OBJECTIVES: To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. METHODS: Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. RESULTS: Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). CONCLUSIONS TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.
Female ; Humans ; Pregnancy ; Echocardiography ; Heart Defects, Congenital/surgery* ; Pulmonary Veins/surgery* ; Retrospective Studies ; Scimitar Syndrome/surgery* ; Infant, Newborn

BACKGROUND: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardiopulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. MATERIAL AND METHOD: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2~41 days) and median body weight was 2,870 grams (ranges; 900~3,530 grams). Preoperative diagnoses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. RESULT: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002~0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26~140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7+/-7.4 mmHg, 53.7+/-11.4 mmHg, and 56.3+/-13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3+/-7.2, 21.0+/-8.4, and 21.2+/-8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin administration. CONCLUSION: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.
Acidosis ; Adult ; Aortic Coarctation ; Arteries ; Blood Pressure ; Body Weight ; Cardiopulmonary Bypass ; Child ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Double Outlet Right Ventricle ; Heart Defects, Congenital* ; Heart Diseases ; Humans ; Hypoplastic Left Heart Syndrome ; Postoperative Care ; Scimitar Syndrome ; Sepsis ; Shock* ; Shock, Septic ; Thoracic Surgery ; Vasodilation ; Vasopressins*

BACKGROUND: It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects.We reviewed our recent surgical experiences on congenital heart defect (other than patent ductus arteriosus)in low birth wei ght babi es.Material and METHOD: From September 1994 to February 2001,31 consecutive infants weighing 2500 g or less underwent cardiac surgery with (OHS group n=12)or without cardiopulmonary bypass (CHS group n=19).A retrospective study was carried out to evaluate short-and intermediate-term outcome.Mean gestational age and age at operation were 36.9 weeks(range,32.3-42weeks)and 32.1days (range,0-87days)respectively.Mean body weight at birth and operation were 1972g (range,1100-2500g)and 2105g (range,1450-2500 g)respectively.There was no difference between the two groups in age and body weight.Defects included ventricular septal defect (VSD)(n=3),VSD with arch anomaly (n=2),total anomalous pulmonary venous return (n=2),transposition of the great arteries (TGA)(n=2),truncus arteriosus (n=2),and univentricular heart with cor triatriatum (n=1)in OHS group,and coarctation of aorta (n=7),tetralogy of Fallot (TOF)(n=3),TOF with pulmonary atresia (n=3), multiple muscular VSDs (n=1),double outlet right ventricle (n=1),pulmonary atresia with intact ventricular septum (n=2),tricuspid atresia (n=1),and TGA with multiple VSD (n=1)in CHS group.13 patients (41.9%)were intubated pre-operatively. RESULT: There were 4 early deaths (<30 days);1 (8.3%)in OHS group and 3 (15.8%)in non-OHS group.All these early deaths were related to the pulmonary artery banding(PAB).There was no operative mortality in infants undergoing complete repair and palliative operations other than PAB.Delayed sternal closure was required in 3 patients.Prolonged postoperative mechanical ventilation (>7days)was required in 7 patients (58.3%)in OHS and 7 (38.8%)in CHS group.Late mortality occurred in 3 patients,two of which were non-cardiac.A patient in OHS group was documented to have neurologic sequelae.All the survivors except two are in NYHA class I. CONCLUSION: Complete repair and palliative operations other than PAB can be performed in low birth weight infants with low operative mortality and an acceptable intermediate-term result.However,about a half of the patients required long-term postoperative mechanical ventilation.
Aortic Coarctation ; Arteries ; Body Weight ; Cardiopulmonary Bypass ; Cor Triatriatum ; Gestational Age ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Mortality ; Parturition ; Pulmonary Artery ; Pulmonary Atresia ; Respiration, Artificial ; Retrospective Studies ; Risk Factors ; Scimitar Syndrome ; Survivors ; Thoracic Surgery* ; Ventricular Septum