Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary
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ORIGINAL ARTICLE
Outcomes of systemic‑to‑pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection Takuya Okamoto1 · Toshihide Nakano1 · Masami Goda1 · Shinichiro Oda1 · Hideaki Kado1 Received: 2 April 2020 / Accepted: 28 August 2020 © The Japanese Association for Thoracic Surgery 2020
Abstract Objectives A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). Methods Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. Results Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. Conclusions Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial. Keywords Congenital heart disease · Univentricular heart · Systemic-to-pulmonary artery shunt · Total anomalous pulmonary venous connection
Introduction Despite numerous advances in diagnosis and perioperative management of congenital heart surgery, systemic-to-pulmonary artery shunt (SPS) still has a poor prognosis; the operative and interim mortality rates for single ventricular heart (SVH) are 15–18% and 19%, respectively [1–4]. It has been reported some predictor for mortality after SPS [1, 2, 5], in particular, the case of SPS with total anomalous pulmonary * Takuya Okamoto [email protected] 1
venous connection (TAPVC) has worse outcomes [6–8]; in the reports of TAPVC, when only SPS with SVH cases were extracted, the early mortality rate was 63–71% [6, 9, 10]. However, studies that evaluated the outcomes of SPS [1, 5, 11–14] did not include TAPVC in the analysis, or even if that was included, the number of TAPVC cases was small. Therefore, we thought that those studies could not determine the effect of TAPVC on SPS. Moreover, to our best knowledge, a few studies have described the outcomes of only S
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