A cyanotic heart defect is any

congenital heart defect A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascula ...

(CHD) that occurs due to deoxygenated

blood Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood in th ...

bypassing the lungs and entering the

systemic circulation The blood circulatory system is a system of organs that includes the heart, blood vessels, and blood which is circulated throughout the entire body of a human or other vertebrate. It includes the cardiovascular system, or vascular system, tha ...

, or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart such as

right-to-left In a script (commonly shortened to right to left or abbreviated RTL, RL-TB or R2L), writing starts from the right of the page and continues to the left, proceeding from top to bottom for new lines. Arabic, Hebrew, Persian, Pashto, Urdu, Kashmir ...

or bidirectional shunting, malposition of the great arteries, or any condition which increases pulmonary vascular resistance. The result may be the development of collateral circulation.

Types

Tetralogy of Fallot Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are: * pulmonary stenosis, which is narrowing of the exit from the r ...

(ToF) *

Total anomalous pulmonary venous connection Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital defect of the pulmonary veins. Total anomalous pulmonary venous connection ''Total anomalous pulmonary venous co ...

* Hypoplastic left heart syndrome (HLHS) * Transposition of the great arteries (d-TGA) * Truncus arteriosus (Persistent) * Tricuspid atresia * Interrupted aortic arch * Pulmonary atresia (PA) * Pulmonary stenosis (critical) * Eisenmenger syndrome (reversal of shunt due to pulmonary hypertension).Page

Signs and symptoms

Presentation includes the following: * Clubbing * The patient assuming a crouching position * Cyanosis - bluish face, particularly the lips; and bluish fingers and toes * Crying * Crabbiness/irritability *

Tachycardia Tachycardia, also called tachyarrhythmia, is a heart rate that exceeds the normal resting rate. In general, a resting heart rate over 100 beats per minute is accepted as tachycardia in adults. Heart rates above the resting rate may be normal ( ...

* Tachypnea * A history of inadequate feeding * Unusually large toe and fingernails * Delayed development (both biological and physiological)

Diagnosis

Management

Morphine Morphine is a strong opiate that is found naturally in opium, a dark brown resin in poppies ('' Papaver somniferum''). It is mainly used as a pain medication, and is also commonly used recreationally, or to make other illicit opioids. Ther ...

during

Tet Tet or TET may refer to: Vietnam *Tết or Tết Nguyên Đán, the Vietnamese new year, Lunar new year *Tet Offensive, a military campaign during the Vietnam War that began in 1968 **Tet 1969 Geography *Têt (river) in Roussillon, France *Tét, ...

spells to decrease associated infundibular spasm. * Prophylactic:

Propranolol Propranolol, sold under the brand name Inderal among others, is a medication of the beta blocker class. It is used to treat high blood pressure, a number of types of irregular heart rate, thyrotoxicosis, capillary hemangiomas, performance a ...

/Inderall *

Prostaglandin The prostaglandins (PG) are a group of physiologically active lipid compounds called eicosanoids having diverse hormone-like effects in animals. Prostaglandins have been found in almost every tissue in humans and other animals. They are der ...

E (to keep the ductus arteriosus patent) * Prophylactic antibiotic to prevent

endocarditis Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves. Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or the ...

* Surgery: Variable. Superior Cavopulmonary Bypass (Bidirectional Glenn or Hemi-Fontan Procedure), Total Cavopulmonary Bypass (Fontan Completion Procedure). The purpose of these operations is to redirect the blood flow of the deoxygenated blood to the lungs by attaching the Vena Cava directly to the Pulmonary Artery causing the blood that flows into the lungs to be oxygenated before entering the chambers on the right side of the heart. Mathematical models are used to address the issue of pressure level alterations of circulation after the procedures. The pulmonary pressure resistance in the cavopulmonary connection is increased, and these models permit clear analyses of the pressure increase allowing doctors to avoid possible venous circulation congestion.

References

External links