Hybrid therapy for hypoplastic left heart syndrome with intact atrial septum

  • Dr Naoki Ohno, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Shinichi Ohtsuki, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Yoshio Okamoto, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Maiko Kondo, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Yoshihiko Kurita, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Takahiro Eitoku, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Tsuneo Morishima, Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
  • Dr Shunji Sano, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan

    • Objective. In cases of hypoplastic left heart syndrome (HLHS) with intact atial septum, we discuss hybrid therapy and the postoperative course.
    Methods. The subjects were two patients with HLHS who underwent hybrid therapy in our hospital. Hybrid therapy: Immediately after birth, needle puncture of the interatrial septum via the right auricular appendage was carried out with the heart beating, static balloon atrioseptostomy (BAS) and bilateral pulmonary artery banding (PAB) was performed.
    Results. Case 1: Stenting for the interatrial septum was performed due to restenosis. A decrease in pulmonary artery pressure (PAP) was indicated by pressure gradient in PAB of 65 mmHg, and Norwood procedure was performed at the age of one month. Bidirectional Glenn procedure was performed at the age of one year and currently waiting for total cavopulmonary connection. Case 2: BAS was performed again twice due to restenosis. But there was a gradual decrease in PAP with pressure gradient in PAB of 30 mmHg. Norwood procedure was performed at the age of 3 months due to unstable hemodynamics. However, postoperative lung biopsy revealed notable hypoplasia of pulmonary small arteries. We considered that there was not surgical indication. The patient was treated with Sildenafil and Bosentan but died from PH crisis .
    Conclusions. Careful consideration must be given to the indication and timing of the Norwood procedure and also indication for further surgery based on changes in the pressure gradient in PAB as an index of PAP after interartial communication has been sufficiently established.