Truncus arteriosus

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Truncus arteriosus

ETP Cardiovascular System

ETP Cardiovascular System

Introduction to the cardiovascular system
Anatomy of the heart
Anatomy of the coronary circulation
Anatomy clinical correlates: Heart
Anatomy of the superior mediastinum
Anatomy of the inferior mediastinum
Anatomy clinical correlates: Mediastinum
Development of the cardiovascular system
Fetal circulation
Cardiac muscle histology
Artery and vein histology
Arteriole, venule and capillary histology
Cardiovascular system anatomy and physiology
Lymphatic system anatomy and physiology
Coronary circulation
Blood pressure, blood flow, and resistance
Pressures in the cardiovascular system
Laminar flow and Reynolds number
Resistance to blood flow
Compliance of blood vessels
Control of blood flow circulation
Microcirculation and Starling forces
Measuring cardiac output (Fick principle)
Stroke volume, ejection fraction, and cardiac output
Cardiac contractility
Frank-Starling relationship
Cardiac preload
Cardiac afterload
Law of Laplace
Cardiac and vascular function curves
Altering cardiac and vascular function curves
Cardiac cycle
Cardiac work
Pressure-volume loops
Changes in pressure-volume loops
Physiological changes during exercise
Cardiovascular changes during hemorrhage
Cardiovascular changes during postural change
Normal heart sounds
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Action potentials in myocytes
Action potentials in pacemaker cells
Excitability and refractory periods
Cardiac excitation-contraction coupling
Electrical conduction in the heart
Cardiac conduction velocity
ECG basics
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Baroreceptors
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Renin-angiotensin-aldosterone system
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Truncus arteriosus
Transposition of the great vessels
Total anomalous pulmonary venous return
Tetralogy of Fallot
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Ventricular septal defect
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Premature atrial contraction
Atrioventricular nodal reentrant tachycardia (AVNRT)
Wolff-Parkinson-White syndrome
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Acyanotic congenital heart defects: Pathology review
Cyanotic congenital heart defects: Pathology review
Atherosclerosis and arteriosclerosis: Pathology review
Coronary artery disease: Pathology review
Peripheral artery disease: Pathology review
Valvular heart disease: Pathology review
Cardiomyopathies: Pathology review
Heart failure: Pathology review
Supraventricular arrhythmias: Pathology review
Ventricular arrhythmias: Pathology review
Heart blocks: Pathology review
Aortic dissections and aneurysms: Pathology review
Pericardial disease: Pathology review
Endocarditis: Pathology review
Hypertension: Pathology review
Shock: Pathology review
Vasculitis: Pathology review
Cardiac and vascular tumors: Pathology review
Dyslipidemias: Pathology review
Sympatholytics: Alpha-2 agonists
Adrenergic antagonists: Presynaptic
Adrenergic antagonists: Alpha blockers
Adrenergic antagonists: Beta blockers
ACE inhibitors, ARBs and direct renin inhibitors
Thiazide and thiazide-like diuretics
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cGMP mediated smooth muscle vasodilators
Class I antiarrhythmics: Sodium channel blockers
Class II antiarrhythmics: Beta blockers
Class III antiarrhythmics: Potassium channel blockers
Class IV antiarrhythmics: Calcium channel blockers and others
Lipid-lowering medications: Statins
Lipid-lowering medications: Fibrates
Miscellaneous lipid-lowering medications
Positive inotropic medications
Cardiomyopathies: Clinical
Congenital heart defects: Clinical
Valvular heart disease: Clinical
Infective endocarditis: Clinical
Pericardial disease: Clinical
Chest trauma: Clinical
Hypertension: Clinical
Pulmonary hypertension
Aortic aneurysms and dissections: Clinical
Raynaud phenomenon
Peripheral vascular disease: Clinical
Heart failure: Clinical
Coronary artery disease: Clinical
Deep vein thrombosis and pulmonary embolism: Pathology review
Fascia, vessels and nerves of the upper limb
Vessels and nerves of the forearm
Vessels and nerves of the hand
Anatomy of the abdominal viscera: Blood supply of the foregut, midgut and hindgut
Fascia, vessels, and nerves of the lower limb
Vessels and nerves of the gluteal region and posterior thigh
Anatomy of the popliteal fossa
Ventilation
Ventilation-perfusion ratios and V/Q mismatch
Gas exchange in the lungs, blood and tissues
Oxygen binding capacity and oxygen content
Oxygen-hemoglobin dissociation curve
Carbon dioxide transport in blood
Trypanosoma cruzi (Chagas disease)
Yellow fever virus
Rickettsia rickettsii (Rocky Mountain spotted fever) and other Rickettsia species
Arteriovenous malformation
Cerebral circulation

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Truncus arteriosus

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Questions

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A 3-day-old newborn male is evaluated in the neonatal intensive care unit for breathing difficulty. The patient was born at 39 weeks gestation via vaginal delivery at home, and this is his first medical evaluation. The mother is a 26-year-old woman who had minimal prenatal care. She reports that he “was fine” after birth, but after the first few days, she noted a constant bluish color of his lips. Temperature is 37°C (98.6°F), pulse is 170/min, respirations are 65/min, and blood pressure is 80/50 mm Hg. Pulse oximetry shows an oxygen saturation of 84% on room air. Physical exam shows low-set ears and bulbous nasal tip. Cardiac auscultation reveals a loud, single second heart sound and bounding peripheral pulses. Chest radiograph shows pulmonary congestion with increased pulmonary markings and decreased soft-tissue attenuation in the right anterior mediastinum. Which of the following cardiac defects is most likely to be seen with further analysis of this patient?  

External References

First Aid

2024

2023

2022

2021

Persistent truncus arteriosus p. 285, 302

Truncus arteriosus

21q10 syndromes p. 304

cyanosis with p. 731

embryologic development p. 284

thymic aplasia p. 114

Transcript

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Content Reviewers

The truncus arteriosus is a big structure that’s present during fetal development; later in development, it divides to form two separate arteries: the aorta and pulmonary artery. Once it’s fully developed, the aorta comes from the left ventricle, and the pulmonary artery comes from the right ventricle.

A persistent truncus arteriosus describes when this developmental structure doesn’t divide into the two separate arteries. Instead, the baby’s left with one giant artery that branches off from both the right and the left ventricles that then splits off into the aorta and the pulmonary artery. Sometimes, this condition is simply referred to as truncus arteriosus, or TA. The cause of TA is unknown; however, a lot of cases seem to be associated with 22q11.2 deletion syndrome, also known as DiGeorge Syndrome.

Let’s switch to a more simplified view of the heart. All right, so usually deoxygenated blood comes in from the body and travels to the right atrium; then, it goes to the right ventricle and is pumped through the pulmonary artery to the lungs to be re-oxygenated. Next, freshly oxygenated blood passes from the lungs to the left atrium, goes to the left ventricle, and gets pumped through the aorta to the body. Then that circuit repeats, right?

If these two great arteries, the aorta and the pulmonary artery, don’t divide, you essentially have this massive artery coming from both ventricles. However, notice that this one big artery does eventually split into the aorta and pulmonary artery. Even though they eventually split off, before they do, it’s just one single vessel; thus, the oxygenated and deoxygenated blood mix. When deoxygenated blood mixes into the systemic circulation, it’ll often present as cyanosis, a bluish-purple discoloration of the skin, which can be seen in a baby within the first days after birth.

Summary

Persistent truncus arteriosus (PTA) is a rare congenital heart defect in which the embryonic structure that normally divides into the pulmonary artery and aorta fails to form properly, resulting in a single large vessel that supplies blood to both the systemic and pulmonary circulations.

Symptoms of PTA may include cyanosis, rapid breathing, poor feeding, and difficulty gaining weight. If left untreated, PTA can lead to heart failure and other complications. Treatment typically involves surgical repair of the defect, which involves separating the pulmonary artery from the aorta and creating a connection between the right ventricle and the pulmonary artery.