bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒05‒26
two papers selected by
Richard James
University of Pennsylvania


  1. J Am Heart Assoc. 2019 May 21. 8(10): e010783
    Gardner MM, Mercer-Rosa L, Faerber J, DiLorenzo MP, Bates KE, Stagg A, Natarajan SS, Szwast A, Fuller S, Mascio CE, Fleck D, Torowicz DL, Giglia TM, Rome JJ, Ravishankar C.
      Background In shunt-dependent, single-ventricle patients, mortality remains high in the interstage period between discharge after neonatal surgery and stage 2 operation. We sought to evaluate the impact of our infant single-ventricle management and monitoring program ( ISVMP ) on interstage mortality and stage 2 outcomes. Methods and Results This retrospective single-center cohort study compared patients enrolled in ISVMP at hospital discharge with historical controls. The relationship of ISVMP to interstage mortality was determined with a bivariate probit model for the joint modeling of both groups, using an instrumental variables approach. We included 166 ISVMP participants (December 1, 2010, to June 30, 2015) and 168 controls (January 1, 2007, to November 30, 2010). The groups did not differ by anatomy, gender, race, or genetic syndrome. Mortality was lower in the ISVMP group (5.4%) versus controls (13%). An ISVMP infant compared with a historical control had an average 29% lower predicted probability of interstage death (adjusted probability: -0.29; 95% CI , -0.52 to -0.057; P=0.015). On stratified analysis, mortality was lower in the hypoplastic left heart syndrome subgroup undergoing Norwood operation (4/84 [4.8%] versus 12/90 [14%], P=0.03) but not in those with initial palliation of shunt only ( P=0.90). ISVMP participants were younger at the time of the stage 2 operation (138 versus 160 days, P<0.001), with no difference in postoperative mortality or length of stay. Conclusions In this single-center study, we report significantly lower interstage mortality for participants with hypoplastic left heart syndrome enrolled in ISVMP . Younger age at stage 2 operation was not associated with postoperative mortality or longer length of stay.
    Keywords:  hypoplastic left heart syndrome; interstage mortality; interstage period; single‐ventricle congenital heart disease
    DOI:  https://doi.org/10.1161/JAHA.118.010783
  2. Congenit Heart Dis. 2019 May 21.
    Monteiro SA, Serrano F, Tsang R, Smith Hollier E, Guffey D, Noll L, Voigt RG, Ghanayem N, Shekerdemian L.
      OBJECTIVE: Neurodevelopmental impairment is common after surgery for congenital heart disease (CHD) in infancy. While neurodevelopmental follow-up of high-risk patients has increased, the referral patterns for ancillary services following initial evaluation have not been reported. The aim of this study is to describe the rates and patterns of referral at the initial visit to our outcomes clinic of patients who underwent surgery for CHD during infancy.OUTCOMES MEASURES: The Cardiac Developmental Outcomes Program clinic at Texas Children's Hospital provides routine longitudinal follow-up with developmental pediatricians and child psychologists for children who required surgery for CHD within the first 3 months of life. Demographic, diagnostic, and clinical data, including prior receipt of intervention and referral patterns at initial presentation, were abstracted from our database.
    RESULTS: Between April 2013 and May 2017, 244 infants under 12 months of age presented for initial evaluation at a mean age of 7 ± 1.3 months. At presentation, 31% (76/244) were referred for either therapeutic intervention (early intervention or private therapies), ancillary medical services, or both. Referral rates for low-risk (STAT 1-3) and high-risk (STAT 4-5) infants were similar (28 vs. 33%, P = .48). Referrals were more common in: Hispanic white infants (P = .012), infants with non-cardiac congenital anomalies (P = .001), history of gastrostomy tube placement (P < .001), and infants with prior therapy (P = .043). Infants of non-English speaking parents were three times more likely to be referred (95% CI = 1.5, 6.4; P = .002).
    CONCLUSIONS: At the time of presentation, nearly 1 in 3 infants required referral. Referral patterns did not vary by traditional risk stratification. Sociodemographic factors and co-morbid medical conditions increased the likelihood of referral. This supports the need for routine follow-up for all post-surgical infants regardless of level of surgical complexity. Further research into the completion of referrals and long-term referral patterns is needed.
    Keywords:  CHD; ancillary services; early intervention; neurodevelopmental outcome; referral
    DOI:  https://doi.org/10.1111/chd.12789