Case history
Case history #1
A 6-year-old girl is brought to her pediatrician for routine well-child care. She is doing well and has never experienced chest pain, palpitations, or syncope. She actively participates in a dance class and reports being able to keep up with her peers. There is no family history of congenital heart defects. She is well-appearing with no apparent distress. Her left ventricular impulse is normal, and there is a subtle right ventricular lift. Her second heart sound is widely split and does not vary with respiration. She has a soft 1-2/6 systolic ejection murmur best heard along the left upper sternal border. The remainder of her physical exam is normal.
Case history #2
A 45-year-old woman seeks treatment for frequent palpitations. The patient did well throughout the first 2 decades of life. In her mid-20s she noticed that she became slightly short of breath with exertion. She has recently been seen twice in the emergency department for atrial tachyarrhythmias. Her left ventricular impulse is normal. Her right ventricular impulse is 2+. She has a 2/6 systolic ejection murmur at her left upper sternal border. The murmur radiates to her back. The remainder of her physical exam is normal.
Other presentations
The clinical presentation depends on the age of the patient and the size of the left-to-right shunt. Children can be completely asymptomatic. Occasionally, patients with interatrial communications have symptoms of heart failure and poor growth in infancy, particularly those born prematurely. This presentation is more common if the patient has an additional source of pulmonary blood flow, such as a patent ductus arteriosus, or in association with partial anomalous pulmonary venous drainage.
Adult patients with an untreated interatrial communication can commonly present with heart failure, atrial arrhythmias, right ventricular failure, paradoxic embolization, cerebral abscess, or pulmonary vascular obstructive disease. Adults typically do not develop heart failure until their fifth decade of life. Patients with sinus venosus-type defects are more prone to developing heart failure. The incidence of atrial arrhythmias increases with age, especially between the fifth and seventh decades of life.[8]
Pulmonary vascular obstructive disease develops in 5% to 10% of patients and is more common in women.[9] The clinical manifestations of pulmonary hypertension are often nonspecific, most commonly including dyspnoea; syncope is uncommon. Examination may include a loud split second heart sound and tricuspid regurgitation murmur.[10] Some patients develop irreversible right-to-left shunting (Eisenmenger physiology). Most women with interatrial communications usually tolerate pregnancy well, including those with a sizable left-to-right shunt. However, patients who have developed pulmonary vascular obstructive disease do not tolerate pregnancy well. Idiopathic pulmonary arterial hypertension may coexist with atrial septal defect. Pulmonary vein stenosis should be ruled out in the setting of associated pulmonary hypertension.
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