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Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection

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Abstract

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57–336; Group A) compared to 14 days (range 11–31; Group B); p < 0.05. Median age at IMO was 87 days (range 8–345) [Group A: 99 days (range 68–345) vs Group B: 25 days (range 8–39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169–218) for Group A compared to 118 days (range 97–161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.

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Abbreviations

ASA:

Acetylosalicylic acid

APVR:

Anomalous pulmonary venous return

AVSD:

Atrioventricular septal defect

CHD:

Congenital heart disease

ECMO:

Extracorcopreal membrane oxygenation

GI:

Gastrointestinal

HS:

Heterotaxy syndrome

ICU:

Intensive care unit

IM:

Intestinal malrotation

IMO:

Operative intervention for intestinal malrotation

S1P:

Stage 1 palliation

SCPC:

Superior cavopulmonary connection

SPS:

Systemic-to-pulmonary shunt

SV:

Single ventricle

UGISBFT:

Upper GI with small bowel follow-through

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The author(s) received no financial support for the research, authorship, and/or publication of this article.

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Authors and Affiliations

  1. Division of Pediatric and Congenital Cardiothoracic Surgery, Children’s Hospital of Georgia, 1120 15th Street BAA 8300, Augusta, GA, 30912, USA

    Lauren Mathis, Brendan Shafer & Anastasios C. Polimenakos

  2. Department of Surgery, Medical College of Georgia, Augusta, GA, USA

    Danielle Crethers & Anastasios C. Polimenakos

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  1. Lauren Mathis
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  2. Brendan Shafer
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  4. Anastasios C. Polimenakos
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Correspondence to Anastasios C. Polimenakos.

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The study was approved by the Institutional Review Board. Need for parental consent was waived.

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Mathis, L., Shafer, B., Crethers, D. et al. Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection. Pediatr Cardiol 40, 1224–1230 (2019). https://doi.org/10.1007/s00246-019-02136-w

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