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Sample records for tetralogy of fallot

  1. Facts about Tetralogy of Fallot

    Science.gov (United States)

    ... Websites Information For… Media Policy Makers Facts about Tetralogy of Fallot Language: English (US) Español (Spanish) Recommend on Facebook ... in the mother’s womb during pregnancy. What is Tetralogy of Fallot? Tetralogy of Fallot is made up of the ...

  2. Tetralogy of Fallot

    NARCIS (Netherlands)

    Gouw, Samantha C.; Le, Thuy-Nga; Sreeram, Narayanswami

    2001-01-01

    The optimal surgical approach and timing for patients with tetralogy of Fallot remain controversial. There are two options in current practice: a two-stage repair (an initial palliative aortopulmonary shunt at an early age followed by complete repair at an older age) or primary complete repair.

  3. What Is Tetralogy of Fallot?

    Science.gov (United States)

    ... people who have repaired tetralogy of Fallot approach middle age, they can develop coronary heart disease (CHD). (Adults ... pregnancy Medicines they can take during pregnancy Any new or worsening symptoms If possible, these women should ...

  4. Cineangiocardiography in tetralogy of Fallot

    International Nuclear Information System (INIS)

    Partridge, J.B.; Fiddler, G.I.

    1981-01-01

    The advantages of angled angiographic projections are demonstrated in a review of 55 cases of tetralogy of Fallot. Particular attention was paid to the origins of the branch pulmonary arteries, whose diameter was measured and compared with that of the descending aorta, showing that in 12 per cent there was narrowing to below 40 per cent of descending aortic diameter. Right aortic arch was present in seven cases (13%) and a major coronary artery anomaly in two (4%). (author)

  5. Prenatal color Doppler ultrasonographic diagnosis of fetal tetralogy of Fallot

    International Nuclear Information System (INIS)

    Tan Buqiao

    2009-01-01

    Objective: To investigate the sonographic findings of tetralogy of Fallot in fetuses. Methods: The data of color Doppler ultrasonography and follow-up results of 5 fetal tetralogy of Fallot were analyzed retrospectively, and their abnormal ultrasound imaging characteristic were summarized. Results: Two cases were proved tetralogy of Fallot by autopsy, and three cases were confirmed to be tetralogy of Fallot by echocardiography after birth. The image features were the main aorta situated above the ventricular septal defect, pulmonary stenosis, no obvious thickening of the right wall. Conclusion: Fetal tetralogy of Fallot have characteristic ultrasound images, prenatal color Doppler ultrasonographic can diagnoses fetal tetralogy of Fallot correctly and has important clinical value. (authors)

  6. Tetralogy of Fallot: work of literature review

    International Nuclear Information System (INIS)

    Campos Alfaro, Franklin R.

    2010-01-01

    A literature search was conducted in PubMed and Medline data base, under the theme Tetralogyan Fallot. The publications before 2000 or without full text were excluded. A total of 30 items were used on tetralogy of Fallot are included; the emphasis with pulmonary artery is treated without deepening [es

  7. MR tomography of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Stark, P.; Agness, M.; Holshouser, B.; Hinshaw, D. Jr.

    1991-01-01

    We present four patients with Tetralogy of Fallot, imaged with magnetic resonance. MRI is useful in clearly depicting the dilated ascending aorta, the infundibular stenosis, ventricular septal defect, right ventricular hypertrophy and dilated bronchial arteries. The pulmonary outflow tract can be visualized better with MR imaging than with ultrasound. (orig.) [de

  8. Adult presentation of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Popeski, P.; Valcheva, E.; Dulgerov, B.

    2013-01-01

    Full text: Introduction: The tetralogy of Fallot is the most common form of cyanotic congenital heart malformation. The term was coined by Abbott in 1924 in honor of Etienne-Louis Fallot, which in 1888, describes physiology of this Congenital Heart Defect (CHD). It is characterized with obstruction in the outflow tract of the right ventricle, bundle defect riding aorta and hypertrophy of the right ventricle. What you will learn: We present a 16- year-old boy with tetralogy of Fallot, which is diagnosed even a newborn. When the patient was 2 years old is the radical correction was made. In the course of ambulatory monitoring a progress of the lung failure due to pulmonary regurgitation has been noticed and four years after the operation the pulmonary insufficiency is estimated to stage III. The patient proceeds to correct pulmonary failure. After clinical and radiological discussion was decided to perform a detailed MDCT preoperative evaluation (morphology and function of the right ventricle and the main branches of the pulmonary artery, coronary anatomy). Discussion: The tetralogy of Fallot represents 10% of all congenital heart disease and is the most common cyanotic CHD. With the increase in the number of surviving patients after radical correction it is showed that initially considered as benign residual pulmonary insufficiency is haemodynamically significant and leads to progressive dilatation of the right ventricular, arrhythmias and cardiac death. This requires the monitoring of these patients, assessment of the degree of pulmonary insufficiency and dilation of the right camera for accurate time assessment for post- surgical correction of pulmonary insufficiency before occurring of irreversible dilatation of the right camera. Conclusion: MDCT is a noninvasive diagnostic image method allowing morphological and functional assessment (contractility of the right ventricle, calculation of volumes - EF, FS and SV and fraction of the right ventricle forcing), which

  9. UNCORRECTED TETRALOGY OF FALLOT IN A 30 - YEARS OLD

    Directory of Open Access Journals (Sweden)

    Dihingia

    2015-03-01

    Full Text Available Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. Survival after the age of 12 years without corrective surgery is rare. We present the case of a 30 year - old man with uncorrected tetralogy of Fallot.

  10. Heart failure complicating tetralogy of Fallot | Ogunkunle | West ...

    African Journals Online (AJOL)

    Background: Heart failure is considered to be an unusual complication of uncorrected tetralogy of Fallot. Patients and method: Three adolescents with tetralogy of Fallot, presenting in congestive cardiac failure are presented. Two died. Myocardial infarction was found in the only patient that underwent autopsy, and is thought ...

  11. Tetralogy of Fallot: General Principles of Management.

    Science.gov (United States)

    Downing, Tacy E; Kim, Yuli Y

    2015-11-01

    Repaired tetralogy of Fallot (TOF) is one of the most common diagnoses encountered when caring for adults with congenital heart disease. Although long-term survival after childhood TOF repair is excellent, morbidity is common and most patients require reintervention in adulthood. This review provides an overview of key surveillance and management issues for adults with TOF, including residual right ventricular outflow tract disease and timing of pulmonary valve replacement, arrhythmias and risk stratification, left-sided heart disease and heart failure, and pregnancy management. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Tetralogy of Fallot: A Surgical Perspective

    Science.gov (United States)

    2012-01-01

    Tetralogy of Fallot (TOF) is an index lesion for all paediatric and congenital heart surgeons. In designing an appropriate operation for children with TOF, the predicted postoperative physiology must be taken into account, both for the short and long term. A favourable balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of biventricular function. A unified repair strategy to limit both residual PS and PI is presented, along with supportive experimental evidence. A strategy for dealing with coronary anomalies and some comments regarding best timing of operation are also included. PMID:22880165

  13. The Nager acrofacial dysostosis syndrome with the tetralogy of Fallot.

    Science.gov (United States)

    Thompson, E; Cadbury, R; Baraitser, M

    1985-10-01

    A male infant is described with mandibulofacial dysostosis and absent thumbs, consistent with the Nager acrofacial dysostosis syndrome. In addition, the tetralogy of Fallot was present. Major congenital heart malformations occur rarely in this syndrome.

  14. Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue

    OpenAIRE

    Mahipat Raj Soni; Deepak A. Bohara; Ajay U. Mahajan; Pratap J. Nathani

    2012-01-01

    In tetralogy of Fallot septal defect is usually large because of malalignment of outlet septum, restrictive defect has been reported rarely. We present a case of tetralogy of Fallot with accessory tricuspid leaflet tissue restricting ventricular septal defect. The report includes echocardiographic and catheter images of this rare presentation of tetralogy of Fallot.

  15. Tetralogy of fallot in down syndrome (trisomy 21) - an uncommon association

    International Nuclear Information System (INIS)

    Rashid, A.K.M.M.; Basu, B.; Rahman, M.M.

    2009-01-01

    Down Syndrome (trisomy 21) is the common disorder among chromosomal anomalies. This is frequently associated with congenital a cyanotic heart disease. Tetralogy of fallot is an uncommon event in the trisomy 21. Tetralogy of fallot presents with cyanosis usually in the later part of infancy, but cyanosis is present since birth if Tetralogy of Fallot is accompanied with Down Syndrome. (author)

  16. Tetralogy of Fallot in monozygotic triplets

    Energy Technology Data Exchange (ETDEWEB)

    Victorica, B.E.; Kumar, A.; Zori, R.T. [Univ. of Florida, Gainesville, FL (United States)

    1994-09-01

    Tetralogy of Fallot (TOF), like most other congenital heart defects, is considered to be of multifactorial inheritance. Occasional families with multiple affected members in one or more generations above been described. A stronger genetic influence in the causation of isolated TOF is also supported by recent demonstration of microdeletions in chromosome 22q11 region. Deletions in this region are also responsible for DiGeorge and velocardiofacial syndrome as well as CHARGE association. We report a set of monozygotic triplets born to healthy parents at 35 weeks of gestation. There was no family history of congenital heart defects. All three had TOF with left aortic arch (documented by cardiac catheterization in 2 and echocardiography in all 3). The degree of right ventricular outflow obstruction varied from mild to complete atresia needing prostaglandin infusion and a subsequent Blalock-Taussig shunt in one. No features of DiGeorge syndrome or any other congenital defects were present. High resolution chromosome analysis of peripheral blood lymphocytes of these infants revealed normal 46,XY male karyotype. Fluorescent in situ hybridization (FISH) using probe D22S75, which maps to chromosome 22q11.2 did not detect any deletion. This pedigree suggests a de novo mutation causing TOF in all 3 monozygotic triplets. Although there is no deletion demonstrable in DiGeorge critical region, a smaller deletion or mutation in this region cannot be excluded.

  17. Arrhythmias After Tetralogy of Fallot Repair

    Science.gov (United States)

    Folino, Antonio Franco; Daliento, Luciano

    2005-01-01

    Tetralogy of Fallot is the most common cyanotic congenital heart disease, with a good outcome after total surgical correction. In spite of a low perioperative mortality and a good quality of life, late sudden death remains a significant clinical problem, mainly related to episodes of sustained ventricular tachycardia and ventricular fibrillation. Fibro-fatty substitution around infundibular resection, intraventricular septal scar, and patchy myocardial fibrosis, may provide anatomical substrates of abnormal depolarization and repolarization causing reentrant ventricular arrhythmias. Several non-invasive indices based on classical examination such as ECG, signal-averaging ECG, and echocardiography have been proposed to identify patients at high risk of sudden death, with hopeful results. In the last years other more sophisticated invasive and non-invasive tools, such as heart rate variability, electroanatomic mapping and cardiac magnetic resonance added a relevant contribution to risk stratification. Even if each method per se is affected by some limitations, a comprehensive multifactorial clinical and investigative examination can provide an accurate risk evaluation for every patient. PMID:16943881

  18. Arrhythmias After Tetralogy of Fallot Repair

    Directory of Open Access Journals (Sweden)

    Antonio Franco Folino

    2005-10-01

    Full Text Available Tetralogy of Fallot is the most common cyanotic congenital heart disease, with a good outcome after total surgical correction. In spite of a low perioperative mortality and a good quality of life, late sudden death remains a significant clinical problem, mainly related to episodes of sustained ventricular tachycardia and ventricular fibrillation. Fibro-fatty substitution around infundibular resection, intraventricular septal scar, and patchy myocardial fibrosis, may provide anatomical substrates of abnormal depolarization and repolarization causing reentrant ventricular arrhythmias. Several non-invasive indices based on classical examination such as ECG, signal-averaging ECG, and echocardiography have been proposed to identify patients at high risk of sudden death, with hopeful results. In the last years other more sophisticated invasive and non-invasive tools, such as heart rate variability, electroanatomic mapping and cardiac magnetic resonance added a relevant contribution to risk stratification. Even if each method per se is affected by some limitations, a comprehensive multifactorial clinical and investigative examination can provide an accurate risk evaluation for every patient.

  19. Prenatal and Postnatal Survival of Fetal Tetralogy of Fallot: A Meta-analysis of Perinatal Outcomes and Associated Genetic Disorders.

    Science.gov (United States)

    Zhao, Yili; Abuhamad, Alfred; Fleenor, Jonathan; Guo, Yajun; Zhang, Wangshu; Cao, Danming; Zeng, Shi; Sinkovskaya, Elena; Zhou, Qichang

    2016-05-01

    The aim of this systematic review was to compare the postnatal outcomes, genetic testing results, and sonographic findings in 3 subtypes of tetralogy of Fallot. Thirty-six articles from the MEDLINE and EMBASE databases were selected for this review. The postnatal outcomes, karyotyping results, and sonographic findings of fetal tetralogy of Fallot with pulmonary stenosis, tetralogy of Fallot with pulmonary atresia, and tetralogy of Fallot with an absent pulmonary valve were collected and compared. The survival rates (termination of pregnancy was considered fetal death) for prenatally diagnosed tetralogy of Fallot with pulmonary atresia and tetralogy of Fallot with an absent pulmonary valve at the end of neonatal period were significantly lower than the rate for tetralogy of Fallot with pulmonary stenosis (P tetralogy of Fallot with pulmonary atresia was also lower at birth (P tetralogy of Fallot with pulmonary stenosis (Ptetralogy of Fallot with pulmonary atresia and tetralogy of Fallot with an absent pulmonary valve (P tetralogy of Fallot with pulmonary stenosis, a right aortic arch was more associated with tetralogy of Fallot with pulmonary atresia (32.6%; P tetralogy of Fallot with an absent pulmonary valve (87.5%; P tetralogy of Fallot. Documenting those details at diagnosis can help specialists better counsel their patients. © 2016 by the American Institute of Ultrasound in Medicine.

  20. Hot topics in tetralogy of Fallot.

    Science.gov (United States)

    Villafañe, Juan; Feinstein, Jeffrey A; Jenkins, Kathy J; Vincent, Robert N; Walsh, Edward P; Dubin, Anne M; Geva, Tal; Towbin, Jeffrey A; Cohen, Meryl S; Fraser, Charles; Dearani, Joseph; Rosenthal, David; Kaufman, Beth; Graham, Thomas P

    2013-12-10

    Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All

  1. Clinical challenges late after correction of tetralogy of Fallot

    NARCIS (Netherlands)

    Bokma, J.P.

    2018-01-01

    This thesis focused on several clinical challenges which may occur late after surgical correction of tetralogy of Fallot. Among several other topics, three important clinical challenges were studied: (1) timing of pulmonary valve replacement, (2) risk stratification, (3) prevention and treatment of

  2. Primary early correction of tetralogy of Fallot irrespective of age.

    Science.gov (United States)

    Kantorova, Andrea; Zbieranek, Kai; Sauer, Henning; Lilje, Christian; Haun, Christoph; Hraska, Viktor

    2008-04-01

    The policy of early repair of patients with tetralogy of Fallot, irrespective of age, as opposed to initial palliation with a shunt, remains controversial. The aim of our study was to analyze the midterm outcome of primary early correction of tetralogy of Fallot. Between 1996 and 2005, a total of 61 consecutive patients less than 6 months of age underwent primary correction of tetralogy of Fallot in two institutions. The median age at surgery was 3.3 months, and 27 patients (44%) were younger than 3 months of age, including 12 (20%) newborns. We analyzed the patients in 2 groups: those younger than 3 months of age, and those aged between 3 and 6 months. There was one early (1.6%), and one late death. Actuarial survival was 98.4%, 96.7%, 96.7% at 1, 5, and 10 years respectively, with a median follow up of 4.5 years. There was no difference in survival, bypass time, lengths of ventilation, and hospital stay between the groups. A transjunctional patch was placed significantly more often in the patients younger than 3 months (p = 0.039), with no adverse effect on survival and morbidity during the follow-up. Freedom from reoperation was 98.2%, 92.2%, and 83% at 1, 5, and 10 years respectively, with no difference between the groups. Elective primary repair of tetralogy of Fallot in asymptomatic patients is delayed beyond 3 months of age. In symptomatic patients, primary repair of tetralogy of Fallot is performed irrespective of age, weight and preoperative state. This approach is safe, and provides an excellent midterm outcome with acceptable morbidity and rates of reintervention. The long-term benefits of this approach must be established by careful follow-up, with particular emphasis on arrhythmias, right ventricular function, and exercise performance.

  3. Digital subtraction angiography in the diagnosis of Fallot's tetralogy

    International Nuclear Information System (INIS)

    Ivanitskij, A.V.; Tereshkin, Ya.A.; Sobolev, A.V.; Stolyar, V.L.; Slyunyastikov, M.A.

    1995-01-01

    The authors analyze the efficacy of digital subtraction angiography (DSA) in the diagnosis of Fallot's tetralogy (FT); this method helps simplity and cut down their scope of investigations but does not deteriorate their informative value. DSA findings in 120 patients with TF are analyzed. 5 refs.; 6 figs

  4. Intermittent′ restrictive ventricular septal defect in Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Sudhir S Shetkar

    2015-01-01

    Full Text Available Ventricular septal defect (VSD in Tetralogy of Fallot (TOF is usually large and non-restrictive with equalization of right and left ventricular pressures. Restrictive VSD in TOF is rare. We present an unusual case of TOF with restriction to VSD caused by accessory tricuspid valve tissue that varied with respiration.

  5. Quantitative Cardiac Assessment in Fetal Tetralogy of Fallot.

    Science.gov (United States)

    Jatavan, Phudit; Tongprasert, Fuanglada; Srisupundit, Kasemsri; Luewan, Suchaya; Traisrisilp, Kuntharee; Tongsong, Theera

    2016-07-01

    The purpose of this study was to quantitatively assess cardiac function and biometric parameters in fetuses with a diagnosis of tetralogy of Fallot and compare them to those in healthy fetuses. Two hundred healthy fetuses and 20 fetuses with a diagnosis of classic tetralogy of Fallot were quantitatively assessed for 16 cardiac parameters, including morphologic characteristics and functions. All recruited fetuses were in the second trimester with correct gestational ages. The measured values that were out of normal reference ranges were considered abnormal. Rates of abnormalities of these parameters were compared between the groups. The significant parameters were further analyzed for their sensitivity, specificity, and likelihood ratio. Of the 16 parameters, rates of abnormalities in 7 parameters, including right ventricular wall thickness, peak systolic velocities (PSVs) in the pulmonary artery and aorta, time to peak velocity, or acceleration time, in the pulmonary artery, aortic valve diameter, pulmonary valve diameter, and aortic-to-pulmonary valve diameter ratio, were significantly higher in fetuses with tetralogy of Fallot (P tetralogy of Fallot.

  6. Magnetic resonance angiography vs. angiography in tetralogy of Fallot.

    Science.gov (United States)

    Rao, Uppalapati Venkateswara; Vanajakshamma, Velam; Rajasekhar, Durgaprasad; Lakshmi, Amancharla Yadagiri; Reddy, Reddivari Niranjan

    2013-08-01

    : To determine whether gadolinium-enhanced three-dimensional magnetic resonance angiography can provide a noninvasive alternative to diagnostic catheterization for evaluation of pulmonary artery anatomy in tetralogy of Fallot. Thirty-five consecutive patients with tetralogy of Fallot, who attended the cardiology outpatient department between January 2008 and December 2009, were included in the study. There were 21 males and 14 females, with a mean age of 9 ± 4.15 years (range, 3-21 years). Thirty-two patients had tetralogy of Fallot with varying severities of valvular and infundibular stenosis. Three patients had tetralogy of Fallot with pulmonary atresia. All patients underwent both cardiac catheterization with X-ray angiography and 3-dimensional magnetic resonance angiography within one month. Measurements of right and left pulmonary arteries and aortopulmonary collaterals were equal by both methods. There was a good correlation between magnetic resonance angiography and catheterization measurements of branch pulmonary arteries. Gadolinium-enhanced three-dimensional magnetic resonance angiography can be used as a reliable noninvasive alternative to X-ray cineangiography for delineation of pulmonary arterial anatomy in sick infants and young children, obviating the need for catheterization.

  7. Nephrotic Syndrome in a Child Suffering from Tetralogy of Fallot: A Rare Association

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    Pépé Mfutu Ekulu

    2015-01-01

    Full Text Available Nephrotic syndrome is an uncommon complication of tetralogy of Fallot and has been rarely reported in pediatric population. We describe a 4-year-old female Congolese child who was referred for investigation for persistent dyspnea, edema, and cyanosis and nephrotic range proteinuria. Our patient presented with a tetralogy of Fallot and nephrotic syndrome. Conclusion. This case reminds us that children with tetralogy of Fallot may develop nephrotic proteinuria.

  8. MR tomography of tetralogy of Fallot. Kernspintomographie der Fallot-Tetralogie

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P.; Agness, M.; Holshouser, B.; Hinshaw, D. Jr. (Loma Linda Univ., CA (USA). Dept. of Radiology)

    1991-08-01

    We present four patients with Tetralogy of Fallot, imaged with magnetic resonance. MRI is useful in clearly depicting the dilated ascending aorta, the infundibular stenosis, ventricular septal defect, right ventricular hypertrophy and dilated bronchial arteries. The pulmonary outflow tract can be visualized better with MR imaging than with ultrasound. (orig.).

  9. Anestesi Pada Operasi Palatoplasty Dengan Penyulit Tetralogy of Fallot

    OpenAIRE

    Munandar, Arief

    2004-01-01

    Di laporkan penatalaksanaan anestesi pada pasien Labiopalatoschisis dengan penyulit Tetralogy of Fallot yang dilakukan operasi palatoplasti pada seorang anak wanita umur 4 tahun, berat badan 9 kg, dengan anestesi umum, status fisik ASA III. Premedikasi dengan midazolam 0,75 mg, sulfas atropin 0,25 mg, ketalar 5 mg. Induksi dengan ketalar 15 mg, fasilitas intubasi dengan Esmeron 6 mg. Pemeliharaan dengan fentanil 20 mg, halotan 0,5% dan oksigen 5 1/mnt. Durante operasi dilakukan monitoring pad...

  10. Reoperation after radical correction tetralogy of Fallot: results and prospects

    Directory of Open Access Journals (Sweden)

    M.F. Zinkovskyi

    2017-06-01

    Full Text Available The aim – to present experience, results and prospects of repeated interventions in the remote period after radical correction of tetralogy of Fallot. Materials and methods. Results of the repeated 65 surgeries in 62 patients after radical correction of tetralogy of Fallot with long-term follow-up for the period from 1981 to 2014 are presented. The interval between the radical tetralogy of Fallot correction and re-operation ranged from 1 month to 30 years (average – 8.54 ± 6.3 years. Results. Depending on the predominance of one or other complications and its causes we observed: 1 residual defects – 25 (40.4 % cases; right ventricular dysfunction – 23 (37.0 % cases; valve pathology – 5 (8.0 % cases; other rare complications – 9 (14.6 % cases. In total, 100 complications were identified and eliminated in 62 patients. The most frequent complications that required second surgical correction were: recanalization of ventricular septal defect (36.0 %, right ventricular aneurysm (19.0 %, tricuspid valve insufficiency (13.0 %, the residual obstruction of the outflow tract of the right ventricle (9.0 %, right ventricular dysfunction caused by pulmonary valve insufficiency (6.0 % and pulmonary trunk aneurysm (5.0 %. Conclusion. Hospital mortality after surgical correction of long-term complications was 8.0 %. All discharged patients with echocardiographic evaluation revealed a positive dynamics of volume and functional parameters of the right and left ventricles

  11. Coronary anomalies in Pakistani children with tetralogy of fallot

    International Nuclear Information System (INIS)

    Hussain, I.; Patel, N.

    2010-01-01

    To determine coronary artery anomalies in tetralogy of Fallot in Pakistani children as seen on angiography. Study Design: Case series. Place and Duration of Study: The National Institute of Cardiovascular Diseases, Karachi, Pakistan from July 2006 to July 2007. Methodology: Children under 15 years of age with echocardiographic diagnosis of tetralogy of Fallot were included in the study. All patients had pre-operative cardiac catheterization and angiography. Coronary arteries were studied with a nonselective aortic root angiogram in standard cranially tilted left anterior oblique view. The frequency of a normal and an anomalous coronary was determined. Results: Of the 83 patients, 78% were male and had a mean age of 8.9 years. Their mean weight was 14.3 kilograms. Seventy six (91.6%) had a normal coronary anatomy while 7 (8.4%) patients had anomalous coronary arteries. Among the patients with coronary anomalies, the commonest was a single origin coronary artery in 04 (57.14%) cases. Three (42.86%) had an anomalous origin of left anterior descending artery from the right coronary artery. Conclusion: Coronary artery anomalies were detected in 8.4% of the cases with tetralogy of Fallot. Single origin coronary artery anomaly was the commonest anomaly. (author)

  12. Penatalaksanaan Anestesi Pasien Tetralogy of Fallot pada Operasi Mouth Preparation

    Directory of Open Access Journals (Sweden)

    Arsy Felisita Dausawati

    2013-08-01

    Full Text Available Tetralogy of Fallot (TOF is a congenital heart disease consisting of a ventricular septal defect, overriding aorta, pulmonary stenosis and right ventricular hypertrophy. Tetralogy of Fallot, including the type of cyanotic congenital heart defects. A boy of 9 years came for treatment and tooth extraction as preparation for the surgical correction of TOF at the Dr. Hasan Sadikin Hospital-Bandung whitin February 2012. Patients with a history of blue as a baby, and on physical examination found the child looking cyanosis, SpO2 70–75%, systolic murmur and finger clubbing. Abnormalities on echocardiography obtained TOF. Anesthetic management of these patients was performed using ketamine and vecuronium for induction and maintenance with O2, N2O and halothane. Cyanotic attacks can occur preoperative, intraoperative and postoperative, who treated by increasing systemic vascular resistance (SVR compared to pulmonary vascular resistance (PVR. In conclusions, perioperatif mangement principal for non cardiac surgery on tetralogy of fallot (TOF is to prevent shunting from right to left by keep the SVR from decline, increase on PVR, and reduce infundibular spasme.

  13. A Rare Triad of Giant Occipital Encephalocele with Lipomyelomeningocele, Tetralogy of Fallot, and Situs Inversus

    Science.gov (United States)

    Franco, Arie; Jo, Stephanie Y; Mehta, Amar S; Pandya, Dave J; Yang, Carina W

    2016-01-01

    Giant encephalocele is an uncommon congenital anomaly with very few published reports available in the English literature. Tetralogy of Fallot associated with situs inversus is also infrequently reported. To our knowledge there are no published reports of an association between giant encephalocele and Tetralogy of Fallot. The additional finding of situs inversus results in a rare pathologic triad, not heretofore described. PMID:27200165

  14. A Rare Triad of Giant Occipital Encephalocele with Lipomyelomeningocele, Tetralogy of Fallot, and Situs Inversus.

    Science.gov (United States)

    Franco, Arie; Jo, Stephanie Y; Mehta, Amar S; Pandya, Dave J; Yang, Carina W

    2016-03-01

    Giant encephalocele is an uncommon congenital anomaly with very few published reports available in the English literature. Tetralogy of Fallot associated with situs inversus is also infrequently reported. To our knowledge there are no published reports of an association between giant encephalocele and Tetralogy of Fallot. The additional finding of situs inversus results in a rare pathologic triad, not heretofore described.

  15. Role of the 3-Vessel and Trachea View in Antenatal Detection of Tetralogy of Fallot.

    Science.gov (United States)

    Palatnik, Anna; Grobman, William A; Cohen, Leeber S; Dungan, Jeffrey S; Gotteiner, Nina L

    2016-08-01

    Prenatal diagnosis of tetralogy of Fallot remains less frequent compared to other major congenital heart defects. In this study, we examined how often the 3-vessel and trachea view was abnormal in a large series of prenatally diagnosed cases of tetralogy of Fallot. In addition, we compared its sensitivity to that of the traditional outflow tract views for detection of tetralogy of Fallot. We found that both views were abnormal in all fetuses with tetralogy of Fallot, showing reversed aortic-to-pulmonary valve and aortic arch isthmus-to-ductus arteriosus ratios in the outflow tract and 3-vessel and trachea views, respectively. However, as a single measured marker, the enlarged aortic arch isthmus on the 3-vessel and trachea view appears to be the most sensitive for tetralogy of Fallot.

  16. Tetralogy of Fallot in a 6-year-old albino ferret (Mustela putorius furo)

    Science.gov (United States)

    Laniesse, Delphine; Hébert, Julie; Larrat, Sylvain; Hélie, Pierre; Pouleur-Larrat, Bénédicte; Belanger, Marie C.

    2014-01-01

    Tetralogy of Fallot associated with bidirectional shunting across a large ventricular septal defect, was found in a 6-year-old ferret. The prognosis associated with tetralogy of Fallot is usually poor. This case is interesting given the advanced age of the ferret. The bidirectional shunting, responsible for an acyanotic disease, may explain the unexpected prolonged survival in this ferret. PMID:24790231

  17. Survival into seventh decade after a potts palliation for tetralogy of Fallot

    NARCIS (Netherlands)

    Oosterhof, Thomas; Jacobs, Monique; Cramer, Maarten-Jan; Mulder, Barbara J. M.

    2007-01-01

    In this case report we present a 62-year-old patient with unrepaired tetralogy of Fallot who underwent a Potts shunt for palliation. Survival into late adulthood of patients with unoperated tetralogy of Fallot is rare. This patient is currently in New York Heart Association (NYHA) class II. A Potts

  18. Survival to the age of 87 years in a woman with unoperated tetralogy of Fallot

    Science.gov (United States)

    Subhawong, Ty K.; Teytelboym, Oleg

    2009-01-01

    Tetralogy of Fallot is the most common cyanotic congenital heart defect, affecting approximately 2700 infants per year born in the United States. The natural history of the tetralogy reflects the adverse physiologic consequences of the underlying structural abnormalities, with only approximately 3% of uncorrected patients surviving past age 40. In this case report, we report an 87-year-old woman with unoperated tetralogy of Fallot, who we believe to be the oldest and only second octogenarian described in the literature. PMID:22470677

  19. An apology for primary repair of tetralogy of Fallot.

    Science.gov (United States)

    Van Arsdell, Glen; Yun, Tae-Jin

    2005-01-01

    The first repair of tetralogy of Fallot (TOF) was 50 years ago, so it would seem that the details for optimal management strategies would be clear. Timing of repair and operative repair strategy for TOF are still a source of debate. Varying institutions have published excellent outcomes with a primary repair strategy or a selective staged repair strategy. In this article, the current and historic strategy for repair of TOF at the Hospital for Sick Children in Toronto is delineated along with associated outcomes. Data from our institution indicate a clear survival advantage for primary repair of TOF.

  20. Cardiovascular disease in late survivors of tetralogy of fallot: a tertiary care center experience.

    Science.gov (United States)

    Bradley, Elisa; Parker, Jeff; Novak, Eric; Ludbrook, Philip; Billadello, Joseph; Cedars, Ari

    2013-01-01

    Patients with tetralogy of Fallot can survive to late adulthood; however, there are few data on cardiovascular outcomes in this population. We conducted a single-center retrospective analysis of cardiovascular outcomes and risk factors in 208 patients with tetralogy of Fallot to better evaluate the burden of cardiovascular disease in this group. Descriptive statistics were used to determine the prevalence of relevant cardiovascular risk factors and outcomes, including a composite analysis of cardiovascular disease. Rates and mean values from the American Heart Association 2011 Heart Disease and Stroke Statistics Update were used as population estimates for comparison. In tetralogy of Fallot patients, cardiovascular disease prevalence was not different from that found in the general population (40% vs. 36%, P=0.3). However, there was significantly more cardiovascular disease in tetralogy of Fallot men aged 20 to 39 years (30% vs. 14%, P tetralogy of Fallot men aged 40 to 59 years (63% vs. 29%, P tetralogy of Fallot men aged 20 to 59 years. These data support the need to routinely screen young adult male survivors of tetralogy of Fallot for asymptomatic heart failure. Further studies are needed to determine the incidence, severity, and long-term effects of cardiovascular disease in the adult congenital heart disease population.

  1. Association of Wolfram syndrome with Fallot tetralogy in a girl.

    Science.gov (United States)

    Korkmaz, Hüseyin A; Demir, Korcan; Hazan, Filiz; Yıldız, Melek; Elmas, Özlem N; Özkan, Behzat

    2016-06-01

    Wolfram syndrome (DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a rare neurodegenerative disorder. Mutations of the WFS1 (wolframin) on chromosome 4 are responsible for the clinical manifestations in majority of patients with Wolfram syndrome. Wolfram syndrome is also accompanied by neurologic and psychiatric disorders, urodynamic abnormalities, restricted joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotrophic hypogonadism in males and diabetic microvascular disorders. There are very limited data in the literature regarding cardiac malformations associated in children with Wolfram syndrome. A 5-year-old girl with Wolfram syndrome and tetralogy of Fallot is presented herein. Sociedad Argentina de Pediatría.

  2. Observations on obesity patterns in tetralogy of Fallot patients from childhood to adulthood.

    Science.gov (United States)

    Briston, David A; Sabanayagam, Aarthi; Zaidi, Ali N

    2017-07-01

    Obesity is increasingly prevalent, and abnormal body mass index is a risk factor for cardiovascular disease. There are limited data published regarding body mass index and CHD. We tested the hypothesis that body mass index and obesity prevalence are increasing in patients with tetralogy of Fallot over time by analysing time since surgery, age, height, weight, and body mass index among tetralogy of Fallot patients and demographic data from age-matched controls. NYHA class and left ventricular ejection fraction were analysed in adults. Body mass index was categorised into normal, overweight, and obese in this single-centre, retrospective chart review. Data were collected from 137 tetralogy of Fallot patients (71 men:66 women), of whom 40 had body mass index >25 kg/m2. Tetralogy of Fallot patients aged tetralogy of Fallot patients, the mean body mass index was 26.5 but not statistically significantly different from the control cohort. Obese adult patients had significantly higher average NYHA class compared with those of normal weight (p=0.03), but no differences in left ventricular ejection fraction by echocardiography (p=0.55) or cardiac MRI (p=0.26) were noted. Lower body mass index was observed initially in tetralogy of Fallot patients, but by late adolescence no significant difference was observed. As adults, tetralogy of Fallot patients with higher body mass index had increased NYHA class but similar left ventricular ejection fraction.

  3. Preoperative Pulmonary Valvuloplasty in Tetralogy of Fallot with Right-To-Left Shunt

    Directory of Open Access Journals (Sweden)

    Baris Bugan

    2014-12-01

    Full Text Available Tetralogy of Fallot is the most common cyanotic congenital heart disease and characterized by right ventricular outflow tract obstruction, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. Right ventricular outflow tract obstruction and ventricular septal defect are the major clinical components of the syndrome. Although most have undergone a corrective operation, an important minority of patients with tetralogy of Fallot have had a preoperative palliative procedure. Herein we reported a succesful palliative percutaneous balloon valvuloplasty of pulmonary stenosis at an 19-year-old female patient with tetralogy of Fallot who was considered as inoperable for corrective surgery due to right -to-left shunt.

  4. Outcomes of Tetralogy of Fallot repair performed after three years of age

    Directory of Open Access Journals (Sweden)

    Ni Putu Veny Kartika Yantie

    2016-07-01

    Conclusion Tetralogy of Fallot repair after 3 years of age appears to not increase ICU LoS or is associated with lower TAPSE, but it is associated with longer QRS duration. [Paediatr Indones. 2016;56:176-83.].

  5. Surgical treatment of tetralogy of Fallot with absent pulmonary valve syndrome.

    Science.gov (United States)

    Tanaka, Yuki; Miyamoto, Takashi; Naito, Yuji; Yoshitake, Shuichi

    2016-06-01

    The patient was a 3-month-old girl weighting 3.6 kg, diagnosed with tetralogy of Fallot and absent pulmonary valve syndrome. We surgically repaired the tetralogy of Fallot by patch closure of the ventricular septal defect, right ventricular outflow tract reconstruction using an expanded polytetrafluoroethylene monocusp patch with a bulging sinus, and removal of the bronchial obstruction by anterior translocation of the pulmonary artery using the Lecompte maneuver. © The Author(s) 2015.

  6. Tetralogy of Fallot in men: quality of life, family, education, and employment.

    Science.gov (United States)

    Bygstad, Elisabeth; Pedersen, Lia C V M; Pedersen, Thais A L; Hjortdal, Vibeke E

    2012-08-01

    Little is known about the quality of life, health, family, education, and employment status among adult men with repaired tetralogy of Fallot. A total of 68 men who underwent repair of tetralogy of Fallot between 1971 and 1991 were studied. Fifty-three patients answered the SF-36 health survey and additional questions regarding offspring, education, and employment status. The men with repaired tetralogy of Fallot were compared with 32 healthy men and 40 women who also underwent repair of tetralogy of Fallot in the same period. The patients scored lower than healthy men in the SF-36 categories physical functioning, general health, and physical component summary. There were no statistically significant differences in the scores from male and female patients except a lower score in bodily pain among women. Educational level for men operated for tetralogy of Fallot was similar to the general male population, whereas fewer were employed and more were retired, undergoing rehabilitation or receiving social benefits. The reproduction rate was lower compared with the general population (0.65 versus 1.02 children per man) but relatively higher than the rate among women with tetralogy of Fallot (0.88 versus 1.84 children per woman). The risk of having a child with congenital heart disease was 8.3%. Men operated for tetralogy of Fallot have good quality of life and educational status. They start a family, although their reproduction rate is two-thirds that of the general population. The risk of having a child with congenital heart disease is higher compared with the background population. The overall quality of life is similar for men and women operated for tetralogy of Fallot.

  7. Whole exome sequencing identifies novel mutation in eight Chinese children with isolated tetralogy of Fallot.

    Science.gov (United States)

    Liu, Lin; Wang, Hong-Dan; Cui, Cun-Ying; Qin, Yun-Yun; Fan, Tai-Bing; Peng, Bang-Tian; Zhang, Lian-Zhong; Wang, Cheng-Zeng

    2017-12-05

    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. Whole exome sequencing was performed among eight small families with Tetralogy of Fallot. Differential single nucleotide polymorphisms and small InDels were found by alignment within families and between families and then were verified by Sanger sequencing. Tetralogy of Fallot-related genes were determined by analysis using Gene Ontology /pathway, Online Mendelian Inheritance in Man, PubMed and other databases. A total of sixteen differential single nucleotide polymorphisms loci and eight differential small InDels were discovered. The sixteen differential single nucleotide polymorphisms loci were located on Chr 1, 2, 4, 5, 11, 12, 15, 22 and X. Among the sixteen single nucleotide polymorphisms loci, six has not been reported. The eight differential small InDels were located on Chr 2, 4, 9, 12, 17, 19 and X, whereas of the eight differential small InDels, two has not been reported. Analysis using Gene Ontology /pathway, Online Mendelian Inheritance in Man, PubMed and other databases revealed that PEX5 , NACA , ATXN2 , CELA1 , PCDHB4 and CTBP1 were associated with Tetralogy of Fallot. Our findings identify PEX5 , NACA , ATXN2 , CELA1 , PCDHB4 and CTBP1 mutations as underlying genetic causes of isolated tetralogy of Fallot.

  8. [Bentall operation after previous repair of tetralogy of Fallot].

    Science.gov (United States)

    Ballore, Luca; Tramontin, Corrado; Manca, Paolo; Tocco, Stefania; Lixi, Giovanni; Matta, Gildo; Cirio, Emiliano Maria; Martelli, Valentino

    2007-04-01

    Pathologies that involve the ascending aorta are described in case reports of patients after complete repair of tetralogy of Fallot. Here, we present a case report. Preoperative tests showed an aortic ratio of > 1.5. The patient was operated on replacement of the ascending aorta according to the Bentall technique. At present, the guidelines for replacement of the ascending aorta recommend an aortic ratio of > 1.5 and in case of Marfan syndrome of > 1.3. In these patient subsets, cystic medial necrosis and an increased risk of aortic dissection have been described. Therefore, as for Marfan syndrome, why an aortic ratio of >1.3 should not be considered a proper surgical indication also for these patients?

  9. Tetralogia de Fallot em cão Tetralogy of Fallot in dogs

    Directory of Open Access Journals (Sweden)

    Rodrigo Ramos de Freitas

    2003-12-01

    Full Text Available A Tetralogia de Fallot é uma moléstia cardíaca congênita caracterizada pela presença de hipoxemia provocada principalmente pela estenose pulmonar e defeito septal interventricular, exteriorizada pelo animal com o quadro de cianose. Atualmente, tem-se vários métodos de diagnóstico de grande precisão, na determinação de tal afecção. Existem vários tratamentos da Tetralogia de Fallot, desde medicamentoso até a sua correção cirúrgica definitiva, com o reparo dos defeitos cardíacos. Aquele que vem sendo mais utilizado é o tratamento cirúrgico paliativo, com a criação de um desvio sistêmico-pulmonar, utilizando-se a técnica de Blalock-Taussig, que cria uma comunicação entre a aorta e a artéria pulmonar com a artéria subclávia esquerda.Tetralogy of Fallot is a congenital cardiac disease characterized by the presence of hypoxemia because of the pulmonary stenosis and the ventricular septal defect. The symptom in the animal is cianosis. Actually, there are many diagnostic methods of this affection. There are many treatments for Tetralogy of Fallot, such as the medicamentosus treatment and the definitive surgical correction, reparing the cardiac defects. The most used is the palliative surgical treatment, creating systemic-pulmonar shunt. The most famous surgical method is the Blalock-Taussig surgery, when the surgeon creates a communication between the aorta and pulmonary artery, using the left subclavian artery.

  10. Repair of tetralogy of Fallot associated with atrioventricular septal defect.

    Science.gov (United States)

    Tláskal, T; Hucín, B; Kostelka, M; Chaloupecký, V; Marek, J; Tax, P; Janouàek, J; Kuèera, V; Hruda, J; Reich, O; Skovránek, J

    1998-01-01

    Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiography alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restoration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2-12.5 years after surgery (median 4.9 years, mean 5.9+/-3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

  11. The effect of transesophageal echocardiography in the surgical treatment of tetralogy of Fallot.

    Science.gov (United States)

    Wang, L-C; Li, S-K; Zhu, F-T; Bian, T; Wang, H-Y

    2018-04-01

    To investigate the effect of transesophageal echocardiography (TEE) in the surgical treatment of tetralogy of Fallot. 98 patients with tetralogy of Fallot received and cured by Zhengzhou Cardiovascular Hospital (Zhengzhou No. 7 People's Hospital) from January 2015 to January 2017 were selected as the study objects. All patients were examined by TEE before surgery, and the pulmonary artery index (PAI) and pulmonary vein index (PVI) were measured, so as to analyze the effect of TEE in the surgical treatment of tetralogy of Fallot. Among the 98 patients, 12 patients were diagnosed with intensive care unit (ICU) retention, 23 patients were diagnosed with respirator assisted respiration extension, 8 patients were diagnosed with low cardiac output syndrome, and 10 patients were diagnosed with respiratory tract infection, which indicated that TEE could diagnose conditions after radical operation of tetralogy of Fallot. The calculation results showed that the PAI was (171.37±58.39) mm2/m2 and the PVI was (282.46±54.37) mm2/m2. The Pearson correlation analysis showed that the correlation between them was good (r=0.821, ptetralogy of Fallot. TEE can predict the occurrence of respirator assisted respiration extension, ICU retention and low cardiac output syndrome of patients after radical surgery by evaluating the PAI and PVI of patients with tetralogy of Fallot.

  12. Clinical value of Tei index in pediatric patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Song, Bing; Qi, Quan; Liu, Ruisheng; Xing, Wang; Tang, Hanbo; Li, Yuanmin

    2015-01-01

    Tetralogy of Fallot is a congenital heart disease characterized by underdevelopment of the right ventricular infundibulum. Present study aimed to explore the clinic value of Tei index in assessing right ventricular function of pediatric patients with repaired Tetralogy of Fallot. A total of 45 pediatric patients with repaired Tetralogy of Fallot were recruited and classified into: group A (Tei index 0.7; n=13, aging 4-14 years). The right ventricular Tei index value was related to the clinical characteristics of Tetralogy of Fallot repair patients. Right ventricular Tei index was positively correlated with ventilation time, drainage volumes, and negatively with drug assistance and Intensive Care Unit (ICU) stay, although time for drug assistance and ICU stay were not statically different between group B and group C. There was no significant difference in left ventricular ejection fraction. Tei index is a sensitive indicator of right ventricular dysfunction, and has important clinical value to better our understanding of right ventricular function after tetralogy of Fallot repair.

  13. Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging.

    Science.gov (United States)

    Bellinger, David C; Rivkin, Michael J; DeMaso, David; Robertson, Richard L; Stopp, Christian; Dunbar-Masterson, Carolyn; Wypij, David; Newburger, Jane W

    2015-02-01

    Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot. In this single-centre cross-sectional study, we enrolled 91 adolescents (13-16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging. Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%). Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.

  14. Alteration of pulmonary blood flow in tetralogy of Fallot

    International Nuclear Information System (INIS)

    Hashimoto, Kazuhiro; Matsui, Michihiko; Kurosawa, Hiromi; Arai, Tatsuta; Nakamura, Yuzuru.

    1992-01-01

    The pulmonary blood distribution was examined in 17 patients with tetralogy of Fallot (TOF) pre and postoperatively with macroaggregates of 99m TC-labeled human serum albumin. Most of the patients with TOF demonstrated an abnormal preoperative distribution pattern. The abnormalities included not only an unbalanced distribution between the right and left lungs but also a maldistribution of peripheral vessels in each lung. The right/left lung counts ratio and pulmonary peripheral index (calculated in order to express the severity of peripheral maldistribution) correlated neither to the diameter nor the cross-sectional area of either right or left pulmonary arteries which were measured angiographically. Postoperatively, the pulmonary blood was shunted toward the developed side of the lung which further contributed to maldistribution of blood flow and unbalanced pulmonary growth. Since the patients with an unbalanced pulmonary blood distribution demonstrated a higher right ventricular pressure one year after the operation, a palliative operation facilitating the growth of the underdeveloped side of the lung might be considered as an effective procedure to precede intracardiac repair. (author)

  15. Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

    Science.gov (United States)

    Qureshi, Muhammad Yasir; Burkhart, Harold M; Julsrud, Paul; Cetta, Frank

    2014-12-01

    Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

  16. Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot - predictors of success and outcome.

    Science.gov (United States)

    Ezzat, Vivienne A; Ryan, Matthew J; O'Leary, Justin; Ariti, Cono; Deanfield, John; Pandya, Bejal; Cullen, Shay; Walker, Fiona; Khan, Fakhar; Abrams, Dominic J; Lambiase, Pier D; Lowe, Martin D

    2017-03-01

    Adults with tetralogy of Fallot experience atrial tachyarrhythmias; however, there are a few data on the outcomes of radiofrequency ablation. We examined the characteristics, outcome, and predictors of recurrence of atrial tachyarrhythmias after radiofrequency ablation in tetralogy of Fallot patients. Methods/results Retrospective data were collected from 2004 to 2013. In total, 56 ablations were performed on 37 patients. We identified two matched controls per case: patients with tetralogy of Fallot but no radiofrequency ablation and not known to have atrial tachyarrhythmias. Acute success was 98%. Left atrial arrhythmias increased in frequency over time. The mean follow-up was 41 months; 78% were arrhythmia-free. Number of cardiac surgeries, age, and presence of atrial fibrillation were predictors of recurrence. Lone cavo-tricuspid isthmus-dependent flutter reduced the likelihood of atrial fibrillation. Right and left atria in patients with tetralogy of Fallot were larger in ablated cases than controls. NYHA class was worse in cases and improved after ablation; baseline status predicted death. Of matched non-ablated controls, a number of them had atrial fibrillation. These patients were excluded from the case-control study but analysed separately. Most of them had died during follow-up, whereas of the matched ablated cases all were alive and the majority in sinus rhythm. Patients with tetralogy of Fallot and atrial tachyarrhythmias have more dilated atria than those without atrial tachyarrhythmias. Radiofrequency ablation improves functional status. Left atrial ablation is more commonly required with repeat procedures. There is a high prevalence of atrial tachyarrhythmias, particularly atrial fibrillation, in patients with tetralogy of Fallot; early radiofrequency ablation may have a protective effect against this.

  17. The impact of not having a ductus arteriosus on clinical outcomes in foetuses diagnosed with tetralogy of Fallot.

    Science.gov (United States)

    Stern, Seth J; Wadekar, Neelum; Mertens, Luc; Manlhiot, Cedric; McCrindle, Brian W; Jaeggi, Edgar T; Nield, Lynne E

    2015-04-01

    Foetuses with simple tetralogy of Fallot almost universally have a patent ductus arteriosus. Two recently identified cases had an absent patent ductus arteriosus, requiring emergent intervention at birth. The objective of this study was to determine whether foetuses diagnosed with tetralogy of Fallot and no patent ductus arteriosus have poorer outcomes compared with those with tetralogy of Fallot+patent ductus arteriosus. All foetal cases of tetralogy of Fallot between January, 2000 and 2012 were retrospectively identified from The Hospital for Sick Children (Toronto, Canada) database. Cases - tetralogy of Fallot+no patent ductus arteriosus confirmed on postnatal echo - and controls - tetralogy of Fallot+patent ductus arteriosus, matched for gestational age - were identified from prenatal records, and both clinical and echocardiographic data were reviewed. Optimal outcome was defined as valve-sparing repair with no residual lesions. Student's t-tests and Fisher's exact χ2 were used to compare groups. n=115 foetuses were diagnosed with tetralogy of Fallot: 11 (9%) had no patent ductus arteriosus, and were matched to 22 controls - mean gestational age at diagnosis 23.2±4.2 weeks, 23.4±6.6 weeks, respectively. Cases had a higher proportion of right aortic arches (64% versus 14%, ptetralogy of Fallot.

  18. Update article for continuous education Tetralogy of Fallot

    International Nuclear Information System (INIS)

    Gonzalez L, Jaime A; Cadavid, Ana M; Aguilera, Damaris; Cazzaniga, Mario

    2008-01-01

    The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in the general population with a general incidence of 0.1/1000 live births. The morphologic diagnostic axis is the left anterior displacement of the infundibular ventricular septum. Towards the right ventricle way out- during the embryogenic period that causes aortic override, ventricular septal defect, subpulmonary stenosis and right ventricular hypertrophy. Without surgical intervention, survival is 66% in 1 year and only 10% to 15% in > 20 years. Clinical presentation is variable and depends on the grade of pulmonary stenosis; when it is already significant in the neonatal period or in infants < 3 to 6 months, hypoxemic crisis that may require urgent medical or surgical intervention may appear. Complete surgical correction of the malformation offers good survival outcomes during decades; although with the first techniques -Trans-annular patch and closure of the interventricular defect- there appear long term problems that may generate an additional morbimortality risk. Severe pulmonary insufficiency, presence of right ventricular dilation and development of potentially fatal arrhythmias are problems that become important and must be recognized in order to assess an early re-intervention and to repair residual defects inductors of arrhythmia. The new surgical techniques try to preserve as much as possible ventricular-pulmonary joint and the valve itself, if anatomy allows it. That will without doubt benefit the patient because the residual problems described have lesser clinical significance

  19. One-stage repair of tetralogy of Fallot with coarctation of the aorta.

    Science.gov (United States)

    Makhija, Zeena; Radhakrishnan, Sitaraman; Goel, Apporva; Sharma, Rajesh

    2014-09-01

    We describe the rare case of a 10-month-old girl who had coarctation of the aorta in association with tetralogy of Fallot. The surgical management and postoperative course is described. This case highlights the rare association of coarctation with tetralogy of Fallot, with a large intracardiac right-to-left shunt. Although an exception to the rule, it challenges the reduced fetal blood flow theory and the smooth muscle cell migration theory as embryological explanations for the development of coarctation. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  20. MRI for therapy control in patients with tetralogy of Fallot

    International Nuclear Information System (INIS)

    Theisen, D.; Reiser, M.F.; Dalla Pozza, R.D.; Malec, E.

    2011-01-01

    With prevalences ranging from 0.26 to 0.8 permille of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation. The timing of pulmonary valve replacement is crucial as right ventricular (RV) volumes have only proven to normalize when preoperative end-diastolic volumes are 2 and end-systolic volumes are 2 . After surgical repair up to 15% of patients have residual or recurrent pulmonary artery stenosis. Distal pulmonary branch stenosis can aggravate PR and lead to right heart failure due to combined pressure and volume overload. Therefore, it has to be diagnosed in time and treated by angioplasty with or without stenting. (orig.) [de

  1. Echocardiographic assessment of the aortic root dilatation in adult patients after tetralogy of Fallot repair.

    Science.gov (United States)

    Cruz, Cristina; Pinho, Teresa; Lebreiro, Ana; Silva Cardoso, José; Maciel, Maria Júlia

    2013-06-01

    Transthoracic echocardiography is an important tool after tetralogy of Fallot repair, of which aortic root dilatation is a recognized complication. In this study we aimed to assess its prevalence and potential predictors. We consecutively assessed adult patients by transthoracic echocardiography after tetralogy of Fallot repair, and divided them into two groups based on the maximum internal aortic diameter at the sinuses of Valsalva in parasternal long-axis view: group 1 with aortic root dilatation (≥38 mm) and group 2 without dilatation (de Cardiologia. Published by Elsevier España. All rights reserved.

  2. Increase in Cardiac Troponin I in a Lamb with Tetralogy of Fallot

    Science.gov (United States)

    NEUWALD, Elisa Barp; SOARES, Frederico Aécio Carvalho; DREYER, Cristina Terres; CARNESELLA, Samuel; WOUTERS, Angelica Terezinha Barth; GONZÁLEZ, Félix Hilario Diaz; DRIEMEIER, David

    2013-01-01

    ABSTRACT This study describes a case of tetralogy of Fallot in a lamb showing failure to thrive and signs of respiratory distress. Physical examination, electrocardiography, thoracic radiographies, echocardiography and cardiac troponin I evaluation were performed. The value of cardiac troponin I was compared with the values of 10 healthy lambs of the same age and breed, and the affected animal demonstrated an increase in cardiac troponin I. Due to the poor prognosis, euthanasia was indicated, and necropsy confirmed the diagnosis. This is the first report of an increase in cardiac troponin I in a lamb with tetralogy of Fallot. PMID:23685750

  3. Surgery for an adult with tetralogy of Fallot and acquired heart disease.

    Science.gov (United States)

    Hamamoto, Masaki; Morifuji, Kiyohiko

    2014-06-01

    We experienced the rare case of an elderly woman with uncorrected tetralogy of Fallot. She also had significant mitral and tricuspid regurgitation with deteriorated ventricular function and ischemic coronary artery disease. We performed a radical repair of the tetralogy of Fallot, valvular operations for the mitral and tricuspid regurgitation, and coronary artery bypass grafting. Although mechanical circulatory support was required postoperatively, she recovered well to New York Heart Association functional class II. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  4. Clinical course of tricuspid regurgitation in repaired tetralogy of Fallot.

    Science.gov (United States)

    Woudstra, O I; Bokma, J P; Winter, M M; Kiès, P; Jongbloed, M R M; Vliegen, H W; Groenink, M; Meijboom, F J; Mulder, B J M; Bouma, B J

    2017-09-15

    Little is known on the clinical course of tricuspid regurgitation (TR) in patients with repaired tetralogy of Fallot (rTOF) and which patients are at particular risk. This study aims to determine TR course, characteristics associated with TR progression, and the prognostic relevance of TR in rTOF patients. In this dualcenter cohort study, rTOF patients from a prospective national registry with ≥1 cardiac magnetic resonance imaging study and ≥2 echocardiograms available were included. Clinical and imaging data were collected. Cox hazards regression analysis was used to assess patient characteristics associated with progression to severe TR and whether severe TR was associated with the combined clinical endpoint of tachyarrhythmia, heart failure, and death, as time-dependent factor. A total of 216 patients were included (57% men, age 34±12years); 11 patients (5%) had severe TR at baseline. During 7.6±3.5years of follow-up, progression to severe TR occurred in 15 patients (7%). NYHA class ≥2 (HR 5.38, 95%-C.I. 1.91-15.16, p=0.001) and moderate baseline TR (HR 13.10, 95%-C.I. 2.95-58.21, p=0.001) were independently associated with progression to severe TR. Adverse events occurred in 47 patients (22%). The occurrence of severe TR was independently associated with adverse events (HR 3.48, 95%-C.I. 1.68-7.21, p=0.001). In this study, severe TR was present in 12% of adult rTOF patients during 7.6years, and progression to severe TR was most likely in symptomatic patients with moderate baseline TR. In these patients, close surveillance is warranted, because the occurrence of severe TR was associated with worse prognosis. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Ventricular emptying performance in patients with tetralogy of Fallot

    International Nuclear Information System (INIS)

    Takeda, Kan; Maeda, Hisato; Nakagawa, Tsuyoshi; Ito, Tsunao; Yamaguchi, Nobuo; Matsuda, Akira

    1989-01-01

    Comparison of emptying patterns between left and right ventricles (LV, RV) was performed with Fourier analysis of gated blood-pool data in patients with tetralogy of Fallot (TF). Using global time-activity curves, the phase and amplitude at the first-harmonic component of Fourier series were calculated and emptying patterns of both ventricles were evaluated by phase difference {D(phase)=RV phase minus LV phase} and RV/LV amplitude ratio {R(amp)}. In 20 patients with normal cardiac function, D(phase) was minimal (mean 2.0±6.6 degrees) and R(amp) was less than 1.0 (mean 0.60±0.19). In 11 patients with TF, D(phase) was significantly larger than normal, with a mean value of 24.3±10.0 degrees (p<0.01) and became greater in a reversed proportion to the ratio of the pulmonary-to-systemic blood flow (p<0.01). In all but one cases with TF, R(amp) was greater than 1.0 with a mean value of 1.4±0.4, significantly larger than normal (p<0.001). Furthermore, using time-activity curves approximated by terms up to the 3rd-harmonic component, the temporal difference in emptying patterns between both ventricles was investigated. In TF cases, the time from end-diastole to minimum count (T2) was significantly larger in RV than in LV (p<0.001). The elongated T2 interval of RV seemed to play an important role in producing RV phase lag. Thus, this non-invasive method is valuable for pathophysiologic investigation of patients with TF and can be of help in estimating the severity of their disease. (author)

  6. Right ventricular outflow tract strategies for repair of tetralogy of Fallot: effect of monocusp valve reconstruction.

    NARCIS (Netherlands)

    Sasson, L.; Houri, S.; Raucher Sternfeld, A.; Cohen, I.; Lenczner, O.; Bove, E.L.; Kapusta, L.; Tamir, A.

    2013-01-01

    OBJECTIVES: The absence of a pulmonary valve (PV) after tetralogy of Fallot (TOF) repair has been shown to impact postoperative right ventricular (RV) function. The purposes of this study were to (i) compare early outcomes after PV-sparing vs transannular patching (TAP) with monocusp valve

  7. Functionally significant, rare transcription factor variants in tetralogy of Fallot.

    Directory of Open Access Journals (Sweden)

    Ana Töpf

    Full Text Available Rare variants in certain transcription factors involved in cardiac development cause Mendelian forms of congenital heart disease. The purpose of this study was to systematically assess the frequency of rare transcription factor variants in sporadic patients with the cardiac outflow tract malformation tetralogy of Fallot (TOF.We sequenced the coding, 5'UTR, and 3'UTR regions of twelve transcription factor genes implicated in cardiac outflow tract development (NKX2.5, GATA4, ISL1, TBX20, MEF2C, BOP/SMYD1, HAND2, FOXC1, FOXC2, FOXH, FOXA2 and TBX1 in 93 non-syndromic, non-Mendelian TOF cases. We also analysed Illumina Human 660W-Quad SNP Array data for copy number variants in these genes; none were detected. Four of the rare variants detected have previously been shown to affect transactivation in in vitro reporter assays: FOXC1 p.P297S, FOXC2 p.Q444R, FOXH1 p.S113T and TBX1 p.P43_G61del PPPPRYDPCAAAAPGAPGP. Two further rare variants, HAND2 p.A25_A26insAA and FOXC1 p.G378_G380delGGG, A488_491delAAAA, affected transactivation in in vitro reporter assays. Each of these six functionally significant variants was present in a single patient in the heterozygous state; each of the four for which parental samples were available were maternally inherited. Thus in the 93 TOF cases we identified six functionally significant mutations in the secondary heart field transcriptional network.This study indicates that rare genetic variants in the secondary heart field transcriptional network with functional effects on protein function occur in 3-13% of patients with TOF. This is the first report of a functionally significant HAND2 mutation in a patient with congenital heart disease.

  8. Right ventricular global longitudinal strain in repaired tetralogy of Fallot.

    Science.gov (United States)

    Toro, Kamill Del; Soriano, Brian D; Buddhe, Sujatha

    2016-10-01

    Echocardiogram has limitations in effectively assessing right ventricular (RV) function in children post tetralogy of Fallot (TOF) repair. We evaluated the utility of speckle tracking echocardiography (STE)-based RV global longitudinal strain (GLS) for the assessment of RV systolic function. All patients with repaired TOF who had both echocardiograms and cardiac MRI (CMR) within a 6-month interval were included. RV volumes and ejection fraction (EF) were obtained by CMRs. Traditional echocardiographic function parameters and RV GLS were compared to CMR-derived RV EF. Subjects were divided into two groups based on CMR RV EF (group I: RV EF ≥45%; and group II: RV EF <45%). A total of 57 subjects were included. Mean age was 13.0±3.6 years and 58% were males. Group I had 39 subjects and group II had 18. Only six of the 18 patients (33%) in group II were identified as having at least mild RV dysfunction by echocardiogram. The mean RV GLS was significantly abnormal in group II (-15.3±3.8%) compared to group I (-20.9±3.3%; P<.001). By ROC analysis, an RV GLS cutoff value of -18% had 78% sensitivity and 77% specificity in identifying RV EF <45% (area under curve .87, P<.001). Intra- and inter-observer reproducibility of RV GLS were good. RV GLS is a simple and effective tool for the assessment of RV systolic function in patients post TOF repair. This technique would help further refine patient selection for timing of CMR and management. © 2016, Wiley Periodicals, Inc.

  9. Identification of TBX5 mutations in a series of 94 patients with Tetralogy of Fallot

    NARCIS (Netherlands)

    Baban, Anwar; Postma, Alex Vincent; Marini, Monica; Trocchio, Gianluca; Santilli, Antonella; Pelegrini, Monica; Sirleto, Pietro; Lerone, Margherita; Albanese, Sonia Bernadette; Barnett, Phil; Boogerd, Cornelis Job; Dallapiccola, Bruno; Digilio, Maria Cristina; Ravazzolo, Roberto; Pongiglione, Giacomo

    2014-01-01

    Tetralogy of Fallot (TOF) (OMIM #187500) is the most frequent conotruncal congenital heart defect (CHD) with a range of intra- and extracardiac phenotypes. TBX5 is a transcription factor with well-defined roles in heart and forelimb development, and mutations in TBX5 are associated with Holt-Oram

  10. Virtual surgical modification for planning tetralogy of Fallot repair

    Science.gov (United States)

    Plasencia, Jonathan; Babiker, Haithem; Richardson, Randy; Rhee, Edward; Willis, Brigham; Nigro, John; Cleveland, David; Frakes, David H.

    2010-01-01

    Goals for treating congenital heart defects are becoming increasingly focused on the long-term, targeting solutions that last into adulthood. Although this shift has motivated the modification of many current surgical procedures, there remains a great deal of room for improvement. We present a new methodological component for tetralogy of Fallot (TOF) repair that aims to improve long-term outcomes. The current gold standard for TOF repair involves the use of echocardiography (ECHO) for measuring the pulmonary valve (PV) diameter. This is then used, along with other factors, to formulate a Z-score that drives surgical preparation. Unfortunately this process can be inaccurate and requires a mid-operative confirmation that the pressure gradient across the PV is not excessive. Ideally, surgeons prefer not to manipulate the PV as this can lead to valve insufficiency. However, an excessive pressure gradient across the valve necessitates surgical action. We propose the use of computational fluid dynamics (CFD) to improve preparation for TOF repair. In our study, pre-operative CT data were segmented and reconstructed, and a virtual surgical operation was then performed to simulate post-operative conditions. The modified anatomy was used to drive CFD simulation. The pressure gradient across the pulmonary valve was calculated to be 9.24mmHg, which is within the normal range. This finding indicates that CFD may be a viable tool for predicting post-operative pressure gradients for TOF repair. Our proposed methodology would remove the need for mid-operative measurements that can be both unreliable and detrimental to the patient.

  11. Correlating the morphological features of tetralogy of Fallot and the Eisenmenger malformation.

    Science.gov (United States)

    Restivo, Angelo; Anderson, Robert H; Carletti, Raffaella; di Gioia, Cira R T

    2017-01-01

    We studied a series of 43 autopsied cases of tetralogy of Fallot, assessing the mode of insertion of the outlet septum relative to the limbs of the septomarginal trabeculation, and compared the findings in retrospective fashion with our previous observations of a group of hearts with the so-called Eisenmenger malformation. In the majority of hearts with tetralogy of Fallot, the outlet septum inserted frontally relative to the septomarginal trabeculation, but in a minority of cases the outlet septum inserted in lateral fashion, as had been observed in all our hearts studied with the Eisenmenger malformation. The different modes of insertion were found to correlate, first, with the axis of anatomical aortic rightward rotation, coincident with the commissure between the right coronary and the left coronary leaflets of the aortic valve. The different modes of insertion of the outlet septum also correlated with the level of attachment of the arterial valvar leaflets on its subpulmonary and subaortic surfaces; concomitantly, correlation was found between the length of the subpulmonary infundibulum and the length of the muscular outlet septum itself. In the majority of hearts showing tetralogy of Fallot, the elongated infundibulum was also uniformly narrow, but in a minority the infundibulum was well expanded, obstructed only at its mouth, but widening at the valvar level. In all the hearts with the Eisenmenger malformation, in contrast, the unobstructed infundibulum was well expanded. The morphological findings of the present study show unequivocally that tetralogy of Fallot and Eisenmenger malformation are two phenotypically different congenital cardiac anomalies.

  12. Study on coordination of ventricular contraction by a phase analysis method in tetralogy of Fallot

    International Nuclear Information System (INIS)

    Chen Xianying; Zhu Hongyu; Li Xinmin; Wang Zhiguo; Zhang Guoxu; Zhang Zhaozhong; Wang Kaigeng

    2001-01-01

    Objective: Quantitative study on the characters of left ventricular (LV) wall motion and assessing degree of satisfaction of surgical repair of tetralogy of Fallot with phase standard deviation (PSD). Methods: PSD was calculated by equilibrium radionuclide ventriculography in 24 normal controls and 59 patients of tetralogy of Fallot before and after operation. Results: LV PSD was (9.7 +- 2.8) degree in 24 normal controls and (20.5 +- 15.5) degree and (10.0 +- 7.2) degree in 51(86.4%) of 59 patients of tetralogy of Fallot before and after surgical repair, respectively, and the difference was statistically significant (P < 0.01). LV PSD was (11.2 +- 7.8) degree and (21.3 +- 9.3) degree, respectively before and after surgical repair in the remaining 8(13.4%) patients and the LV PSD was increased significantly after operation (P < 0.05). Conclusions: LV PSD is coordinate with improving degree of ventricular wall motion and heart function after surgical repair of tetralogy of Fallot. PSD is one of the heart function parameters for reflecting the degree of satisfaction of the surgical repair

  13. Significance of lung anomalies in fetuses affected by tetralogy of Fallot with absent pulmonary valve syndrome.

    Science.gov (United States)

    Tenisch, Estelle; Raboisson, Marie-Josée; Rypens, Françoise; Déry, Julie; Grignon, Andrée; Lapierre, Chantale

    2017-11-01

    Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance. We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome. Abnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series. This study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

  14. Magnetic resonance imaging pre- and postoperative evaluation of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Bernardes, Renata Junqueira Moll; Simoes, Luiz Carlos

    2004-01-01

    The purpose of this study was to assess the usefulness of magnetic resonance imaging (MRI) in the pre- and postoperative evaluation of patients with tetralogy of Fallot. Twenty patients aged 1 to 29 years were prospectively evaluated with black-blood and contrast-enhanced angiographic techniques, 11 with the classic form of tetralogy of Fallot and 9 with tetralogy of Fallot and pulmonary atresia. MRI studies provided adequate visualization of the aorta that was classified as dilated or not dilated, and definition of its position in all cases. The use of contrast-enhanced MR angiographic techniques provided excellent imaging of the main right and left pulmonary arteries. The results suggest that MRI, including contrast-enhanced angiography techniques, is a useful tool in the evaluation of patients with tetralogy of Fallot before and after cardiac surgery since it provides important anatomical information that is not always obtained with echocardiography. MRI can be considered an alternative to cardiac catheterization, particularly in the evaluation of the pulmonary vascular anatomy. (author)

  15. Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair

    NARCIS (Netherlands)

    Wald, Rachel M.; Valente, Anne Marie; Gauvreau, Kimberlee; Babu-Narayan, Sonya V.; Assenza, Gabriele Egidy; Schreier, Jenna; Gatzoulis, Michael A.; Kilner, Philip J.; Koyak, Zeliha; Mulder, Barbara; Powell, Andrew J.; Geva, Tal

    2015-01-01

    Patients with repaired tetralogy of Fallot (TOF) are followed serially by cardiac magnetic resonance (CMR) for surveillance of RV dilation and dysfunction. We sought to define the prevalence of progressive RV disease and the optimal time interval between CMR evaluations. Candidates were selected

  16. Tetralogy of Fallot with Pulmonary Obstruction at the Level of the ...

    African Journals Online (AJOL)

    1974-04-06

    Apr 6, 1974 ... A case of Fallot's tetralogy is described in a Black male who died of acute .... rather than that of Shauer; although the theory expoun- ded by him is very ... The punched-out hole in the interatrial septum reflects a congenital ...

  17. Value of Cardiovascular Magnetic Resonance Imaging in Noninvasive Risk Stratification in Tetralogy of Fallot

    NARCIS (Netherlands)

    Bokma, Jouke P.; de Wilde, Koen C.; Vliegen, Hubert W.; van Dijk, Arie P.; van Melle, Joost P.; Meijboom, Folkert J.; Zwinderman, Aeilko H.; Groenink, Maarten; Mulder, Barbara J. M.; Bouma, Berto J.

    IMPORTANCE Adults late after total correction of tetralogy of Fallot (TOF) are at risk for majorcomplications. Cardiovascular magnetic resonance (CMR) imaging is recommended toquantify right ventricular (RV) and left ventricular (LV) function. However, a commonly usedrisk model by Khairy et al

  18. Multipel graviditet hos kvinder med korrigeret Steno-Fallots tetralogi

    DEFF Research Database (Denmark)

    Pedersen, Lia Mendes; Pedersen, Thais Andreas Bjerregaard; Herskind, Anne Maria

    2008-01-01

    Tetralogy of Fallot is the most common cyanotic heart disease accounting for 5 to 8% of all cases of congenital heart disease. In a cohort of women with corrected Tetralogy of Fallot, followed concerning fertility and pregnancy outcome, we observed two cases of uncomplicated multiple pregnancies...

  19. Congenital intermittent atrio-ventricular dissociation in tetralogy of Fallot (a case report.

    Directory of Open Access Journals (Sweden)

    Karande S

    1991-01-01

    Full Text Available A case of tetralogy of fallot with congenital intermittent atrioventricular dissociation is reported. A review of standard postgraduate books of cardiology fails to describe this condition, thus showing its extreme rarity. The therapeutic dilemma in treating such a case is also discussed.

  20. Oxygen uptake efficiency slope and peak oxygen consumption predict prognosis in children with tetralogy of Fallot.

    Science.gov (United States)

    Tsai, Yun-Jeng; Li, Min-Hui; Tsai, Wan-Jung; Tuan, Sheng-Hui; Liao, Tin-Yun; Lin, Ko-Long

    2016-07-01

    Oxygen uptake efficiency slope (OUES) and peak oxygen consumption (VO2peak) are exercise parameters that can predict cardiac morbidity in patients with numerous heart diseases. But the predictive value in patients with tetralogy of Fallot is still undetermined, especially in children. We evaluated the prognostic value of OUES and VO2peak in children with total repair of tetralogy of Fallot. Retrospective cohort study. Forty tetralogy of Fallot patients younger than 12 years old were recruited. They underwent a cardiopulmonary exercise test during the follow-up period after total repair surgery. The results of the cardiopulmonary exercise test were used to predict the cardiac related hospitalization in the following two years after the test. OUES normalized by body surface area (OUES/BSA) and the percentage of predicted VO2peak appeared to be predictive for two-year cardiac related hospitalization. Receiver operating characteristic curve analysis demonstrated that the best threshold value for OUES/BSA was 1.029 (area under the curve = 0.70, p = 0.03), and for VO2peak was 74% of age prediction (area under the curve = 0.72, p = 0.02). The aforementioned findings were confirmed by Kaplan-Meier plots and log-rank test. OUES/BSA and VO2peak are useful predictors of cardiac-related hospitalization in children with total repair of tetralogy of Fallot. © The European Society of Cardiology 2015.

  1. Young infants with severe tetralogy of Fallot: Early primary surgery versus transcatheter palliation.

    Science.gov (United States)

    Wilder, Travis J; Van Arsdell, Glen S; Benson, Lee; Pham-Hung, Eric; Gritti, Michael; Page, Alexandra; Caldarone, Christopher A; Hickey, Edward J

    2017-11-01

    Infants with severe tetralogy of Fallot may undergo (1) early primary surgical repair (EARLY) or (2) early transcatheter palliation (CATH) before delayed surgical repair. We compared these strategies with (3) elective single-stage tetralogy of Fallot repair (IDEAL). From 2000 to 2012, 453 children underwent tetralogy of Fallot repair (excluding systemic-pulmonary shunts), including 383 in the IDEAL (75%), 42 in the EARLY (9%), and 28 in the CATH (6%) groups. IDEAL repair at The Hospital for Sick Children occurs after 3 months. Risk-adjusted hazard analysis compared freedom from surgical or catheter reintervention. Somatic size, branch pulmonary artery size, and right ventricle systolic pressure were modeled using 2780 echocardiogram reports via mixed-model regression. CATH involved right ventricular outflow tract stent in 18 patients, right ventricular outflow tract balloon in 9 patients, and ductal-stent in 1 patient. Three patients died (1 per group). Risk-adjusted freedom from surgical reoperation was 89% ± 4%, 88% ± 5%, and 85% ± 6% for the IDEAL, EARLY, and CATH groups, respectively, at 10 years. Patients in the EARLY and CATH groups had similar reoperation rates, except for neonates (tetralogy of Fallot. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  2. Stent migration after right ventricular outflow tract stenting in the severe cyanotic Tetralogy of Fallot case

    Directory of Open Access Journals (Sweden)

    Tamaki Hayashi

    2017-01-01

    Full Text Available We report our experience with a stent migration after right ventricle outflow tract stenting and converted to patent ductus arteriosus stenting in Tetralogy of Fallot (TOF with severe infundibular stenosis. Finally, the patient achieved to TOF repair, and the migrated stent was removed without any complication.

  3. 22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.

    Science.gov (United States)

    Mercer-Rosa, Laura; Pinto, Nelangi; Yang, Wei; Tanel, Ronn; Goldmuntz, Elizabeth

    2013-10-01

    We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher exact and Mann-Whitney tests were used for categoric and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome. We studied 208 subjects with tetralogy of Fallot, 164 (79%) without and 44 (20%) with 22q11.2 deletion syndrome. There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and reoperations were also comparable between patients with and without 22q11.2 deletion syndrome. Those with 22q11.2 deletion syndrome had more aortopulmonary shunts preceding complete surgical repair (21% vs 7%, P = .02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2 deletion syndrome had longer cardiopulmonary bypass time (84 vs 72 minutes, P = .02) and duration of intensive care (6 vs 4 days, P = .007). Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population. Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  4. Technical modification enabling pulmonary valve-sparing repair of a severely hypoplastic pulmonary annulus in patients with tetralogy of Fallot.

    Science.gov (United States)

    Ito, Hiroki; Ota, Noritaka; Murata, Masaya; Tosaka, Yuko; Ide, Yujiro; Tachi, Maiko; Sugimoto, Ai; Sakamoto, Kisaburo

    2013-06-01

    Although pulmonary valve-sparing repair is preferable for patients with tetralogy of Fallot, the repair of very small pulmonary valves is challenging. The present study evaluates our modification for preserving severely hypoplastic pulmonary valves in patients with tetralogy of Fallot. Sixty-eight consecutive patients who underwent complete repair of a tetralogy of Fallot between 2005 and 2011 were retrospectively reviewed. Patients with pulmonary atresia, absence of a pulmonary valve, atrioventricular septal defect and/or subarterial ventricular septal defect were excluded. There were 19 (28%) patients with a severely hypoplastic pulmonary annulus determined by preoperative echocardiography (z-score -4 group. In the z tetralogy of Fallot could not be applied in all patients, this strategy enabled acceptable growth of the valve annulus, with only mild stenosis during the early to mid-term follow-up. This modification seems to be an option, even for a very small pulmonary valve.

  5. Anestesia en la tetralogía de Fallot Anesthesia in the tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Lincoln de la Parte Pérez

    2004-12-01

    Full Text Available Se presenta una revisión bibliográfica sobre las características fundamentales de esta enfermedad, la repercusión hemodinámica que produce, las complicaciones que se presentan durante el preoperatorio, los defectos asociados a esta, el tratamiento preoperatorio y el manejo anestésico que se utiliza. Estos pacientes que se programan para una reconstrucción anatómica, están en el mejor de los casos sólo compensados parcialmente, por lo que pueden presentar inestabilidad hemodinámica con la administración de los agentes anestésicos, especialmente durante la inducción anestésica. Por todo lo anterior es que deben ser atendidos con delicadeza y cuidado extremo para evitar empeorar la disfunción cardiovascular preexistente, que crearía un ciclo de agravamiento de la hipoxia, acidosis y disminución del gasto cardíaco.A bibliographic review of the main characteristics of this disease, the hemodynamic repercussion it produces, the complications appearing during the preoperatory, the defects associated with it, the preoperative treatment and the anesthetic management used in it, is made. These patients that are scheduled for an anatomical reconstruction are at best only partially compesated, so they can present hemodynamic unstability with the administration of anesthetic agents, specially during anesthetic induction. Taking all this into account, they should receive a delicate and extremely careful attention so as not to worsen the preexisting cardiovascular dysfunction that will create a cycle of severeness of hypoxia, acidosis and reduction of the cardiac output.

  6. Early postoperative remodelling following repair of tetralogy of Fallot utilising unsedated cardiac magnetic resonance: a pilot study.

    Science.gov (United States)

    DiLorenzo, Michael P; Goldmuntz, Elizabeth; Nicolson, Susan C; Fogel, Mark A; Mercer-Rosa, Laura

    2018-05-01

    IntroductionThe 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 methodSubjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot. Protocol-based cardiac MRI to assess anatomy, function, and flows was performed before hospital discharge using the feed and sleep technique, an unsedated imaging technique. MRI was completed in 16 subjects at a median age of 77 days (interquartile range 114). There was normal ventricular ejection fraction and indexed right ventricular end-diastolic volume (48±13 cc/m2), but elevated right ventricular mass (z score 6.2±2.4). Subjects requiring a transannular patch or right ventricle to pulmonary artery conduit had moderate pulmonary insufficiency (regurgitant fraction 27±16%).DiscussionEarly right ventricular remodelling after surgical repair for tetralogy of Fallot is characterised by significant pulmonary regurgitation, right ventricular hypertrophy, and lack of dilation. Performing cardiac MRI using the feed and sleep technique is feasible in infants younger than 5 months. These results might open new avenues to study longitudinal right ventricular changes in tetralogy of Fallot and to further explore the utility of unsedated MRI in patients with other types of CHDs.

  7. New Insights into the Surgical Management of Tetralogy of Fallot: Physiological Fundamentals and Clinical Relevance.

    Science.gov (United States)

    Bove, Thierry; François, Katrien; De Wolf, Daniel

    2015-01-01

    The surgical treatment of tetralogy of Fallot can be considered as a success story in the history of congenital heart diseases. Since the early outcome is no longer the main issue, the focus moved to the late sequelae of TOF repair, i.e. the pulmonary insufficiency and the secondary adaptation of the right ventricle. This review provides recent insights into the pathophysiological alterations of the right ventricle in relation to the reconstruction of the right ventricular outflow tract after repair of tetralogy of Fallot. Its clinical relevance is documented by addressing the policy changes regarding the optimal management at the time of surgical repair as well as properly defining criteria and timing for late pulmonary valve implantation.

  8. Palliative balloon dilation of pulmonic stenosis in a dog with tetralogy of Fallot.

    Science.gov (United States)

    Weder, C; Ames, M; Kellihan, H; Bright, J; Orton, C

    2016-09-01

    A 6-month-old Beagle with tetralogy of Fallot underwent balloon valvuloplasty of the pulmonary valve. Balloon valvuloplasty was successful and resulted in palliation of clinical signs and an improved quality of life for approximately 9 months. After 9 months, the dog became symptomatic and a modified Blalock-Taussig shunt procedure was successfully performed. Based on this report, balloon valvuloplasty in dogs with tetralogy of Fallot appears to be a feasible technique that may result in improvement of clinical signs. In addition, it may allow for the delay of the more invasive surgical palliation and provide time for weight gain and development of the pulmonary vascular bed for greater ease of surgical shunt creation. Copyright © 2016 Elsevier B.V. All rights reserved.

  9. Clinical application of 64-slice spiral CT in the diagnosis of the Tetralogy of Fallot

    International Nuclear Information System (INIS)

    Wang Ximing; Wu Lebin; Sun Cong; Liu Cheng; Chao, Bao-Ting; Han Bo; Zhang Yunting; Chen Haisong; Li Zhenjia

    2007-01-01

    Objective: To explore the clinical application of 64-slice spiral CT in the diagnosis of the Tetralogy of Fallot. Methods: Eighteen patients with diagnosed Tetralogy of Fallot underwent cardiac CT angiography with 64-slice CT (CTA). Two- and three-dimensional images were used for diagnosing in all cases by means of MPR (coronal, sagital and oblique), cMPR, MIP and VRT. Results: All patients had ventricular septal defect, pulmonary stenosis and hypertrophy of right ventricle. The morphologic features of Tetralogy of Fallot were equal to echocardiography. Patent ductus arteriosus (PDA) were detected on eight patients. Main coronary artery branches and partial sub-branches were visualized in all patients. Abnormal coronary arteries were found in seven cases: both left and right main coronary artery arising from the right aortic sinus one case, right main coronary artery and circumflex (CX) arising from the right aortic sinus one case, left anterior descending (LAD) and CX arising from left aortic sinus directly one case, left and right coronary artery arising from back and front of the aortic sinus, respectively, three cases. Pulmonary artery branch stenosis was found in 12 cases and branch pulmonary artery dilation noticed in 1 case. Double superior vena cava was also found in one case. According to the surgery the diagnostic accuracy of CT and Echocardiography was 95.45 and 83.33%, respectively. Conclusion: Two- and three-dimensional 64-slice CTA not only show the overall anatomical structure of the heart, but also show coronary and pulmonary arteries. With these results, evaluation of coronary anomalies and pulmonary artery stenosis with 64-MSCT is extremely valuable for planning the operative procedure on the patients with Tetralogy of Fallot

  10. Clinical application of 64-slice spiral CT in the diagnosis of the Tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Wang Ximing [Tianjin Medical University, Tianjin City (China) and Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China)], E-mail: wxming369@163.com.cn; Wu Lebin [Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China); Sun Cong [Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China); Shandong University Medical College, Shandong Jinan 250012 (China); Liu Cheng [Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China); Chao, Bao-Ting [Shandong University Medical College, Shandong Jinan 250012 (China); Han Bo [Shandong Provincial Hospital Pediatric Department, Shandong, Jinan 250021 (China); Zhang Yunting [Tianjin Medical University, General Hospital MR Department, Tianjin City (China); Chen Haisong [Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China); Shandong University Medical College, Shandong Jinan 250012 (China); Li Zhenjia [Shandong Medical Imaging Research Institute, Jinan City, Shandong Province 250021 (China)

    2007-11-15

    Objective: To explore the clinical application of 64-slice spiral CT in the diagnosis of the Tetralogy of Fallot. Methods: Eighteen patients with diagnosed Tetralogy of Fallot underwent cardiac CT angiography with 64-slice CT (CTA). Two- and three-dimensional images were used for diagnosing in all cases by means of MPR (coronal, sagital and oblique), cMPR, MIP and VRT. Results: All patients had ventricular septal defect, pulmonary stenosis and hypertrophy of right ventricle. The morphologic features of Tetralogy of Fallot were equal to echocardiography. Patent ductus arteriosus (PDA) were detected on eight patients. Main coronary artery branches and partial sub-branches were visualized in all patients. Abnormal coronary arteries were found in seven cases: both left and right main coronary artery arising from the right aortic sinus one case, right main coronary artery and circumflex (CX) arising from the right aortic sinus one case, left anterior descending (LAD) and CX arising from left aortic sinus directly one case, left and right coronary artery arising from back and front of the aortic sinus, respectively, three cases. Pulmonary artery branch stenosis was found in 12 cases and branch pulmonary artery dilation noticed in 1 case. Double superior vena cava was also found in one case. According to the surgery the diagnostic accuracy of CT and Echocardiography was 95.45 and 83.33%, respectively. Conclusion: Two- and three-dimensional 64-slice CTA not only show the overall anatomical structure of the heart, but also show coronary and pulmonary arteries. With these results, evaluation of coronary anomalies and pulmonary artery stenosis with 64-MSCT is extremely valuable for planning the operative procedure on the patients with Tetralogy of Fallot.

  11. Tetralogy of Fallot: Report of 30 Cases and Dental Considerations with Review of Literature

    OpenAIRE

    S Gedik; R Gedik; TN Gedik

    2015-01-01

    Tetralogy of Fallot (TOF) is one of the most serious cyanotic congenital heart diseases and is characterized by common systemic and oral findings. This study was carried out on 30 patients (3−13 years old) with TOF and 30 subjects as a control group. The patients were examined for oral hygiene, mucous membrane, gingivae, dental caries and any hypoplasia. There appeared to be an increase in dental caries, hypoplasia and periodontal disease in the patients with TOF compared with controls. The d...

  12. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Robida, A.; Fettich, D.

    1985-09-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease.

  13. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    International Nuclear Information System (INIS)

    Robida, A.; Fettich, D.

    1985-01-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease. (orig.)

  14. The impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot.

    Science.gov (United States)

    Ávila, Pablo; Marcotte, François; Dore, Annie; Mercier, Lise-Andrée; Shohoudi, Azadeh; Mongeon, François-Pierre; Mondésert, Blandine; Proietti, Anna; Ibrahim, Reda; Asgar, Anita; Poirier, Nancy; Khairy, Paul

    2016-09-15

    Sudden death of presumed arrhythmic etiology is the leading cause of mortality in adults with tetralogy of Fallot. To assess the impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot. Adults with repaired tetralogy of Fallot were randomized in a 2:1 ratio to an open-label trial of standard care versus 12weeks of supervised combined aerobic/resistance training with continuous Holter monitoring. Proportion of premature ventricular complexes (PVC) and runs of non-sustained ventricular tachycardia (NSVT) were assessed by mixed and Poisson regression models with generalized estimating equations for repeated measures. A total of 152 Holters were performed in 17 patients, median age 35 (interquartile range [IQR] 28, 42) years, 65% male, 13 of whom were randomized to exercise training. Baseline characteristics were similar between groups. Exercise training resulted in significant increases in peak oxygen uptake (11±19%, p=0.028), metabolic equivalents (11±18%, p=0.027), and exercise duration (8±10%, p=0.009) compared to no changes in controls. Frequent (≥30 per hour) PVCs were present in 46% of patients, couplets in 62%, and 3 to 7 beat runs of NSVT in 31%. The median proportion of PVCs was 1.93‰ (IQR 0.41, 5.89) at baseline and 1.45‰ (IQR 0.08, 2.76) during the initial exercise session (p=0.722), and remained stable over time (ß coefficient=-0.031, p=0.408). Runs of NSVT decreased significantly over time (ß coefficient=-0.032, p=0.018). In adults with repaired tetralogy of Fallot, exercise training is safe, improves exercise capacity, and appears to confer a beneficial effect on ventricular arrhythmias. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  15. Primary tetralogy of Fallot repair: predictors of intensive care unit morbidity.

    Science.gov (United States)

    Egbe, Alexander C; Uppu, Santosh C; Mittnacht, Alexander J C; Joashi, Umesh; Ho, Deborah; Nguyen, Khanh; Srivastava, Shubhika

    2014-09-01

    Primary repair of tetralogy of Fallot has low surgical mortality, but some patients still experience significant postoperative morbidity. Our objectives were to review our institutional experience with primary tetralogy of Fallot repair, and identify predictors of intensive care unit morbidity. We reviewed all patients with tetralogy of Fallot who underwent primary repair in infancy from 2001 to 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. Intensive care unit morbidity was defined as prolonged intensive care unit stay (≥ 7 days) and/or prolonged duration of mechanical ventilation (≥ 48 h). 97 patients who underwent primary surgical repair during the study period were included in the study. The median age was 4.9 months (range 1-9 months) and the median weight was 5.3 kg (range 3.1-9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median intensive care unit stay was 6 days (range 2-21 days) and the median duration of mechanical ventilation was 19 h (range 0-136 h). Age and weight were independent predictors of intensive care unit stay, while surgical era predicted the duration of mechanical ventilation. Primary tetralogy of Fallot repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery were significant predictors of morbidity. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  16. Feasibility of a healthcare system-based tetralogy of Fallot patient registry.

    Science.gov (United States)

    Khoury, Audrey L; Jernigan, Eric G; Chowdhury, Muntasir H; Loehr, Laura R; Nelson, Jennifer S

    2018-02-01

    Patient-reported outcomes and epidemiological studies in adults with tetralogy of Fallot are lacking. Recruitment and longitudinal follow-up investigation across institutions is particularly challenging. Objectives of this study were to assess the feasibility of recruiting adult patients with tetralogy of Fallot for a patient-reported outcomes study, describe challenges for recruitment, and create an interactive, online tetralogy of Fallot registry. Adult patients living with tetralogy of Fallot, aged 18-58 years, at the University of North Carolina were identified using diagnosis code query. A survey was designed to collect demographics, symptoms, history, and birth mother information. Recruitment was attempted by phone (Part I, n=20) or by email (Part II, n=20). Data analysis included thematic grouping of recruitment challenges and descriptive statistics. Feasibility threshold was 75% for recruitment and for data fields completed per patient. In Part I, 60% (12/20) were successfully contacted and eight (40%) were enrolled. Demographics and birth mother information were obtained for all enrolled patients. In Part II, 70% (14/20) were successfully contacted; 30% (6/20) enrolled and completed all data fields linked to REDCap database; the median time for survey completion was 8 minutes. Half of the patients had cardiac operations/procedures performed at more than one hospital. Automatic electronic data entry from the online survey was uncomplicated. Although recruitment (54%) fell below our feasibility threshold, enrolled individuals were willing to complete phone or online surveys. Incorrect contact information, privacy concerns, and patient-reported time constraints were challenges for recruitment. Creating an online survey and linked database is technically feasible and efficient for patient-reported outcomes research.

  17. Clinical Progress in the Management of Tetralogy of Fallot in the Dominican Republic: A Case Series.

    Science.gov (United States)

    Perez, María T; Vázquez, Marietta; Canarie, Michael F; Toribio, Janet; León-Wyss, Juan

    2017-09-01

    Definitive surgical interventions for Dominican children with congenital heart disease, like those of other low- and middle-income countries, have been historically limited. We undertook review of a case series focusing on the surgical correction of complex forms of tetralogy of Fallot at a single center, CEDIMAT Centro Cardiovascular, in the Dominican Republic, over a 30-month period. According to our criteria, 43 cases were determined to be complex tetralogy of Fallot repairs from the two-year period. Besides tetralogy of Fallot, the cohort had an additional 55 anatomic anomalies that had to be addressed at the time of surgery. Median age at the time of surgery was notably 30 months, and an average of 42 months elapsed from the time of diagnosis to the time of surgery for this group. Only 33% of the cases reviewed had no hypercyanotic crises before repair. Median time to extubation for this group of patients was one day, with a three-day median length of stay in the intensive care setting. Our study importantly captures the present experience of a surgical congenital heart program that has recently transitioned from a traditional "mission model" to a now self-sustaining local practice. Both the number and the complexity of the lesions corrected in this caseload represent an advance from the level of care previously provided to children in the Dominican Republic.

  18. Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair.

    Science.gov (United States)

    Sullivan, Rachel T; Frommelt, Peter C; Hill, Garick D

    2017-08-01

    The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.

  19. Obstetric Outcomes in Women with Unrepaired Tetralogy of Fallot: A Case Report

    Directory of Open Access Journals (Sweden)

    Burak Karadağ

    2011-08-01

    Full Text Available Tetralogy of Fallot (TOF is the most common form of cyanotic congenital heart diseases and without corrective surgery, natural survival rate into the fourth decade was only about 3%. In pregnant patients with unrepaired TOF, pregnancy-related physiological changes is more difficult to tolerate compared to healthy pregnant woman. Discussed below a case of term pregnancy in a 20- year old woman with unrepaired TOF, was diagnosed at delivery.

  20. [Treatment of Fallot tetralogy with a transannular patch. Six years follow-up].

    Science.gov (United States)

    Galicia-Tornell, Myriam; Reyes-López, Alfonso; Ruíz-González, Sergio; Bolio-Cerdán, Alejandro; González-Ojeda, Alejandro; Fuentes-Orozco, Clotilde

    2015-01-01

    Primary repair of Fallot tetralogy has been performed successfully for the last 45 years. It has low surgical mortality (Fallot tetralogy is the most common form of cyanotic congenital heart disease (including transannular patch) and accounts for 7.5% of all cardiovascular surgical procedures. The mid-term follow-up results are reported. Case series. The study included patients who had complete repair of Fallot tetralogy with transannular patch from January 2000 to December 2009. An analysis was performed on the clinical variables, morbidity and mortality. There were 52 patients in the study, with mean age 4 ± 2 years. Perioperative mortality in 6 patients, with 5 associated with residual right ventricular obstruction and, 1 associated with further surgery. The survival rate was 88% (46) patients, with a follow-up 75 ± 26 months. Late morbidity occurred in 14, due to right ventricular dysfunction in 11, recurrent distal obstruction in 2, and residual ventricular septal defect in 1. Associated risk factors were severe pulmonary insufficiency (p=0.001); QRS > 160 ms, p=0.001); cardiothoracic > 0.60 index, (p=0.048), and tricuspid regurgitation (p=0.001). There was reasonable long-term survival and excellent quality of life after total correction of Fallot tetralogy; however, progressive right ventricular dysfunction requires continuous monitoring, as well as the choice of optimal timing of pulmonary valve replacement. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

  1. Serum Bile Acids in Repaired Tetralogy of Fallot: A Marker for Liver and Heart?

    Science.gov (United States)

    Grangl, Gernot; Zöhrer, Evelyn; Köstenberger, Martin; Jud, Alexandra; Fauler, Günter; Scharnagl, Hubert; Stojakovic, Tatjana; Marterer, Robert; Gamillscheg, Andreas; Jahnel, Jörg

    2015-01-01

    Patients with repaired tetralogy of Fallot may develop chronic right ventricular dysfunction and hepatic congestion over time. We hypothesized that bile acid metabolism is altered in repaired tetralogy of Fallot patients and therefore sought to correlate right ventricular indices with serum bile acid levels. Indexed right ventricular end diastolic volume, as assessed by cardiac magnetic-resonance imaging, was classified as 150ml/m2 (Group 3, n = 6) in 29 patients with repaired tetralogy of Fallot. Pulmonary regurgitation fraction and right ventricular ejection fraction were calculated. The serum bile acid profile, including 15 species, in these patients was determined by liquid chromatography coupled with mass spectrometry. Serum bile acid levels increased from Group 1 to Group 3 (2.5 ± 0.7; 4.1 ± 2.5; 6.0 ± 2.8 μmol/l, respectively) with significantly increased bile acid values in Group 3 compared to Group 1 (p≤0.05). In Group 3, but not in Group 1 and 2, a significant increase in glycine-conjugated bile acids was observed. Pulmonary regurgitation fraction increased (12 ± 1; 28 ± 16; 43 ± 3%, Groups 1-3, respectively) and right ventricular ejection fraction decreased (48.4 ± 6.4; 48.5 ± 6.5; 42.1 ± 5.3%, Groups 1-3, respectively) with rising indexed right ventricular end diastolic volume. These preliminary results suggest that serum bile acid levels are positively correlated with indexed right ventricular end-diastolic volume in patients with repaired tetralogy of Fallot; however, this needs to be confirmed in a larger patient cohort.

  2. The formation of congenital heart defects (tetralogy of Fallot) in children living in adverse environmental areas of Gomel and Mogilev regions of Belarus

    International Nuclear Information System (INIS)

    Rinkevich, E.P.; Kokorina, N.V.; Khmel', R.D.

    2015-01-01

    In this work were analyzed data on the frequency of occurrence UPU tetralogy of Fallot in the Republic of Belarus for the period 2007-2012. Studied comparative characteristics of morbidity of children tetralogy of Fallot in Mogilev, Gomel and Vitebsk regions. (authors)

  3. Impaired arm development after Blalock-Taussig shunts in adults with repaired tetralogy of Fallot.

    Science.gov (United States)

    Le Gloan, Laurianne; Marcotte, François; Leduc, Hugues; Mercier, Lise-Andrée; Dore, Annie; Mongeon, François-Pierre; Ibrahim, Reda; Miro, Joaquim; Asgar, Anita; Poirier, Nancy; Khairy, Paul

    2013-09-30

    Many adults with repaired tetralogy of Fallot have had prior Blalock-Taussig shunts. These shunts may theoretically hinder growth and development of the ipsilateral arm. We prospectively enrolled consecutive patients with tetralogy of Fallot in a cross-sectional study to measure arm length and assess handgrip strength. Bilateral handgrip strength was quantified by a dynamometer in a standing position after instructing patients to clench each hand tightly in succession. The maximum force achieved, in kilograms, was measured. A total of 80 consecutive adults with tetralogy of Fallot, aged 36.0 ± 12.5 years, 49% female, were prospectively enrolled. Thirty-eight (47.5%) patients had prior Blalock-Taussig shunts at a median age of 1.0 year. Twenty-one (55.3%) were left-sided and 23 (60.5%) were classic shunts. All but six patients with right-sided shunts and one without a prior shunt were right-handed. The shunts were present for a median of 4.0 years prior to takedown during corrective surgery. The arm ipsilateral to the shunt was significantly shorter than the contralateral arm (71.5 ± 6.1 versus 73.6 ± 5.6 cm, Ptetralogy of Fallot, Blalock-Taussig shunts may impair normal development of the ipsilateral arm with repercussions in adulthood that include shorter limb length and reduced handgrip strength. These changes are most pronounced in patients with classic end-to-side anastomoses. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  4. Ventricular arrhythmia risk stratification in patients with tetralogy of Fallot at the time of pulmonary valve replacement.

    Science.gov (United States)

    Sabate Rotes, Anna; Connolly, Heidi M; Warnes, Carole A; Ammash, Naser M; Phillips, Sabrina D; Dearani, Joseph A; Schaff, Hartzell V; Burkhart, Harold M; Hodge, David O; Asirvatham, Samuel J; McLeod, Christopher J

    2015-02-01

    Most patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR), but the evaluation for and management of ventricular arrhythmia remain unclear. This study is aimed at clarifying the optimal approach to this potentially life-threatening issue at the time of PVR. A retrospective analysis was performed on 205 patients with repaired tetralogy of Fallot undergoing PVR at our institution between 1988 and 2010. Median age was 32.9 (range, 25.6) years. Previous ventricular tachycardia occurred in 16 patients (8%) and 37 (16%) had left ventricular dysfunction, defined as left ventricular ejection fraction tetralogy of Fallot undergoing PVR with history of ventricular tachycardia or left ventricular dysfunction appear to be associated with a higher risk of arrhythmic events after operation. Events in the first year after PVR are rare, and in select high-risk patients, surgical cryoablation does not seem to increase arrhythmic events and may be protective. © 2014 American Heart Association, Inc.

  5. Magnetic resonance imaging pre- and postoperative evaluation of tetralogy of Fallot; Avaliacao pre e pos-operatoria da tetralogia de Fallot por ressonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Bernardes, Renata Junqueira Moll; Simoes, Luiz Carlos [Instituto Nacional de Cardiologia (INC), Rio de Janeiro, RJ (Brazil). Servico de Cardiologia da Crianca e do Adolescente; Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia]. E-mail: edmarchiori@bol.com.br; Bernardes, Paulo Manuel de Barros; Gonzaga, Maria Beatriz Albano Monzo [Rede Labs/D' Or, Rio de Janeiro, RJ (Brazil)

    2004-08-01

    The purpose of this study was to assess the usefulness of magnetic resonance imaging (MRI) in the pre- and postoperative evaluation of patients with tetralogy of Fallot. Twenty patients aged 1 to 29 years were prospectively evaluated with black-blood and contrast-enhanced angiographic techniques, 11 with the classic form of tetralogy of Fallot and 9 with tetralogy of Fallot and pulmonary atresia. MRI studies provided adequate visualization of the aorta that was classified as dilated or not dilated, and definition of its position in all cases. The use of contrast-enhanced MR angiographic techniques provided excellent imaging of the main right and left pulmonary arteries. The results suggest that MRI, including contrast-enhanced angiography techniques, is a useful tool in the evaluation of patients with tetralogy of Fallot before and after cardiac surgery since it provides important anatomical information that is not always obtained with echocardiography. MRI can be considered an alternative to cardiac catheterization, particularly in the evaluation of the pulmonary vascular anatomy. (author)

  6. Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy.

    Science.gov (United States)

    Drogalis-Kim, Diana E; Reemtsen, Brian L; Reardon, Leigh Christopher

    2016-12-01

    Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.

  7. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    Energy Technology Data Exchange (ETDEWEB)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-07-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.).

  8. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    International Nuclear Information System (INIS)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-01-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.)

  9. Delayed enhancement imaging in a contemporary patient cohort following correction of tetralogy of Fallot.

    Science.gov (United States)

    Preim, Uta; Sommer, Philipp; Hoffmann, Janine; Kehrmann, Jana; Lehmkuhl, Lukas; Daehnert, Ingo; Gutberlet, Matthias; Grothoff, Matthias

    2015-10-01

    To test the hypothesis that myocardial scars after repair of tetralogy of Fallot are related to impaired cardiac function and adverse clinical outcome. A total of 53 patients were retrospectively analysed after repair of tetralogy of Fallot. The median patient age was 20 years (range 2-48). Cardiac MRI with a 1.5 T magnet included cine sequences to obtain volumes and function, phase-sensitive inversion recovery delayed enhancement imaging to detect myocardial scars, and flow measurements to determine pulmonary regurgitation fraction. In addition, clinical parameters were obtained. An overall 83% of patients were in NYHA class I. All patients with the exception of 2 (96%) had pulmonary insufficiency. Mean ejection fraction and end-diastolic volume index were 46% and 128 ml/m2 for the right ventricle and 54% and 82 ml/m² for the left ventricle, respectively. Excluding enhancement of the septal insertion and prosthetic patches, delayed enhancement was seen in 11/53 cases (21%). Delayed enhancement of the right ventricle was detected in 6/53 patients (11%) and of the left ventricle in 5/53 patients (9%). The patient group with delayed enhancement was significantly older (p=0.003), had later repair (p=0.007), and higher left ventricular myocardial mass index (p=0.009) compared with the group without delayed enhancement. This study reveals that scarring is common in patients after surgical repair of tetralogy of Fallot and is associated with older age and late repair. However, there was no difference in right ventricular function, NYHA class, or occurrence of clinically relevant arrhythmias between patients with and those without myocardial scars.

  10. Tetralogy of Fallot: Report of 30 Cases and Dental Considerations with Review of Literature

    Directory of Open Access Journals (Sweden)

    S Gedik

    2015-05-01

    Full Text Available Tetralogy of Fallot (TOF is one of the most serious cyanotic congenital heart diseases and is characterized by common systemic and oral findings. This study was carried out on 30 patients (3−13 years old with TOF and 30 subjects as a control group. The patients were examined for oral hygiene, mucous membrane, gingivae, dental caries and any hypoplasia. There appeared to be an increase in dental caries, hypoplasia and periodontal disease in the patients with TOF compared with controls. The dental aspects of TOF patients are highlighted and their dental management is discussed.

  11. FDG-PET identification of infected pulmonary artery conduit following tetralogy of fallot (TOF) repair

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Yu Yang; Williams, Hadyn; Pucar, Darko [Dept. of Radiology, Medical College of Georgia, Augusta (United States)

    2017-03-15

    Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PET as a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery.

  12. A case of uncorrected adult tetralogy of Fallot for emergency decompressive craniotomy: An anesthetic challenge!!

    Directory of Open Access Journals (Sweden)

    Amruta Vinod Hippalgaonkar

    2016-01-01

    Full Text Available Cyanotic heart disease (CHD includes those anatomical heart defects that produce a limitation in pulmonary blood flow or result in mixing of oxygenated and deoxygenated blood. Both conditions lead to decreased blood oxygen content and cyanosis. The most frequent defects seen in the outpatient adult congenital setting include tetralogy of Fallot (TOF. The cardiovascular anatomy and physiology of adult CHD is complex and requires specific knowledge of the defect and its anesthetic implications. Hence, they should receive care with multidisciplinary collaboration among anesthesiologists, cardiologists, surgeons, and intensivists. We hereby report the anesthetic management of such a case of head injury in an adult uncorrected TOF with a good outcome.

  13. FDG-PET identification of infected pulmonary artery conduit following tetralogy of fallot (TOF) repair

    International Nuclear Information System (INIS)

    Zhang, Yu Yang; Williams, Hadyn; Pucar, Darko

    2017-01-01

    Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PET as a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery

  14. A CHILD WITH TETRALOGY OF FALLOT PRESENTING WITH COMPLICATIONS OF INFECTIVE ENDOCARDITIS, CEREBRAL ABSCESS, AND UNDERNUTRITION

    Directory of Open Access Journals (Sweden)

    AA Ayu Dwi Adelia Yasmin

    2015-10-01

    Full Text Available Tetralogy of Fallot  (TOF  is a congenital heart disease  that consists of  four anatomical anomalies,namely ventricular septal defect, pulmonary stenosis or obstruction of the right ventricular outflowtract, right ventricular hypertrophy, and overriding aorta. Tetralogy of Fallot is frequently associatedwith complications that could affect morbidity and mortality because of its complex cardiac lesions.Good understanding of the natural history and complications of this disease is very important to guidethe management of patients. We reported a 4-year old girl with classic TOF with complications ofinfective  endocarditis,  cerebral  abscess,  and undernutrition. Despite  optimal medical  therapy,  thepatient?s condition showed no satisfying improvement. Since the parents were refused to take moreaggressive measure by undergoing surgical therapy, so that patient care remains focused on supportiveand palliative aspects. [MEDICINA 2015;46:37-41].Tetralogy of Fallot (TOF merupakan suatu penyakit jantung kongenital yang terdiri dari empat anomalianatomis, antara lain ventricular septal defect, pulmonary stenosis atau obstruksi pada right ventricleoutflow tract, hipertrofi ventrikel kanan, dan overriding aorta. Tetralogy of Fallot sering disertai denganberbagai  komplikasi  yang  dapat mempengaruhi morbiditas  dan mortalitas  karena  kelainan  inimerupakan kelainan kongenital kardiak yang kompleks. Pemahaman yang baik mengenai perjalananalamiah  dan komplikasi  penyakit  ini  sangat  penting untuk mengetahui  penatalaksanaan  pasien.Kami melaporkan seorang anak perempuan berusia 4 tahun dengan TOF klasik yang juga menderitakomplikasi endocarditis infektif, abses serebral, dan gizi kurang. Walaupun telah memperoleh terapimedikamentosa  yang  optimal,  kondisi  pasien  tidak menunjukkan  perbaikan  yang memuaskan.Orangtua  pasien  telah menolak  pemberian  tindakan  yang  lebih  agresif,  yaitu  dengan

  15. Frontonasal malformation with tetralogy of Fallot associated with a submicroscopic deletion of 22q11

    Energy Technology Data Exchange (ETDEWEB)

    Stratton, R.F. [South Texas Genetics Center, San Antonio, TX (United States); Payne, R.M. [Central Texas Genetics Center, Austin, TX (United States)

    1997-03-31

    We report on a 14-month-old girl with bifid nasal tip and tetralogy of Fallot. Several similar patients have been described with CNS or eye abnormalities. Chromosome analysis with FISH, using Oncor DiGeorge probes, confirmed a submicroscopic deletion of 22q11. Many patients with Shprintzen (velo-cardio-facial) syndrome have a similar deletion with conotruncal cardiac defects and an abnormal nasal shape, suggesting that a gene in this area, possibly affecting neural crest cells, influences facial and other midline development. 13 refs., 1 fig.

  16. Does early tetralogy of Fallot total correction give better final lung volumes?

    Science.gov (United States)

    Sadeghi, Hasan Allah; Miri, Seyed Reza; Bakhshandeh, Hooman; Mirmesdagh, Yalda; Paziraee, Nazita

    2013-06-01

    Pulmonary blood flow may affect lung development in adulthood. Early total correction of tetralogy of Fallot may affect development of final lung volumes. We evaluated the effect of age at total correction on lung volumes years after the operation. In a retrospective cohort study on patients with totally corrected tetralogy of Fallot (mean age, 13.40 years at the time of follow-up), forced vital capacity, slow vital capacity, forced expiratory volume in 1 s, and other parameters were measured 154.8 ± 46.25 months after the operation. Comparison were made of 3 groups: ≤2-, 2-8-, and >8-years old at the time of total correction surgery. Among 322 enrolled patients, the mean values of the follow-up spirometry results in ≤2-, 2-8-, >8-year-olds and the percentage of predicted values were respectively: vital capacity: 4.46 ± 0.57 L (107% ± 10.96%), 3.89 ± 0.58 L (91.10% ± 12.25%), 3.25 ± 0.48 L (82.35% ± 10.62%), p volume in 1 s: 4.22 ± 0.63 L (104.84% ± 13.64%), 3.66 ± 0.58 L (90.61% ± 12.59%), 3.02 ± 0.48 L (84.31% ± 12%), p volumes and capacities. It is better to consider total correction for all tetralogy of Fallot patients below 2-years old, or at least below 8-years old, if it is technically possible.

  17. B-type natriuretic peptide as prognostic marker in tetralogy of Fallot surgery.

    Science.gov (United States)

    Kapoor, Poonam Malhotra; Subramanian, Arun; Malik, Vishwas; Kiran, Usha; Velayoudham, Devagourou

    2015-02-01

    B-type natriuretic peptide has been extensively studied in patients with cardiovascular disease, but its impact on the perioperative outcome of patients with cyanotic congenital heart defects is still unclear. We assessed the perioperative changes in B-type natriuretic peptide levels and their correlation with preoperative factors and clinical outcomes in a large homogenous group of patients with tetralogy of Fallot undergoing definitive repair at a tertiary care center. A prospective study was undertaken in the cardiac operating room and intensive care unit at a single institution; 250 patients with tetralogy of Fallot undergoing intracardiac repair under cardiopulmonary bypass were studied. B-type natriuretic peptide levels were taken at 3 time points and correlated with clinical variables. Baseline B-type natriuretic peptide levels correlated with the degree of cyanosis in all 4 groups. B-type natriuretic peptide levels at 24 h after admission to the intensive care unit correlated with mortality in the adult subset of patients. B-type natriuretic peptide levels > 290 pg mL(-1) in the intensive care unit predicted an increased probability of adverse clinical outcomes. We demonstrated a rise in serum B-type natriuretic peptide levels in patients with tetralogy of Fallot undergoing definitive repair on cardiopulmonary bypass. B-type natriuretic peptide levels may be monitored to identify patients with cyanosis at increased risk of an augmented inflammatory response to cardiopulmonary bypass. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  18. Peeping through heart′s window: A case report of Fallots tetralogy

    Directory of Open Access Journals (Sweden)

    Bhowate R

    2005-03-01

    Full Text Available Tetralogy of Fallot is a congenital heart defect. A combination of four abnormalities in heart allow oxygen rich and oxygen-depleted blood to mix. The resulting low-oxygen blood then circulates through the body. A child with the condition tends to develop slowly, eat poorly and grow slowly and may experience "blue" spells on crying and dyspnea on exertion. A 12 year old child with this congenital heart defect and cleft palate with cleft lip is discussed. Details of the latest diagnostic and treatment procedures are also presented.

  19. Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution

    Directory of Open Access Journals (Sweden)

    Jonathan Lee

    2017-01-01

    Full Text Available We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.

  20. Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: a review

    NARCIS (Netherlands)

    Oosterhof, Thomas; Mulder, Barbara J. M.; Vliegen, Hubert W.; de Roos, Albert

    2006-01-01

    Cardiovascular magnetic resonance (CMR) is becoming an important tool in the clinical management of patients with congenital heart disease. Because of the diverse problems patients may face after initial correction for tetralogy of Fallot and the large amount of CMR techniques that can be applied,

  1. The Role of Cardiopulmonary Exercise Testing for Decision Making in Patients with Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Dallaire, Frederic; Wald, Rachel M; Marelli, Ariane

    2017-08-01

    Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. As a result of the surgical strategies employed at the time of initial repair, chronic pulmonary regurgitation (PR) is prevalent in this population. Despite sustained research efforts, patient selection and timing of pulmonary valve replacement (PVR) to address PR in young asymptomatic patients with repaired tetralogy of Fallot (rToF) remain a fundamental but as yet unanswered question in the field of congenital heart disease. The ability of the heart to compensate for the chronic volume overload imposed by PR is critical in the evaluation of the risks and benefits of PVR. The difficulty in clarifying the functional impact of PR on the cardiovascular capacity may be in part responsible for the uncertainty surrounding the timing of PVR. Cardiopulmonary exercise testing (CPET) may be used to assess abnormal cardiovascular response to increased physiologic demands. However, its use as a tool for risk stratification in asymptomatic adolescents and young adults with rToF is still ill-defined. In this paper, we review the role of CPET as a potentially valuable adjunct to current risk stratification strategies with a focus on asymptomatic rToF adolescents and young adults being considered for PVR. The role of maximal and submaximal exercise measurements to identify young patients with a decreased or borderline low peak VO 2 resulting from impaired ventricular function is explored. Current knowledge gaps and research perspectives are highlighted.

  2. Assessment of the ductus arteriosus in fetuses with tetralogy of Fallot and the implication for postnatal management.

    Science.gov (United States)

    Tuo, Giulia; Volpe, Paolo; Buffi, Davide; De Robertis, Valentina; Marasini, Maurizio

    2014-01-01

    To describe the antenatal and neonatal echocardiographic morphology and flow pattern of the ductus arteriosus in patients with tetralogy of Fallot. We included patients with a prenatal diagnosis of tetralogy of Fallot between January 2006 and December 2012. Among the 52 fetuses with tetralogy of Fallot the severity of right ventricular outflow obstruction was considered mild in 32, moderate in 14, and severe in 6. In the mild right ventricular outflow obstruction group (n = 32) all had normal ductal morphology and flow pattern, eight (25%) elected for termination of pregnancy and two died in the neonatal period from extracardiac causes. In the moderate right ventricular outflow obstruction group (n = 14) the fetuses had a small ductus arteriosus with antegrade but abnormal flow velocity, one (7%) elected for termination of pregnancy. Immediately after birth the ductus arteriosus was very small or already closed at echocardiographic examination. Two out of 13 patients (15%) developed severe hypoxic spells and underwent modified Blalock-Taussig shunt during the neonatal period. Six fetuses were considered to have severe right ventricular outflow obstruction with flow reversal in the ductus arteriosus, three (50%) of whom elected for termination of pregnancy. The other three newborns underwent modified Blalock-Taussig shunt. In fetuses with tetralogy of Fallot, ductal diameter can be reduced even up to prenatal closure. Prenatal ductal morphology assessment may be useful for improving management of patients with moderate right ventricular outflow obstruction and small ductus arteriosus who may become cyanotic at birth. © 2013 Wiley Periodicals, Inc.

  3. Evaluation of tricuspid annular plane systolic excursion measured with cardiac MRI in children with tetralogy of Fallot.

    Science.gov (United States)

    Soslow, Jonathan H; Usoro, Emem; Wang, Li; Parra, David A

    2016-04-01

    Aneurysmal dilation of the right ventricular outflow tract complicates assessment of right ventricular function in patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is commonly used to estimate ejection fraction. We hypothesised that tricuspid annular plane systolic excursion measured by cardiac MRI approximates global and segmental right ventricular function, specifically right ventricular sinus ejection fraction, in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion was measured retrospectively on cardiac MRIs in 54 patients with repaired tetralogy of Fallot. Values were compared with right ventricular global, sinus, and infundibular ejection fractions. Tricuspid annular plane systolic excursion was indexed to body surface area, converted into a fractional value, and converted into published paediatric Z-scores. Tricuspid annular plane systolic excursion measurements had good agreement between observers. Right ventricular ejection fraction did not correlate with the absolute or indexed tricuspid annular plane systolic excursion and correlated weakly with fractional tricuspid annular plane systolic excursion (r=0.41 and p=0.002). Segmental right ventricular function did not appreciably improve correlation with any of the tricuspid annular plane systolic excursion measures. Paediatric Z-scores were unable to differentiate patients with normal and abnormal right ventricular function. Tricuspid annular plane systolic excursion measured by cardiac MRI correlates poorly with global and segmental right ventricular ejection fraction in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is an unreliable approximation of right ventricular function in this patient population.

  4. Oral and systemic manifestations, and dental management of a pediatric patient with Tetralogy of Fallot. A case report.

    Directory of Open Access Journals (Sweden)

    Carmen Ayala

    2016-03-01

    Full Text Available Introduction: Tetralogy of Fallot is a congenital heart disease and the most common cyanotic heart defect in children. It is clinically characterized by a ventricular septal defect, pulmonary stenosis, overriding aorta over ventricular septal defect and right ventricular hypertrophy. There is little or no information about the oral manifestations in patients with this pathology. A report and discussion of a pediatric patient diagnosed with Tetralogy of Fallot, its clinical manifestations, oral findings and dental management are presented. Case Report: A four-year-old male patient diagnosed with Tetralogy of Fallot and epileptic attacks. The patient has deciduous teeth with many severe early childhood caries, stomatitis and cyanotic mucous membranes, root fragments, periapical abscess and noticeable enamel hypoplasia. Conclusions: In agreement with other authors, children with systemic diseases such as Tetralogy of Fallot have a higher rate of caries, poor oral hygiene, high susceptibility to other infections and bacterial endocarditis, cyanotic mucous membranes and enamel hypoplasia. Primary prevention is critical, proper dental hygiene, regular dental check-ups and the use of antibiotic prophylaxis are particularly important, especially in high-risk patients.

  5. Trends in pulmonary valve replacement in children and adults with tetralogy of fallot.

    Science.gov (United States)

    O'Byrne, Michael L; Glatz, Andrew C; Mercer-Rosa, Laura; Gillespie, Matthew J; Dori, Yoav; Goldmuntz, Elizabeth; Kawut, Steven; Rome, Jonathan J

    2015-01-01

    Operative correction of tetralogy of Fallot frequently results in pulmonary insufficiency and chronic volume overload, which have been linked to increased risk for adverse outcomes. No consensus recommendations for the timing of pulmonary valve replacement (PVR) exist. The aim of this study was to examine the pattern of PVR in the United States from 2004 to 2012. The Pediatric Health Information Systems database was used to perform an observational study of children and adults ≥10 years of age with diagnoses of tetralogy of Fallot who underwent PVR at 35 centers in the United States from 2004 and 2012, to assess the rate of PVR and the age at which is performed. Mixed-effects multivariate regression was used to account for patient-level covariates and center-level covariance. Additional analyses assessed for trends in cost, hospital length of stay (LOS), intensive care unit LOS, and in-hospital mortality over the study period. In total, 799 subjects at 35 centers underwent PVR over the study period. The number of PVRs performed per year increased significantly over the study period. There was significant between-center heterogeneity in age at PVR (p optimal timing of PVR. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Ductal Stent Implantation in Tetralogy of Fallot with Aortic Arch Abnormality

    Science.gov (United States)

    Ergul, Yakup; Saygi, Murat; Ozyilmaz, Isa; Guzeltas, Alper; Odemis, Ender

    2015-01-01

    Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion. We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion. PMID:26175649

  7. Extreme Tetralogy of Fallot With Polycythemia in a Ferret (Mustela putorius furo).

    Science.gov (United States)

    Dias, Sara; Planellas, Marta; Canturri, Albert; Martorell, Jaume

    2017-06-01

    A 5-month-old, intact male ferret (Mustela putorius furo) was presented with apathy, tachypnea and exercise intolerance. On initial physical examination, tachypnea and cyanosis were the two primary clinical signs detected. The complete blood count also revealed a severe polycythemia, with lymphopenia, neutrophilia and eosinophilia. Further diagnostic imaging tests were performed. The thoracic radiographs revealed cardiomegaly and a diffuse alveolar pulmonary pattern. Consequently, an echocardiography was performed and showed an interventricular septal defect, pulmonic artery occlusion, overriding of the aorta and right ventricle hypertrophy. All abnormalities were compatible with a tetralogy of Fallot (TOF). As a treatment plan, a phlebotomy was performed and the ferret was posteriorly maintained with oxygen and fluid therapy. The following day, the hematocrit decreased 11% and the respiratory distress improved. Four days later, the ferret got clinically worse and was euthanized. A necropsy was performed and confirmed the presence of an extreme TOF with a diffused interstitial pneumonia secondary to Pneumocystis carinii. To the author's knowledge, this is the first report of an extreme tetralogy of Fallot with polycythemia in a ferret. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Ventricular kinetic energy may provide a novel noninvasive way to assess ventricular performance in patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Jeong, Daniel; Anagnostopoulos, Petros V; Roldan-Alzate, Alejandro; Srinivasan, Shardha; Schiebler, Mark L; Wieben, Oliver; François, Christopher J

    2015-05-01

    Ventricular kinetic energy measurements may provide a novel imaging biomarker of declining ventricular efficiency in patients with repaired tetralogy of Fallot. Our purpose was to assess differences in ventricular kinetic energy with 4-dimensional flow magnetic resonance imaging between patients with repaired tetralogy of Fallot and healthy volunteers. Cardiac magnetic resonance, including 4-dimensional flow magnetic resonance imaging, was performed at rest in 10 subjects with repaired tetralogy of Fallot and 9 healthy volunteers using clinical 1.5T and 3T magnetic resonance imaging scanners. Right and left ventricular kinetic energy (KERV and KELV), main pulmonary artery flow (QMPA), and aortic flow (QAO) were quantified using 4-dimensional flow magnetic resonance imaging data. Right and left ventricular size and function were measured using standard cardiac magnetic resonance techniques. Differences in peak systolic KERV and KELV in addition to the QMPA/KERV and QAO/KELV ratios between groups were assessed. Kinetic energy indices were compared with conventional cardiac magnetic resonance parameters. Peak systolic KERV and KELV were higher in patients with repaired tetralogy of Fallot (6.06 ± 2.27 mJ and 3.55 ± 2.12 mJ, respectively) than in healthy volunteers (5.47 ± 2.52 mJ and 2.48 ± 0.75 mJ, respectively), but were not statistically significant (P = .65 and P = .47, respectively). The QMPA/KERV and QAO/KELV ratios were lower in patients with repaired tetralogy of Fallot (7.53 ± 5.37 mL/[cycle mJ] and 9.65 ± 6.61 mL/[cycle mJ], respectively) than in healthy volunteers (19.33 ± 18.52 mL/[cycle mJ] and 35.98 ± 7.66 mL/[cycle mJ], respectively; P tetralogy of Fallot. Quantification of ventricular kinetic energy in patients with repaired tetralogy of Fallot is a new observation. Future studies are needed to determine whether changes in ventricular kinetic energy can provide earlier evidence of ventricular dysfunction and guide future medical and

  9. The right ventricular outflow tract in tetralogy of Fallot by preoperative cardiac angiography

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yong Chul; Lee, Jung Sook; Yoo, Soo Woong; Yu, Hoe Sung; Kim, Han Suk [National Medical Center, Seoul (Korea, Republic of)

    1979-06-15

    The underdevelopment of the right ventricular outflow tract is the essence among the main components of the tetralogy of Fallot. The detail and exact information about the states of the outflow tract in each patients with tetralogy of Fallot are very important to cardiac surgery. Fourteen patients of tetralogy of Fallet (12 undergone open heart surgery and 2, palliative) are available for their clinical chart and for cardiac angiography reviews. Analysis of them focusing the right ventricular outflow tract discloses the following results: 1. Average ratios of the systole to diastole of the pulmonary infundibulum are 0.63 {+-} 0.03 with the range of 0.44 to 1.0 cm P-A, and 0.64 {+-} 0.12 with the range of 0.5 to 0.83 on lateral projections. These results indicate relatively fixed infundibular stenosis of TOF. 2. Average infundibular systole to pulmonary bulb ratios are 0.24 {+-} 0.05 on P-A, and 0.36 {+-} 0.17 on lateral projections, which mean the underdeveloped and narrowed infundibulum more than the pulmonary truck in TOF. 3. Average pulmonary to aortic bulb ratios are 0.59 {+-} 0.01 on P-A, 0.54 {+-} 0.01 on lateral projections. These suggest hypoplasia of the pulmonary truck and anteriorly displaced conus septum and truncus septum from a developmental point of view. 4. The overriding degree of the aorta in our series are from 25% to 50% by Kjellberg et al classification.

  10. The right ventricular outflow tract in tetralogy of Fallot by preoperative cardiac angiography

    International Nuclear Information System (INIS)

    Lee, Yong Chul; Lee, Jung Sook; Yoo, Soo Woong; Yu, Hoe Sung; Kim, Han Suk

    1979-01-01

    The underdevelopment of the right ventricular outflow tract is the essence among the main components of the tetralogy of Fallot. The detail and exact information about the states of the outflow tract in each patients with tetralogy of Fallot are very important to cardiac surgery. Fourteen patients of tetralogy of Fallet (12 undergone open heart surgery and 2, palliative) are available for their clinical chart and for cardiac angiography reviews. Analysis of them focusing the right ventricular outflow tract discloses the following results: 1. Average ratios of the systole to diastole of the pulmonary infundibulum are 0.63 ± 0.03 with the range of 0.44 to 1.0 cm P-A, and 0.64 ± 0.12 with the range of 0.5 to 0.83 on lateral projections. These results indicate relatively fixed infundibular stenosis of TOF. 2. Average infundibular systole to pulmonary bulb ratios are 0.24 ± 0.05 on P-A, and 0.36 ± 0.17 on lateral projections, which mean the underdeveloped and narrowed infundibulum more than the pulmonary truck in TOF. 3. Average pulmonary to aortic bulb ratios are 0.59 ± 0.01 on P-A, 0.54 ± 0.01 on lateral projections. These suggest hypoplasia of the pulmonary truck and anteriorly displaced conus septum and truncus septum from a developmental point of view. 4. The overriding degree of the aorta in our series are from 25% to 50% by Kjellberg et al classification.

  11. Effect of Losartan on Right Ventricular Dysfunction: Results From the Double-Blind, Randomized REDEFINE Trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) in Adults With Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Bokma, Jouke P; Winter, Michiel M; van Dijk, Arie P; Vliegen, Hubert W; van Melle, Joost P; Meijboom, Folkert J; Post, Martijn C; Berbee, Jacqueline K; Boekholdt, S Matthijs; Groenink, Maarten; Zwinderman, Aeilko H; Mulder, Barbara J M; Bouma, Berto J

    2018-04-03

    The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot. The REDEFINE trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) is an investigator-initiated, multicenter, prospective, 1:1 randomized, double-blind, placebo-controlled study. Adults with repaired tetralogy of Fallot and RV dysfunction (RV ejection fraction [EF] 0.30 for all). In predefined subgroup analyses, losartan did not have a statistically significant impact on RV EF in subgroups with symptoms, restrictive RV, RV EFtetralogy of Fallot. Future larger studies may determine whether there might be a role for losartan in specific vulnerable subgroups. URL: https://www.clinicaltrials.gov. Unique identifier: NCT02010905. © 2017 American Heart Association, Inc.

  12. Uses of Magnetic Resonance in Post-Surgical Evaluation of Patients with Tetralogy of Fallot

    International Nuclear Information System (INIS)

    Abad, Pedro; Delgado Jorge Andres; Llano Serna, Juan Fernando

    2008-01-01

    The number of patients with tetralogy of Fallot who survive complete reparative surgery is increasing. For this reason the late complications of this procedure, such as pulmonary regurgitation, right ventricular dysfunction, arrhythmias, pulmonary artery stenosis or pseudo aneurysms outflow tract, are becoming more common in clinical practice. Magnetic resonance imaging is the method of choice for the global evaluation of these patients because the ability to evaluate the morphology, quantifying right ventricular function and characterization of the anatomy and function of the pulmonary valve. The knowledge of the embriopathology and the anatomy of this congenital heart disease, and the different interventions, palliative or corrective and the complications are essential for the proper interpretation of the images and making therapeutic decisions.

  13. [The complex origin of ventricular tachycardia after the total correction of tetralogy of Fallot].

    Science.gov (United States)

    Ressia, L; Graffigna, A; Salerno-Uriarte, J A; Viganò, M

    1993-09-01

    Two patients underwent surgical treatment of ventricular tachycardia after repair of tetralogy of Fallot. Both patients had right bundle branch block, moderate pulmonary valve incompetence and right ventricular dilatation, and were refractory to electrophysiologically guided drug therapy. Both patients underwent intraoperative epicardial mapping, which located the arrhythmogenic focus on the right ventricular outflow tract, on the border of the previous ventriculotomy. In one patient removal of the previous scar and endocardial cryoablation was successful in ablating the arrhythmia. In the other, the same procedure was only temporarily effective. VT recurred and was subsequently identified at the superior border of the closed ventricular septal defect. It was ablated by means of transcatheter radiofrequency. While VT from foci located on the right ventricular free wall can be easily detected and ablated, septal origin of VT requires extensive preoperative and intraoperative electrophysiological evaluation and may necessitate combined surgical and transcatheter procedures.

  14. 22q11.2 deletion status and disease burden in children and adolescents with tetralogy of Fallot.

    Science.gov (United States)

    Mercer-Rosa, Laura; Paridon, Stephen M; Fogel, Mark A; Rychik, Jack; Tanel, Ronn E; Zhao, Huaqing; Zhang, Xuemei; Yang, Wei; Shults, Justine; Goldmuntz, Elizabeth

    2015-02-01

    Patients with repaired tetralogy of Fallot experience variable outcomes for reasons that are incompletely understood. We hypothesize that genetic variants contribute to this variability. We sought to investigate the association of 22q11.2 deletion status with clinical outcome in patients with repaired tetralogy of Fallot. We performed a cross-sectional study of tetralogy of Fallot subjects who were tested for 22q11.2 deletion, and underwent cardiac magnetic resonance, exercise stress test, and review of medical history. We studied 165 subjects (12.3±3.1 years), of which 30 (18%) had 22q11.2 deletion syndrome (22q11.2DS). Overall, by cardiac magnetic resonance the right ventricular ejection fraction was 60±8%, pulmonary regurgitant fraction was 34±17%, and right ventricular end-diastolic volume was 114±39 cc/m(2). On exercise stress test, maximum oxygen consumption was 76±16% predicted. Despite comparable right ventricular function and pulmonary regurgitant fraction, on exercise stress test the 22q11.2DS had significantly lower percent predicted: forced vital capacity (61.5±16 versus 80.5±14; Ptetralogy of Fallot. These findings may provide avenues for intervention to improve outcomes, and should be re-evaluated longitudinally because these associations may become more pronounced with time. © 2015 American Heart Association, Inc.

  15. Following up adult patients with tetralogy of fallot: The role of echocardiography.

    Science.gov (United States)

    Daraban, Ana Maria; Jurcuţ, Ruxandra; Bădilă, Elisabeta; Bartoş, Daniela; Dan, Gheorghe Andrei

    2017-02-01

    Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases. With surgical repair and the advances in postoperative care, contemporary mortality has dramatically improved and an increasing number of patients survive into adulthood, leading to a growing number of adult TOF. However, residual anatomic and hemodynamic abnormalities are encountered in nearly all patients, making follow-up mandatory. Furthermore, mortality starts to increase 25 years after surgery, emphasizing that, in adult TOF, closer monitoring is necessary. We review here the role of echocardiography in the follow-up of the TOF patients, emphasizing the role of multiple echocardiographic techniques. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:79-95, 2017. © 2016 Wiley Periodicals, Inc.

  16. Revisiting the utility of technical performance scores following tetralogy of Fallot repair.

    Science.gov (United States)

    Lodin, Daud; Mavrothalassitis, Orestes; Haberer, Kim; Sunderji, Sherzana; Quek, Ruben G W; Peyvandi, Shabnam; Moon-Grady, Anita; Karamlou, Tara

    2017-08-01

    Although an important quality metric, current technical performance scores may not be generalizable and may omit operative factors that influence outcomes. We examined factors not included in current technical performance scores that may contribute to increased postoperative length of stay, major complications, and cost after primary repair of tetralogy of Fallot. This is a retrospective single site study of patients younger than age 2 years with tetralogy of Fallot undergoing complete repair between 2007 and 2015. Medical record data and discharge echocardiograms were reviewed to ascertain component and composite technical performance scores. Primary outcomes included postoperative length of stay, major complications, and total hospital costs. Multivariable logistic and linear regression identified determinants of each outcome. Patient population (n = 115) had a median postoperative length of stay of 8 days (interquartile range, 6-10 days), and a median total cost of $71,147. Major complications occurred in 33 patients (29%) with 1 death. Technical performance scores assigned were optimum in 28 patients (25%), adequate in 59 patients (52%), and inadequate in 26 patients (23%). Neither technical performance score components nor composite scores were associated with increased postoperative length of stay. Optimum or adequate repairs versus inadequate had equal risk of a complication (P = .79), and equivalent mean total cost ($100,000 vs $187,000; P = .25). Longer cardiopulmonary bypass time per 1-minute increase (P technical performance scores were not associated with selected outcomes in our postoperative population. Although returning to bypass and bypass length are not included as components in the current score, these are important factors influencing complications and resource use in our population. Revisions anticipated from a prospective trial should consider including these variables. Copyright © 2017 The American Association for Thoracic Surgery

  17. Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Ho Jin Kim

    2017-04-01

    Full Text Available Background: Cardiac computed tomography (CT has emerged as an alternative to magnetic resonance imaging (MRI for ventricular volumetry. However, the clinical use of cardiac CT requires external validation. Methods: Both cardiac CT and MRI were performed prior to pulmonary valve implantation (PVI in 11 patients (median age, 19 years who had undergone total correction of tetralogy of Fallot during infancy. The simplified contouring method (MRI and semiautomatic 3-dimensional region-growing method (CT were used to measure ventricular volumes. Results: All volumetric indices measured by CT and MRI generally correlated well with each other, except for the left ventricular end-systolic volume index (LV-ESVI, which showed the following correlations with the other indices: the right ventricular end-diastolic volume index (RV-EDVI (r=0.88, p<0.001, the right ventricular end-systolic volume index (RV-ESVI (r=0.84, p=0.001, the left ventricular end-diastolic volume index (LV-EDVI (r=0.90, p=0.001, and the LV-ESVI (r=0.55, p=0.079. While the EDVIs measured by CT were significantly larger than those measured by MRI (median RV-EDVI: 197 mL/m 2 vs. 175 mL/m 2 , p=0.008; median LV-EDVI: 94 mL/m 2 vs. 92 mL/m 2 , p=0.026, no significant differences were found for the RV-ESVI or LV-ESVI. Conclusion: The EDVIs measured by cardiac CT were greater than those measured by MRI, whereas the ESVIs measured by CT and MRI were comparable. The volumetric characteristics of these 2 diagnostic modalities should be taken into account when indications for late PVI after tetralogy of Fallot repair are assessed.

  18. Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot.

    Science.gov (United States)

    Kim, Ho Jin; Mun, Da Na; Goo, Hyun Woo; Yun, Tae-Jin

    2017-04-01

    Cardiac computed tomography (CT) has emerged as an alternative to magnetic resonance imaging (MRI) for ventricular volumetry. However, the clinical use of cardiac CT requires external validation. Both cardiac CT and MRI were performed prior to pulmonary valve implantation (PVI) in 11 patients (median age, 19 years) who had undergone total correction of tetralogy of Fallot during infancy. The simplified contouring method (MRI) and semiautomatic 3-dimensional region-growing method (CT) were used to measure ventricular volumes. All volumetric indices measured by CT and MRI generally correlated well with each other, except for the left ventricular end-systolic volume index (LV-ESVI), which showed the following correlations with the other indices: the right ventricular end-diastolic volume index (RV-EDVI) (r=0.88, p<0.001), the right ventricular end-systolic volume index (RV-ESVI) (r=0.84, p=0.001), the left ventricular end-diastolic volume index (LV-EDVI) (r=0.90, p=0.001), and the LV-ESVI (r=0.55, p=0.079). While the EDVIs measured by CT were significantly larger than those measured by MRI (median RV-EDVI: 197 mL/m 2 vs. 175 mL/m 2 , p=0.008; median LV-EDVI: 94 mL/m 2 vs. 92 mL/m 2 , p=0.026), no significant differences were found for the RV-ESVI or LV-ESVI. The EDVIs measured by cardiac CT were greater than those measured by MRI, whereas the ESVIs measured by CT and MRI were comparable. The volumetric characteristics of these 2 diagnostic modalities should be taken into account when indications for late PVI after tetralogy of Fallot repair are assessed.

  19. Diffuse myocardial fibrosis following tetralogy of Fallot repair: a T1 mapping cardiac magnetic resonance study

    International Nuclear Information System (INIS)

    Kozak, Marcelo F.; Yoo, Shi-Joon; Seed, Mike; Grosse-Wortmann, Lars; Redington, Andrew; Greiser, Andreas

    2014-01-01

    Adverse ventricular remodeling after tetralogy of Fallot (TOF) repair is associated with diffuse myocardial fibrosis. The goal of this study was to measure post-contrast myocardial T1 in pediatric patients after TOF repair as surrogates of myocardial fibrosis. Children after TOF repair who underwent cardiac magnetic resonance imaging with T1 mapping using the modified look-locker inversion recovery (MOLLI) sequence were included. In addition to routine volumetric and flow data, we measured post-contrast T1 values of the basal interventricular septum, the left ventricular (LV) lateral wall, and the inferior and anterior walls of the right ventricle (RV). Results were compared to data from age-matched healthy controls. The scans of 18 children who had undergone TOF repair and 12 healthy children were included. Post-contrast T1 values of the left ventricular lateral wall (443 ± 54 vs. 510 ± 77 ms, P = 0.0168) and of the right ventricular anterior wall (333 ± 62 vs. 392 ± 72 ms, P = 0.0423) were significantly shorter in children with TOF repair than in controls, suggesting a higher degree of fibrosis. In children with TOF repair, but not in controls, post-contrast T1 values were shorter in the right ventricle than the left ventricle and shorter in the anterior wall of the right ventricle than in the inferior segments. In the TOF group, post-contrast T1 values of the RV anterior wall correlated with the RV end-systolic volume indexed to body surface area (r = 0.54; r 2 = 0.30; P = 0.0238). In children who underwent tetralogy of Fallot repair the myocardium of both ventricles appears to bear an abnormally high fibrosis burden. (orig.)

  20. Diffuse myocardial fibrosis following tetralogy of Fallot repair: a T1 mapping cardiac magnetic resonance study

    Energy Technology Data Exchange (ETDEWEB)

    Kozak, Marcelo F.; Yoo, Shi-Joon; Seed, Mike; Grosse-Wortmann, Lars [The Hospital for Sick Children, University of Toronto, Labatt Family Heart Centre in the Department of Paediatrics and Department of Diagnostic Imaging, Toronto (Canada); Redington, Andrew [The Hospital for Sick Children, University of Toronto, Labatt Family Heart Centre in the Department of Paediatrics, Toronto (Canada); Greiser, Andreas [Siemens AG Healthcare Sector, Erlangen (Germany)

    2014-04-15

    Adverse ventricular remodeling after tetralogy of Fallot (TOF) repair is associated with diffuse myocardial fibrosis. The goal of this study was to measure post-contrast myocardial T1 in pediatric patients after TOF repair as surrogates of myocardial fibrosis. Children after TOF repair who underwent cardiac magnetic resonance imaging with T1 mapping using the modified look-locker inversion recovery (MOLLI) sequence were included. In addition to routine volumetric and flow data, we measured post-contrast T1 values of the basal interventricular septum, the left ventricular (LV) lateral wall, and the inferior and anterior walls of the right ventricle (RV). Results were compared to data from age-matched healthy controls. The scans of 18 children who had undergone TOF repair and 12 healthy children were included. Post-contrast T1 values of the left ventricular lateral wall (443 ± 54 vs. 510 ± 77 ms, P = 0.0168) and of the right ventricular anterior wall (333 ± 62 vs. 392 ± 72 ms, P = 0.0423) were significantly shorter in children with TOF repair than in controls, suggesting a higher degree of fibrosis. In children with TOF repair, but not in controls, post-contrast T1 values were shorter in the right ventricle than the left ventricle and shorter in the anterior wall of the right ventricle than in the inferior segments. In the TOF group, post-contrast T1 values of the RV anterior wall correlated with the RV end-systolic volume indexed to body surface area (r = 0.54; r{sup 2} = 0.30; P = 0.0238). In children who underwent tetralogy of Fallot repair the myocardium of both ventricles appears to bear an abnormally high fibrosis burden. (orig.)

  1. Tetralogy of fallot (cyanotic cardiac malformation), trends and variation in a population based study

    International Nuclear Information System (INIS)

    Arif, A.; Zehra, S.; Qamarunnisa, S.; Azhar, A.

    2012-01-01

    Congenital heart diseases (CHDs) are a major threat worldwide for children below the age of five. It has high mortality and morbidity ratio. CHDs can be classified as cyanotic and acyanotic. Cyanotic cardiac malformation accounts 25% of all congenital heart diseases and Tetralogy of Fallot (TOF) is .the most common form of cyanotic CHD. Tetralogy of Fallot is a combination of four anatomical abnormalities. These include a large ventricular septal defect (VSD), right ventricular outflow tract and pulmonary valve obstruction, right ventricular hypertrophy, and over-riding of the aorta. The current investigation was conducted over the span of 2.5 years on the patients presented in OPD and hospitalized at various pediatric cardiology centers. A detailed family history was taken to elucidate the genetic and environmental factors. Diagnosis was confirmed by the cardiologist based on examination of cardiac murmur, chest X-ray, fetal echocardiography (ECG), complete blood count (CBC), echocardiograms and/or echocardiogram (ECHO) reports, cardiac catherization reports, operative notes and MRI of heart, if applicable. This study recruited a cohort of 268 patients and 140 controls, healthy unrelated individuals. The mean age for patients was 2.97+-1.21 and that for controls was 3.14+-2.44 years. Males were shown to have a higher rate of incidence than females (1.26:1) in our population. The genes that are mainly involved are NKX 2.5, GAT A 4, TBX5, JAGI, ZFPM2 and VEGF. The present study focuses on NKX 2.5 and its mutations in our population. (author)

  2. The role of cardiovascular magnetic resonance in assessment of patients after radical surgical correction of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Genova, K.; Dasheva, A.

    2014-01-01

    Surgical management of tetralogy of Fallot (TOF) results in residual anatomic and functional abnormalities in majority of patients. Primarily considered as a benign pulmonary regurgitation with the time results in right ventricular overload, loses the compensatory mechanisms and irreversible right ventricular dilatation and dysfunction. Cardiovascular magnetic resonance (CMR) has evolved during the last two decades as the reference standard imaging modality to assess the anatomic and functional changes in patients with repaired TOF. This article reviews the role of CMR to assess the right ventricular function and to evaluate the degree of pulmonary regurgitation and comments some technical aspects, based to our experience. Key words: Tetralogy of Fallot. Cardiovascular Magnetic Resonance. Pulmonary regurgitation. Right ventricular assessment

  3. Usefulness of right ventricular and right atrial two-dimensional speckle tracking strain to predict late arrhythmic events in adult patients with repaired Tetralogy of Fallot.

    Science.gov (United States)

    Timóteo, Ana T; Branco, Luísa M; Rosa, Sílvia A; Ramos, Ruben; Agapito, Ana F; Sousa, Lídia; Galrinho, Ana; Oliveira, José A; Oliveira, Mário M; Ferreira, Rui C

    2017-01-01

    To determine whether right ventricular and/or atrial speckle tracking strain is associated with previous arrhythmic events in patients with repaired tetralogy of Fallot. We studied right ventricular and atrial strain in 100 consecutive patients with repaired tetralogy of Fallot referred for routine echocardiographic evaluation. Patients were divided into two groups, one with previous documentation of arrhythmias (n=26) and one without arrhythmias, in a median follow-up of 22 years. Patients with arrhythmias were older (pFallot, although a prospective study is required. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. An innovative simple technique of blood conservation in adult patients with tetralogy of Fallot and severely raised hemoglobin.

    Science.gov (United States)

    Neema, Praveen Kumar; Manikandan, Sethuraman; Rathod, Ramesh Chandra

    2007-12-01

    The adult patients of tetralogy of Fallot often present with high hemoglobin levels. High hemoglobin and hematocrit on cardiopulmonary bypass (CPB) are associated with increased hemolysis, plasma free hemoglobin, renal dysfunction or failure, postoperative bleeding, exploration for bleeding, and increased requirement of allogeneic blood and blood products. Despite the presence of high hemoglobin and its association with adverse outcome, blood conservation is rarely practiced in these patients because of the fear of possible hemodynamic instability, and hypoxemic spell. We describe an innovative, simple technique of blood conservation for adult patients of tetralogy of Fallot with severely raised hemoglobin. With this technique, hemoglobin can be normalized on CPB; moreover, there is no fear of hypoxemic spell or hemodynamic instability. Furthermore, the blood conserved is readily available for transfusion in the perioperative period, if needed.

  5. Outcomes of Pulmonary Valve Replacement for Correction Pulmonary Insufficiency after Primary Repair of Tetralogy of Fallot (TOF)

    OpenAIRE

    Mohammad Abbassi Teshnisi; Aliasghar Moeinipour; Hamid Hoseinikhah; Seyedeh Zahra Aemmi; Shahla Shirin Bahador; Nahid Zirak

    2016-01-01

    Background Total correction of Tetralogy of Fallot (TOF) anomaly in early childhood has been practiced in many centers with good results, but in some of patients after few years sever Pulmonary valve insufficiency occurred. Materials and Methods At a cross- sectional study from January 2015 to January 2016, 10 patients who had history of primary repair of TOF with free pulmonary insufficiency (PI) that underwent of pulmonary valve replacement (PVR) with bioprosthetic valves were evaluated. Re...

  6. Fetal MRI correlates with postnatal CT angiogram assessment of pulmonary anatomy in tetralogy of Fallot with absent pulmonary valve.

    Science.gov (United States)

    Sun, Heather Y; Boe, Justin; Rubesova, Erika; Barth, Richard A; Tacy, Theresa A

    2014-01-01

    In tetralogy of Fallot with absent pulmonary valve, pulmonary stenosis and regurgitation results in significant pulmonary artery dilatation. Branch pulmonary artery dilatation often compresses the tracheobronchial tree, causing fluid trapping in fetal life and air trapping and/or atelectasis after birth. Prenatal diagnosis predicts poor prognosis, which depends on the degree of respiratory insufficiency from airway compromise and lung parenchymal disease after birth. Fetal magnetic resonance imaging (MRI) has been useful in evaluating the effects of congenital lung lesions on lung development and indicating severity of pulmonary hypoplasia. This report is the first demonstrating the utility of fetal MRI in tetralogy of Fallot/absent pulmonary valve patients, which predicted postnatal pulmonary artery size and visualized airway compression and lung parenchymal lesions. The distribution of lobar fluid trapping on fetal MRI correlated with air trapping on postnatal computed tomography angiogram. © 2013 Wiley Periodicals, Inc.

  7. Early primary repair of tetralogy of fallot in neonates and infants less than four months of age.

    Science.gov (United States)

    Tamesberger, Melanie I; Lechner, Evelyn; Mair, Rudolf; Hofer, Anna; Sames-Dolzer, Eva; Tulzer, Gerald

    2008-12-01

    The ideal age for correction of tetralogy of Fallot is still under discussion. The aim of this study was to analyze morbidity and mortality in patients who underwent early primary repair of tetralogy of Fallot at the age of less than 4 months and to assess whether neonates, who needed early repair within the first 4 weeks of life, faced an increased risk. From 1995 to 2006, 90 consecutive patients with tetralogy of Fallot and pulmonary stenosis underwent early primary repair. Patient charts were analyzed retrospectively for two groups: group A, 25 neonates younger than 28 days who needed early operation owing to duct-dependent pulmonary circulation or severe hypoxemia; and group B, 65 infants younger than 4 months of age who underwent elective early repair. There was no 30-day mortality; late mortality was 2% after a median follow-up time of 4.7 years. Seven of 88 patients (8%) needed reoperation and twelve of 88 patients (14%) needed reintervention. Groups A and B did not differ significantly in terms of intensive care unit stay, days of mechanical ventilation, overall hospital stay, major or minor complications, or reoperation. Significant differences were found in a more frequent use of a transannular patch (p = 0.045) and more reinterventions (p = 0.046) in group A. Early primary repair of tetralogy of Fallot can be performed safely and effectively in infants younger than 4 months of age and even in neonates younger than 28 days with duct-dependent pulmonary circulation or severe hypoxemia.

  8. Right ventricular outflow tract stent versus BT shunt palliation in Tetralogy of Fallot.

    Science.gov (United States)

    Quandt, Daniel; Ramchandani, Bharat; Penford, Gemma; Stickley, John; Bhole, Vinay; Mehta, Chetan; Jones, Timothy; Barron, David James; Stumper, Oliver

    2017-12-01

    This study sets out to compare morbidity, mortality and reintervention rates after stenting of the right ventricular outflow tract (RVOT) versus modified Blalock-Taussig shunt (mBTS) for palliation in patients with tetralogy of Fallot (ToF)-type lesions. Retrospective case review study evaluating 101 patients (64 males) with ToF lesions who underwent palliation with either mBTS (n=41) or RVOT stent (n=60) to augment pulmonary blood flow over a 10-year period. Procedure-related morbidity, mortality and reintervention rates were assessed and compared. Admission rate to paediatric intensive care unit (PICU) was lower in the RVOT stent group (22% vs 100%; pFallot-type lesions can be accomplished safely, with lower PICU admission rate, a shorter hospital length of stay and shorter duration of palliation until complete repair compared with mBTS palliation. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  9. Twenty Years of Anesthetic and Perioperative Management of Patients With Tetralogy of Fallot With Absent Pulmonary Valve.

    Science.gov (United States)

    Jochman, John D; Atkinson, Douglas B; Quinonez, Luis G; Brown, Morgan L

    2017-06-01

    Review the authors' institutional experience of the induction and perioperative airway management of children with tetralogy of Fallot with an absent pulmonary valve. Retrospective chart review. Large academic children's hospital. Patients with the diagnosis of tetralogy of Fallot with absent pulmonary valve undergoing primary cardiac repair over a 20-year period. None. Forty-four patients were identified with tetralogy of Fallot with an absent pulmonary valve from January 1995 through August 2014. Forty-two patients (95%) required surgery in their first year of life. Sixteen patients (36%) required mechanical ventilation preoperatively, including 11 neonates. Of the 28 patients not intubated preoperatively, only 1 (3.8%) exhibited minor airway obstruction following induction that was managed uneventfully. All intubations in the operating room were performed in the supine position. Five patients who were mechanically ventilated preoperatively in a lateral or prone position were supinated in the operating room without significant cardiopulmonary compromise. There were no patients who suffered cardiopulmonary arrest or required mechanical circulatory support. The median time to extubation was 2 days (range 1-13 days) in those patients who were not mechanically ventilated prior to their surgery. Ultimately, 5 patients required tracheostomy and 1 patient underwent lobectomy. Although, there was no mortality at 30 days, 4 children died within 1 year of their surgery. All the children who died had a genetic syndrome and required mechanical ventilation preoperatively. While there are theoretical concerns specific to the induction and airway management of tetralogy of Fallot with an absent pulmonary valve, there were no episodes of cardiorespiratory arrest or extracorporeal membrane oxygenation in the authors' series. Neonatal age at the time of surgery, preoperative need for mechanical ventilation, and concomitant genetic syndromes are risk factors for respiratory

  10. Late gadolinium enhancement and adverse outcomes in a contemporary cohort of adult survivors of tetralogy of Fallot.

    Science.gov (United States)

    Dobson, Richard J; Mordi, Ify; Danton, Mark H; Walker, Niki L; Walker, Hamish A; Tzemos, Nikolaos

    2017-01-01

    Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes. Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29). Cardiovascular magnetic resonance was performed, and late gadolinium enhancement mass was estimated for the LV using the 5-SD remote myocardium method, and for the RV using a segmental scoring system. Cohort characterization was determined through the use of a computerized database. Survival analysis from time of scan to first adverse event, defined as an episode of atrial arrhythmia, sustained ventricular arrhythmia, hospitalization with heart failure, or implantable cardioverter-defibrillator insertion. Eleven patients experienced an adverse outcome in the follow-up period, although there were no deaths. LV late gadolinium enhancement was associated with adverse outcomes in a univariate model (P = .027). However, when adjusted for age at scan the significant variables included NYHA class (P = .006), peak oxygen uptake (P = .028), number of prior sternotomies (P = .044), and higher indexed RV and LV end diastolic volumes (P = .002 and P tetralogy of Fallot survivors, however assessment particularly of the LV holds promise for the future. © 2016 Wiley Periodicals, Inc.

  11. Usage of 3D models of tetralogy of Fallot for medical education: impact on learning congenital heart disease.

    Science.gov (United States)

    Loke, Yue-Hin; Harahsheh, Ashraf S; Krieger, Axel; Olivieri, Laura J

    2017-03-11

    Congenital heart disease (CHD) is the most common human birth defect, and clinicians need to understand the anatomy to effectively care for patients with CHD. However, standard two-dimensional (2D) display methods do not adequately carry the critical spatial information to reflect CHD anatomy. Three-dimensional (3D) models may be useful in improving the understanding of CHD, without requiring a mastery of cardiac imaging. The study aimed to evaluate the impact of 3D models on how pediatric residents understand and learn about tetralogy of Fallot following a teaching session. Pediatric residents rotating through an inpatient Cardiology rotation were recruited. The sessions were randomized into using either conventional 2D drawings of tetralogy of Fallot or physical 3D models printed from 3D cardiac imaging data sets (cardiac MR, CT, and 3D echocardiogram). Knowledge acquisition was measured by comparing pre-session and post-session knowledge test scores. Learner satisfaction and self-efficacy ratings were measured with questionnaires filled out by the residents after the teaching sessions. Comparisons between the test scores, learner satisfaction and self-efficacy questionnaires for the two groups were assessed with paired t-test. Thirty-five pediatric residents enrolled into the study, with no significant differences in background characteristics, including previous clinical exposure to tetralogy of Fallot. The 2D image group (n = 17) and 3D model group (n = 18) demonstrated similar knowledge acquisition in post-test scores. Residents who were taught with 3D models gave a higher composite learner satisfaction scores (P = 0.03). The 3D model group also had higher self-efficacy aggregate scores, but the difference was not statistically significant (P = 0.39). Physical 3D models enhance resident education around the topic of tetralogy of Fallot by improving learner satisfaction. Future studies should examine the impact of models on teaching CHD that

  12. Rare associations of tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection.

    Science.gov (United States)

    Sen, Supratim; Rao, Suresh G; Kulkarni, Snehal

    2016-06-01

    We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

  13. An Innovative Simple Technique of Blood Conservation in Adult Patients with Tetralogy of Fallot and Severely Raised Hemoglobin

    OpenAIRE

    Neema, Praveen Kumar; Manikandan, Sethuraman; Rathod, Ramesh Chandra

    2007-01-01

    The adult patients of tetralogy of Fallot often present with high hemoglobin levels. High hemoglobin and hematocrit on cardiopulmonary bypass (CPB) are associated with increased hemolysis, plasma free hemoglobin, renal dysfunction or failure, postoperative bleeding, exploration for bleeding, and increased requirement of allogeneic blood and blood products. Despite the presence of high hemoglobin and its association with adverse outcome, blood conservation is rarely practiced in these patients...

  14. Right ventricular function after repair of tetralogy of Fallot: a comparison between bovine pericardium and porcine small intestinal extracellular matrix.

    Science.gov (United States)

    Naik, Ronak; Johnson, Jason; Kumar, T K S; Philip, Ranjit; Boston, Umar; Knott-Craig, Christopher J

    2017-05-29

    The porcine small intestinal extracellular matrix reportedly has the potential to differentiate into viable myocardial cells. When used in tetralogy of Fallot repair, it may improve right ventricular function. We evaluated right ventricular function after repair of tetralogy of Fallot with extracellular matrix versus bovine pericardium. Subjects with non-transannular repair of tetralogy of Fallot with at least 1 year of follow-up were selected. The extracellular matrix and bovine pericardium groups were compared. We used three-dimensional right ventricular ejection fraction, right ventricle global longitudinal strain, and tricuspid annular plane systolic excursion to assess right ventricular function. The extracellular matrix group had 11 patients, whereas the bovine pericardium group had 10 patients. No differences between the groups were found regarding sex ratio, age at surgery, and cardiopulmonary bypass time. The follow-up period was 28±12.6 months in the extracellular matrix group and 50.05±17.6 months in the bovine pericardium group (p=0.001). The mean three-dimensional right ventricular ejection fraction (55.7±5.0% versus 55.3±5.2%, p=0.73), right ventricular global longitudinal strain (-18.5±3.0% versus -18.0±2.2%, p=0.44), and tricuspid annular plane systolic excursions (1.59±0.16 versus 1.59±0.2, p=0.93) were similar in the extracellular matrix group and in the bovine pericardium group, respectively. Right ventricular global longitudinal strain in healthy children is reported at -29±3% in literature. In a small cohort of the patients undergoing non-transannular repair of tetralogy of Fallot, there was no significant difference in right ventricular function between groups having extracellular matrix versus bovine pericardium patches followed-up for more than 1 year. Lower right ventricular longitudinal strain noted in both the groups compared to healthy children.

  15. Evaluation of Tricuspid Annular Plane Systolic Excursion Measured with Cardiac Magnetic Resonance Imaging in Pediatric Patients with Tetralogy of Fallot

    Science.gov (United States)

    Soslow, Jonathan H.; Usoro, Emem; Wang, Li; Parra, David A.

    2015-01-01

    Background Aneurysmal dilation of the right ventricular outflow tract complicates assessment of right ventricular function in patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is commonly used to estimate ejection fraction. We hypothesized that tricuspid annular plane systolic excursion measured by cardiac magnetic resonance imaging approximates global and segmental right ventricular function, specifically right ventricular sinus ejection fraction, in pediatric patients with repaired tetralogy of Fallot. Methods Tricuspid annular plane systolic excursion was measured retrospectively on cardiac magnetic resonance images in 54 patients with repaired tetralogy of Fallot. Values were compared with right ventricular global, sinus, and infundibular ejection fractions. Tricuspid annular plane systolic excursion was: 1) indexed to body surface area, 2) converted into a fractional value, and 3) converted into published pediatric Z-scores. Results Tricuspid annular plane systolic excursion measurements had good agreement between observers. Right ventricular ejection fraction did not correlate with the absolute or indexed tricuspid annular plane systolic excursion and correlated weakly with fractional tricuspid annular plane systolic excursion (r=0.41 and p=0.002). Segmental right ventricular function did not appreciably improve correlation with any of the tricuspid annular plane systolic excursion measures. Pediatric Z-scores were unable to differentiate patients with normal and abnormal right ventricular function. Conclusions Tricuspid annular plane systolic excursion measured on cardiac magnetic resonance imaging correlates poorly with global and segmental right ventricular ejection fraction in pediatric patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is an unreliable approximation of right ventricular function in this patient population. PMID:26279488

  16. Optimal timing of pulmonary valve replacement in tetralogy of Fallot

    NARCIS (Netherlands)

    Straten, Alexander van

    2008-01-01

    Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation which leads tot dilatation of

  17. Aggressive Patch Augmentation May Reduce Growth Potential of Hypoplastic Branch Pulmonary Arteries After Tetralogy of Fallot Repair.

    Science.gov (United States)

    Wilder, Travis J; Van Arsdell, Glen S; Pham-Hung, Eric; Gritti, Michael; Hussain, Sara; Caldarone, Christopher A; Redington, Andrew; Hickey, Edward J

    2016-03-01

    Potential surgical strategies for hypoplastic branch pulmonary arteries (BPAs) during tetralogy of Fallot repair include (1) extensive patch augmentation to the hilum (PATCH), (2) limited extension arterioplasty to the proximal pulmonary artery (EXTENSION), or (3) leaving the native vessels unaugmented (NATIVE). We explored the effect of these strategies on reintervention and BPA growth. From 2000 to 2012, 434 children underwent complete tetralogy of Fallot repair. Risk-adjusted parametric models were used to analyze the risk of BPA reintervention for (1) all children, (2) children with BPAs of 4 mm or smaller, and (3) children with BPAs of 3 mm or smaller. Repeated-measures analysis of more than 2,000 echocardiograms was used to characterize postoperative BPA growth and right ventricular pressure by using nonlinear mixed models. Overall survival (99% [3 deaths]) was excellent. The 10-year freedom from BPA reintervention was 84%. In risk-adjusted models (including baseline BPA z-score), PATCH had a decreased freedom from reintervention (73%; p tetralogy of Fallot tend (∼85%) to grow well without instrumentation. Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. Pulmonary valve replacement in patients with corrected tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Fotios M. Mitropoulos

    2017-06-01

    Results: There were 2 perioperative deaths (2%. One patient developed sternal dehiscence requiring rewiring. Median ICU and hospital stay was 1 and 7 days respectively. Postoperative echocardiography at 6 and 12 months showed excellent bioprosthetic valve performance, significant decrease in size of the right cardiac chambers and reduction of tricuspid regurgitation (TR in the majority of the patients. At mean follow-up of 3.6 ± 2 years, all surviving patients remain in excellent clinical condition. Conclusion: Probability of reoperation for pulmonary insufficiency in patients with surgically corrected TOF increases with time and timely PVR by preventing the development of right heart failure is crucial for long-term survival. Current bioprosthetic valve technology in combination with the beating heart technique provides excellent immediate and short-term results. Further follow-up is necessary to evaluate long-term outcome.

  19. Heart failure complicating tetralogy of Fallot | Ogunkunle | West ...

    African Journals Online (AJOL)

    West African Journal of Medicine. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 23, No 1 (2004) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register · Download this PDF file. The PDF file you selected should ...

  20. MRI for therapy control in patients with tetralogy of Fallot; MRT zur Therapiekontrolle bei Patienten mit Fallot-Tetralogie

    Energy Technology Data Exchange (ETDEWEB)

    Theisen, D.; Reiser, M.F. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Dalla Pozza, R.D. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Abteilung fuer Kinderkardiologie und Paediatrische Intensivmedizin, Kinderherzzentrum, Muenchen (Germany); Malec, E. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Herzchirurgische Klinik, Kinderherzzentrum, Muenchen (Germany)

    2011-01-15

    With prevalences ranging from 0.26 to 0.8 permille of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation. The timing of pulmonary valve replacement is crucial as right ventricular (RV) volumes have only proven to normalize when preoperative end-diastolic volumes are <170 ml/m{sup 2} and end-systolic volumes are <85 ml/m{sup 2}. After surgical repair up to 15% of patients have residual or recurrent pulmonary artery stenosis. Distal pulmonary branch stenosis can aggravate PR and lead to right heart failure due to combined pressure and volume overload. Therefore, it has to be diagnosed in time and treated by angioplasty with or without stenting. (orig.) [German] Die Fallot-Tetralogie (TOF) ist mit einer Praevalenz zwischen 0,26 und 0,8 permille aller Lebendgeburten der haeufigste Herzfehler mit primaerer Zyanose. Durch verbesserte Operationstechniken erreichen heute nahezu alle Patienten das Erwachsenenalter. Bei fast allen Patienten tritt eine postoperative Pulmonalinsuffizienz (PI) auf, die entscheidenden Einfluss auf die langfristige Morbiditaet und Mortalitaet hat. Die kardiale Magnetresonanztomographie (MRT) ist fuer Quantifizierung von Ventrikelfunktion und Klappeninsuffizienzen etabliert und aufgrund ihrer fehlenden Invasivitaet und Strahlenexposition sehr gut fuer regelmaessige Verlaufskontrollen der heranwachsenden Patienten geeignet. Insbesondere der Zeitpunkt fuer einen Pulmonalklappenersatz darf

  1. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    International Nuclear Information System (INIS)

    Holmqvist, C.; Hochbergs, P.; Bjoerkhem, G.; Brockstedt, S.; Laurin, S.

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD

  2. Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

    Energy Technology Data Exchange (ETDEWEB)

    Holmqvist, C.; Hochbergs, P. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology; Bjoerkhem, G. [Univ. Hospital, Lund (Sweden). Dept of Paediatrics; Brockstedt, S.; Laurin, S. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology

    2000-01-01

    To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5{+-}4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

  3. A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta.

    Science.gov (United States)

    Caruana, Maryanne; Grech, Victor

    2017-05-01

    To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes. All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan-Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95-51.21) years). Patients born after 1985 were operated at a younger age (median 1.28 years) compared with patients born before 1985 (median 9.64 years) (P tetralogy of Fallot repair in contemporary patients is very good, cardiac death can occur at any stage and structural reintervention is common. Regular follow-up with imaging and rhythm monitoring remains of utmost importance in all patients. © 2016 Wiley Periodicals, Inc.

  4. Arrhythmias and conduction abnormalities in children after repair of tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Kuzevska-Maneva Konstandina

    2005-01-01

    Full Text Available Aim. To find out types and frequency of cardiac arrhythmias and conduction abnormalities in the group of children who underwent surgery for tetralogy of Fallot (TOF. Methods. Fortysix pedicatric patients who underwent a complete repair of TOF at the age of 1 to 13 (mean 2.89 ± 2.36 were studied. Thirty-eight (82.60% had total correction and 8 (17.40% had palliative operation first, and total correction afterwards. Twenty-four-hour Holter ECG monitoring was performed in all 46 pediatric patients aged from 1 to 16 yrs (mean 6.48 ± 4.04 after surgery as follows: in 1 patient (2.17% after a year, in 20 patients (43.47% after 2 to 5 years and in 25 patients (54.34% after 5 years. Mean age of patients on Holter monitoring was 9.25 ± 4.39 (range 4−19. Twenty of them (43.47% were girls and 28 (56.53% were boys. All the patients were evaluated by standard methods (clinical signs, clinical findings, ECG before surgery, ECG before Holter monitoring and 2D Doppler echocardiography. Results. Types of heart rhythm found out by Holter monitoring were: sinus nodus dysfunction in 1 child (2.17%, significant premature atrial contraction (PAC in 8 (17.39%, supraventricular paroxysmal tachycardia in 3 (6.53%, transient nodal rhythm in 2 (4.34%, premature ventricular contraction (PVC Lown grade I-III in 9 (19.56% and Lown grade IV in 2 (4.34, atrioventricular (AV block grade I in 2, right bundle branch block (RBBB in all 46 (100% and RBBB + left anterior hemiblock (LAH in 4 (8.96%. There was no presence of atrial flutter, ventricular tachycardia or complete AV block. None of them experienced sudden death. Using cross procedure statistical methods, it was found that all the patients with PVC had right ventricular dilatation. There was no relation of other types of arrhythmia found on Holter monitoring to the other parameters from echocardiography, neither to the other standard methods. Children did not need the pace-maker, but 36.95% of the them required

  5. Early versus late pulmonary valve replacement in patients with transannular patch-repaired tetralogy of Fallot.

    Science.gov (United States)

    Dobbels, Bieke; Herregods, Marie-Christine; Troost, Els; Van De Bruaene, Alexander; Rega, Filip; Budts, Werner; De Meester, Pieter

    2017-09-01

    Although the effects of pulmonary regurgitation after tetralogy of Fallot repair are detrimental, timing of pulmonary valve replacement (PVR) is unclear. Our goal was to evaluate the midterm efficacy and safety of early PVR. Patients with tetralogy of Fallot who underwent repair from 1962 to 2015 were included from the local database. Statistical analyses compared patients who underwent early PVR (age ≤16 years), late PVR and no PVR. The timing of the intervention was compared for efficacy-all-cause mortality and the combined end-point of all-cause mortality, ventricular tachycardia and defibrillator implantation-and for safety-the combined end-point of 1-year postoperative mortality after PVR, endocarditis and reintervention. Echocardiographic and electrocardiographic data at the last follow-up examination were compared across the 3 groups. Two hundred seventy-three patients (age 21 ± 5 years; 52% female) were included. The mean follow-up was 24 (95% confidence interval 22.7-26.2) years; the observed median was 21 years (interquartile range 11-31). No significant difference in survival was found between the early PVR (n = 106; 39%), the late PVR (n = 47; 17%) and the no PVR groups (n = 120; 44%) (P = 0.990). No significant difference in the combined efficacy end-point was noted between patients who underwent early PVR compared with patients who underwent late PVR (P = 0.247). Worse event-free survival for the 3-point safety end-point was observed after early PVR (P < 0.001). Right ventricular morphology (P < 0.001) and function (P < 0.001) were better preserved in the patient group that underwent PVR before the age of 16 years. As expected, PVR-related morbidity was higher in patients who underwent early PVR but the midterm outcome was similar. Nevertheless, better preservation of right ventricular morphology and function in the early PVR group might result in better long-term survival. © The Author 2017. Published by Oxford

  6. Peptidomic Analysis of Fetal Heart Tissue for Identification of Endogenous Peptides Involved in Tetralogy of Fallot.

    Science.gov (United States)

    Zhang, Jingjing; Liang, Dong; Cheng, Qing; Cao, Li; Wu, Yun; Wang, Yan; Han, Shuping; Yu, Zhangbin; Cui, Xianwei; Xu, Tianhui; Ma, Dingyuan; Hu, Ping; Xu, Zhengfeng

    2017-06-01

    Tetralogy of fallot (TOF) is one of the most prevalent types of congenital heart diseases. As a category of bioactive molecules, peptides have been proved to participate in various biological processes. However, the role of endogenous peptides in the pathogenesis of TOF has not been studied. In this study, we performed a comparative peptidomic profile in the fetal heart of TOF and the control group for the first time by liquid chromatography-tandem mass spectrometry. Our data demonstrated that a total of 201 peptides derived from 176 precursor proteins were differentially expressed in the heart tissues of TOF fetuses compared with normal controls, including 41 upregulated peptides and 160 downregulated peptides. After analyzing the characteristics of these differentially expressed peptides and their precursor proteins, we found that these peptides were potentially involved in different biological processes, especially cardiogenesis and congenital anomaly of the cardiovascular system. Interestingly, we detected several extracellular matrix-derived peptides involved in our differentially expressed peptidomic profile. In summary, our study constructed a comparative peptidomic profile from the heart tissues of TOF fetuses and normal controls, and it identified a series of peptides that could potentially participate in heart development and TOF formation. The emergence of our peptidomics study indicated a new perspective to explore the pathogenesis of abnormal heart morphology, especially TOF.

  7. Lung scintiscanning in corrective surgery of congenital heart disease, 2. Tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Endo, K. (Iwate Medical Coll., Morioka (Japan). School of Medicine)

    1981-06-01

    Lung scintiscanning was performed to investigate the distribution of pulmonary blood flow before and after surgery in 30 patients with tetralogy of Fallot. Uniformity of lung scintigram and the derangement of the distribution pattern were observed preoperatively and postoperatively. The results were as follows: 1) Preoperatively, uniformity of lung scintigram was 27.7 - 100% (average 63.4%). It is more necessary to construct the outflow tract in most cases, when the PA/Ao diameter ratio was lower than 0.5 and uniformity of lung scintigram was under 50%. Besides, preoperative uniformity of lung scintigram correlated with the degree of polycythemia. In 3 cases who died after corrective surgery, the value of uniformity of lung scintigram was as low as 33.3 - 61.1%. 2) Preoperative pulmonary blood flow disturbances were found in 50% of 30 cases. In all of the cases without pulmonary blood flow disturbances, total correction was successfully performed. On the other hand, in cases with pulmonary blood flow disturbances, the result of total correction was not satisfactory. 3) In cases with the slight and moderately deranged distribution preoperatively, postoperative follow up study by scintiscanning at 1 to 2 months after surgery showed considerately more improvement of pulmonary blood flow.

  8. Managing the right ventricular outflow tract for pulmonary regurgitation after tetralogy of Fallot repair

    Science.gov (United States)

    Hauser, Michael; Eicken, Andreas; Kuehn, Andreas; Hess, John; Fratz, Sohrab; Ewert, Peter; Kaemmerer, Harald

    2013-01-01

    The long-term outcome of patients with tetralogy of Fallot (TOF) with reconstruction of the right ventricular (RV) outflow tract is often complicated by the sequelae of severe pulmonary regurgitation. Progressive enlargement of the right ventricle, biventricular dysfunction and arrhythmia are apparent in more than 50% of the patients in the fourth decade of life. Pathophysiologic implications, clinical assessment and diagnostic modalities are discussed, whereas CMR imaging seems to be the procedure of choice. Therapeutical options for rereconstruction of the RV outflow tract are mentioned, surgical and interventional procedures are explained in detail. The optimal timing of reoperation for significant pulmonary regurgitation after TOF repair is still a matter of controversy given the limited runtime of the lately implanted prostheses and the risk of further reoperation. Early surgery is recommended in these patients before symptoms develop, or RV function has declined. Today we believe that waiting for the patient to become symptomatic is too late. All in all, pulmonary valve replacement is at least indicated in patients developing symptoms due to severe pulmonary regurgitation, particularly if associated with substantial or progressive RV dilatation, tricuspid regurgitation and/or supraventricular or ventricular arrhythmias. PMID:27326099

  9. 4D-MR flow analysis in patients after repair for tetralogy of Fallot

    International Nuclear Information System (INIS)

    Geiger, J.; Markl, M.; Jung, B.; Langer, M.; Grohmann, J.; Stiller, B.; Arnold, R.

    2011-01-01

    Comprehensive analysis of haemodynamics by 3D flow visualisation and retrospective flow quantification in patients after repair of tetralogy of Fallot (TOF). Time-resolved flow-sensitive 4D MRI (spatial resolution ∝ 2.5 mm, temporal resolution = 38.4 ms) was acquired in ten patients after repair of TOF and in four healthy controls. Data analysis included the evaluation of haemodynamics in the aorta, the pulmonary trunk (TP) and left (lPA) and right (rPA) pulmonary arteries by 3D blood flow visualisation using particle traces, and quantitative measurements of flow velocity. 3D visualisation of whole heart haemodynamics provided a comprehensive overview on flow pattern changes in TOF patients, mainly alterations in flow velocity, retrograde flow and pathological vortices. There was consistently higher blood flow in the rPA of the patients (rPA/lPA flow ratio: 2.6 ± 2.5 vs. 1.1 ± 0.1 in controls). Systolic peak velocity in the TP was higher in patients (1.9 m/s ± 0.7 m/s) than controls (0.9 m/s ± 0.1 m/s). 4D flow-sensitive MRI permits the comprehensive evaluation of blood flow characteristics in patients after repair of TOF. Altered flow patterns for different surgical techniques in the small patient cohort may indicate its value for patient monitoring and potentially identifying optimal surgical strategies. (orig.)

  10. 4D-MR flow analysis in patients after repair for tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Geiger, J.; Markl, M.; Jung, B.; Langer, M. [University Hospital Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Grohmann, J.; Stiller, B.; Arnold, R. [University Hospital Freiburg, Department of Congenital Heart Disease and Pediatric Cardiology, Freiburg (Germany)

    2011-08-15

    Comprehensive analysis of haemodynamics by 3D flow visualisation and retrospective flow quantification in patients after repair of tetralogy of Fallot (TOF). Time-resolved flow-sensitive 4D MRI (spatial resolution {proportional_to} 2.5 mm, temporal resolution = 38.4 ms) was acquired in ten patients after repair of TOF and in four healthy controls. Data analysis included the evaluation of haemodynamics in the aorta, the pulmonary trunk (TP) and left (lPA) and right (rPA) pulmonary arteries by 3D blood flow visualisation using particle traces, and quantitative measurements of flow velocity. 3D visualisation of whole heart haemodynamics provided a comprehensive overview on flow pattern changes in TOF patients, mainly alterations in flow velocity, retrograde flow and pathological vortices. There was consistently higher blood flow in the rPA of the patients (rPA/lPA flow ratio: 2.6 {+-} 2.5 vs. 1.1 {+-} 0.1 in controls). Systolic peak velocity in the TP was higher in patients (1.9 m/s {+-} 0.7 m/s) than controls (0.9 m/s {+-} 0.1 m/s). 4D flow-sensitive MRI permits the comprehensive evaluation of blood flow characteristics in patients after repair of TOF. Altered flow patterns for different surgical techniques in the small patient cohort may indicate its value for patient monitoring and potentially identifying optimal surgical strategies. (orig.)

  11. Flow Topology in the Right Ventricle after Tetralogy of Fallot Repair

    Science.gov (United States)

    Mikhail, Amanda; Kadem, Lyes; di Labbio, Giuseppe

    2016-11-01

    Among all of the known congenital heart defects, Tetralogy of Fallot (TOF) is the most common cyanotic defect, accounting for 5% of all detected defects. Approximately 1 in 2518 births will result with TOF, leading to about 1657 cases per year in the United States alone. All of those affected will need surgical repair in order to have a relatively normal life and longer life span. Unfortunately, pulmonary regurgitation (PR) has been observed to appear two to three decades after the initial operation in 50% of operated cases. PR results in abnormal flow patterns in the right ventricle, which are currently poorly understood. In this experimental study, several severities of pulmonary regurgitation were simulated on a newly developed right ventricle using a cardiovascular simulator. The interaction between the tricuspid valve inflow and the pulmonary regurgitation was investigated using Time-resolved particle image velocimetry (TR-PIV). PR resulted in a turbulent jet that disturbed the optimal filling of the RV. Energy losses and viscous shear stresses were observed to significantly increase with the severity of PR. This study can contribute towards a better understanding of the suboptimal performance in patients with repaired TOF.

  12. Tetralogy of Fallot and Hypoplastic Left Heart Syndrome – Complex Clinical Phenotypes Meet Complex Genetic Networks

    Science.gov (United States)

    Lahm, Harald; Schön, Patric; Doppler, Stefanie; Dreßen, Martina; Cleuziou, Julie; Deutsch, Marcus-André; Ewert, Peter; Lange, Rüdiger; Krane, Markus

    2015-01-01

    In many cases congenital heart disease (CHD) is represented by a complex phenotype and an array of several functional and morphological cardiac disorders. These malformations will be briefly summarized in the first part focusing on two severe CHD phenotypes, hypoplastic left heart syndrome (HLHS) and tetralogy of Fallot (TOF). In most cases of CHD the genetic origin remains largely unknown, though the complexity of the clinical picture strongly argues against a dysregulation which can be attributed to a single candidate gene but rather suggests a multifaceted polygenetic origin with elaborate interactions. Consistent with this idea, genome-wide approaches using whole exome sequencing, comparative sequence analysis of multiplex families to identify de novo mutations and global technologies to identify single nucleotide polymorphisms, copy number variants, dysregulation of the transcriptome and epigenetic variations have been conducted to obtain information about genetic alterations and potential predispositions possibly linked to the occurrence of a CHD phenotype. In the second part of this review we will summarize and discuss the available literature on identified genetic alterations linked to TOF and HLHS. PMID:26069455

  13. Correlation between Oxygen Saturation and Hemoglobin and Hematokrit Levels in Tetralogy of Fallot Patients

    Directory of Open Access Journals (Sweden)

    Farhatul Inayah Adiputri

    2016-03-01

    Full Text Available Background: Hemoglobin and hematocrit levels increase in Tetralogy of Fallot (TOF but the oxygen saturation declines. Reduced hemoglobin in circulating blood as a parameter of cyanosis does not indicate rising hemoglobin due to the ‘not-working’ hemoglobins that affect the oxygen saturation. Increasing hematocrit is the result of secondary erythrocytosis caused by declining oxygen level in blood, which is related to the oxygen saturation. This study was conducted to find the correlation between oxygen saturation and hemoglobin and hematocrite levels in TOF patients. Methods: This study was undertaken at Dr. Hasan Sadikin General Hospital in the period of January 2011 to December 2012 using the cross-sectional analytic method with total sampling technique. Inclusion criteria were medical records of TOF patients diagnosed based on echocardiography that included data on oxygen saturation, hemoglobin, and hematocrite. Exclusion criteria was the history of red blood transfusion. Results: Thirty medical records of TOF patiens from Dr. Hasan Sadikin General Hospital Bandung were included in this study. Due to skewed data distribution, Spearman correlation test was used to analyze the data. There was a significant negative correlation between oxygen saturation and hematocrit level (r= -0.412; p=0.024 and insignificant correlation between oxygen saturation and hemoglobin (r=-0.329; p= 0.076. Conclusions: There is a weak negative correlation between oxygen saturation and hematocrite levels

  14. Lung Perfusion SPECT: Application in a Patient With Tetralogy of Fallot and Suspected Pulmonary Thromboemboli

    International Nuclear Information System (INIS)

    Ranji Amjad, Mina; Abbasi, Mehrshad; Farzanehfar, Saeed

    2015-01-01

    A 22-year-old woman presented with acute left-sided pleuritic chest pain and dyspnea 6 days after surgery for revision of the stenotic central aortopulmonary shunt. She had a history of tetralogy of Fallot (TOF), pulmonary valve stenosis, ventricular septal defect and major aortopulmonary collateral artery. Her Waterston shunt was placed when she was 5 years old and stented and re-dilated after stenosis. Acute pulmonary thromboemboli (PTE) was suspected and pulmonary perfusion scan was performed with 4 mCi 99m Technetium labeled macroaggregated albumin. The left lung was globally hypoperfused with evident uptake in the brain, renal parenchyma and thyroid. SPECT images revealed a segmental wedge-shaped peripheral defect in the posterior segment of the left upper lobe. The scan was interpreted as acute/chronic PTE or vascular abnormality. CT angiography excluded PTE; nevertheless the patient was treated with a therapeutic dose of heparin changed to warfarin and was discharged with improvement of the symptoms. Pulmonary artery angiography was not performed

  15. Lactate, endothelin, and central venous oxygen saturation as predictors of mortality in patients with Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Poonam Malhotra Kapoor

    2016-01-01

    Full Text Available Background: Lactate and central venous oxygen saturation (ScVO2 are well known biomarkers for adequacy of tissue oxygenation. Endothelin, an inflammatory marker has been associated with patient′s nutritional status and degree of cyanosis. The aim of this study was to explore the hypothesis that lactate, ScVO2 and endothelin before induction may be predictive of mortality in pediatric cardiac surgery. Methods: We conducted a prospective observational study of 150 pediatric (6 months to 12 years patients who were posted for intracardiac repair for tetralogy of fallot and measured lactate, ScVO2 and endothelin before induction (T1, 20 minutes after protamine administration (T2 and 24 hours after admission to ICU (T3. Results: Preinduction lactate and endothelin levels were found to predict mortality in patients of tetralogy of fallot with an odds ratio of 6.020 (95% CI 2.111-17.168 and 1.292(95% CI 1.091-1.531 respectively. In the ROC curve analysis for lactate at T1, the AUC was 0.713 (95% CI 0.526-0.899 P = 0.019. At the cutoff value of 1.750mmol/lt, the sensitivity and specificity for the prediction of mortality was 63.6% and 65.5%, respectively. For endothelin at T1, the AUC was 0.699 (95% CI 0.516-0.883, P = 0.028 and the cutoff value was ≤2.50 (sensitivity, 63.6%; specificity, 58.3 %. ScVO2 (odds ratio 0.85 at all three time intervals, suggested that improving ScVO2 can lead to 15% reduction in mortality. Conclusions: Lactate, ScVO2 and endothelin all showed association with mortality with lactate having the maximum prediction. Lactate was found to be an independent, reliable and cost-effective measure of prediction of mortality in patients with tetralogy of fallot.

  16. Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries: A 15-Year Experience With 458 Patients.

    Science.gov (United States)

    Bauser-Heaton, Holly; Borquez, Alejandro; Han, Brian; Ladd, Michael; Asija, Ritu; Downey, Laura; Koth, Andrew; Algaze, Claudia A; Wise-Faberowski, Lisa; Perry, Stanton B; Shin, Andrew; Peng, Lynn F; Hanley, Frank L; McElhinney, Doff B

    2017-04-01

    Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction. We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure. Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries. © 2017 American Heart Association, Inc.

  17. Fallot's tetralogy in a European beaver (Castor fiber).

    Science.gov (United States)

    Wenger, Sandra; Gull, Jessica; Glaus, Tony; Blumer, Serafin; Wimmershoff, Julia; Kranjc, Asja; Steinmetz, Hanspeter; Hatt, Jean-Michel

    2010-06-01

    A 20-mo-old, female, 9-kg European beaver (Castor fiber) presented with apathy, reduced appetite of 3-day duration and a grade 5/6 systolic heart murmur. Thoracic radiographs revealed a diffuse broncho-interstitial pattern suspicious for bronchopneumonia. The echocardiographic findings of a hypertrophied right ventricle, ventricular septal defect with overriding aorta, and infundibular pulmonic stenosis were consistent with Fallot's tetralogy. Even though the bronchopneumonia rather than the congenital cardiac defect was considered of primary importance for the presenting clinical signs, the latter was relevant for the decision not to continue any medical treatment. Both disease processes were confirmed on necropsy. Fallot's tetralogy, European beaver, Castor fiber, heart murmur, ultrasound.

  18. Retained fetal lung fluid in two neonates with congenital absence of the pulmonary valve and tetralogy of fallot

    International Nuclear Information System (INIS)

    Strife, J.L.; Towbin, R.B.; Francis, P.; Kuhn, J.P.

    1981-01-01

    Chest radiographs obtained at birth in two neonates with absent pulmonary valve and tetralogy of Fallot demonstrated asymmetrical lung aeration. This finding was attributed to delay in resorption of fetal lung fluid. It is postulated that in the initial hours of life, the dilated pulmonary artery compressed the bronchus and delayed egress of fetal lung fluid. Over a 24-hour interval, the fluid was resorbed, resulting in the more typical pattern of hyperinflated lung and markedly dilated pulmonay artery. These cases are presumably the first of their kind to be reported

  19. Retained fetal lung fluid in two neonates with congenital absence of the pulmonary valve and tetralogy of fallot

    Energy Technology Data Exchange (ETDEWEB)

    Strife, J.L.; Towbin, R.B.; Francis, P.; Kuhn, J.P.

    1981-12-01

    Chest radiographs obtained at birth in two neonates with absent pulmonary valve and tetralogy of Fallot demonstrated asymmetrical lung aeration. This finding was attributed to delay in resorption of fetal lung fluid. It is postulated that in the initial hours of life, the dilated pulmonary artery compressed the bronchus and delayed egress of fetal lung fluid. Over a 24-hour interval, the fluid was resorbed, resulting in the more typical pattern of hyperinflated lung and markedly dilated pulmonay artery. These cases are presumably the first of their kind to be reported.

  20. Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot Infants With Risk Factors for Early Primary Repair.

    Science.gov (United States)

    Sandoval, Juan Pablo; Chaturvedi, Rajiv R; Benson, Lee; Morgan, Gareth; Van Arsdell, Glen; Honjo, Osami; Caldarone, Christopher; Lee, Kyong-Jin

    2016-12-01

    Tetralogy of Fallot with cyanosis requiring surgical repair in early infancy reflects poor anatomy and is associated with more clinical instability and longer hospitalization than those who can be electively repaired later. We bridged symptomatic infants with risk factors for early primary repair by right ventricular outflow tract stenting (stent). Four groups of tetralogy of Fallot with confluent central pulmonary arteries were studied: stent group (n=42), primary repair (aged 3mo group; n=45). Stent patients had the smallest pulmonary arteries with a median (95% credible intervals) Nakata index (mm 2 /m 2 ) of 79 (66-85) compared with the early-PA 139 (129-154), early-PS 136 (121-153), and surg>3mo 167 (153-200) groups. Only stent infants required unifocalization of aortopulmonary collaterals (17%). Stent and early-PA infants had younger age and lower weight than early-PS infants. Stent infants had the most multiple comorbidities. Stenting allowed deferral of complete surgical repair to an age (6 months), weight (6.3 [5.8-7.0] kg), and Nakata index (147 [132-165]) similar to the low-risk surg>3mo group. The 3 early treatment groups had similar intensive care unit/hospital stays and high reintervention rates in the first 12 months after repair, compared with the surg>3mo group. Right ventricular outflow tract stenting of symptomatic tetralogy of Fallot with poor anatomy (small pulmonary arteries) and adverse factors (multiple comorbidities, low weight) relieves cyanosis and defers surgical repair. This allowed pulmonary arterial and somatic growth with clinical results comparable to early surgical repair in more favorable patients. © 2016 American Heart Association, Inc.

  1. Efficacy of dexmedetomidine for the control of junctional ectopic tachycardia after repair of tetralogy of Fallot

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    Randhir S Rajput

    2014-01-01

    Full Text Available Background: Junctional ectopic tachycardia occurs frequently after congenital cardiac surgery and can be a cause of increased morbidity and mortality. Dexmedetomidine (DEX is an a2 adrenoreceptor agonist, has properties of controlling tachyarrhythmia by regulating the sympatho-adrenal system. Objective: To evaluate the efficacy of DEX for control of junctional ectopic tachycardia after repair of Tetralogy of Fallot (TOF. Materials and Methods: Two hundred and twenty pediatric cardiac patients with TOFs were enrolled in a prospective randomized control study. Patients underwent correction surgery. They were divided into two groups, i.e., Group 1 (DEX and Group 2 (control. Heart rate, rhythm, mean arterial pressure (MAP were recorded after the anesthetic induction (T1, after termination of bypass (T2, after 04 hours (T3, and 08 hours after transferring the patient to intensive care unit (ICU; T4. Results: Heart rate was comparable between two groups before starting the drug but statistically significant after bypass until 08 hours after transferring the patient to ICU. Junctional ectopic tachycardia occurred more in Group-2 (20% as compared to Group-1 (9.09%; P = 0.022. Junctional ectopic tachycardia occurs early in Group-2 (0.14 ± 0.527 hours as compared to Group 1 (0.31 ± 1.29 hours; P = 0.042. The duration of junctional ectopic tachycardia was more prolonged in Group-2 (1.63 ± 3.64 hours as compared to Group-1 (0.382 ± 1.60 hours; P = 0.012. The time to withdraw from mechanical ventilation and ICU stay of Group 1 patient was less than of Group 2 patients (P = <0.001. Conclusion: DEX had a therapeutic role in the prevention of junctional ectopic tachycardia in patients undergoing repair for TOF.

  2. Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

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    Dowdle, S.C.; Human, D.G.; Mann, M.D. (Univ. of Cape Town (South Africa))

    1990-08-01

    Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis.

  3. Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

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    Dowdle, S.C.; Human, D.G.; Mann, M.D.

    1990-01-01

    Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis

  4. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome with Tetralogy of Fallot: a very rare combination

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    Deepak eSharma

    2015-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC syndrome is a rare genetic disorder with an incidence of around 1:90,000 live births. It is known with various names which includes split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis.

  5. Early post operative mortality of Total Correction of Tetralogy of Fallot

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    Ali Azari

    2017-12-01

    Full Text Available Introduction: Since 1954, after the first surgical repair of tetralogy of Fallot (TOF, several innovations have occurred in cardiac surgery, especially in children. One stage complete repair of TOF is currently possible even in infancy; however, complications such as hypoxemia, arrhythmia, cardiac dysfunction, sudden death, and valvular disorders may happen. In this study, we evaluated the results of complete surgical repair of TOF with pulmonary stenosis. Material and Methods: We assessed 74 cases of TOF with pulmonary stenosis that underwent surgery in Cardiac Surgical Ward of Imam Reza Hospital, Mashhad, Iran from 2008 to 2010. Results: Mean age was 5.74±3.31 years and more than half of the patients were male. Mean perfusion and cross-clamping times were 55.45±15.06 and 42.63±9.07 min,respectively. The most common coexisting anomaly was atrial septal defect. Further, 83.7% of the patients were symptomatic, and history of spell attacks was positive in 24.3% of the cases. Arrhythmia was reported in 28.4% of the patients. Mortality rate was 12.2% in our study, which was higher in younger patients (P=0.022 or those with lower weight (P=0.008, longer perfusion time during cardiac surgery (P=0.009, or presence of associated cardiac anomalies (P=0.030. Conclusion: Outcomes and mortality rate of one-stage surgical repair of TOF with pulmonary stenosis was acceptable in our center, and arrhythmia was the most common postoperative complication

  6. Proarrhythmic remodelling of the right ventricle in a porcine model of repaired tetralogy of Fallot

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    Benoist, David; Dubes, Virginie; Roubertie, François; Gilbert, Stephen H; Charron, Sabine; Constantin, Marion; Elbes, Delphine; Vieillot, Delphine; Quesson, Bruno; Cochet, Hubert; Haïssaguerre, Michel; Rooryck, Caroline; Bordachar, Pierre; Thambo, Jean-Benoit; Bernus, Olivier

    2017-01-01

    Objective The growing adult population with surgically corrected tetralogy of Fallot (TOF) is at risk of arrhythmias and sudden cardiac death. We sought to investigate the contribution of right ventricular (RV) structural and electrophysiological remodelling to arrhythmia generation in a preclinical animal model of repaired TOF (rTOF). Methods and results Pigs mimicking rTOF underwent cardiac MRI functional characterisation and presented with pulmonary regurgitation, RV hypertrophy, dilatation and dysfunction compared with Sham-operated animals (Sham). Optical mapping of rTOF RV-perfused wedges revealed a significant prolongation of RV activation time with slower conduction velocities and regions of conduction slowing well beyond the surgical scar. A reduced protein expression and lateralisation of Connexin-43 were identified in rTOF RVs. A remodelling of extracellular matrix-related gene expression and an increase in collagen content that correlated with prolonged RV activation time were also found in these animals. RV action potential duration (APD) was prolonged in the epicardial anterior region at early and late repolarisation level, thus contributing to a greater APD heterogeneity and to altered transmural and anteroposterior APD gradients in rTOF RVs. APD remodelling involved changes in Kv4.3 and MiRP1 expression. Spontaneous arrhythmias were more frequent in rTOF wedges and more complex in the anterior than in the posterior RV. Conclusion Significant remodelling of RV conduction and repolarisation properties was found in pigs with rTOF. This remodelling generates a proarrhythmic substrate likely to facilitate re-entries and to contribute to sudden cardiac death in patients with rTOF. PMID:28051771

  7. Evaluation by MRA of aortic dilation late after repair of tetralogy of Fallot.

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    Kay, W Aaron; Cook, Stephen C; Daniels, Curt J

    2013-09-10

    This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA). AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients. All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD. Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo). AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  8. Recent Development in Pulmonary Valve Replacement after Tetralogy of Fallot Repair: The Emergence of Hybrid Approaches

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    Tariq eSuleiman

    2015-06-01

    Full Text Available In the current era approximately 90% of infants born with tetralogy of Fallot (ToF are expected to live beyond 40 years of age making it the fastest growing population amongst patients with congenital heart disease. One of the most common late consequences after repair of ToF, is pulmonary valve regurgitation (PVR. Significant PVR results in progressive dilatation and dysfunction of the right ventricle, decrease in exercise tolerance, arrhythmias, heart failure, and increased risk of sudden death. The conventional approach of dealing with this problem is to perform pulmonary valve replacement using cardiopulmonary bypass (CPB and cardioplegic arrest. However, this approach is associated not only with long operative times but also side effects related to the use of CPB. Development of percutaneous approaches to valve disease is one of the most exciting areas of research and clinical innovation in cardiovascular research. The main development has been that of transcatheter pulmonary valve replacement for the rehabilitation of conduits between the right ventricle and pulmonary artery in patients after surgery for ToF. However, with the percutaneous technique, a limited size of prosthesis can be inserted. Moreover, the technique does not offer the opportunity of treating additional defects that are frequently associated with severe PR, such as pulmonary artery dilatation, and it cannot be used in the significantly dilated native right ventricular outlet tract (RVOT. The advent of the hybrid surgical options for treating cardiac disease has integrated the techniques of interventional cardiology with the techniques of cardiac surgery to provide a form of therapy that combines the respective strengths of both fields.In this review, we present and compare recent advances in procedures to replace the pulmonary valve in patients with ToF presenting with severe PVR and dilated RVOT.

  9. Echocardiographic parameters and brain natriuretic peptide in patients after surgical repair of tetralogy of Fallot.

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    Tatani, Solange B; Carvalho, Antonio Carlos C; Andriolo, Adagmar; Rabelo, Rogério; Campos, Orlando; Moises, Valdir A

    2010-04-01

    Although the residual lesions after surgical correction of tetralogy of Fallot (TOF) can be evaluated by Doppler echocardiography (DE), the relation of DE parameters with the proBNP level, a potential biomarker of right ventricle overload, is not well known. The objective of this study was to evaluate the DE parameters and their relation to proBNP levels. proBNP plasma level and Doppler echocardiography parameters were obtained on the same day in 49 patients later after repair of TOF (mean age of 14.7 years, 51% female, mean PO time of 9.5 years). The DE parameters studied were the dimensions of the right atrium (RA) and ventricle (RV), RV diastolic and systolic function, and residual pulmonary lesions. The relation between them and proBNP levels were analyzed and the cutoff values of DE parameters for elevated proBNP determined. proBNP was elevated in 53% and correlated with RV diastolic diameter (r = 0.41; P = 0.003), RA longitudinal (r = 0.52; P = 0.0001) and transversal (r = 0.47; P = 0.001) diameters, pressure half time of pulmonary regurgitation (PR) velocity (PHT) (r =-0.42; P = 0.005), and the PR index (r =-0.60; P < 0.001). By multivariate analysis, the PR index (r =-597; P = 0,001; CI: -913.2 to -280.8) and RA longitudinal (r = 7.74; P < 0,001; CI 4.18 to 11.31) were independent predictors of elevated proBNP. PHT lower than 64 msec (0.76) and PRi lower than 0.65 (0.81) had the best accuracy for elevated proBNP. proBNP may be increased in patients after surgical repair of TOF, correlated with the size of right cardiac chambers and the severity of PR.

  10. Repair of Tetralogy of Fallot in Infancy via the Atrioventricular Approach

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    Hamid Bigdelian

    2016-02-01

    Full Text Available Background: Tetralogy of Fallot (TOF is a well-recognized congenital heart disease. Despite improvements in the outcomes of surgical repair, the optimal timing of surgery and type of surgical management of patients with TOF remains controversial. The purpose of this study was to assess outcomes following the repair of TOF in infants depending on the surgical procedure used. Methods: This study involved the retrospective review of 120 patients who underwent TOF repair between 2010 and 2013. Patients were divided into three groups depending on the surgical procedure that they underwent. Corrective surgery was done via the transventricular approach (n=40, the transatrial approach (n=40, or a combined atrioventricular approach (n=40. Demographic data and the outcomes of the surgical procedures were compared among the groups. Results: In the atrioventricular group, the incidence of the following complications was found to be significantly lower than in the other groups: complete heart block (p=0.034, right ventricular failure (p=0.027 and mediastinal bleeding (p=0.007. Patients in the atrioventricular group had a better postoperative right ventricular ejection fraction (p=0.001. No statistically significant differences were observed among the three surgical groups in the occurrence of tachycardia, renal failure, and tricuspid incompetence. The one-year survival rates in the three groups were 95%, 90%, and 97.5%, respectively (p=0.395. Conclusion: Combined atrioventricular repair of TOF in infancy can be safely performed, with acceptable surgical risk, a low incidence of reoperation, good ventricular function outcomes, and an excellent survival rate.

  11. Effect of Concomitant Birth Defects and Genetic Anomalies on Infant Mortality in Tetralogy of Fallot.

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    Jernigan, Eric G; Strassle, Paula D; Stebbins, Rebecca C; Meyer, Robert E; Nelson, Jennifer S

    2017-08-15

    A substantial proportion of infants born with tetralogy of Fallot (TOF) die in infancy. A better understanding of the heterogeneity associated with TOF, including extracardiac malformations and chromosomal anomalies is vital to stratifying risk and optimizing outcomes during infancy. Using the North Carolina Birth Defects Monitoring Program, infants diagnosed with TOF and born between 2003 and 2012 were included. Kaplan-Meier survival curves were used to estimate cumulative 1-year mortality, stratified by the presence of concomitant birth defects (BDs) and chromosomal anomalies. Multivariable logistic regression was used to estimate the direct effect of each concomitant BD, after adjusting for all others. A total of 496 infants with TOF were included, and 15% (n = 76) died. The number of concomitant BD systems was significantly associated with the risk of death at 1-year, p < 0.0001. Specifically, the risk of mortality was 8% among infants with TOF with or without additional cardiac defects, 16% among infants with TOF and 1 extracardiac BD system, 19% among infants with 2 extracardiac BD systems, and 39% among infants with ≥ 3 extracardiac BD systems. After adjustment, concomitant eye and gastrointestinal defects were significantly associated increased with 1-year mortality, odds ratio 2.83 (95% confidence interval, 1.08-7.32) and odds ratio 4.43 (95% confidence interval, 1.57, 12.45), respectively. Infants with trisomy 13 or trisomy 18 were also significantly more likely to die, p < 0.0001. Both concomitant BDs and genetic anomalies increase the risk of mortality among infants with TOF. Future studies are needed to identify the underlying genetic and socioeconomic risk factors for high-risk TOF infants. Birth Defects Research 109:1154-1165, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  12. Functional health status of adults with tetralogy of Fallot: matched comparison with healthy siblings.

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    Knowles, Rachel; Veldtman, Gruschen; Hickey, Edward J; Bradley, Timothy; Gengsakul, Aungkana; Webb, Gary D; Williams, William G; McCrindle, Brian W

    2012-07-01

    Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status (p health as good or excellent (p health (p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  13. Comparison of stroke work between repaired tetralogy of Fallot and normal right ventricular physiologies.

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    Lee, Namheon; Das, Ashish; Banerjee, Rupak K; Gottliebson, William M

    2013-01-01

    Adult patients who underwent tetralogy of Fallot repair surgery (rTOF) confront life-threatening ailments due to right ventricular (RV) myocardial dysfunction. Pulmonary valve replacement (PVR) needs to be performed to restore the deteriorating RV function. Determination of correct timing to perform PVR in an rTOF patient remains subjective, due to the unavailability of quantifiable clinical diagnostic parameters. The objective of this study is to evaluate the possibility of using RV body surface area (BSA)-indexed stroke work (SW(I)) to quantify RV inefficiency in TOF patients. We hypothesized that RV SW(I) required to push blood to the lungs in rTOF patients is significantly higher than that of normal subjects. Seven patients with rTOF pathophysiology and eight controls with normal RV physiology were registered for this study. Right ventricular volume and pressure were measured using cardiac magnetic resonance imaging and catheterization, respectively. Statistical analysis was performed to quantify the difference in SW(I) between the RV of the rTOF and control groups. Right ventricular SW(I) in rTOF patients (0.176 ± 0.055 J/m(2)) was significantly higher by 93.4% (P = 0.0026) than that of controls (0.091 ± 0.030 J/m(2)). Further, rTOF patients were found to have significantly higher (P < 0.05) BSA normalized RV end-systolic volume, end-systolic pressure, and regurgitation fraction than control subjects. Ejection fraction and peak ejection rate of rTOF patients were significantly lower (P < 0.05) than those of controls. Patients with rTOF pathophysiology had significantly higher RV SW(I) compared with subjects with normal RV physiology. Therefore, RV SW(I) may be useful to quantify RV inefficiency in rTOF patients along with currently used clinical end points such as RV volume, pressure, regurgitation fraction, and ejection fraction.

  14. Basic arterial blood gas biomarkers as a predictor of mortality in tetralogy of Fallot patients.

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    Bhardwaj, Vandana; Kapoor, Poonam Malhotra; Irpachi, Kalpana; Ladha, Suruchi; Chowdhury, Ujjwal Kumar

    2017-01-01

    Serum lactate and base deficit have been shown to be a predictor of morbidity and mortality in critically ill patients. Poor preoperative oxygenation appears to be one of the significant factors that affects early mortality in tetralogy of Fallot (TOF). There is little published literature evaluating the utility of serum lactate, base excess (BE), and oxygen partial pressure (PO 2 ) as simple, widely available, prognostic markers in patients undergoing surgical repair of TOF. This prospective, observational study was conducted in 150 TOF patients, undergoing elective intracardiac repair. PO 2 , BE, and lactate levels at three different time intervals were recorded. Arterial blood samples were collected after induction (T1), after cardiopulmonary bypass (T2), and 48 h (T3) after surgery in the Intensive Care Unit (ICU). To observe the changes in PO 2 , BE, and lactate levels over a period of time, repeated measures analysis was performed with Bonferroni method. The receiver operating characteristics (ROC) analysis was used to find area under curve (AUC) and cutoff values of various biomarkers for predicting mortality in ICU. The patients who could not survive showed significant elevated lactate levels at baseline (T1) and postoperatively (T2) as compared to patients who survived after surgery (P curve analysis showed that lactate levels (T3) have highest mortality predictive value (AUC: 96.9%) as compared to BE (AUC: 94.5%) and PO 2 (AUC: 81.1%). Serum lactate and BE may be used as prognostic markers to predict mortality in patients undergoing TOF repair. The routine analysis of these simple, fast, widely available, and cost-effective biomarkers should be encouraged to predict prognosis of TOF patients.

  15. Effect of Pregnancy on Ventricular and Aortic Dimensions in Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Cauldwell, Matthew; Quail, Michael A; Smith, Gillian S; Heng, Ee Ling; Ghonim, Sarah; Uebing, Anselm; Swan, Lorna; Li, Wei; Patel, Roshni R; Pennell, Dudley J; Steer, Philip J; Johnson, Mark R; Gatzoulis, Michael A; Babu-Narayan, Sonya V

    2017-07-23

    The aim was to assess whether cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects hemodynamic stability, in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation, or aortic root dilatation. This was a retrospective cohort study of women with repaired TOF with paired cardiovascular magnetic resonance scans before and after their first pregnancy (baseline RV end systolic volume index 49 mL/m 2 and RV end diastolic volume index 118 mL/m 2 ) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline cardiovascular magnetic resonance scan, time between follow-up of cardiovascular magnetic resonance scans, QRS duration, RV ejection fraction, and indexed RV end systolic and diastolic volume at baseline. Effect of pregnancy and time on parameters was assessed using mixed-effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions, or exercise data. There was an effect of pregnancy observed in both left ventricular end diastolic volume and left ventricular stroke volume, consistent with a sustained small increase in left ventricular stroke volume attributed to pregnancy (53-55 mL/m 2 ). Women with repaired TOF and with mild-to-moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that prepregnancy counseling is tailored to their individual clinical status. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  16. Plasma Levels of High Sensitivity Cardiac Troponin T in Adults with Repaired Tetralogy of Fallot

    Science.gov (United States)

    Lai, Clare T. M.; Wong, Sophia J.; Ip, Janice J. K.; Wong, Wai-keung; Tsang, Kwong-cheong; Lam, Wendy W. M.; Cheung, Yiu-fai

    2015-01-01

    Detectable low circulating level of cardiac troponin T (cTnT) may reflect subclinical myocardial injury. We tested the hypothesis that circulating levels of hs-cTnT are altered in adults with repaired tetralogy of Fallot (TOF) and associated with ventricular volume load and function. Eighty-eight TOF patients and 48 controls were studied. Plasma hs-cTnT levels were determined using a highly sensitive assay (hs-cTnT). The right (RV) and left ventricular (LV) volumes and ejection fraction (EF) were measured using 3D echocardiography and, in 52 patients, cardiac magnetic resonance (CMR). The median (interquartile range) for male and female patients were 4.87 (3.83–6.62) ng/L and 3.11 (1.00–3.87) ng/L, respectively. Thirty percent of female but none of the male patients had increased hs-cTnT levels. Female patients with elevated hs-cTnT levels, compared to those without, had greater RV end-diastolic and end-systolic volumes and LV systolic dyssynchrony index (all p < 0.05). For patient cohort only, hs-cTnT levels correlated positively with CMR-derived RV end-diastolic volume and negatively with echocardiography-derived LV and RV EF (all p < 0.05). Multiple linear regression identified sex and RV EF as significant correlates of log-transformed hs-cTnT levels. Increased hs-cTnT levels occur in 30% of female patients after TOF repair, and are associated with greater RV volumes and worse RV EF. PMID:26360613

  17. Prognostic value of left atrial size and function in adults with tetralogy of Fallot.

    Science.gov (United States)

    Baggen, Vivan J M; Schut, Anne-Rose W; Cuypers, Judith A A E; Witsenburg, Maarten; Boersma, Eric; van den Bosch, Annemien E; Roos-Hesselink, Jolien W

    2017-06-01

    Left atrial (LA) size predicts cardiovascular outcome in chronic heart failure. Its prognostic value in adults with repaired tetralogy of Fallot (ToF) is unknown. This study therefore investigated the association of LA size and function with cardiovascular events in adults with ToF. Clinically stable adults with ToF who visited the outpatient clinic between 2011 and 2013 underwent echocardiography and were prospectively followed for the occurrence of death, heart failure, hospitalizations, arrhythmia, thromboembolic events, and re-interventions. LA maximal, minimal and pre-A wave volume, area and length were measured on the apical four-chamber view. Total, passive and active emptying fractions were calculated. In total, 134 patients were included (median age 35 [IQR 29-45] years, 65% male, 91% NYHA I). Median follow-up was 40 [IQR 32-47] months. Patients with a dilated LA (≥34mL/m 2 , 43%) were at higher risk of cardiovascular events (n=33, adjusted HR 2.48 [1.09-5.62], P=0.030). Analysis of LA volumes as continuous variables yielded similar conclusions. In addition, LA length (adjusted HR 2.49 [1.51-4.09], P<0.001), total emptying fraction (adjusted HR 0.96 [0.93-0.99], P=0.008), and active emptying fraction (adjusted HR 0.92 [0.87-0.96], P=0.001) were significantly associated with cardiovascular events. Standardized HRs indicated that LA length was the strongest prognostic marker. In addition, none of the patients with a normally sized LA died or developed heart failure. LA size and function can provide relevant prognostic information in clinically stable adults with repaired ToF. Especially LA length may be a valuable additional tool in the risk stratification of these patients. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

    Science.gov (United States)

    Cortez, Daniel; Barham, Waseem; Ruckdeschel, Emily; Sharma, Nandita; McCanta, Anthony C; von Alvensleben, Johannes; Sauer, William H; Collins, Kathryn K; Kay, Joseph; Patel, Sonali; Nguyen, Duy T

    2017-08-01

    Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias. A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort. We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. History of AFL/IART was documented using electrocardiogram, Holter monitor, exercise stress test, implanted cardiac device, and electrophysiology study. P-R intervals, Pvm, QRS duration, and QRS vector magnitude were assessed from resting sinus-rhythm 12-lead electrocardiograms and identification of those with AFL/IART was determined. Fourteen patients (8.9%) were found to have AFL/IART. Pvm, QRS duration, and QRS vector magnitude significantly differentiated those with AFL/IART from those without on univariate analysis: 0.09 ± 0.04 vs 0.18 ± 0.07 mV, 161.3 ± 21.9 vs 137.7 ± 31.4 ms, and 1.2 (interquartile range, 1.0-1.2) vs 1.6 mV (1.0-2.3), respectively (P < 0.05 for each). The Pvm had the highest area under the ROC curve (0.88) and was the only significant predictor on multivariate analysis, with odds ratio of 0.02 (95% confidence interval: 0.01-0.53). P-R duration, MRI volumes, and right-heart hemodynamics did not significantly differentiate those with vs those without AFL/IART. In TOF patients undergoing pulmonary valve replacement, Pvm has significant value in predicting those with perioperative AFL/IART. These clinical features may help further evaluate TOF patients at risk for perioperative atrial arrhythmias. Prospective studies are warranted. © 2017 Wiley Periodicals, Inc.

  19. Acute and chronic effects of dysfunction of right ventricular outflow tract components on right ventricular performance in a porcine model: implications for primary repair of tetralogy of fallot

    NARCIS (Netherlands)

    Bove, Thierry; Bouchez, Stefaan; de Hert, Stefan; Wouters, Patrick; de Somer, Filip; Devos, Daniel; Somers, Pamela; van Nooten, Guido

    2012-01-01

    This study investigates the contribution of infundibular versus pulmonary valve (PV) dysfunction on right ventricular (RV) function in a porcine model. Clinical outcome after repair of tetralogy of Fallot is determined by the adaptation of the right ventricle to the physiological sequelae of the

  20. Tetralogy of Fallot and aortic root dilation: a long-term outlook.

    Science.gov (United States)

    Nagy, Christian D; Alejo, Diane E; Corretti, Mary C; Ravekes, William J; Crosson, Jane E; Spevak, Philip J; Ringel, Richard; Carson, Kathryn A; Khalil, Sara; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A; Traill, Thomas A; Holmes, Kathryn W

    2013-04-01

    Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1-48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥ 4 cm in 39 % (42 of 109), ≥ 4.5 cm in 21 % (23 of 109), ≥ 5 cm in 8 % (9 of 109), and ≥ 5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.

  1. Risk factors for morbidity in infants undergoing tetralogy of fallot repair

    Directory of Open Access Journals (Sweden)

    Alexander C Egbe

    2014-01-01

    Full Text Available Background: Primary repair of tetralogy of Fallot (TOF has low surgical mortality, but some patients still experience significant postoperative morbidity. Aim: To review our institutional experience with primary TOF repair, and identify predictors of intensive care unit (ICU morbidity. Settings and Design: Medium-sized pediatric cardiology program. Retrospective study. Subjects and Methods: We retrospectively reviewed all the patients with TOF and pulmonic stenosis who underwent primary repair in infancy at our institution from January 2001 to December 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. ICU morbidity was defined as prolonged ICU stay (≥7 days, and/or prolonged duration of mechanical ventilation (≥48 h. Statistical Analysis Used: Multiple logistic regression analysis. Results: Ninety-seven patients underwent primary surgical repair during the study period. The median age was 4.9 months (1-9 months and the median weight was 5.3 kg (3.1-9.8 kg. There was no early surgical mortality. Incidence of junctional ectopic tachycardia (JET and persistent complete heart block was 2 and 1%, respectively. The median length of ICU stay was 6 days (2-21 days and median duration of mechanical ventilation was 19 h (0-136 h. By multiple regression analysis, age and weight were independent predictors of length of ICU stay, while surgical era was an independent predictor of duration of mechanical ventilation. Conclusion: Primary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.

  2. Forced vital capacity predicts morbidity and mortality in adults with repaired tetralogy of Fallot.

    Science.gov (United States)

    Cohen, Katie E; Buelow, Matthew W; Dixon, Jennifer; Brazauskas, Ruta; Cohen, Scott B; Earing, Michael G; Ginde, Salil

    2017-07-01

    Abnormal lung function characterized by a reduced forced vital capacity (FVC) is common in adults with repaired tetralogy of Fallot (TOF) and is associated with previous thoracotomies and sternotomies. The impact of abnormal lung function on clinical outcomes in adult patients with repaired TOF is unclear. The aim of this study was to determine the impact of abnormal lung function on the outcome of hospitalization and death in adults with repaired TOF when analyzed with other traditional cardiac risk factors. Retrospective study of adults with repaired TOF, who underwent spirometry between 2000 and 2014. FVC < 60% of predicted was categorized as moderate-to-severely reduced lung function. Primary outcome measure was the combined clinical endpoint of death, cardiac transplantation, or nonelective hospitalization for primary cardiac or respiratory indication. A total of 122 patients were included. Average age at spirometry testing was 31 ± 10.1 years. FVC was < 60% predicted in 23 (19%) patients. During a mean follow-up period of 3.97 ± 2.65 years, 23 (19%) patients reached the combined clinical outcome of nonelective hospitalization and/or death. FVC < 60% predicted was independently associated with the risk for the combined clinical outcome (RR 6.68 (95% CI 2.49-17.94), P < .001). Abnormal pulmonary function characterized by reduced FVC is common in adults with repaired TOF. Patients with FVC < 60% predicted had a 6 times higher rate of hospitalization and/or death compared to those with FVC ≥ 60%. © 2017 Wiley Periodicals, Inc.

  3. Microarray analysis reveals key genes and pathways in Tetralogy of Fallot

    Science.gov (United States)

    He, Yue-E; Qiu, Hui-Xian; Jiang, Jian-Bing; Wu, Rong-Zhou; Xiang, Ru-Lian; Zhang, Yuan-Hai

    2017-01-01

    The aim of the present study was to identify key genes that may be involved in the pathogenesis of Tetralogy of Fallot (TOF) using bioinformatics methods. The GSE26125 microarray dataset, which includes cardiovascular tissue samples derived from 16 children with TOF and five healthy age-matched control infants, was downloaded from the Gene Expression Omnibus database. Differential expression analysis was performed between TOF and control samples to identify differentially expressed genes (DEGs) using Student's t-test, and the R/limma package, with a log2 fold-change of >2 and a false discovery rate of <0.01 set as thresholds. The biological functions of DEGs were analyzed using the ToppGene database. The ReactomeFIViz application was used to construct functional interaction (FI) networks, and the genes in each module were subjected to pathway enrichment analysis. The iRegulon plugin was used to identify transcription factors predicted to regulate the DEGs in the FI network, and the gene-transcription factor pairs were then visualized using Cytoscape software. A total of 878 DEGs were identified, including 848 upregulated genes and 30 downregulated genes. The gene FI network contained seven function modules, which were all comprised of upregulated genes. Genes enriched in Module 1 were enriched in the following three neurological disorder-associated signaling pathways: Parkinson's disease, Alzheimer's disease and Huntington's disease. Genes in Modules 0, 3 and 5 were dominantly enriched in pathways associated with ribosomes and protein translation. The Xbox binding protein 1 transcription factor was demonstrated to be involved in the regulation of genes encoding the subunits of cytoplasmic and mitochondrial ribosomes, as well as genes involved in neurodegenerative disorders. Therefore, dysfunction of genes involved in signaling pathways associated with neurodegenerative disorders, ribosome function and protein translation may contribute to the pathogenesis of TOF

  4. Rare Copy Number Variations in Adults with Tetralogy of Fallot Implicate Novel Risk Gene Pathways

    Science.gov (United States)

    Costain, Gregory; Merico, Daniele; Migita, Ohsuke; Liu, Ben; Yuen, Tracy; Rickaby, Jessica; Thiruvahindrapuram, Bhooma; Marshall, Christian R.; Scherer, Stephen W.; Bassett, Anne S.

    2012-01-01

    Structural genetic changes, especially copy number variants (CNVs), represent a major source of genetic variation contributing to human disease. Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease, but to date little is known about the role of CNVs in the etiology of TOF. Using high-resolution genome-wide microarrays and stringent calling methods, we investigated rare CNVs in a prospectively recruited cohort of 433 unrelated adults with TOF and/or pulmonary atresia at a single centre. We excluded those with recognized syndromes, including 22q11.2 deletion syndrome. We identified candidate genes for TOF based on converging evidence between rare CNVs that overlapped the same gene in unrelated individuals and from pathway analyses comparing rare CNVs in TOF cases to those in epidemiologic controls. Even after excluding the 53 (10.7%) subjects with 22q11.2 deletions, we found that adults with TOF had a greater burden of large rare genic CNVs compared to controls (8.82% vs. 4.33%, p = 0.0117). Six loci showed evidence for recurrence in TOF or related congenital heart disease, including typical 1q21.1 duplications in four (1.18%) of 340 Caucasian probands. The rare CNVs implicated novel candidate genes of interest for TOF, including PLXNA2, a gene involved in semaphorin signaling. Independent pathway analyses highlighted developmental processes as potential contributors to the pathogenesis of TOF. These results indicate that individually rare CNVs are collectively significant contributors to the genetic burden of TOF. Further, the data provide new evidence for dosage sensitive genes in PLXNA2-semaphorin signaling and related developmental processes in human cardiovascular development, consistent with previous animal models. PMID:22912587

  5. Fetal cardiac axis in tetralogy of Fallot: associations with prenatal findings, genetic anomalies and postnatal outcome.

    Science.gov (United States)

    Zhao, Y; Edington, S; Fleenor, J; Sinkovskaya, E; Porche, L; Abuhamad, A

    2017-07-01

    To compare prenatal findings, associated genetic anomalies and postnatal outcome in fetuses with tetralogy of Fallot (TOF) with normal cardiac axis (CAx) and those with abnormal CAx. In this retrospective cohort study, 85 cases diagnosed with TOF by prenatal ultrasound at our clinic between 2005 and 2015 were reviewed. Follow-up ultrasound and postnatal outcome were available for 68 cases. One case complicated with absent pulmonary valve syndrome and a further seven cases diagnosed postnatally with anomalies other than TOF were excluded from the study. The remaining 60 cases of postnatally confirmed TOF were divided according to CAx into two groups: those with normal CAx (n = 33) and those with abnormal CAx (n = 27). CAx was defined as the angle between the interventricular septum and midline of the fetal thorax at the level of the four-chamber view. CAx > 65° or < 25° was considered abnormal. Prenatal sonographic findings, associated genetic anomalies and postnatal outcome were compared between the two groups. Fetuses with TOF and abnormal CAx were more likely to have pulmonary atresia (40.7% vs 15.2%; P = 0.026) and right-sided aortic arch (48.1% vs 21.2%; P = 0.028) than those with normal CAx. Postnatal death occurred in 30.4% of infants with abnormal CAx vs 6.5% with normal CAx (P = 0.028). Incidence of tested genetic anomalies was similar between the two groups. In fetuses with TOF, abnormal CAx is associated with the presence of pulmonary atresia, right-sided aortic arch and a higher risk of postnatal death. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

  6. Aortopathy in adults with tetralogy of Fallot has a negative impact on the left ventricle.

    Science.gov (United States)

    Shiina, Yumi; Murakami, Tomoaki; Kawamatsu, Naoto; Niwa, Koichiro

    2017-02-01

    Aortic pressure wave reflection is significantly elevated in patients with congenital heart disease, even in children. Excessive aortic pressure wave reflection provokes cardiovascular events. To assess the influences of the enhanced pressure wave reflection on the left ventricle (LV) in adults with repaired tetralogy of Fallot (TOF). Prospectively, 51 consecutive adults with repaired TOF (35.5±11.6yrs., 25 males) were enrolled and non-invasively assessed the pressure wave reflection using HEM 9000AI. A surrogate maker of the aortic pressure wave reflection, radial augmentation index (rAI) was calculated as reflection wave divided by ejection wave. We also evaluated LV function using echocardiography and magnetic resonance images. Patients were divided into two groups: group A with rAI≧1SD and group B with rAI<1SD. The mean rAI in repaired TOF was 76.9±14.3%. In group A, indexed ascending aortic diameter, LV global longitudinal strain (GLS), LV global circumferential strain (GCS), LV early diastolic strain rate (SR), LV E/A, LV e' were significantly higher than them in group B. The indexed ascending aortic diameter significantly correlated with rAI (r=0.31, P<0.05). On univariate logistic analysis, body surface area, indexed ascending aortic diameter, GLS, GCS, early diastolic SR, LV E/A, LV mass index and creatinine were predictive factors of rAI≧1SD. On multivariate logistic analysis, LV E/A was the most significant predictive factor of rAI≧1SD (Odds ratio 0.044, 95%CI 0.002-0.98 and P<0.05). Aortic pressure wave reflection in adults with repaired TOF has a negative impact on LV function, particularly on diastolic function. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. Evaluation of ventricular function of patients with tetralogy of Fallot before and after operation by radionuclide myocardial imaging

    International Nuclear Information System (INIS)

    Zhang Nanbin; Wang Zengwei; Zhang Renfu; Chen Xianying; Liu Zhifan; Zhang Zhaozhong; Wang Kaigen

    1996-01-01

    Radionuclide myocardial imaging was utilized to evaluate the ventricular function of 43 patients with tetralogy of Fallot in preoperative and postoperative periods. The examination was made before and 25∼35 days after operation respectively. Ventricular ejection fraction, peak ejection rate and peak filling rate were used as indices for cardiac function. Before operation, the average left ventricular ejection fraction (LVEF) was 55%, the average left ventricular peak ejection rate (LVPER) was 2.95EDV/s and the average left ventricular peak filling rate (LVPFR) was 3.05EDV/s. After operation, the above three values were increased to 71%, 4.35EDV/s and 5.05EDV/s respectively, all with P<0.01. The function of right ventricle before operation was decreased and was significantly improved after operation (RVEF 42% vs 50%, RVPER 2.35EDV/s vs 3.00EDV/s and RVPFR 2.32EDV/s vs 3.36EDV/s, P<0.01). Poor right ventricular function in patients with tetralogy of Fallot is improved after surgical operation. Evaluation of the right ventricular function status is useful for assessing the degree of obstruction of right ventricular outflow tract

  8. Evaluation of knowledge-based reconstruction for magnetic resonance volumetry of the right ventricle in tetralogy of Fallot

    International Nuclear Information System (INIS)

    Nyns, Emile Christian Arie; Dragulescu, Andreea; Yoo, Shi-Joon; Grosse-Wortmann, Lars

    2014-01-01

    Cardiac magnetic resonance using the Simpson method is the gold standard for right ventricular volumetry. However, this method is time-consuming and not without sources of error. Knowledge-based reconstruction is a novel post-processing approach that reconstructs the right ventricular endocardial shape based on anatomical landmarks and a database of various right ventricular configurations. To assess the feasibility, accuracy and labor intensity of knowledge-based reconstruction in repaired tetralogy of Fallot (TOF). The short-axis cine cardiac MR datasets of 35 children and young adults (mean age 14.4 ± 2.5 years) after TOF repair were studied using both knowledge-based reconstruction and the Simpson method. Intraobserver, interobserver and inter-method variability were assessed using Bland-Altman analyses. Knowledge-based reconstruction was feasible and highly accurate as compared to the Simpson method. Intra- and inter-method variability for knowledge-based reconstruction measurements showed good agreement. Volumetric assessment using knowledge-based reconstruction was faster when compared with the Simpson method (10.9 ± 2.0 vs. 7.1 ± 2.4 min, P < 0.001). In patients with repaired tetralogy of Fallot, knowledge-based reconstruction is a feasible, accurate and reproducible method for measuring right ventricular volumes and ejection fraction. The post-processing time of right ventricular volumetry using knowledge-based reconstruction was significantly shorter when compared with the routine Simpson method. (orig.)

  9. Evaluation of knowledge-based reconstruction for magnetic resonance volumetry of the right ventricle in tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Nyns, Emile Christian Arie; Dragulescu, Andreea [University of Toronto, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto (Canada); Yoo, Shi-Joon; Grosse-Wortmann, Lars [University of Toronto, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto (Canada); University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto (Canada)

    2014-12-15

    Cardiac magnetic resonance using the Simpson method is the gold standard for right ventricular volumetry. However, this method is time-consuming and not without sources of error. Knowledge-based reconstruction is a novel post-processing approach that reconstructs the right ventricular endocardial shape based on anatomical landmarks and a database of various right ventricular configurations. To assess the feasibility, accuracy and labor intensity of knowledge-based reconstruction in repaired tetralogy of Fallot (TOF). The short-axis cine cardiac MR datasets of 35 children and young adults (mean age 14.4 ± 2.5 years) after TOF repair were studied using both knowledge-based reconstruction and the Simpson method. Intraobserver, interobserver and inter-method variability were assessed using Bland-Altman analyses. Knowledge-based reconstruction was feasible and highly accurate as compared to the Simpson method. Intra- and inter-method variability for knowledge-based reconstruction measurements showed good agreement. Volumetric assessment using knowledge-based reconstruction was faster when compared with the Simpson method (10.9 ± 2.0 vs. 7.1 ± 2.4 min, P < 0.001). In patients with repaired tetralogy of Fallot, knowledge-based reconstruction is a feasible, accurate and reproducible method for measuring right ventricular volumes and ejection fraction. The post-processing time of right ventricular volumetry using knowledge-based reconstruction was significantly shorter when compared with the routine Simpson method. (orig.)

  10. Transcatheter balloon dilation for recurrent right ventricular outflow tract obstruction following valve-sparing repair of tetralogy of Fallot.

    Science.gov (United States)

    Gellis, Laura; Banka, Puja; Marshall, Audrey; Emani, Sitaram; Porras, Diego

    2015-10-01

    Valve-sparing repair in patients with tetralogy of Fallot (TOF) carries the risk of residual or recurrent right ventricular outflow tract (RVOT) obstruction, which is often treated with transcatheter balloon dilation (BD). The outcomes and associated complications of BD of the RVOT in this scenario remain unknown. Retrospective review of the records of the Department of Cardiology at Boston Children's Hospital from 2000 to 2013 was performed. 34 patients had initial valve-sparing repair of tetralogy of Fallot followed by BD of the RVOT during the study period. Following BD, the RVOT gradient decreased from a median of 43 mm Hg (range 13 to 79 mm Hg) to 28 mm Hg (range 0 to 73 mm Hg) (P 1 and a final RVOT gradient of ≥40 post-BD were associated with shorter freedom from reintervention (P < 0.001). BD in patients with recurrent RVOT obstruction following valve-sparing repair of TOF acutely reduces the RVOT gradient, but commonly results in increased PR and is associated with a high reintervention rate. Patients with stenosis solely at the level of the valve had a better response to this type of intervention. © 2015 Wiley Periodicals, Inc.

  11. Associations between N-terminal pro-B-type natriuretic peptide and cardiac function in adults with corrected tetralogy of Fallot

    NARCIS (Netherlands)

    J.A. Eindhoven (Jannet); M.E. Menting (Myrthe); A.E. van den Bosch (Annemien); J.A.A.E. Cuypers (Judith); T.P.E. Ruys (Titia); M. Witsenburg (Maarten); J.S. Vletter-McGhie (Jackie); H. Boersma (Eric); J.W. Roos-Hesselink (Jolien)

    2014-01-01

    textabstractBackground Amino-terminal B-type natriuretic peptide (NT-proBNP) may detect early cardiac dysfunction in adults with tetralogy of Fallot (ToF) late after corrective surgery. We aimed to determine the value of NT-proBNP in adults with ToF and establish its relationship with

  12. Lifetime Costs and Outcomes of Repair of Tetralogy of Fallot Compared to Natural Progression of the Disease: Great Ormond Street Hospital Cohort

    Science.gov (United States)

    Hunter, Rachael Maree; Isaac, Mark; Frigiola, Alessandra; Blundell, David; Brown, Kate; Bull, Kate

    2013-01-01

    Background Tetralogy of Fallot is a congenital heart disease that requires surgical repair without which survival through childhood is extremely rare. The aim of this paper is to use data from the mandatory follow-up of patients with Tetralogy of Fallot to model the health-related costs and outcomes over the first 55-years of life. Method A decision analytical model was developed to establish costs and outcomes for patients up to 55 years after diagnosis and first repair of Tetralogy of Fallot compared to natural progression. Data from Adult Congenital Heart Disease (ACHD) centres that follow up Tetralogy of Fallot patients and Great Ormond Street Hospital (GOSH), London, United Kingdom (UK) medical records was used to establish the cost and effectiveness of current interventions. Data from a Czech cohort was used for the natural, no intervention condition. Results The average cost per patient of a repair for Tetralogy of Fallot was £26,938 (SE = £4,140). The full life time cost per patient, with no discount rate, was £65,310 (95% CI £64,981–£65,729); £56,559 discounted (95% CI £56,159–£56,960). Patients with a repair had an average of 35 Quality Adjusted Life Years (QALYs) per patient over 55 years undiscounted and 20.16 QALYs discounted. If the disorder was left to take its natural course, patients on average had a total of 3 QALYs per patient with no discount rate and 2.30 QALYs discounted. Conclusion A model has been developed that provides an estimate of the value for money of an expensive repair of a congenital heart disease. The model could be used to test the cost-effectiveness of making amendments to the care pathway. PMID:23533645

  13. Repair of tetralogy of Fallot in or beyond the fourth decade of life.

    Science.gov (United States)

    Talwar, Sachin; Meena, Ajay; Choudhary, Shiv Kumar; Saxena, Anita; Kothari, Shyam Sunder; Juneja, Rajnish; Airan, Balram

    2014-01-01

    Patients with tetralogy of Fallot (TOF) undergoing surgery in adulthood represent a challenge. We report our experience with such patients in or beyond the fourth decade of life. Retrospective cohort. Multispeciality tertiary level referral center Forty-one (age 30-52 years) with TOF undergoing surgery between January 2002 and March 2013. The hospital records of these patients were analyzed. None. Early and late morbidity and mortality, duration of mechanical ventilatory support, inotropic score, intensive care unit and hospital stay, and correlation with various parameters. Significant aortopulmonary collaterals were present in 28 patients; these were occluded in cardiac catheterization laboratory prior to repair. Median intraoperative right:left ventricular pressure ratio was 0.40 (range 0.2-0.8). Median inotropic score was 10 (range 5-30). Median duration of mechanical ventilation was 12 hours (range 6-48 hours). Preoperative oxygen saturation was negatively correlated with inotropic score (P = .001, r = -0.485), mechanical ventilatory support (P = .003, r = -0.460), intensive care unit stay (P = .004, r = -0.442), and hospital stay (P = .028, r = -353). Inotropic score was higher in patients with aortopulmonary collaterals (n = 28, P = .03), high preoperative hematocrit (n = 29, P = .029), and with right ventricular dysfunction (n = 6, P = .05). Patients with right ventricular outflow tract gradient >80 mm Hg (n = 19) had prolonged hospital stay (P = .002). Patients undergoing pure transatrial repair (n = 24) showed lower inotropic score (P = .045), less intensive care unit (P = .04), and hospital stay (P = .031). There were two early and two late deaths (one from trauma and one from unknown etiology). Median follow-up was 42 months. Thirty-one patients were in New York Heart Association class II and six were in class III. Repair of TOF in and beyond the fourth decade of life is feasible with acceptable results. Patients with high hematocrit, lower oxygen

  14. T wave alternans threshold late after repair of tetralogy of Fallot

    Science.gov (United States)

    Cheung, Michael M H.; Weintraub, Robert G.; Cohen, Richard J.; Karl, Tom R.; Wilkinson, James L.; Davis, Andrew M.

    2002-01-01

    INTRODUCTION: Sustained microvolt-level T wave alternans (TWA) is a marker of increased risk for malignant ventricular arrhythmia. There is a significant risk of arrhythmia and sudden death after repair of congenital heart disease. The aim of this study was to determine the prevalence and characteristics of TWA after repair of tetralogy of Fallot (TOF). METHODS AND RESULTS: TWA was evaluated during bicycle exercise in 49 subjects who had consecutively undergone transatrial-transpulmonary repair. Median values for age, age at repair, and follow-up duration were 14.9 years (11.5-20.8), 1.6 years (0.2-4.9), and 11.6 years (9.4-17.2), respectively. All patients were in New York Heart Association functional class I and were asymptomatic. Median QRS duration was 120 msec (80-150). Sustained TWA was detected in 7 (23%) of 31 subjects with adequate tests. In these 7 subjects, median onset heart rate (HR) was 120 (98-155). Median HR threshold as a percentage of predicted maximum HR (220 - age) was 58% (48-77). Sustained TWA prevalence was not significantly different compared with normal subjects (7/31 vs 9/83; P = 0.1). Onset HR in the TOF group was significantly lower [mean (SD) of 122 (20) vs 139 (12), P < 0.05]. In the TOF group with sustained TWA, the TWA occurred in 4 of 7 at <60% predicted maximum HR versus 1 of 9 normal subjects (P < 0.05); 3 of 7 had onset HR <120 versus 0 of 9 normal subjects (P < 0.03). There was no significant difference in age, gender, transannular patch use, restrictive right ventricular physiology, QRS duration, QTc, QT/QRS dispersion, or nonsustained ventricular tachycardia in subjects with or those without sustained TWA. CONCLUSION: The onset HR for sustained TWA is significantly lower after repair of TOF. Further study is required to determine whether this represents an increased risk for arrhythmia in this patient group.

  15. VEGF Gene Polymorphisms are Associated with Risk of Tetralogy of Fallot

    Science.gov (United States)

    Li, Xiang; Liu, Chao-Liang; Li, Xiao-Xia; Li, Qing-Chen; Ma, Li-Ming; Liu, Gao-Li

    2015-01-01

    Background The aim of this study was to investigate associations of 3 common polymorphisms in the VEGF gene, −2578C>A, −634C>G, and 936C>T, with risk of tetralogy of Fallot (TOF) in Chinese Han children. Material/Methods From January 2010 to June 2013, a total of 400 pediatric subjects were recruited, including 160 cases with TOF (TOF group) and 240 healthy controls (control group). The genotypes of 3 common VEGF polymorphisms, −2578C>A, −634C>G, and 936C>T, were analyzed by polymerase chain reaction restriction fragment length polymorphism. All data were analyzed with SPSS 18.0 software. Results No significant differences were observed in body mass index or sex between TOF patients and controls (both P>0.05), but significant differences in age and family history of TOF were observed between the 2 groups (both PA of VEGF was correlated with a significantly increased risk of TOF, and TOF risk in A allele carrier was 1.54-fold higher than that of C allele carrier (OR=1.54, 95%CI=1.14–2.09, P=0.005); the statistical significance was still present after Bonferroni correction (Pc=0.045). GG genotype in −634C>G of VEGF gene was also associated with an increased risk of TOF, and TOF risk in patients with G allele was 1.62-fold higher compared to patients with C allele (OR=1.62, 95%CI=1.19–2.21, P=0.002); the statistical significance was still present after Bonferroni correction (Pc=0.018). Interestingly, T allele in VEGF 936C>T polymorphism is associated with a decreased TOF risk (OR=0.65, 95%CI=0.49–0.87, P=0.003, the statistical significance was still present after Bonferroni correction (Pc=0.027). The result of logistic regression analysis revealed that −2578C>A, −634C>G, and 936C>T genotypes are independently related to the prevalence of TOF (all PA and −634C>G, may be associated with an increased TOF risk, while 936C>T polymorphism may be associated with decreased TOF risk. PMID:26558525

  16. Basic arterial blood gas biomarkers as a predictor of mortality in tetralogy of Fallot patients

    Directory of Open Access Journals (Sweden)

    Vandana Bhardwaj

    2017-01-01

    Full Text Available Background: Serum lactate and base deficit have been shown to be a predictor of morbidity and mortality in critically ill patients. Poor preoperative oxygenation appears to be one of the significant factors that affects early mortality in tetralogy of Fallot (TOF. There is little published literature evaluating the utility of serum lactate, base excess (BE, and oxygen partial pressure (PO 2 as simple, widely available, prognostic markers in patients undergoing surgical repair of TOF. Materials and Methods: This prospective, observational study was conducted in 150 TOF patients, undergoing elective intracardiac repair. PO 2 , BE, and lactate levels at three different time intervals were recorded. Arterial blood samples were collected after induction (T1, after cardiopulmonary bypass (T2, and 48 h (T3 after surgery in the Intensive Care Unit (ICU. To observe the changes in PO 2 , BE, and lactate levels over a period of time, repeated measures analysis was performed with Bonferroni method. The receiver operating characteristics (ROC analysis was used to find area under curve (AUC and cutoff values of various biomarkers for predicting mortality in ICU. Results: The patients who could not survive showed significant elevated lactate levels at baseline (T1 and postoperatively (T2 as compared to patients who survived after surgery (P < 0.001. However, in nonsurvivors, the BE value decreased significantly in the postoperative period in comparison to survivors (−2.8 ± 4.27 vs. 5.04 ± 2.06 (P < 0.001. In nonsurvivors, there was a significant fall of PO 2 to a mean value of 59.86 ± 15.09 in ICU (T3, whereas those who survived had a PO 2 of 125.86 ± 95.09 (P < 0.001. The ROC curve analysis showed that lactate levels (T3 have highest mortality predictive value (AUC: 96.9% as compared to BE (AUC: 94.5% and PO 2 (AUC: 81.1%. Conclusion: Serum lactate and BE may be used as prognostic markers to predict mortality in patients undergoing TOF repair. The

  17. Ten-year outcomes of monomorphic ventricular tachycardia catheter ablation in repaired tetralogy of Fallot.

    Science.gov (United States)

    Laredo, Mikaël; Frank, Robert; Waintraub, Xavier; Gandjbakhch, Estelle; Iserin, Laurence; Hascoët, Sebastien; Himbert, Caroline; Gallais, Yves; Hidden-Lucet, Françoise; Duthoit, Guillaume

    2017-05-01

    Monomorphic ventricular tachycardia (MVT) is common in adults with repaired tetralogy of Fallot (TOF), and is associated with sudden cardiac death. Management of MVT is not defined, and results of catheter ablation (CA) are limited. To evaluate long-term outcomes of MVT CA in repaired TOF. Thirty-four patients (mean age 32±10.3 years; 59% male) with repaired TOF underwent CA for symptomatic MVT between 1990 and 2012 in our centre; direct-current ablation (DCA) was used in 6%, radiofrequency followed by DCA in 29% and radiofrequency alone in 65%. Right ventricular (RV) dysfunction was present in 35% and left ventricular (LV) dysfunction in 21%. Mean numbers of clinical and induced MVTs were 1 and 2, respectively. Mean VT rate was 225±95bpm. Ablation targeted a single site (range 1-2), which was RV outflow tract in 85%. Primary success, defined as ventricular tachycardia (VT) termination during CA and final non-inducibility, was obtained in 82%. Seven patients (21%) required redo ablation in the first 3 months (before 2004; DCA). No death related to CA occurred. Mean follow-up time was 9.5±5.2 years. Antiarrhythmic therapy was discontinued in 71%. There were two cases of sudden cardiac death and four VT recurrences. Freedom from death and arrhythmia recurrence was 94% at 5 years, 81% at 10 years and 70% at 20 years. Global survival was 91% at 20 years. Baseline LV ejection fraction<60% was significantly associated with ventricular arrhythmia recurrence (hazard ratio 16.4, 95% confidence interval 1.8-147; P=0.01). CA can safely address macroreentrant MVT in repaired TOF patients with an acceptable long-term rate of recurrence in this high-risk population. Anatomical classification of isthmuses with electroanatomical mapping provides reproducible endpoints for CA. Attention should be given to LV systolic function in risk assessment and selection of candidates for implantable cardioverter-defibrillator. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  18. Effects of Balloon Pulmonary Valvuloplasty as Preoperative Palliation for Tetralogy of Fallot.

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    Kim, Geena; Ban, Gil Ho; Lee, Hyoung Doo; Sung, Si Chan; Kim, Hyungtae; Choi, Kwang Ho

    2016-07-01

    We investigated the effect of balloon pulmonary valvuloplasty (BPV) as a palliative procedure for patients with tetralogy of Fallot (TOF). This was a retrospective single-center study conducted between 2008 and 2014. We classified patients into three groups according to palliation: treatment with BPV (group I), with a Blalock-Taussig shunt (group II), and with infundibulectomy (group III). The growth of the pulmonary valve (PV) annulus and need for transannular patching (TAP) during total correction were compared between groups. Forty-eight patients were enrolled: 31, 10, and 7 in groups I, II, and III, respectively. The mean ages at palliation were 26.3 ± 23.6, 21.5 ± 16.4, and 15.2 ± 4.2 days in groups I, II, and III, respectively (P = .867). The success rate of BPV was 90.3%. The initial PV z-score before palliation did not differ between groups I, II, and III (-3.14 ± 1.44, -3.84 ± 1.47, and -3.71 ± 1.68; P = .494); the preoperative PV z-score before total correction was larger in group I than in group II (-1.19 ± 1.14 and -3.05 ± 1.19; P = .001), and also larger in group III than in group II (group III: -1.10 ± 0.90; P = .003). The change in pulse oxygen saturation differed significantly between the three groups (P = .031), particularly between groups I and II (P = .010), and the change in PV z-score was greater in groups I and III than in group II (P < .001). TAP during total correction was performed in 29.0%, 90.0%, and 16.7% of groups I, II, and III, respectively (P = .001; group I vs. II, P = .001; group II vs. III, P = .008). BPV was an effective and safe palliation that increased pulmonary blood flow and promoted growth of the PV annulus in selected symptomatic newborns with TOF. © 2016 Wiley Periodicals, Inc.

  19. Cardiovascular autonomic dysfunction and carotid stiffness in adults with repaired tetralogy of Fallot.

    Science.gov (United States)

    Novaković, Marko; Prokšelj, Katja; Starc, Vito; Jug, Borut

    2017-06-01

    Adults after surgical repair of tetralogy of Fallot (ToF) may have impaired vascular and cardiac autonomic function. Thus, we wanted to assess interrelations between heart rate variability (HRV) and heart rate recovery (HRR), as parameters of cardiac autonomic function, and arterial stiffness, as a parameter of vascular function, in adults with repaired ToF as compared to healthy controls. In a case-control study of adults with repaired ToF and healthy age-matched controls we measured: 5-min HRV variability (with time and frequency domain data collected), carotid artery stiffness (through pulse-wave analysis using echo-tracking ultrasound) and post-exercise HRR (cycle ergometer exercise testing). Twenty-five patients with repaired ToF (mean age 38 ± 10 years) and 10 healthy controls (mean age 39 ± 8 years) were included. Selected HRR and HRV (time-domain) parameters, but not arterial stiffness were significantly reduced in adults after ToF repair. Moreover, a strong association between late/slow HRR (after 2, 3 and 4 min) and carotid artery stiffness was detected in ToF patients (r = -0.404, p = 0.045; r = -0.545, p = 0.005 and r = -0.545, p = 0.005, respectively), with statistical significance retained even after adjusting for age, gender, resting heart rate and β-blockers use (r = -0.393, p = 0.024 for HRR after 3 min). Autonomic cardiac function is impaired in patients with repaired ToF, and independently associated with vascular function in adults after ToF repair, but not in age-matched healthy controls. These results might help in introducing new predictors of cardiovascular morbidity in a growing population of adults after surgical repair of ToF.

  20. Power loss and right ventricular efficiency in patients after tetralogy of Fallot repair with pulmonary insufficiency: clinical implications.

    Science.gov (United States)

    Fogel, Mark A; Sundareswaran, Kartik S; de Zelicourt, Diane; Dasi, Lakshmi P; Pawlowski, Tom; Rome, Jack; Yoganathan, Ajit P

    2012-06-01

    To quantify right ventricular output power and efficiency and correlate these to ventricular function in patients with repaired tetralogy of Fallot. This might aid in determining the optimal timing for pulmonary valve replacement. We reviewed the cardiac catheterization and magnetic resonance imaging data of 13 patients with tetralogy of Fallot (age, 22 ± 17 years). Using pressure and flow measurements in the main pulmonary artery, cardiac output and regurgitation fraction, right ventricular (RV) power output, loss, and efficiency were calculated. The RV function was evaluated using cardiac magnetic resonance imaging. The RV systolic power was 1.08 ± 0.62 W, with 20.3% ± 8.6% power loss owing to 41% ± 14% pulmonary regurgitation (efficiency, 79.7% ± 8.6%; 0.84 ± 0.73 W), resulting in a net cardiac output of 4.24 ± 1.82 L/min. Power loss correlated significantly with the indexed RV end-diastolic and end-systolic volume (R = 0.78, P = .002 and R = 0.69, P = .009, respectively). The normalized RV power output had a significant negative correlation with RV end-diastolic and end-systolic volumes (both R = -0.87, P = .002 and R = -0.68, P = .023, respectively). A rapid decrease occurred in the RV power capacity with an increasing RV volume, with the curve flattening out at an indexed RV end-diastolic and end-systolic volume threshold of 139 mL/m(2) and 75 mL/m(2), respectively. Significant power loss is present in patients with repaired tetralogy of Fallot and pulmonary regurgitation. A rapid decrease in efficiency occurs with increasing RV volume, suggesting that pulmonary valve replacement should be done before the critical value of 139 mL/m(2) and 75 mL/m(2) for the RV end-diastolic and end-systolic volume, respectively, to preserve RV function. Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  1. Tetralogia de Fallot e sua repercussão na saúde bucal Repercussion of tetralogy of Fallot on oral health

    Directory of Open Access Journals (Sweden)

    Cristiane Meira Assunção

    2008-03-01

    Full Text Available OBJETIVO: Relatar o caso de um paciente com tetralogia de Fallot e sua condição bucal. DESCRIÇÃO DO CASO: Paciente do gênero masculino atendido no Curso de Especialização em Odontopediatria da Escola de Aperfeiçoamento Profissional da Associação Brasileira de Odontologia da seção Paraná dos cinco aos sete anos de idade. O exame clínico intrabucal inicial revelou quadro de cárie severa da infância. Durante o acompanhamento, verificou-se a erupção dos primeiros molares permanentes com a presença de defeitos de desenvolvimento de esmalte e perda de estrutura dental. À anamnese, a mãe relatou que o paciente foi portador de tetralogia de Fallot e que a cirurgia corretora foi realizada com um ano e 11 meses de idade. COMENTÁRIOS: A formação do esmalte dental dos primeiros molares permanentes ocorre a partir do primeiro mês de vida e é finalizada entre dois e quatro anos de idade. Neste caso, tal etapa coincidiu com o período anterior à cirurgia para correção da tetralogia de Fallot, fato que pode ter interferido negativamente no processo de mineralização dos primeiros molares permanentes. Os defeitos de desenvolvimento do esmalte podem levar a perda de estrutura dental, favorecendo o aparecimento da cárie dentária. Dessa forma, o odontopediatra deve realizar um diagnóstico precoce dessas alterações e intervir preventivamente para evitar a perda de tais dentes. Uma anamnese bem detalhada e a maior interação entre pediatras e odontopediatras podem proporcionar melhor acompanhamento de crianças com fatores de risco para apresentar defeitos de desenvolvimento do esmalte dentário.OBJECTIVE: To report a case of a child with tetralogy of Fallot and his oral conditions. CASE DESCRIPTION: A male patient was assisted at the Pediatric Dentristry Specialization Course of the Brazilian Dentistry Association in the State of Paraná, Brazil, from five to seven years old. At the first intrabucal examination, the child

  2. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support

    Science.gov (United States)

    2011-01-01

    Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Although right ventricular volume load due to severe pulmonary regurgitation can be tolerated for many years, there is now evidence that the compensatory mechanisms of the right ventricular myocardium ultimately fail and that if the volume load is not eliminated or reduced by pulmonary valve replacement the dysfunction might be irreversible. Cardiovascular magnetic resonance (CMR) has evolved during the last 2 decades as the reference standard imaging modality to assess the anatomic and functional sequelae in patients with repaired TOF. This article reviews the pathophysiology of chronic right ventricular volume load after TOF repair and the risks and benefits of pulmonary valve replacement. The CMR techniques used to comprehensively evaluate the patient with repaired TOF are reviewed and the role of CMR in supporting clinical decisions regarding pulmonary valve replacement is discussed. PMID:21251297

  3. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2005-02-01

    Full Text Available OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1 veia pulmonar encunhada, 2 colaterais aortopulmonares, 3 aorta torácica e 4 ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.OBJECTIVE: To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study. METHODS: Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1 wedged pulmonary vein; 2 aortopulmonary collaterals; 3 thoracic aorta; and 4 ductus arteriosus or systemic-pulmonary shunt. RESULTS: In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus

  4. Left ventricular dysfunction in repaired tetralogy of Fallot: incidence and impact on atrial arrhythmias at long term-follow up.

    Science.gov (United States)

    Ait Ali, Lamia; Trocchio, GianLuca; Crepaz, Roberto; Stuefer, Josef; Stagnaro, Nicola; Siciliano, Valeria; Molinaro, Sabrina; Sicari, Rosa; Festa, Pierluigi

    2016-09-01

    Left ventricle (LV) systolic dysfunction in repaired tetralogy of Fallot (TOF) has been identified as a risk factor for functional status and adverse outcome. The aims of this cross-sectional followed by a prospective study were: (1) to evaluate the prevalence of LV systolic dysfunction in a large cohort of adults with repaired tetralogy of Fallot, (2) to test the relationship between LV systolic dysfunction and other known risk factors and (3) to evaluate the impact of LV systolic dysfunction on adverse cardiac events. In a multicenter study, 237 adults repaired TOF (58 % males, age 30 ± 10 years) were evaluated by cardiac magnetic resonance (CMR). Demographics, surgical history, ECG, Echo-Color Doppler and follow-up data were recorded. LV was dilated (Z value >2) in 16 patients (6 %), however 56 patients (23.6 %) had a reduced LV systolic function left ventricle ejection fraction (LVEF) (Z value <-2). Patients with LV systolic dysfunction were mainly males (82 %), had reduced right ventricle ejection fraction (RVEF), and higher right and left Late Gadolinium Enhanced scores. In a multivariate regression analysis male gender and RVEF resulted to be independent factors associated to LV systolic dysfunction. Atrial arrhythmias were the main adverse cardiac event at the follow-up and were associated to higher biventricular volumes and lower biventricular ejection fraction (EF); however multivariable analysis identified age, right ventricle end-diastolic volume (RVEDVi) and tricuspid regurgitation as independents factors associated to atrial arrhythmias. At long term follow-up at least ¼ of repaired TOF has LV dysfunction. Lower LVEF is associated to male gender and lower RVEF.

  5. Mechanical stress is associated with right ventricular response to pulmonary valve replacement in patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Tang, Dalin; Yang, Chun; Del Nido, Pedro J; Zuo, Heng; Rathod, Rahul H; Huang, Xueying; Gooty, Vasu; Tang, Alexander; Billiar, Kristen L; Wu, Zheyang; Geva, Tal

    2016-03-01

    Patients with repaired tetralogy of Fallot account for a substantial proportion of cases with late-onset right ventricular failure. The current surgical approach, which includes pulmonary valve replacement/insertion, has yielded mixed results. Therefore, it may be clinically useful to identify parameters that can be used to predict right ventricular function response to pulmonary valve replacement. Cardiac magnetic resonance data before and 6 months after pulmonary valve replacement were obtained from 16 patients with repaired tetralogy of Fallot (8 male, 8 female; median age, 42.75 years). Right ventricular ejection fraction change from pre- to postpulmonary valve replacement was used as the outcome. The patients were divided into group 1 (n = 8, better outcome) and group 2 (n = 8, worst outcome). Cardiac magnetic resonance-based patient-specific computational right ventricular/left ventricular models were constructed, and right ventricular mechanical stress and strain, wall thickness, curvature, and volumes were obtained for analysis. Our results indicated that right ventricular wall stress was the best single predictor for postpulmonary valve replacement outcome with an area under the receiver operating characteristic curve of 0.819. Mean values of stress, strain, wall thickness, and longitudinal curvature differed significantly between the 2 groups with right ventricular wall stress showing the largest difference. Mean right ventricular stress in group 2 was 103% higher than in group 1. Computational modeling and right ventricular stress may be used as tools to identify right ventricular function response to pulmonary valve replacement. Large-scale clinical studies are needed to validate these preliminary findings. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  6. Tratamento cirúrgico da tetralogia de Fallot no primeiro ano de vida Surgical treatment of tetralogy of Fallot in the first year of life

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    Fernando MORAES NETO

    2000-06-01

    surgical treatment of the classic of tetralogy of Fallot in the first year of life and particularly to define possible advantages of early primary repair. MATERIAL AND METHODS: Between March 1986 and September 1999, 56 children under one age tetralogy of Fallot underwent surgical treatment. Thirty-six (64.3% were male and 20 (35.7% female, ranging in age from 1 to 11 months (mean 6.5 ± 2.9 mo.. Weight ranged from 3 to 10 kg (mean 6.3 ± 1.8 kg. The patients were divided into 2 groups: Group I, consisted of 26 children operated on between 1986 and 1996, submitted to a Blalock-Taussig shunt; Group II, comprised of 30 children operated on consecutively since 1996 and submitted to intracardiac repair. RESULTS: In Group I, there were 2 (7.6% early and 1 (3.8% late deaths. No postoperative com-plications were observed in the remaining children. In Group II, 2 (6.6% early deaths and 1 (3.3% non-cardiac late death of a have occurred. Only 2 children had non-significant postoperative complications and 16 presented signs of congestive heart failure. The mortality in both groups was not statistically significant. CONCLUSIONS: In the authors' experience, ideal management of children with classical Fallot's tetralogy in the first year of life consists of the intracardiac repair since it has the same surgical risk as palliation.

  7. Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation

    International Nuclear Information System (INIS)

    Chan, Sherwin S.; Whitehead, Kevin K.; Kim, Timothy S.; Fu, Gregory L.; Fogel, Mark A.; Harris, Matthew A.; Keller, Marc S.

    2015-01-01

    There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m 2 vs. 118 ± 30 mL/m 2 ), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation. (orig.)

  8. Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Sherwin S. [Children' s Mercy Hospital and Clinics, Department of Radiology, Kansas City, MO (United States); Whitehead, Kevin K.; Kim, Timothy S.; Fu, Gregory L.; Fogel, Mark A.; Harris, Matthew A. [Children' s Hospital of Philadelphia, Department of Cardiology, Philadelphia, PA (United States); Keller, Marc S. [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States)

    2015-09-15

    There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m{sup 2} vs. 118 ± 30 mL/m{sup 2}), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation. (orig.)

  9. Does the prophylactic and therapeutic use of beta-blockers in preoperative patients with tetralogy of Fallot significantly prevent and treat the occurrence of cyanotic spells?

    Science.gov (United States)

    Fanous, Eliana; Mogyorósy, Gábor

    2017-10-01

    A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Does the use of beta-blockers significantly prevent and treat the occurrence of cyanotic spells in preoperative infants with tetralogy of Fallot?' Altogether, 80 papers were found using the reported search, of which 6 represented the best evidence to answer the clinical question. The author, journal, date, country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. The participants in the papers reviewed were uncorrected (native or palliated) tetralogy of Fallot patients, all younger than 18 years of age, with some patients younger than 1 year. Each study reviewed included at least 10 patients, and all the studies were case series. Although even the most recent studies found were from 30 years ago, their data remain relevant. Several reviews reported either cases of overdosage or changes in efficacy of treatment after long-term usage. Four of the 6 case reviews demonstrated a decrease in the number of recurring cyanotic spells in at least 66% of the participants, following the introduction of beta-blockers. We can therefore conclude that the use of beta-blockers prevents the occurrence of cyanotic spells in preoperative patients with tetralogy of Fallot. There were insufficient data to establish optimum dosages or duration of treatment. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  10. Existence of mutations in the homeodomain-encoding region of NKX2.5 gene in Iranian patients with tetralogy of Fallot

    Science.gov (United States)

    Kheirollahi, Majid; Khosravi, Fereshteh; Ashouri, Saeideh; Ahmadi, Alireza

    2016-01-01

    Background: Tetralogy of Fallot (TOF), the most common cyanotic heart defect and one of the most common congenital heart diseases, occurs mostly sporadically and nonsyndromically. The underlying molecular genetic mechanism is not known. Therefore, the existence of mutations in the homeodomain-encoding region of NKX2.5 gene in Iranian patients with tetralogy of Fallot is evaluated. Materials and Methods: In the present study, we analyzed the peripheral blood samples of27 patients in order to find any mutation in the 180 bp homeodomain-encoding region of NKX2.5 gene, which is known to be involved in heart development and diseases. DNA was extracted and all the samples were amplified by polymerase chain reaction (PCR) and sequenced. Results: Twenty-seven patients were included in the study. Twenty-five of them were infants and children (6 days to 11 years of age), one was a teenager (14-years of age), and another was a 33-year-old man [mean ± standard deviation (SD): 5.80 ± 3.90 years]. Thirteen patents were males (mean ± SD: 6.587077 ± 5.02 years) and 14 were females (mean ± SD: 5.0726 ± 2.81 years). One synonymous variant, i.e., c.543G>A was identified in one patient. Conclusion: Mutations in the homeodomain-encoding region of NKX2.5 gene may not have an outstanding role in etiology of tetralogy of Fallot patients in Iran. PMID:27904570

  11. NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

    Science.gov (United States)

    Paolino, Annalisa; Hussain, Tarique; Pavon, Antonio; Velasco, Maria Nieves; Uribe, Sergio; Ordoñez, Antonio; Valverde, Israel

    2017-02-01

    The goal of this study is to evaluate whether NT-proBNP plasma levels may help as a screening biomarker for monitoring right ventricular dilatation, pulmonary regurgitation and the onset of heart failure in patients with repaired Tetralogy of Fallot. Our single-centre observational prospective study involved 43 patients (15.1 years, SD = 8) with corrected Tetralogy of Fallot. Data collection included: clinical parameters (electrocardiogram, chest X-ray, NYHA scale, time since last surgery), biochemistry (NT-proBNP levels) and MRI values (ventricular volumetry, pulmonary flow assessment). Mean time since last surgery was 13.5 years (SD = 7.8). There was a statistically significant correlation between the NT-proBNP levels (187.4 pg/ml, SD = 154.9) and right ventricular dilatation for both the right ventricular end-diastolic volume (124.9 ml/m 2 , SD = 31.2) (Pearson = 0.19, p Tetralogy of Fallot, NT-proBNP levels correlate with right ventricular dilatation and the degree of pulmonary regurgitation. Ambulatory determination of NT-proBNP might be an easy, readily available and cost-effective alternative for MRI follow-up evaluation of these patients.

  12. A case of schizophrenia comorbid for tetralogy of Fallot treated with clozapine: further considerations on a role for 22q.11.2 in the proneness for seizures

    Directory of Open Access Journals (Sweden)

    Kashiwagi H

    2017-08-01

    Full Text Available Hiroko Kashiwagi,1 Satoru Ikezawa,2 Tomiki Sumiyoshi,3 Atsuko Kadono,4 Kazuhiko Segawa,5 Kazuyoshi Takeda,1 Mayu Omori,1 Hisako Taguchi,1 Naotsugu Hirabayashi1 1Department of Forensic Psychiatry, 2Department of Psychiatry, 3Department of Clinical Epidemiology, Translational Medical Center, National Center Hospital of Neurology and Psychiatry, Kodaira, Tokyo, 4Saitama Psychiatric Medical Center, Kitaadatigun, Saitama, 5Department of General Medicine, National Center Hospital of Neurology and Psychiatry, Kodaira, Tokyo, Japan Abstract: We present a case of schizophrenia comorbid for tetralogy of Fallot, without chromosome 22q.11.2 deletion or duplication, treated successfully with a combination of clozapine and antiepileptic drugs. Although clozapine by itself initially triggered convulsive seizures, we continued it with co-administration of valproate and topiramate. This combined treatment did not affect cardiac function of the patient, who experienced a favorable clinical course in terms of symptomatology and functional outcomes. To our knowledge, we provide the first report on a patient with tetralogy of Fallot, in whom 22q.11.2 was not deleted and clozapine-induced seizures were observed. Keywords: schizophrenia, clozapine, tetralogy of Fallot, seizure, copy number variants

  13. Clinical implications of hypothermic ventricular fibrillation versus beating-heart technique during cardiopulmonary bypass for pulmonary valve replacement in patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Lee, Ji-Hyun; Lee, Ji-Eun; Shin, Jungho; Song, In-Kyung; Kim, Hee-Soo; Kim, Chong-Sung; Kim, Woong-Han; Kim, Jin-Tae

    2017-09-01

    This study aimed to compare the effects of hypothermic ventricular fibrillation and beating-heart techniques during cardiopulmonary bypass (CPB) on postoperative outcomes after simple pulmonary valve replacement in patients with repaired tetralogy of Fallot (TOF). We retrospectively reviewed the data of 47 patients with repaired tetralogy of Fallot at a single institution, who received pulmonary valve replacement under the ventricular fibrillation or beating-heart technique without cardioplegic cardiac arrest during CPB between January 2005 and April 2015. The patients were divided into fibrillation (n = 32) and beating-heart (n = 15) groups. On comparing these groups, the fibrillation group had a larger sinotubular junction (27.1 ± 4.6 vs 22.1 ± 2.4 mm), had a longer operation duration (396 ± 108 vs 345 ± 57 min), required more postoperative transfusions (2.1 ± 2.6 vs 5.0 ± 6.3 units) and had a higher vasoactive-inotropic score at intensive care unit admission (8.0 vs 10, all P tetralogy of Fallot. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  14. Fresh Autologous Pericardium to Reconstruct the Pulmonary Valve at the Annulus When Tetralogy of Fallot Requires a Transannular Patch at Midterm.

    Science.gov (United States)

    Pande, Shantanu; Sharma, Jugal K; Siddartha, C R; Bansal, Anubhav; Agarwal, Surendra K; Tewari, Prabhat; Kapoor, Aditya

    2016-06-01

    Tetralogy of Fallot often requires reconstruction of the right ventricular outflow tract with a transannular patch (TAP), but this renders the pulmonary valve incompetent and eventually leads to right ventricular dysfunction. We retrospectively evaluated the efficacy of a reconstructed pulmonary valve and annulus in 70 patients who underwent, from December 2006 through December 2010, complete correction of tetralogy of Fallot. We divided the 70 patients into 2 groups in accordance with whether they required (n=50) or did not require (n=20) a TAP. We used autologous untreated pericardium to fashion the TAP and to create both an annulus of the correct size and a competent pulmonary valve with native leaflets. We evaluated the efficiency of this procedure both functionally and anatomically. The median age of the patients was 11 years (range, 2-38 yr). There were 56 males, with no significant difference in sexual distribution between groups. The clinical follow-up was 88% for 57.5 months, and the echocardiographic follow-up was 80% for 36 months. There was no significant difference in outflow gradient or in the occurrence of pulmonary insufficiency between the TAP group (none, 31; mild, 12; moderate, 6; and severe, 1) and the No-TAP group (none, 16; moderate, 2; and severe, 2) (P=0.59). Nor was there any thickening or calcification in the constructed valves. We conclude that pulmonary valves constructed of untreated autologous pericardium performed as well as native valves after total tetralogy of Fallot correction at midterm.

  15. Analgesia de parto em paciente com tetralogia de Fallot não corrigida: relato de caso Analgesia de parto en paciente con tetralogía de Fallot no corregida: relato de caso Labour analgesia in parturient with uncorrected tetralogy of Fallot: case report

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    Florentino Fernandes Mendes

    2005-02-01

    obstétrica fue la de parto vía baja, siendo realizada analgesia de parto a través de bloqueo peridural con bupivacaína a 0,125% y fentanil (100 µg y colocación de catéter peridural. Después de 1h30 minutos del inicio de la analgesia, ocurrió el nacimiento. El peso del recién nacido fue 1485 g y el índice de Apgar 6 y 8 en el primero y en el quinto minutos, respectivamente. La paciente permaneció estable y sin alteraciones hemodinámicas y/o electrocardiográficas. CONCLUSIONES: La elección de la técnica anestésica es de fundamental importancia en el manoseo de las pacientes con tetralogía de Fallot no corregidas. Condiciones favorables del cuello y buena dinámica uterina, particularmente en aquellas pacientes sin historia de síncope, se vuelven imprescindibles para una buena indicación de la analgesia de parto.BACKGROUND AND OBJECTIVES: Although tetralogy of Fallot is the most common cyanotic congenital heart disease, national publications correlating this condition with anesthetic practice are scarce. This report aimed at presenting a case of labor epidural analgesia in a patient with uncorrected tetralogy of Fallot diagnosed during gestation. CASE REPORT: Patient 26 years old, 1.54 m, 56 kg, 32 weeks and 5 days of gestational age, who had been diagnosed with tetralogy of Fallot during gestation. Patient was admitted in labour. After obstetric evaluation and decision for natural birth, epidural analgesia was performed with 0.125% bupivacaine associated to 100µg fentanyl through a catheter. Patient gave birth 1 hour and 30 minutes after the procedure. The newborn weighed 1485 grams and had an Apgar score of 6 and 8 at one and five minutes, respectively. Patient remained stable, with no hemodynamic or ECG changes. CONCLUSIONS: Selecting the appropriate anesthetic technique is extremely important when managing patients with uncorrected tetralogy of Fallot. Favorable uterine dynamics and cervical conditions, particularly in patients with no history of

  16. Outcomes of Pulmonary Valve Replacement for Correction Pulmonary Insufficiency after Primary Repair of Tetralogy of Fallot (TOF

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    Mohammad Abbassi Teshnisi

    2016-09-01

    Full Text Available Background Total correction of Tetralogy of Fallot (TOF anomaly in early childhood has been practiced in many centers with good results, but in some of patients after few years sever Pulmonary valve insufficiency occurred. Materials and Methods At a cross- sectional study from January 2015 to January 2016, 10 patients who had history of primary repair of TOF with free pulmonary insufficiency (PI that underwent of pulmonary valve replacement (PVR with bioprosthetic valves were evaluated. Results Themean age of patients was 6.5 + 0.753 years old (ranged 8-12 years old and male to female ratio was 6/4. The mean of Intensive care unit (ICU stay and Hospital stay was 4.5+ 0.712 days (ranged 3-8 and 11.5+ 0.357 days (ranged 9- 16. Mean of cardiopulmonary bypass time and operation time was 45 + 0.684 min (ranged 32-60 and 83 + 0.317 min (ranged 65-112. In this study we did not find any mortality and ventricular arrhythmia and Heart block. There was only one case (10% with superficial wound infection that was controlled. At 6 months follow up, all of patients were alive, but Echocardiography sign of Right Ventricular (RV failure was present in 2 patients (20% recently. Conclusion Although for Pulmonary insufficiency after primary TOF repair there is controversial in studies, but we had good results of PVR with Bioprosthesis in TOF patients.

  17. Identification of Region-Specific Myocardial Gene Expression Patterns in a Chronic Swine Model of Repaired Tetralogy of Fallot.

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    Sabine Charron

    Full Text Available Surgical repair of Tetralogy of Fallot (TOF is highly successful but may be complicated in adulthood by arrhythmias, sudden death, and right ventricular or biventricular dysfunction. To better understand the molecular and cellular mechanisms of these delayed cardiac events, a chronic animal model of postoperative TOF was studied using microarrays to perform cardiac transcriptomic studies. The experimental study included 12 piglets (7 rTOF and 5 controls that underwent surgery at age 2 months and were further studied after 23 (+/- 1 weeks of postoperative recovery. Two distinct regions (endocardium and epicardium from both ventricles were analyzed. Expression levels from each localization were compared in order to decipher mechanisms and signaling pathways leading to ventricular dysfunction and arrhythmias in surgically repaired TOF. Several genes were confirmed to participate in ventricular remodeling and cardiac failure and some new candidate genes were described. In particular, these data pointed out FRZB as a heart failure marker. Moreover, calcium handling and contractile function genes (SLN, ACTC1, PLCD4, PLCZ, potential arrhythmia-related genes (MYO5B, KCNA5, and cytoskeleton and cellular organization-related genes (XIRP2, COL8A1, KCNA6 were among the most deregulated genes in rTOF ventricles. To our knowledge, this is the first comprehensive report on global gene expression profiling in the heart of a long-term swine model of repaired TOF.

  18. Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair

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    Park, Han Ki

    2013-01-01

    Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF. PMID:23807889

  19. Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair.

    Science.gov (United States)

    Yoo, Byung Won; Park, Han Ki

    2013-06-01

    Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.

  20. Pulmonary stenosis and pulmonary regurgitation: both ends of the spectrum in residual hemodynamic impairment after tetralogy of Fallot repair

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    Byung Won Yoo

    2013-06-01

    Full Text Available Repair of tetralogy of Fallot (TOF has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS and free pulmonary regurgitation (PR at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.

  1. Perioperative Management of Subarachnoid Hemorrhage in a Patient with Alagille Syndrome and Unrepaired Tetralogy of Fallot: Case Report

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    Juan Fiorda-Diaz

    2017-12-01

    Full Text Available Alagille syndrome (ALGS is a genetic disorder associated with multisystem dysfunction involving the hepatic, cardiovascular, and neurologic systems. Tetralogy of Fallot (TOF, a congenital cardiac anomaly, is commonly found in these patients. Patients with ALGS may also have an increased risk of cerebrovascular abnormalities and bleeding. Ruptured cerebral aneurysm and subarachnoid hemorrhage (SAH may be developed, increasing the incidence of morbidity and mortality. Advances in neuroimaging and neurosurgery have allowed early identification and treatment of such vascular abnormalities, improving patients’ outcomes and reducing life-threatening complications such as intracranial bleeding. Authors describe the perioperative management of a patient with ALGS and TOF who was admitted to the emergency department due a ruptured intracranial aneurysm with concomitant SAH. Surgical treatment included diagnostic cerebral arteriography with coil embolization of a left posterior communicating artery aneurysm, and placement of right external ventricular drain (EVD. The combination of neuroprotective anesthetic techniques, fast emergence from anesthesia, and maintenance of intraoperative hemodynamic stability led to a successful perioperative management. A multidisciplinary approach in specialized centers is essential for the treatment of patients with SAH, especially in patients with ALGS and complex congenital heart disease such as TOF.

  2. Mechanisms of Left Ventricular Dysfunction Assessed by Layer-Specific Strain Analysis in Patients With Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Yamada, Mariko; Takahashi, Ken; Kobayashi, Maki; Yazaki, Kana; Takayasu, Hirobumi; Akimoto, Katsumi; Kishiro, Masahiko; Inage, Akio; Yoshikawa, Tadahiro; Park, In-Sam; Nakanishi, Keisuke; Kawasaki, Shiori; Shimizu, Toshiaki

    2017-05-25

    Left ventricular (LV) dysfunction in patients with repaired tetralogy of Fallot (rTOF) is an important risk factor for adverse outcomes. The aim of this study was to assess the details and time course of such LV dysfunction using layer-specific strain analysis by echocardiography.Methods and Results:The 66 patients with rTOF (mean age, 16.3±9.3 years) were divided into 3 groups (T1: 4-10 years, T2: 11-20 years, T3: 21-43 years), and 113 controls of similar age (mean age, 17.2±9.3 years) were divided into 3 corresponding groups (C1, C2, and C3). Layer-specific longitudinal strain (LS) and circumferential strain (CS) of 3 myocardial layers (endocardial, midmyocardial, and epicardial) were determined by echocardiography. Basal and papillary endocardial CS values were decreased in T1 compared with C1. With the exception of papillary epicardial CS, basal/papillary CS and LS of all 3 layers decreased in T2 compared with C2. Excepting papillary epicardial CS, all other values were decreased in T3 compared with C3. Potential myocardial damage was found in the endocardium at the basal and papillary levels of the LV in young patients with rTOF, extending from the endocardium to the epicardium and from the base to the apex. This is the possible time course of LV dysfunction in patients with rTOF.

  3. Proper Orthogonal Decomposition and Dynamic Mode Decomposition in the Right Ventricle after Repair of Tetralogy of Fallot

    Science.gov (United States)

    Mikhail, Amanda; Kadem, Lyes; di Labbio, Giuseppe

    2017-11-01

    Tetralogy of Fallot accounts for 5% of all cyanotic congenital heart defects, making it the most predominant today. Approximately 1660 cases per year are seen in the United States alone. Once repaired at a very young age, symptoms such as pulmonary valve regurgitation seem to arise two to three decades after the initial operation. Currently, not much is understood about the blood flow in the right ventricle of the heart when regurgitation is present. In this study, the interaction between the diastolic interventricular flow and the regurgitating pulmonary valve are investigated. This experimental work aims to simulate and characterize this detrimental flow in a right heart simulator using time-resolved particle image velocimetry. Seven severities of regurgitation were simulated. Proper Orthogonal Decomposition (POD) and Dynamic Mode Decomposition (DMD) revealed intricate coherent flow structures. With regurgitation severity, the modal energies from POD are more distributed among the modes while DMD reveals more unstable modes. This study can contribute to the further investigation of the detrimental effects of right ventricle regurgitation.

  4. The Cardiovascular Effects of Obesity on Ventricular Function and Mass in Patients after Tetralogy of Fallot Repair.

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    Fogel, Mark A; Pawlowski, Thomas; Keller, Marc S; Cohen, Meryl S; Goldmuntz, Elizabeth; Diaz, Laura; Li, Christine; Whitehead, Kevin K; Harris, Matthew A

    2015-08-01

    To determine the cardiovascular effects of obesity on patients with tetralogy of Fallot (TOF) repair. Ventricular performance measures were compared between obese (body mass index [BMI] ≥95%), overweight (85% ≤BMI <95%), and normal weight subjects (BMI <85%) in a retrospective review of patients with TOF who underwent cardiac magnetic resonance from 2005-2010. Significance was P < .05. Of 260 consecutive patients with TOF, 32 were obese (12.3%), 48 were overweight (18.5%), and 180 were normal weight (69.2%). Biventricular mass was increased in obese compared with normal weight patients with right ventricular mass more affected than left ventricular mass. Obese patients demonstrated decreased biventricular end-diastolic volume (EDV) and stroke volume (SV) when indexed to body surface area (BSA) with an increased heart rate when compared with normal weight patients; cardiac index, ejection fraction, and pulmonary regurgitation fraction were similar. When indexed to ideal BSA, biventricular EDV and SV were similar. EDV and SV for overweight patients were nearly identical to normal weight patients with ventricular mass in between the other 2 groups. Approximately 12% of patients after TOF repair referred for cardiac magnetic resonance in a tertiary referral center are obese with increased biventricular mass. Obese patients and normal weight patients have similar cardiac indices, however, when indexed to actual BSA, obese patients demonstrate decreased EDV and SV with increased heart rate and similar cardiac indices. When indexed to ideal BSA, no differences in biventricular volumes were noted. Copyright © 2015. Published by Elsevier Inc.

  5. Angiojet thrombectomy for Blalock-Taussig shunt and pulmonary artery thrombus in an infant with tetralogy of fallot

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    Brody Wehman

    2014-01-01

    Full Text Available We describe a new technique for treatment of shunt thrombosis in infants with complex anatomical defects. A 2-month-old girl with Tetralogy of Fallot underwent placement of a modified Blalock-Taussig shunt (MBTS at day of life (DOL 6 with revision at DOL 20. Following this surgery, the patient became hypotensive and hypoxic with new evidence of lack of flow through the MBTS on echocardiography. Angiography showed an occluded MBTS and right pulmonary artery with patent distal branches with normal pulmonary venous return. Balloon angioplasty was attempted but failed to fully recanalize the right pulmonary artery (RPA and MBTS. An AngioJet catheter was then passed through the shunt and RPA to perform rheolyticthrombectomy. Subsequent angiogram showed a widely patent RPA and MBTS. An echocardiogram at 1-month post-thrombectomy showed a widely patent MBTS with continuous flow seen entering both branch pulmonary arteries. The AngioJet system for thrombectomy provides a viable option for complex thrombus removal in patients refractory to other methods.

  6. Left ventricular regional myocardial motion and twist function in repaired tetralogy of Fallot evaluated by magnetic resonance tissue phase mapping

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    Chang, Meng-Chu; Peng, Hsu-Hsia; Wu, Ming-Ting; Weng, Ken-Pen; Su, Mao-Yuan; Menza, Marius; Huang, Hung-Chieh

    2018-01-01

    We aimed to characterise regional myocardial motion and twist function in the left ventricles (LV) in patients with repaired tetralogy of Fallot (rTOF) and preserved LV global function. We recruited 47 rTOF patients and 38 age-matched normal volunteers. Tissue phase mapping (TPM) was performed for evaluating the LV myocardial velocity in longitudinal, radial, and circumferential (Vz, Vr, and VOe) directions in basal, middle, and apical slices. The VOe peak-to-peak (PTP) during systolic phases, the rotation angle of each slice, and VOe inconsistency were computed for evaluating LV twist function and VOe dyssynchrony. As compared to the controls, the rTOF patients presented decreased RV ejection fraction (RVEF) (p = 0.002) and preserved global LV ejection fraction (LVEF). They also demonstrated decreased systolic and diastolic Vz in several LV segments and higher diastolic Vr in the septum (all p < 0.05). A lower VOe PTP, higher VOe inconsistency, and reduced peak net rotation angle (all p < 0.05) were observed. The aforementioned indices demonstrated an altered LV twist function in rTOF patients in an early disease stage. MR TPM could provide information about early abnormalities of LV regional motion and twist function in rTOF patients with preserved LV global function. (orig.)

  7. Left ventricular regional myocardial motion and twist function in repaired tetralogy of Fallot evaluated by magnetic resonance tissue phase mapping

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    Chang, Meng-Chu; Peng, Hsu-Hsia [National Tsing Hua University, Department of Biomedical Engineering and Environmental Sciences, Hsinchu (China); Wu, Ming-Ting [Kaohsiung Veterans General Hospital, Department of Radiology, Kaohsiung (China); National Yang-Ming University, Faculty of Medicine, Taipei (China); Weng, Ken-Pen [National Yang-Ming University, Faculty of Medicine, Taipei (China); Kaohsiung Veterans General Hospital, Department of Pediatrics, Kaohsiung (China); Shu-Zen Junior College of Medicine and Management, Department of Physical Therapy, Kaohsiung (China); Su, Mao-Yuan [National Taiwan University Hospital, Department of Medical Imaging, Taipei (China); Menza, Marius [Medical Center University of Freiburg, Faculty of Medicine, University of Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Huang, Hung-Chieh [Kaohsiung Veterans General Hospital, Department of Radiology, Kaohsiung (China)

    2018-01-15

    We aimed to characterise regional myocardial motion and twist function in the left ventricles (LV) in patients with repaired tetralogy of Fallot (rTOF) and preserved LV global function. We recruited 47 rTOF patients and 38 age-matched normal volunteers. Tissue phase mapping (TPM) was performed for evaluating the LV myocardial velocity in longitudinal, radial, and circumferential (Vz, Vr, and VOe) directions in basal, middle, and apical slices. The VOe peak-to-peak (PTP) during systolic phases, the rotation angle of each slice, and VOe inconsistency were computed for evaluating LV twist function and VOe dyssynchrony. As compared to the controls, the rTOF patients presented decreased RV ejection fraction (RVEF) (p = 0.002) and preserved global LV ejection fraction (LVEF). They also demonstrated decreased systolic and diastolic Vz in several LV segments and higher diastolic Vr in the septum (all p < 0.05). A lower VOe PTP, higher VOe inconsistency, and reduced peak net rotation angle (all p < 0.05) were observed. The aforementioned indices demonstrated an altered LV twist function in rTOF patients in an early disease stage. MR TPM could provide information about early abnormalities of LV regional motion and twist function in rTOF patients with preserved LV global function. (orig.)

  8. Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation.

    Science.gov (United States)

    Ebishima, Hironori; Kurosaki, Kenichi; Yoshimatsu, Jun; Shiraishi, Isao

    2017-08-01

    This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes. The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation. We included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, ptetralogy of Fallot.

  9. Assessment of inter-atrial, inter-ventricular, and atrio-ventricular interactions in tetralogy of Fallot patients after surgical correction. Insights from two-dimensional speckle tracking and three-dimensional echocardiography.

    Science.gov (United States)

    Abd El Rahman, Mohamed; Raedle-Hurst, Tanja; Rentzsch, Axel; Schäfers, Hans-Joachim; Abdul-Khaliq, Hashim

    2015-10-01

    We aimed to assess biatrial size and function, interactions on atrial and ventricular levels, and atrio-ventricular coupling in patients after tetralogy of Fallot repair. A total of 34 patients with a mean age of 20.9±9 years, and 35 healthy controls, underwent two-dimensional speckle tracking echocardiography for ventricular and atrial strain measurements and real-time three-dimensional echocardiography to assess ventricular and atrial volumes. When compared with controls, tetralogy of Fallot patients had significantly reduced right atrial peak atrial longitudinal strain (ptetralogy of Fallot group, left ventricular ejection fraction was negatively related to the right ventricular end-systolic volume normalised to body surface area (r=-0.62, ptetralogy of Fallot patients, biatrial dysfunction exists and can be quantified via two-dimensional speckle tracking echocardiography as well as real-time three-dimensional echocardiography. Different forms of interactions on atrial and ventricular levels are evident among such cohorts.

  10. Low risk of pulmonary valve implantation after a policy of transatrial repair of tetralogy of Fallot delayed beyond the neonatal period: the Melbourne experience over 25 years.

    Science.gov (United States)

    d'Udekem, Yves; Galati, John C; Rolley, Glenda J; Konstantinov, Igor E; Weintraub, Robert G; Grigg, Leeanne; Ramsay, James M; Wheaton, Gavin R; Hope, Sarah; Cheung, Michael H; Brizard, Christian P

    2014-02-18

    The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period. Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown. The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases. There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively). During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction. Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  11. Right ventricular outflow tract systolic function correlates with exercise capacity in patients with severe right ventricle dilatation after repair of tetralogy of Fallot.

    Science.gov (United States)

    Luo, Shuhua; Li, Jianhua; Yang, Dan; Zhou, Yaxin; An, Qi; Chen, Yucheng

    2017-05-01

    The relationship between exercise capacity and right ventricular (RV) components function in repaired tetralogy of Fallot patients with severely dilated right ventricles is poorly understood. The aim of this study was to characterize the exercise capacity and its relationship to RV global and components function in repaired tetralogy of Fallot patients with RV end-diastolic volume index  >150 ml/m 2 , a currently accepted threshold for pulmonary valve replacement. The medical records and results of cardiac magnetic resonance imaging and cardiopulmonary exercise testing of 25 consecutive eligible patients were reviewed. Twenty age- and gender-matched normal subjects were enrolled as cardiac magnetic resonance control. End-diastolic, end-systolic and stroke volumes, and ejection fraction (EF) were determined for the total RV and its components. Of the 25 patients, 44% maintained normal exercise capacity. RV outlet EF was higher ( P  = 0.02) and RV incisions smaller ( P  = 0.04) in patients with normal exercise capacity than those with subnormal exercise capacity. Predicted peak oxygen consumption correlated better with the RV outflow tract EF than with the EF of other components of the RV or the global EF ( r  = 0.59; P  = 0.002). Multivariate analysis showed the RV outflow tract EF to be the only independent predictor of exercise capacity (ß = 0.442; P  = 0.02). Exercise capacity is preserved in some tetralogy of Fallot patients with severe RV dilatation. RV outflow tract EF is independently associated with exercise capacity in such patients, and could be a reliable determinant of intrinsic RV performance. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  12. Avaliação pré e pós-operatória da tetralogia de Fallot por ressonância magnética Magnetic resonance imaging pre- and postoperative evaluation of tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Renata Junqueira Moll Bernardes

    2004-08-01

    Full Text Available A proposta deste trabalho foi estudar, prospectivamente, por ressonância magnética (RM, pacientes com diagnóstico de tetralogia de Fallot e avaliar a validade da RM como exame pré e pós-operatório nesses pacientes. Foram estudados, através de seqüências spin-eco e angiografia por RM (angio-RM com utilização de gadolínio, 20 pacientes com idades entre 1 e 29 anos. Onze pacientes apresentavam a forma clássica da tetralogia de Fallot e nove, a forma extrema (tetralogia de Fallot com atresia pulmonar. O estudo permitiu a avaliação adequada da aorta, possibilitando a análise quanto à presença ou não de dilatação e quanto à posição do arco aórtico em todos os casos. Houve também uma excelente visualização das artérias pulmonares principal, direita e esquerda, possibilitando a avaliação qualitativa quanto à presença de dilatação, hipoplasia, estenose, ou quanto à ausência do vaso. Os resultados obtidos indicam que a RM, incluindo técnicas de angio-RM com meio de contraste, é um método de grande utilidade no estudo pré e pós-operatório de pacientes com tetralogia de Fallot, porque permite a obtenção de informações anatômicas importantes e complementares à ecocardiografia, podendo ser considerada uma alternativa ao cateterismo cardíaco, principalmente na avaliação da anatomia vascular pulmonar.The purpose of this study was to assess the usefulness of magnetic resonance imaging (MRI in the pre- and postoperative evaluation of patients with tetralogy of Fallot. Twenty patients aged 1 to 29 years were prospectively evaluated with black-blood and contrast-enhanced angiographic techniques, 11 with the classic form of tetralogy of Fallot and 9 with tetralogy of Fallot and pulmonary atresia. MRI studies provided adequate visualization of the aorta that was classified as dilated or not dilated, and definition of its position in all cases. The use of contrast-enhanced MR angiographic techniques provided

  13. Regional ventricular performance and exercise training in children and young adults after repair of tetralogy of Fallot: randomized controlled pilot study.

    Science.gov (United States)

    Duppen, Nienke; Geerdink, Lianne M; Kuipers, Irene M; Bossers, Sjoerd S M; Koopman, Laurens P; van Dijk, Arie P J; Roos-Hesselink, Jolien W; De Korte, Chris L; Helbing, Willem A; Kapusta, Livia

    2015-04-01

    Public-health guidelines recommend patients with congenital heart disease to exercise. Studies have shown that patients with congenital heart disease can improve physical exercise capacity. The effect of training on regional ventricular performance has hardly been studied. We performed a pilot study to assess whether an exercise training program would result in adverse changes of regional ventricular performance in patients with corrected tetralogy of Fallot. Multicenter prospective randomized controlled pilot study in patients with tetralogy of Fallot aged 10 to 25 years. A 12-week standardized aerobic dynamic exercise training program (3 one-hour sessions per week) was used. Pre- and post-training cardiopulmonary exercise tests, MRI, and echocardiography, including tissue-Doppler imaging, were performed. Patients were randomized to the exercise group (n=28) or control group (n=20). One patient in the exercise group dropped out. Change in tissue-Doppler imaging parameters was similar in the exercise group and control group (change in right ventricle free wall peak velocity E' exercise group, 0.8±2.6 cm/s; control group, 0.9±4.1; peak velocity A' exercise group, 0.4±2.4 m/s; control group 4.6±18.1 cm/s). This randomized controlled pilot study provides preliminary data suggesting that regional ventricular performance is well maintained during 3-month aerobic dynamic exercise training in children and young adults with repaired tetralogy of Fallot. This information might help patients adhere to current public-health guidelines. URL: http//:www.trialregister.nl. Unique identifier: NTR2731. © 2015 American Heart Association, Inc.

  14. Long-term follow-up in repaired tetralogy of fallot: can deformation imaging help identify optimal timing of pulmonary valve replacement?

    Science.gov (United States)

    Sabate Rotes, Anna; Bonnichsen, Crystal R; Reece, Chelsea L; Connolly, Heidi M; Burkhart, Harold M; Dearani, Joseph A; Eidem, Benjamin W

    2014-12-01

    Novel echocardiographic techniques based on myocardial deformation have not been extensively evaluated to assess right ventricular (RV) and left ventricular (LV) response after pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot. Between 2003 and 2012, 133 patients undergoing first-time PVR after tetralogy of Fallot repair underwent echocardiographic assessment at Mayo Clinic. The last echocardiogram before PVR and 1 year after surgery were retrospectively analyzed with Velocity Vector Imaging. Mean age at PVR was 35.5 ± 16.2 years (54% women). Longitudinal peak systolic strain and strain rate before PVR were low: for the left ventricle, -14.8 ± 3.5% and -0.8 ± 0.2 sec(-1), and for the right ventricle, -16.2 ± 4.1% and -0.9 ± 0.3 sec(-1), respectively. There was no significant change in either parameter after surgery. A close correlation between LV and RV deformational parameters was found before PVR and was maintained after surgery. In the multivariate analysis, patients with better LV and RV peak systolic strain preoperatively were found to have better LV and RV peak systolic strain after surgery (P = .004 and P = .006, respectively). However, patients with the most improvement in deformation were those with worse RV function preoperatively (P = .002). Mean New York Heart Association class at early follow-up improved from 2.2 ± 0.8 to 1.2 ± 0.6 (P tetralogy of Fallot undergoing PVR, and there was no significant change after surgery. However, preoperative systolic deformational parameters were predictive of postoperative ventricular function and New York Heart Association class after PVR and may be helpful to identify optimal timing for surgical intervention in this cohort. Copyright © 2014 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  15. Dilatation of the ascending aorta is associated with presence of aortic regurgitation in patients after repair of tetralogy of Fallot

    Science.gov (United States)

    Ordovas, Karen Gomes; Keedy, Alexander; Naeger, David M.; Kallianos, Kimberly; Foster, Elyse; Liu, Jing; Saloner, David; Hope, Michael D.

    2016-01-01

    To evaluate the association between aortic morphology and elasticity with aortic regurgitation in surgically corrected of tetralogy of Fallot (TOF) patients. We retrospectively identified 72 consecutive patients with surgically corrected TOF and 27 healthy controls who underwent cardiac MRI evaluation. Velocity-encoded cine MRI was used to quantify degree of aortic regurgitation (AR) in TOF patients. Ascending aorta diameters were measured at standard levels on MRA images. Aortic pulse-wave velocity (PWV) was quantified with MRI. Morphological and functional MRI variables were compared between groups of TOF patients with and without clinically relevant AR and controls. The association between aortic morphology and elasticity with the presence of AR was evaluated using univariate and multivariate logistic regression. The majority of TOF patients had only trace AR. Nine TOF patients (12 %) had an AR fraction higher than 15 %. Indexed aorta diameter at the sinotubular junction (p = 0.007), at the RPA level (p = 0.006), and low left ventricular ejection fraction (LVEF) (p = 0.015) showed the strongest associations with the presence of at least mild AR, which persisted after controlling for age and gender. Increased ascending aorta dimension is associated with AR in patients after repair of TOF. LVEF was also low in the group of patients with relevant AR compared to those without, suggesting even mild to moderate AR may contribute to LV dysfunction in these patients. Enlarged ascending aorta may be an indication for precise quantification of regurgitant fraction with MRI, since symptomatic patients may need aortic valve repair when moderate regurgitation is present. PMID:27240599

  16. Delayed onset of tricuspid valve flow in repaired tetralogy of Fallot: an additional mechanism of diastolic dysfunction and interventricular dyssynchrony

    Directory of Open Access Journals (Sweden)

    Benson Lee N

    2011-08-01

    Full Text Available Abstract Background Diastolic dysfunction of the right ventricle (RV is common after repair of tetralogy of Fallot. While restrictive physiology in late diastole has been well known, dysfunction in early diastole has not been described. The present study sought to assess the prevalence and mechanism of early diastolic dysfunction of the RV defined as delayed onset of the tricuspid valve (TV flow after TOF repair. Methods The study population consisted of 31 children with repaired TOF (mean age ± SD, 12.3 ± 4.1 years who underwent postoperative cardiovascular magnetic resonance (CMR. The CMR protocol included simultaneous phase-contrast velocity mapping of the atrioventricular valves, which enabled direct comparison of the timing and patterns of tricuspid (TV and mitral (MV valve flow. The TV flow was defined to have delayed onset when its onset was > 20 ms later than the onset of the MV flow. The TV and MV flow from 14 normal children was used for comparison. The CMR results were correlated with the findings on echocardiography and electrocardiography. Result Delayed onset of the TV flow was observed in 16/31 patients and in none of the controls. The mean delay time was 64.81 ± 27.07 ms (8.7 ± 3.2% of R-R interval. The delay time correlated with the differences in duration of the TV and MV flow (55.94 ± 32.88 ms (r = 0.90, p Conclusions Early diastolic dysfunction with delayed onset of TV flow is common after TOF repair, and is associated with reduced RV ejection fraction. It is a further manifestation of interventricular dyssynchrony and represent an additional mechanism of ventricular diastolic dysfunction.

  17. The role of blood lactate clearance as a predictor of mortality in children undergoing surgery for tetralogy of Fallot

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    Suruchi Ladha

    2016-01-01

    Full Text Available Background: The identification of biomarkers for predicting morbidity and mortality, particularly in pediatric population undergoing cardiac surgery will contribute toward improving the patient outcome. There is an increasing body of literature establishing the clinical utility of hyperlactatemia and lactate clearance as prognostic indicator in adult cardiac surgical patients. However, the relationship between lactate clearance and mortality risk in the pediatric population remains to be established. Objective: To assess the role of lactate clearance in determining the outcome in children undergoing corrective surgery for tetralogy of Fallot (TOF. Methods and Study Design: A prospective, observational study. Setting: A tertiary care center. Study Population: Two hundred children undergoing elective surgery for TOF. Study Method: Blood lactate levels were obtained as baseline before operation (T0, postoperatively at admission to the cardiac intensive care unit after surgery (T1, and then at every 6 h for the first 24 h of Intensive Care Unit (ICU stay (T6, T12, T18, and T24, respectively. The lactate clearance in the study is defined by the equation ([lactate initial - lactate delayed]/lactate initial ×100%. Lactate clearance was determined at T1-T6, T1-T12, T1-T18, and T1-T24 time interval, respectively. The primary outcome measured was mortality. Secondary outcomes measured were the duration of mechanical ventilation, duration of inotropic requirement, and duration of ICU stay. Results: Eleven out of the two hundred patients enrolled in the study died. Nonsurvivors had higher postoperative lactate concentration (P 10%, relative to patients with a lactate clearance 10% after 6 h have improved outcome compared with those with lower lactate clearance.

  18. Evaluation of postoperative pulmonary regurgitation after surgical repair of tetralogy of Fallot: comparison between Doppler echocardiography and MR velocity mapping

    Energy Technology Data Exchange (ETDEWEB)

    Grothoff, Matthias; Spors, Birgit; Gutberlet, Matthias [Charite Campus Virchow Klinikum, Department of Radiology and Nuclear Medicine, Berlin (Germany); Abdul-Khaliq, Hasim [Deutsches Herzzentrum, Department of Congenital Heart Disease/Pediatric Cardiology, Berlin (Germany)

    2008-02-15

    Pulmonary regurgitation is a common finding in patients after correction of tetralogy of Fallot (TOF). Right ventricular impairment and even ventricular arrhythmia have been ascribed to pulmonary valve insufficiency (PI), which is therefore an important issue in follow-up examinations. To compare PI measured by echocardiography (ECHO) with data provided by cardiac MRI (CMR). We studied 54 selected patients (18 female; median age 14.0 years, range 3.8-53.4 years) after surgical correction of TOF. To quantify pulmonary regurgitant fraction (PRF) by CMR, flow velocity mapping was performed. On Doppler ECHO, length, width and localization of regurgitant flow was measured. The severity of PI was categorized as mild, moderate or severe and compared to the data obtained by CMR. On CMR the mean PRF was 29.2 {+-} 13.4%. Patients with a transannular patch had a significantly higher PRF (39.9 {+-} 11.6%) than patients with an intact annular ring (23.6 {+-} 11.4%). Differentiation by Doppler ECHO between the categories mild, moderate and severe PI was confirmed by significant differences in PRF measured by CMR (mild vs. moderate P < 0.04; moderate vs. severe P < 0.014; mild vs. severe P < 0.001). Furthermore, PRF correlated with right ventricular end diastolic volume index (r = 0.45, P < 0.01) and right ventricular end systolic volume index (r = 0.39, P < 0.01). Doppler ECHO can estimate the severity of PI after repair of TOF with acceptable results compared to CMR flow measurement. In univariate analysis there is only a weak influence of PRF on right ventricular volume. (orig.)

  19. Evaluation of postoperative pulmonary regurgitation after surgical repair of tetralogy of Fallot: comparison between Doppler echocardiography and MR velocity mapping

    International Nuclear Information System (INIS)

    Grothoff, Matthias; Spors, Birgit; Gutberlet, Matthias; Abdul-Khaliq, Hasim

    2008-01-01

    Pulmonary regurgitation is a common finding in patients after correction of tetralogy of Fallot (TOF). Right ventricular impairment and even ventricular arrhythmia have been ascribed to pulmonary valve insufficiency (PI), which is therefore an important issue in follow-up examinations. To compare PI measured by echocardiography (ECHO) with data provided by cardiac MRI (CMR). We studied 54 selected patients (18 female; median age 14.0 years, range 3.8-53.4 years) after surgical correction of TOF. To quantify pulmonary regurgitant fraction (PRF) by CMR, flow velocity mapping was performed. On Doppler ECHO, length, width and localization of regurgitant flow was measured. The severity of PI was categorized as mild, moderate or severe and compared to the data obtained by CMR. On CMR the mean PRF was 29.2 ± 13.4%. Patients with a transannular patch had a significantly higher PRF (39.9 ± 11.6%) than patients with an intact annular ring (23.6 ± 11.4%). Differentiation by Doppler ECHO between the categories mild, moderate and severe PI was confirmed by significant differences in PRF measured by CMR (mild vs. moderate P < 0.04; moderate vs. severe P < 0.014; mild vs. severe P < 0.001). Furthermore, PRF correlated with right ventricular end diastolic volume index (r = 0.45, P < 0.01) and right ventricular end systolic volume index (r = 0.39, P < 0.01). Doppler ECHO can estimate the severity of PI after repair of TOF with acceptable results compared to CMR flow measurement. In univariate analysis there is only a weak influence of PRF on right ventricular volume. (orig.)

  20. Lung perfusion scintigraphy in the diagnosis of peripheral pulmonary stenosis in patients after repair of Fallot tetralogy

    International Nuclear Information System (INIS)

    Sabiniewicz, R.; Chojnicki, M.; Alszewicz-Baranowska, J.; Erecinski, J.; Romanowicz, G.; Lass, P.; Bandurski, T.

    2002-01-01

    The frequency of peripheral pulmonary artery stenosis in patients after surgical repair of tetralogy of Fallot (TOF) ranges from 20 to 40%. This can be either primary or secondary to the surgical intervention. The influence of resulting lung perfusion alterations on the life quality of patients is difficult to predict. The aim of this study was to compare the utility of the diagnostic procedures in this group of patients, with particular focus on lung perfusion scintigraphy. This study comprised 104 patients who underwent repair of TOF at ages from 5 months to 25 years. The patients have been followed up for from 4.2 to 25 years. On the basis of chest X-ray peripheral pulmonary artery stenosis was suspected in 11 patients, in 12 on the basis of echocardiography examination. Lung perfusion scintigraphy has been performed on 87 patients. The disturbances in lung perfusion (mostly in the left lung) were show by means of lung perfusion scintigraphy in 43 (49%) of patients. In 27 of them heart catheterisation has been performed. Angiography revealed stenosis of the lung artery branch in 15/43 (34.9%) patients with abnormal perfusion lung scan and in 4/44 (9%) in patients with normal perfusion lung scan. Intervention procedures were carried out on 10 patients. Lung perfusion scintigraphy may prove a valuable, non-invasive screening tool in the assessment of patients after TOF repair, although both false-negative and false positive results may happen. Therefore, it should play an auxiliary role together with other diagnostic modalities. (author)

  1. Ultra high-resolution gene centric genomic structural analysis of a non-syndromic congenital heart defect, Tetralogy of Fallot.

    Directory of Open Access Journals (Sweden)

    Douglas C Bittel

    Full Text Available Tetralogy of Fallot (TOF is one of the most common severe congenital heart malformations. Great progress has been made in identifying key genes that regulate heart development, yet approximately 70% of TOF cases are sporadic and nonsyndromic with no known genetic cause. We created an ultra high-resolution gene centric comparative genomic hybridization (gcCGH microarray based on 591 genes with a validated association with cardiovascular development or function. We used our gcCGH array to analyze the genomic structure of 34 infants with sporadic TOF without a deletion on chromosome 22q11.2 (n male = 20; n female = 14; age range of 2 to 10 months. Using our custom-made gcCGH microarray platform, we identified a total of 613 copy number variations (CNVs ranging in size from 78 base pairs to 19.5 Mb. We identified 16 subjects with 33 CNVs that contained 13 different genes which are known to be directly associated with heart development. Additionally, there were 79 genes from the broader list of genes that were partially or completely contained in a CNV. All 34 individuals examined had at least one CNV involving these 79 genes. Furthermore, we had available whole genome exon arrays from right ventricular tissue in 13 of our subjects. We analyzed these for correlations between copy number and gene expression level. Surprisingly, we could detect only one clear association between CNVs and expression (GSTT1 for any of the 591 focal genes on the gcCGH array. The expression levels of GSTT1 were correlated with copy number in all cases examined (r = 0.95, p = 0.001. We identified a large number of small CNVs in genes with varying associations with heart development. Our results illustrate the complexity of human genome structural variation and underscore the need for multifactorial assessment of potential genetic/genomic factors that contribute to congenital heart defects.

  2. Ventricular emptying performance in patients with tetralogy of Fallot; Assessment with Fourier analysis of gated blood-pool data

    Energy Technology Data Exchange (ETDEWEB)

    Takeda, Kan; Maeda, Hisato; Nakagawa, Tsuyoshi; Ito, Tsunao; Yamaguchi, Nobuo; Matsuda, Akira (Mie Univ., Tsu (Japan). School of Medicine)

    1989-12-01

    Comparison of emptying patterns between left and right ventricles (LV, RV) was performed with Fourier analysis of gated blood-pool data in patients with tetralogy of Fallot (TF). Using global time-activity curves, the phase and amplitude at the first-harmonic component of Fourier series were calculated and emptying patterns of both ventricles were evaluated by phase difference {l brace}D(phase)=RV phase minus LV phase{r brace} and RV/LV amplitude ratio {l brace}R(amp){r brace}. In 20 patients with normal cardiac function, D(phase) was minimal (mean 2.0{plus minus}6.6 degrees) and R(amp) was less than 1.0 (mean 0.60{plus minus}0.19). In 11 patients with TF, D(phase) was significantly larger than normal, with a mean value of 24.3{plus minus}10.0 degrees (p<0.01) and became greater in a reversed proportion to the ratio of the pulmonary-to-systemic blood flow (p<0.01). In all but one cases with TF, R(amp) was greater than 1.0 with a mean value of 1.4{plus minus}0.4, significantly larger than normal (p<0.001). Furthermore, using time-activity curves approximated by terms up to the 3rd-harmonic component, the temporal difference in emptying patterns between both ventricles was investigated. In TF cases, the time from end-diastole to minimum count (T2) was significantly larger in RV than in LV (p<0.001). The elongated T2 interval of RV seemed to play an important role in producing RV phase lag. Thus, this non-invasive method is valuable for pathophysiologic investigation of patients with TF and can be of help in estimating the severity of their disease. (author).

  3. The Effects of Hemodynamic Alterations on Lung Volumes in Fetuses with Tetralogy of Fallot: An MRI Study.

    Science.gov (United States)

    Berger-Kulemann, Vanessa; Berger, Rudolf; Mlczoch, Elisabeth; Sternal, Daniel; Mailath-Pokorny, Mariella; Hachemian, Nilouparak; Prayer, Daniela; Weber, Michael; Salzer-Muhar, Ulrike

    2015-08-01

    This study assessed whether the presence of tetralogy of Fallot (TOF) affects fetal lung development and whether these fetuses are at risk of pulmonary hypoplasia (PH). Furthermore, we investigated whether the degree of the concomitant pulmonary valve (PV) stenosis or a stenosis in the branch pulmonary arteries correlates with the fetal lung volume. Lung volumetry was performed in 16 fetuses with TOF who underwent MRI between gestational weeks 21 and 35 and in 22 controls. Fetal biometric data and the diameters of the PVs were evaluated by ultrasound. PV and branch pulmonary artery diameters were standardized (z-scores), and fetal lung volume/fetal body weight (FLV/FBW) ratios (ml/g) were calculated. The mean FLV/FBW ratio (0.031 ± 0.009 ml/g) in the TOF group was statistically significantly lower than in the control group (0.041 ± 0.009 ml/g; P = 0.003). None of the fetuses with TOF met the criterion for PH. FLV did not correlate with the degree of PV stenosis, but rather with the presence of a significant stenosis in at least one branch pulmonary artery. The presence of TOF moderately affects fetal lung growth, which is apparently not dependent on the degree of the PV stenosis. However, only an additional stenosis in at least one branch pulmonary artery was associated with a small FLV, but not with PH. Thus, reduced pulmonary blood flow may be offset by additional factors, such as the ability to establish a sufficient collateral system and to alter structural vascular size and, thus, pulmonary vascular resistance.

  4. Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI.

    Science.gov (United States)

    Beurskens, Niek E G; Gorter, Thomas M; Pieper, Petronella G; Hoendermis, Elke S; Bartelds, Beatrijs; Ebels, Tjark; Berger, Rolf M F; Willems, Tineke P; van Melle, Joost P

    2017-11-01

    Quantification of pulmonary regurgitation (PR) is essential in the management of patients with repaired tetralogy of Fallot (TOF). We sought to evaluate the accuracy of first-line Doppler echocardiography in comparison with cardiac magnetic resonance imaging (MRI) to identify hemodynamic significant PR. Paired cardiac MRI and echocardiographic studies (n = 97) in patients with repaired TOF were retrospectively analyzed. Pressure half time (PHT) and pulmonary regurgitation index (PRi) were measured using continuous wave Doppler. The ratio of the color flow Doppler regurgitation jet width to pulmonary valve (PV) annulus (jet/annulus ratio) and diastolic to systolic time velocity integral (DSTVI; pulsed wave Doppler) were assessed. Accuracy of echocardiographic measurements was tested to identify significant PR as determined by phase-contrast MRI (PR fraction [PRF] ≥ 20%). Mean PRF was 29.4 ± 15.7%. PHT < 100 ms had a sensitivity of 93%, specificity 75%, positive predictive value (PPV) 92% and negative predictive value (NPV) 78% for identifying significant PR (C-statistic 0.82). PRi < 0.77 had sensitivity and specificity of 66% and 54%, respectively (C-statistic 0.63). Jet/annulus ratio ≥1/3 had sensitivity 96%, specificity 75%, PPV 92% and NPV 82% (C-statistic 0.87). DSTVI had sensitivity 84%, specificity 33%, PPV 84% and NPV 40%, (C-statistic 0.56). Combined jet/annulus ratio ≥1/3 and PHT < 100 ms was highly accurate in identifying PRF ≥ 20%, with sensitivity 97% and specificity 100%. PHT and jet/annulus ratio on Doppler echocardiography, especially when combined, are highly accurate in identifying significant PR and therefore seem useful in the follow-up of patients with repaired TOF.

  5. Tetralogy of Fallot associated with deletion in the DiGeorge region of chromosome 22 (22q11)

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    D`Angelo, J.A.; Pillers, D.M.; Jett, P.L. [Oregon Health Sciences Univ. Portland, OR (United States)] [and others

    1994-09-01

    Cardiac conotruncal defects, such as Tetralogy of Fallot (TOF), are associated with DiGeorge syndrome which has been mapped to the q11 region of chromosome 22 and includes abnormalities of neural crest and branchial arch development. Patients with conotruncal defects and velo-cardio-facial syndrome may have defects in the 22q11 region but not show the complete DiGeorge phenotype consisting of cardiac, thymus, and parathyroid abnormalities. We report two neonates with TOF and small deletions in the DiGeorge region of chromosome 22 (46,XX,del(22)(q11.21q11.23) and 46,XY,del(22)(q11.2q11.2)) using both high-resolution cytogenetics and fluorescence in situ hybridization (FISH). The first patient is a female with TOF and a family history of congenital heart disease. The mother has pulmonic stenosis and a right-sided aortic arch, one brother has TOF, and a second brother has a large VSD. The patient had intrauterine growth retardation and had thrombocytopenia due to maternal IgG platelet-directed autoantibody. Lymphocyte populations, both T and B cells, were reduced in number but responded normally to stimulation. The findings were not attributed to a DiGeorge phenotype. Although she had transient neonatal hypocalcemia, her parathyroid hormone level was normal. The patient was not dysmorphic in the newborn period but her mother had features consistent with velo-cardio-facial syndrome. The second patient was a male with TOF who was not dysmorphic and had no other significant clinical findings and no family history of heart disease. Lymphocyte testing did not reveal a specific immunodeficiency. No significant postnatal hypocalcemia was noted. These cases illustrate that there is a wide spectrum of clinical features associated with defects of the 22q11 region. We recommend karyotype analysis, including FISH probes specific to the DiGeorge region, in any patient with conotruncal cardiac defects.

  6. Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

    Science.gov (United States)

    Mori, Yoshiki; Murakami, Tomotaka; Inoue, Nao; Kaneko, Sachie; Nakashima, Yasumi; Koide, Masaaki

    2017-08-01

    End-diastolic forward flow (EDFF) is recognized as restrictive right ventricular physiology (r-RVP), but conflicting results have been reported about effects on the clinical outcome in repaired tetralogy of Fallot (r-TOF). We hypothesized that the EDFF by Doppler was not specific for diagnosing r-RVP. Sixty-two consecutive patients aged 15.7±11.6years who underwent cardiac catheterization were studied. Patients were divided according to the presence of EDFF (group 1: EDFF+, group 2: EDFF-) and RV size (group A: small RV, group B: large RV [>150ml/m 2 ]). Group 1 (n=23) had higher a right atrial pressure (RAP), pressure gradient between the RAP and pulmonary diastolic pressure (PDP), and atrial natriuretic peptide (ANP) levels than group 2. Four patients (17.4%) in group 1 and 89.7% of patients in group 2 had a normal RAP range (a wave<10mmHg). There were no differences in the RV volume, ejection fraction (EF), B-type natriuretic peptide levels, and severity of pulmonary regurgitation (PR) between groups 1 and 2. Group A had better RV and LVEF than group B, as well as a smaller LV size. The RAP in subgroup 1A was higher than that of the other 3 subgroups. Subgroup 1B had a similar RAP to group 2, and a lower PDP and a more severe PR than subgroup 1A. Patients with EDFF are associated with increased ANP levels. The presence of EDFF may not be specific for r-RVP, since it is observed in some TOF patients with low PDP (severe PR) and normal RAP. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Computer-based assessment of right ventricular regional ejection fraction in patients with repaired Tetralogy of Fallot

    Science.gov (United States)

    Teo, S.-K.; Wong, S. T.; Tan, M. L.; Su, Y.; Zhong, L.; Tan, Ru-San

    2015-03-01

    After surgical repair for Tetralogy of Fallot (TOF), most patients experience long-term complications as the right ventricle (RV) undergoes progressive remodeling that eventually affect heart functions. Thus, post-repair surgery is required to prevent further deterioration of RV functions that may result in malignant ventricular arrhythmias and mortality. The timing of such post-repair surgery therefore depends crucially on the quantitative assessment of the RV functions. Current clinical indices for such functional assessment measure global properties such as RV volumes and ejection fraction. However, these indices are less than ideal as regional variations and anomalies are obscured. Therefore, we sought to (i) develop a quantitative method to assess RV regional function using regional ejection fraction (REF) based on a 13-segment model, and (ii) evaluate the effectiveness of REF in discriminating 6 repaired TOF patients and 6 normal control based on cardiac magnetic resonance (CMR) imaging. We observed that the REF for the individual segments in the patient group is significantly lower compared to the control group (P < 0.05 using a 2-tail student t-test). In addition, we also observed that the aggregated REF at the basal, mid-cavity and apical regions for the patient group is significantly lower compared to the control group (P < 0.001 using a 2-tail student t-test). The results suggest that REF could potentially be used as a quantitative index for assessing RV regional functions. The computational time per data set is approximately 60 seconds, which demonstrates our method's clinical potential as a real-time cardiac assessment tool.

  8. Rehabilitation of a Female Patient after Corrective Surgery for Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Cvetković Jovana

    2015-12-01

    Full Text Available Prikazana je bolesnica stara 25 godina sa tetralogijom Fallot. Ova urođena srčana mana nije tako česta, ali je najčešća među manama sa cijanozom. Lečenje bolesnica sa tetralogijom Fallot je hirurško i može biti palijativno i korektivno. Bolesnica koju smo prikazali imala je palijativnu operaciju, definitivnu korekciju i ponovnu operaciju zbog insuficijencije pulmonalne valvule. Tokom kardiovaskularne rehabilitacije bolesnica je bila ritmički stabilna uz odličnu toleranciju fizičkog napora. Imajući u vidu da se radi o mlađoj ženskoj osobi koja je tokom proteklih godina imala dva spontana abortusa, trudnoća nije apsolutno kontraindikovana, mada su spontani abortusi i komplikacije trudnoće češći kod ovih bolesnica.

  9. [Infections as a preoperative problem in patients with tetralogy of Fallot (TOF) associated with complete atrioventricular canal septal defect (AVC)].

    Science.gov (United States)

    Szydłowski, Lesław; Marek-Szydłowska, Teresa; Rudziński, Andrzej; Pajak, Jacek; Morka, Jacek; Stołtny, Ludwik

    2004-01-01

    Tetralogy of Fallot (TOF) coexisting with atrioventricular canal septal defect (AVC) is a rare combination of anomalies. Additionally, in cases with concomitant Down syndrome, recurrent infections can be a serious problem in patients (pts.) waiting for cardiosurgery treatment. The purpose of the study was an analysis of types of infections and other factors complicating the preoperative period in those patients. The study group consisted of 17 pts. with TOF and AVC aged from 1 day to 9 years (mean 9.4 month). In this group there were 11 pts. with Down syndrome. All of them were subjected to physical examinations, blood analysis, ECG, chest X-ray and echocardiographic study. Additionally, in 8 pts. we performed catheterization. The signs of different types of infection were analyzed and results were compared in two groups: with and without Down syndrome. The differences were observed in the frequency of recurrent or chronic infections (21 v/s 4), time of hospitalization before surgery (17 v/s 9 days), necessity (11 v/s 3) and duration of antibiotic therapy (19 v/s 7 days) in the two studied groups. Elevated body temperature of unknown etiology was noted in 8 cases with Down syndrome, compared to 1 patient without trisomy 21. Also, the children with Down syndrome had to wait 11 days longer (19 v/s 8) for discharge after operation. Infections in children with TOF, AVC and Down syndrome significantly complicate the natural course of this anomaly. Prolonged preoperation time is characteristic of Down syndrome pts. compared to patients without chromosomal abnormalities.

  10. Altered Aortic Upper Wall TDI Velocity Is Inversely Related with Left Ventricular Diastolic Function in Operated Tetralogy of Fallot.

    Science.gov (United States)

    Bassareo, Pier Paolo; Saba, Luca; Marras, Andrea R; Mercuro, Giuseppe

    2016-12-01

    Postoperative tetralogy of Fallot (TOF) patients often develop progressive aortic root dilatation due to an impairment in aortic elastic properties. (1) to assess aortic elasticity at the level of the aortic upper wall by tissue Doppler imaging (TDI); (2) to evaluate the influence of aortic elasticity on left ventricular (LV) diastolic function in TOF patients. Twenty-eight postoperative TOF patients (14 males, 14 females. Mean age: 25.7 ± 1.6 years) and 28 age- and sex-matched normal subjects were examined. Aortic distensibility and stiffness index were calculated. Aortic wall systolic and diastolic velocities, LV systolic and diastolic parameters were assessed by TDI. Aortic distensibility was significantly lower (P = .024), and aortic stiffness index significantly higher (P = .036) in TOF patients compared to controls. E/E' was significantly higher in TOF than in control group (P < .001). Aortic upper wall early diastolic velocity (AWEDV) was significantly correlated with aortic stiffness index (r: -0.42; P < .03), aortic distensibility (r = 0.54; P < .004), left atrial volume (r = -0.62; P = .0004), and E/E' ratio (r = -0.87; P < .0001). The latter relationship remained significant even when excluding the influence of age at surgery (r = -0.60; P < .0007) and of previous palliative surgery (r = -0.53; P < .02). Aortic elastic properties can be directly assessed using TDI to measure AWEDV. Aortic elasticity is significantly lower in postoperative TOF patients, exerting a negative effect also on LV diastolic function, with a potential long-term influence on clinical status. © 2016 Wiley Periodicals, Inc.

  11. Assessment of intracardiac flow and vorticity in the right heart of patients after repair of tetralogy of Fallot by flow-sensitive 4D MRI.

    Science.gov (United States)

    Hirtler, Daniel; Garcia, Julio; Barker, Alex J; Geiger, Julia

    2016-10-01

    To comprehensively and quantitatively analyse flow and vorticity in the right heart of patients after repair of tetralogy of Fallot (rTOF) compared with healthy volunteers. Time-resolved flow-sensitive 4D MRI was acquired in 24 rTOF patients and 12 volunteers. Qualitative flow evaluation was based on consensus reading of two observers. Quantitative analysis included segmentation of the right atrium (RA) and ventricle (RV) in a four-chamber view to extract volumes and regional haemodynamic information for computation of regional mean and peak vorticity. Right heart intra-atrial, intraventricular and outflow tract flow patterns differed considerably between rTOF patients and volunteers. Peak RA and mean RV vorticity was significantly higher in patients (p = 0.02/0.05). Significant negative correlations were found between patients' maximum and mean RV and RA vorticity and ventricular volumes (p tetralogy of Fallot. • Regurgitant flow in the main pulmonary artery is associated with higher right heart vorticity.

  12. Application of Real-Time Three-Dimensional Echocardiography to Evaluate the Pre- and Postoperative Right Ventricular Systolic Function of Patients with Tetralogy of Fallot

    Science.gov (United States)

    Cui, Cunying; Liu, Lin; Fan, Taibing; Peng, Bangtian; Cheng, Zhaoyun; Ge, Zhenwei; Li, Yanan; Liu, Yuanyuan; Zhang, Yanwei; Ai, Feng; Zhang, Lianzhong

    2015-01-01

    Tetralogy of Fallot (ToF) can be challenging for clinicians to both diagnose and treat, given the multiple heart defects that are by definition associated with the illness. This study investigates the value of real-time three- dimensional echocardiography (RT-3DE) in evaluating the pre-and postoperative right ventricular systolic function of patients with tetralogy of Fallot. A total of 41 ToF patients were divided into two groups: the child group (CG) and the adult group (AG) according to age. The right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV), and the right ventricular ejection fraction (RVEF) of ToF patients were measured before surgery, 7 days, and 3 months after the surgery. The correlation between the preoperative Nakata index and RVEF was then analyzed. Compared with the RVEDV and RVESV prior to surgery, those of the postoperative 7-day and 3-month were not statistically significant (p > 0.05). However, RVEF decreased, and the difference was statistically significant (p 0.05). Compared with the pre-and postoperative RVEDV and RVESV of CG, those of AG increased. However, RVEF decreased, and the differences were statistically significant (p < 0.05). Our study indicated that the correlation between preoperative Nakata index and RVEF was good. Ultimately, we did confirm that RT-3DE can quantitatively evaluate the right ventricular volume and systolic function of ToF patients, thereby providing clinical significance in determining postoperative efficacy and prognosis evaluation. PMID:27122891

  13. [Right branch pulmonary artery stenosis with supravalvar aortic stenosis as a complication of Lecompte maneuver for tetralogy of Fallot associated with absent pulmonary valve].

    Science.gov (United States)

    Honda, Yoshihiro; Suzuki, Shoji; Kaga, Shigeaki; Yoshida, Yukiyo; Kimura, Mitsuhiro; Kamiya, Kentaro; Sakakibara, Kenji; Katsu, Masatake

    2015-05-01

    The patient was diagnosed with tetralogy of Fallot associated with absent pulmonary valve syndrome and a low birth weight of 1,912 g. He suffered from respiratory distress on day 14 and received non-invasive positive pressure ventilation. At 5 months of age and 4.1 kg, he underwent intracardiac repair including right ventricular outflow repair with a monocusp patch, patch closure of the ventricular septum defect and right pulmonary transposition to the anterior of the ascending aorta following the Lecompte maneuver for airway decompression. He was subsequently discharged to home and exhibited an uneventful clinical course with non-invasive positive pressure ventilation for 5 months postoperatively. However, right pulmonary artery and supra-aortic stenosis was noted 2 years after the operation. Computed tomography (CT) and angiography showed ascending aorta strangulation by the right pulmonary artery with right ventricular outflow regurgitation. Right pulmonary artery reconstruction using polytetrafluoroethylene graft interposition and repeat right ventricular outflow repair with bicuspid hand-sewn valves was therefore performed;the postoperative course was uneventful. Pre- and post-operative management using non-invasive positive pressure ventilation and airway decompression with pulmonary artery translocation is a useful strategy in patients exhibiting symptomatic tetralogy of Fallot associated with absent pulmonary valve syndrome in the neonatal period.

  14. Evaluation of right atrial dysfunction in patients with corrected tetralogy of Fallot using 3D speckle-tracking echocardiography. Insights from the CSONGRAD Registry and MAGYAR-Path Study.

    Science.gov (United States)

    Nemes, Attila; Havasi, Kálmán; Domsik, Péter; Kalapos, Anita; Forster, Tamás

    2015-11-01

    In recent studies, alterations in ventricular deformations were demonstrated in adult patients with corrected tetralogy of Fallot by three-dimensional speckle-tracking echocardiography. The present study was designed to assess three-dimensional speckle-tracking echocardiography-derived right atrial volumetric and strain parameters in corrected tetralogy of Fallot. A total of 17 patients with corrected tetralogy of Fallot were included in the study. Their results were compared with 18 age- and gender-matched healthy controls. Complete two-dimensional echocardiography and three-dimensional speckle-tracking echocardiography were performed in all cases. Significantly increased right atrial volumes respecting heart cycle were detected in patients with corrected tetralogy of Fallot. Total and passive atrial emptying fractions proved to be significantly decreased in patients with corrected tetralogy of Fallot (26.4 ± 12.4 % vs. 39.1 ± 8.8 %, p = 0.001 and 11.2 ± 6.8 % vs. 19.8 ± 9.0 %, p = 0.003, respectively). Global and mean segmental peak longitudinal (17.3 ± 9.2 % vs. 30.8 ± 11.2 %, p = 0.0007 and 20.6 ± 10.7 % vs. 34.4 ± 10.5 %, p = 0.0005) and area strains (20.1 ± 17.6 % vs. 41.0 ± 19.8 %, p = 0.004 and 28.1 ± 19.8 % vs. 49.1 ± 19.7 %, p = 0.004) as well as global radial peak strain (-9.1 ± 5.1 % vs. -15.0 ± 10.0 %, p = 0.05) were reduced in patients with corrected tetralogy of Fallot compared with controls. The complexity of right atrial dysfunction can be demonstrated by three-dimensional speckle-tracking echocardiography in patients with corrected tetralogy of Fallot.

  15. Genetic variations of VEGF gene were associated with tetralogy of fallot risk in a Chinese Han population.

    Science.gov (United States)

    Yan, Liru; Ge, Quanzhi; Xi, Chunyan; Zhang, Xuna; Guo, Yujie

    2015-05-01

    Tetralogy of Fallot (TOF) is one of the most common forms of congenital heart disease. In this study, we aimed at investigating the associations between genetic variations of vascular endothelial growth factor (VEGF) gene and the risk of TOF in a Chinese Han population. Our findings may contribute to a deeper understanding of TOF pathogenesis and better diagnostic and therapeutic suggestions. A total of 165 TOF patients and 240 controls from a Chinese Han population in Shenyang and Harbin were recruited in the current study. Nine single-nucleotide polymorphisms (SNPs) (-2578C/A, -460T/C, -1154G/A, -634G/C, 534C/T, +398G/A, +963C/T, 752C/T, 913G/A) were genotyped by the MALDI-TOF MassARRAY system. Individual SNPs as well as their haplotypes were analyzed for their associations with TOF risk, using odds ratios and the 95% confidence interval under codominant and dominant models. In the single SNP analyses, the mutant homozygous genotypes of -2578C/A (rs699947) and +963C/T (rs3025039) were related with an increased risk of TOF. In addition, carriers with the mutant A allele of -1154G/A (rs1570360) were supposed to have a significantly elevated TOF risk. Similarly, compared with the wild homozygote GG carriers, the GC carrier of -634G/C (rs2010963) revealed a significant relationship with susceptibility of TOF, but not for the mutant homozygote CC carriers. However, no significant association was found for the other five SNPs. Meanwhile, haplotype analysis revealed that CCA and ATA in block 1 (-2578C/A, -460T/C, and -1154G/A) and TTG and TCA in block 3 (+963C/T, 752C/T, and 913G/A) were significantly related with an increased TOF risk compared with the most common haplotypes. In summary, our results suggested that VEGF variants (-2578C/A, -1154G/A, -634G/C, +963G/A) were involved in the susceptibility of TOF. However, validation of our study needs further study in various ethnics to reveal the functional relationship between VEGF polymorphisms and TOF risk, which may

  16. Echocardiographic indexes help predict the need for nuclear magnetic resonance evaluation of patients with repaired Tetralogy of Fallot

    International Nuclear Information System (INIS)

    Ferreiro, Myriam; Toro, Lida; Andia, Marcelo; Zelada, Pamela; Alcantara, Alex; Castillo, Maria Elisa; Lagos, Rosa; Bareno, Sandra; Uribe, Sergio

    2013-01-01

    Background: Pulmonary insufficiency (PI) frequently appears long-term after repair of Tetralogy of Fallot (TOFr). Cardiac magnetic resonance (CMR) plays a fundamental role in the indication of pulmonary valve replacement, in order to avoid complications of PI. However, CMR is a scarce and expensive resource in our reality, which is why its indication must be optimized. Aim: The objective of this work is to find echocardiographic indices to identify patients with TOFr with dilated RV and reduced ejection fraction (EF) Method: Images from echocardiograms (ECHO) and CMR in 20 patients (9 women, 8-25 years of age, average 15,8 years old) with TOFr were retrospectively reviewed. From ECHO images we obtained measurements for tricuspid annular plane systolic excursion (TAPSE), tissular s wave, isovolumetric acceleration (IVA), and severity of PI (mild, moderate, or severe). From CMR images, we measured EF, end diastolic volume (EDV) of the RV, and regurgitant fraction (RF) of the pulmonary artery. We performed a multivariate statistical analysis to explore the relation between ECHO parameters and CMR findings Results: No correlation was found between individual ECHO parameters and EDV or RV EF. However, we did find a model based on the TAPSE and PI that was able to predict the EDV of the RV with an r2 = 0.6. (FDV-RV = 8.60*TAPSE + 36.19*PI - 77.213). After comparing the PI with RF, we found that that echocardiography could correctly distinguish cases with mild PI from those with severe PI (p<0.001), or moderate from severe (p=0.004), but not mild from moderate PI Conclusion: In this preliminary study, we found a statistical model based on the TAPSE and PI which could be useful in the selection of patients with TOFr that are referred to CMR. While this study needs to be validated on a greater number of patients, it indicates the relevance of some echocardiographic parameters, which should always be included in the evaluation of patients with TOFr

  17. Analysis of circulating microRNAs in patients with repaired Tetralogy of Fallot with and without heart failure.

    Science.gov (United States)

    Abu-Halima, Masood; Meese, Eckart; Keller, Andreas; Abdul-Khaliq, Hashim; Rädle-Hurst, Tanja

    2017-07-10

    MicroRNAs (miRNAs) are a class of regulatory RNAs that regulate gene expression post-transcriptionally. Little, however, is known on the expression profile of circulating miRNAs in Tetralogy of Fallot (TOF) patients late after surgical repair. In this study, we aimed to identify the specific patterns of circulating miRNAs in blood of patients with repaired, non-syndromic TOF and to assess whether these specific miRNAs may be useful to differentiate patients with and without heart failure. SurePrint™ 8 × 60 K Human v16 miRNA arrays were used to determine miRNA expression profiles in 15 healthy controls and 37 patients after TOF repair of whom 3 had symptomatic right heart failure. The expression levels of selected miRNAs have been validated by quantitative reverse transcription polymerase chain reaction (RT-qPCR). Enrichment analyses of altered miRNA expression were predicted using bioinformatic tools. Compared with healthy controls, a total of 49, 58 and 77 miRNAs were found to be significantly altered in TOF patients (TOF-all), TOF patients with (TOF-HF) and without symptomatic right heart failure (TOF-noHF) (>2.0-fold change, adjusted P < 0.05), respectively. Three miRNAs namely miR-181d-5p, miR-206 and miR-625-5p were validated by RT-qPCR in all TOF groups. The area under the receiver operating characteristic curve (AUC) for miR-181d-5p, miR-206 and miR-625-5p were 0.987, 0.993 and 0.769 in TOF-all and 0.990, 0.994 and 0.749 in TOF-noHF, respectively. Moreover, expression levels of miR-625-5p, miR-1233-3p and miR-421 were lower in TOF-HF compared to TOF-noHF (P = 0.012). Altered expression levels of circulating miRNAs were found in TOF patients late after surgical repair and are different to those seen in the right ventricular myocardium of infants with TOF. Expression levels of miR-421, miR-1233-3p and miR-625-5p are lower in TOF patients with symptomatic right heart failure and thus may indicate disease progression in these patients.

  18. Dimensions of the ascending aorta in children and adolescents with repaired Tetralogy of Fallot obtained by cardiac magnetic resonance angiography.

    Science.gov (United States)

    Grothoff, Matthias; Mende, Meinhard; Graefe, Daniel; Daehnert, Ingo; Kostelka, Martin; Hoffmann, Janine; Freyhardt, Patrick; Lehmkuhl, Lukas; Gutberlet, Matthias; Mahler, Anne

    2016-03-01

    Dilatation of the ascending aorta is a common finding in Tetralogy of fallot (TOF). We sought to provide aortic dimensions in children and adolescents after corrected TOF obtained by contrast-enhanced cardiac-magnetic-resonance angiography (CE-CMRA) that could serve as reference values. We enrolled 101 children and adolescents (56 male) with a median age of 10.9 years. All patients underwent CE-CMRA imaging using a 3-dimensional spoiled gradient-echo-sequence. Aortic diameters were measured at the level of the aortic valve (AV), aortic sinus (AS), sino-tubular junction (STJ) and the ascending aorta (AA) and compared with normal values obtained from literature. Sex-specific aortic dimensions are given as percentile curves as well as z scores. Furthermore CMR volumetric and functional parameters as well as clinical and anamnestic data were analyzed to identify parameters that are associated with aortic dilatation. Diameters for aortic size for males were 3.6 + 16.6*BSA(0.5) at the AV level, 7.0 + 19.5*BSA(0.5) at the AS level, 7.0 + 14.4*BSA(0.5) at the STJ level and 7.3 + 15.5*BSA(0.5) at the AA level. Diameters for females were 5.8 + 14.1*BSA(0.5) at the AV level, 7.2 + 17.6*BSA(0.5) at the AS level, 5.2 + 15.4*BSA(0.5) at the STJ level and 2.0 + 17.8*BSA(0.5) at the AA level. All diameters in TOF patients were larger compared with normal values. The postoperative interval and age at examination were the only parameters associated with aortic size at all measured levels. We provide CE-CMRA data of aortic dimensions in children and adolescents after correction of TOF. Our data might be useful for an estimation of the "normal" aortic size in this patient cohort and can serve as a basis for future longitudinal studies adding prognostic data.

  19. Assessment of early diastolic intraventricular pressure gradient in the left ventricle among patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Kobayashi, Maki; Takahashi, Ken; Yamada, Mariko; Yazaki, Kana; Matsui, Kotoko; Tanaka, Noboru; Shigemitsu, Sachie; Akimoto, Katsumi; Kishiro, Masahiko; Nakanishi, Keisuke; Kawasaki, Shiori; Nii, Masaki; Itatani, Keiichi; Shimizu, Toshiaki

    2017-11-01

    Assessment of left ventricular (LV) dysfunction is vital in patients with repaired tetralogy of Fallot (rTOF). The early diastolic intraventricular pressure gradient (IVPG) in the LV plays an important role in diastolic function. IVPG is calculated as the intraventricular pressure difference divided by the LV length, which allows to account for differences in LV size and therefore calculate IVPG in children. We aimed to investigate the mechanisms of LV diastolic dysfunction by measuring mid-to-apical IVPG as an indicator of the active suction force sucking blood from the left atrium into the LV. We included 38 rTOF patients and 101 healthy controls. The study population was stratified based on age group into children (4-9 years), adolescents (10-15 years), and adults (16-40 years). IVPGs were calculated based on mitral inflow measurements obtained using color M-mode Doppler echocardiography. Although total IVPGs did not differ between rTOF patients and controls, mid-to-apical IVPGs in adolescents and adults were smaller among rTOF patients than among controls (0.15 ± 0.05 vs. 0.21 ± 0.06 mmHg/cm, p < 0.05; 0.09 ± 0.07 vs. 0.17 ± 0.05 mmHg/cm, p < 0.001; respectively). Additionally, only mid-to-apical IVPG correlated linearly with peak circumferential strain (ρ = 0.217, p = 0.011), longitudinal strain (ρ = -0.231, p = 0.006), torsion (ρ = -0.200, p = 0.018), and untwisting rate in early diastole (ρ = -0.233, p = 0.006). In rTOF, the mechanisms underlying diastolic dysfunction involve reduced active suction force, which correlates with reduced LV deformation in all directions.

  20. Assessment of Diffuse Ventricular Myocardial Fibrosis Using Native T1 in Children With Repaired Tetralogy of Fallot.

    Science.gov (United States)

    Yim, Deane; Riesenkampff, Eugenie; Caro-Dominguez, Pablo; Yoo, Shi-Joon; Seed, Mike; Grosse-Wortmann, Lars

    2017-03-01

    Myocardial fibrosis is linked with adverse clinical outcomes in adults after tetralogy of Fallot repair (rTOF). Native T1 times (T1) by cardiac magnetic resonance have been shown to be a surrogate marker of diffuse myocardial fibrosis. The objective was to quantify native T1 in children post-rTOF and to evaluate their relationship with surgical, imaging, and clinical factors. A retrospective cross-sectional study was performed. Midventricular native T1 were obtained in 100 children post-rTOF using a modified look-locker inversion recovery cardiac magnetic resonance sequence and compared with 35 pediatric controls. rTOF patients, aged 13.0±2.9 years, had higher indexed right ventricular (RV) end-diastolic (range 85-326 mL/m 2 , mean 148 mL/m 2 ) volumes, and lower RV and left ventricular (LV) ejection fractions compared with controls. RV, but not LV, T1 were higher in patients than in controls (1031±74 versus 954±32 ms, P <0.001) and female patients had higher RV T1 compared with males (1051±79 versus 1017±68 ms, P =0.02). LV T1 correlated with RV T1 ( r =0.45, P <0.001), cardiopulmonary bypass ( r =0.30, P =0.007), and aortic cross-clamp times ( r =0.32, P =0.004). RV T1 correlated inversely with RV outflow tract gradient ( r =-0.28, P =0.02). Longer aortic cross-clamp times were independently associated with LV and RV T1 on multivariable analysis. There was no association between exercise intolerance, arrhythmia, and native T1 or LV extracellular volume. Children after rTOF do not have elevated LV native T1 or LV extracellular volume, but show evidence of increased RV native T1 suggestive of diffuse RV fibrosis, for which volume loading seems to be a risk factor. Surgical bypass and cross-clamp times are associated with fibrotic remodeling over a decade later. © 2017 American Heart Association, Inc.

  1. Pulmonary valve insufficiency in the postoperative out of tetralogy of Fallot: contribution of conventional transthoracic echocardiography for decision making

    International Nuclear Information System (INIS)

    Guzzo de Leon, D.

    2013-01-01

    Pulmonary valve regurgitation is one of the main problems in the late post-op of Tetralogy of F allot, having a great incidence and depending on many aspects . In these paper a review is done considering the usefulness of conventional transtoracic eco cardiography as a tool to evaluate patients with pulmonary regurgitation in the late post-op of Tetralogy of F allot. Main pathophysiologic concepts about these entity are presented, and as a function of that, the most important eco cardiographic parameters to be considered evaluating these patients are also described. Finally guidelines on the opportunity for pulmonary valve replacement are considered

  2. Pulmonary valve replacement after operative repair of tetralogy of Fallot: meta-analysis and meta-regression of 3,118 patients from 48 studies.

    Science.gov (United States)

    Ferraz Cavalcanti, Paulo Ernando; Sá, Michel Pompeu Barros Oliveira; Santos, Cecília Andrade; Esmeraldo, Isaac Melo; de Escobar, Rodrigo Renda; de Menezes, Alexandre Motta; de Azevedo, Orlando Morais; de Vasconcelos Silva, Frederico Pires; Lins, Ricardo Felipe de Albuquerque; Lima, Ricardo de Carvalho

    2013-12-10

    Because the real benefit of pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot who develop pulmonary insufficiency remains unclear, it is necessary to analyze the evidence published around the world. We performed a systematic review of studies that reported data about the effect of PVR in patients with repaired tetralogy of Fallot that developed pulmonary insufficiency, until December 2012. The variables chosen to represent the benefit were both right ventricular (RV) and left ventricular measures, QRS duration, and functional class. The principal summary measures were difference in means with 95% confidence interval and p values (considered statistically significant when p regression were completed with the software Comprehensive Meta-Analysis (version 2, Biostat, Inc., Englewood, New Jersey). Forty-eight studies involving 3,118 patients met the eligibility criteria. The pooled 30-day mortality was 0.87% (47 studies; 27 of 3,100 patients); the pooled 5-year mortality was 2.2% (24 studies; 49 of 2,231 patients); the pooled 5-year re-PVR was 4.9% (15 studies; 88 of 1,798 patients). The results of this meta-analysis demonstrate that after PVR: 1) the RV experiences improvement of its volumes and function; 2) the left ventricle experiences improvement of its function; 3) QRS duration decreases; 4) symptoms improve; 5) pre-operative RV geometry modulates the effect of PVR; and 6) there is important heterogeneity of the effects among the studies, and few publication biases. In conclusion, PVR seems to be a positive approach in the analyzed scenario. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  3. QRS Complex Enlargement as a Predictor of Ventricular Arrhythmias in Patients Affected by Surgically Treated Tetralogy of Fallot: A Comprehensive Literature Review and Historical Overview

    Science.gov (United States)

    Bassareo, Pier Paolo; Mercuro, Giuseppe

    2013-01-01

    Tetralogy of Fallot (TOF) is a congenital heart disease frequently treated by surgical repair to relieve symptoms and improve survival. However, despite the performing of an optimal surgical repair, TOF patients are at times characterized by a poor long-term survival rate, likely due to cardiac causes such as ventricular arrhythmias, with subsequent sudden death. In the 80s it was irrefutably demonstrated that QRS prolongation ≥180 msec at basal electrocardiogram is a strong predictor for refining risk stratification for ventricular tachycardia in these patients. The aim of this research was to undertake a review of all studies conducted to assess the impact of QRS duration on the development of life-threatening ventricular arrhythmias in repaired TOF subjects. PMID:23509638

  4. 3D Printing Provides a Precise Approach in the Treatment of Tetralogy of Fallot, Pulmonary Atresia with Major Aortopulmonary Collateral Arteries.

    Science.gov (United States)

    Anwar, Shafkat; Rockefeller, Toby; Raptis, Demetrios A; Woodard, Pamela K; Eghtesady, Pirooz

    2018-02-03

    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. Three-dimensional (3D) printing is an emerging medical technology that provides added benefits in the management of patients with Tet PA MAPCAs. When used in combination with current diagnostic modalities and procedures, 3D printing provides a precise approach to the management of these challenging, high-risk patients. Specifically, 3D printing enables detailed surgical and interventional planning prior to the procedure, which may improve procedural outcomes, decrease complications, and reduce procedure-related radiation dose and contrast load.

  5. Correlation of Fragmented QRS with Right Ventricular Indexes and Fibrosis in Patients with Repaired Tetralogy of Fallot, by Cardiac Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Zahra Alizadeh Sani

    2017-09-01

    Full Text Available Background: Repair of tetralogy of fallot (TOF is associated with diffuse myocardial fibrosis. Cardiac magnetic resonance imaging (CMR can visualize the areas with myocardial fibrosis. Presence of fragmented QRS (fQRS implies the presence of the underlying myocardial scar. Despite the strong association between fQRS and myocardial pathologies, the impact of fQRS with myocardial fibrosis in post-TOF correction is unknown. Objectives: Here, we evaluated the possible predictive role of fQRS in repaired TOF cases and its relationship with cardiac function. Patients and Methods: Thirty two patients with previous history of repaired TOF were enrolled. The extent of fQRS was evaluated according to the number of leads with fQRS. After electrocardiographic evaluation, the participants underwent CMR. Results: Results showed a significant relationship between the right ventricular (RV systolic diameter and fQRS (P = 0.014. Also, an inverse linear relationship was found between the number of fQRS edges and RVEF (r = 0.77, P = 0.0001. The mean QRS duration in those with positive and negative fQRS was 132 mm and 115.8 mm (P = 0.0001. Furthermore, a linear correlation was observed between the number of edges and the percentage of scar tissue (r = 0.88, P = 0.001. However, no relevance between gender and fQRS was detected (P = 0.26, and the relationship between RV diastolic diameter and fQRS was not significant (P = 0.1. Thus, fQRS could be used as a marker of RV systolic dysfunction in patients with tetralogy of fallot. Conclusions: We suggested the fQRS as a surrogate indicator of RV dysfunction in repaired TOF patients and showed that diagnostic and prognostic information of the patients were available by fQRS.

  6. Influence of contact force on voltage mapping: A combined magnetic resonance imaging and electroanatomic mapping study in patients with tetralogy of Fallot.

    Science.gov (United States)

    Teijeira-Fernandez, Elvis; Cochet, Hubert; Bourier, Felix; Takigawa, Masateru; Cheniti, Ghassen; Thompson, Nathaniel; Frontera, Antonio; Camaioni, Claudia; Massouille, Gregoire; Jalal, Zakaria; Derval, Nicolas; Iriart, Xavier; Denis, Arnaud; Hocini, Meleze; Haissaguerre, Michel; Jais, Pierre; Thambo, Jean-Benoit; Sacher, Frederic

    2018-03-20

    Voltage criteria for ventricular mapping have been obtained from small series of patients and prioritizing high specificity. The purpose of this study was to analyse the potential influence of contact force (CF) on voltage mapping and to define voltage cutoff values for right ventricular (RV) scar using the tetralogy of Fallot as a model of transmural RV scar and magnetic resonance imaging (MRI) as reference. Fourteen patients (age 32.6 ± 14.3 years; 5 female) with repaired tetralogy of Fallot underwent high-resolution cardiac MRI (1.25 × 1.25 × 2.5 mm). Scar, defined as pixels with intensity >50% maximum, was mapped over the RV geometry and merged within the CARTO system to RV endocardial voltage maps acquired using a 3.5-mm ablation catheter with CF technology (SmartTouch, Biosense Webster). In total, 2446 points were analyzed, 915 within scars and 1531 in healthy tissue according to MRI. CF correlated to unipolar (ρ = 0.186; P voltage in healthy tissue (ρ = 0.245; P voltage cutoffs of 5.19 mV for unipolar voltage and 1.76 mV for bipolar voltage, yielding sensitivity/specificity of 0.89/0.85 and 0.9/0.9, respectively. CF is an important factor to be taken into account for voltage mapping. If good CF is applied, unipolar and bipolar voltage cutoffs of 5.19 mV and 1.76 mV are optimal for identifying RV scar on endocardial mapping with the SmartTouch catheter. Data on the diagnostic accuracy of different voltage cutoff values are provided. Copyright © 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  7. Left and right ventricular dyssynchrony and strains from cardiovascular magnetic resonance feature tracking do not predict deterioration of ventricular function in patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Jing, Linyuan; Wehner, Gregory J; Suever, Jonathan D; Charnigo, Richard J; Alhadad, Sudad; Stearns, Evan; Mojsejenko, Dimitri; Haggerty, Christopher M; Hickey, Kelsey; Valente, Anne Marie; Geva, Tal; Powell, Andrew J; Fornwalt, Brandon K

    2016-08-22

    Patients with repaired tetralogy of Fallot (rTOF) suffer from progressive ventricular dysfunction decades after their surgical repair. We hypothesized that measures of ventricular strain and dyssynchrony would predict deterioration of ventricular function in patients with rTOF. A database search identified all patients at a single institution with rTOF who underwent cardiovascular magnetic resonance (CMR) at least twice, >6 months apart, without intervening surgical or catheter procedures. Seven primary predictors were derived from the first CMR using a custom feature tracking algorithm: left (LV), right (RV) and inter-ventricular dyssynchrony, LV and RV peak global circumferential strains, and LV and RV peak global longitudinal strains. Three outcomes were defined, whose changes were assessed over time: RV end-diastolic volume, and RV and LV ejection fraction. Multivariate linear mixed models were fit to investigate relationships of outcomes to predictors and ten potential baseline confounders. One hundred fifty-three patients with rTOF (23 ± 14 years, 50 % male) were included. The mean follow-up duration between the first and last CMR was 2.9 ± 1.3 years. After adjustment for confounders, none of the 7 primary predictors were significantly associated with change over time in the 3 outcome variables. Only 1-17 % of the variability in the change over time in the outcome variables was explained by the baseline predictors and potential confounders. In patients with repaired tetralogy of Fallot, ventricular dyssynchrony and global strain derived from cine CMR were not significantly related to changes in ventricular size and function over time. The ability to predict deterioration in ventricular function in patients with rTOF using current methods is limited.

  8. Perioperative echocardiography-derived right ventricle function parameters and early outcomes after tetralogy of Fallot repair in mid-childhood: a single-center, prospective observational study.

    Science.gov (United States)

    Raj, Ravi; Puri, Goverdhan Dutt; Jayant, Aveek; Thingnam, Shyam Kumar Singh; Singh, Rana Sandip; Rohit, Manoj Kumar

    2016-11-01

    Right ventricular (RV) function alterations are invariably present in all patients after tetralogy of Fallot (TOF) repair. Unlike the developed world where most of the patients with TOF are corrected in infancy, average age of presentation and thus surgery for these patients in the developing world may be higher. We aimed to study the correlation between RV function parameters such as tricuspid annular peak systolic excursion (TAPSE), fractional area change (FAC), and tricuspid annular peak systolic velocity (S') with early outcome variables after intracardiac repair for TOF. Fifty patients with a preoperative diagnosis of tetralogy of Fallot scheduled for corrective surgery were included in this single-center, prospective observational study. A preoperative transthoracic echocardiogram was performed to measure RV function parameters (FAC0, TAPSE0, S'0). Transthoracic echocardiography was repeated postoperatively to measure FAC1, TAPSE1, S'1 (day 1) and FAC2, TAPSE2, and S'2 (day 3). The relationship between preoperative and postoperative RV function parameters with in-hospital mortality, duration of mechanical ventilation, and intensive care unit stay was studied. The median age of patients was 6 years (range 1-14 years). Multiple stepwise logistic regression analysis showed RV FAC as best predictor of clinical outcome. Area under the receiver operating characteristic curve for postoperative RV function parameters, that is, FAC, TAPSE, and S' to predict early or delayed recovery was 0.944, 0.875, and 0.655, respectively. Among the RV function parameters studied, RV FAC best predicted the early outcome variables after TOF repair, followed by TAPSE while lateral tricuspid annular velocity S' being the least predictive. © 2016, Wiley Periodicals, Inc.

  9. The value of right ventricular longitudinal strain in the evaluation of adult patients with repaired tetralogy of Fallot: a new tool for a contemporary challenge.

    Science.gov (United States)

    Almeida-Morais, Luís; Pereira-da-Silva, Tiago; Branco, Luísa; Timóteo, Ana T; Agapito, Ana; de Sousa, Lídia; Oliveira, José A; Thomas, Boban; Jalles-Tavares, Nuno; Soares, Rui; Galrinho, Ana; Cruz-Ferreira, Rui

    2017-04-01

    The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients. Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method. In total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was -16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=-0.40) and right ventricular ejection fraction (r=-0.45) (all ptetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.

  10. Mid-term results of correction of Tetralogy of Fallot with absent pulmonary valve

    Directory of Open Access Journals (Sweden)

    Sachin Talwar

    2017-11-01

    Conclusions: In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood.

  11. Poorer right ventricular systolic function and exercise capacity in women after repair of tetralogy of fallot: a sex comparison of standard deviation scores based on sex-specific reference values in healthy control subjects.

    Science.gov (United States)

    Sarikouch, Samir; Boethig, Dietmar; Peters, Brigitte; Kropf, Siegfried; Dubowy, Karl-Otto; Lange, Peter; Kuehne, Titus; Haverich, Axel; Beerbaum, Philipp

    2013-11-01

    In repaired congenital heart disease, there is increasing evidence of sex differences in cardiac remodeling, but there is a lack of comparable data for specific congenital heart defects such as in repaired tetralogy of Fallot. In a prospective multicenter study, a cohort of 272 contemporary patients (158 men; mean age, 14.3±3.3 years [range, 8-20 years]) with repaired tetralogy of Fallot underwent cardiac magnetic resonance for ventricular function and metabolic exercise testing. All data were transformed to standard deviation scores according to the Lambda-Mu-Sigma method by relating individual values to their respective 50th percentile (standard deviation score, 0) in sex-specific healthy control subjects. No sex differences were observed in age at repair, type of repair conducted, or overall hemodynamic results. Relative to sex-specific controls, repaired tetralogy of Fallot in women had larger right ventricular end-systolic volumes (standard deviation scores: women, 4.35; men, 3.25; P=0.001), lower right ventricular ejection fraction (women, -2.83; men, -2.12; P=0.011), lower right ventricular muscle mass (women, 1.58; men 2.45; P=0.001), poorer peak oxygen uptake (women, -1.65; men, -1.14; Pstandard deviation scores in repaired tetralogy of Fallot suggest that women perform poorer than men in terms of right ventricular systolic function as tested by cardiac magnetic resonance and exercise capacity. This effect cannot be explained by selection bias. Further outcome data are required from longitudinal cohort studies.

  12. Right ventricular function late after total repair of tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Straten, Alexander van; Roos, Albert de [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands); Vliegen, Hubert W. [Leiden University Medical Center, Department of Cardiology, Leiden (Netherlands); Hazekamp, Mark G. [Leiden University Medical Center, Department of Cardiothoracic Surgery, Leiden (Netherlands)

    2005-04-01

    Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. (orig.)

  13. Right ventricular function late after total repair of tetralogy of Fallot

    International Nuclear Information System (INIS)

    Straten, Alexander van; Roos, Albert de; Vliegen, Hubert W.; Hazekamp, Mark G.

    2005-01-01

    Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. (orig.)

  14. Impact of perioperative transesophageal echocardiography on tetralogy of fallot total repair

    Directory of Open Access Journals (Sweden)

    Yasmin Abdel Razek

    2014-03-01

    Conclusion: TEE imaging is a safe, feasible, and accurate tool for anatomical, hemodynamic, and functional assessment in patients with TOF during surgical repair. TEE offers the advantage of permitting visualization of the operative procedure in real time and provides guidance for the surgeon in making decisions inside the operating room.

  15. Exclusion of 22q11 deletion in Noonan syndrome with Tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Digilio, M.C.; Marino, B.; Giannotti, A. [Bambino Gesu Hospital, Rome (Italy); Dallapiccola, B. [Univ. of Tor Vergata, Rome (Italy)]|[Casa Sollievo Sofferenza Hospital, San Giovanni Rotondo (Italy)

    1996-04-24

    We read with interest the report of Robin et al. [1995] published in recent issue of the Journal. The authors described 6 patients with Noonan syndrome (NS) who underwent molecular evaluation for submicroscopic deletion of chromosome band 22q11. None of those patients presented with conotruncal heart defects. Evidence for 22q11 hemizygosity was demonstrated in only one patient. This patient had NS-like manifestations without clinical manifestations of DiGeorge (DG) or velo-cardio-facial (VCF) syndromes. The molecular results obtained in the other 5 patients led the authors to conclude that classical NS is not due to del(22)(q11), even if some patients with del(22)(q11) may present NS-like manifestations. 12 refs., 1 tab.

  16. Management of a patient with dengue virus infection and tetralogy of Fallot

    Science.gov (United States)

    Arfijanto, M. V.; Oktiawan, E.; Purwati; Hadi, U.

    2018-03-01

    TOF belongs to cyanotic heart disease group, which has polycythemia and a high baseline hematocrit. A heart defect that features four problems:a hole between the lower chambers of the heart, an obstruction from the heart to the lungs, the aorta lies over the hole in the lower chambers, the muscle surrounding the lower right chamber becomes overly thickened. Dengue viral infection in patients with TOF would have a greater potential for the occurrence of heart failure because the baseline of hematocrit is already higher compare with non-congenital heart disease. A 22-year-old man complains offever and nausea since three days before hospitalized. The principal treatment is enough rest and rehydration to achieve normal range of hematocrit (around 65%) and to avoid drugs that may precipitate bleeding (like salicylic acid).In the management of this patient, we should be more careful because there wascomorbid with TOF. The focus of the management is to prevent dehydration during the critical phase and aware of overhydration in the recovery phase.

  17. Uncorrected tetralogy of fallot in a 47 year old Nigerian man: a case ...

    African Journals Online (AJOL)

    This has been attributed to relatively small degrees of left ventricular outflow tract (LVOT) obstruction and/or presence of congenital aorto-pulmonary shunts. Very few cases of uncorrected TOF in adults have been reported in Africa. We report this case of uncorrected TOF in a 47 year old Nigerian man to highlight this rare ...

  18. Pulmonary function testing in infants with tetralogy of Fallot and absent pulmonary valve syndrome

    Directory of Open Access Journals (Sweden)

    Adler M Salazar

    2015-01-01

    Conclusion: For our patients with TOF-APVS, airway resistance was elevated. Flow limitation was seen in the medium to small airways with a mild reduction of FVC. PFTs may help guide management of mechanical ventilation for TOF-APVS patients.

  19. Tetralogy of Fallot

    Science.gov (United States)

    ... that increase the risk for this condition during pregnancy include: Alcoholism in the mother Diabetes Mother who is over 40 years old Poor nutrition during pregnancy Rubella or other viral illnesses during pregnancy Children ...

  20. O miocárdio na tetralogia de Fallot: estudo histológico e morfométrico El miocardio en la tetralogía de Fallot: estudio histológico y morfométrico The myocardium in tetralogy of Fallot: a histological and morphometric study

    Directory of Open Access Journals (Sweden)

    Maria Cecília Knoll Farah

    2009-03-01

    ventriculares para estudios morfométricos.BACKGROUND: Patients with Tetralogy of Fallot frequently develop ventricular dysfunction in the postoperative period. The histological basis of this functional alteration has been scarcely studied. OBJECTIVE: To evaluate myocardial remodeling in anatomical specimens, comparing the subepicardial and subendocardial regions, especially because the subendocardial region is easily approached by means of endomyocardial biopsy. METHODS: Transmural sections of myocardium from the right ventricular (RV inflow tract, anterior wall and infundibulum, and from the left ventricular (LV free wall were evaluated regarding the degree of cardiomyocyte hypertrophy, vascularization and interstitial fibrosis were analyzed. RESULTS: The mean diameter of subendocardial cardiomyocytes is similar to that of subepicardial cardiomyocytes in all regions, except for the RV infundibulum, in which subendocardial cardiomyocytes are significantly larger in relation to those of the subepicardium (p=0.007. The amount of interstitial collagen is in the upper limits of normal and was similar in the subendocardial layers in comparison with the subpericardial layer of each region; however, it was greater in the inflow tract and RV anterior wall than in the LV lateral wall. The numerical density of subendocardial capillaries was similar to that of the subepicardium and was lower than the mean minus two standard deviations of normal in all regions and layers, except for the infundibulum, in which the subepicardium showed normal values and the subendocardium showed values lower than the mean minus two standard deviations. CONCLUSION: The postnatal myocardial changes in Tetralogy of Fallot are homogeneously distributed in the subepicardial and subendocardial halves of the ventricular walls, except for the infundibulum, which has peculiar remodeling characteristics and, therefore, is not representative of the other ventricular regions and layers for morphometric studies.

  1. Immediate and Midterm Cardiac Remodeling After Surgical Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study.

    Science.gov (United States)

    Heng, Ee Ling; Gatzoulis, Michael A; Uebing, Anselm; Sethia, Babulal; Uemura, Hideki; Smith, Gillian C; Diller, Gerhard-Paul; McCarthy, Karen P; Ho, Siew Yen; Li, Wei; Wright, Piers; Spadotto, Veronica; Kilner, Philip J; Oldershaw, Paul; Pennell, Dudley J; Shore, Darryl F; Babu-Narayan, Sonya V

    2017-10-31

    Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling. Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y). There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m 2 ; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m 2 ; P <0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m 2 and RVESVi ≤82 mL/m 2 . RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P <0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm 2 /m 2 ; P <0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality ( P =0.03) over a median of 9.5 years of follow-up. Significant right heart structural reverse remodeling takes place immediately after PVR, followed by a continuing process of further biological remodeling

  2. Assessment of intracardiac flow and vorticity in the right heart of patients after repair of tetralogy of Fallot by flow-sensitive 4D MRI

    Energy Technology Data Exchange (ETDEWEB)

    Hirtler, Daniel [University Hospital Freiburg, Department of Congenital Heart Defects and Pediatric Cardiology (Heart Center, University of Freiburg), Freiburg (Germany); Garcia, Julio; Barker, Alex J. [Northwestern University Feinberg School of Medicine, Department of Radiology, Chicago, IL (United States); Geiger, Julia [University Childrens' Hospital Zurich, Department of Radiology, Zurich (Switzerland)

    2016-10-15

    To comprehensively and quantitatively analyse flow and vorticity in the right heart of patients after repair of tetralogy of Fallot (rTOF) compared with healthy volunteers. Time-resolved flow-sensitive 4D MRI was acquired in 24 rTOF patients and 12 volunteers. Qualitative flow evaluation was based on consensus reading of two observers. Quantitative analysis included segmentation of the right atrium (RA) and ventricle (RV) in a four-chamber view to extract volumes and regional haemodynamic information for computation of regional mean and peak vorticity. Right heart intra-atrial, intraventricular and outflow tract flow patterns differed considerably between rTOF patients and volunteers. Peak RA and mean RV vorticity was significantly higher in patients (p = 0.02/0.05). Significant negative correlations were found between patients' maximum and mean RV and RA vorticity and ventricular volumes (p < 0.05). The main pulmonary artery (MPA) regurgitant flow was associated with higher RA and RV vorticity, which was significant for RA maximum and RV mean vorticity (p = 0.01/0.03). The calculation of vorticity based on 4D flow data is an alternative approach to assess intracardiac flow changes in rTOF patients compared with qualitative flow visualization. Alterations in intracardiac vorticity could be relevant with regard to the development of RV dilation and impaired function. (orig.)

  3. Assessment of intracardiac flow and vorticity in the right heart of patients after repair of tetralogy of Fallot by flow-sensitive 4D MRI

    International Nuclear Information System (INIS)

    Hirtler, Daniel; Garcia, Julio; Barker, Alex J.; Geiger, Julia

    2016-01-01

    To comprehensively and quantitatively analyse flow and vorticity in the right heart of patients after repair of tetralogy of Fallot (rTOF) compared with healthy volunteers. Time-resolved flow-sensitive 4D MRI was acquired in 24 rTOF patients and 12 volunteers. Qualitative flow evaluation was based on consensus reading of two observers. Quantitative analysis included segmentation of the right atrium (RA) and ventricle (RV) in a four-chamber view to extract volumes and regional haemodynamic information for computation of regional mean and peak vorticity. Right heart intra-atrial, intraventricular and outflow tract flow patterns differed considerably between rTOF patients and volunteers. Peak RA and mean RV vorticity was significantly higher in patients (p = 0.02/0.05). Significant negative correlations were found between patients' maximum and mean RV and RA vorticity and ventricular volumes (p < 0.05). The main pulmonary artery (MPA) regurgitant flow was associated with higher RA and RV vorticity, which was significant for RA maximum and RV mean vorticity (p = 0.01/0.03). The calculation of vorticity based on 4D flow data is an alternative approach to assess intracardiac flow changes in rTOF patients compared with qualitative flow visualization. Alterations in intracardiac vorticity could be relevant with regard to the development of RV dilation and impaired function. (orig.)

  4. The importance of the toxicological analysis in newborns: clearing the case of a tetralogy of Fallot with a paradoxical reaction to 1-hydroxymidazolam

    Directory of Open Access Journals (Sweden)

    Cristiano Ialongo

    2016-01-01

    Full Text Available Short half-life benzodiazepines like midazolam are used as premedication to relieve intra-partum pain, and are known to have no adverse effects to the newborn. However, such short-acting drugs are known to induce paradoxical reactions when their concentration is abnormally elevated respect to the age of the subject. In a 1 day-old newborn, admitted to the Cardiology Unit of the “Bambino Gesù” Children Hospital of Rome due to a prenatal diagnosis of tetralogy of Fallot, a paradoxical reaction with a spontaneous resolution was observed. The analysis of urines, performed by gas chromatography/mass spectrometry after a drug screening, showed the presence of the active metabolite 1-hydroxymidazolam at 9.7 µg/mL with no detectable trace of its precursor midazolam and of the alternative metabolite 4-hydroxymidazolam. On the basis of these evidences, we speculated that 1-hydroxymidazolam, produced by the mother’s liver enzymes, passed to the newborn whose reduced volume of distribution (for the shunted circulation favoured the onset of such a reaction. Hence, the toxicological approach should be carried out on both mother and child wherever the clinical picture may appear unexplainable or does not match with the other findings collected.

  5. Alteration of pulmonary blood flow in tetralogy of Fallot; Pre- and postoperative study with macroaggregates of [sup 99m]Tc-labeled human serum albumin

    Energy Technology Data Exchange (ETDEWEB)

    Hashimoto, Kazuhiro; Matsui, Michihiko; Kurosawa, Hiromi; Arai, Tatsuta (Jikei Univ., Tokyo (Japan). School of Medicine); Nakamura, Yuzuru

    1992-10-01

    The pulmonary blood distribution was examined in 17 patients with tetralogy of Fallot (TOF) pre and postoperatively with macroaggregates of [sup 99m]TC-labeled human serum albumin. Most of the patients with TOF demonstrated an abnormal preoperative distribution pattern. The abnormalities included not only an unbalanced distribution between the right and left lungs but also a maldistribution of peripheral vessels in each lung. The right/left lung counts ratio and pulmonary peripheral index (calculated in order to express the severity of peripheral maldistribution) correlated neither to the diameter nor the cross-sectional area of either right or left pulmonary arteries which were measured angiographically. Postoperatively, the pulmonary blood was shunted toward the developed side of the lung which further contributed to maldistribution of blood flow and unbalanced pulmonary growth. Since the patients with an unbalanced pulmonary blood distribution demonstrated a higher right ventricular pressure one year after the operation, a palliative operation facilitating the growth of the underdeveloped side of the lung might be considered as an effective procedure to precede intracardiac repair. (author).

  6. Catheter Ablation of a Complex Atrial Tachycardia after Surgical Repair of Tetralogy of Fallot Guided by Combined Noncontact and Contact Mapping

    Directory of Open Access Journals (Sweden)

    Eitaro Fujii, MD

    2010-01-01

    Full Text Available A 34-year-old man with a surgically repaired Tetralogy of Fallot complained of palpitation, fatigue, and presyncope. A 12-lead ECG showed atrial tachycardia with a cycle length of 250 ms and a P wave morphology positive in leads II, III and aVF, and negative in lead V1. Although the EnSite system (version 6.OJ made use of noncontact mapping to delineate the counterclockwise reentry around the crista tenninalis, it was difficult to rule out the incisional atrial reentry because the location of the surgical incision was far from the multi-electrode array. Since the bipolar contact mapping of the EnSite system revealed the location of the atriotomy incision, entrainment mapping during the tachycardia demonstrated the critical reentry circuit around the crista terminalis. Radiofrequency ablation targeting the critical isthmus from the lower position of the crista terminalis to the posterior dense scar which was continuous with the inferior vena cava, and to the atriotomy scar, eliminated the tachycardia.

  7. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    Energy Technology Data Exchange (ETDEWEB)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P. [St. Christopher`s Hospital for Children, Front Street at Erie Avenue, Philadelphia, PA 19134 (United States)

    1997-12-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.) With 2 figs., 3 tabs., 11 refs.

  8. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    International Nuclear Information System (INIS)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P.

    1997-01-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.)

  9. Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries.

    Science.gov (United States)

    Murphy, Lexa K; Compas, Bruce E; Reeslund, Kristen L; Gindville, Melissa C; Mah, May Ling; Markham, Larry W; Jordan, Lori C

    2017-01-01

    The objective of this study is to investigate cognitive and attentional function in adolescents and young adults with operated congenital heart disease. Previous research has indicated that children with congenital heart disease have deficits in broad areas of cognitive function. However, less attention has been given to survivors as they grow into adolescence and early adulthood. The participants were 18 non-syndromic adolescents and young adults with tetralogy of Fallot and d-transposition of the great arteries that required cardiac surgery before the age of 5 years, and 18 healthy, unaffected siblings (11-22 years of age for both groups). Cases with congenital heart disease and their siblings were administered Wechsler Intelligence scales and reported attention problems using the Achenbach System of Empirically Based Assessments. Cases were compared to both healthy siblings and established norms. Cases performed significantly lower than siblings on full scale IQ and processing speed, and significantly lower than norms on perceptual reasoning. Cases also reported more attention problems compared to both siblings and norms. Effect sizes varied with medium-to-large effects for processing speed, perceptual reasoning, working memory, and attention problems. Findings suggest that neurocognitive function may continue to be affected for congenital heart disease survivors in adolescence and young adulthood, and that comparisons to established norms may underestimate neurocognitive vulnerabilities.

  10. Pixel-wise derivation of pulmonary regurgitation index could influence clinical decision: A phase-contrast MR imaging study on patients with repaired tetralogy of Fallot.

    Science.gov (United States)

    Wu, Pei-Hsin; Chung, Hsiao-Wen; Wu, Ming-Ting; Ko, Cheng-Wen

    2017-08-01

    Regurgitant fraction (RF) measured from 2D phase-contrast MRI has been used as a standard to quantitate the degree of pulmonary regurgitation and serves as a determinant indicator of prognosis for tetralogy of Fallot after surgical repair. This study demonstrated the potential underestimate of RF using the conventional definition and its impact on clinical decision when backward flow occurred during systolic periods. Quantitative flow parameters, including forward flow volume (FFV), backward flow volume (BFV), and RF were estimated by two approaches: One derived from conventional ROI-averaged curve (bulk quantity) and the other in a pixel-wise manner to spatially reflect inhomogeneous flow profile (pixel-wise quantity). Eccentricity at systolic peak (Ecc sys ) was adopted as an index reflecting spatial flow inhomogeneity. Flow parameters derived from ROI-averaged curves on main pulmonary artery were significantly smaller than that of pixel-wise measurement (P 0.3 appears greater compared to the group with Ecc sys < 0.3. Thirteen out of 68 RF values (19%) were underrated while using bulk analysis. The spatial-related flow parameters showed more consistency with the qualitative flow profile pattern for pulmonary arteries, and could be a decisive complement for diagnostic classification. Copyright © 2017 Elsevier B.V. All rights reserved.

  11. Effect of ventricular size and function on exercise performance and the electrocardiogram in repaired tetralogy of Fallot with pure pulmonary regurgitation

    International Nuclear Information System (INIS)

    Menon, Shaji C; Kaza, Aditya K; Puchalski, Michael D

    2012-01-01

    In repaired tetralogy of Fallot (TOF), exercise test parameters like peak oxygen uptake and ventilatory efficiency predict mortality. Studies have also suggested cardiac magnetic resonance (CMR)-derived right ventricular (RV) size threshold values for pulmonary valve replacement in repaired TOF. However, effects of proposed RV size on exercise capacity and morbidity are not known. The relationship between CMR-derived ventricular size, function, and pulmonary regurgitation (PR) and NYHA class, exercise performance, and electrocardiogram (ECG) was studied in patients of repaired TOF with pure PR in a retrospective review of records. 46 patients (22 females), mean age 14 years (8–30.8), were studied. There was no relationship between CMR-derived ventricular size, function, or PR and exercise test parameters, or NYHA class. RV end systolic and end diastolic volume correlated positively with the degree of PR. QRS duration on ECG correlated positively with RV end-diastolic volume (P < 0.01, r 2 = 0.34) and PR (P < 0.01, r 2 = 0.52). In repaired TOF and pure PR, there is no correlation between ventricular size or function and exercise performance. RV size increases with increasing PR. Timing of pulmonary valve replacement in TOF with pure PR needs further prospective evaluation for its effect on morbidity and mortality

  12. Quantitative assessment of right ventricular function and pulmonary regurgitation in surgically repaired tetralogy of Fallot using 256-slice CT: comparison with 3-Tesla MRI

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    Yamasaki, Yuzo; Yonezawa, Masato; Matsuo, Yoshio; Kamitani, Takeshi; Higuchi, Ko; Honda, Hiroshi [Graduate School of Medical Sciences, Kyushu University, Department of Clinical Radiology, Higashi-ku, Fukuoka (Japan); Nagao, Michinobu; Kawanami, Satoshi [Graduate School of Medical Sciences, Kyushu University, Department of Molecular Imaging and Diagnosis, Higashi-ku, Fukuoka (Japan); Yamamura, Kenichiro [Graduate School of Medical Sciences, Kyushu University, Department of Pediatrics, Higashi-ku, Fukuoka (Japan); Sakamoto, Ichiro [Graduate School of Medical Sciences, Kyushu University, Department of Cardiovascular Medicine, Higashi-ku, Fukuoka (Japan); Shiokawa, Yuichi [Graduate School of Medical Sciences, Kyushu University, Department of Cardiovascular Surgery, Higashi-ku, Fukuoka (Japan); Yabuuchi, Hidetake [Graduate School of Medical Sciences, Kyushu University, Department of Health Sciences, Higashi-ku, Fukuoka (Japan)

    2014-12-15

    To compare 256-slice cardiac computed tomography (CCT) with cardiac magnetic resonance (CMR) imaging to assess right ventricular (RV) function and pulmonary regurgitant fraction (PRF) in patients with repaired tetralogy of Fallot (TOF). Thirty-three consecutive patients with repaired TOF underwent retrospective ECG-gated CCT and 3-Tesla CMR. RV and left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were measured using CCT and CMR. PRF-CCT (%) was defined as (RVSV - LVSV)/RVSV. PRF-CMR (%) was measured by the phase-contrast method. Repeated measurements were performed to determine intra- and interobserver variability. CCT measurements, including PRF, correlated highly with the CMR reference (r = 0.71-0.96). CCT overestimated RVEDV (mean difference, 17.1 ± 2.9 ml), RVESV (12.9 ± 2.1 ml) and RVSV (4.2 ± 2.0 ml), and underestimated RVEF (-2.6 ± 1.0 %) and PRF (-9.1 ± 2.0 %) compared with CMR. The limits of agreement between CCT and CMR were in a good range for all measurements. The variability in CCT measurements was lower than those in CMR. The estimated effective radiation dose was 7.6 ± 2.6 mSv. 256-slice CCT can assess RV function and PRF with relatively low dose radiation exposure in patients with repaired TOF, but overestimates RV volume and underestimates PRF. (orig.)

  13. Quantitative assessment of right ventricular function and pulmonary regurgitation in surgically repaired tetralogy of Fallot using 256-slice CT: comparison with 3-Tesla MRI

    International Nuclear Information System (INIS)

    Yamasaki, Yuzo; Yonezawa, Masato; Matsuo, Yoshio; Kamitani, Takeshi; Higuchi, Ko; Honda, Hiroshi; Nagao, Michinobu; Kawanami, Satoshi; Yamamura, Kenichiro; Sakamoto, Ichiro; Shiokawa, Yuichi; Yabuuchi, Hidetake

    2014-01-01

    To compare 256-slice cardiac computed tomography (CCT) with cardiac magnetic resonance (CMR) imaging to assess right ventricular (RV) function and pulmonary regurgitant fraction (PRF) in patients with repaired tetralogy of Fallot (TOF). Thirty-three consecutive patients with repaired TOF underwent retrospective ECG-gated CCT and 3-Tesla CMR. RV and left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were measured using CCT and CMR. PRF-CCT (%) was defined as (RVSV - LVSV)/RVSV. PRF-CMR (%) was measured by the phase-contrast method. Repeated measurements were performed to determine intra- and interobserver variability. CCT measurements, including PRF, correlated highly with the CMR reference (r = 0.71-0.96). CCT overestimated RVEDV (mean difference, 17.1 ± 2.9 ml), RVESV (12.9 ± 2.1 ml) and RVSV (4.2 ± 2.0 ml), and underestimated RVEF (-2.6 ± 1.0 %) and PRF (-9.1 ± 2.0 %) compared with CMR. The limits of agreement between CCT and CMR were in a good range for all measurements. The variability in CCT measurements was lower than those in CMR. The estimated effective radiation dose was 7.6 ± 2.6 mSv. 256-slice CCT can assess RV function and PRF with relatively low dose radiation exposure in patients with repaired TOF, but overestimates RV volume and underestimates PRF. (orig.)

  14. Cardiac Magnetic Resonance Feature Tracking Biventricular Two-Dimensional and Three-Dimensional Strains to Evaluate Ventricular Function in Children After Repaired Tetralogy of Fallot as Compared with Healthy Children.

    Science.gov (United States)

    Berganza, Fernando M; de Alba, Cesar Gonzalez; Özcelik, Nazire; Adebo, Dilachew

    2017-03-01

    Cardiac magnetic resonance imaging is an important tool to evaluate cardiac anatomy and ventricular size and function after repaired tetralogy of Fallot. Magnetic resonance tissue tagging is the gold standard for evaluation of myocardial strain. However, myocardial tagging strain requires tagged images to be obtained prospectively, during the scan and with limited temporal resolution. Cardiac magnetic resonance feature tracking is a new tool that allows the retrospective analysis of cine images. There is limited experience with cardiac magnetic resonance feature tracking strain analysis in children. The medical records of patients with repaired tetralogy of Fallot that had a cardiac magnetic resonance (CMR) study from December 2013 to June 2015 were reviewed. The control group included patients who underwent a CMR with normal cardiac anatomy and ventricular function. Global longitudinal, circumferential and radial strain parameters (2D and 3D) were obtained by retrospectively contouring cine images from ventricular short axis, two chamber and four chamber views using post-processing software (Circle CVi 42 , Calgary, Canada). The correlation between conventional ventricular function parameters and ventricular strain was performed using Pearson's correlation. The mean age of tetralogy of Fallot and control subjects was 12.4 and 14.1 years, respectively. In patients after repaired tetralogy of Fallot, the mean left ventricular global 2D and 3D circumferential strains were -17.4 ± 2.9 and -10.1 ± 3, respectively. The mean indexed right ventricular end-diastolic volume was 135.4 cc m 2  ± 46 compared to 75.7 cc m 2  ± 17 in control subjects (P = 0.0001, CI 95%). Left ventricular global circumferential 3D strain showed a statistically significant difference in patients after TOF repair compared to normal subjects (-10.1 ± 3 vs. -14.71 ± 1.9, P = 0.00001). A strong correlation between left ventricular global circumferential 3D strain and right

  15. Predictors of ventricular tachyarrhythmia occurring late after intracardiac repair of tetralogy of Fallot: combination of QRS duration change rate and tricuspid regurgitation pressure gradient

    Science.gov (United States)

    Takahashi, Masashi; Sugimoto, Ai; Tsuchida, Masanori

    2017-01-01

    Background To determine potential predictors of ventricular tachyarrhythmia and sudden cardiac death (SCD) occurring late after repair of tetralogy of Fallot (TOF). Methods 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. Results The mean follow-up period was 24.3 years. During the study period, one patient died of cerebral bleeding, and two patients had SCD. The overall survival rates at 20, 30, and 40 years were 100%, 94.6%, and 94.6%, respectively. Eight (9.0%) patients required re-intervention during the late period associated with right ventricular outflow (n=4), tricuspid valve (n=3), aortic valve (n=2), and others (n=2). Ten (11.2%) patients had a history of ventricular tachycardia (VT) or ventricular fibrillation (VF), and six underwent implantation of an implantable cardiac defibrillator. Multivariate analysis selected the change rate of QRS duration [ms/year; odds ratio (OR), 2.44; 95% confidence interval (CI): 1.28–4.65; P=0.007] and the pressure gradient at tricuspid valve regurgitation on echocardiography (OR, 1.12; 95% CI: 1.02–1.22; P=0.017) as risk factors for VT/VF or SCD. Trans-annular patch (TAP) repair was not an independent risk factor for ventricular arrhythmia. Conclusions The combination of rapid change rate of QRS duration and higher-pressure gradient at tricuspid regurgitation were risk factors for ventricular tachyarrhythmia late after TOF repair. Adequate surgical or catheter intervention for pressure and volume load in the right ventricle might decrease the prevalence of VT/VF and SCD. PMID:29312717

  16. Tricuspid annular plane systolic excursion in the assessment of right ventricular function in children and adolescents after repair of tetralogy of Fallot.

    Science.gov (United States)

    Mercer-Rosa, Laura; Parnell, Aimee; Forfia, Paul R; Yang, Wei; Goldmuntz, Elizabeth; Kawut, Steven M

    2013-11-01

    Assessing right ventricular (RV) performance is essential for patients with tetralogy of Fallot (TOF). The aim of this study was to investigate the reliability and validity of tricuspid annular plane systolic excursion (TAPSE) against cardiac magnetic resonance imaging measures and cardiopulmonary exercise testing. A retrospective study was performed in 125 outpatients with repaired TOF with available protocol-driven echocardiography, cardiac magnetic resonance imaging, and exercise stress testing obtained as part of a cross-sectional study. TAPSE was measured on the two-dimensional apical four-chamber view on echocardiography by two readers. Multivariate linear regression was used to examine the association between TAPSE and measures of RV function and exercise capacity. The mean age was 12.6 ± 3.3 years, 41 patients (33%) were female, and 104 (83%) were white. TAPSE averaged 1.6 ± 0.37 cm, with an interreader intraclass correlation coefficient of 0.78 (n = 18). TAPSE was significantly associated with cardiac magnetic resonance-based RV stroke volume after adjustment for gender and body surface area (β = 13.8; 95% confidence interval, 2.25-25.30; P = .02). TAPSE was not associated with cardiac magnetic resonance-based RV ejection fraction (P = .77). On exercise testing, TAPSE was not associated with peak oxygen consumption, percentage of predicted oxygen consumption, oxygen pulse, or the ventilatory equivalent for carbon dioxide in patients with maximal exercise stress testing (n = 73 [58%]). TAPSE is reproducibly measured by echocardiography in patients with TOF. It is not associated with RV ejection fraction or exercise performance, and its association with RV stroke volume may be confounded by body size. On the basis of these results, TAPSE is not representative of global RV performance in patients with TOF. Copyright © 2013 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.

  17. Feasibility and safety of on table extubation after corrective surgical repair of tetralogy of Fallot in a developing country: A case series

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    Mohammad Irfan Akhtar

    2015-01-01

    Full Text Available Fast-track extubation is an established safe practice in pediatric congenital heart disease (CHD surgical patients. On table extubation (OTE in acyanotic CHD surgical patients is well established with validated safety profile. This practice is not yet reported in tetralogy of Fallot (TOF cardiac surgical repair patients in developing countries. Evidence suggests that TOF total correction patients should be extubated early, as positive pressure ventilation has a negative impact on right ventricular function and the overall increase in post-TOF repair complications such as low cardiac output state and arrhythmias. The objective of the case series was to determine the safety and feasibility of OTE in elective TOF total correction cardiac surgical patients with an integrated team approach. To the best of our knowledge, this is the first reported case series. A total of 8 elective male and female TOF patients were included. Standard anesthetic, surgical and perfusion techniques were used in these procedures. All patients were extubated in the operating room safely without any complications with the exception of one patient who continued to bleed for 3 h of postextubation at 2-3 ml/kg/h which was managed with transfusion of fresh frozen plasma at 15 mL/kg, packed red blood cells 10 mL/kg and bolus of transamine at 20 mg/kg. Apart from better surgical and bypass techniques, the most important factor leading to successful OTE was an excellent analgesia. On the basis of the case series, it is suggested to extubate selected TOF cardiac surgery repair patients on table safely with integrated multidisciplinary approach.

  18. Impact of surgical correction of tetralogy of fallot on short-term right and left ventricular function as determined by 2-dimensional speckle tracking echocardiography

    Science.gov (United States)

    Li, Yuman; Wang, Xinfang; Lv, Qing; Wang, Jing; Yang, YaLi; He, Lin; Yuan, Li; Zhang, Li; Xie, Mingxing

    2016-01-01

    Abstract Right ventricular (RV) and left ventricular (LV) dysfunction is an important determinant of poor clinical status in repaired patients with tetralogy of Fallot (TOF). The purpose of our study is to assess the impact of surgical repair on short-term RV and LV function by 2-dimensional speckle tracking echocardiography (STE). Sixty-seven patients (median age 12 months) with TOF before and 6 months after repair and 35 healthy subjects were studied. The patients were divided into the younger (age at surgery ≤12 months) and older (age at surgery >12 months) subgroups. RV and LV global longitudinal systolic strain and strain rate (SR), and LV global circumferential and radial systolic strain and SR were measured by STE. After repair, RV longitudinal strain and SR increased in the younger patients, whereas RV longitudinal SR was decreased in the older patients. LV deformation parameters were unchanged in all patients. In the multivariate analysis, patients with better RV and LV deformation parameters preoperatively were identified to have better RV and LV strain and SR postoperatively (P < 0.05 for all). The surgical approach of the pulmonary valve ring was predictive of RV and LV systolic function postoperatively (P < 0.05 for all). After TOF repair, short-term RV function improvement is identified in the younger but not in the older patients, whereas LV function is unchanged in all patients. The preoperative RV and LV deformational indices are the determinant of postoperative biventricular function improvement. STE appears to be a valuable tool for assessment of biventricular function after congenital heart disease surgery. PMID:27495064

  19. 4D cardiovascular magnetic resonance velocity mapping of alterations of right heart flow patterns and main pulmonary artery hemodynamics in tetralogy of Fallot

    Science.gov (United States)

    2012-01-01

    Background To assess changes in right heart flow and pulmonary artery hemodynamics in patients with repaired Tetralogy of Fallot (rTOF) we used whole heart, four dimensional (4D) velocity mapping (VM) cardiovascular magnetic resonance (CMR). Methods CMR studies were performed in 11 subjects with rTOF (5M/6F; 20.1 ± 12.4 years) and 10 normal volunteers (6M/4F; 34.2 ± 13.4 years) on clinical 1.5T and 3.0T MR scanners. 4D VM-CMR was performed using PC VIPR (Phase Contrast Vastly undersampled Isotropic Projection Reconstruction). Interactive streamline and particle trace visualizations of the superior and inferior vena cava (IVC and SVC, respectively), right atrium (RA), right ventricle (RV), and pulmonary artery (PA) were generated and reviewed by three experienced readers. Main PA net flow, retrograde flow, peak flow, time-to-peak flow, peak acceleration, resistance index and mean wall shear stress were quantified. Differences in flow patterns between the two groups were tested using Fisher's exact test. Differences in quantitative parameters were analyzed with the Kruskal-Wallis rank sum test. Results 4D VM-CMR was successfully performed in all volunteers and subjects with TOF. Right heart flow patterns in rTOF subjects were characterized by (a) greater SVC/IVC flow during diastole than systole, (b) increased vortical flow patterns in the RA and in the RV during diastole, and (c) increased helical or vortical flow features in the PA's. Differences in main PA retrograde flow, resistance index, peak flow, time-to-peak flow, peak acceleration and mean wall shear stress were statistically significant. Conclusions Whole heart 4D VM-CMR with PC VIPR enables detection of both normal and abnormal right heart flow patterns, which may allow for comprehensive studies to evaluate interdependencies of post-surgically altered geometries and hemodynamics. PMID:22313680

  20. Pulmonary uptake of thallium-201 in patients with congenital heart disease; Comparison between total anomalous pulmonary venous connection and tetralogy of Fallot

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    Kohata, Tohru; Ono, Yasuo; Iwatani, Hajime; Fukushima, Hideki; Kamiya, Tetsuro; Yagihara, Toshikatsu; Nishimura, Tsunehiko; Takamiya, Makoto (National Cardiovascular Center, Suita, Osaka (Japan))

    1992-01-01

    To evaluate the pulmonary extravascular space in patients with congenital heart disease, lung uptake of Tl-201 was quantitatively studied. Patients' diseases consisted of total anomalous pulmonary venous connection (TAPVC)--supracardiac (I), paracardiac (II) and infracardiac (III) types--, tetralogy of Fallot (T/F), ventricular septal defect (VSD), and patent ductus arteriosus (PDA). Tl-201 imaging was performed before operation and in the early and late stages after operation. Twenty-five other patients with arrhythemias or a history of Kawasaki disease without perfusion defects served as controls. Lung uptake of Tl-201 was analyzed with a computer using the anterior image of the chest, and the averge count ratio of the right lung (P) to the left ventricular wall (LV) was calculated. P/LV values were compared between the patients before and after operation, and differences in anatomical types in TAPVC were also evaluated. In TAPVC, P/LV values decreased gradually after operation, but were significantly higher than those of controls even in the late stage. In the late stage after operation, type I TAPVC had significantly higher P/LV values than those of type-II. In T/F, the P/LV values were significantly higher after operation, even in the late stage, than before operation. In the VSD or PDA group, the P/LV value returned to normal after operation and was significantly lower than that before operation. In conclusion, TAPVC patients was considered to have a larger pulmonary extravascular space even in the late stage after operation, suggesting a sign of pulmonary congestion due to intrapulmonary vascular damage in utero. In T/F, scanty pulmonary vascular beds before operation were perfused with increased pulmonary blood flow after operation. Therefore, postoperative increases in pulmonary blood flow may be responsible for the increased pulmonary extravascular space. (N.K.).

  1. Cardiothoracic ratio may be misleading in the assessment of right- and left-ventricular size in patients with repaired tetralogy of Fallot

    International Nuclear Information System (INIS)

    Śpiewak, M.; Małek, Ł.A.; Biernacka, E.K.; Kowalski, M.; Michałowska, I.; Hoffman, P.; Miśko, J.; Demkow, M.; Rużyłło, W.; Marczak, M.

    2014-01-01

    Aim: To assess the relationship between cardiothoracic ratio (CTR) and ventricular and atrial volumes in patients with repaired tetralogy of Fallot (TOF). Materials and methods: Patients with repaired TOF undergoing cardiac magnetic resonance (CMR) and chest radiography within 1 day were included (n = 82; median age: 24.7 years, interquartile range: 21.5–35.9). The CTR was obtained from upright posteroanterior chest roentgenograms. Analyses of CMR images and radiographs were performed in a blinded fashion. Results: There were 35.1% (13/37) of patients with normal CTR (<0.5) who had severe right ventricular (RV) dilatation. There were six patients (13.3%, 6/45) with high CTR with both normal RV and left-ventricular (LV) volumes. CTR did not correlate with either RV or LV volumes but showed a weak correlation with right- and left-atrial volumes (r = 0.43, p = 0.0001; r = 0.27, p = 0.01, respectively). CTR ≥0.5 showed poor ability in the identification of severe RV dilatation (sensitivity: 61.8%, specificity: 50%). The combination of CTR and signs of RV enlargement on lateral radiographs did not improve the diagnostic accuracy of any of those parameters alone. Conclusion: CTR in patients with repaired TOF reflected atrial rather than ventricular dilatation. The use of CTR or lateral radiographs in patients with repaired TOF may lead to false conclusions concerning ventricular size. - Highlights: • Cardiothoracic ratio did not correlate with either right- or left-ventricular volumes. • Cardiothoracic ratio reflected atrial rather than ventricular dilatation. • Normal cardiothoracic ratio did not exclude severe right ventricular dilatation. • Neither RVEF nor LVEF showed correlation with cardiothoracic ratio

  2. Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation.

    Science.gov (United States)

    Karnik, Ruchika; Uppu, Santosh C; Tozzi, Meghan; Doucette, John; Lytrivi, Irene D; Geiger, Miwa; Klas, Berthold; Parness, Ira A; Shenoy, Rajesh; Rajagopal, Hari; Srivastava, Shubhika

    2018-04-11

    Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.

  3. Importance of Non-invasive Right and Left Ventricular Variables on Exercise Capacity in Patients with Tetralogy of Fallot Hemodynamics.

    Science.gov (United States)

    Meierhofer, Christian; Tavakkoli, Timon; Kühn, Andreas; Ulm, Kurt; Hager, Alfred; Müller, Jan; Martinoff, Stefan; Ewert, Peter; Stern, Heiko

    2017-12-01

    Good quality of life correlates with a good exercise capacity in daily life in patients with tetralogy of Fallot (ToF). Patients after correction of ToF usually develop residual defects such as pulmonary regurgitation or stenosis of variable severity. However, the importance of different hemodynamic parameters and their impact on exercise capacity is unclear. We investigated several hemodynamic parameters measured by cardiovascular magnetic resonance (CMR) and echocardiography and evaluated which parameter has the most pronounced effect on maximal exercise capacity determined by cardiopulmonary exercise testing (CPET). 132 patients with ToF-like hemodynamics were tested during routine follow-up with CMR, echocardiography and CPET. Right and left ventricular volume data, ventricular ejection fraction and pulmonary regurgitation were evaluated by CMR. Echocardiographic pressure gradients in the right ventricular outflow tract and through the tricuspid valve were measured. All data were classified and correlated with the results of CPET evaluations of these patients. The analysis was performed using the Random Forest model. In this way, we calculated the importance of the different hemodynamic variables related to the maximal oxygen uptake in CPET (VO 2 %predicted). Right ventricular pressure showed the most important influence on maximal oxygen uptake, whereas pulmonary regurgitation and right ventricular enddiastolic volume were not important hemodynamic variables to predict maximal oxygen uptake in CPET. Maximal exercise capacity was only very weakly influenced by right ventricular enddiastolic volume and not at all by pulmonary regurgitation in patients with ToF. The variable with the most pronounced influence was the right ventricular pressure.

  4. "Pulmonary valve replacement diminishes the presence of restrictive physiology and reduces atrial volumes": a prospective study in Tetralogy of Fallot patients.

    Science.gov (United States)

    Pijuan-Domenech, Antonia; Pineda, Victor; Castro, Miguel Angel; Sureda-Barbosa, Carlos; Ribera, Aida; Cruz, Luz M; Ferreira-Gonzalez, Ignacio; Dos-Subirà, Laura; Subirana-Domènech, Teresa; Garcia-Dorado, David; Casaldàliga-Ferrer, Jaume

    2014-11-15

    Pulmonary valve replacement (PVR) reduces right ventricular (RV) volumes in the setting of long-term pulmonary regurgitation after Tetralogy of Fallot (ToF) repair; however, little is known of its effect on RV diastolic function. Right atrial volumes may reflect the burden of RV diastolic dysfunction. The objective of this paper is to evaluate the clinical, echocardiographic, biochemical and cardiac magnetic resonance (CMR) variables, focusing particularly on right atrial response and right ventricular diastolic function prior to and after elective PVR in adult patients with ToF. This prospective study was conducted from January 2009 to April 2013 in consecutive patients > 18 years of age who had undergone ToF repair in childhood and were accepted for elective PVR. Twenty patients (mean age: 35 years; 70% men) agreed to enter the study. PVR was performed with a bioporcine prosthesis. Concomitant RV reduction was performed in all cases when technically possible. Pulmonary end-diastolic forward flow (EDFF) decreased significantly from 5.4 ml/m(2) to 0.3 ml/m(2) (p volumes by 25% (p = 0.0024): mean indexed diastolic/systolic atrial volumes prior to surgery were 43 ml/m(2) (SD+/-4.6)/63 ml/m(2) (SD+/-5.5), and dropped to 33 ml/m(2) (SD+/-3)/46 ml/m(2) (SD+/-2.55) post-surgery. All patients presented right ventricular diastolic and systolic volume reductions, with a mean volume reduction of 35% (p volumes in keeping with the known reduction in RV volumes, after PVR. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  5. Arrhythmias and sudden death among older children and young adults following tetralogy of Fallot repair in the current era: are previously reported risk factors still applicable?

    Science.gov (United States)

    Arya, Swati; Kovach, Julie; Singh, Harinder; Karpawich, Peter P

    2014-01-01

    Young adult patients (pts) with repaired tetralogy of Fallot (TOF) remain at risk for arrhythmias (Ar) and sudden cardiac death (SCD). Based on past studies with earlier pt subsets, Ar/SCD events were associated with right ventricular (RV) systolic pressures >60 mm Hg, outflow tract gradients >20 mm Hg, and QRS duration >180 ms. However, there are limited recent studies to evaluate these risk factors in the current patient generation. Patients with TOF followed over the past 50 years were grouped by presence of any arrhythmias (group 1), absence of arrhythmias (group 2), and presence of SCD or significant ventricular arrhythmias (group 3) and correlated with current pt age, gender, age at repair, repair types, echocardiogram, cardiac magnetic resonance imaging, electrocardiogram/Holter, hemodynamics, and electrophysiology findings. Of 109 pts, 52 were male aged 17-58 years. Of these, 59 (54%) had Ar, two of whom had SCD. These 59 pts were chronologically older at the time of analysis, with repair at an older age and wider QRS duration (78-240, mean 158 ms) when compared with those without Ar. However, there was no correlation with surgical era, surgical repair, gender, RV pressure >60 mm Hg, right ventricular outflow tract gradient >20 mm Hg, or RV end-diastolic volume on CMRI. Ar/SCD risk continues to correlate with repair age and advancing pt age. QRS duration is longer in these patients but at a shorter interval (mean 158 ms) and less RV pressure (mean 43 mm Hg) than previously reported. In the current TOF patient generation, neither surgical era, type of repair, RV outflow gradient nor RV volume correlate with Ar/SCD. Electrophysiologic testing to verify and identify arrhythmias remains clinically effective. © 2013 Wiley Periodicals, Inc.

  6. Stable right ventricular size and function during short-term follow-up in patients with pulmonary regurgitation after tetralogy of Fallot repair

    International Nuclear Information System (INIS)

    Śpiewak, M.; Małek, Ł.A.; Petryka, J.; Biernacka, E.K.; Hoffman, P.; Demkow, M.; Miśko, J.; Rużyłło, W.

    2013-01-01

    Aim: To assess changes in ventricular size and function over time in conservatively treated adult patients with repaired tetralogy of Fallot (TOF) and significant pulmonary regurgitation (PR). Materials and methods: Patients with repaired TOF who had undergone more than one cardiac magnetic resonance study were identified. To confine the cause of ventricular size and function deviation to PR, patients with residual ventricular septal defect, more than mild regurgitation at a valve other than the pulmonary valve, and known coronary artery disease were excluded. Results: The final analysis included 27 adults with PR fraction >20%. During a follow-up of mean 2.1 ± 0.8 years, there was no change in right ventricular (RV) end-diastolic volume (EDV; 162.1 ± 27.6 versus 164 ± 29.6 ml/m 2 , p = 0.5). Left ventricular (LV) EDV showed a small decrease (85.1 ± 16.2 versus 81.5 ± 14.1 ml/m 2 , p = 0.02). The mean PR fraction, PR volume, and peak RV outflow tract gradient did not change. Additionally, both RV ejection fraction (EF) and LVEF remained stable over the follow-up period (48.1 ± 6.5 versus 48.4 ± 6.7%, p = 0.83, and 57.3 ± 5.4 versus 57.2 ± 5.1 %, p = 0.91, respectively). Only two asymptomatic patients (7.4% of the study group) developed symptoms and the remaining did not deteriorate. Conclusion: The RVEDV, RVEF, and LVEF remained stable over a mean follow-up of approximately 2 years in the majority of adult patients after TOF repair with significant PR and a wide range of RVEDV

  7. Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study

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    Kálmán Havasi

    Full Text Available Abstract Background: Three-dimensional (3D echocardiography coupled with speckle-tracking echocardiographic (STE capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF. Objectives: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods: The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years. They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men. Results: Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions: Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair.

  8. Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study)

    Science.gov (United States)

    Havasi, Kálmán; Domsik, Péter; Kalapos, Anita; McGhie, Jackie S.; Roos-Hesselink, Jolien W.; Forster, Tamás; Nemes, Attila

    2017-01-01

    Background Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF). Objectives To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men). Results Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair. PMID:28327874

  9. Association between left ventricular mechanics and diffuse myocardial fibrosis in patients with repaired Tetralogy of Fallot: a cross-sectional study.

    Science.gov (United States)

    Haggerty, Christopher M; Suever, Jonathan D; Pulenthiran, Arichanah; Mejia-Spiegeler, Abba; Wehner, Gregory J; Jing, Linyuan; Charnigo, Richard J; Fornwalt, Brandon K; Fogel, Mark A

    2017-12-11

    Patients with repaired tetralogy of Fallot (TOF) have progressive, adverse biventricular remodeling, leading to abnormal contractile mechanics. Defining the mechanisms underlying this dysfunction, such as diffuse myocardial fibrosis, may provide insights into poor long-term outcomes. We hypothesized that left ventricular (LV) diffuse fibrosis is related to impaired LV mechanics. Patients with TOF were evaluated with cardiac magnetic resonance in which modified Look-Locker (MOLLI) T1-mapping and spiral cine Displacement encoding (DENSE) sequences were acquired at three LV short-axis positions. Linear mixed modeling was used to define the association between regional LV mechanics from DENSE based on regional T1-derived diffuse fibrosis measures, such as extracellular volume fraction (ECV). Forty patients (26 ± 11 years) were included. LV ECV was generally within normal range (0.24 ± 0.05). For LV mechanics, peak circumferential strains (-15 ± 3%) and dyssynchrony indices (16 ± 8 ms) were moderately impaired, while peak radial strains (29 ± 8%) were generally normal. After adjusting for patient age, sex, and regional LV differences, ECV was associated with log-adjusted LV dyssynchrony index (β = 0.67) and peak LV radial strain (β = -0.36), but not LV circumferential strain. Moreover, post-contrast T1 was associated with log-adjusted LV diastolic circumferential strain rate (β = 0.37). We observed several moderate associations between measures of fibrosis and impaired mechanics, particularly the LV dyssynchrony index and peak radial strain. Diffuse fibrosis may therefore be a causal factor in some ventricular dysfunction in TOF.

  10. Stenting of the right ventricular outflow tract after thrombosis of the modified Blalock-Taussig shunt in a 8 month old infant with tetralogy of Fallot and right pulmonary artery agenesis

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    I. A. Soynov

    2017-01-01

    Full Text Available Pulmonary artery agenesis combined with tetralogy of Fallot is the most rarely seen congenital heart disease. Children with this anomaly are an especially problematic category of patients undergoing staged surgical repair. The postoperative period quite often is complicated with shunt thrombosis while a redo open surgery is associated with a very high risk; therefore, endovascular repair is a preferred procedure. We describe a case of right ventricular outflow tract stenting in a 8 month old girl with tetralogy of Fallot and right pulmonary artery agenesis. The patient was admitted at 3 month after performing of a left-sided modified Blalock-Taussig shunt with severe signs of heart failure and desaturation caused by shunt thrombosis. Assessments performed at 2 months after stenting of the outflow tract demonstrated good oxygen saturation in arterial blood (80% and above and improvement of heart failure symptoms to NYHA II class.

  11. Fallot's tetralogy in combination with an open common atrioventricular canal

    International Nuclear Information System (INIS)

    Alekyan, B.G.; Dvinyaninova, N.B.; Chiaureli, M.R.; Arzhanukhina, S.Yu.

    1987-01-01

    The methods and clinics of diagnostics of Fallot tetrad (FT) combined with the opened general atrioventricular canal (AVC) of the heart are described on the basis of observation of a 6.5-year old patient and analysis of literary data. On the basis of studying clinical data, electro-, phono-, echocardiography and roentgenography, as well as heart catheterization and angiocardiography the conclusion is made that nowadays the most informative method of diagnostics of FT and AVC combination before angiocardiography is a dimer echocardiography which permits to diagnoze AVC. Angiocardiographic study proves this diagnosis and gives a precise anotomical picture of various components of this combination of heart failure

  12. Clinical diagnosis and alternative surgical treatment of tetralogy of Fallot in a dog. A case report Diagnóstico clínico e tratamento cirúrgico alternativo da tetralogia de Fallot em cão. Relato de caso

    Directory of Open Access Journals (Sweden)

    M.H.M.A Larsson

    2000-10-01

    Full Text Available Tetralogy of Fallot was diagnosed in a 30 month-old Cocker Spaniel female, through physical, radiographic, electrocardiographic, and echocardiographic exams. The animal was submitted to a palliative surgical correction, the Blalock-Taussig technique, and 46 months after the surgery, no signs of cardiopulmonary decompensation were observed.Tetralogia de Fallot foi diagnosticada em uma fêmea Cocker Spaniel, de 30 meses de idade, por meio de exames físico, radiográfico, eletrocardiográfico e ecocardiográfico. A paciente foi submetida à correção cirúrgica paliativa, técnica de Blalock-Taussig, e em avaliação recente, 46 meses após a cirurgia, apresentava-se sem qualquer manifestação sintomática de descompensação cardiopulmonar.

  13. Late outcome of right ventricular outflow tract repair using bicuspid pulmonary prosthesis in tetralogy of Fallot surgery repair: case report Resultado tardio da reconstrução da via de saída do ventrículo direito com prótese pulmonar bicúspide na correção da tetralogia de Fallot: relato de caso

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    Miguel Angel Maluf

    2009-12-01

    Full Text Available We present a case of a 19 year-old female patient, with tetralogy of Fallot and hypoplastic pulmonary ring. Diagnosis was performed by echocardiography and cardiac catheterization. The surgical repair was performed at the age of 2 years old. A right ventricular outflow tract remodeling was performed using a porcine pulmonary bicuspid prosthesis. The patient presented a uneventful follow-up during 17 years. Postoperative assessment has shown: mild pulmonary insufficiency and mild pulmonary ventricle gradient, satisfactory right ventricular performance and prosthesis not presenting calcification. This is an option to correct the tetralogy of Fallot adopted by our Institution in the last two decades.É apresentado o caso de uma paciente de 19 anos de idade, sexo feminino, portadora de tetralogia de Fallot com anel pulmonar hipoplásico. O diagnóstico foi feito com ecoDopplercardiograma e cateterismo cardíaco. A correção cirúrgica foi realizada com 2 anos de idade. Para a reconstrução da valva pulmonar e via de saída do ventrículo foi utilizada prótese bivalvular pulmonar porcina. A paciente teve boa evolução clínica durante 17 anos. A avaliação pós-operatória demonstrou: discreta insuficiência pulmonar, discreto gradiente transvalvar pulmonar, desempenho do ventrículo direito preservado e prótese pulmonar sem calcificação. Esse é o procedimento adotado pela nossa Instituição para a correção da tetralogia de Fallot, nos últimos 20 anos.

  14. Opção técnica na tetralogia de Fallot com artéria coronária anômala: relato de casos Technical option on tetralogy of Fallot with abnormal coronary artery: cases report

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    Claudio Roberto Assumpção

    2008-06-01

    Full Text Available Relatamos dois casos um menino de 11 anos e uma menina de um ano e seis meses, portadores de tetralogia de Fallot com artéria coronária de origem anômala, cruzando a via de saída de ventrículo direito. O diagnóstico foi feito com ecocardiografia e cateterismo cardíaco. Utilizamos, na correção, um enxerto externo composto de pericárdio bovino e parede posterior da artéria pulmonar, entre o ventrículo direito e o tronco da artéria pulmonar. As crianças tiveram boa evolução pós-operatória, obtendo alta hospitalarassintomáticas. É mais uma opção técnica na correção da tetralogia de Fallot com artéria coronária anômala.Report on two children, one 11 year-male and another one year-six-month-female with tetralogy of Fallot associated with anomalous origin of coronary artery crossing the right ventricle outlet tract. Diagnosis was done by echocardiography and cardiac catheterization. On surgical correction we used an external composite tubular pericardium bovine graft and posterior wall of the pulmonary artery between the right ventricle and the pulmonary artery trunk. Patients had an uneventful post-operative period and were discharged asymptomatic. It is more an option on correction of tetralogy of Fallot with anomalous coronary artery.

  15. O uso de dexmedetomidina na sedação de crises hipercianóticas em um recém-nascido com tetralogia de Fallot Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine

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    Hideaki Senzaki

    2008-08-01

    Full Text Available OBJETIVO: A sedação é um passo importante para aliviar e prevenir a recorrência de cianose no manejo de pacientes com crises hipercianóticas associadas à tetralogia de Fallot (T4F. Este relato de caso ilustra a eficácia da sedação induzida por dexmedetomidina no manejo de crises hipercianóticas em um recém-nascido com T4F. DESCRIÇÃO: Um paciente recém-nascido a termo de 8 dias de idade com T4F apresentava crises hipercianóticas, indicadas por quedas abruptas no nível de saturação arterial (SpO2, medido por um oxímetro de pulso, de 80% até 50%, quando o paciente ficava agitado ou irritável. Nós começamos a infusão contínua de dexmedetomidina em uma dosagem de 0,2 µg/kg/min sem injeção de ataque em bolus. Cerca de meia hora depois do início da infusão de dexmedetomidina, o paciente atingiu um nível aceitável de sedação, e sua freqüência cardíaca diminuiu aproximadamente 20 batidas por minuto. Não houve nenhuma depressão respiratória aparente ou mudança acentuada em sua pressão arterial. A SpO2 também continuou estável durante a infusão de dexmedetomidina. No dia seguinte à sua hospitalização, o paciente passou com sucesso por uma operação de anastomose de Blalock-Taussig. COMENTÁRIOS: A dexmedetomidina pode ser útil no manejo de crises hipercianóticas em pacientes pediátricos com T4F.OBJECTIVE:Sedation is an important step in the management of patients with hypercyanotic spells associated with tetralogy of Fallot (TOF to ameliorate and prevent recurrence of cyanosis. This case report illustrates the effectiveness of dexmedetomidine-induced sedation in the management of hypercyanotic spells in a neonate with TOF. DESCRIPTION: An 8-day-old term newborn patient with TOF showed hypercyanotic spells, as indicated by an abrupt decrease in arterial saturation (SpO2 level measured by a pulse oximeter from 80% to as low as 50%, when the patient became irritable and agitated. We started continuous

  16. Anestesia para correção de tetralogia de Fallot em paciente adulto: relato de caso Anestesia para corrección de tetralogía de Fallot en paciente adulto: relato de caso Anesthesia for repair of tetralogy of Fallot in an adult patient: case report

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    Michelle Nacur Lorentz

    2007-10-01

    . CONTENIDO: Están definidas las características del síndrome de la infusión del propofol en cuanto a la fisiopatología, características clínicas, tratamiento y recomendaciones de dosis para pacientes gravemente enfermos. CONCLUSIONES: El propofol debe ser usado con cautela cuando se planea su uso bajo el régimen de infusión continua por períodos prolongados. El aparecimiento de señales sugestivas del síndrome de la infusión del propofol indica la suspensión inmediata del fármaco y el inicio de medidas de soporte.BACKGROUND AND OBJECTIVES: Congenital cardiopathy is one of the most common problems at birth, with an incidence of 1 to 1.5 in 10,000 live births. Currently, more than 85% of children with congenital cardiopathy survive to adulthood, partly due to the development of new therapies, but especially because of the tendency for earlier surgical corrections. On the other hand, adult patients with non-corrected tetralogy of Fallot (TOF are hard to find. The objective of the present report was to describe a case of repair of TOF in an adult patient. CASE REPORT: A female patient, 45 years old, with TOF, was admitted for surgical repair of her condition. Physical exam revealed extremities with cyanosis and oxygen saturation (SpO2 of 73%. General balanced anesthesia consisted of ethomidate, fentanyl, pancuronium and sevoflurane. During the surgery, the ventricular communication was closed, placement of a 24 French prosthetic conduit with valve and, posteriorly, the ventricular septum was fenestrated. Milrinone and dopamine were administrated when extracorporeal circulation (ECC was removed. At the end of the surgery SpO2 was 97%. After the procedure the patient was admitted to the ICU where she remained for 72 hours, being discharged from the hospital on the eighth postoperative day in good condition. CONCLUSIONS: This case illustrated the successful surgical repair in a high-risk patient, with a very good interaction among the surgeon, anesthetist, and

  17. Quantification of biventricular myocardial function using cardiac magnetic resonance feature tracking, endocardial border delineation and echocardiographic speckle tracking in patients with repaired tetralogy of fallot and healthy controls

    Science.gov (United States)

    2012-01-01

    Background Parameters of myocardial deformation have been suggested to be superior to conventional measures of ventricular function in patients with tetralogy of Fallot (ToF), but have required non-routine, tagged cardiovascular magnetic resonance (CMR) techniques. We assessed biventricular myocardial function using CMR cine-based feature tracking (FT) and compared it to speckle tracking echocardiography (STE) and to simple endocardial border delineation (EBD). In addition, the relation between parameters of myocardial deformation and clinical parameters was assessed. Methods Overall, 28 consecutive adult patients with repaired ToF (age 40.4 ± 13.3 years) underwent standard steady-state-free precession sequence CMR, echocardiography, and cardiopulmonary exercise testing. In addition, 25 healthy subjects served as controls. Myocardial deformation was assessed by CMR based FT (TomTec Diogenes software), CMR based EBD (using custom written software) and STE (TomTec Cardiac Performance Analysis software). Results Feature tracking was feasible in all subjects. A close agreement was found between measures of global left (LV) and right ventricular (RV) global strain. Interobserver agreement for FT and STE was similar for longitudinal LV global strain, but FT showed better inter-observer reproducibility than STE for circumferential or radial LV and longitudinal RV global strain. Reproducibility of regional strain on FT was, however, poor. The relative systolic length change of the endocardial border measured by EBD yielded similar results to FT global strain. Clinically, biventricular longitudinal strain on FT was reduced compared to controls (P < 0.0001) and was related to the number of previous cardiac operations. In addition, FT derived RV strain was related to exercise capacity and VE/VCO2-slope. Conclusions Although neither the inter-study reproducibility nor accuracy of FT software were investigated, and its inter-observer reproducibility for regional

  18. Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot.

    Science.gov (United States)

    Riesenkampff, Eugénie; Luining, Wietske; Seed, Mike; Chungsomprasong, Paweena; Manlhiot, Cedric; Elders, Bernadette; McCrindle, Brian W; Yoo, Shi-Joon; Grosse-Wortmann, Lars

    2016-10-26

    Unfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors. In this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity. There was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r = 0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p < 0.05). As compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males.

  19. Associations between N-terminal pro-B-type natriuretic peptide and cardiac function in adults with corrected tetralogy of Fallot.

    Science.gov (United States)

    Eindhoven, Jannet A; Menting, Myrthe E; van den Bosch, Annemien E; Cuypers, Judith A A E; Ruys, Titia P E; Witsenburg, Maarten; McGhie, Jackie S; Boersma, Eric; Roos-Hesselink, Jolien W

    2014-07-01

    Amino-terminal B-type natriuretic peptide (NT-proBNP) may detect early cardiac dysfunction in adults with tetralogy of Fallot (ToF) late after corrective surgery. We aimed to determine the value of NT-proBNP in adults with ToF and establish its relationship with echocardiography and exercise capacity. NT-proBNP measurement, electrocardiography and detailed 2D-echocardiography were performed on the same day in 177 consecutive adults with ToF (mean age 34.6 ± 11.8 years, 58% male, 89% NYHA I, 29.3 ± 8.5 years after surgical correction). Thirty-eight percent of the patients also underwent a cardiopulmonary-exercise test. Median NT-proBNP was 16 [IQR 6.7-33.6] pmol/L, and was elevated in 55%. NT-proBNP correlated with right ventricular (RV) dilatation (r = 0.271, p present in 69 patients (39%). Moderate or severe pulmonary regurgitation was not associated with higher NT-proBNP. Tricuspid and pulmonary regurgitation peak velocities correlated with NT-proBNP (r = 0.305, p < 0.001 and r = 0.186, p = 0.045, respectively). LV twist was measured with speckle-tracking echocardiography in 71 patients. An abnormal LV twist (20 patients, 28%) was associated with elevated NT-proBNP (p = 0.030). No relationship between NT-proBNP and exercise capacity was found. NT-proBNP levels are elevated in more than 50% of adults with corrected ToF, while they are in stable clinical condition. Higher NT-proBNP is most strongly associated with elevated pulmonary pressures, and with LV dysfunction rather than RV dysfunction. NT-proBNP has the potential to become routine examination in patients with ToF to monitor ventricular function and may be used for timely detection of clinical deterioration. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  20. Perioperative management of a patient with Dandy Walker malformation with tetralogy of Fallot undergoing total correction and fresh homologous pericardial pulmonary valve conduit implantation: Report of a rare case

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    Vishnu Datt

    2015-01-01

    Full Text Available Perioperative management of a patient with Dandy-Walker malformation (DWM with tetralogy of Fallot (TOF, patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP, conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo-peritoneal (VP shunt should be performed before cardiac surgery. We present the first case report of a 11-month-old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion.

  1. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance.

    NARCIS (Netherlands)

    Oosterhof, T.; Straten, A. van; Vliegen, H.W.; Meijboom, F.J.; Dijk, A.P.J. van; Spijkerboer, A.M.; Bouma, B.J.; Zwinderman, A.H.; Hazekamp, M.G.; Roos, A.; Mulder, B.J.M.

    2007-01-01

    BACKGROUND: To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. METHODS AND RESULTS: Between 1993 and 2006, 71 adult patients with

  2. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance

    NARCIS (Netherlands)

    Oosterhof, Thomas; van Straten, Alexander; Vliegen, Hubert W.; Meijboom, Folkert J.; van Dijk, Arie P. J.; Spijkerboer, Anje M.; Bouma, Berto J.; Zwinderman, Aeilko H.; Hazekamp, Mark G.; de Roos, Albert; Mulder, Barbara J. M.

    2007-01-01

    Background - To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular ( RV) volumes above which no decrease or normalization of RV size takes place after surgery. Methods and Results - Between 1993 and 2006, 71 adult patients with

  3. Interventional catheter methods for complex tetralogy of fallot and 'Critical' PS/pulmonary atresia with intact interventional septum

    International Nuclear Information System (INIS)

    Issenberg, H.J.

    2005-01-01

    TOF is generally accepted as the index lesion used for evaluating the overall quality of a congenital cardiac program. To achieve good results , the evaluation and management of TOF requires an intimate collaboration between all members of the cardiac team involved in the care of these children, especially the interventional catheterizer and the surgeon. Each program has to develop its own approach, relying on the collective strengths of their staff. Any weak link in this chain will often have a profoundly negative effect upon the patient , which may be difficult to correct later. Whereas the cathlab was the sole source of the anatomic and physiologic data for surgical planning, much of the information, especially intracardiac anatomy and physiology, can now be acquired with echocardiography, and if necessary, enhanced with MRI and CT. The role of diagnostic angiography is reserved for the elucidation of complex and small vascular structures beyond the resolution and specificity of less invasive methods, such as pulmonary artery branches, coronary arteries and arterial collaterals. This anatomy becomes particularly important when staging procedures for TOF in the infant with small pulmonary arteries or atresia, when is the greatest potential for enhancing arterial size and vascular bed growth. Our approach is to perform early correction which will insure antegrade flow into the small pulmonary arteries, by opening the outflow tract (patch or conduit), and when feasible, closing the VSD. Aortic to pulmonary arterial shunts are avoided. Direct access into the pulmonary arteries allows additional catheter-based interventions. Which may include balloon dilation and stenting, which avoiding any deformity from peripheral shunts. Aortic collaterals perfusing lung segments competing with antegrade flow into the branch pulmonary arteries may need Giannturco coil occlusion. Those with small pulmonary arteries may require multiple ballooning/stenting interventions to increase

  4. 31. Abordaje transaórtico de la tetralogía de fallot en el adulto

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    M.T. González López

    2012-04-01

    Conclusiones: El cierre de la CIV en la tetralogía de Fallot del adulto mediante abordaje transaórtico resulta sencillo, por las dimensiones de la raíz y el acabalgamiento sobre la misma. En casos de situs inversus, esta técnica facilita sobremanera la corrección final.

  5. Role of myocardial hypertrophy on acute and chronic right ventricular performance in relation to chronic volume overload in a porcine model: relevance for the surgical management of tetralogy of Fallot.

    Science.gov (United States)

    Bove, Thierry; Vandekerckhove, Kristof; Bouchez, Stefaan; Wouters, Patrick; Somers, Pamela; Van Nooten, Guido

    2014-06-01

    The age for correction of tetralogy of Fallot has progressively declined to the postnatal period, often despite an increased rate of transannular patch repair. However, the long-term effect of premature exposure to chronic pulmonary insufficiency on the right ventricle remains unknown. On the basis of the relationship between the duration of pressure overload and age, the role of previous pressure load-related hypertrophy on right ventricular (RV) performance after chronic volume overload was investigated in a porcine model. RV hypertrophy (RVH), induced by pulmonary artery banding, was studied in pigs with (RVH plus pulmonary insufficiency [PI]) and without (RVH) subsequent PI. The effect of volume overload was compared between these 2 groups and pigs without RVH but with PI and controls (sham). Both acute and chronic effects on RV function were studied using conductance technology and validated using echocardiography. After chronic volume overload, the end-systolic and end-diastolic volumes were smaller in the RVH+PI group than in the PI group, including a lower pulmonary regurgitation fraction (25% ± 5% vs 35% ± 5%; P = .002). RVH resulted in better preserved systolic function, confirmed by an increased preload recruitable stroke work slope (14.7 ± 1.8 vs 9.3 ± 1.3 Mw.s/mL; P = .025) and higher RV ejection fraction (51% ± 3% vs 45% ± 4%; P = .05). Myocardial stiffness was impaired in the RVH+PI group versus the PI group (β, 0.19 ± 0.03 vs 0.12 ± 0.02 mL(-1); P = .001), presenting restrictive physiology only in the condition associating RVH and PI. The results of the present study have demonstrated that RVH attenuates the RV remodeling process related to chronic PI. It enables better preservation of contractility but at the cost of sustained diastolic impairment. These findings might help to determine the timing and strategy for repair of tetralogy of Fallot when RV outflow tract morphology indicates a definite need for transannular reconstruction

  6. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome

    Science.gov (United States)

    Silversides, Candice K; Beauchesne, Luc; Bradley, Timothy; Connelly, Michael; Niwa, Koichiro; Mulder, Barbara; Webb, Gary; Colman, Jack; Therrien, Judith

    2010-01-01

    With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org. PMID:20352138

  7. Fourier analysis of multi-gated cardiac blood-pool data in patients with congenital heart diseases, (2). Assessment of diseases with complex cardiac anomalies, especially tetralogy of Fallot

    Energy Technology Data Exchange (ETDEWEB)

    Takeda, Kan; Maeda, Hisato; Yamaguchi, Nobuo; Nakamura, Kazuyoshi; Matsumura, Kaname; Nakagawa, Tsuyoshi; Sakurai, Minoru; Aoki, Kenzo

    1985-04-01

    The clinical usefulness of Fourier analysis of multi-gated cardiac blood-pool data was evaluated in 18 subjects with normal cardiac functions and 14 patients with complex cardiac anomalies (ten with tetralogy of Fallot, two with tricuspid atresia (TA), one with double-outlet right ventricle (DORV), and one with Ebstein's anomaly (EA)). Using global ventricular time-activity curves, the phase and amplitude at fundamental frequency were calculated, and emptying patterns of the left and right ventricles (LV, RV) were evaluated by phase difference (D(phase)=RV phase minus LV phase) and amplitude ratio of RV to LV (R(amp)). In patients with TOF, mean values of D (phase) and R(amp) were 25.3 +- 10.5 degrees and 13.5 +- 0.49 respectively and significantly larger than those of normal subjects. D (phase) became larger in inverse proportion to the ratio of pulmonary-to-systemic blood flow and there was an inverse linear correlation between these two variables. On visual interpretation of functional images, the dynamic property of hypoplastic ventricles could be easily estimated in patients with TA or DORV. In a case with EA, the atrialized RV was shown clearly as a hypokinetic, atrial phase area. This method is valuable for pathophysiologic investigation of diseases with complex cardiac anomalies. (author).

  8. Right-ventricular and left-ventricular function parameters in patients with and without outflow tract patches as determined by radionuclide methods at least 10 years following surgical correction of Fallot's tetralogy in the adult individuals

    International Nuclear Information System (INIS)

    Seidel, P.

    1988-01-01

    During a period starting in 1983 and ending in April 1984 radionuclide ventriculography was performed in 26 patients subjected to surgical correction of Fallot's tetralogy after having reached adulthood. They were divided into two groups according to the surgical methods used. Patients showing no right-ventricular outflow patch were assigned to group I, while group II was made up of individuals that had received outflow tract patches of Dacron or Teflon for pressure reduction in the right ventricle. The parameters registered during radionuclide ventriculography, in which the tracer substance TC 99m was administered into a peripheral vein, included the endiastolic volume, endsystolic volume, stroke volume, global ejection fraction and cardiac index. For resting patients, these values were calculated using the first-pass technique, the determinations during exercise on the ergometer were based on equilibrium radionuclide ventriculography. It could be concluded from the results obtained here that non-invasive endocardial scintigraphy is a sensitive method that can be used both at rest and during exercise on the ergometer to detect function disorders of the right and left ventricles. (orig./MG) [de

  9. Avaliação clínica e funcional tardia de arritmias em crianças operadas de Tetralogia de Fallot Evaluación clínica y funcional tardía de arritmias en niños operados de tetralogía de Fallot Late clinical and functional assessment of arrhythmias in children after repair of Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Maria Eulália Thebit Pfeiffer

    2010-09-01

    . OBJETIVO: Evaluar la ocurrencia de arritmias cardíacas en niños y adolescentes operados de TF, asociándolas a los aspectos clínicos y de exámenes complementarios. MÉTODOS: Estudio transversal en 37 pacientes sometidos a cirugía de TF en el Instituto Estadual de Cardiología Aloysio de Castro (RJ. Tras revisión de prontuarios y evaluación clínica, los pacientes fueron sometidos a electrocardiograma (ECG, ecocardiograma (Eco, Holter 24h (Holter y prueba ergométrica (PE, siendo los resultados sometidos al análisis estadístico. RESULTADOS: Estudiados 37 pacientes, el 54% masculinos, edad 9,7 ± 3,5 años, seguimiento promedio de 4,7 ± 1,9 años. Cambio más prevalentes: ECG: bloqueo de rama derecha (89%; Eco: insuficiencia pulmonar severa (43%, estenosis pulmonar ligera (73%, hipertrofia ventricular derecha (HVD moderada (57%. En la PE, baja capacidad de ejercicio (90%, déficit cronotrópico (40%, arritmias (20%. Al Holter, el 59% presentó arritmias: ventriculares el 44%, supraventriculares el 38% y ambas el 24%, con predominio de extrasístoles poco frecuentes y benignas. Cinco pacientes (15% presentaron extrasístoles polimórficas. Hubo asociación entre arritmia ventricular y HVD moderada y severa (p=0,026 y también con gradiente ventrículo derecho-arteria pulmonar (VD/AP >45 mmHg (p=0,004. A través de la Regresión Logística, el aumento del gradiente VD/AP fue factor predictivo independiente para arritmia ventricular (p=0,017. CONCLUSIÓN: Las arritmias cardíacas fueron un hallazgo común en gran parte de los niños y adolescentes tras reparación quirúrgica de TF, sin embargo, poco frecuentes y benignas, en la mayoría de los casos. El gradiente de presión VD-AP se consideró como un fuerte predictor para arritmia ventricular.BACKGROUND: Cardiac arrhythmias are the major cause of late sudden death in patients undergoing repair of Tetralogy of Fallot (TF. OBJECTIVE: To evaluate the occurrence of cardiac arrhythmias in children and adolescents

  10. Análise de fatores pré e per-operatórios determinantes do resultado cirúrgico da tetralogia de Fallot Analysis of the factors pre and post-operatory, as determinants of the surgical results of the tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Bayard Gontijo Filho

    1987-08-01

    Full Text Available São analisados os resultados pós-operatórios imediatos de 98 pacientes submetidos a tratamento cirúrgico da tetralogía de Fallot (TF num total de 109 procedimentos (29 cirurgias paliativas e 80 correções definitivas. No grupo de cirurgias paliativas, ocorreram 2 óbitos no início de nossa experiência, não havendo mortalidade nos últimos 20 pacientes operados. A técnica de Blalock-Taussig clássica foi a mais utilizada (19 casos. No grupo de correção total, houve 9 óbitos (11,2%, sendo 7 deles no período de 1979 a 1983, ocorrendo apenas 2 óbitos nos últimos 42 casos operados. A presença de anastomose sistêmico-pulmonar prévia (8 casos não interferiu na mortalidade pós-operatória. A análise retrospectiva desta experiência mostrou que os fatores que influenciaram negativamente o resultado cirúrgico foram: o baixo peso, a baixa idade, o alargamento de via de saída e alguns tipos de malformações associadas. A nossa conduta atual, baseada em uma sistematização colocada em prática a partir de 1984, prevê correção cirúrgica em 2 estágios, com tratamento paliativo antes dos 2 anos de idade. A mortalidade combinada observada nos últimos 2 anos foi de 4,7%.We report the immediate postoperative results of 98 patients who underwent surgical treatment for tetralogy of Fallot. A paliative operation was employed in 29 children and total correction was performed in 80 patients (109 operations. There were 2 postoperative deaths in the group of paliative treatment which occurred in the beggining of our experience and no deaths were found in the last 20 operations. In the group of patients who underwent total correction there were 9 deaths (11,2%, but only 2 of these deaths occurred in the last 42 patients operated. The presence of a previous shunt (8 patients did not interfere with the postoperative result. The factors that determined a higher postoperative mortality were: age under 2 years, weight under 10 kg, presence of

  11. Emprego e avaliação em médio prazo da cúspide de homoenxerto decelularizado na correção da tetralogia de Fallot The use and midium-term evaluation of decellularized allograft cusp in the surgical treatment of the tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Leonardo Andrade Mulinari

    2008-06-01

    Full Text Available OBJETIVO: Descrever a técnica de preparo e a evolução ecocardiográfica das cúspides de homoenxerto decelularizado utilizadas em pacientes com tetralogia de Fallot. MÉTODOS: No período de março de 2005 a agosto de 2007, 15 pacientes foram submetidos ao implante deste tipo de enxerto e foram acompanhados clinicamente e com ecocardiograma para avaliar o resultado morfofuncional dos enxertos. RESULTADOS: O acompanhamento médio foi de 12,7 meses (1-25 meses. A análise ecocardiográfica em médio prazo revelou: insuficiência pulmonar leve em nove (60% pacientes, moderada em três (20% e importante em três (20%; a função sistólica do ventrículo direito esteve preservada em 13 (86,7% pacientes e com disfunção leve em dois (13,3%; 11 (73,4% pacientes não apresentaram gradientes na via de saída do ventrículo direito (VD, e em quatro (26,6% pacientes evidenciou-se a presença de estenose leve; a mobilidade da cúspide foi normal em todos os pacientes; não houve espessamento maior de 1,5mm nas cúspides analisadas; não se detectou nenhuma calcificação nas cúspides. Catorze (93,3% pacientes apresentaram Z score entre -1 e 0,7 e um (6,7% paciente apresentou anel pulmonar com Z score de + 2,5. CONCLUSÃO: O retalho de homoenxerto decelularizado parece ser uma boa opção para a ampliação da via de saída do VD nos pacientes submetidos à correção total da tetralogia de Fallot em médio prazo.OBJECTIVE: To describe the technique of implantation and to show the echocardiographic follow-up of the decellularized cusps allografts used in patients with tetralogy of Fallot. METHODS: Fifteen patients underwent this implantation between March 2005 and August 2007 and they were clinically followed-up. An echocardiogram was performed to evaluate the morphofunctional result of the allografts. RESULTS: The mean follow-up was 12.7 months (1-25 months. The echocardiography results showed that pulmonary insufficiency was mild in nine (60

  12. Correção intracardíaca da tetralogia de Fallot no primeiro ano de vida: resultados a curto e médio prazos Intracardiac correction of tetralogy of Fallot in the first year of life: short-term and midium-term results

    Directory of Open Access Journals (Sweden)

    Fernando Ribeiro de Moraes Neto

    2008-06-01

    Full Text Available OBJETIVO: Avaliar os resultados a curto e médio prazos da correção intracardíaca da tetralogia de Fallot no primeiro ano de vida. MÉTODOS: De janeiro de 1996 a outubro de 2004, 67 crianças com idade variando de 1 a 11 meses (média: 7,2 meses e pesando entre 4 a 10 quilos (média: 7,1 kg foram eletivamente submetidas a correção intracardíaca da tetralogia de Fallot. A cirurgia foi realizada com circulação extracorpórea convencional e hipotermia moderada. Ventriculotomia direita foi realizada em 60 (89,5% casos e em sete (10,5% utilizou-se a abordagem do defeito pela via atriopulmonar. RESULTADOS: O tempo de circulação extracorpórea variou de 35 a 147 minutos (média:78,8 ± 21 minutos, e o tempo de pinçamento da aorta variou de 25 a 86 minutos (média: 51,8 ± 15,6 minutos. Ampliação transanular da via de saída do ventrículo direito foi necessária em 50 (64,1% casos. O gradiente entre o ventrículo direito e a artéria pulmonar, após a correção, variou de 0 a 54 mmHg (média: 15,5 ± 10,8 mmHg. Ocorreram dois (2,98% óbitos no pós-operatório imediato. O seguimento tardio variou de 7 a 115 meses (média: 44,0 ± 35 meses. Houve uma morte tardia não-cardíaca. Todos os outros pacientes estão assintomáticos. A curva de sobrevida actuarial, incluindo a mortalidade operatória, revelou que a probabilidade de sobrevida, 12 anos após a operação, é de 97%. Dez pacientes foram avaliados por angiorresonânia magnética nuclear. CONCLUSÕES: A correção intracardíaca da tetralogia de Fallot no primeiro ano de vida pode ser feita com baixas morbidade e mortalidade e bons resultados tardios.OBJECTIVE: To evaluate short-term and medium-term results of intracardiac correction of Tetralogy of Fallot in the first year of life. METHODS: From January 1996 to October 2004, 67 consecutive infants ranging in age from 1 to 11 months (mean: 7.2 months and weighing from 4 to 10 kilograms (mean: 7.1 kilograms underwent elective total

  13. Feto portador de síndrome de turner e tetralogia de fallot associadas à elevação de alfafetoproteína materna Fetal turner syndrome and tetralogy of fallot associated with elevated maternal serum alpha-fetoprotein levels

    Directory of Open Access Journals (Sweden)

    Eduardo Vieira Neto

    1998-06-01

    developed, requiring corticosteroid therapy and premature cesarean section at the 34th week of gestation. The female newborn was transferred to a neonatal ICU and tetralogy of Fallot and Turner syndrome were diagnosed. This case prompted the authors to review the literature on maternal serum biochemical markers in Turner syndrome and congenital heart defects and to propose a protocol for unexplained increased MSAFP.

  14. Anastomosis cava-pulmonar en el tratamiento quirúrgico de la tetralogía de Fallot

    Directory of Open Access Journals (Sweden)

    José Félix Patiño

    1965-01-01

    Full Text Available Se informa sobre la aplicación clínica exitosa de la técnica de anastomosis cava-pulmonar, originalmente descrito por Glenn y Patiño, en un caso de Tetralogía de Fallot. Representa este caso el primer paciente tratado en esta forma en la literatura médica occidental. La anastomosis cava-pulmonar es un procedimiento técnicamente muy sencillo que permite mejorar notablemente la oxigenación en pacientes que presentan ciertas anomalías en el corazón derecho o en la arteria pulmonar. La anastomosis cava-pulmonar parece ser una operación fisiológica fácil de realizar, bien tolerada, que da como resultado buena oxigenación de la sangre venosa desembocada al pulmón derecho, y que en el caso de la Tetralogía de Fallot, presenta notables ventajas sobre las operaciones paliativas clásicas de Blalock y Potts. La anastomosis cava-pulmonar es un procedimiento que puede ser usado en el tratamiento quirúrgico de la Tetralogía de Fallot como operación paliativa definitiva, o como la primera etapa que mejora las condiciones generales del paciente para permitir que más tarde pueda ser llevado a corrección definitiva. Esta anastomosis, que disminuye trabajo al corazón, no necesariamente tiene que ser deshecha a tiempo de la corrección definitiva, a diferencia de las operaciones clásicas de Blalock y Pott, que sí añaden trabajo al corazón al crear un ductus artificial y una hipertensión pulmonar. Se propone el uso clínico de la anastomosis cava-pulmonar en aquellos casos de Tetralogía de Fallot que requieran una operación paliativa extra-cardíaca o como la primera etapa antes de ser sometidos a la corrección definitiva por circulación extracorpórea.

  15. 171. Funcionamiento y resultados del programa de fast-track en el tratamiento de la tetralogía de fallot

    Directory of Open Access Journals (Sweden)

    J. Orrit Palacios

    2010-01-01

    Conclusiones: La corrección completa de la tetralogía de Fallot con el programa de fast-track de anestesia disminuye la ventilación mecánica, la estancia en cuidados intensivos y el tiempo de hospitalización. Asimismo se reducen los costes económicos del tratamiento.

  16. Síndrome de bajo gasto cardíaco en la tetralogía de Fallot

    Directory of Open Access Journals (Sweden)

    Lincoln de la Parte Pérez

    2002-06-01

    Full Text Available Se realiza un estudio retrospectivo de 200 niños operados de tetralogía de Fallot en el Cardiocentro del Hospital Pediátrico Universitario "William Soler" durante el período comprendido entre los años 1990 y 1993. Se observó una relación directamente proporcional entre el tiempo de pinzamiento aórtico (paro anóxico, el tiempo de circulación extracorpórea (CEC y el desarrollo del síndrome de bajo gasto cardíaco. La incidencia de bajo gasto cardíaco en los niños con tiempo de pinzamiento aórtico menor a los 30 min fue nula, en los pacientes con tiempo menor a los 60 min la incidencia fue del 23,2 % (40 pacientes. En los 24 pacientes que sufrieron procedimientos quirúrgicos prolongados y tiempo de pinzamiento aórtico mayor de 1 hora la incidencia fue del 95,8 %. En los pacientes con tiempo de CEC menor de 90 min la incidencia de bajo gasto cardíaco que de sólo el 9,75 %, en los niños con tiempo de CEC entre 91 y 105 min fue de 15,53 %, en los que necesitaron entre 106 y 120 min de CEC fue del 62,5 % y en los procedimientos quirúrgicos prolongados fue del 95,8 %. La mayoría de los pacientes respondió bien al tratamiento, aunque en un pequeño número de ellos se mantuvo la disfunción ventricular a pesar del tratamiento. Siete niños fallecieron en el posoperatorio para una mortalidad del 3,5 %.A retrospective study of 200 children with Tetralogy of Fallot, who were operated on the Cardiology Center of "William Soler" University Pediatric Hospital from 1990 to 1993, was performed. There was a direct proportional relation between time of holding aorta with forceps (anoxic arrest, time of extracorporeal circulation and the development of low cardiac output syndrome. The incidence of low cardiac output in children who underwent holding of aorta with forceps for less than 30 minutes was zero, but the same procedure applied in patients for less than 60 minutes, the incidence was 23,2%(40 patients. The 24 patients that underwent

  17. Tetralogía de Fallot asociada a duplicación distal del brazo largo del cromosoma 11

    Directory of Open Access Journals (Sweden)

    Elodia Torres

    2015-04-01

    Full Text Available La tetralogía de Fallot es una cardiopatía frecuente y puede representar hasta el 11 al 13% de todas las cardiopatías congénitas clínicas, se presenta en 1 de cada 8.500 nacidos vivos. En la mayoría de los casos, se asocia a una microdeleción del cromosoma 22 y con menor frecuencia al síndrome de Down. El síndrome de la dup 11q es una anomalía cromosómica causada por la duplicación de la porción distal del extremo del brazo largo del cromosoma 11, ocasionando una trisomía parcial del mismo, producto de un desbalance cromosómico, con disfunción de los genes involucrados en este material genético adicional que ocasiona anormalidades tanto físicas como mentales en un recién nacido. Se presenta el caso de un niño de 3 meses de vida que es derivado a la consulta genética por fenotipo sindromático, Tetralogía de Fallot y retraso del crecimiento. El estudio citogenético se realizó en sangre periférica, los cromosomas fueron procesados con técnicas de tinción convencional, bandas de alta resolución y centroméricas, observándose una duplicación 11q. Cariotipo: 46, XY, dup11 (q23àqter. Se enfatiza la importancia del estudio cromosómico en recién nacidos con malformaciones congénitas mayores para el diagnóstico de certeza y posterior asesoramiento genético a los progenitores.

  18. Abnormal biventricular performance in asymptomatic adolescents late after repaired Tetralogy of Fallot: Combined two-dimensional speckle tracking and three-dimensional echocardiography study

    Directory of Open Access Journals (Sweden)

    Ken-Pen Weng

    2018-02-01

    Conclusion: Our results suggest asymptomatic adolescents with repaired TOF had abnormal biventricular myocardial performance, as demonstrated by combined 2D speckle-tracking and 3D echocardiography. The implications of these findings for management of adolescents late after repaired TOF remain to be determined.

  19. Right-ventricular and left-ventricular function parameters in patients with and without outflow tract patches as determined by radionuclide methods at least 10 years following surgical correction of Fallot's tetralogy in the adult individuals. Nuklearmedizinische Bestimmung der rechts- und linksventrikulaeren Funktionsparameter von Patienten ohne und mit Ausflusstraktpatch - mindestens 10 Jahre nach operativer Korrektur der Fallot'schen Tetralogie im Erwachsenenalter

    Energy Technology Data Exchange (ETDEWEB)

    Seidel, P.

    1988-12-15

    During a period starting in 1983 and ending in April 1984 radionuclide ventriculography was performed in 26 patients subjected to surgical correction of Fallot's tetralogy after having reached adulthood. They were divided into two groups according to the surgical methods used. Patients showing no right-ventricular outflow patch were assigned to group I, while group II was made up of individuals that had received outflow tract patches of Dacron or Teflon for pressure reduction in the right ventricle. The parameters registered during radionuclide ventriculography, in which the tracer substance TC 99m was administered into a peripheral vein, included the endiastolic volume, endsystolic volume, stroke volume, global ejection fraction and cardiac index. For resting patients, these values were calculated using the first-pass technique, the determinations during exercise on the ergometer were based on equilibrium radionuclide ventriculography. It could be concluded from the results obtained here that non-invasive endocardial scintigraphy is a sensitive method that can be used both at rest and during exercise on the ergometer to detect function disorders of the right and left ventricles. (orig./MG).

  20. Propranolol plasma monitoring in children submitted to surgery of tetralogy of Fallot by a micromethod using high performance liquid chromatography Monitoramento do propranolol plasmático em crianças operadas da tetralogia de Fallot através de micrométodo utilizando a cromatografia líquida de alta eficiência

    Directory of Open Access Journals (Sweden)

    Cristina Sanches

    2007-01-01

    Full Text Available OBJECTIVE: To evaluate the analytical micromethod using liquid chromatography for the quantification of propranolol in children submitted to surgery of tetralogy of Fallot (TLF. Methods: Only 0.2 mL of plasma is required for the assay. Peaks eluted at 8.4 (Propranolol and 17.5 min (verapamil, internal standard from a C18 column, with a mobile phase 0.1 M acetate buffer, pH 5.0, and acetonitrile (60:40, v/v at flow rate 0.7 mL/min, detected at 290 nm (excitation and 358 nm (emission. Surgery was started 776 min of drug administration (8.7mg, mean; seven blood samples were collected from six patients (4M/2F; 2.1yrs;11.5kg; 0.80m; 18.9kg/m². RESULTS: Confidence limits of the method showed high selectivity and recovery, sensitivity of 0.02ng/mL, good linearity (0.05-1000ng/mL, precision of 8.6% and accuracy of 3.1%. The mean duration of surgery was 283.2min, with the patients remaining under cardiopulmonary bypass (CPB for 114min. A declining curve of propranolol plasma concentration was obtained after the last dose in the night that preceded the day of surgery. Plasma concentration also was normalized with hematocrit due to the hemodilution caused by the CPB procedure. On the other hand a decrease on drug plasma concentration was obtained between periods, the beginning of surgery to the postoperative day 2 (7.09 ng/mL and 0.05 ng/mL, pOBJETIVO: Avaliar o micrométodo analítico empregando a cromatografia líquida para quantificação de propranolol em crianças operadas de tetralogia de Fallot (TLF. MÉTODO: Requereu-se apenas volumes de 0,2mL de plasma para a realização do ensaio. Os picos foram eluídos em 8.4 (Propranolol e 17.5 min (verapamil, padrão interno de uma coluna C18, com fase móvel (tampão acetato 0,1 M pH 5,0 e acetonitrila, 60:40, v/v em fluxo de 0,7 mL/min, sendo detectados em 290 nm (excitação e em 358 nm (emissão. A cirurgia iniciou-se 776 min depois da dose administrada (8,7mg, média e sete amostras de sangue foram

  1. Tetralogía de Fallot con síndrome de válvula pulmonar ausente: Presentación de un caso y revisión de la literatura

    OpenAIRE

    Gómez¹, Matías; Vayo¹, María Agustina; Ortiz², Lucía M.; Portis³, Marcelo; Echazarreta4, Diego; Marelli5, Daniel

    2017-01-01

    La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente del adulto. El síndrome de válvula pulmonar ausente constituye una variante poco frecuente, que representa del 3% al 6% de los pacientes con tetralogía de Fallot. Presentamos el caso de un paciente masculino de 29 años de edad, con tetralogía de Fallot y síndrome de válvula pulmonar ausente, los hallazgos del examen físico y los principales exámenes complementarios; como así también su evolución y una revisión de la l...

  2. Testing of RPMI-1640 as a Nutrient Medium for Fresh Semilunar Valve Storage.

    Science.gov (United States)

    1979-01-01

    Tetralogy of Fallot, pulmonary atresia, ventricular septal defect, truncus arteriosis , transposition of the great arteries with intact ventricular septum and...a method of overcoming severe developmental abnormalities such as ventricular atresia, truncus arteriosis , and Tetrology of Fallot. 3 Experimental

  3. On the role of the gap junction protein Cx43 (GJA1 in human cardiac malformations with Fallot-pathology. a study on paediatric cardiac specimen.

    Directory of Open Access Journals (Sweden)

    Aida Salameh

    Full Text Available INTRODUCTION: Gap junction channels are involved in growth and differentiation. Therefore, we wanted to elucidate if the main cardiac gap junction protein connexin43 (GJA1 is altered in patients with Tetralogy of Fallot or double-outlet right ventricle of Fallot-type (62 patients referred to as Fallot compared to other cardiac anomalies (21 patients referred to as non-Fallot. Patients were divided into three age groups: 0-2years, 2-12years and >12years. Myocardial tissue samples were collected during corrective surgery and analysis of cell morphology, GJA1- and N-cadherin (CDH2-distribution, as well as GJA1 protein- and mRNA-expression was carried out. Moreover, GJA1-gene analysis of 16 patients and 20 healthy subjects was performed. RESULTS: Myocardial cell length and width were significantly increased in the oldest age group compared to the younger ones. GJA1 distribution changed significantly during maturation with the ratio of polar/lateral GJA1 increasing from 2.93±0.68 to 8.52±1.41. While in 0-2years old patients ∼6% of the lateral GJA1 was co-localised with CDH2 this decreased with age. Furthermore, the changes in cell morphology and GJA1-distribution were not due to the heart defect itself but were significantly dependent on age. Total GJA1 protein expression decreased during growing-up, whereas GJA1-mRNA remained unchanged. Sequencing of the GJA1-gene revealed only few heterozygous single nucleotide polymorphisms within the Fallot and the healthy control group. CONCLUSION: During maturation significant changes in gap junction remodelling occur which might be necessary for the growing and developing heart. In our study point mutations within the Cx43-gene could not be identified as a cause of the development of TOF.

  4. The role of cardiac CT angiography in the pre- and postoperative ...

    African Journals Online (AJOL)

    2015-12-17

    Dec 17, 2015 ... Memorial Children's Hospital, ... Tornado', first coined the now familiar term of tetralogy of Fallot (TOF) in 1924. ... of this realisation have resulted in improved long-term ... (MDCT) allows fast imaging with very short acquisition.

  5. Tropical Journal of Health Sciences - Vol 19, No 2 (2012)

    African Journals Online (AJOL)

    Cerebral And Myocardial Infarction In A Patient With Uncorrected Tetralogy Of Fallot · EMAIL FULL TEXT EMAIL FULL TEXT DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. WE Sadoh, IN Adigweme, EJ Ogbemudia, 40-41 ...

  6. African Journal of Anaesthesia and Intensive Care - Vol 12, No 2 ...

    African Journals Online (AJOL)

    Cerebral Abscess in a 7yr old with uncorrected tetralogy of Fallot: Anaesthetic management · EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. AA Majekodunmi, OO Kanu, OE Idowu, BA Animasahun, 49-51 ...

  7. Echocardiographic right ventricle longitudinal contraction indices cannot predict ejection fraction in post-operative Fallot children.

    Science.gov (United States)

    Bonnemains, Laurent; Stos, Bertrand; Vaugrenard, Thibaud; Marie, Pierre-Yves; Odille, Freddy; Boudjemline, Younes

    2012-03-01

    To examine in a population of post-operative tetralogy of Fallot patients, the correlation between right ventricle (RV) ejection fractions (EF) computed from magnetic resonance imaging (MRI) and three echocardiographic indices of RV function: TAPSE, longitudinal strain and strain rate. Indeed, these patients present a pulmonary regurgitation which is responsible for progressive dilatation of the RV. An echocardiographic assessment of the RV function would be very useful in determining the timing of pulmonary revalvulation for Fallot patients. However, these indices are generally based on the ventricle contraction in the long axis direction which is impaired in this population and does not seem to correlate with the EF. Thirty-five post-operative tetralogy of Fallot patients and 20 patients with normal RVs were included. In both groups, RVEF, assessed by MRI, was compared with the three echocardiographic indices. Longitudinal strain and strain rates were computed both on the free wall and on the whole RV. No correlation was found between the echocardiographic indices and the MRI EF in our Fallot population. The accuracy of those indices as a diagnostic test of an altered RV was low with Younden's indices varying from -0.18 to 0.5 and areas under the Receiver Operating Characterictic (ROC) curves equal to 0.54 for tricuspid annulus plane systolic excursion, 0.59-0.62 for strain and 0.57-0.63 for strain rate. Three conventional echocardiographic indices based on RV longitudinal contraction failed to assess the EF in our population of post-operative tetralogy of Fallot patients.

  8. Combined radiodiagnosis of pulmonary artery coarctation in Fallot's tetrad

    International Nuclear Information System (INIS)

    Dynnik, I.B.; Shalenkov, V.P.

    1986-01-01

    The paper is concerned with a comparative analysis of the results of routine X-ray examination and those of intubation and angiocardiography in 36 patients with Fallot's tetrad and pulmonary artery coarctation (PAC). The chief method of PAC diagnosis was selective angiocardiography from the right ventricular orifice. The authors presented angiocardiographic semiotics of the PAC main classes according to modern morphological classification which could be of practical value in a cardiosurgical hospital for the planning of operations. In some forms of PAC, transmission chest roentgenograms revealed pathognomonic roentgenologic signs permitting a preliminary diagnosis with a subsequent roentgenosurgical investigation

  9. Effect of ventricular function and volumes on exercise capacity in adults with repaired Tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Natalia Dłużniewska

    2018-01-01

    Conclusion: Exercise intolerance in adults with repaired ToF is markedly depressed. The decreased exercise capacity is correlated with impaired RV function and may be associated also with LV dysfunction, which suggests right-to-left ventricular interaction.

  10. Myocardial visualization on a routine perfusion lung scintigram: Relationship to the amount of right-to-left shunt

    International Nuclear Information System (INIS)

    Seto, H.; Futatsuya, R.; Kamei, T.; Kakishita, M.; Hisada, K.

    1983-01-01

    Three cases of myocardial visualization on a routine perfusion lung scintigram with sup(99m)Tc-macroaggregaed albumin were reported in patients with congenital heart diseases; two cases of tetralogy of Fallot and one case of truncus arteriosus type IV. Large right-to-left shunts greater than 39% and marked hypertrophy of the ventricle suggesting the presence of increased coronary blood flow were noted in all cases. In the two patients with tetralogy of Fallot myocardial activity appeared to be located in the hypertrophic right venticles. (orig.)

  11. Myocardial visualization on a routine perfusion lung scintigram: Relationship to the amount of right-to-left shunt

    Energy Technology Data Exchange (ETDEWEB)

    Seto, H.; Futatsuya, R.; Kamei, T.; Kakishita, M.; Hisada, K.

    1983-11-14

    Three cases of myocardial visualization on a routine perfusion lung scintigram with sup(99m)Tc-macroaggregated albumin were reported in patients with congenital heart diseases; two cases of tetralogy of Fallot and one case of truncus arteriosus type IV. Large right-to-left shunts greater than 39% and marked hypertrophy of the ventricle suggesting the presence of increased coronary blood flow were noted in all cases. In the two patients with tetralogy of Fallot myocardial activity appeared to be located in the hypertrophic right venticles.

  12. Application of digital subtraction angiography in disease of large cardiac vessel

    Energy Technology Data Exchange (ETDEWEB)

    Arisawa, Jun; Sone, Shusuke; Morimoto, Shizuo; Ikezoe, Junpei; Higashibara, Tokuro; Hanayama, Masayuki

    1983-06-01

    Digital subtraction angiography (DSA) was performed in 31 cases of disease of large cardiac vessel. DSA was useful for the diagnosis of aortic aneurysm and malformation of large vessels, follow-up after A-C bypass operation and Blalock's shunt operation for tetralogy of Fallot and as an adjuvant modality in cardiac catheterization.

  13. Application of digital subtraction angiography in disease of large cardiac vessel

    International Nuclear Information System (INIS)

    Arisawa, Jun; Sone, Shusuke; Morimoto, Shizuo; Ikezoe, Junpei; Higashibara, Tokuro; Hanayama, Masayuki

    1983-01-01

    Digital subtraction angiography (DSA) was performed in 31 cases of disease of large cardiac vessel. DSA was useful for the diagnosis of aortic aneurysm and malformation of large vessels, follow-up after A-C bypass operation and Blalock's shunt operation for tetralogy of Fallot and as an adjuvant modality in cardiac catheterization. (Chiba, N.)

  14. Diverticulum of the brachiocephalic trunk - angiography and embryological explanation

    Energy Technology Data Exchange (ETDEWEB)

    Pfefferkorn, J.R.; Lunkenheimer, A.; Loeser, H.; Hilgenberg, F.

    1983-03-01

    A diverticulum of the brachiocephalic trunk is described in 5 children with unilateral absence of the pulmonary artery, in a child with tetralogy of Fallot, and in an other child with Bland-White-Garland syndrome. The diverticulum is a remnant of a contralateral ductus arteriosus, which closed after birth and represents the origin of the distal part of the sixth aortic arch.

  15. Recanalization of an occluded left pulmonary artery: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ali Ibrahim Elarabi

    2017-01-01

    Full Text Available We report an 8-year-old male child with tetralogy of Fallot (TOF, who developed left pulmonary artery (LPA atresia, following surgical repair of TOF and left pulmonary arterioplasty at the age of 6 years. He underwent successful radiofrequency recanalization and stenting of the LPA. The LPA exhibited satisfactory growth for 3 months, following recanalization and stenting.

  16. Two cases of Pentalogy of Cantrell diagnosed antenatally at Korle ...

    African Journals Online (AJOL)

    There was no diaphragm over the liver but a posterior diaphragm over the kidneys. The liver was grossly enlarged with a groove on the upper surface accommodating the heart. The heart was located in the abdominal wall defect, lying freely on top of the liver (Figure 4). Further dissection revealed a Tetralogy of Fallot.

  17. Permanent complete heart block following surgical correction of ...

    African Journals Online (AJOL)

    Conclusion: Permanent post-operative complete heart block occurred in 1.3% of patients undergoing VSD repair and 5.5% of those undergoing repair of conotruncal anomalies (Fallot's tetralogy). The dominant anatomic risk factor was a large perimembranous VSD as an isolated defect or as part of a conotruncal anomaly.

  18. CT demonstration of chicken trachea resulting from complete cartilaginous rings of the trachea in ring-sling complex

    International Nuclear Information System (INIS)

    Calcagni, Giulio; Bonnet, Damien; Sidi, Daniel; Brunelle, Francis; Vouhe, Pascal; Ou, Phalla

    2008-01-01

    We report a 10-month-old infant who presented with tetralogy of Fallot and respiratory disease in whom the suspicion of a ring-sling complex was confirmed by high-resolution CT. CT demonstrated the typical association of left pulmonary artery sling and the ''chicken trachea'' resulting from complete cartilaginous rings of the trachea. (orig.)

  19. CT demonstration of chicken trachea resulting from complete cartilaginous rings of the trachea in ring-sling complex

    Energy Technology Data Exchange (ETDEWEB)

    Calcagni, Giulio; Bonnet, Damien; Sidi, Daniel [University Paris Descartes, Department of Paediatric Cardiology, Hopital Necker-Enfants Malades, AP-HP, Paris (France); Brunelle, Francis [University Paris Descartes, Department of Paediatric Radiology, Hopital Necker-Enfants Malades, AP-HP, Paris Cedex 15 (France); Vouhe, Pascal [University Paris Descartes, Department of Paediatric Cardiovascular Surgery, Hopital Necker-Enfants Malades, AP-HP, Paris (France); Ou, Phalla [University Paris Descartes, Department of Paediatric Cardiology, Hopital Necker-Enfants Malades, AP-HP, Paris (France); University Paris Descartes, Department of Paediatric Radiology, Hopital Necker-Enfants Malades, AP-HP, Paris Cedex 15 (France)

    2008-07-15

    We report a 10-month-old infant who presented with tetralogy of Fallot and respiratory disease in whom the suspicion of a ring-sling complex was confirmed by high-resolution CT. CT demonstrated the typical association of left pulmonary artery sling and the ''chicken trachea'' resulting from complete cartilaginous rings of the trachea. (orig.)

  20. High CpG island methylation ofp16 gene and loss of p16 protein ...

    Indian Academy of Sciences (India)

    Navya

    :Tetralogy of Fallot;p16 gene;p16 protein;CpG islands;Methylation;Promoter regions ... of congenital heart disease, as well as the exclusion of previous history of ..... malignant progression of oral epithelial dysplasia: a prospective cohort study.

  1. High prevalence rate of left superior vena cava determined by ...

    African Journals Online (AJOL)

    2013-10-08

    Oct 8, 2013 ... persistent LSVC (6). There was a similar high prevalence, in the current study, of persistent LSVC in patients with tetralogy of. Fallot and coarctation of the aorta when compared to other studies (6, 8). This can be explained by the fact that the patient sample in the current study with these lesions was small.

  2. Pattern and clinical profile of children with complex cardiac anomaly ...

    African Journals Online (AJOL)

    ... DORV (double outlet right ventricle) with left sided aorta, hypoplastic tricuspid valve with a PDA (patent ductus artriosus), TOF (tetralogy of fallot), prolapse of aortic valve, and pulmonary regurgitation. One of these complex cardiac anomalies presented with Turner's syndrome and another with VACTERAL association.

  3. Parental mental health moderates the efficacy of exercise training on health-related quality of life in adolescents with congenital heart disease

    NARCIS (Netherlands)

    Dulfer, Karolijn; Duppen, Nienke; van Dijk, Arie P. J.; Kuipers, Irene M.; van Domburg, Ron T.; Verhulst, Frank C.; van der Ende, Jan; Helbing, Willem A.; Utens, Elisabeth M. W. J.

    2015-01-01

    To evaluate the moderating influence of parental variables on changes in health-related quality of life (HRQoL) in adolescents with Tetralogy of Fallot (ToF) or a Fontan circulation after participation in standardized exercise training. A multicenter randomized controlled trail in which 56 patients,

  4. Abnormalities of intestinal rotation and congenital intrinsic duodenal ...

    African Journals Online (AJOL)

    ultrasound. Echocardiography was normal in one patient, and in the other, there were features of Fallot's tetralogy. Intraoperatively, one had duodenal atresia with a gap, whereas the other had a duodenal diaphragm (Fig. 2). In the remaining seven patients, the diagnosis of associated malrotation was made intraoperatively.

  5. Spectrum of congenital heart diseases in children with Down

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    septal defect (ASD) while one (3.1%) patient each had isolated ASD and Fallot's tetralogy respectively. Only three (9.4%) patients had surgical closure abroad, with good postoperative outcome in two of the patients. Conclusion: AVSD is the commonest CHD in our series, which is similar to previous reports. Increased.

  6. Practical application of natriuretic peptides in paediatric cardiology

    DEFF Research Database (Denmark)

    Smith, Julie; Goetze, Jens P; Andersen, Claus B

    2010-01-01

    conclude that in premature neonates with persistent arterial ducts; in teenagers with tetralogy of Fallot and pulmonary regurgitation; and in children with heart transplants and potential allograft rejection cardiac peptides can provide the clinician with additional information, but in children with atrial...

  7. Congenital Heart Diseases in Adults: A Review of Echocardiogram ...

    African Journals Online (AJOL)

    The most common congenital anomalies were ventricular septal defects (VSD) ‑ 31.3%, (36/115), atrial septal defects ‑ 28.7% (33/115) and tetralogy of fallot ‑ 10.4% (12/115). Conclusion: VSD are the most common congenital heart diseases in adults presenting for echocardiographic examination in Enugu, Nigeria.

  8. VANISHING CALCIFICATION OF THE BRAIN IN AN INFANT AFTER OPEN-HEART-SURGERY

    NARCIS (Netherlands)

    BEGEER, JH; RUTGERS, AWF; VENCKEN, LM; HOORNTJE, TM; MEUZELAAR, JJ; WOLTERSOMZWIERZYNSKA, BD

    1991-01-01

    Neurological complications after cardiac operations with the aid of cardiopulmonary bypass and hypothermia are well known. A 6 months-old child is described with severe neurological complications after cardiac surgery for Fallots tetralogy. On the CT scan cortical calcification was seen to vanish.

  9. An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

    Science.gov (United States)

    Schwartz, Matthew C; Felix, Donald; Iacono, Karen; Nykanen, David

    2018-04-16

    During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty. © 2018 Wiley Periodicals, Inc.

  10. Anaesthetic management of craniotomy for intracranial lesion in a child with uncorrected Tetrology of Fallot

    International Nuclear Information System (INIS)

    Rafique, N. B.; Hamid, M.

    2013-01-01

    The case of a 16 years old female with uncorrected Tetrology of Fallot, who operated for intracranial lesion in parieto frontal area with midline shift is presented. She had right ventricular hypertrophy, clubbing, central and peripheral cyanosis. Patient was anaesthetized keeping all measures required to avoid haemodynamic swings, tachycardia, desaturation, acidosis and dehydration. Pre-operative antibiotic cover was given to prevent bacterial endocarditis. Neurosurgeon, Paediatric cardiologist, Anaesthesiologist and Intensivist were involved in the preoperative planning and management of the patient. Haemodynamics were maintained and managed by monitoring continuous arterial line secured pre-induction and central line after induction. During surgery pain was controlled with fentanyl boluses intra-operatively and post operatively by tramadol infusion. Patient was extubated post operatively in the recovery room fulfilling the extubation criteria. She remained haemodynamically stable throughout the course. She was discharged on 5th post operative day from the hospital on SpO/sub 2/ of 70-80% at room air. (author)

  11. The Anti-Racist Overtones of a Feminist Historical Novel Tetralogy from the 1940s

    Directory of Open Access Journals (Sweden)

    Judit Kádár

    2011-01-01

    Full Text Available Although the most popular Hungarian historical novels were written in the nineteenth century by the famous romantic writer, Mór Jókai, a revival of the genre occurred in the period following the First World War. Most of the authors, each influenced by a different worldview, were scouring the symbolic space of history for an explanation as to why Hungary had lost the war. “Our knowledge of the past, our cultural heritage is also a symbolic space that is the site of struggle for the self-representation of social groups, a space that is shaped according to the degrees to which certain groups have access to it” – states Györgyi Horváth in her work on the constitutive role of the historical narrative; this, of course, is also true of authors in the Post World War I Era. They represented their own social groups, which happened to be white, middle class, Hungarian men. Although the period between the two World Wars saw the rise of female authorship, and dozens of historical novels were published by women each year, almost all women writers conceived their novels from a dominant masculine perspective. In this paper, I examine one of the few exceptions, a tetralogy of historical novels by Lola Kosáryné Réz, written from the perspective of oppressed women, and I discuss her stance on the relationship between different ethnicities in discourses of war and responsibility.

  12. Acute-on-chronic liver failure due to thiamazole in a patient with hyperthyroidism and trilogy of Fallot: case report

    Directory of Open Access Journals (Sweden)

    Shen Chuan

    2010-08-01

    Full Text Available Abstract Background Thiamazole is a widely used antithyroid agent that has been approved for the treatment of hyperthyroidism. Although thiamazole-induced hepatotoxicity is a main side effect, it may progress to liver failure in a very few cases. Case Presentation We described a 24-year-old patient with hyperthyroidism and trilogy of Fallot, who developed liver failure due to thiamazole. Liver biopsy showed intrahepatic cholestasis, mild inflammatory infiltrates, as well as significant fibrosis, indicating both acute and chronic liver injuries. Although a series of potent therapies were given, the patient deceased due to severe liver decompensation. Conclusions This case suggests that thiamazole-induced hepatotoxicity in the setting of advanced fibrosis increases the risk of poor outcome. Regular liver function monitoring during thiamazole therapy is therefore important.

  13. Acute-on-chronic liver failure due to thiamazole in a patient with hyperthyroidism and trilogy of Fallot: case report

    Science.gov (United States)

    2010-01-01

    Background Thiamazole is a widely used antithyroid agent that has been approved for the treatment of hyperthyroidism. Although thiamazole-induced hepatotoxicity is a main side effect, it may progress to liver failure in a very few cases. Case Presentation We described a 24-year-old patient with hyperthyroidism and trilogy of Fallot, who developed liver failure due to thiamazole. Liver biopsy showed intrahepatic cholestasis, mild inflammatory infiltrates, as well as significant fibrosis, indicating both acute and chronic liver injuries. Although a series of potent therapies were given, the patient deceased due to severe liver decompensation. Conclusions This case suggests that thiamazole-induced hepatotoxicity in the setting of advanced fibrosis increases the risk of poor outcome. Regular liver function monitoring during thiamazole therapy is therefore important. PMID:20707932

  14. A diverticulum of the brachiocephalic trunk - angiography and embryological explanation

    International Nuclear Information System (INIS)

    Pfefferkorn, J.R.; Lunkenheimer, A.; Loeser, H.; Hilgenberg, F.

    1983-01-01

    A diverticulum of the brachiocephalic trunk is described in 5 children with unilateral absence of the pulmonary artery, in a child with tetralogy of Fallot, and in an other child with Bland-White-Garland syndrome. The diverticulum is a remnant of a contralateral ductus arteriosus, which closed after birth and represents the origin of the distal part of the sixth aortic arch. (orig.) [de

  15. Application of nuclear medicine to heart diseases in childhood

    International Nuclear Information System (INIS)

    Tsuji, Atsutoshi; Osano, Mitsuru.

    1981-01-01

    Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, 201 Thallium, and 133 Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and 201 Thallium myocardial imaging. (Kondo, M.)

  16. Application of nuclear medicine to heart diseases in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Tsuji, A. (Yamanashi Medical Coll., Tamaho, Kofu (Japan)); Osano, M.

    1981-06-01

    Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, /sup 201/Thallium, and /sup 133/Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and /sup 201/Thallium myocardial imaging.

  17. Vanishing calcification of the brain in an infant after open heart surgery

    International Nuclear Information System (INIS)

    Begeer, J.H.; Rutgers, A.W.F.; Vencken, L.M.; Hoorntje, T.M.; Meuzelaar, J.J.; Woltersom-Zwierzynska, B.D.

    1991-01-01

    Neurological complications after cardiac operations with the aid of cardiopulmonary bypass and hypothermia are well known. A 6 months-old child is described with severe neurological complications after cardiac surgery for Fallots tetralogy. On the CT scan cortical calcification was seen to vanish. Such calcification has not been reported in similar patients. Possible causes are discussed but the precise pathophysiology of this phenomenon remains unclear. (orig.)

  18. Vanishing calcification of the brain in an infant after open heart surgery

    Energy Technology Data Exchange (ETDEWEB)

    Begeer, J.H.; Rutgers, A.W.F. (Groningen Univ. Hospital (Netherlands). Dept. of Child Neurology); Vencken, L.M. (Groningen Univ. Hospital (Netherlands). Dept. of Neuroradiology); Hoorntje, T.M. (Groningen Univ. Hospital (Netherlands). Dept. of Pediatrics); Meuzelaar, J.J. (Groningen Univ. Hospital (Netherlands). Dept. of Thoracic Surgery); Woltersom-Zwierzynska, B.D. (Groningen Univ. Hospital (Netherlands). Dept. of Anaesthesia)

    1991-08-01

    Neurological complications after cardiac operations with the aid of cardiopulmonary bypass and hypothermia are well known. A 6 months-old child is described with severe neurological complications after cardiac surgery for Fallots tetralogy. On the CT scan cortical calcification was seen to vanish. Such calcification has not been reported in similar patients. Possible causes are discussed but the precise pathophysiology of this phenomenon remains unclear. (orig.).

  19. Genetics Home Reference: Alagille syndrome

    Science.gov (United States)

    ... abnormalities. This combination of heart defects is called tetralogy of Fallot . People with Alagille syndrome may have distinctive facial ... the disorder, such as a heart defect like tetralogy of Fallot, or a characteristic facial appearance. These individuals do ...

  20. Spontaneous expectoration of a Blalock-Taussig shunt a decade after operation

    Directory of Open Access Journals (Sweden)

    Shyam S Kothari

    2015-01-01

    Full Text Available An eleven-year-old boy expectorated a foreign body in cough that was identified as the prosthetic graft used for a Blalock-Taussig shunt. The shunt procedure was done 10 years earlier, and a definitive repair for tetralogy of Fallot was done a year later. He had no other symptoms, and a computed tomography (CT angiogram did not reveal any other significant anomaly. The reason for this extremely rare event is unclear.

  1. Correção total da tétrade de Fallot no primeiro ano de vida

    Directory of Open Access Journals (Sweden)

    Fernando MORAES NETO

    1998-01-01

    Full Text Available De janeiro de 1996 a novembro de 1997, 15 crianças com idade variando de 3 a 11 meses (média: 6 meses e pesando entre 5 kg a 9 kg (média: 7,2 kg foram eletivamente submetidas à correção total de tétrade de Fallot. Treze tinham sintomas de hipoxemia, e 2 eram acianóticos. O diagnóstico definitivo foi obtido em todos os casos por ecocardiografia bidimensional. Utilizou-se circulação extracorpórea convencional e hipotermia moderada. Obteve-se proteção miocárdica com infusão na aorta de solução cardioplégica cristalóide gelada e hipotermia tópica do coração. O tempo de CEC variou de 50 min a 125 min (média: 56 min e o de pinçamento aórtico de 32 min a 86 min (média: 56 min. A correção foi realizada por via transventricular em 14 e por via transatrial em 1. Em 11 casos, utilizou-se enxerto de pericárdio bovino para ampliar a via de saída do ventrículo direito, sendo que em 4 a ampliação foi transanular. Após a correção, o gradiente entre o ventrículo direito e a artéria pulmonar variou de 2 a 25 mmHg (média: 12 mmHg. Não ocorreram óbitos ou complicações significativas nesta série. Conclui-se que a correção total da tétrade de Fallot no primeiro ano de vida pode ser realizada com baixa mortalidade, podendo essa conduta ter vantagens sobre a correção em dois tempos.From January 1996, to November 1997, 15 consecutive infants ranging in age from 3 to 11 months (mean: 6 months and weigh ing from 5 to 9 kilograms (mean: 7.2 kilograms underwent elective total repair of tetralogy of Fallot. Thirteen had symptoms of hypoxemia and two were acyanotic. Definitive diagnosis was established in all cases by two-dimensional echocardiography. Intracardiac correction was accomplished with conventional cardiopulmonary bypass and moderate hypothermia. Myocardial protection was obtained by cold cristaloide cardioplegia infused into the aorta and topical hypothermia of the heart. Bypass time ranged from 50 to 125 minutes

  2. Tetralogia de Fallot no Adulto

    OpenAIRE

    Louro, F; Gomes, A; Almeida, J; Abreu, J; Agapito, A; Magalhães, M; Morgado, AV

    1999-01-01

    Os autores apresentam um caso clínico de tetralogia de Fallot num homem de 47 anos, com quadro clínico atípico desde a infância, sujeito a cirurgia correctiva com sucesso. Discutem-se o timing cirúrgico e as complicações pós-operatórias.

  3. Case report: cytogenetic and molecular analysis of proximal interstitial deletion of 4p, review of the literature and comparison with wolf-hirschhorn syndrome.

    Science.gov (United States)

    Bailey, Nathanael G; South, Sarah T; Hummel, Marybeth; Wenger, Sharon L

    2010-01-01

    We report on a two-year-old female with a de novo proximal interstitial deletion of the short arm of chromosome 4 and a tetralogy of Fallot malformation. The patient had a karyotype of 46,XX,del(4)(p14p15.33) that was further characterized by array comparative genomic hybridization (aCGH). Phenotypic abnormalities for our patient are compared with those of previously reported patients with similar proximal 4p deletions as well as more distal deletions. The functions of genes that are deleted within this segment are reviewed.

  4. Significance of MR imaging in congenital heart disease

    International Nuclear Information System (INIS)

    Mayr, H.; Globits, S.; Frank, H.; Glogar, D.; Nouhold, A.; Imhof, H.

    1989-01-01

    To determine the diagnostic impact of MR imaging in congenital heart disease, the authors used a 0.5- or 1.5-T magnet to examine 85 patients. Multisection spin-echo images were obtained in three planes. Diagnoses included atrial septal defect, trilogy and tetralogy of Fallot, ventricular septal defect, transposition (seven), single ventricle, and other complex disorders. Compared with other noninvasive techniques, MR imaging allowed a much better visualization of anatomic structures and the relationship of great vessels to shunt lesions in complex congenital heart disease. In 53 (63%) of 85 patients, MR imaging made a major contribution to establishing or modifying diagnoses

  5. Diagnosis of common congenital heart anomalies in the dog using survey and nonselective contrast radiography

    International Nuclear Information System (INIS)

    Stickle, R.L.; Anderson, L.K.

    1987-01-01

    The most common canine congenital heart anomalies include patient ductus arteriosus, ventricular septal defects, tetralogy of Fallot, pulmonic stenosis, and aortic stenosis. Survey radiography and nonselective (venous) angiography can allow the practicing veterinarian to confirm the diagnosis in many of these patients. Typical radiographic findings using these diagnostic procedures are reviewed. Nonselective angiocardiography is a relatively easy, rapid, and noninvasive procedure which can be performed using conventional equipment. The major disadvantage of this special procedure is that the superimposition of opacified structures can make the identification of some left-to-right shunts difficult. Dilution of contrast medium can occur if a rapid bolus injection is not made

  6. Pulmonary atresia with intact ventricular septum and agenesis of the ductus arteriosus in a pup

    International Nuclear Information System (INIS)

    Brown, D.J.; Patterson, D.F.

    1989-01-01

    A 7-week-old Wire Fox Terrier was admitted with pulmonary atresia with intact ventricular septum. The right ventricle and tricuspid valve were hypoplastic, and venous return to the right atrium reached the left side through an atrial septal defect. Oxygenation was via hyperplastic bronchial arteries. There was no evidence of the ductus arteriosus. Physical examination, plain and contrast radiography, and electrocardiography were performed. Clinical findings for this combination of defects were similar to those of more common defects (tetralogy of Fallot, patent ductus arteriosus

  7. [Surgical therapy of life-threatening tachycardic cardiac arrhythmias in children].

    Science.gov (United States)

    Frank, G; Schmid, C; Baumgart, D; Lowes, D; Klein, H; Kallfelz, H C

    1989-05-01

    Surgical techniques for tachyarrhythmias refractory to medical treatment are used with increasing frequency. Among 211 patients undergoing antiarrhythmic surgery 10 children (2 to 14 years old) were operated by electrophysiologically directed procedures. 7 patients suffered from WPW syndrome, 2 from focal atrial tachycardias and 1 from recurrent ventricular tachycardia following the repair of Fallot's tetralogy. In all cases preoperative electrophysiologic study and intraoperative mapping preceded operative ablation. Surgical treatment consisted of interruption of the bundle of Kent (3 right-sided, 2 left-sided, 3 septal), ablation of the atrial focus (1 right-sided, 1 left-sided) and right ventricular outflow tract incision. In 7 operations cryo-techniques were added. 2 children with WPW syndrome had two interventions because of tachycardia recurrences due to multiple accessory pathways. In 1 case a VVI-pacemaker was implanted postoperatively due to complete atrioventricular block. Another 2 children with prolonged postoperative bradycardia received a pacemaker prophylactically. Only the child with previous tetralogy of Fallot is still under antiarrhythmic medication while all other children are free of tachycardiac episodes. Our data confirm the efficacy of surgical treatment of tachyarrhythmias in children thereby abolishing the need for life-long antiarrhythmic medication.

  8. Experience with intravenous digital subtraction angiography following shunting in 4 cases of congenital heart diseases

    Energy Technology Data Exchange (ETDEWEB)

    Higashidate, Masafumi; Konno, Susumu; Sumitomo, Naokata; Ito, Sango; Kobayashi, Hiroshi

    1985-01-01

    Intravenous digital subtraction angiography (IV-DSA) was performed in two patients with Fallot's tetralogy who underwent Blalock-Taussing operation, a patient with coarctation of the aortic arch who underwent extra-anastomic bypass between the ascending aorta and the descending aorta, and a patient with stenosis of the tricuspid valve who underwent Fontan type operation (anastomosis between the right atrium and the pulmonary artery). The conventional cardiac angiography was also performed for the comparison with IV-DSA in all patients. IV-DSA revealed the thickness of the subclavian artery anastomosed by Blaloch-Taussing operation, but failed to determine the accurate thickness of the pulmonary artery required for PA-index for radical surgery in cases of Fallot's tetralogy. Bypass from the ascending aorta to the descending aorta was well visualized by IV-DSA because the heart did not overlap on the image. In cases of Fontan type operation, IV-DSA permitted the visualization of blood flow from the right atrium to the pulmonary artery and the shape of the pulmonary artery. It is concluded that IV-DSA is a less invasive, simple technique to evaluate patients' condition following shunting for congenital heart diseases, as compared to the conventional method. (Namekawa, K.).

  9. Experience with intravenous digital subtraction angiography following shunting in 4 cases of congenital heart diseases

    International Nuclear Information System (INIS)

    Higashidate, Masafumi; Konno, Susumu; Sumitomo, Naokata; Ito, Sango; Kobayashi, Hiroshi

    1985-01-01

    Intravenous digital subtraction angiography (IV-DSA) was performed in two patients with Fallot's tetralogy who underwent Blalock-Taussing operation, a patient with coarctation of the aortic arch who underwent extra-anastomic bypass between the ascending aorta and the descending aorta, and a patient with stenosis of the tricuspid valve who underwent Fontan type operation (anastomosis between the right atrium and the pulmonary artery). The conventional cardiac angiography was also performed for the comparison with IV-DSA in all patients. IV-DSA revealed the thickness of the subclavian artery anastomosed by Blaloch-Taussing operation, but failed to determine the accurate thickness of the pulmonary artery required for PA-index for radical surgery in cases of Fallot's tetralogy. Bypass from the ascending aorta to the descending aorta was well visualized by IV-DSA because the heart did not overlap on the image. In cases of Fontan type operation, IV-DSA permitted the visualization of blood flow from the right atrium to the pulmonary artery and the shape of the pulmonary artery. It is concluded that IV-DSA is a less invasive, simple technique to evaluate patients' condition following shunting for congenital heart diseases, as compared to the conventional method. (Namekawa, K.)

  10. Systemic Arterial-to-Pulmonary Artery Shunt Utilization

    African Journals Online (AJOL)

    multiruka1

    In certain circumstances, such as cyanotic neonates with tetralogy of Fallot (4) or cyanotic patients with. Tetralogy of Fallot and hypoplastic pulmonary arteries. (5), better outcomes are obtained if definitive surgery. (total correction or palliation) is preceded by creation of a systemic arterial-to-pulmonary artery shunt (SAPAS).

  11. Genetics Home Reference: 1q21.1 microduplication

    Science.gov (United States)

    ... a particular combination of heart defects known as tetralogy of Fallot . Less commonly, other physical malformations such as the ... times more common in people with schizophrenia or tetralogy of Fallot. Many people with 1q21.1 microduplications are likely ...

  12. Assessment of right ventricular function and anatomy using peripheral vein infusion of krypton 81m

    Energy Technology Data Exchange (ETDEWEB)

    Sugrue, D.D.; Kamal, S.; Deanfield, J.E.; McKenna, W.J.; Myers, M.J.; Watson, I.A.; Oakley, C.M.; Lavender, J.P. (Royal Postgraduate Medical School, London (UK))

    1983-09-01

    A method for imaging the right side of the heart (atrium, ventricle and main pulmonary artery) and for assessment of RV systolic function (ejection fraction and ejection rate) is described. An ultra-short-lived isotope (/sup 81/Krsup(m)) is continuously eluted in 5% dextrose and infused into an ante-cubital arm vein; standard multigated images are acquired using a gamma camera and commercially available software. Preliminary evaluation of the method in 55 subjects (20 with repaired tetralogy of Fallot, 14 with dilated cardiomyopathy and 21 normal volunteers) showed that the technical success rate was 100%; that RV boundaries free from LV overlap can be clearly visualised due to efficient exhalation of /sup 81/Krsup(m) through the lungs and that /sup 81/Krsup(m) measurements of RVEF are reproducible. The technique offers considerable potential for serial non-invasive assessment of RV function.

  13. Assessment of right ventricular function and anatomy using peripheral vein infusion of krypton 81m

    International Nuclear Information System (INIS)

    Sugrue, D.D.; Kamal, S.; Deanfield, J.E.; McKenna, W.J.; Myers, M.J.; Watson, I.A.; Oakley, C.M.; Lavender, J.P.

    1983-01-01

    A method for imaging the right side of the heart (atrium, ventricle and main pulmonary artery) and for assessment of RV systolic function (ejection fraction and ejection rate) is described. An ultra-short-lived isotope ( 81 Krsup(m)) is continuously eluted in 5% dextrose and infused into an ante-cubital arm vein; standard multigated images are acquired using a gamma camera and commercially available software. Preliminary evaluation of the method in 55 subjects (20 with repaired tetralogy of Fallot, 14 with dilated cardiomyopathy and 21 normal volunteers) showed that the technical success rate was 100%; that RV boundaries free from LV overlap can be clearly visualised due to efficient exhalation of 81 Krsup(m) through the lungs and that 81 Krsup(m) measurements of RVEF are reproducible. The technique offers considerable potential for serial non-invasive assessment of RV function. (author)

  14. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch

    Directory of Open Access Journals (Sweden)

    Trilok Chand

    2017-01-01

    Full Text Available The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

  15. In utero premature closure of the ductus arteriosus presenting as isolated right ventricular hypertrophy.

    Science.gov (United States)

    Long, Webb E; Wilson, Allen D; Srinivasan, Shardha; Seeger, Kimberly J; Maginot, Kathleen R

    2009-10-01

    The etiology of isolated right ventricular hypertrophy (RVH) is distinct from other forms of hypertrophic cardiomyopathy. RVH is typically seen in the setting of pulmonary valve stenosis or Tetralogy of Fallot. A rare cause of isolated RVH is premature closure of the patent ductus arteriosus (PDA) in utero that results in pulmonary hypertension. This can have a range of outcomes, from spontaneous resolution to fetal demise. This case report describes a term infant who presented with respiratory distress and striking isolated RVH, pulmonary hypertension, and no PDA. She was treated conservatively with supplemental oxygen. The patient was gradually weaned off oxygen over the course of two weeks and follow-up echocardiography showed resolution of her RVH and pulmonary hypertension by 14 weeks of age. The presentation and course of this patient with severe isolated RVH is consistent with spontaneous premature closure of the ductus arteriosus in utero.

  16. Value of cardiac catheterization and cineangiography in infantile lobar emphysema

    Energy Technology Data Exchange (ETDEWEB)

    Roguin, N.; Peleg, H.; Naveh, Y.; Riss, E.

    1980-01-01

    Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.

  17. Three-dimensional MR imaging of congenital heart disease

    International Nuclear Information System (INIS)

    Laschinger, J.C.; Vannier, M.W.; Knapp, R.H.; Gutierrez, F.R.; Cox, J.L.

    1987-01-01

    Contiguous 5-mm thick ECG-gated MR images of the thorax were edited using surface reconstruction techniques to produce three-dimensional (3D) images of the heart and great vessels in four healthy individuals and 25 patients with congenital heart disease (aged 3 months-30 years). Anomalies studied include atrial and ventricular septal defects, aortic coarctation, AV canal defects, double outlet ventricles, hypoplastic left heart syndrome, and a wide spectrum of patients with tetralogy of Fallot. The results were correlated with echocardiographic and cineradiographic studies, and with surgical findings or pathologic specimens. Three-dimensional reconstructions accurately localized the dimensions and locations of all cardiac and great vessel anomalies and often displayed anatomic findings not diagnosed or visualized with other forms of diagnostic imaging

  18. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

    International Nuclear Information System (INIS)

    Sondheimer, H.M.; Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-01-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease

  19. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of); Choi, Sang Il; Chun, Eun Ju [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of); Choi, Sung Hun [Ulsan University Hospital, Ulsan (Korea, Republic of); Park, Jae Hyung [Seoul National University Hospital, Seoul (Korea, Republic of)

    2010-10-15

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging.

  20. The Cardiac MR Images and Causes of Paradoxical Septal Motion

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Sang Il; Chun, Eun Ju; Choi, Sung Hun; Park, Jae Hyung

    2010-01-01

    Real-time cine MRI studies using the steady-state free precession (SSFP) technique are very useful for evaluating cardiac and septal motion. During diastole, the septum acts as a compliant membrane between the two ventricles, and its position and geometry respond to even small alterations in the trans-septal pressure gradients. Abnormal septal motion can be caused by an overload of the right ventricle, delayed ventricular filling and abnormal conduction. In this study, we illustrate, based on our experiences, the causes of abnormal septal motion such as corrective surgery for tetralogy of Fallot, an atrial septal defect, pulmonary thromboembolism, mitral stenosis, constrictive pericarditis and left bundle branch block. In addition, we discuss the significance of paradoxical septal motion in the context of cardiac MR imaging

  1. The value of cardiac catheterization and cineangiography in infantile lobar emphysema

    International Nuclear Information System (INIS)

    Roguin, N.; Peleg, H.; Naveh, Y.; Riss, E.; Technion-Israel Inst. of Tech., Haifa. Cardiothoraic Surgery; Technion-Israel Inst. of Tech., Haifa. Dept. of Pediatrics)

    1980-01-01

    Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants. (orig.) [de

  2. A Case of Re-Expansion Pulmonary Edema after Rapid Pleural Evacuation

    Directory of Open Access Journals (Sweden)

    SH Shahbazi

    2007-07-01

    Full Text Available Introduction & Objective: Pulmonary edema after chest tube insertion is a rare complication and is associated with high mortality. The cause of this phenomenon is not clear, although causes such as decrease in surfactant and inflammatory process have been defined. Early diagnosis and treatment decrease the mortality. This study introduces a case of re-expansion pulmonary edema after rapid pleural evacuation. Case: The case is a 4.5 y/o boy, a case of Tetralogy of Fallot, who developed respiratory distress after surgery (Total Correction in ICU of Namazi Hospital in 1385. Chest X ray showed pneumothorax of left lung. For the patient, chest tube was inserted and the symptoms improved. After few hours the patient developed tachypnea, tachycardia, and CXR showed pulmonary edema of left lung. Appropriate treatment was done for the patient and his condition improved. Conclusion: Pulmonary edema after sudden evacuation of pleura is a rare phenomenon and early diagnosis decreases the mortality.

  3. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

    Energy Technology Data Exchange (ETDEWEB)

    Sondheimer, H.M. (Upstate Medical Center, Syracuse, NY); Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-05-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease.

  4. Three-dimensional surface reconstruction imaging for evaluation of congenital heart disease from ECG-triggered MR images

    International Nuclear Information System (INIS)

    Vannier, M.W.; Laschinger, J.; Knapp, R.H.; Gutierrez, F.R.; Gronnemeyer, S.A.

    1987-01-01

    Three-dimensional surface reconstruction images of the heart and great vessels were produced from contiguous sequences of electrocardiographically triggered MR images in 25 patients with congenital heart disease and in three healthy subjects. The imaging data were semiautomatically processed to separate the epicardial and endocardial surfaces and to define the outline of the enclosed blood volumes on a section by section basis. Images were obtained at 5-mm intervals in patients aged 3 months to 30 years with anomalies of the great vessels, tetralogy of Fallot, septal defects, pulmonary atresia, and other congenital heart malformations. The results were used to facilitate the surgical treatment of these patients and were compared with echocardiographic and cineradiographic studies, and with surgical findings or pathologic specimens. These surface reconstruction images were useful for communicating the results of diagnostic examinations to cardiac surgeons, for sizing and location of intracardiac defects, for imaging the pulmonary venous drainage, and for assessing regional and global function

  5. Application of the angioskop-koordinat 3 D system for cardiac angiography in children

    International Nuclear Information System (INIS)

    Wermenski, K.

    1981-01-01

    For the angiocardiographic diagnosis of congenital heart diseases were have been applying for several years cinematography with fixed X-ray tube and image intensifier in two planes, i.e. frontal and lateral ray direction. Oblique projections as they appeared several times in publications in the last time, require with a conventional X-ray system a careful and time-consuming positioning of the child as well as the application of different cushions or thorax supports. From all this the request for an angiocardiographic system results which permits any irradiation direction by its unlimited rotability around the patient who is steadily at rest. A system is introduced which answers this requirement and which has successfully been used for more than one year by now. The application of this equipment is illustrated by examples of the examination of children suffering from Fallots tetralogy and ventricular septal defects. (orig./MG) [de

  6. Application of the angioskop-koordinat 3 D system for cardiac angiography in children

    Energy Technology Data Exchange (ETDEWEB)

    Wermenski, K

    1981-01-01

    For the angiocardiographic diagnosis of congenital heart diseases were have been applying for several years cinematography with fixed X-ray tube and image intensifier in two planes, i.e. frontal and lateral ray direction. Oblique projections as they appeared several times in publications in the last time, require with a conventional X-ray system a careful and time-consuming positioning of the child as well as the application of different cushions or thorax supports. From all this the request for an angiocardiographic system results which permits any irradiation direction by its unlimited rotability around the patient who is steadily at rest. A system is introduced which answers this requirement and which has successfully been used for more than one year by now. The application of this equipment is illustrated by examples of the examination of children suffering from Fallots tetralogy and ventricular septal defects.

  7. Encephalopathy and bilateral cataract in a boy with an interstitial deletion of Xp22 comprising the CDKL5 and NHS genes.

    Science.gov (United States)

    Van Esch, Hilde; Jansen, Anna; Bauters, Marijke; Froyen, Guy; Fryns, Jean-Pierre

    2007-02-15

    We describe a male patient with a deletion at Xp22, detected by high resolution X-array CGH. The clinical phenotype present in this infant boy, consists of severe encephalopathy, congenital cataracts and tetralogy of Fallot and can be attributed to the deletion of the genes within the interval. Among these deleted genes are the gene for Nance-Horan syndrome and the cyclin-dependent kinase-like 5 gene (CDKL5), responsible for the early seizure variant of Rett syndrome. This is the first description of a male patient with a deletion of these genes, showing the involvement of CDKL5 in severe epileptic encephalopathy in males. Moreover it illustrates the added value of high resolution array-CGH in molecular diagnosis of mental retardation-multiple congenital anomaly cases. (c) 2007 Wiley-Liss, Inc.

  8. The Association of H1N1 Pandemic Influenza with Congenital Anomaly Prevalence in Europe

    DEFF Research Database (Denmark)

    Luteijn, Johannes Michiel; Addor, Marie-Claude; Arriola, Larraitz

    2015-01-01

    BACKGROUND: In the context of the European Surveillance of Congenital Anomalies (EUROCAT) surveillance response to the 2009 influenza pandemic, we sought to establish whether there was a detectable increase of congenital anomaly prevalence among pregnancies exposed to influenza seasons in general......, and whether any increase was greater during the 2009 pandemic than during other seasons. METHODS: We performed an ecologic time series analysis based on 26,967 pregnancies with nonchromosomal congenital anomaly conceived from January 2007 to March 2011, reported by 15 EUROCAT registries. Analysis...... and tricuspid atresia and stenosis during pandemic influenza season 2009, but not during 2007-2011 influenza seasons. For congenital anomalies, where there was no prior hypothesis, the prevalence of tetralogy of Fallot was strongly reduced during influenza seasons. CONCLUSIONS: Our data do not suggest...

  9. The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema

    International Nuclear Information System (INIS)

    Fink, A. Michelle; Edis, Brian; Massie, John

    2005-01-01

    Congenital lobar emphysema (CLE) is a cause of severe neonatal respiratory distress. Overexpansion of the affected pulmonary lobe in the fetus is due to narrowing of the airway, with a resultant 'ball-valve' effect. At birth, there may be delayed clearance of fetal lung fluid. Early chest radiographs show opacification of the hyperexpanded lobe. The CT findings in the immediate neonatal period have not been previously reported. We describe the imaging in a neonate with tetralogy of Fallot and absent pulmonary valve with secondary CLE. CT demonstrates the hyperexpanded lobe with initial thickening of the interlobular septa and alveolar ground glass attenuation, with subsequent clearing. This resorption of fetal lung fluid via the pulmonary interstitium should not be confused with interstitial lung disease. (orig.)

  10. The CT appearances of delayed amniotic fluid clearance from the lungs in an infant with absent pulmonary valve and congenital lobar emphysema

    Energy Technology Data Exchange (ETDEWEB)

    Fink, A. Michelle [Royal Children' s Hospital, Department of Medical Imaging, Parkville, Victoria (Australia); University of Melbourne, Melbourne, Victoria (Australia); Edis, Brian [Royal Children' s Hospital, Department of Cardiology, Parkville, Victoria (Australia); Massie, John [University of Melbourne, Melbourne, Victoria (Australia); Royal Children' s Hospital, Department of Respiratory Medicine, Parkville, Victoria (Australia); Murdoch Children' s Research Institute, Melbourne, Victoria (Australia)

    2005-09-01

    Congenital lobar emphysema (CLE) is a cause of severe neonatal respiratory distress. Overexpansion of the affected pulmonary lobe in the fetus is due to narrowing of the airway, with a resultant 'ball-valve' effect. At birth, there may be delayed clearance of fetal lung fluid. Early chest radiographs show opacification of the hyperexpanded lobe. The CT findings in the immediate neonatal period have not been previously reported. We describe the imaging in a neonate with tetralogy of Fallot and absent pulmonary valve with secondary CLE. CT demonstrates the hyperexpanded lobe with initial thickening of the interlobular septa and alveolar ground glass attenuation, with subsequent clearing. This resorption of fetal lung fluid via the pulmonary interstitium should not be confused with interstitial lung disease. (orig.)

  11. Percutaneous pulmonary valve replacement after different duration of free pulmonary regurgitation in a porcine model effects on the right and left ventricle

    DEFF Research Database (Denmark)

    Ersboell, Mads; Vejlstrup, Niels; Nilsson, Jens Christian

    2013-01-01

    BACKGROUND: Free pulmonary regurgitation (PR) after surgical correction of Tetralogy of Fallot (ToF) with transannular patching can lead to irreversible right ventricular (RV) failure. However, the optimal timing of valve replacement is still debated. METHODS AND RESULTS: Thirty six pigs were......, respectively) after which PPVR was performed. After 1 month with competent valve the animals were euthanized. Cardiac magnetic resonance (CMR) and right heart catheterization were performed serially. Free PR led to severe dilation of the RV in all three groups compared to matched controls (p.... CONCLUSIONS: Recovery of right ventricular function after free PR by treatment with PPVR was successful in the majority of animals. Increases in RV volume during PR were the only predictor of non-recovery after PPVR and duration of PR did not in itself predict treatment success....

  12. Evaluation of Pulmonary Perfusion Scan in Heart Disease

    Energy Technology Data Exchange (ETDEWEB)

    Lee, J T; Kim, C K; Park, C Y; Choi, B S [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1973-09-15

    Pulmonary perfusion scan with radioactive {sup 113m}In-iron hydroxide particle was performed in the 25 cases of heart disease which had been diagnosed by cardiac catheterization prior to surgery from July, 1972 to July, 1973 at the Department of Radiology and Nuclear Medicine, Yonsei Medical College. It consists of 7 mitral stenosis, 2 mitral insufficiency, 1 aortic insufficiency, 3 atrial septal defect, 5 ventricular septal defect, 2 patent ductus arteriosus, 1 transposition of great vessel and 4 Tetralogy of Fallot. Findings of pulmonary perfusion scan in relation to hemodynamic data of cardiac catheterization were examined. 1) Out of 10 cases of acquired valvular heart disease, In 6 cases of stenosis and 1 case of aortic insufficiency, radioactivity was increased at both upper lung. This finding is noted when pulmonary wedge or venous pressure was elevated above 22 mmHg and arterial systolic pressure above 33 mmHg. 2) Out of 15 cases of congenital heart disease. In almost all cases of artial septal defect and ventricular septal defect except 2 cases, radioactivity was even at both entire lung. In 2 cases of patent ductras arteriosus, radioactivity was decreased especially at the left lung. It is observed that in acyanotic congenital heart disease, radioactivity of lung is not related with pulmonary arterial pressure. In 3 cases of Tetralogy of Fallot, radioactivity was even at both entire lung and in 2 of them, extrapulmonary radioactivity of liver or kidney which depends on size of defect and volume of right to left shunt reversible, was noted.

  13. Evaluation of Pulmonary Perfusion Scan in Heart Disease

    International Nuclear Information System (INIS)

    Lee, J. T.; Kim, C. K.; Park, C. Y.; Choi, B. S.

    1973-01-01

    Pulmonary perfusion scan with radioactive 113m In-iron hydroxide particle was performed in the 25 cases of heart disease which had been diagnosed by cardiac catheterization prior to surgery from July, 1972 to July, 1973 at the Department of Radiology and Nuclear Medicine, Yonsei Medical College. It consists of 7 mitral stenosis, 2 mitral insufficiency, 1 aortic insufficiency, 3 atrial septal defect, 5 ventricular septal defect, 2 patent ductus arteriosus, 1 transposition of great vessel and 4 Tetralogy of Fallot. Findings of pulmonary perfusion scan in relation to hemodynamic data of cardiac catheterization were examined. 1) Out of 10 cases of acquired valvular heart disease, In 6 cases of stenosis and 1 case of aortic insufficiency, radioactivity was increased at both upper lung. This finding is noted when pulmonary wedge or venous pressure was elevated above 22 mmHg and arterial systolic pressure above 33 mmHg. 2) Out of 15 cases of congenital heart disease. In almost all cases of artial septal defect and ventricular septal defect except 2 cases, radioactivity was even at both entire lung. In 2 cases of patent ductras arteriosus, radioactivity was decreased especially at the left lung. It is observed that in acyanotic congenital heart disease, radioactivity of lung is not related with pulmonary arterial pressure. In 3 cases of Tetralogy of Fallot, radioactivity was even at both entire lung and in 2 of them, extrapulmonary radioactivity of liver or kidney which depends on size of defect and volume of right to left shunt reversible, was noted.

  14. Current status of cardiovascular surgery in Japan 2013 and 2014: A report based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.

    Science.gov (United States)

    Hirata, Yasutaka; Hirahara, Norimichi; Murakami, Arata; Motomura, Noboru; Miyata, Hiroaki; Takamoto, Shinichi

    2018-01-01

    We analyzed the mortality and morbidity of congenital heart surgery in Japan using the Japan Cardiovascular Surgery Database (JCVSD). Data regarding congenital heart surgery performed between January 2013 and December 2014 were obtained from JCVSD. The 20 most frequent procedures were selected and the mortality rates and major morbidities were analyzed. The mortality rates of atrial septal defect repair and ventricular septal defect repair were less than 1%, and the mortality rates of tetralogy of Fallot repair, complete atrioventricular septal defect repair, bidirectional Glenn, and total cavopulmonary connection were less than 2%. The mortality rates of the Norwood procedure and total anomalous pulmonary venous connection repair were more than 10%. The rates of unplanned reoperation, pacemaker implantation, chylothorax, deep sternal infection, phrenic nerve injury, and neurological deficit were shown for each procedure. Using JCVSD, the national data for congenital heart surgery, including postoperative complications, were analyzed. Further improvements of the database and feedback for clinical practice are required.

  15. Selective serotonin reuptake inhibitor antidepressant use in first trimester pregnancy and risk of congenital anomalies

    DEFF Research Database (Denmark)

    Wemakor, A.; Casson, K.; Garne, E.

    2015-01-01

    Objective / Background The Selective Serotonin Reuptake Inhibitor (SSRI) antidepressants are widely prescribed in pregnancy, but there is evidence that they may cause congenital anomalies, particularly congenital heart defects (CHD). Objective: To determine the specificity of association between...... first trimester pregnancy exposure to individual SSRI and specific congenital anomalies (CAs). Methods Population-based case-malformed control study covering 3.3 million births from 12 EUROCAT registries 1995-2009. CAs included non-syndromic live births, fetal deaths and terminations of pregnancy......% confidence intervals (CI) were calculated adjusted for registry. Results SSRI use in first trimester pregnancy was associated with CHD overall (OR 1.38, 95 % CI 1.05-1.82, n=109); and with severe CHDs (OR 1.56, 95 % CI 1.03-2.38, n=29). Specific associations between SSRI and Tetralogy of Fallot (OR 3.36, 95...

  16. Value of the Electrocardiogram as a Predictor of Right Ventricular Dysfunction in Patients With Chronic Right Ventricular Volume Overload.

    Science.gov (United States)

    Alonso, Pau; Andrés, Ana; Rueda, Joaquín; Buendía, Francisco; Igual, Begoña; Rodríguez, María; Osa, Ana; Arnau, Miguel A; Salvador, Antonio

    2015-05-01

    Pulmonary regurgitation is a common complication in patients with repaired tetralogy of Fallot or congenital pulmonary stenosis. Electrocardiographic variables have been correlated with parameters used to evaluate right ventricular function. We aimed to analyze the diagnostic value of the width and fragmentation of the electrocardiogram in the identification of patients with right ventricular dysfunction and/or dilation. We selected 107 consecutive patients diagnosed with severe pulmonary insufficiency after repair of pulmonary stenosis or tetralogy of Fallot. The tests included electrocardiography, echocardiography, and magnetic resonance. Each electrocardiogram was analyzed manually to measure QRS duration. We defined QRS fragmentation as the presence of low-voltage waves in the terminal portion of the QRS complex in at least 2 contiguous leads. We found a significant negative correlation between QRS width and right ventricular function, as well as a positive correlation with right ventricular volume. The receiver operating characteristic curve indicated a cut-off point for QRS width of 140ms, which showed good sensitivity for a diagnosis of right ventricular dilation (> 80%) and dysfunction (> 95%). In logistic regression models, a QRS duration > 140ms was found to be the only independent predictor of right ventricular dilation and dysfunction. Electrocardiography is a rapid, widely available, and reproducible tool. QRS width constitutes an independent predictor of the presence of right ventricular dilation and dysfunction. This study is the first to provide a cutoff value for QRS width to screen for right ventricle involvement. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  17. HYPOMAGNESAEMIA AND ROLE OF MAGNESIUM SUPPLEMENTATION DURING CARDIOPULMONARY BYPASS IN PEDIATRICS CARDIAC SURGERY

    Directory of Open Access Journals (Sweden)

    Vivek

    2016-01-01

    Full Text Available BACKGROUND Postoperative Junctional Ectopic Tachycardia (JET remains one of the most common arrhythmias (8%-20% after paediatric cardiac surgery. JET is associated with hemodynamic instability, longer mechanical ventilation time and longer stays in the Cardiac Intensive Care Unit (ICU. AIM The aim of this study is to evaluate the effect of prophylactic administration of magnesium on the occurrence of postoperative arrhythmias in patients undergoing intracardiac repair for Tetralogy of Fallot and to determine the incidence of hypomagnesaemia in paediatric patients undergoing cardiac surgery who require CPB. METHODS Forty five patients with Tetralogy of Fallot undergoing intracardiac repair were enrolled to receive saline, 25mg/kg and 50mg/kg of Magnesium as three groups intraoperatively. Postoperative ECG monitored for JET and magnesium levels measured. RESULTS Hypomagnesaemia was present in 28% of patients. None of the patients who were administered magnesium developed hypomagnesaemia. The incidence of JET was found to be increased (53.3% in the placebo group as compared to 13.3% and 6.7% in the groups receiving 25 and 50mg/kg of magnesium (p<0.001. Eleven patients having JET 7 (64% had hypomagnesaemia and rest of the 4(36% occurred in patients with normal magnesium levels (p<0.01. The mean mechanical ventilation time and the mean length of ICU stay were both prolonged those with hypomagnesaemia. The mean mechanical ventilation time and length of ICU stay were both prolonged in the patients with JET (p<.001 CONCLUSIONS Hypomagnesemia is one of the factors responsible for JET and in turn with prolonged ICU stay and prolonged mechanical ventilation.

  18. The effects of breath-holding on pulmonary regurgitation measured by cardiovascular magnetic resonance velocity mapping

    Directory of Open Access Journals (Sweden)

    Babu-Narayan Sonya V

    2009-01-01

    Full Text Available Abstract Background Pulmonary regurgitation is a common and clinically important residual lesion after repair of tetralogy of Fallot. Cardiovascular magnetic resonance (CMR phase contrast velocity mapping is widely used for measurement of pulmonary regurgitant fraction. Breath-hold acquisitions, usually acquired during held expiration, are more convenient than the non-breath-hold approach, but we hypothesized that breath-holding might affect the amount of pulmonary regurgitation. Methods Forty-three adult patients with a previous repair of tetralogy of Fallot and residual pulmonary regurgitation were investigated with CMR. In each, pulmonary regurgitant fraction was measured from velocity maps transecting the pulmonary trunk, acquired during held expiration, held inspiration, by non-breath-hold acquisition, and also from the difference of right and left ventricular stroke volume measurements. Results Pulmonary regurgitant fraction was lower when measured by velocity mapping in held expiration compared with held inspiration, non-breath-hold or stroke volume difference (30.8 vs. 37.