bims-hylehe Biomed news on
Hypoplastic Left Heart Syndrome
Issue of 2018‒03‒18
three papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr Surg. 2018 Feb 07. doi: 10.1016/j.jpedsurg.2018.02.014
    Abstract:  PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation. RESULTS: Of 1811 neonates with CHD, 3.4% (n=61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n=33/653), compared to 2.4% (n=28/1158) in the non-DD group (p=0.003). RACHS-1 score>2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1≤2 cases, 1.7% (20/1153) (p=0.005). DD patients and complex patients with RACHS-1>2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n=16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups. CONCLUSION: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score>2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients. LEVEL OF EVIDENCE: Level IIb.
    Keywords:  Congenital heart disease; Ductal dependent; Hypoplastic left heart syndrome; NEC; Necrotizing enterocolitis
  2. Heart Lung Circ. 2017 Oct 31. doi: 10.1016/j.hlc.2017.10.008
    Abstract:  BACKGROUND: The atrial switch operation for transposition of the Great Arteries (TGA) (Mustard or Senning Procedure) provides excellent short-term survival. Significant long-term concerns exist for these patients, however, including the ability of the right ventricle to maintain systemic perfusion and the risk of arrhythmia. We seek to describe long-term mortality and morbidity of this group of adult patients. METHODS: Consecutive patients who had undergone an atrial switch procedure, who were aged over 16 years and who were followed up at our tertiary level adult congenital heart disease (ACHD) service in Sydney, Australia since 2000 were included. We documented mortality using a National Death Index and analysed the prospectively defined composite endpoint of "Serious Adverse Events" including death, heart failure hospitalisation and/or documented ventricular arrhythmia. RESULTS: There were 83 patients included; mean age at most recent follow-up was 35 ± 5 years. Overall survival was 82% at 35 years and 22% of patients experienced a serious adverse event. Atrial and ventricular arrhythmias occurred in 45% and 7% of patients respectively. Eighteen (22%) patients required a pacemaker and six (7%) required implantable cardiacdefibrillator (ICD) implantation. Significant right ventricular dysfunction was present in 26% of patients on their most recent visit and this, or requirement for permanent pacing, was associated with an increased risk of serious adverse events (OR 10.22, p<0.001), (OR 4.998, p=0.04) respectively. CONCLUSIONS: Significant mortality and morbidity accrues by mid-adult life after an atrial procedure for TGA. Right ventricular dysfunction and permanent pacing are associated with serious adverse events.
    Keywords:  Atrial switch; Congenital heart disease; Transposition of the great arteries
  3. Int J Cardiol. 2018 Mar 02. doi: 10.1016/j.ijcard.2018.02.118
    Abstract:  BACKGROUND: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD). METHODS AND RESULTS: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. RDW was measured in fresh venous blood samples at inclusion in 592 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at four annual follow-up visits. During 4.3 [IQR 3.8-4.7] years of follow-up, the primary endpoint (death, heart failure, hospitalization, arrhythmia, thromboembolic events, cardiac intervention) occurred in 196 patients (33%). Median RDW was 13.4 (12.8-14.1)% versus 12.9 (12.5-13.4)% in patients with and without the primary endpoint (P < 0.001). RDW was significantly associated with the endpoint when adjusted for age, sex, clinical risk factors, CRP, and NT-proBNP (HR 1.20; 95% CI 1.06-1.35; P = 0.003). The C-index of the model including RDW was slightly, but significantly (P = 0.005) higher than the model without (0.74, 95% CI 0.70-0.78 versus 0.73, 95% CI 0.69-0.78). Analysis of repeated RDW measurements (n = 2449) did not show an increase in RDW prior to the occurrence of the endpoint. CONCLUSIONS: RDW is associated with cardiovascular events in patients with ACHD, independently of age, sex, clinical risk factors, CRP, and NT-proBNP. This readily available biomarker could therefore be considered as an additive biomarker for risk stratification in these patients.
    Keywords:  Adult congenital heart disease; Biomarker; Prognosis; Red cell distribution width; Risk