Anje M. Spijkerboer

Preoperative Thresholds for Pulmonary Valve Replacement in Patients With Corrected Tetralogy of Fallot Using Cardiovascular Magnetic Resonance

Background— To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. Methods and Results— Between 1993 and 2006, 71 adult patients with corrected tetralogy of Fallot underwent pulmonary valve replacement in a nationwide, prospective follow-up study. Patients were evaluated with cardiovascular magnetic resonance both preoperatively and postoperatively. Changes in RV volumes were expressed as relative change from baseline. RV volumes decreased with a mean of 28%. RV ejection fraction did not change significantly after surgery (from 42±10% to 43±10%; P=0.34). Concomitant RV outflow tract reduction resulted in a 25% larger decrease of RV volumes. After correction for surgical RV outflow tract reduction, higher preoperative RV volumes (mL/m2) were independently associated with a larger decrease of RV volumes (RV end-diastolic volume: &bgr;=0.41; P<0.001). Receiver operating characteristic analysis revealed a cutoff value of 160 mL/m2 for normalization of RV end-diastolic volume or 82 mL/m2 for RV end-systolic volume. Conclusions— Overall, we could not find a threshold above which RV volumes did not decrease after surgery. Preoperative RV volumes were independently associated with RV remodeling and also when corrected for a surgical reduction of the RV outflow tract. However, normalization could be achieved when preoperative RV end-diastolic volume was <160 mL/m2 or RV end-systolic volume was <82 mL/m2.

Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial

AIM Patients with Marfan syndrome have an increased risk of life-threatening aortic complications, mostly preceded by aortic dilatation. Treatment with losartan, an angiotensin-II receptor-1 blocker, may reduce aortic dilatation rate in Marfan patients. METHODS AND RESULTS In this multicentre, open-label, randomized controlled trial with blinded assessments, we compared losartan treatment with no additional treatment in operated and unoperated adults with Marfan syndrome. The primary endpoint was aortic dilatation rate at any predefined aortic level after 3 years of follow-up, as determined by magnetic resonance imaging. A total of 233 participants (47% female) underwent randomization to either losartan (n = 116) or no additional treatment (n = 117). Aortic root dilatation rate after 3.1 ± 0.4 years of follow-up was significantly lower in the losartan group than in controls (0.77 ± 1.36 vs. 1.35 ± 1.55 mm, P = 0.014). Aortic dilatation rate in the trajectory beyond the aortic root was not significantly reduced by losartan. In patients with prior aortic root replacement, aortic arch dilatation rate was significantly lower in the losartan group when compared with the control group (0.50 ± 1.26 vs. 1.01 ± 1.31 mm, P = 0.033). No significant differences in separate clinical endpoints or the composite endpoint (aortic dissection, elective aortic surgery, cardiovascular death) between the groups could be demonstrated. CONCLUSION In adult Marfan patients, losartan treatment reduces aortic root dilatation rate. After aortic root replacement, losartan treatment reduces dilatation rate of the aortic arch.

Prosthetic heart valve assessment with multidetector-row CT: imaging characteristics of 91 valves in 83 patients

ObjectivesMultidetector CT (MDCT) has shown potential for prosthetic heart valve (PHV) assessment. We assessed the image quality of different PHV types to determine which valves are suitable for MDCT evaluation.MethodsAll ECG-gated CTs performed in our institutions since 2003 were reviewed for the presence of PHVs. After reconstruction in 3 specific PHV planes, image quality of the supravalvular, perivalvular, subvalvular and valvular regions was scored on a four-point scale (1 = non-diagnostic, 2 = moderate, 3 = good and 4 = excellent) by two independent observers.ResultsEighty-four CT examinations (66 cardiac, 18 limited-dose aortic protocols) of 83 patients with a total of 91 PHVs in the aortic (n = 71), mitral (n = 17), pulmonary (n = 1) and tricuspid (n = 2) position were included. CT was performed on a 16-slice (n = 4), 64-slice (n = 28) or 256-slice (n = 52) MDCT system. Median image quality scores for the supra-, peri- and subvalvular regions and valvular detail were (3.5, 3.3, 3.5 and 3.5, respectively) for bileaflet PHV; (3.0, 3.0, 3.5 and 3.0, respectively) for Medtronic Hall PHV; (1.0, 1.0, 1.0 and 1.0, respectively) for Björk-Shiley and Sorin monoleaflet PHV and (3.5, 3.5, 4.0 and 2.0 respectively) for biological PHV.ConclusionCurrently implanted PHVs have good image quality on MDCT and are suitable for MDCT evaluation.

SCN5A Mutations in Brugada Syndrome Are Associated with Increased Cardiac Dimensions and Reduced Contractility

Background The cardiac sodium channel (Nav1.5) controls cardiac excitability. Accordingly, SCN5A mutations that result in loss-of-function of Nav1.5 are associated with various inherited arrhythmia syndromes that revolve around reduced cardiac excitability, most notably Brugada syndrome (BrS). Experimental studies have indicated that Nav1.5 interacts with the cytoskeleton and may also be involved in maintaining structural integrity of the heart. We aimed to determine whether clinical evidence may be obtained that Nav1.5 is involved in maintaining cardiac structural integrity. Methods Using cardiac magnetic resonance (CMR) imaging, we compared right ventricular (RV) and left ventricular (LV) dimensions and ejection fractions between 40 BrS patients with SCN5A mutations (SCN5a-mut-positive) and 98 BrS patients without SCN5A mutations (SCN5a-mut-negative). We also studied 18 age/sex-matched healthy volunteers. Results SCN5a-mut-positive patients had significantly larger end-diastolic and end-systolic RV and LV volumes, and lower LV ejection fractions, than SCN5a-mut-negative patients or volunteers. Conclusions Loss-of-function SCN5A mutations are associated with dilatation and impairment in contractile function of both ventricles that can be detected by CMR analysis.

Laparoscopy to predict the result of primary cytoreductive surgery in advanced ovarian cancer patients (LapOvCa-trial): a multicentre randomized controlled study.

BackgroundStandard treatment of advanced ovarian cancer is surgery and chemotherapy. The goal of surgery is to remove all macroscopic tumour, as the amount of residual tumour is the most important prognostic factor for survival. When removal off all tumour is considered not feasible, neoadjuvant chemotherapy (NACT) in combination with interval debulking surgery (IDS) is performed. Current methods of staging are not always accurate in predicting surgical outcome, since approximately 40% of patients will have more than 1 cm residual tumour after primary debulking surgery (PDS). In this study we aim to assess whether adding laparoscopy to the diagnostic work-up of patients suspected of advanced ovarian carcinoma may prevent unsuccessful primary debulking surgery for ovarian cancer.MethodsMulticentre randomized controlled trial, including all gynaecologic oncologic centres in the Netherlands and their affiliated hospitals. Patients are eligible when they are planned for PDS after conventional staging. Participants are randomized between direct PDS or additional diagnostic laparoscopy. Depending on the result of laparoscopy patients are treated by PDS within three weeks, followed by six courses of platinum based chemotherapy or with NACT and IDS 3-4 weeks after three courses of chemotherapy, followed by another three courses of chemotherapy. Primary outcome measure is the proportion of PDSs leaving more than one centimetre tumour residual in each arm. In total 200 patients will be randomized. Data will be analysed according to intention to treat.DiscussionPatients who have disease considered to be resectable to less than one centimetre should undergo PDS to improve prognosis. However, there is a need for better diagnostic procedures because the current number of debulking surgeries leaving more than one centimetre residual tumour is still high. Laparoscopy before starting treatment for ovarian cancer can be an additional diagnostic tool to predict the outcome of PDS. Despite the absence of strong evidence and despite the possible complications, laparoscopy is already implemented in many countries. We propose a randomized multicentre trial to provide evidence on the effectiveness of laparoscopy before primary surgery for advanced stage ovarian cancer patients.Trial registrationNetherlands Trial Register number NTR2644

Cardiac magnetic resonance imaging for noninvasive assessment of cardiovascular disease during the follow-up of patients with Kawasaki disease.

Background— Kawasaki disease (KD) is the most common cause of acquired coronary artery disease in childhood. In KD, the American Heart Association recommends echocardiography for routine coronary artery surveillance and nuclear perfusion scans and conventional coronary angiography in select patients. Cardiac MRI (CMRI) may be a noninvasive and radiation-free alternative. We applied CMRI during the follow-up of patients with KD and assessed the performance of CMRI compared with echocardiography. Methods and Results— Patients with KD aged ≥8 years were consecutively included. Sixty-three patients (median age, 14.6 years; 74.6% male sex) underwent a comprehensive CMRI protocol including adenosine stress testing to evaluate coronary artery anatomy, ischemia, and myocardial infarction. All patients underwent CMRI without significant complications. On CMRI, 23 coronary artery aneurysms (CAAs) were identified in 15 patients. CMRI detected thrombus formation in 6 CAAs in 4 patients, wall motion disturbances and ischemia in 4 patients, and delayed hyperenhancement indicating myocardial infarction in 5 patients. Wall motion and perfusion abnormalities were noted in territories supplied by affected coronary arteries. CMRI results were compared with recent echocardiography findings. In 6 of the 15 patients with CAAs on CMRI, CAAs were not detected by echocardiography. Conclusions— A comprehensive CMRI protocol including adenosine stress testing is feasible to identify coronary artery pathology, ischemia, and myocardial infarction in former patients with KD and compares favorably with echocardiography. CMRI may be used as a noninvasive and radiation-free imaging method for coronary artery surveillance during the long-term follow-up of patients with KD.

Retrospective comparison of magnetic resonance imaging features and histopathology in Crohn's disease patients

PURPOSE To retrospectively compare histopathological findings of surgically resected bowel segments with magnetic resonance imaging (MRI) findings on Crohns disease activity. MATERIALS AND METHODS Patients who underwent a MR enterography or enteroclysis before surgery were included after informed consent. MRI features (T1-enhancement, T1 and T2 stratification, T2 signal intensity, bowel wall thickness, presence of ulcerations, comb sign, creeping fat, and disease activity) were assessed by three experienced abdominal radiologists. An acute inflammatory score based on histopathology (parameters: mucosal ulceration, edema, depth and degree of neutrophils) was calculated. Interobserver variability for subjective MRI features was also assessed. RESULTS Thirty-nine segments in 25 patients (mean age 38 years) were included. Of the MRI features, disease activity per segment and bowel wall thickness had a positive association with the acute inflammatory score (p<0.05). T1-enhancement had a positive correlation with disease chronicity. All other MRI features did not have an association with the acute inflammatory score. Interobserver agreement between the three observers was weak to moderate. CONCLUSION MR features bowel wall thickness and disease activity per-segment reflect disease activity in Crohns disease patients.

The value of 3.0 Tesla diffusion-weighted MRI for pelvic nodal staging in patients with early stage cervical cancer

OBJECTIVE The purpose of this study is to investigate the diagnostic accuracy of 3.0Tesla (3T) diffusion-weighted magnetic resonance imaging (MRI) in addition to conventional MRI for the detection of lymphadenopathy in patients with early stage cervical cancer compared to histopathological evaluation of the systematically removed pelvic lymph nodes as reference standard. METHODS 68 fédération internationale de gynécologie obstétrique (FIGO) stage Ia2 to IIb cervical cancer patients were included. Sensitivity and specificity rates for two experienced observers were computed for the detection of lymphatic metastasis. Reproducibility of conventional MRI was tested by kappa statistics. The variables included in the analysis were: size of the long axis, short axis, ratio short to long axis and apparent diffusion coefficient (ADC). RESULTS Nine patients had 15 positive pelvic nodes at histopathological examination. The sensitivity and specificity of lymphatic metastasis detection by predefined conventional MRI characteristics was 33% (95% Confidence Interval (CI) 3-64) and 83% (95% CI 74-93) on patient level, and 33% (95% CI 7-60) and 97% (95% CI 95-99) on regional level respectively for observer 1. For observer 2 the sensitivity was 33% (95% CI 3-64) and the specificity 93% (95% CI 87-100) on patient level, and 25% (95% CI 1-50) and 98% (95% CI 97-100) on regional level, respectively. The kappa-value for reproducibility of metastasis detection on regional level was 0.50. The short axis diameter showed the highest diagnostic accuracy (area under the curve (AUC)=0.81 95% CI 0.70-0.91); ADC did not improve diagnostic accuracy (AUC=0.83 95% CI 0.73-0.93). CONCLUSIONS Diffusion-weighted MRI did not result in additional diagnostic value compared to conventional MRI.

Predicting surgical outcome in patients with International Federation of Gynecology and Obstetrics stage III or IV ovarian cancer using computed tomography: a systematic review of prediction models.

Objective Maximal cytoreduction to no residual disease is an important predictor of prognosis in patients with advanced-stage epithelial ovarian cancer. Preoperative prediction of outcome of surgery should guide treatment decisions, for example, primary debulking or neoadjuvant chemotherapy followed by interval debulking surgery. The objective of this study was to systematically review studies evaluating computed tomography imaging based models predicting the amount of residual tumor after cytoreductive surgery for advanced-stage epithelial ovarian cancer. Methods We systematically searched the literature for studies investigating multivariable models that predicted the amount of residual disease after cytoreductive surgery in advanced-stage epithelial ovarian cancer using computed tomography imaging. Detected studies were scored for quality and classified as model derivation or validation studies. We summarized their performance in terms of discrimination when possible. Results We identified 11 studies that described 13 models. The 4 models that were externally validated all had a poor discriminative capacity (sensitivity, 15%–79%; specificity, 32%–64%). The only internal validated model had an area under the receiver operating characteristic curve of 0.67. Peritoneal thickening, mesenterial and diaphragm disease, and ascites were most often used as predictors in the final models. We did not find studies that assessed the impact of prediction model on outcomes. Conclusions Currently, there are no external validated studies with a good predictive performance for residual disease. Studies of better quality are needed, especially studies that focus on predicting any residual disease after surgery.

Evaluation of an MRI-based score of disease activity in perianal fistulizing Crohn's disease

To evaluate a magnetic resonance imaging (MRI)-based score of disease severity for perianal fistulizing Crohns disease (CD), 16 patients with perianal fistulizing CD underwent 1.5-T MRI before and after infliximab therapy. MRI scores, Perianal Disease Activity Index (PDAI) and C-reactive protein (CRP) were calculated, and fistula drainage was assessed. These items were compared for responders and nonresponders. After therapy, PDAI values and CRP values decreased significantly in responders, but there were no significant differences in the MRI scores. In all responders, MRI findings were indicative of persisting active inflammation. In these patients, a relapse rate of 57% was seen.