Tetralogy of Fallot is the most common cyanotic congenital Congenital Chorioretinitis heart disease. The disease is the confluence of 4 pathologic cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) features: overriding aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy, ventricular septal defect, right ventricular outflow obstruction, and right ventricular hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation. The timing and severity of presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor usually depend on the degree of right ventricular outflow obstruction. Definitive diagnosis is usually established by echocardiogram Echocardiogram Transposition of the Great Vessels. Chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests may show the classic boot-shaped heart. Definitive treatment involves surgical repair. Long-term prognosis Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas is good for surgically corrected disease, but cardiovascular morbidity Morbidity The proportion of patients with a particular disease during a given year per given unit of population. Measures of Health Status is common.
Last updated: 18 Nov, 2021
Tetralogy of Fallot (TOF) is the most common cyanotic congenital Congenital Chorioretinitis heart disease and has the following 4 (tetra) features:
DROP:
PROVe:
Anatomy and physiology of tetralogy of Fallot
Left: normal heart
Right: tetralogy of Fallot
Cardinal features of tetralogy of Fallot: autopsy specimen opened through the anterior wall of the right ventricle
Image: “Tetralogy of Fallot autopsy” by North Carolina Children’s Heart Center, Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA. License: CC BY 2.0Age of presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor is inversely proportional to the degree of RVOT obstruction.
Cyanosis
Cyanosis
A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.
Pulmonary Examination in a
neonate
Neonate
An infant during the first 28 days after birth.
Physical Examination of the Newborn due to
congenital
Congenital
Chorioretinitis heart disease:
The infant in the image displays
cyanosis
Cyanosis
A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.
Pulmonary Examination due to poor perfusion caused by
congenital
Congenital
Chorioretinitis heart disease (in this case,
transposition of the great vessels
Transposition of the Great Vessels
Transposition of the great vessels (TGV) is a cyanotic congenital heart disease characterized by “switching” of the great arteries. There are 2 presentations: the dextro (D)- and levo (L)-looped forms. The L-looped form is rare and congenitally corrected, as the ventricles are also switched.
Transposition of the Great Vessels, but all
cyanosis
Cyanosis
A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.
Pulmonary Examination due to
congenital
Congenital
Chorioretinitis heart disease looks similar).
Cyanosis
Cyanosis
A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.
Pulmonary Examination can be subtle and is most clearly seen around the
lips
Lips
The lips are the soft and movable most external parts of the oral cavity. The blood supply of the lips originates from the external carotid artery, and the innervation is through cranial nerves.
Lips and Tongue: Anatomy and fingertips.
Boot-shaped heart: chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests of a 16-month-old boy with tetralogy of Fallot
Image: “Typical preoperative chest X‐ray of a 16‐month‐old boy with tetralogy of Fallot” by Andrew C. Chatzis et al AL Amyloidosis. License: CC BY 4.0Echocardiogram Echocardiogram Transposition of the Great Vessels of an individual with tetralogy of Fallot:
A parasternal short- axis Axis The second cervical vertebra. Vertebral Column: Anatomy view demonstrating deviation of the outlet septum into the right ventricular outflow tract
Echocardiogram
Echocardiogram
Transposition of the Great Vessels of an individual with tetralogy of Fallot:
A modified parasternal long-
axis
Axis
The second cervical vertebra.
Vertebral Column: Anatomy view demonstrating characteristic features, including a large ventricular septal defect, aortic override (
aorta
Aorta
The main trunk of the systemic arteries.
Mediastinum and Great Vessels: Anatomy displaced over the ventricular septal defect), and right ventricular
hypertrophy
Hypertrophy
General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia).
Cellular Adaptation
Definitive:
Palliative: