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The hepatopulmonary syndrome (HPS) is characterized by arterial oxygenation defect induced by intrapulmonary vascular dilatations in the setting of liver disease. We report a 57-year-old woman with a history of liver cirrhosis presented with progressive cyanosis, exertional dyspnea and a dry cough. Oxyhemoglobin saturation was 88.5% on room air. Contrast transthoracic echocardiography (cTTE) and technetium-99m-macroaggregated albumin ( 99m Tc-MAA) scintigraphy showed an intrapulmonary shunting and confirmed HPS.

Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis., Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS., Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05)., Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09)., Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are pulmonary vascular complications of portal hypertension with divergent clinicopathologic features and management. The presence of intrapulmonary vascular dilatations (IPVDs), detected by agitated saline contrast-enhanced transthoracic echocardiography (cTTE), is an essential feature of HPS but is not typically characteristic of POPH. Although IPVDs have been reported rarely in POPH, the prevalence and significance of this finding have not been systematically studied. We conducted a retrospective chart review of 80 consecutive patients diagnosed with POPH from January 1, 2002 to June 30, 2014 with documentation of cTTE findings, pulmonary hemodynamics, oxygenation, and survival. A total of 34 of the 80 patients (42%) underwent cTTE during initial diagnosis of POPH. IPVDs were detected in 20/34 patients (59%); intracardiac shunting was detected in 9/34 patients (26%; 4 also had IPVDs); and 9 patients (26%) had negative cTTE with no evidence of IPVD or intracardiac shunting. Patients with IPVD had decreased survival as compared to those without IPVD (P = 0.003), a trend that persisted after exclusion of liver transplant recipients (P =  0.07). The IPVD group had a trend toward higher Model for End-Stage Liver Disease score with and without incorporating sodium (MELD or MELD-Na; P = 0.05 for both). The right ventricular index of myocardial performance (RIMP) was lower in the IPVD group (median, 0.4 versus 0.6; P = 0.006). Patients with moderate or large IPVDs (n = 6) had worse oxygenation parameters (partial pressure of arterial oxygen, diffusing capacity of the lung for carbon monoxide, and alveolar-arterial oxygen gradient) as compared to the rest of the cohort. Unexpectedly, IPVDs were frequently documented in POPH and associated with decreased survival. To further understand this observation, we recommend screening for IVPD in all patients with POPH., (© 2015 American Association for the Study of Liver Diseases.)

Pulmonary right-to-left shunting can be encountered using transthoracic contrast echocardiography (TTCE) with agitated saline. Diseases associated with pulmonary shunting on saline TTCE include hereditary hemorrhagic telangiectasia (HHT), hepatopulmonary syndrome, and some congenital heart defects after partial or complete cavopulmonary anastomosis. Furthermore, small pulmonary shunts on saline TTCE are also documented in a proportion of healthy individuals. Pulmonary shunting carries the risk for severe neurologic complications due to paradoxical embolization. In HHT, additional chest computed tomography is recommended in case of any pulmonary shunt detected on saline TTCE, to evaluate the feasibility for transcatheter embolotherapy of pulmonary arteriovenous malformations. Furthermore, antibiotic prophylaxis is advised in case of any pulmonary shunt on saline TTCE to prevent brain abscesses after procedures with risk for bacteremia. The present review provides an overview of important aspects of pulmonary shunting and its detection using saline TTCE. Furthermore, advances in understanding the clinical implications of different pulmonary shunt grades on saline TTCE are described. It appears that small pulmonary shunts on saline TTCE (grade 1) lack any clinical implication, as these shunts cannot be used as a diagnostic criterion for HHT, are not associated with an increased risk for neurologic complications, and represent pulmonary arteriovenous malformations too small for subsequent endovascular treatment. This implies that additional chest computed tomography could be safely withheld in all persons with only small pulmonary shunts on saline TTCE and sets the stage for further discussion about the need for antibiotic prophylaxis in these subjects. Besides further optimization of the current screening algorithm for the detection of pulmonary arteriovenous malformations in HHT, these observations can be of additional clinical importance in other diseases associated with pulmonary shunting and in those healthy individuals with documented small pulmonary shunts on saline TTCE., (Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background and Study Aims: Intrapulmonary vascular dilatations (IPVDs) are a criterion for the diagnosis of hepatopulmonary syndrome in patients with liver cirrhosis. We aimed to show that IPVDs are more common than suspected in a heterogenous cirrhotic population and to identify new diagnostic parameters., Patients and Methods: Forty-three consecutive patients with cirrhosis admitted to our Gastroenterology department were included in this prospective study. History, physical examination, ECG and, when warranted, pulmonary function tests and chest radiograph were used to exclude patients with significant cardiac or pulmonary disease. Contrast enhanced transthoracic echocardiography (CEE) was used to determine the presence of IPVDs. Pulse oximetry readings were taken in the supine and standing positions., Results: We found 12 patients with IPVDs. Statistical analysis proved the correlation between IPVDs and systolic pulmonary artery pressure (sPAP) (p= .049), right ventricle wall width (RVW) (p = .013) and E/A ratio (p = .034) but not left atrial or ventricular diameter. Orthodeoxia was also present more frequently in patients with positive CEE. The difference between supine and standing oxygen saturation (changeSat) proved a fair diagnostic test for detecting IPVDs, with an area under the receiver operated curve (AUROC) of 0.823., Conclusions: Our study shows that RVW, sPAP, E/A and orthodeoxia determined by pulse oximetry are valuable novel predictors of IPVDs, encouraging the routine use of pulse oximetry and echocardiography in cirrhotic patients.

Background: Patients with hepatopulmonary syndrome (HPS) with end-stage liver disease (ESLD) have higher cardiorespiratory mortality than those without. The aims of this study were to determine whether echocardiography could distinguish patients with ESLD with and without HPS and whether the diagnosis of HPS by contrast echocardiography (CE) was altered by the performance of the test in a supine or standing position., Methods: Subjects were recruited prospectively from patients with end-stage liver disease undergoing assessment for liver transplantation. Hepatopulmonary syndrome was diagnosed on the basis of arterial blood gas analysis, lung function testing and agitated saline contrast echocardiography in the absence of primary cardiac or pulmonary disease. Bubble contrast injections were performed supine or standing in a randomised order and read by a blinded observer., Results: CE showed late right-to-left shunting in 13 of 50 consecutive patients with cirrhosis (26%). Eight patients (16%) had definite diagnoses of HPS. CE in the standing position consistently increased both the number and the size of shunts compared with supine injection. CE detected intrapulmonary shunting before a change in arterial blood gases. Standard echocardiographic parameters did not distinguish between those with and without HPS., Conclusion: This study suggests that screening for HPS in patients with advanced cirrhosis should be done using CE with patients in the upright position.

Background: We screened a large number of liver transplant candidates with contrast echocardiography to detect intrapulmonary right to left shunt (IPS). We found that IPS is frequently present in patients with left atrial enlargement. This finding raises a question concerning the correlation between IPS and left atrial size in patients with liver cirrhosis., Objectives: The aim of this prospective study was to evaluate the possible correlation between left atrial size and IPS in patients with liver cirrhosis., Methods: Adult patients (>18 years old) with documented liver cirrhosis underwent trans-thoracic contrast echocardiography with agitated saline. Left atrial dimension was measured by M-mode echocardiography. Stroke volume was calculated using left ventricular M-mode echocardiography. Patients with atrial fibrillation, intracardiac shunt, congenital heart defects, valvular heart disease and diastolic dysfunction were excluded., Results: A total of 92 patients met all study criteria. Of these, 39 (42.3%) had IPS. Cardiac output was significantly greater in patients with IPS compared with those without IPS (5.68 +/- 0.83 L/min vs 4.75 +/- 0.76 L/min, P < 0.01). In a multi-variable model, after adjustment for body surface area and body mass index, left atrial enlargement was the strong predictor of IPS (area under the curve = 0.66) but when controlling for cardiac output, left atrial size was not an independent predictor of IPS., Conclusion: In the context of liver cirrhosis, patients with IPS have greater cardiac output compared with those without shunt. Left atrial enlargement, which reflects one aspect of increased cardiac output, is an indirect marker of IPS and greater left atrial dimension is associated with the presence of intrapulmonary right-to-left shunt.

The hepatopulmonary syndrome must be suspected in patients with end-stage liver disease and refractory hypoxemia. We report a case of a 49-year-old woman with hypoxemia and cirrhosis referred to the cardiac ultrasound laboratory in the evaluation of liver transplantation. Contrast-enhanced echocardiography with intravenous injection of agitated saline solution was crucial to obtain the definitive diagnosis. This imaging modality has proven to be a valuable tool in detecting intrapulmonary right-to-left shunt. The present case emphasizes the usefulness of contrast echocardiography in the diagnosis of a noncardiac disease with important prognosis implications.

Transthoracic echocardiography is a useful tool in the evaluation of patients with intrapulmonary and intracardiac shunts. We describe a case of a 49-year-old female with severe hypoxemia in the setting of aortic stenosis and cirrhosis of the liver. The use of agitated saline contrast during an echocardiography study helped to establish the diagnosis of intrapulmonary arteriovenous shunting consistent with the hepatopulmonary syndrome, thereby confirming the etiology of her symptoms and laboratory findings. This case report highlights the utility of echocardiography in diagnosing intrapulmonary shunts and assists in the understanding of the pathophysiology of hypoxemia in such patients.

We investigated the prevalence and characteristics of intrapulmonary shunt using contrast echocardiography with harmonic imaging in 130 liver transplant candidates. We found a high prevalence of intrapulmonary shunts and a significant correlation between the degree of intrapulmonary shunt and the Child-Pugh classification score.

The aim of this study was to study the prevalence of hepatopulmonary syndrome (HPS) in cirrhotic patients, comparing the results obtained using contrast transthoracic echocardiography (CTTE) and contrast transesophageal echocardiography (CTEE) in the demonstration and grading of pulmonary vasodilatation (PV). We also analyzed the correlation between gas-exchange abnormalities and PV when it was demonstrated with both techniques. The prevalence of PV and HPS with CTEE in the 88 cirrhotic patients was 28% and 22%, respectively, and with CTEE, 42% (P < 0.05) (middle PV: 35% and significant PV: 7%) and 30% (P < 0.05), respectively. Fifty-six percent of patients diagnosed with PV and with CTTE presented with hypoxemia as did 49% using CTEE (83% of patients with significant PV had hypoxaemia). PaCO2 and diffusing capacity of CO were significantly more decreased in patients with PV than in patients without PV when CTEE was employed. We conclude that CTEE is superior to CTTE in the diagnosis and grading of PV in the diagnosis of HPS in cirrhotic patients, being more sensitive and presenting a better correlation with gas-exchange abnormalities. Given its high sensitivity, CTEE should be carried out in all patients with suspicion of HPS and normal or uncertain CTTE.