Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis

An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist with associated cardiac lesions, the conduct of the cardiac surgical repair must accommodate the abnormal venous anatomy, especially in total cavopulmonary connection patients. Herein, we present a rare case of a 7-year-old Chinese boy about 1 year post bilateral superior vena cava pulmonary anastomosis who presented with severe cyanosis and was referred to our department. However, the patient showed an unexpected gradual decrease in blood oxygen saturation to 60–70% after the extracardiac total cavopulmonary connection (ETCPC) operation. Emergency echocardiography and computed tomography confirmed that the LHV entered the right atrium. Subsequently, the patient undergone completion of a staged TCPC with intra-atrial tunnel technique. This illustrative report highlights the essence of improving the preoperative accurate diagnosis to avoid unplanned reoperation in China, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Failure to identify anomalous LHV connection, in this case, will delay effective treatment past the optimal treatment time.

Double tunnel technique reduces technical failure during POEM in cases with severe submucosal fibrosis

Background and study aims Submucosal fibrosis (SMF) is one of the major reasons for technical failure during per-oral endoscopic myotomy (POEM). In this study, we aimed to evaluate the impact of double tunnel technique (DT-POEM) on the technical success of POEM in cases with severe SMF. Patients and methods Data from patients with achalasia in whom SMF was encountered during POEM from Jan 2013 to Jan 2020 at our center were retrospectively evaluated. The technical success and adverse events (AEs) were analyzed and compared between cases with SMF who underwent POEM via single tunnel versus DT-POEM technique. Results A total of 1,150 patients underwent POEM during the study period. There were 14 technical failures (1.2 %). SMF and severe SMF was found in 104 (9 %) and 21 (1.8 %) patients, respectively. The majority of patients (17, 80.9 %) with severe SMF had type I achalasia. Overall, technical failures occurred in 14 cases (1.2 %) and severe SMF resulted in the majority of the technical failures (10, 71.4 %). DT-POEM was utilized in 11 cases (52.4 %) with severe SMF. The median procedure time with DT-POEM was 150 minutes (85–210). There were no major AEs and DT-POEM was technically successful in all the patients. The technical success of POEM in cases with SMF improved after the adoption of the double tunnel technique (98.4 % vs 68.3 %, P = 0.0001). Conclusions Severe SMF is the most common reason for technical failure during POEM. DT-POEM improves the technical success rates of POEM in patients with severe SMF.