Carlos Viana Poyares Jardim

Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease

Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its features in patients with SCD. 80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitant jet velocity (TRV) of ≥2.5 m·s−1 were further evaluated through right heart catheterisation (RHC) to assure the diagnosis of PH. Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiography. RHC (performed in 25 out of 32 patients) confirmed PH in 10% (95% CI 3.4–16.5%) of all patients, with a prevalence of post-capillary PH of 6.25% (95% CI 0.95–11.55%) and pre-capillary PH of 3.75% (95% CI -0.4–7.9%). Patients with PH were older, had worse performance in 6MWTs, and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH. Our study reinforced the use of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced haemolytic profile.

Pulmonary artery distensibility in pulmonary arterial hypertension: an MRI pilot study

Pulmonary arterial hypertension (PAH) is a disease of the small vessels in which there is a substantial increase in pulmonary vascular resistance leading to right ventricle failure and death. Invasive haemodynamic evaluation is mandatory not only for diagnosis confirmation but also to address prognosis and eligibility for the use of calcium-channel blockers through an acute vasodilator challenge. Noninvasive surrogate response markers to the acute vasodilator test have been sought. In the present study, the relationship between pulmonary artery distensibility, assessed using magnetic resonance imaging (MRI), and response to acute vasodilator tests was investigated. In total, 19 patients diagnosed with idiopathic PAH without any specific treatment were evaluated. Within a 48-h window after pulmonary artery catheterisation, patients underwent cardiac MRI. Cardiac index, calculated after the determination (invasively and noninvasively) of cardiac output, showed excellent correlation, as did right atrial pressure and right ventricle ejection fraction. Pulmonary artery distensibility was significantly higher in responders. A receiver operating characteristic curve analysis has shown that 10% distensibility was able to differentiate responders from nonresponders with 100% sensitivity and 56% specificity. The present findings suggest that magnetic resonance imaging and pulmonary artery distensibility may be useful noninvasive tools for the evaluation of patients with pulmonary hypertension.

Trends in pulmonary arterial hypertension

Past decades have witnessed an increasing interest in the field of pulmonary arterial hypertension (PAH). The large number of publications related to PAH reflects this interest. As a result, in contrast to 15 yrs ago, there are now at least seven different drugs as specific therapy targeting the pulmonary circulation that are approved for use in the USA and European Union, with some of them also approved for use in Canada, Japan, Australia and in some countries in Latin America. These medications have proved to increase exercise capacity [1–5], haemodynamics [2, 3, 6], quality of life [7–9] and even survival [5]. Nevertheless, there is still room for further improvements, since the mortality rate remains significantly high [10, 11]. Haemodynamically, PAH is characterised by progressive increase in pulmonary vascular resistance, as a consequence of vascular remodelling within the pre-capillary territory, leading to right ventricular failure and, eventually, death [12]; numerically, this is translated to the presence of a mean pulmonary artery pressure >25 mmHg, with normal pulmonary artery occlusion pressure (<15 mmHg) [13]. This definition, although extremely important for the proper characterisation of a pre-capillary impairment, might be misleading when focusing on specific patients or, more properly, specific forms of PAH. The most recent published nomenclature for pulmonary hypertension date from 2003 [13], and despite the fact that it has been recently revised (at the International Symposium of Pulmonary Hypertension, held in Dana Point, CA, USA, in the first quarter of 2008) it still includes in group 1 (PAH) several diseases that share the haemodynamic profile of pre-capillary pulmonary hypertension but present a completely distinct clinical course, such as idiopathic PAH, scleroderma-associated PAH and portopulmonary hypertension [10, 14, …

Other causes of PAH (schistosomiasis, porto-pulmonary hypertension and hemolysis-associated pulmonary hypertension).

Recent years have witnessed a significant increase in the knowledge about the pathophysiology of pulmonary arterial hypertension (PAH) and the availability of multiple drugs specifically aimed at pulmonary circulation. Although most of this is related to the idiopathic form of PAH, this development has also turned attention to other forms of pulmonary hypertension such as schistosomiasis-associated PAH (Sch-PAH), portopulmonary hypertension (POPH), and hemolysis-associated pulmonary hypertension. The importance of these different forms of pulmonary hypertension spans their epidemiology and the multiple pathophysiological mechanisms associated with their development and complications. Taken together, Sch-PAH and hemolysis-associated pulmonary hypertension may represent the most prevalent forms of PAH worldwide. Portopulmonary hypertension is particularly important if the morbidity and mortality that it adds to patients with liver disease is considered. Although clear progress has been reached in these various forms of PAH, there are many different aspects yet to be addressed that may contribute to the basis of specific treatment applied to these subgroups of patients.

Schistosomiasis and pulmonary hypertension

Schistosomiasis is one of the most prevalent chronic infectious diseases in the world. One of its most severe complications, pulmonary hypertension, occurs in up to 5% of patients with hepatosplenic schistosomiasis. The prevalence of schistosomiasis is so overwhelming that schistosomiasis-associated pulmonary hypertension (Sch-PH) may be the most prevalent cause of pulmonary hypertension around the world. Multiple pathways have been described as potential mechanisms of disease in Sch-PH, such as egg embolism, inflammatory disease or pulmonary blood overflow. The possible physiopathological mechanisms will be discussed in this article, as well the disease’s clinical course and response to the treatment available.

Schistosomiasis associated pulmonary hypertension

Schistosomiasis is one of the most prevalent infectious diseases, endemic in more than 70 countries, mainly within the developing world. More than 200 million people might be infected worldwide; about 20 million of those might develop severe disease. The hepatosplenic form of schistosomiasis is the most prevalent form of chronic disease, characterised by the presence of periportal fibrosis and portal hypertension. Pulmonary hypertension is a well‐recognised complication of hepatosplenic schistosomiasis. Recent prevalent studies revealed that schistosomiasis patients may develop precapillary and postcapillary forms of pulmonary hypertension, reinforcing the role of invasive haemodynamic measurements for the proper diagnosis. These studies also demonstrated that schistosomiasis associated pulmonary arterial hypertension may represent the most prevalent form of pulmonary arterial hypertension (PAH). Many aspects regarding the appropriate management of Sch‐PAH patients still remain to be elucidated, as the use of specific PAH therapy. Although the ongoing control programmes that started within the 1980s have clearly improved the schistosomiasis cenario worldwide, Sch‐PAH will be seen for decades after proper control is reached, strengthening the current need for comprehensive studies aiming to clarify the multiple mechanisms involved in the pathophysiology of this particular subgroup of PAH.

Left ventricular dysfunction in patients with suspected pulmonary arterial hypertension

OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH). METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH-in the absence of severe left ventricular dysfunction (LVD), significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism-between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH) was confirmed in 302 patients (78.6%). The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3%) and 178 (81.7%) were diagnosed with PH associated with LVD (PH-LVD) and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001). CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant.

Doença veno-oclusiva pulmonar: alternativas diagnósticas e terapêuticas

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Surgical biopsy was usually required for diagnostic confirmation. However, the morbidity, mortality and limited benefit of this procedure have generated discussion regarding noninvasive diagnostic techniques. We present the case of a female patient with progressive dyspnea, hypoxemia and pulmonary hypertension, the last diagnosed via catheterization. Computed tomography revealed septal thickening and diffuse micronodules. Bronchoalveolar lavage revealed occult alveolar hemorrhage. Treatment with an endothelin antagonist was started, resulting in symptomatic and functional improvement. Occult alveolar hemorrhage differentiates PVOD from idiopathic pulmonary hypertension. We believe that this finding, in combination with characteristic tomographic findings, is sufficient to establish a diagnosis of PVOD.

Contemporary issues in pulmonary hypertension

In recent years, important contributions to the understanding of pulmonary hypertension (PH) have been published in core clinical journals [1]. Relevant and thought-provoking studies evidencing the high quality of research in the PH field have been carried out by specialists worldwide. We have decided to focus on special issues we believe to be, on the one hand, pertinent to the understanding of what has happened hitherto and, on the other hand, useful to lay the new foundations on which future research will be based. Amongst the articles published in the last couple of years, we believe that the following groups of interest deserve special attention: the summary of the fourth World Symposium on PH; the guidelines on PH (both the American and the European); and the studies that shed new light on the discussion of survival in PH. In June 2009, the results of the discussion of working groups on specific issues of PH were published in 11 articles and one editorial in the Journal of the American College of Cardiology [2–13]. The articles covered a vast array of topics on PH, from basic research (comprising development, pathology, inflammation, genetics, and cellular and molecular basis of PH) to clinical issues, such as classification, diagnosis, the role of surgery and medical treatment in pulmonary arterial hypertension (PAH). Interestingly, there were also papers on end-points and clinical trials, and on future perspectives for the treatment of PAH. The article entitled “Updated clinical classification of pulmonary hypertension” [3] aimed at grouping together different PH manifestations with similar pathophysiological mechanisms, clinical presentation and therapeutic options. In spite of the maintenance of the general architecture of the classification compared to the previous classifications (Second and Third World Symposium on Pulmonary Hypertension in 1998 (Evian, France) and 2003 …

Clinical response to sildenafil in pulmonary hypertension associated with Gaucher disease.

SummaryThere are few reports of pulmonary hypertension in Gaucher disease. We report a patient who showed significant clinical improvement after treatment with sildenafil.