Outcomes of hypoplastic left heart syndrome: analysis of National Inpatient Sample Database 1998–2004 versus 2005–2014

Hamzah, Mohammed; Othman, Hasan F.; Baloğlu, Orkun; Aly, Hany

doi:10.1007/s00431-019-03508-3

Cited by 13 publications

(14 citation statements)

References 33 publications

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“…The incidence of HLHS during the fetal stage could be higher. Currently, surgical palliative techniques and improved postoperative care have significantly improved survival, with ~60-70% HLHS neonates surviving for at least 5 years following repair [4][5][6]. Mortality however is highest in the first year of life, with 30% dying or requiring heart transplant before one year old.…”

Section: Introductionmentioning

confidence: 99%

Novel Protein-Protein Interactions Highlighting the Crosstalk between Hypoplastic Left-Heart Syndrome, Ciliopathies and Neurodevelopmental Delays

Karunakaran¹,

Gabriel²,

Balakrishnan³

et al. 2022

Preprint

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Hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease (CHD) affecting 1 in 5,000 newborns. We constructed the interactome of 74 HLHS-associated genes identified from a large-scale mouse mutagenesis screen, augmenting it with 408 novel protein-protein interactions (PPIs) using our High-precision Protein-Protein Interaction Prediction (HiPPIP) model. The interactome is available on a webserver with advanced search capabilities (http://severus.dbmi.pitt.edu/wiki-HLHS). 364 genes including 73 novel interactors were differentially regulated in tissues/iPSC-derived cardiomyocytes of HLHS patients. Novel PPIs facilitated the identification of TOR signaling and endoplasmic reticulum stress modules. 60.5% of the interactome consisted of housekeeping genes that may harbor large-effect mutations and drive HLHS etiology but show limited transmission. Network proximity of diabetes, Alzheimer’s disease, and liver carcinoma-associated genes to HLHS genes suggested a mechanistic basis for their comorbidity with HLHS. Interactome genes showed tissue-specificity for sites of extracardiac anomalies (placenta, liver and brain). The HLHS interactome shared significant overlaps with the interactomes of ciliopathy and microcephaly-associated genes, with the shared genes respectively enriched for genes involved in intellectual disability and/or developmental delay, and neuronal death pathways. This supported the increased burden of ciliopathy variants and prevalence of neurological abnormalities observed among HLHS patients with developmental delay and microcephaly respectively.

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Section: Introductionmentioning

confidence: 99%

Novel Protein-Protein Interactions Highlighting the Crosstalk between Hypoplastic Left-Heart Syndrome, Ciliopathies and Neurodevelopmental Delays

Karunakaran¹,

Gabriel²,

Balakrishnan³

et al. 2022

Preprint

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“…In addition, the incidence of complications of total anomalous pulmonary venous connection (TAPVC) in patients with hypoplastic left heart syndrome is low (2.3%), and the prognosis is poor. The prognosis may even be poorer if the condition is further complicated by pulmonary venous stenosis 2,3 …”

Section: Introductionmentioning

confidence: 99%

Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection

et al. 2022

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Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. This case suggests that compared to non‐pulsatile continuous pulmonary venous flow, the nutmeg lung pattern can only be observed with severe pulmonary congestion and advanced pulmonary lymphangiectasia.

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“…Outcomes for neonates with hypoplastic left-heart syndrome (HLHS) have vastly improved over the past 3 decades [1][2][3][4][5][6]. However, for the approximately 20% [7] of HLHS patients with restrictive atrial septum (RAS), morbidity and mortality remain high [8,9].…”

Section: Introductionmentioning

confidence: 99%

Maternal Hyperoxygenation Testing in Fetuses with Hypoplastic Left-Heart Syndrome: Association with Postnatal Atrial Septal Restriction

et al. 2021

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Introduction: In fetuses with hypoplastic left-heart syndrome (HLHS), maternal hyperoxygenation (MHO) may aid risk stratification. We hypothesized that pulmonary vein (Pvein) velocity time integral (VTI) change with MHO would more reliably identify neonates who undergo emergent atrial septoplasty (EAS) than changes in pulmonary arterial pulsatility index (PA PI). Methods: Fetuses with HLHS who underwent MHO testing at our institution between 2014 and 2019 were identified. Data were reviewed in a blinded, retrospective manner. Pvein VTI ratio (prograde:retrograde) was calculated. The primary outcome was neonatal EAS. Results: Twenty-seven HLHS fetuses underwent MHO, and 5 (19%) underwent EAS. Without MHO, a Pvein VTI ratio <3 conferred 60% sensitivity and 100% specificity for EAS. With MHO, a Pvein VTI ratio <6.5 conferred 100% sensitivity and specificity. For an intermediate group of fetuses with a baseline Pvein VTI ratio 3–7, the ratio decrease with MHO conferred 100% sensitivity and specificity. Compared to the Pvein VTI ratio, PA PI was less accurate in identifying EAS neonates. Discussion/Conclusion: Addition of MHO appears to improve the diagnostic ability of the Pvein VTI ratio to identify HLHS fetuses who undergo EAS. The Pvein VTI ratio change may more accurately identify fetuses who undergo EAS than change in PA PI and has less interobserver variability.

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Outcomes of hypoplastic left heart syndrome: analysis of National Inpatient Sample Database 1998–2004 versus 2005–2014

Cited by 13 publications

References 33 publications

Novel Protein-Protein Interactions Highlighting the Crosstalk between Hypoplastic Left-Heart Syndrome, Ciliopathies and Neurodevelopmental Delays

Novel Protein-Protein Interactions Highlighting the Crosstalk between Hypoplastic Left-Heart Syndrome, Ciliopathies and Neurodevelopmental Delays

Fetal case of rare association of hypoplastic left heart syndrome and absent atrial septum accompanied by mixed form of supracardiac total anomalous pulmonary venous connection

Maternal Hyperoxygenation Testing in Fetuses with Hypoplastic Left-Heart Syndrome: Association with Postnatal Atrial Septal Restriction

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