Survival in Schistosomiasis-Associated Pulmonary Arterial Hypertension

Fernandes, Constantino José; Jardim, Carlos; Hovnanian, Andre; Hoette, Susana; Dias, Bruno; Souza, Silvia; Humbert, Marc; Souza, Rogério

doi:10.1016/j.jacc.2010.03.065

Cited by 68 publications

(73 citation statements)

References 32 publications

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“…This study suggests that the etiology of pulmonary hypertension may be closely associated to portal hypertension, a physiopathogeny that shows no significant pulmonary vasoreactivity 8,3 , which is in disagreement with other studies that indicate many similarities between these etiologies, with SPAH being recently reclassified from group IV to group I 17,[27][28][29] . The findings in the analysis are consistent with the fact that patients with SPAH are vasoreactive, but at a value below those recorded for idiopathic PAH, which is around 20% 30 .…”

Section: Discussioncontrasting

confidence: 91%

“…Moreover, there was no association with mean pulmonary artery pressure. Of the 84 patients with SPAH, it was observed that 3.5% of them showed criterion for a positive vasoreactivity test with nitric oxide, below the value found for the idiopathic form (10% -20%) 30,35,36 and different from that found by Fernandes et al 8 , in which of 54 schistosomiasis patients, there were no vasoreactive cases. This finding consists in new data that would show that the pulmonary vasoreactivity test is useful in the assessment of severity in patients with schistosomiasis.…”

Section: Discussioncontrasting

confidence: 59%

“…In the study by Fernandes et al designed to analyze survival in patients with SPAH, the study of 54 schistosomiasis patients versus 95 idiopathic ones showed that no patient with schistosomiasis was vasoreactive, against 16.2% of positive vasoreactivity in idiopathic ones 8 . This study suggests that the etiology of pulmonary hypertension may be closely associated to portal hypertension, a physiopathogeny that shows no significant pulmonary vasoreactivity 8,3 , which is in disagreement with other studies that indicate many similarities between these etiologies, with SPAH being recently reclassified from group IV to group I 17,[27][28][29] .…”

Section: Discussionmentioning

confidence: 99%

“…Some less advanced cases have been prospectively studied, with a poor prognosis; however, it has been demonstrated that some patients may remain clinically stable for years, indicating a more benign clinical course when compared to other forms of PAH (3-year mortality of 14.1% versus one-year mortality of 15%, in idiopathic PAH) 7,8 . PAH is defined when there is an increase in mean pulmonary artery pressure at rest ≥ 25 mmHg, measured by right cardiac catheterization 3 .…”

Section: Introductionmentioning

confidence: 99%

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Japyassú¹,

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Bandeira³

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Section: Discussioncontrasting

confidence: 91%

Section: Discussioncontrasting

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Perfil hemodinâmico de gravidade ao teste de vasorreatividade pulmonar em esquistossomóticos

Japyassú¹,

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Bandeira³

et al. 2012

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“…These multiple possible pathways may somehow explain the better clinical course of Sch-PAH. When compared to IPAH, Sch-PAH patients present with a better haemodynamic profile at diagnosis and better prognosis; nonetheless, they still have a 3-yr mortality rate of ,15% [28]. Since schistosomiasis is a highly prevalent disease worldwide and given the non-negligible mortality rate, the reclassification of Sch-PAH in group 1 based on pathological and haemodynamic studies may warrant trials with specific treatments for this highly prevalent condition.…”

Section: Fourth World Symposium On Phmentioning

confidence: 99%

Contemporary issues in pulmonary hypertension

Jardim

Hoette²,

Souza³

2010

European Respiratory Review

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Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

et al. 2016

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We read with interest the recently published article by Fussner et al. (1) in Liver Transplantation. The authors showed that intrapulmonary vascular dilatations (IPVDs), detected by agitated saline contrast-enhanced transthoracic echocardiography (cTTE), were common in patients with portopulmonary hypertension (POPH)-59% of patients. Moreover, this finding was associated with decreased survival, and the authors suggest the possibility of a pathophysiological overlap between POPH and hepatopulmonary syndrome in patients with end-stage liver disease. (1) Interestingly, this feature was previously described in patients with schistosomiasis. (2,3) Early reports of the disease identified a unique pattern of pulmonary involvement in patients with schistosomiasis: pulmonary hypertension with central cyanosis in the absence of intracardiac shunt. (2) In 1 study, IPVD was found in 20% of patients with schistosomiasis cor pulmonale. (3) According to necroscopic studies, cyanosis was associated with arteriovenous fistulae, possibly due to bypass of Schistosoma eggs in pulmonary circulation. Furthermore, other investigators confirm this finding and hypothesized that it could be related to repeated worm infestation, common in endemic areas. The pathophysiology of schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is not completely understood, but it probably shares some similarities with POPH, in a model without liver cirrhosis.Sch-PAH is potentially the most common cause of pulmonary hypertension worldwide and is associated with higher survival rates compared to IPAH. (4) Our group recently published an article contrasting physiological responses to incremental exercise in cardiopulmonary exercise tests (CPETs) between naive patients with Sch-PAH (n 5 8) and with idiopathic pulmonary arterial hypertension (IPAH) (n 5 9) with similar resting hemodynamics. Interestingly, Sch-PAH showed less impaired physiological responses to incremental exercise than IPAH patients, suggesting a more preserved cardiopulmonary response to exercise in Sch-PAH, which could be related to its better clinical course compared to IPAH. (5) However, at that time, the mechanisms involved were not identified. Even though none of the patients with Sch-PAH had cyanosis or hypoxemia (only 3 had SpO 2 90% at the end of the incremental CPET), cTTE was performed after the publication, and IPVD was detected in 6/8 (75%) Sch-PAH patients (data not published). It is noteworthy that despite the high prevalence of IVPD in this study, only 2 patients died to date (1 of them was without IPVD), which contrasts with the poor prognosis of the presence of IPVD in patients with cirrhosis described by Fussner et al. (1) The clinical relevance of the presence of IPVD in patients with Sch-PAH is not clear, but it is possible to hypothesize that IPVD could attenuate right ventricular stress during exercise. Further studies are needed in order to obtain a better understanding of the natural history of IPVD associated with schistosomiasis as well as to assess...

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Survival in Schistosomiasis-Associated Pulmonary Arterial Hypertension

Abstract: Sch-PAH has a more benign clinical course than IPAH despite a lack of demonstrable acute vasoreactivity at hemodynamic evaluation.

Cited by 68 publications

References 32 publications

Perfil hemodinâmico de gravidade ao teste de vasorreatividade pulmonar em esquistossomóticos

Perfil hemodinâmico de gravidade ao teste de vasorreatividade pulmonar em esquistossomóticos

Contemporary issues in pulmonary hypertension

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

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