Safe treatment of congenital left atrial appendage aneurysm using lateral thoracotomy on a 3-year-old patient.

Limited literatures report the management of congenital left atrial appendage aneurysm (LAAA) which is extremely rare. Chest X-ray firstly showed an enlarged left cardiac silhouette for a 3-year-old patient with pneumonia. Echocardiography and magnetic resonance imaging confirmed a large cyst attached to the left atrium. Aneurysmectomy was performed through lateral thoracotomy using step-by-step method and under the guidance of transoesophageal echocardiography. We aim to show the safety and efficacy of this approach applied to children associated with congenital LAAA.

Surgical Treatment of Loffler Endocarditis in an 8-Year-Old Girl.

BACKGROUND: Idiopathic hyper eosinophilic syndrome (HES) is a rare disease characterized by a sustained increase in eosinophilia. Heart involvement is called Loffler endocarditis. Loffler endocarditis is a serious complication of hyper eosinophilia syndrome, which is characterized by a special type of fibrotic endocarditis. Loffler endocarditis is an inflammatory cardiac condition characterized by eosinophilic infiltration in the heart. The overall prognosis for patients with Loffler endocarditis is very poor. METHODS: In this article we report an 8-year-old girl who was diagnosed as having Loffler endocarditis in thrombotic phase and was successfully treated with surgery. RESULTS: Our patient had a good prognosis during the half-year follow-up. She had no symptoms of heart failure and echocardiography findings were normal. CONCLUSION: The cardiac damage occurred in a three-stage process: the necrotic, thrombotic, and fibrotic stages. This unusual but sometimes life-threatening disease is often detected in the late phase, resulting in no curative strategy available to reverse the disease process. The overall prognosis of patients with Loffler endocarditis is very poor. Current treatments include anticoagulation and anti-eosinophils therapy, and surgery only in selected cases. Surgical treatment of HES in adolescents is very rare. The present case illustrates that with well-controlled peripheral eosinophilia, proper surgical treatment in selected patients can improve their prognosis in the near future but long-term follow-up is necessary.

Lipocalin-2 induced LDHA expression promotes vascular remodelling in pulmonary hypertension.

Aerobic glycolysis is involved in the pathogenesis of pulmonary hypertension (PH). The mechanisms by which glycolysis is increased and how it contributes to pulmonary vascular remodelling are not yet fully understood. In this study, we demonstrated that elevated lipocalin-2 (LCN2) in PH significantly enhances aerobic glycolysis in human pulmonary artery smooth muscle cells (PASMCs) by up-regulating LDHA expression. Knockout of Lcn2 or having heterozygous LDHA deficiency in mice significantly inhibits the progression of hypoxic PH. Our study reveals that LCN2 stimulates LDHA expression by activating Akt-HIF-1α signalling pathway. Inhibition of Akt or HIF-1α reduces LDHA expression and proliferation of PASMCs. Both Akt and HIF-1α play critical roles in the development of PH and are suppressed in the pulmonary vessels of hypoxic PH mice lacking LCN2. These findings shed light on the LCN2-Akt-HIF1α-LDHA axis in aerobic glycolysis in PH.

Long-term outcomes in children undergoing vascular ring division: a multi-institution experience.

OBJECTIVES: Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division. METHODS: A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed. RESULTS: The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up. CONCLUSIONS: The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.