bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒08‒26
thirteen papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2018 Aug 18.
    Taggart NW, Gordon BM, Morgan GJ, Goldstein BH.
      The complex nature of congenital heart disease (CHD) has hindered the establishment of management standards for peri-catheterization anticoagulation. We sought to describe anticoagulation practice variability among providers performing cardiac catheterization in children and adults with CHD. A web-based survey ( http://www.surveymonkey.com ) was distributed to pediatric and congenital interventional cardiologists. Respondents were queried on their training, practice setting, years in practice, and case volume. Clinical questions focused on general anticoagulation strategies and on five common clinical scenarios: two diagnostic (biventricular circulation, single ventricle physiology) and three interventional cardiac catheterizations (atrial septal defect closure, pulmonary artery stenting in Fontan circulation, stent placement for coarctation of aorta). Seventy-seven pediatric and congenital interventional cardiologists responded to the survey (81% in the United States). Twenty-six (36%) worked in a public medical institution; 57% worked in a free-standing children's hospital. Twenty-six percent had been in practice for < 5 years and 32% for > 15 years; 75% completed additional training in interventional congenital cardiology. The median number of cases performed was 200/year (IQR 110); median number of interventional cases was 100/year (IQR 100). Responses to general queries and specific clinical scenarios suggested significant variation in anticoagulation practices, including monitoring of anticoagulation during catheterization, protamine use, and outpatient anticoagulation after catheterization. Practices not only varied between providers but also between different clinical scenarios. Practice patterns did not correlate with provider experience or case volume. Management of anticoagulation in the congenital cardiac catheterization lab varies from operator to operator. Our study may provide some initial insight and context for discussion regarding anticoagulation in a field of increasingly heterogeneous interventional techniques and patient substrates. Future studies would be helpful to better define "best practices" for peri-procedural thromboprophylaxis in patients with congenital heart disease.
    Keywords:  Anticoagulation; Cardiac catheterization; Quality improvement; Survey
    DOI:  https://doi.org/10.1007/s00246-018-1960-2
  2. Pediatr Cardiol. 2018 Aug 18.
    Tsaur S, Gleason L, Kim Y.
      Quality indicators for adult congenital heart disease (ACHD) were recently published due to a lack of consensus regarding delivery of care to adults with congenital heart disease (CHD). The objective of this study was to examine adherence to quality indicators for the care of patients with tetralogy of Fallot. Adults with tetralogy of Fallot seen in outpatient cardiology clinics at a tertiary care facility between July 2014 and June 2015 were included, and electronic medical records for each visit were reviewed. Completion rates for eight proposed quality indicator metrics were recorded and results for ACHD and non-ACHD cardiologists were compared. A total of 96 eligible patients completed 179 cardiology visits (134 ACHD and 45 non-ACHD). The quality indicator completion rates were over 80% for 7 of the 8 indicators. Metric 5 (cardiac magnetic resonance imaging every five years) had the lowest completion rate at 38.7%. Compared to non-ACHD cardiologists, ACHD cardiologists had higher completion rates for QRS assessment (88.1% vs. 75.6%, p = 0.04), echocardiogram by CHD expert (97.8% vs. 80.0%, p < 0.001), and infective endocarditis counseling (95.9% vs. 77.4%, p = 0.001). In this single center study, there was a wide range of quality indicator completion rates for tetralogy of Fallot. Routine cardiac MRI by an expert in CHD was identified as an area for improvement. There were significant differences in quality indicator completion between ACHD and non-ACHD cardiologists.
    Keywords:  Adult congenital heart disease; Quality improvement; Quality indicators; Tetralogy of Fallot
    DOI:  https://doi.org/10.1007/s00246-018-1954-0
  3. Pediatr Cardiol. 2018 Aug 18.
    Patel A, Michelson K, Andrei AC, Pahl E, Gossett JG.
      Ethical issues in pediatric heart transplantation (Htx) include resource allocation, benefit, and burden assessment in high-risk recipients, and informed consent. Practice patterns and decision-making was investigated using an internet survey with 47-multiple choice items and vignette-based questions. Of 43 pediatric Htx cardiologists contacted, 28 (65%) responded. Respondents reported that an overall median 1-month survival of 73% (range 50-100%), 1-year survival of 70% (range 50-85%), 5-year survival of 50% (range 40-85%), and 10-year survival of 50% (range 25-85%) was adequate to offer Htx. Based on vignettes presented, 100% of those surveyed would offer Htx to a straightforward 12-year old with end-stage dilated cardiomyopathy and a 7-year old with hypoplastic left heart syndrome with protein losing enteropathy. Thirty percent of physicians would offer Htx to a patient status post a Fontan procedure with mutliple co-morbidities. Seventy-five percent of physicians would offer Htx despite proven non-adherence. Considerable variability exists in the practice patterns of pediatric heart transplant cardiologists with regards to decision-making while evaluating patients for listing. Disagreements among pediatric Htx cardiologists exist when there are concerns for non-adherence and associated multiple co-morbidities. Further work is needed to understand these variations and develop consensus for pediatric Htx organ allocation.
    Keywords:  Ethics; Heart transplantation; Pediatric
    DOI:  https://doi.org/10.1007/s00246-018-1965-x
  4. Pediatr Cardiol. 2018 Aug 18.
    Şişli E, Tuncer ON, Şenkaya S, Doğan E, Şahin H, Ayık MF, Atay Y.
      The study aimed to revisit the in-hospital predictors of shunt thrombosis (ST) in the foreground of the pulmonary artery size in patients who received modified Blalock-Taussig shunt (mBTS) as the first-stage palliation. Data from 80 patients who received mBTS as their initial palliative procedure between February 2012 and January 2017 was retrospectively collected. The median age and weight of the patients at the time of their mBTS procedure was 4 days (IQR 2-22 days) and 3.2 kg (IQR 2.8-3.7 kg), respectively. Of the 80 patients in the study, 11 (13.8%) developed ST. The diameter and corresponding z scores of the pulmonary arteries were significantly lower in patients with ST. The median shunt size/shunted pulmonary artery size (S/PA) ratio was considerably higher in patients with ST. In logistic regression analysis, pulmonary artery hypoplasia (PAH) [odds ratio (OR) = 13.7 (0.06-0.21), p < 0.001], S/PA ratio ≥ 0.9 [OR = 8.1 (0.03-0.53), p = 0.03], prematurity [OR = 9.5 (0.05-0.33), p = 0.003], and shunt size/weight (S/W) ratio ≥ 1.3 [OR = 6.4 (0.04-0.67), p = 0.012] were found to have a significant impact on ST. The best combination of sensitivity and specificity of the S/W (0.73 and 0.75) and the S/PA ratio (0.73 and 0.80) were achieved at the cut-off value of 1.3 and 0.9, respectively. The Youden index of S/PA was 0.52. While the area under the curve (AUC) of the S/W ratio was 0.686 ± 0.12 (p = 0.049), the AUC of the S/PA ratio was 0.791 ± 0.08 (p = 0.002). In conclusion, instead of weight, considering the size of the target pulmonary artery and thereby, the S/PA ratio would be more instructive in determining shunt size. There were a high number of patients in our study who showed PAH having received a shunt size based on their body weight. By contrast, our results showed that the S/PA ratio of ≥ 0.9 would be a good predictor of in-hospital ST.
    Keywords:  Blalock–Taussig shunt, modified; Hypoplasia; In-hospital mortality; Pulmonary artery
    DOI:  https://doi.org/10.1007/s00246-018-1958-9
  5. Int J Cardiol. 2018 Aug 14. pii: S0167-5273(18)34400-0. [Epub ahead of print]
    Varadarajan P, Pai RG.
      
    DOI:  https://doi.org/10.1016/j.ijcard.2018.08.041
  6. Int J Cardiovasc Imaging. 2018 Aug 18.
    Hu L, Sun A, Guo C, Ouyang R, Wang Q, Yao X, Zhong Y.
      To evaluate the use of the tissue tracking (TT) technique to measure myocardial strain left ventricular in post-Fontan children with preserved ejection fraction (pEF). Nineteen (male/female, 10/9) patients with univentricular hearts after completion of the Fontan circulation (post-Fontan group) and 19 age- and gender-matched healthy children (control group) were retrospectively enrolled. Cardiovascular magnetic resonance (CMR) imaging was conducted on a 1.5-T MRI scanner. Global and regional strains of the left ventricle in post-Fontan patients (EF > 55%) and controls were obtained using CMR-TT software. The Mann-Whitney U test was used to compare parameters between the two groups. Correlation between EF and strain was investigated using Pearson correlation coefficients. The Bland-Altman method was used to identify the inter- and intra-observer agreement in measurement of global strain. Global longitudinal strain was lower in post-Fontan patients than in healthy controls (- 18.87 ± 4.61 vs. -19.72 ± 1.58; P = 0.54), though the difference was not statistically significant. Global circumferential strain and global radial strain were significantly lower in post-Fontan patients than in healthy controls (- 14.55 ± 3.79 vs. -19.91 ± 1.97; P < 0.001; and 29.62 ± 8.41 vs. 36.85 ± 5.95; P = 0.01; respectively). The regional circumferential strain (RCS) decrease was marked in regional segments compare with post-Fontan patients and controls (basal, - 11.81 ± 2.98 vs. - 16.21 ± 2.72, P < 0.001; mid, - 15.05 ± 3.31 vs. - 20.17 ± 2.28, P = 0.005; apical, - 16.86 ± 3.09 vs. - 23.37 ± 2.62, P < 0.001). All circumferential and longitudinal parameters had an inter-observer ICC of ≥ 0.85, but this coefficient was lower for radial parameters. CMR-TT appears to be a feasible technique for identification of early myocardial dysfunction in post-Fontan with pEF.
    Keywords:  Cardiac magnetic resonance; Fontan; Pediatric; Strain
    DOI:  https://doi.org/10.1007/s10554-018-1440-z
  7. Pediatr Neonatol. 2018 Aug;pii: S1875-9572(18)30488-1. [Epub ahead of print]59(4): 327-328
    Shao PL.
      
    DOI:  https://doi.org/10.1016/j.pedneo.2018.07.009
  8. Pediatr Cardiol. 2018 Aug 18.
    Bhatt SM, Elci OU, Wang Y, Goldmuntz E, McBride M, Paridon S, Mercer-Rosa L.
      Exercise performance is variable and often impaired in patients with repaired tetralogy of Fallot (rTOF). We sought to identify factors associated with exercise performance by comparing high to low performers on cardiopulmonary exercise testing (CPET) in patients with rTOF. We conducted a cross-sectional study of subjects presenting for CPET who underwent echocardiograms at rest and peak exercise. Patients with pacemakers and arrhythmias were excluded. Right ventricular (RV) global longitudinal strain was used as a measure of systolic function. Pulmonary insufficiency (PI) was assessed with the diastolic systolic ratio and the diastolic systolic time-velocity integral ratio by Doppler interrogation of the pulmonary artery. CPET measures included percent-predicted maximum [Formula: see text] [Formula: see text], percent-predicted maximum work and oxygen pulse. High versus low performers were identified as those achieving [Formula: see text] of at least 80% or falling below, respectively. Differences in echocardiographic parameters from rest to peak exercise were examined using mixed-effects regression models. Compared to the low performers (n = 17), high performers (n = 12) were younger (12.8 ± 3.3 years vs. 18.3 ± 4.8 years), had normal chronotropic response (peak heart rate > 185 bpm) with greater heart rate reserve and superior physical working capacity. High performers also had a greater reduction in PI at peak exercise, despite greater PI severity at rest. Oxygen pulse was comparable between groups. For both groups, there was no association of PI severity and RV systolic function at rest with exercise parameters. There was no group difference in the magnitude of change in RV strain and diastolic parameters from rest to peak exercise. Chronotropic response to exercise appears to be an important parameter with which to assess exercise performance in rTOF. Chronotropic health should be taken into consideration in this population, particularly given that RV function and PI severity at rest were not associated with exercise performance.
    Keywords:  Cardiopulmonary exercise testing; Congenital heart disease; Stress echocardiography; Tetralogy of Fallot
    DOI:  https://doi.org/10.1007/s00246-018-1962-0
  9. J Am Coll Cardiol. 2018 Aug 09. pii: S0735-1097(18)36849-9. [Epub ahead of print]
    Zaragoza-Macias E, Zaidi AN, Dendukuri N, Marelli A.
      Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs. The inclusion criteria included age ≥18 years, systemic RVs, and at least 3 months of treatment with ACE inhibitor, ARB, beta blocker, or aldosterone antagonist. The outcomes included RV end-diastolic and end-systolic dimensions, RV ejection fraction, functional class, and exercise capacity. EMBASE, PubMed, and Cochrane databases were searched. The selected data were pooled and analyzed with the DerSimonian-Laird random-effects meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. A Bayesian meta-analysis model was also used in the event that heterogeneity was low. Bias assessment was performed with the Newcastle-Ottawa Scale and Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. Six studies met the inclusion criteria, contributing a total of 187 patients; treatment with beta blocker was the intervention that could not be analyzed because of the small number of patients and diversity of outcomes reported. After at least 3 months of treatment with ACE inhibitors, ARBs, or aldosterone antagonists, there was no statistically significant change in mean ejection fraction, ventricular dimensions, or peak ventilatory equivalent of oxygen. The methodological quality of the majority of included studies was low, mainly because of a lack of a randomized and controlled design, small sample size, and incomplete follow-up. In conclusion, pooled results across the limited available studies did not provide conclusive evidence with regard to a beneficial effect of medical therapy in adults with systemic RV dysfunction. Randomized controlled trials or comparative-effectiveness studies that are sufficiently powered to demonstrate effect are needed to elucidate the efficacy of ACE inhibitors, ARBs, beta blockers, and aldosterone antagonists in patients with systemic RVs.
    DOI:  https://doi.org/10.1016/j.jacc.2018.08.1030
  10. J Pediatr Surg. 2018 Aug 03. pii: S0022-3468(18)30481-0. [Epub ahead of print]
    Karila K, Anttila A, Iber T, Pakarinen M, Koivusalo A.
      BACKGROUND: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) are the most common abdominal surgical conditions in preemies. Associated mortality remains high and long periods of parenteral nutrition (PN) may be required. We assessed the developments in the outcomes of surgically treated NEC and SIP in the two largest Finnish neonatal intensive care units (NICU).METHODS: Retrospective observational study based on hospital records during 1986-2014. Main outcome measures were three-month survival during 1986-2000 compared with 2001-2014 and predictors of mortality.
    RESULTS: Included were 225 patients (NICU A 131 and NICU B 94) with NEC in 142 (63%) and SIP 83 (37%). The median birth weight (BW) (870 vs 900 g) and gestation age (GA) (27 vs 27 weeks, p = 0.96) were similar in NEC and SIP. Small intestine was affected in 85% of NEC and 76% of SIP patients (p = 0.12). In 5% of patients NEC was panintestinal. Median small intestinal loss was 25% in NEC and 4.0% in SIP (p < 0.001). Ileocecal valve was resected in 29% of NEC and 14% of SIP patients (p = 0.01). Enterostomy was performed in 78% of patients and primary anastomosis in 18%; 4% died of extensive NEC without definitive surgery. Overall survival was 74% (NEC 73%, SIP 77%, p = 0.48; NICU A 82%, NICU B 65%, p = 0.003). From 1986-2000 to 2001-2014 overall survival increased from 69 to 81% (p = 0.04). Treating NICU was the strongest predictor of survival, RR = 2.8 (95% CI = 1.4-5.1), p = 0.003.
    CONCLUSIONS: Overall survival improved significantly from the early (1986-2000) to the late (2001-2014) study period. Strongest predictor of mortality was the treating neonatal intensive care unit.
    LEVEL OF EVIDENCE: III.
    Keywords:  Mortality; Necrotizing enterocolitis; Parenteral nutrition; Spontaneous intestinal perforation; Surgical methods
    DOI:  https://doi.org/10.1016/j.jpedsurg.2018.07.020
  11. Pediatr Cardiol. 2018 Aug 18.
    Weber RW, Stiasny B, Ruecker B, Fasnacht M, Cavigelli-Brunner A, Valsangiacomo Buechel ER.
      We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.
    Keywords:  Foetal echocardiography; Outcome; Prenatal diagnosis; Single ventricle
    DOI:  https://doi.org/10.1007/s00246-018-1961-1