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Anomalies of Great Veins, Congenital

Congenital anomaly of the inferior vena cava


Presentation

  • […] if the condition was present at the time of inpatient admission.[icd.codes]
  • Presentation varies with the severity of the obstruction. Critical coarctation of the aorta presents with cyanosis and shock when the ductus arteriosus closes.[ahcmedia.com]
  • Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery[icd10data.com]
  • Among the seven patients with no DVT history two presented agenesis of the IVC intrahepatic portion, three presented double IVC, one presented transposition of the IVC and another presented retrocaval ureter.[scielo.br]
  • The greater the number of pulmonary veins successfully returning to the right side, the more severe are the presenting symptoms.[vhlab.umn.edu]
Turkish
  • Scimitar syndrome is named for the crescent- or turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.[icd10data.com]
  • The anomalous vein gently curves to the right cardiophrenic angle and is shaped like a Turkish sword ('Scimitar') Right lower lobe anomalous venous return into the azygos vein. On the left another right lower lobe anomalous return.[radiologyassistant.nl]
Short Stature
  • Frequent structural anomaly Males 2x more then females Turner's syndrome (45, X), short stature, low-set ears, Shield chest, café au lait spots, left neck, lymphedema, short fourth metacarpals, primary amenorrhea due to accelerated loss of oocytes, rib[quizlet.com]
  • stature Q872Congenital malformation syndromes predominantly involving limbs Q873Congenital malformation syndromes involving early overgrowth Q8740Marfan's syndrome, unspecified Q87410Marfan's syndrome with aortic dilation Q87418Marfan's syndrome with[cms.gov]
Developmental Disabilities
  • As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image.[cdc.gov]
Claustrophobia
  • In patients with a cardiac pacemaker or claustrophobia, CT may be a better choice.[radiologykey.com]
Epistaxis
  • […] for embolization, namely, embolic microspheres in the form of particles to treat hemorrhages, arteriovenous malformations, hemangioendothelioma, cerebral aneurysms, muscle hypertrophy, hypertrophic cardiomyopathy, gastrointestinal bleeding, trauma, epistaxis[ro.glosbe.com]
Excitement
  • The treatment depends on the type of tachycardia, and the aim is to either decrease the excitability or increase the conduction time to block a re-entrant mechanism.[sonoworld.com]
  • To reduce the sensitivity of SSFP to field inhomogeneities, which can be problematic for large field-of-view imaging, a nonselective radio frequency excitation is used to shorten the repetition time.[radiologykey.com]
Lethargy
  • Infants present with cyanosis, lethargy, tachypnea, respiratory infections, and poor growth. An anomalous pulmonary venous return in which the right pulmonary vein is not connected to the left atrium but to the inferior vena cava.[icd10data.com]
Agitation
  • This was confirmed with transesophageal echocardiography after injection of agitated saline in the left arm. Echocardiography image showing bubbles first entered the LA ( green arrows ).[radiologykey.com]

Workup

Mediastinal Mass
  • A brachiocephalic vein aneurysm can manifest as a mediastinal mass on a supine radiograph but can be barely seen with the patient in the erect position. In this case CT or MR can be diagnostic (Fig. 26.4 ).[radiologykey.com]

Treatment

  • These may also require a series of treatments about six weeks apart to block all of the abnormal vessels.[hopkinsmedicine.org]
  • Treatment Reimplantation of coronary arteries Aortocoronary bypass[amboss.com]
  • However, since most CVMs do not need treatment, or it is delayed until the need for treatment is more obvious, it is now rarely necessary to get angiograms as a first step.[vascularcures.org]
  • Today, an alternative treatment called embolization provides significant improvements. Embolization Embolization, in which the blood vessels are intentionally closed, is used as a treatment for many vascular malformations.[ucsfbenioffchildrens.org]
  • Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants.[icdlist.com]

Prognosis

  • Prognosis: Cardiac failure is never seen in fetal life as well as postnatally.[sonoworld.com]
  • Prognosis is good after surgery. Prognosis is poor in neonates with obstructive TAPVR. Long term follow up is needed to assess restenosis and late arrhythmias.[hawaii.edu]
  • Heart failure and cardiogenic shock, if untreated Treatment Initially : continuous infusion of PGE1 prior to heart surgery Definite therapy Staged surgical correction in three steps (Norwood, Glenn, and Fontan procedure) Alternative: heart transplant Prognosis[amboss.com]
  • Absence of a main branch pulmonary artery No treatment required, prognosis good. Absence of a lung (agenesis) Compatible with normal life. C. Pulmonary Arteriovenous fistulas Requires surgical treatment. D. Alteration of Pulmonary Circulation.[anatomyatlases.org]
  • Frequently a tethering of anterior and/or posterior tricuspid leaflet to RV wall is present. ( Fig. 5 ) These modifications lead to atrialization of a RV part. 1 The atrialized RV/total RV ratio more than 30% indicates severe disease with poor prognosis[tmj.ro]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • References: [19] [1] [1] [20] [2] [21] [22] [23] [24] [25] Truncus arteriosus Definition Failed separation of aorta and pulmonary artery during development single trunk that receives output from both ventricles Etiology Failure of cardiac neural crest[amboss.com]
  • The echocardiography offers a complete positive, etiologic (congenital, rheumatic, carcinoid syndrome, infectious, or tumor that mimicks the TS), severity and functional diagnosis.[tmj.ro]
  • WPW is the most common underlying etiology. The treatment may include administration of adenosine.[ahcmedia.com]
  • Etiology: The etiology of heart defects is heterogeneous and probably depends on the interplay of multiple genetic and environmental factors, including maternal diabetes mellitus or collagen disease, exposure to drugs such as lithium, and viral infections[sonoworld.com]

Epidemiology

  • References: [15] Tricuspid valve atresia Definition Absent or rudimentary tricuspid valve, resulting in no blood flow between the right atrium and the right ventricle Epidemiology Third most common cyanotic heart defect Almost always accompanied by ASD[amboss.com]
  • The epidemiology and genetics of congenital heart disease. Clin in Perinatol 2001; 28 : 1–10. 3 Kohner EM, Allen EM, Saunders KB, Emery VM, Pallis C.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • Overview of cyanotic congenital heart defects General pathophysiological processes Heart defects may lead to the formation of connections between the right and the left heart ( see “Pathophysiology” in acyanotic congenital heart defects ).[amboss.com]
  • The general physical symptoms of these pathophysiological alterations are growth retardation, decreased exercise tolerance, recurrent respiratory infections, dyspnea, tachypnea, tachycardia, cyanosis, tissue hypoxia, and murmurs, all of which vary in[medical-dictionary.thefreedictionary.com]
  • (Click picture to show/hide bloodflows) Pathophysiology Patients with partial anomalous pulmonary venous return are usually asymptomatic.[vhlab.umn.edu]
  • The pathophysiology is almost total RVOT resulting in increased right-to-left shunt through the VSD.[ahcmedia.com]
  • DORV is not a single malformation from a pathophysiological point of view.[sonoworld.com]

Prevention

  • Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age.[nemours.org]
  • While they can't be prevented, many treatments are available for the defects and related health problems.[kidshealth.org]
  • As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image.[cdc.gov]
  • In most cases, transposition of the great arteries can't be prevented.[mayoclinic.org]
  • Treatment Infusion of prostaglandin ( PGE1 ) to prevent closure of the ductus arteriosus in ductal-dependent defects (see “General treatment concepts” in acyanotic congenital heart defects ) In cases of heart failure Antibiotic prophylaxis for bacterial[amboss.com]

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