Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia

an Algorithmic Approach

Kaitlin Balduf, T. K.Susheel Kumar, Umar Boston, Shyam Sathanandam, Marc V. Lee, Timothy Jancelewicz, Christopher J. Knott-Craig

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

Original languageEnglish (US)
Pages (from-to)191-196
Number of pages6
JournalSeminars in Thoracic and Cardiovascular Surgery
Volume30
Issue number2
DOIs
StatePublished - Jun 1 2018

Fingerprint

Hypoplastic Left Heart Syndrome
Newborn Infant
Congenital Diaphragmatic Hernias
Norwood Procedures
Fontan Procedure
Lung
Mitral Valve Stenosis
Aortic Valve Stenosis
Diaphragm

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia : an Algorithmic Approach. / Balduf, Kaitlin; Kumar, T. K.Susheel; Boston, Umar; Sathanandam, Shyam; Lee, Marc V.; Jancelewicz, Timothy; Knott-Craig, Christopher J.

In: Seminars in Thoracic and Cardiovascular Surgery, Vol. 30, No. 2, 01.06.2018, p. 191-196.

Research output: Contribution to journalArticle

@article{b84e528d0ca94a0ebbb7a7d4d51f6686,
title = "Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach",
abstract = "Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1{\%}-5{\%}. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.",
author = "Kaitlin Balduf and Kumar, {T. K.Susheel} and Umar Boston and Shyam Sathanandam and Lee, {Marc V.} and Timothy Jancelewicz and Knott-Craig, {Christopher J.}",
year = "2018",
month = "6",
day = "1",
doi = "10.1053/j.semtcvs.2018.02.010",
language = "English (US)",
volume = "30",
pages = "191--196",
journal = "Seminars in Thoracic and Cardiovascular Surgery",
issn = "1043-0679",
publisher = "W.B. Saunders Ltd",
number = "2",

}

TY - JOUR

T1 - Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia

T2 - an Algorithmic Approach

AU - Balduf, Kaitlin

AU - Kumar, T. K.Susheel

AU - Boston, Umar

AU - Sathanandam, Shyam

AU - Lee, Marc V.

AU - Jancelewicz, Timothy

AU - Knott-Craig, Christopher J.

PY - 2018/6/1

Y1 - 2018/6/1

N2 - Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

AB - Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

UR - http://www.scopus.com/inward/record.url?scp=85044121265&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85044121265&partnerID=8YFLogxK

U2 - 10.1053/j.semtcvs.2018.02.010

DO - 10.1053/j.semtcvs.2018.02.010

M3 - Article

VL - 30

SP - 191

EP - 196

JO - Seminars in Thoracic and Cardiovascular Surgery

JF - Seminars in Thoracic and Cardiovascular Surgery

SN - 1043-0679

IS - 2

ER -