Abstract 14310: Elephant Sitting on My Chest: A Tale of Two Vessels

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Anudeep K Dodeja ◽  
Patrick I McConnell ◽  
Elisa A Bradley ◽  
Curt Daniels ◽  
Laxmi S Mehta ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Symptomatic Relief ◽  
Pulmonary Veins ◽  
Main Pulmonary Artery ◽  
Laboratory Data ◽  
Venous Drainage ◽  
Sinus Venosus ◽  
Exercise Intolerance ◽  
Extrinsic Compression ◽  
Sinus Venosus Defect

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Understanding the spectrum of sinus venosus interatrial communications

2016 ◽  
Vol 27 (3) ◽  
pp. 418-426 ◽  
Author(s):  
Justin T. Tretter ◽  
Sathish Chikkabyrappa ◽  
Diane E. Spicer ◽  
Carl L. Backer ◽  
Ralph S. Mosca ◽  
...  
Keyword(s):  
Cardiac Development ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Interatrial Septum ◽  
Sinus Venosus ◽  
Group 13 ◽  
Common Wall ◽  
Sinus Venosus Defect ◽  
Autopsy Specimens ◽  
Anomalous Pulmonary Venous Connections

AbstractBackgroundIt is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects.MethodsWe reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens.ResultsWe evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases.ConclusionAppreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

scholarly journals The morphogenesis and associated anomalous pulmonary venous drainage in sinus venosus defect

2020 ◽  
Vol 61 (1) ◽  
pp. 92-99
Author(s):  
Hsin-Mao Hsu ◽  
Ya-Ting Chang ◽  
Wen-Jen Su ◽  
Jaw-Ji Chu ◽  
Yu-Sheng Chang ◽  
...  
Keyword(s):  
Venous Drainage ◽  
Sinus Venosus ◽  
Anomalous Pulmonary Venous Drainage ◽  
Sinus Venosus Defect

Absence of the coronary sinus with coronary venous drainage into the main pulmonary artery

2012 ◽  
Vol 23 (5) ◽  
pp. 759-762
Author(s):  
Kiyoshi Ogawa ◽  
Takashi Hishitani ◽  
Kenji Hoshino
Keyword(s):  
Coronary Angiography ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Contrast Medium ◽  
Coronary Arteries ◽  
Coronary Sinus ◽  
Main Pulmonary Artery ◽  
Venous Drainage ◽  
Anterior Wall ◽  
The Right

AbstractWe describe the case of a 9-year-old girl demonstrating isolated absence of the coronary sinus with abnormal coronary venous drainage into the main pulmonary artery. Coronary angiography showed normal coronary arterial trees and contrast medium from both coronary arteries drained into the main pulmonary artery via an abnormal cardiac vein on the anterior wall of the right ventricle.

scholarly journals The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

2020 ◽  
Vol 5 (2) ◽  
pp. 1-4
Author(s):  
Merjema Karavdic ◽  
Keyword(s):  
Neonatal Period ◽  
Pulmonary Veins ◽  
Arteriovenous Shunt ◽  
Sinus Venosus ◽  
Vein Of Galen ◽  
Cardiac Defect ◽  
Congenital Cardiac Defect ◽  
Sinus Venosus Defect

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

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

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Ruiz-Zamora ◽  
L Alvarez-Roy ◽  
G Pinillos-Francia ◽  
A Gutierrez-Fernandez ◽  
M Gomez-Llorente ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Vein ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Sinus Venosus ◽  
The Right

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

The Physical Properties of Human Pulmonary Arteries and Veins

1978 ◽  
Vol 55 (5) ◽  
pp. 477-484 ◽  
Author(s):  
J. Banks ◽  
F. V. McL. Booth ◽  
E. H. MacKay ◽  
B. Rajagopalan ◽  
G. De J. Lee
Keyword(s):  
Pulmonary Artery ◽  
Physical Properties ◽  
Lung Disease ◽  
Left Atrium ◽  
Pulmonary Veins ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Post Mortem ◽  
Arteries And Veins

1. We have studied the extensibility of circumferential strips of main pulmonary artery and large pulmonary veins obtained at post mortem from patients of all ages, dying from conditions other than heart and lung disease. 2. The vessel strips were submitted to increasing loads in a tension balance. The pulmonary arteries were found to be readily extensible. This extensibility became less with increasing age. The pulmonary veins were virtually inextensible at all ages. 3. It is postulated that the large extraparenchymal pulmonary veins have a capacitative role in supplying blood from the lungs to the left atrium. This may be accomplished by their collapsible nature, as they have little capability of distension.

scholarly journals An aberrant mediastinal mediobasal segmental pulmonary artery in a patient with lung cancer undergoing right lower lobectomy: a case report

2021 ◽  
Author(s):  
Kazuyuki Komori ◽  
Hiroshi Hashimoto ◽  
Kotaro Yoshikawa ◽  
Koji Kameda ◽  
Shinichi Taguchi ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Pulmonary Artery ◽  
Ct Angiography ◽  
Multidetector Ct ◽  
Mediastinal Lymph Node ◽  
Pulmonary Veins ◽  
Main Pulmonary Artery ◽  
Lower Lobectomy ◽  
The Right ◽  
Right Lower Lobectomy

Abstract Background A mediastinal mediobasal segmental pulmonary artery (A7) from the right main pulmonary artery is extremely rare. Case presentation: We have reported a case of a 71-year-old woman with aberrant A7 who underwent right lower lobectomy for lung cancer (cT1bN0M0, stage IA2). Preoperative three-dimensional computed tomography (CT) angiography revealed an aberrant mediastinal A7 from the right main pulmonary artery. Right lower lobectomy and mediastinal lymph node dissection were performed. Intraoperatively, A7 was observed between the superior and inferior pulmonary veins, and at the front of the lower bronchus near the anterior hilum. The artery was carefully dissected from the caudal side after dissection of the inferior pulmonary vein. Then, the lung parenchyma, which was within the fissure due to poor lobulation between the middle and lower lobes, was safely divided. Conclusions Thoracic surgeons need to evaluate CT angiography or enhanced multidetector CT carefully at preoperative conferences and always keep this anomaly in mind.

DEVELOPMENT OF THE PULMONARY VEINS

PEDIATRICS ◽  
1956 ◽  
Vol 18 (6) ◽  
pp. 880-887
Author(s):  
Catherine A. Neill
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Venous Drainage ◽  
Atrial Septum ◽  
Sinus Venosus ◽  
Human Embryos ◽  
Left Auricle ◽  
Serial Sections ◽  
The Common ◽  
Common Pulmonary Vein

The examination of serial sections of human embryos between 24 and 34 days (3 to 11 mm) and the use of plastic reconstructions, showed that the common pulmonary vein develops as an outgrowth from the medial superior wall of the left auricle and unites with the angioblastic plexus of the developing lung bud. No evidence was found that the vein connects directly with the sinus venosus in the early stages, and later shifts in position as the atrial septum grows. Anomalous pulmonary venous drainage is classified in four main types, and theories of development are briefly discussed.

Septum primum malposition defect and inferior sinus venosus defect: a rare association

2014 ◽  
Vol 25 (7) ◽  
pp. 1389-1392 ◽  
Author(s):  
Deepa Prasad ◽  
Christopher Snyder ◽  
Ravi Ashwath
Keyword(s):  
Respiratory Distress ◽  
Pulmonary Veins ◽  
Atrial Septum ◽  
Sinus Venosus ◽  
Rare Association ◽  
Septum Primum ◽  
Sinus Venosus Defect ◽  
The Right ◽  
Inferior Sinus Venosus Defect ◽  
Septum Secundum

AbstractWe present a case of a 2-year-old girl who presented with respiratory distress and umbilical abscess, and was found to have an inferior sinus venosus defect, malposition of the atrial septum primum, absent septum secundum, and anomalous drainage of the right upper and lower pulmonary veins to the right atrium.

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