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Blue Babies : Past, present and future! Author:Dr Maitri Chaudhuri MD(Pediatrics), Fellow National Board(Ped Cardiology) Consultant Cardiologist Vikram Hospitals, Bangalore Email:[email protected] www.babyheart.in Introduction: Some children are blue at birth or infancy. A careful look at their lips, nails and tongue reveals blue/ dusky coloration. This is a medical condition called “CYANOSIS”. The word "cyanosis" comes from the Greek "cyanos" meaning dark blue. In this situation, there is deficiency of oxygen and the amount of deoxygenated hemoglobin in blood is more than 5G/deciliter. Often cyanosis is caused by serious heart or lung or hemoglobin (blood pigment) problem. These babies need thorough and careful examination and prompt treatment. Cyanotic Heart Disease: We will now discuss a very serious condition in children called Congenital Cyanotic Heart Disease. It is actually not one single disease but a group of diseases where baby is blue and is born with a defect in the heart. How to recognize that a newborn child is actually a blue baby? 1. 2. 3. 4. 5. 6. 7. 8. Baby ‘s lips and fingers are blue Even if the baby is warm, this bluishness persists Breathing difficulty Baby has very fast breathing or the chest is moving up and down with each breath Uncontrolled crying Baby is very cold Any other defect in baby If your pediatrician(baby doctor) is giving oxygen to the child, hearing an abnormal heart sound(Murmur), unable to feel good pulses or says your baby is in shock - Always Exclude Heart Disease A newborn with cyanotic heart disease given oxygen through a ventilator Cyanotic Heart Disease in Older Babies 1. Usually bluishness starts by 2-3 months of age 2. In the early stages, cyanosis appears when the baby is irritable or crying or passing motion and later stages, bluishness is seen always. 3. Baby cannot walk beyond a short distance and sits down (SQUATTING POSTURE) 4. Gets Drowsy or Lethargic or has Fits after prolonged exertion or crying 5. Fingertips get swollen. Medically this is called as “Clubbing” 6. Fever, headache etc 7. Joint pain Common Heart Diseases In Children with Cyanosis: The usually found diseases are: 1. Tetralogy of Fallot 2. Transposition of Great Arteries 3. Tricuspid Atresia 4. Pulmonary Atresia 5. Ebstein’s Anomaly 6. Eisenmenger’s syndrome Tetralogy of Fallot: A French Doctor, Arthur Etienne Louis Fallot discovered this disease and it was named after him. Has 4 things: 1. 2. 3. 4. Ventricular septal defect(hole) Pulmonary stenosis (obstructed blood circulation to lungs) Right Ventricular Hypertrophy(thick right lower chamber) Overriding of Aorta(Main artery of body is partially committed to right side) Usually presents by 2-3 months of age with bluishness, crying and irritability. One of the commonest heart diseases in babies. Diagnosis: Chest X-Ray: Boot Shaped Heart Echocardiogram showing VSD(blue arrow), overriding Aorta, Thickened right ventricle RV-Right Ventricle, Ao-Aorta, LV –Left Ventricle, LA – Left Atrium Treatment of Tetralogy of Fallot: The definite treatment is surgery called Total Correction. Surgically, the hole is closed and obstruction to lungs is corrected. It is an Open Heart Surgery, takes 6-8 hours, done under general anesthesia and usual recovery time is 2 weeks. The best time for surgery is six months age and six kg weight. Palliative/Temporary Surgery : Sometimes a newborn baby gets too much bluish from Tetralogy of Fallot. Then a temporary surgery called BT Shunt (Blalock- Taussig Shunt) is done followed by total correction at a later date. Precautions after BT Shunt 1. 2. 3. 4. 5. A medicine called Aspirin is given to prevent clot formation inside the shunt Aspirin dose is 3-5mg/kg. So, dose is as per the baby’s weight Don’t miss even a single dose of Aspirin without doctor’s advise Baby should not get dehydration( lack of water in body) If baby gets diarrhea, vomiting, excessive sweating replace the lost fluid by ORS or visit your pediatrician 6. If baby gets sudden breathing problem or turns too blue, BT shunt is getting obstructed. Rush to the cardiologist 7. Every 3 months visit your cardiologist and get an echo done. 8. Remember BT Shunt is Temporary. It does not grow with your child. Does medicines help in Tetralogy of Fallot? There is no medicine which cures completely Tetralogy of Fallot. 1. While waiting for sugery, doctors often prescribe an oral tablet called Inderal (Propranolol) taken two to three times a day. 2. Also the child should not have anemia and if Hemoglobin is less than 15 G%, better to take oral iron tonic. 3. If your child is very blue and crying unconsolably, try to calm him, loosen his clothes, take him to an open space and put in Knee- Chest Position. Rush to a doctor. Transposition of great arteries (TGA) This is a heart disease where the two main arteries(pipes) of our body arise from opposite cardiac chambers. RA LA RV LV In normal circulation, deoxygenated blood passes through right side of heart and goes to lungs for oxygenation. This oxygen rich blood from lungs return to left side of heart and then distributed throughout the body via aorta. RA – Right Atrium(Right Upper Chamber) LA- Left Atrium(Left Upper Chamber) PA AO RV – Right Ventricle(Right Lower Chamber) LV- left Ventricle (Left Lower Chamber) PA- Pulmonary Artery(Pipe to Lungs) LUNGS Aorta – Pipe to Body RA LA RV LV AO PA In Transposition of great arteries, the body pipe Aorta is connected to the right heart and the lung pipe ,Pulmonary Artery is connected to the left side. So, bad blood (deoxygenated) circulates throughout the body and oxygenated blood keeps returning in a closed circle to the lungs. There is no mixing of bad and oxygen rich blood. Baby remains intensely blue after birth. Presentation: 1. Within 2-3 days after birth, baby becomes blue 2. Often happens when mother has diabetes in pregnancy. 3. Usually large for age babies, weight 3 -3.5 Kg 4. Pulse oximetry: saturations between 60-85% 5. Giving oxygen to the baby has no significant change