Percutaneous bicaval dual lumen cannula for extracorporeal life support Veno-venous extracorporeal membrane oxygenation (ECMO) is a useful mechanical device for pediatric patients with severe respiratory failure. Conventional veno-venous ECMO using double cannulation, however, is not feasible due to size limitations in pediatric patients who have small femoral vessels. Recently, percutaneous bicaval dual-lumen cannula can be inserted using single cannulation via the right internal jugular vein. Herein, we report the case of a pediatric patient with severe respiratory failure who was weaned off the ECMO successfully after treatment with bicaval dual-lumen cannulation for 5 days despite the small body size and immunocompromised condition due to chemotherapy for hemophagocytic lymphohistiocytosis.

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OBJECTIVES Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7–106.9 ml/m2, P < 0.001), end-systolic volume (101.4–64.9 ml/m2, P < 0.001), stroke volume (66.8–48.0 ml/m2, P = 0.007) and cardiac output (5.1–3.6 l/m2, P = 0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5–69.5%) and oxygen pulse (95.0–83.0%) changed without statistical significance. CONCLUSIONS Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.

Objectives Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot type anomalies. We tried to find how fast the aortic structures dilate and how to manage this phenomenon. Methods Among 801 patients who underwent corrective surgery of Fallot type anomalies, [tetralogy of Fallot (TOF) and Fallot type of double outlet right ventricle (DORV)] from 2004 to 2020, 66 patients who had follow-up cardiac computed tomography angiography (CT) images at least over 5-year interval after the initial CT study were enrolled in this retrospective study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of aortic annulus, sinus of Valsalva, sinotubular junction and ascending aorta between initial and follow-up CTs. "Dilatation” was defined as a z-score over 2 in each aortic structure. Results The median age at initial and follow-up CTs were 5.9 years (Interquartile range (IQR): 0.4~12.4) and 15.9 years (IQR: 9.3~23.4), respectively. The median CT interval (initial ~ latest CT) was 9.5 years (IQR: 6.6~12.0). Sinus of Valsalva was the aortic structure that dilated the most rapidly (0.94 mm/year) and significantly (32.8 mm at follow-up CT) across the study period. The AH ratio increased significantly in the four aortic structures. Patient’s age was significantly associated with higher AH in follow-up CT. Aortic dilatation was found in 74.2% at initial CT and 86.4% at follow-up CT. Conclusions In Fallot type anomalies, the AH ratio of aortic root structures significantly increased over about 9.5 years. The number of the patients diagnosed within the range of aortic dilatation also increased. Shorter interval for regular follow-up should be considered for these young patients because it could reach a significant dilatation in their mid-20s according to our observation in this study.

Backgrounds: Dilatation of aortic root or ascending aorta is often seen in the patients with conotruncal anomalies (CA). How fast these structures dilate and how we manage this phenomenon are not clear. Methods: We reviewed medical records of patients who underwent biventricular repair of CA such as tetralogy of Fallot (TOF), double outlet right ventricle (DORV) and truncus arteriosus (TA) from 2004 to 2018. We included 99 patients who had follow-up cardiac computed tomography angiography (CT) images at least 5 years since initial CT in this study. We analyzed changes of diameters of aortic root structures and ascending aorta between initial and follow-up CT images. Results: Median follow-up duration was 16.1 years (interquartile range (IQR): 11.8~26.2 years) after operation. Median interval between initial and follow-up CT examination was 9.4 years (IQR: 7.1~11.5 years). Median age of initial and follow-up CT was 4.3 years (IQR: 0.3~12.3 years) and 14.4 years (IQR: 9.2~22.9 years). The median z-scores (with IQR) of sinus of Valsalva (SV, 2.0 (0.8~3.3) → 2.4 (1.4~3.3), p=0.006) and sinotubular junction (STJ, 1.3 (-0.2~2.6) → 2.2 (0.4~3.3), p=0.001) were significantly changed, whereas aortic annulus (AA, 2.4 (0.9~3.4) → 2.3 (0.8~3.3), p=0.21) and ascending aorta (AsA, 1.6 (0.4~2.9) → 1.6 (0.03~2.6), p=0.27) did not show significant changes in CT images. Median increasing rate of diameter of AA, SV, STJ and AsA was 0.72, 0.93, 0.83 and 0.51 mm/year, respectively. Dilatation rate of AA (0.69 in TOF vs. 0.99 in TA, p=0.042) and SV (0.81 in TOF vs. 1.53 in TA, p=0.008) of TA was significantly higher than of TOF. Conclusions: In TOF, DORV and TA, SV and STJ significantly dilated, whereas AsA and AA did not significantly dilated for 9 years of follow-up. Although chance of surgical intervention for dilatation of aortic structures was rare, it might be necessary to observe closely aortic valvular function associated with structural deterioration of SV or STJ.