I love you so much

I am reading about the surgery that you are going to have after you are born and I can hardly breathe.  My little baby...my sweet, precious baby...why can't things just be normal for you?  My God, you are not even born yet and you have already struggled for every stage of your life.

I am pasting the description of your surgery and outcomes below so when you read this later, you will know just how brave and courageous you are.

Gracie, I love you so much.  I just want you to be alright.  That's it.  Just be alright.  I will deal with anything else that you need.  I promise you.

Tetralogy of Fallot (ToF) Repair

Related Clinics & Centers

Adult/Pediatric Congenital Heart Surgery Service

Related Diseases

Tetralogy of Fallot (TOF)

Related Physicians

Charles D. Fraser, Jr., MD

Jeffrey S. Heinle, MD

E. Dean McKenzie, MD

Complete Tetralogy of Fallot (ToF) repair:

Tetralogy of Fallot is the most common congenital heart defect causing cyanosis (bluish discoloration of skin caused by oxygen-poor blood reaching the general circulation). An embryologic failure of the right ventricular outflow tract to form properly results in the 4 cardiac abnormalities characteristic of TOF:

• ventricular septal defect – VSD (an abnormal opening between the two lower chambers of the heart)
• pulmonic stenosis – PS (a narrowed area within the main pulmonary artery, at, above or below the pulmonary valve)
• a malpositioned aorta in which the entrance to the aorta over-rides the VSD
  an overly muscular right ventricle (ventricular hypertrophy)

A complete TOF repair is an open-heart procedure performed to correct this congenital heart defect.

What do we need to do before congenital heart surgery?

1. If at all possible, it is important that the patient be free of infection prior to going to surgery. This includes dental cavities, so a dental check-up and any dental work required should be completed within 6 weeks prior to surgery. A letter is required from the dentist clearing the patient for surgery. In addition, if the patient develops other signs and symptoms of an infection, such as a fever, runny nose, diarrhea, or vomiting, contact the surgeon’s office immediately.
2. Please contact your insurance company to verify insurance and determine whether a referral is required. If your insurance requires a referral, have your Primary Care Physician’s office fax the referral to Congenital Heart Surgery at (832) 825-2013.
3. If the patient is taking aspirin, contact the cardiologist to ask when to discontinue taking the aspirin. Aspirin is generally discontinued 14 days prior to surgery to minimize the risk of excessive bleeding. However, there may be a medical reason to continue the aspirin, so it is important to check with the cardiologist.
4. Patients who live more than an hour’s drive away from Texas Children’s Hospital will need to make arrangements to stay in Houston for at least one full week after discharge from the hospital. This is important to ensure a rapid response to any early post-operative issues. The Texas Children’s Hospital Social Worker can assist if any help is needed making arrangements for this.
5. If the patient is due for immunizations within a week of surgery, contact the Congenital Heart Surgery Clinic and ask to speak to the clinic nurse.
6. Patient’s undergoing cardiac surgery frequently need blood products. Patients and families interested in donating blood should call the Congenital Heart Surgery Clinic and ask to speak to a clinic nurse. Families and friends may donate blood in one of two ways:
   • Credit: For each unit of blood donated, a credit will be given on the patient’s bill, but the blood donated will not specifically be designated for the patient. Blood donated can be any blood type.
   • Designated (or Directed) Donation: Blood donated will be used for the patient designated. For congenital heart surgery patients, blood donors must be the same blood type as the patient. For “Designated Donation,” it is very important before you donate that you call the surgeon’s office to let us help you coordinate the donation. The pertinent patient information we need when you call is:
     • the patient’s weight
     • blood type
     • surgery date
     • type of surgery
   • The best time to donate blood is one week prior to surgery and no later than three days prior to surgery. There is a cost associated with directed donor blood that may not be covered by your insurance. It will be the family’s responsibility to cover the cost of directed donor blood that is not covered by insurance.
7. If the patient is taking “blood thinners” such as Coumadin or Lovenox, please contact the Congenital Heart Surgery Clinic and ask to speak to the clinic nurse. These patients may need to be hospitalized a few days prior to surgery to discontinue these medications and convert to Heparin prior to surgery. This decision will be coordinated between the patient, surgeon and cardiologist.
8. Registration and admission into Texas Children’s Hospital is usually scheduled to occur the day before surgery. Patients and their families should arrive at 8:00 a.m. in the Congenital Heart Surgery Clinic located on the 20th floor of the West Tower. We conduct a very thorough pre-operative work-up in order to attain the very best surgical outcome for the patient, so the preoperative work-up will take several hours. A cardiologist performs a physical exam and multiple tests will be performed to ensure the patient is well for surgery. A chest x-ray (CXR), an electrocardiogram (EKG), and lab work will be performed as part of the preoperative process. A Congenital Heart Surgery clinic nurse will facilitate your day. You will also meet many members of the Heart Center team who will familiarize you with the preoperative routines and answer any questions. These team members include: the Social Worker, Child Life Specialist, Chaplain, and the Congenital Heart Surgery Anesthesiologist. At the end of the pre-operative work-up, you will either be admitted to the 15 Tower Cardiac Inpatient Unit or sent home to return the following early morning for surgery. Valet parking is available at the West Tower building. If you choose to valet park the day of your preoperative work-up, bring your parking ticket with you and the Congenital Heart Surgery clinic nurse will validate parking for the day of your preoperative work-up. It will be your responsibility to pay for parking throughout the remainder of your hospital stay.

What happens on the day of surgery?

For patients who are hospitalized the night before surgery, an intravenous (IV) catheter may be placed. This will allow intravenous fluids to be given once NPO (nothing by mouth) status begins.

All patients – whether they are admitted to the hospital or not - will be placed on “NPO” (nothing by mouth) status after midnight the night before surgery. Clear liquids may be allowed at the specific instructions of the anesthesiologist.

On the morning of surgery, your family will meet our staff on the 18th floor of the West Tower. You will be greeted by a receptionist and given a private waiting room. Approximately one hour prior to surgery, the patient will be taken to the holding area, dressed in hospital pajamas, and placed in a crib or bed. The anesthesiologist will order some medication to be given that reduces anxiety. This medication is given by mouth or IV, and will allow the patient to relax and become sleepy.

What type of anesthesia will be used?

Anesthesiologists who specialize in pediatric cardiovascular anesthesia will manage the anesthetic care of the patient throughout the surgery. They collaborate closely with the surgeon and the rest of the OR team to monitor and manage the patient’s care in the operating room. Based on their pre-operative assessment, they will individualize the anesthetic to meet each patient’s physiologic needs. In most cases, a general anesthetic approach is used which results in the patient being completely unconscious and pain-free during the entire surgery as well as shortly afterward. During the surgery, several specialized neurophysiologic monitors are used to ensure that the anesthesia is effectively maintaining the patient in a fully unconscious and pain-free condition.

What happens during surgery and how is it performed?

The type of surgery your child will undergo depends on your child’s heart defect. Prior to the surgery, the surgeon will have a detailed consultation with the family to explain the specific surgical procedure, discuss risks and benefits, and answer any questions the family may have. Throughout the actual operation, a surgical nurse clinician will update the family approximately every hour during your child’s surgery. These updates will be given in the private waiting room assigned by the receptionist.

The following steps are commonly followed in all types of congenital heart surgery:

Preparation: The anesthesiologist and the operating room nurses will escort the patient into the operating room. A heart monitor will be connected to the patient that will show the OR team a continuous read-out of the heart rate and rhythm throughout the surgery. The patient will be given a mask through which they will breathe a gas that causes them to fall deeply asleep.

Once the patient is asleep, the anesthesiologist will put a breathing tube (endo-tracheal tube or ET tube) into their windpipe. This tube is attached to a breathing machine (ventilator) that will do the breathing for the patient during their surgery. Once the ventilator is secured, the anesthesiologist will place several intravenous (IV) catheters in the patient’s veins. The sites typically used are the large vein in the neck or the groin. There may be one or two more IVs placed.

Once the IVs are secured, intravenous fluids and medication are given through them throughout the operation. Another special catheter (arterial line) is placed in an artery (the blood vessel which has a pulse). The arterial line is used to monitor blood pressure during and after surgery. This special catheter is also used to draw samples of blood to obtain various laboratory values. The sites usually used are in the wrist or the groin. A nasogastric (NG) tube is placed in the nose and gently guided down to the stomach. The stomach will continue to produce juices even though there is no food in it. This may cause the patient to become nauseated and vomit. The NG tube will empty the stomach and prevent vomiting. Another catheter (Foley catheter) is placed in the patient’s urinary opening and guided gently to the bladder. This catheter is attached to a device that drains and measures the urine produced during surgery. All of these procedures are performed after the patient is deeply asleep. This whole process may take from one to one and a half hours.

Once all the lines and tubes are in place, a transesophageal echocardiogram (TEE) is performed. A cardiologist will place a probe into the patient’s mouth and gently guide the probe down the esophagus. The TEE probe rests behind the heart and provides the surgeon with a continuous picture of the structures of the heart during the operation. When the TEE is completed, it is time for the surgeon to begin the operation.

Incision and operation:

A median sternotomy incision is used for “open-heart” surgery (surgery that occurs inside the heart). This incision usually begins at or below the top of the breastbone (sternal notch) and goes straight down the sternum (breastbone). The breastbone is then separated to expose the heart. The patient is then placed on the heart-lung bypass machine, a device that provides blood flow to the body and “bypasses” the patient’s heart and lungs. Diverting the heart’s blood flow to the bypass pump allows the surgeon to open the heart and operate on the structures inside the heart. The heart-lung bypass machine provides continuous oxygenated blood to the other organ systems during the open-heart surgery. Depending on the location of the defect, an incision will be made in either the right atrium, the pulmonary artery, or the outflow tract of the right ventricle (infundibulum). The right atrial approach in which the VSD is patched by working through the open valve (tricuspid valve) between the right atrium and the right ventricle is preferred. This approach prevents having to make an incision in the right ventricle, which can lead to future right ventricular failure. The patch for the VSD is created by the surgeon from either the patient’s own pericardial tissue or a synthetic material such as Dacron, then sutured into place to close the defect. Any obstructive tissue in the right ventricular outflow tract is cut away (resected). A second incision is made in the main pulmonary artery, and this approach is used to complete the VSD closure and any additional resection of obstructive tissue. Additional work may be needed to widen a narrow pulmonary artery or valve, such as a patch. In some cases of severe obstruction, or absence of the pulmonary artery, a conduit may be placed between the right ventricle and pulmonary arteries to provide an open communication for blood to flow to the lungs. After making these repairs, the atrial, pulmonary artery and/or infundibular incisions are then closed with sutures.

Once the surgical procedure is completed, the patient will be weaned gradually off of the heart-lung bypass machine until the newly repaired heart is managing all the blood flow again. Chest tubes will then be placed to drain the surgical area. These tubes are positioned at the base of the incision. There will be 1-3 chest tubes placed for most surgical procedures. Temporary pacing wires are also placed at this time. These are very small wires that are positioned on one or both sides of the incision. These pacing wires may be used temporarily to pace the heart rate and rhythm if needed in the post-operative period. Intracardiac monitoring lines may be placed depending on the type of surgical repair. These special catheters are placed in the chambers and vessels of the heart to provide the surgeon and the postoperative team with valuable information about the pressures within the heart and lungs. A postoperative TEE will be performed which provides the surgeon with valuable information after the surgical repair. Once the TEE is completed, the surgeon will close the sternum. The sternal bone is brought together, and stainless steel wire secures the sternum.

The type of skin closure the surgeon uses is dependent on age and weight:

• Clear, absorbable skin suture that runs the length of the incision on the inside of the chest. A clear knot is seen at the top and the bottom of the incision. To secure the outside of the incision, adhesive strips (steri-strips) are applied to the surface of the skin along the length of the incision.
• Surgical staples (used occasionally in larger patients)

Completion and transfer to recovery room:

After closure of the incision, a dressing is placed that remains in place for the first 24 hours after surgery. The anesthesiologist and the operating room nurses then secure all the patient’s tubes and lines, and prepare for transport from the OR. The patient is moved from the operating room table onto an intensive care bed and transported to the Cardiovascular Intensive Care Unit (CVICU).

What happens after the surgery?

The patient will be transferred to the Cardiovascular Intensive Care Unit (CVICU) by the cardiovascular anesthesiologist and the team of operating room nurses. The CVICU is situated directly adjacent to the cardiovascular operating rooms to maximize patient safety. This makes the transition from the operating room to the CVICU a smooth process. Once the patient arrives in the CVICU, a thorough report of the surgical procedure is given to the Cardiologist and the CVICU nursing team who will be managing care in the CVICU. The surgeon will update the family on the operation once the patient is settled into the CVICU. Family members may usually visit the patient within an hour after arrival to the CVICU.

While in the CVICU, the patient will continue to be monitored closely. In addition to monitoring heart rate and rhythm, and respiratory rate, the monitor will also display other pressures and waveforms, which assist the cardiologist in managing care. A chest x-ray (CXR) and lab work will be performed on arrival and periodically throughout the stay in the CVICU.

The CVICU is open to visitation 24 hours a day. No more than two visitors are permitted to visit in the CVICU at a time. Children under the age of fourteen years are not allowed on the 18th floor in order to protect the patients and the children from infection. A limited number of rooms are available for sleeping at night, and they are assigned on a first come, first served basis. The receptionist will provide you with the CVICU visiting policy and the process for the use of the sleeping rooms.

The CVICU team will continually assess the patient’s comfort level. Medication will be given to reduce any pain or anxiety they may experience after their surgery. These medications are initially given by IV and then gradually changed to medications that can be taken by mouth. Comfort measures provided by the family are encouraged. Talking quietly to the patient is important even if the ETT is still in place (the patient will not be able to speak when the ETT is in place). Hearing your voice usually will assure the patient that you are close by and may make them feel more secure. Other comfort measures such as listening to soft music or helping patients change their position in bed regularly may also decrease their anxiety or pain.

Once the patients are awake enough to breathe on their own, the breathing tube (ETT or endotracheal tube) is removed. Depending on the surgery performed, this may take anywhere from a few hours to a few days following surgery. Once the surgical dressing is removed, the incision will remain open to air. The nurses will cleanse the incision with a Betadine solution twice a day. A small gauze dressing will be placed over the insertion sites of the chest tubes, intracardiac lines, and pacing wires. Gradually the tubes, wires, and intravenous lines will be removed as the patient’s condition improves.

When the patient has a stable blood pressure, a stable heart rate and rhythm, and can breath well on their own without the ventilator, they will be transferred to the cardiac inpatient floor, 15 Tower. As the patient improves, their activity level will gradually increase until they are doing well enough to be discharged from the hospital. Before leaving, the family will be instructed in how to care for the patient at home, including information on medications, incisional care, and activity limitations.

How long will we be in the hospital?

The length of time in the hospital will depend on a variety of factors:

• The type of congenital heart defect, surgical repair and post-operative course
• The patient’s ability to maintain/gain weight and activity level
• The family’s understanding and ability to provide for the patient’s post-operative needs

In general, patients can expect to stay anywhere from 3-10 days, depending on the above factors. Patients who live greater than an hour’s drive from Houston may need to make arrangements to stay in the Houston area until after their 1st post-operative cardiology clinic visit (1 week after discharge).

What are the risks associated with congenital heart surgery?

Risks associated with congenital heart surgery vary widely according to the specific procedure and the patient’s condition at the time of surgery. They can include:

• Bleeding
• Infection
• Need for re-operation
• Damage to nerves in the chest area
• Brain damage
• Death

Your surgeon will discuss the specific risks associated with your child’s congenital heart surgery at the time of your consult. In our program, the risk of serious complications occurring, such as brain damage or death, is very low. Our surgeons have a very large experience and can provide specific data to you defining the surgical risk for your particular procedure. We believe this important information must be shared with parents to make the process less stressful.

What should we watch out for after surgery?

Before the patient is discharged from the hospital, your family will be given detailed instructions that are individualized for the specific procedure performed. However, in general, patients should contact their doctor for any of the following after congenital heart surgery:

• redness, swelling, or oozing/bleeding from incision
• fever
• altered mental status, excessive fatigue
• feeding/eating problems
• prolonged or worsening pain

Patients who have had a sternotomy should avoid any activity that causes strain on the chest for at least 6-8 weeks, so that the breastbone can heal. For infants, avoid picking them up by arms (“scoop” them up instead); for older children and adults, avoid activities that involve pulling or pushing with arms as well as contact games/sports.