of the heart and associated great blood vessels that are present at birth. Congenital heart diseases affect almost 1%, Etiology and types of congenital heart diseases Although the etiology of CHD is not completely understood, data suggest that a combination of genetic and environmental factors is responsible for the development of cardiovascular malformations in the neonate. Congenital heart diseases are associated with other syndromes in about 28% of cases, including trisomy 21 (Down syndrome), Treacher Collins syndrome, DiGeorge syndrome, Turner syndrome, Noonan syndrome, and Klinefelter syndrome (Kosiv et al., 2023). Environmental factors may include Cyanotic defects Cyanotic defects occur when poorly oxygenated blood is pumped from the left side of the heart, through the aortic valve, and out to the rest of the body (MedlinePlus, 2023). Of the several types of cyanotic defects possible, tetralogy of Fallot and transposition of the great vessels are the most likely to occur in this category. As the name suggests, tetralogy of Fallot has four components (CDC, 2023b): 1. Pulmonary valve stenosis, leading to decreased blood flow to the lungs; 2. A large ventricular septal defect, with a right to left shunting of unoxygenated blood; Septal defects Septal defects occur in the wall separating the right and left sides of the heart. These defects can occur between either the left and right atria or left and right ventricles. The atrial septal defect has a prevalence of 2.5 out of every 1,000 live births and is the result of communication between the left and right atria (Muroke et al., 2023). In this situation, oxygenated blood from the left side of the heart can mix with unoxygenated blood on the right side of the heart before flowing to the lungs. This inefficient flow of blood can lead to an enlargement of the right atrium and ventricle, impeding heart function. Arrhythmias can develop as a result of this impediment. The increase of blood flow through the pulmonary artery can also lead to pulmonary hypertension, further decreasing the lungs’ ability to properly oxygenate blood. Ventricular septal defect (VSD) is the most commonly occurring type of CHD, accounting for 80% of all CHD Obstructive defects In obstructive defects, a blockage (atresia) or narrowing (stenosis) prevents normal blood flow through the four heart valves. In aortic stenosis, which accounts for 5% of CHD in pediatric patients, the narrowed aortic valve restricts the flow of oxygenated blood through the aorta and out to the rest of the body (Ross et al., 2023). This inhibition of blood flow can lead to an enlarged heart and its associated symptoms of fatigue, abnormal clotting, and diminished wound healing. Pulmonary stenosis, a narrowing of the pulmonary valve leading to the pulmonary artery, has a prevalence of about 1.7 cases per 100,000 children under the age of 2 (McLennan et al., 2021). This condition restricts blood flow to the lungs from the right ventricle, leading to increased Medical/surgical treatment For patients presenting with a lone ASD or VSD, nonsurgical treatment may include medication to increase cardiac contractility or to decrease the load on the cardiovascular system. Many cases of ASD and VSD spontaneously resolve, requiring no further treatment. For the obstructive and cyanotic defects, surgical repair may include valve
or about 40,000, births in the population each year (Centers for Disease Control and Prevention [CDC], 2023a).
maternal diabetes, rubella, and medications or alcohol consumed by the mother during the prenatal period. The American Heart Association (AHA) reports the existence of at least 14 distinct types of CHD. In general, CHD fall into one of two classifications: Cyanotic or acyanotic defects. Acyanotic defects can be further classified as either left-to- right hunts or outflow obstructions (AHA, n.d.). 3. Right ventricular hypertrophy secondary to pulmonary valve stenosis; and 4. Overriding aorta, which allows unoxygenated blood to flow from the right ventricle through the aorta. Both tetralogy of Fallot and transposition of the great vessels lead to unoxygenated blood flowing through the body. The resultant inadequate oxygen supply leads to a bluish hue to the skin (cyanosis) and the child’s inability to thrive. anomalies (Dakkak & Oliver, 2023). In VSD, there is communication between the right and left ventricles of the heart, allowing oxygenated blood from the left side of the heart to mix with unoxygenated blood on the right side of the heart. As in ASD, the right side of the heart can enlarge and therefore decrease its capacity to pump blood through the pulmonary artery and into the lungs. For both types of septal defects, depending on the severity and location, children will exhibit fatigue, shortness of breath, rapid breathing, and slow growth and maturation. Although no qualitative difference exists between the two types of septal defects, surgical repair of either is dictated by the size and location of each. For the unrepaired defect, medications such as digoxin (Digitek, Lanoxin) or furosemide (Lasix, Delone, Furocot) can be used to minimize symptoms and improve overall cardiovascular function if necessary. workload for the right ventricle, with sequelae similar to those found in ASD. The third obstructive cardiac abnormality is called coarctation of the aorta and is defined as a narrowing of the blood vessel downstream of the aortic valve. Postnatal symptoms in the infant include pale skin, irritability, heavy sweating, and difficulty with breathing. As the infant develops through the first several weeks of life, the lack of blood flow to the body and increased workload for the heart will necessitate a procedure to increase the lumen size of the aorta. Symptoms in the older child include hypertension, shortness of breath, headache, leg cramps, and nosebleeds (Mayo Clinic, 2018).
replacement, palliative shunts, or use of prosthetic patches or devices. Postoperative medical care may include the use of anticoagulants such as warfarin (Coumadin, Jantoven) or antiplatelet medications such as aspirin or clopidogrel (Plavix).
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