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Persistent Left Superior Vena Cava

Persistent Left Superior Caval Vein Connecting to the Left-Sided Atrium


Presentation

  • We hereby present a preterm infant who presented with a triad of PLSVC, laryngeal atresia, and esophageal atresia.[ncbi.nlm.nih.gov]
  • He had presented with a history of worsening dyspnoea. To the best of our knowledge, this is the first reported case of a single patient with such a vast constellation of anomalies.[ncbi.nlm.nih.gov]
  • We present the first reported case of ACS and PLSVC occurring after thrombosis of the innominate vein (IV) after central venous catheter placement. The patient presented with atypical subacute chest pain and recurrent extrasystoles.[ncbi.nlm.nih.gov]
  • In the present case, previously performed enhanced CT with its routine manner, i.e., contrast through the right arm, missed this anomaly.[ncbi.nlm.nih.gov]
  • A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient.[ncbi.nlm.nih.gov]
Hypoxemia
  • A 54-year-old woman with a history of multiple cardiac surgeries suffered from hypoxemia caused by a right-to-left intra-cardiac shunt due to coronary sinus (CS) anomaly with persistent left superior vena cava (PLSVC).[ncbi.nlm.nih.gov]
  • This then places the patient at risk for systemic hypoxemia, paradoxical embolic events, and cerebral abscess.[ncbi.nlm.nih.gov]
  • Findings in these patients include hypotension, hypoxemia, seizures, and impairment of cognitive ability.[howlingpixel.com]
  • Iflarge and numerous, however, these lesionsmay cause hypoxemia, stroke, and paradoxicemboli.[vdocuments.site]
Pathologist
  • All clinicians and scientists interested in birth defects, including pediatricians, geneticists, genetic counselors, obstetricians, and pediatric pathologists, will find this book to be an invaluable source of information.[books.google.com]
Intravenous Drugs
  • الصفحة 77 - Influence of human immunodeficiency virus i infection and degree of immunosuppression in the clinical characteristics and outcome of infective endocarditis in intravenous drug users. Arch Intern Med... ‏[books.google.com]
Dental Abscess
  • The primary source of infection was likely his dental abscesses. He underwent intravenous antibiotic therapy for a total of 6 weeks and was then referred to a specialised vascular centre for further management of his congenital anomaly.[ncbi.nlm.nih.gov]
Heart Disease
  • Broberg, Adult Congenital Heart Disease, Critical Heart Disease in Infants and Children, 10.1016/B978-1-4557-0760-7.00076-0, (905-910.e2), (2019).[doi.org]
  • The potential importance of PLSVC for the surgical risk during operation for congenital heart disease and for instability of the cardiac rhythm is emphasized.[ncbi.nlm.nih.gov]
  • An associated heart disease must always be excluded in these patients.[ncbi.nlm.nih.gov]
  • KEYWORDS: Coarctation of the aorta; Incidence in congenital heart disease; Incidence in normal population; Persistent left superior vena cava; Trisomy 21[ncbi.nlm.nih.gov]
  • Author information 1 Fetal Heart Disease Maternal Fetal Medicine Research Laboratories, Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, No. 2 Anzhen Road, Chaoyang District, 100029, Beijing, China. 2 Fetal Heart Disease[ncbi.nlm.nih.gov]
Vascular Disease
  • Jihun Ahn, Sang-Ho Park, Dohoi Kim, Taehoon Kim, Seongsik Jo, Hyeokgyu Lee and Ara Cho, Role of Echocardiography in Sinus Venosus Atrial Septal Defect Combined with Systemic and Pulmonary Vascular Disease, Journal of Cardiovascular Ultrasound, 20, 1,[doi.org]
S3 Gallop
  • Physical examination revealed grade III systolic murmur on the apex, S3 gallop, and a prominent jugular venous pulse.[innovationsincrm.com]
Diastolic Murmur
  • The cardio-respiratory physical examination was normal except for a mild diastolic murmur at the lower left sternal border.[oatext.com]
Hemianopsia
  • Visual loss and central venous catheterization: cortical blindness and hemianopsia after inadvertent subclavian artery entry. J Neuroophthalmol 2000; 20: 32–4. 7. Mooney DP, Snyder CL, Holder TM.[journals.lww.com]
Altered Mental Status
  • During his hospitalisation, he also developed altered mental status which was found to be secondary to systemic emboli by a head MRI. The primary source of infection was likely his dental abscesses.[ncbi.nlm.nih.gov]

Workup

  • Further workup to evaluate for an infectious source revealed an incidental finding of persistent left superior vena cava via transesophageal echocardiography. This finding was confirmed by cardiac MRI.[ncbi.nlm.nih.gov]
  • Abstract An echocardiogram is the most common diagnostic imaging done in chest pain evaluation workup. Persistent left superior vena cava is a rare thoracic congenital anomaly but commonly reported one in the literature.[elynsgroup.com]
  • Presentation Isolated UCS defects may not express any symptoms and can be found during the workup for a concomitant lesion in asymptomatic patients. [12, 13] When present, symptoms may range from mild cardiomegaly to overt congestive heart failure.[emedicine.medscape.com]
Left Axis Deviation
  • On the electrocardiogram, left axis deviation may be noted. Chest radiograph may show an unusual vertical left upper mediastinal silhouette. Persistent left SVC does not, in itself, produce any physiologic derangement.[journals.lww.com]
  • In 10% of patients, a PLSVC may drain into LA. [18] A large RΰL shunt requires surgical correction ECG may show left axis deviation of the P wave with shortened PR interval.[annals.in]
Prolonged PR Interval

Treatment

  • This case reflects a 5-month-old boy who acquired a severe viral pneumonia leading to respiratory failure and ECMO treatment.[ncbi.nlm.nih.gov]
  • The complex of UCS and PLSVC is one such abnormality and its treatment requires careful assessment of other concomitant cardiac abnormalities to prevent post-treatment haemodynamic complications.[ncbi.nlm.nih.gov]
  • Medicines and surgery are the two main treatments for aneurysms.[icdlist.com]
  • The complex structural anomalies presented great technical challenges for interventional treatments.[ncbi.nlm.nih.gov]
  • Proper and advanced image studies including ultrasound, contrast-enhanced venography, CT, and magnetic resonance imaging may be necessary for understanding the potential vascular abnormality and guiding the following treatment. Copyright 2013.[ncbi.nlm.nih.gov]

Prognosis

  • Most cases are associated with anomalous systemic venous return, such as persistent left superior vena cava.The diagnosis of this lesion is important to the prognosis of the patient, but it is often difficult because of nonspecific clinical features.Here[ncbi.nlm.nih.gov]
  • […] arteries, aortic stenosis, bicuspid aortic valve, truncus arteriosus is more common than the isolated form of complete IAA. [2], [6] In the neonatal or early childhood period, patient presents with symptoms of congestive heart failure which carries poor prognosis[avicennajmed.com]
  • Long-term prognosis in abnormal SVC development is good in the absence associated CHD [ 4 ].[bmcresnotes.biomedcentral.com]
  • Treatment and prognosis Except in cases where a large right to left shunt is present, a left-sided SVC has essentially no physiologic impact and is entirely asymptomatic.[radiopaedia.org]
  • Interauricular Septum Interventricular Septum Absence of Cardiac Septa Aortic Septum Pulmonary Stenosis and Atresia Pulmonary Artery Dilatation Aortic Stenosis or Atresia Primary Patency and Ductus Arteriosus Aorta Coarctation Aorta Hypoplasia Diagnosis Prognosis[embryology.med.unsw.edu.au]

Etiology

  • Cerebral abscess is a serious neurological condition that is often of unclear etiology. Management is usually medical therapy with or without direct drainage, and when patients have recurrent episodes a structural abnormality should be considered.[ncbi.nlm.nih.gov]
  • […] is only common anomaly of SVC (90% of this anomaly) In other 10%, persistent SVC connects to left atrium Most with connection to left atrium have associated ASD or heterotaxy syndromes This produces a right-to-left shunt of a rather small magnitude Etiology[learningradiology.com]
  • Complete evaluation of the pulmonary venous anatomy is important in determining the presence of other pathways for right-to-left shunting as possible etiologies for paradoxical embolism.[invasivecardiology.com]
  • PLSVC is not the only cause of a dilated coronary sinus, however, with other etiologies including elevated right atrial pressure (most common), coronary arterio-venous fistula, partial anomalous pulmonary venous return, or an "unroofed" coronary sinus[cardiovascularultrasound.biomedcentral.com]
  • Additional etiologies of a dilated coronary sinus include elevated right atrial pressure, partial anomalous pulmonary venous return to the coronary sinus, and coronary arteriovenous fistula [3].[oatext.com]

Epidemiology

  • The radiological evaluations, epidemiology, embryology and clinical features of the anomalies are discussed.[ncbi.nlm.nih.gov]
  • This study examines the epidemiologic, clinical, and morphologic characteristics of a cohort of patients with PLSVC draining into the coronary sinus.[ncbi.nlm.nih.gov]
  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.com]
  • Hypothesis : This study examines the epidemiologic, clinical, and morphologic characteristics of a cohort of patients with PLSVC draining into the coronary sinus.[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • 0.5% of the general population, with 8% draining into the left atrium. [5] UCS is seen in 75% of patients with a PLSVC that drains into the left atrium and is usually associated with other forms of congenital heart disease and heterotaxy syndromes. [1] Pathophysiology[emedicine.medscape.com]
  • Theretwo pathophysiologic entities that will be discussed: un-fed coronary sinus syndromes and baffle obstruction intients with complete TGA (transposition of the great arter-) that have undergone atrial switch procedures.The most common systemic venous[vdocuments.mx]

Prevention

  • For primary prevention of sudden death, the patient was also fitted with an additional subcutaneous implantable cardioverter defibrillator (S-ICD).[ncbi.nlm.nih.gov]
  • The complex of UCS and PLSVC is one such abnormality and its treatment requires careful assessment of other concomitant cardiac abnormalities to prevent post-treatment haemodynamic complications.[ncbi.nlm.nih.gov]
  • In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery. 2017, Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • To prevent PVO, sufficient tension-free anastomosis is necessary. When dilated, the coronary sinus becomes an obstacle for obtaining sufficient incision length in the left atrial cavity.[ncbi.nlm.nih.gov]
  • In addition, during heart surgery, awareness of such variations may prevent major complications, such as hemorrhage or damage to vascular structures, and possibly also provide new insights and perspectives to cardiovascular surgeries.[ncbi.nlm.nih.gov]

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