Hypoplastic left heart syndrome: Duration of survival without surgical intervention

Hoshino, Kenji; Ogawa, Kiyoshi; Hashizume, Takumi; Kitazawa, Reiko; Uehara, Ritei

doi:10.1016/s0002-8703(99)70503-x

Cited by 28 publications

(12 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Prior to the establishment of these procedures, survival was low; in HLHS patients, survival to 1 y of age was virtually 0% (4). More recently, the Single Ventricle Reconstruction Trial found that overall 3-y transplant-free survival in HLHS patients was 64%.…”

mentioning

confidence: 99%

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

et al. 2017

View full text Add to dashboard Cite

Background:We compared brain cortical and subcortical gray matter volumes and cortical thickness between postFontan patients and healthy controls, and examined brain anatomical associations with operative and medical history characteristics. Methods: Post-Fontan (n = 128 volumes; n = 115 thickness) and control subjects (n = 48 volumes; n = 45 thickness) underwent brain MRI at ages 10-19 y. Subcortical and cortical volumes and cortical thicknesses were measured for intergroup comparison. Associations between brain measures and clinical measures were assessed in the Fontan group. results: Widespread, significant reduction in brain volumes and thicknesses existed in the Fontan group compared to controls, spanning all brain lobes and subcortical gray matter. Fontan subjects treated with vs. without the Norwood procedure had smaller volumes in several terminal clusters, but did not differ in cortical thickness. Older age at first operation and increasing numbers of cardiac catheterizations, operative complications, and catheterization complications were associated with lower regional volumes and thicknesses. Increasing numbers of operative complications and cardiac catheterizations were associated with smaller regional volumes in the Norwood group. conclusion: The post-Fontan adolescent brain differs from the normal control brain. Some of these differences are associated with potentially modifiable clinical variables, suggesting that interventions might improve long-term neurocognitive outcome.c ongenital heart disease (CHD) is the most common congenital anomaly, with single ventricle (SV) defects occurring in nearly 10% of patients with CHD (1). Patients with SV, particularly hypoplastic left heart syndrome (HLHS), have higher mortality and morbidity than those with other types of CHD, but their survival has steadily increased for several decades (2). The Fontan procedure, as well as staged palliation

show abstract

mentioning

confidence: 99%

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

et al. 2017

View full text Add to dashboard Cite

show abstract

“…The three major determinants of the hemodynamic disturbances are the gradual decrease in pulmonary vascular resistance, the spontaneous constriction of the ductus arteriosus and the inadequacy of the interatrial connection (31). Coarctation of the aorta may impede retrograde blood flow to a diminutive ascending aorta and, thus, decrease the adequacy of coronary blood flow (32,33). As these changes occur, systemic and coronary perfusion are decreased, leading to tissue hypoxia, metabolic acidosis and, eventually, vascular shock and death (31,32).…”

Section: Diagnosismentioning

confidence: 99%

“…Most infants die within the first two weeks of life, with an average age at death of 4.5 days (31,33,34). Some patients with HLHS, however, can survive beyond sixty days, without any surgical intervention through the development of pulmonary hypertension (8,33).…”

Section: Diagnosismentioning

confidence: 99%

“…Therefore, immediate therapy of infants with HLHS is aimed at maintaining the patency of the ductus arteriosus with an intravenous prostaglandin E 1 infusion (32). Diuretic infusion, inotropic support, sodium bicarbonate infusion, mechanical ventilation and sedation may be necessary to treat the respiratory and cardiac insufficiency complicated by metabolic acidemia (33). Higher arterial oxygen pressure is paradoxically an unfavourable sign, indicating pulmonary overcirculation with progressive congestive failure and consequent systemic undercirculation and poor peripheral perfusion (31,35).…”

Section: Diagnosismentioning

confidence: 99%

“…Less commonly, there may be a persistance of high pulmonary arteriolar resistance that does not diminish in the postnatal period or when there is marked restriction at the interatrial septum (31). There is a significant correlation of survival with smaller interatrial communications; however, severe intractable hypoxemia also limits survival (33). An interatrial flow jet width 3 mm or less, measured by colour flow Doppler, is thought to be associated with a greater risk of death (36).…”

Section: Diagnosismentioning

confidence: 99%

See 2 more Smart Citations