BLOCK 8: Congenital Heart Defects (PART 2)
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Learning Objectives By Lesson
1-Eisenmenger Physiology
  • Define Eisenmenger physiology
  • Identify etiologies of Eisenmenger physiology
  • Describe the pathophysiology of Eisenmenger syndrome
  • Describe the clinical manifestations of Eisenmenger physiology
  • Identify the common causes of mortality related to Eisenmenger physiology
  • Describe the physical examination findings associated with Eisenmenger physiology
  • Describe potential reasons for why patients with Eisenmenger syndrome tend to live longer than patients with other forms of pulmonary arterial hypertension
  • Describe the long-term prognosis of Eisenmenger physiology
  • Describe the general approach to management issues that might arise with Eisenmenger physiology
2-Tetralogy of Fallot
  • Describe the epidemiology of tetralogy of Fallot (TOF)
  • Identify and describe the four characteristic features of tetralogy of Fallot (TOF)
  • Describe how anterior malalignment of the conal septum can account for the four charactersitic features of tetralogy of Fallot (TOF)
  • Describe the physiologic and hemodynamic findings expected in tetralogy of Fallot (TOF)
  • Identify what determines the direction and magnitude of flow across the ventricular septal defect (VSD)
  • Describe the pathophysiology underlying "tet" (hypercyanotic) spells
  • Describe the clinical examination in tetralogy of Fallot
  • Identify genetic and anatomic associations with tetralogy of Fallot (TOF)
  • Describe the general principles of management for tetralogy of Fallot (TOF)
3-Pulmonary Atresia with Ventricular Septal Defect
  • Describe the characteristic features pulmonary atresia with ventricular septal defect (PAVSD)
  • Identify the genetic and anatomic assocaitions of pulmonary atresia with ventricular septal defect (PAVSD)
  • Identify the anatomic components of pulmonary atresia with ventricular septal defect (PAVSD)
  • Describe the hemodynamic effects of pulmonary atresia with ventricular septal defect (PAVSD)
  • Describe the source of blood for the native pulmonary arteries
  • Describe the surgical goals and considerations for pulmonary atresia with ventricular septal defect (PAVSD)
  • Describe the unifocalization procedure for pulmonary atresia with ventricular septal defect (PAVSD)
4-Ebstein's Anomaly
  • Define Ebstein's anomaly and its characteristic features
  • Describe the clinical presentation of patient's with Ebstein's anomaly
  • Describe the pathophysiology in Ebstein's anomaly
  • Describe the leaflet abnormalities that can be observed with Ebstein's anomaly
  • Describe classification approaches for Ebstein's anomaly
  • Identify associated congenital abnormalities assocaited with Ebstein's anomaly
  • Describe potential ECG and chest X-ray features associated with Ebstein's anomaly
  • Describe the clinical utility of the echocardiogram and cardiac magnetic resnonance imaging in the evaluation of Ebstein's anomaly
  • Identify indications for surgery in patient's with Ebstein's anomaly
  • Describe surgical considerations in patient's with Ebstein's anomaly
5-Coarctation of the Aorta
  • Describe the anatomical findings in coarctation of the aorta
  • Describe what the isthmus of the aorta refers to
  • Describe important contemporary considerations in the evaluation of coarctation of the aorta
  • Describe a surgical classification schema for coarctation of the aorta
  • Identify associations with coarctation of the aorta
  • Describe the pathophysiologic mechanisms associated with coarctation of the aorta
  • Describe the collateral blood flow associated with coarctation of the aorta
  • Describe the clinical presentation of coarctation of the aorta and factors that influence it
  • Describe the diagnostic evaluation of coarctation of the aorta
  • Identify considerations for interventions in coarctation of the aorta
  • Describe various surgical and cather-based approaches for coarctation of the aorta
  • Identify surgical complications that can result following surgery for coarctation of the aorta
6-Complete Transposition of the Great Arteries (D-TGA)
  • Describe the anatomical findings in complete transposition of the great arteries (D-TGA)
  • Describe what is meant by transposition (ventriculoarterial discordance)
  • Describe what is meant by complete transposition
  • Describe what is meant by congenitally corrected transposition
  • Describe what is meant by malposition of the great arteries
  • Describe the embrylogical development of the heart and how it gives rise to future cardiac structures
  • Identify other defects associated with complete transposition of the great arteries (D-TGA)
  • Describe the physiology in complete transposition of the great arteries (D-TGA)
  • Describe the clinical presentation of complete transposition of the great arteries (D-TGA)
  • Describe the management approach for complete transposition of the great arteries (D-TGA)
  • Describe the atrial switch procedure for complete transposition of the great arteries (D-TGA)
  • Describe the arterial switch procedure for complete transposition of the great arteries (D-TGA)
  • Describe the Rastelli procedure for complete transposition of the great arteries (D-TGA)
  • Describe clinical outcomes in patients with complete transposition of the great arteries (D-TGA)
7-Congenitally Corrected Transposition of the Great Arteries (CC-TGA)
  • Describe the anatomical findings in congenitally corrected transposition of the great arteries (CC-TGA)
  • Identify other terms that are synonymous with congenitally corrected transposition of the great arteries (CC-TGA)
  • Describe the embrylogical development of the heart and how it gives rise to future cardiac structures
  • Describe different variations of congenitally corrected transposition of the great arteries (CC-TGA)
  • Describe what determines the physiological consequences of congenitally corrected transposition of the great arteries (CC-TGA)
  • Identify other defects associated with congenitally corrected transposition of the great arteries (CC-TGA)
  • Describe the clinical presentation of congenitally corrected transposition of the great arteries (CC-TGA), including in the unoperated adult patient
  • Describe the conventional and anatomic repair approaches for congenitally corrected transposition of the great arteries (CC-TGA)
  • Describe the double switch operation for congenitally corrected transposition of the great arteries (CC-TGA)
  • Identify candidacy requirements for undergoing the double switch operation
  • Identify various potential complications of an anatomic repair approach for congenitally corrected transposition of the great arteries (CC-TGA)
  • Describe clinical outcomes in patients with congenitally corrected transposition of the great arteries (CC-TGA)
8-Congenital Heart Disease Surgeries (Pt 1)
  • Describe the goals of palliative and staged surgeries for congenital heart disease
  • Identify congenital heart disease surgergies that improve pulmonary blood flow and those that protect pulmonary circulation
  • Describe the purpose of, indications for, and anatomical connections associated with the original and modified Blalock-Taussig shunt
  • Describe the anatomical connections and disadvantages associated with the Waterson and Potts' shunts
  • Describe the purpose of and anatomical connections associated with the Sano shunt
  • Describe the purpose and goals of pulmoary artery banding (e.g., in the setting of transposition of the great arteries)
  • Describe the purpose of and anatomical connections associated with a Glenn shunt
  • Describe and identify a long-term complication assocaited with a bidirectional Glenn shunt
9-Congenital Heart Disease Surgeries (Pt 2)
  • Describe the goals of, indications for, and anatomical connections created during the Fontan operation
  • Describe what is meant by hypoplastic left heart syndrome (HLHS)
  • Describe the goals of, indications for, and stages of the Norwood procedure
  • Describe the difference between D- and L-transposition of the great arteries (TGA)
  • Describe the surgical precordures (i.e., Mustard and Senning, Jantene, and Rastelli procedures) for transposition of the great arteries (TGA)
  • Describe the Rastelli procedure and its indications
  • Describe the Damus-Kaye-Stansel (DKS) procedure and its indications
Contributors: Nandan Anavekar, MBBCh; Mahmoud Ismayl, MD; Khalid Saeed Al-Asad, MD; Adnan Halboni, MD; Adolfo Martinez Salazar, MD; Anthony Kashou, MD
Last updated: January 2023.
Progress

Teachers
GCU Education Team
Physician Educators