A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.

BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04). CONCLUSION: The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

What is the role of airway pressure release ventilation in the management of acute lung injury?

The lack of published evidence supporting the use of APRV in the pediatric critical care patient population may diminish its effective application in respiratory failure. The effect of APRV on the number of ventilator days, ICU stay, and mortality still remains to be studied. Further application of APRV in the role of rest settings for ECMO especially in the pediatric cardiac patient population needs to be investigated. Will the use of APRV decrease the time for adequate lung recruitment, decrease sheer trauma, and/or promote earlier decannulation upon the restoration of tolerable cardiac function? Can APRV be utilized as a re-recruitment maneuver? A comparison of APRV over sustained in a randomized-controlled fashion, will there be a significant difference in ventilator days, length of ICU stay, and/or mortality? Does re-recruitment at plateau pressures during suctioning, patient position changes, or in the face of increased airway resistance decrease the number of ventilator days, length of ICU stay, and/or mortality? Does the use of continuous monitoring of carbon dioxide production aid in optimizing P(high)? The list of questions, both speculative and scientific are too numerous to list. Speculation leads to inquiry which over time drives science. More focus is needed on randomized, controlled trials. Initially the comparison of APRV to HFOV needs to be the primary focus for a proactive approach for ALI. Once a comfort level is established with this modality, further scientific inquires will follow. In the meantime, its use is likely to remain controversial.