Hiroo Kinami

All Publications

• Kabuki patients with CHDs: outcomes after cardiac surgery CARDIOLOGY IN THE YOUNG Lam, R. W., Haro, V., Tabbutt, S., Kinami, H., Reddy, V., Kim, M. 2025: 1-4

  Abstract

  This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

  View details for DOI 10.1017/S1047951125000435

  View details for Web of Science ID 001433308300001

  View details for PubMedID 40007435

• Right ventricular outlet tract reconstruction for tetralogy of fallot: systematic review and network meta-analysis. Interdisciplinary cardiovascular and thoracic surgery Yamaguchi, A., Shimoda, T., Kinami, H., Yasuhara, J., Takagi, H., Fukuhara, S., Kuno, T. 2024

  Abstract

  Concerns persist regarding pulmonary regurgitation after transannular patch repair (TAP) for Tetralogy of Fallot. Despite various architectural preservation techniques being introduced, the optimal strategy remains controversial. We aimed to compare different right ventricular outlet tract reconstruction techniques.PubMed, EMBASE and Cochrane Central were searched through March 2024 to identify comparative studies on right ventricular outlet tract reconstruction techniques (PROSPERO ID: CRD42024519404). The primary outcome was mid-term pulmonary regurgitation, with secondary outcomes including postoperative mortality, postoperative pulmonary regurgitation, length of intensive care unit stays, postoperative right ventricular outlet tract pressure gradient, and mid-term mortality. We performed a network meta-analysis to compare outcomes among TAP, valve-repairing (VR), TAP with neo-valve creation (TAPN), and valve-sparing (VS).Two randomized controlled studies and 32 observational studies were identified with 8,890 patients. TAP carried a higher risk of mid-term pulmonary regurgitation compared to TAPN (HR, 0.53; 95％CI [0.33; 0.85]) and VS (HR, 0.27; 95% CI [0.19; 0.39]), with no significant difference compared to VR. VS was also associated with reduced postoperative mortality compared to TAP (RR, 0.31; 95% CI [0.18; 0.56]), in addition to reduced ventilation time. TAP also carried an increased risk of postoperative pulmonary regurgitation compared to the other groups. The groups were comparable in terms of length of intensive care unit stay, right ventricular outlet tract pressure gradient, and mid-term mortality.VR was associated with a reduced risk of postoperative pulmonary regurgitation, while TAPN was associated with reduced risks of both postoperative and mid-term pulmonary regurgitation.

  View details for DOI 10.1093/icvts/ivae180

  View details for PubMedID 39499166