Coarctation of the aorta is a birth defect in which a part of the aorta is narrower than usual. If the narrowing is severe enough and if it is not diagnosed, the baby may have serious problems and may need surgery or other procedures soon after birth. For this reason, coarctation of the aorta is often considered a critical congenital heart defect. The defect occurs when a baby’s aorta does not form correctly as the baby grows and develops during pregnancy. The narrowing of the aorta usually happens in the part of the blood vessel just after the arteries branch off to take blood to the head and arms, near the patent ductus arteriosus, although sometimes the narrowing occurs before or after the ductus arteriosus. In some babies with coarctation, it is thought that some tissue from the wall of ductus arteriosus blends into the tissue of the aorta. When the tissue tightens and allows the ductus arteriosus to close normally after birth, this extra tissue may also tighten and narrow the aorta.

The narrowing, or coarctation, blocks normal blood flow to the body. This can back up flow into the left ventricle of the heart, making the muscles in this ventricle work harder to get blood out of the heart. Since the narrowing of the aorta is usually located after arteries branch to the upper body, coarctation in this region can lead to normal or high blood pressure and pulsing of blood in the head and arms and low blood pressure and weak pulses in the legs and lower body.

If the condition is very severe, enough blood may not be able to get through to the lower body. The extra work on the heart can cause the walls of the heart to become thicker in order to pump harder. This eventually weakens the heart muscle. If the aorta is not widened, the heart may weaken enough that it leads to heart failure. Coarctation of the aorta often occurs with other congenital heart defects.

The heart is an organ, about the size of a fist. It is made of muscle and pumps blood through the body. Blood is carried through the body in blood vessels, or tubes, called arteries and veins. The process of moving blood through the body is called circulation. Together, the heart and vessels make up the cardiovascular system.

Structure of the Heart

The heart has four chambers (two atria and two ventricles). There is a wall (septum) between the two atria and another wall between the two ventricles. Arteries and veins go into and out of the heart. Arteries carry blood away from the heart and veins carry blood to the heart. The flow of blood through the vessels and chambers of the heart is controlled by valves.

Blood Flow Through the Heart

(Abbreviations refer to labels in the illustration)

The heart pumps blood to all parts of the body. Blood provides oxygen and nutrients to the body and removes carbon dioxide and wastes. As blood travels through the body, oxygen is used up, and the blood becomes oxygen poor.

1. Oxygen-poor blood returns from the body to the heart through the superior vena cava (SVC) and inferior vena cava (IVC), the two main veins that bring blood back to the heart.
2. The oxygen-poor blood enters the right atrium (RA), or the right upper chamber of the heart.
3. From there, the blood flows through the tricuspid valve (TV) into the right ventricle (RV), or the right lower chamber of the heart.
4. The right ventricle (RV) pumps oxygen-poor blood through the pulmonary valve (PV) into the main pulmonary artery (MPA).
5. From there, the blood flows through the right and left pulmonary arteries into the lungs.
6. In the lungs, oxygen is put into the blood and carbon dioxide is taken out of the blood during the process of breathing. After the blood gets oxygen in the lungs, it is called oxygen-rich blood.
7. Oxygen-rich blood flows from the lungs back into the left atrium (LA), or the left upper chamber of the heart, through four pulmonary veins.
8. Oxygen-rich blood then flows through the mitral valve (MV) into the left ventricle (LV), or the left lower chamber.
9. The left ventricle (LV) pumps the oxygen-rich blood through the aortic valve (AoV) into the aorta (Ao), the main artery that takes oxygen-rich blood out to the rest of the body.

Heart: Heart Defects

One very common form of interatrial septum pathology is patent foramen ovale, which occurs when the septum primum does not close at birth, and the fossa ovalis is unable to fuse. The word patent is from the Latin root patens for “open.” It may be benign or asymptomatic, perhaps never being diagnosed, or in extreme cases, it may require surgical repair to close the opening permanently. As much as 20–25 percent of the general population may have a patent foramen ovale, but fortunately, most have the benign, asymptomatic version. Patent foramen ovale is normally detected by auscultation of a heart murmur (an abnormal heart sound) and confirmed by imaging with an echocardiogram. Despite its prevalence in the general population, the causes of patent ovale are unknown, and there are no known risk factors. In nonlife-threatening cases, it is better to monitor the condition than to risk heart surgery to repair and seal the opening.

Coarctation of the aorta is a congenital abnormal narrowing of the aorta that is normally located at the insertion of the ligamentum arteriosum, the remnant of the fetal shunt called the ductus arteriosus. If severe, this condition drastically restricts blood flow through the primary systemic artery, which is life threatening. In some individuals, the condition may be fairly benign and not detected until later in life. Detectable symptoms in an infant include difficulty breathing, poor appetite, trouble feeding, or failure to thrive. In older individuals, symptoms include dizziness, fainting, shortness of breath, chest pain, fatigue, headache, and nosebleeds. Treatment involves surgery to resect (remove) the affected region or angioplasty to open the abnormally narrow passageway. Studies have shown that the earlier the surgery is performed, the better the chance of survival.

A patent ductus arteriosus is a congenital condition in which the ductus arteriosus fails to close. The condition may range from severe to benign. Failure of the ductus arteriosus to close results in blood flowing from the higher pressure aorta into the lower pressure pulmonary trunk. This additional fluid moving toward the lungs increases pulmonary pressure and makes respiration difficult. Symptoms include shortness of breath (dyspnea), tachycardia, enlarged heart, a widened pulse pressure, and poor weight gain in infants. Treatments include surgical closure (ligation), manual closure using platinum coils or specialized mesh inserted via the femoral artery or vein, or nonsteroidal anti-inflammatory drugs to block the synthesis of prostaglandin E2, which maintains the vessel in an open position. If untreated, the condition can result in congestive heart failure.

Septal defects are not uncommon in individuals and may be congenital or caused by various disease processes. Tetralogy of Fallot is a congenital condition that may also occur from exposure to unknown environmental factors; it occurs when there is an opening in the interventricular septum caused by blockage of the pulmonary trunk, normally at the pulmonary semilunar valve. This allows blood that is relatively low in oxygen from the right ventricle to flow into the left ventricle and mix with the blood that is relatively high in oxygen. Symptoms include a distinct heart murmur, low blood oxygen percent saturation, dyspnea or difficulty in breathing, polycythemia, broadening (clubbing) of the fingers and toes, and in children, difficulty in feeding or failure to grow and develop. It is the most common cause of cyanosis following birth. The term “tetralogy” is derived from the four components of the condition, although only three may be present in an individual patient: pulmonary infundibular stenosis (rigidity of the pulmonary valve), overriding aorta (the aorta is shifted above both ventricles), ventricular septal defect (opening), and right ventricular hypertrophy (enlargement of the right ventricle). Other heart defects may also accompany this condition, which is typically confirmed by echocardiography imaging. Tetralogy of Fallot occurs in approximately 400 out of one million live births. Normal treatment involves extensive surgical repair, including the use of stents to redirect blood flow and replacement of valves and patches to repair the septal defect, but the condition has a relatively high mortality. Survival rates are currently 75 percent during the first year of life; 60 percent by 4 years of age; 30 percent by 10 years; and 5 percent by 40 years.

In the case of severe septal defects, including both tetralogy of Fallot and patent foramen ovale, failure of the heart to develop properly can lead to a condition commonly known as a “blue baby.” Regardless of normal skin pigmentation, individuals with this condition have an insufficient supply of oxygenated blood, which leads to cyanosis, a blue or purple coloration of the skin, especially when active.

Septal defects are commonly first detected through auscultation, listening to the chest using a stethoscope. In this case, instead of hearing normal heart sounds attributed to the flow of blood and closing of heart valves, unusual heart sounds may be detected. This is often followed by medical imaging to confirm or rule out a diagnosis. In many cases, treatment may not be needed. Some common congenital heart defects are illustrated in Figure.

Figure. Congenital Heart Defects (a) A patent foramen ovale defect is an abnormal opening in the interatrial septum, or more commonly, a failure of the foramen ovale to close. (b) Coarctation of the aorta is an abnormal narrowing of the aorta. (c) A patent ductus arteriosus is the failure of the ductus arteriosus to close. (d) Tetralogy of Fallot includes an abnormal opening in the interventricular septum.

During prenatal development, the fetal circulatory system is integrated with the placenta via the umbilical cord so that the fetus receives both oxygen and nutrients from the placenta. However, after childbirth, the umbilical cord is severed, and the newborn’s circulatory system must be reconfigured. When the heart first forms in the embryo, it exists as two parallel tubes derived from mesoderm and lined with endothelium, which then fuse together. As the embryo develops into a fetus, the tube-shaped heart folds and further differentiates into the four chambers present in a mature heart. Unlike a mature cardiovascular system, however, the fetal cardiovascular system also includes circulatory shortcuts, or shunts. A shunt is an anatomical (or sometimes surgical) diversion that allows blood flow to bypass immature organs such as the lungs and liver until childbirth.

The placenta provides the fetus with necessary oxygen and nutrients via the umbilical vein. (Remember that veins carry blood toward the heart. In this case, the blood flowing to the fetal heart is oxygenated because it comes from the placenta. The respiratory system is immature and cannot yet oxygenate blood on its own.) From the umbilical vein, the oxygenated blood flows toward the inferior vena cava, all but bypassing the immature liver, via the ductus venosus shunt (image). The liver receives just a trickle of blood, which is all that it needs in its immature, semifunctional state. Blood flows from the inferior vena cava to the right atrium, mixing with fetal venous blood along the way.

Although the fetal liver is semifunctional, the fetal lungs are nonfunctional. The fetal circulation therefore bypasses the lungs by shifting some of the blood through the foramen ovale, a shunt that directly connects the right and left atria and avoids the pulmonary trunk altogether. Most of the rest of the blood is pumped to the right ventricle, and from there, into the pulmonary trunk, which splits into pulmonary arteries. However, a shunt within the pulmonary artery, the ductus arteriosus, diverts a portion of this blood into the aorta. This ensures that only a small volume of oxygenated blood passes through the immature pulmonary circuit, which has only minor metabolic requirements. Blood vessels of uninflated lungs have high resistance to flow, a condition that encourages blood to flow to the aorta, which presents much lower resistance. The oxygenated blood moves through the foramen ovale into the left atrium, where it mixes with the now deoxygenated blood returning from the pulmonary circuit. This blood then moves into the left ventricle, where it is pumped into the aorta. Some of this blood moves through the coronary arteries into the myocardium, and some moves through the carotid arteries to the brain.

The descending aorta carries partially oxygenated and partially deoxygenated blood into the lower regions of the body. It eventually passes into the umbilical arteries through branches of the internal iliac arteries. The deoxygenated blood collects waste as it circulates through the fetal body and returns to the umbilical cord. Thus, the two umbilical arteries carry blood low in oxygen and high in carbon dioxide and fetal wastes. This blood is filtered through the placenta, where wastes diffuse into the maternal circulation. Oxygen and nutrients from the mother diffuse into the placenta and from there into the fetal blood, and the process repeats.

The heart folds quickly like origami and now starts beating. This begins with the formation of two tubes and beats spontaneously by week 4 of development.

Developing rapidly and early, the heart is the first organ to function in the embryo, and it takes up most of the room in the fetus's midsection in the first few weeks of its life. During its initial stages of development, the fetal heart actually resembles those of other animals. In its tubelike, two-chambered phase, the fetal heart resembles that of a fish. In its three-chambered phase, the heart looks like that of a frog. As the atria and then the ventricles start to separate, the human heart resembles that of a turtle, which has a partial septum in its ventricle. The final, four-chambered design is common to mammals and birds. The four chambers allow low-pressure circulation to the lungs and high pressure circulation to the rest of the body.

Development of the Heart

The human heart is the first functional organ to develop. It begins beating and pumping blood around day 21 or 22, a mere three weeks after fertilization. This emphasizes the critical nature of the heart in distributing blood through the vessels and the vital exchange of nutrients, oxygen, and wastes both to and from the developing baby. The critical early development of the heart is reflected by the prominent heart bulge that appears on the anterior surface of the embryo.

The heart forms from an embryonic tissue called mesoderm around 18 to 19 days after fertilization. Mesoderm is one of the three primary germ layers that differentiates early in development that collectively gives rise to all subsequent tissues and organs. The heart begins to develop near the head of the embryo in a region known as the cardiogenic area. Following chemical signals called factors from the underlying endoderm (another of the three primary germ layers), the cardiogenic area begins to form two strands called the cardiogenic cords (Figure 19.36). As the cardiogenic cords develop, a lumen rapidly develops within them. At this point, they are referred to as endocardial tubes. The two tubes migrate together and fuse to form a single primitive heart tube. The primitive heart tube quickly forms five distinct regions. From head to tail, these include the truncus arteriosus, bulbus cordis, primitive ventricle, primitive atrium, and the sinus venosus. Initially, all venous blood flows into the sinus venosus, and contractions propel the blood from tail to head, or from the sinus venosus to the truncus arteriosus. This is a very different pattern from that of an adult.

Development of the Human Heart This diagram outlines the embryological development of the human heart during the first eight weeks and the subsequent formation of the four heart chambers.

The five regions of the primitive heart tube develop into recognizable structures in a fully developed heart. The truncus arteriosus will eventually divide and give rise to the ascending aorta and pulmonary trunk. The bulbus cordis develops into the right ventricle. The primitive ventricle forms the left ventricle. The primitive atrium becomes the anterior portions of both the right and left atria, and the two auricles. The sinus venosus develops into the posterior portion of the right atrium, the SA node, and the coronary sinus.

As the primitive heart tube elongates, it begins to fold within the pericardium, eventually forming an S shape, which places the chambers and major vessels into an alignment similar to the adult heart. This process occurs between days 23 and 28. The remainder of the heart development pattern includes development of septa and valves, and remodeling of the actual chambers. Partitioning of the atria and ventricles by the interatrial septum, interventricular septum, and atrioventricular septum is complete by the end of the fifth week, although the fetal blood shunts remain until birth or shortly after. The atrioventricular valves form between weeks five and eight, and the semilunar valves form between weeks five and nine.

A normal pregnancy lasts nine months. Each three-month period of pregnancy is called a trimester. During each trimester, the fetus grows and develops. Regular medical checkups and prenatal tests are very important. They can:

• Help keep you and your baby healthy
• Spot problems with your baby (if there are any). In some cases, health care professionals can treat the problem before your baby is born. But even when they cannot, it can still be helpful to know about the problem early on. That gives you time to learn about your baby's condition and prepare for any challenges you may face after the baby is born.
• Prevent problems during delivery. For example, if your baby is breech (bottom first or feet first, instead of head first), you may need to have a Cesarean section to avoid complications.

Besides getting medical care, there are other things you can do to keep your baby as healthy as possible. It's important not to drink or smoke. Try to eat a healthy diet and make sure to take care of any health problems you have during pregnancy.

In a developing embryo, the heart has developed enough by day 21 post-fertilization to begin beating. Circulation patterns are clearly established by the fourth week of embryonic life. It is critical to the survival of the developing human that the circulatory system forms early to supply the growing tissue with nutrients and gases, and to remove waste products. Blood cells and vessel production in structures outside the embryo proper called the yolk sac, chorion, and connecting stalk begin about 15 to 16 days following fertilization. Development of these circulatory elements within the embryo itself begins approximately 2 days later. You will learn more about the formation and function of these early structures when you study the chapter on development. During those first few weeks, blood vessels begin to form from the embryonic mesoderm. The precursor cells are known as hemangioblasts. These in turn differentiate into angioblasts, which give rise to the blood vessels and pluripotent stem cells, which differentiate into the formed elements of blood. (Seek additional content for more detail on fetal development and circulation.) Together, these cells form masses known as blood islands scattered throughout the embryonic disc. Spaces appear on the blood islands that develop into vessel lumens. The endothelial lining of the vessels arise from the angioblasts within these islands. Surrounding mesenchymal cells give rise to the smooth muscle and connective tissue layers of the vessels. While the vessels are developing, the pluripotent stem cells begin to form the blood.

Vascular tubes also develop on the blood islands, and they eventually connect to one another as well as to the developing, tubular heart. Thus, the developmental pattern, rather than beginning from the formation of one central vessel and spreading outward, occurs in many regions simultaneously with vessels later joining together. This angiogenesis—the creation of new blood vessels from existing ones—continues as needed throughout life as we grow and develop.

Blood vessel development often follows the same pattern as nerve development and travels to the same target tissues and organs. This occurs because the many factors directing growth of nerves also stimulate blood vessels to follow a similar pattern. Whether a given vessel develops into an artery or a vein is dependent upon local concentrations of signaling proteins.

As the embryo grows within the mother’s uterus, its requirements for nutrients and gas exchange also grow. The placenta—a circulatory organ unique to pregnancy—develops jointly from the embryo and uterine wall structures to fill this need. Emerging from the placenta is the umbilical vein, which carries oxygen-rich blood from the mother to the fetal inferior vena cava via the ductus venosus to the heart that pumps it into fetal circulation. Two umbilical arteries carry oxygen-depleted fetal blood, including wastes and carbon dioxide, to the placenta. Remnants of the umbilical arteries remain in the adult. (Seek additional content for more information on the role of the placenta in fetal circulation.)

There are three major shunts—alternate paths for blood flow—found in the circulatory system of the fetus. Two of these shunts divert blood from the pulmonary to the systemic circuit, whereas the third connects the umbilical vein to the inferior vena cava. The first two shunts are critical during fetal life, when the lungs are compressed, filled with amniotic fluid, and nonfunctional, and gas exchange is provided by the placenta. These shunts close shortly after birth, however, when the newborn begins to breathe. The third shunt persists a bit longer but becomes nonfunctional once the umbilical cord is severed. The three shunts are as follows (image):

• The foramen ovale is an opening in the interatrial septum that allows blood to flow from the right atrium to the left atrium. A valve associated with this opening prevents backflow of blood during the fetal period. As the newborn begins to breathe and blood pressure in the atria increases, this shunt closes. The fossa ovalis remains in the interatrial septum after birth, marking the location of the former foramen ovale.
• The ductus arteriosus is a short, muscular vessel that connects the pulmonary trunk to the aorta. Most of the blood pumped from the right ventricle into the pulmonary trunk is thereby diverted into the aorta. Only enough blood reaches the fetal lungs to maintain the developing lung tissue. When the newborn takes the first breath, pressure within the lungs drops dramatically, and both the lungs and the pulmonary vessels expand. As the amount of oxygen increases, the smooth muscles in the wall of the ductus arteriosus constrict, sealing off the passage. Eventually, the muscular and endothelial components of the ductus arteriosus degenerate, leaving only the connective tissue component of the ligamentum arteriosum.
• The ductus venosus is a temporary blood vessel that branches from the umbilical vein, allowing much of the freshly oxygenated blood from the placenta—the organ of gas exchange between the mother and fetus—to bypass the fetal liver and go directly to the fetal heart. The ductus venosus closes slowly during the first weeks of infancy and degenerates to become the ligamentum venosum.

Overview

Blood vessels begin to form from the embryonic mesoderm. The precursor hemangioblasts differentiate into angioblasts, which give rise to the blood vessels and pluripotent stem cells that differentiate into the formed elements of the blood. Together, these cells form blood islands scattered throughout the embryo. Extensions known as vascular tubes eventually connect the vascular network. As the embryo grows within the mother’s womb, the placenta develops to supply blood rich in oxygen and nutrients via the umbilical vein and to remove wastes in oxygen-depleted blood via the umbilical arteries. Three major shunts found in the fetus are the foramen ovale and ductus arteriosus, which divert blood from the pulmonary to the systemic circuit, and the ductus venosus, which carries freshly oxygenated blood high in nutrients to the fetal heart.

In a 2019 study, using 2010-2014 data from 39 population-based birth defects surveillance programs, the Centers for Disease Control and Prevention (CDC) estimated that about 4 of every 10,000 babies born had coarctation of the aorta. This means about 2,200 babies are born each year in the United States with coarctation of the aorta. In other words, about 1 in every 1,800 babies born in the United States each year are born with coarctation of the aorta.

The causes of heart defects, including coarctation of the aorta, among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. Heart defects, like coarctation of the aorta, are also thought to be caused by a combination of genes and other risk factors, such as things the mother comes in contact with in the environment, what the mother eats or drinks, or medicines the mother uses.

Coarctation of the aorta is usually diagnosed after the baby is born. How early in life the defect is diagnosed usually depends on how mild or severe the symptoms are. Newborn screening using pulse oximetry during the first few days of life may or may not detect coarctation of the aorta.

In babies with a more serious condition, early signs usually include:

• pale skin
• irritability
• heavy sweating
• difficulty breathing

Detection of the defect is often made during a physical exam. In infants and older individuals, the pulse will be noticeably weaker in the legs or groin than it is in the arms or neck, and a heart murmur—an abnormal whooshing sound caused by disrupted blood flow—may be heard through a doctor’s stethoscope. Older children and adults with coarctation of the aorta often have high blood pressure in the arms.

Once suspected, an echocardiogram is the most commonly used test to confirm the diagnosis. An echocardiogram is an ultrasound of the heart that can show problems with the structure of the heart and the blood flow through it, and how well the heart is working. It will show the location and severity of the coarctation and whether any other heart defects are present. Other tests to measure the function of the heart may be used including chest x-ray, electrocardiogram (EKG), magnetic resonance imaging (MRI), and cardiac catheterization.

Coarctation of the aorta is often considered a critical congenital heart defect (critical CHD) because if the narrowing is severe enough and it is not diagnosed, the baby may have serious problems soon after birth. CCHDs also can be detected with newborn pulse oximetry screening. Pulse oximetry is a simple bedside test to determine the amount of oxygen in a baby’s blood. Low levels of oxygen in the blood can be a sign of a CCHD. Newborn screening using pulse oximetry can identify some infants with a CCHD, like coarctation of the aorta, before they show any symptoms.

No matter what age the defect is diagnosed, the narrow aorta will need to be widened once symptoms are present. This can be done with surgery or a procedure called balloon angioplasty, which is done during a cardiac catheterization. A balloon angioplasty is a procedure that uses a thin, flexible tube, called a catheter, which is inserted into a blood vessel and directed to the aorta. When the catheter reaches the narrow area of the aorta, a balloon at the tip is inflated to expand the blood vessel. Sometimes a mesh-covered tube (stent) is inserted to keep the vessel open. The stent is used more often to initially widen the aorta or re-widen it if the aorta narrows again after surgery has been performed. During surgery to correct a coarctation, the narrow portion is removed and the aorta is reconstructed or patched to allow blood to flow normally through the aorta.

Even after surgery, children with a coarctation of the aorta often have high blood pressure that is treated with medicine. It is important for children and adults with coarctation of the aorta to follow up regularly with a cardiologist (a heart doctor) to monitor their progress and check for other health conditions that might develop as they get older.

Coarctation of the aorta is a birth defect in which a part of the aorta is narrower than usual. To learn what it’s like to live with this condition or how families are affected, read these real stories from people living with coarctation of the aorta.

Nicholas’s Story– Written by his mom, Amy

Nicholas was born with a critical congenital heart defect (critical CHD) called coarctation of the aorta. Read his inspirational story below about the hardships he faced early in life, but how he is now a poster child for successful heart surgery.

“Are you sitting down? Your baby has a serious heart defect.” This phone call was supposed to be my mother, congratulating us as we brought our baby home for the first time. Instead, it marked the beginning of a whirlwind, which resulted in three hospitals, a helicopter ride and heart surgery, all in the first four days of Nicholas’ life. Nicholas was born with a serious heart defect called critical coarctation of the aorta, a severe narrowing in his aorta cutting off blood flow to the lower half of his body.

The first couple of years of Nick’s life were filled with caution, fear and anxiety, and they were consumed by doctors’ visits. This was compounded by the fact that there were always two other siblings in tow. The inside of my minivan was decorated with the hundreds of “good patient” stickers the three of them had collected. These stickers meant Nicholas had made it another day.

Truthfully, Nicholas is a poster child for successful surgery. He has done so well that the doctors’ visits have tapered off to a bi-annual cardiology visit. He is able to participate in sports with soccer and swimming topping his list, although hip-hop dancing is his favorite. Most days, it’s easy to forget that he has a serious defect. Yet, occasionally, I catch myself wondering what his future holds. Until recently, children with severe heart defects didn’t survive and there is a huge gap of information about adults with heart defects.

There are still questions: How? Why? What’s next? No one really knows. I do know, that I will do everything I can to make sure he lives a long and healthy life.

Shandler’s Story – Written by his mom, Tricia

Shandler was born with a critical congenital heart defect (CCHD). His mother shares the family’s story about how Shandler beat all odds against him for survival. Today, Shandler is a happy, energetic boy. Read his full story below.

Our twins, a boy and girl, were born six weeks premature in 2002. Our son, Shandler, was born with two congenital heart defects: coarctation of the aorta and bicuspid aortic valve. Coarctation of the aorta is a narrowing of part of the aorta (the major artery that supplies oxygenated blood to the entire body). Bicuspid aortic valve is an aortic valve that has two leaflets, instead of three. Shandler was fairly small, weighing only 4 pounds. At four days old, he began to have difficulty breathing. The doctors detected a heart murmur, and diminished pulses in his legs and feet. Through echocardiography (ultrasound of the heart), the congenital heart defects were found. Three days later, the heart surgeon repaired the coarctation. There is no intervention for his bicuspid aortic valve.

Our family was absolutely terrified that he wouldn’t survive major heart surgery because of how tiny he was. He did well for about two weeks after surgery, and then he developed complications (a risk with any surgical procedure). Fluid collected around his lungs, making it difficult for him to breathe. He required a ventilator to assist with breathing and chest tubes placed to drain the fluid. After six weeks of no improvement in his condition, as well as developing bacterial meningitis (which almost took his life), he was taken back into surgery. Within 24 hours, he was able to breathe on his own again. After 11 weeks in the newborn intensive care unit, Shandler was finally able to come home, to be reunited with his twin sister and live with our family. However, he was discharged with a feeding tube. This was necessary because his heart condition caused fatigue during feedings, and he was not able to take the full amount necessary by mouth.

Shortly after coming home, Shandler developed a condition called dysphagia, due to severe acid reflux. Dysphagia is a problem with swallowing. Shandler started refusing to eat. He later had a feeding tube surgically placed, which was how he received his nutrition for several years. Just before turning four, he was finally eating and drinking enough to have the tube removed.

His heart condition made him extremely fragile the first few years of life. As an infant, he was hospitalized for several weeks (in critical condition) in the pediatric intensive care unit, with complications from flu. At age two, he contracted respiratory syncytial virus (RSV) and was hospitalized for 1 week due to breathing difficulty.

Seeing Shandler today, you wouldn’t know he had such a rough start in life. Although he is small for his age, he is able to run and play with his peers, and eats everything in sight!! However, he does require yearly follow-up by a cardiologist, a heart doctor. Through echocardiography, they look for any signs of aortic narrowing again, as well as checking the function of his bicuspid aortic valve. Complications of bicuspid aortic valve include: congestive heart failure, leakage of blood through the valve back into the heart, and narrowing of the valve’s opening. The valve will require repair or replacement if this occurs. Shandler is never allowed to participate in contact sports (football, hockey and wrestling). Despite this restriction, he is a bright, happy boy, who loves to play jokes on others (especially his sisters). He enjoys building things, reading Harry Potter books, and is a whiz in math and other school subjects. He will always have his “battle scar”, although he jokes that it “makes him look tough”!! We have been truly blessed to have Shandler in our family.