Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome Introduction Heart diseases comprise of some of the most complicated conditions that human beings suffer from since time immemorial. In addition to heart diseases being genetically oriented they are hard to manage. Hypoplastic left heart syndrome is one of the heart diseases that affect a considerable proportion of infants in the world (Anderson, Pozzi & Hutchinson, 2004). This condition is referred to as the hypoplastic left heart syndrome since it mostly mainly affects the parts of the heart located on the left side. This paper through qualitative assessment of published work, journal articles and other legitimate sources is going to look at the etiology, pathophysiology, clinical signs, and diagnosis and complications of this disease in addition to its management.

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Definition Hypoplastic left heart syndrome is one of the rare heart conditions that obtain their names from the side of the heart they affect. This disease has been shown to affect major parts of the heart on the left side including the left ventricle, the aorta and the aortic valve (Anderson, Pozzi & Hutchinson, 2004). The parts affected by this disease do not develop completely, thereby predisposing a child to grave danger. The hearts of infants presenting with this disease do not pump blood efficiently to vital parts of the body. The work of pumping blood to various parts of the body is left to right side of the heart. The right side is therefore overworked, an aspect that aggravates an already grim situation. This disease is considered to be congenital since it is usually present at birth.

Etiology Hypoplastic left heart syndrome is not a common heart disorder. It is a congenital heart disease implying that it has no known cause. The disease is more common in males as compared to females. Research has revealed that a large number of those suffering from this disease usually present with a wide range of birth defects (Connor & Thiagarajan, 2007). Problems associated with this condition usually develop when the left ventricle and other structures located on the left side of the heart fail to develop normally. The aorta and the left ventricle are, as a result, described as being hypoplastic or poorly formed.

Complications It is important to note that the heart does not have the capacity to efficiently supply blood to the left side of the body in patients presenting with this disease. As a result, the right ventricles are forced to pump harder in order to maintain supply of blood to the entire body. The right

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ventricle can only prop up supply of blood to the vital organs for a while. The extra workload finally leads to the right ventricles becoming overloaded, thereby minimizing their capacity to pump blood. In infants presenting with this disorder, blood from the right ventricle passes through the ductus arteriosus to various parts of the body (Rychik & Wernovsky, 2003).

However, the ductus arteriosus eventually closes thereby exposing an infant to the risk of death.

Medications are used to keep the ductus arteriosus open in order to increase the chances of survival for patients.

Pathophysiology Hypoplastic left heart syndrome, also abbreviated as HLHS, is a congenital cardiac condition that affects a significant proportion of children in the United States. This condition usually affects the major vessels of the heart. This condition is characterized by incomplete development of major parts located in the left side of the heart. Considering that the left ventricle is more muscular than the right ventricle, conditions affecting this region greatly hampers the heart’s main role of pumping blood to major organs in the body (Rychik & Wernovsky, 2003).

Clinical Signs and Diagnosis Even though infants presenting with this condition may appear normal, research has revealed that they present with a wide range of underlying conditions. Symptoms associated with this disease start occurring within the first few hours of birth. However, it may take some days for these symptoms to be fully noticeable (Connor & Thiagarajan, 2007). Some common symptoms of the hypoplastic left heart syndrome include: Poor skin color, also referred to as cyanosis, lethargy and shortness of breath, poor pulse and rapid breathing, in addition to pounding heart. The bluish skin color in the hands and feet is a response of the extremities to coldness. It is actually a sign that blood supply to these regions is insufficient.

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Management In a manner similar to other heart diseases, hypoplastic left heart syndrome presents health care practitioners with a delicate situation to handle. Numerous tests are carried out in order to determine whether the child is actually presenting with this disorder. Upon diagnosis with this disorder, the baby is immediately admitted to neonatal intensive care unit. Life support gadgets such as the breathing machine are used in order to make it easy for the baby to breathe.

Medications such as prostaglandins are also made use of to maintain the ductus arteriosus in an open status (Anderson, Pozzi & Hutchinson, 2004). Surgery is, however, pertinent since these measures do not offer a lasting solution. Norwood operation, which is carried out within the first few weeks of life, is a procedure aimed at reconstructing the aorta using blood vessels from other parts of the body. A second operation is carried out to connect major vessels from the heart to the lungs (Hennein, Edward & Bove, 2002). The final operation, also referred to as the Fotan procedure, is carried out after about three years for purposes of connecting vessels carrying blue blood from the body to the lungs for oxygenation. In extreme cases transplantation may be carried out. Even though transplantation is the best management strategy, the main challenge associated with it is that donor organs are extremely scarce.

Conclusion It can therefore be concluded that even though a rare condition, hypoplastic left heart syndrome is a severe heart disorder that predisposes patients to a high risk of death. Patients presenting with this disorder, which is characterized by underdevelopment of the left region of the heart, present with symptoms such as cyanosis, increased breathing rate and pounding heart among others. Even though medications such as prostaglandins may be used to enhance supply

HYPOPLASTIC LEFT HEART SYNDROME of blood to the left side of the hearth through the ductus arteriosus, surgery remains the most effective treatment strategy.

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HYPOPLASTIC LEFT HEART SYNDROME References Anderson, R., Pozzi, M., & Hutchinson, S. (2004). Hypoplastic left heart syndrome.

Burmingham, UK: Springer, Connor, J., & Thiagarajan, R. (2007). Hypoplastic left heart syndrome. Orphanet Journal of Rare Diseases, 2(23), 1750-1172 Hennein, H., Edward, L., & Bove, M. (2002). Hypoplastic left heart syndrome. Armonk, NY: John Wiley & Sons.

Rychik, J., & Wernovsky, G. (2003). Hypoplastic left heart syndrome. Norwell, Massachusetts: Springer.

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