Paul’s relapse

Brian had difficulty breathing and the physician noticed he had blue-tinged skin, a heart murmur (an abnormal whooshing sound caused by turbulent blood flow).

Several tests were ordered.

Brian’s Initial diagnostic tests

• Chromosomal karyotype (blood test): To diagnosis congenital abnormalities
• Echocardiogram: Uses sound waves to produce moving images, providing information on structure and function  of the heart
• Electrocardiogram: Detects cardiac abnormalities by measuring the electrical activity generated by the heart
• Chest x-ray: Can show complications related to lung and heart problems
• Oxygen level measurement: Estimates the amount of oxygen in your blood
• Cardiac catheterization: Used to evaluate heart function and diagnose cardiovascular conditions

Electrical Conduction System of the Heart

• Sinoatrial node
• Atrioventricular node
• Bundle of His
• Left bundle branch
• Left posterior fascicle
• Left anterior fascicle
• Left ventricle
• Ventricular septum
• Right ventricle
• Right bundle branch

Chambers & Circulation Through the Heart

• Blood flows from the right atrium to the right ventricle, where it is pumped into the pulmonary circuit.
• The blood in the pulmonary artery branches is low in oxygen but relatively high in carbon dioxide.
• Gas exchange occurs in the pulmonary capillaries (oxygen into the blood, carbon dioxide out), and blood high in oxygen and low in carbon dioxide is returned to the left atrium.
• From here, blood enters the left ventricle, which pumps it into the systemic circuit.
• Following exchange in the systemic capillaries (oxygen and nutrients out of the capillaries and carbon dioxide and wastes in), blood returns to the right atrium and the cycle is repeated.

Facts About Tetralogy of Fallot

• Birth defect that affects normal blood flow through the heart.
• Happens when the heart does not form correctly as the baby grows & develops in the womb.
• Made up of 4 defects of the heart and its blood vessels.

The 4 Abnormalities

Pulmonary valve stenosis

Pulmonary valve stenosis is a narrowing of the pulmonary valve — the valve that separates the lower right chamber of the heart (right ventricle) from the main blood vessel leading to the lungs (pulmonary artery).

Narrowing (constriction) of the pulmonary valve reduces blood flow to the lungs. The narrowing might also affect the muscle beneath the pulmonary valve. In some severe cases, the pulmonary valve doesn’t form properly (pulmonary atresia) and causes reduced blood flow to the lungs.

Ventricular septal defect

A ventricular septal defect is a hole (defect) in the wall (septum) that separates the two lower chambers of the heart — the left and right ventricles. The hole allows deoxygenated blood in the right ventricle — blood that has circulated through the body and is returning to the lungs to replenish its oxygen supply — to flow into the left ventricle and mix with oxygenated blood fresh from the lungs.

Blood from the left ventricle also flows back to the right ventricle in an inefficient manner. This ability for blood to flow through the ventricular septal defect reduces the supply of oxygenated blood to the body and eventually can weaken the heart.

Overriding aorta

Normally the aorta branches off the left ventricle. In tetralogy of Fallot, the aorta is shifted slightly to the right and lies directly above the ventricular septal defect.

In this position the aorta receives blood from both the right and left ventricles, mixing the oxygen-poor blood from the right ventricle with the oxygen-rich blood from the left ventricle.

Right ventricular hypertrophy

When the heart’s pumping action is overworked, it causes the muscular wall of the right ventricle to thicken. Over time this might cause the heart to stiffen, become weak and eventually fail.

Tetralogy of Fallot

Brian’s Treatment

Brian was two weeks old when he required temporary surgery due to his underdeveloped pulmonary arteries (hypoplastic).  A bypass (shunt) was created between a large artery that branches off from the aorta and the pulmonary artery.

After six months, the cardiologist deemed Brian strong enough to undergo ‘intracardiac repair’.  This is an open-heart surgery that involves several repairs.

Surgery is the only effective treatment for tetralogy of Fallot.

Intracardiac repair

• Usually done within the 1st year after birth, involves several repairs
• Patch over the ventricular septal defect to close the hole between the ventricles
• Repairs or replaces the narrowed pulmonary valve and widens the pulmonary arteries
• The right ventricle will go back to normal thickness (doesn’t need to work as hard)

Temporary surgery

• May be done if premature birth or pulmonary arteries are undeveloped (hypoplastic)
• A shunt is inserted between a large artery that branches off the aorta and the pulmonary artery
• Intracardiac surgery will be performed when baby is ready and shunt will be removed

Post surgical complications

• Chronic pulmonary regurgitation (right ventricle)
• Other heart valve problems
• Continued leaks after the patch repair, may require a re-repair
• Enlarged right ventricle or left ventricle
• Arrhythmias
• Coronary artery disease
• Aortic root dilation (ascending aorta enlarges)
• Sudden cardiac death

The surgery was a success

Brian was eventually discharged home. He had around-the-clock care which included: Nursing, Physiotherapist, Occupational therapist, Respiratory therapist, Play workers and a Tutor. Brian required regular medical follow-ups to maintain good health.

• Routine follow-up care – regular check-ups with a cardiologist, primary physician routine exams, medications that are prescribed, routine dental care
• Heart-healthy lifestyle – heart-healthy eating, physical activity, maintaining healthy weight
• Emotional health – may feel isolated, sadness, and frustration

On-going Care

• Lifelong care with a cardiologist trained in treating congenital heart disease
• Routine follow-up appointments which include physical exam, blood tests, echocardiogram, ECG
• Monitor physical activity if there is any pulmonary leakage or obstruction or arrhythmias
• Antibiotics for dental procedures to prevent endocarditis

Coping and Support

• Support groups-provide hope, encouragement and support
• Family physician-provide local resources
• Family & friends-give you a break
  

Keep a written record of:

• Diagnosis
• Medications
• Surgeries and dates
• Cardiologist’s name and number

Brian’s Development and Sense of Accomplishment: Adulthood

• Brian’s father, Hugh, was concerned about Brian’s long-term health and well-being. Brian’s parents were now older with multiple health concerns.
• Brian was frustrated with being isolated at home for much of his life. Hugh was able to help Brian get a part-time job at the local grocery store once he finished his schooling. Brian earned a modest salary and really enjoyed interacting with his co-workers.
• With his modest salary, Brian needed to live with his father, but decided to participate in a local recreational swimming program at the private fitness club located next to his work.