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Congenital Heart Defects: A Lifelong Challenge for Our Tiniest Patients

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  • October 31, 2019
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Congenital Heart Defects: A Lifelong Challenge for Our Tiniest Patients

From the womb to adulthood, know the statistics, treatment, and medical coding associated with these birth defects.

Cardiac patients are generally thought to be older adults being seen for cardiovascular disease, angina, heart attack, or congestive heart failure; however, cardiac patients can also be newborns, who require completely different care than that used in adult cardiology. Let’s explore the types of congenital heart defects (CHDs) in detail to better understand the obstacles these young patients face.

Early Detection Can Help with Better Outcomes

CHDs are the No. 1 birth defect in the United States, with one out of every 100 to 115 babies born with a CHD every year.
CHDs are usually detected at the 20-week fetal ultrasound. When a CHD is detected, the parents are directed to a pediatric cardiology facility for a consultation and to prepare for their infant’s special needs upon birth. Usually, parents are encouraged to deliver their infant in the same facility where their pediatric cardiologist works so their infant can get immediate care.
If the CHD is not detected during prenatal ultrasonography, the symptoms to be aware of are cyanosis (bluish discoloration of the skin and mucous membranes), failure to thrive, lethargy, sweating while feeding, edema (swelling), and fussiness. These same symptoms can be seen in older children in addition to an inability to keep up with their peers, chest pain, and heart palpitations.
Infants with a complex critical defect usually need surgical intervention within the first few weeks of life; whereas, those with mild defects are generally watched closely with periodic visits to their pediatric cardiologist. Besides surgical procedures, these infants and children will undergo electrocardiograms, echocardiograms, cardiac catheterization, cardiac magnetic resonance imaging, stress tests, Holter monitoring, and 30-day monitoring.

Developmental and Neurological Effects are Ongoing

CHDs also cause neurological problems in these patients. Many struggle with developmental delays, depression, anxiety, increased susceptibility to infections, difficulty gaining weight, and issues with cold or hot temperatures. These patients may also require physical and occupational therapy, a feeding tube, and antibiotic care. As patients age, these issues may continue to plague them.
Developmental delays can continue into the school years, so the patient’s physician needs to work with the parents and educators to develop programs that will help the child thrive in the school system. A child with a CHD needs to stay up to date on all vaccines and antibiotic care. Certain illnesses that a heart-healthy child would normally recover from could be deadly to a child with a CHD.
Reaching adulthood brings its own set of needs to consider such as dating, college, working, stress management, healthy eating and exercise, drug and alcohol use, and the possibility of pregnancy. As such, more physicians than just pediatric cardiologists need to be knowledgeable about CHDs and how to provide proper care for these cardiac patients throughout their lifetime.

Mild Defects

CHD includes defects in the heart’s structure and the heart’s electrical conduction system. Out of the more than 36 known defects, examples of mild defects include atrial septal defect (ASD) and a ventricular septal defect (VSD). These are also known as holes in the heart.
The hole could be between the upper chambers, the atria (Q21.1 Atrial septal defect), or the lower chambers, the ventricles (Q21.0 Ventricular septal defect). Sometimes, these holes close without intervention. Some patients do not discover they have an ASD or a VSD until adulthood. If these defects require intervention, a surgical procedure is done via heart catheterization.

Critical Defects

Two commonly known critical defects are transposition of the great vessels (Q20.3 Discordant ventriculoarterial connection) and Tetralogy of Fallot (TOF) (Q21.3 Tetralogy of Fallot). Both require surgical intervention.
Transposition of the great vessels is basically the switching of the aorta and the pulmonary trunk. This causes the circulatory system to be two closed loops, instead of one large loop. The surgical intervention is to switch these arteries back to their appropriate locations. In TOF, the pulmonary artery is narrowed, the right ventricle is enlarged, and the aorta exits the heart just above the VSD present. This surgery patches the VSD and enlarges the pulmonary trunk leaving the right ventricle.

Complex Critical Defects

Two examples of complex critical heart defects are hypoplastic right heart syndrome (HRHS) (Q22.6 Hypoplastic right heart syndrome) and hypoplastic left heart syndrome (HLHS) (Q23.4 Hypoplastic left heart syndrome), with the left heart defect being more serious than the right.
In HRHS, particularly, the right ventricle is underdeveloped and unable to pump the blood to the lungs to pick up oxygen. This will cause the infant to be cyanotic (blue). The Blalock-Taussig shunt procedure is commonly used to correct this defect.
33750     Shunt; subclavian to pulmonary artery (Blalock-Taussig type operation)
This shunt connects the left subclavian artery to the left pulmonary artery, allowing the blood leaving the left side of the heart to return to the lungs for oxygen.

Complex Critical Defect Surgery Is a 3-step Process

In HLHS, the left side of the heart is underdeveloped, causing an inability to pump blood out to the body. The first surgery for these patients is the Norwood.
33619     Repair of single ventricle with aortic outflow obstruction and aortic arch hypoplasia (hypoplastic left heart syndrome) (eg, Norwood procedure)
The Norwood places a shunt between the aorta and the pulmonary trunk, making the right ventricle the main pumping chamber of the heart and allowing blood to flow to both the lungs and the body.
The second surgery is the Glenn, which is performed in both HRHS and HLHS. There are two types of Glenn procedures.
33766     Shunt; superior vena cava to pulmonary artery to flow to 1 lung (classical Glenn procedure)
33767     Shunt; superior vena cava to pulmonary artery for flow to both lungs (bidirectional Glenn procedure)
The Glenn takes the superior vena cava and attaches it to the right pulmonary artery, allowing blood from the head and the arms to return to the pulmonary circulation to pick up oxygen and bypass the heart.
The third surgery is the Fontan.
33615     Repair of complex cardiac anomalies (eg, tricuspid atresia) by closure of atrial septal defect and anastomosis of atria or vena cava to pulmonary artery (simple Fontan procedure)
33617     Repair of complex cardiac anomalies (eg, single ventricle) by modified Fontan procedure)
The Fontan has gone through many variations over the years. Each variation, however, involves taking the inferior vena cava and linking it to the right or left pulmonary artery to allow the blood from the lower body back to the lungs, bypassing the heart.
These surgeries are enabling more and more children to live beyond their first year of life and a greater number of individuals reaching adulthood. No matter how successful the surgery, however, these patients need lifetime follow-up visits with a cardiologist who understands CHD. The treatments in childhood might need to be redone or the patient may require a heart or heart and lung transplant. The Fontan, alone, is known to cause liver failure, heart rhythm issues requiring a pacemaker, plastic bronchus, and protein-losing enteropathy. All these surgeries, except for the mild forms of CHD, are palliative treatments and not cures.
Considering CHD is not a well-known birth defect and has very limited research, it’s encouraging to know these surgical interventions have probably led to 2 to 3 million survivors in the United States. Until recently, however, there have been no long-term studies about the longevity of CHD patients. The survivors will always have special healthcare needs requiring lifelong management.

The Children’s Heart Foundation., It’s My Heart. Jan. 1, 2004.
Pediatric Congenital Heart Association, New to CHD – Start Here.
The Children’s Heart Foundation, CHD Facts.

About Has 2 Posts

Kathy J. Ware, MCLS, RHIT, CPC, CPB, AAPC Instructor, is a professor of health information management at Lord Fairfax Community College. Her local chapters are both Winchester and Harrisonburg, Va. Wares has had both types of migraines since childhood.

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