Children with Congenital Heart Diseases

INFORMATION 8 % of term infants born with CHD Rate is higher in preterms Male = Female Specific defects show sex differences ASD & PDA - girls Cyanotic heart diseases boys

CAUSES failure of heart structure to progress beyond an early stage of embryonic development Maternal rubella Familial

CLASSIFICATION Classified based on physical signs of cyanosis 1. Acyanotic Heart Disease Stricture or shunt Left to right shunt Increase in pulmonary pressure Right ventricular hypertrophy Right sided heart failure Except Aortic stenosis and coarction of the aorta

2. Cyanotic Heart Disease Right to left shunt Unoxygenated to oxygenated

Left ventricular hypertrophy Left sided heart failure

** Children with Acyanotic can develop cyanotic heart disease ** Some children with cyanotic disease may not exhibit cyanosis until they are seriously ill SECOND CLASSIFICATION 1. Acyanotic Heart Disease o Disorders with Increased Pulmonary Blood Flow a. Ventricular Septal Defect b. Arterial Septal Defect c. Patent Ductus Arteriosus d. Atrioventricular Canal Defect

Disorders with Obstructive Lesion a. Pulmonary Stenosis b. Aortic Stenosis c. Coarctation of the Aorta

Disorder with Mixed Blood Flow a. Transposition of the Great Arteries b. Total Anomalous Pulmonary Venous Return c. Truncus Arteriosus d. Hypoplastic Left Heart Syndrome

2. Cyanotic Heart Disease Disorders with the Decreases Pulmonary Blood Flow a. Pulmonary Atresia b. Tetralogy of Fallot c. Tricuspid Atresia

GENERAL INFORMATION Congestive heart failure is the complication of all congenital heart disease if left untreated Echocardiogram diagnostic tool used in diagnosing all congenital heart diseases All congenital heart disease have heart murmurs but they differ in the type of murmur Grade 1 Grade 2 Grade 3 Grade 4 Grade 5 Grade 6

VENTRICULAR SEPTAL DEFECT most common type of congenital cardiac disorder an opening is present in the septum between the 2 ventricles may not be evident at birth left and right shunting of blood SIGNS AND SYMPTOMS Small holes in the ventricular septum usually produce no symptoms but are often recognized by the child's health care provider when a loud heart murmur along the left side of the lower breast bone or sternum is heard. Large holes typically produce symptoms 1-6 months after an infants birth. The left ventricle begins to fail, producing the following symptoms:

due to higher pressure in left side of heart

Fast breathing Sweating Pallor Very fast heartbeats Decreased feeding Poor weight gain

When a ventricular septal defect is not detected early in life, it can cause more severe problems and more severe symptoms as time goes on. The biggest concern is development of high pressure in the lungs (pulmonary hypertension). If the

ventricular septal defect is not surgically closed, irreversible pulmonary hypertension can develop, and the child is no longer operable and has a poor prognosis. The following are typical symptoms of pulmonary hypertension:

Fainting Shortness of breath Chest pain Bluish discoloration of the skin (cyanosis)

The skin turns faintly bluish when the tissues are not receiving quite enough oxygen. This condition is often termed "hypoxemia" or "hypoxia." MANAGEMENT over 3 mm require open heart surgery - scheduled before the child is 2 years old - if left open heart failure

ARTERIAL SEPTAL DEFECT abnormal communication with the two atria

SIGNS AND SYMPTOMS Signs and symptoms of large or long-standing atrial septal defects may include:

Shortness of breath Fatigue Swelling of legs, feet or abdomen Heart palpitations or skipped beats

MANAGEMENT Surgery done between 1 and 3 years of age (open heart surgery) Closure is important

PATENT DUCTUS ARTERIOSUS failure to close when the baby reaches 3 months of age blood shunts from the aorta(oxygenated) to the pulmonary artery (unoxygenated)

GIRLS >> BOYS

SIGNS AND SYMPTOMS

Bounding pulse Fast breathing Poor feeding habits Shortness of breath Sweating while feeding Tiring very easily Poor growth

MANAGEMENT Give Indomethacin side effect : nephrotoxic Surgery (done at 6 months to 1 year) not open heart surgery

ARTRIOCENTRICULAR CANAL DEFECT Atrioventricular canal is a congenital (present at birth) heart defect. Other terms used to describe this defect are endocardial cushion defect and atrioventricular septal defect (AVSD). As the fetus is growing, something occurs to affect heart development during the first 8 weeks of pregnancy, and certain areas of the heart do not form properly. Normally, oxygen-poor blood returns to the right atrium from the body, travels to the right ventricle, then is pumped into the lungs where it receives oxygen. Oxygenrich blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta. An atrial septal defect allows oxygen-rich blood to pass from the left atrium, through the opening in the septum, and then mix with oxygen-poor blood in the right atrium. A ventricular septal defect allows oxygen-rich blood to pass from the left ventricle, through the opening in the septum, and then mix with oxygen-poor blood in the right ventricle. Abnormalities of the mitral or tricuspid valves allow blood that should be moving forward from the ventricle into either the pulmonary artery or the aorta to instead flow backward into the atria. Atrioventricular canal defects occur in about 5 percent of all congenital heart disease cases and are more common in infants with Down syndrome. SIGNS AND SYMPTOMS Symptoms occur in infancy. The following are the most common symptoms of AVC. However, each child may experience symptoms differently. Symptoms may include:

fatigue sweating pale skin cool skin rapid breathing heavy breathing rapid heart rate congested breathing disinterest in feeding, or tiring while feeding poor weight gain

MANAGEMENT

digoxin - helps strengthen the heart muscle, enabling it to pump more efficiently. diuretics - the body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body. ACE (angiotensin-converting enzyme) inhibitors - dilates the blood vessels, making it easier for the heart to pump blood forward into the body.

PULMONIC STENOSIS narrowing of the pulmonary valve or the pulmonary artery just distal to the valve inability of the right ventricle to evacuate blood by way of the pulmonary artery because of the obstruction leads to right ventricular hypertrophy

SIGNS AND SYMPTOMS

Turning blue, called cyanosis Rapid breathing Fainting Low energy

MANAGEMENT Balloon angioplasty -procedure of choice -a catheter with an uninflated balloon at its tip is inserted and passed through the heart into the stenosed valve - balloon is inflated breaking the adhesions relieve stenosis

AORTIC STENOSIS stricture of the aortic valve prevents passage of blood from the left ventricle into the aorta causes increase in the pressure in the left ventricle hypertrophy of the left ventricle

SIGNS AND SYMPTOMS

Chest pain (angina) or tightness Feeling faint or fainting with exertion Shortness of breath, especially with exertion Fatigue, especially during times of increased activity Heart palpitations sensations of a rapid, fluttering heartbeat Heart murmur

MANAGEMENT Beta blockers or calcium channel Balloon valvulopasty Artificial valve replacement

COARCTION OF THE AORTA narrowing of the lumen of aorta due to a constricting band occurs frequently in boys than in girls leading cause of congestive heart failure in the first few months

PATHOPHYSIOLOGY Difficult for blood to pass through the lumen of the aorta BP increases proximal to the coarctation and decreases distal to it Increase BP in the upper portion of body vertigo, eistaxis Decrease BP in the lower extremities

SIGNS AND SYMPTOMS Babies with severe coarctation of the aorta usually begin having signs and symptoms shortly after birth. These include:

Pale skin Irritability Heavy sweating Difficulty breathing

Left untreated, aortic coarctation in babies may lead to heart failure and death. Older children and adults with the condition often don't have symptoms, because they tend to have less severe narrowing of the aorta. If signs or symptoms appear, the most common sign is high blood pressure (hypertension) measured in the arm. Signs and symptoms may include:

High blood pressure (hypertension) Shortness of breath, especially during exercise Headache Muscle weakness Leg cramps or cold feet Nosebleeds

MANAGEMENT Give digoxin and diuretics Surgery

TRANSPOSITION OF THE GREAT ARTERIES Transposition of the great arteries is a heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition changes the way blood circulates through the body, leaving a shortage of oxygen in blood flowing from the heart to the rest of the body. Without an adequate supply of oxygen-rich blood, the body can't function properly. SIGNS AND SYMPTOMS

Blue color of the skin (cyanosis) Shortness of breath Lack of appetite Poor weight gain

MANAGEMENT Before surgery Your baby's doctor may recommend several options to help manage the condition before corrective surgery. They include:

Medication. The medication prostaglandin E-1 helps keep open the ductus arteriosus a connection between the aorta and pulmonary artery to increase blood flow and improve mixing of oxygen-poor and oxygen-rich blood.

Atrial septostomy. This procedure usually done using cardiac catheterization rather than surgery enlarges a natural connection between the heart's upper chambers (atria). It allows for the oxygen-rich and oxygenpoor blood to mix and results in improved oxygen delivery to your baby's body.

Surgery Surgical options include:

Arterial switch operation. This is the surgery that doctors most often use to correct transposition of the great arteries. Doctors usually perform this surgery within the first month of life. During an arterial switch operation, the pulmonary artery and the aorta are moved to their normal positions: The pulmonary artery is connected to the right ventricle, and the aorta is connected to the left ventricle. The coronary arteries also are reattached to the aorta. If your baby has a ventricular septal defect or an atrial septal defect, those holes usually are closed during surgery. In some cases, however, the doctor may leave small ventricular septal defects to close on their own.

Atrial switch operation. In this surgery, the surgeon makes a tunnel (baffle) between the heart's two upper chambers (atria). This diverts the oxygen-poor blood to the left ventricle and the pulmonary artery and the oxygen-rich blood to the right ventricle and the aorta. With this procedure, the right ventricle must pump blood to the entire body, instead of just to the lungs as it would do in a normal heart. The atrial switch was used frequently beginning in the 1960s. Since the 1980s, however, it has been largely replaced by the arterial switch operation because the latter is not associated with many of the long-term complications of the atrial switch, such as arrhythmias, baffle obstructions or leaks, and problems with right ventricle function.

After surgery After corrective surgery, your baby will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease to monitor his or her heart health. The cardiologist may recommend that your child limit physical activity. Your child may need to take antibiotics before dental procedures and other surgical procedures to prevent infections. Many people who undergo the arterial switch operation don't need additional surgery. However, some complications, such as arrhythmias, heart valve leaks or problems with the heart's pumping, may require treatment.

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

Total anomalous pulmonary venous return is a congenital heart disease (present at birth) in which none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart). SIGNS AND SYMPTOMS

Lethargy Poor feeding Rapid breathing Poor growth Frequent respiratory infections Cyanosis (blue discoloration of the skin)

MANAGEMENT - Early complete surgical repair is needed. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed. - If left untreated, death may occur by age 1 in babies with more severe defects. With surgery, early repair provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. - There is no known way to prevent TAPVR.

TRUNCUS ARTERIOSUS
- Truncus arteriosus is a rare type of congenital heart disease characterized by a single blood vessel arising from the right and left ventricles, instead of the normal two (pulmonary artery and aorta).

There are four subtypes of truncus arteriosus, depending on the specific anatomy of the single vessel.
SIGNS AND SYMPTOMS

Heart failure Lethargy Poor feeding Shortness of breath (dyspnea) Rapid breathing (tachypnea) Fatigue Cyanosis (blue discoloration of skin) Delayed growth or growth failure Broadening of the finger tips (clubbing)

MANAGEMENT

Surgery is needed to treat this condition. Two procedures are available. One is banding of the pulmonary arteries coming off the truncus, but it is rarely used anymore. The other procedure is called complete repair. Complete repair appears to be the preferred option but as the child grows, repeat surgical procedures may be necessary. Complete repair usually provides good results. Re-operation may be necessary as the patient grows. Untreated cases have a poor outcome, usually leading to death between during the first year of life. Rarely, the diagnosis is missed until early adulthood; these patients generally need a heart and a lung transplant.

HYPOPLASTICLEFT HEART SYNDROME

Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle aortic valve, and aorta) do not develop completely. The condition is congenital (present at birth).

SIGNS AND SYMPTOMS Lethargy Poor suckling and feeding Shortness of breath Rapid breathing Cold extremities Enlarged liver Poor pulse Pounding heart Bluish (cyanosis) or poor skin color Sudden death

MANAGEMENT Once the diagnosis of hypoplastic left heart is made, the baby will be admitted to the neonatal intensive care unit. A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to maintain circulation of blood to the body. These measures do not solve the problem. The condition always requires surgery. The first surgery, called the Norwood operation, occurs within the first few days of life. Stage I of the Norwood procedure consists of building a new aorta, and an artificial shunt is inserted to maintain blood flow to the lungs. Afterwards, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done.

Stage II of the operation is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the body (the superior vena cava) directly to blood vessels to the lungs to get oxygen. The surgery is usually done when the child is 4 - 6 months of age. During stage I and II, the child may still appear somewhat blue (cyanotic). Stage III, the final step, is called the Fontan procedure. The remainder of the veins carrying blue blood from the body (the inferior vena cava) is connected directly to the blood vessels to the lungs, and the right ventricle now serves as the only pumping chamber for the heart. This surgery is usually performed between the ages of 18 months and 3 years of age. After this final step, the baby is no longer blue (cyanotic). Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure.

In some hospitals, heart transplantation is considered a better choice than the 3-step surgery process. However, there are few donated hearts available for small infants.
TETRALOGY OF FALLOT four anomalies present 1. pulmonary stenosis 2. large Ventricular septal defect 3. overriding of the aorta 4. hypertrophy of the right ventricle PATHOPHYSIOLOGY pulmonary stenosis pressure build up in the right side of the heart blood shunts from this area of increased pressure via the VSD into the left ventricle and the overriding of the aorta extra effort involved to pump hypertrophy of the right ventricle blood eventually causes

SIGNS AND SYMPTOMS

A bluish coloration of the skin caused by blood low in oxygen (cyanosis) Shortness of breath and rapid breathing Loss of consciousness (fainting) Clubbing of fingers and toes (an abnormal, rounded shape of the nail bed) Lack of appetite Poor weight gain

Tiring easily during play Irritability

MANAGEMENT Surgery ( Brock Procedure) - a surgical technique for correction of pulmonic stenosis in which there is excision of the fibromuscular obstruction in the right ventricle using a rongeur inserted through the wall of the right ventricle. Blalock Taussig procedure - surgical construction of a shunt between the right subclavian artery and the right pulmonary artery as a temporary measure to overcome congenital heart malformations, such as tetralogy of Fallot, in which there is insufficient pulmonary blood flow. Echocardiography is used to assess the malformation. General anesthesia and a cardiac bypass machine are used for the operation. The subclavian artery is joined end to side with the pulmonary artery, directing blood from the systemic circulation to the lungs. Thrombosis of the shunt is the major postoperative complication. Permanent surgical correction is performed in early childhood. Keep hypoxic episodes to a minimum administering oxygen assume knee-chest / squatting position

TRICUSPID ATRESIA Tricuspid atresia is a type of congenital heart disease in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle.

SIGNS AND SYMPTOMS

Cyanosis (bluish discoloration of the skin) Easily fatigued Shortness of breath (dyspnea) Clubbing of fingers or toes Heart failure Fast breathing Poor growth

MANAGEMENT Once the diagnosis is made, the baby will be admitted to the neonatal intensive care unit (NICU). A breathing machine (ventilator) may be needed to help the baby breathe. A medicine called prostaglandin E1 is used to maintain circulation of blood to the body. The condition always requires surgery. If the heart is unable to pump enough blood out to the lungs and rest of the body, the first surgery usually occurs within the first

few days of life. In this procedure, an artificial shunt is inserted to maintain blood flow to the lungs. Afterwards, the baby usually goes home. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be done. Stage II of the operation is called the Glenn shunt or Hemifontan procedure. This procedure connects half of the veins carrying blue blood from the body directly to blood vessels to the lungs that get oxygen. The surgery is usually done when the child is between 4 - 6 months of age. During stage I and II, the child may still appear somewhat blue (cyanotic). Stage III, the final step, is called the Fontan procedure. The remainder of the veins carrying blue blood from the body is connected directly to blood vessels leading to the lungs, and the right ventricle now serves as the only pumping chamber for the heart. This surgery is usually performed between the ages of 18 months and 3 years of age. After this final step, the baby is no longer blue (cyanotic). Some patients may need more surgeries in their 20s or 30s if they develop hard to control arrhythmias or other complications of the Fontan procedure. In some hospitals, heart transplantation is considered to be a better alternative to the 3-step surgery process, but there are few donated hearts available for small infants.

Sources http://www.mayoclinic.com http://www.emedicinehealth.com http://health.nytimes.com http://www.nlm.nih.gov Maternal and Child Health Nursing 5th edition by Adele Pilliteri