Chairman, Cardiac Sciences, Fortis Hospital, Noida
Part 7 of 8 in Transforming Cardiac Care: Advances in Diagnosis, Surgery and Management
Tetralogy of Fallot and Eisenmenger Syndrome: Two Very Different Outcomes
December 1, 2024
Two congenital and pulmonary vascular conditions came up directly in the discussion of Dr. Ajay Kaul's Jivo Healthcare masterclass for doctors across Africa: tetralogy of Fallot and Eisenmenger syndrome. Dr. Kaul, Chairman of Cardiac Sciences at Fortis Hospital, Noida, addressed both, and the contrast between them makes a clear case for early referral.
Tetralogy of Fallot Is Highly Treatable
Tetralogy of Fallot, a combination of congenital heart defects, is very well treatable when operated on in childhood, according to Dr. Kaul. Children who are operated on go on to lead normal lives; he pointed to his own patients who are now 25 to 30 years old and doing well. The exact outcome does depend on which specific variant of tetralogy the patient has, but results are generally very good.
Eisenmenger Syndrome Is What Happens Without Early Treatment
Eisenmenger syndrome represents the other end of the same spectrum. It develops when an operable heart defect is left untreated for long enough that the abnormal blood flow damages the lungs, eventually making the lungs too stiff for any corrective heart procedure to help. At that point, the only remaining option is a combined heart and lung transplant, a far more difficult undertaking than treating the original defect would have been, with a success rate of only around 40 to 50 percent.
The Real Variable Is Time
The distance between these two outcomes, a normal life after childhood surgery versus a difficult heart-lung transplant decades later, is set almost entirely by how early the underlying defect is caught and referred for treatment. For a doctor evaluating a child with a congenital heart defect, that makes the timing of referral itself a clinical decision with lasting consequences.
This guide is based on a live Jivo Masterclass: Dr. Ajay Kaul taught doctors across Africa on December 1, 2024.
FROM THE LIVE Q&A
Moderator
How does an artificial heart actually work?
Dr. Ajay Kaul
The heart is essentially a pump: blood comes in from the body, is pushed to the lungs to be purified, and is then pushed back out to the body. Building an artificial pump that does not damage or clot the blood took nearly 50 years to perfect. The device is based on magnetic levitation technology, similar to Japan's high-speed trains that run without touching the rails, so the pump's rotor never actually makes contact with the blood it is moving. In India, one artificial heart costs around 1.5 crore rupees, which is the major cost barrier.
Frequently Asked Questions
Is heterozygous (mismatched donor-recipient) heart transplantation possible?▼
It is technically possible, but very few doctors would attempt it. A heart is a precious, scarce organ with a long waiting list of patients, so we always give it to the best-matching recipient rather than risk it in a less compatible match where rejection is more likely.
What happens to the donor in a heart transplant?▼
The donor is already dead by the time of donation. It is not a live donation, so nothing happens to the donor.
What can someone who is currently healthy do in everyday life to keep their heart healthy?▼
Some factors cannot be changed: genetics (a family history of coronary artery disease, hypertension or diabetes), age, and sex. Some factors are partially avoidable, like living in a polluted city or staying in a highly stressful job or relationship. The fully preventable factors are the most important: smoking or chewing tobacco has no offsetting benefit and damages the heart, lungs or mouth; alcohol should be limited to two or three drinks, two or three times a week; walking at least 10,000 steps a day and taking the stairs down instead of the lift helps; and the single best exercise is a 3-kilometer walk in 30 minutes, twice a day. On checkups: if there is a family history of heart disease or diabetes, get a cardiac checkup from age 20; if there is no family history, get one by age 40. And before starting any strenuous new exercise routine, such as joining a gym, get a cardiac checkup first, because the heart often gives no warning symptoms until it fails outright, the same way a car needs servicing even when it seems to be running fine.
Does a transplant recipient have to stay on rejection-suppression therapy, and what is the average monthly cost?▼
Yes, anyone who has had an organ transplant, whether liver, lung, or heart, has to remain on anti-rejection therapy. In India, that costs on average about 25,000 to 35,000 rupees a month, and can range from about 20,000 up to 50,000. I'm not able to speak to what it costs in other countries.
Most chest abnormalities are often confused with cardiac disease. What is the major difference between the two?▼
The chest includes the lungs, the esophagus, the heart, and the blood vessels. What we are discussing today covers only diseases of the heart and blood vessels, not lung disease, muscular disease, disease of the bones or spine, or even the breast, even though these are also considered part of the chest.
Is tetralogy of Fallot treatable?▼
Yes, very well. Children who are operated on go on to lead normal lives, with patients now 25 to 30 years post-surgery doing well.
What causes Eisenmenger syndrome?▼
It develops when an operable heart defect is left untreated long enough that it damages the lungs irreversibly.
What is the treatment for Eisenmenger syndrome, and how successful is it?▼
A combined heart and lung transplant, with a success rate of only around 40 to 50 percent.
In This Series: Transforming Cardiac Care: Advances in Diagnosis, Surgery and Management
- 1.Transforming Cardiac Care
- 2.Minimally Invasive Cardiac Surgery: A New Standard for Heart Operations
- 3.Valve Replacement and Coronary Bypass Surgery Without a Full Sternotomy
- 4.Treating Aortic and Carotid Aneurysms With a Minimally Invasive Stent
- 5.Heart Failure and the Case for Heart Transplantation
- 6.The Artificial Heart (LVAD): A Bridge to Transplant
- 7.Tetralogy of Fallot and Eisenmenger Syndrome: Two Very Different Outcomes
- 8.A Cardiac Surgeon's Guide to Preventing Heart Disease