Tetralogy of fallot surgical techniques

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]

## Overview

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Repair of tetralogy of Fallot reduces mortality. we can either perform palliative surgery which involves forming an anastomosis between the subclavian artery and the pulmonary artery. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients or total surgical repair which involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, repairing the VSD using a Gore-Tex or Dacron patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.The repair could be done by either of the approaches i.e.transatrial or transpulmonary.

## Surgery

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### Palliative Surgery

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* The condition was initially thought untreatable until surgeon Alfred Blalock, cardiologist Helen B. Taussig, and lab assistant Vivien Thomas at Johns Hopkins University developed a surgical procedure. It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work.
* The surgery involved forming an anastomosis between the subclavian artery and the pulmonary artery. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients.
* The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results.
* The Pott shunt and the Waterson procedure are other shunt procedures which were developed for the same purpose.
* Waterson shunt: Shunt between right pulmonary artery and ascending aorta.
* Potts shunt: Shunt between left pulmonary artery and descending aorta.
* The technique has been modified and is usually performed using a Gortex tube to create the connection.
* Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for some conditions like-
* Severe variants such as tetralogy of Fallot with pulmonary atresia
* Premature babies
* Coronary artery origin anomalies
* The Blalock-Taussig procedure was the only surgical treatment until the first total repair was performed in 1954.
* They are usually performed through lateral thoracotomy.

### Disadvantages

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* Shunt placement causes pulmonary artery distortion such as kink, thrombosis and occlusion
* Large shunt produces pulmonary hypertension and pulmonary vascular disease.
* Large shunt causes volume overload of left ventricle.

### Total Surgical Repair

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* The total repair was performed by C. Walton Lillehei at the University of Minnesota in 1954 on a 10-month boy.
* Total repair initially carried a high mortality risk which has consistently improved over the years.
* Surgery is now often carried out in infants 1 year of age or younger with a <5% perioperative mortality.
* The surgery generally involves
* Making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle
* Repairing the VSD using a Gore-Tex or Dacron patch or a homograft.
* Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.
* The repair could be done by either of the approaches i.e.transatrial or transpulmonary
* Patients who have undergone "total" repair of tetralogy of Fallot often have good to excellent cardiac function after the operation with some to no exercise intolerance and have the potential to lead normal lives. Surgical success and long-term outcome greatly depends on the particular anatomy of the patient and the surgeon's skill and experience with this type of repair.
* Pulmonary valve should be replaced before right ventricle dysfunction occurs.

## Video

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### After Repair

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Arrythmia may occur after the surgical repair of TOF.

## 2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1]

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### Evaluation and Follow-up in the Repaired Patients of Tetralogy of Fallot (DO NOT EDIT)[1]

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Class I

"1. Patients with repaired tetralogy of Fallot should have at least annual follow-up with a cardiologist who has expertise in adult congenital heart disease (ACHD). (Level of Evidence: C)"
"2. Patients with tetralogy of Fallot should have echocardiographic examinations and/or magnetic resonance imagings (MRIs) performed by staff with expertise in ACHD. (Level of Evidence: C)"
"3. Screening for heritable causes of their condition (e.g., 22q11 deletion) should be offered to all patients with tetralogy of Fallot. (Level of Evidence: C)"
"4. Before pregnancy or if a genetic syndrome is identified, consultation with a geneticist should be arranged for patients with tetralogy of Fallot. (Level of Evidence: B)"
"5. Patients with unrepaired or palliated forms of tetralogy should have a formal evaluation at an ACHD center regarding suitability for repair. (Level of Evidence: B)"

### Surgery for Adults with Previous Repair of Tetralogy of Fallot (DO NOT EDIT)[1]

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Class I

"1. Surgeons with training and expertise in congenital heart disease (CHD) should perform operations in adults with previous repair of tetralogy of Fallot. (Level of Evidence: C)"
"2. Pulmonary valve replacement is indicated for severe pulmonary regurgitation and symptoms or decreased exercise tolerance. (Level of Evidence: B)"
"3. Coronary artery anatomy, specifically the possibility of an anomalous anterior descending coronary artery across the RVOT, should be ascertained before operative intervention. (Level of Evidence: C)"
Class IIa

"1. Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following:"
"a. Moderate to severe right ventricular dysfunction. (Level of Evidence: B)"
"b. Moderate to severe RV enlargement. (Level of Evidence: B)"
"c. Development of symptomatic or sustained atrial and/or ventricular arrhythmias. (Level of Evidence: C)"
"d. Moderate to severe tricuspid regurgitation (TR). (Level of Evidence: C)"
"2. Collaboration between ACHD surgeons and ACHD interventional cardiologists, which may include preoperative stenting, intraoperative stenting, or intraoperative patch angioplasty, is reasonable to determine the most feasible treatment for pulmonary artery stenosis. (Level of Evidence: C)"
"3. Surgery is reasonable in adults with prior repair of tetralogy of Fallot and residual RVOT obstruction (valvular or subvalvular) and any of the following indications:"
"a. Residual RVOT obstruction (valvular or subvalvular) with peak instantaneous echocardiography gradient greater than 50 mm Hg. (Level of Evidence: C)"
"b. Residual RVOT obstruction (valvular or subvalvular) with RV/LV pressure ratio greater than 0.7. (Level of Evidence: C)"
"c. Residual RVOT obstruction (valvular or subvalvular) with progressive and/or severe dilatation of the right ventricle with dysfunction. (Level of Evidence: C)"
"d. Residual ventricular septal defect (VSD) with a left-to-right shunt greater than 1.5:1. (Level of Evidence: B)"
"e. Severe aortic regurgitation (AR) with associated symptoms or more than mild LV dysfunction. (Level of Evidence: C)"
"f. A combination of multiple residual lesions (e.g., VSD and RVOT obstruction) leading to RV enlargement or reduced RV function. (Level of Evidence: C)"

## References

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1. ↑ 1.0 1.1 1.2 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)

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