Citations

Étienne‐Arthur Louis Fallot (1850–1911) is one of the most significant medical figures of the 19th century with an eponymous congenital cardiac malformation. His initial account of the four anatomical features making up his tetralogy proved remarkably significant in the progression of clinical descriptions of the lesions producing cyanotic congenital cardiac disease. Although subsequently the cause of appreciable controversy, the accuracy of his initial descriptions underscore the current diagnostic and therapeutic approach to the malformation now known uniformly as tetralogy of Fallot. Clin. Anat. 27:958–963, 2014. © 2014 Wiley Periodicals, Inc.

PurposeThe purpose of this work is to compare the behavior of the signal‐to‐noise ratio (SNR) and contrast‐to‐noise ratio (CNR) in contrast‐enhanced MR angiography with background suppression performed by either a Dixon‐type or subtraction‐type method.Theory and MethodsTheoretical expressions for the SNR and CNR for both background suppression techniques were derived. The theoretical Dixon:subtraction SNR and CNR ratios were compared to empirical ratios measured from phantom and in vivo studies for Dixon techniques utilizing one, two, and three echoes. Specifically, the SNR and CNR ratios were compared as the concentration of contrast material in the blood changed.ResultsEmpirical measurements of the SNR and CNR ratios compared favorably with the ratios predicted by theory. As the contrast concentration was reduced, the SNR advantage of the Dixon techniques increased asymptotically. In the ideal case, the SNR improvement over subtraction contrast‐enhanced MR angiography was at least twofold for one‐ and two‐echo Dixon techniques and at least a factor of for the three‐echo Dixon technique.ConclusionExpressions showing a contrast concentration‐dependent SNR and CNR improvement of at least a factor of two when Dixon‐type contrast‐enhanced MR angiography is used in place of subtraction‐type contrast‐enhanced MR angiography were derived and validated with phantom and in vivo experiments. Magn Reson Med 74:81–92, 2015. © 2014 Wiley Periodicals, Inc.

Anomalies in the course and drainage of the Inferior Vena Cava (IVC) may complicate normal functioning, correct diagnosis, and therapeutic interventions within the abdomen. Development of the IVC occurs during the 4th to 8th week of gestation, and due to its developmental complexity, there are many opportunities for malformations to occur. Although most IVC anomalies are clinically silent and are usually discovered incidentally on abdominal imaging, aberrations may be responsible for formation of thrombosis, back pain, and anomalous circulation of blood to the heart. In this review, we will discuss the most common variations and abnormalities of the IVC, which include the posterior cardinal veins, the subcardinal veins, the supracardinal veins, persistent left IVC, IVC duplication, situs inversus, left retroaortic renal vein, left circumaortic renal collar, scimitar syndrome, and IVC agenesis. For each abnormality outlined above, we aim to discuss relevant embryology and potential clinical significance with regards to presentation, diagnosis, and treatment as is important for radiologists, surgeons, and clinicians in current clinical practice. Clin. Anat. 27:1234–1243, 2014. © 2014 Wiley Periodicals, Inc.

AbstractBackgroundAutologous stem cell transplant (ASCT) is the current standard of care for most patients with multiple myeloma (MM) who are transplant eligible, yet the timing ofASCTis disputed due to a similar overall (OS) and progression‐free survival with an earlyASCT(eASCT) or a delayedASCT(dASCT) approach.ObjectiveWe developed a decision analytic model to perform cost‐effectiveness analysis of the two commonly used treatment strategies forMM.MethodsData on disease progression and treatment effectiveness came from 2001 to 2008 cohort treated at the Mayo Clinic and from published studies. Cost analysis was performed from a third‐party payer perspective.ResultsThe Consumer Price Index adjusted 2012 costs ofeASCTanddASCTwere $249 236 and $262 610, respectively.eASCTcohort had a benefit of 1.96 quality‐adjusted life years (QALYs), 0.23 QALYs more thandASCT, implying thateASCTis preferred (dominant) overdASCT. The most critical variables in one‐way sensitivity analysis were treatment‐related mortality and OS associated witheASCTstrategy.ConclusionsWe conclude thateASCTcould potentially be a relatively cost‐effective treatment option for appropriate patients with MM, and these results would help patients, providers, and payers in decision making for timing of ASCT.

BackgroundWe aimed to determine the long‐term outcomes of percutaneous lead extraction and stent placement in patients with pacemaker‐induced superior vena cava (SVC) syndrome.MethodsThe study retrospectively screened patients who underwent lead extraction followed by central vein stent implantation at Mayo Clinic (Rochester, MN, USA), from January 2005 to December 2012, to identify the patients with pacemaker‐induced SVC syndrome. Demographic, clinical, and follow‐up characteristics of those patients were collected from electronic medical records.ResultsSix cases were identified. The mean (standard deviation) age was 56 (15) years (male, 67%). All patients had permanent dual‐chamber pacemakers, with a mean 11‐year history of pacemaker placement. The entire device system was explanted in five patients; one patient had a 21‐year‐old pacemaker lead that could not be removed. Eight stents were implanted in six patients: five patients had one stent, one patient had three. A new pacemaker system was reimplanted through the stented vein in five patients. Technical success was achieved in all patients, without any complication. Symptoms rapidly resolved in all patients after stent deployment. The mean follow‐up duration was 48 months (range, 10–100 months). Three patients remained symptom free. Reintervention with percutaneous balloon venoplasty was successful in three patients with symptom recurrence.ConclusionPercutaneous stent implantation after lead removal followed by reimplantation of leads is a feasible alternative therapy for pacemaker‐induced SVC syndrome, although some cases may require repeat intervention.

ABSTRACT Histoplasma urine antigen (UAg) detection is an important biomarker for histoplasmosis. The clinical significance of low-positive (<0.6 ng/ml) UAg results was evaluated in 25 patients without evidence of prior Histoplasma infection. UAg results from 12/25 (48%) patients were considered falsely positive, suggesting that low-positive UAg values should be interpreted cautiously.

It is controversial whether acute myeloid leukemia (AML) patients with 20–29% bone marrow (BM) blasts, formerly referred to as refractory anemia with excess blasts in transformation (RAEBT), should be considered AML or myelodysplastic syndrome (MDS) for the purposes of treatment and prognostication. We retrospectively studied 571 de novo AML in patients aged >50 years, including 142 RAEBT and 429 with ≥30% blasts (AML30), as well as 151 patients with 10–19% BM blasts (RAEB2). RAEBT patients were older and had lower white blood count, but higher hemoglobin, platelet count, and karyotype risk scores compared to AML30, while these features were similar to RAEB2. FLT3 and NPM1 mutations and monocytic morphology occurred more commonly in AML30 than in RAEBT. RAEBT patients were treated less often with induction therapy than AML30, whereas allogeneic stem cell transplant frequency was similar. The median and 4‐year OS of RAEBT patients were longer than those of AML30 patients (20.5 vs 12.0 months and 28.6% vs 20.4%, respectively, P = 0.003); this difference in OS was manifested in patients in the intermediate UKMRC karyotype risk group, whereas OS of RAEBT patients and AML30 patients in the adverse karyotype risk group were not significantly different. Multivariable analysis showed that RAEBT (P < 0.0001), hemoglobin (P = 0.005), UKMRC karyotype risk group (P = 0.002), normal BM karyotype (P = 0.004), treatment with induction therapy (P < 0.0001), and stem cell transplant (P < 0.0001) were associated with longer OS. Our findings favor considering de novo RAEBT as a favorable prognostic subgroup of AML. Am. J. Hematol. 89:E193–E199, 2014. © 2014 Wiley Periodicals, Inc.

Abstract Waldenström macroglobulinemia (WM) is a distinct B-cell lymphoproliferative disorder for which clearly defined criteria for the diagnosis, initiation of therapy, and treatment strategy have been proposed as part of the consensus panels of International Workshops on WM (IWWM). As part of the IWWM-7 and based on recently published and ongoing clinical trials, the panels updated treatment recommendations. Therapeutic strategy in WM should be based on individual patient and disease characteristics (age, comorbidities, need for rapid disease control, candidacy for autologous transplantation, cytopenias, IgM-related complications, hyperviscosity, and neuropathy). Mature data show that rituximab combinations with cyclophosphamide/dexamethasone, bendamustine, or bortezomib/dexamethasone provided durable responses and are indicated for most patients. New monoclonal antibodies (ofatumumab), second-generation proteasome inhibitors (carfilzomib), mammalian target of rapamycin inhibitors, and Bruton's tyrosine kinase inhibitors are promising and may expand future treatment options. A different regimen is typically recommended for relapsed or refractory disease. In selected patients with relapsed disease after long-lasting remission, reuse of a prior effective regimen may be appropriate. Autologous stem cell transplantation may be considered in young patients with chemosensitive disease and in newly diagnosed patients with very-high-risk features. Active enrollment of patients with WM in clinical trials is encouraged.