ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
ObjectiveTo evaluate the long-term clinical effect and risk factors of tricuspid valve replacement (TVR) as a relief treatment for adult patients with congenitally corrected transposition of the great artery (CCTGA).Method We retrospectively analyzed the clinical data of 47 adult patients with CCTGA who underwent tricuspid valve replacement in Fuwai Hospital between 2000 and 2017 year. There were 27 males and 20 females with operation age of 14–62 (38.8±13.5) years. Preoperative echocardiography showed moderate or more tricuspid regurgitation in all patients. The basic data of patients before and during operation were recorded. Survival was followed up by telephone and ultrasound report.ResultsThe average follow-up time was 6.5±3.7 years. The 1-year, 5-year and 10-year survival rate or the incidence of heart transplant-free was 94.6%, 90.5% and 61.7%, respectively. During the follow-up period, the long-term right ventricular ejection fraction of most patients (>90%) was still greater than or equal to 40%. Increased preoperative right ventricular end diastolic diameter (RVEDD) was a risk factor for death or heart transplantation (risk ratio 1∶11, P=0.04). The survival rate of patients with RVEDD (>60 mm) before operation was significantly reduced (P=0.032).ConclusionTVP is a feasible treatment for adult patients with CCTGA. The increase of preoperative RVEDD is a risk factor for long-term mortality.
ObjectiveTo summarize the treatment of a patient with congenital portosystemic shunt (CPS) complicated with hepatic focal nodular hyperplasia (FNH), and to explore the feasibility and safety of combined laparoscopy and interventional radiology therapy at the same time.MethodsThe clinicopathological data of a patient with CPS complicated with hepatic FNH who admitted to West China Hospital of Sichuan University in March 2019 was retrospectively analyzed.ResultsThe patient underwent laparoscopic liver nodule resection and digital subtraction angiography (DSA) guided jugular portal portosystemic shunt fistula embolization. The laparoscopic surgery operation time was 180 min and the intraoperative blood loss was 50 mL, and for interventional procedure was 230 min and 10 mL respectively. There were no complications after operation and the patient was successfully discharged on the 8th day after surgery. The patient was followed up for six months and in good condition.ConclusionsCPS patient should develop individualized treatment under the discussion of multidisciplinary cooperation group. The combination of laparoscopy and interventional technique can be minimally invasive and efficient to solve portal vein-avitary shunt fistula and benign hepatic nodules at the same time.
ObjectiveTo analyze the short-term outcomes of cardiac surgery via minimally invasive approach under thoracoscope in a single-center. Methods The clinical data of patients who underwent cardiac surgery via minimally invasive approach under thoracoscope between July 2017 and February 2022 were retrospectively analyzed. Results A total of 453 patients were enrolled, including 150 males and 303 females at an average age of 42.2±14.6 years. The main disease types included atrial septal defect in 314 patients, partial atrioventricular septal defect in 26 patients, and cardiac tumor in 105 patients. There was no death during the perioperative period. The mean operative time was 3.9±0.8 h, cardiopulmonary bypass time was 101.2±34.0 min, aortic occlusion time was 42.1±25.1 min, ventilator assistance time was 11.6±9.4 h, ICU stay time was 22.6±13.9 h and postoperative hospital stay was 6.0±1.7 d. Intraoperative and postoperative complications occurred in 18 (4.0%) patients, including 2 patients with conversion to sternotomy, 3 with incision expand, 3 with reoperation for bleeding, 1 with of redo operation, 4 with incision infection, 2 with respiratory insufficiency, 2 with cerebrovascular accident, and 1 with ventricular fibrillation. The follow-up time was 22.6±15.4 months, during which 1 patient died, 4 patients had moderate mitral regurgitation, 1 patient had mild-moderate mitral regurgitation, and 1 patient had mild-moderate tricuspid regurgitation. Conclusion Minimally invasive cardiac surgery under thoracoscope is safe with small invasions and few complications, and has satisfactory short-term outcomes.
ObjectiveTo explore the hemodynamic effects of inhaled nitric oxide (iNO) on postoperative hemodynamic in patients with cyanotic congenital heart disease (CHD) combined with decreased pulmonary blood flow.MethodsFrom 2014 to 2018, there were 1 764 patients who received corrective repair of cyanotic CHD with decreased pulmonary blood flow in the Department of Pediatric Cardiac Surgery of Fuwai Hospital. We included 61 patients with the ratio of right ventricular systolic pressure to systolic blood pressure (SBP) ≥75% after weaning from cardiopulmonary bypass. There were 41 males and 20 females, with the age of 20.5 (9.0, 39.0) months and weight of 12.5±7.8 kg. The patients were divided into two groups: a conventional group (33 patients, conventional therapy only) and a combined therapy group (28 patients, iNO combined with conventional therapy). The hemodynamics during the first 24 hours after iNO therapy and the in-hospital outcomes of the two groups were investigated and compared.ResultsThere was no statistical difference between the two groups in demographic characteristics and surgical parameters (P>0.05). The hemodynamic effects of iNO within 24 hours included the decrease in the vasoactive inotropic score (VIS, 21.6±6.6 vs. 17.3±7.2, P=0.020) along with the increase in blood pressure (SBP: 73.7±9.7 mm Hg vs. 90.8±9.1 mm Hg, P<0.001) , the decrease in central venous pressure (10.0±3.1 mm Hg vs. 7.9±2.1 mm Hg, P=0.020), the decrease in lactate (2.2±1.7 mmol/L vs. 1.2±0.5 mmol/L, P<0.001) and increase in urine output [2.8±1.7 mL/(kg·h) vs. 4.9±2.2 mL/(kg·h), P<0.001]. The decrease of VIS at 24 h after the surgery in the conventional therapy group was not statistically significant (22.1±7.9 vs. 20.0±8.5, P=0.232). Besides, we discovered that the need for renal replacement therapy (RRT) was less in the combined therapy group than that in the conventional therapy group, especially in the moderate complicated surgery [risk adjustment in congenital heart surgery (RACHS-1) ≤3] subgroup (9.5% vs. 40.7%, P=0.016).ConclusionIn pediatric patients after corrective repair of cyanotic and pulmonary blood follow decreased CHD with increased pulmonary vascular resistance, iNO combined with conventional therapy can improve the hemodynamics effectively. Compared with the conventional therapy, the combined therapy with iNO can decrease the VIS and the need for RRT, which is beneficial to the postoperative recovery of patients.
Objective To explore the feasibility of transcatheter closure of congenital heart disease (CHD) under the guidance of transthoracic echocardiography (TTE). Methods A total of 37 patients with CHD who received transcatheter closure under the guidance of transthoracic echocardiography from November 2013 through November 2015 in our hospital were recruited. There were 15 males and 22 females, aged 1 to 16 years. Among them 32 patients suffered atrial septal defect and 5 patients had patent ductus arteriosus. The transcatheter closure of CHD was performed under the guidance of TTE. The patients underwent echocardiography follow-up at one, three and six months after surgery. Results Closure devices were successfully implanted in 37 patients under TTE guidance. The procedure was simple and safe. During the follow-up, no severe complication such as valvular injury, pericardial effusion, residual shunt and peripheral vascular injury occurred. Conclusion Transcatheter closure of CHD under TTE guidance is a feasible method and worth further clinical application.
Objective To evaluate the functional and aesthetic evaluation of external fixator lengthening through plantar approach for fourth brachymetatarsia. Methods A retrospective analysis was conducted on 20 patients (23 feet) with fourth brachymetatarsia who met the selection criteria between January 2016 and January 2024, including 3 males and 17 females, with 8 left, 9 right, and 3 bilateral cases. The mean age was 24.7 years (range, 14-51 years). The preoperative metatarsal shortening length was (13.8±3.2) mm. The preoperative American Orthopaedic Foot and Ankle Society (AOFAS) forefoot score was 79.5±3.9, the visual analogue scale (VAS) score of appearance satisfaction was 1.7±0.8, and the appearance index (AI) score was 13.6±0.9. All patients underwent external fixator lengthening through plantar approach. The lengthening length of metatarsal bone, lengthening ratio, healing time, and healing index were recorded. Functional outcomes were assessed using the AOFAS forefoot score, VAS score of appearance satisfaction, and quality-of-life impact with AI questionnaire. Results All 20 patients were followed up 14-55 months with an average of 36.3 months. During the follow-up, complications occurred in 4 cases (17.4%), including 2 cases of metatarsophalangeal joint stiffness, which had no significant effect on the function and appearance. Delayed union of osteotomy occurred in 1 case (healed at 12 weeks after operation). Pin loosening occurred in 1 case and recovered after outpatient reinforcement. No complications related to plantar scar occurred. At last follow-up, the lengthening length of metatarsal bone was (13.9±3.1) mm, and the lengthening ratio was 25.8%±5.6%. All cases achieved bony union, with a mean healing time of (64.3±12.5) days and a healing index of (46.9±4.8) d/cm. At last follow-up, AOFAS score was 98.9±2.1, the VAS score of appearance satisfaction was 9.3±0.7, and the AI score was 0.6±0.8, which significantly improved when compared with those before operation (t=27.398, P<0.001; t=32.994, P<0.001; t=56.135, P<0.001). Conclusion External fixator lengthening through plantar approach is a safe and effective technique for fourth brachymetatarsia, achieving satisfactory functional and aesthetic outcomes.
Our country has made great progress in the surgical treatment of congenital heart disease, but after entering the new era, congenital cardiac surgery also presents some new features and trends. The quality of data of Guangdong congenital heart defects monitoring network was recognized by international organizations. We analyzed the data of the incidence of congenital heart disease, the rate of surgical treatment, the mortality of surgical procedures and the quality of surgical treatment in Guangdong province from Guangdong congenital heart defects monitoring network, and then accurately understood the development characteristics of congenital cardiac surgery. It is very helpful to clarify the regional status of congenital cardiac surgery, which is conducive to the development of a more reasonable surgical strategy for congenital heart disease, and finally promote the further development of congenital cardiac surgery in China.
Fibropolycystic liver diseases (FLDs) is a rare genetic disorder, including bile duct hamartomas, congenital hepatic fibrosis, polycystic liver disease, Caroli’s disease, and choledochal cysts. Fibropolycystic liver diseases has received little clinical attention and exhibits a variety of imaging manifestations, leading to a high likelihood of missed diagnosis and misdiagnosis. Through this case, we delineate the characteristic imaging manifestations of the disease and its underlying pathological mechanisms. Our objective is to enhance readers' comprehension of the disease and thereby reduce the rate of missed diagnosis and misdiagnosis of the disease.
ObjectiveThough investigating the causes of untreated congenital heart disease among children aged 3–14 years in Panzhihua city, to provide the basis for formulating intervention measures in the next step.MethodsA survey was conducted on the children aged 3 to 14 years in Panzhihua city from March 2015 to December 2017. The causes of untreated congenital heart disease were analyzed. Results Of 148 children with untreated congenital heart diseases, 82 were found to have congenital heart disease or heart murmur before screening. Among them, 59 parents (71.95%) thought that hospitalization was expensive; 42 parents (53.84%) thought that their children were asymptomatic and did not need treatment. Doctors suggested that 13 children (15.85%) be observed. Sixty-six patients of congenital heart disease were found for the first time in this screening, with an average age of 7.11±1.23 years. The patients in the rural population (n=44, 81.82%) were significantly more than the patients in the urban (n=12, 18.18%, P<0.01). Atrial septal defect ranked the first among the diseases, and 45 (68.18%) children did not regularly participate in child health care after birth.ConclusionMost families with untreated congenital heart disease in Panzhihua city think that the cost of treatment is high and there is no need for treatment. And most of them do not participate in child health care regularly. Atrial septal defect ranks first among the diseases. Free screening and treatment of congenital heart disease is of great significance in Panzhihua city.