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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/28329232/mid-term-results-of-bilateral-lung-transplant-with-postoperatively-extended-intraoperative-extracorporeal-membrane-oxygenation-for-severe-pulmonary-hypertension%C3%A2
#3
Jawad Salman, Fabio Ius, Wiebke Sommer, Thierry Siemeni, Christian Kuehn, Murat Avsar, Dietmar Boethig, Ulrich Molitoris, Christoph Bara, Jens Gottlieb, Tobias Welte, Axel Haverich, Marius M Hoeper, Gregor Warnecke, Igor Tudorache
OBJECTIVES: In severe pulmonary hypertension, diastolic dysfunction of the left ventricle causes significant morbidity and mortality after lung transplantation, which may be successfully reversed using a protocol based on perioperative veno-arterial extracorporeal membrane oxygenation (ECMO) and early extubation. Here, we present echocardiographic data and mid-term outcomes. METHODS: The records of lung transplanted patients at our institution between May 2010 and January 2016 were retrospectively reviewed...
March 2, 2017: European Journal of Cardio-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
#4
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
#5
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/28329164/large-animal-model-of-functional-tricuspid-regurgitation-in-pacing-induced-end-stage-heart-failure
#6
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/28329111/hypoplastic-left-heart-syndrome-a-novel-surgical-strategy-for-small-volume-centres-%C3%A2
#7
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/28329065/right-ventricular-outflow-tract-systolic-function-correlates-with-exercise-capacity-in-patients-with-severe-right-ventricle-dilatation-after-repair-of-tetralogy-of-fallot
#8
Shuhua Luo, Jianhua Li, Dan Yang, Yaxin Zhou, Qi An, Yucheng Chen
OBJECTIVES: The relationship between exercise capacity and right ventricular (RV) components function in repaired tetralogy of Fallot patients with severely dilated right ventricles is poorly understood. The aim of this study was to characterize the exercise capacity and its relationship to RV global and components function in repaired tetralogy of Fallot patients with RV end-diastolic volume index  >150 ml/m 2 , a currently accepted threshold for pulmonary valve replacement. METHODS: The medical records and results of cardiac magnetic resonance imaging and cardiopulmonary exercise testing of 25 consecutive eligible patients were reviewed...
February 16, 2017: Interactive Cardiovascular and Thoracic Surgery
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
#9
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/28325720/giant-right-ventricle-secondary-to-severe-pulmonary-hypertension
#10
Kunal Kishor Jha, Nuwadatta Subedi, Durgesh Prasad Chaudhary, Manoj Lamsal
No abstract text is available yet for this article.
March 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28325518/the-impact-of-age-and-gender-on-right-ventricular-diastolic-function-among-healthy-adults
#11
Antonello D'Andrea, Olga Vriz, Andreina Carbone, Francesco Ferrara, Marco Di Maio, Rosangela Cocchia, Gianluigi Tagliamonte, Edwige Acri, Caterina Driussi, Enrica Pezzullo, Rodolfo Citro, Antonio Cittadini, Raffaele Calabrò, Maria Giovanna Russo, Eduardo Bossone
BACKGROUND: Doppler echocardiography is ideally suited for assessment of diastolic function, being widely available, non-invasive, and less expensive than other techniques. However, data regarding age- and gender-matched reference values of right ventricular diastolic function are limited. This study aims to explore the physiologic variations of right ventricle (RV) diastolic function in a large cohort of healthy adults, and to investigate clinical and echocardiographic correlates. METHODS: From June 2007 to February 2014, 1168 healthy Caucasian subjects [mean age 45...
March 18, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28325006/a-simplified-state-space-model-of-biventricular-assist-device-cardiovascular-system-interaction
#12
Vivian C A Koh, Einly Lim, Boon Chiang Ng, Yong Kuen Ho, Nigel H Lovell
A simplified state-space model of biventricular assist device (BiVAD)-cardiovascular system (CVS) interaction is presented. The state-space equations includes a six-compartments CVS model incorporating the ventricles, the pulmonary and systemic circulations as well as the non-linear behavior of the valve flow, together with a left ventricular assist device (LVAD) and a right ventricular assist device (RVAD) component. The left and right pump speeds serve as the input variables for the state-space model. The model is simulated with three operational modes, i...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28321539/secoisolariciresinol-diglucoside-attenuates-cardiac-hypertrophy-and-oxidative-stress-in-monocrotaline-induced-right-heart-dysfunction
#13
Stephanie Puukila, Rafael Oliveira Fernandes, Patrick Türck, Cristina Campos Carraro, Jéssica Hellen Poletto Bonetto, Bruna Gazzi de Lima-Seolin, Alex Sander da Rosa Araujo, Adriane Belló-Klein, Douglas Boreham, Neelam Khaper
Pulmonary arterial hypertension (PAH) occurs when remodeling of pulmonary vessels leads to increased pulmonary vascular resistance resulting in increased pulmonary arterial pressure. Increased pulmonary arterial pressure results in right ventricle hypertrophy and eventually heart failure. Oxidative stress has been implicated in the pathogenesis of PAH and may play a role in the regulation of cellular signaling involved in cardiac response to pressure overload. Secoisolariciresinol diglucoside (SDG), a component from flaxseed, has been shown to reduce cardiac oxidative stress in various pathophysiological conditions...
March 20, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28321067/the-histological-features-of-a-myocardial-biopsy-specimen-in-a-patient-in-the-acute-phase-of-reversible-catecholamine-induced-cardiomyopathy-due-to-pheochromocytoma
#14
Miyuki Miura, Hiroaki Kawano, Takeo Yoshida, Yuki Yamagata, Tomoo Nakata, Seiji Koga, Satoshi Ikeda, Kan Kageyama, Kuniko Abe, Koji Maemura
A 63-year-old Japanese woman with an adrenal tumor was transferred to our hospital due to cardiogenic shock. Right and left ventriculography showed severe hypokinesis of the middle segment and the apex in both ventricles, and an endomyocardial biopsy demonstrated a small number of necrotic myocytes and cellular infiltration. She was diagnosed with pheochromocytoma and quickly recovered after treatment with an α-blocker. The functional disability of both the right and left ventricles with less myocardial damage due to an excessive level of catecholamine seemed to be related to the early recovery the present patient with catecholamine-induced cardiomyopathy due to pheochromocytoma...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28320896/estrogen-maintains-mitochondrial-content-and-function-in-the-right-ventricle-of-rats-with-pulmonary-hypertension
#15
Aiping Liu, Jennifer Philip, Kalyan C Vinnakota, Francoise Van den Bergh, Diana M Tabima, Timothy Hacker, Daniel A Beard, Naomi C Chesler
The typical cause of death in pulmonary hypertension (PH) is right ventricular (RV) failure, with females showing better survival rates than males. Recently, metabolic shift and mitochondrial dysfunction have been demonstrated in RV failure secondary to PH In light of evidence showing that estrogen protects mitochondrial function and biogenesis in noncardiovascular systems, we hypothesized that the mechanism by which estrogen preserves RV function is via protection of mitochondrial content and oxidative capacity in PH We used a well-established model of PH (Sugen+Hypoxia) in ovariectomized female rats with/without estrogen treatment...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28318665/the-relation-between-local-repolarization-and-t-wave-morphology-in-heart-failure-patients
#16
Francesco Maffessanti, Joris Wanten, Mark Potse, Francois Regoli, Maria Luce Caputo, Giulio Conte, Daniel Sürder, Annekatrin Illner, Rolf Krause, Tiziano Moccetti, Angelo Auricchio, Frits W Prinzen
BACKGROUND: Both duration and morphology of the T-wave are regarded important parameters describing repolarization of the ventricles. Conventionally, T-wave concordance is explained by an inverse relation between the time of depolarization (TD) and repolarization (TR). Little is known about T-wave morphology and TD-TR relations in patients with heart failure. METHODS: Electro-anatomic maps were obtained in the left (LV) and right ventricle (RV) and in the coronary sinus (CS) in patients with heart failure with narrow (nQRS, n=8) and wide QRS complex with (LBBB, n=15) and without left bundle branch block (non-LBBB, n=7)...
February 22, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28318537/cardiac-magnetic-resonance-imaging-in-suspected-blunt-cardiac-injury-a-prospective-pilot-cohort-study
#17
Aidan Jc Burrell, David M Kaye, Mark C Fitzgerald, David J Cooper, James L Hare, Benedict T Costello, Andrew J Taylor
INTRODUCTION: The aim of this study was to evaluate the incidence and severity of blunt cardiac injury (BCI) as determined by cardiac magnetic resonance imaging (CMR), and to compare this to currently used diagnostic methods in severely injured patients. MATERIALS AND METHODS: We conducted a prospective, pilot cohort study of 42 major trauma patients from July 2013 to Jan 2015. The cohort underwent CMR within 7 days, enrolling 21 patients with evidence of chest injury and an elevated Troponin I compared to 21 patients without chest injury who acted as controls...
February 27, 2017: Injury
https://www.readbyqxmd.com/read/28317159/surgical-planning-for-a-complex-double-outlet-right-ventricle-using-3d-printing
#18
Puneet Bhatla, Justin T Tretter, Sathish Chikkabyrappa, Sujata Chakravarti, Ralph S Mosca
Rapid prototyping may be beneficial in properly selected cases of complex congenital heart disease, providing detailed anatomical understanding that helps to guide potential surgical and cardiac catheterization interventions. We present a case of double-outlet right ventricle, where the decision to obtain a three-dimensional printed model helped for better understanding of the anatomy, with the additional advantage of surgical simulation in planning the surgical approach and type of surgical repair.
March 19, 2017: Echocardiography
https://www.readbyqxmd.com/read/28316327/obstructive-primary-cardiac-t-cell-lymphoma-a-case-report-from-senegal
#19
Simon Antoine Sarr, Abdou Majib Gaye, Fatou Aw, Jean de Dieu Nzambaza, Malick Bodian, Kana Babaka, Mouhamadou Bamba Ndiaye, Adama Kane, Maboury Diao, Serigne Abdou Ba
BACKGROUND Cardiac lymphoma is a rare entity, defined by the non-extra cardiac location at diagnosis. CASE REPORT Our patient was a 32-year-old female with no particular medical history, who presented with right heart failure with recurrent ascites and pleural effusion. There was a progressive worsening exertional dyspnea. On admission, examination revealed an irregular tachycardia at 170 beats per minute (bpm) and congestive heart failure. The electrocardiogram scored full tachyarrhythmia by atrial fibrillation with an average ventricular rate of 179 cycles per minute...
March 20, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28316073/arrhythmic-effects-of-epac-mediated-ryanodine-receptor-activation-in-langendorff-perfused-murine-hearts-are-associated-with-reduced-conduction-velocity
#20
Mengye Li, Sandeep Hothi, Samantha C Salvage, Kamalan Jeevaratnam, Andrew A Grace, Christopher L-H Huang
Recent papers have attributed arrhythmic substrate in murine RyR2-P2328S hearts to reduced action potential (AP) conduction velocities (CV), reflecting acute functional inhibition and/or reduced expression of sodium channels. We explored for acute effects of direct Epac (exchange protein directly activated by cAMP)-mediated ryanodine receptor-2 (RyR2) activation on arrhythmic substrate and CV. Monophasic action potential recordings demonstrated that initial steady (8-Hz) extrinsic pacing elicited ventricular tachycardia (VT) in 0 of 18 Langendorff-perfused wild-type mouse ventricles before pharmacological intervention...
March 18, 2017: Clinical and Experimental Pharmacology & Physiology
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