Type Of Total Anomalous Pulmonary Venous Connection Research Articles

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults.

Patients with total anomalous pulmonary venous connection (TAPVC) generally have symptoms during the neonatal period and infancy, and the fatality rate is extremely high. Most patients do not survive to adulthood. This study analyzed the clinical and transthoracic echocardiographic (TTE) manifestations of adult patients with TAPVC, summarized the echocardiographic characteristics of TAPVC, and identified the factors influencing pulmonary hypertension. Data from adult patients with TAPVC from Beijing Anzhen Hospital, China, were retrospectively collected for analyses, including sex, age, history of gestation, clinical manifestations, echocardiographic parameters, and blood oxygen levels. Patients were grouped for comparative analyses based on their pulmonary artery systolic pressure (PASP) (≥60 vs. <60 mmHg); 32 atrial septal defect (ASD) patients were included as a control group. (I) Sixteen patients were identified with TAPVC (11 women and 5 men; mean age: 32.2±9.5 years), including 8, 4, and 4 patients with supra-cardiac, mixed, and intracardiac type TAPVC, respectively. Furthermore, 10 patients had moderate or severe tricuspid regurgitation, and 6 had a PASP of ≥60 mmHg. Echocardiography misdiagnosed 2 patients with an ASD. (II) The TAPVC group patients had a smaller left atrium (LA) and a lower aorta/pulmonary artery ratio than ASD-only group patients. However, the right ventricular diameter (RVd) and right atrium were larger in patients with TAPVC than in those with only ASD. (III) The RVd was larger and the LA was smaller in patients with a PASP of ≥60 mmHg than in those with a PASP of <60 mmHg. (IV) Of those with a PASP of ≥60 mmHg, TAPVC patients had a smaller LA and a larger RVd than those with only ASD. (V) Pregnancy affected the PASP (adjusted odds ratio: 15.000, 95% confidence interval: 1.031-218.300, P=0.047). (VI) Echocardiography indicated that TAPVC patients with ASD had a right to left shunt at the atrial level and the pulmonary vein (PV) was not connected to the LA. Searching for the PV by TTE is necessary for patients with ASDs, which may help avoid misdiagnosis. Moreover, pregnancy affects the PASP. Patients with TAPVC may present with a larger right heart, smaller LA, and lower aorta/pulmonary artery ration than those with only ASD.

Total Anomalous Pulmonary Venous Connection with Persistent Left Superior Vena Cava: A Retrospective Surgical Series

Introduction: Total anomalous pulmonary venous connection (TAPVC) is a common cyanotic congenital heart disease seen in developing countries at tertiary referral centers. Association with persistent left superior vena cava (LSVC) without innominate vein (H-connection) is an extremely rare condition which makes the surgical correction extremely challenging. Materials and Methods: This was a retrospective case record review of 18 patients with TAPVC in the presence of LSVC without innominate vein (without H-connection) at our institution from January 2007 to February 2021. We detail our experience of our modified surgical technique for this complex anatomy using various modifications for each type of TAPVC with LSVC in the absence of innominate vein. Results: Out of 18 patients of TAPVC with LSVC, 17 patients survived and are being followed up regularly. There was one mortality (5.5%) in a 5-month-old infant with double-outlet right ventricle with ventricular septal defect with severe pulmonary arterial hypertension with infracardiac TAPVC who died in intensive care unit on the 5th postoperative day. Conclusion: In our experience, the modified surgical technique for treating this complex variant of TAPVC is especially useful for favorable long-term outcomes with minimal incidence of recurrent pulmonary venous obstruction.

The Clinical Characteristics of 88 Patients with Total Anomalous Pulmonary Venous Connection and Risk Factors Associated with Early Postoperative Death.

ObjectiveThis study aimed to analyze the outcomes and risk factors of early postoperative death (within 30 days after surgery) in a single-center after repair of total anomalous pulmonary venous connection (TAPVC).MethodsThe clinical data of 88 children who had been diagnosed with TAPVC and underwent radical operation in the Shandong Provincial Hospital Affiliated with Shandong First Medical University (China) from January 2015 to July 2021 were retrospectively analyzed. All the patients were divided into the survival group (n = 81) and the death group (n = 7) for the analysis of preoperative and postoperative clinical data. The variables associated with early postoperative death were statistically analyzed to obtain the risk factors for early postoperative death of TAPVC.ResultsOf the 88 patients included in this study, 7 (7.95%) patients died early, including 4 supracardiac and 3 infracardiac cases. Recurrent pulmonary vein obstruction occurred in 2 patients after discharged from hospital, and both were intracardiac TAPVC. Delayed death occurred in 2 children, both of which were intracardiac TAPVC cases. According to univariate analysis, the risk factors statistically significantly associated with the early postoperative death included infracardiac type (P = 0.08), preoperative maximum pulmonary vein flow velocity (P = 0.031), preoperative mechanical ventilation (P = 0.043), preoperative maximum pulmonary artery pressure (P = 0.000), intraoperative cardiopulmonary bypass time (P = 0.003) and intraoperative aortic cross-clamp time (P = 0.000).ConclusionInfracardiac type of TAPVC, preoperative maximum pulmonary vein flow velocity, preoperative mechanical ventilation, preoperative maximum pulmonary artery pressure, intraoperative cardiopulmonary bypass time and aortic cross-clamp time are the risk factors for early postoperative death.

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis

OBJECTIVESA meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC).METHODSData bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC. Publications were screened by 2 authors independently for criteria inclusion, methodological quality assessment and data extraction. The Newcastle-Ottawa Scale and the Agency for Healthcare Research and Quality checklist were obtained to assess the quality of the studies. Data were pooled by the random effect model or the fixed effect model according to the heterogeneity test.RESULTSA total of 16 studies (2,385 participants) were included in the meta-analysis. All included studies were retrospective studies. Six potential risk factors were pooled, 5 of which were significantly associated with postoperative PVO. Patients with preoperative PVO were more likely to suffer from postoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01]. Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. A mixed type TAPVC plays a critical role in postoperative PVO [OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04]. Inverse variance analysis showed that longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO. Heterotaxy [OR = 1.18, 95% CI = 0.13, 10.45, P = 0.88] was not statistically significant as a risk factor for postoperative PVO.CONCLUSIONSThis meta-analysis may provide a perspective on the risk factors for postoperative PVO after TAPVC, thus leading to more studies predicting postoperative PVO after TAPVC with our findings.

Predictors of mortality and morbidity in total anomalous pulmonary venous connection with biventricular physiology: A 10-year Indian single centre experience of 492 patients.

Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains associated with significant mortality despite advances in intra-operative and postoperative management. We retrospectively analyzed 492 consecutive TAPVC patients with biventricular physiology, who were operated at our centre, with regard to predictors of mortality, morbidity, and intermediate-term outcomes. A total of 492 TAPVC patients with biventricular physiology were operated at our centre from August 2009 to November 2019. Their medical records were reviewed and were followed up during March-April 2020 for any symptoms of cardiac disease. Of 492, 302 (61.38%) were healthy at follow-up, 29 (5.89%) had postoperative mortality, 23 (4.67%) had mortality during the follow-up period, and 138 (28.05%) were lost to follow up. Age <1 month and weight <2.5 kg were associated with higher mortality with odds ratios (OR) of 6.37 and 5.56, respectively. There was no difference in mortality in different types of TAPVC. Obstructed TAPVC was associated with higher mortality with OR of 3.05. Acute kidney injury requiring peritoneal dialysis and sepsis were associated with higher mortality with ORs of 10.17 and 3.29, respectively. All follow-up mortality occurred in <1 year from the index operation. Anastomotic gradients were significantly higher in patients who died. Although peri-operative TAPVC mortality has reduced, mortality on follow-up continues to occur and is partly due to the obstruction of pulmonary venous pathway. Meticulous follow-up holds the key in further reducing the mortality. Larger studies are needed for the identification of risk factors for pulmonary venous obstruction and its preventive strategies.

Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning. To evaluate the role of cardiac computed tomography (CT) in this setting. Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo). Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre- and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in >1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p < .00001, Fisher exact test). Cardiac CT is essential to evaluate pre- and postoperative TAPVC in SV for surgical decision making and long term follow up.

Mid-Term Results and Risk Factors For 10 Years of Functional Single Ventricle Associated With Total Anomalous Pulmonary Venous Connection

There are few surgical treatment results in elderly patients with functional single ventricle (FSV) and total anomalous pulmonary venous connection (TAPVC). We retrospectively analyzed 10 years of mid-term surgical treatment results and risk factors of these age-specific people. Between March 2008 and December 2018, 43 consecutive patients with FSV and TAPVC received initial surgical palliation in our center. There were 20 cases of supracardiac TAPVC, 21 of cardiac type, and two cases of mixed type. Initial surgical palliation procedures involved pulmonary artery banding (PAB) for patients, modified Blalock-Taussing shunt (mBTs) for five patients, and bidirectional Glenn (BDG) for 34 patients. TAPVC repair was performed in 12 patients during BDG. The 1-year and 5-year overall survival rates were 69.7% and 62.8%, respectively. In TAPVC repair group and non-TAPVC repair group, the 1-year overall survival rates after initial surgical palliation were 41.7 and 80.5%, respectively, and the 3-year ones were 25% and 77%, respectively. There were significant differences in the type of TAPVC (P < 0.001), preoperative pulmonary venous obstruction (P = 0.001), and overall mortality (P = 0.001) between these two groups. Cox univariate and multivariable analysis indicated concomitant TAPVC repair was the only risk factor for mortality. The mid-term results of surgical treatment of FSV and TAPVC, especially for patients who underwent concomitant TAPVC repair, remain poor. TAPVC repair may be a priority over single-ventricular palliative surgery for patients with FSV and TAPVC.

Strategy of delayed repair of total anomalous pulmonary venous connection in right atrial isomerism and functional single ventricle

ObjectiveRepair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. MethodsTwenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). ResultsPatients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan–Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. ConclusionsFor neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

BIDIRECTIONAL GLENN SHUNT AND REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION IN A 3 YEARS OLD CHILD WITH HETEROTAXY SYNDROME: A CASE REPORT

Right Atrial Isomerism (RAI) and total anomalous pulmonary venous connection (TAPVC) is a rare condition with very high mortality in neonatal period, but outcomes may be better in older children. A cyanotic 3 years old boy with dyspnea and growth retardation diagnosed with RAI and supracardiac type of TAPVC undergone a successful repair of TAPVC and simultaneous Glenn shunt. Older patients with RAI may be a distinct, more suitable group for surgical repair.

A patient with atrial septal defect with cyanosis with diagnostic dilemma turned out to be a case of Total Anomalous Pulmonary Venous Connection (TAPVC)

Total anomalous pulmonary venous connection (TAPVC) is a congenital cyanotic heart disease where all 4 pulmonary veins do not open directly to left atrium. There are 4 types of TAPVC. Supra cardiac type forms a confluence and may open to Innominate vein or SVC. Cardiac type usually opens to coronary sinus. Infracardiac type opens to hepatic veins or other veins. Mixed type is the combination of others. Of these 4 types infracardiac type is most vulnerable and presents with early features of cyanosis, pulmonary hypertension, pulmonary vein obstruction. We present the case of an 18 year old lady with atrial septal defect (ASD) with cyanosis. Pre operative echo showed ASD with 3rd chamber behind LA, CT angiogram revealed large ASD, with tongue like extended chamber in posteromedial aspect of RA. Preoperative angiogram report was inconclusive. Despite the diagnostic dilemma, we took the challenge and the patient went for open heart surgery. The patient recovered well and discharged on 10 th post operative day. Post operative echocardiogram is encouraging and she is doing fine. KYAMC Journal.2021;12(02): 107-110

A single center, retrospective analysis of total anomalous pulmonary venous connection repair early outcome at a tertiary care center in India.

Context:In recent years, increasing awareness and early detection has made total anomalous pulmonary venous connection (TAPVC) a relatively common congenital heart condition presenting to children’s heart centers in India. The condition was associated with significant morbidity and mortality in the past due to various reasons. Improvement in perioperative management has markedly changed the outcomes of TAPVC even in a developing country.Material and Methods:All patients with TAPVC operated between June 2013 and February 2018 at our center were included in the study. Post repair 30-days mortality and morbidity were analyzed.Results:A total of 166 patients were divided into supracardiac (91), infracardiac (45), cardiac (18), and mixed type (12). It also divided our cohort into obstructed and unobstructed types. The duration of inotrope usage in the obstructed group was significantly higher compared to the unobstructed group. Statistically, significant difference was noticed for the duration of ventilation 85.17 ± 80.94 h in obstructed type versus 49.23 ± 60.7 h in the unobstructed group, and in ICU stay (days) in obstructed (9.64 ± 5.96) and unobstructed group (6.29 ± 5.12). The morbidity parameters such as duration of an inotrope, ventilation, and length of ICU stay had a negative correlation between body surface area (BSA) of the patient but no correlation in respect to duration of CPB and ACC time. Mortality was found to be higher in lower BSA, infracardiac type (7/9), and obstructed variants of TAPVC (9/9) patients.Conclusions:Longer duration of inotrope usage, mechanical ventilation, and ICU stay were seen in obstructed TAPVC in comparison to unobstructed TAPVC patients. Duration of CPB or aortic cross-clamp had no effects on morbidity parameters. In our cohort of TAPVC patients, lower BSA was strongly associated with the longer requirement of inotropes, prolong ventilation time, and ICU stay. The risk factors for mortality in our study include lower BSA, infracardiac, and obstructed type of TAPVC.

Mixed type total anomalous pulmonary venous connection: Early results and midterm outcomes of surgical correction.

The morphological heterogeneity of anomalous pulmonary venous drainage in mixed type total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical repair resulting in high mortality in these patients. A retrospective review of 14 patients with mixed type TAPVC undergoing biventricular repair between January 2012 and December 2019 was conducted. A descriptive analysis was done, highlighting the anatomic variation, diagnostic and surgical approach, and surgical outcomes in these patients. The most common anatomic pattern was "3 by 1" (79%) followed by "2 by 2" (21%). The correct diagnosis by transthoracic echocardiography was made in 10 (71%) of the 14 patients. In contrast, preoperative computed tomographic (CT) angiography was performed in 10 patients and correct diagnosis was obtained in 8 (80%) of them. Pulmonary venous obstruction was seen in one patient before surgery. The in-hospital mortality was 14% (2/14). Four patients had pulmonary hypertensive crisis in the postoperative period. The average follow-up was 54 ± 27 months (range: 17-98 months) after surgical repair, and all surviving patients were asymptomatic. There was no late death. No clinically apparent sequelae were seen in six patients in whom isolated left superior pulmonary vein drainage was left uncorrected. An accurate diagnosis of anatomic pattern in mixed type TAPVCcan be difficult to establish in all the patients before surgery. Detailed intraoperative assessment, individualized surgical approach, and aggressive perioperative management may reduce surgical mortality. Operative survivors have good midterm outcome.

Integrated model for the prenatal diagnosis and postnatal surgical treatment of total anomalous pulmonary venous connection: A multidisciplinary collaborative experience and preliminary results.

This study aimed to evaluate an integrated model for the prenatal diagnosis and postnatal treatment of total anomalous pulmonary venous connection (TAPVC). From January 2014 to December 2018, 11 patients were considered as a prenatally diagnosed group, who would accept the integrated model for prenatal diagnosis and postnatal treatment of TAPVC. Besides, 25 patients as postnatally diagnosed group underwent emergency surgery during the corresponding period at the same age. The perioperative status, survival and risk factors for death were compared between the two groups. In a prenatally diagnosed group, three pregnant women chose termination; eight patients followed the integrated model, and their newborns were rapidly transported to a children's hospital within 24 hours after birth. Other than one patient who was prenatally diagnosed with infracardiac type was later confirmed as a mixed type of TAPVC, the prenatal and postnatal diagnoses of the other seven patients were consistent. The 30-day, 1-year, and 5-year survival rates in the prenatally diagnosed group were 100%, 100%, and 100%, while those in the postnatally diagnosed group were 92%, 87.8%, and 87.8%, without significant difference (P > .05). Although Fisher's exact test indicated that an oxygen saturation <70% at admission might be an independent predictor of mortality (P < .01), none of the risk factors for death were significantly different by multivariate Cox regression analysis. The integrated model of prenatal diagnosis and postnatal treatment by multidisciplinary collaboration could lead to satisfactory outcomes, and prenatal diagnosis combined with postnatal oxygen saturation evaluation would facilitate early intervention for TAPVC.

Long-term outcomes of total correction for isolated total anomalous pulmonary venous connection: lessons from 50-years' experience.

Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity. A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach. There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively). Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.

Antenatal diagnosis of total anomalous pulmonary venous connection in functional single ventricle hearts: Outcomes over 13-year period.

A functionally single cardiac ventricle seen on foetal ultrasound scan carries a guarded prognosis. The antenatal diagnosis of anomalous pulmonary venous connection (APVC) remains challenging, if there is no associated structural cardiac abnormality. Antenatally, a combination of complex cardiac anomaly with suspected isomerism should raise the possibility of associated total anomalous pulmonary venous connection (TAPVC). There needs to be a high index of suspicion for TAPVC, in functional single ventricle and suspected isomerism, as this carries a very grim outcome postnatally. We illustrate foetal echocardiographic findings of suspected TAPVC and review outcomes of antenatal versus postnatal diagnosis of TAPVC with functional single ventricle. We retrospectively reviewed our database over 13 years, focusing on foetal cardiac diagnosis, pregnancy outcomes, management and outcomes of livebirths with diagnosis of TAPVC with functional single ventricle. Thirteen patients were included in the review. For the nine antenatal patients, three pregnancies were terminated and six babies were born alive (four babies had compassionate care, two babies had cardiac surgery). One baby is alive at 8.5 years, after Fontan surgery. For the four postnatal patients, three babies had compassionate care (one alive at age 8.1 years) and one baby had cardiac surgery (died age nine weeks). Ten of the 13 patients have right atrial isomerism. Of these 10 patients, only two are alive. For the three non-isomeric babies, only one baby is still alive. There is heterogeneity of the type of TAPVC diagnosed with no particular group that offered better survival. Antenatal diagnosis of TAPVC, even in the context of functional single ventricle remains challenging. If isomerism is suspected, targeted evaluation of pulmonary venous connection should be done. This combination of cardiac lesion carries a very grim outcome. The ability to make this diagnosis antenatally will add to the information and counselling given to these parents.

Mixed Type Total Anomalous Pulmonary Venous Connection in a Newborn

: Total anomalous pulmonary venous connection (TAPVC) is referred to a specific condition in which there is no direct connection between any pulmonary veins and the left atrium. In this report, we describe a case of mixed type TAPVC in a newborn. The surgical correction of anomaly was successful. The child had an uneventful post-op recovery and was discharged.

Total Anomalous Pulmonary Venous Connection in Children: Preoperative Evaluation with Low-Dose Multidetector Computed Tomographic Angiography.

We report the results of our retrospective evaluation, from February 2011 through August 2014, of the anatomic features of total anomalous pulmonary venous connection (TAPVC) and its associated cardiovascular anomalies in a pediatric population. In all 43 patients under study (23 female; average age, 9 mo [range, 4 d-7.1 yr]), these examinations had been performed with a dual-source 256-detector scanner. The type of TAPVC, the presence of obstruction, and the association with other cardiovascular anomalies were investigated and recorded. In accordance with the absence or presence of these accompanying anomalies, patients were subdivided into 2 groups: isolated and complex. In the 43 patients, 22 (51%) TAPVCs were supracardiac, 10 (23%) were cardiac, 6 (14%) were infracardiac, and 5 (12%) were mixed. Obstruction was detected in 7 patients. Seventeen patients were in the isolated group and 26 in the complex group. The diagnostic agreements between multidetector computed tomographic angiographic and surgical results were 100% in both the isolated and complex groups. The overall average effective radiation dose was 0.66 mSv (range, 0.15-1.11 mSv); and it was 0.52 mSv (range, 0.12-0.72 mSv) in patients younger than 1 year of age. We conclude that computed tomographic angiography with a dual-source 256-slice multidetector scanner is a reliable imaging method that enables, despite lower radiation doses, the detailed and comprehensive anatomic imaging of TAPVC in neonates and children.

Total Anomalous Pulmonary Venous Connection in Children

Abstract Introduction: The aim of this study was to study the anatomical types of total anomalous pulmonary venous connection (TAPVC), the associated cardiac and extracardiac congenital malformations, clinical manifestations, and postoperative evolution. Materials and methods: Twenty-four patients with a mean age of 125 days, admitted to the Clinic of Pediatric Cardiology III between January 1, 2009 and December 31, 2015 and diagnosed with TAPVC were included in the study. The patients were evaluated clinically, electrocardiographically and echocardiographically, both pre- and postoperatively. Postoperative evolution was monitored at 1, 3, 6, 12 and 24 months. Results: The anatomical types of TAPVC were: supracardiac in 50% of cases (12 patients), cardiac in 37.5% (9 patients) and mixed type in 12.5% of cases (3 patients). The first clinical manifestation was cyanosis in 72.2% of cases. Surgical correction was performed at a mean age of 37 days in obstructive forms, and 254 days in non-obstructive forms. From the study lot, 8.4% of patients had associated extracardiac malformations (anorectal agenesis and Ivemark syndrome). Early postoperative complications included pulmonary hypertension crisis (60% of cases), supraventricular arrhythmias (35% of cases) and chylothorax (8.4% of cases). Late postoperative complications included: reintervention in 8% of patients with mixed type TAPVC. Conclusions: The most frequently encountered type was supracardiac TAPVC, which had a favorable postoperative evolution. Mixed type TAPVC had the highest rate of reintervention.

Comparison of Sutureless and Conventional Techniques to Repair Total Anomalous Pulmonary Venous Connection

Surgical repair of total anomalous pulmonary venous connection (TAPVC) is challenging. This study aimed to compare the outcomes of the sutureless and conventional techniques for primary repair. From October 2007 to December 2013, 179 consecutive patients underwent repair of TAPVC (sutureless, n = 81; conventional, n = 98). Propensity score matching was used to submit 140 patients to stratified analysis by the presence or absence of preoperative pulmonary venous obstruction (pre-PVO). Surgeons׳ performance differences were assessed with multilevel mixture survival analysis. Freedom from death and postoperative PVO (post-PVO) were evaluated with Kaplan-Meier curves. Risk factors for death and post-PVO were explored using Cox proportional hazard model. Surgeons׳ multilevel effects did not exist in this study. Following matching, Kaplan-Meier curves showed that for patients with pre-PVO (totally 38 cases), rates of freedom from death and post-PVO at 1 year were 80.0% (59.8%-100.0%) in the sutureless group, which was significantly better than that in the conventional group (38.3% [15.2%-61.4%], P = 0.02). For patients without pre-PVO (totally 102 cases), rates of freedom from death and post-PVO at 1 year were 96.1% [95% CI: 90.8%-100.0%] and 86.7% [76.5%-96.9%] in the sutureless and conventional groups, respectively (P = 0.15). Conventional technique was a risk factor for death (hazard ratio = 4.14, 95% CI: 1.29-13.28) and post-PVO (hazard ratio = 5.56, 95% CI: 1.18-26.27) adjusting for type of TAPVC, pre-PVO, and other confounders. In conclusions, the sutureless strategy for primary repair of TAPVC is safe and effective. For patients with pre-PVO, this strategy may associate with decreased mortality and post-PVO with statistical significance.

Distinctive Mediastinal Appearance in Chest Radiograph of a Patient with Total Anomalous Pulmonary Venous Connection

Abstract Despite technological advancements, diagnosis of total anomalous pulmonary venous connection (TAPVC) can be challenging in neonates and infants, particularly in asymptomatic cases. We report a case of a 22-month-old boy who presented to the Emergency department with a history of intermittent fever and cough, initially diagnosed as pneumonia. The presence of an enlarged mediastinal mass was noted in a chest radiograph and was interpreted as a potential malignancy. However, the presence of a supracardiac type of TAPVC with significant right-sided cardiac dilation and a large unobstructed ascending vertical vein was confirmed by echocardiogram. Elective and uneventful surgical correction was performed with subsequent follow-up to confirm cardiac stability and normalization. This case presents the “snowman” or “figure-of-eight” appearance characteristic of TAPVC. Moreover, our patient’s chest radiograph is suggestive of a well-defined, smooth and linear vascular shadow with the presence of a normal lung parenchyma along with increased pulmonary vascularity indicating a left-sided vertical vein in keeping with the supracardiac type of TAPVC. Not commonly reported in previous literature, these unique radiographic findings may hold significant value for pediatricians and imaging specialists as a diagnostic tool for the supracardiac variant of this congenital pathology