Tetralogy of Fallot: Facts and Resources About the Congenital Heart Disorder

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Tetralogy of Fallot (TOF) is a condition consists of four congenital heart defects that are accompanied with each other.  Tetralogy means four.  The complexity of this disorder includes a hole in the ventricular septum locating between the two lower pumping chambers of the heart (interventricular communication).  Another problem is a muscular obstruction causing the decrease of flow of blood through the right ventricle.  The enlargement of the right ventricle causes the third problem as it tries to pump blood past the same obstruction into the pulmonary artery.  The fourth problem is aorta sitting over the ventricular septal defect by shifting towards the right side of the heart (biventricular connection of the aortic root).   The clinical description of the physiology created by the anatomic abnormalities is La Maladie Bleue, as described in 1888 by Louis Arthur Etienne Fallot, a French physician.

Almost 50% of patients that are diagnosed with tetralogy of Fallot after their mothers gave give birth.  The newborns, while they were being developed in their mother’s womb, had their right side of the heart not properly developed.  Differential diagnosis of it includes primary pulmonary causes of cyanosis in accordance with other cyanotic heart lesions.

Living Past Childhood

Based on studies of immediate and long-term follow-up care, patients who were born with Tetralogy of Fallot during the 1970s displays that more than eighty-five percent of them are able to survive for the long-term.  Moreover, they are able to lead normal lives as evidenced, for example, by the ability to carry pregnancies successfully to full-term.  They must also avoid serious residues from surgeries and procedures to live a long life.  Chronic issues that adults with TOF currently face after their surgical repair include recurrent or residual pulmonary stenosis, ventricular arrhythmias, and the haemodynamic manifestations of chronic pulmonary regurgitation.  The enhancements of the strategies for medical and surgical management have currently improved the morbidity and mortality of the newborns with TOF.  Thus, the prognosis of babies born in the current era is expected to continue to ameliorate dramatically.  The main key to living a long and normal life is to perpetually manage the heart with cardiologists that are trained in congenital heart defects.

Congenital

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, which is known to be connected with other congenital disorders including DiGeorge syndrome, Goldenhar syndrome, and velo-cardio-facial syndrome.  TOF affects one out of every one thousand babies born with congenital heart disease, and it is a little more common among males.

Low in Oxygen

Tetralogy spell (hypercyanosis) happens when the arterial oxygen saturation of babies with tetralogy can drop quickly and significantly.  It is caused by abrupt greater compression of the outflow tract to the lungs making the pulmonary blood flow more limited.  Children will first become exceedingly bad-tempered in response to the critical low levels of oxygen.  They then might become sleepy or unresponsive if the distress from cyanosis continues on.  Sudden dwindle in arterial oxygen level causes babies to have their lips and skin be more keenly blue.  Whenever that happens, assuage the baby and also flex the knees forward and upward.  This technique may not always succeed; calling for immediate medical attention is usually the action that is called for if the baby isn’t feeling more soothing.

Alleviating the Heart Defect

Infants with ductal-dependent flow to the lungs will temporarily receive hormone-like substances (prostaglandins) to maintain ductal patency until the main surgery is performed.  Initial intervention may be palliative, such as the common surgical creation of a systemic-to-pulmonary arterial shunt (Blalock-Taussig shunt).  Surgeons create a connection between one of the major arteries of the body and the right pulmonary artery to increase the amount of red blood reaching the lungs and relieve cyanosis from the patient.  This will enable babies to grow big enough to have complete surgical repairs  Yet, there is a trend to perform the repairs on infants. Medical centers that take on neonatal palliation will perform the complete repair at the approximate age of four to six months.

Resources About Tetralogy of Fallot

Children’s Hospital Boston

Orphanet Journal of Rare Diseases

University of Michigan – C. S. Mott Children’s Hospital

Cincinnati Children’s Hospital Medical Center

Emedicine Health – Experts for Everyday Emergencies

Resource by S.Lee

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