Cogenital heart defects in children: some facts and mythologies

Introduction:
Last month, January 2017, was “Birth Defects Prevention Month” closely followed this month of February by the “Congenital Heart Defect Awareness Week” (February 7-14) and “Heart Failure Awareness Week” (February 14-20), three related conditions in children occurring all in quick succession in a season that is remarkably linked with felicitations, the heart and love.
Every 2-4 minutes a baby is born with birth defect, and congenital heart defect (CHD) is the most frequent and leading cause of birth defect-related deaths worldwide across the lifespan, while heart failure is the most common complication of CHDs in children. This newspaper article is intended to contribute to efforts to create and raise awareness on congenital heart defects, and highlight some facts and myths linked with CHDs.

What is a Congenital Heart Defect (CHD)?
The word “congenital” means inborn or existing at birth. Congenital heart defects (CHDs) refer to abnormal development of heart and/or the major blood vessels that affect the structure and function of the heart.

What are the Facts about CHDs?
•    CHD is the most frequent birth malformation and the leading cause of birth defect-related death across the life span and the life-cycle-fetus (unborn baby), newborn, infants, pre-school and school age children, adolescents, adults, pregnant women and the elderly.
•    Birth defects can occur in any family regardless of race, ethnicity, health history, economic status, or level of education;
•    About 8-12 of every 1000 live born babies are born with CHD every year, which implies one (1) case of CHD is born every 15 minutes! and if we have 1,000,000 live births in a year, then we expect about 8,000-12,000 babies with CHD born every year.
•    Many cases of sudden cardiac death in young athletes, some strokes and pregnancy-related heart failure and death are caused by undiagnosed CHDs and childhood-onset heart disease.
•    CHDs are 60 times more prevalent than childhood cancer and more children die from CHD than from all forms of childhood cancer combined and from HIV/AIDS, yet research funding for paediatric cancer 5-10 times higher than for CHD.
•    A baby’s risk of being born with a CHD is increased by 3 times if the mother, father, or sibling has a CHD.
•    Although most causes of CHDs are unknown, there are established and emerging high risk factors that include genetic, environmental and a combination of both. The diagrams above show the proportionate distribution of causes of death in newborns and children.

What are the Risk Factors of CHDs?
CHD may be inherited when a first degree relative (parent, sibling) has CHD. Environmental risk factors include exposure to organic solvents, pesticides, carbon monoxide, nitrous oxide (laughing gas), ozone (ground) and sulfur dioxide. Also, consumption of alcohol including ethanol-containing local concoctions, cigarette smoking, certain antibiotics, painkillers, anticonvulsants, antimalarials, antihypertensives, antidepressants, contraceptives, caffeine and illicit drugs (cocaine, heroin, amphetamine); and maternal diabetes mellitus, rubella infection and obesity have been associated with CHDs. Presence of other birth defects increases the risk of CHD.

What are the Common Health Problems Associated with CHD?
At birth, signs of serious CHD include blueness, jitteriness, sweatiness, lethargy (reduced physical activity/weakness), fast and difficult breathing within 24 to 72 hours in a newborn baby who was previously well.

Infants with CHD may have feeding difficulties such as inability to suck long enough on the breast leading to intermittent rests at short intervals of sucking. Other signs of CHD in infants include sweatiness during feeds, poor growth, repeated coughs, or chest infections (pneumonias), delayed development

Toddlers and children below five years of age have poor growth and development, repeated chest infection, and get easily tired following normal physical activity for their age. Bluish discoloration of the finger tips and lips or episodic blueness, squatting, episodic restless/weakness, convulsions, stroke, bleeding tendencies, swelling of the finger and toe nail areas, which may appear as drumsticks occur in “blue” heart defects. 

Older children, adolescents, and adults with CHD experience irregular heartbeats, palpitations (awareness of one’s heart beat), fainting spells, and chest pain, poor growth and development. Other signs include effort/exercise intolerance (easily tired), difficult breathing on exertion such as climbing stairs, walking to school, and participation in non-competitive sports

Myth #1: Children are Innocent and Do Not Suffer Heart Diseases!
Truth: Heart diseases are more frequent in children and the causes are more diverse than in adults. Also, some heart diseases (acquired and congenital) in adults originate during childhood
Myth #2: Congenital heart defects (CHDs) in children are mainly “holes in the heart, which do not pose any danger because they close naturally.

Truth: There are over 40 different types of heart defects of varying seriousness and complexity ranging from simple types to immediately life-threatening or fatal complex types.

Common examples include holes in different areas of the heart, obstructed major blood vessels, narrow or leaky heart valves. Every suspected heart defect including hole in the heart should be referred promptly to a Paediatric Cardiologist.
Myth #3: Many children who are born with a CHDs do not survive to adulthood.
Truth:  Today, in developed countries, most children who are born with complex heart defects survive, and survive well, into adulthood because they are identified, treated early and followed up after treatment.

CHD can be Prevented and Controlled by
•    ensuring adolescent girls, women of child bearing age and mothers have a healthy, balanced, nutritious diet including a wide variety of vegetables and fruit; and maintain a healthy weight;
•    preparing for pregnancy and maintaining preconception physical, emotional and nutrition health; undertaking gynaecological and wellness examination, and taking a multivitamin daily that includes the recommended 400mcg of folic acid and other essential B vitamins;
•    ensuring mothers avoid harmful medications and substances including alcohol and tobacco;
•    avoidance of travel by pregnant women (and sometimes women of child-bearing age) to regions experiencing outbreaks of infections known to be associated with congenital anomalies;
•    reducing or eliminating environmental exposure to hazardous substances (such as pesticides) during pregnancy;
•    controlling diabetes prior to and during pregnancy through counselling, weight management, diet and administration of insulin when required;
•    ensuring that any exposure of pregnant women to medications or medical radiation (such as X-  rays) is justified and based on careful health risk–benefit analysis;
•    vaccination, especially against the rubella virus, for children and women;
•    obtaining genetic counselling and birth defect screening especially if there is a positive family history of CHD or other birth defects and if you are 35 years of age and over;
•    screening unborn babies during pregnancy (prenatal screening) and immediately after birth (postnatal screening);
•    screening children in rural and urban communities to detect those with CHD and referring them for appropriate care;
•    conducting population-based surveillance and research on CHDs and other birth defects.

Conclusion
Congenital heart defects (CHDs) are common, costly, and critical but have received little attention from stakeholders in the public and private sectors. Early identification and treatment of CHDs will save lives and improve the quality of life of affected persons. There is need to support screening and educational programs in communities including schools. Partnership and collaboration with stakeholders in the private and public sectors are required to support and facilitate screening and surveillance programs, educational programs on case identification, documentation and referral pathways, appropriate management of children with CHD, and epidemiological life course research, if we must reverse the current narrative of CHD and other birth defects in Nigeria and other developing countries.

Professor Okoromah is of the College of Medicine University of Lagos and Consultant Paediatrician/Paediatric Cardiologist
Lagos University Teaching Hospital, Idi-Araba
Email: partnership4chd@gmail.com; advocacy4birthdefects@gmail.com