Pulmonary schistosomatic arteriovenous fistulas producing a new cyanotic syndrome in Manson's schistosomiasis

Faria, J. Lopes de; Barbas, J. V. Filho; Fujioka, Terutaka; Lion, M F; Silva, U. de Andradre e; Décourt, Luiz V.

doi:10.1016/0002-8703(59)90090-0

Cited by 29 publications

(7 citation statements)

References 12 publications

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“…The involvement of lung tissue has been reported in patients with schistosomiasis, which was also described in an experimental study involving mice chronically infected with S. mansoni (de Faria et al 1959, Fairfax et al 2012). It should be noted that although the involvement of lung tissue was not a focus of our study, this might have contributed to the widened AaPO 2 , irrespective of the presence of IPVD, resulting in an overestimation of the frequency of HPS.…”

Section: Discussionmentioning

confidence: 63%

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

Gonçalves-Macedo

Lopes

Domingues

et al. 2017

Mem. Inst. Oswaldo Cruz

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BACKGROUND Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS).OBJECTIVES To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis.METHODS We evaluated patients with HSS with or without concomitant liver cirrhosis. All patients underwent laboratory testing, ultrasound, endoscopy, contrast echocardiography, and arterial blood gas analysis.FINDINGS Of the 121 patients with HSS, 64 were also diagnosed with liver cirrhosis. HPS was diagnosed in 42 patients (35%) and was more frequent among patients with concomitant liver cirrhosis than in those without cirrhosis (42% vs. 26%), but the difference was not significant (p = 0.069). HPS was more common in those with spider naevi, Child-Pugh classes B or C and high model for end stage liver disease (MELD) scores (p < 0.05 each).MAIN CONCLUSIONS The prevalence of HPS was 35% in this study. The occurrence of liver cirrhosis concomitantly with HSS may have influenced the frequency of patients presenting with HPS.

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Section: Discussionmentioning

confidence: 63%

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

Gonçalves-Macedo

Lopes

Domingues

et al. 2017

Mem. Inst. Oswaldo Cruz

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“…(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.…”

Section: To the Editormentioning

confidence: 99%

“…(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.…”

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confidence: 99%

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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“…It can be found in 10 % of patients with the hepatosplenic form of schistosomiasis [ 12 ]. This form was previously defi ned as the cyanotic form of schistosomiasis, a more severe phase of hepatopulmonary syndrome, described by Faria et al [ 13 ]. Schistosomiasis-associated pulmonary arterial hypertension (PAH) (Sch-PAH) is defi ned as an increase in mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest and pulmonary wedge pressure (PWP) ≤15 mmHg in the setting of schistosomotic disease [ 13 ].…”

Section: Schistosomiasismentioning

confidence: 99%

Schistosomiasis-Associated Pulmonary Arterial Hypertension

Ferreira

Bandeira

Domingues

2015

Diagnosis and Management of Pulmonary Hypertension

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Schistosomiasis is the third most common endemic disease and has been considered the most common cause of pulmonary arterial hypertension (PAH) in the world. Around 6 % of people with chronic schistosomiasis in endemic areas are affected. Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) is classifi ed by the World Health Organization as group 1 pulmonary arterial hypertension because it has clinical, pathological, and hemodynamic characteristic that are similar to idiopathic pulmonary arterial hypertension. Sch-PAH is a severe disease that affects mainly middle-aged women. Symptoms and signs of right-heart insuffi ciency may be present, such as dyspnea, syncope, and peripheral edema. It has been demonstrated that Sch-PAH has a more benign clinical course and a better survival than idiopathic pulmonary hypertension. There are few reports of any benefi cial effect from specifi c therapy directed toward group 1 PAH in this population of patients. The mechanisms responsible for the development of pulmonary hypertension in patients with schistosomiasis are unknown. There is growing evidence that vessel obstruction and granulomatous reaction to pulmonary embolization of eggs is not the sole mechanism that causes the disease. Further studies are needed to better elucidate the pathogenesis of Sch-PAH and to develop targeted therapies for this devastating disease.

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Pulmonary schistosomatic arteriovenous fistulas producing a new cyanotic syndrome in Manson's schistosomiasis

Cited by 29 publications

References 12 publications

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

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

Schistosomiasis-Associated Pulmonary Arterial Hypertension

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