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.

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.

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.

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.

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

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

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.

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.).

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.

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

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.

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.

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.

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

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)

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)

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.

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)

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.

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

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.

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.

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.

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

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.

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.

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.

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.

[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.

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

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.

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.

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

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

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.

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.

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.

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

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.

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

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.

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.

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.

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.

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.

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.

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.

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

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.].

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 ...

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.

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.

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

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.

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.

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.

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)

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.

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.

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

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

Directory of Open Access Journals (Sweden)

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

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.

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.).

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.)

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.

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.

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.

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.

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.

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.

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...

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.

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.

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.

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.

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

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

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.

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.

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

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 �

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

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.

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.

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

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.

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

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.

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

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.

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

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

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.

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.

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

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

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.

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

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

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,

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.

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.

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.

[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?

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.

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.

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.)

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.

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.

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.

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.

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.

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...

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.

31. Abordaje transaórtico de la tetralogía de fallot en el adulto

Directory of Open Access Journals (Sweden)

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.

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.

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

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.

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.

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.

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...

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.

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.

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.

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.

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

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

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.

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.

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

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

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

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.

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.

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.

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.

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...

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.

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.

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.

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)

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.

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...

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.

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.

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.)

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.)

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.)

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.)

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)

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.

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.

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.

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

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.

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

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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.

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.

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.

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.

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

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.

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

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

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

Directory of Open Access Journals (Sweden)

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.

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.

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.

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.

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

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

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.)

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.)

[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.

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

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

Directory of Open Access Journals (Sweden)

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.

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

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.

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

[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.

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.

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.

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

Energy Technology Data Exchange (ETDEWEB)

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.

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

International Nuclear Information System (INIS)

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

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

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.

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.

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.

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

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)

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.

Early post operative mortality of Total Correction of Tetralogy of Fallot

Directory of Open Access Journals (Sweden)

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

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.

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.

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.

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

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

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.

Repair of Tetralogy of Fallot in Infancy via the Atrioventricular Approach

Directory of Open Access Journals (Sweden)

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.

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.

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.

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.

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

Directory of Open Access Journals (Sweden)

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.

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

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.

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.

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.

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.)

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.)

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.

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.

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

Directory of Open Access Journals (Sweden)

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.

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

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

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.

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

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.

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

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.

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

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.

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.

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

Directory of Open Access Journals (Sweden)

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.

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.

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

Science.gov (United States)

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.

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

Science.gov (United States)

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.

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.

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.

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

Science.gov (United States)

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

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.

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

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

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

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

Directory of Open Access Journals (Sweden)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Science.gov (United States)

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.

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.

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

Directory of Open Access Journals (Sweden)

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.

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

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

Science.gov (United States)

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.

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.

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.

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

Science.gov (United States)

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.

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

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).

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

Science.gov (United States)

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.

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

Science.gov (United States)

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.

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.

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)

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.

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

International Nuclear Information System (INIS)

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.)

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

Energy Technology Data Exchange (ETDEWEB)

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.)

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

Science.gov (United States)

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.

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

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.

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.

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

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

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

Directory of Open Access Journals (Sweden)

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.

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

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

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.

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

Directory of Open Access Journals (Sweden)

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.

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.

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.

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.

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

Science.gov (United States)

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

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.