Heart

Stenosis

Spinal stenosis is a medical condition in which the spinal canal narrows and compresses the spinal cord and nerves. This is usually due to the spinal degeneration that commonly occurs with aging. It can also sometimes be caused by osteoporosis, spinal disc herniation, or a tumor.

Spinal stenosis may affect the cervical, thoracic or lumbar spine.

Symptoms include:

  • Pain
  • Weakness
  • Tingling of the legs

Surgery for spinal stenosis is the most common spinal operation in people over the age of 50. However, spinal stenosis caused by developmental narrowing of the spinal canal may occur in people in their 20s and 30s.

Operations used to treat stenosis include:

  • Anterior Cervical Discectomy and Fusion — A small incision in the front of the neck is used to access the upper spine. The ruptured or herniated disc is removed and replaced with a small bone plug, which eventually grows to connect the two adjacent vertebrae.
  • Cervical Corpectomy — Part of the vertebra and discs are removed and replaced with a bone graft or a metal plate and screws to support the spine.
  • Decompressive Laminectory — The roof of the vertebrae, called the lamina, is surgically removed. The procedure also may include removing part of the disc or fusing the vertebrae (spinal fusion).
  • Foramenotomy — The area where nerve roots leave the spinal canal, called the foramen, is removed. This procedure can be performed using a minimally invasive approach with an endoscope, an instrument that allows the surgeon to see inside the body through a tiny incision. The surgeon can then use other tiny incisions to perform the surgery, avoiding the discomfort and muscle atrophy associated with the traditional open technique that uses a large incision.
  • Laminoplasty — The compressive bone in the back of the neck is gently lifted off of the spinal cord creating a new “roof” over the spinal cord and nerve roots. This procedure effectively decompresses the spinal cord over multiple segments without the need for fusion or hardware. It also minimizes the chance of spinal instability or deformity that may result from the traditional laminectomy procedure.
  • Laminotomy — Only a small portion of the lamina is removed.
  • Medial Facetectomy — Part of the bone structure in the spinal canal, called the facet, is removed.
  • Cervical Disc Replacement — Instead of fusing the affected area, the natural disc material is replaced with a metal and plastic prosthesis that maintains or restores the motion segment. This will hopefully prevent degeneration of the next disc level.

Cervical Stenosis

Stenosis in the neck, also called the cervical spine, affects the upper part of the body including the arms and hands. Stenosis is the narrowing of the bony canal that protects the spinal cord and its branching nerves to the point where it injures the spinal cord or nerves.

This may be caused by a number of conditions including:

  • Bone spurs
  • Rupture of the spinal discs

Cervical stenosis may cause pain, numbness, or weakness in the legs. The pain may move from one part of the body to another but is often most noticeable in the neck.

If the stenosis is severe and is not responding to other treatment methods, surgery to widen the spinal canal may be necessary. Because bone continues to deteriorate, additional treatment may be needed several years after even successful surgery.

Lumbar Stenosis

Stenosis in the lower back is called lumbar stenosis. It is often characterized by radiating pain in the buttocks and legs.

Frequently people afflicted with lumbar stenosis have varying degrees of low back discomfort. The pain typically occurs most often during activities and is relieved by resting, sitting or bending forward. In some cases, the pain is centralized in the lower legs and feet. In severe cases, it also can impact continence (bowel and bladder control) and sexual function.

The initial treatment for stenosis is to treat the symptoms rather than the condition itself. These treatments include:

  • Rest
  • Posture changes, such as lying with the knees drawn up to the chest or leaning forward while walking, may relieve the pressure on the nerves
  • Medication such as aspirin or ibuprofen to relieve inflammation and pain
  • Physical therapy
  • Losing weight
  • Corticosteroid injections to reduce inflammation and relieve pain
  • A cervical collar

If several months of treatment have not improved the symptoms, and if the stenosis is severe, surgery to widen the spinal canal may be necessary. Because bone continues to deteriorate, additional treatment may be needed several years after even successful surgery.

Henoch-Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a disease that causes small blood vessels in the skin to leak because of inflammation.

HSP can occur any time in life, but it usually happens in children between the ages of 2 and 11.

The causes of HSP are not fully understood. One theory is that it may develop as an immune response to an infection. Once the immune cells have rid the body of the germ cells, they continue to attack other cells in the body. This theory is based on the fact that, in many cases, HSP symptoms recur or worsen during upper respiratory infections. HSP has also been associated with insect bites and exposure to cold weather. Other cases have developed after a person received vaccination for typhoid, measles, cholera, hepatitis B, or yellow fever. Some foods, drugs, or other chemical toxins may trigger HSP as well. Often no cause can be found.

HSP has four main symptoms:

  • Rashes and bruising. Leaking blood vessels in the skin cause rashes that look like bruises or small red dots to develop on the legs, buttocks, and back of the arms. The rash may first look like hives, then change to look like bruises. Rarely, the rash may spread to the upper part of the body, but it is usually on the parts of the body that “hang down,” like the legs, buttocks, elbows, and even earlobes. The rash does not disappear or turn pale when you press on it.
  • Abdominal pain. About two-thirds of people with HSP experience pain in the stomach that may cause vomiting or blood in the stool. This pain and bleeding can vary from mild to severe.
  • Arthritis. About 80 percent of people with HSP have pain and swelling in their joints, usually in the knees and ankles, less frequently in the elbows and wrists. These joint symptoms have no long-lasting effects, although they can be very uncomfortable while they’re present.
  • Kidney involvement. Blood in the urine (hematuria) occurs in about 40 percent of people with HSP. Often the blood cannot be seen by the naked eye, but it can be measured with a laboratory test called a urinalysis. In most people the hematuria goes away without permanent kidney damage. Protein in the urine or development of high blood pressure (hypertension) suggests more severe kidney problems.

In most cases, HSP lasts 4 to 6 weeks, with no long-term consequences. Sometimes symptoms come and go during this time period. About one in three people have more than one episode (recurrence) of HSP. Recurrences usually occur within a few months and are usually less severe than the initial episode. Even when it lasts longer than a few months, HSP can still resolve completely.

There is no specific treatment for HSP. The main goals of treatment are to relieve symptoms such as joint pain, abdominal pain, or swelling. In most cases, you can use over-the-counter medicines, such as acetaminophen (Tylenol), for the pain. In some patients with severe arthritis, the doctor may prescribe prednisone, a steroid medicine.

Between 20 and 50 percent of children with HSP develop some kidney problems, but only 1 percent progress to total kidney failure. Progression to kidney failure may take as long as 10 years. Your doctor will check your kidney function with blood and urine tests even after the main symptoms of HSP disappear. People who develop kidney disease usually show signs within 3 to 6 months after the initial rash appears. These immunosuppressive drugs may be prescribed to keep kidney disease from progressing to permanent kidney failure. A person with severe kidney failure must receive a blood cleansing treatment called dialysis or a kidney transplant if the damage is permanent.

Another rare complication of HSP is intussusception of the bowel, or intestine. With this condition, a section of the bowel folds into itself like a telescope. The bowel may become blocked as a result. Prednisone may be prescribed by your doctor, or the problem may need to be corrected with surgery.

Transposition of the Great Arteries

Transposition of the great arteries (TGA) is when the two major arteries leaving the heart are connected to the wrong ventricles, the lower pumping chambers of the heart. The result is that blood containing oxygen from the lungs is pumped back into the lungs. Blood that lacks oxygen, which is necessary to nourish the body, is pumped throughout the body.

The heart consists of four chambers. There are two upper chambers, called atria, where blood enters the heart; and the two lower chambers, called ventricles, where blood is pumped out of the heart. The flow between the chambers and between the arteries is controlled by a set of valves that act as one-way doors.

The heart also can be viewed as two side-by-side pumps with one side — an atrium and a ventricle with valves connecting them to blood vessels — pumping blood into the lungs and the other side pumping blood from the lungs back to the body.

Blood is pumped from the right side of the heart up through the pulmonary valve and the pulmonary artery to the lungs. In the lungs, blood is filled with oxygen. From the lungs, the blood travels back down to the heart’s left atrium and left ventricle. Then, it’s pumped through a big blood vessel called the aorta to the rest of the body.

A baby with transposed arteries is blue, or cyanotic, shortly after birth. The blueness doesn’t go away even if the baby is given extra oxygen. For a baby with transposed arteries to survive, blood flow between the right and left sides of the heart must be increased. These infants rarely survive without surgical intervention so it is extremely rare for a person to grow to adulthood without treatment.

Surgical atrial septectomy and balloon atrial septostomy are two palliative therapies for TGA. Physiological procedure (atrial switch operation) and anatomic repair (arterial switch operation) are also available treatments. The survival rate for infants with transposition of the great arteries today is greater than 90%.

Syncope (Fainting) in Children

Syncope (fainting) is common during childhood.

Syncope is caused by a sudden decrease in blood pressure, which temporarily deprives the brain of a sufficient amount of oxygen. Dizziness often occurs before syncope and many patients complain of dizziness without syncope.

Syncope has a variety of causes. Occasionally, syncope is caused by a neurologic problem such as a seizure or migraine headache. Other non-cardiac causes of syncope include:

  • Breath-holding spells
  • Rapid breathing (hyperventilation)
  • Hysteria
  • Exposure to certain drugs or toxins

Cardiac causes of syncope during childhood are rare but are the most worrisome because they can be life-threatening.

  • Secondary to obstruction to blood flow – aortic valvar stenosishypertrophic cardiomyopathy, primary pulmonary hypertension, Eisenmenger’s syndrome
  • Heart rhythm abnormalities – ventricular tachycardia, Wolff-Parkinson-White syndrome, long QT syndrome, sinus node dysfunction, atrioventricular block, arrhythmogenic right ventricular dysplasia
  • Diminished heart function – ventricular dysfunction from a variety of causes including:
    • Dilated cardiomyopathy
    • Inflammatory diseases such as acute myocarditis and Kawasaki disease
    • Ischemic heart disease secondary to an anomalous coronary artery, Kawasaki’s disease, or hypercholesterolemia

By far the most common cause of syncope during childhood, accounting for more than 90 percent of syncope in children, is:

  • Secondary to irregularities in a normal involuntary (autonomic) reflex resulting in an inappropriate relaxation of the blood vessels (vasodilation)
  • Lowering of the heart rate (bradycardia) at a time when the body actually needs the blood vessels to constrict and the heart rate to increase

These changes result in an inappropriately low blood pressure (hypotension) resulting in dizziness and syncope.

This common, non-life threatening form of syncope has been given several names including neurally mediated syncope, neurocardiogenic syncope, vaso-vagal syncope, and vasodepressor syncope.

Most causes of syncope can be successfully treated. Syncope secondary to neurologic causes can be treated with medications. Cardiac causes of syncope have a wide range of treatment options dependent upon the specific cause.

Neurally mediated syncope can often be treated without medications by avoiding situations that may provoke syncope, avoiding caffeine, increasing one’s salt intake, and by staying well hydrated. Fluid intake should be increased to the point that one’s urine is colorless.

Various maneuvers can also be performed to prevent dizziness from progressing to syncope. These include:

  • Lying down
  • Squatting
  • Tensing one’s abdominal muscles
  • Crossing one’s legs at the ankles
  • Placing one foot on a stool or chair while the other foot remains on the ground

If non-pharmacologic measures fail to adequately decrease the frequency and severity of one’s symptoms, a number of different medications can be tried. The most commonly prescribed medication for children with neurally mediated syncope is Florinef. This medication works by helping the kidneys retain fluid and sodium. Rare side effects include minimal weight gain, excessively high blood pressure (hypertension), and leg cramping. Leg cramping is caused by low potassium. Patients on Florinef should increase their potassium intake by eating bananas and drinking fruit juices.

Beta-blockers (propranalol, atenolol, nadolol, metoprolol) are also frequently prescribed. These medications work by preventing the inappropriate reflex that leads to syncope. Potential side effects include fatigue, decreased exercise performance, moodiness, and depression.

Disopyramide (Norpace), another medicine occasionally prescribed, also works by preventing the inappropriate reflex from occurring. Common side effects of Disopyramide (Norpace) include dry mouth, blurred vision, and constipation.

In rare cases of neurally mediated syncope, pacemaker implantation is warranted.

Most patients with neurally mediated syncope will eventually outgrow their symptoms, though this may take several years.

Syncope (Fainting)

Syncope, commonly called fainting or “passing out”, is a medical term used to describe a temporary loss of consciousness due to the sudden decline of blood flow to the brain.

Syncope can occur in otherwise healthy people and affects all age groups, but occurs more often in the elderly.

There are several types of syncope:

  • Vasovagal syncope usually has an easily identified triggering event such as emotional stress, trauma, pain, the sight of blood, or prolonged standing.
  • Carotid sinus syncope happens because of constriction of the carotid artery in the neck and can occur after turning the head, while shaving, or when wearing a tight collar.
  • Situational syncope happens during urination, defecation, coughing, or as a result of gastrointestinal stimulation.

Syncope can also be a symptom of heart disease or abnormalities that create an uneven heart rate or rhythm that temporarily affect blood volume and its distribution in the body.

Syncope isn’t normally a primary sign of neurological disorders, but it may indicate an increased risk for neurologic disorders such as Parkinson’s disease, postural orthostatic tachycardia syndrome (POTS), diabetic neuropathy, and other types of neuropathy.

Certain classes of drugs are associated with an increased risk of syncope, including:

  • Diuretics
  • Calcium antagonists
  • ACE inhibitors
  • Nitrates
  • Antipsychotics
  • Antihistamines
  • Levodopa
  • Narcotics
  • Alcohol

If an individual is about to faint, he or she will:

  • Feel dizzy, lightheaded, or nauseous
  • Their field of vision may “white out” or “black out”
  • Their skin may be cold and clammy

As they lose consciousness they drop to the floor. After fainting, an individual may be unconscious for a minute or two, but will revive and slowly return to normal.

The immediate treatment for an individual who has fainted involves checking first to see if their airway is open and they are breathing. They should remain lying down for at least 10-15 minutes, preferably in a cool and quiet space. If this isn’t possible, have them sit forward and lower their head below their shoulders and between their knees. Ice or cold water in a cup is refreshing.

Syncope is a dramatic event and can be life-threatening if not treated properly. Generally, however, people recover completely within minutes to hours. If syncope is symptomatic of an underlying condition, then the prognosis will reflect the course of the disorder.

For individuals who have problems with chronic fainting spells, therapy should focus on recognizing the triggers and learning techniques to keep from fainting. At the appearance of warning signs such as lightheadedness, nausea, or cold and clammy skin, counter-pressure maneuvers that involve gripping fingers into a fist, tensing the arms, and crossing the legs or squeezing the thighs together can be used to ward off a fainting spell. If fainting spells occur often without a triggering event, syncope may be a sign of an underlying heart disease.

Single Ventricle Anomalies

The term “single ventricle anomaly” is purposely non-specific. It is used to describe a group of cardiac defects that may differ quite dramatically from each other but share the common feature that only one of the two ventricles is of adequate functional size.

Some of the anomalies described as single ventricle defects include:

The ultimate plan for reconstruction is actually quite similar for most of these anomalies. All will generally undergo staged reconstructive procedures ultimately resulting in a Fontan circulation.

In the normal heart each ventricle does a separate job. The right ventricle pumps blood to the lungs, and the left ventricle pumps blood to the body. In a single ventricle heart, there is only one ventricle large enough to do the normal job of pumping blood.

Whenever there is only one ventricle large enough to do a normal job of pumping blood, we need to configure the circulation to maximize the efficiency of this single ventricle without overworking it. This ultimately requires committing the single ventricle to doing the harder work of the heart, pumping blood to the body. The job of getting blood to the lungs must be done without a pump.

How long a heart with a single ventricle reconstruction can function is not known. The first children to have a successful Fontan operation are just now 30 years old and many improvements in surgical technique and medical management have occurred over this time period.

Late complications including irregular rhythms and heart failure may occur. Some speculate that most single ventricle hearts will not function efficiently beyond 30 to 40 years, but improvements in surgical technique and medical care may increase this age significantly. In some cases, if the ventricular function deteriorates significantly, heart transplantation may be considered. Because of the possibility of late complications in patients with single ventricle anamoly, continued regular follow-up with a cardiologist for the life of a patient is essential.

Fontan Circulation

The “Fontan circulation” refers to the configuration where the single ventricle pumps blood returning from the lungs to the body, and the blood returning from the body travels to the lungs by direct blood vessel connections without a pumping chamber. In any individual child there may be different procedures needed to achieve this goal.

For a heart with a Fontan reconstruction to work well, there are a few crucial features that must be maintained:

  • The single ventricle must not be overworked for a long period of time, in terms of either having to pump too much blood or pump at too high a pressure.
  • The pulmonary arteries must grow well without stenosis (narrowing) and must remain low resistance (or be very relaxed). If the pulmonary arteries are narrow or if the resistance in these vessels is high, blood will not be able to flow into them without a pump, so the Fontan will not be successful.
  • Leaky or tight valves may adversely affect the function of the ventricle or the flow of blood to the lungs.

The type of operation needed in the newborn period is quite varied depending on the specific type of single ventricle cardiac defect. Whatever is needed in the newborn period, the aim is typically to balance the blood flow between the lungs and the body, achieving stable oxygen levels and adequate heart function. Some of the operations performed at this stage include:

  • Systemic to pulmonary artery shunt or Blalock-Taussig (BT) shunt
  • A procedure to restrict blood flow to the lungs
  • The Norwood procedure

Rarely, a baby with a single ventricle anomaly will have “just right” flow into the lungs so that an equal amount of blood flows to the body and the lungs. These babies do not require intervention in the newborn period.

The second stage for most children with single ventricle anomolies is undertaken around three to six months of age. The operation is called a “bi-directional Glenn” or sometimes a “hemi-Fontan”. During the Glenn operation the large vessel that drains blood from the head and upper body back to the heart (the superior vena cava) is taken off the heart and sewn directly to the pulmonary artery. If a prior BT shunt was present, it is removed. If a pulmonary artery band was previously placed, it may be removed but can also be left in place in some situations.

The Glenn operation removes some of the work from the single ventricle so that the ventricle will no longer have to pump all of the blood to the lungs in addition to all of the blood to the body. This replaces the risk for early heart failure. In most cases this stage is tolerated the best of all the stages with a survival rate of 95 percent or better. After the Glenn operation most children will have oxygen saturation levels of 75 percent to 85 percent.

The third and final stage in the reconstruction of a single ventricle heart defect is the Fontan completion operation. This operation is usually performed at 2 or 3 years of age, based on the child’s size and clinical status.

During the Fontan operation, the blood vessels returning blood to the heart from the lower half of the body (inferior vena cava) is connected directly to the pulmonary arteries. Until now this blood has bypassed the lungs and has been pumped directly to the body resulting in oxygen levels lower than normal. After a Fontan operation, oxygen levels will be nearly normal (90s).

Currently, when patients have been well prepared for Fontan completion, the success rates are 90 percent and higher.

After a successful Fontan surgery, the limitations children experience due to their heart defect can vary greatly. At one end of the spectrum there are children with Fontan circulations who have participated in competitive sports such as swimming and gymnastics. Other children may have significant limitation in their capacity for exercise. Most children fall somewhere in between the extremes.

Most children are on a blood thinner called coumadin after their Fontan to prevent clots from forming in the Fontan circuit. When a child is on a blood thinner, you must take extra careful to avoid falls or head trauma as they are at increased risk for internal bleeding.

Pulmonary Valvar Stenosis

Pulmonary stenosis is a condition characterized by obstruction to blood flow from the right ventricle to the pulmonary artery.

This obstruction is caused by narrowing (stenosis) at one or more points from the right ventricle to the pulmonary artery. It includes obstruction from thickened muscle below the pulmonary valve, narrowing of the valve itself, or narrowing of the pulmonary artery above the valve. The most common form of pulmonary stenosis is obstruction at the valve itself, referred to as pulmonary valvar stenosis.

The normal pulmonary valve consists of three thin and pliable valve leaflets. When the right ventricle ejects blood into the pulmonary artery, the normal pulmonary valve leaflets spread apart easily and cause no obstruction (blockage) to outflow of blood from the heart. Most commonly with pulmonary valvar stenosis, the pulmonary valve leaflets are thickened and fused together along their separation lines (commissures). When the tissue is thickened, the leaflets become less pliable than normal, which contributes to the obstruction. At times, the diameter of the pulmonary valve itself is small or hypoplastic.

When the pulmonary valve is obstructed, the right ventricle must work harder to eject blood into the pulmonary artery. To compensate for this additional workload, the muscle of the right ventricle (the myocardium) gradually thickens to provide additional strength to right ventricular ejection. The increased right ventricular muscle, known as hypertrophy, is rarely a problem in itself, but instead is an indication that significant valve obstruction exists.

When the pulmonary valve is severely obstructed, especially in newborns with critical degrees of pulmonary stenosis, the right ventricle cannot eject sufficient volume of blood flow into the pulmonary artery. In these instances, blue blood bypasses the right ventricle flowing from the right atrium to left atrium, through the foramen ovale, a communication or “hole” between these two chambers that is normally present in newborns. Newborns with critical pulmonary stenosis therefore will have cyanosis (blue discoloration of the lips and nailbeds) due to lower oxygen levels in their blood.

Right ventricular failure rarely occurs with pulmonary valve stenosis.

Children with pulmonary valvar stenosis are usually asymptomatic and in normal health. A heart murmur is the most common sign detected by a physician indicating that a valve problem may be present. Children with mild-to-moderate degrees of pulmonary valve stenosis have easily detectable heart murmurs, but typically do not have any symptoms. Symptoms occur only with severe pulmonary valve stenosis.

A newborn with critical pulmonary valve stenosis develops cyanosis in the first few days of life. This is due to diminished volume of blood flow into the lungs, together with a shunt of blue blood from right to left atrium. This an emergency situation that requires immediate treatment, either balloon dilation of the valve or surgery.

In an older child, severe pulmonary valve stenosis may cause easy fatigue or shortness of breath with physical exertion. Severe pulmonary valve stenosis rarely results in right ventricular failure or sudden death.

Children with mild pulmonary valve stenosis rarely require treatment. Patients with mild pulmonary valve stenosis are healthy, can participate in all types of physical activities and sporting events, and lead normal lives. Mild pulmonary valve stenosis in childhood rarely progresses after the first year of life. However, mild pulmonary stenosis in a young infant may progress to more severe degrees and requires careful follow-up.

Children with moderate-to-severe degrees of pulmonary stenosis require treatment, the timing of which is often elective. The type of treatment required depends on the type of valve abnormality present. Most commonly, the stenotic pulmonary valve is of normal size, and the obstruction is due to fusion along the commissures or lines of valve leaflet opening. This “typical” form of pulmonary valve stenosis responds very nicely to balloon dilation. Balloon dilation valvuloplasty is performed at the time of cardiac catheterization and does not require open-heart surgery. In the newborn, balloon dilation for critical pulmonary valve stenosis can be a technically challenging procedure as these newborns are often critically ill and unstable. More typically, in older children the procedure is performed electively on an outpatient basis.

Results of balloon dilation valvuloplasty for pulmonary stenosis have been excellent. Importantly, balloon dilation cannot make an abnormal valve “normal”. Instead, the procedure decreases the degree of pulmonary valve obstruction from severe to mild in the large majority of patients. For children and adolescents with “typical” pulmonary valve stenosis, a single balloon dilation procedure is usually the only therapy ever needed. It is rare that an older child will have return of significant pulmonary valve obstruction following a successful balloon dilation procedure.

Newborns and young infants with very severe pulmonary valvar obstruction will also have an excellent response to balloon dilation, unless the valve is underdeveloped in size. However, recurrence of significant pulmonary stenosis does occur in approximately 20 percent of newborns and young infants even though the initial response to balloon dilation has been excellent. These patients may require a second balloon dilation, or an open-heart surgical procedure if the valve is small.

Open-heart surgical procedures are required for more complex valves, where balloon dilation is not sufficient therapy. These valves may be obstructed by thick and dysplastic leaflet tissue (such as in patients with Noonan Syndrome), and the diameter of the valve itself may be small in some cases. For these conditions surgical pulmonary valvotomy (opening of the valve), partial valvectomy (removal of a portion of the leaflet), and possibly a transannular patch (patch from the right ventricle to pulmonary artery) may be required during the open-heart surgery repair.

The long-term outcomes for open-heart surgery in patients with severe pulmonary valvar stenosis are also excellent. Because balloon dilation is usually the only treatment necessary for most patients, open-heart surgery is generally only performed in patients with more complex forms of pulmonary valve obstruction. However, patients with very thick valve leaflets or underdeveloped pulmonary valve sizes also have an outstanding long-term outcome following open-heart surgical repair. If there is no other associated heart disease, these children are expected to lead normal, active lives.

It is important that all children with pulmonary valve stenosis, even after very successful balloon dilation or open-heart surgery, be re-evaluated at regular intervals. Long-term follow-up with evaluation by a qualified cardiologist is essential to helping provide the highest quality outcome for patients with pulmonary valve stenosis.

Pulmonary Hypertension

Pulmonary hypertension is a rare condition of high blood pressure in the blood vessels of the lungs as a result of various disorders. The pressure in the pulmonary artery leading from the right side of heart to the lungs rises above normal levels and imposes a big strain on the right side of the heart. The right side of the heart can become so enlarged and weakened that it eventually fails leading to poor quality of life and death.

Primary pulmonary hypertension (PPH) — which is pulmonary hypertension that isn’t a side effect of (or secondary to) another condition such as emphysema or lupus — strikes only between one and two people per million.

Pulmonary hypertension is classified into five different types:

  • Pulmonary Arterial Hypertension (PAH) — This form affects the blood vessels in the lungs that carry blood from the heart into the lungs where it picks up oxygen. This category is subdivided into two types:
    1. Primary pulmonary hypertension (PPH), which can occur for no discernable reason or is inherited (10% of cases). It is very rare. It most often occurs in young adults and is more than twice as common in women as men.
    2. PAH related to exposure to toxins including diet drugs such as fenphen; street drugs including cocaine and methamphetamine; HIV; collagen vascular diseases including sclerodermalupus and rheumatoid arthritis; chronic liver disease; and congenital heart diseases.
  • Pulmonary Venous Hypertension (PVH) — This form is caused by diseases of the left side of the heart, such as heart failure or mitral valve disease. This can increase pulmonary artery blood pressure but usually doesn’t become severe PAH.
  • Respiratory System — Pulmonary hypertension can be associated with diseases of the respiratory system including interstitial lung disease, emphysema, asthmatic bronchitis, sleep apnea and chronic exposure to high altitude.
  • Chronic Blood Clots — Blood clots in the lung blood vessels.
  • Blood Vessel Disorders — Pulmonary hypertension due to disorders directly affecting the blood vessels in the lungs such as parasites, or inflammation of the blood vessels.

There are no specific signs and symptoms in the early stages of the disease. The symptoms that may occur — such as shortness of breath, fatigue and chest pain — also are common to many other conditions. Other symptoms that may occur include dizziness, swollen ankles and legs, fainting and a bluish cast to lips and skin.

Over time, pulmonary arterial hypertension can damage your heart to the point of danger and result in complications that can interfere with your daily life. Complications from pulmonary hypertension include:

  • Enlarged right ventricle
  • Blood clots

Treatment is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. Since pulmonary venous hypertension is synonymous with congestive heart failure, the treatment is to optimize left ventricular function by the use of medications or to repair/replace the mitral valve or aortic valve. Lifestyle changes, digoxin, diuretics, oral anticoagulants, and oxygen therapy are considered conventional therapy for pulmonary hypertension.

Fast Arrhythmias in Children

The normal heartbeat originates from the heart’s normal pacemaker called the sinus node. An arrhythmia occurs when the electrical activity of the heart originates from a location other than this normal pacemaker. This location can be a site in the upper or lower chambers of the heart, or it can be a “circuit” composed of parts of the upper chambers, the lower chambers, or both.

The normal heart rate varies with age and activity. Babies have a faster heart rate than adults. For each age group, normal ranges have been established. If the heart rate exceeds this limit, a fast rhythm (tachycardia) exists.

If the electrical activity for this fast rhythm originates from the normal pacemaker of the heart, the rhythm is “sinus tachycardia”. If the mechanism is not normal, a fast arrhythmia or “tachy-arrhythmia” exists.

There are two basic mechanisms for fast rhythms: automatic and re-entry. Arrhythmias are also divided based on location of their origin. Fast arrhythmias that originate from the lower chambers (i.e., the ventricles) are called ventricular tachycardias. Those that originate from the upper chambers (i.e., the atria) are termed supra-ventricular tachycardias (SVT).

After diagnosis of fast arrhythmias, the first objective needs to be consultation with your cardiologists regarding the risks of the fast rhythm and the risks and benefits of its treatment. If the fast rhythm has a “benign” course with little impact on daily life, close observation may be warranted. On the other hand, an infrequent fast rhythm that is a cause for concern to either the patient, family, or physician may require therapy.

There are many medications available for treatment of fast arrhythmias. The choice of medication depends on the mechanism of the fast rhythm. In selected cases, a catheterization procedure can be performed to treat the arrhythmia.

Supra-Ventricular Tachycardias

Supra-ventricular tachycardias are usually not dangerous. If they occur very frequently or for prolonged periods of time (hours to days) then they can cause difficulty with the pumping action of the heart. They are not due to a “heart attack” and do not cause sudden death.

Types of supra-ventricular tachycardias include:

  • Atrial tachycardia – A location or an area of the upper chambers takes over the pacemaker activity of the heart. This is an automatic tachycardia and is relatively uncommon in children.
  • Atrial Flutter/Atrial Fibrillation – When a large area of the upper chamber is involved in a circuit pattern, atrial flutter can develop. This rhythm can be seen in children who have had previous heart surgery involving the upper chambers, for example the Fontan procedure.Children with atrial flutter are at risk for developing clots in the upper chambers because the flow in these chambers is slow and disorganized. There is often swirling of blood in the atrium seen on echocardiogram.
  • Atrio-ventricular re-entrant tachycardia (AVRT) – An extra electrical connection (called “accessory pathway”) between the upper and lower chamber allows the formation of a circuit that conducts electrical activity faster than the normal pacemaker. This is the most common form of fast arrhythmias in infancy.The majority of infants with atrio-ventricular re-entrant tachycardia “outgrow” the tachycardia during the first year of life. The specific diagnoses falling in this category include Wolff-Parkinson-White Syndrome (WPW) and Permanent Junctional Reciprocating Tachycardia (PJRT).
  • Atrio-ventricular nodal re-entrant tachycardia (AVNRT) – The atrio-ventricular node (A-V node) is located between the upper and lower chambers of the heart. It is the only area that normally allows the electrical activity of the heart to pass from the upper chambers to the lower chambers. Sometimes the region of the A-V node can become a source for a tachycardia. This is the most common form of fast arrhythmias in adolescence.
  • Junctional tachycardia – The origin of the tachycardia is the “junction” between the upper and lower chambers. This is an automatic tachycardia. This tachycardia is seen in patients who have had recent surgery involving this area, for example repair of a ventricular septal defect (VSD), atrioventricular septal defect or Tetralogy of Fallott.

Ventricular Tachycardia

Ventricular tachycardia are more serious than supra-ventricular tachycardias and can lead to serious heart dysfunction. When the source of the fast heart rate is the lower chambers (ventricles), ventricular tachycardia (VT) is present. These types of tachycardias are usually associated with symptoms such as passing out, lightheadedness, or dizziness.

Ventricular tachycardia is relatively uncommon in children. It can be seen in patients with congenital heart disease, especially those who, despite surgery, continue to have problems with heart function. Ventricular tachycardia is also seen with other conditions. Among the most common are prolonged QT syndrome, Hypertrophic Cardiomyopathy, and Myocarditis.

In older children and adolescents, the fast heart rate is often felt as palpitations. They recognize that a fast heart rate is occurring at an inappropriate time such as while at rest, doing homework, or eating dinner. Fast arrhythmias may also cause children or adolescents to pass out (syncope). Younger children may have difficulty describing this sensation and may complain of chest pain.

In infants, fast arrhythmias are often better tolerated. The infant’s parents become concerned when they note the fast heart rate while cuddling their infant or during feedings. Some infants may develop poor feeding, irritability, or pallor (unnatural paleness).

Patent Ductus Arteriosus

While still on the mother’s womb a baby’s lungs are not needed to supply oxygen because the baby receives its oxygen via the mother’s lungs and placenta. Since a baby’s lungs do not provide any oxygen, there is no need for energy to be expended pumping blood to the lungs. The ductus arteriosus is a blood vessel that is present in all babies while still in the womb that allows blood to bypass the pathway to the lungs; it allows blood to flow from the pulmonary artery to the aorta.

When the baby is born and the umbilical cord is cut, the lungs are now needed to supply oxygen. The lungs expand, their blood vessels relax to accept more flow and the ductus arteriosus usually closes within the first hours of life. On occasion, however, the ductus arteriosus does not close on its own and this is referred to as a patent (“patent” means open) ductus arteriosus (PDA). While this condition is much more often seen in premature babies, it may also appear in term infants.

The symptoms of PDA depend on the size of the ductus and how much blood flow it carries. After birth, the pressures and resistance are much tighter in the aorta than the pulmonary artery, so if a ductus arteriosis is present, blood will flow from the aorta into the pulmonary artery. This extra blood flow into the lungs can overload the lungs and put an additional burden on the heart to pump this extra blood. This situation may not be well tolerated in a premature baby who already has problems related to immaturity of the lungs themselves. These babies may need more support from the ventilator and have symptoms of congestive heart failure.

A newborn with a patent ductus arteriosus, may have:

  • Fast breathing
  • An increase in the work of breathing
  • More frequent respiratory infections
  • Tiring more easily
  • Poor growth

However, if the patent ductus arteriosus is not large, it may cause absolutely no symptoms at all and may be detected only upon further evaluation of a heart murmur. Even in the absence of symptoms, the turbulent flow of blood through the patent ductus arteriosus puts a person at a higher risk for a serious infection known as endocarditis.

Because of turbulent blood flow from the high pressure aorta to the low pressure pulmonary artery, a PDA causes a characteristic heart murmur that is heard on physical exam. The presence of the characteristic murmur along with symptoms of heart failure in a premature infant most frequently leads to the diagnosis of patent ductus arteriosus. The chest X-ray will show an enlarged heart and evidence of an excessive amount of blood flow to the lungs (except in older children, where an x-ray may appear normal). An echocardiogram is performed to confirm the diagnosis. This will demonstrate the size of the ductus arteriousus and will demonstrate if the heart chambers have become enlarged due to the extra blood flow.

In a newborn, the PDA still has the potential to close on its own without intervention. Thus, in newborns, additional time may be allowed for this to happen if the heart failure can be easily managed. If symptoms are severe, such as in a premature infant, or if it is felt unlikely to close on its own, medical or surgical closure is pursued.

If a PDA is still present beyond the newborn period, it will generally never close on its own. Closure is recommended in such cases to prevent the future risk of endocarditis.

In newborns, a medication such as indomethacin or ibuprofen can be given. These medications are given in the stomach and can constrict the muscle in the wall of the PDA and promote closure. These drugs do have side effects, however, such as kidney injury or bleeding, so not all babies can receive them. Because of the potential side effects, the baby must have lab values checked before medications can be given. If the lab values are not normal or if the medications do not work, surgery can be performed and the PDA tied off (ligated).

Medications are generally only successful in newborn patients. In older infants and children, treaments include surgery or closure in the cardiac catheterization laboratory with a device or coil.

  • During the cardiac catheterization procedure, the patient is sedated and catheters are placed into blood vessels in the groin. The catheters are then fed to the heart and pictures are taken of the ductus arteriosus with dye (called an angiogram). Two methods can be used to close the ductus. If it is small, a coil may be placed within the vessel which will expand to block the blood flow. If the ductus is larger, a flexible device can be placed within the ductus as a “plug”.
  • During surgery, a small incision is made between the ribs on the left side and the ductus arteriosus is tied and cut. Surgical closure of the patent ductus arteriosus can be performed at any age, and is specifically recommended in some situations such as a very large PDA or other unusual anatomy.

The risk of complications with any of these treatments is low, determined mostly by how ill the child is prior to treatment.

In some cases, having a patent ductus arteriosus can be a good thing. Some babies have heart defects that require the patent ductus arteriosus to remain open for them to survive. In some heart defects, such as pulmonary atresia (an underdeveloped or blocked pulmonary valve), the PDA supplies the only adequate source of blood flow to the lungs so that oxygen can be delivered to the blood. In these patients, the ductus arteriosus supplies blood to the lungs from the aorta.

In other anomalies, such as underdeveloped or severely narrowed aorta (like that seen in hypoplastic left heart syndrome), the PDA is crucial to allow adequate blood flow to the body. In these patents, the ductus arteriosus supplies blood to the body from the pulmonary artery.

Medication is given intravenously (IV) to keep the PDA open and requires the baby to be closely monitored in the intensive care unit. This allows stabilization of the newborn until more definitive treatment, usually surgical, can be carried out.