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https://www.readbyqxmd.com/read/27914124/atypical-neonatal-marfan-syndrome-with-p-glu1073lys-mutation-of-fbn1-the-first-case-in-korea
#1
Ju Sun Heo, Joo Young Song, Eun Young Choi, Eun Hee Kim, Ji Hee Kim, So Eun Park, Ji Hyun Jeon
Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna...
January 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27913078/pulmonary-artery-dissection-following-balloon-valvuloplasty-in-a-dog-with-pulmonic-stenosis
#2
K A Grint, H B Kellihan
A 3-month-old, 9.9 kg, male pit bull cross was referred for evaluation of collapse. A left basilar systolic heart murmur graded V/VI and a grade IV/VI right basilar systolic heart murmur were ausculted. Echocardiography showed severe pulmonic stenosis characterized by annular hypoplasia, leaflet thickening, and leaflet fusion. After 1 month of atenolol therapy, a pulmonic valve balloon valvuloplasty procedure was performed, and the intra-operative right ventricular pressure was reduced by 43%. Echocardiography, performed the following day, showed apparent rupture of a pulmonary valve leaflet and a membranous structure within the pulmonary artery consistent with a dissecting membrane...
November 30, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/27909513/assessment-of-sinoatrial-node-function-in-patients-with-persistent-and-long-standing-persistent-forms-of-atrial-fibrillation-after-maze-iii-procedure-combined-with-mitral-valve-operation
#3
Kulikov Aleksei A, Bokeria Leo A
RESEARCH OBJECTIVE: Assessment of sinoatrial node function after Maze III procedure combined with a mitral valve operation. METHODS: 100 patients were included in the research with persistent and long-standing persistent forms of atrial fibrillation (AF) and need of operative treatment concerning valve disease. The following preoperative preparation methods were executed to all patients: Electrocardiogram in 12 standard assignments;Two-dimensional echocardiographic with assessment of systolic and diastolic functions of the left ventricle, size of the left atrium and grade of valve disease;Transesophageal echocardiography for exclusion of blood clots in the left atrium and left atrial appendage;Coronary angiography for exclusion of coronary heart disease;Computer tomography for examination of cardiac chambers and anatomic characteristics of pulmonary veins...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909274/-three-mitral-valve-operations-in-a-patient-with-trousseau-syndrome-and-nonbacterial-thrombotic-endocarditis-caused-by-ovarian-cancer
#4
Masahito Saito, Naoki Asano, Kazunori Ota, Kazuho Niimi, Koyu Tanaka, Shigeyoshi Gon, Hiroshi Takano
A 52-year-old woman was admitted to our hospital for acute right hemi-paresis, left homonymous hemianopia, and fever. Magnetic resonance imaging of the brain showed multiple cerebral infarctions and transesophageal echocardiography (TEE) revealed a vegetation on the posterior leaflet of her mitral valve. Mitral valve repair was performed under a diagnosis of infective endocarditis (IE). Further multiple cerebral infarctions occurred on the 11th postoperative day, resulting in left hemiplegia and dysarthria...
December 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/27904846/complete-congenital-heart-block-in-a-neonate-with-a-complex-congenital-heart-defect-in-africa
#5
Clovis Nkoke, Edvine Yonta Wawo, Liliane Kuate Mfeukeu, Larissa Makamte, Sandrine Dikosso Edie, Flore Esiene Balana
Congenital heart block (CHB) is rare disorder that has a higher mortality when associated with structural congenital heart defects. Very few cases have been reported in Sub-Saharan Africa (SSA). We present a case of complete CHB associated with a complex congenital heart defect in a neonate in Cameroon. A 1-month-old neonate in Cameroon was referred for the evaluation of bradycardia. The obstetrical ultrasound done during pregnancy revealed fetal bradycardia without further evaluation. Clinical examination showed well a developed neonate with bradycardia at 62 beats/minute, and mild cyanosis with oxygen saturation at 93% at room air...
October 2016: Cardiovascular Diagnosis and Therapy
https://www.readbyqxmd.com/read/27903371/-impact-of-reduced-left-ventricular-ejection-fraction-on-all-cause-mortality-of-75-years-old-and-over-patients-with-moderate-to-severe-aortic-stenosis
#6
Z Li, Y Q Ye, M Y Wang, H Y Xu, S Y Teng, J Qian, W Wang, X Wang, Y J Wu
Objective: To evaluate the impact of left ventricular systolic dysfunction on all-cause mortality of 75 years old and over patients with moderate to severe aortic stenosis. Methods: Clinical data of 301 consecutive aged (≥75 years old) patients with a diagnosis of non-rheumatic moderate to severe aortic stenosis by echocardiography between January 1, 2008 and January 1, 2015 in our hospital were retrospectively analyzed. According to the LVEF, patients were divided into normal LVEF subjects and low LVEF subjects, and the low LVEF level was set at below 60%(≤60%(n=171)vs...
October 24, 2016: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/27898500/patterns-and-trends-of-transcatheter-aortic-valve-implantation-in-italy-insights-from-rispeva
#7
Arturo Giordano, Nicola Corcione, Giuseppe Biondi-Zoccai, Sergio Berti, Anna Sonia Petronio, Carlo Pierli, Patrizia Presbitero, Pietro Giudice, Gennaro Sardella, Antonio L Bartorelli, Roberto Bonmassari, Ciro Indolfi, Alfredo Marchese, Elvis Brscic, Alberto Cremonesi, Luca Testa, Nedy Brambilla, Francesco Bedogni
AIMS: Clinical trials have shown that transcatheter aortic valve implantation for aortic stenosis compares favorably to surgical replacement in high-risk patients and is superior to medical therapy in those at prohibitive risk. There is uncertainty however on patterns and trends in transcatheter aortic valve implantation, especially focusing on Italy. METHODS: The RISPEVA study is a prospective Italian registry including 21 institutions. Patients have been enrolled since late 2012, and data collection includes several baseline, procedural, in-hospital, and follow-up details...
November 24, 2016: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/27898434/the-continuing-challenge-of-evaluating-diastolic-function-by-echocardiography-in-children-developing-concepts-and-newer-modalities
#8
Wadi Mawad, Mark K Friedberg
PURPOSE OF REVIEW: Assessment of diastolic function by echocardiography is challenging but important. Left ventricular filling has been more extensively studied than the right ventricle, and predominantly in adult populations. Although multiple parameters exist to assess diastolic function, they all have limitations, including load and heart rate dependency, which make assessment of diastolic function particularly challenging. The purpose of this article is to review evolving concepts and modalities for echo assessment of diastolic function in children...
January 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27895692/impact-of-postoperative-duration-of-aspirin-use-on-longevity-of-bioprosthetic-pulmonary-valve-in-patients-who-underwent-congenital-heart-disease-repair
#9
Tae-Woong Hwang, Sung-Ook Kim, Sang-Yun Lee, Seong-Ho Kim, Eun-Young Choi, So-Ick Jang, Su-Jin Park, Hye-Won Kwon, Hyo-Bin Lim, Chang-Ha Lee, Eun-Seok Choi
PURPOSE: Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases. METHODS: We retrospectively reviewed the clinical data of 137 patients who underwent PVR using a bioprosthetic valve between January 2000 and December 2003...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27894069/association-between-left-atrial-dilatation-and-invasive-hemodynamics-at-rest-and-during-exercise-in-asymptomatic-aortic-stenosis
#10
Nicolaj Lyhne Christensen, Jordi Sanchez Dahl, Rasmus Carter-Storch, Rine Bakkestrøm, Kurt Jensen, Flemming Hald Steffensen, Eva Vad Søndergaard, Lars Videbæk, Jacob Eifer Møller
BACKGROUND: Transition from an asymptomatic to symptomatic state in severe aortic stenosis is often difficult to assess. Identification of a morphological sign of increased hemodynamic load may be important in asymptomatic aortic stenosis to identify patients at risk. METHODS AND RESULTS: Thirty-nine patients with asymptomatic severe aortic stenosis (aortic valve area <1 cm(2), peak jet velocity >3.5 m/s) underwent exercise testing with simultaneous invasive hemodynamic monitoring and Doppler echocardiography...
October 2016: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27892890/trial-design-rivaroxaban-for-the-prevention-of-major-cardiovascular-events-after-transcatheter-aortic-valve-replacement-rationale-and-design-of-the-galileo-study
#11
Stephan Windecker, Jan Tijssen, Gennaro Giustino, Ana H C Guimarães, Roxana Mehran, Marco Valgimigli, Pascal Vranckx, Robert C Welsh, Usman Baber, Gerrit-Anne van Es, Peter Wildgoose, Albert A Volkl, Ana Zazula, Karen Thomitzek, Melanie Hemmrich, George D Dangas
BACKGROUND: Optimal antithrombotic treatment after transcatheter aortic valve replacement (TAVR) is unknown and determined empirically. The direct factor Xa inhibitor rivaroxaban may potentially reduce TAVR-related thrombotic complications and premature valve failure. DESIGN: GALILEO is an international, randomized, open-label, event-driven, phase III trial in more than 1,520 patients without an indication for oral anticoagulation who underwent a successful TAVR (ClinicalTrials...
October 31, 2016: American Heart Journal
https://www.readbyqxmd.com/read/27890843/total-endovascular-aortic-repair-in-a-patient-with-marfan-syndrome
#12
Mau Amako, Rafaëlle Spear, Rachel E Clough, Adrien Hertault, Richard Azzaoui, Teresa Martin Gonzalez, Jonathan Sobocinski, Stéphan Haulon
OBJECTIVE: To describe a total endovascular aortic repair with branched and fenestrated endografts in a young patient with Marfan syndrome and a chronic aortic dissection. Open surgery is the gold standard to treat aortic dissections in patients with aortic disease and Marfan syndrome. METHODS: In 2000, a 38-year-old man with Marfan syndrome underwent open ascending aorta repair for an acute type A aortic dissection. One year later, a redo sternotomy was performed for aortic valve replacement...
November 24, 2016: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/27888571/melody-valve-implantation-within-freestyle-stentless-porcine-aortic-heterograft
#13
James A Kuo, Ashlea A Feezel, Traci N Putnam, Deborah A Schutte
BACKGROUND: The Melody transcatheter pulmonary valve has been implanted successfully worldwide since its first implant in 2000. The vast majority of these valves have been implanted in pulmonary homografts. In our institution, the most common valve used for pulmonary valve replacement is the Medtronic Freestyle stentless porcine aortic heterograft. OBJECTIVE: We describe our experience implanting the Melody valve within the Freestyle heterograft. METHODS: Retrospective chart review was performed...
November 26, 2016: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/27884776/inverted-orientation-improves-decellularization-of-whole-porcine-hearts
#14
Po-Feng Lee, Eric Chau, Rafael Cabello, Alvin T Yeh, Luiz C Sampaio, Andrea S Gobin, Doris A Taylor
In structurally heterogeneous organs, such as heart, it is challenging to retain extracellular matrix integrity in the thinnest regions (eg, valves) during perfusion decellularization and completely remove cellular debris from thicker areas. The high inflow rates necessary to maintain physiologic pressure can distend or damage thin tissues, but lower pressures prolong the process and increase the likelihood of contamination. We examined two novel retrograde decellularization methods for porcine hearts: inverting the heart or venting the apex to decrease inflow rate...
November 21, 2016: Acta Biomaterialia
https://www.readbyqxmd.com/read/27879530/transaortic-edge-to-edge-repair-for-functional-mitral-regurgitation-during-aortic-valve-replacement-a-13-year-experience
#15
Christos G Mihos, Maiteder Larrauri-Reyes, Judy Hung, Orlando Santana
OBJECTIVE: The study evaluated the feasibility of a transaortic edge-to-edge mitral valve repair (Alfieri stitch) for moderate or greater (≥2+) functional mitral regurgitation (MR) in high-risk patients undergoing aortic valve replacement. METHODS: We retrospectively evaluated 40 consecutive patients who underwent aortic valve replacement combined with a transaortic edge-to-edge mitral valve repair for 2+ or greater functional MR, between February 2002 and April 2015...
November 22, 2016: Innovations: Technology and Techniques in Cardiothoracic and Vascular Surgery
https://www.readbyqxmd.com/read/27878632/utility-of-echocardiography-in-the-assessment-of-left-ventricular-diastolic-function-and-restrictive-physiology-in-children-and-young-adults-with-restrictive-cardiomyopathy-a-comparative-echocardiography-catheterization-study
#16
Thomas D Ryan, Peace C Madueme, John L Jefferies, Erik C Michelfelder, Jeffrey A Towbin, Jessica G Woo, Rashmi D Sahay, Eileen C King, Roberta Brown, Ryan A Moore, Michelle A Grenier, Bryan H Goldstein
The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27878333/transcatheter-pulmonary-valve-replacement-and-acute-increase-in-diastolic-pressure-are-associated-with-increases-in-both-systolic-and-diastolic-pulmonary-artery-dimensions
#17
Ryan Callahan, Lisa Bergersen, James E Lock, Audrey C Marshall
Stable positioning of a transcatheter pulmonary valve (TPV) in native outflow tracts depends on a clear understanding of underlying anatomy and outflow tract dimensions. We hypothesized that restoration of pulmonary competence may acutely alter these dimensions. A retrospective single-center review of consecutive patients after TPV placement from 2007 to 2014 was performed. Patients with less than moderate pulmonary regurgitation were excluded. We reviewed acute catheterization data on 46 patients, most with tetralogy of Fallot (70%)...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27875478/functional-mitral-stenosis-following-mitral-valve-repair
#18
Vincent Chan, Thierry Mesana, Subodh Verma
PURPOSE OF REVIEW: This review summarizes the evidence describing functional mitral stenosis (MS) following surgical mitral valve repair. RECENT FINDINGS: Functional MS is an evolving concept and has been implicated in the repair of functional and organic mitral regurgitation. Following repair of functional mitral regurgitation, an elevation in peak transmitral gradient and pulmonary artery pressure has been observed under physiologic stress. These changes have been associated with worse functional status early after restrictive annuloplasty...
November 19, 2016: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27873373/cardiac-output-and-blood-flow-redistribution-in-the-fetus-with-d-loop-transposition-of-the-great-arteries-and-intact-ventricular-septum-insights-into-the-pathophysiology
#19
Max E Godfrey, Kevin G Friedman, Monika Drogosz, Abraham M Rudolph, Wayne Tworetzky
OBJECTIVES: Although the postnatal physiology of D-loop transposition of the great arteries with intact ventricular septum (DTGA-IVS) is well established, little is known about fetal DTGA-IVS. In the normal fetus, the pulmonary valve (PV) is larger than the aortic valve (AoV), there is exclusive right to left flow at the foramen ovale (FO) and ductus arteriosus (DA), and the left ventricle (LV) ejects 40% of combined ventricular output (CVO) through the aorta, primarily to the brain. In DTGA-IVS, the LV ejects O2 -rich blood to the pulmonary artery, theoretically leading to pulmonary vasodilation, increased branch pulmonary arterial flow and reduced DA flow...
November 22, 2016: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/27873344/impact-of-residual-mitral-regurgitation-on-right-ventricular-systolic-function-after-left-ventricular-assist-device-implantation
#20
Serkan Ertugay, Hatice S Kemal, Umit Kahraman, Catagay Engin, Sanem Nalbantgil, Tahir Yagdi, Mustafa Ozbaran
Significant mitral regurgitation (MR) is thought to decrease after left ventricular assist device (LVAD) implantation, and therefore repair of mitral valve is not indicated in current practice. However, residual moderate and severe MR leads to pulmonary artery pressure increase, thereby resulting in right ventricular (RV) dysfunction during follow-up. We examined the impact of residual MR on systolic function of the right ventricle by echocardiography after LVAD implantation. This study included 90 patients (mean age: 51...
November 22, 2016: Artificial Organs
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