The NCCPA™ PANCE Cardiology System Content Blueprint covers five types of congenital heart disease. Congenital heart disease can be divided into two categories: cyanotic and noncyanotic
Congenital Heart Disease Quick Cram
| Atrial septal defect | Systolic murmur heard best at the upper left sternal border with a wide, fixed, split s2 |
| Coarctation of the aorta | Grade II/VI murmur along the left sternal border that radiates into the left axilla and the left side of the back. Femoral pulses are decreased bilaterally. rib notching on x-ray |
| Patent ductus arteriosus | A rough, continuous, "machinery murmur" at the upper left sternal border (pulmonary area) |
| Ventricular septal defect | Loud, harsh, pansystolic murmur at the lower left sternal border |
| Tetralogy of Fallot | Failure to thrive. "tet spells", baby with cyanosis and loss of consciousness with crying
Four features: PROVe
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Non Cyanotic heart defects
| Atrial septal defect | Patient will present as → a healthy 7-year-old girl who has reached all developmental milestones. On examination, the precordium is hyperdynamic with a prominent right ventricular heave. A grade III/VI systolic ejection murmur is present in the 2nd left intercostal space (pulmonic position) with an early to mid-systolic rumble and a fixed splitting of the second heart sound (s2) during inspiration and expiration. An echocardiogram is shown here.
Noncyanotic Foramen ovale fails to close. Ostium Secundum is most common Physical Exam: Wide fixed split second heart sound (S2). Systolic ejection murmur at the second left intercostals space with an early to mid-systolic rumble. Failure to thrive. Diagnosis: Best diagnosed by passing a catheter through defect Treatment:
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| Coarctation of aorta | Patient will present as → an 8-year-old boy who is seen for the first time in your office. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mmHg. You notice that his lower extremities are slightly atrophic with a mottling appearance. Upon further examination, he is found to have very weak and delayed femoral pulses with a blood pressure of 96/60 in the lower extremities. He has a late systolic ejection murmur on cardiac auscultation. CXR is performed demonstrating a “figure of 3 sign“ and angiography is seen here.
Noncyanotic - Typically found just after the vessels are given off to the left arm. This is a cause of high blood pressure, as the kidneys do not "see" as high of a blood pressure as they would like.
Ejection murmur is heard at the aortic area and left sternal border that radiates into the left axilla and left back A bicuspid valve is seen in 50% of the cases •- also increase incidence of cerebral berry aneurysm Diagnosis:
 The figure 3 sign is seen in aortic coarctation and is formed by prestenotic dilatation of the aortic arch and left subclavian artery, indentation at the coarctation site (also known as the "tuck"), and poststenotic dilatation of the descending aorta. TX:
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| Patent ductus arteriosus | Patient will present as → a 2-week-old infant with a history of prematurity presents with a pink torso and upper extremity and blue lower extremities. On cardiac auscultation, you notice a rough, continuous “machinery murmur” heard over the left sternal border at the 2nd intercostal space (pulmonary area).
Noncyanotic - Ductus arteriosus is a normal fetal structure => If it remains open it's called a patent ductus arteriosus
Rough “Machinery” murmur late in systole at the time of S2 making it loud
Diagnose: Echocardiogram Treatment: Indomethacin has been used to help close a PDA |
| Ventricular septal defect | Patient will present as → a 4-year-old boy who is brought to your office by his parents because he gets tired very easily and cannot keep up with the other children. On exam, you hear a loud, harsh, holosystolic murmur at the left lower sternal border without radiation to the axillae.
Noncyanotic - VSD is the most common pathologic murmur in childhood
Loud, harsh, holosystolic murmur, left to right - heard best at the lower left sternal border
DX: Echocardiogram Treatment: Most close by age 6, surgery if large |
Cyanotic defects
| Tetralogy of Fallot | Patient will present as → a 2-week-old newborn brought to the ER by his mom who reports a sudden loss of consciousness during feeding and with crying. She also has noticed that the infants lips have turned blue on three occasions during feeding. blood pressure is 75/45 mmHg, a pulse is 170/min, and respirations are 44/min. A grade 3/6 harsh systolic ejection murmur is heard at the left upper sternal border. A CXR shows a small boot shaped heart and decreased pulmonary vascular markings.
Cyanotic Presentation: Difficult feeding, failure to thrive. "tet spells", baby with cyanosis and loss of consciousness with crying Four features: PROVe
Physical exam: Crescendo-decrescendo, holosystolic at LSB radiating to the back Chest radiograph: Boot shaped heart |
