Total anomalous pulmonary venous return
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Cardiovascular system pathology review
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
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AssessmentsTotal anomalous pulmonary venous return
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Total anomalous pulmonary venous return
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Total anomalous pulmonary venous return
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USMLE® Step 1 style questions USMLE
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Preview
A 2-day-old male newborn is being evaluated in the neonatal intensive care unit for progressive cyanosis, breathing difficulty and poor feeding. He was born to a 22-year-old mother who received no prenatal care. Temperature is 37.0° C (98.6° F), pulse is 160/min, blood pressure is 87/55 mm Hg, and respiration rate is 55/min. On physical examination, no craniofacial deformities are seen. Central cyanosis is present, and cardiac auscultation reveals fixed splitting of the second heart sound (S2) over the left upper sternal border. Chest radiograph is obtained and shown below:
Reproduced from: Radiopaedia
Which of the following is the most likely diagnosis?
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Summary
Anomalous pulmonary venous connection is a rare cyanotic congenital heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. Normally, pulmonary veins return oxygenated blood from the lungs to the left atrium where it can then be pumped to the rest of the body. A patent foramen ovale, patent ductus arteriosa or an atrial septal defect must be present, or else the condition is fatal due to a lack of systemic blood flow.
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