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Supravalvular Ring Mitral Stenosis

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Parachute mitral valve: Morphology and surgical management.

Shi-Min Yuan,

Background:This review aims to discuss morphology and surgical management of parachute mitral valve. Methods:A total of 62 articles in the English language with 330 parachute mitral valve patients were retrieved from the PubMed, HighWire Press, and Cochrane Library databases using specific MeSH terms and keywords between January 2000 and December ... Read more >>

Turk Gogus Kalp Damar Cerrahisi Derg (Turk gogus kalp damar cerrahisi dergisi)
[2020, 28(1):219-226]

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Novel ELN mutation in a Japanese family with a severe form of supravalvular aortic stenosis.

Kaori Sugiyama, Hitoshi Horigome, Lisheng Lin, Takashi Murakami, Junko Shiono, Yoshito Yamashiro, Hiroyuki Matsuura, Hitoshi Yoda, Hiromi Yanagisawa,

BACKGROUND:Supravalvular aortic stenosis (SVAS) is one of the congenital cardiovascular diseases characterized by stenosis of the aorta. The stenotic lesions occur anywhere above the aortic valve in the aortic tree as well as pulmonary arteries and eventually leads to circulatory failure. The disease gene has been identified on the elastin ... Read more >>

Mol Genet Genomic Med (Molecular genetics & genomic medicine)
[2019, 7(11):e986]

Cited: 1 time

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Role of perioperative echocardiography in repair of incomplete shone complex: A case series.

Alok Kumar, Imran Hussain Bhat, Bhupesh Kumar, K S T Shyam,

Multilevel obstruction of left-sided heart structures was originally characterized by Shone et al. The formulation of an appropriate operative strategy remains challenging and needs to be individualized for this complex subset of patients. Intraoperative transesophageal echocardiography (TEE) not only helps in delineating spatial anatomy but also reveals associated anomalies that ... Read more >>

Ann Card Anaesth (Annals of cardiac anaesthesia)
[2019, 22(4):444-448]

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Avoidance of malignant arrhythmia caused by displacement of the right coronary artery ostium in surgical correction of supravalvular aortic stenosis.

Bo Peng, Qiang Wang,

J. Int. Med. Res. (The Journal of international medical research)
[2019, 47(11):5702-5710]

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Investigation of Chromosomal Abnormalities and Microdeletion/ Microduplication(s) in Fifty Iranian Patients with Multiple Congenital Anomalies.

Akbar Mohammadzadeh, Susan Akbaroghli, Ehsan Aghaei-Moghadam, Nejat Mahdieh, Reza Shervin Badv, Payman Jamali, Roxana Kariminejad, Zahra Chavoshzadeh, Saghar Ghasemi Firouzabadi, Roxana Mansour Ghanaie, Ahoura Nozari, Sussan Banihashemi, Fatemeh Hadipour, Zahra Hadipour, Ariana Kariminejad, Hossein Najmabadi, Yousef Shafeghati, Farkhondeh Behjati,

Objective:Major birth defects are inborn structural or functional anomalies with long-term disability and adverse impacts on individuals, families, health-care systems, and societies. Approximately 20% of birth defects are due to chromosomal and genetic conditions. Inspired by the fact that neonatal deaths are caused by birth defects in about 20 and ... Read more >>

Cell J (Cell journal)
[2019, 21(3):337-349]

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A rare late progression form of Sly syndrome mucopolysaccharidosis.

Nathalie Guffon, Roseline Froissart, Alain Fouilhoux,

Mucopolysaccharidoses VII, or Sly syndrome, is linked to mutations in the beta-glucuronidase encoding gene. Sly syndrome is a rare condition and presentation is highly variable, ranging from a prenatal form with severe, lethal fetal hydrops to more benign adolescent or adult forms with simple thoracic kyphosis. Molecular diagnosis of this ... Read more >>

JIMD Rep (JIMD reports)
[2019, 49(1):1-6]

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Dimensional Accuracy and Clinical Value of 3D Printed Models in Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Ivan Wen Wen Lau, Zhonghua Sun,

The aim of this paper is to summarize and evaluate results from existing studies on accuracy and clinical value of three-dimensional printed heart models (3DPHM) for determining whether 3D printing can significantly improve on how the congenital heart disease (CHD) is managed in current clinical practice. Proquest, Google Scholar, Scopus, ... Read more >>

J Clin Med (Journal of clinical medicine)
[2019, 8(9):]

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Adult ALCAPA: from histological picture to clinical features.

Hiroshi Kubota, Hidehito Endo, Hikaru Ishii, Hiroshi Tsuchiya, Yusuke Inaba, Katsunari Terakawa, Yu Takahashi, Mio Noma, Kazuya Takemoto, Seiichi Taniai, Konomi Sakata, Kyoko Soejima, Hiroaki Shimoyamada, Hiroshi Kamma, Hayato Kawakami, Yukihiro Kaneko, Satoru Hirono, Daisuke Izumi, Kazuyuki Ozaki, Tohru Minamino, Hideaki Yoshino, Kenichi Sudo,

BACKGROUND:Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. CASE ... Read more >>

J Cardiothorac Surg (Journal of cardiothoracic surgery)
[2020, 15(1):14]

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Application of the Boston Technical Performance Score to intraoperative echocardiography.

Hannah R Bellsham-Revell, Antigoni Deri, Silvia Caroli, Andrew Durward, Owen I Miller, Sujeev Mathur, Jelena Saundankar, David R Anderson, B Conal Austin, Caner Salih, Kuberan Pushparajah, John M Simpson,

Background:The Technical Performance Score (TPS) developed by Boston Children's Hospital showed surgical outcomes correlate with adequacy of technical repair when implemented on pre-discharge echocardiograms. We applied this scoring system to intraoperative imaging in a tertiary UK congenital heart surgical centre. Methods:After a period of training, intraoperative TPS (epicardial and/or transesophageal ... Read more >>

Echo Res Pract (Echo research and practice)
[2019, 6(3):63-70]

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Cor triatriatum sinister mimicking mitral stenosis.

Onur Işık, Muhammet Akyüz, Ali Rahmi Bakiler,

Turk Gogus Kalp Damar Cerrahisi Derg (Turk gogus kalp damar cerrahisi dergisi)
[2019, 27(2):261-262]

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Echocardiographic assessment of the right heart in adults: a practical guideline from the British Society of Echocardiography

Abbas Zaidi, Daniel S Knight, Daniel X Augustine, Allan Harkness, David Oxborough, Keith Pearce, Liam Ring, Shaun Robinson, Martin Stout, James Willis, Vishal Sharma, ,

The structure and function of the right side of the heart is influenced by a wide range of physiological and pathological conditions. Quantification of right heart parameters is important in a variety of clinical scenarios including diagnosis, prognostication, and monitoring response to therapy. Although echocardiography remains the first-line imaging investigation ... Read more >>

Echo Res Pract (Echo research and practice)
[2020, 7(1):G19-G41]

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Youngest documented rheumatic mitral stenosis with regurgitation in a 28-month-old girl.

Santosh Kumar Sinha, Mohammad Asif, Vikas Mishra, Mahmodulla Razi, Vinay Krishna,

BACKGROUND:Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are the leading causes of acquired diseases in children and young adults in developing countries carrying considerable morbidity and mortality. Rheumatic fever (RF) commonly affects children between 5-15 years old and is rarely seen in < 5 years old. Mitral stenosis ... Read more >>

ARYA Atheroscler (ARYA atherosclerosis)
[2019, 15(6):288-291]

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Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases.

Anita Saxena, Jay Relan, Ravi Agarwal, Neeraj Awasthy, Sushil Azad, Manisha Chakrabarty, Kulbhushan S Dagar, Velayoudam Devagourou, Baiju S Dharan, Saurabh K Gupta, Krishna S Iyer, M Jayranganath, Raja Joshi, Brj Kannan, Ashish Katewa, Vikas Kohli, Shyam S Kothari, K M Krishnamoorthy, Snehal Kulkarni, R Manoj Kumar, R Krishna Kumar, Sunita Maheshwari, Krishna Manohar, Ashutosh Marwah, Smita Mishra, Smruti R Mohanty, K Samba Murthy, K Nageswara Rao, P V Suresh, S Radhakrishnan, Palleti Rajashekar, S Ramakrishnan, Nitin Rao, Suresh G Rao, H M Chinnaswamy Reddy, Rajesh Sharma, Krishnanaik Shivaprakash, Raghavan Subramanyan, R Suresh Kumar, Sachin Talwar, Munesh Tomar, Sudeep Verma, R Vijaykumar,

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(3):254-286]

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Computed tomography imaging characteristics of shone syndrome.

Alan True, Melvin Baidya, Christopher Lui, German Kilimnik, Michael Sadler,

Shone syndrome was first described in 1963 by Dr JD Shone. It is a constellation of congenital abnormalities compromising approximately 0.6% of all cases of congenital cardiac abnormalities. Shone syndrome is also known as Shone complex, involving several characteristic cardiac abnormalities: coarctation of the aorta, subaortic stenosis, supravalvular mitral ring, ... Read more >>

Radiol Case Rep (Radiology case reports)
[2019, 14(2):164-167]

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Incomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient.

Beatrice Chia-Hui Shih, Jae Hong Lim, Jooncheol Min, Eung Re Kim, Jae Gun Kwak, Woong-Han Kim,

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, ... Read more >>

Korean J Thorac Cardiovasc Surg (The Korean journal of thoracic and cardiovascular surgery)
[2019, 52(2):100-104]

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Congenital anomalies of the pulmonary arteries: an imaging overview.

Joanna G Escalon, Lorna P Browne, Tami J Bang, Carlos S Restrepo, Daniel Ocazionez, Daniel Vargas,

Congenital pulmonary artery anomalies represent a diverse group of abnormalities, ranging from asymptomatic incidental findings to causes of sudden cardiac death. While some may be recognized in childhood, others may be found incidentally in adulthood. We review the clinical and imaging findings in patients with congenital anomalies of the pulmonary ... Read more >>

Br J Radiol (The British journal of radiology)
[2018, :20180185]

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Significant incidental cardiac disease on thoracic CT: what the general radiologist needs to know.

Maren Krueger, Paul Cronin, Mohamed Sayyouh, Aine Marie Kelly,

OBJECTIVE:Incidental cardiac findings are often found on chest CT studies, some of which may be clinically significant. The objective of this pictorial review is to illustrate and describe the appearances and management of the most frequently encountered significant cardiac findings on non-electrocardiographically gated thoracic CT. Most radiologists will interpret multidetector ... Read more >>

Insights Imaging (Insights into imaging)
[2019, 10(1):10]

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Parachute mitral valve assessed by cardiac magnetic resonance.

Grazia Casavecchia, Matteo Gravina, Stefano Zicchino, Silvana Capalbo, Matteo Di Biase, Natale Daniele Brunetti,

Parachute mitral valve (PMV) is a congenital cardiac malformation where typically all tendons chordae converge and insert into one major papillary muscle. We report the case of 27-year-old man affected by Hodgkin's lymphoma-mixed cellularity subtype with polychemotherapy treatment and Barrett's esophagus who came to our attention for routine echocardiographic exam ... Read more >>

Interv Med Appl Sci (Interventional medicine & applied science)
[2019, 11(1):65-67]

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Magnetic resonance imaging of the papillary muscles of the left ventricle: normal anatomy, variants, and abnormalities.

Prabhakar Rajiah, Nicholas Lim Fulton, Michael Bolen,

Left ventricular papillary muscles are small myocardial structures that play an important role in the functioning of mitral valve and left ventricle. Typically, there are two groups of papillary muscles, namely the anterolateral and the posteromedial groups. Cardiovascular magnetic resonance (CMR) is a valuable imaging modality in the evaluation of ... Read more >>

Insights Imaging (Insights into imaging)
[2019, 10(1):83]

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Cost-effectiveness of Humanitarian Pediatric Cardiac Surgery Programs in Low- and Middle-Income Countries.

Marcelo Cardarelli, Sumeet Vaikunth, Katie Mills, Thomas DiSessa, Frank Molloy, Elizabeth Sauter, Karen Bowtell, Roslyn Rivera, Andrew Y Shin, William Novick,

Importance:Endorsement of global humanitarian interventions is based on either proven cost-effectiveness or perceived public health benefits. The cost-effectiveness and long-term benefits of global humanitarian pediatric cardiac surgery are unknown, and funding for this intervention is insufficient. Objectives:To determine the cost-effectiveness of the intervention (multiple 2-week-long humanitarian pediatric cardiac surgery program ... Read more >>

JAMA Netw Open (JAMA network open)
[2018, 1(7):e184707]

Cited: 2 times

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Syndromes with aortic involvement: pictorial review.

Evan J Zucker,

A variety of syndromes are associated with thoracoabdominal aortic pathologies. While these diseases are collectively rare, the presence of advanced or unusual aortic disease at a young age should raise suspicion of an underlying syndrome. Similarly, patients with a known syndrome require close monitoring in anticipation of future aortic disease. ... Read more >>

Cardiovasc Diagn Ther (Cardiovascular diagnosis and therapy)
[2018, 8(Suppl 1):S71-S81]

Cited: 1 time

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Isolated adult congenital uni-leaflet severe mitral valve stenosis, a case report, and review of literature.

Hesham A Naeim, Elsayed A Taha, Reham A Taha, Faisal O Alatawi, Osama Amodi, Reda Abuelatta,

Reported cases of uni-leaflet mitral valve (MV) were related to the absence or dysplasia of the posterior mitral leaflet with ample anterior mitral leaflet. We present here a new entity of uni-leaflet MV where the MV appears as a membrane-like structure with a single slit-like orifice at its lateral part ... Read more >>

J Cardiol Cases (Journal of Cardiology Cases)
[2019, 19(6):177-181]

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Shone's syndrome: Insights from three-dimensional echocardiography.

Martín F Vivas, María T Politi, Laura M Riznyk, María F Castro, Gustavo Avegliano, Ricardo Ronderos,

Shone's syndrome is a rare congenital anomaly defined as the presence of at least two of the following heart obstructions: a mitral supravalvular ring, a "parachute" mitral valve stenosis, subaortic stenosis, and aortic coarctation. A 58-year-old man presented with a mitral ring and a "parachute" mitral valve on two-dimensional transthoracic ... Read more >>

Echocardiography (Echocardiography (Mount Kisco, N.Y.))
[2018, 35(3):417-419]

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Subvalvular aortic stenosis: a review of current literature.

Subodh R Devabhaktuni, Eyas Chakfeh, Ali O Malik, Joshua A Pengson, Jibran Rana, Chowdhury H Ahsan,

Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. ... Read more >>

Clin Cardiol (Clinical cardiology)
[2018, 41(1):131-136]

Cited: 1 time

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Influence of factor XIII activity on post-operative transfusion in congenital cardiac surgery-A retrospective analysis.

Fabian B Fahlbusch, Thomas Heinlein, Manfred Rauh, Sven Dittrich, Robert Cesnjevar, Julia Moosmann, Jennifer Nadal, Matthias Schmid, Frank Muench, Michael Schroth, Wolfgang Rascher, Hans-Georg Topf,

OBJECTIVES:Coagulation factor XIII (FXIII) plays a key role in fibrin clot stabilization-an essential process for wound healing following cardiothoracic surgery. However, FXIII deficiency as a risk for post-operative bleeding in pediatric cardiac surgery involving cardiopulmonary bypass (CPB) for congenital heart disease (CHD) is controversially discussed. Thus, as primary outcome measures, ... Read more >>

PLoS ONE (PloS one)
[2018, 13(7):e0199240]

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