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.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair

2021 ◽  
Vol 12 (5) ◽  
pp. 628-634
Author(s):  
Elizabeth H. Stephens ◽  
Brian L. Wolfe ◽  
Abhinav A. Talwar ◽  
Angira Patel ◽  
Joseph A. Camarda ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Demographic Data ◽  
Right Ventricular Outflow Tract ◽  
Patch Repair ◽  
Transannular Patch ◽  
Z Scores ◽  
Valve Sparing ◽  
Tetralogy Of Fallot Repair

Background: Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. Methods: Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. Results: Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient ( P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from −2.3 ± 1.0 on predischarge echocardiogram of to −1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. Conclusions: Obtaining a postrepair pulmonary valve z-score of −2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

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.

Abstract 14576: Durability of Valve-sparing Tetralogy of Fallot Repair

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Elizabeth H Stephens ◽  
Bryan L Wolfe ◽  
Abhinav A Talwar ◽  
Angira Patel ◽  
Joseph Camarda ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Pulmonary Valve Replacement ◽  
Patch Repair ◽  
Transannular Patch ◽  
Z Scores ◽  
Valve Sparing ◽  
Tetralogy Of Fallot Repair

Introduction: While valve-sparing repair is ideal for Tetralogy of Fallot (TOF), it’s durability and which patients may benefit from a transannular patch remains unclear. To this end, we reviewed our experience with valve-sparing TOF repair. Methods: Retrospective review was performed of all primary TOF operations at our institution from 1/2008 to 12/2018. Standard demographic, operative, and echo data were collected, along with clinical outcomes. Transannular patch and valve-sparing repair groups were then compared. Results: Sixty-eight patients underwent TOF repair with a mean age of 4.1±2.2 months and weight of 5.7±1.8 kg. There was no difference in age or weight between patients who underwent a transannular patch repair and valve-sparing repair (Table). There was also no difference in the frequency of hypercyanotic spells or beta-blocker use. As expected the pre-operative pulmonary valve size and z-score were significantly different between groups. Bypass times were longer in the transannular patch group (176±40 vs. 144±40 minutes, p=0.005). There were no differences in post-operative complications. At last follow-up (median 41.5 months) there was a trend of a higher peak pulmonary valve gradient (p=0.07) in the valve-sparing group, but no difference in pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3±1.0 on pre-discharge echocardiogram to -1.2±1.6 on last follow-up, with the peak gradient on pre-discharge 20 (33) mmHg stable on last follow-up at 18 (29) mmHg and degree of pulmonary regurgitation stable. There was one reoperation in the cohort: a pulmonary valve replacement in a patient who had undergone a transannular patch repair 6 years prior. Conclusions: Valve-sparing TOF patients demonstrated stable repairs with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from re-intervention during follow-up.

Two-patch transannular repair of tetralogy of Fallot with complete atrioventricular canal defect

2021 ◽  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Canal ◽  
Atrioventricular Canal Defect ◽  
Valve Annulus ◽  
Patch Technique ◽  
The Right ◽  
Complete Atrioventricular

We demonstrate the repair in an infant of tetralogy of Fallot with complete atrioventricular canal defect using a 2-patch technique with transannular reconstruction of the right ventricular outflow tract due to a diminutive pulmonary valve annulus. This approach is reproducible and particularly valuable to surgeons who routinely use a 2-patch technique to repair an isolated complete atrioventricular canal defect.

scholarly journals Late complete heart block post-tetralogy of Fallot repair: a possible new predicting, precipitating factor and review of related cases

2019 ◽  
Vol 12 (4) ◽  
pp. e228642
Author(s):  
Rayan Hejazi ◽  
Marwan Balubaid ◽  
Jameel Alata ◽  
Rahaf Waggass
Keyword(s):  
Tetralogy Of Fallot ◽  
Heart Block ◽  
Complete Heart Block ◽  
Corrective Surgery ◽  
Patient Assessment ◽  
Severe Tricuspid Regurgitation ◽  
Atrioventricular Dissociation ◽  
Complete Atrioventricular ◽  
Tetralogy Of Fallot Repair

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease beyond the age of 3 months. Complete heart block (CHB) is rare but a serious sequalae post-repair of TOF. We present a case of an 18-month-old child who developed late CHB after around 1 year of the corrective surgery of the congenital anomaly. On the regular follow-up visit, the patient assessment was unremarkable. However, there was bradycardia, 55 beats/min. The ECG showed complete atrioventricular dissociation. Echocardiogram was done and demonstrated severe tricuspid regurgitation (TR). The patient required a permanent pacemaker and he is currently well. We are presenting this case as a late unexpected CHB, with a possibility of progressive right-side dilatation as a contributing factor to CHB due to severe TR.

Surgical management of complete atrioventricular septal defect with tetralogy of Fallot: results from a national audit database

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Dorobantu ◽  
R Tulloh ◽  
S Stoica
Keyword(s):  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
National Audit ◽  
Treatment Pathways ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Repair ◽  
Complete Atrioventricular

Abstract Background Complete atrioventricular septal defect (CAVSD) with tetralogy of Fallot (CAVSD-ToF) is an uncommon association, and the management is more difficult than for each lesion in isolation. Depending on the anatomy, clinical status and ventricular morphology, definitive repair is offered either as staged or single operation with few undergoing single or one and a half ventricle procedures. Currently available data are limited to single centre studies. Purpose We aim to use data from a national audit dataset to describe outcomes in the surgical management of CAVSD-ToF with data from the national audit. Methods All children with CAVSD and tetralogy of Fallot (including double outlet right ventricle and pulmonary atresia variants) undergoing the first cardiac procedure between 2000–2013 were included and all subsequent procedures extracted. Three treatment pathways were defined: definitive primary procedure (“primary complete repair”), staged repair or “unbalanced ventricles repair” (Glenn shunt or modified Fontan). Continuous data is expressed as median (range). Survival data obtained using the Kaplan Meier method and subgroup comparisons done using the log-rank test. Results A total of 125 patients were included, 50.4% male, first procedure at 150 (1–2770) days. Down syndrome was present in 79.4% (63/79 of those with reported non-cardiac comorbidities), while 8% (10/125) had heterotaxy syndrome. “Primary complete repair” was performed in 40% (n=50), at 319 (33–2770) days. Staged repair was undertaken in 28% (n=35), first palliation at 80 (1–733) days. An “unbalanced ventricles” approach was used in 16.8% (n=21), first procedure at 135 (1–1742) days). The other 15.2% (n=19), first procedure at 53 (range 1–436) days, had at least one palliation procedure, but no identifiable committed pathway. Survival at 5 years from the first procedure in “primary complete repair” compared to the remainder of the group is shown in the Figure. No significant differences in survival were found. Specifically in the “unbalanced ventricle repair” group, just 1 patient died during follow-up. A total of 85 patients (68%) achieved either primary or staged “complete repair”. Early mortality (30 day) after the “complete repair” was 6.6% (similar for both approaches, p=0.9). Survival at 5 years after “complete repair” was 82.7% (similar for both approaches, p=0.8). Freedom from any cardiac re-intervention at 5 years was 69.5% (77.6% after primary, 58.1% after staged repair, p=0.03). Conclusion Early-life mortality in this heterogeneous group of CAVSD-ToF remains high, even in patients receiving definitive “complete repair”. We found no survival differences in the medium term between primary repair and a palliation approach, but more cardiac re-interventions were undertaken after staged complete repair. Surprisingly good results were seen in the unbalanced ventricles group, but longer follow up is needed to examine their attrition and longer term outcome. Survival at 5 years by treatment option Funding Acknowledgement Type of funding source: None

scholarly journals Complete atrioventricular canal associated with tetralogy of Fallot

1984 ◽  
Vol 87 (5) ◽  
pp. 756-766 ◽  
Author(s):  
Gideon Uretzky ◽  
Francisco J. Puga ◽  
Gordon K. Danielson ◽  
Robert H. Feldt ◽  
Paul R. Julsrud ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Atrioventricular Canal ◽  
Complete Atrioventricular

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.

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