Lessons from a case with stiff left atrial syndrome complicated by iatrogenic inter-atrial shunts: a case report

In patients with stiff left atrial (LA) syndrome, reservoir function is significantly impaired due to extensive LA fibrosis; consequently, the increase in LA pressure during haemodynamic stress is prominent, easily leading to pulmonary venous hypertension and subsequent pulmonary congestion, and eventually results in intractable heart failure.

A 79-year-old female with mitral stenosis and atrial fibrillation underwent valve replacement, Cox-Maze IV procedure, LA plication, and appendage ligation 4 years prior to presentation. Thereafter, she underwent a total of two catheter ablation procedures for recurrent atrial tachycardia. Transthoracic echocardiography revealed two continuous colour jets across the interatrial septum, with a peak pressure gradient of 23 mmHg, which was consistent with the residual puncture hole at the catheter ablation procedures. Although transoesophageal echocardiography showed no evidence of prosthetic valve dysfunction, the pulmonary venous flow signal showed a significantly blunted systolic forward flow, extremely small retrograde reversal flow during atrial contraction, and prominent diastolic flow velocities, all of which indicated significantly impaired LA function. Cardiac catheter examination revealed a characteristic pulmonary capillary wedge pressure waveform, which consisted of a steep ascending limb of v wave with a large peak, consistent with stiff LA syndrome.

Treatment of patients with stiff LA syndrome is quite challenging and restricted to the use of diuretics only, which has limited efficacy and eventually results in intractable heart failure. In this case, owing to the inter-atrial pressure-relieving gateway, the patient was only mildly symptomatic despite the existence of a non-compliant LA.