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'Tetralogy of Fallot' (11162)


Feb
2018

The optimal timing for pulmonary valve replacement after Tetralogy of Fallot (TOF) repair remains controversial. In this study, we estimated the feasibility of using flow energy loss (FEL) to predict right ventricular (RV) deterioration due to pulmonary regurgitation after TOF repair. We examined RV outflow tract (RVOT) flow in nine patients who underwent TOF or double-outlet right ventricle repair in the intervention group (Group I) and compared them with three healthy children in the control group (Group C).

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Feb
2018

Introduction The right ventricular adaptations early after surgery in infants with tetralogy of Fallot are important to understand the changes that occur later on in life; this physiology has not been fully delineated. We sought to assess early postoperative right ventricular remodelling in patients with tetralogy of Fallot by cardiac MRI. Materials and method Subjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot.

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Feb
2018

The adult congenital heart disease (ACHD) population will grow with medical advances, but data are limited. We investigated the epidemiological profile of ACHD in Taiwan, a country with a congenital heart disease program since 1955, population of 23 million, and easily accessible high-standard medical care.
ACHD patients, born after 1954, were identified from the nationwide database 2000-2014.

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Feb
2018

The present study aimed to investigate the capabilities of the cardiovascular virtual endoscopy (VE) system in diagnosing tetralogy of Fallot (TOF) and performing measurements. A total of 37 patients underwent two-dimensional echocardiography (2-DE) and multi-detector computed tomography (MDCT) examinations. The obtained MDCT images were applied to a cardiovascular VE system.

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Jan
2018

Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients.

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Mar
2018

Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients.

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Feb
2018

Tetralogy of Fallot (TOF) is one of the most severe forms of cyanotic congenital heart disease (CHD) and is also the most common. Previous genome-wide association study (GWAS) and replication studies have suggested that a polymorphism in the neuropilin 1 (NRP1) gene is significantly associated with the risk of TOF. To further confirm the association between the NRP1 polymorphism and the risk of TOF and to identify additional positive functional single-nucleotide polymorphisms (SNPs) for TOF risk, we systematically screened for functional polymorphisms throughout the regulatory and coding regions of the NRP1 gene.

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Feb
2018

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies.

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Dec
1969

Marfan syndrome is an autosomal dominant genetic disorder that affects connective tissue and is caused by mutations in the fibrillin 1 gene present at chromosome 15. Aortic aneurysm is its main complication, and along the dilation of the aorta root and its descending portion (60-100%), with secondary aortic insufficiency, it increases risk of acute aortic dissection and death. Coronary artery anomalies affect between 0.

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Mar
2018

The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included.

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Feb
2018

The aim of the paper is to present the risk of pregnancy for mother and her child in a young patient who had a surgery to repair Tetralogy of Fallot (ToF), who gave a birth to her firstborn by having a cesarean section.
23 years old patient, in 28 weeks of pregnancy was admitted to the clinic due to her medical record. She did not have any subjective complaints.

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Feb
2018

Patients with tetralogy of Fallot, pulmonary atresia, and multiple aortopulmonary collateral arteries (Tet PA MAPCAs) have a wide spectrum of anatomy and disease severity. Management of these patients can be challenging and often require multiple high-risk surgical and interventional catheterization procedures. These interventions are made challenging by complex anatomy that require the proceduralist to mentally reconstruct three-dimensional anatomic relationships from two-dimensional images.

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Dec
2017

Stented bioprosthetic valves (BPVs) are commonly used for surgical pulmonary valve (PV) replacement in postoperative congenital heart disease, but develop structural failure in a time-related fashion. The Melody transcatheter PV (TPV) (Medtronic, Minneapolis, Minn) has been used to treat BPV dysfunction, but there have been few studies in this population.
We performed a retrospective, multicenter study to evaluate Melody valve function in patients who underwent TPV replacement (TPVR) into a dysfunctional pulmonary BPV.

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Jan
2018

Stillbirth, defined as death of a fetus in utero after 20 weeks of gestation, occurs in 1 to 2% of pregnancies in the United States. Many of these stillborn infants have associated malformations, including chromosome abnormalities, neural tube defects, and malformation syndromes. Other causes are abnormalities of the placenta and maternal conditions, such as pre-eclampsia and obesity.

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Dec
1969

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232.

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Jan
2018

We adopted an operative technique of pulmonary valve (PV) annular enlargement with valve repair in tetralogy of Fallot (TOF) correction to reduce postoperative pulmonary regurgitation (PR) 16 years ago. Here, we have evaluated the long-term results.
Between April 2000 and August 2005, 43 patients (26 men) with tetralogy of Fallot with pulmonary stenosis underwent PV annular enlargement with valve repair.

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Mar
2018

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. It involves anatomical abnormalities that change the normal flow of blood through the heart resulting in low oxygenation. Although not all of the underlying causes of TOF are completely understood, the disease has been associated with varying genetic etiologies including chromosomal abnormalities and Mendelian disorders, but can also occur as an isolated defect.

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Jan
2018

To investigate roaming paths planning for diagnosis of congenital heart diseases (CHD) using a cardiovascular virtual endoscopy (VE) system. Forty children were enrolled. VE system was applied to support in establishing a diagnosis.

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Feb
2018

To evaluate ascending aortic strain (AAS) with cardiac magnetic resonance (CMR) in a large consecutive series of patients with different types of cardiovascular disease (CVD).
Two-dimensional phase-contrast gradient-echo sequences of the ascending aorta were retrospectively reviewed in 1027 patients (726 males, 301 females). Aortic lumen area was segmented using a semi-automatic approach to calculate AAS values.

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Jan
2018

It is suggested that folic acid and/or multivitamins, taken periconceptionally, have a role in the prevention of many congenital anomalies. The aim of this study was to determine the serum micronutrient levels in mother-infant pairs with CHD compared with those with healthy newborns and their mothers.
Serum levels of folic acid, homocysteine, zinc, vitamin A, vitamin D, and vitamin B12 were measured from 108 newborns with CHD (study group) and 103 healthy newborns (control group).

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Jan
2018

Right ventricular (RV) volume overload increases morbidity and mortality after tetralogy of Fallot (TOF) repair. Surgical strategies like pulmonary leaflets sparing and tricuspid valve repair at time of primary repair may decrease RV overload. Our objective is to evaluate early and midterm results of pulmonary leaflets sparing with infundibular preservation and tricuspid valve repair in selected TOF patients with moderate pulmonary annular hypoplasia.

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Jan
2018

Transcatheter, bilateral branch pulmonary artery (PA) valve implantation is a novel treatment for patients with severe pulmonary insufficiency and oversized right ventricle (RV) outflow tract. There is scarce data on efficacy and safety of this approach.
This was a retrospective study of 8 patients with repaired tetralogy of fallot (TOF) who underwent bilateral branch PA valve implantation.

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Jan
2018

This study aimed to identify the clinical predictors of the degree of right ventricular (RV) myocardial fibrosis in patients with repaired tetralogy of Fallot (TOF) with special focus on the RV pressure load.Methods and Results:From April 2004 to March 2017, 30 patients with repaired TOF underwent pulmonary valve replacement and concomitant RV myocardial biopsy. The stroke volume ratio (RV stroke volume/left ventricular stroke volume), RV end-diastolic volume index, and right-to-left ventricular systolic pressure ratio were evaluated with respect to their prognostic value for the degree of RV myocardial fibrosis.

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Jan
2018

The prognostic role of B-type natriuretic peptide (BNP) level was confirmed in chronic heart failure and congenital heart diseases irrespective of the aetiology.
The aim of this study was to compare NT‑proBNP measured in the clinical practice and important clinical and echocardiographic parameters in patients with adult congenital heart diseases under our care.
Data of a total of 70 patients were analysed; 34 patients had corrected tetralogy of Fallot and 19 patients had corrected transposition of the great arteries.

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Dec
2017

Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.

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Dec
1969

The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g.

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Dec
1969

The right ventricle (RV) is not designed to sustain high pressure leading to failure. There are no current medications to help RV contraction, so further information is required on adaption of the RV to such hypertension.
The Right Ventricle in Children (RVENCH) study assessed infants with congenital heart disease undergoing cardiac surgery with hypertensive RV.

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Mar
2018

Adults with repaired tetralogy of Fallot (ToF) have impaired exercise capacity, vascular and cardiac autonomic function, and quality of life (QoL). Specific effects of high-intensity interval or moderate continuous exercise training on these parameters in adults with repaired ToF remain unknown.
Thirty adults with repaired ToF were randomized to either high-intensity interval, moderate intensity continuous training (36 sessions, 2-3 times a week) or usual care (no supervised exercise).

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Jan
2018

The Society of Thoracic Surgeons Congenital Heart Surgery Database is a comprehensive clinical outcomes registry which captures almost all pediatric cardiac surgical operations in the United States. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This article summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency.

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Jan
2018

Cyanotic congenital heart disease presents an increased tendency to bleed in view of subtle coagulation defects. Airway bleeding can be particularly difficult to manage while maintaining an adequate ventilation. An isolated lung bleed with the exclusion of possible traumatic, medical and surgical causes of bleeding, should alert the attending anesthesiologist to the possibility of the collateral-related bleeding.

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Jan
2018

Ventricular tachyarrhythmias (VAs) are the most common cause of death in patients with repaired Tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. Electrophysiological study (EPS) has been proposed to risk stratify TOF patients.
We sought to evaluate a perioperative EPS-guided approach to risk stratify TOF patients undergoing PVR and guide concomitant cryoablation.

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Jan
2018

The increasing survival in the adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death HF than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries.

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Dec
2017

With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF.

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Jan
2018

We present our clinical experience with coronary artery bypass grafting (CABG) in children.
Ten children who underwent CABG between July 1995 and August 2017 were retrospectively analyzed. Data including congenital cardiac malformations, previous surgical procedures, age and sex, type of coronary complications, ischemic events preceding surgery, and ventricular function before and after CABG were recorded.

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Jan
2018

The right ventricular (RV) contractile reserve is a measure of the dynamic function of the RV and is a sensitive indicator of volume load. This can be measured noninvasively using the tricuspid annular plane systolic excursion (TAPSE) during exercise. We studied the RV contractile reserve of patients after tetralogy of Fallot (TOF) repair with varying degree of RV dilation and pulmonary regurgitation (PR), and compared them to a control group.

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Jan
2018

We report the results of a bicuspid expanded polytetrafluoroethylene (ePTFE) valved conduit used for right ventricular outflow tract reconstruction (RVOTR).
Between November 2005 and February 2009, 12 conduits were used for RVOTR. The mean age and weight of patients were 43.

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Dec
2017

To determine potential predictors of ventricular tachyarrhythmia and sudden cardiac death (SCD) occurring late after repair of tetralogy of Fallot (TOF).
Since 1964, 415 patients had undergone total repair for TOF at Niigata University Hospital. Of these, 89 patients who were followed for more than 10 years at our institute were retrospectively reviewed.

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Jan
2018

This report documents the outcomes of cardiac surgical mission trips organized by the International Children's Heart Foundation (ICHF), a nongovernmental organization that provides congenital heart surgery services to the developing world, and discusses factors associated with a reduction of mortality and morbidity in this setting.
A retrospective review of a prospectively maintained database was conducted to identify any patient who underwent surgical intervention during the course of an ICHF mission trip.
From 2008 to 2016, a total of 223 trips were made to 23 countries and 3,783 operations were performed.

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Jan
2018

Traditional palliation for biventricular cyanotic congenital heart lesions often involves staging with systemic-to-pulmonary arterial shunts to secure pulmonary blood flow (PBF) in the newborn period prior to complete repair. However, shunts may lead to life-threatening events secondary to shunt occlusion or acute coronary steal. They may be associated with morbidity secondary to diastolic runoff, systemic steal and volume loading, and do not provide pulsatile flow which has the potential to promote pulmonary artery (PA) growth.

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Jan
2018

Postoperative arrhythmias are a known complication after cardiac surgical repairs for congenital heart disease.
Data were reviewed pertaining to incidence, diagnosis, potential risk factors, and management of postoperative arrhythmias in 369 consecutive patients under 18 years of age, undergoing elective open heart surgery. All children were admitted to the intensive care unit and continuous electrocardiographic monitoring was performed.

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Jan
2018

Rotational angiography (RA) has proven to be an excellent method for evaluating congenital disease (CHD) in the cardiac cath lab, permitting acquisition of 3D datasets with superior spatial resolution. This technique has not been routinely implemented for 3D printing in CHD. We describe our case series of models printed from RA and validate our technique.

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Jan
2018

In tetralogy of Fallot (TOF), the dominant ventricular tachycardia substrates are slow-conducting anatomical isthmuses. Surgical correction has evolved, which might have influenced isthmus presence and dimensions.
One hundred and forty-two postmortem TOF specimens (84/58 corrected/uncorrected) were studied for isthmus presence.

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Feb
2018

miR-138 modulates cardiac morphogenesis in zebrafish. We explored whether a genetic polymorphism in miR-138 might contribute to the occurrence of sporadic congenital heart disease (CHD) and the potential mechanism. We performed a case-control study consisting of 857 CHD cases and 938 non-CHD controls by genotyping miR-138 in a Chinese population.

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Jan
2018

Surgery of the pulmonary valve, right ventricular outflow tract, and pulmonary artery falls under the domain of paediatric cardiac surgery. However, 97 adult patients underwent such operations in our establishment from 1993 to 2016.
This study aims to analyse preoperative risk factors, intraoperative data, postoperative outcomes, and long-term survival to identify the potential predictors of mortality and high-risk patients.

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Dec
2017

Tetralogy of Fallot is the most common cyanotic congenital heart disease. However, its pathogenesis remains to be clarified. The purpose of this study was to identify the genetic variants in Tetralogy of Fallot by whole exome sequencing.

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Dec
1969

Congenital heart disease (CHD) is the most common birth defect. Studies on the development of children with CHD point towards deficits in motoric, cognitive and language development. However, most studies are cross-sectional and there is a gap in the knowledge concerning developmental trajectories, risk and protective factors and a lack of research concerning environmental predictors.

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Dec
2017

In our practice, we noticed an increased frequency of tracheobronchial branching abnormalities (TBAs) in patients with tetralogy of Fallot (ToF). This study aimed to determine whether an association exists between congenital TBAs and ToF with or without pulmonary atresia.
The frequency of TBAs on chest computed tomography was assessed in 55 patients with ToF without pulmonary atresia, 34 patients with ToF with pulmonary arteria, and 100 control patients.

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Dec
2017

It is hypothesized that diminished cerebral vascular resistance or the "brain sparing effect" is associated with fetuses with complex congenital heart defects (CHD) and may affect their neurodevelopmental outcome. An alternative explanation is that it is related to the location, cardiac output, pressure, and resistance in left heart obstructive CHDs. We sought to determine the effects of various left and right heart obstructive defects on the cerebral and placental hemodynamics and to evaluate the utility of these variables for the assessment and prognosis of CHDs.

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