congenital heart disease

Congenital Heart Disease : 

Congenital Heart Disease JOSEPH WALTER GROUP-34 4TH COURSE VSMU

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A congenital heart defect (CHD) is a defect in the structure of the heart and great vessels of a newborn. Most heart defects either obstruct blood flow in the heart or vessels near it or cause blood to flow through the heart in an abnormal pattern, although other defects affecting heart rhythm (such as long QT syndrome) can also occur. Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths. Approximately 1 in 125 babies are born each year in the US with a Congenital Heart Defect.

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Congenital heart defects are heart problems that develop before birth. They can occur in the heart's chambers, valves or blood vessels. A baby may be born with only one defect or several defects. Of the dozens of heart defects, some are mild and may need little or no medical treatment even through adulthood. Other types of congenital heart defects are life-threatening, either immediately to the newborn or over time.

The Heart : 

The Heart

Causes : 

Causes Heredity may play a role in some heart defects. For example, a parent who has a congenital heart defect may be more likely than other people to have a child with the condition. In rare cases, more than one child in a family is born with a heart defect. Children who have genetic disorders, such as Down syndrome, often have congenital heart defects. In fact, half of all babies who have Down syndrome have congenital heart defects. Smoking during pregnancy also has been linked to several congenital heart defects, including septal defects. Scientists continue to search for the causes of congenital heart defects.

Signs and Symptoms : 

Signs and Symptoms Symptoms and signs are related to the type and severity of the heart defect. Some children have no signs while others may exhibit shortness of breath, cyanosis, chest pain, syncope, sweating, heart murmur, respiratory infections, under-developing of limbs and muscles, poor feeding, or poor growth, build up of blood and fluid in lungs, feet, ankles and legs. Congenital heart defects cause abnormal heart structure resulting in production of certain sounds called heart murmur. Doctors can sometimes detect it with stethoscope; however, not all heart murmurs are caused by congenital heart defects. CHD symptoms frequently present early in life, but it's possible for some CHDs to go undetected throughout the life span.

Classification of Congenial Heart Disease : 

Classification of Congenial Heart Disease Obstructive Congenital Heart Lesions Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow

Obstructive Congenital Heart Lesions : 

Obstructive Congenital Heart Lesions Impede the forward flow of blood and increase ventricular afterloads. Pulmonary Stenosis Aortic Stenosis Coarctation of the Aorta

Pulmonary Stenosis : 

Pulmonary Stenosis No symptoms in mild or moderately severe lesions. Cyanosis and right-sided heart failure in patients with severe lesions. High pitched systolic ejection murmur maximal in second left interspace. Ejection click often present.

Pulmonary Stenosis : 

Pulmonary Stenosis

Aortic Stenosis : 

Aortic Stenosis Valvular Aortic Stenosis Subaortic Stenosis Supravalvular Aortic Stenosis Asymmetric Septal Hypertrophy (Idiopathic Hypertrophic Subaortic Stenosis)

Valvular Aortic Stenosis : 

Valvular Aortic Stenosis Most common type, usually asymptomatic in children. May cause severe heart failure in infants. Prominent left ventricular impulse, narrow pulse pressure. Harsh systolic murmur and thrill along left sternal border, systolic ejection click.

Valvular Aortic Stenosis : 

Valvular Aortic Stenosis Predominantly in males Thickened, fibrotic, malformed aortic leaflets. Fused commissures Bicuspid aortic valve.

Valvular Aortic Stenosis : 

Valvular Aortic Stenosis

Coarctation of the Aorta : 

Coarctation of the Aorta Absent or weak femoral pulses. Systolic pressure higher in upper extremities than in lower extremities; diastolic pressures are similar. Harsh systolic murmur heard in the back.

Coarctation of the Aorta : 

Coarctation of the Aorta Males twice as frequently as females. 98% of all coarctations at segment of aorta adjacent to ductus arteriosus. Produced by both an external narrowing and an intraluminal membrane. Blood flow to the lower body maintained through collateral vessels.

Coarctation of the Aorta : 

Coarctation of the Aorta

Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow : 

Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow Atrial Septal Defect Complete Atrioventricular Canal Ventricular Septal Defect Patent Ductus Arteriosis Total Anomalous Pulmonary Venous Connection Truncus Arteriosus

Atrial Septal Defect : 

Atrial Septal Defect Acyanotic; asymptomatic, or dyspnea on exertion. Right ventricular lift. Fixed, widely split second heart sound.

Atrial Septal Defect : 

Atrial Septal Defect Average life expectancy reduced because of right ventricular failure, dysrhythmias, and pulmonary vascular disease. Surgical closure is recommended.

Atrial Septal Defect : 

Atrial Septal Defect

Atrial Septal Defect : 

Atrial Septal Defect

Atrial Septal Defect : 

Atrial Septal Defect

Complete Atrioventricular Canal : 

Complete Atrioventricular Canal Heart failure common in infancy. Cardiomegaly, blowing pansystolic murmur, other variable murmurs. Deficiencies of both atrial and ventricular septal cushions and abnormalities of both mitral and tricuspid valves.

Complete Atrioventricular Canal : 

Complete Atrioventricular Canal Partial and complete AV canal defects frequently accompany Down’s syndrome. Early surgical correction. Reconstruction of the AV valves and closure of the septal defects by a single or double patch technique.

Complete Atrioventricular Canal : 

Complete Atrioventricular Canal

Complete Atrioventricular Canal : 

Complete Atrioventricular Canal

Ventricular Septal Defect : 

Ventricular Septal Defect Asymptomatic if defect is small. Heart failure with dyspnea, frequent respiratory infections, and poor growth if defect is large. Pansystolic murmur maximal at the left sternal border.

Ventricular Septal Defect : 

Ventricular Septal Defect Often one component of another more complex congenital heart lesion. Heart is enlarged and lung fields are overcirculated. Many of the defects will close spontaneously by age 7-8 years.

Ventricular Septal Defect : 

Ventricular Septal Defect

Ventricular Septal Defect : 

Ventricular Septal Defect

Patent Ductus Arteriosis : 

Patent Ductus Arteriosis Murmur usually systolic, sometimes continuous, “machinery” Poor feeding, respiratory distress, and frequent respiratory infections in infants with heart failure. Physical exam and echocardiography.

Patent Ductus Arteriosis : 

Patent Ductus Arteriosis Indomethacin, a prostaglandin E1 inhibitor may close a PDA. Surgical treatment after one week, by ligation, clipping, or division.

Patent Ductus Arteriosis : 

Patent Ductus Arteriosis

Patent Ductus Arteriosis : 

Patent Ductus Arteriosis

Total Anomalous Pulmonary Venous Connection : 

Total Anomalous Pulmonary Venous Connection Pulmonary veins do not make a direct connection with the left atrium. Blood reaches the left atrium only through an atrial septal defect or patent foramen ovale. Pulmonary congestion, tachypnea, cardiac failure, and variable cyanosis.

Total Anomalous Pulmonary Venous Connection : 

Total Anomalous Pulmonary Venous Connection Diagnosis by cardiac catherization or echocardiography. Operative repair in all cases.

Truncus Arteriosus : 

Truncus Arteriosus Single large vessel overrides the ventricular septum and distributes all the blood ejected from the heart. Large VSD is present.

Truncus Arteriosus : 

Truncus Arteriosus

Truncus Arteriosus : 

Truncus Arteriosus Corrective operation with a valved conduit between right ventricle and pulmonary vessels. Conduit will need to be changed as child grows but likelihood to develop pulmonary vascular disease is greatly reduced.

Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow : 

Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow Tetralogy of Fallot Transposition of the Great Arteries Tricuspid Atresia Ebstein’s Anomaly

Tetralogy of Fallot : 

Tetralogy of Fallot Pulmonary stenosis VSD of the membranous portion Overriding aorta Right ventricular hypertrophy due to shunting of blood

Tetralogy of Fallot : 

Tetralogy of Fallot Addition of an atrial septal defect falls in the category of Pentalogy of Fallot. Hypoxic spells and squatting. Cyanosis and clubbing.

Tetralogy of Fallot : 

Tetralogy of Fallot

Transposition of the Great Arteries : 

Transposition of the Great Arteries Aorta from right ventricle, pulmonary artery from left ventricle. Cyanosis from birth, hypoxic spells sometimes present. Heart failure often present. Cardiac enlargement and diminished pulmonary artery segment on x-ray.

Transposition of the Great Arteries : 

Transposition of the Great Arteries Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA.

Transposition of the Great Arteries : 

Transposition of the Great Arteries

Transposition of the Great Arteries : 

Transposition of the Great Arteries

Tricuspid Atresia : 

Tricuspid Atresia Tricuspid valve is completely absent in about 2% of newborns with congenital heart disease. Blood flows from right atrium to left atrium through foramen ovale. Early cyanosis.

Tricuspid Atresia : 

Tricuspid Atresia Repair consists of shunt from right atrium to pulmonary artery or rudimentary right ventricle (Fontan procedure).

Ebstein’s Anomaly : 

Ebstein’s Anomaly Septal and posterior leaflets of the tricuspid valve are small and deformed, usually displaced toward the right ventricular apex. Most patients have an associated ASD or patent foramen. Cyanosis and arrhythmias in infancy are common.

Ebstein’s Anomaly : 

Ebstein’s Anomaly Right heart failure in half of patients. Operative repair with tricuspid valve replacement.

Diagnosis : 

Diagnosis Echocardiography Electrophysiology Heart catheterization Magnetic resonance imaging (MRI) Medical genetics Molecular cardiology

Treatments : 

Treatments A congenital heart defect may have no long-term effect on your child's health — in some instances, such defects can safely go untreated. Sometimes they aren't even discovered until adulthood. 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:

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Procedures using catheters Open heart surgery Heart transplantation Medications Long-term treatment Some children with congenital heart disease 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 lifelong medical care, even after corrective surgery.