The Use of Reconstructive Surgery to Improve Quality of Life and Survival in Prenatal Hypoplastic Left Heart Syndrome

Abstract

Outcomes for hypoplastic left heart syndrome have improved substantially in the era of prenatal diagnosis. Current stage 1 survival rates are in excess of 90% in the absence of risk factors. However, fetuses with an intact atrial septum continue to have poor postnatal survival. Accurate diagnosis of these fetuses with an intact atrial septum relies upon careful assessment of the patent foramen ovale, pulmonary venous Doppler flow patterns and branch pulmonary artery Doppler flow patterns. Prenatal and perinatal interventions, such as the placement of an atrial stent in utero or delivery at a center where a stent may be placed immediately after birth to relieve left atrial hypertension, may improve survival in this particularly high-risk group.

“Functionally” Univentricular Hearts: Impact of Pre-Natal Diagnosis

“…In the specific cases of pre-natal diagnosis of hypoplastic left heart, the presence of restrictive inter-atrial communication, and the type and direction of the flow through the inter-atrial communication itself ( 3 , 5 , 8 , 9 , 15 ), as well as the responsiveness of the fetal pulmonary vasculature to maternal hyperoxygenation ( 5 ), are indispensable for a prognostic evaluation and for the organization of the immediate post-natal treatment. Also the pre-natal evidence of endocardial fibroelastosis has clear negative prognostic implications ( 20 , 24 ).…”

“…Survival is of course the most important parameter when evaluating the impact of the pre-natal diagnosis on the outcomes, and this issue has been addressed by many studies ( 3 , 6 , 9 , 23 , 25 – 32 ).…”

“…When dealing with the most complex congenital heart defects, such as “functionally” univentricular hearts, because of the presence of multi-factorial elements influencing the outcomes, the discrepancy on the opinions about the impact of pre-natal diagnosis on survival becomes even more evident ( 3 , 6 , 9 , 23 , 27 , 29 – 32 ).…”

“…In the more recent period, with substantially improved the peri-operative care, a dichotomy clearly divided the reports demonstrating improved survival with pre-natal diagnosis ( 3 , 9 , 30 – 32 ) from the studies showing similar survival between cases with pre-natal and post-natal diagnosis ( 6 , 23 , 26 , 27 , 29 ).…”

“…General agreement exists on the observations that prompt referral after pre-natal diagnosis allows reducing the pre-operative metabolic acidosis and the subsequent need for bicarbonate administration, minimizing the needs for pre-operative inotropic support, reducing the incidence and degree of ventricular dysfunction and right atrio-ventricular valve regurgitation, and almost abolishing the occurrence of end-organs dysfunction ( 3 , 6 , 26 , 27 , 29 – 32 ). The pre-natal diagnosis allows also early intervention in the case of identification of restrictive inter-atrial communication ( 9 ).…”