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Treatments and drugs
By Mayo Clinic staffThere's currently no way to replace the defective tricuspid valve. Treatment for tricuspid atresia involves surgery to ensure adequate blood flow through the heart and into the lungs, allowing your baby's body to receive the proper amount of oxygen-rich blood. Often, this requires more than one surgical procedure.
Surgery
A surgery called the Fontan procedure is the standard treatment of tricuspid atresia. During a Fontan surgery, the surgeon creates a path for the oxygen-poor blood returning to the heart to flow directly into the pulmonary arteries, which then transport the blood into the lungs.
However, most children with tricuspid atresia don't undergo the Fontan procedure until they are at least 2 years old.
Some children with tricuspid atresia aren't good candidates for the Fontan procedure. If that is the case, your child's doctors will discuss other options, including the possibility of a heart transplant.
Before surgery
Before the Fontan surgery, other measures — including medications, procedures and other surgeries — are often necessary to stabilize your child's medical condition and ensure proper growth. These may include:
- Taking prostaglandin drugs. Before surgery, your child's cardiologist may recommend that your child take the medication prostaglandin to help widen (dilate) the blood vessels and keep the ductus arteriosus and the foramen ovale open.
- Preventive antibiotics. The cardiologist also will likely recommend that your child take preventive antibiotics before certain dental and other procedures to prevent bacteria from entering the bloodstream and infecting the inner lining of the heart (infective endocarditis). Practicing good oral hygiene — brushing and flossing teeth, getting regular dental checkups — is another good way of preventing infection.
- Preliminary procedures. The so-called rescue surgeries your baby may need before the Fontan procedure depend on how much blood is reaching his or her lungs. Following are some of the procedures babies with tricuspid atresia may require:
- Atrial septostomy. This procedure creates or enlarges the opening between the heart's upper chambers (atria) to allow more blood to flow from the right atrium to the left atrium.
- Shunting. Creating a bypass (shunt) from the main blood vessel leading out of the heart (aorta) to the pulmonary arteries allows for adequate blood flow to the lungs. Surgeons typically implant a shunt during the first four to eight weeks of life. However, babies usually outgrow this shunt and may need another surgery to replace it.
- Glenn procedure. When babies outgrow the first shunt, they often require a surgery that sets the stage for the Fontan procedure. Doctors usually perform the Glenn procedure when a child is about 6 months old. It connects one of the large veins that return blood to the heart (superior vena cava) to the pulmonary artery. This allows oxygen-poor blood to flow directly to the lungs. The procedure reduces the workload on the left ventricle, decreasing the risk of damage to it.
Follow-up care
To monitor his or her heart health, your baby will need lifelong follow-up care with a cardiologist who specializes in congenital heart disease. Your child's cardiologist will tell you whether your child needs to continue taking preventive antibiotics before dental and other procedures. In some cases, your child's cardiologist may recommend limiting vigorous physical activity.
The short- and intermediate-term outlook for children who have a Fontan procedure is generally promising. Outcomes for those having surgery later in life are generally worse. A variety of complications may occur over time and sometimes require additional procedures. If the circulation system created by the Fontan procedure fails, then a heart transplant may be necessary. Talk to your child's doctor about his or her specific situation.
- Sondheimer HM, et al. Cardiovascular diseases. In: Hay WW Jr, et al. Current Diagnosis and Treatment: Pediatrics. 19th ed. New York, N.Y.: McGraw Hill; 2009. http://www.accessmedicine.com/content.aspx?aID=3402846. Accessed April 28, 2010.
- Single-ventricle defects. American Heart Association. http://www.americanheart.org/print_presenter.jhtml?identifier=11072. Accessed April 28, 2010.
- Harris IS, et al. Congenital heart disease in adults. In: Crawford MH. Current Diagnosis & Treatment: Cardiology. 3rd ed. New York, N.Y.: McGraw Hill; 2009 http://www.accessmedicine.com/content.aspx?aID=3649722. Accessed April 28, 2010.
- Keane JF, et al. Tricuspid atresia: Clinical manifestations. In: Keane JF, et al. Nadas' Pediatric Cardiology. Philadelphia, Pa.: Elsevier; 2006. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-1-4160-2390-6..50050-7--cesec5&uniq=198820213&isbn=978-1-4160-2390-6&sid=991370226. Accessed April 30, 2010.
- Congenital heart defects. March of Dimes. http://www.marchofdimes.com/professionals/14332_1212.asp. Accessed April 28, 2010.
- Tricuspid atresia. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/print/sec19/ch287/ch287i.html. Accessed April 28, 2010.
- Sittiwangkul R, et al. Outcomes of tricuspid atresia in the Fontan era. Annals of Thoracic Surgery. 2004;77:889.
- Berg C, et al. Prenatal diagnosis of tricuspid atresia: Intrauterine course and outcome. Ultrasound in Obstetrics and Gynecology. 2010;35:183.
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