Primary pulmonary hypertension is a rare, progressive disease with high mortality rates. The treatment of primary pulmonary hypertension is still based on high-cost drugs, not yet available in developing countries. Recently, the use of sildenafil as an alternative drug for primary pulmonary hypertension treatment has been reported. It is reported a case of a 21 year-old female patient with primary pulmonary hypertension, who presented an acute worsening of symptoms and decrease of oxygen saturation. The investigation revealed the existence of a patent oval foramen not previously seen, with right-to-left shunt. Sildenafil treatment was instituted in escalating doses. After 40 days of treatment, the echocardiogram showed resolution of the shunt, concomitant to oxygenation improvement. The authors believe that sildenafil is a feasible alternative for primary pulmonary hypertension treatment, although larger clinical trials are necessary to determine its clinical safety and efficacy.

Keywords: Pulmonary hypertension/therapy. Vasodilator agents/administration & dosage.

Severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. Pleuropulmonary complications of systemic lupus erythematosus occur in 50-70% of patients. Severe symptomatic pulmonary hypertension in systemic lupus erythematosus is rare and carries a bad prognosis because a fatal outcome can occur within months. The authors describe, for the first time, a patient with systemic lupus erythematosus with severe pulmonary hypertension and secondary antiphospholipid syndrome who responded favorably to oral sildenafil, after unsuccessful use of prednisone, intravenous cyclophosphamide, warfarin and diltiazem.

Keywords: Vasodilator agents/therapeutic use. Lupus erythematosuos systemic. Antiphospholipid antibodies. Phosphodiesterase inhibitors.

Idiopathic pulmonary arterial hypertension (PAH) patients with a positive response to acute vasodilator challenge and a clinical response to calcium channel blockers (CCBs) for at least one year are traditionally designated true responders. Nevertheless, little is known about a sustained response to CCBs over longer periods of time. We evaluated the loss of response to CCBs after long-term treatment in a cohort of idiopathic PAH patients previously classified as being true responders. Our data suggest that idiopathic PAH patients can lose clinical response to CCBs even after one year of clinical stability, reinforcing the need for constant multidimensional reevaluation to assess the need for targeted PAH therapies and to classify these patients correctly.

Keywords: Pulmonary hypertension; Vasodilator agents; Calcium channel blockers.

Background: The nucleoside adenosine is a potent vasodilator. Although its effect on the pulmonary arteries is well known, its influence on capillaries and veins has yet to be described. Objective: To evaluate the pre- and post-administration effects of adenosine on arterial and venous resistance in the pulmonary circulation of patients with primary pulmonary hypertension. Method: The study involved 7 patients with primary pulmonary hypertension and presenting a positive response to adenosine on the acute test. Before and after adenosine administration, arterial and venous resistances were determined by estimating pulmonary capillary pressure through analysis of pulmonary artery pressure decay curves. Results: Following adenosine administration, there was an increase in the cardiac index (from 1.71 ± 0.23 to 2.72 ± 0.74 L/min-1/m-2) and a decrease in pulmonary vascular resistance (from 2924 ± 1060 to 1975 ± 764 dynes/s/cm-5/m-2) with no significant variations in mean pulmonary artery pressure (pre: 75.6 ± 16.8 mmHg; post: 78.1 ± 18.8 mmHg), pulmonary wedge pressure (pre: 15.3 ± 1.5 mmHg; post: 15.4 ± 1.9 mmHg) and pulmonary capillary pressure (pre: 43.8 ± 5.8 mmHg; post: 44.5 ± 4.9 mmHg). The ratio between arterial resistance and total pulmonary vascular resistance also presented a less than significant variation (pre: 50 ± 15%; post: 49 ± 17%). These findings suggest that adenosine affects the capillaries and veins as well as the arteries. Conclusion: We can conclude that the adenosine mechanism is not restricted to the arterial aspect of the pulmonary circulation, and that analysis of pulmonary capillary pressure could prove useful in the study of various drugs that affect the pulmonary circulation.

Keywords: Key Words: Adenosine/pharmacocinetic. Adenosine/uso terapêutico. Blood pressure. Hypertension pulmonary.

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 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.

Objective: To correlate semiquantitative evaluation of intrapulmonary vascular dilatations (IPVD) with quantitative evaluation of shunt levels, as well as to describe clinical and pulmonary function findings in a sample of liver disease patients with IPVD. Methods: Patients presenting transthoracic echocardiography (TTE) positivity for IPVD underwent clinical evaluation, pulmonary function tests and pulmonary shunt quantification (scintigraphy with technetium-99m-labeled macroaggregated albumin and blood gas analysis after pure oxygen breathing). Results: A total of 28 liver cirrhosis patients were studied (mean age, 47.5 years; 60.7% were Child-Pugh class B). A 4-point, ascending scale was used as a measure of IPVD intensity, which was scored as 1, 2, 3 and 4, respectively, in 13 (46.4%), 9 (32.1%), 2 (7.1%) and 4 (14.3%) of the patients. Patients were divided into a low-intensity group (scores 1 and 2) and a high-intensity group (scores 3 and 4). The mean shunt assessed using scintigraphy was 14.9% in the sample as a whole and was lower in the low-intensity group (11.7% vs. 26.3%; p = 0.01). The mean shunt by blood gas analysis was higher in the high-intensity group (8.3% vs. 16.3%; p < 0.001). Mean PaO2 was lower in the high-intensity group. There was a negative correlation between DLCO and IPVD severity (r = −0.406, p = 0.01). Conclusions: TTE is a safe, useful tool for assessing IPVD severity in liver disease patients. The IPVD intensity assessed using TTE correlated with the intrapulmonary shunt values obtained through the quantitative methods evaluated, as well as with pulmonary gas exchange abnormalities.

Keywords: Anoxia; Liver cirrhosis; Hepatopulmonary syndrome; Echocardiography.

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.

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.

Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin.

Background: The aim of this study was to describe the signs of active tuberculosis in patients submitted to high resolution computerized tomography and the distribution of lesions along the pulmonary parenchyma. Material and methods: The high resolution CTs performed on 42 patients with tuberculosis either bacteriologically diagnosed or clinical suspected and later confirmed by bacteriological tests were studied. Results: The signs of disease activity observed were airspace nodule (83%), centrilobular nodule (74%), tree-in-bud pattern (67%), cavitations (67%), bronchial thickening (55%), parenchymal consolidations (48%), ground glass opacity (21%) and thickening of the inter and intralobular septa (9%). Conclusion: High resolution computed tomography can be strongly suggestive of disease activity, is particularly helpful in patients with negative smear and/or undetermined radiographs, and allows the establishment of proper treatment, even before mycobacteria are identified in means of culture.

Keywords: Pulmonary tuberculosis. X-ray computed tomography.

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.

A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia), organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP) foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses) foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.

Keywords: Síndrome POEMS; Hipertensão pulmonar; Glucocorticoides.

We describe a case of pulmonary Langerhans cell histiocytosis with a close temporal relationship between smoking cessation and radiological improvement. High-resolution computed tomography revealed multiple small nodules located in the upper and middle lobes of both lungs. Microscopy of these lesions showed histiocytic infiltration that reacted strongly to staining for S100 protein. The histiocytes resembling Langerhans cells showed strong reactivity for S100 protein. Smoking cessation was recommended and the patient complied. Chest X-ray and computed tomography performed 6 and 24 months later revealed almost complete resolution of the radiographic abnormalities. Despite the possibility that this evolution was attributable to spontaneous remission, in this case, the lesions did, in fact, disappear rapidly after smoking cessation.

Keywords: Key words: Histiocytosis. Langerhans-Cell. Tabaco use cessation.

Objective: To conduct a cross-cultural adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) as an instrument to evaluate the perception of symptoms, functional limitation, and health-related quality of life (HRQoL) in subjects diagnosed with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in Colombia. Methods: The adaptation process involved 3 phases: translation, cognitive debriefing interviews, and a validation survey. To evaluate the psychometric properties, we recruited individuals ≥ 18 years of age who had been diagnosed with PAH or CTEPH to take part in the latter two stages of the adaptation process. All individuals were being followed on an outpatient basis by the pulmonary hypertension programs at Hospital Universitario San Ignacio, Fundación Clínica Shaio,and Clínicos IPS, all located in the city of Bogotá, Colombia. Results: A Spanish-language version of the CAMPHOR was developed for use in Colombia. The internal consistency was excellent for the symptoms, functioning, and quality of life scales (Cronbach's alpha coefficients of 0.92, 0.87, and 0.93, respectively). Test-retest reliability was above 0.70. The evaluation of the convergent validity and known group validity of the CAMPHOR scales confirmed that there were moderate and strong correlations with the related constructs of the Medical Outcomes Study 36-item Short-Form Health Survey, version 2, as well as showing their capacity to discriminate disease severity. Conclusions: The Spanish-language version of the CAMPHOR developed for use in Colombia was the result of a translation and cultural adaptation process that allows us to consider it equivalent to the original version, having shown good psychometric properties in the study sample. Therefore, its use to assess the impact of interventions on the HRQoL of patients with PAH or CTEPH is recommended, in research and clinical practice.

Keywords: Quality of life; Hypertension, pulmonary; Pulmonary embolism; Psychometrics.

We assessed the effects of lodenafil on hemodynamics and inflammation in the rat model of monocrotaline-induced pulmonary hypertension (PH). Thirty male Sprague-Dawley rats were randomly divided into three groups: control; monocrotaline (experimental model); and lodenafil (experimental model followed by lodenafil treatment, p.o., 5 mg/kg daily for 28 days) Mean pulmonary artery pressure (mPAP) was obtained by right heart catheterization. We investigated right ventricular hypertrophy (RVH) and IL-1 levels in lung fragments. The number of cases of RVH was significantly higher in the monocrotaline group than in the lodenafil and control groups, as were mPAP and IL-1 levels. We conclude that lodenafil can prevent monocrotaline-induced PH, RVH, and inflammation.

Keywords: Hypertension, pulmonary; Monocrotaline; Interleukin-1.

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.

Actinomycosis is a chronic suppurative bacterial infection characterized by multiple abscesses, fistulous pathways, and fibrosis involving the face, neck, chest, and abdomen. It is caused by an anaerobic Gram-positive saprophytic bacterium ( Actinomyces). Primary actinomycosis of the lung is a rare disease that probably results from aspiration of oropharyngeal secretions. It can present as a chronic respiratory disease. The treatment of choice is antibiotic therapy with penicillin. The authors report the case of a 55-year-old female diagnosed with pulmonary actinomycosis and successfully treated with levofloxacin.

Keywords: Actinomycosis; Infection; Ofloxacin; Medical records.