Total Anomalous Pulmonary Vein Connection Research Articles

Surgical Outcomes of Patients With Heterotaxy Syndrome - The Japanese Congenital Cardiovascular Surgery Database.

Cardiac malformations are a major component of heterotaxy syndrome and result in significant mortality and morbidity. This multicenter nationwide Japanese study evaluated mortality and morbidity after initial surgical palliation for patients with heterotaxy syndrome and determined predictors for mortality and morbidity among patients enrolled in the study. The Japanese Congenital Cardiovascular Surgery Database (JCCVSD) collects clinical data from 119 domestic institutions specializing in congenital heart disease, covering almost all major congenital heart surgery programs in Japan. Clinical data on preoperative, operative, and postoperative characteristics and survival data within 30 and 90 days were available from the JCCVSD database. Of the 561 patients with heterotaxy syndrome who underwent any of 8 specific initial cardiovascular surgeries, 45 (8.2%) and 75 (13.4%) had died at 30 and 90 days, respectively. Preoperative emergency transport, type of heterotaxy syndrome, low hospital volume, the repair of total anomalous pulmonary vein connection, and the repair of a common atrioventricular valve were identified as significant predictive factors for operative mortality. Improvements in some medical circumstances, such as fetal diagnosis and the patient transport system, will be needed to improve outcomes for severely ill patients with heterotaxy syndrome. This study describes early outcomes for the largest number of main cardiovascular surgeries to date in infants with heterotaxy syndrome.

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction: A Rare Case Report

Norwood procedure with right ventricle to pulmonary artery conduit: a single-centre 20-year experience.

The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex. A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed. Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing <2.5 kg at the time of the Norwood procedure, intact atrium septum, total anomalous pulmonary vein connection and more than mild atrioventricular regurgitation at the time of the Norwood procedure. Overall survival was 80.9%, 72.3% and 62.8% at 1, 5 and 20 years, respectively. Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.

Progression of vascular remodeling in pulmonary vein obstruction

ObjectivesPulmonary vein obstruction (PVO) frequently occurs after repair of total anomalous pulmonary vein connection with progression of intimal hyperplasia from the anastomotic site toward upstream pulmonary veins (PVs). However, the understanding of mechanism in PVO progression is constrained by lack of data derived from a physiological model of the disease, and no prophylaxis has been established. We developed a new PVO animal model, investigated the mechanisms of PVO progression, and examined a new prophylactic strategy. MethodsWe developed a chronic PVO model using infant domestic pigs by cutting and resuturing the left lower PV followed by weekly hemodynamic parameter measurement and angiographic assessment of the anastomosed PV. Subsequently, we tested a novel therapeutic strategy with external application of rapamycin-eluting film to the anastomotic site. ResultsWe found the pig PVO model mimicked human PVO hemodynamically and histopathologically. This model exhibited increased expression levels of Ki-67 and phospho-mammalian target of rapamycin in smooth muscle-like cells at the anastomotic neointima. In addition, contractile to synthetic phenotypic transition; that is, dedifferentiation of smooth muscle cells and mammalian target of rapamycin pathway activation in the neointima of upstream PVs were observed. Rapamycin-eluting films externally applied around the anastomotic site inhibited the activation of mammalian target of rapamycin in the smooth muscle-like cells of neointima, and delayed PV anastomotic stenosis. ConclusionsWe demonstrate the evidence on dedifferentiation of smooth muscle-like cells and mammalian target of rapamycin pathway activation in the pathogenesis of PVO progression. Delivery of rapamycin to the anastomotic site from the external side delayed PV anastomotic stenosis, implicating a new therapeutic strategy to prevent PVO progression.

Role of four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation in detecting fetal pulmonary veins.

Prenatal diagnosis of fetal total anomalous pulmonary vein connection (TAPVC) remains challenging for most screening sonographers. The purpose of this study was to evaluate the use of four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation (4D-HDFI) in identifying pulmonary veins in normal and TAPVC fetuses. We retrospectively reviewed and performed 4D-HDFI in 204 normal and 12 fetuses with confirmed diagnosis of TAPVC. Cardiac volumes were available for postanalysis to obtain 4D-rendered images of the pulmonary veins. For the normal fetuses, two other traditional modalities including color Doppler and HDFI were used to detect the number of pulmonary veins and comparisons were made between each of these traditional methods and 4D-HDFI. For conventional echocardiography, HDFI modality was superior to color Doppler in detecting more pulmonary veins in normal fetuses throughout the gestational period. 4D-HDFI was the best method during the second trimester of pregnancy in identifying normal fetal pulmonary veins. 4D-HDFI images vividly depicted the figure, course, and drainage of pulmonary veins in both normal and TAPVC fetuses. HDFI and the advanced 4D-HDFI technique could facilitate identification of the anatomical features of pulmonary veins in both normal and TAPVC fetuses; 4D-HDFI therefore provides additional and more precise information than conventional echocardiography techniques.

Neonatal screening to detect critical congenital cardiac disease. A revolution in pediatrics

There is solid evidence that demonstrate the usefulness of routine oxygen saturation testing in every apparently healthy newborn after 24 hours of life and before 48 hours. This procedure is known as “newborn screening for critical congenital heart disease” and serves to detect timely those congenital structural cardiac malformations with hypoxema, such as heart syndrome, pulmonary valve atresia, truncus arteriosus, total anomalous pulmonary vein connection, complete transposition of the great arteries, tetralogy of Fallot and tricuspid valve atresia. This test has been included in the mandatory neonatal screening panel of many countries and its generalization all over the world, seems imminent.

Contrast study of cardiac output and oxygen metabolism in infants with congenital heart disease after cardiopulmonary bypass

To conduct a contrast study of postoperative cardiac output and oxygen metabolism in infants with congenital heart disease undergoing cardiopulmonary bypass. Retrospective analysis was conducted for 55 case of congenital heart disease from January 2006 to January 2009 at our hospital. There were 34 males and 21 females. And they were divided into simple group (n = 30) and complex group (n = 25). In the simple group, all had pulmonary arterial hypertension and there were simple ventricular septal defect (VSD) (n = 15), atrial septal defect (ASD) + VSD (n = 9) and ASD + VSD + patent ductus arteriosus (PDA) (n = 6); in the complex group, there were tetralogy of Fallot (TOF) (n = 12), double outlet of right ventricular with pulmonary stenosis (DORV) (n = 8) and total anomalous pulmonary vein connection (TAPVC) (n = 5). All completed cardiopulmonary bypass procedures under venous injection and inhalation anesthesia. Cardiac outputs were measured by the thermodilution method with a 4 F Swan-Ganz floating catheter at operation completion and postoperative 4, 8, 12, 24, 48, 72 h. Arterial and mixed venous blood specimens were collected through radial artery and floating catheter for blood gas analysis. The parameters of cardiac index (CI), oxygen supply index (DO₂I), oxygen consumption index (VO₂I) and oxygen intake rate (O₂ER) were calculated with PHLIPS M: 8007 A. (1) At postoperative 8 h, ScVO₂ was minimal (simple group 68% ± 14%; complex group 65% ± 9%); and postoperative 12 h CI (L×min⁻¹×m⁻²) bottomed out (simple group 3.29 ± 0.65; complex group 2.88 ± 0.54); DO₂I (492 ± 153) ml×min⁻¹×m⁻² and VO₂I(138 ± 45) ml×min⁻¹×m⁻² were minimal in complex group. (2) DO₂I, VO₂I, O₂ER and ScVO₂ changed with CI and simple group was higher than complex group. (3) Postoperative CI showed a positive correlation with DO₂I, VO₂I, ScVO₂ and a negative correlation with O₂ER. The postoperative cardiac output decreases and oxygen metabolism becomes disordered in congenital heart disease. It is most obvious at postoperative 12 h. And complex CHD is more serious. Cardiac output should be actively boosted to improve tissue oxygen metabolism during an early postoperative period.

Impact of the Evolution of the Fontan Operation on Early and Late Mortality: A Single-Center Experience of 405 Patients Over 3 Decades

Postoperative mortality has decreased in patients undergoing the Fontan operation, and the determinants of such mortality may also have changed significantly. We conducted a study intended to focus on clarifying the determinants of mortality in 405 consecutive patients who had undergone a Fontan operation (62 patients after an atriopulmonary connection, 105 after an intra-atrial rerouting, and 238 patients after an extracardiac rerouting) between 1979 and 2010. The overall 1-year, 5-year, 10-year, and 15-year rates of survival were 87.1%, 84.6%, 83.4%, and 81.6%, respectively, and the type of procedure as well as heterotaxy syndrome, ventricular ejection fraction, and atrioventricular valve repair at the time of the Fontan operation were independent predictors of overall mortality (p < 0.05 for all). Heterotaxy syndrome and atrioventricular (AV) valve repair were independent predictors of early (less than 6 months postoperative) mortality and the type of procedure was an independent predictor of late (6 or more months postoperative) mortality (p < 0.05 to 0.01). In the era of intra-atrial rerouting, heterotaxy syndrome was the only independent predictor of total, early, and late mortality (p < 0.05 for all), whereas a low ejection fraction, AV valve repair, and repair of a total anomalous pulmonary vein connection, rather than heterotaxy syndrome, were independent predictors of total or early mortality or both (p < 0.05 for all) in the era of extracardiac rerouting. Even in the modern era of extracardiac rerouting in the Fontan operation, a low ventricular ejection fraction, AV valve dysfunction, or a total anomalous pulmonary vein connection remain significant risk factors for mortality in patients with a single-ventricle physiology.

Left cor triatriatum: A rare potential cause of total anomalous pulmonary vein connection obstruction

Total anomalous pulmonary venous connection (TAPVC) is a cardiac malformation in which there is no direct connection between any pulmonary vein and left atrium. It results from failure to develop the common pulmonary vein with persistence of the embryonic pulmonary-to-systemic venous connections. Left cor triatriatum (LCT) is a rare form of intra-atrial obstruction resulting from abnormal incorporation of the common pulmonary vein into the left atrium after its connection to the pulmonary venous plexus.

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Total anomalous pulmonary vein connection: Diagnosis, management, and outcome

Total anomalous pulmonary venous connection (TAPVC) is a congenital cardiac malformation that requires surgical therapy. The majority of patients will present either in the neonatal period or early infancy, with cyanosis and signs of cardiac failure (eg, tachypnea, feeding difficulties, diaphoresis, failure to thrive). Surgical repair is best carried out on diagnosis, as an elective procedure, to avoid potential long-term problems associated with right heart volume and pressure overload, or long-standing cyanosis. Occasionally, in severely sick patients with obstructed forms of pulmonary venous drainage, emergency surgical repair may be required. The operative mortality for isolated TAPVC using current management techniques is less than 10%, and the long-term outlook for the majority of patients is excellent. In contrast, patients with associated complex cardiac malformations have a less favorable prognosis, determined both by the need to palliate the associated defects and the often underdeveloped pulmonary vascular bed. The major long-term complication is pulmonary venous obstruction, which may be recurrent in up to 10% of patients. This requires aggressive management strategies, involving reoperation, and balloon angioplasty or stent implantation. The prognosis for these patients is determined by the number of vessels involved and the degree of stenosis, which can often be recurrent and progressive. Late tachy- and bradyarrhythmias have also been described, requiring antiarrhythmic drug therapy or pacemaker implantation, emphasizing the need for continued long-term follow-up of all patients with TAPVC.

Chest X-ray film patterns in children with isolated total anomalous pulmonary vein connection

Chest-X-ray films of 61 children with isolated total anomalous pulmonary vein connection (TAPVC) were re-evaluated retrospectively. A small heart silhouette with normal pulmonary perfusion and signs of pulmonary oedema is typical of TAPVC with obstruction. There is no typical pattern in TAPVC without obstruction. The "snowman" or "figure of eight" does not develop within the first months of life.