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Developmental Anatomy & Congenital Heart Defects in Children

Explore the amazing journey of heart development in children, from embryology to postnatal changes and common defects. Learn about congenital heart conditions and their complications. Understand obstructive and mixed defects.

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Developmental Anatomy & Congenital Heart Defects in Children

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  1. Alterations of Cardiovascular Function in Children Chapter 31

  2. Developmental Anatomy of the Cardiovascular System • Embryology • Cardiogenesis begins at approximately 3 weeks’ gestation • The heart arises from the mesenchyme • Develops as an enlarged blood vessel with a large lumen and muscular wall • Midsection grows faster than the ends • The heart tube elongates and rotates to the right, creating a bulboventricular loop • Fetal heart contractions begin by approximately the 28th day

  3. Developmental Anatomy of the Cardiovascular System

  4. Developmental Anatomy of the Cardiovascular System • Cardiac septation • Endocardial cushions • Septum primum and the septum secundum • Ostium primum • Ostium secundum • Foramen ovale • Ductus arteriosus

  5. Developmental Anatomy of the Cardiovascular System

  6. Transitional Circulation • Circulatory changes take place that affect blood flow, vascular resistance, and oxygen tension • Closure of fetal shunts • Ductus venosus • Foramen ovale • Ductus arteriosus

  7. Postnatal Development • Changes in the position of the heart • Changes in the size of the right ventricle • Hemodynamics • Decreased pulmonary vascular resistance • Increased systemic vascular resistance • Heart rate ranges from 100 to 180 beats per minute • Newborns have a high oxygen demand

  8. Congenital Heart Defects • Underlying cause is known in only 10% of defects • Prenatal, environmental, and genetic risk factors • Maternal rubella, insulin-dependent diabetes, alcoholism, PKU, and hypercalcemia • Drugs • Chromosome aberrations

  9. Complications of Congenital Heart Defects • Congestive heart failure • Hypoxemia • Cyanosis • Eisenmenger syndrome

  10. Defects Increasing Pulmonary Blood Flow • Patent ductus arteriosus (PDA) • Failure of the ductus arteriosus to close • PDA allows blood to shunt from the pulmonary artery to the aorta

  11. Patent Ductus Arteriosus (PDA)

  12. Defects Increasing Pulmonary Blood Flow • Atrial septal defect • Abnormal communication between the atria • Three major types • Ostium primum defect • Ostium secundum defect • Sinus venosus defect

  13. Atrial Septal Defect

  14. Defects Increasing Pulmonary Blood Flow • Ventricular septal defect (VSD) • Abnormal communication between the ventricles • Most common type of congenital heart lesion • Types • Perimembranous VSD • Muscular VSD • Supracristal VSD • AV canal VSD

  15. Ventricular Septal Defect (VSD)

  16. Defects Increasing Pulmonary Blood Flow • Atrioventricular canal defect (AVC) • Results from nonfusion of the endocardial cushions • Demonstrates abnormalities in the atrial and ventricular septa and atrioventricular valves • Complete, partial, and transitional AVCs

  17. Atrioventricular Canal Defect

  18. Defects Decreasing Pulmonary Blood Flow • Tetralogy of Fallot • Syndrome represented by four defects • Ventricular septal defect (VSD) • Overriding aorta straddles the VSD • Pulmonary valve stenosis • Right ventricle hypertrophy

  19. Tetralogy of Fallot

  20. Defects Decreasing Pulmonary Blood Flow • Tricuspid atresia • Imperforate tricuspid valve • Lack of communication between the right atrium and right ventricle • Additional defects • Septal defect • Hypoplastic or absent right ventricle • Enlarged mitral valve and left ventricle • Pulmonic stenosis

  21. Tricuspid Atresia

  22. Obstructive Defects • Coarctation of the aorta • Narrowing of the lumen of the aorta that impedes blood flow • Coarctation of the aorta is almost always in a juxtaductal position, but it can occur anywhere between the origin of the aortic arch and the bifurcation of the aorta in the lower abdomen

  23. Coarctation of the Aorta

  24. Obstructive Defects • Aortic stenosis • Narrowing of the aortic outflow tract • Caused by malformation or fusion of the cusps • Causes an increased workload on the left ventricle

  25. Aortic Stenosis

  26. Obstructive Defects • Pulmonary stenosis • Narrowing of the pulmonary outflow tract • Abnormal thickening of the valve leaflets • Narrowing of the valve • Pulmonary semilunar valve atresia

  27. Pulmonary Stenosis

  28. Obstructive Defects • Hypoplastic left heart syndrome • Abnormal development of the left-sided cardiac structures • Obstruction to blood flow from the left ventricular outflow tract • Under development of the left ventricle, aorta and aortic arch, and mitral atresia or stenosis

  29. Hypoplastic Left Heart Syndrome

  30. Mixed Defects • Transposition of the great arteries • Aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle • Results in two separate, parallel circuits • Unoxygenated blood circulates continuously through the systemic circulation • Oxygenated blood circulates continuously through the pulmonary circulation • Extrauterine survival requires communication between the two circuits

  31. Transposition of the Great Arteries

  32. Mixed Defects • Total anomalous pulmonary venous connection (TAPVC) • Pulmonary veins connect to the right side of the heart, directly or indirectly through one or more systemic veins that drain into the right atrium

  33. Total Anomalous Pulmonary Venous Connection (TAPVC)

  34. Mixed Defects • Truncus arteriosus • Failure of the embryonic artery and the truncus arteriosus to divide into the pulmonary artery and the aorta • The trunk straddles an always present VSD

  35. Truncus Arteriosus

  36. Acquired Cardiovascular Disorders • Kawasaki disease • Also known as mucocutaneous lymph node syndrome • Acute, self-limiting systemic vasculitis that may result in cardiac sequelae

  37. Kawasaki Disease • Stages • One (0-12 days): capillaries, venules, arterioles, and the heart become inflamed • Two (12-35 days): inflammation of larger vessels; coronary aneurysms appear • Three (26-40 days): medium-sized arteries begin granulation process; small vessel inflammation decreases • Four (day 40 and beyond): scarring of vessels, thickening of tunica intima, calcification, coronary artery stenosis

  38. Kawasaki Disease • Diagnosis (5 of 6 major findings) • Fever for 5 or more days (unresponsive to antibiotics) • Bilateral conjunctivitis without exudation • Erythema of oral mucosa (strawberry tongue) • Changes in the extremities, such as peripheral edema and erythema with desquamation of palms and soles • Polymorphous rash • Cervical lymphadenopathy

  39. Acquired Cardiovascular Disorders • Systemic hypertension • Hypertension in children differs from adult hypertension • Often have an underlying disease • Renal disease or coarctation of the aorta • A cause of the hypertension in children is almost always found • Children with hypertension are commonly asymptomatic

  40. Acquired Cardiovascular Disorders • Childhood obesity • Multivariable and multidimensional • Risk factors • Race, socioeconomic status, and lack of health insurance • Childhood nutrition, level of physical activity, and engagement of sedentary activities (TV, computer use, etc.) • Association with parental obesity

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