bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒09‒16
seven papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2018 Sep 08.
    Chauhan JC, Deb R.
      CO2 removal by the lungs depends upon ventilation and pulmonary blood flow, with end tidal CO2 (EtCO2) as surrogate for it. We studied indices based on EtCO2 measured routinely during anesthesia for cardiac catheterization, along with simultaneously calculated Qp:Qs (pulmonary-to-systemic-blood-flow ratio) in children with complex congenital heart disease to assess the relationship between these measures. A retrospective, single-center, correlational cohort study was conducted at a tertiary-care, free-standing children's hospital. All included subjects had Qp:Qs calculated as well as EtCO2 and PaCO2 documented during a single cardiac catheterization. Children with stage-1 single ventricle or complex biventricular repair with highly variable Qp:Qs were defined as Group 1, and Group 2 comprised those with stage 2 or 3 repairs with less variable Qp:Qs. Exclusion criteria were uncuffed artificial airway, EtCO2 > PaCO2, and abnormally high Qp:Qs. EtCO2 indices were defined as EtCO2:PaCO2 (alveolar functional fraction) and EtCO2 gap (PaCO2-EtCO2). Correlation coefficients were obtained between Qp:Qs and EtCO2 indices in both groups. A total of 29 patients in Group 1 and 24 in Group 2 underwent final analysis. Even with highly variable Qp:Qs, Group 1 showed a strong correlation between Qp:Qs and EtCO2:PaCO2 (r = 0.83, p < 0.0001). A similarly strong correlation was maintained in Group 2 (r = 0.79, p < 0.0001) and in both groups combined (r = 0.86, p < 0.0001). A very strong negative correlation was present between Qp:Qs and EtCO2 gap (r = - 0.77, p < 0.0001). EtCO2:PaCO2 has a very strong correlation with Qp:Qs simultaneously calculated during catheterization. It can be an additional parameter to estimate Qp:Qs in critical management of children with congenital heart disease. Our results also provide a basis for future prospective studies to assess dynamic changes in EtCO2-based indices and Qp:Qs.
    Keywords:  Cardiac catheterization; Children; Congenital heart disease; EtCO2; PaCO2; Qp:Qs
    DOI:  https://doi.org/10.1007/s00246-018-1976-7
  2. Int J Cardiol. 2018 Sep 02. pii: S0167-5273(18)30459-5. [Epub ahead of print]
    Li JJ, Liu Y, Xie SY, Zhao GD, Dai T, Chen H, Mu LF, Qi HY, Li J.
      BACKGROUND: Pulse oximetry screening for critical congenital heart disease (CHD) is inapplicable to high altitude due to the variedly decreased arterial saturations and rare complex CHD. We examined the incidence and spectrum of CHD in newborns using echocardiography at high altitude and followed up their outcomes.METHODS: A total of 1337 babies were studied. Echocardiography was performed in 1002 asymptomatic newborns (3-5 days). In the same period, retrospectively studied 394 newborns (≤2 days) admitted to the NICU where echocardiograph was performed in 335. In both groups, follow-up was made at 1-3, 6 and 12-18 months.
    RESULTS: The incidence of CHD in asymptomatic newborns was 27.8%, consisting secundum atrial septal defect (ASD) [175 (62.7%)], patent ductus arteriosus (PDA) [61 (21.9%)], ventricular septal defect (VSD) [8 (2.9%)] and multiple defects [35 (12.6%)]. And 19.4% in NICU patients with similar spectrum, except for 2 with complex CHD who died before discharge. By 12-18 months of follow-up, 30% of CHD remained open. Thirteen patients developed mild to severe pulmonary arterial hypertension (PAH), and 2 of them died of heart failure.
    CONCLUSIONS: The incidence of CHD in newborns at high altitude is about 20 times higher than that at low altitude, consisting mostly of simple forms with left to right shunt, with rare complex CHD. By 12-18 months, the incidence of CHD is still about 10 times higher than that at low altitude. About 8% patients developed PAH or death. Follow-up must be reinforced in order to provide early intervention and prevent from PAH or death.
    Keywords:  Echocardiography screening; High altitude; Incidence of congenital heart defect; Newborns
    DOI:  https://doi.org/10.1016/j.ijcard.2018.08.102
  3. Semin Thorac Cardiovasc Surg. 2018 Sep 05. pii: S1043-0679(18)30208-9. [Epub ahead of print]
    Su JA, Kumar SR, Mahmoud H, Bowdish ME, Toubat O, Wood JC, Kung GC.
      OBJECTIVE: Troponin-I (TN-I) levels are elevated following pediatric cardiac surgery with speculation that particular patterns may have prognostic significance. There is lack of procedure-specific data regarding postoperative TN-I levels in infants undergoing cardiac surgery. We hypothesized that TN-I elevation varies with type of surgery and persistent elevation predicts poor prognosis.METHODS: We prospectively measured serial TN-I levels (preoperatively, 4, 8, 12, 24, and 48 hours postoperatively) in 90 infants (age < 1 year) undergoing cardiac surgery: off cardiopulmonary bypass (CPB) (n = 15), on CPB (n = 43), and on CPB with ventricular incision (CPB with ventricular incision) (n = 32).
    RESULTS: All patients had undetectable baseline TN-I levels. The area under the curve (AUC) of TN-I levels over the 48-hour period was significantly different among the surgical groups (p < 0.002), and highest in patients with CPB with ventricular incision. Generally, TN-I levels peaked by 4 hours after surgery and returned to near-normal levels within 48 hours. A persistent TN-I rise beyond 8 hours after surgery was a strong predictor of postoperative hypoperfusion injury (defined as a composite endpoint of end-organ injury resulting from inadequate perfusion, OR 21.5; p = 0.001) and mortality (30% in those with persistently high TN-I, compared with 3.5% in the remaining patients; p < 0.001), independent of patient age, anatomy/complexity of surgery, and level of postoperative support.
    CONCLUSIONS: Our data provide benchmark values for TN-I levels following cardiac surgery in infants. Extent of TN-I elevation correlates with type of surgery. Persistent TN-I elevation beyond 8 hours after surgery is strongly associated with postoperative hypoperfusion injury and mortality.
    DOI:  https://doi.org/10.1053/j.semtcvs.2018.08.010
  4. Semin Fetal Neonatal Med. 2018 Aug 20. pii: S1744-165X(18)30099-4. [Epub ahead of print]
    Hong CR, Han SM, Jaksic T.
      Necrotizing enterocolitis (NEC) is a potentially devastating condition that preferentially affects premature and low birth weight infants, with approximately half requiring acute surgical intervention. Surgical consult should be considered early on, and deterioration despite maximal medical therapy or the finding of pneumoperitoneum are the strongest indications for emergent surgical intervention. There is no clear consensus on the optimal surgical approach between peritoneal drainage and laparotomy; the best course of action likely depends on the infant's comorbidities, hemodynamic status, size, disease involvement, and available resources. Patients who develop surgical NEC are at a significant risk for morbidity and mortality, with long-term complications including short bowel syndrome, growth failure, and neurodevelopmental impairment. Further research into strategies that optimize outcomes following surgery for NEC in the neonatal intensive care unit and long-term are paramount.
    Keywords:  Laparotomy; Necrotizing enterocolitis; Neonatal intensive care unit; Neurodevelopment; Peritoneal drainage; Prematurity; Short bowel syndrome
    DOI:  https://doi.org/10.1016/j.siny.2018.08.007
  5. Int J Cardiol. 2018 Sep 01. pii: S0167-5273(18)32453-7. [Epub ahead of print]
    Ephrem G, Alshawabkeh L.
      BACKGROUND: Care delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined.METHODS: A 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous.
    RESULTS: Of the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation.
    CONCLUSIONS: The new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.
    Keywords:  Adult; Congenital; Disease; Heart; Job; Training
    DOI:  https://doi.org/10.1016/j.ijcard.2018.08.086
  6. Pediatr Cardiol. 2018 Sep 08.
    Wu Y, Xin L, Zhou Y, Kuang H, Jin X, Li Y, Wu C.
      A meta-analysis was performed for a comparison of outcomes between sutureless technique and conventional surgery for primary repair for total anomalous pulmonary venous connection (TAPVC). Electronic databases including PubMed, EMbase, Scopus, and Cochrane Library were searched systematically for the single-arm studies regarding sutureless repair or conventional surgery, and two-arm studies compared the outcomes of sutureless repair and conventional surgery for TAPVC. Corresponding data were extracted and the methodological quality was assessed by two reviewers independently. 26 studies were included, involving a total of 2702 patients. It was observed that compared with conventional surgery, sutureless technique was associated with a lower occurrence rate of post-operative pulmonary veins obstruction (PVO) (4.6% vs. 13.5%, OR 0.54 in favor of sutureless technique) and re-operations due to PVO (3.4% vs. 12.4%, 0.25 in favor of sutureless technique). However, meta-analyses of post-operative early (OR 0.57; 95% CI 0.27-1.19; P = 0.13), late (OR 0.37; 95% CI 0.13-1.06; P = 0.13), and overall (OR 0.61; 95% CI 0.36-1.03; P = 0.07) mortality showed no significant difference between sutureless technique and conventional surgery. Compared with conventional surgery, sutureless technique was associated with a lower occurrence rate of post-operative PVO and re-operations due to PVO. Meanwhile, post-operative early, late, and overall mortality were not statistically different between two surgical approaches. Sutureless technique is beneficial in the primary repair of TAPVC regarding post-operative PVO and re-operations due to PVO. However, the level of evidence was low and randomized controlled trials should be designed to evaluate the safety and effectiveness of sutureless technique for TAPVC.
    Keywords:  Congenital heart disease; Conventional surgery; Meta-analysis; Sutureless technique; Total anomalous pulmonary venous connection
    DOI:  https://doi.org/10.1007/s00246-018-1948-y
  7. Int J Cardiol. 2018 Aug 25. pii: S0167-5273(18)34885-X. [Epub ahead of print]
    Kaemmerer H, Apitz C, Brockmeier K, Eicken A, Gorenflo M, Hager A, de Haan F, Huntgeburth M, Kozlik-Feldmann RG, Miera O, Diller GP.
      In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article.
    Keywords:  Adult; Congenital heart disease; Medical therapy; Pulmonary hypertension; Shunt
    DOI:  https://doi.org/10.1016/j.ijcard.2018.08.078