Surgery For Tetralogy Of Fallot Research Articles

第17回 心臓性急死研究会 Fallot四徴症の根治術後遠隔期における心室頻拍の発症の予測における加算平均心電図の有用性

第17回 心臓性急死研究会 Fallot四徴症の根治術後遠隔期における心室頻拍の発症の予測における加算平均心電図の有用性

Impairment of tricuspid annular plane systolic excursion and tricuspid annular peak systolic velocity after ventricular septal defect correction

We read with interest the article ‘Disparity in right vs left ventricular recovery during follow-up after ventricular septal defect correction in children’ by Klitsie et al. [1]. In our opinion, this is an interesting manuscript describing a possible long-term effect of open-heart surgery on the remodelling of the right ventricle (RV) in children with ventricular septal defect (VSD). The authors found a reduced tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (S0) in their VSD patients compared with age-related normal values and that RV systolic performance, in contrast to LV systolic performance, remained significantly impaired up to 20 months postoperatively. We completely agree with the findings of Klitsie et al. [1] that this decrease of RV systolic performance after surgical correction of VSD may be due to the detrimental effects of open-heart surgery with cardiopulmonary bypass. We wish to mention that in children after open-heart surgery for tetralogy of Fallot, the TAPSE and S0 values are decreased and also worsen continuously over time [2]. Performing VSD subtype analyses, Klitsie et al. [1] found no significant difference in echocardiographic parameters, perhaps due to the small size of this subgroup. For the convenience of the readership of the European Journal of Cardiothoracic Surgery, we would like to mention that, in our opinion, if a comparison of TAPSE and S0 data from their VSD patients with available TAPSE and S0 paediatric normal values and Z-scores [3, 4] is made, this lack of statistical power in their subgroup analysis could be overcome. Klitsie et al. [1] also support the notion that the RV is highly susceptible to postoperative function impairment in paediatric patients with a VSD. We would like to thank the authors for addressing the need for careful and systematic evaluation of the RV in patients with congenital heart diseases before and after surgical correction. We hope that, with more interesting studies like this from Klitsie et al. [1], the quantification of systolic RV function will become a routine measurement in postoperative VSD patients.

PP-279 ABLATION OF THE TYPICAL CAVOTRICUSPID ISTHMUS-DEPENDENT ATRIAL FLUTTER AND ATRIOVENTRICULAR NODAL REENTRY TACHYCARDIA IN A PATIENT WITH OPERATED TETRALOGY OF FALLOT

Introduction: Cavotricuspid isthmus (CTI)-dependent atrial flutter (AFL) is the most common arrhythmia in operated Tetralogy of Fallot (ToF) patients. Here, we report the successful ablation of a typical counterclockwise AFL and reverse clockwise AFL in a patient with operated ToF. Case report: A 35-year-old male had previously undergone total corrective surgery for ToF 27 years before admitted to our clinic. He suffered from tachyarrhythmias for 3 months, and his symptoms had worsened in the last 1 month with the manifestation of paroxysmal typical AFL. Baseline ECG revealed first degree AV block and right ventricular hypertrophy signs. Upon examination of the 12-lead ECG recorded during palpitation, we observed no visible P waves and inferior axis and thus considered the possibility of AVNRT. Transthoracic and transesophageal echocardiography revealed an incomplete closure of the tricuspid valve with severe tricuspid regurgitation and a dilated right atrium in the apical 4-chamber view. Electrophysiological investigation revealed both clockwise and anticlockwise AFL (Figures 1a,b).

Unusual systemic venous Collateral channels to left atrium causing desaturation after Fontan operation closed percutaneously

We present an unusual cause of progressive cyanosis in a child appearing 2 years after successful Fontan surgery for tetralogy of Fallot with hypoplastic right ventricle. The cause of cyanosis was identified as one large venous channel draining into the left atrium. The channel was closed by Amplatzer vascular plug resulting in improvement of oxygen saturation.

Valve-Sparing Options in Tetralogy of Fallot Surgery

Given late outcomes of patients with tetralogy of Fallot repaired in the 1970s and 1980s, as well as a better understanding of the late deleterious effects of pulmonary regurgitation, there is a tendency toward preservation of the pulmonary valve function during primary repair of tetralogy of Fallot. The bar keeps moving downward, to include smaller and more dysmorphic pulmonary valves. This article reviews some useful indications and techniques for valve-sparing options, including intraoperative balloon dilation and cusp reconstruction using a patch. Just like other valve repair techniques, no one technique can be applied uniformly, and surgeons must master a wide armamentarium of techniques.

Radiofrequency Catheter Ablation of Ventricular Tachycardia after Repair of Tetralogy of Fallot

Sustained monomorphic ventricular tachycardia (SMVT) is a late complication of corrective surgery for tetralogy of Fallot. The success rate of conventional radiofrequency ablation is <50% due to patients’ characteristics. Three-dimensional electroanatomic mapping system is associated with favorable outcomes. We describe the case of a 53 year-old male patient with a history of previous corrective surgery for tetralogy of Fallot and an implantable cardioverter defibrillator due to syncope secondary to SMTV. Despite being treated with amiodarone, radiofrequency catheter ablation was indicated due to recurrences. In addition, we describe three-dimensional electroanatomic mapping system as a complement to conventional electrophysiology for an adequate characterization of the arrhythmogenic circuits undergoing radiofrequency catheter ablation. To our understanding, this is the first case published in Argentina of radiofrequency catheter ablation of a SMVT in patients with corrective surgery for tetralogy of Fallot.

Results of reparative surgery for tetralogy of Fallot: data from the European Association for Cardio-Thoracic Surgery Congenital Database

Right ventricular (RV) dysfunction and pulmonary insufficiency (PI) after tetralogy of Fallot (TOF) repair may contribute to early and late morbidity and mortality. RV dysfunction may be related to the ventriculotomy employed in the transventricular repair technique, particularly when it is combined with a transannular patch (TAP). Transatrial/transpulmonary (TA/TP) repair without ventriculotomy has been advocated as a method potentially diminishing such adverse events. However, the prevalence and early as well as the late results of these different surgical approaches in Europe have not been studied. To ascertain 'the current prevalence and associated early mortality' of various surgical approaches for repair of TOF, relevant data in the European Association for Cardio-Thoracic Surgery Congenital Database were analysed. The study population was all types of reparative operations (n = 6654) for patients with primary diagnosis of TOF reported between 1999 and 2011. Overall hospital mortality (HM) was 2.58% (172 of 6654). Repair via ventriculotomy with TAP was the most prevalent technique (n = 3827, 57.5%), with HM 3.11%. Repair via ventriculotomy with non-TAP was performed in 1309 patients (19.7%, HM = 1.53%). Repair without ventriculotomy was performed in 1214 patients (18.2%, HM = 1.48%). Logistic regression analysis showed statistically significant association between HM risk and the type of surgery. In particular, ventriculotomy with TAP is associated with increased mortality risk significantly compared with ventriculotomy with non-TAP (crude odds ratio [OR] 2.13 (95% confidence interval [CI]: 1.33-3.63). Similar results were obtained by analysing for operative mortality risk (30-day mortality, OM). Operations that have been performed before 2005 have resulted in increased surgical risk compared with those performed after 2005 (ORs for OM 1.45, 95% CI: 1.03-2.013). Overall HM for TOF repair is low. TOF repair by means of ventriculotomy with TAP is the most prevalent approach and is associated with higher mortality. Repair with ventriculotomy but no TAP and repair without ventriculotomy are both less prevalent and with lower mortality. Surgical risk appears to be decreasing over time.

Comparison of Different Doses of ε-Aminocaproic Acid in Children for Tetralogy of Fallot Surgery: Clinical Efficacy and Safety

The purpose of this study was to compare 2 different doses of ε-aminocaproic acid (EACA) and assess their relative efficacy and safety in children undergoing corrective surgery for tetralogy of Fallot (TOF). A prospective, randomized, controlled study. A tertiary care center. One hundred twenty children undergoing corrective surgery for TOF using cardiopulmonary bypass (CPB). Group 1 received 100 mg/kg of EACA after induction, upon initiation of CPB, and after protamine. Group 2 received 75 mg/kg of EACA after induction, followed by a maintenance infusion of 75 mg/kg/h until chest closure, and an additional 75 mg/kg upon initiation of CPB. Group 3 did not receive any antifibrinolytic agent or placebo. Cumulative mean blood loss, total packed red blood cells, and fresh frozen plasma requirements were significantly less in group 2 (p ≤ 0.01). There were no significant differences in the total platelet concentrate transfused, re-exploration rate, incidence of renal failure, arrhythmias, neurologic complications, mortality, or length of intensive care unit stay among the 3 groups. The incidences of perioperative ST/T changes and chest closure time were significantly less in group 1 and group 2 (p ≤ 0.01). The duration of mechanical ventilation was significantly less in group 2 (p ≤ 0.01). EACA was effective in reducing the postoperative blood loss and transfusion requirements in children undergoing corrective cardiac surgery on CPB for TOF. The dose regimen of 75 mg/kg after induction, followed by a maintenance infusion of 75 mg/kg/h until chest closure, and an additional 75 mg/kg upon initiation of CPB were more effective.

Impact of 22q11.2 Deletion on the Postoperative Course of Children After Cardiac Surgery

The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. Demographic information, cardiac diagnoses, noncardiac abnormalities, preoperative factors, intraoperative details, surgical procedures performed, postoperative complications, and in-hospital deaths were collected. The outcome data collected included days of inotrope use, need for dialysis, length of mechanical ventilation, intensive care unit (ICU) length of stay (LOS), hospital LOS, and mortality. The study enrolled 173 patients: 65 patients in the 22q11 group and 108 patients in the control group. Of the 65 patients in the 22q11 group, 36 (55 %) underwent repair for TOF, 13 (20 %) for IAA, and 16 (25 %) for TA. The two groups did not differ in terms of age or weight. The preexisting conditions were similar in the two groups. Unplanned noncardiac operations were more common in the children with del22q11, but delayed chest closure was similar in the two groups. The incidence of postoperative noncardiac complications such as reintubation, vocal cord paralysis, and diaphragmatic paralysis was similar in the two groups. However, increasing numbers of patients in del22q11 group needed dialysis in one form or the other during the immediate postoperative stay. The incidence of fungal infection and wound infection was higher in the del22q11 group than in the control group. Duration of mechanical ventilation, ICU LOS, and hospital LOS were similar in the two groups, except in certain subgroups. Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.

Predictors of mid-term event-free survival in adults with corrected tetralogy of Fallot

Objectives Patients who underwent corrective surgery for tetralogy of Fallot (TOF) have increased long-term risk of cardiovascular morbidity and mortality. Yet, limited information is available on how to evaluate the risk in this population. Therefore, the aim of this study was to investigate the prognostic value of aerobic exercise capacity, along with other related parameters, at medium-term follow-up in adult patients with tetralogy of Fallot.Methods and results Between 2000 and 2003, 92 adults (age 26.2 ± 7.8 years; 63 male) with corrected TOF or TOF-type morphology underwent a cardiopulmonary exercise test (CPET) until exhaustion and echocardiography. During a mean follow-up of 7.3 ± 1.2 years (range 0.9 to 9.3 years), 2 patients died and 26 patients required at least 1 cardiac-related intervention at a mean age of 28.9 ± 7.9 years. Event-free survival tended to be higher in patients with the classical type of TOF (P = 0.061). At multivariate Cox analysis, age at CPET [hazard ratio (HR): 1.13, P= 0.006], age at correction (HR: 0.82, P= 0.037), right ventricular (RV) function (HR: 4.94, P= 0.001), QRS duration (HR: 1.02, P= 0.007), percentage of predicted peak oxygen uptake (peak VO2%) (HR: 0.96, P= 0.029) and ventilatory effi ciency slope (VE/VCO2 slope) (HR: 1.13, P= 0.021) were signifi cantly related to the incidence of death/cardiac-related intervention during medium follow-up.Conclusions Early corrective surgery and a well-preserved RV are associated with a better outcome in adults with corrected TOF. Furthermore, CPET provides important prognostic information; peak VO2% and VE/VCO2 slope are independent predictors for event-free survival in patients with corrected TOF.

The incidence of congenital scoliosis in infants with tetralogy of Fallot based on chest radiographs

Early diagnosis is critical in patients with congenital scoliosis to identify vertebral defects that have a high risk for progression. A review of the medical records and chest radiographs for 562 patients who underwent corrective surgery for tetralogy of Fallot (TOF) at our institution between 1992 and 2007 was conducted. Of the 364 patients examined in the study, 12 patients (3.3%), six girls and six boys, had a positive diagnosis of congenital scoliosis confirmed by examination of radiographs. This incidence is significantly higher than both the incidence reported in prior studies (0-1.4%) and in the general population (0.05-0.10%). Given that patients with TOF routinely undergo chest radiograph, physicians examining chest radiographs of TOF patients should be aware of the potential for congenital scoliosis to provide early diagnosis and referral for orthopaedic evaluation and treatment.

Relationship between ventricular function assessed by tissue Doppler imaging and exercise capacity in patients after repair of tetralogy of Fallot: an observational study

The present study aims to study the relationship between tissue Doppler echocardiography (TDE) indices of right ventricle and exercise capacity in patients after total correction for tetralogy of Fallot (ToF). This cross-sectional observational study included 20 patients, after undergoing total correction procedure for ToF diagnosed with mild/moderate pulmonary regurgitation and 30 age-matched healthy children. In the postoperative period, patients were invited to hospital for evaluation of the ventricular functions by 2D, M-mode, Doppler (DE) echocardiography and TDE and exercise testing to evaluate the effort capacity. Statistical analysis was performed using Mann-Whitney U and Chi-square tests, and Pearson correlation analysis. Compared with the controls; the mitral annular peak systolic flow velocity (Sm) value was significantly lower, while isovolumic contraction time (IVCT), isovolumic relaxation time (IVRT) and myocardial performance index (MPI) values obtained at the tricuspid and mitral (MV) valves were significantly higher (p<0.05 for all) in patients after ToF repair. There was a negative correlation between the exercise period and the total correction age (r=-0.20, p=0.015) and the same negative correlation existed between the exercise period and METS (r=-0.25, p=0.010). MV IVCT with DE and TDE was found to be correlated with METS (r=-0.45, p=0.04). Left ventricular MPI was found to be correlated with maximum heart rate (r=-0.20, p=0.03). By DE, tricuspid valve deceleration time and Sm peak flow velocity with TDE were significantly correlated with METS (r=-0.30, p=0.04; r=-025, p=0.005, respectively). MPI calculated with TDE was correlated with maximum heart rate (r=-0.15, <0.01). Even if patients, undergone total correction surgery for ToF were asymptomatic or had minimal clinical symptoms, MPI index assessed by pulse wave TDE and exercise testing may allow early diagnosis of right ventricle dysfunction.

Functional Health Status of Adults With Tetralogy of Fallot: Matched Comparison With Healthy Siblings

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 < 0.001), were less likely to rate their health as good or excellent (p < 0.001), and had lower SF-36 scores for physical functioning and general 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.

Unusual operation for tetralogy of Fallot

Unusual operation for tetralogy of Fallot

A mysterious case of fatal embolism after the repair of tetralogy of Fallot : secondary to arrhythmia? : case report

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease. The most important late complications observed after repair of TOF are atrio-ventricular arrhythmias and sudden cardiac death. We present a rare case of fatal embolism and sudden cardiac death in a 36-year-old patient, 30 years after the operation for TOF.

Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot

Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.

Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot

Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot

Valve-Sparing Options in Tetralogy of Fallot Surgery

Given late outcomes of patients with tetralogy of Fallot repaired in the 1970s and 1980s, as well as a better understanding of the late deleterious effects of pulmonary regurgitation, there is a tendency toward preservation of the pulmonary valve function during primary repair of tetralogy of Fallot. The bar keeps moving downward, to include smaller and more dysmorphic pulmonary valves. This article reviews some useful indications and techniques for valve-sparing options, including intraoperative balloon dilation and cusp reconstruction using a patch. Just like other valve repair techniques, no one technique can be applied uniformly, and surgeons must master a wide armamentarium of techniques.

Airway pressure release ventilation improves pulmonary blood flow in infants after cardiac surgery*

We hypothesized that spontaneous inspiratory effort transmitted to the pleural space during airway pressure release ventilation would result in increased lung perfusion after surgery for tetralogy of Fallot or following a cavopulmonary shunt as a consequence of transient decreases in intrapleural pressure. Prospective crossover cohort study. A tertiary care cardiac pediatric intensive care unit. Children after tetralogy of Fallot repair, cavopulmonary shunt, or Fontan operation. Lung perfusion and cardiac output were measured during airway pressure release ventilation and pressure control ventilation with pressure support, both with and without spontaneous ventilation. Oxygen consumption was measured (mass spectrometer) and lung perfusion/cardiac output calculated (Fick equation). Constant levels of CO₂ and mean airway pressure were targeted in all study phases. Twenty patients were enrolled in the study, nine after repair of tetralogy of Fallot and 11 after a cavopulmonary shunt. In the absence of spontaneous ventilation, there were no differences in lung perfusion or any of the measured gas exchange or hemodynamic parameters. In the presence of spontaneous ventilation for all patients, mean pulmonary blood flow increased from 2.4 to 2.9 L·min⁻¹M⁻² (p = .02). Oxygen delivery increased from 594 to 774 mL/min/m² (p = .05) in the patients with tetralogy of Fallot patients and from 473 to 518 L·min⁻¹M⁻² (p = .07) in the cavopulmonary shunt group. Ventilation with airway pressure release ventilation (at comparable mean airway pressure) improves lung perfusion compared with pressure control ventilation in children after tetralogy of Fallot repair and cavopulmonary shunt operations. Although this study focused on tetralogy of Fallot and cavopulmonary shunt operations, the improved cardiopulmonary interactions may be beneficial in other situations in which hemodynamics are impaired by positive pressure ventilation.

The Role of Cardiovascular Magnetic Resonance in Adults with Congenital Heart Disease

The comprehensive coverage and versatility of cardiovascular magnetic resonance (CMR), providing functional as well as anatomical information, make it an important facility in a center specializing in the care of adults with congenital heart disease. Imaging specialists using CMR to investigate acquired heart disease should also be able to recognize and evaluate previously unsuspected congenital malformations. Conditions that may present or be picked up during imaging in adulthood include atrial septal defect, anomalously connected pulmonary veins, double-chambered right ventricle, congenitally corrected transposition of the great arteries, aortic coarctation, and patent arterial duct. To realize its full potential and to avoid pitfalls, CMR of adults with congenital heart disease requires specific training and experience. Appropriate pathophysiological understanding is needed to evaluate cardiovascular function after surgery for tetralogy of Fallot, after transposition of the great arteries, and after Fontan operations. For these and other more complex cases, CMR should ideally be undertaken by specialists committed to long-term collaboration with the clinicians and surgeons managing the patients in a tertiary referral center.