Congenital Heart Defects, Ellen’s Story: Part 2
When we left off at the end of Ellen’s Story: Part 1, we were awaiting the results of her monitor and recheck with her cardiologist. Luckily, all is well on that front. No new surprises. Actually, her heart looks better on echo and the cardiologist was thrilled with her progress. That means, thankfully, this is not a post about pediatric arrhythmias and EKGs or other surprises we found, but it is about congenital heart defects. It is also about newborn feeding difficulties. Why the heck is a paramedic blog talking about newborn feeding difficulties? Well, we will get there, but first, congenital heart defects.
Shopping cart command established, big brother is off looking for cookies.
Congenital heart defects affect about 1%, or 40,000, births per year in the US. They are the most common birth defect, with 1 in 4 of those with a heart defect having a critical heart defect that will require surgery in the first year of life. They are the leading cause of birth-defect associated infant illness and death. Deaths typically occur in the first 28 days of life. About 15% of congenital heart defects are related to genetics, while other causes are generally unknown. They are thought to be associated with environmental factors, mom’s health and diet, and maternal medication use. 97% of newborns born with non-critical congenital heart defects survive to one year, with 75% of those born with critical congenital heart defects surviving to one year. Ventricular septal defects are the most common, occurring in about 1 in every 240 births in the US according to the CDC. Most heart defects are co-occurring, meaning most kids don’t have just one type. Source
Critical congenital heart defects are defined as those that will require surgery in the first year of life. Non-critical heart defects may still require surgery, however can typically be managed medically initially.
Heart defects are also classified as cyanotic or acyanotic congenital heart disease. Cyanotic heart disease reduces the amount of oxygen delivered to the body. Acyanotic disease pumps blood abnormally throughout the body, but it is oxygenated.
Non-critical congenital heart defects:
Atrial Septal Defect: A hole between the left and right atria, causing a back flow from the left atria to the right which increases the amount of blood pumped to the lungs. This can lead to right heart enlargement, exertional dyspnea such as with feeding, fatigue, edema, arrythmias or a heart murmur. Left untreated, these can also lead to stroke, pulmonary hypertension, and early death. Depending on their size, some may close on their own, others require repair through placement of a patch or plug via cardiac catheterization or open heart surgery. In Ellen’s case, and in most kids, they have serial echocardiograms, EKGs, and Ellen was placed on furosemide to reduce some of the workload on the right side of her heart. Other kids are placed on beta blockers for arrythmias or on anticoagulants to reduce the risk of clots. Source
These differ from a Patent Foramen Ovale, in that the ASD is a condition you are born with, or is present before birth and is considered a structural abnormality. Everyone has a PFO, and most close on their own, and are not considered a congenital heart defect. Source
Atrioventricular Septal Defect: In this condition, an ASD and VSD are present with atrioventricular valves not formed. These require surgical repair as they may lead to pulmonary hypertension and heart failure. They may be related to genetic conditions such as Down syndrome. Can be detected before birth, but symptoms include exertional dyspnea, especially while feeding, fatigue, sweating while feeding, arrythmias. Source
Ventricular Septal Defect: These are the most common, with small holes only causing a heart murmur, while larger ones cause exertional dyspnea, trouble feeding, growth delays and exercise intolerance. Some may close on their own, however larger ones will need surgical repair. Source
Critical congenital heart defects.
Coarctation of the Aorta: A narrowing of the aorta that causes the left ventricle to work harder to pump blood against the increased resistance. Symptoms include sweating, difficulty feeding, dyspnea, irritability. May lead to heart failure, chronic high blood pressure, aortic aneurysm, aortic dissection, coronary artery disease and death. Source
Double-outlet Right Ventricle: Aorta connects to the right ventricle instead of the left. No arteries connect to the left ventricle. Oxygenated blood moves through a VSD from the left ventricle to the right to allow some oxygenation to occur. Symptoms can include tachypnea, tachycardia, poor feeding, fatigue, sweating, edema, cyanosis. Source
dextro-Transposition of the Great Arteries: The pulmonary artery and the aorta are switched in position; occurs in about 1,153 births each year. In this condition, oxygen rich blood from the left side of the heart is pumped back to the lungs via the pulmonary artery, and oxygen poor blood from the right side of the heart is returned to systemic circulation via the aorta. Typically, these kids also have VSDs that allow for some mixture of oxygenated blood to be delivered to the body. These kids have dyspnea, hypotension, poor feeding, and cyanosis. Source
Ebstein Anomaly: Tricuspid valve is not formed correctly, causing tricuspid regurgitation; can cause hypertrophy leading to heart failure. Symptoms include exertional dyspnea, cyanosis, fatigue, palpitations. Source
Hypoplastic Left Heart Syndrome: The left side of the heart is not formed correctly, including the left ventricle, the mitral and aortic valves, and the ascending aorta. Affects 1 in 3841 births. Symptoms include dyspnea, hypotension, cyanosis, pounding heart. Source
Interrupted Aortic Arch: Part of the aortic arch is missing; broken into three types depending on the location of the interruption. These patients often have a genetic condition called DiGeorge syndrome with includes low calcium, developmental delay and immune system abnormalities. Source
Pulmonary Atresia: Condition in which the pulmonary valve does not develop, preventing blood from going from the right ventricle to the lungs. The foramen ovale typically remains open to allow for blood flow to the lungs. Source
Single Ventricle: Condition in which one or both of the ventricles are under developed and cannot work effectively; or they are missing a valve. These are one of the most complex of the heart defects, typically needing multiple surgeries, but they are thankfully rare occurring in about 5 of 100,000 births. Source
Tetralogy of Fallot: Made up of four defects: ventricular septal defect, pulmonary stenosis, enlarged aorta that arises from both ventricles directly over the VSD and right ventricular hypertrophy. This occurs in 1 in 2518 births. Source
Total Anomalous Pulmonary Venous Return: The four pulmonary veins connect to the right atrium, instead of the left. There are three types, all include an atrial septal defect to allow for blood flow to the left atrium. Source
Tricuspid Atresia: The tricuspid valve is missing and the right ventricle is underdeveloped. Occurs in 2 of every 10,000 live births. Symptoms include cyanosis, dyspnea, fatigue, especially during feedings, poor growth, edema. Source
Truncus Arteriosus: Condition in which only one great vessel leaves the heart. Instead of a separate pulmonary artery and aorta, there is only one vessel with one large valve, typically sitting over both the left and right ventricle. These kids have cyanosis. Congestive heart failure typically occurs in the first one to two weeks of life. Source
In writing this, I am thankful that Ellen’s condition is not worse, and is so far seeming to be manageable with little to no long term effect on her quality of life overall. Lets get back to why the heck a paramedic blog is talking about feeding difficulties. Remember that babies are basic creatures. They want to be warm, they want to eat, and they want to poop. Sleep is optional, or so it seems at times. They don’t have a lot of cardiovascular endurance, and not much functional or metabolic reserve. Feeding is the most physically taxing thing they do. Now, not every baby that has feeding difficulties has heart disease, however most of the conditions above have sweating during feeding or difficulty feeding on the symptom list. We chased GERD as a reason Ellen was having feeding trouble. We had good prenatal care, and we have a great pediatrician that we trust, and it still took a minute to figure it out. Now think about the people in our communities that do not have the same resources, or had no prenatal care. Keep congenital heart defects on the radar for babies who are a little listless, or fatigued, having trouble feeding, and are a little dyspneic. Ask if they get sweaty with exertion, especially feeding. Do they tire easily with a strong crying fit? Are they just not as active as their brothers, sisters, or cousins when they were the same age? The next person might not think to ask, and you could catch something life limiting for that baby. Again, don’t read this and go out panicking parents, just something to keep in mind. Next time, we talk about BRUEs, formally known as ALTEs. Until then, be safe, make good life choices, and reach out if you need anything.
