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.

Recent studies have suggested an association between pulmonary arterial hypertension (PAH) and thyroid diseases (hypothyroidism and hyperthyroidism). This combination has a good prognosis, because the increase in the pulmonary artery pressure is usually slight and reverses after the treatment of the thyroid disease. Although the exact mechanism involved in the pathogenesis of this combination has not yet been established, it has been hypothesized that thyroid hormones and autoimmunity have a direct influence. Due to the high prevalence of thyroid disease in patients with PAH, thyroid function tests should be considered in the investigation of every patient with PAH. In this review, we describe the prevalence of PAH in patients with thyroid diseases and the prevalence of thyroid disease in patients with PAH, as well as addressing the principal effects that thyroid diseases have on the respiratory system. In addition, we report the treatment effects in patients with these diseases.

Keywords: Hypertension, pulmonary; Graves disease; Hyperthyroidism; Hypothyroidism; Thyroid hormones; Echocardiography.

Behçet disease has been associated to pulmonary manifestations and may be lethal if not treated correctly. The authors describe a case of a 53 year-old male with aneurysmatic pulmonary artery dilatation, pulmonary hypertension, and hemoptysis. A review is presented about the diagnosis and the medical and surgical treatment of this disease.

Keywords: Behcet's syndrome. Pulmonary artery. Pulmonary hypertension. Aneurysm. Hemoptysis.

Pulmonary hypertension occurs when the pressure of the pulmonary artery is disproportionally high for a certain level of pulmonary blood flow. Values of mean pulmonary artery pressure of more than 25 mm Hg at rest or 30 mm Hg during exercise allow the diagnosis of pulmonary hypertension. Sustained or chronic pulmonary hypertension may be secondary to known diseases, mainly to those of cardiac or pulmonary nature, or may be a primary abnormality of the pulmonary circulation, with or without identification of associated conditions. Advances in the knowledge of the mechanisms of vasoconstriction and vascular remodeling have brought better prospects for the treatment of the disease. The correct use of vasodilators and anticoagulants, the new vasodilators, as epoprostenol and its analogs, and surgical techniques have increased the survival of many patients. Pneumologists can view cases of pulmonary hypertension as complications of pulmonary diseases or as a result of dyspnea investigation. Despite its etiology, pulmonary hypertension represents a clear abnormality that affects the right ventricle and can be potentially fatal to patients. Image methods have made the diagnosis of pulmonary hypertension more accessible and non-invasive. Six cases of patients with pulmonary hypertension of different causes are presented and discussed. In conclusion, according to new concepts, idiopathic pulmonary hypertension is no longer an irreversible condition, and the identification of associated conditions with potential treatments can be favorable in the management of the patients with pulmonary hypertension.

Keywords: pulmonary hypertension, vascular resistance, lung diseases, vasodilator agents

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.

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.

Behçet's disease is a systemic disease with clinical manifestations, whose underlying histopathologic lesion is a non-specific vasculitis. Pulmonary artery aneurysms and, more rarely, pleuropulmonary manifestations may be found. Some reports are available in the literature about the use of immunosuppressive agents in these conditions. The authors describe a case in whom clinical remission and radiologic resolution were observed after corticosteroid and cyclophophamide therapy.

Keywords: Behçet's disease. Pulmonary artery aneurysms.

Intimal sarcoma of the pulmonary artery is a rare and potentially lethal tumor, the diagnosis of which is difficult and therefore frequently delayed. The clinical signs and symptoms are nonspecific, often mimicking chronic pulmonary thromboembolism (CPTE). We report the case of a 45-year-old male under treatment for CPTE associated with pulmonary arterial hypertension and chronic cor pulmonale. There was no response to treatment with anticoagulants and sildenafil. We emphasize the difficulties in diagnosing intimal sarcoma of the pulmonary artery, the need to investigate this neoplasm in the differential diagnosis of CPTE and the systematic use of criteria for the appropriate prescription of new medications for pulmonary artery hypertension.

Keywords: Pulmonary embolism; Hypertension, pulmonary; Hemangiosarcoma; Diagnosis, differential.

Pulmonary hypertension is a common complication of sickle cell anemia. Despite the fact that the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of sickle cell anemia-related pulmonary hypertension is multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients, pulmonary arterial hypertension is the main cause of elevated pulmonary artery pressures. However, pulmonary venous hypertension also plays a role in a subgroup of patients. Specific data on the effects of treatment modalities for pulmonary hypertension in patients with sickle cell anemia are scarce. It is likely that all patients would benefit from maximization of sickle cell anemia therapy, and that patients with the severe form of the disease would benefit from treatment with selective pulmonary vasodilators and antiproliferative agents. Large trials evaluating the effects of treatment for pulmonary hypertension in the sickle cell anemia population are underway.

Keywords: Anemia, Sickle cell; Hemolysis; Hypertension, pulmonary; Nitric oxide.

Primary pulmonary hypertension (PPH) is a disease of rare, unclear etiology, leading to elevations in pulmonary artery pressure and pulmonary vascular resistance. The vascular lesions range from mild - consisting of neo-intimal proliferation - to severe - characterized by concentric intimal fibrosis, plexiform lesions, and necrotizing arteritis. There have been since 1987 reports describing the association between human immunodeficiency virus (HIV) and PPH, with as many as 60 patients in 1997. The role of HIV in the pathogenesis remains unclear. It is reported a case of a woman in whom this association was observed.

Keywords: Primary pulmonary hypertension. Human immunodeficiency virus.

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.

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.