Corrected Tetralogy Of Fallot Research Articles

Risk Factors of Prolonged Postoperative Pleural Effusion After Repair of Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and total correction is the definitive treatment. Chest tube drainage of pleural effusion (PE) is essential after surgery. Prolonged PE (> 7 days) is one of the complications; it may increase hospital stay and the risks of morbidity and mortality. The aim of this study was to investigate and analyze the possible risk factors for prolonged PE after total correction of TOF. Thirty-seven patients who received total correction of TOF between July 1999 and April 2001 were included in this study. They were divided into 2 groups according to the duration of chest tube drainage for postoperative PE: Group I had postoperative PE < or = 7 days; Group II had postoperative PE > 7 days. Detailed records were taken on patients' demographic characteristics, blood parameters, surgery, electrocardiographic and radiologic data, and angiographic and echocardiographic findings. The data of the 2 groups were compared using the Wilcoxon rank-sum test and Fisher's exact test. Risk factors were analyzed by logistic regression and model selection. Of the 37 patients, 16 were male and 21 were female. There were 32 patients (86.5%) in Group I and 5 (13.5%) in Group II. Mean patient age at repair was 1.82 +/- 1.29 years (range, 0.53-3.11 years). Significant differences (p < 0.05) between the 2 groups were noted for gender, age at repair, body weight, presence of wound infection, duration on heart-lung machine (bypass time), oxygen saturation before surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. These risk factors were analyzed by logistic regression and model selection. Two models were set up: Model 1--oxygen saturation before surgery, presence of wound infection, age at repair; Model 2--oxygen saturation before surgery, presence of wound infection. Prolonged PE is a significant morbidity after TOF repair. The risk factors for prolonged PE are gender, age at repair, body weight, bypass time, low oxygen saturation before surgery, wound infection after surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. Oxygen saturation before surgery and wound infection were major risk factors while age at repair was a confounder.

Comparison Between QRS Duration at Standard ECG and Signal‐Averaging ECG for Arrhythmic Risk Stratification After Surgical Repair of Tetralogy of Fallot

Surgical repair of tetralogy of Fallot is complicated by the occurrence of ventricular tachycardia (VT). Among different indexes proposed to assess prognosis of these patients, the study of QRS and repolarization provided useful information. Controversial results come from the analysis of signal-averaging ECG (SAECG). The aim of our study was to identify patients operated for tetralogy of Fallot at higher risk of sudden death by means of SAECG. Sixty-six consecutive patients, mean age 26 +/- 10 years, were studied 17.7 +/- 5.8 years after total correction for tetralogy of Fallot using standard ECG, 24-hour Holter recordings, SAECG, and echocardiography. The following variables were measured: standard QRS duration, filtered QRS duration (fQRS), high-frequency and low-amplitude signal duration (HFLA), root mean square of the mean voltage in the terminal portion of filtered QRS (RMS), left and right end-diastolic volumes, and ejection fractions. During a mean follow-up period of 7.3 +/- 3.1 years, 12 patients had episodes of sustained VT and two of them suddenly died. All patients had complete right bundle branch block. Patients with VT were characterized by a significantly longer fQRS duration at all filter settings. On the contrary, there was no difference in standard QRS duration in patients with or without VT. At a multivariate analysis, left ventricular ejection fraction and fQRS were independent predictors for VT. A longer fQRS duration is associated with an increased risk in developing malignant ventricular arrhythmias in asymptomatic patients after total correction of tetralogy of Fallot.

Repair of tetralogy of Fallot with absent pulmonary valve using a new approach

Although several techniques for the correction of tetralogy of Fallot (TOF) with absent pulmonary valve have been described, the method of choice is controversial. Symptomatic infants, in particular, have a poor prognosis because of severe central pulmonary artery dilatation and bronchial compression. An alternative technique for primary repair of TOF with absent pulmonary valve syndrome is suggested. Apart from correction of TOF, this approach includes translocation of the pulmonary artery anterior to the aorta and away from the airways. This technique has a potential to reduce or eliminate bronchial compression by the pulmonary artery. Insertion of valve homograft with anterior and/or posterior plication of the pulmonary artery is considered especially in symptomatic newborns and infants. The intermediate-term functional outcomes have been encouraging, with zero mortality events even in the youngest age group of patients and with the disappearance of respiratory symptoms in the majority of them.

The Significance of P Wave Duration and P Wave Dispersion for Risk Assessment of Atrial Tachyarrhythmias in Patients with Corrected Tetralogy of Fallot

The aim of the present study was to determine the potential role of P wave duration and P wave dispersion for risk assessment of atrial tachyarrhythmias in patients with corrected tetralogy of Fallot (ToF). The maximum P wave duration, minimum P wave duration, and the P wave dispersion from the 12-lead surface electrocardiogram of the patients and controls were measured. Electrophysiological study was performed only in the patient group. The study group consisted of 25 patients with corrected ToF with a mean age of 16.4 +/- 4.25 years and 25 age-matched healthy control subjects. Patients underwent repair at a mean age of 4.6 +/- 3.41 years (range: 1-19), and the mean duration of follow-up of 11.8 +/- 1.7 years (range: 9-15) after surgery. On electrophysiological study sinus node dysfunction was detected in 3 patients (12%), atrial tachyarrythmias-atrial flutter or fibrillation-in 5 patients (20%), both sinus node dysfunction and atrial flutter in 1 patient (4%), and AV conduction delay in 1 patient (4%). P wave dispersion is significantly higher in patients with atrial tachyarrhythmia inducible by electrophysiological study than in other patients (P < 0.05). A P wave dispersion value of >35 ms has a high predictive accuracy (sensitivity = 83% and specificity = 89%) for inducible atrial tachyarrhythmia in patients with corrected tetralogy of Fallot. P wave dispersion is an easily measured electrocardiographic marker with a good sensitivity and specificity for predicting atrial arrhythmias in patients after correction of ToF.

Incidence and indications for reoperations late after correction of tetralogy of fallot

Objectives: Long-term results of corrective surgery of tetralogy of Fallot (TOF) are excellent with most of the patients reaching adulthood. Nevertheless reoperations late after correction may complicate results. The aim of this study was to investigate incidence and indications for reoperations late after correction of TOF.

P9-09 Treadmill exercise study after total correction for tetralogy of Fallot

P9-09 Treadmill exercise study after total correction for tetralogy of Fallot

Right ventricular myocardial dysfunction in adult patients late after repair of tetralogy of fallot

Aim of the study: To detect in adult patients late after repair of Tetralogy of Fallot (TOF) possible correlation between myocardial parameters assessed at rest by Tissue Doppler (TD) and cardiac performance during physical effort. Methods: Doppler echo, treadmill test and pulsed TD of both mitral and tricuspid annulus were performed in 25 healthy subjects and in 40 adult patients who had undergone surgery for TOF at a mean age of 1.4±0.5 years. Exclusion criteria were echocardiographic evidence of residual pulmonary, either stenosis or regurgitation. By use of TD, the following parameters were assessed: systolic peak velocities (Sm), pre-contraction time, contraction time, early ( E m) and late ( A m) diastolic velocities, E m/ A m ratio, relaxation time. By treadmill test, we measured: maximal heart rate (HR), systolic blood pressure (SBP), rate-pressure product, maximal workload, time duration of the exercise. Results: the two groups were comparable for left ventricular measurements and for all transmitral and transtricuspid Doppler indexes, while tricuspid ring diameter was increased in TOF. TD analysis showed in TOF lower S m, E m and E m/ A m ratio and prolonged PCT m and Rt m at tricuspid annulus level, despite comparable TD mitral annulus indexes. By treadmill test, TOF showed reduced time of exercise, number of METS reached and rate-pressure product. Multiple linear regression models evidenced in TOF independent positive association between tricuspid Em velocity and time of exercise ( p<0.0001), achieved METS at peak effort ( p<0.001) and rate-pressure product ( p<0.001). An E m peak velocity of tricuspid annulus lower than 0.13 m/s showed 90% sensitivity and 93% specificity in identifying TOF patients unable to perform maximal exercise test. Conclusions: despite normal Doppler parameters, adult patients late after correction of TOF showed impaired right ventricular myocardial function. In these patients pulsed TD may be taken into account as a valuable supporting tool to predict the effort response and possibly to assess long-term follow-up of cardiac functional reserve.

Endogenously acquired deep sternal wound infection after congenital cardiac surgery

A 10-month-old child from the Middle East received complete correction for tetralogy of Fallot. Despite an initially uneventful postoperative course, he developed severe deep sternal wound infection after 7 days. This complication was endogenously acquired caused by a resistant and rarely present in Europe, Streptococcus pneumonia. Following surgical debridement, betadine rinsing for 3 days and with a course of specific antibiotic therapy, the patient was discharged in good health.

Correction of Tetralogy of Fallot: Does the Time Period of Surgery Influence the Outcome?

The perioperative outcome of patients with tetralogy of Fallot (TOF) seems to have improved over the last four decades. To prove this hypothesis, we retrospectively analysed the data of 269 TOF patients operated on between 1975 and 1999 in our institution. Over the years, younger patients (median age 1975 - 1980: 4.5 years, 1995 - 1999: 0.9 years) were operated on with a lower mortality (1975 - 1980: 8.6 %, 1995 - 1999: 2.4 %). Residual defects such as pulmonary stenosis or insufficiency and VSD occurred with a similar frequency over time, whereas rhythm disturbances were significantly reduced (1981 - 1985: 51.2 %, 1995 - 1999: 24.4 %, p = 0.012). Postoperative length of hospital stay was significantly (p < 0.05) shorter in the years 1995 - 1999 (11.0 - 11.4 days) than in 1975 - 1980 (16.9 +/- 16.5 days). Over time periods, there was a trend towards lower mortality and towards operating on patients in a younger age. The rate of rhythm disturbances and the LOS after surgery proved to be reduced during the last decade. These differences did not turn out to be statistically significant. Therefore, we conclude that the time period of surgery has only little impact on the early outcome of patients after definitive correction of TOF.

Status of paediatric cardiac surgery in China

Epidemiological data show that in China, approximately 100 000 neonates annually are born with congenital heart disease, an incidence equivalent to that found internationally. However, surgical treatment is available to only one-fifth of these individuals. Access to surgery is generally becoming more readily available in the urbanised coastal areas than in the rural interior. Over 1000 procedures annually are performed at the two largest facilities, the Fu Wei Hospital, Beijing, and the Shangai Children's Medical Centre. The 300 paediatric cardiac surgery facilities across China perform approximately 30 000 operations each year, with the quality of treatment varying according to location and procedure type: data on Tetralogy of Fallot correction, for example, show that standards are equivalent to those seen internationally, with a mortality rate of 1.1–5%. For other procedures, although outcomes have improved since the beginnings of Chinese paediatric cardiac surgery in the 1970s, there is still a process of quality improvement, especially for the more severe and complex disorders.

Membranous Subaortic Stenosis Presenting Decades After Surgical Correction for Tetralogy of Fallot

Subaortic stenosis is rare in the middle-aged adult. Its association with tetralogy of Fallot is extremely rare. We report two middle-aged patients, presenting three decades after surgical repair of tetralogy of Fallot, with the rare association of subaortic stenosis. The clinical presentation in each patient mimicked residual pulmonary stenosis and insufficiency, but echocardiography revealed subaortic stenosis with associated valvular aortic regurgitation. (J Am Soc Echocardiogr 1998;11;206–8.)

Tetralogy of Fallot with Absent Pulmonary Valve: A Continuing Challenge

Absent pulmonary valve syndrome (APVS); the combination of tetralogy of Fallot (TOF) with agenesis of the pulmonary valve, is a relatively rare cardiac malformation. Despite the anatomic similarity with classic TOF, the pathophysiology is strikingly different. Data on 10 patients (3 male, 7 female) with APVS, treated between January 1978 and December 1995, were retrospectively reviewed. During this period a total of 2920 children underwent correction of a variety of congenital cardiac anomalies, of which 246 patients (8%) had a correction for TOF. Two patients with APVS presented within the first four months of life with severe cardiorespiratory distress and required several operative procedures. The remaining eight patients had only mild to moderate respiratory and/or cardiac symptoms and elective intracardiac repair was performed on those between the ages of 10 months and 9.5 years. Associated cardiac anomalies seen in five patients included aberrant coronary artery, absent or interrupted left pulmonary artery, partial AVSD and aberrant azygos continuation. In those electively corrected, the strategies used were ventriculotomy (7), pulmonary homograft (3) and aneurysmorrhaphy (2). There were two deaths, one in each group of patients, as a result of progressive respiratory insufficiency and cardiac tamponade, respectively. The follow-up of the eight survivors ranged from 2 to 11 years (median 6.75). All have a normal effort tolerance; only one child is on digoxin therapy, and one child continues to suffer bronchospastis episodes. Our experience with infants with this lesion is limited but underlines the different approaches required, depending on the age of presentation.

Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair

Objectives. We sought to analyze risk factors for long-term survival (up to 36 years) after surgical repair of tetralogy of Fallot (TOF).Background. Survival after repair is excellent, but data >20 years are rare.Methods. From 1958 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 ± 8.6 years [mean ± SD], range 2 to 67), 39.7% had a previous palliation. Operative (n = 139) and 1-year (n = 29) deaths were excluded for long-term calculations, resulting in a study group of 490 patients.Results. Actuarial 10-, 20-, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively. Mortality increased 25 years postoperatively from 0.24%/year to 0.94%/year (p = 0.003). The most common cause of death was sudden death (n = 13), followed by congestive heart failure (n = 6). Multivariate correlates of impaired long-term survival were date of operation (before 1970, p = 0.0104), preoperative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079). Postoperative systolic RV/left ventricular pressure ratio and age showed no influence. Patients without preoperative polycythemia and an RV outflow patch (n = 164) had a 36-year actuarial survival rate of 96% and normal life expectancy.Conclusions. Cyanosis, operative experience of the surgeon and an RV outflow tract patch influence long-term outcome after repair of TOF in older children. Early repair by experienced surgeons to avoid polycythemia and excessive RV hypertrophy is supported by this study. However, mortality risk increases 25 years postoperatively, and thus heart monitoring should be intensified.

Intrathoracic Bifurcation of the Left Common Carotid Artery with a Symptomatic Internal Carotid Artery Stenosis

A forty-eight-year-old woman was evaluated for left hemispheric and transient left monocular symptoms referable to the carotid bifurcation. She was found to have an intrathoracic bifurcation of the left common carotid artery. The angiogram showed a 95% stenosis of the proximal left internal carotid artery arising at an intrathoracic location off a short trunk of the left common carotid artery. She had a history of a Blalock-Taussig shunt and a later total correction of Fallot's tetralogy. Operative correction of the carotid lesion consisted of cervical transposition of the left internal carotid artery to the left external carotid artery. Her postoperative course was uneventful, and follow-up for nearly a year continues to demonstrate a widely patent reconstruction with resolution of her symptoms. The carotid bifurcation may be found at varying locations in the neck; however, a review of the English literature did not disclose any reports of an intrathoracic carotid bifurcation. Given the potential for misdiagnosis, this abnormality should be considered when the bifurcation is not found in its more usual locale on duplex interrogation of this artery.

Right ventricular outflow muscle in tetralogy of Fallot: Histologic and immunohistochemical monoclonal antibody analysis

To evaluate progressive muscular right ventricular outflow tract (RVOT) obstruction in tetralogy of Fallot (TOF), we hypothesized that this tissue would demonstrate more prominent selected immunohistochemical markers of myogenous cell differentiation, growth factor/receptor, and extracellular matrix. Sections of formalin-fixed, paraffin-embedded myectomy tissue obtained from RVOT at the time of surgical correction of TOF ( n = 32; ages = 3 months through 13 years) were compared with age-matched tissue from the RVOT of normal control hearts ( n = 12) obtained at autopsy after non-cardiac death. Examining by light microscopy slides stained with a combination of hematoxylin and eosin and elastic trichrome revealed cardiomyocyte (CM) hypertrophy, extensive myofiber disarray, trabeculation, multinucleation (more than two nuclei per myocyte), fibrosis, and thick-walled coronary arteries within the myocardium of TOF tissue. The endocardium from TOF specimens was thickened and hypercellular with prominent fibrosis and elastosis. Mitosis was not observed. At the interface between the endocardium and myocardium, the TOF specimens demonstrated myocyte dispolarity (orientation of CMs perpendicular to the endocardial surface), vascularization, and fibrosis. Immunohistochemical studies were performed using monoclonal antibodies to vimentin, desmin, muscle-specific actin (MSA), epidermal growth factor (EGF), epidermal growth factor receptor (EGFR), and laminin. Compared to the tissue from controls, TOF tissue showed a pattern of upregulated expression of epitopes within the endocardium and adjacent subendocardial myocardium. Decoration for MSA, vimentin, desmin, and EGFR highlighted the zonal nature of this tissue hyperactivity. Laminin prominently outlined endocardial cells, subendocardial CMs, and interface vessels in TOF tissue compared to the remainder of the myocardium and tissue from controls. Overall, changes in TOF were age-related, with older patients showing less zonal myocardial reactivity. These findings provide evidence for an ongoing, complex remodeling of the RVOT muscle in TOF.

Clinical characteristics of patients over 20 years old without total correction in tetralogy of Fallot

Clinical characteristics of patients over 20 years old without total correction in tetralogy of Fallot

Reoperation for residual defects following intracardiac repair for tetralogy of Fallot

Reoperation was carried out in two patients who had previously undergone correction for tetralogy of Fallot and presented with severe pulmonary hypertension. One patient who had undergone intracardiac repair five years earlier had a residual ventricular septal defect (VSD), right ventricular outflow (RVOT) obstruction, patent ductus arteriosus and severe pulmonary hypertension. The second patient, who had undergone intracardiac repair four years earlier, also had a residual VSD and RVOT obstruction. Both underwent VSD closure, RVOT resection and pulmonary valve replacement and did well postoperatively. We recommend aggressive surgical treatment in such patients even in the presence of pulmonary hypertension.

Life-threatening arterial haemorrhage: Results of treatment by transcatheter embolization using home-made steel coils

Major arterial haemorrhage can be fatal. Its management by percutaneous embolization using commercially available embolizing agents is well established. We describe the use of home-made steel coils in the treatment of five patients. These coils were designed during the procedure in order to conform to the dimension of the bleeding artery. The group included two patients with life-threatening haemoptysis after surgical correction for tetralogy of Fallot; one patient each with exsanguinating haemorrhage from a branch of the left profunda femoral artery following violent trauma; massive haematuria due to an inoperable hypernephroma; and haemoptysis due to post-tubercular bronchiectasis. The bleeding vessel was evaluated by selective angiography in each patient. After assessing its length and diameter, steel coils of desired dimensions were made by utilizing a conventional 0.032-in wide guidewire and these were released into the bleeding artery. Occlusion of the bleeding vessel was obtained in all patients, without complication. Fabrication of coils of desired length and diameter can be done within a few minutes during the procedure. These coils provide an effective method of treating life-threatening arterial haemorrhage in selected cases.

Anesthetic management for a palliative surgical procedure in a 72-year-old patient with tetralogy of Fallot

T ETRALOGY OF Fallot (TOF) is the most common form of congenital heart disease producing a right-toleft intracardiac shunt and arterial hypoxemia. The malformation consists of: (1) pulmonary artery outflow obstruction; (2) a large ventricular septal defect; (3) dextroposition of the aorta (biventricular origin or the aorta); and (4) right ventricular hypertrophy. Intracardiac repair of TOF is usually undertaken during infancy or childhood. However, some patients can reach adulthood without total correction or palliation. Indeed, case reports of patients surviving to the seventh decade of life have been reported. The oldest recorded patient with TOF was 69 years old,’ the oldest patient to have a total correction of TOF was 67 years old,2 and the oldest to undergo a systemic-pulmonary shunt for palliative operation for congenital heart disease was 22 years old.” The anesthetic management of a 72-year-old woman undergoing a modified right Blalock-Taussig shunt for palliation of TOF is reported.

Complete correction of the tetralogy of Fallot in adults using separated extracorporeal circulation to block intrathoracic collateral circulation and improve perfusion of the renal vasculature.

During the last 10 years, complete correction of the tetralogy of Fallot (TOF) has been performed on 28 adult patients using the "separated extracorporeal circulation" (separated ECC) technique developed by us. In addition to the usual ascending aortic and dual right arterial cannulations for ECC, the separated ECC also involved femoral arterial cannulation and the insertion of a catheter with two balloons via the other femoral artery. Flow via the ascending aorta and femoral artery were maintained separately during ECC. The urine output during separated ECC was significantly better than that during ordinary ECC (P < 0.01) and the operative field for intracardiac correction was clearer as a result of blocking the collateral circulation. Thus we believe separated ECC to be a superior method for total correction of TOF in adults, which may enhance the operative outcome.