Hypoplastic right heart syndrome is a congenital heart disorder in which the right atrium and right ventricle do not develop. This defect causes inadequate blood flow to the lungs and thus, blue or cyanotic baby.
Video Hypoplastic right heart syndrome
Symptoms
Common symptoms include greyishness (cyanosis) on the skin, lips, nails and other body parts. Other symptoms spoken can be fast/difficulty breathing, lack of eating, cold hands or feet, or inactive and drowsy. "In infants with hypoplastic hypoplastic syndrome, if the natural relationship between the left and right sides of the heart (the foramen oval and ductus arteriosus) is allowed to close, he may experience shock." Signs of shock may include cold or damp skin, a weak or rapid pulse, and enlarged pupils.
Maps Hypoplastic right heart syndrome
Cause
The Notch-signaling pathway is involved in several processes during cardiac development, along with Wnt signaling. [6] Differentiation of cardiomyocytes, different patterns of the heart region, valve development, ventricular trabeculation, and outlet development have all been shown to depend on the activity of specific Notch-signaling elements. [6] The importance of the Notch signal in human disease is evident from the discovery that many mutations in this path component are inherited in some genetic disorders and are acquired, although one can not obtain hypoplastic right heart syndrome. [6] Some mutations are considered on chromosome 2. [9] Other removal is considered the arm of chromosome 9q34 and 20p12, containing NOTCH1 and JAG1 and the removal of band q22 or q24. [6] Wnt signaling also plays a role along with Notch 1.2 and JAG in the formation of cardiac tissue and cardiac development. [2] When there are mutations in the Notch and Wnt lines, Notch and Wnt are not marked or blocked. [2] This pathway should signal proteins and other pathways, such as Tbx2, 3 and 20, Bmp2, 10, and Hey1 and 2 that play a major role in various parts of the developing heart. When proteins and other pathways are not characterized or forms of abnormalities are inhibited [6]
Pathogenesis
When the right side of the heart is more backward than the left side, this is known as right heart syndrome hypoplasia. HRHS is known for pulmonary atresia valves, tricuspid valves, and pulmonary arteries of hypoplasia fail to form properly. HRHS also causes the right ventricle to be smaller than the left side.
In people with hypoplastic right heart syndrome, the heart is unable to pump blood to the lungs. The result of this is an inadequate supply of oxygenated blood to circulate to the body. The severity of underdevelopment varies for each individual. A special team of pediatric cardiologists is required to develop a treatment plan.
Anatomy
A healthy heart has four valves, separated by a flap that opens and closes to control blood flow between the rooms. When the heart beats, poor blood of oxygen enters the right atrium. The blood then flows into the right ventricle, where it enters the pulmonary artery to travel to the lungs for oxygen. The oxygen-rich blood returns to the left atrium, where it then moves into the left ventricle. The left ventricle pushes oxygenated blood into the aorta to circulate throughout the body.
The heart is an organ that comes from mesoderm; Mesoderm is a layer of central germ of an embryo, whose formation is governed by various genes. Initially, the most important is the expression of Nkx2.5, CR1, pitx2, anf and mhc2a, responsible for distinguishing types of cardiomyocytes to determine which part of the heart they are headed. [5] Later in differentiation, activation of hand1, hand2 and other genes is revealed to aid in the development. [5] Expression of gene expression is governed by a variety of processes, including transcription and growth factors, as well as proteins such as fibrillin, Wnt, BMP2, BMP4, BMP5, BMP7, which aid in different cardiovascular development features such as valves and septum. , and other substances, such as retinoids and folic acid. [5] Important steps in heart formation are ventricular development and atrial formation, as well as the formation of septation and valves. Any disruption of the process can cause a variety of congenital heart diseases and disabilities that can be triggered by various genetic, epigenetic or environmental factors. The most common cardiac malformations of genetic or epigenetic problems are: aortic and pulmonary stenosis, which are vessel constriction, atrial and/or ventricular septal defects, tricuspid atresia, left hypoplastic and right heart. When you have a right hipoplastik or heart left more than one of these problems occur together [5]
Diagnosis
Treatment
There is no cure for hypoplastic right heart syndrome. A three-stage surgical procedure is usually used to treat this condition. The surgery rearranges blood flow inside the heart and allows the left ventricle to do the work for the undeveloped right side of the heart. These three operations are spread over several patients in the first few years of life. The first procedure, usually the Norwood or BT shunt procedure, is usually done within the first few days or weeks of life. The second procedure, called the Glenn procedure, is usually done between four and twelve months. The last operation, known as the Fontan procedure, is usually done around the age of 18 months and older. These operations change the blood flow to the lungs so there is always oxygenated blood. In the Norwood stage 1 procedure for hypoplastic right heart, the main pulmonary artery is separated from the left and right portion of the pulmonary artery and joins the upper part of the aorta. [7] The proximal pulmonary artery is connected to the aortic arch, while the narrowed segment of the pulmonary trunk is repaired. An aortopulmonary shunt is made to connect the aorta to the main pulmonary artery to provide pulmonary blood flow to the lungs. [7] The Glen procedure breaks the superior cava vein of the heart and connects it to the right pulmonary artery so that deoxygenated blood from the upper body goes directly to the lungs. [10] The Fontan procedure, performed usually after a two-year-old patient, cuts the inferior vena cava of the heart and connects it directly to other pulmonary arteries so that deoxygenated blood from the lower body is then sent directly to the lungs. [1]
Follow-up care
With a series of surgeries or even a heart transplant, newborns can be treated but can not be cured. Young people who have undergone reconstructive surgery should refer to cardiologists who are experienced in congenital heart disease, "Children with HLHS are at an increased rate for developing endocarditis." Children who have been diagnosed with HRHS should limit the physical activity they follow in their own endurance levels.
Prevalence
Right hypoplastic heart syndrome is less common than the hypoplastic cardiac left syndrome that occurs in 4 out of every 10,000 births. [3]. This rare anomaly requires a prenatal diagnosis because it requires immediate and emergency treatment. Pregnant women whose pregnancies are complicated by these anomalies should be referred to a level 3 hospital with pediatric cardiology and pediatric cardiothoracic surgery teams. [3]
This may be associated with aortic stenosis.
References
External links
Source of the article : Wikipedia
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