14. Congenital Heart Disease.pptx

Congenital Heart Diseases in children PRESENTED BY MRS CHULU  Definition Theses are Abnormalities of the heart/ great vessels present at birth  Incidence Incidence higher in premature infants Faulty embryogenesis during 3-8 weeks of Int...

Congenital Heart Diseases in children PRESENTED BY MRS CHULU  Definition Theses are Abnormalities of the heart/ great vessels present at birth  Incidence Incidence higher in premature infants Faulty embryogenesis during 3-8 weeks of Intrauterine life  Cause The actual cause is unknown but some predisposing factors include: genetic rubella infection, drugs, heavy drinking during pregnancy  Classification 1. Malpositions of Heart Dextrocardia (1 in 12,000 new borns) 2. Shunts (acyanotic and cyanotic CHD) 3. Obstructions (Obstructive CHD)  Shunts These are abnormal communications between 2 chambers or blood vessels; In these shunts blood flows according to pressure gradient  A. Left to Right shunts (Acyanotic or Late Cyanotic group) cyanosis presents several months or years after birth 1. Ventricular septal defect (VSD) -25-30% 2. Atrial septal defect (ASD)- 10-15% 3. Patent ductus arteriosus (PDA)- 10-20% Left to Right shunt: Increase in pulmonary blood flow leads to increase in pulmonary pressure which results in overwork of the Right Ventricle muscle potentiating a cardiac failure Increased pulmonary blood flow leads to medial hypertrophy and intimal proliferation to prevent pulmonary edema.  Prolonged increased pulmonary pressure results in reversing the flow of blood from Right to Left: unoxygenated blood in systemic circulation leads late cyanosis Once significant pulmonary Hypertension develops, surgical treatment of cardiac defects not possible  Ventricular Septal Defect (VSD) This is an opening between the two ventricles, incomplete closure of ventricular septum Left to right shunt – majority Dilated right heart too much blood to lungs increase in pulmonary pressure Morphology: 90% in membranous septum 10% lie below pulmonary valve or within muscular septum Mostly single. Multiple VSDs in muscular septum  Clinical features They depend on size of lesion Small lesions recognized later or may spontaneously close Large VSDs recognized early in life, cause Left to Right shunts leading to hypertrophy of Right Ventricle causing pulmonary Hypertension since birth.  Ultimately, shunt reversal, cyanosis and death If not corrected till 1 year to wait for spontaneous closure  Atrial Septal Defect This is an Abnormal fixed opening in atrial septum caused by incomplete tissue formation It usually goes unnoticed in infancy and childhood It usually presents late in life (30), late cyanotic heart disorder.  Left to Right shunt at atrial level (pulmonary vascular resistance is less than systemic and compliance of Right ventricle is greater than Lt) Pulmonary blood flow increased to 2-4 times, hypertrophy of RA and RV Pulmonary Hypertension, Right Heart Failure are unusual Morphology: 3 types according to location Secundum ASD (90%)- deficient or fenestrated oval fossa near centre of septum Primum ASD- occur adjacent to AV valves Sinus venosus- near entrance of Superior Vena Cava AVSD – Atrio ventricular septal defect  Patent Ductus Arteriosus Ductus arteriosus is normal connection between aorta and bifurcation of pulmonary Artery Normally closes at 1st or 2nd day of life Any period more than 3months persistence is abnormal Cause: possibly due to Increase in levels of PGE2 after birth - seen in children with respiratory distress syndrome - pharmacologic closure with indomethacin (PGE2 inhibitor) Most often does not produce functional difficulties at birth  A narrow ductus has no effect on growth and development during childhood  Right to Left shunts (Cyanotic CHD) B. Right to Left shunts (Cyanotic group) 1. Tetralogy of Fallot (TOF)- 6-15% 2. Transposition of great arteries -4-10% 3. Persistent truncus arteriosus – 2% 4. Tricuspid atresia and stenosis 1% Cyanosis in early postnatal life  There is decrease in pulmonary blood flow leads to poor oxygenation of blood which enters the Left side of the heart, this blood then flows to the systemic circulation resulting in dusky blueness of mucus membranes and skin (Cyanosis)  Functional anemia leads to increased synthesis of Hb + RBCs resulting in mass (polycythemia) Clubbing of tips of fingers and toes due to reduced oxygen  Tetralogy of Fallot This involves a combination of shunts with obstruction with functional impairment There is shunting of blood Its one of the most common cyanotic heart disease It involves 4 features: 1. VSD 2. Displacement of aorta to right side so that it overrides the septal defect 3. Sub-pulmonary stenosis (obstruction) 4. Right ventricular hypertrophy Clinical manifestations dependant on the extent of pulmonary stenosis & VSD 2 types : Cyanotic and Acyanotic (pink tetralogy) Cyanotic: - Pulmonary stenosis is greater causing an increase in resistance to flow of blood in RV, it flows to the LV causing Cyanosis Effects: - Pressure hypertrophy of RA and RV - Small tricuspid valve - Small left atrium & ventricle - Enlarged aortic orifice Acyanotic tetrology: - VSD larger, pulmonary stenosis is mild: L→ R shunt, It has characteristics like VSD Boot shaped heart  Transposition of Great arteries Regular transposition: - Aorta arises from RV and Pulmonary A from LV - Cyanosis from birth Corrected Transposition: - Aorta arises from Right Ventricle, Pulmonary Artery from Left Ventricle and the Pulmonary veins drain into Right Atrium, Superior and Inferior vena cava drain into Left atrium - Physiologically corrected circulation  Persistent Truncus Arteriosus Arch that separates aorta from pulmonary Artery fails to develop. A single large vessel receives blood from both the ventricles Often associated Ventricular Septal Defect Early systemic cyanosis Poor prognosis  Tricuspid Atresia and stenosis - Often associated with pulmonary stenosis or atresia - Atresia- absence of tricuspid orifice, there is dimple in floor of right atrium - Stenosis- tricuspid ring is small and hardened  Obstructions (Obstructive CHD) 1. Coarctation of Aorta 5-7% 2. Aortic stenosis and atresia 4-6% 3. Pulmonary stenosis and atresia 5- 7%  COARCTATION OF AORTA Localized narrowing in any part of the aorta More common in males, females with Turner syndrome Post ductal or adult type: - Obstruction is just distal to ductus arteriosus which is closed - Characterized by hypertension in upper extremities, weak pulses and low BP in the lower extremities, effects of arterial insufficiency such as coldness and claudication (muscle pain due to reduced oxygen) Preductal or Infantile type: - narrowing proximal to ductus arteriosus which remains patent - lower half of body cyanosed while upper part of body receives blood from aorta  AORTIC STENOSIS AND ATRESIA Most common anomaly of aorta is congenital bicuspid valve. Not much functional significance except Congenital aortic is atresia rare & incompatible with life Aortic stenosis- congenital or acquired(RHD) 3 types of congenital AS: 1.Valvular: cusps thickened and malformed 2.Subvalvular: thick fibrous ring under the aortic valve 3.Supravalvular: uncommon May be associated with hypoplastic heart syndrome: fatal in neonates  PULMONARY STENOSIS AND ATRESIA Stenosis - commonest form of obstructive CHD - occurs as component of Tetralogy Of Fallot or isolated defect - fusion of cusps of pulmonary valve forming diaphragm like obstruction Atresia - no communication between right ventricle & lungs - blood goes to left heart through interatrial septal defect and enters lungs via Patent Ductus Arteriosus  INVESTIATIONS Echocardiogram; it reveals a congenital heart defect after your child has been born. Electrocardiogram; This noninvasive test records the electrical activity of your child's heart and can help diagnose heart defects or rhythm problems.  Chest X-ray. Reveals if the heart is enlarged, or if the lungs have extra blood or other fluid in them. These could be signs of heart failure. Pulse oximetry. This test measures how much oxygen is in your child's blood. A sensor is placed over the end of your child's finger to record the amount of oxygen in your child's blood. Too little oxygen could suggest your child has a heart problem.  Cardiac catheterization. It gives much more detailed view of your child's heart defect than an echocardiogram. Cardiovascular magnetic resonance imaging (MRI). This type of imaging is becoming increasingly used to diagnose and evaluate congenital heart defects in adolescents and adults.  Treatment A congenital heart defect may have no long-term effect on your child's health in some instances, such defects can safely go untreated. Certain defects, such as small openings, may even correct themselves as your child ages.  Some heart defects, however, are serious and require treatment soon after they're found. Depending on the type of heart defect your child has, doctors treat congenital heart defects with: Procedures using catheterization. Catheterization techniques, allow the repair to be done without surgically opening the chest and heart. Catheter procedures can often be used to fix holes or areas of narrowing.  Open-heart surgery. Depending on the child's condition, he or she may need surgery to repair the defect. Many congenital heart defects are corrected using open-heart surgery. In open-heart surgery, the chest has to be opened.  Minimally invasive heart surgery may be an option. This type of surgery involves making small incisions between the ribs and inserting instruments through them to repair the defect.  Heart transplant. If a serious heart defect can't be repaired, a heart transplant may be an option. Medications. Some mild congenital heart defects, especially those found later in childhood or adulthood, can be treated with medications that help the heart work more efficiently.  Drugs known as angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers (ARBs) and beta blockers and medications that cause fluid loss (diuretics) can help ease stress on the heart by lowering blood pressure, heart rate and the amount of fluid in the chest. Certain medications can also be prescribed to help irregular heartbeats (arrhythmias).  Sometimes, a combination of treatments is necessary. In addition, some catheter or surgical procedures have to be done in steps, over a period of years. Others may need to be repeated as a child grows.  Long-term treatment Some children with congenital heart defects require multiple procedures and surgeries throughout life. Although the outcomes for children with heart defects have improved dramatically, most people, except those with very simple defects, will require ongoing care, even after corrective surgery.  Monitoring and treatment The child will undergo Lifelong monitoring and treatment. Even if your child has surgery to treat a heart defect, the child's condition will need to be monitored for the rest of his or her life. Initially, the child with a congenital heart defect will be monitored and have regular follow-up appointments with a pediatric cardiologist.  As child grows older, his or her care will transition to an adult congenital cardiologist, who can monitor his or her condition over time. A congenital heart defect can affect the child's adult life, as it can contribute to other health problems. Adults who have congenital heart defects may need other treatments for their condition.  As the child ages, it's important to remind him or her of the heart condition that was corrected and the need for ongoing, lifelong care by doctors experienced in evaluating and treating congenital heart disease. Encourage child to keep his or her doctor informed about the heart defect and the procedures performed to treat the problem.  Exercises Exercise should be restricted. Parents of children with congenital heart defects may worry about the risks of rough play and activity even after treatment. Although some children may need to limit the amount or type of exercise, many can participate in normal or near-normal activity.  Your child's doctor can tell you which activities are safe for your child. If some activities do pose distinct dangers, encourage your child to participate in other activities instead of focusing on what he or she can't do. Although every child is different, most children with congenital heart defects grow up to lead healthy, productive lives.  Infection prevention Depending on the type of congenital heart defect your child had, and the surgery used to correct it, your child may need to take extra steps to prevent infection. Children who are most likely to have a higher risk of infection include those whose defect was repaired with a prosthetic material or device, such as an artificial heart valve.  Sometimes, a congenital heart defect can increase the risk of infections either in the lining of the heart or heart valves (infective endocarditis). Because of this risk, the child may need to take antibiotics to prevent infection before additional surgeries or dental procedures.