Total anomalous pulmonary venous return: Difference between revisions

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==Background==
==Background==
[[File:tapvr.jpg|thumb|]]
[[File:tapvr.jpg|thumb|]]
* A cyanotic congenital heart defect
*A cyanotic congenital heart defect
* All four pulmonary veins drain into the systemic venous circulation rather than into the left atrium.  
*All four pulmonary veins drain into the systemic venous circulation rather than into the left atrium.  
* Four anatomic forms:
*Four anatomic forms:
**Supracardiac (46%): pulmonary veins connect to the cardinal venous system (left innominate vein, superior vena cava, azygous vein)
**Supracardiac (46%): pulmonary veins connect to the cardinal venous system (left innominate vein, superior vena cava, azygous vein)
**Cardiac (20%): pulmonary veins connect to the posterior aspect of the coronary sinus or directly to the right atrium
**Cardiac (20%): pulmonary veins connect to the posterior aspect of the coronary sinus or directly to the right atrium
**Infracardiac (23%): Pulmonary veins drain into the portal vein, hepatic vein, or IVC
**Infracardiac (23%): Pulmonary veins drain into the portal vein, hepatic vein, or IVC
** Mixed (11%): Combination of connections that enter at 2 or more levels.  
**Mixed (11%): Combination of connections that enter at 2 or more levels.  
* Firth most common cause of cyanotic [[congenital heart disease]] <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.</ref>
*Firth most common cause of cyanotic [[congenital heart disease]] <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.</ref>


===Physiology===
===Physiology===
*All oxygenated pulmonary venous blood mixes with deoxygenated blood from the systemic venous system
*All oxygenated pulmonary venous blood mixes with deoxygenated blood from the systemic venous system
*Mixed/partially oxygenated blood then shunted right to left (through patent foramen ovale, [[PDA]] or an [[Atrial septal defect |ASD]]), resulting in cyanosis
*Mixed/partially oxygenated blood then shunted right to left (through patent foramen ovale, [[PDA]] or an [[Atrial septal defect |ASD]]), resulting in cyanosis
*The right atrium and ventricle become dilated because it receives blood from both pulmonary and systemic venous systems.  
*The right atrium and ventricle become dilated because the right side of the heart receives blood from both pulmonary and systemic venous systems.  
*In obstructed forms of TAPVR, the pulmonary venous system is compressed by surrounding structures
*In obstructed forms of TAPVR, the pulmonary venous system is compressed by surrounding structures
**May lead to RV hypertrophy and pulmonary vascular changes that may result in RV failure
**May lead to RV hypertrophy and pulmonary vascular changes that may result in RV failure


==Clinical Features==
==Clinical Features==
* Varies with the degree of obstruction and the degree of left-to-right shunting.
*Varies with the degree of obstruction and the degree of left-to-right shunting.
*Respiratory distress and tachypnea
*[[Shortness of breath (peds)|Respiratory distress]] and tachypnea
*Hypotension
*[[Pediatric shock|Hypotension]]
*Poor feeding, failure to thrive. 
*Poor feeding, [[failure to thrive (peds)|failure to thrive]]
*Fixed S2 split due to RV overload
*Fixed S2 split due to RV overload
*Systolic ejection murmur due to increased stroke volume across the pulmonary valve
*Systolic ejection [[murmur]] due to increased stroke volume across the pulmonary valve
*Hepatomegaly due to right-sided failure
*[[Hepatomegaly]] due to right-sided failure
*Variable degrees of cyanosis
*Variable degrees of cyanosis


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==Evaluation==
==Evaluation==
[[File:Tapvrsnowman.jpeg|thumb|Snowman sign]]
[[File:Tapvrsnowman.jpeg|thumb|Snowman sign]]
*Echocardiography
*[[Echocardiography]]
*Angiography: gold standard but generally no longer required given other less invasive diagnostic modalities
*Angiography: gold standard but generally no longer required given other less invasive diagnostic modalities
* CT or MRA
*CT or MRA
*[[Chest x-ray]]
*[[Chest x-ray]]
**Nonspecific findings, cannot be used to make diagnosis
**Nonspecific findings, cannot be used to make diagnosis
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==Management==
==Management==
*Stabilize cardiopulmonary function prior to surgery
*Stabilize cardiopulmonary function prior to surgery
*Supplemental oxygen
*Supplemental [[oxygen]]
*Mechanical ventilation
*Mechanical ventilation
*Inotropic agents (eg, [[dopamine]] and [[dobutamine]]) improve myocardial contractility
*Inotropic agents (eg, [[dopamine]] and [[dobutamine]]) improve myocardial contractility

Latest revision as of 18:30, 26 September 2019

Background

Tapvr.jpg
  • A cyanotic congenital heart defect
  • All four pulmonary veins drain into the systemic venous circulation rather than into the left atrium.
  • Four anatomic forms:
    • Supracardiac (46%): pulmonary veins connect to the cardinal venous system (left innominate vein, superior vena cava, azygous vein)
    • Cardiac (20%): pulmonary veins connect to the posterior aspect of the coronary sinus or directly to the right atrium
    • Infracardiac (23%): Pulmonary veins drain into the portal vein, hepatic vein, or IVC
    • Mixed (11%): Combination of connections that enter at 2 or more levels.
  • Firth most common cause of cyanotic congenital heart disease [1]

Physiology

  • All oxygenated pulmonary venous blood mixes with deoxygenated blood from the systemic venous system
  • Mixed/partially oxygenated blood then shunted right to left (through patent foramen ovale, PDA or an ASD), resulting in cyanosis
  • The right atrium and ventricle become dilated because the right side of the heart receives blood from both pulmonary and systemic venous systems.
  • In obstructed forms of TAPVR, the pulmonary venous system is compressed by surrounding structures
    • May lead to RV hypertrophy and pulmonary vascular changes that may result in RV failure

Clinical Features

  • Varies with the degree of obstruction and the degree of left-to-right shunting.
  • Respiratory distress and tachypnea
  • Hypotension
  • Poor feeding, failure to thrive
  • Fixed S2 split due to RV overload
  • Systolic ejection murmur due to increased stroke volume across the pulmonary valve
  • Hepatomegaly due to right-sided failure
  • Variable degrees of cyanosis

Differential Diagnosis

Congenital Heart Disease Types

Evaluation

Snowman sign
  • Echocardiography
  • Angiography: gold standard but generally no longer required given other less invasive diagnostic modalities
  • CT or MRA
  • Chest x-ray
    • Nonspecific findings, cannot be used to make diagnosis
    • Prominence of pulmonary arteries
    • Enlarged cardiac silhouette
    • Classic snowman sign (for supracardiac TAPVC)
  • ECG
    • May be normal
    • May have evidence of right ventricular hypertrophy or right atrial enlargement

Management

  • Stabilize cardiopulmonary function prior to surgery
  • Supplemental oxygen
  • Mechanical ventilation
  • Inotropic agents (eg, dopamine and dobutamine) improve myocardial contractility
  • Diuretic therapy, (eg, furosemide) reduce preload, relieve volume overload and pulmonary congestion
  • Prostaglandin E1 may be required to prevent closure of ductus arteriosus to maintain systemic cardiac output
  • Primary surgical repair

Disposition

  • Admit

See Also

External Links

References

  1. ↑ Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.
  2. ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
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