Scimitar Syndrome in a Pediatric Cohort.

BACKGROUND: Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. METHODS: We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. RESULTS: Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. CONCLUSIONS: All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.

Scimitar syndrome with vein stenosis in an infant.

Scimitar syndrome is characterised by right lung hypoplasia and abnormal pulmonary venous return, known as the 'scimitar vein'. We report the case of an infant girl with scimitar syndrome who developed a severe respiratory distress mimicking asthma. Pulmonary hypertension (PH) was diagnosed, attributed to scimitar vein stenosis and a left-to-right shunt. Scimitar vein stenosis, a rare complication of scimitar syndrome, can lead to severe PH, highlighting the importance of prompt management in specialised care centres.

Modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection as an alternative to sutureless technique.

BACKGROUND: The modified L-shaped incision technique (MLIT) was successfully applied to the repair of supracardiac total anomalous pulmonary venous connection (TAPVC) with promising mid-term outcomes. It is, however, unclear whether or not MLIT could be an alternative to sutureless technique (ST). METHODS: All patients ( n =141) who underwent MLIT or ST repair for supracardiac TAPVC between June 2009 and June 2022 were included and a propensity score-matched analysis was performed to reduce the heterogeneity. RESULTS: MLIT was performed in 80.9% (114/141), whereas ST was performed in 19.1% (27/141). Patients who underwent MLIT repair had a lower incidence of pulmonary veinous obstruction (PVO)-related reintervention (1.8 vs. 18.5%, P =0.002), and late mortality (2.6 vs. 18.2%, P =0.006). Overall survival at 10 years was 92.5% (87.7-97.7%) for MLIT and 66.8% (44.4-100%) for ST ( P =0.012). Freedom from postoperative PVO at 10 years was 89.1% (83.2-95.5%) for MLIT and 79.9% (65.6-97.4%) for ST ( P =0.12). Cox proportional hazards regression identified prolonged mechanical ventilation duration, postoperative PVO, respiratory dysfunction, and low cardiac output syndrome were associated with postoperative death and PVO-related reintervention. CONCLUSIONS: The MLIT strategy is a safe, technologically feasible, and effective approach for supracardiac TAPVC, which is associated with more favorable and promising freedom from death and PVO-related reintervention.

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

BACKGROUND: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques. METHOD: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically. RESULTS: When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6-10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy. CONCLUSION: Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.

[Scimitar syndrome with pulmonary sequestration containing carcinoma: a case report].

We reported the manifestations, auxiliary examination, and treatment courses of a case of scimitar syndrome with pulmonary sequestration containing carcinoma. The clinical characteristics of scimitar syndrome with pulmonary sequestration, pulmonary sequestration containing carcinoma were summarized based on the data of this case and the related literatures before January 2022. Scimitar syndrome can coexist with ipsilateral pulmonary sequestration. Because sequestered lung tissue has a risk of malignant transformation, a cancer screening test is useful for early diagnosis and timely treatment.

Primary sutureless repair concomitant with the Warden procedure.

A 2-month-old girl who had supracardiac total anomalous pulmonary venous connection (Darling classification type 1b) was referred to our institution. Computed tomography showed that multiple right upper pulmonary veins drained into the vertical vein, near the entry to the superior vena cava. The common pulmonary venous chamber was located lower right than usual, and right upper pulmonary veins were far from the common chamber. We successfully performed primary sutureless repair concomitant with the Warden procedure. Postoperative computed tomography showed unobstructed pulmonary veins and superior vena cava routes, and the vertical vein between right upper and lower pulmonary veins shrank slightly.

Fetal Diagnosis of Scimitar Syndrome in the Presence of Complex Congenital Heart Disease.

Fetal diagnosis of scimitar syndrome requires a high index of suspicion. We present two fetal cases of complex congenital heart disease associated with scimitar syndrome, one of them is diagnosed with scimitar syndrome in utero. We emphasize prenatal echocardiographic findings that may assist with arriving at the correct prenatal diagnosis. We also discuss potential challenges in suspecting the presence of scimitar syndrome in utero. The postnatal echocardiographic findings and course are described for both patients. We reviewed the available literature on prenatal diagnosis of scimitar syndrome in the presence of complex congenital heart disease. We describe a new association of VACTERL, imperforate anus, scimitar syndrome, and double-outlet right ventricle all on the same patient, as well as the first prenatal diagnosis of scimitar syndrome associated with hypoplastic left heart syndrome with restrictive atrial septum. Advanced imaging modalities such as a fetal lung Magnetic Resonance Imaging is suggested as a confirmatory test when scimitar syndrome is suspected in utero in the presence of complex congenital heart disease.

Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return.

The percent of children who can achieve a normal and physiologic pulmonary venous gradient and flow following the repair of Total Anomalous Pulmonary Venous Return (TAPVR) is not known. Pulmonary venous confluence gradients from infants with supra-, infra-, or mixed TAPVR, repaired using a direct anastomotic connection were measured. Data from age, weight, and gender-matched controls established the normal pulmonary venous gradient range (0.30-0.94 mmHg). TAPVR subjects were divided into three groups: (I) pulmonary venous gradient < 2 × normal with multiphasic flow (II) pulmonary venous gradient > 2 × normal with multiphasic flow, and (III) pulmonary venous gradient > 2 × normal with monophasic flow. From 63 children following TAPVR repair and 63 matched controls, pulmonary venous gradients were significantly lower [0.5 mmHg (IQR:0.4, 0.6) vs 1.6 mmHg (IQR:1.0, 2.4); p < 0.001], and multiphasic flow more frequent (100 vs. 84.1%; p = 0.001) within the control group. There were 38 children (60.3%) in group I, 15 (23.8%) in group II, and 10 (15.8%) in group III. Children in Group I were significantly older at the time of repair, had shorter cardiopulmonary bypass times, and did not utilize deep hypothermic circulatory arrest (DHCA). Multivariate analysis confirmed that avoiding DHCA [Odds Ratio 0.931 (0.913,0.994; p = 0.002)] and shorter cardiopulmonary bypass times [Odds Ratio 0.962 (0.861,0.968; p = 0.02)] during repair were associated with the lowest pulmonary venous gradients and multiphasic flow. Following TAPVR repair with a direct anastomosis, the majority of children can achieve a gradient two times normal or less with multiphasic pulmonary venous flow.

Ductus arteriosus banding to regulate excessive pulmonary blood flow in a neonate with necrotizing enterocolitis and complex congenital heart disease, including pulmonary atresia and total anomalous pulmonary venous return: a case report.

BACKGROUND: Patients with right isomerism have accompanying complex congenital heart disease, which is characterized by pulmonary atresia and total anomalous pulmonary venous return. Balanced regulation of the systemic and pulmonary circulation is essential for successful management, especially for cases complicated with necrotizing enterocolitis (NEC). CASE PRESENTATION: A 6-day-old male neonate with a single ventricle, pulmonary atresia, patent ductus arteriosus (DA), and total anomalous pulmonary venous return associated with right isomerism was admitted because of dyspnea, cyanosis, and melena. The patient presented circulatory incompetence due to excessive pulmonary blood flow, resulting in NEC. The patient underwent DA banding and colectomy following continuous intravenous infusion of prostaglandin E1 at six days. Subsequently, his condition improved, reaching a systemic oxygen saturation of around 80%. He underwent a bidirectional Glenn procedure and closure of colectomy at the ages of 5 and 6 months, respectively. CONCLUSION: DA banding can be an alternative to placing an aortopulmonary shunt, which is conventional in patients with ductus-dependent pulmonary circulation, because DA banding is feasible without cardiopulmonary bypass.

Successful rapid staged hybrid approach for obstructed total anomalous pulmonary venous connection.

Obstructed total anomalous pulmonary venous connection (TAPVC) represents a true pediatric cardiac emergency. The patient may present in extremis secondary to severe pulmonary hypertension and cardiogenic shock which increases perioperative mortality. We present a neonate who underwent a successful staged hybrid approach for an Infradiaphragmatic obstructed TAPVC.

Tetralogy of Fallot associated with a rare type partially anomalous pulmonary venous connection and levoatrial cardinal vein.

Tetralogy of Fallot is rarely associated with partially anomalous pulmonary venous connection. Unidentified partially anomalous pulmonary venous connection, however, might increase the risk of pulmonary valve replacement in repaired tetralogy of Fallot patients. Here, we present a case of a 19-year-old male who received a correction of tetralogy of Fallot 18 years ago and a rare type of partially anomalous pulmonary venous connection with levoatrial cardinal vein was identified during the follow-up period. The anomalous pulmonary veins were connected to the left hepatic vein and the right superior caval vein. Performing a pulmonary valve replacement, partially anomalous pulmonary venous connection was also corrected with a new approach using the venous plexus between the hepatic veins.

Superior sinus venosus defect with partial anomalous pulmonary venous return in a patient with tetralogy of Fallot.

We report a case of a 2-year-old boy with tetralogy of Fallot-pulmonary atresia where CT angiography additionally demonstrated the presence of superior sinus venosus defect with partial anomalous pulmonary venous return. Our case highlights the role of CT angiography in the preoperative evaluation in patients with tetralogy of Fallot in identifying such uncommon anomalies, which would help avoid postoperative complications and re-exploration in these patients.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

PURPOSE: The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. RESULTS: A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%; p < .001). CONCLUSIONS: The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

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

OBJECTIVES: A 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). METHODS: Data 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. RESULTS: A 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. CONCLUSIONS: This 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.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Regression of oesophageal varices in total anomalous pulmonary venous connection.

The coexisting of oesophageal varices with total anomalous pulmonary venous connection is extremely rare but contains a potential leading to a lethal haemorrhage. The fate of the oesophageal varices after total anomalous pulmonary vein connection repair remains largely unknown. We herein report a case with infracardiac type total anomalous pulmonary venous connection with remarkable oesophageal varices. In the present case, of note, the oesophageal varices were completely regressed after total anomalous pulmonary venous connection repair without any intervention. This case might help a surgical team reduce the hesitation to repair the total anomalous pulmonary venous connection regardless of oesophageal varices, a potentially fatal condition.

Misclassification of Pulmonary Hypertension in Partial Anomalous Pulmonary Venous Return.

Partial anomalous pulmonary venous return is a rare congenital heart defect that can escape detection until adulthood and can be misdiagnosed as pulmonary hypertension and managed with vasodilators before the anomaly is identified. This report describes the cases of 3 patients with pulmonary hypertension whose hemodynamics and symptoms improved after repair of anomalous veins. Anomalous pulmonary veins are difficult to identify on a transthoracic echocardiogram, so a high index of suspicion and early use of additional imaging modalities are important to avoid a delayed diagnosis and progression to irreversible disease.