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.
