bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒10‒21
three papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2018 Oct 10. pii: S0003-4975(18)31442-5. [Epub ahead of print]
    Ali WB, Bouhout I, Khairy P, Bouchard D, Poirier N.
      BACKGROUND: There is growing awareness of the long-term impact of a Fontan circulation on the associated morbidity and mortality. Comparative data on the incidence of supraventricular arrhythmia and sinus node dysfunction following extra cardiac (EC) and lateral tunnel (LT) Fontans are controversial. We performed a meta-analysis pooling all available long-term results comparing the EC and LT Fontan with a special focus on arrhythmia.METHODS: We performed a systematic search of PubMed, EMBASE, and Cochrane Library for articles reporting long-term results of Fontan comparing the EC and the LT Fontan.
    RESULTS: 12 studies were selected with 3330 patients (1729 EC, 1601 LT). Freedom from tachyarrhythmia was significantly higher in the EC group (92% versus 83% at 15 years; p <0.0001) while there was no difference in term of bradyarrhythmias (p= 0.7). The survival was 93% and 89% at 20 years respectively in the EC and LT groups (p=0.007). The risk of thromboembolic events was 2.87% patients-years in the EC group vs 0.9% in the LT group (OR= 2.15 [0.95; 4.85]; p=0.07).
    CONCLUSIONS: The EC Fontan confers long-term survival advantage over the LT without a higher rate of reoperations. The EC Fontan preserves the sinus node function and reduces significantly the incidence of long-term postoperative arrhythmia.
    Keywords:  Extra-cardiac; Fontan procedure; Lateral tunnel; arrhythmias
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.08.041
  2. JACC Heart Fail. 2018 Oct 04. pii: S2213-1779(18)30533-X. [Epub ahead of print]
    Amsallem M, Mercier O, Kobayashi Y, Moneghetti K, Haddad F.
      In the last decade, there has been renewed interest in the study of the right ventricle. It is now well established that right ventricular function is a strong predictor of mortality, not only in heart failure but also in pulmonary hypertension, congenital heart disease, and cardiothoracic surgery. The right ventricle is part of a cardiopulmonary unit with connections to the pulmonary circulation, venous return, atria, and left ventricle. In this context, ventriculoarterial coupling, interventricular interactions, and pericardial constraint become important to understand right ventricular adaptation to injury or abnormal loading conditions. This state-of-the-art review summarizes major advances that occurred in the field of right ventricular research over the last decade. The first section focuses on right ventricular physiology and pulmonary circulation. The second section discusses the emerging data on right ventricular phenotyping, highlighting the importance of myocardial deformation (strain) imaging and assessment of end-systolic dimensions. The third section reviews recent clinical trials involving patients at risk for or with established right ventricular failure, focusing on beta blockade, phosphodiesterase inhibition, and mechanical support of the failing right heart. The final section presents a perspective on active areas of research that are most likely to translate in clinical practice in the next decade.
    Keywords:  heart failure; imaging; pulmonary hypertension; right heart; ventricular-assist devices
    DOI:  https://doi.org/10.1016/j.jchf.2018.05.022
  3. Ann Thorac Surg. 2018 Oct 10. pii: S0003-4975(18)31455-3. [Epub ahead of print]
    Hopkins KA, Brown JW, Darragh RK, Kay WA.
      Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. While rarely performed now, patients with this physiology often face severe complications requiring re-intervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present two successful cases to illustrate this idea.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.07.097