The Hypoplastic Left Heart Syndrome

Noonan, Jacqueline A.; Nadas, Alexander S.

doi:10.1016/s0031-3955(16)30727-1

Cited by 390 publications

(146 citation statements)

References 30 publications

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“…A growing body of evidence shows that there is a major genetic contribution underlying nonsyndromic HLHS (33,36). Although the designation of HLHS as a "syndrome" is thought to be a misnomer from a human genetics perspective, extracardiac anomalies are frequently encountered, including CNS and renal abnormalities (5,35,37). The shared genetic basis of these findings suggests the possibility that HLHS is a true heart-brain syndrome.…”

Section: Microcephaly and Outcomes In Hlhsmentioning

confidence: 99%

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

et al. 2013

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Background: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. Methods: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. results: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). Allcause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. conclusion: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.

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Section: Microcephaly and Outcomes In Hlhsmentioning

confidence: 99%

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

et al. 2013

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“…The term HLHS was introduced by Noonan and Nadas in 1958 to describe the morphological characteristics of combined mitral and aortic atresia 1 . However, the concept of HLHS may be extended to cases in which the mitral and aortic valves are present but where the underdevelopment of the left ventricle makes it unable to maintain the systemic circulation 2 .…”

Section: Introductionmentioning

confidence: 99%

Síndrome da hipoplasia do coração esquerdo: a influência da estratégia cirúrgica nos resultados

Silva¹,

Fonseca²,

Baumgratz³

et al. 2007

Arq. Bras. Cardiol.

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SummaryObjectives: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch, and to compare the results of the modified Blalock- Taussig Results: In-hospital survival for the entire cohort was 74.64%, or 67.57% and 82.35% for the mBT shunt and RV-PA conduit groups, respectively (p=0.1808). Mortality rates between the first and second palliation stages were 40% and 4.4% for the mBT shunt and RV-PA conduit groups, respectively (p=0.0054). Hypothermic circulatory arrest times were 45.79±1.99 min and 36.62±1.62min (p=0.0012), respectively. Late coarctation of the aorta occurred in five patients (7.2%).Conclusions: This surgical strategy resulted in short circulatory arrest time, low mortality and favorable morphology of the neoaorta, with low incidence of late coarctation of the aorta. The higher rate of survival to first palliation stage with the RV-PA conduit was not significant, but interstage mortality was statistically lower when compared with the modified Blalock-Taussig shunt procedure.

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“…Hypoplastic left heart syndrome (HLHS) is defined by the presence at birth of underdeveloped left-sided cardiac structures such as a small left ventricle, a small mitral valve, or a small aortic valve (Noonan & Nadas 1958) (Figure 1). Within this syndrome, the anomalies of the left-sided cardiac structures have a varying spectrum of severity.…”

Section: Definition and Epidemiologymentioning

confidence: 99%

“…Until the early 1980s, families were offered comfort care as the only therapeutic option. The first surgery for HLHS was attempted in 1961 but the first report of survivors was not until the 1970s (Ohye et al 2016;Noonan & Nadas 1958).…”

Section: History Of Surgical Repair In Hlhsmentioning

confidence: 99%

Risk Factors for Death or Heart Transplant for Patients with Right Ventricular Dysfunction After the Norwood Procedure: A Secondary Analysis of the Single Ventricle Reconstruction Trial

Jean‐St‐Michel

Meza

Coles

et al. 2017

The Journal of Heart and Lung Transplantation

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We investigated patient and procedural factors associated with failure of staged palliation after the Norwood procedure in patients with right ventricular dysfunction (RVDfx).Patients from the Pediatric Heart Network Single Ventricle Reconstruction (SVR) Trial dataset were selected if RVDfx was present, defined as an ejection fraction of less than 44% and a fractional area of change of less than 35% on the post-Norwood echocardiogram. Transplantfree survival after the Norwood procedure was analyzed using multiphase parametric hazard analysis and factors associated with death or transplant were identified.In the SVR cohort, 123 (34%) infants had RVDfx. The six-month transplant-free survival was 87% (70%CI 82-91%). The independent factors associated with increased risk of death or transplant were BT shunt, increased RV size, infectious disease complications and low surgeon volume.Patient factors can help identify which patient with RVDfx is at higher risk of death and help decision making around early transplant listing.iii

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The Hypoplastic Left Heart Syndrome

Cited by 390 publications

References 30 publications

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome

Síndrome da hipoplasia do coração esquerdo: a influência da estratégia cirúrgica nos resultados

Risk Factors for Death or Heart Transplant for Patients with Right Ventricular Dysfunction After the Norwood Procedure: A Secondary Analysis of the Single Ventricle Reconstruction Trial

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