Back to Fetal Physiology - An Option for the Palliation of Severe Postcapillary Pulmonary Hypertension in Shone´s Complex?

Background: The prognosis of severe pulmonary hypertension in congenital heart disease is poor. Alternative therapeutic strategies, which enable a decompression of the right heart may offer bridging of these patients until the performance of a heart-lung transplantation.

Introduction: We present a 15-month-old patient with Shone's complex who had initially undergone neonatal repair for aortic coarctation in another institution. Due to multiple life-threatening pulmonary hypertensive (PH) crises re-surgery was performed at the age of nine months by resection of left ventricular outflow tract obstruction and aortic valve reconstruction. Most recently he was still suffering from suprasystemic postcapillary pulmonary hypertension with recurrent PH crises and right heart failure ensuing in cardiopulmonary resuscitation. The patient was referred to our institution for the evaluation of cardiac transplantation. A Ross-Konno procedure was not amenable due to a severe discrepancy between the aortic and pulmonary valves due to severe pulmonary hypertension and compromised left ventricular function. We performed a palliative procedure with bilateral pulmonary artery banding (bPAB), atrioseptectomy and “re-ducting” by placing an 8 mm PTFE-prosthesis between the main pulmonary artery and the distal aortic arch. The “reverse” shunt allowed decompression of the right ventricle whereas atrioseptectomy and bPAB avoided volume overload of the pulmonary circulation. The surgery was well tolerated. The patient is doing well and free of PH crises ten months postoperatively.

Discussion: The palliative strategy using a “reverse” aortopulmonary shunt in severe pulmonary hypertension had already been described. The presented approach aimed to imitate cardiac physiology as in a “fetal parallel circulation” and might serve as a temporary palliation with bridge to cardiac transplantation in selected patients.