“Hypoplastic left heart syndrome accounts for 9% of all critically ill newborns with congenital cardiac disease, causing the largest number of cardiac deaths in the first year of life.(2) ” HLHS is a severe heart defect that is present at birth. HLHS combines different defects that result in an underdeveloped left side of the heart. This syndrome is one of the most challenging and difficult to manage of all of the congenital heart defects. Multiple portions on the left side of the heart are affected including the left ventricle, the mitral and aortic valve, and the ascending aorta. These structures are greatly reduced in size, or completely nonexistent causing the functionality of the left heart to be reduced, or non-functional all together. …show more content…
If the necessary steps of palliative care are not taken, this syndrome will be fatal to a diagnosed newborn. Since the left side of the heart is responsible for pumping blood out to the body, this disease greatly reduces normal blood flow through the heart and out to the body. While a baby is still in the womb, two holes develop normally, called the ductus arteriosus and the foramen ovale. These two openings between the right side and left side of the heart are still present after birth. In a newborn with hypoplastic left sided syndrome, this opening will allow for blood to bypass the poorly functioning left side for a short period of time. This natural opening allows the baby survival for the first few days of life, however these holes will usually close by the fourth day after birth. In about twenty percent of cases, the opening will close within the first day, and in over eighty percent of cases the opening can close on the second day. After the closure of the ductus arteriouses, and foramen ovale, the right heart must now work twice as hard to make up for the decreased function of the left side of the heart. Now the right side will now not only have to send oxygen low blood to the lungs, it now has to send the oxygen enriched blood out to the entire body. On top of a doubled workload, the right side of the heart is much less muscular than the left, adding more strain to the lower ventricle. These factors cause a decrease in blood flow not only through the heart, but to the whole body, which will also decrease perfusion to vital organs. When the baby and it's organs are not being well perfused with blood, everything begins to shut down, and the baby will enter shock. If immediate action is not taken death will ensue. The cause of HLHS in babies is not known, but occurs in almost 960 babies each year (CDC) . The defect occurs when the heart is forming in the fetus around the fifth week of pregnancy. Researching and knowing ones family history of this defect is the only clear risk factor for this disease, but some other factors have been considered. In some cases it has been thought that a change in the genes of the fetus may cause the defect to occur. An expecting mother can take vitamins such as folic acid daily, avoid drinking and smoking to help reduce the risk of their baby being born with a heart defect. Certain medicines, enviromental factors, and exposure to those with an illness where a fever is present should be avoided as well. Avoiding these possible teratogens are a few suggestion an expected mother can follow for the well-being of her baby. It should also be noted that if you have one child with this defect, your chances for having another child with the same diagnosis is increased. Having a family history of heart defects also increases your chance of a child with HLHS. In cases of family history a genetic counselor and cardiologist should be inquired regarding future births. If there is not a diagnosis of hypoplastic left heart syndrome before birth, there are some common signs and symptoms that the disease will cause shortly after birth. Since the blood flow is mixing in the right chambers before being pumped out to the body, a low blood oxygen saturation may be present. This low amount of oxygen attached to the blood will then cause blueish gray cyanotic coloring in the baby. The baby will have an increased work of breathing, due to increased pulmonary circulation. The reduced blood sent out to the body from the heart will cause a decrease in circulation, leading to cold limbs. This reduced circulation a decreased heart function will also induce tiredness, and cause a baby to tire easily during feedings, and the baby may not tolerate feedings well or seem uninterested in feeding. Since the baby cannot eat as much, they will not get all of their necessary nutritional needs to thrive. A doctor may also hear a murmur when listening to the babies heart, which will lead to further tests to confirm a diagnosis. If diagnosis is not made before birth, and the signs and symptoms are not immediately addressed, shock may in-sue once the openings between the left and right heart have closed. Shock can be present in conscious and unconscious babies, indicating a loss of circulation and the body starting to shut down. The babies skin may be cold, and clammy with cyanosis once shock occurs. Their pulse will feel very weak, and fast when palpated. Their eyes will lack shine, with dilated pupils, along with a staring gaze. The babies breathing can be either shallow and drawn-out, or very fast. Immediate medical attention is needed in this case, and 911 should be dialed. The diagnosis of hypoplastic left heart syndrome is usually discovered while the baby is still in the womb with an ultrasound.
During pregnancy an echocardiogram of the fetus can be done to produce images of the heart by sending ultrasonic sound waves to the vital organ. These sound waves create an image for the physician to analyze the babies heart function, structure sizes, and blood flow. A positive diagnosis before birth has shown to improve chances of survival, and will allow for appropriate care to be readily available at birth. If a baby is born without being diagnosed with the heart defect, some symptoms previous noted such as low oxygen levels can be suggestive of hypoplastic left heart syndrome. The baby may not display any symptoms or signs for hours after birth because of the openings allowing for blood to be pumped to the rest of the body. However, listening to the babies heart can revel a murmur indicating an irregular flow of blood in the heart. If a murmur is heard, or signs of the defect are observed, diagnostic tests will be ordered and performed. An echocardiogram is still the go-to test once the baby is born to evaluate the heart. The echocardiogram will diagnose the newborn, by revealing the underdeveloped left ventricle, mitral and aortic valve, and the ascending aorta commonly seen in
HLHS. If the baby has been diagnosed before birth, it is advised that the delivery take place at a hospital with a cardiac surgery center so that all of the necessary resources are available for the babies treatment. Prebirth diagnosis also permits the medical team to have any necessary equipment needed at the time of birth if the baby is delivered and is unstable. Treatment of hypoplastic left heart syndrome begins right after birth, with stabilization measures to ensure that the baby does not enter an unstable or life threatening situation. Medications such as prostaglandin and caffeine infusions are delivered intravenously to keep vessels dilated, and to prevent the closing of the patent opening that supplies adequate blood flow to the body system. If the newborn is having a difficult time breathing, intervention through mechanical ventilation may be needed to prevent respiratory failure. Nutritional considerations should also be made due to the fact that these newborns tire easily, and may need a NG tube to provide them their nutritional needs. Babies with hypoplastic left heart syndrome also “require volumes 10% to 20% higher than do healthy neonates to compensate for the capillary leak that occurs with prostaglandin therapy and for additional calories burned because of the elevated cardiac work” (1). Once these measures have been completed, the newborn will start a three-stage surgery over a period of years. These pre-planned operations are all classified as open heart, and will reroute the flow of blood to more adequately benefit cardiopulmonary circulation. The first surgery will occur within the first seven days of life, called the Norwood procedure. This surgery connects the lower right ventricle to a newly built aorta, replacing the underdeveloped aorta with a larger one. This gives the right ventricle the ability to send blood to the lungs as well as out to the body. There will still be some cyanosis present after the first surgery, due to the mixing of high and low levels of oxygenated blood in the heart. The Norwood surgery is the most complex and high risk of the three. The second procedure is done around the age of four to six months, and is called the Bi-directional Glenn Shunt Procedure. This procedure takes one of the pulmonary vessels and directly connects it to the superior vena cava. The superior vena cava normally returns blood to the heart from the upper portion of the body, but attatching it to a pulmonary vein will send the low oxygen saturated blood directly to the lungs. This second surgery will also decrease the workload impended on the right ventricle by bypassing the ventricle, and sending the blood with low oxygen levels straight to the lungs where the blood can pick up its needed oxygen. The final procedure is called the Fontan Procedure, and is done once the babies lungs have matured at ages eighteen months to three years of age. This surgery includee the pulmonary artery being directly connected to the inferior vena cava. The inferior vena cava sends blood low in oxygen into the pulmonary arteries, and the arteries then send the blood to the lungs. This final procedure removes the mixing of oxygen-enriched and oxygen low blood, and cyanosis should subside postoperatively. In a study done where 23 newborns survived to Norwood surgery, only nine survived the procedure due to its many complications and high risk. “While the survival rate following Stage I was 39% (9/23), all the patients who went through Stages II and III survived. (3)” Another option outside of reconstructive cardiac surgery is a heart transplant. However, heart transplants are difficult in this case due to the scarce availability of small sized hearts. A transplant may be considered when the defect is extremely severe, or when the heart is weaken from surgery. In receiving a heart transplant the baby will be need anti-rejection therapy for the rest of their life so their body does not reject the organ. Fortunately, the chances of rejection in newborns is the lowest of all transplant incidences. After this series of three surgeries or a transplant, the newborn will be closely monitored, and can be kept in hospitals postoperatively anywhere from weeks to months. Weight and Sp02 levels should be monitored at home after and between the first and second stages of surgery to ensure the newborn is thriving as expected and reaching certain goals set by the physician. Complications are possible after surgery and throughout life and require lifelong checkups specific to each patient. This potentially fatal defect is never fully cured, but it can be treated, and lived with once treated. Along with the cause of this syndrome, the life expectancy for these patients is also unknown. Each patient is different with what restrictions if any they will have throughout their life. Considerations such as medications, physical activity, or pregnancy should be made with between the patient and their primary doctor that are appropriate to their situation. Continuous check ups into adulthood are important in deciding what is best for the patient by helping to make special considerations and alterations to their self-care to optimize their health. As a HLHS patient ages and gets older, their heart may produce new problems, and may need medical attnetion including medication or surgeries. Many babies born with hypoplastic left heart syndrome grow up to live healthy, independent lives (granted they have had palliative surgery). The treatments and management of this syndrome are continuing to improve, helping the overall outlook of this diagnosis. Although the initial diagnosis of this defect can be intimidating to those effected, treatment, and management can lead to happy healthy lives.
Hypertrophic Cardiomyopathy, also known as HCM, is a type of heart disease that affects the Cardiac Muscles and Cardiac Muscle cells. This disease occurs if the Cardiac Muscle cells enlarge, which causes the wall of the heart’s ventricles (most often the left ventricle) to thicken. It can also cause stiffness in the ventricles, as well as mitral valve and cellular changes.
Of the two representations of the “Tell-Tale Heart”, the live action version is best. The live action was more accurate to the original story than the animated version was. The animated version was mostly for entertainment and got some facts wrong. In the live action, he killed the man in the same way and it had all the narrative of the story. The narrator wasn't Poe, like he was in the animated version. He disposed of the body the same and acted the way the character did in the original book. In the live action, the old man’s eye was completely covered by the film. He also panicked the same way as the book.
Ebstein’s Anomaly is a rare congenital condition, present at birth, in which the tricuspid valve is malformed and the valve itself is not in the correct anatomic place (Mayo Clinic Staff). This anomaly affects the right side of the heart – the tricuspid valve is located too deep into the ventricle, causing a smaller and weaker right ventricle. The space above the decreased right ventricle is made up of atrial tissue and this can be referred to as right ventricle dysplasia or an atrialized right ventricle (Reynolds). Typically the tricuspid valve has three freely moving leaflets, but in Ebstein’s anomaly one or two of those leaflets get fused to the heart walls causing regurgitation. Since the heart does not work as efficiently in those who have this anomaly, the heart usually compensates and becomes enlarged. It...
Cardiomyopathy, by definition, means the weakening of the heart muscle. The heart is operated by a striated muscle that relies on the autonomic nervous system to function. Cardiomyopathy is diagnosed in four different ways based on what caused the illness and exactly what part of the heart is weakened. The four main types of cardiomyopathy are dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogenic right ventricular dysplasia. One other category of cardiomyopathy that is diagnosed is “unclassified cardiomyopathy.” Unclassified cardiomyopathy is the weakening of the heart that does not fit into the main four categories.
First and foremost, Eisenmenger syndrome was initially described in 1897 when German physician, Victor Eisenmenger, reported on a patient with symptoms of dyspnea and cyanosis from infancy that subsequently developed heart failure (Connolly, 2014). The postmortem description was revealed and a ventricular septal defect was discovered (El-Chami et al., 2014a). With that being said, this had been the first time that the link between a large congenital cardiac shunt defect and the development of pulmonary hypertension had ever been noted (El-Chami et al., 2014b). The normal heart has four chambers. The two upper chambers are separated from each other by the atrial septum (NORD, 2014a). The two lower chambers are known as ventricles and are separated from each other by the ventricular septum (NORD, 2014b).
Eisenmenger Syndrome (ES) is a heart defect that was first giving the name in 1897 (Fukushima, 2015). This syndrome happens when the birth defect is not treated before the lungs’ arteries become damaged. Eisenmenger Syndrome is named after Victor Eisenmenger a man who had a patient who showed symptoms such as, breathing complications and skin that was turning a bluish color. The autopsy of this patient lead him to discover a ventricular septal defect [VSD] (El-Chami, 2014), that causes a hole in the wall on the right and left ventricular. This is the defect that begins when signaling for pulmonary artery hypertension, which progresses into more advanced stages of ES. This birth defect eventually causes patients to have various
Hypertrophic Cardiomyopathy: Effects on Young Athletes Alyssa Trimm 130568370 Wilfrid Laurier University Dr. Kalmar KP 122 Section A
During a physical examination, a specialist may hear a heart murmur which will prompt a referral to a pediatric cardiologist for an analysis. Diagnostic testing will vary by the child’s age, clinical condition, and institutional preferences. Such test may incorporate a chest X-ray, electrocardiogram, echocardiography and cardiac catheterization. A chest X-ray uses unseen X-ray beams to cr...
Tetralogy of Fallot is a congenital heart disease which involves four different heart defects in one. The four different heart defects are a large ventricular septal defect (VSD), Pulmonary Stenosis, Right ventricular hypertrophy, and an overriding aorta. These congenital defects change the normal flow of blood thro...
Hypertrophic cardiomyopathy is an inherited disease that affects the cardiac muscle of the heart, causing the walls of the heart to thicken and become stiff. [1] On a cellular level, the sarcomere increase in size. As a result, the cardiac muscles become abnormally thick, making it difficult for the cells to contract and the heart to pump. A genetic mutation causes the myocytes to form chaotic intersecting bundles. A pathognomonic abnormality called myocardial fiber disarray. [2,12] How the hypertrophy is distributed throughout the heart is varied. Though, in most cases, the left ventricle is always affected. [3] The heart muscle can thicken in four different patterns. The most common being asymmetrical septal hypertrophy without obstruction. Here the intraventricular septum becomes thick, but the mitral valve is not affected. Asymmetrical septal hypertrophy with obstruction causes the mitral valve to touch the septal wall during contraction. (Left ventricle outflow tract obstruction.) The obstruction of the mitral valve allows for blood to slowly flow from the left ventricle back into the left atrium (Mitral regurgitation). Symmetrical hypertrophy is the thickening of the entire left ven...
This is called newborn screening. Within newborn screening there three test normally given. This screening occurs not long after birth and can provide details about developmental, genetic and metabolic disorders. Two test completed in the screening are for hearing loss and for critical congenital heart disease (CCHD). The hearing test is completed by placing either a tiny earpiece of microphone in the infants’ ear with a sound playing to see whether the infant responds or not. Another way the test is given is by using tiny earphones and electrodes that are placed on the infant’s head to detect the brains response to sound (National Institute of Child Health and Human Development, 2015). The other tests that may be performed is the CCHD screen which the amount of oxygen in an infant’s blood is measured. In order to do this a sensor is placed on the infants’ skin for a couple of minutes, while the sensor measures the level of oxygen in the blood. Within in newborn screening there is also another tests which is the most important
Technology has had a very prominent influence on electronic fetal monitoring since its appearance in the 1960’s and 1970’s. For many years, fetal monitoring was simply done by listening to a fetal heartbeat through a stethoscope. Dramatic changes in the heartbeat, such as a long period or a drop in the rate or intensity, could be detected,. Now, not only is the electronic fetal monitor used on the outside of the womb by strapping electrodes to the mother’s abdomen but electrodes can also be inserted during the first stage of labor and placed directly on the baby’s head. With advanced technologies such as this the acidity of the infant’s blood as well as the heart rate can be measured.
(Slide 2) What is Cardiomyopathy? If we break down the word we can see “Cardio” which means of the heart, “myo” which means muscle, and “pathy” which means disease, therefore cardiomyopathies are diseases of the heart muscle. (Slide 3) There are 3 main types of cardiomyopathies; hypertrophic, dilated, and restrictive. I will only be discussing dilated cardiomyopathy, which is characterized by the enlargement of the hearts chambers with impaired systolic function. It is estimated that as many as 1 of 500 adults may have this condition. Dilated cardiomyopathy is more common in blacks than in whites and in males than in females. It is the most common form of cardiomyopathy in children and it can occur at any age (CDC).
Why is it that by 21 days into the fetal development the baby's heart begins to
In the fetal circulation, the right and left ventricles work in a parallel circuit. The ductus venosus, foramen ovale, and ductus arteriosus are three cardiovascular structures that are important for maintaining this circulation. The umbilical vein carries relatively well-oxygenated blood from the placenta to the IVC through the ductus venosus, where it partially mixes with poorly oxygenated IVC blood from the lower part of the fetal body. When it reaches the right atrium, oxygenated blood is preferentially shunted across the foramen ovale and then enters the left atrium, where it mixes with blood from the pulmonary veins before entering the left ventricle. From the LV, the ascending aorta sends fully oxygenated blood to the coronary arteries, the head, and the upper extremities. Only a small portion of the LV cardiac output streams through the aortic arch and supplies flow to the thoracic aorta.