Congenital Heart Defects

Defects with Increased Pulmonary Blood Flow

Atrial Septal Defect

An abnormal connection between the R and L atria and is illustrated in the diagram.

Clinical Manifestations- generally asymptomatic- with soft systolic murmur- more classically a widely split S2 unaffected by respiratory pattern.

Diagnostics- presence of murmur- CXR – cardiomegaly- ECG - demonstrates size of defect.- cardiac death is not routinely indicated for dx of a n isolated ASD.

Treatment- preoperative interaction- Diuretics- surgical repair is performed in the preschool age.- sternotomy

Ventricular Septal Defect

Abnormal connection bet R. and L Ventricles.

Clinical Manifestations- asymptomatic- large defect – CHF- tachypneic, diaphoretic, fatigues easily, underweight for age- tires before feeding is completed.

Diagnostics- loud holosystic murmur- Normal CXR- large defects (cardiomegaly, increased in pulmonary blood flow)- ECG (shows size of defect)

Patent Ductus Arteriosus

Direct connection between the main pulmonary artery and the aorta.

Term newborn – the ductus closes within 12 hours and should be closed by 2-3 weeks.

s/s- if small : asymptomatic- if large : CHF

Atrioventricular Septal Defects

Asso. With septal defects in the atrium and ventricle as well as involvement of the AV Valves.

s/s:- s/s of CHF- with murmur- virtually all are asymptomatic

Truncus Arteriosus

Single arterial trunk arises from the heart.

s/s:- cyanosis- w/ s/s of CHF- with loud continuous murmur

- symptoms always develop in the 1st month of life.

Defects with decrease Pulmonary Blood Flow

Pulmonary Stenosis- refers to narrowing of the pulmonary valve and obstruction to blood flow from the R ventricle to the lungs.- s/s :

- mild to moderate – asymptomatic with mumur

- normal growth- dyspneas, cyanosis

Tetralogy of Fallot

Made up of VSD, PS and R Ventricular hypertrophy, overriding aorta

s/s:- TET spells- cyanosis- hyperpnea

Transposition of the Great Aorta

The aorta and the pulmonary artery is transposed or reversed.

Tricuspid Atresia

Characterized by absence of or complete closure of the tricuspid valve and therefore no connection bet the RA and the RV.

Includes ASD, VSD, and varying degrees of RV hypoplasia.

Maybe due to underdeveloped / incomplete development.

CardiomyopathyCardiomyopathy

disease of the heart muscle in which the heart loses its ability to pump blood effectively the heart muscle becomes enlarged or abnormally thick or rigid. In rare cases, the muscle tissue in the heart is replaced with scar tissue. As cardiomyopathy progresses the heart becomes weaker and less able to pump blood through the body to heart failure, arrhythmias, systemic and pulmonary edema and, more rarely, endocarditisThe 3 main types of cardiomyopathy are: Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy

CardiomyopathyCardiomyopathy

Dilated CardiomyopathyDilated Cardiomyopathy

most common form of cardiomyopathy generally occurs in adults aged 20 to 60 years more common in men

the heart muscle begins to dilate or stretch and become thinner

Ventricular chamber size

over time, the heart becomes weaker heart failure

symptoms of heart failure: fatigue, edema, and SOB can also lead to heart valve problems (regurgitation), arrhythmias, and blood clots in the heart (poor blood flow), emboli formation a common reason for needing a heart transplant.

Types and Causes:Types and Causes: Ischemic cardiomyopathy - caused by CAD & MI , w/c leave

scars in the heart muscle Idiopathic cardiomyopathy - the cause is unknown. Hypertensive cardiomyopathy - seen in people who

have high BP for a long time, particuarly when it has gone untreated for years.

Infectious cardiomyopathy - HIV, viral myocarditis Alcoholic cardiomyopathy - usually begins about 10 years

after sustained, heavy alcohol consumption. Toxic cardiomyopathy – due to cocaine, amphetamines,

and some chemotherapy drugs (doxorubicin, daunorubicin) Peripartum cardiomyopathy: This type appears in women

during the last trimester of pregnancy or after childbirth. Radiotherapy (cobalt) diabetes and thyroid disease

Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

occurs when the heart muscle thickens abnormally (left ventricle) 1.) obstructive type - the septum thickens and bulges into the left ventricle blocks the flow of blood into the aorta the ventricle must work much harder to pump blood past the blockage and out to the body

- symptoms can include chest pain, dizziness, shortness of breath, or fainting.

- can also affect the mitral valve, causing blood to leak backward through the valve. 2.) non-obstructive type - the entire ventricle may become thicker (symmetric ventricular hypertrophy) or it may happen only at the bottom of the heart (apical hypertrophy). The right ventricle also may be affected.

Pathophysiology: Left ventricular hypertrophy (thick ventricular wall) ventricular chamber size hold less blood CO pressure in the ventricles and lungs changes in the cardiac muscles interfere with the heart's electrical signals, leading to arrhythmias sudden cardiac arrest

Causes: inherited because of a gene mutation develop over time because of high blood pressure or aging often, the cause is unknown.

Hypertrophic CardiomyopathyHypertrophic Cardiomyopathy

Restrictive CardiomyopathyRestrictive Cardiomyopathy tends to mostly affect older adults the ventricles become stiff and rigid due to replacement of the normal heart muscle with abnormal tissue, such as scar tissue. As a result, the ventricles cannot relax normally and expand to fill with blood, which causes the atria to become enlarged. Eventually, blood flow in the heart is reduced, and complications such as heart failure or arrhythmias occur.Causes: radiation treatments, infections, or scarring after surgery Hemochromatosis - a condition in which too much iron is deposited into tissues, including heart tissue Amyloidosis, a disease in which abnormal proteins are deposited into heart tissue Sarcoidosis, a disease in which inflammation produces tiny lumps of cells in various organs in the body, including the heart

Major Risk FactorsMajor Risk Factors

Having a family history of cardiomyopathy, heart failure, or sudden cardiac death Having a disease or condition that can lead to cardiomyopathy, such as:

Coronary artery disease A previous heart attack Myocarditis

Diseases that can damage the heart (for example, hemochromatosis, sarcoidosis, or amyloidosis) Long-term alcoholism Long-term high blood pressure Diabetes and other metabolic diseases

Signs and SymptomsSigns and Symptoms some have no symptoms in the early stages of the disease as cardiomyopathy progresses and the heart weakens, signs and symptoms of heart failure usually appear. These signs and symptoms include:Tiredness Weakness Shortness of breath after exercise or even at rest Swelling of the abdomen, legs, ankles, and feet Other signs and symptoms: dizziness, lightheadedness, fainting during exercise, abnormal heart rhythms, murmurs

InterventionsInterventionsThe main goals of treating cardiomyopathy are to: Manage any conditions that cause or contribute to the cardiomyopathy Control symptoms so that the person can live as normally as possible Stop the disease from getting worse Reduce complications and the chance of sudden cardiac death Medications: Diuretics, which remove excess fluid and sodium from the body. Angiotensin-converting enzyme (ACE) inhibitors, which lower blood pressure and reduce stress on the heart. Beta-blockers, which slow the heart rate by reducing the speed of the heart's contractions. These medicines also lower BP Calcium channel blockers, which slow a rapid heartbeat by reducing the force and rate of heart contractions, decrease BP

MedicationsMedications Digoxin - increases the force of heart contractions and slows the heartbeat. Anticoagulants, which prevent blood clots from forming. Anticoagulants are often used in the treatment of dilated cardiomyopathy. Antiarrhythmia medicines, which keep the heart beating in a normal rhythm. Antibiotics, which are used before dental or surgical procedures. Antibiotics help to prevent endocarditis, an infection of the heart walls, valves, and vessels. Corticosteroids, which reduce inflammation.

SurgerySurgery Septal myectomy - also called septal myomectomy - is open-heart surgery for hypertrophic obstructive cardiomyopathy - generally used in younger patients and when medicines aren't working well. Procedure: 1. a surgeon removes part of the thickened septum that is bulging into the left ventricle this widens the pathway in the ventricle that leads to the aortic valve and improves blood flow through the heart and out to the body2. If necessary, the mitral valve can be repaired or replaced at the same time. This surgery is often successful, and the person can return to a normal life with no symptoms.

Surgery (cont.)Surgery (cont.) Surgically implanted devices. - Surgeons can place several different types of devices in the heart to help it beat more effectively. 1. A left ventricular assist device (LVAD) - helps the heart pump blood to the body - LVAD can be used as a long-term therapy or as a short-term treatment for people who are waiting for a heart transplant.2. An implantable cardioverter defibrillator (ICD) - is used in people who are at risk of life-threatening arrhythmia or sudden cardiac death. - This small device is implanted in the chest and connected to the heart with wires. If the ICD senses a dangerous change in heart rhythm, it will send an electric shock to the heart to restore a normal heartbeat. Heart Transplant

Left Ventricular Left Ventricular Assist DeviceAssist Device(LVAD)(LVAD)

Lifestyle ChangesLifestyle ChangesThe doctor may recommend lifestyle changes to manage a condition that is causing the cardiomyopathy. These changes may help reduce symptoms. Lifestyle changes may include:

Quitting smoking Losing excess weight Eating a low-salt diet Getting moderate exercise, such as walking, and avoiding strenuous exercise Avoiding the use of alcohol and illegal drugs Getting enough sleep and rest Reducing stress Treating underlying conditions, such as diabetes and high blood pressure

Heart TransplantHeart Transplant an operation in which the diseased heart in a person is replaced with a healthy heart from a deceased donor. 90% of heart transplants are performed on patients with end-stage heart failure --- condition has become so severe that all treatments, other than heart transplant, have failed.

Survival rates: 88 % of patients survive the first year after transplant 72 % survive for 5 years 50 % survive for 10 yrs. 16 % survive 20 years.

Patients who might not be candidates for heart transplant surgery, because the procedure is less likely to be successful.

Advanced age - most transplant surgery isn't performed on patients older than 70 years. Poor blood circulation throughout the body, including the brain. Diseases of the kidney, lungs, or liver that can't be reversed. History of cancer or malignant tumors. Inability or unwillingness to follow lifelong medical instructions after a transplant. Pulmonary arterial hypertension (high blood pressure in the lungs) that can't be reversed. Active infection throughout the body.

Heart Transplant (cont.)Heart Transplant (cont.)

Organs are matched for blood type and size of donor and recipient.

The Donor Heart Guidelines on how a donor heart is selected : the donor meet the legal requirement for brain death consent forms are signed younger than 65 years of age have little or no history of heart disease or trauma to the chest not exposed to hepatitis or HIV donor heart must be transplanted w/in 4 hrs. after removal from the donor

Heart Transplant (cont.)Heart Transplant (cont.)

Heart Transplant (cont.)Heart Transplant (cont.)

A bypass machine is hooked up to the arteries and veins of the heart. The machine pumps blood through the patient's lungs and body while the diseased heart is removed and the donor heart is sewn into place. Preventing Rejection Immunosuppressants used: cyclosporine, tacrolimus, MMF (mycophenolate mofetil), and steroids such as prednisone. Watching for Signs of Rejection

Shortness of breath Fever Fatigue Weight gain Reduced amounts of urine

Preventing Infection

Heart Transplant (cont.)Heart Transplant (cont.)

What Are the Risks of a Heart What Are the Risks of a Heart Transplant? Transplant?

Failure of the donor heart Primary Graft Dysfunction Rejection of the Donor Heart Cardiac Allograft Vasculopathy - the walls of the new heart's

coronary arteries become thick, hard, and lose their elasticity. - can cause heart attack, heart failure, dangerous arrhythmias,

and sudden cardiac arrest Complications from medicines - risk of infection, diabetes, osteoporosis , high blood pressure, kidney damage, and cancer Infection Cancer – lymphoma and skin cancer (due to suppression of the immune system)

End of Lecture…..