Monday, January 18, 2010

January 15th - LBJ - ASD and Congenital Heart Disease

Atrial Septal Defects are a commonly tested question on boards, and are a very complicated topic.  However, here are the basics you'll need to know for boards. 

Even though it is a congenital heart defect, the various types of ASDs can be asymptomatic until adolescence or adulthood.  Therefore, physical exam features are clues into the presence of the defect before symptoms develop

In general, patients can present with symptoms attributable to atrial fibrillation, right-sided heart failure, and rarely Pulmonary HTN. 

There are 4 types of ASD, each with subtle features to know.  You'll most likely be given a patient with an ostium secundum defect, so know this one well.  A lot of the concepts are also relevant to the other types of ASD, so just remember what makes the others different (which I'll list)

Ostium Secundum - The most common Type of ASD, and the 2nd most common congenital abnormality in adults (the most common being a bicuspid aortic valve)

Exam: Fixed Split S2 and systolic murmur at LUSB. Loud P2 if patient get Pulmonary HTN
EKG: Right Axis Deviation and RBBB
CXR:  Enlarged Right Ventricle, prominent pulmonary vasculature
Diagnosis: Echocardiogram
Treatment: Surgical Closure of the ASD.  This is done in symptomatic patients, and in asymptomatic patients that a have pulmonary: system shunt ratio >2:1. If there is pulmonary HTN, surgical closure usually cannot be done, but percutaneous closure can be done. 
Complications:Patients can get TIA, Strokes, and Eisenmenger's Syndrome

Ostium Primum
Exam: Pansystolic Murmur of MR or TR
EKG: First Degree AV Block, Left Axis Deviation and RBBB
Treatment: Treatment of choice is percutaneous closure

Sinus Venosus - Know patients have anomalous pulmonary venous return, treat with surgery

Coronary Sinus - Know that patients usually have a Left SVC, treat with surgery

Okay, so what else do you need to know for boards regarding congenital heart disease? Here are the very basic minimums to know about most of the congenital diseases in adults that you will encounter on boards:

Diagnose with agitated saline and TTE
Evidence to suggest there is an association b/w PFO and migraines and cryptogenic stroke
Some advocate closure of a PFO in those with cryptogenic stroke, but this is controversial

Exam with holosystolic systolic murmur at LLSB, Prolonged Split of S2, as well as murmurs of AR/TR

Diagnose with Echo (suggestive) but need cath to tell difference in O2 Sat b/w Right Atrium and Ventricle

Patients with large VSDs, Significant Shunt, Progressive AR and Volume Overload undergo closure

If left untreated, patients will develop Eisenmenger Syndrome

More common in females, may have history of congenital Rubella infection.

Can present with symptoms of heart failure, and can get pulmonary HTN

Exam wth a Machine-like murmur at LUSB. May have bounding peripheral pulses

Interestingly, if Einsenmeger's occurs there is "differential cyanosis" - only in the lower extremities

Diagnose with Echo, Cath can reveal difference in O2 sat b/w Right Ventricle and Pulmonary Artery

Treatment is closure if the patient has symptoms (but not if the patient has with Pulmonary HTN!)

Coarction of Aorta:
Usually associated with Bicuspid Aortic Valve, and patients can get Mitral Valve disease, as well as LVH

Patients can present with HTN, symptoms of CHF and leg claudication

Exam with a delayed pulse between radial/brachial and femoral areas. Femoral Pulse can be absent. HTN can be present in the Upper Extremities compared to Lower Extremities. Patients usually also have a systolic murmur

CXR with Rib Notching (due to collateral vessels) and a "Figure 3 Sign" (dilated aorta above and below coarcted area)


Diagnosis - MRI can visualize best, cath usually done to measure degree and gradient

Treatment - Can do balloon with stenting or surgical repair. Usually done in younger patients, or those with an increased gradient

Outcome - patients have risk for aortic dissection and advanced HTN

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