Pulmonary Venous Drainage Research Articles

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Right subaxillary muscle-sparing thoracotomy for less invasive open-heart surgery: flap-double patch repair of sinus venosus atrial septal defect and partially anomalous pulmonary venous drainage.

Totally anomalous pulmonary venous drainage presenting with cardiovocal (Ortner's) syndrome.

Totally anomalous pulmonary venous drainage presenting with cardiovocal (Ortner's) syndrome.

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.

Use of advanced 3D modelling and printing for congenital heart disease at a large surgical center

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Rachel Cooper Innovative Technologies Fund Background 3D cardiac models, digital and physical, are being used for pre-procedural planning for management of congenital heart disease. Purpose We describe the application of 3D cardiac modelling with details regarding which indications were typically useful to 3D print at our high volume congenital cardiac surgical center. Methods We reviewed patients with congenital heart disease for whom 3D models were created at our institution from January 2018 to December 2022. The diagnosis and indications for the 3D model were collected. Indications for model were separated into 1) complex intracardiac anatomy 2) ventricular assist device/transplant planning 3) anomalous coronary artery origins and 4) other. The mode of 3D modelling was identified as digital model or 3D print. Results A total of 61 models were created over the 5 year period. Indications for advanced modelling were: 30 with complex intracardiac anatomy, 17 ventricular assist device/transplant planning, 9 with anomalous coronary artery and 6 with other indications. Patients included in the "other" category were those with extracardiac congenital anomalies. A total of 25 3D printed cardiac models were created. These included 12/30 of the complex intracardiac anatomy patients, 12/17 of the ventricular assist device/transplant planning patients and 1/6 of the patients with complex extracardiac anatomy (Figure 1). An example of a 3D printed cardiac model used for presurgical planning for a patient with anomalous right pulmonary venous drainage to the inferior vena cava is demonstrated in Figure 2. Right atrium (RA), left atrium (LA), inferior vena cava (IVC), right pulmonary artery (RPA), aorta (Ao). Conclusion 3D printed cardiac models were more commonly utilized at our center than digital models for ventricular assist device and transplant planning than for any other indication including complex intracardiac anatomy.

Minimally Invasive Surgery for Septal Defects Inchild: Literature Review

Nowadays a minimally invasive approach is a rapidly evolving strategy in particular in the field of congenital heart surgery. The main advantage of minimally invasive approaches is less trauma to a patient which positively effects early postoperative period and recovery after surgery. Own to continuous technological progress and growing experience minimally invasive approaches become widely used in cardiac surgery as well as in treatments of congenital heart defects. This review highlights the main problems and their potential solutions in using minimally invasive approaches in surgical treatment of atrial septal defect, ventricular septal defect, partial atrioventricular canal, partial anomalous pulmonary venous drainage. We describe specific considerations of cardiopulmonary bypass, operative techniques and results of minimally invasive approanes.

Аnalysis of preoperative and postoperative electrocardiogram in children with total anomalous pulmonary venous connection

Introduction. TAPVC is a condition that requires diagnosis and surgical treatment in a short period of time. As the result of an absence of the direct connection between pulmonary veins and left atrium, systemic and pulmonary venous return drainage to the right heart with volume and pressure overload. At the same time, left heart preload is depending on atrial septal defect. The hemodynamic features of the TAPVC create conditions for abnormal spread of electric impulses, whereas necessity of surgical reconstruction on the atrial level — reasons for rhythm and conduction disturbance in postoperative period.Aim of the study: detection of specific EKG markers of TAPVC for differential diagnosis of critical conditions in children, analysis of basis preand postoperative EKG to determine EKG pattern in children with TAPVC, registration of EKG changing in early and late postoperative period to define the potential rhythm and conduction disturbance in patients with TAPVC.Materials and methods. The data of biventricular TAPVC patients were reviewed. We estimated characteristic of P, PQ, QRS, QT, rhythm and conduction disturbance and evaluated preand postoperative EKG data, with their comparison in 30 patients. Results. Preoperative EKG (9,3 ± 6,5 days) showed sinus rhythm in all cases, P wave was 62,6 ± 10,6 (40–80) msec, PQ — 104,1 ± 15 (80–160) msec, QRS — 64,3 ± 13,5 (40–100) msec, QT — 253,1 ± 29,5 (210–350) msec, electric axis of the heart — 130,9 ± 44,4 (-70 ± 185). Postoperative rhythm was sinus in 83,3 % cases, atrial ectopic — 13,3 %, AV node rhythm — 3,3 %. The comparison of preand postoperative EKG data showed significant (p < 0,05) difference in P, QT, and electric axis of the heart measurements. In late postoperative period we detected non-pathological differences in P, PQ, QRS, QT compared early posleoperative period, but sinus node dysfunction frequency was high in late postoperative period.Conclusion. There are no specific ECG markers in preoperative TAPVC patients. The standard EKG showed right heart volume and pressure overloud with positive changing of it after surgical correction of TAPVC. The early postoperative EKG did not showed “typical” rhythm and conduction disturbance after surgery at the atrial level, but patients require a long-time dynamic observation after TAPVC surgery because of higy frequency of sinus node dysfunction in late postoperative period.

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection is a rare CHD with a wide variation in pulmonary venous anatomy and drainage. We present a ten-day-old newborn with a rare bizarre subtype of mixed total anomalous pulmonary venous connection.

Left Ventricular Outflow Obstruction in D-Transposition of Great Arteries: A Rare Etiology

A 2-day old neonate [gender: male; weight: 3.2 kg; height: 53cm] presented to the authors institution with an arterial oxygen saturation of 74%. The child was receiving 6 cm H2O continuous positive airway pressure therapy [FIO2: 0.5] and was treated with a prostaglandin E1 infusion [10ng/kg/min]. Transthoracic echocardiography revealed situs solitus, levocardia, normal systemic and pulmonary venous drainage and a 6 mm non-restrictive atrial septal defect with a left to right shunt. The atrioventricular connections were concordant. The aorta was arising from the right ventricle with no right ventricular outflow obstruction [Figure 1]. The pulmonary artery was arising from the left ventricle with confluent good size branch pulmonary arteries with posterior deviation of the conal septum [Figure 2]. A large perimembranous (conoventricular type)1 ventricular septal defect with an inlet [6 mm] and outlet [6 mm] extension and an additional tiny apical defect were demonstrated [Figure 3]. A patent ductus arteriosus [2.7 mm] shunting left to right was also present (not shown). A 2-dimensional echocardiography image in the parasternal long axis view displayed an obstruction to the left ventricular outflow tract [white arrow] and a color Doppler blood flow map demonstrated turbulence in the outflow tract [Figure 4A,B]. In the subcostal view with the probe tilted anteriorly, color flow Doppler mapping showed the ventricular septal defect and also the blood flow acceleration across the left ventricular outflow tract [Figure 5, videolip-1]. At the area of turbulence, the jet width was 0.234 cm[Figure 6A]. A continuous wave Doppler interrogation of the area demonstrated a gradient of ≈ 25 mmHg [Figure 6B]. 2-dimensional transthoracic echocardiography interrogation in the subcostal view identified the etiology of the left ventricular outflow tract obstruction [Figure 7, Videoclip-2]. What is the diagnosis? Figure 2Transthoracic echocardiography image showing the relationship between the pulmonary artery and the left ventricle. View Large Image Figure Viewer Download Hi-res image Figure 3Subcostal 4-chamber transthoracic echocardiography image showing the perimembranous ventricular septal defect. View Large Image Figure Viewer Download Hi-res image Figure 4A&B. Parasternal long axis transthoracic echocardiography 2-dimensional image showing the obstruction in the left ventricular outflow tract ‘white arrow’ [A], and turbulence in the outflow tract by a color Doppler blood flow map ‘white arrow’[B]. View Large Image Figure Viewer Download Hi-res image Figure 5Subcostal transthoracic echocardiography image showing color Doppler flow acceleration over the left ventricular outflow tract and a ventricular septal defect. View Large Image Figure Viewer Download Hi-res image Figure 6A&B. Subcostal transthoracic echocardiography image showing the width of the obstruction [A] and the pressure gradient across the obstruction [B]. View Large Image Figure Viewer Download Hi-res image Figure 7A subcostal transthoracic echocardiography view showing the etiology of the left ventricular outflow tract obstruction in systole. View Large Image Figure Viewer Download Hi-res image

A Patient with Right-to-Left Shunt Atrial Septal Defect Without Pulmonary Hypertension

We present a case of atrial septal defect (ASD) in a 27-year-old young woman who presented with a right-to-left shunt despite normal pulmonary artery pressure without anomalous pulmonary venous drainage and only mild tricuspid regurgitation (TR), but it was associated with right ventricular dysfunction. Considering the dysfunction of the right heart and significant dilatation of the tricuspid annulus, the surgeon still decided to repair ASD and perform tricuspid valvuloplasty. The surgery lasted approximately three hours, and the whole procedure was smooth and uneventful. Her postoperative recovery was uneventful and discharged in good condition. The occurrence of right-to-left (R-L) shunting in patients with ASD is uncommon and is usually an ominous sign, heralding irreversible pulmonary hypertension (PH). There is little information about similar shunts for patients without PH and tricuspid regurgitation (TR), and the majority of these patients have an anatomic anomaly that favors this type of shunt. ASD with R-L shunt without Eisenmenger physiology is rarely reported and may be underestimated due to difficulty in its diagnosis. Intraoperative transesophageal echocardiography (TEE) examination should not only focus on cardiac structure but also the evaluation of ventricular function. Right-to-left shunt is possible in ASD patients even with normal pulmonary arterial pressure.

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.

An uncommon variant of partial anomalous pulmonary venous drainage causing right chamber dilatation

An uncommon variant of partial anomalous pulmonary venous drainage causing right chamber dilatation

A rare case of the combination of total abnormal pulmonary vein drainage with congenital lung lymphangiectasia

The article describes a clinical report of a combination of congenital heart disease due to total anomalous pulmonary venous drainage into the right atrium with congenital lung disease — lymphoangiectasia in a newborn infant. The role of extracardiac pathology on the course and outcome of cardiac surgery and the complexity of early diagnosis of a rare lung pathology are presented

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.

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. Between March 2010 and December 2020, 58patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2cases, the procedure was unsuccessful; the remaining 34were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

Double anterograde/retrograde approach for embolization of pulmonary arteriovenous malformation presenting with acute respiratory failure.

A 54-year-old male with multiple cardiovascular risk factors and past medical history of long-standing dyspnea was admitted for acute respiratory failure. Physical examination revealed near-normal blood pressure, sinus tachycardia, tachypnea, and reduced oxygen saturation (90%) with mild distal-limb cyanosis. On admission ECG, chest X-ray, transthoracic echocardiography, and laboratory tests were performed to exclude new-onset heart failure and/or an acute coronary syndrome. Then, he underwent pulmonary CT angiography seeking for pulmonary embolism that showed a voluminous pulmonary arterio-venous malformation (PAVM) with the feeding artery coming from the right pulmonary artery and venous drainage confluent into the right pulmonary vein (Panel 1A and C). A thrombophilic screening showed a heterozygous mutation for the methylenetetrahydrofolate reductase-1 gene and reduced serum-free protein S levels. The invasive left–right heart catheterization revealed mild-to-moderate pulmonary hypertension and moderate arterial desaturation (O2 saturation, 91%). Considering the large size of both the feeding artery and vein drainage (10 mm) and in order to reduce postprocedural embolization risk and recanalization rate,1,2 a contemporary percutaneous embolization of both PAVM branches (Panel 1C and D; Supplementary material online, Videos S1–3) with two Amplatzer Vascular Plug (AVP) II® 20 mm (Abbott, Plymouth, MN, USA) was planned using a double access technique, transfemoral trans-septal (retrograde) and transfemoral transpulmonary (anterograde) with a post-procedural improvement of arterial saturation (up to 99%). The patient was discharged with a marked clinical improvement (NYHA Class I) and complete exclusion of the PAVM at six months follow-up (Panel 1E and F).

Midline crossing right pulmonary veins with dual drainage to the left atrium.

We report the exceptional case of transcatheter treatment of a partial anomalous pulmonary venous drainage of the right lung to the innominate vein and dual drainage to the left atrium.

The Impact of Prenatal Diagnosis in the Evolution of Newborns with Congenital Heart Disease.

Congenital heart malformations are cardiac and/or vascular structural abnormalities that appear before birth, the majority of which can be detected prenatally. The latest data from the literature were reviewed, with reference to the degree of prenatal diagnosis regarding congenital heart malformations, as well as its impact on the preoperative evolution and implicitly on mortality. Studies with a significant number of enrolled patients were included in the research. Prenatal congenital heart malformations detection rates were different, depending on the period in which the study took place, the level of the medical center, as well as on the size of enrolled groups. Prenatal diagnosis in critical malformations such as hypoplastic left heart syndrome, transposition of great arteries and totally aberrant pulmonary venous drainage has proven its usefulness, allowing an early surgical intervention, thus ensuring improved neurological development, increasing the survival rate and decreasing the rate of subsequent complications. Sharing the experience and results obtained by each individual therapeutic center will definitely lead to drawing clear conclusions regarding the clinical contribution of congenital heart malformations prenatal detection.

Complex Adult Congenital Heart Disease: A Rare Case of Dextrocardia with Double-outlet Right Ventricle

Abstract Double-outlet right ventricle (DORV) is a complex and rare congenital heart disease (CHD), which is usually symptomatic early in childhood and thus diagnosed early. Dextrocardia is another rare CHD that can occur either as an isolated cardiac abnormality or may be associated with other complex cardiac defects. However, the co-occurrence of DORV and dextrocardia is rare, especially in adult life. We present a 35-year-old Nigerian lady with situs inversus totalis and complex CHD, which included dextrocardia with multiple congenital intracardiac shunts, DORV occurring with L-malpositioning of the great vessels, atrial septal defect, and ventricular septal defect with partial anomalous pulmonary venous drainage, persistent left superior vena cava as well as severe pulmonary stenosis.

540 RAGHIB SYNDROME WITH VERY LATE CLINICAL PRESENTATION.

Abstract A 72-year-old woman who had been diagnosed with pulmonary emphysema and permanent atrial fibrillation was admitted to our emergency department with signs and symptoms of heart failure (NYHA class III). Transthoracic echocardiography (TTE) revealed dilatation of both right and left atria, dilatation and systolic dysfunction of the right ventricle (RV) and severe pulmonary hypertension. Combined severe mitral and tricuspid regurgitation was also noted and, after clinical stabilization, transesophageal echocardiography (TEE) was performed to better assess valvular anatomy and function. TEE revealed clear evidence of a pre-tricuspid left-to-right shunt with signs of significant RV volume overload, but with difficulties in defining the characteristics of a suspected atrial septal defect (ASD). CMR confirmed the presence of a large coronary sinus ASD (Panel A) as responsible for a significant left-to-right shunt (Qp/Qs ∼ 3,5, measured by phase-contrast imaging after correcting for the combined mitral and tricuspid regurgitation; Panel B), as well as severe RV dilatation and dysfunction. Pulmonary venous drainage was normal. Persistent left superior vena cava (SVC; Panel C) draining directly into the left atrium (LA) and a completely unroofed coronary sinus were unveiled. The extremely rare combination of an unroofed coronary sinus with abnormal systemic venous drainage from double SVC circulation (usually as a persistent left SVC) is also known as Raghib syndrome (or Raghib complex). In the ASD with completely unroofed coronary sinus there is no common wall separating the coronary sinus from the LA, making the depiction of the shunt anatomy particularly difficult to assess with echocardiography. When unrecognized in the perinatal age, clinical natural course of Raghib syndrome can vary based on the severity of RV volume overload, and late (or very late) clinical presentations have been rarely reported. CMR, with its large field-of-view capability and multiparametric approach, provides accurate definition of cardiac and great vessels anatomy and function allowing the full non-invasive depiction of most congenital abnormalities. In many cases, multimodality imaging is the key to reach a full depiction of these complex conditions, particularly when adult patients with possible comorbidities need to be evaluated.

MODIFIED DEEP TRANSGASTRIC TRANSESOPHAGEAL ECHOCARDIOGRAPHY IMAGING OF SUPERIOR VENA CAVA

<h3>Background</h3> Superior vena cava is usually interrogated intraoperatively by a standard bi-caval view as suggested by American Society of Echocardiography. A modified deep transgastric transesophageal echocardiography view is suggested that will superior interrogation of the superior vena cava intraoperatively. <h3>Objective</h3> Evaluation of a modified deep transgastric imaging of superior vena cava in patients undergoing cardiac surgery. <h3>Methods and material</h3> A modified deep transgastric imaging of superior vena cava was performed in children undergoing cardiac surgery. The deep transgastric interrogation with color Doppler blood flow map showed the venous return pattern. A pulse wave Doppler signal analysis was also feasible. The position of the tip of a central venous catheter tip that was inserted through the internal jugular vein could be seen. An agitated saline contrast injected into the left upper limb could exclude the presence of a left superior vena cava. In patients undergoing a undergoing a bi-directional Glenn shunt, considerable length of superior vena cava was visualized. In a child with a partial anomalous pulmonary venous drainage and a superior sinus venosus ASD, a point of the pulmonary vein joining the superior vena cava could be identified as it was possible to interrogate a considerable length of superior vena cava compared to the standard mid esophageal bi-caval view. The deep transgastric TEE interrogation of superior vena cava revealed a residual gradient in the superior vena cava following a sinus venosus atrial septal defect repair that was corrected at the same sitting. A color Doppler blood flow map across the superior vena cava following removal of a directly inserted venous cannula showed turbulence and a continuous-wave Doppler signal analysis revealed a residual gradient that was immediately corrected by the surgeon in another patient. <h3>Conclusion</h3> In conclusion, the deep transgastric TEE view of the superior vena cava is clinically useful. The doppler alignment is parallel to SVC flow (less than 20 degree) which is superior to bi caval view, where the doppler alignment is almost perpendicular to the flow. The probable difficulties that may be encountered that would interfere with proper visualization are: (i) poor contact of the probe with cephalad aspect of the stomach, (ii) air in the stomach, (iii) use of inadequate contact jelly, and (iv) lack of experience. There is a deep learning curve that can be a practical impediment but once honed, it could be a really useful adjunct to our routine trans-esophageal views.