Interim mortality in pulmonary atresia with intact ventricular septum

Kathleen N. Fenton, Frank A. Pigula, Sanjiv K. Gandhi, Linda Russo, Kim F. Duncan

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum. At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair). The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1%), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15%) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation. These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15%), and is somewhat higher (24%) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated.

Original languageEnglish (US)
Pages (from-to)1994-1998
Number of pages5
JournalAnnals of Thoracic Surgery
Volume78
Issue number6
DOIs
StatePublished - Dec 1 2004

Fingerprint

Hypoplastic Left Heart Syndrome
Mortality
Heart Ventricles
Pulmonary Atresia
Infant Mortality
Pulmonary Atresia with Intact Ventricular Septum
Cardiology
Pulmonary Artery
Databases
Transplants
Weights and Measures
Right ventricle hypoplasia
Incidence
Population
Therapeutics

Keywords

  • 21

ASJC Scopus subject areas

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

Cite this

Interim mortality in pulmonary atresia with intact ventricular septum. / Fenton, Kathleen N.; Pigula, Frank A.; Gandhi, Sanjiv K.; Russo, Linda; Duncan, Kim F.

In: Annals of Thoracic Surgery, Vol. 78, No. 6, 01.12.2004, p. 1994-1998.

Research output: Contribution to journalArticle

Fenton, Kathleen N. ; Pigula, Frank A. ; Gandhi, Sanjiv K. ; Russo, Linda ; Duncan, Kim F. / Interim mortality in pulmonary atresia with intact ventricular septum. In: Annals of Thoracic Surgery. 2004 ; Vol. 78, No. 6. pp. 1994-1998.
@article{1d31757543f64ec0a05429b54ddc648a,
title = "Interim mortality in pulmonary atresia with intact ventricular septum",
abstract = "Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum. At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair). The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1{\%}), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15{\%}) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation. These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15{\%}), and is somewhat higher (24{\%}) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated.",
keywords = "21",
author = "Fenton, {Kathleen N.} and Pigula, {Frank A.} and Gandhi, {Sanjiv K.} and Linda Russo and Duncan, {Kim F.}",
year = "2004",
month = "12",
day = "1",
doi = "10.1016/j.athoracsur.2004.06.015",
language = "English (US)",
volume = "78",
pages = "1994--1998",
journal = "Annals of Thoracic Surgery",
issn = "0003-4975",
publisher = "Elsevier USA",
number = "6",

}

TY - JOUR

T1 - Interim mortality in pulmonary atresia with intact ventricular septum

AU - Fenton, Kathleen N.

AU - Pigula, Frank A.

AU - Gandhi, Sanjiv K.

AU - Russo, Linda

AU - Duncan, Kim F.

PY - 2004/12/1

Y1 - 2004/12/1

N2 - Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum. At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair). The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1%), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15%) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation. These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15%), and is somewhat higher (24%) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated.

AB - Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum. At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair). The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1%), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15%) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation. These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15%), and is somewhat higher (24%) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated.

KW - 21

UR - http://www.scopus.com/inward/record.url?scp=9344222750&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=9344222750&partnerID=8YFLogxK

U2 - 10.1016/j.athoracsur.2004.06.015

DO - 10.1016/j.athoracsur.2004.06.015

M3 - Article

C2 - 15561017

AN - SCOPUS:9344222750

VL - 78

SP - 1994

EP - 1998

JO - Annals of Thoracic Surgery

JF - Annals of Thoracic Surgery

SN - 0003-4975

IS - 6

ER -