Hypoplastic left heart syndrome

Август 22, 2022

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Hypoplastic left heart syndrome

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2. Phylogenic Disorders Of Circulatory System…
3. Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). The
4. Babies born with hypoplastic left heart syndrome usually are seriously ill soon after birth. Hypoplastic left
5. Pathophysiology At birth, the ductus arteriosus is still open, and there is higher than normal resistance
6. Hypoplastic left heart syndrome can be diagnosed prenatally or after birth via echocardiography. Typical findings include
7. 95% of untreated infants with HLHS die in the first weeks of life. Early survival has
8. Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an anomaly of the
9. Symptoms are caused by vascular compression of the airway, esophagus or both. Presentation is often within
10. Myocardial ischemia occurs when the blood flow through one or more of your coronary arteries is
11. Surgical correction is indicated in all double aortic arch patients with obstructive symptoms (stridor, wheezing, pulmonary
12. Complete vascular rings represent about 0.5-1% of all congenital cardiovascular malformations. The majority of these are
13. Transposition of the great vessels (TGV) is a group of Congenital heart defects involving an abnormal
14. Transposed vessels can present a large variety of atrio venous, ventriculoarterial and/or arteriovenous discordance. The effects
15. On chest X-ray, transposition of the great vessels typically shows a cardio-mediastinal silhouette appearing as an
16. Treatment For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which
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Phylogenic Disorders Of
Circulatory System…

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Hypoplastic left heart syndrome is a complex and rare heart defect

present at birth (congenital). The left side of the heart is critically underdeveloped in hypoplastic left heart syndrome.
If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body. Instead, the right side of the heart must pump blood to the lungs and to the rest of the body.
Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past.

Hypoplastic Left Heart
Syndrome

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Babies born with hypoplastic left heart syndrome usually are seriously ill

soon after birth. Hypoplastic left heart syndrome symptoms include:
Grayish-blue skin color (cyanosis)
Rapid, difficult breathing
Poor feeding
Cold hands and feet
Weak pulse
Being unusually drowsy or inactive

Symptoms

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Pathophysiology
At birth, the ductus arteriosus is still open, and there is higher

than normal resistance to blood flow in the lungs. This allows for adequate oxygenation via mixing between the atria and a normal appearance at birth. When the ductus begins to close and pulmonary vascular resistance decreases, blood flow through the ductus is restricted and flow to the lungs is increased.
In typical anatomy, the left side of the heart receives oxygen-rich blood from the lungs and pumps it to the rest of the body. In people with HLHS, the aorta and left ventricle are underdeveloped (beginning in utero), and the aortic and mitral valves are either too small to allow sufficient blood flow or are atretic (closed) altogether. As blood returns from the lungs to the left atrium, it cannot be pumped to the rest of the body by the left ventricle. The neonate is reliant on blood flowing through an atria septal defect to mix oxygenated and deoxygenated blood, and on a patent ductus arteriosus to allow blood to reach the aorta and the systemic circulation via the right ventricle. This is what defines HLHS as a "single ventricle" defect.

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Hypoplastic left heart syndrome can be diagnosed prenatally or after birth

via echocardiography. Typical findings include a small left ventricle and aorta, abnormalities of the mitral and aortic
valves, retrograde flow in the transverse arch
of the aorta, and left-to-right flow
between the atria. It is often
recognized during the second
Trimester of pregnancy, between 18
and 24 weeks’
gestation.

Diagnosis

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95% of untreated infants with HLHS die in the first weeks

of life.
Early survival has improved since the introduction of the Norwood procedure. Since there are no long-term studies of HLHS adults, statistics are usually derived from post-Fontan patients; it is estimated that 70% of HLHS patients may reach adulthood.
Prognosis is dependent upon the health of
the child, as there is an increased demand on
respiratory and heart rate in infants during
common childhood illnesses. This fragile
population has little cardiac reserve to
accommodate these demands and provide
hemodynamic stability during illnesses.

Prognosis

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Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is

an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Most commonly there is a larger (dominant) right arch behind and a smaller (hypoplastic) left aortic arch in front of the trachea/esophagus. The two arches join to form the descending aorta which is usually on the left side (but may be right-sided or in the midline). In some cases the
end of the smaller left aortic arch closes
(left atretic arch) and the vascular tissue
becomes a fibrous cord. Although in these cases a
complete ring of two patent aortic arches is not
present, the term ‘vascular ring’ is the accepted
generic term even in these anomalies.

Double Aortic Arch

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Symptoms are caused by vascular compression of the airway, esophagus or

both. Presentation is often within the first month (neonatal period) and usually within the first 6 months of life. Starting at birth an inspiratory and expiratory stridor (high pitch noise from turbulent airflow in trachea) may be present often in combination with an expiratory wheeze. The severity of the stridor may depend on the patient’s
body position. It can be worse when
the baby is lying on their back rather
than their side. Sometimes the stridor
can be relieved by extending the
neck (lifting the chin up).

Symptoms

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Myocardial ischemia occurs when the blood flow through one or more

of your coronary arteries is decreased. The low blood flow decreases the amount of oxygen your heart muscle receives.
Myocardial ischemia can develop slowly as arteries become blocked over time. Or it can occur quickly when an artery becomes blocked suddenly.
Conditions that can cause myocardial ischemia include:
1. Coronary artery disease (atherosclerosis). Plaques made up mostly of cholesterol build up on your artery walls and restrict blood flow. Atherosclerosis is the most common cause of myocardial ischemia.

Causes

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Surgical correction is indicated in all double aortic arch patients with

obstructive symptoms (stridor, wheezing, pulmonary infections, poor feeding with choking). If symptoms are absent a conservative approach (watchful waiting) can be reasonable. Children with very mild symptoms may outgrow their symptoms but need regular follow-up.
1. Anesthesia and intraoperative monitoring.
2. Open division of vascular ring.
3. Postoperative care.

Treatment

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Complete vascular rings represent about 0.5-1% of all congenital cardiovascular malformations.

The majority of these are double aortic arches. There is no known gender preference, i.e. males and females are about equally affected. There is also no known ethnic or geographic disposition.
Associated cardiovascular anomalies are found in 10-15% of patients. These include:
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Tetralogy of Fallot
Transposition of the great arteries

Epidemiology

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Transposition of the great vessels (TGV) is a group of
Congenital heart defects involving

an abnormal spatial
arrangement of any of the great vessels: superior and/or
inferior venae cavae, pulmonary artery, pulmonary veins,
and aorta. Congenital heart diseases involving only the
primary arteries (pulmonary artery and aorta)
belong to a sub-group called transposition
of the great arteries.

Transposition Of The
Great Vessels

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Transposed vessels can present a large variety of atrio venous, ventriculoarterial and/or arteriovenous discordance. The effects may

range from a change in blood pressure to an interruption in circulation, depending on the nature and degree of the misplacement and which vessels are involved.
Although "transposed" literally means "swapped", many types of TGV involve vessels that are in abnormal positions, while not actually being swapped with each other. The terms TGV and TGA are most commonly used in reference to dextro-TGA – in which the arteries are in swapped positions; however, both terms are also commonly used, though to a slightly lesser extent, in reference to levo-TGA – in which both the arteries and the ventricles are swapped; while other defects in this category are almost never referred to by either of these terms.

Types

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On chest X-ray, transposition of the great vessels typically
shows a cardio-mediastinal silhouette

appearing as an
"egg on a string", wherein in which the enlarged heart
represents an egg on its side and the narrowed,
atrophic thymus of the superior mediastinum represents
the string.

Diagnosis

X-ray showing characteristic finding in case of Transposition of the great vessels which is called egg on side sign.

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Treatment
For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which allows

mixing of the otherwise isolated pulmonary and systemic circuits. Thus oxygenated blood that recirculates back to the lungs can mix with blood that circulates throughout the body. The arterial switch operation is the definitive treatment for dextro-transposition. Rarely the arterial switch is not feasible due to particular coronary artery anatomy and an atrial switch operation is preferred.