Abstract
Objectives: the purpose of this study was to demonstrate early and midterm postoperative results, after changes in the current strategy for the management of patients with pulmonary atresia and intact ventricular septum, and determines risk factors of an unfavorable outcome.
Material and methods. Retrospective analysis of 21 patients with pulmonary atresia and intact ventricular septum treated at the Bakoulev National Medical Research Center for Cardiovascular Surgery within the period from 2014 to 2020. All patients were divided into four groups, depending from right ventricle (RV) size. The first group were patients with normal RV (n = 8), second group – with moderate RV hypoplasia (n=6), third group – patients with severe RV hypoplasia (n = 4) and fourth group – patients with enlarged RV (n = 3). Median age at hospitalization was 19.4 ± 38.4 days (from 1 day to 5 months). Median weight was 3.55 ± 1.1 (from 2.4 to 7.07) kg. Body surface area was 0.23 ± 0.05 (from 0.17 to 0.37) m2. All patients were operated by one of 3 ways: 2-ventricle, 1.5-ventricle and 1-ventricle correction. Patients who died after operation in case of heart failure (n = 6) become control group.
Results. The distribution of RV sizes was normal in 8 (38%), moderate hypoplasia in 6 (28.6%), severe hypoplasia in 4 (19%) and enlarged RV in 3 (14.4%) patients. RV-to coronary-artery connections were present in 7 (33.3%) and RV coronary dependence in 2 (9.5%) patients. Tricuspid valve (TV) z-score at hospitalization in I group was 0.08 ± 1.11 (from –0.96 to 1.68), in II group –1.26 ± 0.35 (ranged from –0.9 to –1.9), in III group –4.2 ± 1.02 (from –3.2 to –5.27) and in IV group 4.0 ± 2.26 (ranged from 2.6 to 6.62). General mortality at 21.0 ± 14.8 month (from 4 month to 4.5 years) time interval was 28.5%. The end status of the patient’s hemodynamics state was: biventricular repair in 6 (28%), 1.5-ventricle repair in 2 (10%), 1-ventricle repair in 3 (14%), and still awaiting repair in 4 (19%). Two-year survival was 71.5% (95% confidence interval 67.3–75.7%). Independent predictors of mortality were the presence of RV-to-coronary-artery connections and RV coronary dependence.
Conclusion. Patients with severe RV and TV hypoplasia (z-score less than –4) or in case of presence of RV-to coronary-artery connections with RV coronary dependence, should initially be directed to the univentricular repair. All remained patients should be directed to biventricular repair with early RV decompression.
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About the authors
- Sergey V. Kalashnikov, Cand. Med. Sci., Cardiovascular Surgeon, Researcher; ORCID
- Irina Yu. Baryshnikova, Cand. Med. Sci., Researcher; ORCID
- Anatoliy E. Tagaev, Cardiovascular Surgeon; ORCID
- Inessa E. Nefedova, Dr. Med. Sci., Head of Department; ORCID
