Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: Survival analysis after stage i palliation

Shyam K. Sathanandam, Anastasios Charalanpos Polimenakos, David A. Roberson, Chawki F. Elzein, Andrew Van Bergen, Tarek S. Husayni, Michel N. Ilbawi

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. Methods: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. Results: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). Conclusions: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.

Original languageEnglish (US)
Pages (from-to)1599-1608
Number of pages10
JournalAnnals of Thoracic Surgery
Volume90
Issue number5
DOIs
StatePublished - Jan 1 2010
Externally publishedYes

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Hypoplastic Left Heart Syndrome
Mitral Valve Stenosis
Survival Analysis
Survival
Aortic Valve Stenosis
Mortality
Aortic Valve
Mitral Valve

ASJC Scopus subject areas

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

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Mitral stenosis and aortic atresia in hypoplastic left heart syndrome : Survival analysis after stage i palliation. / Sathanandam, Shyam K.; Polimenakos, Anastasios Charalanpos; Roberson, David A.; Elzein, Chawki F.; Van Bergen, Andrew; Husayni, Tarek S.; Ilbawi, Michel N.

In: Annals of Thoracic Surgery, Vol. 90, No. 5, 01.01.2010, p. 1599-1608.

Research output: Contribution to journalArticle

Sathanandam, Shyam K. ; Polimenakos, Anastasios Charalanpos ; Roberson, David A. ; Elzein, Chawki F. ; Van Bergen, Andrew ; Husayni, Tarek S. ; Ilbawi, Michel N. / Mitral stenosis and aortic atresia in hypoplastic left heart syndrome : Survival analysis after stage i palliation. In: Annals of Thoracic Surgery. 2010 ; Vol. 90, No. 5. pp. 1599-1608.
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abstract = "Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. Methods: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. Results: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90{\%} for the entire cohort and 88.9{\%} for the MS-AA subtype (not significantly different). Six-month survival was 70{\%} overall and 70.4{\%} for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93{\%} survival (versus 50{\%} for MS-AA without VCC; p < 0.01). Conclusions: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.",
author = "Sathanandam, {Shyam K.} and Polimenakos, {Anastasios Charalanpos} and Roberson, {David A.} and Elzein, {Chawki F.} and {Van Bergen}, Andrew and Husayni, {Tarek S.} and Ilbawi, {Michel N.}",
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T1 - Mitral stenosis and aortic atresia in hypoplastic left heart syndrome

T2 - Survival analysis after stage i palliation

AU - Sathanandam, Shyam K.

AU - Polimenakos, Anastasios Charalanpos

AU - Roberson, David A.

AU - Elzein, Chawki F.

AU - Van Bergen, Andrew

AU - Husayni, Tarek S.

AU - Ilbawi, Michel N.

PY - 2010/1/1

Y1 - 2010/1/1

N2 - Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. Methods: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. Results: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). Conclusions: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.

AB - Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. Methods: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. Results: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). Conclusions: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.

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