Cardiac findings in bilharziasis without pulmonary arterial hypertension]

1. A systematic search was made for cardiac abnormalities (clinical, radiological and ECG) and haemodynamic disorders (catheterisation of the right side of the heart and pulmonary artery) in 37 cases of confirmed biharziasis. It was generally found that:--14 patients (37.8%) had no symptoms;--8 patients (21.6%) had pulmonary hypertension (PH) with the corresponding cardiac signs (these will be reported separately);--15 patients (40.5%) had cardiac signs with no PH; these are studied in this paper. 2. Iatrogenic myocarditis was noted on the ECG. It was found in 3 of the 37 cases (8.1%). This points to the importance of careful monitoring of treatment. There was also ECG evidence of anaemyic myocardial changes in 5 cases of the 37 (13.5%). In 3 of these 5 cases this was found to be due to bleeding. Hypertensive myocardial disease was found in 1 case (2.7%), a patient with renal disease. Myocardial signs were also found in 3 cases because of coexistant disease. 3. Three cases (8.1%) remain in whom there were ECG changes, but without PH and without any other definable cause. These could have been due to bilharzia myocarditis; the authors review the experimental and clinical evidence for such a possibility. The real incidence of this condition remains to be determined. 4. Although there was no such case in this series, the authors suggest that bilharziasis might cause endomyocardial fibrosis (EMCF). 5. The authors put forward the hypothesis that the pulmonary arteritis is a tissue immunological reaction, and also that the myocarditis (and possibly the EMCF) is a manifestation of circulating antibodies.     

Effects of long-term bosentan in children with pulmonary arterial hypertension

OBJECTIVES: This study investigated the long-term outcome of children with pulmonary arterial hypertension (PAH) treated with bosentan therapy, with or without concomitant prostanoid therapy. BACKGROUND: Bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, improves hemodynamics and exercise capacity in adults with PAH; however, limited data are available on its long-term effects in children. METHODS: In this retrospective study, 86 children with PAH (idiopathic, associated with congenital heart or connective tissue disease) started bosentan with or without concomitant intravenous epoprostenol or subcutaneous treprostinil therapy. Hemodynamics, World Health Organization (WHO) functional class, and safety data were collected. RESULTS: At the cutoff date, 68 patients (79%) were still treated with bosentan, 13 (15%) were discontinued, and 5 (6%) had died. Median exposure to bosentan was 14 months. In 90% of the patients (n = 78), WHO functional class improved (46%) or was unchanged (44%) with bosentan treatment. Mean pulmonary artery pressure and pulmonary vascular resistance decreased (64 +/- 3 mm Hg to 57 +/- 3 mm Hg, p = 0.005 and 20 +/- 2 U x m2 to 15 +/- 2 U x m2, p = 0.01, respectively; n = 49). Kaplan-Meier survival estimates at one and two years were 98% and 91%, respectively. The risk for worsening PAH was lower in patients in WHO functional class I/II at bosentan initiation than in patients in WHO class III/IV at bosentan initiation. CONCLUSIONS: These data suggest that bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, with or without concomitant prostanoid therapy, is safe and efficacious for the treatment of PAH in children.     

70例肺栓塞心电图的分析

目的:探讨肺栓塞(PE)及合并冠心病(CAD)患者的心电图(ECG)变化。方法:回顾我院心内科2003年1月至2005年7月确诊为肺栓塞70例患者的ECG变化。患者均经核素肺通气灌注扫描和冠状动脉造影明确诊断。其中50例患者PE合并CAD。结果:70例PE患者ECG异常的发生率为70.0%。其中有助于PE诊断的ECG变化包括:肺型P波、SⅠQⅢTⅢ、完全性右束支传导阻滞(RBBB)以及心前导联T波倒置的总发生率为22.9%。比较单纯PE与PE合并CAD患者ECG异常变化,2组患者ECG异常发生率差异无统计学意义(χ2=2.99,P=0.084)。结论:ECG异常变化对临床PE提供的诊断价值较低。     

Electrocardiographic and hemodynamic correlations in primary pulmonary hypertension

In order to evaluate the pulmonary hemodynamics in primary pulmonary hypertension, the relation between the standard 12-lead electrocardiogram (ECG) and pulmonary hemodynamics as determined by right-heart catheterization was analyzed. Significant positive correlations were noted between amplitude of the R in V1, the R/S ratio in V1, and the pulmonary artery systolic pressure (r = 0.46 and 0.50, respectively, p less than 0.01). An amplitude of the R in V1 of more than 1.2 mV indicated a pulmonary artery systolic pressure of more than 90 mmHg with a sensitivity of 94% and a specificity of 47%. The cardiac index showed a significant positive relationship with amplitude of the R in V5 and V6 and the R/S ratio in V5 and V6 (r = 0.46, 0.46, 0.39, and 0.48, respectively; each with a p less than 0.01). Moreover, an AQRS greater than or equal to 100 degrees, and either an SV6 greater than or equal to 0.7 mV, or R/SV6 less than or equal to 2 indicated a cardiac index of less than 2.8L/min/m2 with a sensitivity of 82% and 84% and a specificity of 86% and 100% respectively. This study suggests, therefore, that the 12-lead ECG is useful for the evaluation of the severity of pulmonary hypertension by its ability to predict pulmonary artery systolic pressure and cardiac index with clinically useful accuracy.     

肺动脉高压的生物标志物

肺动脉高压(PAH)是一类预后不良的进展性疾病,其特点是血管收缩和重构引起肺动脉压升高及右心衰。PAH的早期诊断有利于提高患者长期生存率,对精准治疗尤为重要。生物标志物是PAH诊断、评估预后和治疗反应的非侵入性客观指标。该文介绍了与右心功能不全和神经内分泌激素激活、心肌损伤、炎性反应和氧化应激、血管损伤和重建、终末器官损伤等相关的生物标记物在PAH中的应用。     

Inflammation in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling of the precapillary pulmonary arteries, with excessive proliferation of vascular cells. Although the exact pathophysiology remains unknown, there is increasing evidence to suggest an important role for inflammation. Firstly, pathologic specimens from patients with PAH reveal an accumulation of perivascular inflammatory cells, including macrophages, dendritic cells, T and B lymphocytes, and mast cells. Secondly, circulating levels of certain cytokines and chemokines are elevated, and these may correlate with a worse clinical outcome. Thirdly, certain inflammatory conditions such as connective tissue diseases are associated with an increased incidence of PAH. Finally, treatment of the underlying inflammatory condition may alleviate the associated PAH. Underlying pathologic mechanisms are likely to be "multihit" and complex. For instance, the inflammatory response may be regulated by bone morphogenetic protein receptor type 2 (BMPR II) status, and, in turn, BMPR II expression can be altered by certain cytokines. Although antiinflammatory therapies have been effective in certain connective-tissue-disease-associated PAH, this approach is untested in idiopathic PAH (iPAH). The potential benefit of antiinflammatory therapies in iPAH is of importance and requires further study.     

Prognostication in pulmonary arterial hypertension

Despite the advances in diagnostic and treatment strategies, and contemporary survival estimates suggesting improved outcomes, the prognosis of patients with pulmonary arterial hypertension (PAH) remains poor. To date, there is no consensus on which prognostic variables or risk prediction strategies best predict survival, at baseline and at different time points in the disease course. Even less clear is whether current prognostic variables accurately reflect disease severity and can sufficiently guide therapeutic decisions. This article reviews the most common factors used for prognostication in PAH, emphasizing that proper strategies for identifying patients at greatest risk are paramount.