Severe Intrapulmonary Shunting Associated With Metastatic Carcinoid

Lee, Daniel F.; Lepler, Lawrence S.

doi:10.1378/chest.115.4.1203

Cited by 17 publications

(7 citation statements)

References 8 publications

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“…A single case of HPS has been noted in a patient with metastatic carcinoid, normal liver function, and no evidence of portal hypertension. 16 This observation raises the possibility that vasoactive substances, possibly released from the tumor, may have triggered intrapulmonary vasodilation in the absence of liver disease or portal hypertension.…”

Section: Clinical Featuresmentioning

confidence: 99%

Pulmonary dysfunction in chronic liver disease

2000

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Pulmonary abnormalities and symptoms are common in patients with chronic liver disease. If questioned, up to 70% of cirrhotic patients undergoing evaluation for liver transplantation complain of dyspnea. 1 In screening studies of patients with chronic liver disease, arterial blood gas abnormalities are found in as many as 45% and abnormal pulmonary function tests in as many as 50%. 2 A variety of causes for pulmonary dysfunction in liver disease have been identified and include intrinsic cardiopulmonary disorders not specifically related to liver disease as well as unique problems associated with the presence of liver disease and/or portal hypertension (Table 1). The recognition that a subset of patients with hepatic disease develop significant pulmonary vascular alterations, either microvascular dilation leading to the hepatopulmonary syndrome (HPS) or arteriolar vasoconstriction leading to portopulmonary hypertension, indicates that unique changes in the pulmonary vasculature may occur in liver disease. These pulmonary vascular syndromes significantly impact morbidity and mortality in affected patients and influence candidacy for liver transplantation. This review will focus on the most common abnormality in the pulmonary vasculature in liver disease: HPS. It will address the differential diagnosis, clinical features, diagnostic evaluation, therapy, and pathogenesis of this increasingly well-recognized syndrome. HEPATOPULMONARY SYNDROMEHPS is caused by intrapulmonary microvascular dilation that occurs in a subgroup of patients with liver disease and/or portal hypertension. It is defined by the presence of hepatic dysfunction or portal hypertension, a widened age-corrected alveolar-arterial oxygen gradient on room air with or without hypoxemia and intrapulmonary vasodilation. 3,4 Although the association between pulmonary dysfunction and liver disease has been recognized for more than 100 years, 5 the term "hepatopulmonary syndrome" was not used until 1977 6 as the concept that intrapulmonary vasodilation caused the gas exchange abnormalities in these patients emerged. At present, studies have shown that as many as 40% of cirrhotic patients have detectable intrapulmonary vasodilation 7 and that up to 8% to 15% will develop impaired oxygenation, which results in significant functional limitations. 3 Early definitions emphasized that the exclusion of intrinsic cardiopulmonary disease or hepatic hydrothorax were required to make the diagnosis of HPS. 4 However, it is now clear that HPS may occur in the setting of other cardiopulmonary abnormalities 8,9 and contribute significantly to gas exchange abnormalities in these patients. CLINICAL SIGNIFICANCE OF HPSThe importance of detecting HPS in patients with liver disease and/or portal hypertension derives from a number of clinical observations in patients who have both hepatic and pulmonary dysfunction. First, what is known about the natural history of HPS suggests that most patients develop progressive intrapulmonary vasodilation and worsening gas exchange over time...

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Section: Clinical Featuresmentioning

confidence: 99%

Pulmonary dysfunction in chronic liver disease

2000

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“…One patient even proceeded to subsequent lung transplantation [3] . Intrapulmonary shunting has also been reported in squamous cell carcinoma and carcinoid of the lung [8–11] .…”

Section: Discussionmentioning

confidence: 96%

Palliative embolisation for intrapulmonary shunting in lepidic predominant adenocarcinoma of the lung

Tan

Walters

Poon

et al. 2015

Respiratory Medicine Case Reports

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Lepidic predominant adenocarcinoma (LPA) (formerly known as bronchioalveolar carcinoma) has rarely been reported to cause refractory hypoxia with intrapulmonary shunting [1–7]. We describe a case who underwent the palliative strategy of intravascular right lower pulmonary artery embolisation with an 18 mm Amplatzer II vascular plug to reduce intrapulmonary shunting. This is the first report we are aware of using this minimally invasive procedure to treat this condition.

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“…Severe intrapulmonary shunting, considered to be a manifestation of a hepato-pulmonary-like syndrome, has been described in some rare cases of patients with NET. 11 Originally, HPS consists of the triad of pronounced liver dysfunction, intrapulmonary vascular dilation, and abnormal blood oxygenation. 6 The pathophysiology of the HPS lies on the impaired catabolism of regulators of the vascular tone, which in turn cause vasodilation of pulmonary veins leading to intrapulmonary shunting.…”

Section: Discussionmentioning

confidence: 99%

Development of severe intrapulmonary shunting in a patient with carcinoid heart disease after closure of a persistent foramen ovale: a case report

Schüttler

Mourouzis

Auernhammer

et al. 2021

European Heart Journal - Case Reports

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Background Neuroendocrine tumors (NETs) can affect the cardiopulmonary system causing carcinoid heart disease and valve destruction. Persistent foramen ovale (PFO) occlusion is indicated in patients with carcinoid heart disease and shunt-related left-heart valve involvement. Case Summary We report the case of a 54-year-old female patient with metastatic NET originating from the small bowel. The patient was on medication with octreotide and telotristat. One year after diagnosis, cardiac involvement of carcinoid developed with regurgitation of right-sided and, due to PFO, left-sided heart valves. Closure of PFO was performed (Occlutech 16/18 mm). One year later she presented with recurrent severe dyspnoea. The PFO-occluder was in situ without residual shunt. Valvular heart disease, including left-sided disease, and metastatic spread of NET were stable. Blood gas analysis revealed arterial hypoxemia (pO2 = 44 mmHg/5.87 kPa), which was related to extensive intrapulmonary shunting (31% shunt fraction) confirmed using contrast-enhanced echocardiography. The patient was prescribed long-term oxygen supplementation as symptomatic therapy and anti-tumoral therapy was intensified with selective internal radiotherapy of the liver metastases in order to improve biochemical control of the carcinoid syndrome. Discussion An echocardiographic assessment of the presence of a PFO is recommended in patients with NET as PFO closure minimizes the risk of left-sided carcinoid valve disease. Deterioration of symptomatic status in metastasized NET might also be due to a hepatopulmonary-like physiology with intrapulmonary shunting and arterial desaturation thought to be caused by vasoactive substances secreted by the tumor. This is a rare case describing the development of this syndrome after PFO closure.

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Severe Intrapulmonary Shunting Associated With Metastatic Carcinoid

Cited by 17 publications

References 8 publications

Pulmonary dysfunction in chronic liver disease

Pulmonary dysfunction in chronic liver disease

Palliative embolisation for intrapulmonary shunting in lepidic predominant adenocarcinoma of the lung

Development of severe intrapulmonary shunting in a patient with carcinoid heart disease after closure of a persistent foramen ovale: a case report

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