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Referral and follow up
All patients should have access to a local cardiologist and should also receive at least one initial assessment at a specialist CHD centre. Adults with moderate or severely complicated CHD should have their care shared between their local centre and a specialist CHD centre.
Patients with CHD require lifelong follow up. Emphasis is on quality and longevity of life rather than cure.
Treatment options
- Surgical Management: Many patients receive surgery when they are babies or during childhood. For some patients this involves multiple surgeries. There are very few operations that offer a “cure” for their heart defect and instead these are described as palliative operations or repairs. As adults, the decision to offer surgery depends on a number of factors. Patients require regular reviews to look for changes in their symptoms, haemodynamic status or exercise tolerance, and reviewing echo and MRI imaging for evidence of changes in their heart. Some of these operations include valve replacements or repairs, aortic root replacements, coractation repairs, closing ASD’s or VSD’s and. in some cases, heart or heart and lung transplantation.
- Trans Catheter: There have been many advances in transcatheter interventions in recent years which provide an opportunity for some patients to receive a much less invasive treatment. For example, some ASDs, VSDs, and PDAs can be closed with devices and some coarctations of the aorta and stenosed pulmonary arteries can be stented, all through a transcatheter approach.
Complications
Heart failure
Heart failure is a common problem for CHD patients and, due to the lack of research into heart failure specifically in the congenital context, ESC guidelines suggest that patients are treated in line with current, general heart failure management recommendations. However, the effectiveness of these treatments (particularly for patients with systemic right ventricles or Fontan circulations) is unknown.
Arrhythmias
Arrhythmias are the main reason for hospitalisation in ACHD patients. Arrhythmias are often a sign of haemodynamic deterioration in CHD patients and they often need urgent Direct Current Cardioversion (DCCV), particularly in the case of patients who have Fontan circulations. Antiarrhythmic drug therapy is often poorly tolerated due to the negative inotropic effects.
Individuals with unexplained syncope (particularly in patients with Tetralogy of Fallot; Transpostion of the Great Arteries and congenitally corrected Transposition of the Great Arteries; Univentricular hearts; and Aortic Stenosis) are at highest risk of sudden cardiac death.
Pulmonary Arterial Hypertention (PAH)
The development of PAH occurs in patients who have untreated systemic to pulmonary shunts (blood flowing through a defect in the heart from the left side to the right side of the heart).
In cases such as Eisenmenger’s Syndrome this shunting process eventually leads to PAH and causes irreversible damage to the lungs which then, in turn, results in a reversal of the shunting. This reversal (blood flowing from the right side of the heart, through a defect and into the left side of the heart) results in the patient becoming cyanosed.
Patients who have PAH in the context of CHD can be offered drug therapies, which need to be managed by a specialist congenital centre. These medications include prostanoids, endothelin receptor antagonists (ERAs) and phosphodiesterase type-5 inhibitors.
Patients with PAH and cyanosis require complex management and the condition will involve multiple organs. Some factors to consider (as per ESC guidelines):
- High risk of both clotting and bleeding. High risk of cerebral events and abscesses. Should receive prophylactic antibiotics for high risk dental procedures.
- Important to keep hydrated and avoid acute exposure to heat such as saunas, hot tubs etc. Avoid iron deficiency and anaemia, respiratory infections and agents that can lead to renal impairment.
- Avoid pregnancy, moderate to severe strenuous exercise and high altitude.
- Use air filters in IV lines to prevent air embolisms, as high risk of entering system.
Diagnosis of a congenital heart condition is based on the investigations outlined in the previous section and include the following conditions:
Patent Ductus Arteriosus (PDA): the ductus arteriosus does not close after birth, allowing blood to mix between the aorta and pulmonary artery.
Obstruction: a narrowing which affects the flow of blood:
- Pulmanory Stenosis (PS): the pulmonary valve is narrowed or obstructed
- Aortic Stenosis (AS): narrowing or obstruction of the aortic valve
- Coarctation of the Aorta: narrowing of aorta obstructs blood flow to the body
- Bicupsid Aortic Valve: where the aortic valve does not develop normally
- Subaortic Stenosis: narrowing of the left ventricle just below the aortic valve
- Ebstein’s Anomaly: the tricuspid valve between the the right atrium and right ventricle is in the wrong position
Hypoplastic Left Heart Syndrome: the left side of the heart is underdeveloped.
Septal: blood flows between the right and left chambers of the heart:
- Atrial Septal Defect (ASD): often known as “hole in the heart”, where the wall in the atria does not close completely
- Ventricular Septal Defect (VSD): an opening of the wall between the left and right ventricles
- Eisenmenger’s Complex: an opening of the wall between the left and right ventricles and increased blood flow to the lungs
- Atrioventricular Septal Defect (AVSD) / Atrioventricular canal defect: hole(s) in the atrial and ventricular septal
Cyanotic: blood pumped to the body has less oxygen causing the skin to discolour blue:
- Teralogy of Fallot (ToF): A hole between the left and right ventricles, thicker right ventricle, aorta connects to both left and right ventricle and a norrowing of the pulmonary valve
- Tricuspid Atresia: the tricuspid valve between the right atrium and right ventricle has not developed properly
- Pulmonary Atresia: the pulmonary valve has not developed properly
- Truncus Arteriosus (TA): there is only one, instead of two, blood vessels from the heart
- Total Anomalous Pulmanory Venous Connection (TAPVC): the pulmanory veins from the lungs attach to the wrong positions of the heart
- Transposition of the Great Arteries (TGA): the aorta and the pulmonary artery have swapped places
Endocarditis: inflammation of the lining of the heart.
Pulse point
Endocarditis prevention is very important in patients with congenital cardiac conditions.
The European Society of Cardiology (ESC) and the National Institute for Clinical Excellence (NICE) guidelines for endocarditis management have been updated in 2015. It is worth noting that the ESC now advocate prophylactic antibiotics for the highest risk patients (patients who have had previous IE: patients who have cyanotic CHD: and patients with prosthetic material in their heart) who are undergoing dental procedures that involve manipulation of the gingival or periapical region of the teeth, or perforation of the oral mucosa. NICE, however, do not advocate prophylaxic antibiotics. Patients must be educated on the importance of 6 monthly dental checks, maintaining good oral hygiene and avoiding piercings and tattoo’s.
