Search

Objective: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice., Methods: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement., Results: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation., Conclusions: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Expected benefits of modified ultrafiltration (MUF) include increased hematocrit, reduction of total body water and inflammatory mediators, improved left ventricular systolic function, and improved systolic blood pressure and cardiac index (CI) following cardiopulmonary bypass (CPB). This prospective randomized trial tested this hypothesis., Methods: Seventy-nine patients undergoing intracardiac repair of tetralogy of fallot were randomized to conventional ultrafiltration (CUF) + MUF (n = 39) or only CUF group (n = 40). The primary outcome was a change in hematocrit. Secondary outcomes were changes in peak airway pressures, ventilatory support, blood transfusions, time to peripheral rewarming, mean arterial pressure, central venous pressure, inotrope score (IS), and CI. Serum inflammatory markers were measured., Results: Baseline hematocrit was 50.6 ± 10.02 in the only CUF group whereas it was 43.9 ± 5.55 in the CUF + MUF group (p = .36). Following MUF, the CUF + MUF group had higher hematocrit (44.7 ± 0.50 g/dl) compared to the only CUF group (37.2 ± 0.49 g/dl), p ≤ .001 after adjusting for baseline hematocrit. Central venous pressure (mmHg) immediately following sternal closure was 9.27 ± 3.12 mmHg in the CUF + MUF group and 10.52 ± 2.2 mmHg in the only CUF group (p = .04). In the intensive care unit (ICU), they were 11.52 ± 2.20 mmHg in the only CUF group and 10.84 ± 2.78 mmHg in the CUF + MUF group (p = .02). Time to peripheral rewarming was 6.30 ± 3.91 h in the CUF + MUF group and 13.67 ± 3.91 h in the only CUF group (p = .06). Peak airway pressures in ICU were 17 ± 2 mmHg versus 20.55 ± 2.97 mmHg in CUF + MUF group & only CUF group, respectively, p < .001). Duration of mechanical ventilation was 6.3 ± 2.7 h in CUF + MUF group compared to 14.7 ± 3.5 h in the only CUF group (p = .002). IS was 11.52 ± 2.20 in the only CUF group compared to 10.84 ± 2.78 in CUF + MUFs group. Eight of 39 (20.5%) patients in the CUF + MUF group had IS > 10 compared to 22 of 40 (55%) patients in the only CUF group (p = .02). Serum Troponin-T and interleukin-6 levels were lower in the CUF + MUF group; TNF-α and CPK-MB were similar. ICU and hospital stay were similar., Conclusion: Patients undergoing a combination of CUF and MUF had higher postoperative hematocrit, decreased duration of mechanical ventilation, lower need for inotropes and lower interleukin-6 and Troponin-T levels. This group had better postoperative outcomes. This study was registered with the Clinical trials registry of India (CTRI/2017/11/010512) before commencement., (© 2021 Wiley Periodicals LLC.)

A 14-year-old girl admitted for corrective surgery for tetralogy of Fallot was found to have pulmonary arteriovenous malformations on pre-operative evaluation. In anticipation of a complicated postoperative course, percutaneous closure of the pulmonary arteriovenous malformations was undertaken first followed by a successful surgical outcome. Importance of this rare association and its clinical implication is hereby highlighted.

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed., (© 2019 Wiley Periodicals, Inc.)

A combination of ruptured sinus of Valsalva (RSOV) aneurysm with tetralogy of Fallot (TOF) is a very rare condition. We report a 32-year-old male who underwent repair of a RSOV aneurysm with TOF., (© 2018 Wiley Periodicals, Inc.)

Objective: To determine effects of allopurinol administration on outcomes following intracardiac repair of tetralogy of Fallot (TOF)., Materials and Methods: Fifty patients undergoing TOF repair were randomized to 2 groups of 25 each: the allopurinol group (n = 25) and the placebo group (n = 25). Postoperatively, inotropic score, rhythm, duration of mechanical ventilation, cardiac output, intensive care unit (ICU) stay, and hospital stay were assessed. Plasma troponin-I, superoxide dismutase (SOD), interleukin (IL) 1-ß, IL-6, and malondialdehyde were measured serially., Results: Inotropic score was lower in the allopurinol compared with placebo group (11.04 ± 5.70 vs 17.50 ± 7.83; P = .02). Duration of ICU and hospital stay was lower in the allopurinol group. Plasma levels of SOD preoperative were (2.87 ± 1.21 U/mL vs 4.5 ± 2.08 U/mL; P = .012), immediately following release of crossclamp (2.32 ± 0.98 U/mL vs 5.32 ± 2.81 U/mL; P < .001), and after termination of CPB (2.18 ± 1.0.78 U/mL vs 3.44 ± 1.99 U/mL; P = .003) between the placebo versus allopurinol group, respectively. Postoperative levels of IL1-ß and IL-6 were lower in the allopurinol group. Malondialdehyde levels following CPB were lower in the allopurinol group (11.80 ± 2.94 pg/mL in the placebo vs 9.16 ± 3.02 g/mL in the allopurinol group; P < .001)., Conclusions: Allopurinol administration in patients undergoing intracardiac repair of TOF is associated with reduced inotropic scores, duration of mechanical ventilation, ICU stay, and hospital stay and favorable biochemical markers of inflammation. Further studies in multiple setups are needed before recommending it as a routine practice., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression., Methods: We performed a retrospective review of 73 consecutive patients who underwent repair for TOF/APV between January 2005-August 2015. Mean age was 6.4±5.6years (28days-22years). The right ventricular outflow tract (RVOT) was reconstructed using varied techniques. Freedom from RVOT gradients and re-operation was studied., Results: There were four (5.5%) early deaths, two each in infants and older children. Median ICU stay was 2days (range, 1-12days). Mean ICU stay for, infants, children and adults, was 6.5±6.04, 2.75±2.45, and 2.33±1.03days, respectively (p=0.0762). Median hospital stay was 6days (range, 4 to 15days). Mean hospital stay for, infants and children and adults was 7±2, 6.75±2.39, and 6.33±1.63days, respectively (p=0.325). Mean follow up was 65±36.6months (median 56 months, range 7-126 months). On follow up echocardiography, 14 (21.21%) had no pulmonary regurgitation. 21 (31.81%) had mild PR patients, 8 (12.12%) moderate PR and 19 (28.78%) had severe PR. There were five (7.5%) reoperations. Five and ten-year survival was 95%±2.12 and 92.3%±3.45 respectively. Freedom from RVOT reoperation was 93±2.62% and 89±3.87% at 5 and 10 years., Conclusions: In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood., (Copyright © 2017. Published by Elsevier B.V.)

We conducted a prospective randomized trial to compare del Nido (DN) cardioplegia with conventional cold blood cardioplegia (St Thomas [STH]) in pediatric patients. We randomized 100 pediatric patients aged ≤12 years undergoing elective repair of ventricular septal defects and tetralogy of Fallot to the DN and the STH groups. In the DN group, a 20 mL/kg single dose was administered. In the STH group, a 30 mL/kg dose was administered, followed by repeated doses at 25- to 30-minute intervals. The primary outcome was cardiac index that was measured 4 times intra- and postoperatively. Troponin-I, interleukin-6, and tissue necrosis factor-alpha were measured. Myocardial biopsy was obtained to assess electron-microscopic ultrastructural changes. Cardiac indices were significantly higher in the DN group than in the STH group 2 hours after termination of cardiopulmonary bypass (P = 0.0006), after 6 hours (P = 0.0006), and after 24 hours (P ≤ 0.0001). On repeated measure regression analysis, the cardiac index was on an average 0.50 L/min/m 2 higher in the DN group than in the STH group at any time point (P = 0.002). Duration of mechanical ventilation (P = 0.01), intensive care unit stay (P = 0.01), and hospital stay (P = 0.0007) was significantly lower in the DN group. Patients in the DN group exhibited lower troponin-I release 24 hours following cardiopulmonary bypass (P = 0.021). Electron microscopic studies showed more myofibrillar disarray in the STH group (P = 0.02). Use of long-acting DN cardioplegia solution was associated with better preservation of cardiac index, lesser troponin-I release, and decreased morbidity. Ultrastructural changes showed better preservation of myofibrillar architecture., (Copyright © 2017 Elsevier Inc. All rights reserved.)

A 30-month-old female was admitted with recurrent spells and severe cyanosis. Preoperative echocardiography was diagnostic of tetralogy of Fallot with an atrial septal defect of the primum type, unroofed coronary sinus, and a left superior vena cava draining into the left atrium. At surgery the patient was found to have a complete atrioventricular septal defect in addition to these anomalies. Complete anatomical correction was achieved through the right atrial approach., (© 2015 Wiley Periodicals, Inc.)

Surgical correction of patients with tetralogy of Fallot with pulmonary atresia is now one of the routine procedures performed by pediatric cardiac surgeons. In one variant, the pulmonary arterial supply is derived from a fistulous communication from the coronary arteries. This rare and interesting situation poses a diagnostic and therapeutic dilemma, as well as providing specific management challenges to the surgical team. Here, we discuss important aspects of this rare variant, specifically its morphology, presentation, evaluation and management., (© The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Objective: 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., Design: Retrospective cohort., Setting: Multispeciality tertiary level referral center, Patients: Forty-one (age 30-52 years) with TOF undergoing surgery between January 2002 and March 2013. The hospital records of these patients were analyzed., Interventions: None., Outcome Measures: Early and late morbidity and mortality, duration of mechanical ventilatory support, inotropic score, intensive care unit and hospital stay, and correlation with various parameters., Results: 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., Conclusion: Repair of TOF in and beyond the fourth decade of life is feasible with acceptable results. Patients with high hematocrit, lower oxygen saturation, right ventricular dysfunction, aortopulmonary collaterals, and high preoperative right ventricular outflow tract gradients have a prolonged postoperative course., (© 2014 Wiley Periodicals, Inc.)

Objectives: Tetralogy of Fallot (TOF) with hemitruncus (HT) is a rare entity. In this report, we present our experience with this condition over the last 20 years., Methods: Between January 1994 and June 2013, 11 patients with HT and TOF underwent surgery at the All India Institute of Medical Sciences, New Delhi, India. All available clinical, radiographic, echocardiographic, cardiac catheterization, operative and follow-up data were reviewed., Results: The mean age was 73 ± 7.1 months (range 7 months to 18 years) and the mean weight was 15.7 ± 1.2 kg. The mean preoperative saturation was 79.3 ± 11.7% (range 62-92%). Six patients had anomalous left pulmonary artery (PA), whereas 5 had an anomalous right PA arising from the aorta. Surgical procedures consisted of complete intracardiac repair of TOF with direct implantation of the anomalous PA into the main PA (n = 7), intracardiac repair of TOF with an interposition saphenous vein graft between the right PA and main PA (n = 1), and reconstruction of the left PA with autologous pericardium with intracardiac repair of TOF (n = 1), direct implantation of the anomalous PA into the main PA with an innominate to right pulmonary artery shunt (n = 1) and a right PA banding with innominate to left PA shunt (n = 1). There were two early deaths. Follow-up ranged from 3 to 73 months. All survivors are in NYHA Class I and follow-up echocardiograms did not show any residual lesions., Conclusions: Surgical repair of HT with TOF results in acceptable early outcomes. The surgical strategy needs to be individualized to the anatomy of the patient., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We report a 3-year-old patient with tetralogy of Fallot (TOF), dextrocardia, situs inversus (SI), and total anomalous pulmonary venous return. This combination of anomalies is extremely unusual. The management is discussed and the literature is reviewed., (© 2013 Wiley Periodicals, Inc.)

In medical schools worldwide, the traditional clinical approach is based on the law of parsimony. Physicians in daily clinical practice attempt to fulfill this very simplified yet complex concept of unifying the diagnosis in a given clinical scenario. However, failures are not uncommon. This report presents a case of tetralogy of Fallot who presented with heart failure at young age of 18 months. This is the first description of coexisting tubercular chronic constrictive pericarditis and tetralogy of Fallot. This case is reported for its unique association of two relatively uncommon clinical entities. The logistic dictum of Hickam in similar circumstances is discussed.

Coronary arteries to pulmonary artery fistulas are rare in patients with Tetralogy of Fallot. Even rarer is the occurrence of these fistulas from a solitary coronary system. We report one such patient in whom the preoperative diagnosis was missed. The importance of a high index of suspicion for accurate preoperative diagnosis is discussed.

Two cases of tetralogy of Fallot (TOF) with double aortic arch and absent left pulmonary artery are reported. Single-stage repair was performed using a left thoracotomy for division of the non-dominant arch followed by complete repair through a median sternotomy. In both patients, the left pulmonary artery was reconstructed using either a homograft saphenous vein or a homograft common iliac artery.

We report a patient with tetralogy of Fallot, total anomalous pulmonary venous return to the right atrium, large atrial septal defect, anomalous course and termination of the superior vena cava, anomalous origin and course of the right coronary artery, and a defect in the diaphragm. This interesting combination of anomalies was managed successfully.

Background: The association of tetralogy of Fallot with total anomalous pulmonary venous drainage (TAPVD) is rare. We report our experience with this condition and review the literature., Methods: Between January 1997 and May 2008, 6 patients (aged 3 months to 5 years; median weight, 10 kg) with combined tetralogy of Fallot with TAPVD underwent complete primary repair at All India Institute of Medical Sciences, New Delhi, India. Their records were retrospectively reviewed., Results: A correct preoperative diagnosis was available in 5 patients by echocardiography and cardiac catheterization. Four patients had supracardiac TAPVD, and 1 each had TAPVD to the coronary sinus and right atrium. There were no early or late deaths. Median follow-up was 37.5 months +/- 46.8 months (range, 2 to 112). All patients are in New York Heart Association class I. Follow-up echocardiograms have revealed no significant abnormalities and have documented normal biventricular function and pulmonary artery pressures. One patient underwent a 24-hour Holter examination at 68 months of follow-up for investigation of a new-onset 2:1 atrioventricular block, for which a permanent pacemaker implantation is planned., Conclusions: When diagnosed accurately, complete primary repair is possible in patients with tetralogy of Fallot and TAPVD and the outcomes are satisfactory with planned surgery.

Background: The aim of this study was to analyze the feasibility and early results of transatrial total correction of tetralogy of Fallot (TOF)., Methods: Of the 860 patients undergoing total correction for TOF between January 2000 and July 2005, 334 patients were considered morphologically suitable for transatrial total correction. The ventricular septal defect (VSD) closure, infundibular resection, and pulmonary valvotomy were performed through the right atrium without a right ventriculotomy. Age ranged from 6 months to 40 years (median, 2.8 years), and weight ranged from 5.5 to 70 kg (median, 14 kg)., Results: Peroperatively, 34 patients required right ventriculotomy and transannular patch; hence, they were excluded from the study. In addition, pulmonary arteriotomy was required in 71 patients (22.9%). There were 4 hospital deaths. There were 4 early reoperations (residual/additional VSD in 3 and tricuspid regurgitation in 1). Two patients had complete heart block requiring permanent pacemaker. Echocardiography at discharge showed a peak right ventricular outflow tract gradient of 20 +/- 5.2 mm Hg. Mean follow-up was 26.8 +/- 4.2 months (range, 1 to 52 months). The right ventricular outflow tract gradients reduced to 13 +/- 4.2 mm Hg after a mean interval of 18.8 +/- 5.2 months. Follow-up New York Heart Association class was I in 240 cases (82%), II in 49 (16%), and III in 7 (2%). There were no late deaths or reoperations., Conclusions: Transatrial total correction of TOF can be accomplished in selected patients with good early results. In 300 cases (90%), the feasibility of transatrial total correction could be predicted accurately.

A coronary artery crossing the right ventricular outflow tract is a subset of a larger pathomorphological cohort known as an anomalous coronary artery (ACA) in the tetralogy of Fallot (TOF). The best possible outcome in a patient with TOF and ACA is decided by judicious selection of optimum preoperative investigative information, the timing of surgery, astute assessment of preoperative surgical findings, and appropriate surgical technique from a wide array of choices. In most instances, the choice of surgical technique is determined by the size of the pulmonary annulus and the anatomical relation of ACA to the pulmonary annulus. In the present era, complete, accurate preoperative diagnosis and primary repair is a routine procedure with strategies to avoid a right ventricle-to-pulmonary artery conduit. [ABSTRACT FROM AUTHOR]

The authors comment on the single-stage approach to pulmonary atresia (PA), ventricular septal defect (VSD) and major aorto pulmonary collaterals (MAPCA). They describe VSD as the severest form of tetrology of Fallot. Increasing the central pulmonary artery flow is one of the aims of the staged approach. They point out that individualization of the approach to any given patient is needed due to extreme variation in the anatomy.

Abstract Objectives We conducted a prospective randomized controlled trial to compare del Nido (DN) and histidine-tryptophan-ketoglutarate (HTK) cardioplegia solution in pediatric patients undergoing intracardiac tetralogy of Fallot repair. Methods One hundred consecutive patients 12 years of age or younger, undergoing intracardiac repair of tetralogy of Fallot were randomized into DN (n = 50) and HTK (n = 50) groups. Cardioplegia strategy consisted of a single dose of DN (20 mL/kg) or HTK (6 mL/kg/min for 6 minutes). Primary outcome was cardiac index (CI). Secondary outcomes were ventricular arrhythmias post cross-clamp release, time to peripheral rewarming, duration of mechanical ventilation, inotropic score, intensive care unit and hospital stay, and serum levels of troponin-I, interleukin-6, and tumor necrosis factor-α. Ultrastructural changes in the myocardium were assessed. Results CI was significantly higher in the DN group compared with the HTK group at 6 (P =.005) and 24 hours (P <.001) after surgery. It was on an average 0.44 L/min/m2 higher in the DN group at any time point (P =.004). Time for complete cessation of electrical activity was longer in the HTK group (P =.01) and more patients in the HTK group had ventricular arrhythmias post cross-clamp release (P =.03). Duration of mechanical ventilation (P =.006), intensive care unit stay (P =.05), and hospital stay (P <.001) were lower in the DN group. Patients in the DN group had lower troponin I levels 24 hours after cardiopulmonary bypass (P <.001). Electron microscopic studies showed more myocardial edema (P =.02) and myofibrillar disarray (P =.04) in the HTK group along with lower glycogen stores (P =.04). DN cardioplegia was more cost-effective than HTK cardioplegia (P <.001). Conclusions DN cardioplegia was associated with better preservation of CI, less duration of mechanical ventilation, shorter intensive care unit and hospital stays, lower inotropic scores, and less release of troponin-I. Electron microscopy showed less myocardial edema and better preservation of the myofibrillar architecture and glycogen stores in the DN group. [ABSTRACT FROM AUTHOR]