Heart Defects

Heart Defects

Congenital heart disease (CHD) is a vast topic and is listed frequently in preadoption referrals. We have outlined the most common defects encountered in children, below. It is important to understand that all cardiac defects occur on a spectrum of disease so that two patients with the same diagnosis may have very different outcomes depending on which end of the spectrum their heart disease lies. It is important to discuss any referral and information with a medical professional that can interpret the heart information provided as well as look at the whole child for signs of other related issues.

Below we have listed the defects in “minor” and “major” categories. Minor heart defects, for the purpose for this paper, are categorized as lesions that will either not require intervention or when they do require intervention, the surgery or intervention will typically result in an anatomically normal heart with very little impact on long term morbidity and mortality. Major heart defects refer to lesions that almost always require surgical intervention, often in the neonatal period or immediately when they come to the USA; children with major heart defects often need repeat procedures, life-long follow up with cardiologists, and in some cases, they may have shortened life spans.

The majority of the time we receive partial reports of echocardiograms (“echo” or ultrasounds of the heart) for children with CHD. These reports are often limited compared to reports obtained from US medical centers. However, we are usually able to conclude a fair assessment of the long and short term impacts these lesions will have on the children. Much depends on the size of the defect, the age at the time of repair, and any resulting effects on the lungs and brain.

I. Murmurs: 

A heart murmur simply refers to an extra sound in the heart. Over 50% of children have an innocent heart murmur at some point in their childhood. An innocent heart murmur refers to a normal extra heart sound and has no impact on a child’s heart, health, or lifespan. There are also pathological murmurs, and these refer to extra sounds in the heart either from holes in the heart or valve abnormalities. Echocardiograms are often required to delineate the type and extent of heart disease in the presence of pathological murmurs and if the referral notes a murmur, then we almost always have further information with an echo.

II. Minor heart defects:

The most common types of CHD are ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), and patent formale ovale (PFO). Not all of these defects will require surgical correction, and the ones that do should result in an anatomically normal heart with little to no long-term consequences to the child.

An ASD is a hole between the top two chambers of the heart. Small defects often close by themselves over time and require no intervention. Moderate sized defects may get smaller over time and even close, but require closer monitoring. Large defects often require closure, either surgical or with a catheterization.1 Optimal time for closure is around 4 years of age, but closure later in childhood is still possible and yields excellent short and long-term results.2

A PFO is similar to an ASD as it is a hole between the top two chambers of the heart. However, a PFO is a normal hole that all babies are born with and it often closes within the first year of life. With that said, approximately 25% of adults still have a PFO3 and are unaware as it very rarely causes any symptoms. It is exceedingly uncommon for children to require PFO closure. Very few adults require PFO closure, but when needed, complications are rare and closure can be performed without surgery.

A VSD is a hole between the bottom two pumping chambers of the heart. There are many different types of VSD. The largest factor that impacts on the severity of a VSD is its size and if there are any associated defects. Small VSD’s are of very little clinical consequence and approximately 80% will close by themselves in the first year of life.4 Larger defects can lead to heart failure and need surgical closure within the first year or two of life. After a VSD is closed (either spontaneously or surgically), if there are no complications with surgery, the child should have a normal life span with no long term consequences.

A PDA is an artery that connects the two major arteries that leave the heart, the aorta and the pulmonary artery. Like the PFO, it is a normal structure that all babies are born with and it normally closes within the first few weeks of life. Some PDA’s do not close in infancy, small PDA’s cause very little problems but large PDA’s can cause heart failure symptoms like large VSD’s do. If a PDA does not close spontaneously, it will often need to be closed surgically (very small babies) or with a catheterization (larger children).5 In either case, long term outcomes are excellent and children should have a normal life span with no long term consequences.

ASD PFO VSD PDA

III. Major heart defects:

There are a wide variety of major heart defects, often divided into two groups: cyanotic (those that cause the baby to be “blue” due to a lack of oxygen in the blood) and acyanotic heart disease (those that do not cause the baby to be “blue”). These types of CHD can be very complicated. The most common types we come across are Tetralogy of Fallot (TOF), transposition of the great arteries (TGA), hypoplastic left heart syndrome (HLHS), endocardial cushion defect or atrioventricular canal defects (AVC), and valve stenosis or atresia (failure for the valve to form properly). The details behind these lesions are very complex and beyond the scope of this paper. It is very important to understand that all of these lesions require open heart surgery and often multiple surgeries throughout the child’s life. It is also important to understand that there is a huge variation of disease even within a single diagnosis, meaning that if you have two children with the same diagnosis one may do very well after surgery whereas the other may not and may need multiple more surgeries and may have limitations to their physical abilities and even lifespan. Major heart disease can also be linked to syndromes or problems with chromosomes such as Down syndrome, Velocardiofacial syndrome and should also be assessed by a medical provider who is screening the preadoption information.

Other than being prepared for the medical and surgical interventions needed for these children with serious heart conditions, another consideration is long term educational and developmental outcomes. The good thing is that generally, despite long term low oxygen levels in children with cyanotic (blue) CHD, these children have normal IQ’s as a group. However, they often demonstrate lower scores in certain academic areas such as verbal IQ and comprehension as well as minor behavioral challenges like attention deficit hyperactivity disorder.6,7 They may require extra services both inside and outside of school, but with the proper ongoing care they can have long, happy, and productive lives.

References

1. Helgason H, Jonsdottir G. Spontaneous closure of atrial septal defects. Pediatr Cardiol. 1999;20(3):195-199.

2. Roos-Hesselink J, Meijboom F, Spitaels S, et al. Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. Eur Heart J. 2003;24(2):190.

3. Hagen PT, Scholz DG, Edwards WD. Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. . 1984;59(1):17-20.

4. Miyake T, Shinohara T, Nakamura Y, et al. Spontaneous closure of ventricular septal defects followed up from< 3 months of age. Pediatrics International. 2004;46(2):135-140.

5. Thanopoulos B, Eleftherakis N, Tzannos K, Stefanadis C. Transcatheter closure of the patent ductus arteriosus using the new Amplatzer duct occluder:: Initial clinical applications in children. Am Heart J. 2008;156(5):917. e1-917. e6.

6. Spijkerboer A, Utens E, Bogers A, Verhulst F, Helbing W. Long?term intellectual functioning and school?related behavioural outcomes in children and adolescents after invasive treatment for congenital heart disease. British Journal of Developmental Psychology. 2008;26(4):457-470.

7. Majnemer A, Limperopoulos C, Shevell M, Rohlicek C, Rosenblatt B, Tchervenkov C. Developmental and functional outcomes at school entry in children with congenital heart defects. J Pediatr. 2008;153(1):55-60. e1.

This article was generously shared by Dr. Judith Eckerle of the University of Minnesota,  Adoption Medicine Clinic. Child referrals are becoming increasingly complex, and adoption medicine professionals are identifying many more children with special needs from all countries that participate in intercountry adoption, as well as from the United States. To help families prepare for these changes, Dr. Eckerle and other medical specialists are writing a book: Health Topics For Preadoptive Families.

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