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

A 56-year-old Chinese man presented with giddiness and vertigo. Subsequent chest radiography showed the classic scimitar sign of an abnormal pulmonary venous return. Further evaluation with non-contrast computed tomography substantiated the finding of a partial anomalous venous drainage pattern and identified an associated rare lung anomaly, horseshoe lung. The imaging findings of scimitar syndrome and its association with horseshoe lung are reviewed.
Diagnosis, Differential ; Humans ; Lung ; abnormalities ; diagnostic imaging ; Male ; Middle Aged ; Radiography, Thoracic ; Scimitar Syndrome ; diagnostic imaging ; Tomography, X-Ray Computed

With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute. We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return.
Cardiopulmonary Bypass ; Dissent and Disputes ; Extracorporeal Membrane Oxygenation* ; Heart Diseases ; Humans ; Infant, Low Birth Weight ; Infant, Newborn* ; Infant, Premature ; Infant, Very Low Birth Weight ; Risk Factors ; Scimitar Syndrome* ; Weaning

BACKGROUND: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. METHODS: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015. All patients were newborns or infants who underwent in situ TAPVR repair without TCA. RESULTS: Four anatomic subtypes of TAPVR were included in this study: supracardiac (20 cases, 69.0%), cardiac (4 cases, 13.8%), infracardiac (4 cases, 13.8%), and mixed (1 case, 3.4%). The median follow-up period for all patients was 42.9 months. Two (6.9%) early mortalities occurred, as well as 2 (6.9%) cases of postoperative pulmonary venous obstruction (PVO). Preoperative ventilator care (p=0.027) and preoperative PVO (p=0.002) were found to be independent risk factors for mortality. CONCLUSION: In situ repair of TAPVR without TCA was associated with encouraging mid-term outcomes. Preoperative ventilator care and preoperative PVO were found to be independent risk factors for mortality associated with TAPVR repair.
Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Mortality ; Reoperation ; Retrospective Studies ; Risk Factors ; Scimitar Syndrome* ; Ventilators, Mechanical

53-year-old female was admitted to our institution with alternating atrial flutter and junctional bradycardia. The patient had undergone the Warden procedure to correct sinus venosus type atrial septal defect combined with partial anomalous pulmonary venous return, and ring tricuspid annuloplasty for severe tricuspid regurgitation. She also had persistent left superior vena cava (PLSVC). With the assistance of a 3D electroanatomic mapping system, catheter ablation therapy was used successfully to treat atrial flutter associated with a channel in the right atrial scar, and a pacemaker was implanted through the PLSVC because of resulting symptomatic bradycardia.
Atrial Flutter ; Bradycardia* ; Cardiac Surgical Procedures ; Catheter Ablation ; Cicatrix ; Female ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Humans ; Middle Aged ; Pacemaker, Artificial ; Scimitar Syndrome ; Tachycardia* ; Tricuspid Valve Insufficiency ; Vena Cava, Superior*

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.
Early Diagnosis ; Heart ; Heart Murmurs ; Humans ; Infant, Newborn ; Korea ; Recurrence ; Scimitar Syndrome* ; Siblings*

This paper is to compare dual-source computed tomography (DSCT) with trans-thoracic echocardiography (TTE) in the evaluation of anomalous pulmonary venous connection (APVC) to explore the advantages of DSCT. Thirty-one patients with APVC detected by DSCT were enrolled in this study. In this cohort, 16 patients underwent echocardiography followed by operative treatments; and among the remained 15 patients who did not received surgical treatment, echocardiography was performed in 10 patients. The numbers and drainage sites of anomalous pulmonary veins, and accompanied defects on DSCT and TTE were retrospectively analyzed. In terms of diagnosis of the numbers and the drainage sites of anomalous pulmonary veins, the accuracy of DSCT was 98% (49/50) and 93.8% (15/16), while the accuracy of echocardiography was 80% (40/50) and 56.3% (9/16), respectively. There were statistical differences between the two modalities (P < 0.05). Although the detection rates of accompanied defects were 89.1% (41/46) and 78.3% (36/46) for DSCT and echocardiography, respectively, no statistically significant difference was found between the two modalities (P > 0.05). The 15 patients without receiving surgical treatments were partial APVC confirmed by DSCT, but APVC was found in 40% (4/10) patients by echocardiography. In conclusion, DSCT could be superior to evaluating APVC in comparison with echocardiography, and should be recommended as an important procedure for preoperative assessment of the anomaly in patients with APVC.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Echocardiography ; Evaluation Studies as Topic ; Female ; Humans ; Infant ; Male ; Middle Aged ; Radiographic Image Interpretation, Computer-Assisted ; Retrospective Studies ; Scimitar Syndrome ; diagnostic imaging ; Tomography, X-Ray Computed ; methods ; Young Adult

Adolescent ; Female ; Heart Septal Defects, Atrial ; complications ; Humans ; Hypertension, Pulmonary ; complications ; Scimitar Syndrome ; complications ; Tomography, X-Ray Computed

Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlobular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.
Autopsy ; Biopsy ; Dilatation ; Humans ; Infant ; Lung ; Prognosis ; Pulmonary Veins ; Scimitar Syndrome

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.
Arteries ; Decision Making ; Diagnosis, Differential ; Ductus Arteriosus, Patent ; Early Intervention (Education) ; Emergencies ; Heart ; Heart Diseases ; Humans ; Hypertension, Pulmonary ; Hypoplastic Left Heart Syndrome ; Infant, Newborn ; Lung Diseases ; Parturition ; Referral and Consultation ; Scimitar Syndrome ; Sepsis