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Congenital Pulmonary Veins Atresia or Stenosis


Presentation

  • Cyanosis, when present for 6 mo, may result in clubbing.[merckmanuals.com]
  • Tricuspid atresia: complete absence of the tricuspid valve; an ASD must be present to maintain blood flow.[patient.info]
  • Congenital pulmonary vein (PV) stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents[ncbi.nlm.nih.gov]
  • Presentation Children born with pulmonary atresia and ventricular septal defect (PA-VSD) may have unpredictable presentations owing to the variability of the lesion. The age at presentation may vary depending on the amount of pulmonary blood flow.[emedicine.medscape.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]
Renal Artery Stenosis
  • .- ) Q27.0 Congenital absence and hypoplasia of umbilical artery Single umbilical artery Q27.1 Congenital renal artery stenosis Q27.2 Other congenital malformations of renal artery Congenital malformation of renal artery NOS Multiple renal arteries Q27.3[apps.who.int]
  • artery stenosis Q27.2 Other congenital malformations of renal artery Q27.3 Arteriovenous malformation (peripheral) Q27.30 Arteriovenous malformation, site unspecified Q27.31 Arteriovenous malformation of vessel of upper limb Q27.32 Arteriovenous malformation[icd10data.com]
  • artery stenosis Q272Other congenital malformations of renal artery Q2730Arteriovenous malformation, site unspecified Q2731Arteriovenous malformation of vessel of upper limb Q2732Arteriovenous malformation of vessel of lower limb Q2733Arteriovenous malformation[cms.gov]
  • Renal artery stenosis —A disorder in which the arteries that supply blood to the kidneys are narrowed or constricted. Septal —Relating to the septum, the thin muscle wall dividing the right and left sides of the heart.[healthofchildren.com]
Dyspnea
  • […] stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea[ncbi.nlm.nih.gov]
  • 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]
  • Infants with increased pulmonary blood flow usually show signs of heart failure (eg, tachypnea, dyspnea with feeding, poor weight gain, diaphoresis) by age 4 to 6 wk.[merckmanuals.com]
  • Other symptoms can include: shortness of breath (dyspnea); chest pain (angina); fainting (syncope), or cyanosis (blueness). If significant pulmonary stenosis is present then the child may feel tired when playing or doing any physical activity.[chd-uk.co.uk]
  • […] or bearing down, indicative of dynamic and increasing right ventricular outflow tract obstruction, which results in increased right-to-left shunting through intracardiac shunt (VSD, ASD) Exercise Intolerance History of exercise intolerance, including dyspnea[learn.pediatrics.ubc.ca]
Tachypnea
  • […] or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea, tachypnea[ncbi.nlm.nih.gov]
  • Infants with increased pulmonary blood flow usually show signs of heart failure (eg, tachypnea, dyspnea with feeding, poor weight gain, diaphoresis) by age 4 to 6 wk.[merckmanuals.com]
  • […] 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]
  • 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]
  • Clinical description PL may present at birth as a stillbirth or with severe respiratory distress, tachypnea, and cyanosis, with a very high mortality rate at or within a few hours of birth [ 2 ].[ojrd.biomedcentral.com]
Hip Dislocation
  • Congenital hip dislocation: A defect in which the head of the femur does not articulate with the acetabulum of the pelvis because of an abnormal shallowness of the acetabulum.[fbdr.org]

Workup

  • Yet the diagnostic workup of pulmonary venous stenosis is elusive, which is why the different diagnostic procedures to detect pulmonary venous stenosis are discussed here.[err.ersjournals.com]

Treatment

  • Treatment Reimplantation of coronary arteries Aortocoronary bypass[amboss.com]
  • After treatment A good result from a balloon valvoplasty or surgery usually means that further treatment is not required.[chd-uk.co.uk]
  • In cases where the right ventricle is normal in size, treatment of critical stenosis or atresia is performed soon after birth. Treatment options include open-heart surgery and interventional cardiac catheterization.[luriechildrens.org]
  • The prognosis in certain subsets of patients remains grim in spite of advances in treatment. Larger studies using newer modalities of treatment would help to improve prognosis in the difficult to treat subsets.[medresearch.in]

Prognosis

  • PMID: 17911076 Prognosis Arunakumar P, Ayyappan A, Sasikumar D, Krishnamoorthy KM Echocardiography 2018 May;35(5):733-734. Epub 2018 Apr 12 doi: 10.1111/echo.13880.[ncbi.nlm.nih.gov]
  • Prognosis: Cardiac failure is never seen in fetal life as well as postnatally.[sonoworld.com]
  • The prognosis in certain subsets of patients remains grim in spite of advances in treatment. Larger studies using newer modalities of treatment would help to improve prognosis in the difficult to treat subsets.[medresearch.in]
  • Prognosis The prognosis for children with congenital heart disease has improved dramatically over the last few decades.[patient.info]
  • Conclusions: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor.[link.springer.com]

Etiology

  • Etiology Pediatr Cardiol 2016 Jan;37(1):125-30. Epub 2015 Aug 14 doi: 10.1007/s00246-015-1249-7. PMID: 26271471 Bonello B, Trivedi KR, Fraisse A Catheter Cardiovasc Interv 2015 Oct;86(4):E190-3. Epub 2015 May 27 doi: 10.1002/ccd.25920.[ncbi.nlm.nih.gov]
  • In most cases the etiology is unknown , probably multifactorial.[atlases.muni.cz]
  • 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]
  • Pulmonary vein stenosis: Etiology, diagnosis and management.[wjgnet.com]
  • Etiology The etiology of PL is not known.[ojrd.biomedcentral.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]
  • Some disease registries, like the cancer registry, closely resemble public health surveillance systems and have epidemiologic value. Renal agenesis/hypoplasia: Absence or incomplete development of the kidney.[fbdr.org]
  • Epidemiology Frequency The best estimates of the relative frequency of pulmonary atresia with ventricular septal defect are 2.5-3.4% of all congenital cardiac malformations with a prevalence of 0.07 per 1000 live births. [2, 3] Pulmonary atresia with[emedicine.medscape.com]
  • Epidemiology The incidence of PL is not clearly defined. Any attempt to provide precise statistics regarding the incidence of PL would be misleading considering that to date only a few isolated cases or small series have been reported.[ojrd.biomedcentral.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]
  • Pathophysiology To understand the pathophysiology of haemoptysis in cases of pulmonary venous stenosis, it is important to know that both the pulmonary and the bronchial circulation drain via the pulmonary veins into the left atrium.[err.ersjournals.com]
  • Each of these types can be subdivided based on the degree of obstruction to pulmonary blood flow: A—pulmonary arteries are atretic B—pulmonary stenosis or hypoplasia C—no obstruction to pulmonary flow (Click picture to show/hide bloodflows) Pathophysiology[vhlab.umn.edu]
  • Classification Classification into five groups based on pathophysiology has been suggested as it would be helpful in planning management [ 1 ].[patient.info]
  • Summary of Cyanotic Congenital Heart Defects Here, we present a brief summary of the pathophysiology of common congenital heart defects.[learn.pediatrics.ubc.ca]

Prevention

  • Endocarditis Prevention Antibiotics to prevent endocarditis are needed before certain dental procedures. See the section on Endocarditis for more information.[heart.org]
  • One-way valves are present within the heart to prevent blood from flowing back into it in the wrong direction.[savetheheartbeat.org]
  • Prevention & Risk Assessment Prevention & Risk Assessment What causes pulmonary stenosis? Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth.[childrensnational.org]
  • References Mai CT, Riehle-Colarusso T, O’Halloran A, Cragan JD, Olney RS, Lin A, Feldkamp M, Botto LD, Rickard R, Anderka M, Ethen M, Stanton C, Ehrhardt J, Canfield M; National Birth Defects Prevention Network.[cdc.gov]
  • 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]

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