1 CONGENITAL HEART DISORDERS Congenital Heart Diseases 2 CLASSIFICATIONS 1.

Acyanotic type - there is shunting of blood from the left side of the heart to the right side of the heart - there is no abnormal connection between pulmonary and systemic circulation - the connection within the heart chambers forces blood to from the arterial (left) to the venous (right) side of the heart, where it is reoxygenated - if left untreated, it will lead to heart failure - Signs of Left-sided Heart Failure: a. Feeding difficulty - usually the first sign because of easy fatigability when sucking or feeding b. Pallor c. tachypnea or rapid breathing - signs of Right-sided Heart Failure a. Hepatomegaly, abdominal pain b. Anorexia c. jugular vein distention d. diaphoresis e. Edema - late sign - management: 1. digitalis- has a positive inotropic and negative chronotropic 2. reduce cardiac work load a. Complete bed rest b. Orthopneic position c. small frequent feeding d. minimal handling 3. remove fluid accumulation a. Diuretics d. weigh daily b. Low sodium diet c. I and O monitoring 4. administer oxygen to improve tissue perfusion

2 Ventricular Septal Defect (VSD) - there is abnormal opening between the ventricles

- manifestations: 1. Low harsh murmur heard throughout the systole pathognomonic sign 2. Right ventricular hyperthropy 3. Bradycardia, dyspnea and slowing of growth pattern 4. Prone to URTI and bacterial endocarditis - Management: 1. Large septal defect- closure with the use of plastic knitted dacron patch in a cardiopulmonary bypass through the right atrium and the tricuspid valve 2. Small septal defect- will close spontaneously or with the use of purse-string approach Patent Ductus Arteriosus (PDA) - failure of the fetal aorta and pulmonary artery to close - commonly associated to mother with puerperial rubella infection

3 - manifestations: 1. Shunting of blood leading to pulmonary hypertension and cardiomegaly 2. Machinery like murmur heard throughout the heartbeat in the left 2nd or 3rd intercostal area - pathognomonic sign 3. Prominent radial pulse in the newborn 4. Limited growth and physical activity 5. LEFT Ventricular enlargement on ECG and Chest x-ray - Management: 1. Closure of the opening at the 1 to 4 years through ligation or division 2. Use of endomethacin (prostaglandin inhibitor) is successful in closing a patent ductus arteriosus - Common complication: 1. Bacterial endocarditis due streptococcal infection Coarctation of the Aorta - narrowing of the aorta within the ductus

arteriosus

- manifestations: 1. Absence of femoral pulse- pathognomonic 2. Increased systemic circulation 3. BP is higher in the upper extremities due to bounding radial and carotid pulse, 4. Intermittent claudication, cold feet, muscle 5. Left ventricular hyperthropy

sign peripheral vascular resistance resulting to headache, dizziness and epistaxis spasm, weak, delayed or absent pulse

4 - management: For COA 1. Resection of the defect and anastomosis of the end of the aorta or enlargement of the constricted section using a graft of prosthetic material or a portion of left subclavian artery ; surgery until 8-10 years old 2. Balloon angioplasty is a primary intervention for COA is being performed in some centers Aortic Stenosis - narrowing of the aortic valve

- Manifestations: 1. Increased workload on the left ventricle and the lowered pressure in the aorta reduces coronary artery flow - management: 1. Valvotomy or balloon angioplasty or division of the stenotic valve 2. Cyanotic type - shunt from the right to left side of the heart - indicates an abnormality that permits the systemic venous return (unoxygenated blood) to bypass the lungs and enter the general circulation directly - Characteristics of Cyanotic type 1. Abnormal connection between the pulmonary and systemic circulation 2. Venous (unoxygenated) blood enters systemic circulation 3. Polycythemia occurs as the body compensate for inadequate supply of oxygen 4. Squatting or Knee chest position is preferred to lessen the venous return by constricting the femoral vein

5 Tetralogy of Fallot - has four associated defects: a. Pulmonary vein stenosis b. Ventricular septal defect c. Overriding of the aorta- receiving blood from both ventricle or an aorta arising from the right ventricle d. Right ventricular hyperthropy

- manifestations: 1. Cyanosis BLUE BABY 2. Clubbing of finger and toes 3. Growth retardation 4. Exertional dyspnea relieved SQUATTING- due to rapid saturation return 5. Polycythemia 6. Right ventricular hyperthropy x-ray result 7. Boot shaped configuration of the heart - management: 1. Blalock-Taussig procedure subclavian artery to pulmonary artery anastomosis 2. Complete repair closure of the ventricular septal defect and resection of the infundibular stenosis

6 Transposition of Great Vessels - aorta arise from the right ventricle and the pulmonary artery arises from the left ventricle - incompatible for life

- Management A. Palliative Management - performed to prevent pulmonary vascular resistance and CHF until the child id able to tolerate complete repair 1. Rashkind procedure enlargement of an existing atrial septal defect by pulling a balloon through the defect during cardiac catheterization 2. Pulmonary artery banding to decrease the blood flow to the lungs and increase shunting of oxygenated blood intraventricularly into the aorta 3. Pharmacologic dilation of PDA with the use of prostaglandin 4. Blalock-hanton surgical creation ASD B. Complete Repair: 1. Rastellis procedure - closure of VSD 2. Mustard or Seening Procedure removing and entire atrial septum and creating a new atrial septum from existing pericardium 3. Jatene Operation transposing the great vessel to correct the anatomic placement with reimplantation of the coronary arteries Tricuspid Atresia - absence of tricuspid valve - incompatible for life unless with ASD, VSD or PDA - management: 1. Palliative treatment procedure same as with TOF 2. Complete repair a. Modified Fontan procedure conversion of the right atrium into an outlet for pulmonary artery Truncus Arteriosus - single great vessel arising from base of the heart, serving as pulmonary and aorta

7 - manifestations: 1. Systolic murmur with a loud second heart sound that is not soft prosthetic valve split - Management: 1. Palliative banding of pulmonary arteries to decrease blood flow to the lungs 2. Complete repair Rastellis procedure wherein the existing pulmonary arteries from the aorta and attaching them to the RV means Sickle Cell Anemia - is a severe, chronic, hemolytic anemia occurring in persons who are homozygous for the sickle gene - is a group of hemoglobinopthies, in which the normal adult haemoglobin A (HbA) is partly or completely replaced by abnormal sickle hemoglobin ( HbS ) - statistics: -With high incidence among BLACKS - 8% among Blacks

Clinical manifestations: 1. Signs of Anemia a. Hemoglobin 6g/dl to 9g/dl b. Loss of appetite c. Paleness and weakness d. Fever and irritability e. Jaundice due to increase hemolysis resulting to hemosiderosis 2. Crises Manifestations: crises- acute symptoms of the disease exacerbation - predisposing factor for Crises: a. Dehydration f. Cold exposure b. Infection g. hypoxia c. trauma h. acidosis d. strenuous physical exercises e. Extreme fatigue

occurring during the period of

- Types of Crises A. Vaso-occlusive disease - preferably called painful episodes - extremities: a. Bony destructions related to hyperplasia of the marrow b. Bone pain and swelling joint c. dactylitis (hand-foot syndrome) - aseptic infarction of carpals and metacarpals 3. spleen a. Abdominal pain

9 b. Spleenomegaly- due to RBC destruction c. increase the risk of infection d. spleen becomes atrophied and fibrotic 4. cerebral occlusion a. Stroke, hemiplegia b. Retinal damage leading to blindness c. seizure 5. pulmonary infarction 6. altered renal function- enuresis and hematuria 7. impaired liver function 8. Priapism- abnormal, recurrent, prolonged and painful penile erection B. Sequestration crises - due to large amount of blood pooled in the spleen 1. Spleen becomes massively enlarge 2. Great decrease in RBC mass 3. signs of circulatory collapse 4. hepatomegaly C. Aplastic crises 1. diminished RBC production resulting to profound anemia Diagnostic Procedure: 1. Sickle cell prep (sickling test) - done by finger or heel stick - oxygen is removed from the dropped blood - the blood is observed under the microscope for sickle shape - test done to distinguish person with sickle cell trait and disease 2. Sickle turbidity test (sickledex) - done by finger stick - a small amount of blood is placed in the solution containing chemical reducing agent - sickle hemoglobin is indicated if the solutions turn turbid 3. Hemoglobin electrophoresis - requires venipuncture - is an accurate, rapid and specific test for detecting homozygous and heterozygous form of the disease Management: 1. Bed rest to minimize the energy expenditures 2. Hydration through oral and IV therapy 3. Electrolyte replacement 4. Analgesic for severe pain associated with vaso-occlusion 5. Blood replacement to treat anemia and to reduce the viscosity of the sickled blood 6. Antibiotics to treat any infection - administration of pneumococcal and meningococcal vaccine and hepa B vaccine

10