Among some of the symptoms consistent with pulmonary valve stenosis are the following:

- Heart murmur

- Cyanosis

- Dyspnea

- Dizziness

- Upper thorax pain

- Developmental disorders

In regards to the cause of pulmonary valve stenosis a very high percentage are congenital, the right ventricular flow is hindered (or obstructed by this). The cause in turn is divided into: valvular, external and intrinsic (when it is acquired).

A right ventricular outflow tract obstruction (RVOTO) may be due to a defect in the pulmonic valve, the supravalvar region, the infundibulum, or the pulmonary artery.

- Pulmonary atresia

- Pulmonary valve stenosis

- Hypoplastic right heart syndrome

- Tetralogy of Fallot

A left ventricular outflow tract obstruction (LVOTO) may be due to a defect in the aortic valve, or a defect located at the subvalvar or supravalvar level.

- Aortic valve stenosis

- Supravalvar aortic stenosis

- Coarctation of the aorta

- Hypoplastic left heart syndrome

A mild diastolic murmur can be heard during auscultation caused by the blood flow through the stenotic valve. It is best heard over the left sternal border with rumbling character and tricuspid opening snap with wide-splitting S1. It may increase in intensity with inspiration (Carvallo's sign). The diagnosis will typically be confirmed by an echocardiograph, which will also allow the physician to assess its severity.

Left to right shunting heart defects include:

- Ventricular septal defect (VSD) (30% of all congenital heart defects)

- Atrial septal defect (ASD)

- Atrioventricular septal defect (AVSD)

- Patent ductus arteriosus (PDA)

- Previously, Patent ductus arteriosus (PDA) was listed as acyanotic but in actuality it can be cyanotic due to pulmonary hypertension resulting from the high pressure aorta pumping blood into the pulmonary trunk, which then results in damage to the lungs which can then result in pulmonary hypertension as well as shunting of blood back to the right ventricle. This consequently results in less oxygenation of blood due to alveolar damage as well as oxygenated blood shunting back to the right side of the heart, not allowing the oxygenated blood to pass through the pulmonary vein and back to the left atrium.

- (Edit - this is called Eisenmenger's syndrome and can occur with Atrial septal defect and ventricular septal defect as well (actually more common in ASD and VSD) therefore PDA can still be listed as acyanotic as, acutely, it is)

Others:

- levo-Transposition of the great arteries (l-TGA)

Acyanotic heart defects without shunting include:

- Pulmonary stenosis (a narrowing of the pulmonary valve)

- Aortic stenosis

- Coarctation of the aorta

In mild cases, children may show no signs or symptoms at first and their condition may not be diagnosed until later in life. Some children born with coarctation of the aorta have other heart defects too, such as aortic stenosis, ventricular septal defect, patent ductus arteriosus or mitral valve abnormalities.

Coarctation is about twice as common in boys as it is in girls. It is common in girls who have Turner syndrome.

Symptoms may be absent with mild narrowings (coarctation). When present, they include: difficulty breathing, poor appetite or trouble feeding, failure to thrive. Later on, children may develop symptoms related to problems with blood flow and an enlarged heart. They may experience dizziness or shortness of breath, faint or near-fainting episodes, chest pain, abnormal tiredness or fatigue, headaches, or nosebleeds. They have cold legs and feet or have pain in their legs with exercise (intermittent claudication).

In more severe cases, where severe coarctations, babies may develop serious problems soon after birth because not enough blood can get through the aorta to the rest of their body.

Arterial hypertension in the arms with low blood pressure in the lower extremities is classic. In the lower extremities, weak pulses in the femoral arteries and arteries of the feet are found.

The coarctation typically occurs after the left subclavian artery. However, if situated before it, blood flow to the left arm is compromised and asynchronous or radial pulses of different "strength" may be detected (normal on the right arm, weak or delayed on the left), termed "radio-radial delay". In these cases, a difference between the normal radial pulse in the right arm and the delayed femoral pulse in the legs (either side) may be apparent, whilst no such delay would be appreciated with palpation of both delayed left arm and either femoral pulses. On the other hand, a coarctation occurring after the left subclavian artery will produce synchronous radial pulses, but "radio-femoral delay" will be present under palpation in either arm (both arm pulses are normal compared to the delayed leg pulses).

Pulmonary and tricuspid valve diseases are right heart diseases. Pulmonary valve diseases are the least common heart valve disease in adults.

Pulmonary valve stenosis is often the result of congenital malformations and is observed in isolation or as part of a larger pathologic process, as in Tetralogy of Fallot, Noonan syndrome, and congenital rubella syndrome . Unless the degree of stenosis is severe individuals with pulmonary stenosis usually have excellent outcomes and treatment options. Often patients do not require intervention until later in adulthood as a consequence of calcification that occurs with aging.

Pulmonary valve insufficiency occurs commonly in healthy individuals to a very mild extent and does not require intervention. More appreciable insufficiency it is typically the result of damage to the valve due to cardiac catheterization, aortic balloon pump insertion, or other surgical manipulations. Additionally, insufficiency may be the result of carcinoid syndrome, inflammatory processes such a rheumatoid disease or endocarditis, or congenital malformations. It may also be secondary to severe pulmonary hypertension.

Tricuspid valve stenosis without co-occurrent regurgitation is highly uncommon and typically the result of rheumatic disease. It may also be the result of congenital abnormalities, carcinoid syndrome, obstructive right atrial tumors (typically lipomas or myxomas), or hypereosinophilic syndromes.

Minor tricuspid insufficiency is common in healthy individuals. In more severe cases it is a consequence of dilation of the right ventricle, leading to displacement of the papillary muscles which control the valve's ability to close. Dilation of the right ventricle occurs secondary to ventricular septal defects, right to left shunting of blood, eisenmenger syndrome, hyperthyroidism, and pulmonary stenosis. Tricuspid insufficiency may also be the result of congenital defects of the tricuspid valve, such as Ebstein's anomaly.

Symptoms related to aortic stenosis depend on the degree of stenosis. Most people with mild to moderate aortic stenosis do not have symptoms. Symptoms usually present in individuals with severe aortic stenosis, though they may occur in those with mild to moderate aortic stenosis as well. The three main symptoms of aortic stenosis are loss of consciousness, anginal chest pain and shortness of breath with activity or other symptoms of heart failure such as shortness of breath while lying flat, episodes of shortness of breath at night, or swollen legs and feet. It may also be accompanied by the characteristic "Dresden china" appearance of pallor with a light flush.

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).

Signs and symptoms of mitral stenosis include the following:

- Heart failure symptoms, such as dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea (PND)

- Palpitations

- Chest pain

- Hemoptysis

- Thromboembolism in later stages when the left atrial volume is increased (i.e., dilation). The latter leads to increase risk of atrial fibrillation, which increases the risk of blood stasis (motionless). This increases the risk of coagulation.

- Ascites and edema and hepatomegaly (if right-side heart failure develops)

Fatigue and weakness increase with exercise and pregnancy.

The resulting syndrome depends on the structure affected.

Examples of vascular stenotic lesions include:

- Intermittent claudication (peripheral artery stenosis)

- Angina (coronary artery stenosis)

- Carotid artery stenosis which predispose to (strokes and transient ischaemic episodes)

- Renal artery stenosis

The types of stenoses in heart valves are:

- Pulmonary valve stenosis, which is the thickening of the pulmonary valve, therefore causing narrowing

- Mitral valve stenosis, which is the thickening of the mitral valve (of the left heart), therefore causing narrowing

- Tricuspid valve stenosis, which is the thickening of the tricuspid valve (of the right heart), therefore causing narrowing

- Aortic valve stenosis, which is the thickening of the aortic valve, therefore causing narrowing

Stenoses/strictures of other bodily structures/organs include:

- Pyloric stenosis (gastric outflow obstruction)

- Lumbar, cervical or thoracic spinal stenosis

- Subglottic stenosis (SGS)

- Tracheal stenosis

- Obstructive jaundice (biliary tract stenosis)

- Bowel obstruction

- Phimosis

- Non-communicating hydrocephalus

- Stenosing tenosynovitis

- Atherosclerosis

- Esophageal stricture

- Achalasia

- Prinzmetal angina

- Vaginal stenosis

When pulmonic stenosis (PS) is present, resistance to blood flow causes right ventricular hypertrophy. If right ventricular failure develops, right atrial pressure will increase, and this may result in a persistent opening of the foramen ovale, shunting of unoxygenated blood from the right atrium into the left atrium, and systemic cyanosis. If pulmonary stenosis is severe, congestive heart failure occurs, and systemic venous engorgement will be noted. An associated defect such as a patent ductus arteriosus partially compensates for the obstruction by shunting blood from the left ventricle to the aorta then back to the pulmonary artery (as a result of the higher pressure in the left ventricle) and back into the lungs.

There are three types of aortic coarctations:

1. Preductal coarctation: The narrowing is proximal to the ductus arteriosus. Blood flow to the aorta that is distal to the narrowing is dependent on the ductus arteriosus; therefore severe coarctation can be life-threatening. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. This is the type seen in approximately 5% of infants with Turner syndrome.

2. Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus. This kind usually appears when the ductus arteriosus closes.

3. Postductal coarctation: The narrowing is distal to the insertion of the ductus arteriosus. Even with an open ductus arteriosus, blood flow to the lower body can be impaired. This type is most common in adults. It is associated with notching of the ribs (because of collateral circulation), hypertension in the upper extremities, and weak pulses in the lower extremities. Postductal coarctation is most likely the result of the extension of a muscular artery (ductus arteriosus) into an elastic artery (aorta) during fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth subsequently narrows the aortic lumen.

Aortic coarctation and aortic stenosis are both forms of aortic narrowing. In terms of word root meanings, the names are not different, but a conventional distinction in their usage allows differentiation of clinical aspects. This spectrum is dichotomized by the idea that aortic coarctation occurs in the aortic arch, at or near the ductus arteriosis, whereas aortic stenosis occurs in the aortic root, at or near the aortic valve. This naturally could present the question of the dividing line between a postvalvular stenosis and a preductal coarctation; nonetheless, the dichotomy has practical use, as most defects are either one or the other.

d vessels can present a large variety of , and/or . The effects may range from a change in blood pressure to an interruption in circulation, depending on the nature and degree of the misplacement and which vessels are involved.

Although "transposed" literally means "swapped", many types of TGV involve vessels that are in abnormal positions, while not actually being swapped with each other. The terms TGV and TGA are most commonly used in reference to dextro-TGA – in which the arteries "are" in swapped positions; however, both terms are also commonly used, though to a slightly lesser extent, in reference to levo-TGA – in which both the arteries and the ventricles are swapped; while other defects in this category are almost never referred to by either of these terms.

In dextro-Transposition of the great arteries (dextro-TGA) deoxygenated blood from the right heart is pumped immediately through the aorta and circulated to the body and the heart itself, bypassing the lungs altogether, while the left heart pumps oxygenated blood continuously back into the lungs through the pulmonary artery. In effect, two separate "circular" (parallel) circulatory systems are created. It is called a cyanotic congenital heart defect (CHD) because the newborn infant turns blue from lack of oxygen.

Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth.

VSD is an acyanotic congenital heart defect, aka a left-to-right shunt, so there are no signs of cyanosis in the early stage. However, uncorrected VSD can increase pulmonary resistance leading to the reversal of the shunt and corresponding cyanosis.

- Pansystolic (Holosystolic) murmur along lower left sternal border (depending upon the size of the defect) +/- palpable thrill (palpable turbulence of blood flow). Heart sounds are normal. Larger VSDs may cause a parasternal heave, a displaced apex beat (the palpable heartbeat moves laterally over time, as the heart enlarges). An infant with a large VSD will fail to thrive and become sweaty and tachypnoeic (breathe faster) with feeds.

The restrictive VSDs (smaller defects) are associated with a louder murmur and more palpable thrill (grade IV murmur). Larger defects may eventually be associated with pulmonary hypertension due to the increased blood flow. Over time this may lead to an Eisenmenger's syndrome the original VSD operating with a left-to-right shunt, now becomes a right-to-left shunt because of the increased pressures in the pulmonary vascular bed.

An acyanotic heart defect, also known as non-cyanotic heart defect, is a class of congenital heart defects. In these, blood is shunted (flows) from the left side of the heart to the right side of the heart due to a structural defect (hole) in the interventricular septum. People often retain normal levels of oxyhemoglobin saturation in systemic circulation.

This term is outdated, because a person with an acyanotic heart defect may show cyanosis (turn blue due to insufficient oxygen in the blood).

Obstruction defects occur when heart valves, arteries, or veins are abnormally narrow or blocked. Common defects include pulmonic stenosis, aortic stenosis, and coarctation of the aorta, with other types such as bicuspid aortic valve stenosis and subaortic stenosis being comparatively rare. Any narrowing or blockage can cause heart enlargement or hypertension.

Stenoses of the vascular type are often associated with unusual blood sounds resulting from turbulent flow over the narrowed blood vessel. This sound can be made audible by a stethoscope, but diagnosis is generally made or confirmed with some form of medical imaging.

Children with tetralogy of Fallot may develop "tet spells". These are acute hypoxia spells, characterized by shortness of breath, cyanosis, agitation, and loss of consciousness. This may be initiated by any event leading to decreased oxygen saturation or that causes decreased systemic vascular resistance, leading to increased venous return, which in turn leads to increased shunting through the ventricular septal defect.

Tet spells are characterized by a sudden, marked increase in cyanosis followed by syncope, and may result in hypoxic brain injury and death.

Older children will often squat during a tet spell. This increases systemic vascular resistance and allows for a temporary reversal of the shunt. It increases pressure on the left side of the heart, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation.

Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the ventricular septal defect (VSD) and preferential flow of the mixed blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve. This is known as a right-to-left shunt. The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. If the baby is not cyanotic then it is sometimes referred to as a "pink tet". Other symptoms include a heart murmur which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia. The baby may turn blue with breast feeding or crying.

Angina in setting of heart failure also increases the risk of death. In people with angina, the 5-year mortality rate is 50% if the aortic valve is not replaced.

Angina in the setting of AS occurs due to left ventricular hypertrophy (LVH) that is caused by the constant production of increased pressure required to overcome the pressure gradient caused by the AS. While the muscular layer of the left ventricle thickens, the arteries that supply the muscle do not get significantly longer or bigger, so the muscle may not receive enough blood supply to meet its oxygen requirement. This ischemia may first be evident during exercise when the heart muscle requires increased blood supply to compensate for the increased workload. The individual may complain of anginal chest pain with exertion. At this stage, a cardiac stress test with imaging may be suggestive of ischemia.

Eventually, however, the heart muscle will require more blood supply at rest than can be supplied by the coronary artery branches. At this point there may be signs of "ventricular strain pattern" (ST segment depression and T wave inversion) on the EKG, suggesting subendocardial ischemia. The subendocardium is the region that is most susceptible to ischemia because it is the most distant from the epicardial coronary arteries.

Hypoplasia can affect the heart, typically resulting in the underdevelopment of the right ventricle or the left ventricle. This causes only one side of the heart to be capable of pumping blood to the body and lungs effectively. Hypoplasia of the heart is rare but is the most serious form of CHD. It is called hypoplastic left heart syndrome when it affects the left side of the heart and hypoplastic right heart syndrome when it affects the right side of the heart. In both conditions, the presence of a patent ductus arteriosus (and, when hypoplasia affects the right side of the heart, a patent foramen ovale) is vital to the infant's ability to survive until emergency heart surgery can be performed, since without these pathways blood cannot circulate to the body (or lungs, depending on which side of the heart is defective). Hypoplasia of the heart is generally a cyanotic heart defect.

Major symptoms of Lutembacher's syndrome as a result of ASD and MS can range from heart failure to pulmonary congestion.

- Right ventricular overload and Right-sided heart failure: Both are caused by a large ASD and MS (moderate to severe).

- Palpitations: This is caused by blood flowing from left atrium to the right atrium causing a higher left atrial pressure and leading to mitral stenosis. Both atria will be dilated (stretched or open)leading to future atrial arrhythmias or atrial fibrillation (Riaz).

- Pulmonary congestion: When blood or fluid pools within the lungs; this is usually a symptom of mitral stenosis and a small ASD.

- Loud mitral S1 and wide fixed split of pulmonary S2: The loud sound of the mitral S1 and the wide fixed split of pulmonary S2 is a symptoms of mitral stenosis. The sounds often are caused by a reduced pressure gradient in the mitral area that was caused from decompression of the left atrium from the ASD and a displacement (moving from normal position) of the left ventricular lower portion of the heart to the a large right ventricle. The second heart sound (S2) split is caused by the increase right heart blood flow through the ASD causing a late closing of the pulmonary component of the S2 as well as decreased left ventricular and aortic blood flow.

- III/IV mid diastolic murmur, early systolic murmur: This heart murmur is caused by an increase blood flow through the tricuspid valve due to ASD; it is heard best in the left lower sternal area or the bottom of the heart (apex).