Chronic thromboembolic pulmonary hypertension is the only potentially curable form of pulmonary hypertension, assuming that surgical treatment is possible. However, there are hindrances to making a definitive, noninvasive diagnosis. We present the case of a 40-year-old female patient with idiopathic pulmonary arterial hypertension, confirmed in 1994. This patient developed thrombi in pulmonary vessels (as an overlap syndrome) mimicking chronic thromboembolic pulmonary hypertension. The identification of these conditions, which present high intraoperative mortality and unsatisfactory surgical resolution, is quite difficult in clinical practice. We discuss the current approach to candidate selection for surgical treatment of chronic thromboembolic pulmonary hypertension and the possible repercussions of inappropriate selection.

Keywords: Hypertension, pulmonary/diagnosis; Hypertension, pulmonary/therapy; Pulmonary embolism; Endarterectomy.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.

Keywords: Hypertension, pulmonary/diagnosis; Hypertension, pulmonary/surgery; Hypertension, pulmonary/therapy; Hypertension, pulmonary/drug therapy; Pulmonary artery/pathology; Pulmonary embolism/complications.

Pulmonary thromboendarterectomy has been established as the standard method for the treatment of chronic thromboembolic pulmonary hypertension, with excellent results. However, repeat pulmonary thromboendarterectomy due to recurrence of pulmonary embolism has never been reported in the Brazilian literature. Its safety and effectiveness remain obscure. We report the case of a patient presenting recurrence of chronic thromboembolic pulmonary hypertension five years after the first pulmonary thromboendarterectomy and requiring a second operation for resolution of the symptoms.

Keywords: Hypertension, pulmonary; Pulmonary embolism; Endarterectomy/methods.

Objectives: Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed at reporting outcomes of CTEPH patients undergoing PEA within 10 years, focusing on advances in anesthetic and surgical techniques. Methods: We evaluated 102 patients who underwent PEA between January 2007 and May 2016 at the Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Changes in techniques included longer cardiopulmonary bypass, heating, and cooling times and mean time of deep hypothermic circulatory arrest and shortened reperfusion time. Patients were stratified according to temporal changes in anesthetic and surgical techniques: group 1 (January 2007-December 2012), group 2 (January 2013-March 2015), and group 3 (April 2015-May 2016). Clinical outcomes were any occurrence of complications during hospitalization. Results: Groups 1, 2, and 3 included 38, 35, and 29 patients, respectively. Overall, 62.8% were women (mean age, 49.1 years), and 65.7% were in New York Heart Association functional class III-IV. Postoperative complications were less frequent in group 3 than in groups 1 and 2: surgical complications (10.3% vs. 34.2% vs. 31.4%, p=0.035), bleeding (10.3% vs. 31.5% vs. 25.7%, p=0.047), and stroke (0 vs. 13.2% vs. 0, p=0.01). Between 3 and 6 months post-discharge, 85% were in NYHA class I-II. Conclusion: Improvements in anesthetic and surgical procedures were associated with better outcomes in CTEPH patients undergoing PEA during the 10-year period.

Keywords: Pulmonary embolism; Pulmonary hypertension; Endarterectomy; Hospital mortality; Survival analysis; Postoperative complications.

Objective: A significant number of patients with chronic thromboembolic pulmonary hypertension (CTEPH) are not eligible for pulmonary endarterectomy and may be treated with balloon pulmonary angioplasty (BPA). Although BPA programs have recently been developed in Brazil, no results have yet been published. The objective of this study was to assess the clinical and hemodynamic progression of the first patients treated with BPA at our center. Methods: This was an observational study of 23 patients with CTEPH enrolled in the BPA program of a specialized center in Brazil between 2015 and 2020. Results: After a mean of 5.6 ± 1.3 sessions and 11 ± 2.8 treated segments/patient (at a mean of 6.7 ± 2.9 months post-BPA), there was a 26% decrease in mean pulmonary artery pressure (51 ± 11 vs. 38 ± 11 mmHg; p < 0.0001), a 43% decrease in pulmonary vascular resistance (10 ± 3.7 vs. 5.7 ± 3.3 WU; p < 0.0001), and a 22.5% increase in the cardiac index (2.38 ± 0.6 vs. 2.95 ± 0.6 L/min/m2; p < 0.0001). There was an increase in the six-minute walk distance and an improvement in functional class. Acute lung injury with clinical manifestations was observed after 7% of the BPA sessions. None of the patients required intubation. During a mean outpatient follow-up period of 38 ± 22 months, two patients were referred for additional BPA sessions due to clinical worsening and new hospitalizations. Two deaths were recorded (due to CTEPH progression and gastrointestinal bleeding, respectively). Conclusions: Among this first group of patients treated with BPA in Brazil, there was significant short- and long-term clinical improvement, together with a low frequency of complications.

Keywords: Hypertension, pulmonary; Pulmonary embolism; Angioplasty, balloon.

Schistosomiasis is a disease that, in Brazil, is caused by Schistosoma mansoni and is transmitted by snails of the genus Biomphalaria. This species occurs in Africa, the Antilles and South America. The parasite, developing to the adult stage in the vascular system of the host, migrates to the mesenteric veins. Each female lays approximately 400 eggs per day, and these travel from the submucosa to the intestinal lumen. There have been reported cases in which S. mansoni eggs were identified in the lungs of patients with no evidence of liver fibrosis. We report a case with this atypical presentation of the disease. The patient presented nonpleuritic chest pain, significant weight loss and dry cough. A CT scan revealed an irregular tumor in the left lower lobe. However, a lung biopsy revealed non-viable S. mansoni eggs. The patient presented no clinical signs of pulmonary or portal hypertension; nor was either identified through diagnostic tests.

Keywords: Schistosomiasis; Schistosoma mansoni; Lung diseases, parasitic.

Objectives: To examine the relationship between chronic bacterial infection and pulmonary hypertension, using Doppler echocardiography, in patients with cystic fibrosis (CF). Methods: A prospective cross-sectional study involving CF patients (≥ 16 years of age) admitted to a program for adults with the disease. The study included 40 patients with a mean age of 23.7 ± 6.3 years. Patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, chest X-rays and sputum cultures of Pseudomonas aeruginosa and Burkholderia cepacia. Results: In terms of the following variables, no significant differences were found between P. aeruginosapositive patients and P. aeruginosa-negative patients: clinical score (p = 0.472); forced expiratory volume in one second (FEV1; p = 0.693); radiological score (p = 0.760); tricuspid regurgitant jet velocity (TRV, p = 0.330); diameter of the right ventricle (DRV, p = 0.191); and right ventricular/pulmonary artery (RV/PA) systolic acceleration time (SAT, p = 0.330). B. cepacia-positive patients presented significantly lower FEV1 than did B. cepacia-negative patients (p = 0.011). No significant differences were found between B. cepacia-positive patients and B. cepacia-negative patients regarding the following variables: clinical score (p = 0.080); radiological score (p = 0.760); TRV (p = 0.613); DRV (p = 0.429); and RV/PA SAT (p = 0.149). Conclusions: Chronic infection with P. aeruginosa or B. cepacia presented no association with pulmonary hypertension in adult CF patients. Pulmonary function was worse in B. cepacia-positive patients than in P. aeruginosa-positive patients.

Keywords: Cystic fibrosis; Bacterial infections; Hypertension, pulmonary; Echocardiography, Doppler.

Over the last five years, knowledge in the field of pulmonary hypertension has grown consistently and significantly. On the basis of various clinical studies showing the usefulness of new diagnostic tools, as well as the efficacy of new medications and drug combinations, new diagnostic and treatment algorithms have been developed. Likewise, in order to simplify the clinical management of patients, the classification of pulmonary hypertension has been changed in an attempt to group the various forms of pulmonary hypertension in which the diagnostic and therapeutic approaches are similar. The objective of this review was to discuss these modifications, based on the 2005 Brazilian guidelines for the management of pulmonary hypertension, emphasizing what has been added to the international guidelines.

Keywords: Hypertension, pulmonary/diagnosis; Hypertension, pulmonary/therapy; Clinical protocols.

Cough is the main physiological mechanism responsible for the clearance of secretions from airways, acting as an important defense mechanism. However, the presentation of chronic cough is one of the most important causes for patients to seek medical attention all over the world, thus the significance of the correct recognition of all the factors related to the process of cough. Among those factors, some are of extreme importance, since they may be present in almost 95% of the cases: post-nasal drip syndrome, asthma and gastroesophageal reflux disease. The main step for a successful therapy for chronic cough is a rational and progressive diagnostic approach, narrowing the list of possible diagnosis and allowing the institution of a treatment with a better cost-effectiveness. The proposal of an algorithm focusing on the most common causes of chronic cough may be helpful in this initial approach.

Keywords: Chronic cough. Diagnostic algorithm. Asthma. Postnasal drip syndrome. Gastroesophageal reflux disease.

Pulmonary thromboembolism (PTE) should be suspected in patients with risk factors for deep venous thrombosis (DVP) who present, either isolated or in association, malaise, dyspnea/tachypnea, sudden collapse or pulmonary hemorrhage syndrome. The diagnostic approach starts with basic tests (chest X-rays electrocardiographs, and arterial blood gas tensions) the results of which associated to medical history and to physical examination allow the classification of clinical suspicion as high, intermediate or low probability. This phase is important, both to support clinical suspicion of PTE and to exclude alternative diagnoses. In order to confirm or exclude PTE, one or several sophisticated imaging exams (lung isotope scanning, leg imaging, and pulmonary angiography) should be performed next, according to the findings of the chest X-ray. If there is undue delay in arranging investigations for patients with high clinical probability, heparin should be started, unless high risk to anticoagulation is present. In this review the diagnostic methods are discussed and some strategies for rapid investigation of PTE are shown with the main purpose of adapting them to Brazilian reality.

Keywords: Pulmonary thromboembolism. Diagnosis. Management strategy.

Objective: To determine the efficacy of using forced expiratory volume in six seconds (FEV6) as a surrogate for forced vital capacity (FVC) in the diagnostic screening for chronic obstructive pulmonary disease (COPD) by comparing FEV1/FVC ratios with FEV1/FEV6 ratios. Methods: In November of 2003, on World COPD Day, we conducted a campaign of diagnostic screening for COPD. The participants completed the clinical questionnaire of the Global Initiative for Obstructive Lung Disease, and those who responded affirmatively to at least three questions underwent spirometry. Results: A total of 134 individuals responded to three questions affirmatively and underwent spirometry. Of those, 59 were excluded: 45 for being non-smokers and 14 due to the fact that their tests did not meet the American Thoracic Society criteria for satisfactory spirometry. The number of tests in which the FEV1/FEV6 ratio was below 70% was similar to that found for the FEV1/FVC ratio. The sensitivity of FEV1/FEV6 in diagnosing airway obstruction (defined as FEV1/FVC below 70%) was 92%, and its specificity was 99%. The positive predictive value was 100%, and the negative predictive value was 98%. The Kendall correlation test revealed r = 0.99 (p < 0.0001). The t-test for paired samples revealed a negative correlation: t = −5.93 (p < 0.0001). Conclusion: The FEV1/FEV6 proved efficient for use in the diagnostic screening for COPD. There is a strong correlation between FEV1/FVC and FEV1/FEV6.

Keywords: Pulmonary disease, Chronic obstructive; Diagnosis; Spirometry; Forced expiratory volume; Vital capacity.

Schistosomiasis mansoni is the third most prevalent endemic parasitic disease in the world. It is estimated that over 200 million people are infected with parasites belonging to one of the Schistosoma species. Of those, 270,000 people (4.6%) suffer from pulmonary arterial hypertension, which is associated with the hepatosplenic form of the disease. This high prevalence makes schistosomiasis-associated pulmonary hypertension the leading cause of pulmonary hypertension worldwide. However, no specific treatment for the pulmonary vascular component of the disease has yet been devised. We report the case of a patient with schistosomiasis-associated pulmonary hypertension who was treated satisfactorily with a phosphodiesterase-5 inhibitor (sildenafil).

Keywords: Schistosomiasis mansoni; Hypertension, pulmonary; Drug therapy.

Pulmonary artery dissection is a fatal complication of long-standing pulmonary hypertension, manifesting as acute, stabbing chest pain, progressive dyspnea, cardiogenic shock, or sudden death. Its incidence has been underestimated, and therapeutic options are still scarce. In patients with pulmonary hypertension, new chest pain, acute chest pain, or cardiogenic shock should raise the suspicion of pulmonary artery dissection, which can result in sudden death.

Keywords: Hypertension, pulmonary; Chest pain; Pulmonary artery.

Chronic necrotizing pulmonary aspergillosis is one of the forms of pulmonary aspergillosis typically found in mildly immunocompromised patients. We report the case of a female patient with complaints of chronic productive cough, fever and asthenia. She reported previous corticosteroid use. Computed tomography of the chest revealed consolidation with interposed cavitation in the right upper lobe. Fiberoptic bronchoscopy revealed purulent fluid within the tracheobronchial tree and an endobronchial exophytic lesion. The results of the biopsy of that lesion and the transbronchial biopsy were consistent with aspergillosis. Based on the clinical, radiological and histopathological findings, the patient was diagnosed with chronic necrotizing pulmonary aspergillosis. Treated with itraconazole, the patient presented a favorable clinical-radiological evolution.

Keywords: Aspergillosis; Lung diseases, fungal; Itraconazole. Resumo

Histoplasmosis is a systemic mycosis caused by the thermally dimorphic fungus Histoplasma capsulatum, which can be isolated from soil contaminated with droppings from birds or bats. Chronic cavitary pulmonary histoplasmosis is one of the rarest clinical presentations of this disease. The differential diagnosis with tuberculosis should be made in patients presenting with cavitated lesions in upper lung segments. We report the case of a female patient with chronic cavitary pulmonary histoplasmosis who had presented with progressive dyspnea and worsening of the radiological pattern over a four-year period.

Keywords: Histoplasmosis; Cavitation; Pulmonary emphysema.