Section VII: Congenital Heart Diseases

The decision to close an atrial septal defect (ASD) is based upon the size, location, degree of shunting and RV function.

HPI: May be asymptomatic. Dyspnea, exercise intolerance, palpitations.

PMH: Arrhythmias, pulmonary hypertension, stoke, thromboembolism, 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, orthodeoxia-platypnea.

ASYM = ASYMptomatic ASD (small shunt and normal RV size).

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 2–3 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 physiology. Mortality is not improved with closure at this point. Prognosis is poor (pulmonary artery pressure >2/3 systemic pressure, pulmonary vascular resistance >2/3 systemic vascular resistance).

MED-TX = Medical management. There is no medicine to correct the anatomical defect. Can give medicines to alleviate right heart failure and pulmonary hypertension.

PE = Paradoxical Embolism.

OP = Orthodeoxia Platypnea.

PAP < 2/3 SBP = Pulmonary artery pressure less than two thirds systemic levels.

PVR < 2/3 SVR = Pulmonary vascular resistance less than two thirds systemic vascular resistance.

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

PE = Closure

OP = Closure

PVR < 2/3 SVR = Closure

PAP< 2/3 SVR = Closure

Most patients with ASD are asymptomatic for decades and have a relatively normal physical examination 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 ...