Tetralogy of Fallot

ESC CardioMed ◽  
2018 ◽  
pp. 814-818
Author(s):  
Robert Yates

Primary repair is now the favoured approach for most patients with tetralogy of Fallot, and has excellent results. Pulmonary regurgitation is the major management issue during long-term follow-up, and late pulmonary valve replacement by surgery or percutaneous approach may be required.

Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi

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.


2014 ◽  
Vol 114 (6) ◽  
pp. 901-908 ◽  
Author(s):  
Anna Sabate Rotes ◽  
Benjamin W. Eidem ◽  
Heidi M. Connolly ◽  
Crystal R. Bonnichsen ◽  
Jordan K. Rosedahl ◽  
...  

2019 ◽  
Vol 57 (4) ◽  
pp. 635-643
Author(s):  
Eva van den Bosch ◽  
Ad J J C Bogers ◽  
Jolien W Roos-Hesselink ◽  
Arie P J van Dijk ◽  
Marie H E J van Wijngaarden ◽  
...  

Abstract OBJECTIVES Our goal was to report the long-term serial follow-up after transatrial–transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome. METHODS We included all patients with TOF who had undergone transatrial–transpulmonary repair between 1970 and 2012. Records were reviewed for patient demographics, operative details and events during the follow-up period (death, pulmonary valve replacement, cardiac reinterventions and hospitalization/intervention for arrhythmias). In patients with elective early primary repair of TOF after 1990, a subanalysis of the optimal timing of TOF repair was performed. RESULTS A total of 453 patients were included (63% male patients; 65% had transannular patch); 261 patients underwent primary elective repair after 1990. The median age at TOF repair was 0.7 years (25th–75th percentile 0.3–1.3) and decreased from 1.7 to 0.4 years from before 1990 to after 2000, respectively (P < 0.001). The median follow-up duration after TOF repair was 16.8 years (9.6–24.7). Events developed in 182 (40%) patients. In multivariable analysis, early repair of TOF (<6 months) [hazard ratio (HR) 3.06; P < 0.001] and complications after TOF repair (HR 2.18; P = 0.006) were found to be predictive for an event. In a subanalysis of the primary repair of TOF after 1990, the patients (n = 125) with elective early repair (<6 months) experienced significantly worse event-free survival compared to patients who had elective repair later (n = 136). In multivariable analysis, early repair (HR 3.00; P = 0.001) and postoperative complications (HR 2.12; P = 0.010) were associated with events in electively repaired patients with TOF. CONCLUSIONS Transatrial–transpulmonary repair of TOF before the age of 6 months may be associated with more events during the long-term follow-up period.


2019 ◽  
Vol 10 (5) ◽  
pp. 543-551 ◽  
Author(s):  
Pasangi Madhuka Wijayarathne ◽  
Peter Skillington ◽  
Samuel Menahem ◽  
Amalan Thuraisingam ◽  
Marco Larobina ◽  
...  

Background: Following corrective surgery in infancy/childhood for tetralogy of Fallot (TOF) or its variants, patients may eventually require pulmonary valve replacement (PVR). Debate remains over which valve is best. We compared outcomes of the Medtronic Freestyle valve with that of the pulmonary allograft valve following PVR. Methods: A retrospective study was undertaken from a single surgical practice of adult patients undergoing elective PVR between April 1993 and March 2017. The choice of valve was at the surgeon’s discretion. There was a trend toward the almost exclusive use of the more readily available Medtronic Freestyle valve since 2008. Results: One hundred fifty consecutive patients undergoing 152 elective PVRs were reviewed. Their mean age was 33.8 years. Ninety-four patients had a Medtronic Freestyle valve, while 58 had a pulmonary allograft valve. There were no operative or 30-day mortality. The freedom from reintervention at 5 and 10 years was 98% and 98% for the pulmonary allograft and 99% and 89% for the Medtronic Freestyle. There was no significant difference in the rate of reintervention, though this was colored by higher pulmonary gradients across the Medtronic Freestyle despite its shorter follow-up. Conclusions: Pulmonary valve replacement following previous surgical repair of TOF or its variants was found to be safe with no significant differences in mortality or reintervention between either valve. Although the Medtronic Freestyle valve had a greater tendency toward pulmonary stenosis, additional follow-up is needed to further document its long-term outcomes.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Cocomello ◽  
M Meloni ◽  
M J Baquedano ◽  
M V Ordonez ◽  
G Biglino ◽  
...  

Abstract Background Tetralogy of Fallot (TOF) repair results in long term chronic pulmonary regurgitation requiring pulmonary valve replacement (PVR). Homograft and stented bio-prosthesis are currently used for PVR but whether one should be considered superior to another remains unknown. Aim To compare echocardiographic and clinical outcomes after PVR with Homograft vs stented bioprosthesis in patients with previous TOF repair. Methods 137 patients who underwent PVR with stented bioproshesis were compared with 80 patients who received an homograft using Mixed linear model and multivariate Cox regression. Results Homograft were associated with a significantly lower transpulmonary gradient postoperatively (P=0.04) and after a mean follow-up 6 years (table). Homograft were associated with a significantly lower risk of reintervention (HR 0.24; 95% CI 0.07–0.85, p=0.026) while long term mortality was comparable between the two groups (P=0.1). Echocardiographic findings BIO HOMOGRAFT P N 137 80 Preoperatively   TV regurgitation (mean, sd) 1.80 (0.63) 1.94 (0.81) 0.353   TV regurgitation pressure drop (mean,sd) 35.15 (15.49) 45.34 (25.24) 0.009   PV regurgitation (mean,sd) 3.63 (0.52) 3.34 (0.64) 0.004   PV systolic peak gradient (mean, sd) 25.07 (17.05) 30.68 (21.70) 0.138 Early postoperatively   TV regurgitation (mean,sd) 1.52 (0.54) 1.54 (0.55) 0.858   TV regurgitation pressure drop (mean,sd) 30.10 (13.82) 29.13 (13.98) 0.733   PV regurgitation (mean,sd) 1.50 (0.54) 1.64 (0.53) 0.224   PV systolic peak gradient (mean,sd) 24.05 (11.10) 20.16 (14.19) 0.045 Latest follow_up   TV regurgitation (mean,sd) 1.63 (0.61) 1.83 (0.80) 0.195   TV regurgitation pressure drop (mean,sd) 37.46 (18.84) 32.58 (13.47) 0.233   PV regurgitation (mean,sd) 2.06 (0.77) 2.07 (0.94) 0.982   PV systolic peak gradient (mean,sd) 32.22 (18.17) 21.25 (13.47) 0.001 TAPSE: Tricuspid annular plane systolic excursion; TV: tricuspid valve; PV: pulmonary valve. PV re-intervention Conclusions When compared to stented bio-prostheses, homografts were associated better early and late hemodynamic profile and a significantly lower risk of re-intervention. Homograft should be considered the first choice in patients undergoing PVR after TOF repair.


Heart ◽  
2017 ◽  
Vol 104 (9) ◽  
pp. 738-744 ◽  
Author(s):  
Jouke P Bokma ◽  
Tal Geva ◽  
Lynn A Sleeper ◽  
Sonya V Babu Narayan ◽  
Rachel Wald ◽  
...  

ObjectiveTo determine the association of pulmonary valve replacement (PVR) with death and sustained ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTOF).MethodsSubjects with rTOF and cardiac magnetic resonance from an international registry were included. A PVR propensity score was created to adjust for baseline differences. PVR consensus criteria were predefined as pulmonary regurgitation >25% and ≥2 of the following criteria: right ventricular (RV) end-diastolic volume >160 mL/m2, RV end-systolic volume >80 mL/m2, RV ejection fraction (EF) <47%, left ventricular EF <55% and QRS duration >160 ms. The primary outcome included (aborted) death and sustained VT. The secondary outcome included heart failure, non-sustained VT and sustained supraventricular tachycardia.ResultsIn 977 rTOF subjects (age 26±15 years, 45% PVR, follow-up 5.3±3.1 years), the primary and secondary outcomes occurred in 41 and 88 subjects, respectively. The HR for subjects with versus without PVR (time-varying covariate) was 0.65 (95% CI 0.31 to 1.36; P=0.25) for the primary outcome and 1.43 (95% CI 0.83 to 2.46; P=0.19) for the secondary outcome after adjusting for propensity and other factors. In subjects (n=426) not meeting consensus criteria, the HR for subjects with (n=132) versus without (n=294) PVR was 2.53 (95% CI 0.79 to 8.06; P=0.12) for the primary outcome and 2.31 (95% CI 1.07 to 4.97; P=0.03) for the secondary outcome.ConclusionIn this large multicentre rTOF cohort, PVR was not associated with a reduced rate of death and sustained VT at an average follow-up of 5.3 years. Additionally, there were more events after PVR compared with no PVR in subjects not meeting consensus criteria.


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