bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒06‒10
four papers selected by
Richard James
University of Pennsylvania


  1. Interact Cardiovasc Thorac Surg. 2018 May 30.
    Manuri L, Calaciura RE, De Zorzi A, Oreto L, Raponi M, Lehner A, Haas N, Agati S.
      Transcatheter creation or enlargement of an atrial septal defect has been used to promote adequate blood flow and mixing in some forms of congenital heart defects or as a relief valve in right or left atrial hypertension, resulting in better cardiac output and/or systemic saturation. We report a case of a 4-year-old male affected by complex congenital heart disease who was admitted for management of severe cyanosis following a staged pericardial fenestrated Fontan procedure. Transoesophageal echocardiogram showed a wide fenestration of 9 mm in size with a severely dilated pericardial Fontan system. To avoid a new surgical procedure and as part of a compassionate use programme, we decided to implant an atrial flow regulator device (4 mm in diameter) with percutaneous approach with the goal of reducing the right-to-left shunt and increasing the pulmonary flow. Preprocedural oxygen saturation was 75%, whereas after 2 months of follow-up, we observed a progressive increase of up to 95% with significant reduction in the pericardial Fontan system dimensions at echocardiography.
    DOI:  https://doi.org/10.1093/icvts/ivy165
  2. Rev Esp Cardiol (Engl Ed). 2018 May 30. pii: S1885-5857(18)30183-X. [Epub ahead of print]
    de Torres-Alba F, Kaleschke G, Baumgartner H.
      Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Early surgical repair has dramatically improved the outcome of this condition. However, despite the success of contemporary approaches with early complete repair, these are far from being curative and late complications are frequent. The most common complication is right ventricle outflow tract (RVOT) dysfunction, affecting most patients in the form of pulmonary regurgitation, pulmonary stenosis, or both, and can lead to development of symptoms of exercise intolerance, arrhythmias, and sudden cardiac death. Optimal timing of restoration of RVOT functionality in asymptomatic patients with RVOT dysfunction after TOF repair is still a matter of debate. Percutaneous pulmonary valve implantation, introduced almost 2 decades ago, has become a major game-changer in the treatment of RVOT dysfunction. In this article we review the pathophysiology, the current indications, and treatment options for RVOT dysfunction in patients after TOF repair with a focus on the role of percutaneous pulmonary valve implantation in the therapeutic approach to these patients.
    Keywords:  Cardiopatías congénitas; Congenital heart disease; Percutaneous; Percutáneo; Pulmonary valve; Tetralogy of Fallot; Tetralogía de Fallot; Válvula pulmonar
    DOI:  https://doi.org/10.1016/j.rec.2018.05.001
  3. Arch Cardiovasc Dis. 2018 May 30. pii: S1875-2136(18)30087-1. [Epub ahead of print]
    Karsenty C, Zhao A, Marijon E, Ladouceur M.
      Adult congenital heart disease (ACHD) is a constantly expanding population with challenging issues. Initial medical and surgical treatments are seldom curative, and the majority of patients still experience late sequelae and complications, especially thromboembolic events. These common and potentially life-threating adverse events are probably dramatically underdiagnosed. Better identification and understanding of thromboembolic risk factors are essential to prevent long-term related morbidities. In addition to specific situations associated with a high risk of thromboembolic events (Fontan circulation, cyanotic congenital heart disease), atrial arrhythmia has been recognized as an important risk factor for thromboembolic events in ACHD. Unlike in patients without ACHD, thromboembolic risk stratification scores, such as the CHA2DS2-VASc score, may not be applicable in ACHD. Overall, after a review of the scientific data published so far, it is clear that the complexity of the underlying congenital heart disease represents a major risk factor for thromboembolic events. As a consequence, prophylactic anticoagulation is indicated in patients with complex congenital heart disease and atrial arrhythmia, regardless of the other risk factors, as opposed to simple heart defects. The landscape of ACHD is an ongoing evolving process, and specific thromboembolic risk scores are needed, especially in the setting of simple heart defects; these should be coupled with specific trials or long-term follow-up of multicentre cohorts.
    Keywords:  Anticoagulation; Arrhythmia; Arythmie; Cardiopathie congénitale; Congenital heart disease; Thromboembolie; Thromboembolism; Traitement anticoagulant
    DOI:  https://doi.org/10.1016/j.acvd.2018.04.003
  4. J Thorac Cardiovasc Surg. 2018 May 03. pii: S0022-5223(18)31237-6. [Epub ahead of print]
    Lynch JM, Ko T, Busch DR, Newland JJ, Winters ME, Mensah-Brown K, Boorady TW, Xiao R, Nicolson SC, Montenegro LM, Gaynor JW, Spray TL, Yodh AG, Naim MY, Licht DJ.
      BACKGROUND: Hypoxic-ischemic white matter brain injury commonly occurs in neonates with critical congenital heart disease. Recent work has shown that longer time to surgery is associated with increased risk for this injury. In this study we investigated changes in perinatal cerebral hemodynamics during the transition from fetal to neonatal circulation to ascertain mechanisms that might underlie this risk.METHODS: Neonates with either transposition of the great arteries (TGA) or hypoplastic left heart syndrome (HLHS) were recruited for preoperative noninvasive optical monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using diffuse optical spectroscopy and diffuse correlation spectroscopy, 2 noninvasive optical techniques. Measurements were acquired daily from day of consent until the morning of surgery. Temporal trends in these measured parameters during the preoperative period were assessed with a mixed effects model.
    RESULTS: Forty-eight neonates with TGA or HLHS were studied. Cerebral oxygen saturation was significantly and negatively correlated with time, and oxygen extraction fraction was significantly and positively correlated with time. Cerebral blood flow did not significantly change with time during the preoperative period.
    CONCLUSIONS: In neonates with TGA or HLHS, increasing cerebral oxygen extraction combined with an abnormal cerebral blood flow response during the time between birth and heart surgery leads to a progressive decrease in cerebral tissue oxygenation The results support and help explain the physiological basis for recent studies that show longer time to surgery increases the risk of acquiring white matter injury.
    Keywords:  CHD; cerebral blood flow; neonate; timing of surgery
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.04.098