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https://www.readbyqxmd.com/read/29782307/cutaneous-manifestations-of-tuberous-sclerosis
#1
Mirjana Bakić, Marina Ratković, Branka Gledović, Balsa Vujović, Danilo Radunović, Vera Babić, Vladimir Prelević
Dear Editor, Tuberous sclerosis (TS) is an autosomal dominant multisystem disease, which occurs due to genetically determined hyperplasia of ectodermal and mesodermal cells. Clinical manifestations present on the skin and in the nervous system, kidneys, heart, and other organs. Recent studies estimate the incidence of TS at 1/6000 to 1/10,000 live births, and a prevalence in the general population of approximately 1 in 20,000 (1). There are two different genetic loci responsible for TS: 9q34 (TSC1-hamartin) and 16p13...
April 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#2
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29761247/beneficial-effects-of-fenofibrate-in-pulmonary-hypertension-in-rats
#3
Palak Galhotra, Pankaj Prabhakar, Himanshu Meghwani, Soheb A Mohammed, Sanjay Kumar Banerjee, Sandeep Seth, Milind P Hote, K H Reeta, Ruma Ray, Subir Kumar Maulik
Pulmonary hypertension (PH) is a morbid complication of cardiopulmonary as well as several systemic diseases in humans. It is rapidly progressive and fatal if left untreated. In the present study, we investigated the effect of PPARα agonist fenofibrate (FF) on monocrotaline (MCT)-induced PH in rats. FF, because of its pleiotropic property, could be helpful in reducing inflammation, oxidative stress, and reactive oxygen species. On day 1, MCT (50 mg/kg, s.c.) was given to all the rats in MCT, sildenafil, and FF group except normal control rats...
May 14, 2018: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/29759779/16-years-of-cardiac-resynchronization-pacing-among-congenital-heart-disease-patients-direct-contractility-dp-dt-max-screening-when-the-guidelines-do-not-apply
#4
Peter P Karpawich, Neha Bansal, Sharmeen Samuel, Yamuna Sanil, Kathleen Zelin
OBJECTIVES: The purpose of this study was to use direct cardiac resynchronization therapy (CRT)-paced contractility (dP/dt-max) response as a pre-implantation evaluation among patients with congenital heart disease (CHD) and follow clinical parameters and contractility indexes after CRT implantation. BACKGROUND: Patients with CHD often develop early heart failure with few therapeutic options, leading to heart transplantation (HT). Unfortunately, guidelines for CRT do not apply, and function evaluations by cardiac ultrasound are often inaccurate among CHD anatomies...
August 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29750156/right-ventricle-remodeling-and-function-in-scleroderma-patients
#5
REVIEW
Roxana Cucuruzac, Iolanda Muntean, Imre Benedek, Andras Mester, Nora Rat, Adriana Mitre, Monica Chitu, Theodora Benedek
Scleroderma, known also as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease. PAH has a higher prevalence in patients with SSc compared to the general population and represents a significant predictor of mortality in SSc. In patients with SSc, the morphological remodeling and alteration of RV function begin even before the setting of PAH and lead to development of a specific adaptive pattern of the RV which is different from the one recorded in patients with IAPH...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29744563/diagnostic-workup-etiologies-and-management-of-acute-right-ventricle-failure-a-state-of-the-art-paper
#6
REVIEW
Antoine Vieillard-Baron, R Naeije, F Haddad, H J Bogaard, T M Bull, N Fletcher, T Lahm, S Magder, S Orde, G Schmidt, M R Pinsky
INTRODUCTION: This is a state-of-the-art article of the diagnostic process, etiologies and management of acute right ventricular (RV) failure in critically ill patients. It is based on a large review of previously published articles in the field, as well as the expertise of the authors. RESULTS: The authors propose the ten key points and directions for future research in the field. RV failure (RVF) is frequent in the ICU, magnified by the frequent need for positive pressure ventilation...
May 9, 2018: Intensive Care Medicine
https://www.readbyqxmd.com/read/29722859/contact-force-with-magnetic-guided-catheter-ablation
#7
Francis Bessière, Christopher Zikry, Lena Rivard, Katia Dyrda, Paul Khairy
Aims: Achieving adequate catheter tip-tissue contact is essential for delivering robust radiofrequency (RF) ablation lesions. We measured the contact force generated by a remote magnetic-guided catheter navigation system. Methods and results: A plexiglass model with an integrated scale was fashioned to mimic transvenous and retrograde access to sites in the right atrium and right and left ventricles. An 8 Fr RF ablation catheter was steered by remote magnetic guidance at fields of 0...
May 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29700862/the-quantitative-assessment-of-epicardial-fat-distribution-on-human-hearts-implications-for-epicardial-electrophysiology
#8
Alexander R Mattson, Mario J Soto, Paul A Iaizzo
INTRODUCTION: Epicardial electrophysiological procedures rely on dependable interfacing with the myocardial tissue. For example, epicardial pacing systems must generate sustainable chronic pacing capture, while epicardial ablations must effectively deliver energy to the target hyper-excitable myocytes. The human heart has a significant adipose layer which may impede epicardial procedures. The objective of this study was to quantitatively assess the relative location of epicardial adipose on the human heart, in order to define locations where epicardial therapies might be performed successfully...
April 26, 2018: Clinical Anatomy
https://www.readbyqxmd.com/read/29686545/effects-of-eplerenone-on-markers-of-myocardial-fibrosis-6-minute-walk-distance-and-quality-of-life-in-adults-with-tetralogy-of-fallot-and-complete-transposition-of-the-great-arteries
#9
Sandeep S Sodhi, Tony J Zhang, Rachel McDonald, Vanessa Al Rashida, Nitin Kondapalli, Philip Barger, Philip Ludbrook, Ari M Cedars
Ventricular dysfunction is common among patients with repaired cyanotic congenital heart disease. To date, no pharmacologic intervention has been demonstrated to be beneficial in this setting. To begin addressing this knowledge gap, we conducted a single-center prospective, randomized, open-label pilot study to investigate the effects of eplerenone on serologic markers of collagen turnover and inflammation, 6-minute walk distance, and quality of life in patients with tetralogy of Fallot (TOF) or transposition of the great arteries with a systemic right ventricle (transposition of the great arteries [TGA])...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29677206/synergistic-interaction-between-a-pde5-inhibitor-sildenafil-and-a-new-adenosine-a2a-receptor-agonist-lassbio-1359-improves-pulmonary-hypertension-in-rats
#10
Allan K Alencar, Fábio I Carvalho, Ananssa M Silva, Sabrina T Martinez, Jorge A Calasans-Maia, Carlos M Fraga, Eliezer J Barreiro, Gisele Zapata-Sudo, Roberto T Sudo
INTRODUCTION: Pulmonary hypertension (PH) is characterized by enhanced pulmonary vascular resistance, which causes right ventricle (RV) pressure overload and results in right sided heart failure and death. This work investigated the effectiveness of a combined therapy with PDE5 inhibitor (PDE5i) and a new adenosine A2A receptor (A2AR) agonist in mitigating monocrotaline (MCT) induced PH in rats. METHODS: An in vitro isobolographic analysis was performed to identify possible synergistic relaxation effect between sildenafil and LASSBio 1359 in rat pulmonary arteries (PAs)...
2018: PloS One
https://www.readbyqxmd.com/read/29674379/relative-position-of-the-atrioventricular-canal-determines-the-electrical-activation-of-developing-reptile-ventricles
#11
Martina Gregorovicova, David Sedmera, Bjarke Jensen
Squamate reptiles appear to lack the specialized His/Purkinje system that enables the cardiac ventricle to be activated from apex to base as in mammals and birds. Instead, activation may simply spread from where the atrioventricular canal connects to the base. Gja5 , which encodes Cx40 that allows fast impulse propagation, was expressed throughout the ventricles of developing anole lizards. Activation was optically recorded in developing corn snake and central bearded dragon. Early embryonic ventricles were broad in shape, and activation propagated from the base to the right...
April 19, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29664959/contemporary-survival-of-patients-with-pulmonary-arterial-hypertension-and-congenital-systemic-to-pulmonary-shunts
#12
Chodchanok Vijarnsorn, Kritvikrom Durongpisitkul, Paweena Chungsomprasong, Densiri Bositthipichet, Salisa Ketsara, Yuttapon Titaram, Prakul Chanthong, Supaluck Kanjanauthai, Jarupim Soongswang
OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded...
2018: PloS One
https://www.readbyqxmd.com/read/29663123/creation-of-a-restrictive-atrial-communication-in-heart-failure-with-preserved-and-mid-range-ejection-fraction-effective-palliation-of-left-atrial-hypertension-and-pulmonary-congestion
#13
Anna Bauer, Markus Khalil, Monika Lüdemann, Jürgen Bauer, Anoosh Esmaeili, Roberta De-Rosa, Norbert F Voelkel, Hakan Akintuerk, Dietmar Schranz
BACKGROUND: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. METHODS AND RESULTS: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %...
April 16, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
https://www.readbyqxmd.com/read/29629260/acute-right-ventricular-failure-postintubation-in-a-mitral-stenosis-patient
#14
Sridhar Reddy Musuku, Saroj Pani, John Cagino
Mitral stenosis (MS) is prevalent in 0.02-0.2% of the population in developed countries. The pathophysiology of MS results in elevated left atrial pressures and over-time results in pulmonary hypertension (HTN) which ultimately affects the right ventricle. In addition, MS restricts the diastolic filling of the left ventricle. Therefore, during induction patients with MS are limited by their ability to increase cardiac output by increasing stroke volume. Anesthesia goals in severe MS are to avoid sudden changes in heart rate, as well as systemic and pulmonary artery pressures...
January 2018: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/29625509/adult-congenital-heart-disease-with-pregnancy
#15
REVIEW
Koichiro Niwa
The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk...
April 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29623530/novel-insights-and-treatment-strategies-for-right-heart-failure
#16
REVIEW
Weiqin Lin, Ai-Ling Poh, W H Wilson Tang
PURPOSE OF REVIEW: The function of the right ventricle (RV) is intimately linked to its preload (systemic volume status) and afterload (pulmonary vasculature). In this review, we explore current knowledge in RV physiology, RV function assessment, causes of right heart failure (RHF), and specific treatment strategies for RHF. RECENT FINDINGS: We examine the evidence behind new pharmacological therapies available, such as macitentan and riociguat in the treatment of specific etiologies of RHF...
April 6, 2018: Current Heart Failure Reports
https://www.readbyqxmd.com/read/29619223/respiratory-exacerbation-in-a-young-adult-with-cystic-fibrosis-and-tricuspid-atresia
#17
Jamie Wood, Abbey Sawyer, Siobhain Mulrennan, Andrew Bullock
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures...
July 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29603461/metaboreceptor-activation-in-heart-failure-with-reduced-ejection-fraction-linking-cardiac-and-peripheral-vascular-hemodynamics
#18
Zachary Barrett-O'Keefe, Joshua F Lee, Amanda Berbert, Melissa A H Witman, Jose Nativi-Nicolau, Josef Stehlik, Russell S Richardson, D Walter Wray
This study sought to evaluate the muscle metaboreflex in heart failure patients with reduced ejection fraction (HFrEF), with an emphasis on the interaction between cardiac and peripheral vascular hemodynamics across multiple levels of metaboreceptor activation. In 23 HFrEF patients (63 ± 2 yrs) and 15 healthy controls (64 ± 3 yrs), we examined changes in mean arterial pressure (MAP), cardiac output (CO), systemic vascular conductance (SVC), effective arterial elastance (Ea), stroke work (SW), and forearm deoxyhemoglobin concentration during metaboreceptor activation elicited by post-exercise circulatory occlusion (PECO) following three levels of static-intermittent handgrip exercise (15, 30, and 45% maximal voluntary contraction (MVC))...
March 30, 2018: Experimental Physiology
https://www.readbyqxmd.com/read/29600485/diffuse-fibrosis-is-common-in-the-left-but-not-in-the-right-ventricle-in-patients-with-transposition-of-the-great-arteries-late-after-atrial-switch-operation
#19
Nerejda Shehu, Christian Meierhofer, Daniel Messroghli, Naira Mkrtchyan, Stefan Martinoff, Peter Ewert, Heiko Stern
In adult patients with transposition of the great arteries (TGA) late after atrial switch operation (AtSO), each of the ventricles is faced with a profoundly different pressure regimen from the one they are meant to support in normal conditions. The extent of diffuse fibrosis of the right ventricle (RV) and left ventricle (LV) in these patients remains incompletely investigated. Aim of this study was to quantify the degree of fibrosis of the unloaded LV and of the overloaded RV by determining the myocardial extracellular volume (ECV) with non-invasive techniques as T1 mapping...
March 29, 2018: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29579196/safety-and-efficacy-of-delivering-high-power-short-duration-radiofrequency-ablation-lesions-utilizing-a-novel-temperature-sensing-technology
#20
Guy Rozen, Leon M Ptaszek, Israel Zilberman, Victoria Douglas, E Kevin Heist, Christopher Beeckler, Andres Altmann, Jeremy N Ruskin, Assaf Govari, Moussa Mansour
Aims: Delivery of high-power short-duration radiofrequency (RF) ablation lesions is not commonly used, in part because conventional thermocouple (TC) technology underestimates tissue temperature, increasing the risk of steam pop, and thrombus formation. We aimed to test whether utilization of an ablation catheter equipped with a highly accurate novel TC technology could facilitate safe and effective delivery of high-power RF lesions. Methods and results: Adult male Yorkshire swine were used for the study...
March 22, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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