Skip to main content

Donate Today

Every gift, no matter the size, helps change children’s lives.

Twin-Twin Transfusion Syndrome (TTTS)

Twin-Twin Transfusion Syndrome (TTTS)

Learn more about the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment

What is twin-twin transfusion syndrome (TTTS)

Twin-twin transfusion syndrome (also called TTTS or twin to twin transfusion syndrome) is a condition in which the blood flows unequally between twins that share a placenta (monochorionic twins). TTTS occurs in about 10 to 15 percent of monochorionic, diamniotic (two amniotic sacs) twins.

What causes TTTS?

Illustration of twin-to-twin transfusion syndrome showing donor twin with low fluid and recipient twin with excess fluid
In twin-to-twin transfusion syndrome (Stage 1), one baby (right) has too much amniotic fluid while the other (left) has too little.

In twin-twin transfusion syndrome, there is an unequal sharing of blood that passes between twins through blood vessel connections on the surface of the placenta. One twin (called the donor twin) pumps blood to the other twin (called the recipient). This causes the recipient twin to receive too much blood and the donor twin to receive too little.

There are no known genetic causes of TTTS.

 

What are the symptoms of TTTS?

In TTTS, the increased volume of blood causes the recipient twin to produce more than the usual amount of urine, which can result in a large bladder, too much amniotic fluid (known as polyhydramnios) and hydrops, a prenatal form of heart failure.

The donor twin, who receives too little blood, produces less than the usual amount of urine. This results in low or no amniotic fluid surrounding it (oligohydramnios) and a small or absent bladder.

Without intervention, the condition can be fatal for both twins.

Women carrying babies with TTTS may experience abdominal discomfort from polyhydramnios, and if left untreated, potential rupture of membranes and premature labor.

Evaluation and diagnosis of TTTS

TTTS ultrasound
A fetal ultrasound helps doctors identify signs of TTTS and monitor both babies closely.

The most important step in evaluating a twin pregnancy for twin-twin transfusion syndrome is to determine whether the twins share a single placenta. An ultrasound examination early in pregnancy (during the first trimester) can best make this distinction.

Other features of TTTS include twins of the same gender, a difference in size between the twins, and a difference in the volume of amniotic fluid surrounding the two fetuses. A detailed sonographic assessment rules out any additional structural anomalies.

Twin-to-twin transfusion syndrome (TTTS) shown on 3D fetal ultrasound
Advanced 3D ultrasound imaging provides a clearer view of twins affected by TTTS.

Twin-twin transfusion syndrome can also have a huge impact on the cardiovascular system in twins. It is important that twins with TTTS are thoroughly evaluated for heart problems while in utero.

If you are referred to CHOP’s Center for Fetal Diagnosis and Treatment, you will be scheduled for a comprehensive, one-day evaluation. During this first visit, you will meet with multiple team members, including a genetic counselor, nurse coordinator and maternal-fetal medicine specialist. You’ll also meet with a research coordinator to discuss ongoing research studies.

Your evaluation will involve the following exams:

  • High-resolution ultrasound – An examination to rule out structural abnormalities in the twins. This exam will also confirm placental location and umbilical cord insertions into the placenta, assess amniotic fluid levels in each sac and assess blood flow patterns in specific blood vessels for each twin.
  • Fetal echocardiogram – A focused ultrasound of each twin’s heart to look for any related heart conditions. Twin-twin transfusion syndrome may cause serious cardiac strain in the recipient twin, resulting in enlargement of the heart and cardiac dysfunction. All twins evaluated for TTTS should undergo a fetal echocardiogram. Fetal cardiology experts from CHOP’s Fetal Heart Program have developed a cardiac scoring tool to determine how severe the condition has become for the twins. This score helps the medical team determine need for treatment. (See “The twin-twin transfusion syndrome: Spectrum of cardiovascular abnormality and development of a cardiovascular score to assess severity of disease.”)
  • Genetic amniocentesis – A procedure in which a small amount of amniotic fluid is removed from the sacs surrounding the fetuses and tested. In some cases, this genetic test may be needed if imaging studies identify structural malformations in addition to TTTS.

Accurate diagnosis is extremely important in distinguishing TTTS from other related anomalies, such as selective intrauterine growth restriction (sIUGR), also called selective fetal growth restriction (sFGR). TTTS and sIUGR/sFGR can both occur in monochorionic, diamniotic pregnancies, but are differentiated by the lack of polyhydramnios/oligohydramnios in sIUGR/sFGR. The management of the two conditions are different, and therefore it is important to have a thorough evaluation to determine the most accurate diagnosis.

  • Mark P. Johnson, MD, MS: Sometimes families come to us with the diagnosis of TTTS, and it turns out to be something more like Selective Intrauterine Growth Restriction or sIUGR. TTTS and sIUGR are related because they involve the same common shared placenta between the two babies. What differentiates them is the vascular connections between the two babies.

    Jack Rychik, MD: Often the differences between sIUGR and Twin-to-Twin Transfusion Syndrome can in fact be very subtle. Through the use of Doppler ultrasound, we can help tease out the facts that support one particular diagnosis versus another. And through Doppler echocardiography, we can identify the direction of blood flow the velocity of blood flow, and also determine patterns of blood flow.

    Mark P. Johnson, MD, MS: One of the things that we look at, of course, is cardiac changes. In TTTS, there is a very characteristic progressive series of changes that happens in the larger twin, due to the extra volume that comes from the smaller twin to the larger twin. 

    Jack Rychik, MD: Blood is exchanged from one twin, the donor, into the recipient, and then as a consequence, there's a huge cascade of hormonal changes that take place, that then brings about the cardiovascular manifestations in the recipient.

    Mark P. Johnson, MD, MS: In selective IUGR, there isn't this transfer of volume. You know, there's not this net shift of volume from one twin to the other. It's much more balanced. But that balance, you know, can be a tenuous balance. 

    Jack Rychik, MD: In the smaller twin, there is an abnormality of placental sharing. 

    Mark P. Johnson, MD, MS: The smaller baby has a much smaller portion of the placenta.

    Jack Rychik, MD: And the resistance in the umbilical artery of that particular twin, is much higher than normal, resulting in alterations in growth in the smaller twin. 

    Mark P. Johnson, MD, MS: Selective intrauterine growth restriction has been recognized for a long time, but it's only been in the last decade that we've been able to see that there are different forms of it.

    There's a milder form, which we call type 1. There's a more severe form that we call type 2, and then there's a new entity that's really only becoming understood, that's come out in the last few years. It's the type 3, selective IUGR. So if we were to look at a normal placenta, there would probably be a large number of connections between both fetuses.

    There's kind of a sense of balance there, so that shifts in blood in one direction would be offset by shifts in blood in the other direction. In type 1 selective IUGR, what you notice is perhaps a 60/40 distribution of placental area, but you see a decrease in the number of connections. That means that there can't be as dynamic a shift in blood volume between the two twins.

    When you go to the type 2 selective IUGR, you start to see a much smaller portion of placenta for the one that develops the intrauterine growth restriction. You also notice that the number of vessels, again, decreases even more. They tend to be balanced so that the number of artery to vein connections from the smaller to the bigger is still offset by artery to veins in the other direction, but the numbers are much, much fewer.

    And so again, it's this idea of dynamic sharing that is more restrictive, and that forces the fetus with the smaller portion of placenta to really try to survive on what it's got as far as placental mass. And the less volume of the placenta it has, the more it struggles. In the type 3 selective IUGR, they have just small, small proportion of the placenta.

    There tends to be a higher proportion of arteries connecting to veins, from the normal baby to the smaller baby, but it's a very small placental area, and that's led to the concept of rescue transfusion, the larger baby being able to send blood to the other side of the placenta. And so those connections are absolutely vital for the smaller baby's survival.

    The other characteristic feature in type 3 selective IUGR is a very big artery to artery connection. There can be rapid shifts in blood in either direction. These high volume shifts of blood from one baby to the other, result in the blood pressure going up and going down and going up and going down, and that appears to result in injury.

    So while this connection is vital to keeping the smaller baby alive, it can potentially result in a brain injury to the normal baby, and it can actually kill the smaller baby because of this just sudden rapid shift in blood pressure and volume. So in type 3 selective IUGR, it's a very, very dependent and very, very dynamic relationship between the two twins and the connections in the placenta.

    Jack Rychik, MD: The science and the practice of managing twin complications such as twin to twin transfusion syndrome or sIUGR perhaps, in effect has only existed for about a decade. 

    Mark P. Johnson, MD, MS: Understanding the TTTS story has been vital in our understanding of how to differentiate that problem from the selective IUGR problem.

    Jack Rychik, MD: High volume exposure to these patients is critical in being able to learn what to expect. 

    Mark P. Johnson, MD, MS: That high volume, that experience, and that collaboration is what makes us a really good place and allows us to really tailor management for each individual patient to optimize and try to achieve the best outcome possible.

Transcript Transcript

Your test results and specific treatment options will be discussed in detail on the day of your evaluation. If fetal intervention is recommended, you can also expect to meet with an advanced practice nurse to complete preoperative testing, and a social worker to arrange temporary accommodations in the Philadelphia area. Our team is here to help and support you and your family throughout this experience.

(Healthcare professionals: for more diagnostic tools and resources to help in the management of patients with complicated twin pregnancies, please refer to our monochorionic pregnancy resources for clinicians.)

Stages of TTTS

In TTTS, there is a characteristic series of pregnancy changes that happens due to the extra blood coming from the donor twin to the recipient twin.

Stage 1

In TTTS, unequal blood flow between twins sharing a placenta results in recipient twin (right) having too much amniotic fluid (polyhydramnios), and donor twin (left) with little or no amniotic fluid (oligohydramnios). This illustration depicts stage 1 TTTS.

Illustration of twin-to-twin transfusion syndrome showing donor twin with low fluid and recipient twin with excess fluid
Stage 1 TTTS: Unequal blood flow between twins sharing a placenta causes imbalance of amniotic fluid.

Stage 2

In stage 2 TTTS, the donor twin’s bladder (left) is not filling or emptying, because blood is being diverted from the kidneys to the brain, heart, and adrenal glands. This is a survival mode mechanism to preserve the functioning of these vital organs. The kidneys and bladder are still functioning, but more slowly than normal, so you don't see the bladder filling and emptying as normal.

Twin-to-twin transfusion syndrome (TTTS) stage 2 illustration
Stage 2 TTTS: Donor twin’s bladder does not fill or empty as expected.

Stage 3

In stage 3 TTTS, abnormal blood flow begins to affect one or both babies' heart function.

Twin-to-twin transfusion syndrome (TTTS) stage 3 illustration
Stage 3 TTTS: Abnormal blood flow affecting heart function.

 

Stage 4

In stage 4 TTTS, massive fluid buildup (hydrops) can develop, which puts both babies and mother at serious risk. Hydrops signals heart failure in the recipient twin. The recipient's heart is struggling to process all the extra blood being sent from the donor twin. 

Twin-to-twin transfusion syndrome (TTTS) stage 4 with hydrops illustration
Stage 4 TTTS: Massive fluid buildup (hydrops) signals heart failure in recipient twin and puts both babies and mother at serious risk.

 

Stage 5

In stage 5 TTTS, death of one or both twins while still in the uterus can occur.
 

Twin-twin transfusion syndrome treatment

Treatment for twin-twin transfusion syndrome may include any of the following:

  • Expectant management – In situations where surgery is not yet indicated (Stage 1 without additional risk factors), close monitoring with periodic ultrasound examinations is used to evaluate the condition of both twins and look for signs of progression. In some cases, a follow-up fetal echocardiogram is used as well to look for signs of cardiac changes; these may sometimes be seen before other changes.
  • Fetoscopic selective laser ablation – A minimally invasive surgery performed on the placenta to disconnect the communicating blood vessels. This procedure, also called selective laser photocoagulation (SLPC), stops the sharing of blood from the donor to the recipient, with the goal of halting the progression and then resolving the twin-twin transfusion syndrome. This is typically the preferred treatment for TTTS, depending on gestational age at presentation, location of the placenta and stage of TTTS.
  • Amnioreduction – Removal of excess amniotic fluid from the larger twin (recipient) which may help ease any pain or discomfort experienced by the mother due to fluid buildup. This is a temporary treatment option and may need to be repeated.
  • Selective cord occlusion – A minimally invasive surgery that stops the blood flow to one twin in order to maximize the outcome for the other twin. Selective cord occlusion procedures include radiofrequency ablation (RFA) and bipolar cord coagulation (BCC). This is considered a last resort option when the disease is very advanced and the at-risk twin is not going to survive. This intervention can protect the co-twin from neurologic impairment and/or death.

Mothers undergoing a fetal surgery procedure will stay in our Garbose Family Special Delivery Unit, the first birthing unit within a pediatric hospital dedicated to healthy mothers carrying babies with serious and life-threatening birth defects.

Watch our educational video series to learn more about the diagnosis and treatment of twin-to-twin transfusion syndrome. En Español »

  • Dennis Hasson: That day in the ultrasound room when that technician looked at us like, "You two know you're having twins?" 

    Marie Hasson: On the ultrasound you could see, you know, a lot of detail because it was a level II ultrasound. 

    Mark P. Johnson, MD, MS: It's very uncommon. Only 10, 15% of monochorionic twins develop this type of a syndrome. 

    Marie Hasson: You could tell they were both girls.

    Dennis Hasson: And for one moment it was exciting. 

    Nahal Khaled, MD, MPH: We still don't know the exact reason behind why certain monochorionic pregnancies are affected by twin-to-twin transfusion syndrome. 

    Marie Hasson: I could see the screen that something was wrong. There wasn't any fluid in the one sac at all. 

    Nahal Khaled, MD, MPH: It results from an imbalance in the flow between specific blood vessels that are in the placenta of monochorionic pregnancies.

    Mark P. Johnson, MD, MS: They don't share the placenta equally.

    Dennis Hasson: That doctor came in, took a look at these ultrasounds- 

    Marie Hasson: And he didn't say a word. He just was looking and kinda taking notes, and then he brought us into his office and shut the door and he said, "Man, this is gonna ... It's gonna be a very difficult pregnancy."

    Mark P. Johnson, MD, MS: Twin-to-twin transfusion syndrome is a complicated disorder. 

    Joy N. MacDonald, RN, BSN: The serious complication of monochorionic or identical twin pregnancies. 

    Mark P. Johnson, MD, MS: Monochorionic meaning that they share a placenta. 

    Susan R. Miesnik, MSN, CRNP: In a monochorionic diamniotic pregnancy there is one chorion, which is the outer sac, and each twin is in its own amnion so that it's like a single balloon with two filled balloons inside it.

    N. Scott Adzick, MD, MMM: One twin, the recipient twin, gets too much blood and the other twin, the donor twin, doesn't get enough blood. 

    Mark P. Johnson, MD, MS: Because the amount of oxygen and nutrition going to the donor baby is less than to the recipient baby, they grow at different rates. 

    Susan R. Miesnik, MSN, CRNP: One twin will end up being very large, having excessive amniotic fluid around it or polyhydramnios.

    Mark P. Johnson, MD, MS: The opposite kind of occurs in the smaller baby or the donor baby. 

    Susan R. Miesnik, MSN, CRNP: The other twin on ultrasound will show itself as being very small for gestational age. It will have decreased amniotic fluid or actually no fluid. 

    Mark P. Johnson, MD, MS: Over time the amniotic fluid around the donor baby shrinks away until literally the baby becomes shrink-wrapped along the side of the uterine wall or placenta.

    Susan R. Miesnik, MSN, CRNP: As this disease progresses you end up seeing cardiac changes in both of the babies. 

    Jack Rychik, MD: When we begin to see cardiac changes one can have significant complications as a consequence which can lead to, either residual issues that these fetuses face, or even death in either the recipient or the donor.

Transcript Transcript

Follow-up care for TTTS

If you undergo fetal intervention, your care team will provide detailed postoperative care and instructions. We will schedule you for an ultrasound exam at our Center one week after your procedure to re-evaluate the health of your twins.

After that exam, we recommend returning to your local maternal-fetal medicine specialist for weekly ultrasound examinations for at least three weeks. Ultrasound exams will then be scheduled according to your doctor for the duration of your pregnancy.

Volumes & outcomes

When seeking the best hospital for TTTS treatment, we encourage you to ask about any treatment team about their volumes and outcomes. Our team at the Center for Fetal Diagnosis and Treatment cares for a high volume of monochorionic twin pregnancies each year. Since 1995, more than 4,978 complicated multiple gestation pregnancies have been referred to the Center for Fetal Diagnosis and Treatment, including 2,592 referrals for TTTS.

This experience helps to continuously expand our understanding of these complex pregnancies and our ability to differentiate between conditions such as TTTS, selective fetal growth restriction (sFGR), twin anemia polycythemia sequence (TAPS), and twin reversed arterial perfusion (TRAP) sequence.

Tour

Tour our Fetal Center

The Wood Center for Fetal Diagnosis and Treatment has cared for many families and will help you through your journey, too.

Ultrasound at CHOP

What to expect

From the moment of referral through delivery and postnatal care, your family can expect a supportive experience when you come to us with a diagnosis of a birth defect.

Resources to help

Twin-Twin Transfusion Syndrome (TTTS) Resources

Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment Resources

Learning your baby has a birth defect is a life-changing experience. We want you to know that you are not alone. To help you find answers to your questions, we've created this list of educational health resources.

Patient stories

Our Stories
When their twins were diagnosed prenatally with twin-to-twin transfusion syndrome, a New Jersey couple found hope at Children’s Hospital of Philadelphia.
Our Stories
Identical twins Vale and Shea were diagnosed before birth with twin-twin transfusion syndrome (TTTS), and underwent fetoscopic laser ablation at CHOP.
Jump back to top