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Congenital Tricuspid Malformation

Congenital Anomaly of the Tricuspid Valve 


Presentation

  • 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, are[icd10data.com]
  • Stenosis - when the valve doesn't open enough and blocks blood flow Valve problems can be present at birth or caused by infections, heart attacks, or heart disease or damage.[icdlist.com]
  • The first reported instance of tricuspid atresia and Ebstein's anomaly in siblings is presented.[link.springer.com]
  • A right ventricular myopathy with varying degrees of failure of tricuspid valve delamination is present in all cases. Consequently, tricuspid valve morphology is highly variable and complex.[mayoclinic.pure.elsevier.com]
  • Tricuspid atresia: complete absence of the tricuspid valve; an ASD must be present to maintain blood flow.[patient.info]
Atrial Septal Defect
  • 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]
  • 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]
  • It’s usually associated with an atrial septal defect. Other heart conditions associated with Ebstein’s anomaly Three common conditions are: Atrial septal defect.[chd-uk.co.uk]
  • In HLHS, blood returning from the lungs must flow through an opening in the wall between the atria (atrial septal defect).[heart.org]
  • Some children with Ebstein’s anomaly of the tricuspid valve also have an atrial septal defect (ASD), a hole in the wall between the two upper chambers of the heart, or other heart defects.[chop.edu]
Surgical Procedure
  • Our research was a review of the surgical procedures conducted (and of their outcomes), as a number of different strategies can be performed, many with quite high rates of failure.[surgsoc.org.au]
  • Some surgical procedures have been performed with mixed results. Arrhythmias are treated with medications or pacemaker placement. History and etymology It is named after Wilhelm Ebstein, a German physician (1836-1912) 4.[radiopaedia.org]
  • Often it's necessary to do a surgical procedure, called a shunt, to increase blood flow to the lungs. This improves the cyanosis. Some children with tricuspid atresia have too much blood flowing to the lungs.[heart.org]
  • In the most severe cases (for example, hypoplastic heart), multiple palliative surgical procedures or a heart transplant may be required. The type and extent of the correction required will depend on the underlying anomaly.[patient.info]
  • procedure to separate the pulmonary and systemic circulation Without treatment, about 75% of patients with tricuspid atresia die in early childhood![amboss.com]
Fatigue
  • Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms.[icdlist.com]
  • Taking medications at the right dose and the right time can help improve symptoms such as racing heartbeats, fatigue and shortness of breath. Stay active. Be as physically active as your doctor allows.[chd-uk.co.uk]
  • Symptoms of Ebstein's anomaly include some or all of the following: Blue or purple tint to lips, skin and nails (cyanosis) Heart murmur and clicks: extra heart sounds heard when a doctor listens with a stethoscope Racing heart Shortness of breath Fatigue[chop.edu]
  • […] flows from the right to the left heart via a shunt deoxygenated blood enters the systemic circulation cyanosis General clinical features “Blue b abies” : pale gray or blue skin color caused by cy anosis Nail clubbing Exertional dyspnea, tachypnea, and fatigue[amboss.com]
  • Chronic severe TR may culminate in fatigue and poor exercise tolerance due to reduced cardiac output. Symptoms of associated diseases may also be present. Signs The severity and chronicity of the disease determine the clinical findings present.[clinicaladvisor.com]
Intermittent Claudication
  • claudication intestine intracranial joint joint prosthesis kidney knee laceration lacrimal lower limb lung malignant molar pregnancy multiple muscle Neoplasm nerve newborn nondisplaced nontraumatic obstetrical trauma obstruction organ Osteochondrosis[books.google.com]
Sepsis
  • Neoplasm nerve newborn nondisplaced nontraumatic obstetrical trauma obstruction organ Osteochondrosis pelvic percent third degree posterior postprocedural prosthesis psychogenic F45.8 pulmonary Puncture region renal respiratory second degree secondary sepsis[books.google.com]
Tachypnea
  • […] shunt : blood flows from the right to the left heart via a shunt deoxygenated blood enters the systemic circulation cyanosis General clinical features “Blue b abies” : pale gray or blue skin color caused by cy anosis Nail clubbing Exertional dyspnea, tachypnea[amboss.com]
Regurgitation
  • Sometimes it causes regurgitation. Stenosis - when the valve doesn't open enough and blocks blood flow Valve problems can be present at birth or caused by infections, heart attacks, or heart disease or damage.[icdlist.com]
  • Tetralogy of Fallot 230 Managing the Right Ventricular Outflow 233 Early and Late Effects 241 Pulmonary Regurgitation in Relation to Pulmonary Artery Compliance and Other Variables 246 Current Approaches to Risk Stratification 251 Timing and Outcome of[books.google.com]
  • Q22 Congenital malformations of pulmonary and tricuspid valves Q22.0 Pulmonary valve atresia Q22.1 Congenital pulmonary valve stenosis Q22.2 Congenital pulmonary valve insufficiency Inclusion term(s): Congenital pulmonary valve regurgitation Q22.3 Other[icd10coded.com]
  • […] not for use on maternal records Type 2 Excludes inborn errors of metabolism ( E70-E88 ) Congenital malformations, deformations and chromosomal abnormalities Approximate Synonyms Congenital insufficiency of tricuspid valve Congenital tricuspid valve regurgitation[icd10data.com]
  • There can be concurrent tricuspid regurgitation with or without stenosis.[radiopaedia.org]
Heart Disease
  • […] defect (Medical Encyclopedia) [ Read More ] Heart Valve Diseases Also called: Valvular heart disease Your heart has four valves.[icdlist.com]
  • Article First Online: 30 April 2014 Abstract The occurrence of familial heart disease in association with tricuspid atresia is rare. The first reported instance of tricuspid atresia and Ebstein's anomaly in siblings is presented.[link.springer.com]
  • Risk factors The relative risk of recurrence of congenital heart disease increases if a first-degree relative has a congenital heart disease.[patient.info]
  • WPW is seen in other types of congenital heart disease but of all of the WPW cases with congenital heart disease, one-third of them have Ebstein’s malformation. WPW is usually of type B (RV free wall bypass tract).[pedcard.rush.edu]
Cyanosis
  • Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms.[icdlist.com]
  • Mild obstruction left-to-right shunt via VSD more pronounced no cyanosis Severe obstruction right-to-left shunt via VSD more pronounced severe cyanosis Clinical findings Mild cyanosis Tet spells Intermittent hypercyanotic, hypoxic episodes with a peak[amboss.com]
  • As the ductus closes the cyanosis worsens. If the cyanosis is severe enough, the baby will develop symptoms of poor oxygenation such as fast breathing or poor feeding. Occasionally, there is an increased amount of blood flow to the lungs.[cincinnatichildrens.org]
  • This causes a bluish discoloration of the lips and skin (cyanosis).[chd-uk.co.uk]
Heart Murmur
  • The main sign of heart valve disease is an unusual heartbeat sound called a heart murmur. Your doctor can hear a heart murmur with a stethoscope. But many people have heart murmurs without having a problem.[icdlist.com]
  • Others are diagnosed at various ages subsequent to the detection of a heart murmur as part of routine screening or during clinical examination for intercurrent illness. Heart murmurs.[patient.info]
  • murmur sweating, especially during feedings a weaker pulse If you notice any of these signs in your baby or child, call your doctor right away.[kidshealth.org]
  • Recognize the innocent heart murmurs that occur during infancy. Identify the signs and symptoms of congestive heart failure in infants. Describe the pathophysiology of the more common cyanotic heart defects.[pedsinreview.aappublications.org]
  • Further symptoms include failure to thrive, characteristic heart murmurs, and symptoms of heart failure. The diagnosis is confirmed through visualization of the defect on echocardiography.[amboss.com]
Hypertension
  • […] contusion cornea device Diabetes diagnosis disease classified elsewhere disease or syndrome Disorder displaced duct ectopic or molar embolism external cause eyelid female finger foot foreign body Fracture gallbladder genital gland heart hemorrhage hepatic Hypertension[books.google.com]
  • […] various causes of pulmonary hypertension.[clinicaladvisor.com]
  • A left-to-right shunt does not cause cyanosis but the high volume pumped by the right side may result in pulmonary hypertension and, if this builds up and exceeds systemic pressure, the shunt may reverse from right to left.[patient.info]
  • […] over lower left sternal border Treatment Initially : infusion of prostaglandin ( PGE1 ) Optional temporary measures In cases with concurrent pulmonary valve atresia or severe stenosis: systemic-to-pulmonary shunt to improve perfusion Risk of pulmonary hypertension[amboss.com]
  • The presence of pulmonary hypertension in neonates increases this atrial shunting and can lead to profound cyanosis in newborn infants.[emedicine.medscape.com]
Mitral Valve Prolapse
  • If they don't, you could have Regurgitation - when blood leaks back through the valve in the wrong direction Mitral valve prolapse - when one of the valves, the mitral valve, has "floppy" flaps and doesn't close tightly.[icdlist.com]
  • (valve) prolapse I342Nonrheumatic mitral (valve) stenosis I348Other nonrheumatic mitral valve disorders I349Nonrheumatic mitral valve disorder, unspecified I350Nonrheumatic aortic (valve) stenosis I351Nonrheumatic aortic (valve) insufficiency I352Nonrheumatic[cms.gov]
Ulcer
  • […] pulmonary Puncture region renal respiratory second degree secondary sepsis sinus skin specified NEC specified site NEC specified type NEC spinal status epilepticus subacute subcategory superficial syphilitic Table of drugs thoracic traumatic tuberculous ulcer[books.google.com]
Fracture
  • NEC condition congenital connective tissue Continued contusion cornea device Diabetes diagnosis disease classified elsewhere disease or syndrome Disorder displaced duct ectopic or molar embolism external cause eyelid female finger foot foreign body Fracture[books.google.com]
Contusion
  • Elsevier ‏ معاينة محدودة - 2018 عبارات ومصطلحات مألوفة abdomen abnormal Abscess abuse acquired acute ankle artery benign Bite bone brain breast cardiac cell cerebral cervical cervix chronic complication NEC condition congenital connective tissue Continued contusion[books.google.com]
Lacrimation
  • […] eyelid female finger foot foreign body Fracture gallbladder genital gland heart hemorrhage hepatic Hypertension idiopathic implant infarction infection Injury intermittent claudication intestine intracranial joint joint prosthesis kidney knee laceration lacrimal[books.google.com]
Suggestibility
  • The presence of these two distinct pathologic variants of tricuspid valve malformations in siblings suggests that these malformations result from a common abnormality occurring during the development of the inlet portion of the right ventricle.[link.springer.com]
  • Any patient with a murmur suggestive of TI or other clinical features suggestive of right heart failure or pulmonary hypertension should undergo an echocardiogram.[clinicaladvisor.com]
  • Characteristic ECG findings include: A large P wave, some studies suggest that a large P wave correlates with decreased oxygen saturation, severe symptoms and increased risk of death with or without pre-excitation.[pedcard.rush.edu]
  • Before any surgery is planned, the doctors may suggest medicines to help the heart work better if it is already coping poorly. Surgery if it is necessary is aimed at making the tricuspid valve less leaky and normalising the flows in the heart.[gosh.nhs.uk]
  • Evidence suggests that the anterior leaflet forms earlier in development than the septal and posterior leaflets.[emedicine.medscape.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]
  • Treatment Reimplantation of coronary arteries Aortocoronary bypass[amboss.com]
  • If signs and symptoms become troublesome, or if the heart is enlarging or becoming weaker, treatment for Ebstein’s anomaly may be necessary. Treatment options include medications and surgery.[chd-uk.co.uk]
  • In all cases of moderate or severe TR, treatment of any underlying cause, including medical treatment of pulmonary hypertension as per guidelines, is indicated.[clinicaladvisor.com]
  • Much research is being conducted both into the cause and the treatment of congenital heart defects. Many types of congenital heart defects are repairable.[americanpregnancy.org]

Prognosis

  • Treatment and prognosis As the anomaly is of variable severity, so is the prognosis. Severity is related to the amount of RVOT dysfunction and tricuspid regurgitation. Sudden death from arrhythmia may occur.[radiopaedia.org]
  • As a result, prognosis is improved from earlier diagnosis.[escardio.org]
  • Prognosis Severe TR of any cause generally has a poor prognosis long term due to RV dysfunction and/or systemic venous congestion.[clinicaladvisor.com]
  • Prognosis The prognosis for children with congenital heart disease has improved dramatically over the last few decades.[patient.info]
  • 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]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • When significant TI accompanies left-sided heart disease or pulmonary hypertension of other etiology, the natural history is determined by the primary disease.[clinicaladvisor.com]
  • Etiologic Syndrome Frequency of Cardiac Anomalies † Distinguishing Features All (%) Distinctive or Most Common Autosomal Dominant Adams-Oliver syndrome 20 Left-sided obstruction (eg, COA, parachute MVP), TOF Scalp cutis aplasia, terminal transverse limb[pedsinreview.aappublications.org]
  • 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]
  • Etiology The exact embryologic cause of Ebstein anomaly is unknown. The tricuspid valve leaflets form by a process of delamination of the inner layers of the inlet portion of the right ventricle.[emedicine.medscape.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]
  • Epidemiology The reported total birth prevalence of congenital heart disease in Europe is 8.2 per thousand live births which is higher than reported birth prevalence in the USA (6.9 per thousand live births).[patient.info]
  • Epidemiology Pathological TR is more prevalent in women and in those with a past history of myocardial infarction and heart failure.[clinicaladvisor.com]
  • Epidemiology This rare heart condition accounts for only 1% of congenital heart defects but its impact is significant, especially to those directly affected. About 10% of cases are associated with chronic maternal lithium use.[chd-uk.co.uk]
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]
  • Describe the pathophysiology of the more common cyanotic heart defects. Introduction Significant congenital heart disease (CHD) may be diagnosed at virtually any age.[pedsinreview.aappublications.org]
  • Classification Classification into five groups based on pathophysiology has been suggested as it would be helpful in planning management [ 1 ].[patient.info]
  • Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: pathophysiologic and clinical implications. J Am Coll Cardiol. 1992 Apr. 19(5):1049-53. [Medline]. Patel V, Nanda NC, Rajdev S, et al.[emedicine.medscape.com]
  • Pathophysiology of congenital heart disease in the adult: part III: Complex congenital heart disease.[ghrnet.org]

Prevention

  • Can congenital heart defects be prevented? Most congenital heart defects cannot be prevented. A woman can do some things to reduce the risk of congenital heart defects though.[americanpregnancy.org]
  • Endocarditis Prevention Antibiotics to prevent endocarditis are needed before certain dental procedures. See the section on Endocarditis for more information.[heart.org]
  • Prevention Although many forms of congenital heart disease are not currently preventable, the avoidance of known risk factors such as drugs and alcohol during pregnancy will help to reduce the risk.[patient.info]
  • While they can't be prevented, many treatments are available for the defects and related health problems.[kidshealth.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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