Truncus arteriosus: Difference between revisions
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==Background== | ==Background== | ||
* A cyanotic congenital heart defect | [[File:Truncus arteriosus.jpg|thumb|Truncus arteriosus anatomy]] | ||
* Blood is pumped through a single truncal valve into a truncal artery which gives rise to the aorta and the pulmonary arteries | *A cyanotic [[congenital heart disease|congenital heart defect]] | ||
* Associated with large VSD in most patients | *Blood is pumped through a single truncal valve into a truncal artery which gives rise to the aorta and the pulmonary arteries | ||
* Incidence ranges from 6 to 10 per 100,000 live births, accounts for 4% of all [[congenital heart disease]] <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13. </ref> | *Associated with large [[VSD]] in most patients | ||
*Incidence ranges from 6 to 10 per 100,000 live births, accounts for 4% of all [[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=== | ||
*PVR is high in the newborn infant, so there is little left to right shunting at birth | *PVR is high in the newborn infant, so there is little left to right shunting at birth | ||
*Over first several weeks of life, PVR drops, left to right shunting increases, leading to heart failure | *Over first several weeks of life, PVR drops, left to right shunting increases, leading to [[heart failure]] | ||
*Mixing of systemic and pulmonary blood leads to mild to moderate cyanosis | *Mixing of systemic and pulmonary blood leads to mild to moderate cyanosis | ||
*Pulmonary vascular obstructive disease may develop in surgically uncorrected patients | *Pulmonary vascular obstructive disease may develop in surgically uncorrected patients | ||
==Clinical Features== | ==Clinical Features== | ||
* Most present within the first weeks of life with | *Most present within the first weeks of life with | ||
** Cyanosis | **[[hypoxemia|Cyanosis]] | ||
** Pulmonary congestion and heart failure | **Pulmonary congestion and [[heart failure]] | ||
***Poor feeding, lethargy, signs of respiratory distress (tachypnea, costal-sternal retractions, grunting, and nasal flaring), tachycardia, hyperdynamic precordium, and hepatomegaly | ***[[failure to thrive (peds)|Poor feeding]], [[altered mental status (peds)|lethargy]], signs of [[shortness of breath (peds)|respiratory distress]] (tachypnea, costal-sternal retractions, grunting, and nasal flaring), [[tachycardia]], hyperdynamic precordium, and [[hepatomegaly]] | ||
** Cardiovascular findings | **Cardiovascular findings | ||
***Loud second heart sound | ***Loud second heart sound | ||
***Prominent ejection click at the apex or left sternal border | ***Prominent ejection click at the apex or left sternal border | ||
***Systolic ejection murmur at the left sternal border | ***Systolic ejection [[murmur]] at the left sternal border | ||
***Bounding peripheral pulses | ***Bounding peripheral pulses | ||
***Increased pulse pressure | ***Increased pulse pressure | ||
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**Increased pulmonary vascular markings | **Increased pulmonary vascular markings | ||
**May see absent thymus in patients with TA associated with [[DiGeorge syndrome]] | **May see absent thymus in patients with TA associated with [[DiGeorge syndrome]] | ||
* | *[[Echocardiography]] | ||
==Management== | ==Management== | ||
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*Inotropic agents (eg, [[dopamine]] and [[dobutamine]]) improve myocardial contractility | *Inotropic agents (eg, [[dopamine]] and [[dobutamine]]) improve myocardial contractility | ||
*[[ACEI]] reduces afterload | *[[ACEI]] reduces afterload | ||
*Noninvasive positive pressure ventilation often used in patients with respiratory distress due to pulmonary congestion | *[[Noninvasive positive pressure ventilation]] often used in patients with respiratory distress due to pulmonary congestion | ||
*Supportive care to correct metabolic acidosis, hypoglycemia, and anemia that may contribute to heart failure | *Supportive care to correct [[metabolic acidosis]], [[hypoglycemia]], and [[anemia]] that may contribute to heart failure | ||
*[[Prostaglandin E1]] if patient also has critical coarctation of the aorta | *[[Prostaglandin E1]] if patient also has critical [[coarctation of the aorta]] | ||
*Primary surgical repair during the neonatal period (less than 30 days of age) | *Primary surgical repair during the neonatal period (less than 30 days of age) | ||
**Improved survival rate at one year of age of >80% vs 15% in uncorrected patients <ref>Thompson LD. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. 2001;72(2):391-5.</ref> | **Improved survival rate at one year of age of >80% vs 15% in uncorrected patients <ref>Thompson LD. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. 2001;72(2):391-5.</ref> | ||
Latest revision as of 21:11, 6 October 2019
Background
- A cyanotic congenital heart defect
- Blood is pumped through a single truncal valve into a truncal artery which gives rise to the aorta and the pulmonary arteries
- Associated with large VSD in most patients
- Incidence ranges from 6 to 10 per 100,000 live births, accounts for 4% of all congenital heart disease [1]
Physiology
- PVR is high in the newborn infant, so there is little left to right shunting at birth
- Over first several weeks of life, PVR drops, left to right shunting increases, leading to heart failure
- Mixing of systemic and pulmonary blood leads to mild to moderate cyanosis
- Pulmonary vascular obstructive disease may develop in surgically uncorrected patients
Clinical Features
- Most present within the first weeks of life with
- Cyanosis
- Pulmonary congestion and heart failure
- Poor feeding, lethargy, signs of respiratory distress (tachypnea, costal-sternal retractions, grunting, and nasal flaring), tachycardia, hyperdynamic precordium, and hepatomegaly
- Cardiovascular findings
- Loud second heart sound
- Prominent ejection click at the apex or left sternal border
- Systolic ejection murmur at the left sternal border
- Bounding peripheral pulses
- Increased pulse pressure
Differential Diagnosis
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[2]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Evaluation
- ECG
- May be normal
- May have evidence of left or right ventricular hypertrophy
- Chest x-ray
- Enlarged cardiac silhouette
- Increased pulmonary vascular markings
- May see absent thymus in patients with TA associated with DiGeorge syndrome
- Echocardiography
Management
- Stabilize cardiopulmonary function prior to surgery
- Diuretic therapy, (eg, furosemide) reduce preload, relieve volume overload and pulmonary congestion
- Inotropic agents (eg, dopamine and dobutamine) improve myocardial contractility
- ACEI reduces afterload
- Noninvasive positive pressure ventilation often used in patients with respiratory distress due to pulmonary congestion
- Supportive care to correct metabolic acidosis, hypoglycemia, and anemia that may contribute to heart failure
- Prostaglandin E1 if patient also has critical coarctation of the aorta
- Primary surgical repair during the neonatal period (less than 30 days of age)
- Improved survival rate at one year of age of >80% vs 15% in uncorrected patients [3]
Disposition
- Admit
See Also
External Links
References
- ↑ Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
- ↑ Thompson LD. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. 2001;72(2):391-5.