Anomalous Drainage Research Articles

Utilizing three-dimensional computer graphics for anomalous drainage of the inferior vena cava

Utilizing three-dimensional computer graphics for anomalous drainage of the inferior vena cava

Appraisal of partial anomalous pulmonary venous drainage through a lumped-parameter mathematical model: a new pathophysiological proof of concept.

Haemodynamic determinants of the ratio between pulmonary and systemic flow (Qp/Qs) in partial anomalous pulmonary venous return (PAPVR) are still not fully understood. Indeed, among patients with the same number of lung segments draining anomalously, a great variability is observed in terms of right ventricular overload. The aim of this study was to test the hypothesis that the anatomic site of drainage, affecting the total circuit impedance, independently influences the magnitude of shunt estimated by Qp/Qs. A zero-dimensional (0D) lumped parameter mathematical model was developed and validated on a sample of patients. We developed a 0D lumped parameter model, using time varying elastances for heart chambers, RLC Windkessel circuits for the systemic and pulmonary circulations. Patients, were categorized into vena cava (VC) type (including left drainage to anomimous vein) and right atrium type (RA). The mathematical model is a system of ordinary differential equations that are numerically solved by means of the ode15s solver in the Matlab environment. The model showed an increase of Qp/Qs with the increase of the number of anomalous veins. With the same number of anomalous veins, Qp/Qs was lower in patients with anomalous drainage to the vena cava (VC) as compared with right atrium (RA) The validation sample consisted of 49 patients (27, 55% females). As predicted by the model, patients with PAPVR with VC type displayed a lower invasive and CMR Qp/Qs as compared with drainage to RA: 1.4 (1.2-1.7) and 1.45 (1.25-1.6) versus 2 (1.75-2.1) and 1.9 (1.6-2) p < 0.05. After stratifying for number of lung territories a lower Qp/Qs was measured in patients with VC PAPVR as compared with RA. In patients with PAPVR, the site of anomalous drainage modulates the Qp/Qs. According to the model, this effect is mediated by the post-capillary impedance of the circuit and significantly decreases with the increase of pulmonary vascular resistances.

Small cardiac vein draining into the inferior vena cava.

Cardiac venous anomalies are rare congenital anatomical anomalies, which are most commonly found on computed tomography scans as an accidental finding. We report a case of a 14-year-old child who was operated for an atrial septal defect, and during the operation, we came across an anomalous drainage of the small cardiac vein into the inferior vena cava. The child's postoperative course was uneventful.

Diagnosis of Congenital Heart Disease in Adulthood: How Often, How Relevant?

Congenital heart disease (CHD) is typically detected during fetal life, infancy, or early childhood. However, there is no published data regarding the proportion of congenital heart defects that are diagnosed in adulthood or the impact of these defects. Retrospective analyses of all consecutive patients (n = 1,010) referred to an adult CHD unit between 2018 and 2023. We analyzed the proportion of cases diagnosed in adulthood, defining the type of defect, reasons for diagnosis, complications, and need for intervention. In total, 26.5% of patients were diagnosed in adulthood (mean age 47 ± 16 years). Overall, 75% were in New York Heart Association class I/IV. Most were mild complexity lesions (57.5%). The most common diagnoses were pre-tricuspid shunts, including ostium secundum atrial septal defect (ASD, 23.9%), partial anomalous pulmonary vein drainage (18.3%), and other types of ASD (5.9%). Bicuspid aortic valve (16.8%) and aortic coarctation (8.2%) were common. Other diagnoses included Ebstein's anomaly (5.6%), ventricular septal defect (4.5%), patent ductus arteriosus (2.6%), or congenitally corrected transposition of the great arteries (2.6%). The main reason for diagnostic work-up was cardiac symptoms (28.4%) such as dyspnea (19%) and palpitations (7.1%), followed by incidental findings on imaging (25.4%). A total of 47.4% had some complications, the most common being pulmonary hypertension (24.3%). Surgical repair was required in 27.2% and 25.4% underwent percutaneous intervention. About one-fourth of patients with CHD were diagnosed in adulthood, and up to 42.5% had moderate or severe complexity lesions. A significant proportion had developed complications at the time of diagnosis and half of them required intervention.

Intralobar Pulmonary Sequestration: Varied Presentations and Different Treatment Modalities

Abstract Pulmonary sequestration, accounting for 0.15%–6.4% of congenital abnormalities, is a dysplastic lung tissue that receives an abnormal systemic arterial supply and has an abnormal tracheobronchial connection. There are two types, namely, extralobar and intralobar. The intralobar sequestration, a dysplastic lung tissue, is characterized by the absence of its own visceral pleura and has its arterial supply from the anomalous systemic artery and venous drainage into the pulmonary vein. These three cases highlight the various presentations and treatment modalities available.

Percutaneous closure of a superior sinus venosus atrial septal defect with partial pulmonary anomalous venous drainage: An option also for children.

Percutaneous closure of a superior sinus venosus atrial septal defect with partial pulmonary anomalous venous drainage: An option also for children.

Different mechanisms for persistent and residual left-to-right shunt after transcatheter sinus venosus defect closure and their management.

Transcatheter closure of superior vena cava (SVC) form of sinus venosus defects (SVDs) using covered stents is emerging as an alternative to surgery in the current decade. A covered stent placed in the cavoatrial junction creates a roof for the right upper pulmonary vein (RUPV) that stops the left-to-right shunt and redirects the vein to the left atrium. While surgical literature has clearly documented the incidence of stenosis of SVC and RUPV, sinus nodal dysfunction, and persistent residual shunts following surgical correction, it is imperative to have similar data after this new transcatheter intervention on the incidence of complications and follow-up outcomes. Since patients with pretricuspid shunts are often clinically asymptomatic, correction is primarily performed to prevent a persistent right heart volume overload and allow remodeling of the heart chambers. Any residual left-to-right shunt after a correction will result in persistent right heart dilatation. Residual flows can result from various mechanisms, including lack of apposition of the covered stent to the free edge of the SVD, fabric breach, and persistent anomalous drainage of additional right-sided pulmonary veins that drain very high in the SVC or can be due to a coexistent defect in the oval fossa. This review analyzes the different mechanisms, explains the transesophageal and angiographic images for each one, and offers solutions tailored for various reasons. Different mechanisms warrant different treatment principles. A solution for residual shunt from one mechanism may not be appropriate for residual flow through another mechanism. A thorough understanding would aid the operator in effective interventions for these SVDs.

Analysis of risk factors and outcomes of acute kidney injury in young children after cardiac surgery

The aim of this study was to analysis of risk factors and outcomes of acute kidney injury (AKI) in young children with congenital heart disease (CHD) after cardiac surgery. The study included 137 young children with CHD after various types of cardiac surgery. The stages of AKI and indications for peritoneal dialysis (PD) were determined based of Kidney Disease: Improving Global Outcomes (KDIGO) guidelines. The incidence of AKI in young children in the general group was 40.9% (n=56). Stage 1 AKI was diagnosed in 21.9% (n=29) of patients, stage 2 AKI in 12.4% (n=17), and stage 3 AKI in 7.3% (n=10) of patients. Peritoneal dialysis was performed in 11.7% (n=16) of children. The incidence of AKI development after radical correct transposition of the great vessels (TGV) was 55.5% (n=5), truncus arteriosus (TA) was 100%, pulmonary atresia (PA)=25%, tetralogy of Fallot (TF)=38.1%, total anomalous pulmonary vein drainage (TAPVD)=60%, partial anomalous pulmonary vein drainage (PAPVD)=37.5%, atrioventricular canal (AVC)=44.4%, double outlet of main vessels from the right ventricle (DOMV from the RV)=60% , interventricular septal defect with high pulmonary hypertension (VSD)=21.6%, and combined operations was 46.6%. The need for PD after TGV correction was 22.2% (n=2), after TA=100%, after TF=33.3%, after TAPVD=20%, after AVC=11.1%, after VSD=1.9%, and after combined operations was 13.3%. Risk factors for AKI in young children were: younger age, initial heart failure, type of operation, prolonged cardiopulmonary bypass (CPB) and aortic clamping (AC), low cardiac output syndrome, inotropic and vasopressor therapy, hyperlactatemia. The development of AKI led to increased length of stay in the intensive care unit, overall hospitalization and infant mortality.

Anomalous hepatic drainage and platypnea-orthodeoxia in cyanotic children.

The aetiology of cyanosis could be unclear in children, even for specialised paediatricians. Two cases were reported: first, a 6-year-old child with features of left isomerism and Fallot was fortuitously diagnosed with anomalous hepatic venous drainage before complete repair. Second, a newborn with an antenatal diagnosis of ductus venosus agenesis had an isolated intermittent right-to-left atrial shunt when upright, with favourable outcome, in contrast to the association with significant heart malformations including inferior caval vein interruption. Multimodality imaging and 3D printing helped to rule out extracardiac causes of persistent cyanosis and plan surgical repair.

Use of French Broad River Drainage Basin Topographic Map Evidence Upstream from Asheville, North Carolina to Test a New Geology and Glacial History Paradigm, USA

Topographic map evidence in the western North Carolina French Broad River drainage basin (upstream from Asheville) was used to determine if a new geology and glacial history paradigm (new paradigm) can explain previously unexplained (and anomalous) drainage system evidence. The new paradigm claims: 1) the Eastern Continental Divide was uplifted as the southeastern rim of a continental ice sheet created deep &amp;ldquo;hole&amp;rdquo; (in which the ice sheet was located) as immense and prolonged southwest-oriented meltwater floods flowed across it, 2) headward erosion of south and southeast-oriented valleys (in sequence from the southwest to the northeast) diverted floodwaters more directly to the Atlantic Ocean, and 3) headward erosion of north- and northwest-oriented valleys from the developing deep &amp;ldquo;hole&amp;rdquo; (in sequence from the southwest to the northeast) diverted floodwaters to deep &amp;ldquo;hole&amp;rdquo; space (located between the rising deep &amp;ldquo;hole&amp;rdquo; rim and the ice sheet margin) and then toward deep &amp;ldquo;hole&amp;rdquo; southern exits (eventually the Mississippi River valley became the only southern exit). The new paradigm permitted the following types of drainage system evidence to be explained:1) numerous barbed tributaries flowing to a northeast-oriented French Broad River segment, 2) a larger than required northeast-oriented French Broad River valley, 3) and diverging and converging valley complexes which are found throughout the northeast-oriented oriented French Broad River headwaters drainage basin. In addition, the map evidence could be interpreted to show: 1) headward erosion of the north- and northwest-oriented French Broad River valley captured southwest-oriented flow to the north-oriented Pigeon River valley which had captured flow to the northwest-oriented Little Tennessee River valley, 2) headward erosion of the south-oriented Broad River valley captured southwest-oriented flow to the northeast- and north-oriented French Broad River and the south-oriented Toxaway River and 3) and multiple gaps identify locations where southwest-oriented water entered and exited the present-day French Broad River headwaters drainage basin.

Isolated persistent left superior vena cava associated with anomalous left hepatic vein drainage into the right atrium accidentally discovered after sternotomy.

Isolated persistent left superior vena cava associated with anomalous left hepatic vein drainage into the right atrium accidentally discovered after sternotomy.

Sinus node dysfunction during transcatheter assessment and stent correction of sinus venosus atrial septal defects.

Covered stent correction of a superior sinus venosus atrial septal defect is increasingly performed as an alternative to surgical repair.While sinus node dysfunction requiring pacemaker implantation may be required after surgical repair, this has not been previously reported after covered stent implantation. We reviewed the experience in two interventional centers.Balloon inflation in the superior vena cava was used to confirm the anomalous pulmonary vein drainage would be unobstructed after stent implantation. During balloon testing in 62 consecutive patients, we assessed gradients across the pulmonary vein to left atrium while monitoring the rhythm. We observed the outcomes after covered stent correction in 51 patients.In a single patient, significant bradycardia and pauses developed on repeat balloon testing and the procedure was abandoned without stent implantation. In another patient, there was no sign of sinus node dysfunction during balloon testing but several hours after stent implantation, the patient became symptomatic from sinus bradycardia and pauses and had a pacemaker implanted 3 days later. Over a year later there are some signs of improvement in sinus node function.While sinus node dysfunction has not been described previously during balloon testing or after stent implantation, this report demonstrates for the first time that it may occur. Larger registries are therefore required to monitor for this uncommon complication.

Case Report: Removal of septal occluder device and closure of atrial septal defect with pulmonary vein flow redirection

BACKGROUND: Atrial septal defect represents 10 to 15% of congenital heart disease and is more frequent in women with a 2:1 ratio. Sinus venosus atrial septal defect is usually associated with partial anomalous pulmonary venous drainage from the right superior pulmonary vein to the right atrium directly or indirectly through the superior vena cava. Rarer still, is the inferior sinus venosus, which may be associated with partial anomalous venous drainage from the right inferior pulmonary vein. CASE REPORT: 13-year-old patient with a history of intervention for interatrial defect closure with Amplatzer occluder at the age of six. He was brought to receive medical attention for fatigue and dyspnea that had been progressing for 2 years. Cardiac CT reported partial anomalous connection of pulmonary veins and echocardiogram reported a residual atrial septal defect of 10 mm and mild to moderate pulmonary hypertension. Based on the findings, surgical treatment was decided. EVOLUTION: In correction surgery, we observed Amplatzer occluder incorrectly positioned, the device was removed and flow was redirected from right pulmonary veins to left atrium with bovine pericardium patch. After the surgical intervention, the patient had a favorable evolution without no post-surgical complications. At follow-up, 15 days after medical discharge, the patient was asymptomatic, and a cardiac CT scan showed the corrected interatrial defect. CONCLUSION: The treatment of atrial septal defect should be individualized, since it will depend on the size of the defect, the age at the time of diagnosis, the magnitude of the shunt, the severity of the symptoms and the presence of other conditions. If the defect requires repair, it is important to weigh the benefits of choosing percutaneous closure vs. surgical intervention to avoid the need for reintervention, as in the present case. Closure and redirection of pulmonary vein flow with bovine patch is a safe and effective option.

Double Jeopardy: Truncus Arteriosus with Obstructed Infracardiac Total Anomalous Pulmonary Venous Connection

Truncus arteriosus (TA) is a congenital cyanotic heart disease with admixture physiology at the great arterial level. It can have associated anomalies of the pulmonary arteries, truncal valve narrowing, etc., Total pulmonary venous anomalous drainage associated with TA is extremely rare. We report a sick young infant who presented with type I TA and obstructed infracardiac total anomalous pulmonary venous return.

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

Lateral growth of Safeen anticline as deduced from drainage analysis, Iraqi Kurdistan region

Safeen anticline is one of the largest anticlines in the Iraqi Kurdistan Region (IKR), with NW – SE trend. The anticline shows clear lateral growth as deduced from different anomalous drainage patterns and forms, besides different geomorphological forms, which are indicative of lateral growth. Among those forms are water gaps, wind gaps, abandoned alluvial fans, and different shapes of valleys. Two hanging synclines exist on both sides of the anticline forming unique geomorphological and structural forms in IKR. The anticline is developed in the High Folded Zone (HFZ) of Iraq. It is part of the Zagros Fold–Thrust Belt (ZFTB), with the exposed rocks of the Qamchuqa Formation (Early Cretaceous), which consists mainly of limestone and dolostone, which are the oldest rocks in the core of the anticline. Two main streams named Shaqlawa and Kori dissect the anticline from the northern and southern sides. We have explained their trends and abnormal courses. We have used geological maps and high-resolution satellite images to interpret geomorphological forms and to confirm the lateral growth of the anticline. A field investigation was carried out to check and confirm the interpreted data; accordingly, we identified the anticline's lateral growth based on the interpreted geomorphological and structural forms.

A rare constellation of horseshoe lung with lung malformation and hypoplastic left heart syndrome: a case report

Introduction. The aim of the paper is demonstrating the possibilities of CT angiography in newborns with suspected combinations of a congenital heart defect and pulmonary malformations.Materials and methods. The article presents a clinical case of a rare constellation of horseshoe lung with hypoplastic left heart syndrome, right lung hypoplasia and intralobar pulmonary sequestration of the right lung.Results. Contrast-enhanced computed tomography is mandatory in visualization of rare complex congenital heart and bronchovascular anomaly.Discussion. Horseshoe lung is a rare congenital anomaly of childhood in which the caudal and basal segments of the lungs are joined together anterior to the aorta and behind the left ventricle. This anomaly was described for the first time by Spenser in 1962. Horseshoe lung is often associated with unilateral lung hypoplasia, most commonly involving the right lung, and can occur in conjunction with scimitar syndrome, which includes hypoplasia of the right lung, abnormal right pulmonary venous return, and abnormal arterial supply to the right lung.Conclusion. Presented case report demonstrates current postnatal examination possibilities (CT, angiography) in the diagnostics of a rare congenital heart and bronchovascular anomaly — hyperplastic left heart, intralobar pulmonary sequestration of the right lung, anomalous venous return, anomalous hepatic venous drainage. The incidence of this disorder is approximately 1–3 per 100 000 births.

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

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

ОПЫТ ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ РЕБЕНКА ГРУДНОГО ВОЗРАСТА С СИНДРОМОМ ЯТАГАНА: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

Scimitar syndrome (SS) is an extremely rare congenital heart disease (CHD). There are two main clinical forms of the disorder: adult and infant. The usual course of the infantile form is characterized by an unfavorable prognosis. According to some reports the mortality could reach 100% without surgical treatment. The results of surgical treatment in this age group are currently unsatisfactory. Authors represent a clinical case of SS in a 4.5 months old male patient, who was in moderate and relatively stable condition upon admission. The severity of the patient’s condition was mainly caused by the progressive cardiac and respiratory failure. The diagnoses of CHD, SS were established during the clinical and instrumental examination: partial anomalous drainage of the right pulmonary veins into the inferior vena cava, hypoplasia of the right branch of the pulmonary artery, hypoplasia of the right lung, sequestration of the right lung, dextrocardia, secondary atrial septal defect (ASD), therefore, the indications for surgical correction were also identified. Ligation of the systemic pulmonary anastomoses, reimplantation of the right pulmonary vein collector into the left atrium, suturing of ASD under cardiopulmonary bypass and hypothermic circulatory arrest had been performed. The postoperative period was difficult: the patient was supported with an artificial lung ventilation for long time. He was transferred to the general somatic department on the 28th day after the surgical intervention, which was then followed by a favorable long-term result. Conclusion: Authors represent a successful case of radical surgical treatment of a child aged 4.5 months old with a rare CHD using the non-standard technique for reimplantation of the pulmonary vein collector as well as the features of intraoperative support.

An incidentally detected Partial Anomalous Pulmonary Venous Connection (PAPVC) into supra hepatic IVC- Scimitar syndrome: A case report

Anomalous Pulmonary venous connection is drainage of one or more pulmonary veins outside of left atrium. Anomalous Pulmonary Venous Connection is most common on Left side left upper pulmonary vein drains directly into left innominate vein to ultimately drain into right atrium. Right side Partial Anomalous venous drainage can be into Superior Vena Cava, Right Atrium, Innominate vein or rarely Inferior Vena Cava.