Case presentation

A 53-year-old woman was referred for evaluation for lung transplant due to severe pulmonary hypertension in the setting of an atrial septal defect. She noted exertional dyspnea and peripheral edema of 3 years duration, with frequent nonsustained palpitations.

Physical examination revealed mild tachycardia and a resting oxygen saturation of 92% on room air. Jugular veins were distended while sitting, and there was an increased precordial activity with a right ventricular heave. A harsh systolic ejection murmur was present along the left sternal border, but diastole was clear. The abdominal exam was notable for a liver distended 3 centimeters below the right costal margin. Her lower extremities had edema to the ankles but intact peripheral pulses.

A 12-lead surface electrocardiogram demonstrated sinus rhythm with right axis deviation and a narrow RSR’ in V1, consistent with right ventricular enlargement. Transthoracic echocardiography demonstrated a markedly dilated right ventricle (Figure 52-1 and Video 52-1) and a tricuspid regurgitant Doppler signal consistent with severe pulmonary hypertension (Figure 52-2). Subcostal imaging demonstrated an atrial septal defect with significant left-to-right shunt across the atrial septum (Figure 52-3 and Video 52-2). There was normal left ventricular size and systolic function. She was referred for percutaneous closure of the atrial septal defect.

FIGURE 52-1 Parasternal long-axis view showing enlargement of the right ventricle.

FIGURE 52-2 Doppler evaluation found significant tricuspid regurgitation with increased velocities consistent with severe pulmonary hypertension.

FIGURE 52-3 Subcostal image showing flow across the atrial septal defect.

Cardiac catheterization

The procedure was performed using conscious sedation. Vascular access initially consisted of a 6 French sheath in the right femoral artery and both 8 and 11 French sheaths in the right femoral vein. The patient received 4000 U of unfractionated heparin intravenously along with 1 g of cefazolin. A 10.5 French intracardiac echocardiography catheter was then inserted through the 11 French sheath and advanced to the right atrium, allowing imaging and sizing of the atrial septal defect as well as confirming normal pulmonary venous return to the left atrium (Figure 52-4