Abstract
Around 3-4 children born with congenital heart diseases have
univentricular hearts, where the prognosis of univentricular hearts is
poor in the past, with a survival rate of less than 50% during the
first year and 10% during the first ten years of life. Based on a
literature search, current advances in perioperative management of
neonates with complex congenital heart diseases have increased their
survival rate by 85%. To aid cardiothoracic surgeons worldwide, this
comprehensive literature review will focus on the perioperative
management of staged palliation surgery for functional univentricular
hearts, considering current trends as well as how we do it in our
centre. Our review article specifially discusses perioperative
strategies regarding surgical considerations, current techniques, to
deal with overshunting and undershunting during the first stage of
palliation surgery. This article also gives an overview on when a
patient is suitable to go through with the next stage of the procedure,
which is the implementation of a bidirectional cavopulmonary shunt or
the Hemi-Fontan procedure. Lastly, this article gives a comprehensive
approach regarding perioperative strategies of the Fontan procedure,
which include patient criteria, current surgical techniques,
postoperative management, as well as the use of anticoagulants after the
Fontan procedure.
Keywords: fontan procedure, perioperative management, staged
palliation, univentricular hearts
Congenital heart diseases (CHD) are the most frequent congenital defect
found in neonates, accounting for nearly one-third of major congenital
anomalies.1 CHD that cannot be surgically
reconstructed to achieve normal anatomic conditions of biventricular
circulation are often described as functional univentricular hearts
(UVH). It is reported that the incidence of functional UVH is 3 to 4 per
100 children born with CHD.2 The natural history of
functional UVH is poor in the past, with a reported survival rate of
less than 50% at one year and 10% at ten years. Advances in
perioperative management for neonates with complex CHD have increased
survival rates by more than 85%.3, 4
The functional univentricular heart can result from a variety of
anatomic lesions. Regardless of the etiology, newborns with these
lesions mix systemic and pulmonary venous blood. Perioperative
management is becoming as important in patients with UVHs as
preoperative patient management focuses on the balance of competing
circulations, where systemic and pulmonary circulations exist in
parallel rather than in series, as in normal circulation. Thus, most
patients will require surgical intervention to provide unobstructed
systemic blood flow (Qs) and restrictive pulmonary blood flow
(Qp).5 Intraoperative management focuses on selecting
ideal candidates for each staged palliation surgical procedure such as
pulmonary artery size and pulmonary artery resistance index, while
postoperative patient management focuses on optimizing systemic output,
respiratory status, and mitigating the effects of cardiopulmonary
bypass.5