Anomalous Pulmonary Venous Connection Research Articles

Partial anomalous pulmonary venous return with sinus venosus atrial septal defect: a case report

Partial anomalous pulmonary venous return with sinus venosus atrial septal defect: a case report

Outcome of obstructed total anamalous pulmonary venous connection (TAPVC) repair patients with milrinone versus milrinone and inhaled nitric oxide (INO): A prospective randomized observational study.

Obstructed total anomalous pulmonary venous connection (TAPVC) typically present with severe cardiovascular decompensation and requires urgent surgical management. Pulmonary arterial hypertension (PAH) is a major risk factor affecting mortality. Perioperative management focuses on providing inotropic support and managing potential pulmonary hypertensive episodes. Milrinone and inhaled nitric oxide (iNO) efficiently reduce pulmonary artery pressure (PAP) and help to improve the outcome. The aim was to determine the outcome of patients with high PAP with milrinone alone and a combination of iNO and milrinone. After ethical committee approval, the study was conducted over a period of 3 years in 80 patients with obstructed TAPVC repair. A total of 80 patients having severe PAH (supra systemic arterial pressure) randomly divided into two groups with 40 patients in each (M & MN). Group M (milrinone) patients received milrinone and Group MN (milrinone & iNO) patients received both milrinone (after opening aortic cross clamp) and iNO (post operative ICU). Ventilation time, hospital stay, ICU stay, complications, in hospital mortality were compared between both groups. Ventilation time, Intensive Care Unit (ICU) stay, hospital stay for group M was 8.02 ± 5.74 days, 11.25 ± 7.33 day, 14.92 ± 8.55 days, respectively, and for group MN was 5.02 ± 1.78 days, 8.27 ± 3.24 days, 10.3 ± 3.18 days, respectively. In hospital mortality for group M and MN was 10% and 2.5%, respectively. P value for each variable was significant < 0.05 (except mortality). Most of the patients with obstructed TAPVC had severe PAH. Management of severe PAH with a combination of milrinone with iNO had a better outcome than milrinone alone.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

Multiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment.

We report a rare congenital heart disease characterized by multiple ventricular septal defects associated to anomalous systemic and pulmonary venous returns, marked apical myocardial hypertrophy of both ventricles and of right outflow, and hypoplastic mitral anulus. Multimodality imaging is mandatory to assess anatomical details.

Palliation of a Heterotaxy Single Ventricle Neonate with Pulmonary Atresia and Obstructed TAPVR

Patients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis.

Total Anomalous Pulmonary Venous Connection: Surgical Outcomes and Risk Factors for Postoperative Pulmonary Vein Obstruction.

Total anomalous pulmonary venous connection (TAPVC) is a fatal congenital cardiac anomaly that requires urgent surgical intervention. The development of postoperative pulmonary vein obstruction (PVO) negatively impacts long-term survival. The present study aimed to evaluate the surgical outcomes of TAPVC repair and risk factors associated with postoperative PVO. Patients who underwent primary TAPVC repair at our institute between 2004 and 2022 were retrospectively enrolled, and those with right atrial isomerism and single ventricle physiology were excluded. Factors associated with survival and postoperative PVO were analyzed. A total of 116 patients were enrolled in the present study. The early mortality rate was 6.9%. Nineteen patients (16.4%) developed postoperative PVO within a median time of 59 days of the primary repair, of whom 10 were successfully relieved without any recurrent obstruction. In long-term follow-up, patients with postoperative PVO had significantly lower long-term survival rates than those without postoperative PVO [57.9%, 95% confidence interval (CI) = 34.8-79.5%; vs. 90.4%, 95% CI = 83-96.6% at 10 years, p < 0.001]. Risk factors for postoperative PVO development included lower body weight, younger age, preoperative mechanical ventilation, preoperative inotrope use, and emergency operation. Postoperative PVO was significantly associated with a higher long-term mortality rate after primary TAPVC repair, with the risk being higher in patients with critical preoperative status. The long-term outcome was good for patients in whom the obstruction was successfully relieved. Early detection and prompt intervention for postoperative PVO after TAPVC repair can improve overall survival in these patients.

Fetal left-atrial posterior-space-to-diagonal ratio at 17-37 weeks' gestation for prediction of total anomalous pulmonary venous connection.

To develop normative data for the left-atrial posterior-space-to-diagonal (LAPSD) ratio and post-left atrium space (PLAS) index in fetuses from 17 to 37 weeks' gestation, and determine the optimal cut-offs of the LAPSD ratio and PLAS index to discriminate between normal fetuses and those with total anomalous pulmonary venous connection (TAPVC). This was a prospective cross-sectional assessment of 428 structurally normal fetuses between 17 and 37 weeks' gestation and a retrospective study of 27 TAPVC fetuses. The fetal LAPSD ratio was calculated by dividing the left atrium-descending aorta distance (LDD) by the left atrial diagonal diameter (LA). The PLAS index was calculated as the ratio of the LDD to the descending aorta diameter (DA). Pearson's correlation analysis was used to examine the correlation of cardiac parameters with gestational age (GA) and fetal somatic growth. The PLAS index and LAPSD ratio were compared between the normal and TAPVC groups to assess their usefulness in the prenatal diagnosis of TAPVC. LDD, LA and DA measures showed moderate to strong positive correlation, whereas both the LAPSD ratio and PLAS index showed a slight decrease with increasing GA and biometric variables. The fetal LAPSD ratio and PLAS index in TAPVC cases were significantly greater compared with those of fetuses with a normal heart (both P < 0.001). There were no significant differences in the PLAS index and LAPSD ratio between the isolated and complex TAPVC groups (both P = 1). No significant associations of the PLAS index and LAPSD ratio with fetal gender, four-chamber view type (apical, basal or lateral) or TAPVC type were found. Using values of ≥ 0.35 for the LAPSD ratio and of ≥ 1 for the PLAS index exhibited similar excellent diagnostic performance, with a sensitivity of 100% and specificity of 97.0% or 95.1% for detecting TAPVC. Our study demonstrates that the LAPSD ratio is a practical and effective screening tool for diagnosing fetal TAPVC, similar to the PLAS index. Incorporating these parameters into routine cardiac scanning may enhance the prenatal detection of TAPVC. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Total Anomalous Pulmonary Venous Return.

Supplemental material is available for this article.

Safety and efficacy of balloon angioplasty compared to stent-based-strategies with pulmonary vein stenosis: A systematic review and meta-analysis.

Pulmonary vein stenosis (PVS) is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous re-vascularization strategies of pulmonary vein balloon angioplasty (PBA) or pulmonary vein stent implantation (PSI). To study the safety and efficacy outcomes of PBA vs PSI in all patient categories with PVS. We performed a literature search of all studies comparing outcomes of patients evaluated by PBA vs PSI for PVS. We selected all published studies comparing PBA vs PSI for PVS with reported outcomes of restenosis and procedure-related complications in all patient categories. In adults, PVS following atrial fibrillation ablation and in children PVS related to congenital etiology or post-procedural PVS following total or partial anomalous pulmonary venous return repair were included. The patient-centered outcomes were risk of restenosis requiring re-intervention and procedural-related complications. The meta-analysis was performed by computing odds ratios (ORs) using the random effects model based on underlying statistical heterogeneity. Eight observational studies treating 768 severe PVS in 487 patients met our inclusion criteria. The age range of patients was 6 months to 70 years and 67% were males. The primary outcome of the re-stenosis requiring re-intervention occurred in 196 of 325 veins in the PBA group and 111 of 443 veins in the PSI group. Compared to PSI, PBA was associated with a significantly increased risk of re-stenosis (OR 2.91, 95%CI: 1.15-7.37, P = 0.025, I 2 = 79.2%). Secondary outcomes of the procedure-related complications occurred in 7 of 122 patients in the PBA group and 6 of 69 in the PSI group. There were no statistically significant differences in the safety outcomes between the two groups (OR: 0.94, 95%CI: 0.23-3.76, P = 0.929), I 2 = 0.0%). Across all patient categories with PVS, PSI is associated with reduced risk of re-intervention and is as safe as PBA and should be considered first-line therapy for PVS.

Repair of Partial Anomalous Pulmonary Venous Connection to the Azygous Vein via Right Thoracotomy.

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism (P < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism (P = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.

Critical congenital heart disease beyond HLHS and TGA: neonatal brain injury and early neurodevelopment.

Characterization of brain injury and neurodevelopmental (ND) outcomes in critical congenital heart disease (cCHD) has primarily focused on hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). This study reports brain injury and ND outcomes among patients with heterogeneous cCHD diagnoses beyond HLHS and TGA. This prospective cohort study included infants with HLHS, TGA, or heterogenous "Other cCHD" including left- or right-sided obstructive lesions, anomalous pulmonary venous return, and truncus arteriosus. Brain injury on perioperative brain MRI and ND outcomes on the Bayley-II at 30 months were compared. A total of 218 participants were included (HLHS = 60; TGA = 118; "Other cCHD" = 40, including 8 with genetic syndromes). Pre-operative (n = 209) and post-operative (n = 189) MRI showed similarly high brain injury rates across groups, regardless of cardiopulmonary bypass exposure. At 30 months, participants with "Other cCHD" had lower cognitive scores (p = 0.035) compared to those with HLHS and TGA, though worse ND outcome in this group was driven by those with genetic disorders. Frequency of brain injury and neurodevelopmental delay among patients with "Other cCHD" is similar to those with HLHS or TGA. Patients with all cCHD lesions are at risk for impaired outcomes; developmental and genetic screening is indicated. This study adds to literature on risk of brain injury in patients with critical congenital heart disease (cCHD) diagnoses other than hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), a heterogenous cohort of patients that has often been excluded from imaging studies. Children with cCHD beyond HLHS and TGA have similarly high rates of acquired brain injury. The high rate of neurodevelopmental impairment in this heterogenous group of cCHD diagnoses beyond HLHS and TGA is primarily driven by patients with comorbid genetic syndromes such as 22q11.2 deletion syndrome.

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. Case resolution

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. Case resolution

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. How would I approach it?

Percutaneous treatment of partial anomalous pulmonary venous connection with dual drainage. How would I approach it?

Assessment of left atrial deformation in patients with total anomalous pulmonary venous connection by two-dimensional speckle-tracking echocardiography.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by impaired left ventricle growth and diastolic dysfunction. We hypothesized that the patients with TAPVC reduced blood flow into the left heart prenatally could affect left atrium (LA) not just growth but function. We compared the age-related changes in LA deformation using two-dimensional speckle-tracking echocardiography (2DSTE) in Patients with TAPVC. This single-center, retrospective cohort study was conducted on consecutive isolated TAPVC patients who underwent neonatal surgery between January 1, 2009 and January 1, 2022. The LA datasets in TAPVC patients were analyzed before surgery (n = 28) and follow-ups at 1-2 (n = 24) and 5-7years of age (n = 13) and compared with those of age-matched healthy controls (January 2009-2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) strains. LA pressure was evaluated by periodic follow-up catheterization after repair. Compared to the controls, the TAPVC patients had significantly smaller LA maximum volume preoperatively, and with age, the LA maximal volumes reached normal levels, while the LA minimal volumes were larger. All 2DSTE-determined LA strains showed significant reductions at all time points in the TAPVC group compared to those in the control (median εR, εCD, and εCT; before surgery: 17.0% vs. 26.0%, 12.9% vs. 15.9%, and 6.3% vs. 10.4%; follow-up at 1-2years: 30.0% vs. 45.7%, 23.2% vs. 29.6%, and 6.1% vs. 16.3%; follow-up at 5-7years: 31.2% vs. 43.1%, 25.0% vs. 31.2%, and 5.2% vs. 10.8%, respectively; p < 0.05). Only εCT did not represented a significant change over time even though after correction of blood flow (median εCT: 6.0% → 5.9%). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. This study showed that nevertheless maximum volume of LA was recovered within the normal range, reduced LA strains, especially contractile function lasted from birth even after repair in Patients with TAPVC.

Capnography for catheter location confirmation in minimally invasive surfactant administration.

Minimally Invasive Surfactant Treatment (MIST) is a common method for administering surfactant as a treatment for respiratory distress syndrome. However, tracheal catheter placement can be difficult to confirm. We assessed the presence of carbon dioxide (CO2) in tracheal and esophageal gas aspirated using CO2 detector. Retrospective arm: 20 infants, MIST catheter placement was assessed with a CO2 detector in two techniques and confirmed with clinical response. Prospective arm-10 infants, aimed to check for CO2 presence in aspirated esophageal gas during routine nasogastric tube insertion. Retrospective arm: All infants had positive capnography. One infant that had no clinical response to MIST was diagnosed with total anomalous pulmonary venous return. All 10 infants of the prospective arm had a Negative capnography (P < 0.001, Fisher's exact test). Readily available CO2 detectors can distinguish between tracheal and esophageal placement of MIST catheters prior to MIST.

Infracardiac obstructive total anomalous pulmonary venous return with ventricular septal defect: a rare anomaly

The infracardiac variant of total anomalous pulmonary venous return occurs only rarely and is frequently obstructive in nature, thereby presenting in the early days of life [1]. An atrial septal defect is present in most cases and is necessary for survival, but a ventricular septal defect is extremely rare; only a few cases have been reported. Surgery is the only treatment option but carries a high risk of morbidity and mortality.

364 - Partial anomalous pulmonary venous return in the setting of an inferior sinus venosus atrial septal defect and the utility of three dimensional echocardiography

364 - Partial anomalous pulmonary venous return in the setting of an inferior sinus venosus atrial septal defect and the utility of three dimensional echocardiography

Early Mortality in Infants Born with Neonatally Operated Congenital Heart Defects and Low or Very-Low Birthweight: A Systematic Review and Meta-Analysis.

Mortality outcomes of children with isolated neonatally operated congenital heart defects (CHDs) born with a low (LBW), moderately low (MLBW) or very-low birthweight (VLBW) remain ambiguous. We searched Medline and Embase (inception until October 2021) and included studies that evaluated early mortality. The risk of bias was assessed using the Critical Appraisal Skills Program cohort checklist. Meta-analysis involved random-effects models. We explored variability in mortality across birthweight subgroups, CHD types, and study designs. From 2035 reports, we included 23 studies in qualitative synthesis, and the meta-analysis included 11 studies (1658 CHD cases), divided into 30 subcohorts. The risk of bias was low in 4/11 studies included in the meta-analysis. Summary mortality before discharge or within one month after surgery was 37% (95%CI 27-47). Early mortality varied by birthweight (VLBW 56%, MLBW 15%, LBW 16%; p = 0.003) and CHD types (hypoplastic left heart syndrome (HLHS) 50%, total anomalous pulmonary venous return (TAPVR) 47%, transposition of the great arteries (TGA) 34%, coarctation of the aorta (CoA) 16%; p = 0.13). Mortality was higher in population-based studies (49% vs. 10%; p = 0.006). One-third of infants born with neonatally operated isolated CHDs and LBW, MLBW, or VLBW died within 30 days after surgery. Mortality varied across infant and study characteristics. These results may help clinicians assess neonatal prognosis. PROSPERO registration CRD42020170289.