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.
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.
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.
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.

暂无摘要。

Transthoracic echocardiography is the first-line imaging modality in the assessment of right-sided valve disease. The principle objectives of the echocardiographic study are to determine the aetiology, mechanism and severity of valvular dysfunction, as well as consequences on right heart remodelling and estimations of pulmonary artery pressure. Echocardiographic data must be integrated with symptoms, to inform optimal timing and technique of interventions. The most common tricuspid valve abnormality is regurgitation secondary to annular dilatation in the context of atrial fibrillation or left-sided heart disease. Significant pulmonary valve disease is most commonly seen in congenital heart abnormalities. The aetiology and mechanism of tricuspid and pulmonary valve disease can usually be identified by 2D assessment of leaflet morphology and motion. Colour flow and spectral Doppler are required for assessment of severity, which must integrate data from multiple imaging planes and modalities. Transoesophageal echo is used when transthoracic data is incomplete, although the anterior position of the right heart means that transthoracic imaging is often superior. Three-dimensional echocardiography is a pivotal tool for accurate quantification of right ventricular volumes and regurgitant lesion severity, anatomical characterisation of valve morphology and remodelling pattern, and procedural guidance for catheter-based interventions. Exercise echocardiography may be used to elucidate symptom status and demonstrate functional reserve. Cardiac magnetic resonance and CT should be considered for complimentary data including right ventricular volume quantification, and precise cardiac and extracardiac anatomy. This British Society of Echocardiography guideline aims to give practical advice on the standardised acquisition and interpretation of echocardiographic data relating to the pulmonary and tricuspid valves.

There is no systematic evidence review of the long-term results of surgical pulmonary valve replacement (PVR) dedicated to adults with repaired tetralogy of Fallot (rTOF) and pulmonary regurgitation.
Our primary objective was to determine whether PVR reduced long-term mortality in adults with rTOF compared with conservative therapy. Secondary objectives were to determine the postoperative incidence rate of death, the changes in functional capacity and in right ventricular (RV) volumes and ejection fraction after PVR, and the postoperative incidence rate of sustained ventricular arrhythmias. A systematic search of multiple databases for studies was conducted without limits.
No eligible randomized controlled trial or cohort study compared outcomes of PVR and conservative therapy in adults with rTOF. We selected 10 cohort studies (total 657 patients) reporting secondary outcomes. After PVR, the pooled incidence rate of death was 1% per year (95% confidence interval [CI] 0-1% per year) and the pooled incidence rate of sustained ventricular arrhythmias was 1% per year (95% CI 1%-2% per year). PVR improved symptoms (odds ratio for postoperative New York Heart Association functional class > II 0.08, 95% CI 0.03-0.24). Indexed RV end-diastolic (-61.29 mL/m2, -43.64 to -78.94 mL/m2) and end-systolic (-37.20 mL/m2, -25.58 to -48.82 mL/m2) volumes decreased after PVR, but RV ejection fraction did not change (0.19%, -2.36% to 2.74%). The effect of PVR on RV volumes remained constant regardless of functional status.
Studies comparing PVR and conservative therapy exclusively in adults with rTOF are lacking. After PVR, the incidence rates of death and ventricular tachycardia are both 1 per 100 patient-years. Pooled analyses demonstrated an improved functional status and a reduction in RV volumes.

暂无摘要。

暂无摘要。

The authors sought to study variation in the practice of balloon aortic (BAV) and pulmonary valvuloplasty (BPV).
The IMPACT (IMProving Adult and Congenital Treatment) registry provides an opportunity to study practice variation in transcatheter interventions for congenital heart disease.
The authors studied BAV and BPV in the IMPACT registry from January 1, 2011, to September 30, 2015, using hierarchical multivariable models to measure hospital-level variation in: 1) the distribution of indications for intervention; and 2) in cases with \"high resting gradient\" as the indication, consistency with published guidelines.
A total of 1,071 BAV cases at 60 hospitals and 2,207 BPV cases at 75 hospitals were included. The indication for BAV was high resting gradient in 82%, abnormal stress test or electrocardiogram (2%), left ventricular dysfunction (11%), and symptoms (5%). Indications for BPV were high resting gradient in 82%, right-left shunt (6%), right ventricular dysfunction (7%), and symptoms (5%). No association between hospital characteristics and distribution of indications was demonstrated. Among interventions performed for \"high resting gradient,\" there was significant adjusted hospital-level variation in the rates of cases performed consistently with guidelines. For BAV, significant differences were seen across census regions, with hospitals in the East and South more likely to practice consistently than those in the Midwest and West (p = 0.005). For BPV, no association was found between hospital factors and rates of consistent practice, but there was significant interhospital variation (median rate ratio: 1.4; 95% confidence interval: 1.2 to 1.6; p < 0.001).
There is measurable hospital-level variation in the practice of BAV and BPV. Further research is necessary to determine whether this affects outcomes or resource use.

暂无摘要。

暂无摘要。

暂无摘要。