Atrial thrombus after total anomalous pulmonary venous connection repair.

A 6-year-old boy had previously undergone total anomalous pulmonary venous connection repair and postoperative pulmonary vein stenosis release. Magnetic resonance imaging revealed blood stasis caused by a collision between the inflow from the pulmonary veins and the outflow from the left atrial appendage. A surgical specimen revealed evidence of advanced thrombus attachment. Infra-cardiac total anomalous pulmonary venous connection with an antler appearance may be a risk factor for thrombus formation in the left atrial appendage and for postoperative pulmonary venous stenosis due to blood flow collision in the left atrium after total anomalous pulmonary venous connection repair.

Outcomes of Adults With Unrepaired Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum.

BACKGROUND: The purpose of this study was to describe the correlates and outcomes in adults with unrepaired partial anomalous pulmonary venous return and intact atrial septum (PAPVR-IAS). METHODS AND RESULTS: We identified adults with PAPVR-IAS who received care at the Mayo Clinic, while those with unrepaired PAPVR-IAS comprised the reference group. Clinical indices (New York Heart Association class, peak oxygen consumption, and NT-proBNP [N-terminal pro-B-type natriuretic peptide]) and echo-derived right heart indices (right atrial [RA] volume, RA reservoir strain, right ventricular [RV] free wall strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline and 3-year and 5-year follow-up. There were 80 patients and 38 patients with unrepaired versus repaired PAPVR-IAS, respectively. The clinical predictors of surgical repair were the number of anomalous veins, RA volume, and RV end-diastolic area. The PAPVR-IAS risk score, derived from these clinical predictors, was associated with surgical repair (adjusted odds ratio, 1.37 [95% CI, 1.24-1.65] per unit increase in risk score; area under the curve, 0.742). Among patients with unrepaired PAPVR-IAS with 3-year (n=73) and 5-year follow-up (n=36), there was no temporal change in clinical indices (New York Heart Association class, predicted peak oxygen consumption, and NT-proBNP) and right heart indices (RA volume index, RA reservoir strain, RV end-diastolic area index, RV free wall strain, and RV systolic pressure). CONCLUSIONS: The PAPVR-IAS risk score can be used to assess the odds of requiring surgical repair. Furthermore, there was no temporal deterioration in clinical and right heart indices during follow-up in adults with unrepaired PAPVR-IAS.

Percutaneous closure of a sinus venosus defect with partial anomalous pulmonary venous return.

A 73-year-old man with a history of hypertension, hyperlipidemia, and obesity presented for cardiovascular evaluation. He was experiencing mild fatigue and dyspnea on exertion. Transthoracic echocardiogram (TTE) showed right ventricular dilation, which was otherwise unremarkable.

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia.

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes.

BACKGROUND: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients. METHODS: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates. RESULTS: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029). CONCLUSIONS: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

Rare case of right ventricular dilatation associated with anomalous pulmonary venous drainage, sinus venosus atrial septal defect and persistent left superior vena cava.

A 34-year-old woman was seen in the emergency department for shortness of breath and chest pain. During a pandemic, it is easy to 'think horses and not zebras', and with a patient presenting with the classic coronavirus symptoms it would have been easy to jump to that as her diagnosis. After a careful history and examination, it became clear that there was another underlying diagnosis. Chest X-ray, echocardiogram and CT scan revealed marked right ventricular dilatation and pulmonary hypertension, alongside a persistent left superior vena cava (PLSVC). Further investigation with cardiac MRI and coronary angiography at a tertiary centre demonstrated that she not only have a PLSVC but also a partial anomalous pulmonary venous drainage and sinus venosus atrial septal defect. This case highlights the importance of considering all differentials and approaching investigations in a logical manner.

Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment.

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as "the scimitar sign", is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

It is not asthma! An arcane case of 'Scimitar' syndrome: A case report.

'Scimitar' syndrome in adulthood is usually asymptomatic. Significant structural abnormalities symptoms usually manifest early during infancy or young childhood with features of congestive heart failure from significant shunting of the anomalous pulmonary venous drainage. Diagnosis of 'Scimitar' Syndrome in adults is rare and usually an incidental finding on chest radiograph. Here, we report a case of an adult who presented with symptoms in her 40's. This syndrome has never been reported nor discussed in Malaysia. This is the first case report of 'Scimitar' Syndrome in Malaysian literature. The diagnostic dilemma, medical management, and multi-disciplinary management by cardiology, physiotherapy and pulmonary rehabilitation teams are discussed.

Peculiarities of Histological Structure of Pulmonary Veins in Patients with Total Anomalous Pulmonary Venous Drainage as Morphological Substrates Relating to Formation of Postoperative Pulmonary Venous Obstruction.

The histological and immunohistochemical methods were employed to examine the peculiarities of histological structure of pulmonary veins and left atrium of the heart in norm and in various types of total anomalous drainage of pulmonary veins. In contrast to normal pulmonary vein covered with external multiple muscle layers (myocardial sleeve), such sleeve is absent in veins that have no connection with the left atrium irrespective of the type of the defect. In patients with total anomalous pulmonary venous drainage, the structure of left atrium was heterogeneous featuring either the presence or absence of inner angiomural lining in this atrium. The structural peculiarities are important for insight into etiology of the development of postoperative pulmonary venous obstruction in patients with total anomalous pulmonary venous drainage.

A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience.

BACKGROUND: Different methods for surgical correction of partial anomalous pulmonary venous connection (PAPVC) exist. We evaluated the outcomes of four techniques regarding morbidity and mortality. METHODS: A total of 116 patients underwent PAPVC repair in our institution over a period of 20 years. Single-patch technique (n = 82 [71%], mean age: 18.59 ± 20.49 years), double-patch technique (n = 13 [11%], mean age: 43.18 ± 25.14 years), Warden's technique (n = 7 [6%], mean age: 10.04 ± 10.47 years), and direct implantation of anomalous pulmonary veins (n = 14 [12%], mean age: 14.42 ± 18.58 years) were examined. RESULTS: Out of the 116 patients, one patient (0.9%) developed pulmonary hypertension after discharge and three patients (2.6%) with normal right cardiac function showed right ventricular failure. In total, a pacemaker was inserted in seven cases (6%). Three patients (2.6%) presented with persistent nonsinus rhythm during follow-up. This complication was most frequently seen in the double-patch group being significantly increased compared with the other groups (p = 0.035). One patient presented with a mild stenosis of the superior vena cava. There were two early, nonsurgery-related deaths and no late mortality. CONCLUSION: Operative therapy of PAPVC has low postoperative morbidity and mortality. Therefore, surgical repair of this cardiac anomaly is a safe and reproducible treatment independent of the applied method. The surgical technique must be selected based on the anatomy and possible accompanying congenital heart defects. Special care should be taken when using the double-patch technique because of significant more frequent nonsinus rhythm events postoperatively.

Outcomes of Double-patch and Warden Techniques in Patients With Supracardiac Partial Anomalous Pulmonary Venous Connection.

AIM: This study compared outcomes following the double-patch and Warden procedures for correcting partial anomalous connection of the right pulmonary veins to the superior vena cava. METHODS: Eighty (80) patients, aged <18years old, and with partial anomalous connection of the right pulmonary veins to the superior vena cava were randomly assigned into double-patch method (n=40) and Warden procedure (n=40) groups. The median follow-up was 22.5 (range, 12-39) months. The primary endpoint was sinus node dysfunction at the mid-term follow-up period. RESULTS: No early or late mortality occurred. In the early postoperative period, sinus node dysfunction was observed in 27.5% and 5% of cases after double-patch correction and the Warden procedure, respectively (risk ratio, 5.50; 95% confidence interval, 1.30-23.25; p=0.01). At follow-up, sinus node dysfunction persisted in two (5%) patients after double-patch correction. All patients had normal sinus rhythm after the Warden procedure. No early or late pacemaker implantation occurred in either group. No patients had significant pulmonary veins or superior vena cava stenosis. CONCLUSIONS: The double-patch technique and Warden procedure both showed excellent early and mid-term results with no mortality and minimal morbidity. The Warden procedure was associated with less sinus node dysfunction in the early postoperative period than the double-patch technique. There was no significant between-group difference in sinus node dysfunction at the mid-term follow-up.

Midterm Outcomes of Surgical Repair of Partial Anomalous Pulmonary Venous Connection to SVC.

OBJECTIVES: We explore midterm results after surgical treatment of partial anomalous pulmonary venous connection (PAPVC) to superior vena cava (SVC) in our institution. METHODS: From 2008 to 2017, 78 patients underwent surgical repair for PAPVC to SVC. Patients were divided into three groups based on surgical techniques: Single-patch repair (n = 20, group A), double-patch repair (n = 31, group B), and Warden repair (n = 27, group C). Their median age was 1.9 years (range: 3 months-13.8 years); median weight was 11.4 kg (range: 4.4-39.7 kg). Clinical, electrocardiographic and echocardiographic were available for all patients. RESULTS: There were no early or late mortality. The mean follow-up duration was 1.8 ± 2.1 years (range: 0.6 months to 8 years). No pulmonary venous obstruction occurred and no residual left-to-right shunts sustained during the follow-up. Reoperation for SVC obstruction was required: 1 (5.3%) in group A, 1 (3.2%) in group B, and 2 (7.4%) in group C (p = 0.78). Four patients (3 in group B, 1 in group C, p = 0.7) presented transient rhythm disturbance at discharge and one patient in group B remains nonsinus rhythm during follow-up. Pacemaker was not required in all patients. CONCLUSION: PAPVC to SVC can be safely managed by multiple techniques. Careful manipulation nearby sinus node must be emphasized during double-patch repair to prevent injury of sinus node. Obstruction of postoperative SVC stenosis should be paid attention to after Warden procedure. For young patients, operation should not be performed that early, but until preschool age.