bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒08‒05
six papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2018 Jul 28.
    VanLoozen DH, Murdison KA, Polimenakos AC.
      Right ventricle dependent coronary circulation (RVDCC) in pulmonary atresia with intact ventricular septum (PA/IVS) is associated with significant mortality risk in the immediate post-operative period following the initial stage of surgical palliation. Prognosis remains guarded during the interstage period towards conversion to the superior cavopulmonary shunt physiology. Current literature is scarce regarding this specific patient population. Cardiac troponin-I is widely used as a marker of coronary ischemia in adults, but its use for routine monitoring of neonatal myocardial tissue injury due to supply/demand perfusion mismatch is, yet to be determined. We sought to evaluate the clinical correlation of cTnl perioperative use in a PA/IVS RVDCC case and assess its interplay with established clinical, echocardiographic, and laboratory variables in guiding a real-time (dynamic) management strategy following systemic-to-pulmonary shunt palliation.
    Keywords:  Congenital; Post-operative; Pulmonary atresia; Right ventricle dependent coronary circulation; Troponin-I
    DOI:  https://doi.org/10.1007/s00246-018-1940-6
  2. Ann Thorac Surg. 2018 Jul 25. pii: S0003-4975(18)31031-2. [Epub ahead of print]
    Jacobsen RM, Mitchell ME, Woods RK, Loomba RS, Tweddell JS.
      BACKGROUND: Norwood palliation typically requires patch augmentation of the ascending aorta and aortic arch. Patients having undergone Norwood palliation are at risk of recurrent arch obstruction, the risk of which may be impacted by the type of patch material used at the time of Norwood palliation. We sought to determine the freedom from neo-aortic arch re-intervention and overall survival in patients who underwent Norwood palliation utilizing porcine small intestinal submucosa (PSIS) as the patch material.METHODS: Retrospective chart review was performed to identify patients who underwent a Norwood operation utilizing PSIS material at our institution. Cardiac diagnosis, age at surgery, shunt type, need for re-intervention, and outcome (survival, transplant, and death) were evaluated.
    RESULTS: Forty-four patients had PSIS material utilized for arch reconstruction at the time of Norwood palliation. There were only 5 (11.4%) neo-aortic arch re-interventions in 4 patients. An additional 10 re-interventions, unrelated to the PSIS patch, were performed, including 5 shunt revisions and 5 branch pulmonary artery interventions. There were 3 deaths and 5 patients were transplanted. Median follow-up was 387.5 days (range, 4-1513 days).
    CONCLUSIONS: Freedom from neo-aortic arch re-intervention and survival following Norwood palliation with PSIS patch material is promising. PSIS appears non-inferior and may be an appropriate tissue choice for Norwood palliation. Studies with longer follow-up are needed to determine the rate of neo-aortic re-intervention over time.
    Keywords:  A congenital surgeon; Allograft; Anatomy; Aorta/aortic; Homograft; aortic arch
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.06.033
  3. Hepatology. 2018 Jul 28.
    Mazzarelli C, Cannon MD, Hudson M, Heaton N, Sarker D, Kane P, Quaglia A, Suddle A.
      Liver disease is a recognised complication of the Fontan procedure for cyanotic congenital heart disease. The Fontan procedure is a complex cardio-surgical procedure that diverts systemic venous blood in patients with univentricular hearts directly into the pulmonary arteries, bypassing the right ventricle.[1] The operation has dramatically increased the survival of patients with single ventricle physiology, with 83% surviving to 20 years, without need for cardiac transplantation. This article is protected by copyright. All rights reserved.
    DOI:  https://doi.org/10.1002/hep.30194
  4. Ann Thorac Surg. 2018 Jul 25. pii: S0003-4975(18)31030-0. [Epub ahead of print]
    Jacobs ML, Jacobs JP, Hill KD, O'Brien SM, Pasquali SK, Vener D, Kumar SR, Chiswell K, Habib RH, Shahian DM, Fernandez FG.
      The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) is the largest congenital and pediatric cardiac surgical clinical data registry in the world. The most recent bi-annual feedback report to participants includes analysis of data submitted from 125 hospitals, representing nearly all centers performing pediatric and congenital heart operations in the United States and Canada. In addition to serving as a platform for reporting of outcomes and for quality improvement, the database continues to be a primary data source for clinical research and for innovations related to quality measurement. Over the past year, several investigative teams reported analyses of data in the STS CHSD pertaining to various processes of care, including assessment of variation in specific practices and patient characteristics across centers participating in the STS CHSD, and their associations with outcomes. Additional ongoing projects involve the development of new or refined metrics for quality measurement and reporting of outcomes and center level performance. To meet the needs of investigators, the STS Research Center and Workforce on Research Development have created multiple pathways through which investigators may propose, and ultimately perform outcomes research projects based on STS CHSD data. This article reviews published outcomes research and quality improvement projects from the past year, and describes ongoing research related to quality measurement.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.06.032
  5. Dev Period Med. 2018 ;22(2): 113-122
    Stasiak A, Niewiadomska-Jarosik K, Kędziora P.
      INTRODUCTION: Essentially, preexcitation syndrome is the presence of an accessory pathway in the heart, which can lead to serious consequences, ranging from atrioventricular reentrant tachycardia to sudden cardiac death. Wolff-Parkinson-White syndrome is the most common preexcitation syndrome.AIM OF THE STUDY: The aim of the study was to evaluate the clinical course of the disease, as well as the treatment of children and adolescents hospitalized in the Department of Pediatric Cardiology in the years 2008-2015.
    MATERIALS AND METHODS: The study was carried out in 45 children (62 % male, 38% female; the mean age 11 years). During the study we analyzed 12-lead ECG, 24-hour Holter ECG, echocardiography and the cycloergometric exercise test. The results of treatment were also discussed.
    RESULTS: Apart from the typical features of preexcitation, the most prevalent abnormality found in ECG was atrioventricular reentrant tachycardia. In 24-hour Holter ECG the most frequently detected disorders were premature ventricular beats and premature atrial contractions. Structural heart defects were detected in 8.9% of the children. The cycloergometric exercise test was positive in 8.9% of patients. The mean duration of symptoms before the diagnosis was 2.5 years. 25% of the patients were asymptomatic. 42.2% of the children needed antiarrhythmic therapy, while 44.4% had accessory pathways ablated.
    CONCLUSIONS: The most common symptom of preexcitation in the study group were heart palpitations. The most frequent type of arrhythmia in children with preexcitation syndrome was orthodromic atrioventricular reentrant tachycardia. For the majority of older children ablation of the accessory pathway was a recommended form of treatment. In younger children the standard preventive pharmacological treatment was applied for 6 to 12 months.
    Keywords:   WPW syndrome ; child ; preexcitation syndromes
  6. Rev Esp Cardiol (Engl Ed). 2018 Jul 25. pii: S1885-5857(18)30295-0. [Epub ahead of print]
    Pulignani S, Andreassi MG.
      
    DOI:  https://doi.org/10.1016/j.rec.2018.06.030