For newborns with transposition, prostaglandins can be given to keep the ductus arteriosus open which allows mixing of the otherwise isolated pulmonary and systemic circuits. Thus oxygenated blood that recirculates back to the lungs can mix with blood that circulates throughout the body. The arterial switch operation is the definitive treatment for dextro- transposition. Rarely the arterial switch is not feasible due to particular coronary artery anatomy and an atrial switch operation is preferred.

The effect of statins on the progression of AS is unclear. The latest trials do not show any benefit in slowing AS progression, but did demonstrate a decrease in ischemic cardiovascular events.

In general, medical therapy has relatively poor efficacy in treating aortic stenosis. However, it may be useful to manage commonly coexisting conditions that correlate with aortic stenosis:

- Any angina is generally treated with beta-blockers and/or calcium blockers. Nitrates are contraindicated due to their potential to cause profound hypotension in aortic stenosis.

- Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers.

- Any heart failure is generally treated with digoxin and diuretics, and, if not contraindicated, cautious administration of ACE inhibitors.

While observational studies demonstrated an association between lowered cholesterol with statins and decreased progression, a randomized clinical trial published in 2005 failed to find any effect on calcific aortic stenosis. A 2007 study did demonstrate a slowing of aortic stenosis with the statin rosuvastatin.

In adults and children found to have coarctation, treatment is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 1944. In some cases angioplasty can be performed to dilate the narrowed artery, with or without the placement of a stent graft.

For fetuses at high risk for developing coarctation, a novel experimental treatment approach is being investigated, wherein the mother inhales 45% oxygen three times a day (3 x 3–4 hours) beyond 34 weeks of gestation. The oxygen is transferred via the placenta to the fetus and results in dilatation of the fetal lung vessels. As a consequence, the flow of blood through the fetal circulatory system increases, including that through the underdeveloped arch. In suitable fetuses, marked increases in aortic arch dimensions have been observed over treatment periods of about two to three weeks.

The long term outcome is very good. Some patients may, however, develop narrowing (stenosis) or dilatation at the previous coarctation site. All patients with unrepaired or repaired aortic coarctation require follow up in specialized Congenital Heart Disease centers.

Globally more than 250,000 people have received transcatheter aortic valve replacement (TAVR). For people who are not candidates for surgical valve replacement and most patients who are older than 75, TAVR may be a suitable alternative.

In treating pulmonary insufficiency, it should be determined if pulmonary hypertension is causing the problem to therefore begin the most appropriate therapy as soon as possible (primary pulmonary hypertension or secondary pulmonary hypertension due to thromboembolism). Furthermore, pulmonary insufficiency is generally treated by addressing the underlying condition, in certain cases, the pulmonary valve may be surgically replaced.

The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population.

Surgical treatment involves resection of the stenosed segment and re-anastomsis. Two complications specific to this surgery are Left recurrent nerve palsy and chylothorax, as the recurrent laryngeal nerve and thoracic duct are in the vicinity. Chylothorax is a troublesome complication and is usually managed conservatively by adjusting the diet to eliminate long chain fatty acids and supplementing medium chain triglycerides. When conservative management fails surgical intervention is required. Fluorescein dye can aid in the localisation of chyle leak.

The treatment of choice is percutaneous balloon valvuloplasty and is done when a resting peak gradient is seen to be >60mm Hg or a mean >40mm Hg is observed.

In terms of treatment for pulmonary valve stenosis, valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case.

Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used.

Since 1981 Lecompte has put his Lecompte manoeuvre in use. This is used with the REV (Réparation à l'Etage Ventriculaire). This surgery is like the Rastelli procedure, but with the use of the pulmonary artery without a conduit.

It was Bex who introduced in 1980 the possibility of aortic translocation. But Nikaidoh has put the procedure in practice in 1984. It results in an anatomical normal heart, even better than with an ASO, because also the cones are switched instead of only the arteries as with an ASO.

It has as contra-indication coronary anomalies.

The following table includes the main types of valvular stenosis and regurgitation. Major types of valvular heart disease not included in the table include mitral valve prolapse, rheumatic heart disease and endocarditis.

Simple l-TGA has a very good prognosis, with many individuals being asymptomatic and not requiring surgical correction.

In a number of cases, the (technically challenging) "double switch operation" has been successfully performed to restore the normal blood flow through the ventricles.

Treatment is not necessary in asymptomatic patients.

The treatment options for mitral stenosis include medical management, mitral valve replacement by surgery, and percutaneous mitral valvuloplasty by balloon catheter.

The indication for invasive treatment with either a mitral valve replacement or valvuloplasty is NYHA functional class III or IV symptoms.

Another option is balloon dilatation. To determine which patients would benefit from percutaneous balloon mitral valvuloplasty, a scoring system has been developed. Scoring is based on 4 echocardiographic criteria: leaflet mobility, leaflet thickening, subvalvar thickening, and calcification. Individuals with a score of ≥ 8 tended to have suboptimal results. Superb results with valvotomy are seen in individuals with a crisp opening snap, score < 8, and no calcium in the commissures.

Treatment also focuses on concomitant conditions often seen in mitral stenosis:

- Any angina is treated with short-acting nitrovasodilators, beta-blockers and/or calcium blockers

- Any hypertension is treated aggressively, but caution must be taken in administering beta-blockers

- Any heart failure is treated with digoxin, diuretics, nitrovasodilators and, if not contraindicated, cautious inpatient administration of ACE inhibitors

Preexisting diabetes mellitus of a pregnant mother is a risk factor that has been described for the fetus having TGV.

In the first stage of restenosis, administering anti-platelet drugs (called IIb/IIIa inhibitors) immediately after surgery greatly reduces the chance of a thrombosis occurring.

Drug-eluting stents are now being trialled in Europe, Canada and the USA, as well as in Asia-Pacific. These stents are coated with pharmaceuticals that inhibit tissue growth and thus reduce the risk of restenosis from scar-tissue and cell proliferation.

There has been some success with these new stents in reducing the occurrence of restenosis, with clinical studies showing an incidence rate of 5% or lower.

The Norwood procedure is a procedure to correct fetal aortic stenosis that occurs after birth. This typically consists of three surgeries creating and removing shunts. The atrial septum is removed, the aortic arch is reconstructed to remove any hypoplasia, and then the main pulmonary artery is connected into this reconstructed arch, resulting in the right ventricle ejecting directly into systemic circulation. In the end, the right ventricle is pumping blood to systemic circulation and to the lungs. However, this procedure carries a very high risk of failure and the patient will likely require a heart transplant.

Another treatment option is to correct the stenosis in utero. In this procedure, fetal positioning is crucial. It is important that the left chest is located anteriorly, and that there are no limbs between the uterine wall and the apex of the left ventricle. The LV apex needs to be within 9 cm of the abdominal wall and the left ventricle outflow track has to be parallel to the intended cannula course in order for the wire to be blindly directed at the aortic valve. A 11.5 cm long, 19-gauge cannula and stylet needle passes through the mother’s abdomen, uterine wall, and fetal chest wall into the left ventricle of the fetus. Then a 0.014 inch guide wire is passed across the stenosis aortic valve, where a balloon is inflated to stretch the aortic annulus.

An alternative to the Norwood procedure is known as the hybrid procedure, was developed in 2008. In the hybrid procedure, bilateral pulmonary artery bands are positioned to limit pulmonary flow while, at the same time, placing a stent in the ductus arteriosus to hold it open. This maintains the connection between the aorta and the systemic circulation. A balloon atrial septostomy is also done. This ensures that there is enough of a connection between the two atria of the heart to provide open blood flow and mixing of oxygen rich and poor blood This procedure spares the baby from undergoing open heart surgery until they are older. They typically come back at 4–6 months of age when they are stronger for the open heart surgery.

Stenosis of the pulmonary artery is a condition where the pulmonary artery is subject to an abnormal constriction (or stenosis). Peripheral pulmonary artery stenosis may occur as an isolated event or in association with Alagille syndrome, Berardinelli-Seip congenital lipodystrophy type 1, Costello syndrome, Keutel syndrome, nasodigitoacoustic syndrome (Keipert syndrome), Noonan syndrome or Williams syndrome.

It should not be confused with a pulmonary valve stenosis, which is in the heart, but can have similar hemodynamic effects. Both stenosis of the pulmonary artery and pulmonary valve stenosis are causes of pulmonic stenosis.

In some cases it is treated with surgery.

Mitral valvuloplasty is a minimally invasive therapeutic procedure to correct an uncomplicated mitral stenosis by dilating the valve using a balloon.

Under local anaesthetic, a catheter with a special balloon is passed from the right femoral vein, up the inferior vena cava and into the right atrium. The interatrial septum is punctured and the catheter passed into the left atrium using a "trans-septal technique." The balloon is sub-divided into 3 segments and is dilated in 3 stages. First, the distal portion (lying in the left ventricle) is inflated and pulled against the valve cusps. Second, the proximal portion is dilated, in order to fix the centre segment at the valve orifice. Finally, the central section is inflated, this should take no longer than 30 seconds, since full inflation obstructs the valve and causes congestion, leading to circulatory arrest and flash pulmonary edema.

With careful patient pre-selection, percutaneous balloon mitral valvuloplasty (PBMV) is associated with good success rates and a low rate of complications. By far the most serious adverse event is the occurrence of acute severe mitral regurgitation. Severe mitral regurgitation usually results from a tear in one of the valve leaflets or the subvalvular apparatus. It can lead to pulmonary edema and hemodynamic compromise, necessitating urgent surgical mitral valve replacement.

Other serious complications with PBMV usually relate to the technique of trans-septal puncture (TSP). The ideal site for TSP is the region of the fossa ovalis in the inter-atrial septum. Occasionally, however, the sharp needle used for TSP may inadvertently traumatize other cardiac structures, leading to cardiac tamponade or serious blood loss.

Although the immediate results of PBMV are often quite gratifying, the procedure does not provide permanent relief from mitral stenosis. Regular follow-up is mandatory, to detect restenosis. Long-term follow-up data from patients undergoing PBMV indicates that up to 70-75% individuals can be free of restenosis 10 years following the procedure. The number falls to about 40% 15 years post-PBMV.

Pulmonic stenosis, also known as pulmonary stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood.

Pulmonic stenosis is usually due to isolated valvular obstruction (pulmonary valve stenosis), but it may be due to subvalvular or supravalvular obstruction, such as infundibular stenosis. It may occur in association with other congenital heart defects as part of more complicated syndromes (for example, tetralogy of Fallot).

If restenosis occurs without a stent, it is usually treated with more angioplasty. Once restenosis has occurred and been treated by angioplasty, the chances of restenosis occurring again are increased by a factor of 2. This treatment is also used if restenosis occurs at either the proximal or distal end of the stent.

If restenosis occurs within a stent (also known as in-stent stenosis), it may be treated with repeated angioplasty and insertion of another stent inside the original, sometimes with a drug-eluting stent.

Over the past 5 years, ISR is preferentially treated with a drug eluting balloon, which is a balloon coated with the same anticancer drugs that prevent restenosis. The Balloon avoids the need for a double layer of metal which is used when an in-stent restenosis is treated with another stent within the original stent

Alternative treatments include brachytherapy, or intracoronary radiation. The radiation kills cells and inhibits tissue growth (similar to a patient undergoing cancer therapy).

Tet spells may be treated with beta-blockers such as propranolol, but acute episodes require rapid intervention with morphine or intranasal fentanyl to reduce ventilatory drive, a vasopressor such as phenylephrine, or norepinephrine to increase systemic vascular resistance, and IV fluids for volume expansion.

Oxygen (100%) may be effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs by decreasing shunting of deoxygenated blood from the right to left ventricle through the VSD. There are also simple procedures such as squatting and the knee chest position which increase systemic vascular resistance and decrease right-to-left shunting of deoxygenated blood into the systemic circulation.

Treatment is with neonatal surgical repair, with the objective of restoring a normal pattern of blood flow. The surgery is open heart, and the patient will be placed on cardiopulmonary bypass to allow the surgeon to work on a still heart. The heart is opened and the ventricular septal defect is closed with a patch. The pulmonary arteries are then detached from the common artery (truncus arteriosus) and connected to the right ventricle using a tube (a conduit or tunnel). The common artery, now separated from the pulmonary circulation, functions as the aorta with the truncal valve operating as the aortic valve. Most babies survive this surgical repair, but may require further surgery as they grow up. For example, the conduit does not grow with the child and may need to be replaced as the child grows. Furthermore, the truncal valve is often abnormal and may require future surgery to improve its function.

There have been cases where the condition has been diagnosed at birth and surgical intervention is an option. A number of these cases have survived well into adulthood.

The condition was initially thought untreatable until surgeon Alfred Blalock, cardiologist Helen B. Taussig, and lab assistant Vivien Thomas at Johns Hopkins University developed a palliative surgical procedure, which involved forming a side to end anastomosis between the subclavian artery and the pulmonary artery. This first surgery was depicted in the film "Something the Lord Made". It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month-old Eileen Saxon on November 29, 1944 with dramatic results.

The Potts shunt and the Waterston-Cooley shunt are other shunt procedures which were developed for the same purpose. These are no longer used.

Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia (pseudotruncus arteriosus).

Inflammation of the heart valves due to any cause is called valvular endocarditis; this is usually due to bacterial infection but may also be due to cancer (marantic endocarditis), certain autoimmune conditions (Libman-Sacks endocarditis, seen in systemic lupus erythematosus) and hypereosinophilic syndrome (Loeffler endocarditis). Certain medications have been associated with valvular heart disease, most prominently ergotamine derivatives pergolide and cabergoline.

Valvular heart disease resulting from rheumatic fever is referred to as "rheumatic heart disease". Damage to the heart valves follows infection with beta-hemolytic bacteria, such as typically of the respiratory tract. Pathogenesis is dependent on cross reaction of M proteins produced by bacteria with the myocardium. This results in generalized inflammation in the heart, this manifests in the mitral valve as vegetations, and thickening or fusion of the leaflets, leading to a severely compromised buttonhole valve.

Rheumatic heart disease typically only involves the mitral valve (70% of cases), though in some cases the aortic and mitral valves are both involved (25%). Involvement of other heart valves without damage to the mitral are exceedingly rare.

While developed countries once had a significant burden of rheumatic fever and rheumatic heart disease, medical advances and improved social conditions have dramatically reduced their incidence. Many developing countries, as well as indigenous populations within developed countries, still carry a significant burden of rheumatic fever and rheumatic heart disease and there has been a resurgence in efforts to eradicate the diseases in these populations.