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https://www.readbyqxmd.com/read/28436133/population-trends-in-prenatal-detection-of-transposition-of-great-arteries-impact-of-obstetrical-screening-ultrasound-guidelines
#1
Ravi Prasad, Mills Lindsay, Fruitman Deborah, Savard Winnie, Colen Timothy, Khoo Nee, Serrano-Lomelin Jesus, Hornberger Lisa K
BACKGROUND: D-transposition of the great arteries (D-TGA) is one of the most common critical neonatal heart defects. Although there are clinical advantages to a prenatal diagnosis, D-TGA has had low prenatal detection internationally. Recent obstetrical ultrasound guidelines incorporating screening of cardiac outflows may improve prenatal detection of D-TGA. We sought to evaluate trends in the prenatal detection of D-TGA with/without ventricular septal defect (VSD) in Alberta over the past 13 years, examining the potential impact of enhanced ultrasound guidelines in 2009-2010 and 2013 and factors impacting detection...
April 24, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28435681/a-hol-e-y-predicament
#2
Shaiva Ginoya Meka, Daniel Shelden, Amy Mertens, Paul Christensen, Meet Patel
Endocardial cushion defects are congenital abnormalities that result in valvular dysfunction as well as defects (or "holes") in the septa of the heart. They are typically diagnosed in early infancy; presentation late in life is rare. We present the case of a 72-year-old female admitted to the hospital with dyspnoea and palpitations. She was found to have multifocal atrial tachycardia. She suffered cardiac arrest associated with refractory hypoxaemia that required mechanical ventilation and vasodilator therapy with inhaled nitric oxide...
July 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28434921/identification-of-a-novel-and-functional-mutation-in-the-tbx5-gene-in-a-patient-by-screening-from-354-patients-with-isolated-ventricular-septal-defect
#3
Huan-Xin Chen, Xi Zhang, Hai-Tao Hou, Jun Wang, Qin Yang, Xiu-Li Wang, Guo-Wei He
Ventricular septal defect (VSD) is the most frequently occurring congenital heart disease (CHD) in newborns. A number of genetic studies have linked TBX5 mutations to cardiac abnormalities. We aimed to identify potential pathogenic mutations in TBX5 and to provide insights into the etiology of sporadic and isolated VSD. Case-control mutational and functional analyses were performed in 354 sporadic patients with isolated VSD and 341 controls. All the coding exons and intron-exon boundaries of TBX5 were first sequenced in a group of VSD patients and controls...
April 18, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28434546/surgical-strategy-toward-biventricular-repair-for-severe-ebstein-anomaly-in-neonates-and-infancy
#4
Shu-Chien Huang, En-Ting Wu, Shyh-Jye Chen, Chi-Hsiang Huang, Jin-Chung Shih, Hen-Wen Chou, Chung-I Chang, Ing-Sh Chiu, Yih-Sharng Chen
BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan...
April 20, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28431723/successful-biventricular-conversion-late-after-primary-one-and-one-half-ventricle-repair
#5
Takaya Hoashi, Masataka Kitano, Koji Kagisaki, Hajime Ichikawa
A 6-year-old girl with unbalanced atrioventricular septal defect, hypoplastic right ventricle, and severe common atrioventricular valve regurgitation developed patient-prosthetic mismatch. At 6 months old, she underwent primary one and one-half ventricle repair and replacement of left side atrioventricular valve. A catheter examination showed that her right ventricular end-diastolic volume increased from 39.4 mL/m(2) 1 year after to 70 mL/m(2) 3.5 years after the previous surgery. Thus, at the timing of redo left side atrioventricular valve replacement, she was converted successfully to biventricular circulation...
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28427801/-multimodality-imaging-in-the-cardiac-catheterization-laboratory
#6
Karine Warin-Fresse, Sébastien Hascoet, Patrice Guérin
Cardiac catheterization has greatly contributed to the progress made in the management of congenital heart diseases (CHD). Initially used in diagnosis, it allowed the understanding of heart diseases, their anatomy and hemodynamics. Gradually, the development of interventional cardiology has played a major role in the management of these malformations (Patent ductus arteriosus [PDA] and atrial septal defect [ASD] closure, pulmonary dilatation, percutaneous pulmonary valve implantation…). The development of such technology was made possible through the concomitant development of imaging techniques: fluoroscopy, ultrasound, MRI and CT...
April 17, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28426680/echocardiographic-findings-in-infants-with-presumed-congenital-zika-syndrome-retrospective-case-series-study
#7
Danielle Di Cavalcanti, Lucas V Alves, Geraldo J Furtado, Cleusa C Santos, Fabiana G Feitosa, Maria C Ribeiro, Paulo Menge, Izabelle M Lira, Joao G Alves
OBJECTIVE: To report the echocardiographic evaluation of 103 infants with presumed congenital Zika syndrome. METHODS: An observational retrospective study was performed at Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Recife, Brazil. 103 infants with presumed congenital Zika syndrome. All infants had microcephaly and head computed tomography findings compatible with congenital Zika syndrome. Zika IgM antibody was detected in cerebrospinal fluid samples of 23 infants...
2017: PloS One
https://www.readbyqxmd.com/read/28424444/a-successful-cesarean-delivery-without-fetal-or-maternal-morbidity-in-an-eisenmenger-patient-with-cor-triatriatum-sinistrum-double-orifice-mitral-valve-large-ventricular-septal-defect-and-single-ventricle-who-was-under-long-term-bosentan-treatment
#8
Hacer Ceren Tokgöz, Cihangir Kaymaz, Nertila Poci, Özgür Yaşar Akbal, Selçuk Öztürk
Presently described is successful cesarean delivery in a pregnancy superimposed on long-term bosentan treatment in an Eisenmenger syndrome patient with cor triatriatum sinistrum, double-orifice mitral valve, and large ventricular septal defect resulting in single functioning ventricle with double outlets. Cesarean delivery was performed at 27th week of gestation without maternal or fetal morbidity. The infant had no congenital cardiovascular abnormality or any probable teratogenic effect of bosentan treatment during pregnancy...
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28423128/peratrial-device-closure-of-atrial-septal-defect-under-transesophageal-echocardiographic-guidance-without-fluoroscopy-compared-to-conventional-on-pump-surgical-closure
#9
Zhixiang Guo, Chengxin Zhang, Huan Wang, Shenglin Ge
Objective: This study is designed to evaluate the advantages between peratrial device closure under transesophageal echocardiographic guidance and open heart surgery in atrial septal defect. Methods: From November 2011 to September 2014, 28 patients with atrial septal defect were treated. Fourteen patients received peratrial device closure under transesophageal echocardiographic guidance (TEE group) and 14 patients received cardiopulmonary bypass (CPB group). Clinical parameters during intraoperative and postoperative periods were examined...
January 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28416195/pitavastatin-versus-pravastatin-in-adults-with-hiv-1-infection-and-dyslipidaemia-intrepid-12-week-and-52-week-results-of-a-phase-4-multicentre-randomised-double-blind-superiority-trial
#10
Judith A Aberg, Craig A Sponseller, Douglas J Ward, Vladimir A Kryzhanovski, Stuart E Campbell, Melanie A Thompson
BACKGROUND: People living with HIV-1 infection are at greater risk for cardiovascular disease than seronegative adults. Treatment of dyslipidaemia with statins has been challenging in people with HIV because of an increased potential for drug interactions due to competing cytochrome P450 metabolism between statins and commonly used antiretroviral agents. Neither pitavastatin nor pravastatin depend on cytochrome P450 for primary metabolism. We aimed to assess the safety and efficacy of pitavastatin versus pravastatin in adults with HIV and dyslipidaemia...
April 13, 2017: Lancet HIV
https://www.readbyqxmd.com/read/28415904/wire-atrial-septostomy
#11
Masataka Kitano, Masanori Tsukada, Mistuhiro Fujino
Both balloon atrial septostomy and static balloon dilation are often ineffective in creating a large atrial septal defect in patients with a thickened atrial septum. In such situations, blade atrial septostomy and atrial septal stenting are alternative treatments. We have devised and performed a new technique, called wire atrial septostomy, which uses a thin soft wire to create a large defect. The details of the procedure are presented.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28415099/p594contrast-transthoracic-echocardiography-as-a-gatekeeper-for-patent-foramen-ovale-closure
#12
M Muratori, G Italiano, E Innocenti, L Fusini, M Mapelli, G Tamborini, S Ghulam Ali, P Gripari, A Maltagliati, F Celeste, M Pepi
Background.: The presence of patent foramen ovale (PFO) has been linked to many illness, including cryptogenic stroke, transient ischemic attack, migraine, platypnea-orthodeoxia syndrome and decompression sickness in scuba divers. Transesophageal echocardiography is the gold standard technique for the visualization of atrial septal anatomy, but it is a secondary level exam, not always available, with additional associated costs and not completely free from procedural risks. Standard transthoracic echocardiography (TTE) has a too low sensitivity for PFO screening...
December 1, 2016: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28414679/misses-opportunity-tof-diagnoses-in-4th-decade-of-life
#13
O Baniahmad, T Jarreau, A Johnson
CASE: A 40 year old woman with a history of HIV, congestive heart failure secondary to an unknown congenital heart defect, and hypertension presented to our emergency department with worsening edema. On room air, oxygen saturation was 55 percent . On 5L of oxygen via nasal cannula, oxygen saturation was 88 percent . Physical examination was notable for central cyanosis, facial and lid edema, a II/VI holosystolic murmur across right chest radiating to entire right back hemithorax, decreased breath sounds at bases with pulmonary crackles, clubbing of fingers and edema of bilateral lower extremities...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28412961/perventricular-double-device-closure-of-wide-spaced-multi-hole-perimembranous-ventricular-septal-defect
#14
Fei Liang, Li Hongxin, Hai-Zhou Zhang, Guo Wenbin, Cheng-Wei Zou, Zeeshan Farhaj
BACKGROUND: Device closure of a wide-spaced multi-hole PmVSD is difficult to succeed in percutaneous approach. This study is to evaluate the feasibility, safety and efficacy of perventricular device closure of wide-spaced multi-hole PmVSD using a double-device implanting technique. METHODS: Sixteen patients with wide-spaced multi-hole PmVSD underwent perventricular closure with two devices through an inferior median sternotomy approach under transesophageal echocardiographic guidance...
April 17, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28411047/immunodepression-after-cpb-cytokine-dynamics-and-clinics-after-pediatric-cardiac-surgery-a-prospective-trial
#15
Georgia Justus, Christoph Walker, Lisa-Maria Rosenthal, Felix Berger, Oliver Miera, Katharina Rose Luise Schmitt
BACKGROUND: Corrective surgery for congenital heart defects is known to trigger a severe immune reaction. There has been extensive research on the effects of inflammation after cardiopulmonary bypass (CPB). Interestingly, monocytes are observed to be non-responsive to stimulation with lipopolysaccharide (LPS) under these conditions, indicating a state of immunodepression, which lays the ground for second hit infections after cardiosurgery with CPB. OBJECTIVES: The aim of this prospective study was to analyze immunodepression after pediatric cardiopulmonary bypass and to differentiate the effects of monocytic anergy on postoperative outcome...
April 11, 2017: Cytokine
https://www.readbyqxmd.com/read/28406175/nkx2-5-and-sarcospan-genetically-interact-in-the-development-of-the-muscular-ventricular-septum-of-the-heart
#16
Adam A Panzer, Suk D Regmi, DePorres Cormier, Megan T Danzo, Iuan-Bor D Chen, Julia B Winston, Alayna K Hutchinson, Diana Salm, Claire E Schulkey, Rebecca S Cochran, David B Wilson, Patrick Y Jay
The muscular ventricular septum separates the flow of oxygenated and de-oxygenated blood in air-breathing vertebrates. Defects within it, termed muscular ventricular septal defects (VSDs), are common, yet less is known about how they arise than rarer heart defects. Mutations of the cardiac transcription factor NKX2-5 cause cardiac malformations, including muscular VSDs. We describe here a genetic interaction between Nkx2-5 and Sarcospan (Sspn) that affects the risk of muscular VSD in mice. Sspn encodes a protein in the dystrophin-glycoprotein complex...
April 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28405996/ebstein-s-anomaly-with-paradoxical-embolism-to-lower-limb
#17
Gouranga Santra, Shinjan Patra
Ebstein's anomaly (EA) is a rare congenital heart disease. Paradoxical embolism (PDE) may occur in EA due to associated atrial septal defect (ASD). An 18 year old male with EA presented with left lower limb ischemia due to PDE to the limb. No features of cerebral embolism was present. PDE is a potential complication of EA. High level of awareness is needed for early diagnosis of PDE in EA to save a limb in case of embolism to the extremity.
December 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28404481/clinical-utility-and-diagnostic-accuracy-of-palm-held-mini-sized-ultrasonocardiographic-scanner-in-congenital-heart-disease
#18
Mao-Hung Lo, Chien-Fu Huang, I-Chun Lin, Ying-Jui Lin, Hsuan-Chang Kuo, Kai-Sheng Hsieh
BACKGROUND/PURPOSE: To investigate whether a palm-held ultrasonocardiographic scanner would be useful for screening and follow-up in congenital heart disease (CHD). METHODS: We retrospectively reviewed the echocardiographic images from June 1, 2014 to November 1, 2014. All patients underwent two ultrasonographic examinations including palm-held scanner examination and standard echocardiography. To compare the quality of the two instruments, we developed a diagnostic scoring system ranging from 1 point to 10 points, with 10 points indicating the best quality...
April 9, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28403078/renal-dysplasia-characterized-by-prominent-cartilaginous-metaplasia-lesions-in-vacterl-association-a-case-report
#19
Takeo Nakaya, Taiju Hyuga, Yukichi Tanaka, Shina Kawai, Hideo Nakai, Toshiro Niki, Akira Tanaka
BACKGROUND: Renal dysplasia is the most important cause of end-stage renal disease in children. The histopathological characteristic of dysplasia is primitive tubules with fibromuscular disorganization. Renal dysplasia often includes metaplastic cartilage. Metaplastic cartilage in renal dysplasia has been explained as occurring secondary to vesicoureteral reflux (VUR). Additionally, renal dysplasia is observed in renal dysplasia-associated syndromes, which are combinations of multiple developmental malformations and include VACTERL association...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28401252/evaluation-of-ventricular-septal-defect-with-special-reference-to-the-spontaneous-closure-rate-subaortic-ridge-and-aortic-valve-prolapse-ii
#20
Ayse Guler Eroglu, Sezen Ugan Atik, Esma Sengenc, Gulnaz Cig, Irfan Levent Saltik, Funda Oztunc
The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2...
April 12, 2017: Pediatric Cardiology
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