Outcome of surgical repair of patients with left-to-right (LR) shunt lesion and severe pulmonary arterial hypertension (PAH)

  • Dr Soo In Jeong, Department of Pediatrics, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Dr Cha Gon Lee, Department of Pediatrics, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Soo Han Choi, Department of Pediatrics, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Dr June Huh, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Korea
  • Dr Heung-Jae Lee, Department of Pediatrics, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Dr Ji-Hyuk Yang, Department of Thoracic Surgery, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Dr Tae-Gook Jun, Department of Thoracic Surgery, Samsung Medical Center, SungKyunKwan University School of Medicine, Korea
  • Dr I-Seok Kang, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Korea

    • Objective:
    To report outcome of surgical closure of LR shunt lesions associated with severe PAH.

    We reviewed medical records of 21 patients who underwent surgical closure of LR shunt lesions associated with Rp above 8 Wood unit (Wu) after 1 year of age.

    results:
    Median age of operation was 26 years (range: 1-58). Shunt lesions were ASD(n=11), VSD(n=4), VSD with PDA (n=4) and PDA(n=2). NYHA class was III in 10 patients and II in 11 patients. Median Rp was 10.8 Wu(8.3~29.7). Median Rp/Rs was 0.32(0.15-0.72). Median PAP was 58 mmHg(40-91). There were response to pulmonary vasodilator in 4 patients, partial response in 2 patients and no response in 15 patients. After test occlusion of shunt with or without pulmonary vasodilator, six of 8 non-responders showed decrease in pressure.
    Complete closure was done in 5 patients, and partial closure(n=12) or ASD creation(n=4) in others. During postoperive follow-up of median 27months(4-117), estimated RVSP was decreased in 20 patients and was normalized in 9 patients. NYHA class was improved in 20 patients and unchanged in 1 patient whose RVSP didn’t decrease. Postoperatively we used pulmonary vasodilators in 16 patients and discontinued it in 6 patients during follow up. Immediate postoperative PAP was correlated with that of test occlusion. There was no mortality or significant morbidity.
    Conclusion:
    Surgical repair of LR shunt with PAH can be performed safely in selected subjects. With recent advances in intensive care and pulmonary vasodilator therapy, guideline of surgical repair of LR shunt with PAH should be reconsidered.