Section VII: Congenital Heart Diseases

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AMA Citation
Congenital Heart Diseases. In: Higgins JP, Ali A, Filsoof DM. Higgins J.P., Ali A, Filsoof D.M. Eds. John P. Higgins, et al.eds. Cardiology Clinical Questions New York, NY: McGraw-Hill; . http://accesscardiology.mhmedical.com/content.aspx?bookid=1819&sectionid=126678398. Accessed September 18, 2018.

MLA Citation
. "Congenital Heart Diseases." Cardiology Clinical Questions Higgins JP, Ali A, Filsoof DM. Higgins J.P., Ali A, Filsoof D.M. Eds. John P. Higgins, et al. New York, NY: McGraw-Hill, , http://accesscardiology.mhmedical.com/content.aspx?bookid=1819&sectionid=126678398.

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Does a patient with an atrial septal defect require closure?

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	The decision to close an atrial septal defect (ASD) is based upon the size and degree of shunting.
	HPI: May be asymptomatic. Dyspnea, exercise intolerance, palpitations. PMH: Arrhythmias, pulmonary hypertension, congestive heart failure (CHF) FH: Family History of congenital heart disease
	May have a normal examination. Prominent right ventricular (RV) impulse, wide fixed splitting of S2, midsystolic pulmonary ejection murmur.
	Prolonged PR interval. Secundum ASD = Right axis deviation + right atrial enlargement + incomplete right bundle branch block (IRBBB). Primum ASD = Left axis deviation + IRBBB.
	Chest X-ray: RV and RA enlargement; prominent pulmonary artery; increased pulmonary vascularity. Echocardiogram: Direct visualization of ASD or shunting (especially with agitated saline study), dilation of the right atrium/ventricle, elevated pulmonary pressure, Qp/Qs (pulmonary to systemic ratio) to quantitate shunt.
	SYM = SYMptomatic ASD = Dyspnea on exertion, fatigue, palpitations, evidence of atrial arrhythmias. ASYM = ASYMptomatic ASD SIG-SHUNT = Significant left to right shunt (usually caught on echo), Qp:Qs > 1.5. STR-CHANGE = Evidence of STRuctural CHANGEs in the heart such as elevated pulmonary pressure, right atrial or right ventricular enlargement or hypertrophy. PxE = History of a paradoxical embolic event. CLOSURE = CLOSURE of ASD. Can be performed percutaneously or surgically depending on size and location of ASD. FOLLOW = Clinical follow up and echocardiogram every 2 years or sooner if symptoms develop. END-STAGE = The clinical picture from an uncorrected significant ASD over time. May have severe irreversible pulmonary hypertension or Eisenmenger's physiology. Mortality is not improved with closure at this point. Prognosis is poor. MED-TX = Medical management. There is no medicine to correct the anatomical defect. Can give medicines to alleviate right heart failure and pulmonary hypertension.
	SYM + (NO END-STAGE) = CLOSURE ASYM + SIG-SHUNT = CLOSURE ASYM + STR-CHANGE = CLOSURE ASYM + PxE = CLOSURE ASYM + (NO SIG-SHUNT) + (NO STR-CHANGE) = FOLLOW END-STAGE = MED-TX
	Most patients with ASD are asymptomatic for decades and have a relatively normal physical exam making ASD the most common adult uncorrected congenital anomaly. The natural history for an ASD with a significant shunt is to develop pulmonary hypertension and right heart remodeling from pulmonary vascular overload. The mortality once severe pulmonary hypertension has developed is high. Closure of ASD has a very low incidence of morbidity and mortality from the procedure itself and can be done percutaneously or surgically. The goal is to close significant ASDs early before complications develop. Very small ASDs without significant shunting rarely become clinically significant.
	– Closure is not recommended in patients with severe irreversible pulmonary artery hypertension.
	– Most patients with ASD will be asymptomatic for decades. – Mortality is increased with significant shunts due to pulmonary vascular overload, and can be improved with closure even when patients are older or asymptomatic. – Once severe irreversible pulmonary hypertension has ...