INTRODUCTION The Normal Heart has four chambers. Consisting of the 2 basic circulation; The pulmonary circulation carrying the deoxygenated blood and the Systemic circulation carrying Oxygenated blood supply. In an event this processes does not occur properly, oxygen and nutrients are not delivered to various tissues leading to different problems in the body. Cyanosis can occur independently of the heart. Example Raynaud Phenomenon.

INTRODUCTION Cyanotic heart defect is a group-type of congenital heart defects (CHDs) that occurs due to deoxygenated blood by-passing the lungs and entering the systemic circulation.

Signs & Symptoms  Clubbing  Cyanosis - facial discolouration (particularly the lips) and digit discolouration (fingers & toes).  Irritability  Tachycardia  Tachypnea  Unusually large toe & fingernails.

Categories Cyanotic heart Disease  Tetralogy of Fallot (ToF)  Transposition of the great arteries (d-TGA)  Truncus arteriosus (Persistent)  Tricuspid atresia  Patent ductus arteriosus Non cyanotic heart disease  Atrial septal defect  Ventricular septal defect  Coarctation of aorta

Tetralogy of Fallot (ToF) A congenital heart defect involving four anatomical abnormalities of the heart;  Stenosis of right ventricular outflow tract  Right ventricular hypertrophy  Ventricular septal defect  Aorta overides the ventricular septal defect Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions.

‘Boot Shaped‘ on Xray

Transposition of the great arteries  Congenital heart diseases involving the primary arteries (pulmonary artery and aorta).  Preexisting diabetes mellitus of an expectant mother is a risk factor that has been described for the fetus having Transposition of great arteries.

Truncus arteriosus (Persistent) A rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. Deoxygenated blood from the Right ventricle mixes with oxygenated blood from Left ventricle before pulmonary and aortic circulations seperation.

Tricuspid atresia A form of congenital heart disease whereby there is a complete absence of the tricuspid valve. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing.

Patent ductus arteriosus A congenital disorder in the heart where in a neonate's ductus arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life include increased work of breathing and poor weight gain.

Exams and Tests Physical examination confirms cyanosis.  Chest xray  Echocardiogram (ECG)  Complete Blood count  Testing the Heart Electrical System

Possible Complications Complications of cyanotic heart disease include:  Abnormal heart rhythms and sudden death  Chronic high blood pressure in the blood vessels of the lung  Heart failure  Infection in the heart  Stroke

Pharmacological Treatment & Management  Prophylactic: Propranolol/Inderall  Prostaglandin E (to keep the ductus arteriosus patent)  Prophylactic antibiotic to prevent endocarditis  Surgery

Case Study  A 27-year-old G2 P1 woman has a screening ultrasound performed at 18 weeks gestation. The fetus is appropriate in size for 18 weeks. The fetal kidneys, liver, head, and extremities appear normal. However, the fetus has a heart with a membranous ventricular septal defect, overriding aorta, and marked pulmonic atresia. If the baby were to be liveborn, which of the following characteristics on physical examination would most likely result from these cardiac defects?

References  Heart Disease: A Textbook of Cardiovascular Medicine, 11th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 65.  Wikipedia  Webpath  Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Zipes DP, Libby P, Bonow RO, eds. Braunwald's