the same individual. MPMs are classified as synchronous when tumors are diagnosed within six months of each other. The most common malignancies in MPMs are melanoma, breast, lung, and prostate cancer. Synchronous lymphoma and solid tumors are relatively rare. In these cases, a multi-disciplinary approach to treatment is essential. The early detection of additional primary malignancies such as myeloid and lymphatic tumors will enable prompt management with curative intent. The authors present a case of diffuse B-cell non-Hodgkin lymphoma and invasive lobular breast carcinoma presented as a chylous pleural effusion.

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.

Graves' Disease Caused by SARS-CoV-2 Infection.

Graves' disease is an autoimmune disorder that results in hyperthyroidism, caused by autoantibodies to the thyrotropin receptor (TRAbs) stimulating thyroid hormone synthesis, giving rise to a variety of systemic manifestations such as goitre, dermatopathy and orbitopathy. The authors present the case of a 28-year-old man admitted to hospital for a 3-week history of fatigue, shortness of breath, palpitations and diffuse goitre, after recent mild SARS-CoV-2 infection. Laboratory investigation revealed hyperthyroidism with TRAbs elevation. Thyroid ultrasound confirmed a diffusely heterogeneous and irregular thyroid gland and a nodular image below the sternal notch. Thyroid scintigraphy excluded the nodule and confirmed a Graves' disease pattern. Following the initiation of methimazole, the patient had complete resolution of symptoms and normalization of thyroid values. The results suggest a possible association between Graves' disease and SARS-CoV-2 infection acting as a trigger. Graves' disease is an important differential diagnosis to keep in mind when patients present with hyperthyroidism after COVID-19 disease. LEARNING POINTS: Graves' disease may be induced after SARS-CoV-2 infection by a possible autoimmune pathway.Graves' disease induced by SARS-CoV-2 infection responds well to antithyroid medication.

Continuous Aspiration Mechanical Thrombectomy for the management of intermediate- and high-risk pulmonary embolism: Data from the first cohort in Portugal.

INTRODUCTION: This study describes the experience of a reference center using continuous aspiration mechanical thrombectomy for acute high- and intermediate-high-risk pulmonary embolism (PE). METHODS: Twenty-nine consecutive patients with acute central PE (48.3% high-risk PE; 82.8% in class >III from the original Pulmonary Embolism Severity Index score; median Charlson Comorbidity Index of 4) were treated with the Indigo® Mechanical Thrombectomy System between March 2018 and March 2020. Technical success was defined as successful placement of the device and initiation of aspiration thrombectomy. Clinical success was defined as any improvement in hemodynamic and/or oxygenation parameters, pulmonary hypertension or right heart strain at 48 hours, and survival to hospital discharge. Safety was defined as freedom from severe adverse events potentially related to the procedure. Three-month follow-up results were collected. RESULTS: Technical success was 96.6%. Miller index and systolic pulmonary arterial pressure were significantly reduced after the procedure (-5.5±3.0, and -10.2±11.5 mmHg, respectively, both p<0.001). There was a significant improvement in mean paO2/FiO2 ratio (+77.1±103.2; p=0.001), shock index (-0.4±0.4; p<0.001), need for aminergic support at 48 h after the procedure (-75.0%, p=0.006) and improvement in right ventricular function in 66.6% (p=0.008). Clinical success was 75.9%. Severe adverse event rate was 10.3%: two deaths during the procedure and one pulmonary macroembolization during device progression. In-hospital and three-month survival rates were 82.8% and 72.4%, respectively. CONCLUSIONS: Aspiration thrombectomy for acute high- and intermediate-high-risk PE is feasible with a high technical and clinical success rate. Nevertheless, all-cause mortality is still high, probably related to the baseline high-risk features of the studied population and associated comorbidities.

Time course of lung ultrasound findings in patients with COVID-19 pneumonia and cardiac dysfunction.

BACKGROUND: Lung ultrasound (LUS) is a valuable tool to predict and monitor the COVID-19 pneumonia course. However, the influence of cardiac dysfunction (CD) on LUS findings remains to be studied. Our objective was to determine the effect of CD on LUS in hospitalized patients with COVID-19 pneumonia. MATERIAL AND METHODS: Fifty-one patients with COVID-19 pneumonia participated in the study. Focused echocardiography (FoCUS) was carried out on day 1 to separate patients into two groups depending on whether they had FoCUS signs of CD (CD+ vs CD-). LUS scores, based on the thickness of the pleural line, the B-line characteristics, and the presence or not of consolidations, were obtained three times along the patient's admission (D1, D5, D10) and compared between CD+ and CD- patients. A correlation analysis was carried out between LUS scores and the ratio of the arterial partial pressure of oxygen to the fraction of the inspired oxygen (P/F ratio). RESULTS: Twenty-two patients were CD+ and 29 patients were CD-. Among the CD+ patients, 19 were admitted to the intensive care unit (ICU), seven received invasive mechanical ventilation (IMV), and one did not survive. Among the CD- patients, 11 were admitted to the ICU, one received IMV and seven did not survive. CD+ patients showed a significantly lower P/F ratio than CD- patients. However, LUS scores showed no between-group differences, except for fewer subpleural consolidations in the upper quadrants of CD+ than on CD- patients. CONCLUSION: In patients with COVID-19, CD contributed to a worse clinical course, but it did not induce significant changes in LUS. Our findings suggest that pathophysiological factors other than those reflected by LUS may be responsible for the differences in clinical condition between CD+ and CD- patients.

Protein-Losing Enteropathy Resolved by Helicobacter pylori Eradication.

Protein-losing enteropathy (PLGE) is an uncommon condition with a multifactorial origin, that is characterized by excessive loss of serum proteins into the gastrointestinal tract, resulting in hypoproteinaemia and oedema. The authors present the case of a 24-year-old man admitted to hospital for a 2-month history of lower extremity oedema and diarrhoea with a secretory pattern. Blood analysis revealed hypoalbuminaemia and iron deficiency anaemia. Liver disease and severe proteinuria were excluded as possible aetiologies. Upper gastrointestinal endoscopy revealed signs of chronic Helicobacter pylori gastritis. After completion of H. pylori eradication, the patient had complete resolution of clinical and laboratory abnormalities. The results suggest the need to consider less frequent aetiologies for peripheral oedema and hypoproteinaemia, such as PLGE, especially those caused by prevalent bacterial agents like H. pylori. LEARNING POINTS: Protein-losing enteropathy may be related to Helicobacter pylori infection.Protein-losing enteropathy and its associated symptoms may be resolved by H. pylori eradication.

Intraocular Lymphoma: When to Suspect a Sinister Cause of Ocular Haemorrhage.

Intraocular lymphoma (IOL) is a rare and life-threatening condition whose aetiology is unclear. Blurred vision, reduced vision, and floaters are common initial symptoms. Posterior vitreous detachment and haemorrhage rarely occur. The authors present the case of a 79-year-old man who initially presented with a 3-month history of fever, night sweats, significant weight loss, bilateral peri-orbital haematoma, red eyes and retro-orbital headache. Physical examination revealed fever, bilateral peri-orbital haematoma, subconjunctival haemorrhage and palpable cervical lymphadenopathy. CT scans detected conical intra-orbital lesions, cervical adenomegalies, expansive lesions in the adrenal glands, and thrombosis of the splenomesenteric confluent and posterior segment of the right branch of the portal vein. These findings were suggestive of a lymphoproliferative disorder. Aspiration cytology of the adrenal mass and inguinal adenopathies was compatible with diffuse large B-cell lymphoma with areas of transformation to Burkitt's lymphoma. We describe a rare form of lymphoma, and a very unusual presentation of primary intraocular lymphoma with atypical symptoms. LEARNING POINTS: Intraocular lymphoma is a rare disease and can mimic common infectious or non-infectious uveitis.Bleeding or periocular haematoma are suggestive of intraocular lymphoma.

Stroke and refractory hypoxaemia: complications of pulmonary embolism.

Acute pulmonary embolism is one of the main causes of cardiovascular mortality. Treatment should be guided according to mortality risk stratification, but an individualised and multidisciplinary approach is often required. Concomitant persistent hypoxaemia can be present in cases of intracardiac shunt. In this report, we describe a 46-year-old woman with a history of surgery, presenting with pulmonary embolism with refractory hypoxaemia and simultaneous ischaemic stroke. Fibrinolysis was successfully performed, and the patient made a full recovery. Additional investigations identified a patent foramen ovale, which was later closed. She had no recurrent thrombotic events.

Pleural Sarcoidosis and Occult Lymphatic Anthracosis: An Unusual Symptomatic Association.

Sarcoidosis is a chronic multisystemic inflammatory disease of unknown aetiology. Virtually any organ or system can be involved, resulting in a wide range of clinical presentation. Pleural sarcoidosis is rare. Pleural effusion can only be attributed to pleural sarcoidosis in the presence of pleural non-caseating epithelioid granulomas and after excluding other granulomatous diseases. Anthracosis is a pneumoconiosis associated with thoracic adenopathies and bronchial disease, and it is usually asymptomatic. The authors present a case of a middle-aged man hospitalized due to cough, right-sided pleuritic chest pain and trepopnoea. LEARNING POINTS: To pursue a systematic and exhaustive investigation of pleuraleffusion until a definitive diagnosis is established.To recognize atypical presentations of sarcoidosis with a high degree of suspicion whenever the initial investigation is inconclusive.

Pericardial Effusion as an Initial Presentation of Panhypopituitarism.

Pericardial effusion has a broad spectrum of clinical presentation, ranging from an incidental finding on imaging to a potentially fatal emergency such as pericardial tamponade, the most severe presentation. The authors present a case of a middle-aged male hospitalized due to shortness of breath. Initial work-up was positive for massive pericardial effusion with haemodynamic compromise. Additional study revealed panhypopituitarism. The acromegalic phenotype was suggestive of acromegaly secondary to pituitary adenoma, which had probably evolved to apoplexy. Hormone replacement was started with clinical improvement. At the 3-year follow-up, there was no evidence of recurrence of pericardial effusion. Panhypopituitarism is a relatively rare entity, but can lead to life-threatening complications such as adrenal crisis, coma and myxoedema-associated cardiac failure. Pericardial effusion is an extremely rare manifestation of secondary hypothyroidism. LEARNING POINTS: To recognize the clinical presentation of cardiac tamponade.To recognize atypical causes of pericardial effusion, such as severe panhypopituitarism.Hormonal replacement is efficient in treating panhypopituitarism.

Methicillin-sensitive S taphylococcus aureus bacterial endarteritis associated with vascular closure device.

Percutaneous endovascular procedures (PEPs) are increasingly common in clinical practice. Percutaneous closure devices (PCD) ensure safe and immediate haemostasis, reducing the length of hospitalisation and improving patient comfort. Infectious complications are rare. We present the case of a 65-year-old man who was admitted to hospital because of fever and weight loss. He had a history of carotid arterial disease, having been submitted to a PEP 3 weeks before. On admission, he presented feverishly. Anaemia and elevated inflammatory parameters were detected on basic chemistry. Blood cultures isolated methicillin-sensitive Staphylococcus aureus and antibiotic therapy was started. He maintained fever and developed signs of right lower limb ischemia. Bacterial endocarditis was ruled out. Positron emission tomography (PET)-scan revealed inflammatory activity involving the right femoral artery (RFA). Bacterial femoral endarteritis was confirmed on surgical exploration, which documented the presence of infected PCD and occlusion of RFA. After surgery, apyrexia and improvement of ischaemic signs were achieved.

Marantic endocarditis and paraneoplastic pulmonary embolism.

Cancer is frequently associated with a hypercoagulable state. Almost 15% of patients with cancer will suffer a thromboembolic event during their clinical course. The aetiology of this hypercoagulable state is multifactorial and includes procoagulant factors associated with malignancy as well as the host's inflammatory response. Cancer-associated thrombophilia can present as venous thromboembolism, migratory superficial thrombophlebitis, arterial thrombosis, disseminated intravascular coagulation, thrombotic microangiopathy and rarely non-bacterial thrombotic endocarditis (NBTE). In this paper, we will describe an uncommon presentation of lung cancer on a non-smoker middle-aged woman, with recent diagnosis of pulmonary embolism, who develops malignant recurrent pleural effusion, NBTE with cutaneous and neurological manifestations, with a rapid evolution into shock, culminating in death. Diagnosis of NBTE requires a high degree of clinical suspicion. The mainstay of treatment is systemic anticoagulation to prevent further embolisation and underlying cancer control whenever is possible.

Scleredema associated with Sjögren's syndrome.

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren's syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren's syndrome and scleredema adultorum of Buschke.

Scleredema associated with Sjögren’s syndrome

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

Giant left atrial thrombus: an unexpected finding.

The incidence of left atrial thrombi is higher in patients with mitral valve stenosis. Its presence and location have important implications in deciding on the therapeutic approach, particularly the need for valve repair. We describe the case of a 63-year-old patient, with asymptomatic moderate mitral stenosis, hospitalized due to community-acquired pneumonia, in whom investigation to exclude pulmonary thromboembolism revealed a giant left atrial thrombus, which required urgent surgery.

Intraventricular gradients and acute coronary syndromes: a case report.

Left ventricular outflow tract obstruction (LVOTO) has traditionally been associated with hypertrophic obstructive cardiomyopathy, but can occur in other clinical scenarios such as acute myocardial infarction (AMI). In some patients, LVOTO is absent at rest, being detectable only with provocation tests such as stress echocardiography. Timely diagnosis of this phenomenon is very important, as it has therapeutic implications, and relies on clinical suspicion and on recognizing substrates in which LVOTO can occur. We report a case of syncope and AMI associated with LVOTO with systolic anterior motion of the mitral valve and a significant intraventricular gradient.

[Inflammatory myopathy with an unusual evolution]. / Miopatia inflamatória com evolução menos comum.

Inflammatory myopathies are a heterogeneous group of conditions characterized by proximal muscle weakness, nonsuppurative inflammation of skeletal muscle, with elevated muscle enzyme levels and characteristic electromyography and muscle biopsy findings. The authors describe a clinical case of a young woman, admitted with a four day history of bilateral thigh myalgia. She was afebrile and without skin, mucosal or joint involvement. Thigh muscle palpation was painful. Complete blood count revealed leukopenia and thrombocytopenia. High levels of creatine kinase, serum aminotransferases and myoglobin were detected. Metabolic, toxic and drug-related causes were excluded as well as infectious diseases, malignant tumours and endocrine myopathies. Auto-antibodies for connective diseases were negative. Magnetic resonance imaging and electromyography of lower limbs were suggestive of inflammatory myopathy. Generalized muscle weakness and dysphagia were reported subsequently. Clinical and laboratorial improvement was seen after corticotherapy. Muscle biopsy revealed myopathy signs without inflammatory changes or vasculitis. After prednisolone reduction, presently without treatment, she remains asymptomatic with normal laboratorial findings. The authors emphasize in this case of inflammatory myopathy the unusual clinical and laboratory evolution and the importance of a cautious differential diagnosis.