Background Left pulmonary artery (LPA) sling is a rare anomaly characterized by the origin of the LPA from the right pulmonary artery with a course between the trachea and esophagus. It is often associated with airway and cardiac anomalies. Methods This is a retrospective case series of consecutive patients who underwent LPA sling repair (LPASR) at a tertiary care center over a 35-year period with a focus on tracheal and/or LPA reinterventions and survival. Results Between June 1983 and July 2018, 42 patients were identified: isolated LPASR was performed in 16 (38%), LPASR/intracardiac repair in 10 (24%), and LPASR/tracheal repair in 16 (38%). There were 5 (12%) in-hospital and 4 (10%) late deaths. Survival rates (15 years) were as follows: 100% (isolated LPASR), 65% (concomitant intracardiac repair), and 52% (concomitant tracheal surgery). Preoperative intensive care unit (ICU) hospitalization was associated with future intervention on the LPA/trachea (61%, 11/18). The median distribution of blood flow to the left lung post-index surgery was 38%. Freedom from isolated LPA intervention was 100% after isolated LPASR, 93% after LPASR/tracheal surgery, and 69% after LPASR/intracardiac repair. Freedom from isolated tracheal intervention was 92% after isolated LPASR, 73% after LPASR/tracheal surgery, and 78% after LPASR/intracardiac repair. Conclusions ICU hospitalization prior to index surgery may indicate the severity of associated cardiac/tracheal abnormalities as this is associated with increased morbidity and mortality. Patients who underwent LPASR/intracardiac surgery were more likely to undergo isolated LPA intervention and those who underwent LPASR/tracheal surgery were more likely to undergo isolated tracheal intervention.
Management of complex left ventricular outflow tract obstruction (LVOTO) can be achieved with a Konno or Modified Konno procedure to enlarge the LVOT. We hypothesized that patients who undergo a Modified Konno procedure would have a higher rate of LVOT re-intervention compared to the Konno procedure. Patients who underwent a Konno or Modified Konno procedure for LVOTO at a single tertiary care center between 1990 and 2014 were retrospectively reviewed. The primary outcome was LVOT re-intervention post-discharge from index Konno or Modified Konno procedure. Cox regression and Kaplan–Meier estimates were used for time-to-event analysis of LVOT re-interventions, any unplanned re-interventions, and transplant-free survival. The study included 122 patients: 51 (41.8%) in the Konno group and 71 (58.2%) in the Modified Konno group. Median age at surgery was 8.2 (IQR 3–16) years in the Konno group and 3.9 (IQR 1.5–11) years in the Modified Konno group. Multiple left heart lesions were less prevalent in Modified Konno patients. There were 36 (29.5%) patients with LVOT re-interventions: 8 (16%) in the Konno group and 28 (39.4%) in the Modified Konno group ( p = 0.01). Transplant-free survival at five years was 87.2% for the Konno group and 93.5% for the Modified Konno group. A higher rate of LVOT re-intervention was found in the Modified Konno group although the Konno and Modified Konno techniques were applied to different patient populations. This finding suggests that careful preoperative decision-making can direct therapy appropriately and that fundamental diagnosis affects procedure choice. Supplementary Information The online version contains supplementary material available at 10.1007/s00246-020-02522-9.
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