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https://www.readbyqxmd.com/read/28329610/generalized-woolly-hair-with-diventricular-arrythmogenic-cardiomyopathy-a-rare-variant-of-naxos-disease
#1
Abhijit Dutta, Sudip Kumar Ghosh, Biswajit Majumder, Rahul Majumdar
Woolly hair may occur as an isolated problem of cosmetic concern or can be a part of a systemic disease (woolly hair syndrome) with underlying fatal cardiomyopathy. Two characteristic associations of woolly hair syndrome are Naxos disease and Carvajal syndrome. Naxos disease is characterized by woolly hair, palmoplantar keratoderma, and arrythmogenic right ventricular cardiomyopathy.In this report we describe a case of a young girl who presented with heart failure and was subsequently diagnosed as a case of generalized woolly hair with biventricular arrythmogenic cardiomyopathy...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28329464/left-ventricular-retraining-and-double-switch-in-patients-with-congenitally-corrected-transposition-of-the-great-arteries
#2
Ali N Ibrahimiye, Richard D Mainwaring, William L Patrick, Laura Downey, Vamsi Yarlagadda, Frank L Hanley
PURPOSE: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28329333/pre-and-postoperative-tricuspid-regurgitation-in-patients-with-severe-symptomatic-aortic-stenosis-importance-of-pre-operative-tricuspid-annulus-diameter
#3
Claire Dumont, Elena Galli, Emmanuel Oger, Maxime Fournet, Erwan Flecher, Christophe Leclercq, Jean-Philippe Verhoye, Erwan Donal
Aims: Secondary tricuspid regurgitation (STR) is commonly found in patients with aortic stenosis and is associated with increased morbidity. The study sought to evaluate the prevalence of pre-operative STR and its progression after surgical aortic valve replacement (SAVR) or transcatheter aortic valve implantation (TAVI). Also, it sought to analyse the predictors of post-operative changes in STR. Methods and results: We prospectively evaluated 116 patients (aged 75...
March 7, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28329315/seraphin-haemodynamic-sub-study-the-effect-of-the-dual-endothelin-receptor-antagonist-macitentan-on-haemodynamic-parameters-and-nt-probnp-levels-and-their-association-with-disease-progression-in-patients-with-pulmonary-arterial-hypertension
#4
Nazzareno Galiè, Pavel Jansa, Tomás Pulido, Richard N Channick, Marion Delcroix, Hossein-Ardeschir Ghofrani, Franck-Olivier Le Brun, Sanjay Mehta, Loïc Perchenet, Lewis J Rubin, B K S Sastry, Gérald Simonneau, Olivier Sitbon, Rogério Souza, Adam Torbicki
Aims: The effect of macitentan on haemodynamic parameters and NT-proBNP levels was evaluated in pulmonary arterial hypertension (PAH) patients in the SERAPHIN study. Association between these parameters and disease progression, assessed by the primary endpoint (time to first morbidity/mortality event), was explored. Methods and results: Of the 742 randomized patients, 187 with right heart catheterization at baseline and month 6 participated in a haemodynamic sub-study...
February 28, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28329284/contemporary-outcome-of-unplanned-right-ventricular-assist-device-for-severe-right-heart-failure-after-continuous-flow-left-ventricular-assist-device-insertion
#5
Daisuke Yoshioka, Hiroo Takayama, Reshad A Garan, Veli K Topkara, Jiho Han, Paul Kurlansky, Melana Yuzefpolskaya, Paolo C Colombo, Yoshifumi Naka, Koji Takeda
OBJECTIVES: The timely use of a right ventricular assist device (RVAD) becomes necessary for severe right heart failure (RHF) after left ventricular assist device (LVAD) insertion. This study evaluates outcomes in patients who required unplanned RVAD support early after continuous-flow (CF) LVAD insertion. METHODS: We retrospectively reviewed 305 patients who underwent HeartMate II/HeartWare CF-LVAD insertion between 2009 and 2014. Twenty-seven (9%) patients required unplanned RVAD for severe RHF early after LVAD insertion...
March 1, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329231/cardiac-protective-effects-of-remote-ischaemic-preconditioning-in-children-undergoing-tetralogy-of-fallot-repair-surgery-a-randomized-controlled-trial
#6
Qingping Wu, Tingting Wang, Shiqiang Chen, Quanjun Zhou, Haobo Li, Na Hu, Yinglu Feng, Nianguo Dong, Shanglong Yao, Zhengyuan Xia
Aims: Remote ischaemic preconditioning (RIPC) by inducing brief ischaemia in distant tissues protects the heart against myocardial ischaemia-reperfusion injury (IRI) in children undergoing open-heart surgery, although its effectiveness in adults with comorbidities is controversial. The effectiveness and mechanism of RIPC with respect to myocardial IRI in children with tetralogy of Fallot (ToF), a severe cyanotic congenital cardiac disease, undergoing open heart surgery are unclear. We hypothesized that RIPC can confer cardioprotection in children undergoing ToF repair surgery...
February 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28329229/isolated-severe-leftward-displacement-of-the-septum-primum-anatomic-and-3d-echocardiographic-findings-and-surgical-repair
#7
Fabio Cuttone, Khaled Hadeed, François Lacour-Gayet, Hugues Lucron, Sebastien Hascoet, Philippe Acar, Bertrand Leobon, Richard Van Praagh
OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage...
February 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329216/heart-mechanics-at-high-altitude-6%C3%A2-days-on-the-top-of-europe
#8
Claire Maufrais, Thomas Rupp, Pierre Bouzat, Gregory Doucende, Samuel Verges, Stéphane Nottin, Guillaume Walther
Aims: The aim of this study was to analyse the underlying mechanisms of left and right ventricular (LV and RV) functional alterations during several days in high-altitude hypoxia. Methods and results: Resting evaluations of LV and RV function and mechanics were assessed by Speckle Tracking Echocardiography on 11 subjects at sea level (SLPRE), 3 ± 2 h after helicopter transport to high altitude (D0), at day 2 (D2), day 4 (D4) and day 6 (D6) at 4350 m and 5 ± 2 h after return to sea level (SLPOST)...
December 22, 2016: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28329164/large-animal-model-of-functional-tricuspid-regurgitation-in-pacing-induced-end-stage-heart-failure
#9
Marcin Malinowski, Alistair G Proudfoot, David Langholz, Lenora Eberhart, Michael Brown, Hans Schubert, Jeremy Wodarek, Tomasz A Timek
OBJECTIVES: Functional tricuspid regurgitation (FTR) is common in patients with advanced heart failure and frequently complicates left ventricular assist device implantation yet remains poorly understood. We set out to establish large animal model of FTR that could serve as a research platform to investigate the pathogenesis of FTR associated with end-stage heart failure. METHODS: : Through right thoracotomy, ten adult sheep underwent implantation of pacemaker with epicardial LV lead, five sonomicrometry crystals on the right ventricle, and left and right ventricular telemetry pressure sensors during a beating heart off-pump procedure...
February 21, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329138/small-intestinal-submucosa-tricuspid-valve-tube-graft-shows-growth-potential-remodelling-and-physiological-valve-function-in-a-porcine-model%C3%A2
#10
Diana M Ropcke, Christine Ilkjaer, Marcell J Tjornild, Soren N Skov, Steffen Ringgaard, Vibeke E Hjortdal, Sten L Nielsen
OBJECTIVES: Tricuspid valve reconstruction using small intestinal submucosal extracellular matrix (ECM) designed as a tube graft is possible in severe valve pathology. Using cardiac magnetic resonance imaging (MRI) and digital photograph imaging, valves were evaluated after 6 months in a porcine model. METHODS: Ten 60 kg pigs received a tricuspid valve tube graft. A baseline MRI was performed after 2 weeks ( n  = 8). A follow-up MRI was performed in three of five 6 months' survivors to assess function and haemodynamics of the tricuspid tube graft...
February 27, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329112/comparison-of-paracorporeal-and-continuous-flow-ventricular-assist-devices-in-children-preliminary-results%C3%A2
#11
Mohamed S Nassar, Asif Hasan, Teresa Chila, Stephan Schueler, Carola Pergolizzi, Zdenka Reinhardt, Stephen Lord, Fabrizio De Rita, Lee Ferguson, Jon Smith, Simon Haynes, John O'Sullivan, Bari Murtuza
OBJECTIVES: With the scarcity of organs, a durable, reliable ventricular assist device (VAD) is required. The Berlin Heart EXCOR ® (BH) remains the most established VAD in the paediatric population. Implantable continuous flow (CF) VADs have been introduced to the paediatric field with encouraging early results. In this study, we compared the results of a newly introduced CF VAD (HeartWare VAD [HVAD] ® ) to results in a matched group of BH recipients. METHODS: The study included patients aged <16 years who received mechanical left VAD (LVAD) support between December 2005 and January 2016...
February 16, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28329111/hypoplastic-left-heart-syndrome-a-novel-surgical-strategy-for-small-volume-centres-%C3%A2
#12
Margaux Pontailler, Régis Gaudin, Marien Lenoir, Ayman Haydar, Diala Kraiche, Damien Bonnet, Pascal Vouhé, Olivier Raisky
OBJECTIVES: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach. METHODS: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia...
February 17, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28329100/long-term-cardiovascular-changes-following-creation-of-arteriovenous-fistula-in-patients-with-end-stage-renal-disease
#13
Yogesh N V Reddy, Masaru Obokata, Patrick G Dean, Vojtech Melenovsky, Karl A Nath, Barry A Borlaug
Aims: Short-term studies have reported left ventricular (LV) dilatation following surgical creation of arteriovenous fistulas (AVF) or arteriovenous grafts (AVGs), but chronic cardiac structural and functional changes have not been examined or related to clinical outcomes following AVF/AVG. We sought to characterize the long-term changes in cardiac structure and function in patients undergoing shunt creation for haemodialysis. Methods and results: A retrospective analysis was performed of patients undergoing echocardiography before and after surgical AVF/AVG creation for the initiation of haemodialysis...
March 6, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28329042/longitudinal-cardiovascular-outcomes-of-sleep-disordered-breathing-in-children-a-meta-analysis-and-systematic-review
#14
Zarmina Ehsan, Stacey L Ishman, Thomas R Kimball, Nanhua Zhang, Yuanshu Zou, Raouf S Amin
Objectives: The presence of sleep disordered breathing (SDB) is known to impact long-term cardiovascular morbidity in adults; however, the long-term effects in children are poorly understood. We aimed to systematically review and synthesize studies published to date on the long-term effects of SDB in children. Study Design: Meta-analysis and systematic review using PubMed, CINAHL,Embase and Scopus (all indexed years). Methods: We searched for English-language articles containing original human data from prospective studies, with ≥7 participants, in children ≤18 years of age...
January 28, 2017: Sleep
https://www.readbyqxmd.com/read/28328950/repeated-anaesthesia-with-isoflurane-and-medetomidine-midazolam-fentanyl-in-guinea-pigs-and-its-influence-on-physiological-parameters
#15
Sabrina Schmitz, Sabine Tacke, Brian Guth, Julia Henke
Repeated anaesthesia may be required in experimental protocols and in daily veterinary practice, but anaesthesia is known to alter physiological parameters in GPs (Cavia porcellus, GPs). This study investigated the effects of repeated anaesthesia with either medetomidine-midazolam-fentanyl (MMF) or isoflurane (Iso) on physiological parameters in the GP. Twelve GPs were repeatedly administered with MMF or Iso in two anaesthesia sets. One set consisted of six 40-min anaesthesias, performed over 3 weeks (2 per week); the anaesthetic used first was randomized...
2017: PloS One
https://www.readbyqxmd.com/read/28328585/mechanical-support-with-impella-during-malignant-arrhythmia-ablation-a-case-report-on-the-growing-trend-in-the-electrophysiology-laboratory
#16
Adam C Adler, Ramesh Kodavatiganti
Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease in which the persistence of the right ventricle as the systemic ventricle leads to heart failure, tricuspid valve insufficiency, and arrhythmia. Supraventricular arrhythmias are especially common in these patients. We discuss the anesthetic management of a 33-year-old patient with congenitally corrected transposition of the great arteries who required a ventricular assist device to maintain cardiac output during ablation of supraventricular tachyarrythmia...
March 17, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28328277/finding-the-right-frameworks
#17
Sam Foster
Sam Foster, Chief Nurse at Heart of England NHS Foundation Trust, discusses the issues around job descriptions, curricula and opportunities for progression needed to ensure care delivery is fit for existing and future patients.
March 9, 2017: British Journal of Nursing: BJN
https://www.readbyqxmd.com/read/28328117/noonan-syndrome-ptpn11-mutations-and-brain-tumors-a-clinical-report-and-review-of-the-literature
#18
Aurore Siegfried, Claude Cances, Marie Denuelle, Najat Loukh, Maïté Tauber, Hélène Cavé, Marie-Bernadette Delisle
Noonan syndrome (NS), an autosomal dominant disorder, is characterized by short stature, congenital heart defects, developmental delay, and facial dysmorphism. PTPN11 mutations are the most common cause of NS. PTPN11 encodes a non-receptor protein tyrosine phosphatase, SHP2. Hematopoietic malignancies and solid tumors are associated with NS. Among solid tumors, brain tumors have been described in children and young adults but remain rather rare. We report a 16-year-old boy with PTPN11-related NS who, at the age of 12, was incidentally found to have a left temporal lobe brain tumor and a cystic lesion in the right thalamus...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28326855/impact-of-postoperative-pulmonary-hypertension-on-outcome-after-heart-transplantation
#19
Jakob Lundgren, Carl Söderlund, Göran Rådegran
OBJECTIVES: We wanted to investigate the effects of postoperative pulmonary hypertension (PHpostop: mean pulmonary artery pressure [MPAP] ≥ 25 mmHg), diastolic pressure gradient (DPG), pulmonary vascular resistance (PVR), and repeated hemodynamic measurements on long-term survival after heart transplantation (HT). DESIGN: Eighty-nine patients who underwent HT at Skåne University Hospital in Lund in the period 1988-2010 and who were evaluated with right-heart-catheterization at rest, prior to HT and repeatedly during the first postoperative year, were grouped based on their MPAP, DPG, and PVR...
March 22, 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/28326680/the-effect-of-desmopressin-on-the-amount-of-bleeding-and-transfusion-requirements-in-patients-undergoing-heart-transplant-surgery
#20
Alireza Jahangirifard, Mohammad Reza Razavi, Zargham Hosein Ahmadi, Mohammad Forozeshfard
One of the most common risks after a heart transplant is bleeding. In this study, the effect of desmopressin administration on the amount of bleeding and transfusion requirements following heart transplant surgery was investigated. In a double-blind clinical trial, 48 patients who were candidates for heart transplant surgery were randomly assigned to two groups. In the intervention group, patients received desmopressin 0.3 μg/kg, 30 min. before surgery. Patients in the control group received normal saline at the same amount and time...
March 22, 2017: Basic & Clinical Pharmacology & Toxicology
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