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.

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.

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.

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.

The case of the missing pulmonary vein: A focused update on anomalous pulmonary venous connection in congenital cardiovascular disease.

Partial anomalous pulmonary venous connection is defined by one or more of the pulmonary veins draining to the heart into a location other than the left atrium. Depending on the location of the anomalous venous connection, they can be categorized as supracardiac, infracardiac, cardiac, and mixed types. In some cases, there is no hemodynamic consequence; in others, it can result in tricuspid regurgitation, right heart dilation, and pulmonary hypertension. Frequently, the reason for referral can be asymptomatic right heart dilation of unknown significance. Diagnosis is often difficult by transthoracic echocardiogram unless there is a high index of suspicion, and the appropriate views are obtained. Cardiac CT (computed tomography) or cardiac MRI (magnetic resonance imaging) can provide more precise anatomic detail as needed. The current article reviews the etiology and pathophysiology of partial anomalous pulmonary venous connection, and also reviews the current knowledge on their treatment.

Cardiorespiratory Interactions in Paediatrics: 'It's (almost always) the circulation stupid!'

The interaction of the heart and lungs is probably the most important aspect of life and survival. Fortunately, it is not difficult to understand the fundamentals. The purpose of the lungs and their ventilation is to present oxygen to the circulation via the alveoli and to receive carbon dioxide from the circulation and then expel it. The relations of the heart and lungs and the matching of blood flow to the various organs with ventilation and lung perfusion may be disrupted by a variety of congenital or acquired heart malformations. They include those giving rise to an increased or reduced pulmonary blood flow, elevated pulmonary venous pressure or external physical pressure on the airways or lung parenchyma. Respiratory disorders which compromise cardiac function include states with reduced alveolar ventilation, those with a barrier to ventilation or perfusion, ventilation/perfusion mismatch and pulmonary vascular disease. There is also a fascinating group in which congenital disorders of the heart and lung co-exist to produce very particular modes of abnormal cardiopulmonary interaction.

Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.

Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques. Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years. Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.

Anomalous left atrial drainage of the right superior caval vein with partial anomalous pulmonary venous return.

We present a case of anomalous direct connection of the right superior caval vein to the left atrium. The diagnosis was made in a 2-year-old child with unexplained cyanosis and apparently normal heart. Corrective surgery was performed, and the child recovered completely.

Obstructed Infradiaphragmatic Total Anomalous Pulmonary Venous Return in a 13-Day-Old Infant Presenting Acutely to the Emergency Department: A Case Report.

BACKGROUND: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital heart defect. Obstructed forms are more severe, and typically present earlier in life, usually in the immediate newborn period, with symptoms of severe cyanosis and respiratory failure. CASE REPORT: A 13-day-old boy presented to the emergency department (ED) with respiratory extremis. He appeared cyanotic and limp, and was found to have significant hypoxia with oxygen saturation of 40%. He had no improvement of oxygenation with bag-valve-mask ventilation despite a fraction of inspired oxygen near 100%. This gave clear indication that the hypoxia was caused by a shunt and not by hypoventilation, a ventilation/perfusion mismatch, or a barrier to diffusion. Next, the patient was intubated emergently. Broad spectrum antibiotics and fluid resuscitation with normal saline were initiated. A chest radiograph showed evidence of pulmonary edema vs. diffuse interstitial disease. Cardiology was consulted and evaluated the child with an echocardiogram, which revealed TAPVR with infradiaphragmatic obstructed veins. Once stabilized, he was transferred for definitive surgical repair. This is, to our knowledge, the first reported case of TAPVR with infradiaphragmatic obstruction presenting to the ED with hemodynamic and respiratory compromise beyond the first week of life. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Despite improvements in antenatal and newborn screening, congenital heart disease often remains an elusive diagnosis. Some patients with these critical lesions are discharged home before the manifestation of their disease becomes apparent. Once symptomatic, these patients often present to the ED in extremis. We conclude that it is important to recognize this presentation to ensure proper evaluation and early diagnosis. If misdiagnosed, many of the usual therapies for other diseases could be detrimental.

Successful transcatheter occlusion of an anomalous pulmonary vein with dual drainage to the left atrium.

We describe a case of a scimitar syndrome "variant" where dual drainage existed from the right upper and middle pulmonary veins to the inferior vena cava and left atrium. Device closure of the anomalous vein at the level of the connection to the IVC was successful in achieving diversion of pulmonary venous flow to the left atrium. Vigilance during work-up of anomalous pulmonary venous drainage (whether isolated or associated with other cardiac defects that may be amenable to device closure) is important to define the presence of dual connections to the left atrium, in which case a less-invasive transcatheter approach may be feasible.

Mixed Supra- and Intracardiac Totally Anomalous Pulmonary Venous Connection in an Adult Female: Pre- and Postoperative Evaluation with Emphasis on MDCT Angiographic Advantages.

All cases with total anomalous pulmonary venous connection (TAPVC) were reported in neonates and children in the previous literature. This report describes the only case in which a mixed supra- and intracardiac TAPVC was discovered in a 25-year-old adult female and multidetector-row computed tomography (MDCT) angiography was used in its pre- and postoperative evaluation. Multidetector-row computed tomography angiography is superior to echocardiography in showing the draining sites and courses of the anomalous connected pulmonary veins, as well as postoperative evaluation in a patient with mixed TAPVC. It indicates that MDCT angiography may be a more suitable diagnostic modality for use in the pre- and postoperative evaluation of the mixed TAPVC.

Simultaneous transcatheter closure of intralobar pulmonary sequestration and patent ductus arteriosus in a patient with infantile Scimitar syndrome.

Scimitar syndrome is a rare disease associated with a right lung sequestration vascularised by arteries arising from the abdominal aorta and abnormal venous drainage into the inferior vena cava. The infantile form is generally presented with severe heart failure, pulmonary hypertension and respiratory distress. It may be associated with various intracardiac defects, including atrial septal defects, ventricular septal defects, patent ductus arteriosus or more complicated structural congenital heart defects. Here, we present a 2-month-old girl with Scimitar syndrome whose pulmonary arterial pressure decreased after transcatheter patent ductus arteriosus closure and embolization of the anomalous systemic arterial supply.

Scimitar syndrome: the curved Turkish sabre.

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. Patients are either diagnosed early with severe symptoms (infantile type) or late with minimal symptoms (childhood/adult type). In this chapter we reviewed the history, pathophysiology, presentation, diagnosis, and treatment methods of Scimitar syndrome in the current era.

Fontan completions over 10 years after Glenn procedures.

OBJECTIVE: Despite the broadened indications for Fontan procedure, there are patients who could not proceed to Fontan procedure because of the strict Fontan criteria during the early period. Some patients suffer from post-Glenn complications such as hypoxia, arrhythmia, or fatigue with exertion long after the Glenn procedure. We explored the possibility of Fontan completion for those patients. METHODS: Between 2004 and 2010, five consecutive patients aged between 13 and 31 years (median 21) underwent Fontan completion. These patients had been followed up for more than 10 years (10 to 13, median 11) after Glenn procedure as non-Fontan candidates. We summarise these patients retrospectively in terms of their pre-operative physiological condition, surgical strategy, and problems that these patients hold. RESULTS: Pre-operative catheterisation showed pulmonary vascular resistance ranging from 0.9 to 3.7 (median 2.2), pulmonary to systemic flow ratio of 0.3 to 1.6 (median 0.9), and two patients had significant aortopulmonary collaterals. Extracardiac total cavopulmonary connections were performed in three patients, lateral tunnel total cavopulmonary connection in one patient, and intracardiac total cavopulmonary connection in one patient, without a surgical fenestration. Concomitant surgeries were required including valve surgeries--atrioventricular valve plasty in three patients and tricuspid valve replacement in one patient; systemic outflow tract obstruction release--Damus-Kaye-Stansel procedure in two patients and subaortic stenosis resection in one patient; and anti-arrhythmic therapies--maze procedure in two patients, cryoablation in two patients, and pacemaker implantation in two patients. All patients are now in New York Heart Association category I. CONCLUSION: Patients often suffer from post-Glenn complications. Of those, if they are re-examined carefully, some may have a chance to undergo Fontan completion and benefit from it. Multiple lesions such as atrioventricular valve regurgitation, systemic outflow obstruction, or arrhythmia should be surgically repaired concomitantly.

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.

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.

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.