Pulmonary Venous Drainage Research Articles

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.

HYPOXIA, RIGHT-LEFT SHUNT, AND A UNFORTUNATELY LOCATED MASS

Cardiac myxomas are benign intracavitary neoplasms known to cause exertional dyspnea and presyncope due to obstructed pulmonary venous drainage or impaired flow across the atrio-ventricular valves. A left atrial (LA) myxoma should not typically lead to hypoxia even with a concomitant PFO. A 75-year

Use of 3-dimensionally printed heart models in the planning and simulation of surgery in patients with Raghib syndrome (coronary sinus defect with left superior vena cava).

Use of 3-dimensionally printed heart models in the planning and simulation of surgery in patients with Raghib syndrome (coronary sinus defect with left superior vena cava).

Total anomalous pulmonary venous drainage repair: the effect of anatomical type and pulmonary vein stenosis on outcomes

BackgroundSurgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting.ResultsBetween 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used.Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein stenosis occurred in three patients, and reoperation was done in one patient. Survival free from reoperation was 98%, 98%, and 90% at 1, 24, and 60 months in the absence of pulmonary vein stenosis. However, all three patients who developed pulmonary vein stenosis died, at 0.5, 2.7, and 6.3 months of follow-up, respectively. We were unable to detect a significant effect of anatomical type, preoperative obstruction, associated single ventricle, or heterodoxy syndrome on operative mortality or long-term freedom from death or reoperation. Patients who presented with obstruction and infracardiac or supracardiac TAPVD had longer hospital stay.ConclusionTAPVD repair outcomes are excellent except for patients who develop postoperative pulmonary venous stenosis. Anatomical type, obstructed presentation, associated single ventricle, or heterotaxy are not significant predictors of survival.

Transcatheter correction of sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage: The procedure of choice in selected patients?

Transcatheter correction of sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage: The procedure of choice in selected patients?

Unroofed coronary sinus syndrome: A case report.

Unroofed coronary sinus syndrome (UCSS), also named coronary sinus septal defect, is a rare type of atrial septal defect with the incidence less than 1% of the total number of atrial septal defects. It is caused by incomplete formation of left atrial venous folds during embryonic development. Here we reported a patient with UCSS, who was treated in the Second Xiangya Hospital of Central South University. The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise. Color Doppler echocardiography showed coronary sinus dilatation (17 mm×14 mm), indicating the possibility of permanent left superior vena cava. Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium, followed by the right atrium and right ventricle, indicating a partial anomalous pulmonary venous drainage (intracardiac type). Finally, the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava (PLSVC) went into the left atrium and the coronary sinus, respectively, while the coronary sinus septum was absent and the PLSVC was connected with the left atrium. The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.

Isomerism of the atrial appendages: morphology and terminology.

Our aim is to identify the pathognomonic anatomical markers and the best terminology to describe the cardiac malformations associated with absent or multiple spleens, which are known as asplenia or polysplenia syndromes or isomerism. We have reviewed 65 hearts with isomerism of atrial appendages of the Anatomical Collections of Congenital Heart Disease, Institute of Pathological Anatomy of the University of Padua consisting of 1800 specimens. All the hearts were classified according to sequential segmental classification. The incidence of isomerism was 3.6%. Of the total, 45 hearts with isomerism of right atrial appendages showed bilateral trilobed lungs, short bronchi, and absent spleen. The atrioventricular junction was univentricular in 49% of cases with a common atrioventricular valve in 91%. Pulmonary atresia and double outlet right ventricle were present in 40% and 47% of cases, respectively. Total anomalous pulmonary venous drainage and absent coronary sinus were always present. In 20 hearts with isomerism of left atrial appendages, bilateral bilobed lungs with long bilateral bronchi and multiple spleens were always found. The biventricular atrioventricular connection was present in 65% with a common valve in 30% of the hearts. The ventriculoarterial connection was concordant in 45% of cases, and aortic atresia and pulmonary atresia were both noted in 15% of each. An anomalous symmetric pulmonary venous drainage was observed in 65% of the hearts and interruption of inferior vena cava was found in 75% of cases. We believe that the appropriate terminology is based on the symmetrical morphology of the atrial appendages. The absence of the coronary sinus and the total anomalous pulmonary venous drainage are the markers of isomerism of the right atrial appendages. Symmetric pulmonary venous drainage and interruption of inferior vena cava are the markers of isomerism of left atrial appendages. In recent years, thanks to the improvement of clinical diagnosis and of surgical techniques these patients have the possibility to survive to adult age.

P1335 Cor triatriatum sinister: the importance of multimodality imaging

Abstract A 67-year-old woman who had previous history of hypertension and hyperlipidemia was referred to the cardiologist because of nonspecific electrocardiographic abnormalities. During physical exam a systolic murmur was detected. Transthoracic echocardiography (TTE) showed aortic valve sclerosis with no evidence of valvular stenosis. An unexpected finding was the presence of a membrane at left atrium (LA) and a transesophageal echocardiography (TEE) was recommended with suspicion of cor triatriatum. No signs of obstruction to LA flow were demonstrable with pulsed-wave or colour Doppler analysis. On 3D TEE imaging, a structure compatible with a left atrial tendon was visualized. To complete the study computed tomography (CT) was performed, revealing the presence of a real membrane dividing the LA into two components, separating the pulmonary veins from the true LA chamber with the left atrial appendage (LAA), being the patient diagnosed of cor triatriatum. Cor triatriatum sinister (CTS) is a rare but well-recognised form of congenital heart disease. It is believed to reflect failure of incorporation of the common pulmonary vein into the LA. Defining the relationship between the CTS membrane and the LAA allows differentiation from a supra­mitral ring. In CTS, the membrane is located superior to the LAA (between the LAA and pulmonary veins), while a supra­mitral ring is located inferior to the LAA and is often adher­ent to, and constitutes part of, the mitral valve leaflets. The diagnosis of CTS may be made at any age. Most cases involve a nonobstructive membrane, diagnosis of which is facilitated by advances in imaging technology. TTE is adequate to define the clinical relevance of CTS but it may be more precisely tailored with cardiac CT or cardiac magnetic resonance (CMR) imaging. This case emphasizes the importance of a multimodality imaging approach. CTS can present as an isolated lesion, but it is more commonly seen in association with other congenital cardiac anomalies (patent foramen ovale, atrial septal defect, patent ductus arterio­sus, coarctation of the aorta, persistent left superior vena cava, ventricular septal defect, anomalous pulmonary venous drainage, and a variety of left-sided cardiac abnormalities). Nonobstructive asymptomatic adults do not require intervention. However, onset of any cardiovascular symptom should prompt detailed evaluation. A turbulent jet arising from the opening in an obstructive CTS membrane may produce jet lesions on the structurally normal mitral valve causing damage and secondary mitral regurgitation. Surgi­cal resection is the first line of management for an obstructive membrane in the LA. The development of atrial fibrillation in an individual with nonobstructive CTS may also constitute an indication for surgi­cal resection as the presence of the membrane may increase the risk of thromboembolic events. Abstract P1335 Figure. Cor triatriatum sinister (TTE,3D-TEE,CT)

P649 Before and after: sinus venosus atrial septal defect

Abstract Introduction The diagnosis of sinus venosus atrial septal defect (SVASD) is complex and requires special imaging. Surgery is the conventional treatment; however, transcatheter repair may become an attractive option. Case report A 60 year-old woman was admitted to the cardiology department with several episodes of paroxysmal atrial flutter, atrial fibrillation and atrioventricular nodal reentrant tachycardia. She reported a 10-year history of occasional palpitations which had not been studied. A transthoracic echocardiography revealed severe right ventricle dilatation and moderate dysfunction. Right volume overload appeared to be secondary to a superior SVASD with partial anomalous pulmonary venous drainage. A transesophageal echocardiography confirmed the diagnosis revealing a large SVASD of 16x12 mm (Figure A) with left-right shunt (Qp/Qs 2,2) and two right pulmonary veins draining into the right superior vena cava. Additionally, it demonstrated coronary sinus dilatation secondary to persistent left superior vena cava. CMR and cardiac CT showed right superior and middle pulmonary veins draining into the right superior vena cava 18 mm above the septal defect (Figures B and C). After discussion in clinical session, a percutaneous approach was planned to correct the septal defect and anomalous pulmonary drainage. For this purpose, anatomical data obtained from CMR and CT was needed to plan the procedure. During the intervention two stents graft were deployed in the right superior vena cava. The distal stent was flared at the septal defect level so as to occlude it while redirecting the anomalous pulmonary venous flow to the left atrium (Figure D). Control CT confirmed the complete occlusion of the SVASD without residual communication from pulmonary veins to the right superior vena cava or the right atrium (Figure E). Anomalous right superior and middle pulmonary veins drained into the left atrium below the stents. Transthoracic echocardiographies showed progressive reduction of right atrium and ventricle dilatation. The patient also underwent successful ablation of atrial flutter and intranodal tachycardia. She is currently asymptomatic, without dyspnea or arrhythmic recurrences. Conclusions In this case, multimodality imaging played a key role in every stage of the clinical process. First, it provided the diagnosis and enabled an accurate understanding of the patient’s anatomy, particularly of the anomalous pulmonary venous connections. Secondly, it allowed a transcatheter approach by supplying essential information to guide the procedure. Finally, it assessed the effectiveness of the intervention and the improvement in cardiac hemodynamics during follow-up. Abstract P649 Figure.

P648 Right heart volume overload after surgical correction of atrial septal defect sinus venosus type with partial anomalous pulmonary veins drainage

Abstract 40 year-old male with history of congenital heart disease. Atrial septal defect (ASD) sinus venosus type associated with partial anomalous pulmonary venous drainage (PAPVD) was diagnosed during childhood and surgically repaired at the age of three. Since then the patient was asymptomatic and he was lost to follow up when reached adulthood. A transthorathic echocardiogram was performed during a hospitalization because of a complicated pneumonia. A severe dilatation of right chambers and main pulmonary artery was observed. The estimated Qp:Qs by this technique was 2.0 but the atrial septum seemed to be intact. Agitated saline was administered in this procedure and no passage of microbubbles was observed. A cardiac MRI was performed because of suspicion of anomalous pulmonary venous drainage. The findings observed in the echocardiogram were confirmed and an uncommon PAPVD was demonstrated by this technique: the superior left pulmonary vein drainaged into a dilated innominated vein. Also, a small pulmonary vein from the right upper lobe emptied into a "venous conduct" (yellow circle) located posterior to the superior cava vein (SCV) that later drainaged into the right atrium under the mouth of the SVC, posterior to the interatrial septum. Surgically correctioin of the PAPVD was performed. Discussion PAPVD is a congenital heart disease characterized by the drainage of one or several pulmonary veins –but not all of them- into the right atrium or systemic veins, which leads to left-to-right shunting. The estimated incidence of this disease ranges between 0.1 and 0.2% of the general population. The right superior pulmonary vein is the most frequently involved and is commonly associated to ASD sinus venosus type. Bilateral anomalous drainage, as occurred in this case, is exceptional. Furhermore, this case highlights the importance of long-term follow-up in patients with congenital heart disease due to the fact that, even when the disease is considered to be cured, long term complications could appear compromising the prognosis of the patient. Abstract P648 Figure. Unusual PAPVD

Outcomes of the Warden Procedure for Partial Anomalous Pulmonary Venous Drainage.

Partial anomalous pulmonary venous drainage (PAPVD) is a common congenital heart disease. If the insertion of an anomalous pulmonary vein (PV) is high into the superior vena cava (SVC), the traditional 1-patch or 2-patch surgical repair might be challenging. Unlike patch procedures, the cavoatrial anastomosis technique (Warden procedure) theoretically reduces complications such as sinus node dysfunction and venous obstruction. We investigate outcomes of the Warden procedure (WP) at a single institution. A total of 67 patients (42 males and 25 females) with PAPVD who underwent the WP between January 2011 and December 2018 were consecutively enrolled. The median age was 2.8years (3-61years old). 52 cases were younger than 18years old. perioperative and follow-up clinical data were collected. In addition, we selected possible risk factors (a total of 18 risk factors) of SVC stenosis or obstruction. Univariate and multivariate analyses were conducted to confirm the independent risk factors. The average Cardiopulmonary bypass (CPB) time was 132.3 ± 46.3min, and the aortic cross-clamp time was 85.2 ± 35.7min. One patient died postoperatively in hospital. The mean follow-up time of the remaining patients (n = 66) was 15.8 ± 14.5months (3-64months). No abnormal cardiac function or sinus node dysfunction cases were identified during the follow-up. Of the survival patients, no venous obstruction was presented before the discharge. Numbers of patients identified with mild PV and SVC stenosis were 1 (1.5%) and 7 (10.6%, 4 of them turned negative eventually) at discharge. At the last follow-up, no PV obstruction was identified, whereas, 4 cases (6%) had SVC obstruction. Numbers of PV and SVC stenosis cases were 3 (4.5%) and 8 (12%). Univariate and multivariate analyses showed that only the diameter of SVC less than 10mm was an independent risk factor for SVC stenosis or obstruction at the last follow-up. Warden procedure can effectively treat PAPVD (anomalous drainages into SVC), with satisfactory early and mid-term postoperative results. Different modifications of the Warden procedure may be selected according to the anatomic characteristics. The diameter of SVC less than 10mm predicts SVC stenosis or obstruction after Warden procedure.

Long-Term Quality of Life Outcomes in Adult Survivors After Anomalous Pulmonary Venous Drainage Repair

We sought to determine the long-term quality of life after repair of anomalous pulmonary venous drainage using the Short Form (SF)-36 questionnaire in adult survivors. All patients who underwent repair of partial or total anomalous pulmonary venous drainage (PAPVD or TAPVD) and were 18 years of age or older with a current contact number were identified from the hospital database. The mean age of the 101 patients was 26 ± 7 years (range, 18-49) old. Patients completed the SF-36 quality of life questionnaire via telephone. The results of the 8 domains of the SF-36 questionnaire and the derived health state summary score (SF-6-Dimension) were compared against an age-matched Australian population data. Compared with Australian population age-matched data, the 18- to 24-year-old TAPVD/PAPVD patients ranked their health higher in 1 of 8 domains; however the SF-6-Dimension scores were similar (0.75 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P= .2). In the 25-50 age group TAPVD/PAPVD patients ranked their health higher in 3 of 8 domains. However the SF-6-Dimension scores were similar to Australian age-matched population (0.78 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P= .51). Young adult survivors after anomalous pulmonary venous drainage repair have similar quality of life outcomes as age-matched Australian control subjects as measured by SF-6-Dimension.

Retrospective analysis of pulmonary venous drainage in 90 patients with Turner syndrome demonstrates abnormalities are common; Is it time to review the guidelines?

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Reversed Differential Cyanosis in Two Neonates with Obstructed Supracardiac Total Anomalous Pulmonary Venous Drainage

Generalized cyanosis in newborn can be caused by many pathological conditions and cyanotic congenital heart disease represents one of the important causes. Differential cyanosis refers to the situation where upper limb saturation is higher than lower limb and it is well documented to be caused by persistent pulmonary hypertension in newborn

Simple Radiography can Rescue from Dilemma

24 year old young lady came to us with a diagnosis of atrial septal defect (ASD) secundum who lost her follow up since her 4 years of age. Later her history, physical findings and investigations change the diagnosis into supracardiac total anomalous pulmonary venous drainage. Typical ‘snowman sign’ in chest x-ray and transthoracic echo helped to review the case. Proper history taking and careful evaluation by investigation is needful for each individual.
 Cardiovasc. j. 2019; 12(1): 75-78

OP15.03: Prenatal and newborn screening for congenital heart disease

To investigate the value of prenatal and postnatal echocardiogram in the diagnosis of congenital heart disease. Pregnant women who were born in the hospital from 2017.1 to 2017.12 were included in the study. The fetal structure was routinely screened during the second trimester. The newborns were screened for congenital heart disease by pulse oximetry and auscultation. The positive ones were examined by neonatal echocardiography. At 42 days postpartum, the returning infants underwent routine auscultationand those who were examined positive were further examined by echocardiography. A total of 13,849 pregnant women were collected during the study. 62 cases of congenital heart disease were found before birth, including 34 cases of severe congenital heart disease and 28 cases of mild congenital heart disease. In the neonatal period, 192 cases of congenital heart disease were found, including 6 cases of severe congenital heart disease and 186 cases of mild congenital heart disease. The incidence of congenital heart disease is 18.3%. The most common severe congenital heart disease is tetralogy of Fallot, a total of 17 cases. The cases of prenatal missed diagnosis were 3 cases of tetralogy of Fallot, 2 cases of total abnormal pulmonary venous drainage and 1 case of transposition of great arteries. The more common congenital heart diseases in the neonatal period were atrial septal defect and ventricular septal defect. Fetal echocardiography is more sensitive to the examination of severe congenital heart disease, and postpartum neonatal echocardiography can detect more mild congenital heart disease. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Repair of Sinus Venosus Defects With Partial Anomalous Pulmonary Venous Connection in Children by Modified Right Vertical Infra Axillary Thoracotomy.

To evaluate the safety and reproducibility of modified right vertical infra axillary thoracotomy (RVIAT) for repair of sinus venosus defects with right-sided partial anomalous pulmonary venous connection (PAPVC) in children. Between March 2017 and February 2018, we performed intracardiac repair for sinus venosus defects with right-sided PAPVC in 14 children through modified RVIAT. Median age and weight were 9.5 years and 21 kg, respectively. We modified RVIAT by avoiding central venous cannulation and used total peripheral venous cannulation (right internal jugular vein and right femoral vein). In all children double-patch technique was followed, using untreated autologous pericardium. Intracardiac repair was safely performed in all children. There was no conversion to another approach and there were no complications related to peripheral venous cannulation. All children were in sinus rhythm with no residual defects, with non-obstructive pulmonary venous drainage at the time of discharge and during subsequent follow-ups. Modified RVIAT can be safely performed for repair of sinus venosus defects with right-sided PAPVC, without compromising on the quality of repair. With this modification not only the intracardiac repair was easier, also it provided more working space with minimal rib spreading.

Newborn pulse oximetry screening in the context of a high antenatal detection rate of critical congenital heart disease.

AimAssess the potential additional benefit from pulse oximetry screening in the early detection of critical congenital heart disease in a country with a well‐developed antenatal ultrasound screening programme.MethodsLive‐born infants, pregnancy terminations and stillbirths from 20 weeks’ gestational age, between 2013 and 2015, with critical cardiac defects defined as primary or secondary targets of pulse oximetry screening were identified. Critical defects were those resulting in the death of a fetus or an infant in the first 28 days after birth, or a defect requiring intervention in the first 28 days.ResultsTwo hundred and sixty‐eight infants and Fetuses were identified. Antenatal detection rates improved from 69% to 77% over the study period. An associated co‐morbidity improved antenatal detection rates. Twenty‐seven live‐born infants were diagnosed after discharge: 15 aortic arch obstruction (AAO); 10 d‐loop transposition of the great arteries (d‐TGA), and two total anomalous pulmonary venous drainage (TAPVD). Of these, five with AAO, nine with d‐TGA and likely both with TAPVD could potentially have been detected with oximetry screening.ConclusionThe antenatal detection of critical cardiac anomalies continues to improve in New Zealand. Despite high antenatal detection rates for most lesions, universal postnatal oximetry screening has the potential to improve early detection.

Imperforate anus associated with anomalous pulmonary venous return in scimitar syndrome. Case report from a tertiary hospital in Ethiopia

BackgroundScimitar syndrome is a rare form of partial anomalous pulmonary venous drainage associated with pulmonary hypertension and congestive heart failure that may lead to death in the newborn infant. Although it is described with anomalies of the lung, heart and their vascular structure, extremely rare association with imperforate anus had been reported. The third case of Scimitar syndrome and imperforate anus will be reported in this case report.Case presentationA 3 days old male neonate with imperforate anus presented with abdominal distention. Loop colostomy was done to relieve abdominal distension.The chest x-ray revealed a curved shadow on the right mid lung zone extending to the diaphragm abutting and indenting the inferior vena cava (scimitar sign).Abdominal ultrasound, transthoracic echocardiography and computerized tomographic angiography confirmed the presence of Scimitar vein and associated dextro-position of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, secundum atrial septal defect with bidirectional shunt, patent ductus arteriosus, pulmonary hypertension, left superior vena cava, and systemic collateral arteries feeding the lower lobe of the right lung.The rare association of scimitar syndrome with imperforate anus is discussed.ConclusionScimitar syndrome associated with imperforate anus with and without VACTERL association has been reported previously only in four cases. The knowledge of association between imperforate anus and Scimitar syndrome helps for early detection and management of cases. It is recommended to have high index of suspicion in every newborn with imperforate anus to check for symptoms of dextro-position of the heart, right lung hypoplasia which may be indicate scimitar syndrome.

Supracardiac Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which there is developmental absence of connection of all four pulmonary veins with the left atrium. To report a rare case and share our experience in surgery and post-operative management for supracardiac TAPVC. Patient with supracardiac TAPVC with atrial septal defect (ASD) secundum variety with rudimentary patent ductus arteriosus (PDA) underwent rechanneling of pulmonary veins to left atrium (LA) with gluteryldehye treated autologous pericardial patch closure of ASD with ligation of ascending vertical vein and ligation of rudimentary PDA.Post operatively there were no events of pulmonary hypertensive crisis, low cardiac output syndrome, right heart failure or conduction defect were observed and echocardiogram showed adequate pulmonary venous drainage with no residual shunt across the interatrial septum. Marked development in surgical results of TAPVC has been observed in recent years with declining mortality rate from 65% in early sixties to 5% in current surgical scenerio.
 KYAMC Journal Vol. 10, No.-2, July 2019, Page 118-121