Hybrid stenting through direct ventricular access for severe stenosis of the pulmonary venous baffle after atrial switch operation for transposition of the great arteries: a case report

Background: Atrial switch repair was the first surgical intervention to result in long-term survival in patients with ventriculo-arterial discordance or transposition of the great arteries. However, the natural history after atrial switch is not uneventful with frequent atrial arrhythmia, development of baffle stenosis, and eventually heart failure. For this, new interventions might be necessary but are often associated with increased risk. Case summary: We present the case of a 49-year-old woman born with ventriculo-arterial discordance or dextro-transposition of the great arteries who underwent atrial switch repair according to Mustard at the age of 1 year. She presented with shortness of breath and reduced exercise capacity. The echocardiography revealed prominent turbulent flow at the level of the pulmonary venous baffle (PVB). This was confirmed on cardiac computed tomography. After multidisciplinary discussion, a hybrid approach was considered as the preferred strategy. In this, the cardiac surgeon provided apical access by left lateral thoracotomy. The PVB was accessed retrograde through right ventricular apical access, and stenting with a covered stent with subsequent balloon dilatation up to 13 mm was performed. This reduced the peak gradient on echocardiography from 18 to 11 mmHg. Clinical follow-up was uneventful with improved functional capacity 6 months after discharge. Discussion: This case provides an alternative access to the PVB by left lateral mini-thoracotomy and apical ventricular access. Furthermore, we highlight the challenges in decision-making and the importance of the multidisciplinary collaboration between adult congenital cardiologist, the echocardiographer, and cardiac surgeon as well as the flexibility in interventional techniques to individualize the management of such cases.