PANCE Blueprint Cardiology (13%)

Congenital Heart Disease (PEARLS)

The NCCPA™ PANCE Cardiology System Content Blueprint covers 5 different 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

  1. ulmonary Stenosis
  2. R ight ventricular hypertrophy
  3. verriding aorta
  4. V entricular septal defect
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 heard sound (s2) during inspiration and expiration. An echocardiogram is shown here.

Atrial Septal Defect

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:

  • Symptomatic: Diuretics, ACE inhibitors, digoxin
  • Definitive: Surgical closure
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.

Coarctation of the Aorta

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.

  • This leads them to release substances to raise the pressure/renin
  • The person is in their teens, twenties need to r/o in a young adult with HTN
  • The key finding is elevated blood pressure in the arms, with low blood pressure in the legs.
  • Pulses in the leg may be decreased in intensity, or delayed compared with their occurrence in the arm.

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:

  • 1st Echocardiogram
  • EKG= LVH
  • CXR=rib notching “figure of 3 sign”
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.

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:

  • Prostaglandins E1
  • Generally, require surgical repair with dilating the segment with balloons
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).

Patent Ductus Arteriosus

Noncyanotic - Ductus arteriosus is a normal fetal structure

  • If it remains open it's called a patent ductus arteriosus.
  • 3-6-week infants can present with tachypnea, diaphoresis, inability or difficulty with feeding, and no weight gain.
  • low birth weight premature infant Adults with PDA may present with signs and symptoms of heart failure

Rough “Machinery” murmur late in systole at the time of S2 making it loud

  • Murmur is best heard at the pulmonic area 2nd ICS left sternal border and inferior to the clavicle bounding pulses and a widened pulse pressure
  • Because Prostaglandin E-2 is responsible for keeping the ductus patent - inhibitors of prostaglandin will close it
    • This is why NSAIDs are the treatment and are contraindicated in pregnancy

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.

Ventral Septal Defect

Noncyanotic - VSD is the most common pathologic murmur in childhood

  • In this case, the hole or "defect" is in the heart muscle is between the ventricles (the intraventricular "septum").
  • As in the case of the ASD, the heart can dilate, the muscle can become weak, and the pressures in the pulmonary arteries can increase (pulmonary hypertension) due to the increase in blood flow.

Loud, harsh, holosystolic murmur, left to right - heard best at the lower left sternal border

  • Like ASD’s, the size and therefore the clinical course of these defects is quite variable.
  • Some remain large, while others become smaller over time.
  • It is not unusual for small-to-medium sized VSD’s to eventually close spontaneously.

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.

Tetralogy of Fallot

Cyanotic

Presentation: Difficult feeding, failure to thrive. "tet spells", baby with cyanosis and loss of consciousness with crying

Four features: PROVe

  1. ulmonary Stenosis
  2. R ight ventricular hypertrophy
  3. verriding aorta
  4. V entricular septal defect

Physical exam: Crescendo-decrescendo, holosystolic at LSB radiating to the back

Chest radiograph: Boot shaped heart

Ventricular tachycardia (ReelDx + Lecture) (Prev Lesson)
(Next Lesson) Brian Wallace PA-C Podcast: Congenital Heart Disorders
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