Detection of Intrapulmonary Shunts in Schistosomal Cor Pulmonale

Rafla, Samir; S, Sourour

doi:10.1378/chest.104.4.1280

Cited by 4 publications

(4 citation statements)

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“…The pathogenesis, although unknown, is suspected to be similar to that of HPS without the presence of Schistosoma infection, 50 that is, the formation of intrapulmonary vascular dilatations resulting in intrapulmonary shunting and pronounced hypoxia without evidence of PH. [51][52][53] The prevalence of HPS is estimated to be approximately 6% in patients with schistosomiasisassociated liver fibrosis or as high as 11% in all patients with hepatosplenic schistosomiasis, versus 5.3%-16% in cirrhosis of various etiologies in patients awaiting liver transplantation in Brazil. 54 One of the interesting questions posed with regard to Sch-PH is which patient will develop it and why.…”

Section: Natural History Of Hepatosplenic Schistosomiasis and Sch-phmentioning

confidence: 99%

Schistosomiasis‐Associated Pulmonary Hypertension

et al. 2014

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Schistosomiasis, a parasite-borne disease, is highly prevalent in Africa and Asia; it is estimated that close to 20 million people worldwide have a severe form of the disease. The chronic form can affect the gastrointestinal system and lead to hepatosplenic disease, and it may cause cardiopulmonary complications, including pulmonary hypertension. The exact pathogenesis of schistosomiasis-associated pulmonary hypertension (Sch-PH) remains unclear, although several mechanisms, including parasitic arterial embolization, pulmonary arteriopathy, and portopulmonary hypertension-like pathophysiology, have been suggested. The immunopathology of the disease is also unclear, although there are similarities with the immunology of idiopathic pulmonary arterial hypertension (PAH). Finally, the treatment of Sch-PH has not been well studied. There is some evidence on treating the underlying infection, with unclear effect on Sch-PH, and advanced PAH therapies are now being suggested, but more studies are needed to confirm their efficacy.

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Section: Natural History Of Hepatosplenic Schistosomiasis and Sch-phmentioning

confidence: 99%

Schistosomiasis‐Associated Pulmonary Hypertension

et al. 2014

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“…Various liver diseases have been associated with this syndrome, and the presence of intrapulmonary shunt has been shown to occur in schistosomal cor pulmonale without liver failure (6). To our knowledge this is the first report of HPS associated with liver failure due to schistosomal liver disease without cor pulmonale.…”

Section: Discussionmentioning

confidence: 71%

Hepatopulmonary Syndrome Associated with Schistosomal Liver Disease

Al-Moamary¹,

Altraif²

1997

Canadian Journal of Gastroenterology

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H epatopulmonary syndrome (HPS) is the triad of chronic liver dysfunction, abnormal arterial oxygenation and pulmonary vascular shunting (1). It is estimated to occur in 8% to 25% of liver transplant candidates (2-9). There are no reports of HPS associated with schistosomal liver disease in the literature. We report the first such case of HPS complicating schistosomal liver disease. CASE PRESENTATIONA 55-year-old man known to have biopsy-proven hepatic fibrosis secondary to previously treated schistosoma mansonirelated liver disease. He presented in 1988 with hypersplenism for which he underwent splenectomy. In the summer of 1994 he developed dyspnea on minimal exertion, platypnea, dry cough and cyanosis upon standing. He was maintained on diuretics, and lactulose for increasing ascites and encephalopathy.Physical examination revealed a thin, afebrile man with stable hemodynamic status, jaundice, cyanosis with stigmata of chronic liver disease and marked digital clubbing. His chest had bilateral basal lung crackles. He had previous splenectomy and moderate ascites.Complete blood cell count was normal with hemoglobin of 130 g/L. Bilirubin was 98 mmol/L (normal 0 to 17) and albumin was 11 g/L (38 to 50). Aspartate aminotransferase was 132 IU/L (normal 15 to 37), alanine aminotransferase 67 IU/L (30 to 65) and alkaline phosphates 243 IU/L (50 to 136). Hepatitis B and C serology was negative. Antinuclear antibody level was less than 40, and ceruloplasmin, iron studies and alpha 1 antitrypsin levels were normal. Endoscopic ret- Echocardiogram documented the intrapulmonary shunt by positive microbubbles test (microbubbles were seen in the right ventricle after four to five beats). Swan-Ganz catheterization study showed normal right ventricle and pulmonary artery pressures (right atrial pressure 1 mmHg, right ventricle pressure 17/1 mmHg, pulmonary artery pressure 16/1 mmHg and pulmonary capillary wedge pressure 7 mmHg).The patient underwent orthotropic liver transplantation because he was progressively symptomatic and had liver failure with worsening hypoxemia. The initial postliver transplant course showed gradual improvement in oxygenation. On the ninth post-transplantation day he was successfully extubated and maintained on 2 L/min oxygen with a saturation of 94% to 96%.Eleven days after transplantation, bilateral lung infiltrates developed with shortness of breath and desaturation, for which he was reintubated. His sputum grew Actinobacter species and Klebsiella pneumoniae. Despite appropriate antibiotic coverage he continued to require a high fraction of inspired oxygen; for several days he was maintained on 100% oxygen. On day 42 post-liver transplant the patient died from severe hypoxemia, sepsis and multi-organ failure. DISCUSSIONFor the past four decades attention has focused on the association of oxygen desaturation with chronic liver disease. HPS was first suggested in 1977 by Kennedy and Knudson (3). It was proposed to be due to a remarkable dilation of the peripheral branches of the pulmonary vessels, re...

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“…(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.…”

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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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Detection of Intrapulmonary Shunts in Schistosomal Cor Pulmonale

Cited by 4 publications

References 3 publications

Schistosomiasis‐Associated Pulmonary Hypertension

Schistosomiasis‐Associated Pulmonary Hypertension

Hepatopulmonary Syndrome Associated with Schistosomal Liver Disease

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

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