Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection

Bahaaldin Alsoufi, Cedric Manlhiot, Mamdouh Al-Ahmadi, Zohair Al-Halees, Brian W. McCrindle, Ahmed Yehia Mousa, Yasser Al-Heraish, Avedis Kalloghlian

Research output: Contribution to journalArticle

Abstract

Objectives: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Methods: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Results: Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Conclusions: Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

Original languageEnglish (US)
JournalJournal of Thoracic and Cardiovascular Surgery
Volume142
Issue number1
DOIs
StatePublished - Jan 1 2011
Externally publishedYes

Fingerprint

Norwood Procedures
Mortality
Hypoplastic Left Heart Syndrome
Ventricular Function
Lung
Fontan Procedure
Survival
Heart Transplantation
Vascular Diseases
Vasodilation
Vascular Resistance
Lung Diseases
Ventilation
Nitric Oxide
Referral and Consultation
Pathology
Oxygen
Weights and Measures

ASJC Scopus subject areas

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

Cite this

Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection. / Alsoufi, Bahaaldin; Manlhiot, Cedric; Al-Ahmadi, Mamdouh; Al-Halees, Zohair; McCrindle, Brian W.; Mousa, Ahmed Yehia; Al-Heraish, Yasser; Kalloghlian, Avedis.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 142, No. 1, 01.01.2011.

Research output: Contribution to journalArticle

Alsoufi, Bahaaldin ; Manlhiot, Cedric ; Al-Ahmadi, Mamdouh ; Al-Halees, Zohair ; McCrindle, Brian W. ; Mousa, Ahmed Yehia ; Al-Heraish, Yasser ; Kalloghlian, Avedis. / Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection. In: Journal of Thoracic and Cardiovascular Surgery. 2011 ; Vol. 142, No. 1.
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abstract = "Objectives: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Methods: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Results: Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53{\%} had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10{\%} had impaired ventricular function, 11{\%} had moderate atrioventricular valve regurgitation, and 32{\%} had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39{\%} had died, and 57{\%} underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15{\%} had died, and 85{\%} underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53{\%}. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Conclusions: Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.",
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AU - Alsoufi, Bahaaldin

AU - Manlhiot, Cedric

AU - Al-Ahmadi, Mamdouh

AU - Al-Halees, Zohair

AU - McCrindle, Brian W.

AU - Mousa, Ahmed Yehia

AU - Al-Heraish, Yasser

AU - Kalloghlian, Avedis

PY - 2011/1/1

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N2 - Objectives: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Methods: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Results: Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Conclusions: Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

AB - Objectives: Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Methods: Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Results: Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Conclusions: Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

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