Abstract
Infants with hypoplastic left heart syndrome (HLHS) commonly undergo initial surgical palliation during the first week of life. Few data exist on optimal preoperative management strategies; therefore, the management of these infants prior to surgery is anecdotal and variable. To more fully define this variability in preoperative care of infants with HLHS, a survey was designed to describe current preoperative management practices in the infant with HLHS. The questionnaire explored management styles as well as preoperative monitoring techniques and characteristics of the respondent’s health care institution. The responses were compiled and are reported. A striking lack of consistency in preoperative management techniques for infants with HLHS is apparent. The impact of these preoperative strategies is unknown. Despite challenges in anatomic and hemodynamic variability at presentation, a prospective randomized controlled trial comparing ventilatory management techniques, enteral feeding strategies, and the utility of various monitoring tools on short- and long-term outcome is needed.
Similar content being viewed by others
References
Azakie A, Merklinger S, McCrindle, et al. (2001) Evolving strategies and improving outcomes of the modified Norwood procedure: a 10-year single-institution experience. Ann Thorac Surg 72:1349–1353
Barnea O, Santamore P, Rossi A, et al. (1998) Estimation of oxygen delivery in newborns with a univentricular circulation. Circulation 98:1407–1413
Chang R, Chen A, Klitzner T (2002) Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988–1997. Pediatrics 110:292–298
Gutgesell H, Gibson J (2002) Management of hypoplastic left heart syndrome in the 1990s. Am J Cardiol 89:842–846
Mahle W, Clancy R, McGaurn S, Goin J, Clark B (2001) Impact of prenatal diagnosis on survival and early neurologic morbidity in neonates with hypoplastic left heart syndrome. Pediatrics 107:1277–1282
Mahle W, Spray T, Wernovsky G, Gaynor J, Clark B (2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 102(Suppl III):III-136–III-141
Ramamoorthy C, Tabbutt S, Kurth CD, et al. (2002) Effects of inspired hypoxic and hypercapnic gas mixtures on cerebral oxygen saturation in neonates with univentricular heart defects. Anesthesiology 96:283–288
Smith JB, Vernon-Levett P (1993) Care of infants with hypoplastic left heart syndrome. AACN Clin Issues Crit Care Nurs 4:329–339
Tabbutt S, Ramamoorthy C, Montenegro L, et al. (2001) Impact of inspired gas mixtures on preoperative infants with hypoplastic left heart syndrome during controlled ventilation. Circulation 104(12 Suppl 1):I159–I164
Tweddell J, Hoffman G, Mussatto K, et al. (2002) Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 106(Suppl 1):I-82–I-89
Acknowledgment
We thank Dr. Gil Wernovsky for his assistance in the distribution and collection of the surveys.
Author information
Authors and Affiliations
Corresponding author
Appendix 1
Appendix 1
Survey of Preoperative HLHS Management as Distributed
Please circle the most appropriate answer to the questions below regarding your usual preoperative management of uncomplicated HLHS
1 = always (routinely); 2 = frequently; 3 = sometimes; 4 = rarely; 5 = never
-
1.
Infants with HLHS are electively intubated upon initiation of prostaglandins
1 2 3 4 5
-
2.
How frequently are the following maneuvers utilized to balance circulation?
-
a
Subambient FiO2 using inspired nitrogen
1 2 3 4 5
-
b
Hypercarbia using inspired carbon dioxide
1 2 3 4 5
-
c
Hypoventilation with intubation and sedation
1 2 3 4 5
-
d
Use of muscle relaxation
1 2 3 4 5
-
e
Other (please specify)
-
a
-
3.
Preoperative use of digoxin
1 2 3 4 5
-
4.
Preoperative use of routine Lasix
1 2 3 4 5
-
5.
Preoperative use of anticoagulation
1 2 3 4 5
-
6.
Preoperative initiation of enteral feeding
-
a
PO 1 2 3 4 5
-
b
NG 1 2 3 4 5
-
a
-
7.
Parameters followed preoperatively to direct clinical decision making (please circle all that apply)
-
a
Arterial saturation?
-
b
Mixed venous saturation
-
c
Cerebral oxygen saturation using infrared spectroscopy
-
d
Urine output
-
e
Metabolic acidosis
-
f
Other (please specify)
-
a
-
8.
Usual dose of PGE1 used to maintain ductal patency
-
a
<0.03
-
b
0.03–0.05
-
c
>0.05
-
a
-
9.
Average length of time from birth to initial surgical procedure
-
a
<3 days
-
b
3–7 days
-
c
>7 days
-
a
-
10.
Most of the HLHS patients cared for at your institution are:
-
a
Inborn
-
b
Outborn
-
c
Equal number of inborn and outborn
-
a
-
11.
The proportion of HLHS patients who are diagnosed prenatally is:
-
a
Nearly none
-
b
∼25%
-
c
∼50%
-
d
∼75%
-
e
Nearly all
-
a
-
12.
The most common operative procedure performed at your institution for HLHS is:
-
a
Staged palliation
-
b
Transplant
-
a
-
13.
Number of neonates with HLHS cared for at your institution each year
-
a
< 10
-
b
10–20
-
c
21–30
-
d
>30
-
a
-
14.
Nonintervention is offered as an option to families with infants diagnosed with HLHS at your institution
-
a
True
-
b
False
-
a
-
15.
Please specify the zip code of the institution at which you work:
-
16.
Please specify your occupation:
-
a
Cardiologist
-
b
Surgeon
-
c
Intensivist
-
d
Neonatologist
-
e
Pediatrician
-
f
Nurse
-
g
Perfusionist
-
a
Other (please specify)
Rights and permissions
About this article
Cite this article
Johnson, B.A., Mussatto, K., Uhing, M.R. et al. Variability in the Preoperative Management of Infants with Hypoplastic Left Heart Syndrome. Pediatr Cardiol 29, 515–520 (2008). https://doi.org/10.1007/s00246-007-9022-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-007-9022-1