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Tetralogy of Fallot With Pulmonary Stenosis

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Long-term follow-up after transatrial-transpulmonary repair of tetralogy of Fallot: influence of timing on outcome.

Eva van den Bosch, Ad J J C Bogers, Jolien W Roos-Hesselink, Arie P J van Dijk, Marie H E J van Wijngaarden, Eric Boersma, Aagje Nijveld, Linda W G Luijten, Ronald Tanke, Laurens P Koopman, Willem A Helbing,

OBJECTIVES:Our goal was to report the long-term serial follow-up after transatrial-transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome. METHODS:We included all patients with TOF who had undergone transatrial-transpulmonary repair between 1970 and 2012. Records were reviewed for patient ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, 57(4):635-643]

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Haploinsufficiency of the basic helix-loop-helix transcription factor HAND2 causes congenital heart defects.

Ana S A Cohen, Christopher Simotas, Bryn D Webb, Huanzhi Shi, Wahab A Khan, Lisa Edelmann, Stuart A Scott, Ram Singh,

Congenital heart defects (CHDs) are caused by a disruption in heart morphogenesis, which is dependent, in part, on a network of transcription factors (TFs) that regulate myocardial development. Heterozygous sequence variants in the basic helix-loop-helix TF gene heart and neural crest derivatives expressed 2 (HAND2) have been reported among some ... Read more >>

Am. J. Med. Genet. A (American journal of medical genetics. Part A)
[2020, :]

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Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

Catherine Deshaies, Helen Trottier, Paul Khairy, Mohammed Al-Aklabi, Luc Beauchesne, Pierre-Luc Bernier, Santokh Dhillon, Sanjiv K Gandhi, Christoph Haller, Camille L Hancock Friesen, Edward J Hickey, David Horne, Frédéric Jacques, Marla C Kiess, Jean Perron, Maria Rodriguez, Nancy C Poirier, ,

BACKGROUND:Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES:This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on ... Read more >>

J. Am. Coll. Cardiol. (Journal of the American College of Cardiology)
[2020, 75(9):1033-1043]

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Venoarterial extracorporeal membrane oxygenation in heart surgery post-operative pediatric patients: A retrospective study at Christus Muguerza Hospital, Monterrey, Mexico.

Gerardo Vargas-Camacho, Verónica Contreras-Cepeda, Rene Gómez-Gutierrez, Guillermo Quezada-Valenzuela, Adriana Nieto-Sanjuanero, Jesús Santos-Guzmán, Francisco González-Salazar,

Objectives:Extracorporeal membrane oxygenation is a life support procedure developed to offer cardiorespiratory support when conventional therapies have failed. The purpose of this study is to describe the findings during the first years using venoarterial extracorporeal membrane oxygenation in pediatric patients after cardiovascular surgery at Christus Muguerza High Specialty Hospital in ... Read more >>

SAGE Open Med (SAGE Open Medicine)
[2020, 8:2050312120910353]

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Iron-deficiency anaemia in children with congenital heart diseases at a teaching hospital in Ghana.

Inna Ossei, Kwame Ohene Buabeng, Paul Poku Sampene Ossei, Samuel Blay Nguah, William Gilbert Ayibor, Berko Panyin Anto, Agyemang-Duah Eric, Mahama Duwiejua,

Background:Uncorrected congenital heart lesions in children keep them in a state of constant hypoxia with compromised quality of life and reduced life expectancy. This requires early diagnosis and interventions including prevention and treatment of the resultant anaemia. Unfortunately, congenital heart disease (CHD) often goes unrecognized and thus untreated. Objectives:We determined ... Read more >>

Heliyon (Heliyon)
[2020, 6(2):e03408]

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Congenital heart disease in school children in Lagos, Nigeria: Prevalence and the diagnostic gap.

Ekanem N Ekure, Ogochukwu Sokunbi, Nnenna Kalu, Akinsanya Olusegun-Joseph, Oyewole Kushimo, Casmir Amadi, Olayinka Hassan, Desmond Ikebudu, Sophia Onyia, Chinonso Onwudiwe, Victor Nwankwo, Remi Akinwunmi, Fukpode Awusa, Zainab Akere, Olaolu Dele-Salawu, Elizabeth Ajayi, Olagoke Ale, Dorothy Muoneke, Maximillian Muenke, Paul Kruszka, Andrea Beaton, Craig Sable, Adebowale Adeyemo,

Congenital heart disease (CHD) in low-and-middle income countries (LMIC) is often characterized by late presentation resulting from inadequate screening and healthcare access in these regions. Accurate estimates of the burden of CHD among school children are often lacking. The objective of this study was to determine the prevalence and distribution ... Read more >>

Am J Med Genet C Semin Med Genet (American journal of medical genetics. Part C, Seminars in medical genetics)
[2020, 184(1):47-52]

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Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle.

Hyun Woo Goo,

OBJECTIVE:To demonstrate the feasibility of using cardiothoracic CT for quantification of the initial right ventricle (RV) dimensions in infants with congenital heart disease (CHD) and a hypoplastic RV and to compare these measurements with those obtained in a control group with CHD without a hypoplastic RV. MATERIALS AND METHODS:Initial RV ... Read more >>

Korean J Radiol (Korean journal of radiology)
[2020, 21(2):203-209]

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Left ventricular filling pressure in Tetralogy of Fallot: Correlation between invasive and noninvasive indices.

Alexander C Egbe, Keerthana Banala, Rahul Vojjini, Raja Jadav, Mahir Sufian, Patricia A Pellikka, Naser M Ammash,

Background:Left heart filling pressures, as measured by pulmonary artery wedge pressure (PAWP), is associated with heart failure related mortality. Because of the prognostic importance of PAWP, several echocardiographic indices have been proposed for noninvasive assessment of PAWP. However, these indices have not been validated in the congenital heart disease population. ... Read more >>

Int J Cardiol Heart Vasc (International journal of cardiology. Heart & vasculature)
[2020, 26:100457]

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Use of argatroban in combination with nafamostat mesilate in open-heart surgery for a pediatric patient with heparin-induced thrombocytopenia type II: a case report.

Shuji Kawamoto, Eriko Kusudo, Kazuhiko Fukuda,

BACKGROUND:Heparin-induced thrombocytopenia type II (HIT II) is a rare, immune-mediated complication of heparin therapy and can cause life-threatening thromboembolism. However, perioperative anticoagulation therapy for patients with a complication of HIT II has not been established. CASE PRESENTATION:A 6-year-old boy with tetralogy of Fallot underwent radical intracardiac repair with administration of ... Read more >>

JA Clin Rep (JA clinical reports)
[2020, 6(1):3]

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Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes.

Elisabeth Martin, Richard D Mainwaring, R Thomas Collins, Kirstie L MacMillen, Frank L Hanley,

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was ... Read more >>

Semin. Thorac. Cardiovasc. Surg. (Seminars in thoracic and cardiovascular surgery)
[2020, :]

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The Cardiac Genome Clinic: implementing genome sequencing in pediatric heart disease.

Miriam S Reuter, Rajiv R Chaturvedi, Eriskay Liston, Roozbeh Manshaei, Ritu B Aul, Sarah Bowdin, Iris Cohn, Meredith Curtis, Priya Dhir, Robin Z Hayeems, S Mohsen Hosseini, Reem Khan, Linh G Ly, Christian R Marshall, Luc Mertens, John B A Okello, Sergio L Pereira, Akshaya Raajkumar, Mike Seed, Bhooma Thiruvahindrapuram, Stephen W Scherer, Raymond H Kim, Rebekah K Jobling,

PURPOSE:This study investigated the diagnostic utility of nontargeted genomic testing in patients with pediatric heart disease. METHODS:We analyzed genome sequencing data of 111 families with cardiac lesions for rare, disease-associated variation. RESULTS:In 14 families (12.6%), we identified causative variants: seven were de novo (ANKRD11, KMT2D, NR2F2, POGZ, PTPN11, PURA, SALL1) ... Read more >>

Genet. Med. (Genetics in medicine : official journal of the American College of Medical Genetics)
[2020, :]

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Is Carotid Intima-Media Thickness Increased in Adults With Congenital Heart Disease?

Barbara Reiner, Renate Oberhoffer, Anna-Luisa Häcker, Peter Ewert, Jan Müller,

Background Because of the increasing numbers of congenital patients surviving into adulthood, early diagnosis and prevention of acquired cardiovascular disease is reasonable. The aim of this study was to detect diagnostic subgroups of adults with congenital heart disease (ACHD) that have increased carotid intima-media thickness (cIMT), a subclinical marker of ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(3):e013536]

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Patient-Specific 3-Dimensional-Bioprinted Model for In Vitro Analysis and Treatment Planning of Pulmonary Artery Atresia in Tetralogy of Fallot and Major Aortopulmonary Collateral Arteries.

Martin L Tomov, Alexander Cetnar, Katherine Do, Holly Bauser-Heaton, Vahid Serpooshan,

Background Tetralogy of Fallot with major aortopulmonary collateral arteries is a heterogeneous form of pulmonary artery (PA) stenosis that requires multiple forms of intervention. We present a patient-specific in vitro platform capable of sustained flow that can be used to train proceduralists and surgical teams in current interventions, as well as ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2019, 8(24):e014490]

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Different CMR Imaging Modalities for Native and Patch-Repaired Right Ventricular Outflow Tract Sizing: Impact on Percutaneous Pulmonary Valve Replacement Planning.

Irene Ferrari, Nerejda Shehu, Naira Mkrtchyan, Stefan Martinoff, Andreas Eicken, Heiko Stern, Peter Ewert, Christian Meierhofer,

Percutaneous pulmonary valve replacement (PPVI) in native or patched right ventricular outflow tract (RVOT) has proven to be feasible. The procedure is highly dependent on the size of the RVOT. Several methods exist to evaluate the size of the RVOT by cardiovascular magnetic resonance (CMR). We evaluated different CMR modalities ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, 41(2):382-388]

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An Unusual Cause of Hypoxia: Ventricular Septal Defect, Pulmonary Artery Atresia, and Major Aortopulmonary Collaterals Diagnosed in the Adult Cardiac Catheterization Lab.

Katia Bravo-Jaimes, Brian Walton, Poyee Tung, Richard W Smalling,

The association of pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA) is an extreme form of tetralogy of Fallot (TOF). It carries a high mortality risk if not intervened on during infancy with only 20% of unoperated patients surviving into adulthood. We present the case of a ... Read more >>

Case Rep Cardiol (Case reports in cardiology)
[2020, 2020:4726529]

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Right ventricular outflow tract reconstruction with the Labcor® stentless valved pulmonary conduit.

Jill Jussli-Melchers, Jens Scheewe, Jan Hinnerk Hansen, Christina Grothusen, Julia Steer, Inga Voges, Jana Logoteta, Peter Dütschke, Hans-Heiner Kramer, Tim Attmann,

OBJECTIVES:The right ventricular outflow tract reconstruction is a common necessity in congenital cardiac surgery. As homograft availability is limited, alternatives need to be evaluated. The Labcor® conduit consists of a porcine tricomposite valve assembled inside a bovine pericardium tube. This study presents intermediate-term results for its utilization for right ventricular ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, 57(2):380-387]

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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. Abridged Secondary Publication.

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, B R J Kannan, Ashish Katewa, Vikas Kohli, Nageswara Rao Koneti, Shyam S Kothari, K M Krishnamoorthy, Snehal Kulkarni, Rohit Manoj Kumar, Raman Krishna Kumar, Sunita Maheshwari, Krishna Manohar, Ashutosh Marwah, Smita Mishra, Smruti R Mohanty, Kona Samba Murthy, P V Suresh, S Radhakrishnan, Palleti Rajashekar, Sivasubramanian Ramakrishnan, Nitin Rao, Suresh G Rao, Chinnaswamy Hm Reddy, Rajesh Sharma, Krishnanaik Shivaprakasha, Raghavan Subramanyan, R Suresh Kumar, Sachin Talwar, Munesh Tomar, Sudeep Verma, Vijayakumar Raju, ,

JUSTIFICATION:A number of guidelines are available for management of congenital heart diseases 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 congenital heart diseases, as often these patients present late ... Read more >>

Indian Pediatr (Indian pediatrics)
[2020, 57(2):143-157]

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Multi-detector computed tomography angiographic evaluation of right ventricular outflow tract obstruction and other associated cardiovascular anomalies in tetralogy of Fallot patients.

Rani Kunti R Singh, Neeraj Jain, Sunil Kumar, Naveen Garg,

Purpose:To evaluate various types of right ventricular outflow tract obstruction associated with tetralogy of Fallot (TOF) with emphasis on the abnormality of pulmonary arterial system and other associated cardiovascular anomalies using computed tomography (CT) angiography. Material and methods:We retrospectively evaluated 184 consecutive previously diagnosed TOF patients who underwent CT angiography ... Read more >>

Pol J Radiol (Polish journal of radiology)
[2019, 84:e511-e516]

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A novel index equivalent to the myocardial performance index for right ventricular functional assessment in children and adolescent patients.

Yasunobu Hayabuchi, Yukako Homma, Shoji Kagami,

The aims of the present study were to develop and check the utility and feasibility of a novel right ventricular (RV) functional index (RV angular velocity; RVω, s-1) derived from the angular velocity in harmonic oscillator kinematics obtained from the RV pressure waveform. We hypothesized that RVω reflects the myocardial ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):19975]

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Surgical treatment of tetralogy of Fallot in symptomatic neonates and young infants.

Bobae Jeon, Dong-Hee Kim, Bo Sang Kwon, Eun Seok Choi, Chun Soo Park, Tae-Jin Yun,

OBJECTIVES:Optimal management of tetralogy of Fallot in symptomatic neonates and young infants remains controversial. METHODS:A retrospective review of 53 patients (26 male) with symptomatic tetralogy of Fallot who underwent primary repair (group 1, n = 22) or initial palliation (group 2, n = 31) within 2 months (60 days) after birth between 2005 and 2018 was ... Read more >>

J. Thorac. Cardiovasc. Surg. (The Journal of thoracic and cardiovascular surgery)
[2020, 159(4):1466-1476.e2]

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The Rare Association of Tetralogy of Fallot and ALCAPA.

Alexandre Noboru Murakami, Gabriela Guimarães Baston, Mariana Ribeiro Rodero Cardoso, Carlos Henrique de Marchi, Ulisses Alexandre Croti,

Braz J Cardiovasc Surg (Brazilian journal of cardiovascular surgery)
[2019, 34(5):624-626]

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Pregnancy outcomes among 31 patients with tetralogy of Fallot, a retrospective study.

Kana Wang, Junguo Xin, Xiaodong Wang, Haiyan Yu, Xinghui Liu,

BACKGROUND:Tetralogy of Fallot (TOF) is a severe type of congenital heart disease (CHD) and it confers substantial risk to mother and fetus for pregnant women. However, the outcome of pregnancy in women with TOF has not been well studied. METHODS:Women with TOF who have been seen and/or delivered at our ... Read more >>

BMC Pregnancy Childbirth (BMC pregnancy and childbirth)
[2019, 19(1):486]

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Complete sternal cleft with tetralogy of Fallot.

Debasis Das, Nilanjan Dutta, Pratik Pandey, Adhish Basu, Manish Kumar Sharma, Rangan Koley, Amitabha Chattopadhyay,

Complete sternal cleft is a very rare congenital midline defect of the sternum. It is not uncommonly associated with intracardiac defects. We report a case of a 2-year-old child with complete sternal cleft and tetralogy of Fallot who presented with cyanotic spells. The child underwent total correction, followed by chest ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2020, 13(1):81-83]

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Invasive cardiovascular magnetic resonance (iCMR) for diagnostic right and left heart catheterization using an MR-conditional guidewire and passive visualization in congenital heart disease.

Surendranath R. Veeram Reddy, Yousef Arar, Riad Abou Zahr, Vasu Gooty, Jennifer Hernandez, Amanda Potersnak, Phillip Douglas, Zachary Blair, Joshua S. Greer, Sébastien Roujol, Mari Nieves Velasco Forte, Gerald Greil, Alan W. Nugent, Tarique Hussain,

BackgroundToday’s standard of care, in the congenital heart disease (CHD) population, involves performing cardiac catheterization under x-ray fluoroscopy and cardiac magnetic resonance (CMR) imaging separately. The unique ability of CMR to provide real-time functional imaging in multiple views without ionizing radiation exposure has the potential to be a powerful tool for ... Read more >>

J Cardiovasc Magn Reson (Journal of Cardiovascular Magnetic Resonance)
[2020, 22:]

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Spectrum of heart diseases in children presenting to a paediatric cardiac echocardiography clinic in the Lake Zone of Tanzania: a 7 years overview.

Antke Zuechner, Tumaini Mhada, Naizihijwa G Majani, Godwin G Sharau, William Mahalu, Matthias W Freund,

BACKGROUND:Congenital heart diseases (CHD) are among the most common congenital malformations. It is estimated that the incidence of CHD is constant worldwide, but data are rare for most African countries including Tanzania. Even less data are available on the prevalence of acquired heart diseases (AHD) in African children. Rheumatic heart ... Read more >>

BMC Cardiovasc Disord (BMC cardiovascular disorders)
[2019, 19(1):291]

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