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Cyanotic Congenital Heart Disorders

• In most cases cyanotic disorders have to do with right to left shunting of blood.
• Deoxygenated blood returning from the body bypasses the lungs altogether and is recirculated without becoming oxygenated.

Tetralogy of Fallot

• Right ventricular outflow obstruction (think pulmonary stenosis)
• Right ventricular hypertrophy (the right ventricle has to work against the the outflow issues)
• Ventricular septal defect (A communication between the two ventricles)
• Overriding aorta (attaches to both the right and left ventricle)

I came in to see my physician assistant because of…

• Untreated children may have a tet spell consisting of extreme hypoxia, cyanosis, syncope and hypercapnia.
• Clubbing of fingers
• Easy fatigability

Physical Exam

Murmur is a crescendo-decrescendo holosystolic along the the left sternal border and radiating to the back.

Lab & Studies

• Chest x-ray
  • Historically significant
  • Classic boot shaped heart due to the RVH
• No interstitial lung markings
• Echocardiogram is used for diagnosis

Treatment

• Surgical correction

Pulmonary atresia

• Atresia is an opening that is either closed or absent
• Pulmonary atresia is a closed pulmonary valve
• Treatment is by surgical intervention

Hypoplastic Left Heart Syndrome

• Hypoplastic =  small and underdeveloped
• Hypoplastic left heart syndrome = small undeveloped left ventricle and aorta

Transposition of the Great Vessels

• A complete transposition of the pulmonary artery and the aorta.
• At birth prostaglandins are given to keep the ductus arteriosus patent.  This allows the two circulatory systems to mix.
• Surgical intervention is the only long term treatment.

Noncyanotic Congenital Disorders

Atrial Septal Defect

• An opening between the right and left atrium.  This most commonly occurs when the foramen ovale does not close or is to large.  This can create either a right to left shunt or more commonly left to right shunt of blood.

Physical Exam

• A systolic ejection murmur in the  2nd and 3rd intercostal spaces.
• There may also be an early to mid systolic rumble.

Labs & Studies

• ECG
  • Right ventricular hypertrophy
  • Right bundle branch block
• Chest X-ray
  • Enlarged right heart
  • Large pulmonary arteries
• Echo
  -Diagnostic
• Cardiac Cath
  • More detailed information

Treatment

• A small shunt may require no treatment
• Surgical closure of the defect is necessary for larger defects.

Patent Ductus Arteriosus (PDA)

• The ductus arteriosus is a blood vessel which connects the pulmonary artery to the aorta.  In utero this allows most of the blood to bypass the lungs.
• Patent = open or not obstructed.
• Usually the ductus arteriosus closes at birth

I came in to see my physician assistant today because of…

• Signs of left ventricular failure
• Pulmonary hypertension

Physical Exam

• Machine like murmur
• Thrill
• Wide pulse pressure

Labs & Studies

• ECG is normal
• Echo is normal
• MRI/CT/Cardiac cath is best for visualizing the shunt

Treatment

• NSAIDs help close a small shunt
  • They inhibit prostoglandin production and prostaglandins keep the shunt open.
  • IV indomethacin in first 10–14 days of life.
• Surgical correction

Coarctation of the Aorta

• Coarctation = a narrowing or constricting
• Narrowing of the aorta

I came in to see my physician assistant today because of…

• Failure to thrive
• Chest pain
• Lightheadedness
• Shortness of breath
• Heart failure in an infant
• HTN
• HTN leading to left heart failure

Physical Exam

• Delayed or weak femoral pulse
• Harsh systolic murmur heard in the back
• Hypertension in the upper extremities with hypotension in the lower extremities.

Labs & Studies

• ECG
  • Left ventricular hypertrophy
• Echo
  • Diagnostic
• Cath/CT/MRI may all be used for more information

Treatment

• Patients younger than 50 open surgical repair
• Patients older than 50 stenting is the often the best option

Study Tip

Be interested during your rotations.