Tetralogy Of Fallot In Infancy Research Articles

The role of primary surgical repair technique on late outcomes of Tetralogy of Fallot: a multicentre study.

Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique. This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV. We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7-11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1-21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022). Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity.

Impact of timing on in-patient outcomes of complete repair of tetralogy of Fallot in infancy: an analysis of the United States National Inpatient 2005\u20132011 database

BackgroundThis study aimed to investigate whether age at complete repair of tetralogy of Fallot (TOF) impacts postoperative morbidity and length of hospital stay in infants less than 365 days of age.MethodsThe United States Nationwide Inpatient Sample was searched for infants 0–365 days of age that underwent complete repair of TOF between 2005 and 2011. Patients were categorized based on age at time of repair: 0–30 days; 31–90 days; 91–180 days; > 180 days.ResultsA total of 1112 infants were included in the study. Multivariate analysis showed the risk of postoperative complications was 40% lower in infants ≥91 days old at the time of repair as compared to those ≤30 days old. In addition, children > 30 days old at the time of repair had a significantly shorter length of hospital stay than those aged ≤30 days. In the subgroup with elective repair, older age was associated with a shorter length of hospital stay as compared to those ≤30 days old at repair, while association between age at complete repair of TOF and postoperative complication was not significant among the groups after adjusting for confounders.ConclusionsIn children < 1 year old, postoperative complications and length of hospital stay are affected by the timing of complete repair of TOF.

Management of tetralogy of Fallot with Blalock–Taussig shunt alone in a low-resource setting

The outcomes of management of Tetralogy of Fallot (TOF) in children with only BT shunts have not been widely reported. Therefore, we present a 29-year-old man with complaints of progressive easy fatigability and effort intolerance. He was diagnosed with TOF in infancy and had both left- and right-sided BT shunts without corrective surgery. Examination revealed an asthenic young man with conjunctival plethora, cyanosis, digital clubbing, and hypertension. Chest X-ray, electrocardiography, and echocardiography revealed the typical anomalies of TOF. He has been referred for corrective surgery. Despite two previous BT shunts, the investigations still revealed the structural anomalies of TOF; this confirms BT shunt is not the definitive treatment but a palliative measure. Total corrective surgery remains the definitive treatment of TOF. Patients and their caregivers should be counseled on this and on the need for early corrective surgery.

Современные паллиативные методы лечения новорожденных и грудных детей с тетрадой Фалло. Часть 2. Эндоваскулярное лечение

Во второй части статьи представлено современное состояние паллиативного эндоваскулярного лечения новорожденных и грудных детей с тетрадой Фалло, у которых возможно выполнение транслюминальной баллонной вальвулопластики клапанного стеноза легочной артерии (КСЛА), стентирование открытого артериального протока (ОАП) и стентирование выводного отдела правого желудочка (ВОПЖ). Транслюминальная баллонная вальвулопластика КСЛА сопровождается хорошими клиническими результатами при отсутствии выраженного подклапанного стеноза ЛА, приводит к увеличению насыщения артериальной крови кислородом, росту легочных артерий и фиброзного кольца ЛА. Стентирование ОАП наиболее предпочтительно при аномалии коронарных артерий, когда они пересекают ВОПЖ, при гипоплазии легочных артерий с большими аортолегочными коллатеральными сосудами, при выраженном стенозе или атрезии устья левой ЛА, когда кровоснабжение левого легкого осуществляется через боталлов проток. Стентирование ВОПЖ – эффективная процедура, обеспечивающая достаточный антеградный кровоток по ЛА и приводящая к росту и развитию гипоплазированных ЛА. Эндоваскулярные методы лечения приводят к быстрой стабилизации клинического состояния за счет устранения артериальной гипоксемии и могут быть альтернативой открытым паллиативным хирургическим вмешательствам у определенных групп пациентов.

Современные паллиативные методы лечения новорожденных и грудных детей с тетрадой Фалло. Часть 1. Хирургическое лечение

Современные паллиативные методы лечения новорожденных и грудных детей с тетрадой Фалло. Часть 1. Хирургическое лечение

Constrictive Pericarditis Following Correction of Partial Anomalous Pulmonary Venous Drainage

We report the case of a 23-year-old man who developed constrictive pericarditis within four months after pulmonary valve replacement and repair of partial anomalous pulmonary venous connection. He had previously undergone repair of tetralogy of Fallot in infancy. After an unsuccessful trial of medical management for persistent right heart failure, magnetic resonance imaging was done, which showed a thickened pericardium. He underwent a radical pericardiectomy with a good outcome. The case is presented to illustrate a less well-recognized cause of cardiac failure following congenital cardiac surgery, which may otherwise be attributed to the failure of surgery or residual complications.

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

BackgroundTetralogy 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.MethodsThis 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.ResultsIn 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).ConclusionCombined 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.

Primary repair of tetralogy of Fallot in infants: Transatrial/transpulmonary or transventricular approach

Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of tetralogy of Fallot (TOF). Transatrial/transpulmonary repair avoids a ventriculotomy (in contrast to the transventricular approach) in order to preserve the structure and function of the right ventricle. We performed a pilot prospective randomized controlled trial in infants with TOF undergoing primary repair. A pilot prospective controlled clinical trial was conducted in infants with TOF undergoing primary repair between January 2008 and December 2009. One hundred and six patients were recruited in the trial and divided into a transatrial-transpulmonary approach group (Group A; n=53) and a transventricular approach group (Group B; n=53), depending on the different surgical techniques used. Preoperative patient characteristics and procedure-related variables were similar. There were no deaths in Group A, while two patients died in Group B. There were significant differences in cardiopulmonary bypass time (95.02±23.8 vs. 85.23±22.63 minutes, p=0.032), cross-clamp time (69.4±10.36 vs. 61.17±9.38 minutes, p=0.035), inotropic support (1.63±0.97 vs. 2.1±1.09 days, p=0.02), intubation time (26.62±12.48 vs. 33.02±17.55 hours, p=0.033), duration of stay in the intensive care unit (ICU) (2.25±1.28 vs. 2.85±1.46 days, p=0.026), and the incidence of arrhythmia [3 patients (5.7%) vs. 10 patients (18.9%), p=0.038]. No significant differences in right/left ventricular pressure ratio and hospital stay were observed. Transatrial/transpulmonary repair of TOF is associated with excellent surgical results and immediately follow-up.

LINE-1 methylation status and its association with tetralogy of fallot in infants

BackgroundMethylation levels of long interspersed nucleotide elements (LINE-1) are representative of genome-wide methylation status and play an important role in maintaining genomic stability and gene expression. To derive insight into the association between genome-wide methylation status and tetralogy of fallot (TOF), we compared the methylation status of LINE-1 element between TOF patients and controls. The methylation of the NKX 2–5, HAND 1, and TBX 20 promoter regions was also evaluated.MethodsGenomic DNA from right ventricular tissue samples was obtained from 32 patients with TOF and 15 control subjects. Sequenom MassARRAY platform was performed to examine the methylation levels of LINE-1, NKX2-5, HAND1 and TBX20. Mann–Whitney U test was used to compare differences in methylation levels between two groups.ResultsThe methylation level of LINE-1 was significantly lower in patients with TOF, with a median of 57.95% (interquartile range [IQR]: 56.10%–60.04%), as opposed to 59.70% in controls (IQR: 59.00%–61.30%; P = 0.0021). The highest LINE-1 methylation level was 61.3%. The risk of TOF increased in subjects with the lowest methylation levels (less than or equal to 59.0%; OR = 14.7, 95% CI: 1.8–117.7, P = 0.014) and in those with medium methylation levels (59.0%–61.3%; OR = 2.0, 95% CI: 0.3–14.2, P = 0.65). An ROC curve analysis showed a relatively high accuracy of using the LINE-1 methylation level in predicting the presence of TOF (AUC = 0.78, 95% CI: 0.65–0.91; P = 0.002). The association of the LINE-1 methylation level with TOF was only observed in males (P = 0.006) and not in females (P = 0.25). Neither age nor gender was found to be associated with the LINE-1 methylation level in patients or controls. Higher methylation levels of NKX2-5 and HAND1 and lower methylation levels of TBX20 were also observed in patients with TOF than in controls. No association was found between the methylation levels of NKX2-5, HAND1 and TBX 20 with the LINE-1 methylation level.ConclusionsLower LINE-1 methylation levels are associated with increased risk of TOF and may provide important clues for the development of TOF.

Heart Failure in Children

Why is heart failure in children important? If we just consider the number of individuals affected, adult heart failure is clearly a more compelling public health problem. However, the relatively small numbers belie the overall economic and social impact of pediatric heart failure. When a child is admitted to the hospital for heart failure, the costs are considerably higher for children than adults because of the frequent need for surgical or catheter-based intervention. The demands of medical care can fray the family structure and adversely affect parental economic productivity. When a child dies of heart failure, the economic impact is magnified enormously because of the number of potentially productive years lost per death. For these and other reasons, heart failure in children is a serious public health concern. In addition, growing numbers of children with heart failure are reaching adulthood because of the successful application of medical and surgical heart failure therapies and the improved outcomes of congenital heart surgery. A greater understanding of the pathophysiology of heart failure in childhood may help inform therapeutic strategies once these children become adults. Furthermore, given the recent explosion of research in the impact of cardiac development and cardiogenetics on pediatric cardiovascular disease, it is not outside the realm of possibility for a pediatric discovery to be made that will also benefit adults with heart failure. We may not yet be able to agree on a definition of heart failure, but the cardinal symptoms, dyspnea, anasarca (“dropsy”), and cachexia, were well recognized in antiquity.1 These symptoms were not specific for cardiac pathology, and it was not until the 17th century, when William Harvey definitively identified the heart as an organ that pumped blood rather than generating heat, that the heart could begin to be understood as a source of dyspnea, edema, and …

Management of complications after operation for tetralogy of Fallot on infants and follow-up of one year

Objective To review the early postoperative complications and its treatment in infants with tetralogy of Fallot(TOF).Methods From January 2004 to March 2007,35 infant patients with TOF,aged 4 to 12 months,underwent corrective procedure with hypothermic cardiopulmonary bypass(CPB) and general anaesthesia in Anhui provincial children's hospital.Of all the patients,30 cases received radical surgery and the other 5 cases with initial palliation.Results The early postoperative complications occurred in 10 cases,of which 6 cases comlplicated with low cardiac output,2 cases had severe arrhythmia,2 cases manifested as perfusion lung,2 cases were symptomized by residual right ventricular outflow tract(RVOT) obstruction and 1 case suffered from residual ventricular septal defect(VSD).There were 3 cases of early postoperative death with 8.6% of hospital mortality.Conclusion The operative mortality can be effectively reduced by careful preparations for surgery,reliable surgical procedure,effective myocardial protection and timely treatment for postoperative complications.This study demonstrated that repair of TOF in infancy can be done with excellent results. Key words: Tetralogy of Fallot; Infants; Postoperative complications; Follow-up

Invited Commentary

Invited Commentary

Analysis of 614 cases of tetralogy of fallot in infancy before three years old by radical correction

目的 临床评价婴幼儿期施行法乐四联症根治术的手术疗效.方法 对614例3周岁以下婴幼儿法乐四联症根治术进行总结.年龄2～36个月,体质量3.5～15.0 kg.在中度低温体外循环下手术.除11例外,均采用自体心包补片修补室间隔缺损.采用自体心包补片扩大右心室流出道.结果 手术死亡38例,手术死亡率为6.19%,手术死亡原因和术后并发症主要为低心排综合征和心律失常.结论 婴幼儿期法乐四联症根治术的手术有其自身特点,改进手术技术,加强术后监护处理,可提高手术成功率。

Genetic Syndromes and Outcome After Surgical Correction of Tetralogy of Fallot

Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact of genetic syndromes on surgical repair of TOF in infancy is still under investigation. This retrospective study reviews the outcome of 306 consecutive patients (median age, 5.1 months) who underwent primary (266) or staged (40) repair of TOF between 1994 and 2004. Total follow-up was 1,188 patient-years (mean, 57 months). Genetic syndromes were documented in 85 patients (27.8%), including 22q11 deletion (27), trisomy 21 (13), vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities (VACTERL, 12), and others (33). Hypoplastic pulmonary arteries (PA) were more common in syndromic (19/85 = 22.3%) than nonsyndromic TOF (20/221 = 9.04%) (p < 0.001). Primary repair was performed in 82.4% syndromic and 88.7% nonsyndromic TOF (p = not significant [NS]). Ten-year actuarial survival was 94.1 +/- 2.3% in nonsyndromic and 84.3 +/-4.2% in syndromic TOF (p < 0.001). Ten-year survival was 96.3 +/- 3.6% for del22q11, 100% for trisomy 21, 63.6 +/- 14.5% for VACTERL, and 78.5 +/- 7.3% for patients with other syndromes (p = 0.022). Survival in syndromic TOF with normal PA anatomy was 89.6 +/- 4.2% for primary repair and 85.7 +/- 12.8% for staged repair (p = NS); freedom from reoperation after complete repair was 74.4 +/- 6.4% for primary correction and 56.3 +/- 11.9% for staged repair (p = 0.04). Cox proportional hazard identified the presence of genetic syndrome (p = 0.011) and central PA hypoplasia (p = 0.002) as independent predictors of mortality. Pulmonary arborization defects and genetic syndromes other than del22q11 or trisomy 21, are associated with worse outcome after correction of TOF. Primary TOF repair in syndromic patients with normal PA anatomy is a valid surgical strategy, with no additional risk for mortality and higher freedom from reintervention.

Complete repair of tetralogy of Fallot in infancy

We reviewed our long-term results of complete repair of tetralogy of Fallot (TOF) in infant. One hundred and sixty infants diagnosed as TOF underwent complete repair between January 1990 and April 2002. Mean age at the operation was 8.1+/-2.6 months. Correction was accomplished through a short right ventriculotomy of less than 30% of the ventricular height in all patients. A transannular patch was used in 78 patients (49%). There were four early deaths and no late death. Follow-up was complete in all survivors. All patients are currently in New York Heart Association functional class I or II. Actuarial freedom from reoperation at 1 and 10 years were 94.0 and 87.5%, respectively. Echocardiographic studies at follow-up showed excellent right ventricular function in most patients. Our results suggest that early complete repair of TOF yielded acceptable results with low mortality and morbidity. Transventricular repair of intracardiac pathology can be safely applied to yield good postoperative right ventricular function.

Relation of the discrete subaortic stenosis position to mitral valve function

1. Daliento L. Total correction of tetralogy of Fallot: late clinical follow-up. Ital Heart J 2002;3:24–27. 2. Rhodes J, Dave A, Pulling MC. Effect of pulmonary artery stenoses on the cardiopulmonary response to exercise following repair of tetralogy of Fallot. Am J Cardiol 1998;81:1217–1219. 3. Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN. Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation. Br Heart J 1992;60:57–65. 4. Dudekem Y, Rubay J, Ovaert C. Failure of right ventricular recovery of fallot patients after pulmonary valve replacement: delay of reoperation or surgical technique? J Am Coll Cardiol 2001;37:2008–2009. 5. Cheung YF, Leung MP, Lee JW, Chau AK, Yung TC. Evolving management for critical pulmonary stenosis in neonates and young infants. Cardiol Young 2000;10:186–192. 6. Yetman AT, Lee KJ, Hamilton R, Morrow WR, McCrindle BW. Exercise capacity after repair of tetralogy of Fallot in infancy. Am J Cardiol 2001;87: 1021–1023. 7. Unnithan VB, Wilson J, Buchanan D, Timmons JA, Paton JY. Validation of the Sensormedics metabolic cart for pediatric exercise testing. Can J Appl Physiol 1994;19:472–479. 8. Wasserman K. Principles of Exercise Testing and Interpretation. 2nd Ed. Philadelphia, PA: Lea & Febinger, 1994:44. 9. O’Connor BK, Beekman RH, Lindauer A, Rocchini A. Intermediate-term outcome after pulmonary balloon valvuloplasty: comparison with a matched surgical control group. J Am Coll Cardiol 1992;20:169–173. 10. Gatzoulis MA, Clark AL, Cullen S, Newman CG, Redington AN. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Restrictive physiology predicts superior exercise performance. Circulation 1995; 91:1775–1781.

Exercise capacity after repair of tetralogy of fallot in infancy

Exercise capacity after repair of tetralogy of fallot in infancy

Influence of perioperative factors on outcomes in children younger than 18 months after repair of tetralogy of Fallot

Background. There has been a trend toward advocating earlier repair of tetralogy of Fallot and avoiding palliative procedures. The impact of this trend on perioperative outcomes has not been adequately documented.Methods. Data from consecutive patients undergoing repair of tetralogy of Fallot at less than 18 months of age from May 1987 to September 1994 were reviewed. Independent factors associated with duration of stay in the intensive care unit were sought.Results. Repair was performed in 89 infants at a median age of 13 months (range, 15 days to 18 months). A systemic–pulmonary artery shunt was present in 24% of patients. Mean duration of cardiopulmonary bypass was 119 ± 37 minutes; 63% of patients received a transannular patch. There were six deaths (7%), all occurring less than 48 hours after repair. The median duration of stay in the intensive care unit was 5 days (range, 1 day to 8 months). Significant independent factors associated with increasing length of intensive care unit stay included younger age at repair, previous shunt, malformation syndrome, increased total dose and number of inotropic agents used, and respiratory complications. Hemodynamic variables serially recorded in the first 48 hours after repair were independently associated with death or prolonged (>7 days) duration of stay.Conclusions. Although outcomes after repair of tetralogy of Fallot in infants are good, both younger age at repair and previous palliative procedures were associated with longer duration of stay in the intensive care unit.

Surgical experience with total correction of tetralogy of Fallot in infancy

Fifty two patients less than one year old with tetralogy of Fallot underwent primary repair between January 1991 and December 1994. Age range was three to twelve months (mean 10.09 ± 2.01 months) and body weight ranged from 4.5 to 9 kg (mean 8.38 ± 2.79 kg). Transatrial-transpulmonary repair was performed in 36 patients and the classical transventricular approach was used in 16 patients. Six patients underwent emergency surgery for severe cyanosis and spells. Five patients had left pulmonary artery plasty for pulmonary artery bifurcation stenosis and two out of the five patients who had anomalous coronary arteries needed a right ventricle to pulmonary artery conduit. Mean post repair peak right ventricular/systemic pressure ratio was 0.74 ± 0.18 in the transventricular group and 0.71 ± 0.26 in the transatrial-transpulmonary group. There were three hospital deaths. Follow-up ranged from 3 to 46 months (mean 21.18 months). Forty patients underwent echocardiography and twenty patients underwent cardiac catheterisation six to eighteen months after surgery. Mean right ventricular outflow tract gradient on echocardiography was 20.35 ± 10.12 and, at cardiac catheterisation, 17.51 ± 13.49 mmHg with mean post repair peak right ventricle/left ventricle pressure ratio of 0.44 ± 0.11. These were significantly less than the values obtained in the operating room. Only one patient had residual ventricular septal defect with left to right shunt of 1.6:1 at cardiac recatheterisation. There was one late death after reoperation for residual obstruction. Encouraging results with primary repair of tetralogy of Fallot in infancy prompt us to continue this policy in suitable cases.

Surgical experience with total correction of tetralogy of Fallot in infancy

Surgical experience with total correction of tetralogy of Fallot in infancy