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.
Congenital heart disease (CHD) is a series of structural cardiac abnormalities resulting from abnormal fetal heart development. With the prolongation of survival time, their cognitive function problems begin to be concerned. From fetus period to adulthood, people with complex CHD are more likely to have abnormalities in brains. Children with complex CHD have a significantly increased risk of developmental disorders in cognitive functions, such as intelligence, attention and psychosocial disorders. These diseases persist into their adulthood. Adults with CHD have earlier neurocognitive decline, poorer performance in intelligence, executive function, attention and academic achievement, and are more likely to have mood disorders, higher incidence of mental disorders and lower quality of life. This paper reviews the studies on cognitive function of adult patients with CHD from the dimension of the whole life cycle.
Bidirectional superior cavopulmonary anastomosis(BCPA)is a palliative method used in the single ventricular repair. It mainly includes bidirectional Glenn shunt and hemi-Fontan operation. The indications of BCPA are those as an intermediate option of total cavopulmonary anastomosis, partial biventricular or 1 1/2 ventricle repair and a practical approach to complex congenital heart surgery. The choise of age,influence on pulmonary artery maturation,remain of additional pulmonary flow,formation of collaters and time to Fontan are demand of study.
ObjectiveTo evaluate the possibility of monitoring regional tissue oxygen saturation by near-infrared spectroscopy (NIRS) for early predicting adverse events in patients with pulmonary atresia.MethodsTwenty-six patients aged under 3 months who were diagnosed with pulmonary atresia and admitted to cardiovascular intensive care unit in our hospital between January 2016 and May 2017, accepted regional tissue oxygenation (cerebral and splanchnic) by near-infrared spectroscopy. There were 19 males and 7 females at age of 2–89 days. A total of 625 times of heart rate, blood pressure, pulse saturation, regional tissue oxygenation, and 98 serum lactate were retrospectively analyzed. The relationship of the tissue oxygen saturation and clinical adverse events was explored.ResultsThe adverse event by routine monitoring was 69 (11.04%) person-time: isolated hypoxia in 27, hypoxia combined increased lactate in 16, hypotension in 6, hypotension combined increased lactate in 17, isolated increased lactate in 3. A reduction of 12.80% in cranial oxygen predicted the high probability of adverse events, with a sensitivity of 85.30% and a specificity of 87.00%. A reduction of 20.60% in splanchnic oxygen predicted the high probability of adverse event, with a sensitivity of 73.50% and a specificity of 91.2%. On average, the splanchnic oxygenation had fell 3 minutes before a reduction of blood pressure, or 45 minutes before an increase in lactate.ConclusionFor preoperative patients with pulmonary atresia, a fall of 12.80% in cranial oxygen saturations, or of 20.60% in splanchnic oxygen saturation, should attract clinician’s awareness.
Objective To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.
At present, the operative results of complex congenital heart disease are suboptimal which is closely correlated to the understanding of the anatomy and function of complex congenital heart disease, and operative techniques. With the further understanding to pathology and physiology of congenital heart disease, strategies and techniques in well-known operations and complex procedures have developed in recent years. Currently, designing and applying individual operative method in terms of patient’s characteristics of anatomy and physiology is very important trend. This article reviewed the advances of knowledgement and techniques in some representive complex congenital heart disease including complete atrioventricular septal defect, unifocalization for major aortopulmonary collateral arteries, transopsition of the great artery and Fontan type operation.
ObjectiveTo investigate the clinical efficacy and application value of percutaneous interventional treatment for structural heart diseases under guidance of ultrasound.MethodsThe clinical data of 1 010 patients with structural heart diseases treated by transcutaneous ultrasound-guided occlusion in our hospital from December 2, 2015 to December 31, 2019 were retrospectively reviewed, including 360 males and 650 females, aged 1-50 years. There were 692 patients of atrial septal defect (603 with central type, 9 combined with arterial catheter, 80 with ethmoid type), 116 patent foramen ovale, 25 ventricular septal defects (3 combined with atrial septal defect), 132 patent ductus arteriosus, 32 pulmonary valve stenosis (3 combined with atrial defect), 1 main pulmonary artery window, and 3 aneurysm rupture of aortic sinus. All patients were diagnosed by transthoracic echocardiography (TTE) before operation. Treatment was accomplished intraoperatively through TTE or transesophageal echocardiography (TEE) via the femoral artery or femoral vein. After operation, echocardiography, electrocardiogram and chest radiograph were reexamined.ResultsSatisfactory results were obtained in 1 005 patients, and 1 patient failed to seal the ventricular defect and was repaired under direct vision, occluder detachment occurred in 5 patients after operation (3 patients of atrial septal defects underwent thoracotomy for Amplatzer device and were repaired, 1 patient of atrial septal defects was closed after removing Amplatzer device, 1 patient of patent ductus arteriosus underwent thoracotomy for Amplatzer device and was sutured), mild pulmonary valve regurgitation occurred after balloon dilation in 2 patients with pulmonary stenosis, a small amount of residual shunt was found in 2 patients with ventricular defect, which disappeared after 3 months of follow-up, and 1 patient of right bundle branch block occurred and disappeared after 1 week. After follow-up of 1-24 months, 3 patients of ethmoidal atrial septal defect were reexamined with mild shunt. The occluder was in good position and the pressure difference of pulmonary valve was significantly reduced. There was no complication such as hemolysis, arrhythmia, embolism or rupture of chordae tendinae.ConclusionPercutaneous transfemoral artery and vein guided by TTE or TEE is safe and effective, with little trauma, no radiation or contrast agent damage, and has significant clinical efficacy and application values.
Objective To investigate the genotype and phenotype in patients with leber congenital amaurosis (LCA), and offer accurate genetic counseling and prenatal diagnosis for those families. Methods Three LCA patients and their parents were recruited for this study and received detailed collection of medical history and family history from March to August 2016. The three patients received fundus fluorescein angiography examination and their parents received slit-lamp microscope and indirect ophthalmoscopy examinations. DNA was extracted from the patients and their family members. Whole-exome sequencing method was used for genetic diagnosis and typing of the three LCA patients and their parents. Results The three patients with different clinical features had a definite clinical diagnosis of LCA. Patient 1 showed pale disc, attenuated vessels aroud the optic disc and the salt-and-pepper appearance of the retina, had the homozygous c.744.745insT (p.249, L>Ffs4) mutation inSPATA7. Patient 2 showed optic disc pallor and attenuated retinal vessels, had the heterozygous c.535G>A, p.A179T mutation inWFS1. Patient 3 showed pale disc, atrophic macular and retinal and choroidal degeneration, had the heterozygous mutation in CRB1, RPGRIP1, SPATA7. Conclusion LCA has characteristics of genetic heterogeneity and clinical and phenotypic diversity.
Objective To investigate the risk factors of prolonged postoperative mechanical ventilation for adult patients with atrioventricular septal defect (AVSD). Methods We retrospectively analyzed the clinical data of 76 patients with AVSD aged more than 18 years in our hospital from January 1, 2011 to December 31, 2017. The patients ventilated longer than 24 hours were described as a prolonged ventilation group (n=27) and the others as a normal group (n=49). There were 9 males and 18 females aged 32.22±9.64 years in the prolonged ventilation group, and 16 males and 33 females aged 35.98±11.34 years in the normal group. Perioperative variables between the two groups were compared and selected, and then analyzed by logistic regression analysis. Results The result of univariate analysis showed that there was a statistical difference in weight, preoperative pulmonary artery systolic pressure, duration of cardiopulmonary bypass, the level of postoperative platelet, hemoglobin, blood glucose, lactic acid and serum creatinine, postoperative maximum heart rate and postoperative infection rate between the prolonged ventilated group and the normal group. Multivarable logistic regression showed that preoperative pulmonary artery hypertension (OR=1.056, 95%CI 1.005 to 1.110, P=0.030), prolonged duration of cardiopulmonary bypass (OR=1.036, 95%CI 1.007 to 1.066, P=0.016) and the low postoperative hemoglobin level (OR=0.874, 95%CI 0.786 to 0.973, P=0.014) were the risk factors of prolonged postoperative mechanical ventilation. Conclusion Preoperative pulmonary artery hypertension, long duration of cardiopulmonary bypass and postoperative anaemia are the risk factors associated with prolonged postoperative mechanical ventilation.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.