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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]

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]

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]

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]

Stenosis, or other defects, of valves and/or vessels may also be present. When no other heart defects are present it is called 'simple' TGV; when other defects are present it is called 'complex' TGV. [en.wikipedia.org]

Entire Body System

  • Atrial Septal Defect

    septal defect; one had perimembranous ventricular septal defect with secundum atrial septal defect; and one had coarctation of the aorta. [adc.bmj.com]

    Postoperative complications include conduction defects, such as transient right bundle branch block. ATRIAL SEPTAL DEFECT Atrial septal defects may occur as sinus venosus, secundum or primum type. [aafp.org]

    The left side of the heart (left atrium and left ventricle) are filled only by mixed blood shunting across a hole in the atrial wall (atrial septal defect – ASD). [cincinnatichildrens.org]

    Atrial septal defects An atrial septal defect (ASD) is where there's a hole between the two collecting chambers of the heart (the left and right atria). [nhs.uk]

    septal defect Sinus venosus atrial septal defect Sinus venosus defect with overriding inferior vena cava Sinus venosus defect with overriding superior vena cava The code Q26.8 is grouped in the following Diagnostic Related Group(s) (MS-DRG v36.0) Cardiac [healthprovidersdata.com]

  • Coarctation of the Aorta

    COARCTATION OF THE AORTA Narrowing of the aorta may occur anywhere along its length, but 98 percent of cases occur just below the origin of the left subclavian artery. 9 The classic clinical sign of coarctation of the aorta is a higher blood pressure [aafp.org]

    Another notable association was that between coarctation of the aorta and vein of Galen malformations. [adc.bmj.com]

    Kinds of congenital cardiac anomalies include atrial septal defect, coarctation of the aorta, tetralogy of Fallot, transposition of the great vessels, tricuspid atresia, and ventricular septal defect. Also called congenital heart disease. [medical-dictionary.thefreedictionary.com]

    Surgery 42:101–120 PubMed Google Scholar Craaford C, Nylin G (1945) Congenital coarctation of the aorta and its surgical treatment. [link.springer.com]

    Coarctation of the aorta Coarctation of the aorta (CoA) is where the main artery (the aorta) has a narrowing, which means that less blood can flow through it. [nhs.uk]

  • Pain

    These malformations swell after treatment with alcohol, and the swelling and pain may last for three to five days. During this time, we give patients medication for any pain or swelling they may have. [hopkinsmedicine.org]

    To learn more about congenital heart disease, visit SickKids on the following topics: Introduction to the Heart Heart tests Heart medication Heart Catheterization Heart surgery Pacemaker surgery After heart surgery: caring for your child Pain after an [heartandstroke.ca]

    This weakening will be felt by patients as signs of heart failure: Shortness of breath Fatigue Chest pain Decreased exercise ability Treatments with medicines are almost always helpful and can restore some of the heart function and improve symptoms. [nationwidechildrens.org]

    The remaining patients were submitted to the exams for other reasons, such as abdominal pain, hematuria and tumor staging. [scielo.br]

  • Pulmonary Valve Stenosis

    Pulmonary valve stenosis Pulmonary valve stenosis is a defect where the pulmonary valve, which controls the flow of blood out of the right heart pumping chamber (the right ventricle) to the lungs, is narrower than normal. [nhs.uk]

    Pulmonary valve stenosis is the most common valve defect. [my.clevelandclinic.org]

    This type of abnormality occurs with congenital disease, such as pulmonary valve stenosis, or advanced acquired disease, such as mitral stenosis with moderately severe pulmonary hypertension. [rjmatthewsmd.com]

    More information about Patent Ductus Arteriosis (PDA) Pulmonary Valve Stenosis A thickened or fused heart valve that does not fully open. The pulmonary valve allows blood to flow out of the heart, into the pulmonary artery and then to the lungs. [heart.org]

    […] and tricuspid valves Q22.0 Pulmonary valve atresia Q22.1 Congenital pulmonary valve stenosis Q22.2 Congenital pulmonary valve insufficiency Congenital pulmonary valve regurgitation Q22.3 Other congenital malformations of pulmonary valve Congenital malformation [apps.who.int]

  • Trisomy 21

    Heart defects are found in more than 90% of fetuses with trisomy 18 or 13, 50% of trisomy 21, and 40% of those with Turner syndrome, deletions or partial trisomies involving a variety of chromosomes. [sonoworld.com]

    21 or 22 G 758.1 Patau's syndrome Trisomy: 13 D1 758.2 Edward's syndrome Trisomy: 18 E3 758.3 Autosomal deletion syndromes 758.31 Cri-du-chat syndrome Deletion 5p 758.32 Velo-cardio-facial syndrome Deletion 22q11.2 758.33 Other microdeletions Miller-Dieker [theodora.com]

Respiratoric

  • Dyspnea

    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]

    Retrograde coronary artery perfusion Clinical findings May be asymptomatic at birth if the open PDA provides adequate systemic perfusion Aggravation of symptoms following closure of the PDA Postnatal cyanosis Dyspnea and metabolic acidosis ↓ BP, ↓ peripheral [amboss.com]

    It may compress the trachea, leading to stridor, cough and dyspnea. This compression decreases with age and these patients will outgrow it. [radiologyassistant.nl]

    […] infundibular stenosis increases, progressive cyanosis develops (due to less pulmonary blood flow), and infants and children may develop cyanotic or hypoxic spells, which consist of sudden onset of increased cyanosis, excessive crying, hypoxemia, acidosis, dyspnea [hawaii.edu]

    In mild cases, patients with AS present with exercise intolerance and in more severe cases patients may have poor feeding and dyspnea. On physical exam, patients present with a systolic thrill over the RUSB, suprasternal notch, or carotid arteries. [ahcmedia.com]

  • Respiratory Distress

    Symptoms and Signs Neonates with obstructed pulmonary venous return present with respiratory distress, pulmonary edema, and marked cyanosis. [merckmanuals.com]

    Most infants with double aortic arch show signs of severe respiratory distress within the first 6 months of life. A high index of suspicion is required to make the diagnosis. Dysphagia or feeding difficulties may accompany respiratory distress. [ots1.narod.ru]

    Infants with CHF typically present with sweating, feeding difficulties, failure to thrive, tachypnea, tachycardia, respiratory distress, wheezing, peripheral edema, hepatomegaly, gallop rhythm, cyanosis or shock. [ahcmedia.com]

    He is alert and active in mild respiratory distress, with visible cyanosis. HEENT exam is negative. His heart rhythm is tachycardic. He has a mild right precordial heave with a grade 3/6 ejection murmur at ULSB and a diminished 2nd heart sound. [hawaii.edu]

Gastrointestinal

  • Regurgitation

    Commonly the tricuspid regurgitation is severe with low velocity on continuous wave Doppler. [tmj.ro]

    […] process during cardiac septation → right AV valve does not separate normally from the ventricular myocardium ; Displaced valve reduces the ventricular volume → regurgitation into the right atrium ( tricuspid regurgitation ) → atrial dilatation ; poorly [amboss.com]

    The characteristic physical findings at this stage are those of aortic regurgitation and often tricuspid regurgitation, an otherwise unusual combination. [ots1.narod.ru]

    Approach Browse Doctors lipid disorder Our Approach Browse Doctors long QT syndrome Browse Doctors lymphatic disease Browse Doctors mediastinal disease Browse Doctors mesenteric ischemia Our Approach Browse Doctors metabolic syndrome Browse Doctors mitral regurgitation [nyulangone.org]

    Most of the hemodynamic problems associated with this abnormality are caused by the ventricular septal defect, although mitral regurgitation or left-ventricle-to-right-atrium regurgitation, or both, may add to the pulmonary overload. [aafp.org]

Cardiovascular

  • Heart Disease

    Approach Browse Doctors heart throbbing Browse Doctors heart tumor Browse Doctors heart valve disease Our Approach To: Adult Congenital Heart Disease Aortic Valve Disease in Adults Mitral Valve Disease in Adults Browse Doctors high blood pressure disorder [nyulangone.org]

    The influence of congenital heart disease on psychological conditions in adolescents and adults after corrective surgery. Congenital Heart Disease 1(6):282–288. Perloff, J. K., and C. A. Warnes. 2001. [nap.edu]

    NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]

    What is congenital heart disease? Congenital heart disease is condition you are born with. The word congenital means “present at birth.” [heartandstroke.ca]

    Hypoplastic Left Heart Syndrome Hypoplastic left heart syndrome (HLHS) is associated with the highest mortality of all congenital heart diseases. [ahcmedia.com]

  • Tachycardia

    Fetuses with supraventricular tachycardia that occasionally convert to sinus rhythm can tolerate well the condition. Sustained tachycardias of greater than 200 bpm frequently result in fetal hydrops. [sonoworld.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]

    Start or Resume CME Test (Opens in a new Tab/Window ) Arrhythmias Supraventricular tachycardia (SVT) is the most common tachyarrhythmia in neonates. [ahcmedia.com]

    Approach Browse Doctors ventricular arrhythmia Our Approach Browse Doctors ventricular fibrillation Browse Doctors ventricular septal defect Our Approach To: Adult Congenital Heart Disease Congenital Heart Defects in Children Browse Doctors ventricular tachycardia [nyulangone.org]

    Long-term management Surgery : performed within the first year of life, if possible Follow-up care : to prevent long-term complications such as heart failure, arrhythmias (e.g., ventricular tachycardia ), and neurodevelopmental impairment References: [amboss.com]

Skin

  • Ulcer

    At their surface, the vessels may be vulnerable to injury and bleed, or may even break down and ulcerate. [vascularcures.org]

Neurologic

  • 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]

Treatment

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]

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]

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]

Manifestation code identifies conditions for which it is important to record both the etiology and the symptom of the disease. [books.google.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]

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]

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]

Health science Providing basic knowledge of medical terminology, anatomy and physiology, and pathophysiology of diseases and conditions. [icd-10online.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]

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]

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]

Prevention Congenital cardiovascular defects cannot be prevented. [healthofchildren.com]

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