St SW Research Articles

Letter to the editor of Acta Neurochirurgica: Intracranial venous drainage of a lumbar spinal dural arteriovenous fistula

Dear Editor, We would like to report the findings of intracranial venous drainage of a spinal dural arteriovenous fistula (sDAVF). While intracranial dural arteriovenous fistulas have long been recognized to have spinal venous drainage with characteristic myelopathy, to the best of our knowledge, intracranial venous drainage of a spinal AVF has not been previously documented [2, 3]. A 73 year old man was discovered to have a L4 (sDAVF) with retrograde intracranial drainage to the deep venous system (Fig. 1). This lesion was found incidentally during aortic angiography for repair of an abdominal aneurysm endo-leak. The patient denied previous symptoms consistent with sDAVF including myelopathic changes, bowel or bladder incontinence, or sensory changes [7]. He further denied any sign or symptom related to the intracranial venous drainage. The patient was counseled to undergo treatment based upon the pattern of drainage but ultimately elected to forgo further evaluation and treatment. Intracranial dural arteriovenous fistulas are well known to have perimedullary spinal venous drainage and cause myelopathy [3, 4]. However, to our knowledge, retrograde intracranial drainage from a sDAVF of the lumbar spine has not been described before. A patient with a lumbar sDAVF and brainstem symptoms has been reported by Wu and coworkers, but the draining vein did not extend intracranially [8]. Another report by Hashimoto et al., found perimesencephalic hemorrhage in conjunction with a sDAVF [5]. In comparison, our patient was asymptomatic despite the high level of venous drainage. This was probably related to prevalent involvement of the anterior venous drainage of the cord (Fig. 1) with relative sparing of the usually more robust dorsal draining system. He was thus spared the symptoms consistent with congestion of this system [1, 3, 6]. It is possible that the fistula developed in the setting of abnormal vasculature of the radicular arteries due both to the aneurysm and its endovascular treatment. A. M. Burrows :G. Lanzino (*) Department of Neurologic Surgery, Mayo Clinic, 200 1st St SW, Rochester, MN 55901, USA e-mail: lanzino.giuseppe@mayo.edu

Nervy Vasculature

HomeArteriosclerosis, Thrombosis, and Vascular BiologyVol. 32, No. 7Nervy Vasculature Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBNervy Vasculature Victoria M. Bedell and Stephen C. Ekker Victoria M. BedellVictoria M. Bedell From the Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN. Search for more papers by this author and Stephen C. EkkerStephen C. Ekker From the Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN. Search for more papers by this author Originally published1 Jul 2012https://doi.org/10.1161/ATVBAHA.112.251272Arteriosclerosis, Thrombosis, and Vascular Biology. 2012;32:1546–1547Blood vessel formation is a critical process during development and in response to normal wound healing. Precocious vasculature formation is associated with a variety of diseases such as cancer. The zebrafish (Danio rerio) has become a powerful model system to illuminate the molecular mechanisms underlying vasculogenesis. Some inherent advantages of the zebrafish include the following: external development that does not require vessel neogenesis for embryo formation, transparent embryos and larva, ready panel of transgenics that use fluorescent proteins for noninvasive imaging, and a full molecular genetic toolbox.See accompanying article on page 1563Recent work has shown that the evolutionary origin of many vascular factors is tied to their biochemical history in nervous system formation.1,2 One mechanism for the deployment of neural patterning genes in blood vessel formation is genome duplication followed by hijacking for use.1,2 In the study by Rissone et al, the authors show a role for more neural players in blood vessel formation, β-neurexin (β-NRXN1a), and neuroligin (NLGN1). NRXNs are cell adhesion molecules that play a role in synaptic activity, whereas NLGNs interact with NRXNs in synapse biology.3–5 The role of NLGNs in vessel formation has not been previously studied.Rissone et al focused on the vascular structure called the caudal plexus (CP). In fish embryos, this region undergoes angiogenesis with subsequent anastomosis and later remodeling.6,7 The present article carefully documents this progression through embryonic development and defines β-NRXN1a and NLGN1 expression in the CP during normal development. In β-NRXN1a knockdown animals, vascular remodeling fails, with a dramatic looping phenotype observed in the blood vessels.Using the strength of their model organism, the authors used morpholino oligonucleotides to coknockdown several known angiogenic genes to look for genetic interactions. Specifically, the authors explore the connection between NRXN signaling and the extracellular matrix (ECM). Prior work demonstrates that ECM interacting forms of vascular endothelial growth factor A are critical for vessel formation in mammals.8–12 In zebrafish, the ECM-modifying enzyme HS6ST-2 is also critical for CP formation and vascular branching.13 Rissone et al show that β-NRXN1a and NLGN1 genetically interact with vascular endothelial growth factor A, and their noted loss-of-function phenotypes are similar to the loss of HS6ST-2.13 Together, these results implicate the ECM as a critical component in β-NRXN1a and NLGN signaling during vascular formation.Rissone et al14 use the ability of the zebrafish to readily knockdown multiple genes to study interactions that would be difficult in other model organisms. Furthermore, they begin to define how the endothelial cells work together with the ECM to correctly pattern new blood vessels and remodel existing vessels.With this data, we can now think about how the CP can be used as a new model for precocious vascular formation, such as cancer vessels. Because tumor vasculature is a combination of angiogenesis and remodeling (see Nagy et al15 for review), the CP could be an excellent way to study normal vascular remodeling within a time frame that makes gene manipulation relatively simple. Does NRXN/NLGN signaling participate in the progression of cancer or other blood vessel–based disease?The evolutionary relationship between critical components of nervous system and vascular development is intriguing. However, most of the data to date have been finding neural patterning genes play a role in vascular development. Is it possible that the evolution goes both ways? For example, is the role of the ECM, as seen in vascular development, a mechanism that also plays a role in neural plasticity?DisclosuresNone.FootnotesCorrespondence to Stephen C. Ekker, Mayo Clinic, 200 1st St SW, 1342C Guggenheim, Rochester, MN 55905. E-mail [email protected]

Improvement in Myocardial Delayed Enhancement After Autologous Stem Cell Transplant in a Patient With Light Chain Amyloidosis

HomeCirculation: Heart FailureVol. 5, No. 3Improvement in Myocardial Delayed Enhancement After Autologous Stem Cell Transplant in a Patient With Light Chain Amyloidosis Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessBrief ReportPDF/EPUBImprovement in Myocardial Delayed Enhancement After Autologous Stem Cell Transplant in a Patient With Light Chain Amyloidosis Crystal R. Bonnichsen, MD, James F. Glockner, MD, PhD and Martha Grogan, MD Crystal R. BonnichsenCrystal R. Bonnichsen From the Division of Cardiovascular Diseases (C.R.B., M.G.) and Department of Radiology (J.F.G.), Mayo Clinic, Rochester, MN. Search for more papers by this author , James F. GlocknerJames F. Glockner From the Division of Cardiovascular Diseases (C.R.B., M.G.) and Department of Radiology (J.F.G.), Mayo Clinic, Rochester, MN. Search for more papers by this author and Martha GroganMartha Grogan From the Division of Cardiovascular Diseases (C.R.B., M.G.) and Department of Radiology (J.F.G.), Mayo Clinic, Rochester, MN. Search for more papers by this author Originally published1 May 2012https://doi.org/10.1161/CIRCHEARTFAILURE.112.967935Circulation: Heart Failure. 2012;5:e55A 55-year-old woman presented with progressive heart failure symptoms and was diagnosed by endomyocardial biopsy with light chain amyloidosis. Magnetic resonance imaging (MRI) at baseline showed slightly increased wall thickness of the right and left ventricles and abnormal nulling of the myocardium with a dark blood pool (Figure A) on postgadolinium images, consistent with amyloidosis. Baseline N-terminal prohormone of brain natriuretic peptide (NT-Pro BNP) level was 1429 pg/mL (normal range 10–168 pg/mL). Three years after autologous peripheral blood stem cell transplant (SCT), she continued to have complete hematologic response and had improvement of the heart failure symptoms. NT-Pro BNP level improved to 277 pg/mL. Repeat MRI showed regression of the mildly increased ventricular wall thickness and normal nulling of the myocardium (Figure B), with faint residual areas of myocardial delayed enhancement.Download figureDownload PowerPointFigure. A, Baseline midventricular short axis magnetic resonance imaging (MRI) obtained 10 minutes after administration of intravenous gadolinium shows diffusely abnormal nulling of the myocardium with a characteristic dark blood pool, consistent with cardiac amyloidosis. B, Three years after stem cell transplant, postgadolinium midventricular short axis MRI image shows normal nulling of the myocardium with faint patchy areas of residual delayed enhancement. RV indicates right ventricle; LV, left ventricle.Extent of cardiac involvement is the major determinant of outcome in light chain amyloidosis.1 Cardiac MRI with gadolinium administration can be used to detect the presence of amyloid deposition, which is seen as either global transmural or subendocardial delayed enhancement or suboptimal myocardial nulling,2 as was seen in this case. The improvement in the imaging findings after SCT in this case suggests regression of the amyloid infiltration of the myocardium and correlates with the improvement in the heart failure symptoms and the NT-Pro BNP levels.DisclosuresNone.FootnotesCorrespondence to Dr Martha Grogan, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail grogan.[email protected]edu

Combined surgical and ablative cure for localized sternal compression–induced cardiomyopathy and ventricular tachyarrhythmia

FIGURE 1. A, Preprocedure electrocardiogram showing monomorphic nonsu block morphology. B, Postprocedure electrocardiogram showing sinus rhythm From the Departments of Internal Medicine, Cardiology, Pediatric Surgery, and the Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn. Disclosures: Authors have nothing to disclose with regard to commercial support. Received for publication Feb 17, 2012; accepted for publication March 16, 2012; available ahead of print April 16, 2012. Address for reprints: Samuel J. Asirvatham, MD, Divisions of Cardiovascular Diseases and Internal Medicine and Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905 (E-mail: asirvatham.samuel@mayo.edu). J Thorac Cardiovasc Surg 2012;144:e85-7 0022-5223/$0.00 Published byElsevier Inc. on behalf of The American Association for Thoracic Surgery http://dx.doi.org/10.1016/j.jtcvs.2012.03.033

Developing Nurse Leaders

This study aims to determine whether participation in the Nursing Leadership Perspectives Program (NLPP) at Mayo Clinic in Rochester, Minnesota, produced a change in leadership skills, increased professional activities, leadership promotion, and retention rates of participants. The NLPP is an educational program designed to enhance leadership skills and promote professionalism of registered nurses. The 6-month program provides participants with theoretical knowledge, core competencies, and opportunities to practice application of leadership skills. Outcome metrics were collected from registered nurses who completed the program (n = 15). Data analysis included descriptive and nonparametric methods. Participants reported statistically significant changes in their leadership skills after participation in the program (P = .007) on the Leadership Practices Inventory. Changes in professional behavior were also statistically significant as rated by the Nursing Activity Scale (P = .001). Participants demonstrated a change in leadership skills and professional behavior following the program.

Vascular Dysfunction in Sleep Apnea

See related article, pp 417–424 Cardiovascular disease remains one of the leading causes of morbidity and mortality. Obstructive sleep apnea (OSA) has been increasingly implicated in a breadth of cardiovascular diseases, including systemic hypertension, heart failure, stroke, arrhythmias, myocardial ischemia, and pulmonary arterial hypertension.1 The importance of this association, especially in the context of OSA as an etiologic factor, is compellingly relevant to disease management strategies, given the high prevalence of OSA and its likely rising trajectory secondary to the ongoing obesity epidemic.2 OSA may be present in approximately 20% of American adults, only a minority of whom have ever been diagnosed; this is true even for patients with established cardiovascular disease3 and despite the widespread availability of several effective treatment modalities (including continuous positive airway pressure [CPAP]). It is only over the past 2 decades that serious investigative interest has been paid to the interactions between OSA and cardiac and vascular disease and dysfunction. Deciphering the mechanisms, causal relationships, and potential therapeutic targets in the links between OSA and cardiovascular morbidity has not been an easy task, in part because of the constellation of comorbidities that characterize a large proportion of sleep apnea patients. OSA leads to multiple sources of cardiovascular stress, including nocturnal hypoxemia and hypercapnia, precipitous intrathoracic pressure changes, repetitive arousals, and poor sleep quality with consequent sleep deprivation. These, in turn, have been linked to endothelial dysfunction,4 systemic inflammation, sympathetic activation,5 metabolic dysregulation, hypercoagulability, and atrial enlargement, the combination of which may contribute to the initiation and progression of a range of cardiovascular diseases. Of these, endothelial dysfunction …

Surgery 2010: Finding the Silver Lining on a Cloud of Regulation

Division of Gastroenterologic and General Surgery, Mayo Clinic, Rochester, MN Correspondence address: Michael B Farnell, MD, FACS, Department of Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905 E-mail: [email protected] Submitted January 3, 2011; accepted January 12, 2011. Disclosure Information: Nothing to disclose. Presented at Western Surgical Association 118th Scientific Session, Chicago, IL, November 2010.

AJR Teaching File: Left Neck Mass Evaluated by PET/CT

Received February 20, 2010; accepted after revision June 10, 2010. 1Department of Radiology, Mayo Clinic, 200 First St SW, Charlton 1-219, Rochester, MN 55905. Address correspondence to P. J. Peller (peller.patrick@mayo.edu). 2Department of Pathology, Mayo Clinic, Rochester, MN. 3Department of Radiology, Boston University School of Medicine and Boston Medical Center, Boston, MA. AJR 2011; 196:WS78–WS82 0361–803X/11/1966–WS78 © American Roentgen Ray Society Clinical History A 31-year-old healthy man presented with a 1-month history of a palpable left neck mass and was referred to a tertiary care center for further evaluation. On clinical examination, there was a spongy, nontender, supraclavicular mass deep in relation to his left sternocleidomastoid muscle. CT of the neck and chest performed at an outside institution showed a 6-cm round heterogeneous mass in the inferior left carotid space with no surrounding lymphadenopathy and a normal thyroid. The radiologic differential diagnosis initially favored a neurogenic tumor over a metastatic lymph node. An initial blind fine-needle aspiration of the mass performed before referral was nondiagnostic. Neck MRI was then ordered, likely intended to help narrow the differential diagnosis. The initial pathologic evaluation of an ultrasound-guided core needle biopsy of the mass was consistent with a metastatic poorly differentiated neoplasm. PET/CT was performed for identification of the primary site of tumor and failed initially to identify a primary malignancy. A complete blood count was normal. Later, further pathologic evaluation of the biopsy suggested that a germ cell neoplasm was a possibility, and testicular ultrasound was performed. There was no initial clinical detection of a primary focus of malignancy, but physical examination later confirmed a hard mass in the inferior pole of the left testicle.

AJR Teaching File: Incidental Breast Cancer Detected With 18F-FDG PET/CT

Received February 19, 2010; accepted after revision June 10, 2010. 1Department of Radiology, Mayo Clinic, 200 First St SW, Charlton 1-219, Rochester, MN 55905. Address correspondence to P. J. Peller (peller.patrick@mayo.edu). 2Department of Radiology, Boston University School of Medicine and Boston Medical Center, Boston, MA. AJR 2011; 196:WS83–WS85 0361–803X/11/1966–WS83 © American Roentgen Ray Society Clinical History A 51-year-old woman presented for staging and planned wide local reexcision of a Merkel cell carcinoma of the left upper arm. Merkel cell carcinoma is a rare, aggressive cutaneous neuroendocrine cell malignancy with some properties similar to melanoma. The diagnostic excisional biopsy showed lymphovascular invasion and positive microscopic margins. PET/ CT from the mid calvaria to the mid thighs was performed.

Outcomes Research and Epidemiology

Outcomes research was defined in 1998 as “the study of the end results of health services that takes patients' experiences, preferences, and values into account … intended to provide scientific evidence relating to decisions made by all who participate in health care.”1 Inherent to this definition is the fact that outcomes research is multidisciplinary and relies on diverse domains of expertise, with an explicit goal of direct relevance to the care delivered to patients. Within this multidisciplinary framework, epidemiology is one of the disciplines related to the field of outcomes research. Epidemiology is the study of distribution and determinants of disease frequency in human populations, with a particular focus on the occurrence of disease as categorized by time, place, and persons.2 Sometimes referred to as the basic science in medicine,3,4 epidemiology is founded on principles and methods (including methods of statistical analysis) that form the basis of clinical research.3,4 The fundamental constructs that epidemiology embraces are key to the conduct of outcomes research today. Indeed, envisioning the burden of disease through the lenses of the traditional epidemiological constructs of time and person can provide powerful guidance to shape the direction of outcomes research and assess its impact. This Editor's Perspective shares a few examples to illustrate this point. In a Shattuck Lecture published in the New England Journal of Medicine , Eugene Braunwald5 designated heart failure as a new epidemic of cardiovascular disease. This eloquent statement generated thought-provoking questions: Was there, indeed, an epidemic of heart failure in the epidemiological sense of the word? If so, is the epidemic driven by increase in incidence, improvement in survival, or both? These questions inspired epidemiologists to conduct the needed formal investigation of the heart failure epidemic, which meant bringing to bear rigorous epidemiological …

CT Dose Index and Patient Dose: They AreNotthe Same Thing

Estimates of individual patient risk, and epidemiologic studies assessing potential late effects, must use patient size–specific dose estimates—they cannot use only scanner output (volume CT dose index or dose-length product).

Teaching Points With 3-Dimensional Mapping of Cardiac Arrhythmias

Before creating a detailed 3-dimensional map of an arrhythmia, the electrophysiologist should already have a reasonable idea regarding which cardiac chamber contains either the focal site of origin of an automatic tachycardia or the relevant substrate for reentry. Typically, a combination of careful review of the ECG during tachycardia and knowledge of the location of abnormal substrate (such as myocardial infarction, atriotomy scar, etc) will allow focused mapping in the appropriate chamber. Prior teaching points have already discussed1,2 the fact that an isolated “early site of activation” is essentially meaningless for guiding ablation of macroreentry. However, in some circumstances, even for a focal “automatic” tachycardia, the apparently early site is not a suitable site to target for ablation. After constructing the activation map during tachycardia before ablation, especially if the earliest site of activation obtained does not appear to be particularly early compared with the surface ECG reference, the following should be considered. ### Overlapping Cardiac Structures Mapping a chamber other than the actual chamber where the tachycardia originates will clearly lead to a wrong conclusion. For example, when mapping premature ventricular tachycardias or monomorphic ventricular tachycardia in a structurally normal heart, a detailed map of the right ventricular outflow tract may show the “earliest site” of activation to be in the posterior wall of the right ventricular outflow tract. The true site of origin may, however, be in a coronary cusp or the subvalvar anterior left ventricular outflow tract. The posterior right ventricular outflow tract may appear to be early because a far-field signal is detected on the mapping electrode or because of breakthrough in the right ventricular outflow tract from the wave front originating on the other side of the region.3,4 After mapping the left ventricle and coronary cusps and combining the maps, the earliest activation …

A New Signal From B-Type Natriuretic Peptide in ST-Elevation Myocardial Infarction

A New Signal From B-Type Natriuretic Peptide in ST-Elevation Myocardial Infarction

Congenital Intracranial Teratoma

Received January 6, 2010, from the Department of Obstetrics and Gynecology, Mayo Clinic, Rochester, Minnesota USA. Revision requested February 8, 2010. Revised manuscript accepted for publication February 25, 2010. Address correspondence to Zaraq Khan, MBBS, Department of Obstetrics and Gynecology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 USA. E-mail: khan.zaraq@mayo.edu Abbreviations MRI, magnetic resonance imaging 32-year-old patient, gravida 2, para 0010, was noted to have a discrepancy in uterine size with dates. At 24 weeks’ gestation, her fundal height measured 28 cm. She had previously had a normal pregnancy course and a normal anatomy scan at 19 weeks (Figures 1 and 2). On imaging at 24 weeks’ gestation, the fetal brain had an irregular shape, and the midline was deviated to the left side (Figures 3–7). A biparietal diameter of 9.9 cm and head circumference of 35.9 cm were both greater than the 99th percentile. The differential diagnosis was a fetal cranial neoplasm versus an intracranial bleed. Results of platelet antibody tests, obtained to rule out neonatal alloimmune thrombocytopenia, were negative. Options for pregnancy termination including cephalocentesis were discussed, and the patient chose to proceed with the pregnancy expectantly. At 27 weeks, fetal magnetic resonance imaging (MRI) showed a cranium of 14 × 11 cm filled with amorphous soft tissue containing multiple cysts. No identifiable brain tissue could be seen (Figure 8), consistent with a teratoma. The patient came to labor and delivery with spontaneous onset of preterm labor at 29 weeks. She was admitted at 7 cm dilation. A cephalic presentation was noted. Intrauterine fetal death was noted by abdominal sonography. Cephalocentesis was again offered to allow vaginal delivery, but the patient refused. She spontaneously dilated to 9 cm, and then artificial rupture of membranes was performed. Soon after, vaginal bleeding was noted, and fetal brain tissue began to pass from the vagina, consistent with cranial rupture. Soon thereafter, she had a vaginal delivery of the fetal body. On postmortem examination congenital intracranial teratoma was identified; no other congenital abnormalities were noted.

Pomalidomide: A new IMiD with remarkable activity in both multiple myeloma and myelofibrosis

Pomalidomide: A new IMiD with remarkable activity in both multiple myeloma and myelofibrosis

Patient Education to Reduce Prehospital Delay Time in Acute Coronary Syndrome

Reducing time from symptom onset to reperfusion therapy decreases infarct size and improves survival in patients with ST-elevation myocardial infarction (STEMI). National quality improvement initiatives, including the Door-to-Balloon (D2B) Quality Alliance, have successfully disseminated evidence-based strategies to reduce door-to-balloon time.1,2 Many hospital systems have achieved the targeted door-to-balloon time <90 minutes for 75% of patients with STEMI presenting directly to a hospital with percutaneous coronary intervention capability.3 However, prehospital delay from time of symptom onset to hospital arrival remains the largest portion of the total delay time and has been refractory to interventions aimed at improving patient responsiveness. Article see p 524 For patients with STEMI, longer prehospital delay is associated with higher in-hospital mortality even after adjusting for patient-, hospital-, and system-level variables including door-to-balloon or door-to-needle time.4,5 In the past 15 years, 2 randomized controlled trials have tested whether educational interventions to improve patient knowledge about acute coronary syndrome (ACS) symptoms and to promote patient action to call 9-1-1 would reduce prehospital delay time. Luepker and colleagues randomized 20 US cities to serve as controls or to receive 18 months of education targeting mass media, community and professional organizations, and patients and clinicians in the Rapid Early Action for Coronary Treatment (REACT) trial.6 The median prehospital delay time was 2.3 hours at baseline and decreased slightly (4.7% per year) during the 18 months in the intervention group; however, this trend was not significantly different to that observed in the control group ( P =0.54). The use of emergency medical services (EMS) increased modestly in the …

Sharing Decision Making About Cardiac Surgery

Aortic valve replacement surgery is the only available treatment option able to improve survival in patients with symptomatic and severe aortic valve stenosis. Without surgical intervention, patients with symptomatic dyspnea and severe aortic valve stenosis have a median survival less than 2 years. In an ideal, patient-centered healthcare system, the decision whether to undergo risky yet potentially life-prolonging surgery should not only reflect judicious application of the evidence of safety and efficacy of the surgery, but it also should be consistent with the patient’s clinical and personal contexts, and their goals, values, and preferences for life and healthcare. Article see p 533 The report by Bach et al1 is the latest in their series of investigations to uncover why cardiac valve replacement surgery is underused among eligible candidates. In this report, they seek explanations among patient, clinician, and hospital-level characteristics and suggest the following hypothesis: clinicians, particularly cardiologists, are making apparently inconsistent judgments as to which patients should be referred to cardiac surgeons for consideration of aortic valve replacement. Among 191 patients with severe aortic valve stenosis who did not undergo cardiac surgery, a cardiologist evaluated 182 (95%), but referred only 57 (30%) to a cardiac surgeon. In this editorial, we will place this finding in the context of the evolving field of shared decision-making and suggest how this focus could improve the quality of care delivered to patients with valvular heart disease. Shared decision making is a 3-step process.2 The first step involves knowledge transfer. In its idealized form, shared decision making requires symmetrical and bidirectional exchange of information between patients and clinicians. This exchange aims to identify and distinguish the available options under consideration, including the option of no treatment. The other 2 steps require the patient and clinician to (1) actively engage in deliberating (ie, …

Atrial Fibrillation: Focal or Reentrant or Both?

Ablation for atrial fibrillation (AF) is a genuinely viable and increasingly used option for improving quality of life in patients with symptomatic drug-refractory AF. What is remarkable about the limited success achieved with these procedures is that progress has occurred despite a near complete lack of knowledge about what causes or maintains AF. Focal origins for AF were first demonstrated decades ago1–4 with topical application of acetylcholine to junctional tissues and atrial sites triggering AF. More recently, there has been convincing documentation of rapid tachycardia arising from the pulmonary veins inciting AF, thus supporting the concept of “trigger elimination” contributing to the success of pulmonary vein isolation. However, studies also support the presence of multiple reentrant circuits maintaining and possibly initiating AF.5, 6 The ablation era has taught us7–9 that in persistent and chronic AF, trigger elimination alone is not enough and that substrate modification is probably necessary to decrease the likelihood of reentrant wavelet continuance and thereby increase success rates. Even the most ardent supporter of either hypothesis recognizes the limitations of each. How can a focal source exist in this milieu indefinitely, producing and maintaining chronic AF, and how do multiple reentrant wavelets arise spontaneously? Article see p 384 In this issue of Circulation: Arrhythmia and Electrophysiology , Niu et al10 provide important, interesting, and novel insights into solving this age-old riddle using a previously unexplored angle-autonomic manipulation. Acetylcholine was used to induce AF in 31 canines. In 12 of the animal studies, propafenone was administered. They then ablated the ganglionated plexi (GP). Before ablation, AF could still be induced despite administration of propafenone. After ablation, there was a consistent pattern of electrogram organization and atrial synchronization. In addition, subsequent administration of propafenone resulted in the inability to induce atrial tachyarrhythmia. There are several …

Uncommon Variant of Ebstein Anomaly With Tricuspid Stenosis

A 27-year-old previously healthy woman with no prior history of exercise limitations, shortness of breath, cyanosis, or arrhythmia became short of breath 10 months after her second successful pregnancy. Her shortness of breath was at rest accompanied by progressive edema, ascites, and later anasarca. An ECG revealed normal sinus rhythm with right atrial enlargement (Figure 1A), and chest x-ray revealed normal lung parenchyma with mildly enlarged cardiac silhouette (Figure 1B). A transthoracic echocardiogram demonstrated reduced left ventricular size with an ejection fraction estimated at 50%, severe right atrial enlargement, and severe right ventricular enlargement with dysfunction and was suspicious for severe displacement of the tricuspid valve leaflets toward the right ventricular apex, suggestive of Ebstein anomaly (EA) (Figure 2). She responded to intravenous diuresis with improvement in her right-sided heart failure. In light of her dilated, poorly functioning right ventricle, she was started on Coumadin. Repeat transthoracic echocardiogram 2 months later demonstrated displacement of the tricuspid valve into …

Soluble Guanylate Cyclase

Arthur Kornberg, who shared with Severo Ochoa the 1959 Nobel Prize for Physiology or Medicine, stated in his autobiography that he had never met a dull enzyme.1 We strongly believe that soluble guanylate cyclase (sGC; E.C.4.6.1.2) is no exception to this. Article see p 2781 Nitric oxide (NO) plays an important physiological role as a signaling molecule as well as a cytotoxic agent. It is produced by 3 distinct NO synthases, specifically endothelial, neuronal, and inducible NO synthase. In the vasculature, endothelial NO synthase plays an important role in vascular homeostasis because NO generated in endothelial cells promotes vascular smooth muscle cell relaxation and inhibits platelet aggregation. Importantly, bioavailability of NO can be reduced in cardiovascular diseases, a condition which has been termed “endothelial dysfunction.” This phenomenon provides a rationale for therapeutic intervention. The primary target of NO is sGC, which is a heterodimeric enzyme consisting of an α- and β-subunit and a prosthetic heme group, which is located in the β-subunit. The heme group contains a ferrous iron atom (Fe2+). Binding of NO to this iron changes the conformation of the enzyme and leads to a >100-fold increase in catalytic rate (ie, the conversion of guanosine triphosphate to the second messenger cyclic guanosine monophosphate [cGMP]). The NO-induced cGMP signal modulates intracellularly the activity of several effector molecules: cGMP-dependent protein kinases, cGMP-regulated phosphodiesterases, and cGMP-gated ion channels. Of note, both heme iron oxidation (Fe3+) and heme removal render sGC unresponsive to NO (Figure). These 2 conditions could therefore provide a mechanism for “vascular smooth muscle cell dysfunction” in cardiovascular disease states, which are often characterized by marked vasoconstriction either systemically or in specific organs. Figure. Schematic illustrating 3 different forms of soluble guanylate cyclase and their respective responsiveness to nitrovasodilators, sGC stimulators (eg, BAY 41–2272), and sGC …