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 cardiovascular ...

(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 the c ...

bypassing the

lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...

s 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 ...

bidirectional Bidirectional may refer to: * Bidirectional, a roadway that carries traffic moving in opposite directions * Bi-directional vehicle, a tram or train or any other vehicle that can be controlled from either end and can move forward or backward with e ...

shunting, malposition of the

great arteries The great arteries are the primary arteries that carry blood away from the heart, which include: * ''Pulmonary artery'': the vessel that carries oxygen-depleted blood from the right ventricle to the lungs. * ''Aorta'': the blood vessel through whic ...

, or any condition which increases

pulmonary vascular resistance Vascular resistance is the resistance that must be overcome to push blood through the circulatory system and create flow. The resistance offered by the systemic circulation is known as the systemic vascular resistance (SVR) or may sometimes be ca ...

. 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 ri ...

(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 con ...

Hypoplastic left heart syndrome Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital hea ...

(HLHS) *

Transposition of the great arteries Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital ...

(d-TGA) * Truncus arteriosus (Persistent) *

Tricuspid atresia Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventri ...

Interrupted aortic arch In digital computers, an interrupt (sometimes referred to as a trap) is a request for the processor to ''interrupt'' currently executing code (when permitted), so that the event can be processed in a timely manner. If the request is accepted, ...

Pulmonary atresia Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located ...

(PA) *

Pulmonary stenosis Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood. Signs and symptoms Cause Pulmonic stenosis is usually due to isolated valvula ...

(critical) *

Eisenmenger syndrome Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, pat ...

(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 (su ...

* 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 analgesic, pain medication, and is also commonly used recreational drug, recreationally, or to make ...

during Tet 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 anx ...

/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 derive ...

E (to keep the

ductus arteriosus The ''ductus arteriosus'', also called the ''ductus Botalli'', named after the Italian physiologist Leonardo Botallo, is a blood vessel in the developing fetus connecting the trunk of the pulmonary artery to the proximal descending aorta. It a ...

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