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Variability in the Preoperative Management of Infants with Hypoplastic Left Heart Syndrome

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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.

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Acknowledgment

We thank Dr. Gil Wernovsky for his assistance in the distribution and collection of the surveys.

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Correspondence to B. A. Johnson.

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. 1.

    Infants with HLHS are electively intubated upon initiation of prostaglandins

    1      2      3      4      5

  1. 2.

    How frequently are the following maneuvers utilized to balance circulation?

    1. a

      Subambient FiO2 using inspired nitrogen

      1      2      3      4      5

    2. b

      Hypercarbia using inspired carbon dioxide

      1      2      3      4      5

    3. c

      Hypoventilation with intubation and sedation

      1      2      3      4      5

    4. d

      Use of muscle relaxation

      1      2      3      4      5

    5. e

      Other (please specify)

  1. 3.

    Preoperative use of digoxin

    1      2      3      4      5

  2. 4.

    Preoperative use of routine Lasix

    1      2      3      4      5

  3. 5.

    Preoperative use of anticoagulation

    1      2      3      4      5

  4. 6.

    Preoperative initiation of enteral feeding

    1. a

      PO    1      2      3      4      5

    2. b

      NG    1      2      3      4      5

  1. 7.

    Parameters followed preoperatively to direct clinical decision making (please circle all that apply)

    1. a

      Arterial saturation?

    2. b

      Mixed venous saturation

    3. c

      Cerebral oxygen saturation using infrared spectroscopy

    4. d

      Urine output

    5. e

      Metabolic acidosis

    6. f

      Other (please specify)

  1. 8.

    Usual dose of PGE1 used to maintain ductal patency

    1. a

      <0.03

    2. b

      0.03–0.05

    3. c

      >0.05

  1. 9.

    Average length of time from birth to initial surgical procedure

    1. a

      <3 days

    2. b

      3–7 days

    3. c

      >7 days

  1. 10.

    Most of the HLHS patients cared for at your institution are:

    1. a

      Inborn

    2. b

      Outborn

    3. c

      Equal number of inborn and outborn

  1. 11.

    The proportion of HLHS patients who are diagnosed prenatally is:

    1. a

      Nearly none

    2. b

      ∼25%

    3. c

      ∼50%

    4. d

      ∼75%

    5. e

      Nearly all

  1. 12.

    The most common operative procedure performed at your institution for HLHS is:

    1. a

      Staged palliation

    2. b

      Transplant

  1. 13.

    Number of neonates with HLHS cared for at your institution each year

    1. a

      < 10

    2. b

      10–20

    3. c

      21–30

    4. d

      >30

  1. 14.

    Nonintervention is offered as an option to families with infants diagnosed with HLHS at your institution

    1. a

      True

    2. b

      False

  1. 15.

    Please specify the zip code of the institution at which you work:

  2. 16.

    Please specify your occupation:

    1. a

      Cardiologist

    2. b

      Surgeon

    3. c

      Intensivist

    4. d

      Neonatologist

    5. e

      Pediatrician

    6. f

      Nurse

    7. g

      Perfusionist

Other (please specify)

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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

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  • DOI: https://doi.org/10.1007/s00246-007-9022-1

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