Noncardiac surgery in children with hypoplastic left heart syndrome

Torres, Adalberto; DiLiberti, John H.; Pearl, Richard H.; Wohrley, Julie; Raff, Gary W.; Bysani, G. Kris; Bond, Linda M; Geiss, Dale M.

doi:10.1053/jpsu.2002.35377

Cited by 53 publications

(43 citation statements)

References 11 publications

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“…Although long-term outcomes have improved during 2 decades, various co-morbidities remain. CHD adds a significant risk of mortality in children requiring noncardiac surgery [5,6].…”

Section: Introductionmentioning

confidence: 99%

Anesthetic management of noncardiac surgery for patients with single ventricle physiology

2011

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Patients with congenital heart diseases are a growing population, and noncardiac surgeries will become an important health care issue. Patients with single ventricle physiology are a particularly challenging population who will undergo staged, palliative repair toward a final step of Fontan circulation. Although Fontan surgery creates a serial circulation in which the ventricle pumps blood to the systemic circuit, pulmonary blood flow occurs without a dedicated ventricle. Despite progress in outcomes, this abnormal circulation remains associated with various co-morbidities such as ventricular dysfunction, arrhythmias, protein losing enteropathy, and plastic bronchitis. Health care professionals must prepare for these patients to present to noncardiac surgery at any stage of intervention, possibly with complications. Given that staged, palliative repair has undergone multiple modifications, patients who present for surgery can vary in types and timing of the repair. Anesthesiologists who care for them must be familiar with perioperative issues to optimize outcomes, especially because congenital heart disease is a risk factor for increased mortality for noncardiac surgery.

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“…Although long-term outcomes have improved during 2 decades, various co-morbidities remain. CHD adds a significant risk of mortality in children requiring noncardiac surgery [5,6].…”

Section: Introductionmentioning

confidence: 99%

Anesthetic management of noncardiac surgery for patients with single ventricle physiology

2011

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“…HLHS accounts for 4–9% of congenital heart disease in children, and it is the largest specific cause of cardiovascular infant mortality caused by birth defects [3]. HLHS was nearly uniformly fatal before 1980.…”

Section: Discussionmentioning

confidence: 99%

“…HLHS was nearly uniformly fatal before 1980. In fact, HLHS was responsible at one time for 25% of all cardiac deaths in the early days of life and has recently been reported to have 5-year survival rates of 60–75% for patients undergoing staged cardiac operations [1, 3, 7]. After Fontan and Baudet [8] published their preliminary report in 1971 about the effective separation of the systemic circulation from the pulmonary venous circulation, mortality rates decreased [1, 2].…”

Section: Discussionmentioning

confidence: 99%

“…After Fontan and Baudet [8] published their preliminary report in 1971 about the effective separation of the systemic circulation from the pulmonary venous circulation, mortality rates decreased [1, 2]. Torres et al [3] reported the survival rate in HLHS to increase from 36% for patients operated on during the 1989 through 1992 interval to 75% during the 1993 through 1995 interval due to intraoperative cardiac anesthetic management advances, dedicated postoperative pediatric intensive care unit facilities, and technical expertise.…”

Section: Discussionmentioning

confidence: 99%

“…The mortality rate among the children with HLHS after cardiac repair is well documented, but comparable data after noncardiac surgical procedures is not well known. With a decrease in the cardiac mortality, an increasing number of noncardiac procedures are being performed on children with HLHS, and these procedures are associated with high rates of morbidity and mortality [3]. Medically intractable epilepsy is one of those problems, which arises as a complication of cardiovascular surgery in HLHS and necessitates neurosurgical intervention [4, 5].…”

Section: Introductionmentioning

confidence: 99%

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Implications of a Neurosurgical Intervention in a Patient with a Surgically Repaired Hypoplastic Left Heart Syndrome

2007

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Hypoplastic left heart syndrome (HLHS) accounts for 4–9% of congenital heart disease in children. The mortality rate among children with HLHS undergoing cardiac repair is well documented, but comparable data for noncardiac surgical procedures are not well known. Medically intractable epilepsy is one of those problems, which arises as a complication of cardiovascular surgery in HLHS and necessitates neurosurgical intervention. There is no published knowledge about neurosurgical procedures performed in children with HLHS in the English literature. Thus, we present a 10-year-old boy who developed medically intractable epilepsy after cardiac surgery for HLHS. The aim of this study is to outline the pre-, intra- and postoperative precautions needed for neurosurgical intervention in HLHS patients to decrease morbidity and mortality.

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