Covered transjugular intrahepatic portosystemic shunt versus endoscopic therapy + ß-blocker for prevention of variceal rebleeding.

UNLABELLED: Gastroesophageal variceal bleeding in patients with cirrhosis is associated with significant morbidity and mortality, as well as a high rebleeding risk. Limited data are available on the role of transjugular intrahepatic portosystemic shunt (TIPS) with covered stents in patients receiving standard endoscopic, vasoactive, and antibiotic treatment. In this multicenter randomized trial, long-term endoscopic variceal ligation (EVL) or glue injection + ß-blocker treatment was compared with TIPS placement in 72 patients with a first or second episode of gastric and/or esophageal variceal bleeding, after hemodynamic stabilization upon endoscopic, vasoactive, and antibiotic treatment. Randomization was stratified according to Child-Pugh score. Kaplan-Meier (event-free) survival estimates were used for the endpoints rebleeding, death, treatment failure, and hepatic encephalopathy. During a median follow-up of 23 months, 10 (29%) of 35 patients in the endoscopy + ß-blocker group, as compared to 0 of 37 (0%) patients in the TIPS group, developed variceal rebleeding (P = 0.001). Mortality (TIPS 32% vs. endoscopy 26%; P = 0.418) and treatment failure (TIPS 38% vs. endoscopy 34%; P = 0.685) did not differ between groups. Early hepatic encephalopathy (within 1 year) was significantly more frequent in the TIPS group (35% vs. 14%; P = 0.035), but during long-term follow-up this difference diminished (38% vs. 23%; P = 0.121). CONCLUSIONS: In unselected patients with cirrhosis, who underwent successful endoscopic hemostasis for variceal bleeding, covered TIPS was superior to EVL + ß-blocker for reduction of variceal rebleeding, but did not improve survival. TIPS was associated with higher rates of early hepatic encephalopathy.

Combining radiological imaging and gastrointestinal tonometry: a minimal invasive and useful approach for the workup of chronic gastrointestinal ischemia.

BACKGROUND: The established approach for patients suspected of chronic gastrointestinal ischemia (CGI) includes assessment of medical history, vascular imaging, such as by digital subtraction angiography, and, more recently, computed tomography angiography (CTA) or magnetic resonance angiography. Mucosal perfusion assessment techniques have recently been shown to be of additional diagnostic value, including visible light spectroscopy and gastric exercise tonometry. Gastric exercise tonometry, however, is cumbersome and impossible to perform in a considerable proportion of patients. An alternative approach is provided by 24 h gastrointestinal tonometry (TM). We challenged the use of TM in combination with CTA as an alternative approach to evaluate patients suspected of CGI. METHODS: Patients referred for suspected CGI were prospectively evaluated using CTA and TM, and discussed in a multidisciplinary team, where a consensus diagnosis was made. CGI patients were offered therapy. Persistent symptom relief after adequate therapy during follow-up was used as the 'gold standard' and was defined as a definitive diagnosis of CGI. RESULTS: In 31 months, 186 patients were included (men 69, mean age 63 years). A consensus diagnosis of CGI was made in 128 (69%) patients: 94 with occlusive and 34 with nonocclusive CGI. After a median follow-up of 21 months after a therapeutical intervention, 91% of the CGI patients were free from symptoms. CONCLUSION: In patients clinically suspected of CGI, the combination of CTA and TM provides a minimally invasive, reliable diagnostic approach, which seems to be very useful in clinical practice and to have an outcome similar to the established diagnostic workup.

Outcome of four-dimensional stereotactic radiotherapy for centrally located lung tumors.

PURPOSE: To assess local control, overall survival, and toxicity of four-dimensional, risk-adapted stereotactic body radiotherapy (SBRT) delivered while tracking respiratory motion in patients with primary and metastatic lung cancer located in the central chest. METHODS: Fifty-eight central lesions of 56 patients (39 with primary, 17 with metastatic tumors) were treated. Fifteen tumors located near the esophagus were treated with 6 fractions of 8 Gy. Other tumors were treated according to the following dose escalation scheme: 5 fractions of 9 Gy (n = 6), then 5 fractions of 10 Gy (n = 15), and finally 5 fractions of 12 Gy (n = 22). RESULTS: Dose constraints for critical structures were generally achieved; in 21 patients the coverage of the PTV was reduced below 95% to protect adjacent organs at risk. At a median follow-up of 23 months, the actuarial 2-years local tumor control was 85% for tumors treated with a BED >100 Gy compared to 60% for tumors treated with a BED ≤ 100 Gy. No grade 4 or 5 toxicity was observed. Acute grade 1-2 esophagitis was observed in 11% of patients. CONCLUSION: SBRT of central lung lesions can be safely delivered, with promising early tumor control in patients many of whom have severe comorbid conditions.

Percutaneous treatment of peripheral vascular malformations in children: long-term clinical outcome.

PURPOSE: This study was designed to assess the rate of complications and clinical failure at 3 and 12 months after percutaneous treatment of vascular malformations in children. Furthermore, we describe patient satisfaction of treatment results during 5 years of follow-up. METHODS: In a retrospective cohort study, we evaluated 26 patients younger than aged 19 years who were treated for symptomatic vascular malformations. Data on treatment outcomes and patient satisfactions were obtained with a precoded structured questionnaire. Patient files and imaging data were retrieved to obtain information regarding the vascular malformations and treatment. Clinical success was defined as disappearance or partial improvement of the complaints. Patient satisfaction was declared whenever patients answered in the questionnaire that they were satisfied with the treatment results. RESULTS: Of 26 eligible patients, we included 23 (88%). The mean follow-up was 36 (range, 15-127) months. Posttreatment, 87% (20/23; 95% confidence interval (CI), 66-97%) of patients reported clinical success at 3 months. At 1, 2, 3, 4, and 5 years of follow-up this percentage was 74%, 59%, 59%, 59%, and 59%, respectively. Eleven (48%, 95% CI 27-69%) patients had experienced complications and 22% (95% CI 7-44%) had major complications, of which 5 had required additional treatment. In all, 83% (19/23) of the patients reported satisfaction with the treatment. CONCLUSIONS: Percutaneous treatment of vascular malformations improved clinical symptoms in 87% of the patients at 3 months and were sustainable for half of all patients during a 5-year follow-up period. However, major complications were seen in 22%.

Retrograde transvenous ethanol embolization of high-flow peripheral arteriovenous malformations.

PURPOSE: To report the clinical efficiency and complications in patients treated with retrograde transvenous ethanol embolization of high-flow peripheral arteriovenous malformations (AVMs). Retrograde transvenous ethanol embolization of high-flow AVMs is a technique that can be used to treat AVMs with a dominant outflow vein whenever conventional interventional procedures have proved insufficient. METHODS: This is a retrospective study of the clinical effectiveness and complications of retrograde embolization in five patients who had previously undergone multiple arterial embolization procedures without clinical success. RESULTS: Clinical outcomes were good in all patients but were achieved at the cost of serious, although transient, complications in three patients. CONCLUSION: Retrograde transvenous ethanol embolization is a highly effective therapy for high-flow AVMs. However, because of the high complication rate, it should be reserved as a last resort, to be used after conventional treatment options have failed.

Radial force measurements in carotid stents: influence of stent design and length of the lesion.

PURPOSE: To assess the differences in radial force of carotid stents and whether the length of the lesion influences the measurements. MATERIALS AND METHODS: Different models of tapered stents of similar size (length, 30 mm) were used. The tapered nitinol Acculink, Protégé, and Cristallo Ideale carotid artery stents and the straight, braided Elgiloy carotid Wallstent were compared. A measurement device consisting of three film loops along the stent body connected to aluminium rods with copper strain gauges was developed. Five stents of each type were deployed within 3-mm stenoses in simulated long (26 mm) and short (8 mm) stenoses. RESULTS: In the short stenosis simulation, the greatest radial force was seen in the Protégé stent, at 3.14 N ± 0.45, followed by the Cristallo Ideale stent (1.73 N ± 0.51), Acculink (1.16 N ± 0.21), and Wallstent (0.84 N ± 0.10; P < .001). In the long stenosis simulation, peak radial force again was highest in the Protégé stent (1.67 N ± 0.37), but the Acculink stent was second (0.95 N ± 0.12) and the Wallstent third (0.80 N ± 0.06). The Cristallo Ideale stent, in contrast to the short stenosis simulation, produced the least radial force (0.44 N ± 0.13) in the long stenosis simulation (P = .001). CONCLUSIONS: Radial forces exerted by carotid stents vary significantly among stent designs. Differences between stent types are dependent on the length of the stenosis. An understanding of radial force is necessary for a well-considered choice of stent type in each individual patient.

Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis.

Thoracic aortic aneurysms and dissections are a main feature of connective tissue disorders, such as Marfan syndrome and Loeys-Dietz syndrome. We delineated a new syndrome presenting with aneurysms, dissections and tortuosity throughout the arterial tree in association with mild craniofacial features and skeletal and cutaneous anomalies. In contrast with other aneurysm syndromes, most of these affected individuals presented with early-onset osteoarthritis. We mapped the genetic locus to chromosome 15q22.2-24.2 and show that the disease is caused by mutations in SMAD3. This gene encodes a member of the TGF-ß pathway that is essential for TGF-ß signal transmission. SMAD3 mutations lead to increased aortic expression of several key players in the TGF-ß pathway, including SMAD3. Molecular diagnosis will allow early and reliable identification of cases and relatives at risk for major cardiovascular complications. Our findings endorse the TGF-ß pathway as the primary pharmacological target for the development of new treatments for aortic aneurysms and osteoarthritis.

Endoscopic visible light spectroscopy: a new, minimally invasive technique to diagnose chronic GI ischemia.

BACKGROUND: The diagnosis of chronic GI ischemia (CGI) remains a clinical challenge. Currently, there is no single simple test with high sensitivity available. Visible light spectroscopy (VLS) is a new technique that noninvasively measures mucosal oxygen saturation during endoscopy. OBJECTIVE: To determine the diagnostic accuracy of VLS for the detection of ischemia in a large cohort of patients. DESIGN: Prospective study, with adherence to the Standards for Reporting of Diagnostic Accuracy. SETTING: Tertiary referral center. PATIENTS: Consecutive patients referred for evaluation of possible CGI. INTERVENTIONS: Patients underwent VLS along with the standard workup consisting of evaluation of symptoms, GI tonometry, and abdominal CT or magnetic resonance angiography. MAIN OUTCOME MEASUREMENTS: VLS measurements and the diagnosis of CGI as established with the standard workup. RESULTS: In 16 months, 121 patients were included: 80 in a training data set and 41 patients in a validation data set. CGI was diagnosed in 89 patients (74%). VLS cutoff values were determined based on the diagnosis of CGI and applied in the validation data set, and the results were compared with the criterion standard, resulting in a sensitivity and specificity of VLS of 90% and 60%, respectively. Repeated VLS measurements showed improvement in 80% of CGI patients after successful treatment. LIMITATIONS: Single-center study; only 43% of patients had repeated VLS measurements after treatment. CONCLUSIONS: VLS during upper endoscopy is a promising easy-to-perform and minimally invasive technique to detect mucosal hypoxemia in patients clinically suspected of having CGI, showing excellent correlation with the established ischemia workup.

Radiological imaging and gastrointestinal tonometry add value in diagnosis of chronic gastrointestinal ischemia.

BACKGROUND & AIMS: The diagnosis of chronic gastrointestinal ischemia (CGI) remains a clinical challenge. We aimed to assess the diagnostic value of clinical features, visualization of the gastrointestinal arteries, and evaluation of mucosal perfusion in patients clinically suspected of CGI. METHODS: A total of 186 patients referred for suspicion of CGI were prospectively included and followed up. All patients had an extensive diagnostic work-up, including visualization of the gastrointestinal arteries with computed tomography, magnetic resonance, or conventional angiography, and mucosal perfusion with tonometry. The reference standard for CGI was persistent clinical response after adequate therapy. The diagnostic value of individual and combined tests was assessed with multivariable logistic regression analysis. RESULTS: A total of 116 (62%) patients were diagnosed with CGI. In a multivariable model solely based on clinical features, the strongest predictors for CGI were the presence of postprandial pain, weight loss per month in kilograms, concomitant cardiovascular disease, and presence of an abdominal bruit. However, this model showed limited discriminative ability for the presence or absence of CGI (c-statistic, 0.62). Adding radiologic imaging to the prediction model improved the discriminative ability substantially (c-statistic, 0.81). Adding tonometry to the prediction model further improved the discriminative ability of the model (c-statistic, 0.90). The combination of clinical features and tonometry with a c-statistic of 0.88 approximated the discriminative ability of the latter model. CONCLUSIONS: Clinical features alone have a limited value to assess CGI correctly. Visualization of the gastrointestinal arteries and evaluation of mucosal perfusion substantially improve the diagnosis of CGI. The strongest diagnostic contribution comes from mucosal perfusion assessment.

Standardized definitions and clinical endpoints in carotid artery and supra-aortic trunk revascularization trials.

BACKGROUND: Endovascular therapy has emerged as a promising alternative to open surgery for stroke prevention in patients with obstructive disease of the supra-aortic arteries. Although most previous studies have used similar safety and efficacy endpoints, differences in definitions, timing of assessments, and standards of reporting have hampered direct comparisons across various trials. METHODS AND RESULTS: The DEFINE group, an informal collaboration of multidisciplinary physicians, involved in the therapy of patients with obstructive disease of the supra-aortic arteries in Europe and the United States reviewed the current literature and, after extensive correspondence and meetings, proposed the definitions outlined in the present manuscript. Three meetings including all authors of the manuscript, along with representatives of the United States Food and Drug Administration (FDA) and commercial device manufacturers were held in Barcelona, Spain, in May 2008, in Munich, Germany, in July 2008, and in New York in November 2008. The proposed definitions encompass baseline clinical and anatomic characteristics, clinical and radiologic outcomes, complications, standards of reporting, and timing of assessment. CONCLUSIONS: Considering the broad consensus between the multidisciplinary scientific members and the regulatory authorities, the proposed definitions are expected to find adoption in future clinical investigations. These definitions can be applied to both endovascular and open surgery trials and will allow reliable comparisons between these two revascularization methods.

Quality of life after stereotactic radiotherapy for stage I non-small-cell lung cancer.

PURPOSE: To determine the impact of stereotactic radiotherapy on the quality of life of patients with inoperable early-stage non-small-cell lung cancer (NSCLC). Overall survival, local tumor control, and toxicity were also evaluated in this prospective study. METHODS AND MATERIALS: From January 2006 to February 2008, quality of life, overall survival, and local tumor control were assessed in 39 patients with pathologically confirmed T1 to 2N0M0 NSCLC. These patients were treated with stereotactic radiotherapy. The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ) C30 and the QLQ LC13 lung cancer-specific questionnaire were used to investigate changes in quality of life. Assessments were done before treatment, at 3 weeks, and at 2, 4, 6, 9, and 12 months after treatment, until death or progressive disease. Toxicity was evaluated using common terminology criteria for adverse events version 3.0. RESULTS: Emotional functioning improved significantly after treatment. Other function scores and QLQ C30 and QLQ LC13 lung symptoms (such as dyspnea and coughing) showed no significant changes. The overall 2-year survival rate was 62%. After a median follow-up of 17 months, 1 patient had a local recurrence (3%). No grade 4 or 5 treatment-related toxicity occurred. Grade 3 toxicity consisted of thoracic pain, which occurred in 1 patient within 4 months of treatment, while it occurred thereafter in 2 patients. CONCLUSIONS: Quality of life was maintained, and emotional functioning improved significantly after stereotactic radiotherapy for stage I NSCLC, while survival was acceptable, local tumor control was high, and toxicity was low.

Legal aspects of cross-border teleradiology.

The growth of cross-border teleradiology has created legal challenges that are insufficiently addressed by nation health laws. New legislation is currently under development at the European level. This article will look at the details of the existing and proposed legislation and the still unsettled issues and will discuss the implications for international teleradiology.

Transjugular intrahepatic portosystemic shunt in patients with chronic portal vein occlusion and cavernous transformation.

GOALS: To determine the feasibility of transjugular intrahepatic portosystemic shunt (TIPS) creation as a possible salvage intervention in patients with variceal bleeding and chronic portal vein thrombosis with cavernous transformation, refractory to endoscopic therapy. BACKGROUND: TIPS is technically feasible in partial portal vein occlusion or complete occlusion due to fresh thrombosis. However, when the portal vein occlusion is complete and chronic, placement of TIPS is technically difficult. STUDY: In a tertiary referral center setting 4 patients with portal hypertension associated complications, received TIPS, as salvage therapy. In all patients a covered stent was placed to the cavernous transformation. RESULTS: Creation of TIPS to the dilated veins of a cavernous transformation was feasible in patients for whom recanalization of the portal vein was not possible. However, the collaterals need to be suitably wide for placement of TIPS and the high-pressure collaterals should communicate with the varices. CONCLUSIONS: TIPS should be considered as salvage therapy when endoscopic treatment is unsuccessful in patients with chronic portal vein thrombosis and cavernous transformation.

Stent placement in patients with atherosclerotic renal artery stenosis and impaired renal function: a randomized trial.

BACKGROUND: Little is known about the efficacy and safety of renal artery stenting in patients with atherosclerotic renal artery stenosis (ARAS) and impaired renal function. OBJECTIVE: To determine the efficacy and safety of stent placement in patients with ARAS and impaired renal function. DESIGN: Randomized clinical trial. Randomization was centralized and computer generated, and allocation was assigned by e-mail. Patients, providers, and persons who assessed outcomes were not blinded to treatment assignment. SETTING: 10 European medical centers. PARTICIPANTS: 140 patients with creatinine clearance less than 80 mL/min per 1.73 m(2) and ARAS of 50% or greater. INTERVENTION: Stent placement and medical treatment (64 patients) or medical treatment only (76 patients). Medical treatment consisted of antihypertensive treatment, a statin, and aspirin. MEASUREMENTS: The primary end point was a 20% or greater decrease in creatinine clearance. Secondary end points included safety and cardiovascular morbidity and mortality. RESULTS: Forty-six of 64 patients assigned to stent placement had the procedure. Ten of the 64 patients (16%) in the stent placement group and 16 patients (22%) in the medication group reached the primary end point (hazard ratio, 0.73 [95% CI, 0.33 to 1.61]). Serious complications occurred in the stent group, including 2 procedure-related deaths (3%), 1 late death secondary to an infected hematoma, and 1 patient who required dialysis secondary to cholesterol embolism. The groups did not differ for other secondary end points. LIMITATION: Many patients were falsely identified as having renal artery stenosis greater than 50% by noninvasive imaging and did not ultimately require stenting. CONCLUSION: Stent placement with medical treatment had no clear effect on progression of impaired renal function but led to a small number of significant procedure-related complications. The study findings favor a conservative approach to patients with ARAS, focused on cardiovascular risk factor management and avoiding stenting.

Long-term patient satisfaction after percutaneous treatment of peripheral vascular malformations.

PURPOSE: To determine long-term patient satisfaction for percutaneous treatment by using sclerosing agents (sclerotherapy) and/or arterial embolization for peripherally located vascular malformations (VMs). This treatment has been described as successful; however, there is a relative paucity of published long-term results. MATERIALS AND METHODS: This retrospective study was institutional review board approved; 107 patients treated for symptomatic VM were evaluated. After informed consent was obtained, 66 patients were sent a questionnaire regarding treatment effectiveness and patient satisfaction. Patient files and imaging data were retrieved to obtain information regarding the VMs and VM treatment. Kaplan-Meier survival curves were constructed to analyze clinical success rates over time. RESULTS: The most frequent reasons for patients to seek treatment were pain (89%, n = 59) and swelling (91%, n = 60). The majority of VMs were the low-flow venous type (83%, n = 55). Three months after treatment, clinical success was reported for 58% (n = 38) of patients and clinical failure was reported for 42% (n = 28). At 1-, 2-, 3-, 4-, and 5-year follow-up, clinical success was 49%, 49%, 42%, 42%, and 42%, respectively. Twenty-seven (40%) patients experienced complications, 12 of which required additional treatment. In all, 35 (53%) patients reported being satisfied with their treatment. Patient satisfaction was closely correlated with clinically successful long-term outcome of treatment. CONCLUSION: Initial partial or complete relief of VM complaints after percutaneous treatment is expected in 58% of patients, irrespective of VM size or classification. These results were durable over a 5-year follow-up period.

Intermittent claudication: clinical effectiveness of endovascular revascularization versus supervised hospital-based exercise training--randomized controlled trial.

PURPOSE: To compare clinical success, functional capacity, and quality of life during 12 months after revascularization or supervised exercise training in patients with intermittent claudication. MATERIALS AND METHODS: This study had institutional review board approval, and all patients gave written informed consent. Between September 2002 and September 2005, 151 consecutive patients who presented with symptoms of intermittent claudication were randomly assigned to undergo either endovascular revascularization (angioplasty-first approach) (n = 76) or hospital-based supervised exercise (n = 75). The outcome measures were clinical success, functional capacity, and quality of life after 6 and 12 months. Clinical success was defined as improvement in at least one category in the Rutherford scale above the pretreatment level. Significance of differences between the groups was assessed with the unpaired t test, chi(2) test, or Mann-Whitney U test. To adjust outcomes for imbalances of baseline values, multivariable regression analysis was performed. RESULTS: Immediately after the start of treatment, patients who underwent revascularization improved more than patients who performed exercise in terms of clinical success (adjusted odds ratio [OR], 39; 99% confidence interval [CI]: 11, 131; P < .001), but this advantage was lost after 6 (adjusted OR, 0.9; 99% CI: 0.3, 2.3; P = .70) and 12 (adjusted OR, 1.1; 99% CI: 0.5, 2.8; P = .73) months. After revascularization, fewer patients showed signs of ipsilateral symptoms at 6 months compared with patients in the exercise group (adjusted OR, 0.4; 99% CI: 0.2, 0.9; P < .001), but no significant differences were demonstrated at 12 months. After both treatments, functional capacity and quality of life scores increased after 6 and 12 months, but no significant differences between the groups were demonstrated. CONCLUSION: After 6 and 12 months, patients with intermittent claudication benefited equally from either endovascular revascularization or supervised exercise. Improvement was, however, more immediate after revascularization.

Assessment of biventricular functional reserve and NT-proBNP levels in patients with RV volume overload after repair of tetralogy of Fallot at young age.

PURPOSE: To assess biventricular functional reserve (FR), NT-proBNP levels and exercise performance, in relation to right ventricular volume in patients with pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) at young age. METHODS: In 53 TOF patients (maximum age at repair 2.0 years, interval since repair 15 (5) years) without residual lesions except PR, biventricular FR (derived from magnetic resonance imaging with dobutamine stress), NT-proBNP levels, maximal workload, and peak oxygen uptake were assessed. RESULTS: Mean right ventricular end-diastolic volume was 140(38) ml/m(2). Median pulmonary regurgitant fraction was 37% (range 0-57%). Biventricular systolic stress response was normal: mean (SD) ESV decreased (DeltaRVESV -17(8) ml/m(2), DeltaLVESV -11(5)), SV increased (DeltaRVSV +12(9) ml/m(2), DeltaLVSV +9(6)), FR was positive in all (RV-FR +11(5)%, LV-FR +13(6)). No serious adverse effects to dobutamine were encountered. NT-proBNP was increased in 2 patients. Median level was 10 pmol/L (range 2-42). NT-proBNP correlated with PR-percentage but not with right ventricular size. High-risk levels of NT-proBNP indicated a smaller RV-FR and a smaller decrease of biventricular ESV. Mean (SEM) VO2(max) was 96(3)%, mean Workload(max) 89(2)% of predicted. CONCLUSION: At mid to long term follow-up overall NT-proBNP levels are normal and biventricular functional reserve and exercise tolerance are well preserved in TOF repaired at young age, irrespective of RV volume. This questions the validity of isolated PR or RV volume criteria for pulmonary valve replacement in this group. Low-dose dobutamine stress testing is well tolerated and may be a useful additional tool for clinical decision making.

Diarrhoea caused by a stenosis of the coeliac artery: suggestive for mesenteric steal.

The classical triad of postprandial pain, weight loss and an abdominal bruit is thought to be the most common presentation of chronic gastrointestinal ischaemia. We describe a patient with severe diarrhoea as an uncommon presenting symptom of small bowel ischaemia, suggesting a mesenteric steal phenomenon due to a significant atherosclerotic coeliac artery stenosis. The stenosis and concomitant steal effect was successfully treated by stent placement. The latter is supported by the patient's uneventful course after stent placement. This case illustrates that chronic gastrointestinal ischaemia has to be considered in patients with otherwise unexplained diarrhoea.

Pulmonary artery size and function after Fontan operation at a young age.

PURPOSE: To assess pulmonary artery (PA) size, flow variables, and wall shear stress (WSS) in patients after Fontan operation at a young age. MATERIALS AND METHODS: Flow in the branch PA was obtained with phase contrast velocity-encoded cardiovascular magnetic resonance imaging in 14 patients before and after low-dose dobutamine stress (7.5 microg/kg/min) and in 17 healthy controls at rest. RESULTS: At rest, stroke index, total flow, average, and peak flow rate were all statistically significantly lower in patients than in controls (P<0.001). With stress-testing, all variables increased in patients (P<0.001), apart from stroke index, which did not change. At rest, branch PA area did not differ between patients and controls. Distensibility was lower in patients than in controls (P<0.001). With stress-testing, area and distensibility did not change. At rest, WSS was lower in patients than in controls (P<0.001). WSS increased with stress-testing (P<0.001), but not to the same levels as during resting conditions of the control group. CONCLUSION: PA size is normal long-term after Fontan operation at a young age. Flow variables, distensibility, and WSS are significantly lower compared to healthy controls, and do not show adequate reactions with stress-testing, which is suggestive of pulmonary artery endothelial and/or vascular dysfunction.