Management of adults with Tetralogy of Fallot

2013 ◽  
Vol 23 (6) ◽  
pp. 921-932 ◽  
Author(s):  
Richard M. Martinez ◽  
Jeremy M. Ringewald ◽  
Hector L. Fontanet ◽  
James A. Quintessenza ◽  
Jeffrey P. Jacobs
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Heart Association ◽  
Pulmonary Insufficiency ◽  
Multiple Sources ◽  
Multiple Components ◽  
Congenital Cardiac Disease ◽  
European Society Of Cardiology ◽  
New Strategies

AbstractTetralogy of Fallot is the most common form of cyanotic congenital cardiac disease. Patients with previously repaired tetralogy of Fallot are the most common patients seen in the Program for Adults with Congenital Heart Disease at The Johns Hopkins All Children's Heart Institute. Guidelines for the management of these patients are available from multiple sources including The American College of Cardiology (ACC) and The American Heart Association (AHA), The Canadian Cardiovascular Society, and The European Society of Cardiology (ESC). These guidelines describe multiple components related to the care for these patients including strategies for medical follow-up, the management of arrhythmias and electrophysiological diseases, and the treatment of chronic pulmonary insufficiency and stenosis. Several new strategies are available for replacement of the pulmonary valve including transcatheter replacement of the pulmonary valve and replacement of the pulmonary valve with a self-manufactured bicuspid polytetrafluoroethylene pulmonary valve.

scholarly journals Fresh Autologous Pericardium to Reconstruct the Pulmonary Valve at the Annulus When Tetralogy of Fallot Requires a Transannular Patch at Midterm

2016 ◽  
Vol 43 (3) ◽  
pp. 207-213 ◽  
Author(s):  
Shantanu Pande ◽  
Jugal K. Sharma ◽  
C.R. Siddartha ◽  
Anubhav Bansal ◽  
Surendra K. Agarwal ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Right Ventricular Dysfunction ◽  
Pulmonary Insufficiency ◽  
Autologous Pericardium ◽  
Transannular Patch ◽  
Significant Difference

Tetralogy of Fallot often requires reconstruction of the right ventricular outflow tract with a transannular patch (TAP), but this renders the pulmonary valve incompetent and eventually leads to right ventricular dysfunction. We retrospectively evaluated the efficacy of a reconstructed pulmonary valve and annulus in 70 patients who underwent, from December 2006 through December 2010, complete correction of tetralogy of Fallot. We divided the 70 patients into 2 groups in accordance with whether they required (n=50) or did not require (n=20) a TAP. We used autologous untreated pericardium to fashion the TAP and to create both an annulus of the correct size and a competent pulmonary valve with native leaflets. We evaluated the efficiency of this procedure both functionally and anatomically. The median age of the patients was 11 years (range, 2–38 yr). There were 56 males, with no significant difference in sexual distribution between groups. The clinical follow-up was 88% for 57.5 months, and the echocardiographic follow-up was 80% for 36 months. There was no significant difference in outflow gradient or in the occurrence of pulmonary insufficiency between the TAP group (none, 31; mild, 12; moderate, 6; and severe, 1) and the No-TAP group (none, 16; moderate, 2; and severe, 2) (P=0.59). Nor was there any thickening or calcification in the constructed valves. We conclude that pulmonary valves constructed of untreated autologous pericardium performed as well as native valves after total tetralogy of Fallot correction at midterm.

scholarly journals Risk of infective endocarditis among patients with tetralogy of fallot

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Havers-Borgersen ◽  
J.H Butt ◽  
M Groening ◽  
M Smerup ◽  
G.H Gislason ◽  
...  
Keyword(s):  
Infective Endocarditis ◽  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Pulmonary Valve ◽  
Cox Regression ◽  
Pulmonary Valve Replacement ◽  
Varying Coefficients ◽  
Background Population ◽  
Increased Risk

Abstract Introduction Patients with tetralogy of Fallot (ToF) are considered at high risk of infective endocarditis (IE) as a result of altered hemodynamics and multiple surgical and interventional procedures including pulmonary valve replacement (PVR). The overall survival of patients with ToF has increased in recent years. However, data on the risk of adverse outcomes including IE are sparse. Purpose To investigate the risk of IE in patients with ToF compared with controls from the background population. Methods In this nationwide observational cohort study, all patients with ToF born in 1977–2017 were identified using Danish nationwide registries and followed from date of birth until occurrence of an outcome of interest (i.e. first-time IE), death, or end of study (July 31, 2017). The comparative risk of IE among ToF patients versus age- and sex-matched controls from the background population was assessed. Results A total of 1,156 patients with ToF were identified and matched with 4,624 controls from the background population. Among patients with ToF, 266 (23.0%) underwent PVR during follow-up. During a median follow-up time of 20.4 years, 38 (3.3%) patients and 1 (0.03%) control were admitted with IE. The median time from date of birth to IE was 10.8 years (25th-75th percentile 2.8–20.9 years). The incidence rates of IE per 1,000 person-years were 2.2 (95% confidence interval (CI) 1.6–3.0) and 0.01 (95% CI 0.0001–0.1) among patients and controls, respectively. In multivariable Cox regression models, in which age, sex, pulmonary valve replacement, and relevant comorbidities (i.e. chronic renal failure, diabetes mellitus, presence of cardiac implantable electronic devices, other valve surgeries), were included as time-varying coefficients, the risk of IE was significantly higher among patients compared with controls (HR 171.5, 95% CI 23.2–1266.7). Moreover, PVR was associated with an increased risk of IE (HR 3.4, 95% CI 1.4–8.2). Conclusions Patients with ToF have a substantial risk of IE and the risk is significantly higher compared with the background population. In particular, PVR was associated with an increased risk of IE. With an increasing life-expectancy of these patients, intensified awareness, preventive measures, and surveillance of this patient group are advisable. Figure 1. Cumulative incidence of IE Funding Acknowledgement Type of funding source: None

scholarly journals Mid- to long-term follow-up of pulmonary valve replacement with BioIntegral injectable valve

2020 ◽  
Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi
Keyword(s):  
Cardiopulmonary Bypass ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Pulmonary Valve Replacement

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.

scholarly journals One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy

2018 ◽  
Vol 82 (12) ◽  
pp. 3064-3068
Author(s):  
Jae Gun Kwak ◽  
Woong-Han Kim ◽  
Eung Re Kim ◽  
Jae Hong Lim ◽  
Jooncheol Min
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
One Year

Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair

2017 ◽  
Vol 8 (2) ◽  
pp. 189-195
Author(s):  
Elizabeth H. Stephens ◽  
Jennifer Tingo ◽  
Marc Najjar ◽  
Betul Yilmaz ◽  
Stéphanie Levasseur ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Right Ventricular Outflow Tract ◽  
Patch Repair ◽  
Perioperative Outcomes ◽  
Pulmonary Insufficiency ◽  
Atrioventricular Canal ◽  
Atrioventricular Valve Regurgitation ◽  
Valve Sparing ◽  
Complete Atrioventricular

Background: Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention. Methods: We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014). A two-patch repair was used in all patients to correct the CAVC defect. Tetralogy of Fallot repair included transannular patch in 11 (65%) patients and valve-sparing in 6 (35%) patients. Results: The average age at surgery was 72 ± 122 weeks, 40% were male, and 80% had trisomy 21. Mean echo follow-up was 3.0 ± 3.0 years. There were no in-hospital or late mortalities. The rate of reoperation was 20%. At the latest follow-up, moderate left atrioventricular valve regurgitation was present in three (15%) patients and mild stenosis present in seven (35%) patients. One (5%) patient had moderate right ventricular outflow tract (RVOT) obstruction. The valve-sparing population was smaller at the time of surgery than the non-valve-sparing cohort (body surface area: 0.28 ± 0.04 vs 0.42 ± 0.11, P = .002) and less likely to have had a previous shunt (0% vs 64%, P = .01). Among the valve-sparing patients (six), at the latest follow-up, moderate pulmonary insufficiency was present in two (33%) patients. Conclusion: Repair of CAVC concomitant with TOF can be performed with low mortality and acceptable perioperative morbidity. Management of the RVOT remains a challenge for the long term.

scholarly journals Follow-Up After Pulmonary Valve Replacement in Adults With Tetralogy of Fallot

2010 ◽  
Vol 56 (18) ◽  
pp. 1486-1492 ◽  
Author(s):  
Roderick W.C. Scherptong ◽  
Mark G. Hazekamp ◽  
Barbara J.M. Mulder ◽  
Olivier Wijers ◽  
Cees A. Swenne ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Pulmonary Valve ◽  
Pulmonary Valve Replacement
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