scholarly journals Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Neerod Kumar Jha ◽  
Haitham Talo ◽  
Laszlo Kiraly ◽  
Nishant Shah ◽  
Aref Al Hakami ◽  
...  
Keyword(s):  
Venous Return ◽  
Pulmonary Veins ◽  
Posterior Wall ◽  
Right Atrial ◽  
Excellent Outcome ◽  
Venous Circulation ◽  
Echocardiographic Image ◽  
Anomalous Pulmonary Venous Return ◽  
Septum Primum ◽  
Absent Pericardium

Abstract Background Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of pulmonary vein at various levels. Case presentation A 3-month-old infant was presented to us with history of poor feeding, respiratory distress and desaturations. The routine echocardiographic investigation initially confirmed the diagnosis of an atrial septal defect with dextrocardia. However, due to disproportionate severity of symptoms and congestive heart failure a cardiac computer tomography angiogram was done that revealed a rare finding of connection of pulmonary veins fused with the posterior atrium, but on the rightward side of the deviated atrial septum. Therefore, pulmonary veins entered a sinus that drains directly into the right atrial superior-posterior wall. During surgical repair, we found an area of absent pericardium in the diaphragmatic surface of the heart. The patient underwent total repair of the TAPVR and patch reconstruction of the pericardial defect. The patient is doing well at 6-month follow up. Conclusions The septum primum malposition defect resulting in TAPVR is a very rare congenital anomaly that can be rarely seen without any heterotaxy. The anomalous features including absent pericardium and dextrocardia were present in this patient have not been described previously with TAPVR. Therefore, we have hypothesized the embryological correlation of absent pericardium and cardiac malposition in such case. Transthoracic echocardiography with Doppler interrogation is a reliable method for diagnosing this condition. In case of suboptimal echocardiographic image due to cardiac position, unclear anatomy or unexplained symptoms, advanced imaging such as computer tomographic angiography or cardiac magnetic resonance imaging can be very helpful. Preoperative proper diagnosis of this anomaly facilitates successful surgical management with excellent outcome.

Single-Patch Fold-Back Technique for Augmentation of the Superior Vena Cava in Sinus Venosus Atrial Septal Defect Associated with Partial Anomalous Pulmonary Venous Return

2015 ◽  
Vol 17 (6) ◽  
pp. 282
Author(s):  
Suguru Ohira ◽  
Kiyoshi Doi ◽  
Takeshi Nakamura ◽  
Hitoshi Yaku
Keyword(s):  
Atrial Septal Defect ◽  
Superior Vena Cava ◽  
Septal Defect ◽  
Venous Return ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Sinus Venosus ◽  
Right Atrial ◽  
Anomalous Pulmonary Venous Return

Sinus venosus atrial septal defect (ASD) is usually associated with partial anomalous pulmonary venous return (PAPVR) of the right pulmonary veins to the superior vena cava (SVC), or to the SVC-right atrial junction. Standard procedure for repair of this defect is a patch roofing of the sinus venosus ASD and rerouting of pulmonary veins. However, the presence of SVC stenosis is a complication of this technique, and SVC augmentation is necessary in some cases. We present a simple technique for concomitant closure of sinus venosus ASD associated with PAPVR and augmentation of the SVC with a single autologous pericardial patch.

Pięciomiesięczne niemowlę z całkowitym nieprawidłowym spływem żył płucnych – opis przypadku

2018 ◽  
Vol 22 (1) ◽  
Author(s):  
Agnieszka Pskit ◽  
Anna Piórecka-Makuła ◽  
Małgorzata Gołąbek-Dylewska ◽  
Danuta Roik ◽  
Bożena Werner
Keyword(s):  
Heart Failure ◽  
Left Atrium ◽  
Primary Care Physicians ◽  
Venous Return ◽  
Pulmonary Veins ◽  
Main Pulmonary Artery ◽  
Vena Cava ◽  
Right Atrial ◽  
Oxygen Saturation Level ◽  
Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return (TAPVR) is a rare congenital cyanotic heart disease, in which the lack of connection between all four pulmonary veins and left atrium is present. The interatrial communication allows the oxygenated blood flow to systemic circulation through the left atrium. The authors present a 5-month-old girl with heart failure symptoms. Poor weight gain, feeding difficulties, tachypnoe with use of accessory breathing muscles, hepatomegaly and low oxygen saturation level were observed. Serum concentration of NT-proBNP was elevated. Electrocardiography revealed right atrial and right ventricular enlargement. Chest X-Ray showed cardiomegaly and increased pulmonary vascular markings. The echocardiographic evaluation revealed pulmonary venous drainage to superior vena cava, right-sided heart chambers enlargement, main pulmonary artery dilation and atrial septal defect. Computed tomographic angiography was performed in order to acquire more accurate image of pulmonary veins connection. Supracardiac type of total anomalous pulmonary venous return was diagnosed and cardiosurgical correction was performed. The authors pay attention to the importance of recognizing the clinical symptoms of heart failure in infants by primary care physicians.

scholarly journals A rare case of obstructive supracardiac TAPVR (Total Anomalous Pulmonary Venous Return) with aberrant right subclavian artery.

2021 ◽  
Vol 28 (7) ◽  
pp. 1058-1060
Author(s):  
Fazal ur Rehman ◽  
◽  
Sabiha Khan ◽  
Waqas Ali ◽  
Asif Ali Khuhro ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Subclavian Artery ◽  
Right Atrium ◽  
Venous Return ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Aberrant Right Subclavian Artery ◽  
Vertical Vein ◽  
Anomalous Pulmonary Venous Return ◽  
Tract Infections

Congenital aortic arch malformations manifest a broad-spectrum of differences and abnormalities that come from disturbed embryogenesis of branchial arches. Current case was a 10 months old baby girl with length of 69 cm (less than –3 SD) and weight of 5.5 kg (less than –3 SD). The patient had history of recurrent lower respiratory tract infections since the time of birth and failure to gain adequate weight since the time of birth. The patient has been having multiple check-ups with registered medical practitioners in the nearby locality and multiple courses of antibiotics with only partial resolution of symptoms. The 2-D echocardiogram showed her to be a case of supracardiac type of “Total Anomalous Pulmonary Venous Return (TAPVR)”. All pulmonary veins making a confluence and draining into the right atrium. Significant turbulence observed at the level of superior vena cava to right atrium junction. A level of obstruction was recorded at the junction of the confluence of pulmonary veins and the vertical vein. There was aberrant right subclavian artery from the aortic arch as its third branch with no obstruction or aneurysm formation, having retrotracheal and esophageal course.

Abnormalities of Pulmonary Venous Return

Chest Imaging ◽  
2019 ◽  
pp. 533-537
Author(s):  
Kristopher W. Cummings
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Venous Return ◽  
Pulmonary Veins ◽  
Lower Lobe ◽  
Scimitar Syndrome ◽  
Systemic Vein ◽  
Anomalous Pulmonary Venous Return ◽  
The Right

Abnormalities of pulmonary venous return in adults result from anomalous drainage of one or more pulmonary veins into a systemic vein, resulting in a left-to-right shunt. Partial anomalous pulmonary venous return (PAPVR) is most commonly encountered in adults in the upper lobes. PAPVR in the right upper lobe is commonly associated with a sinus venous atrial septal defect, whereas in the right lower lobe it is commonly encountered in association with other anomalies in Scimitar syndrome. Left upper lobe PAPVR is usually isolated. In some instances, patients can develop pulmonary over-circulation and hypertension, necessitating intervention. This chapter emphasizes CT and MR features key to recognizing and diagnosing these anomalies.

Abstract 17056: Report Of An Adult Living With Undiagnosed Total Anomalous Pulmonary Venous Return

Circulation ◽  
2021 ◽  
Vol 144 (Suppl_2) ◽  
Author(s):  
Kent Beam ◽  
Jorge Alegria ◽  
Matthew Schwartz
Keyword(s):  
Venous Return ◽  
Pulmonary Veins ◽  
Innominate Vein ◽  
Exercise Intolerance ◽  
Pulmonary Venous Obstruction ◽  
X Ray ◽  
Venous Structure ◽  
Vertical Vein ◽  
Anomalous Pulmonary Venous Return ◽  
Chest X Ray

Case Presentation: 25-year-old male presented with cellulitis and abscess of the lower extremity. He was an immigrant from Guatemala. He worked in landscaping and played soccer with limited exertion. His exam was notable for cyanosis and clubbing of all digits. A II/VI systolic ejection murmur at the left upper sternal border and a mildly accentuated P2 was auscultated. ECG was notable for right axis deviation and right ventricle hypertrophy. Chest x-ray showed a widened mediastinum with perihilar vascular prominence. CT chest with contrast demonstrated all pulmonary veins forming a large, coalescing venous structure draining into the left innominate vein. Transthoracic echocardiogram confirmed total pulmonary venous return by way of a large vertical vein draining into a dilated innominate vein and a 2.7 cm ostium secundum ASD. Discussion: Total anomalous pulmonary venous return (TAPVR) is a rare, often deadly condition. Most patients die in infancy. In the rare adult patient with unrepaired TAPVR, exercise intolerance, cyanosis, clubbing of the digits, and growth retardation is often noted. A right ventricular heave with auscultation of a fixed, split second heart sound with a loud pulmonic component is common. On chest x-ray, the classic “snowman” appearance is seen with vertical vein connections as in our patient. Right heart enlargement represents the base, and an enlarged connecting vertical vein and SVC make up the upper portion. Echocardiography can demonstrate the anomalous pulmonary venous conduit coursing behind the left atrium. CT angiography can also reveal TAPVR and the patient’s specific anatomy. Amongst the few surviving adult TAPVR patients, similar characteristics include large ASDs, short anomalous veins, and absence of pulmonary venous obstruction. A large interatrial connection provides adequate systemic flow. The latter two factors reduce pulmonary vascular resistance, hence improving pulmonary flow and systemic oxygen concentrations.

Severely obstructed totally anomalous pulmonary venous return with residual connection to the left atrium

2007 ◽  
Vol 17 (4) ◽  
pp. 441-444
Author(s):  
Lik Eng Loh ◽  
Katherine Brown ◽  
Jan Marek
Keyword(s):  
Left Atrium ◽  
Pulmonary Disease ◽  
Conventional Therapy ◽  
Venous Return ◽  
Pulmonary Veins ◽  
Diagnostic Difficulty ◽  
Anomalous Pulmonary Venous Return

AbstractTotally anomalous pulmonary venous return may present diagnostic difficulty to clinicians, as it often appears similar to severe neonatal pulmonary disease. We describe a neonate who presented with severely obstructed pulmonary venous return, but with a residual venous connection to the left atrium. The unusual anatomy confounded the diagnosis. To our knowledge, this particular permutation has not previously been described. Our experience indicates that echocardiographic interrogation should be repeated in cases where there is no response to conventional therapy, and all individual pulmonary veins should be observed carefully.

Nichtinvasive Diagnostik von Vorhofseptumdefekten und fehlmündenden Lungenvenen mit der Kernspintomographie

1989 ◽  
Vol 28 (05) ◽  
pp. 172-180 ◽  
Author(s):  
P. Theissen ◽  
U. Sechtem ◽  
U. Mennicken ◽  
H. H. Hilger ◽  
H. Schicha
Keyword(s):  
Venous Return ◽  
Spin Echo ◽  
Pulmonary Veins ◽  
Volume Overload ◽  
Interatrial Septum ◽  
Gradient Echo ◽  
Anomalous Pulmonary Venous Return ◽  
Mri Diagnosis ◽  
Magnetic Resonance Imaging Mri ◽  
Spin Echo Technique

The present study was to evaluate the diagnostic information that may be obtained in patients with atrial septal defect (ASD) and/or with anomalous pulmonary venous return using spin echo and gradient echo magnetic resonance imaging (MRI). The interatrial septum and pulmonary veins were studied in 31 patients and in 16 healthy volunteers. In all patients cardiac anatomy and dilatation due to right-sided volume overload was well depicted. In 87% of the patients examined by spin echo technique and in 100% of the patients examined by gradient echo technique the defect was reliably documented. All cases of anomalous pulmonary venous return were shown. MRI diagnosis of ASD was improved by combined use of spin echo and gradient echo techniques. The new MRI sequence was particularly helpful in patients with small ASDs and anomalous pulmonary venous return.

scholarly journals Right Upper Lobe Partial Anomalous Pulmonary Venous Connection

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Christos Tourmousoglou ◽  
Christina Kalogeropoulou ◽  
Efstratios Koletsis ◽  
Nikolaos Charoulis ◽  
Christos Prokakis ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Right Atrium ◽  
Venous Return ◽  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Systemic Vein ◽  
Anomalous Pulmonary Venous Return ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Partial anomalous pulmonary venous return (PAPVR) is a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. We report a case of a 45-year-old male with PAPVR to superior vena cava which was incidentally discovered during a right lower bilobectomy for lung cancer.

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