Skip to main content

Critical congenital heart disease (CCHD), defined as heart disease requiring either surgical or catheter-based intervention in the first year of life, affects 2-3 out of every 1,000 live births. Specific defects include hypoplastic left heart syndrome, pulmonary atresia, tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia, and truncus arteriosus. The majority of CCHD is identified prior to discharge from the newborn nursery. Many patients have already been detected prenatally. A good portion of those not identified prenatally will present with signs or symptoms in the first 24 hours of life. These may include an audible murmur, abnormal heart sounds, the absence of palpable pulses, cyanosis, pallor, or tachypnea. Nevertheless, a small percentage of patients with CCHD may inevitably "slip through the cracks." Usually these are infants with ductal-dependent heart lesions whose ductus arteriosus has remained open and therefore either have sufficient pulmonary blood flow to prevent the noticeable detection of cyanosis, or have sufficient cardiac output to prevent signs of shock. It almost all cases, however, these patients will have a level of oxygen desaturation that may identify them as potential "risks" despite appearing normal to an examiner's eye.

The technical term for a specialized sonogram of a baby’s heart before they are born is a fetal echocardiogram (fetal echo). This type of examination is usually performed for the first time at 20-24 weeks gestation. As is true with any test, before you have a fetal echo it is important to understand what it can and cannot diagnose.

Your child has recently had an episode of fainting, and your pediatrician has decided that they should be evaluated by a cardiologist. While there are many causes for fainting, or syncope, one of the most important to rule out is a malfunction of the heart. Luckily, in an otherwise healthy child, heart related causes of fainting are extremely uncommon. In fact, in most cases, after a visit to the cardiologist, parents and children are told that the reason for the dizziness and fainting is vasovagal syncope. But what exactly is vasovagal syncope?

Most children who visit the cardiologist’s office will have several tests done to evaluate the heart. One test typically performed is called an EKG or an ECG, both of which stand for electrocardiogram. While most parents understand that this is a quick test that evaluates the heart, many people do not actually understand what the doctors are actually testing. What IS an EKG?

An EKG, ECG or electrocardiogram is a recording of the electrical activity in the heart. Our heartbeats are created by an electrical signal that begins in one part of our heart and then moves through special conducting cells which carry the electrical signal to the heart muscle and cause it to contract. This contraction is a heartbeat and the electrical signal is creating the heart rhythm. In a normal heart rhythm, each heartbeat, or contraction, is generated by a new electrical signal.

Many families who deal with congenital heart disease in their children often feel quite isolated. After all, with only 1.2 out of every 100 babies afflicted with some form of CHD, it is easy to think that you are alone in your battle. Fortunately, incredible support groups such as Amazing Little Hearts bring families together, and introduce them to the fact that they are not alone!

It is also fun (and surprising) to reveal how many celebrities and public figures also deal with congenital heart disease. One of the most famous athletes for Team USA, Shaun White, has won many gold medals in Olympic snowboarding and skateboarding, all while being born with Tetralogy of Fallot!