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.

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

A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.

The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children in the United States. It is also the type that will most commonly require surgical intervention, comprising over 80% of cases.

Membranous ventricular septal defects are more common than muscular ventricular septal defects, and are the most common congenital cardiac anomaly.

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).

There are numerous types, differentiated by the extent of the defect. These types are:

- Type I: simple defects leading to communication between the ascending aorta and pulmonic trunk

- Type II: defects that extend to the origin of the right pulmonary artery

- Type III: anomalous origin of the right pulmonary artery from the ascending aorta

It is also classified as simple or complex. Simple defects are those that do not require surgical repair, occur with no other defects, or those that require minor stright-forward repair (ductus arteriosus, atrial septal defect). Complex defects are those that occur with other anatomical anomalies or require non-standard repair.

Aortopulmonary septal defect is a rare congenital heart disorder accounting for only 0.1-0.3% of congenital heart defects worldwide. It is characterized by a communication between the aortic and pulmonary arteries, with preservation of two normal semilunar valves. It is the result of an incomplete separation of the aorticopulmonary trunk that normally occurs in early fetal development with formation of the spiral septum. Aortopulmonary septal defects occur in isolation in about half of cases, the remainder are associated with more complex heart abnormalities.

The clinical course of HCM is variable. Many people with HCM are asymptomatic or mildly symptomatic, and many of those carrying disease genes for HCM do not have clinically detectable disease. The symptoms and signs of HCM include shortness of breath due to stiffening and decreased blood filling of the ventricles, exertional chest pain (sometimes known as angina) due to reduced blood flow to the coronary arteries, uncomfortable awareness of the heart beat (palpitations), as well as disruption of the electrical system running through the abnormal heart muscle, lightheadedness, weakness, fainting and sudden cardiac death.

Dyspnea is largely due to increased stiffness of the left ventricle (LV), which impairs filling of the ventricles, but also leads to elevated pressure in the left ventricle and left atrium, causing back pressure and interstitial congestion in the lungs. Symptoms are not closely related to the presence or severity of an outflow tract gradient. Often, symptoms mimic those of congestive heart failure (esp. activity intolerance and dyspnea), but treatment of each is different. Beta blockers are used in both cases, but treatment with diuretics, a mainstay of CHF treatment, will exacerbate symptoms in hypertrophic obstructive cardiomyopathy by decreasing ventricular preload volume and thereby increasing outflow resistance (less blood to push aside the thickened obstructing tissue).

Major risk factors for sudden death in individuals with HCM include prior history of cardiac arrest or ventricular fibrillation, spontaneous sustained ventricular tachycardia, family history of premature sudden death, unexplained syncope, LV thickness greater than or equal to 30 mm, abnormal exercise blood pressure and nonsustained ventricular tachycardia.

Ventricular inversion, also known as atrioventricular discordance, is a condition in which the anatomic right ventricle of the heart is on the left side of the interventricular septum and the anatomic left ventricle is on the right.

Upon cardiac catheterization, catheters can be placed in the left ventricle and the ascending aorta, to measure the pressure difference between these structures. In normal individuals, during ventricular systole, the pressure in the ascending aorta and the left ventricle will equalize, and the aortic valve is open. In individuals with aortic stenosis or with HCM with an outflow tract gradient, there will be a pressure gradient (difference) between the left ventricle and the aorta, with the left ventricular pressure higher than the aortic pressure. This gradient represents the degree of obstruction that has to be overcome in order to eject blood from the left ventricle.

The Brockenbrough–Braunwald–Morrow sign is observed in individuals with HCM with outflow tract gradient. This sign can be used to differentiate HCM from aortic stenosis. In individuals with aortic stenosis, after a premature ventricular contraction (PVC), the following ventricular contraction will be more forceful, and the pressure generated in the left ventricle will be higher. Because of the fixed obstruction that the stenotic aortic valve represents, the post-PVC ascending aortic pressure will increase as well. In individuals with HCM, however, the degree of obstruction will increase more than the force of contraction will increase in the post-PVC beat. The result of this is that the left ventricular pressure increases and the ascending aortic pressure "decreases", with an increase in the LVOT gradient.

While the Brockenbrough–Braunwald–Morrow sign is most dramatically demonstrated using simultaneous intra-cardiac and intra-aortic catheters, it can be seen on routine physical examination as a decrease in the pulse pressure in the post-PVC beat in individuals with HCM.

In cardiology, Ventricular dyssynchrony is a difference in the timing, or lack of synchrony, of contractions in different ventricles in the heart. Large differences in timing of contractions can reduce cardiac efficiency and is correlated with heart failure.

Three chief presentations of dyssynchrony can occur. Atrioventricular (AV) dyssynchrony occurs when there is an unfavorable difference in timing between atrial and ventricular contractions. Interventricular dyssynchrony occurs when there is a difference in timing between right ventricular (RV) and left ventricular (LV) Systole. Intraventricular dyssynchrony occurs when the timing in a sequence of activations and contractions of segments of the LV wall becomes abnormal. In all three types, changes in timing lead to changes in the dynamic behavior of the myocardial tissues, leading to "mechanical dyssynchrony". All three presentations allow distinct and easily reproducible electrical signatures as illustrated by left and right bundle branch blocks, hemiblocks, etc. The concise measurement of the time and morphology of the QRS interval allows the interventional ability to manipulate this interval with biventricular pacemakers.

It is important to distinguish ventricular dyssynchrony from ventricular "dyssynergy". Dyssynergy refers to changes in relative strength of contractions, whereas dyssynchrony is a change in relative timing of contractions. Once again the terms inotropy and chronotropy apply to the pathology under examination. Biventricular pacing strongly favors chronotropy over inotropy.

Cardiac fibroma is a slow-growing tumor that can cause heart electrical transmission defects and arrhythmias. Some features may be seen in the ventricle wall separating the right and left lower chambers or the ventricle muscle. This tumor is rarely seen in atrial locations. Cardiac fibromas are mostly single and well-circumscribed and the average size of the tumor is circular and is 5 cm. Sometimes signs and symptoms are difficult to find in 35% of individuals. Situations like this, the tumor is incidentally diagnosed during a health checkup for other medical conditions. An individual may have abnormal heart sounds, such as a heart murmur.

In 65% of individuals, signs and symptoms are more obvious due to the large size of the tumor. Also, there is blood flow obstruction, especially into or out of the valves. The valves function becomes affected, which leads to heart failure. An individual might experience bluish skin (cyanosis), severe arrhythmias, dizziness, fainting, and other obstructive symptoms may be present.

Parasystole is a kind of arrhythmia caused by the presence and function of a secondary pacemaker in the heart, which works in parallel with the SA node. Parasystolic pacemakers are protected from depolarization by the SA node by some kind of "entrance block". This block can be complete or incomplete.

Parasystolic pacemakers can exist in both the atrium or the ventricle. Atrial parasystolia are characterized by narrow QRS complexes

Two forms of ventricular parasystole have been described in the literature, fixed parasystole and modulated parasystole. Fixed ventricular parasystole occurs when an ectopic pacemaker is protected by entrance block, and thus its activity is completely independent from the sinus pacemaker activity. Hence, the ectopic pacemaker is expected to fire at a fixed rate.

Therefore, on ECG, the coupling intervals of the manifest ectopic beats will wander through the basic cycle of the sinus rhythm. Accordingly, the traditional electrocardiographic criteria used to recognize the fixed form of parasystole are:

- the presence of variable coupling intervals of the manifest ectopic beats;

- inter-ectopic intervals that are simple multiples of a common denominator;

- fusion beats.

According to the modulated parasystole hypothesis, rigid constancy of a pacemaker might be expected if the entrance block were complete, but if there is an escape route available for the emergence of ectopic activity, then clearly there must be an effective ionic communication, not complete insulation, between the two tissues. If there is an electrical

communication between the two, then the depolarization of the surrounding ventricle may influence the ectopic pacemaker. That influence will be electrotonic; depolarization of the surrounding field will induce a partial depolarization

of the pacemaker cells. Therefore, appropriate diagnosis of modulated parasystole relies upon the construction of a “phase response curve” as theoretical evidence of modulation of the ectopic pacemaker cycle length by the electrotonic activity generated by the sinus discharges across the area of protection. In this case, the timing of the arrival of the electronic stimulus will serve to delay or advance the subsequent pacemaker activation. In this case, the coupling intervals between the manifest ectopic and sinus discharges will be either fixed or variable, depending on the cycle length relations between the two pacemakers.

Symptoms of myocardial rupture are recurrent or persistent chest pain, syncope, and distension of jugular vein. Sudden death caused by a myocardial rupture is sometimes preceded by no symptoms.

Cardiac fibroma, also known as cardiac fibromatosis, is a rare benign tumor of the heart that occurs primarily in infants and children. Benign tumors are typically a solitary, firm grey-white, non-encapsulated tumor that is composed of fibrous and dense connective tissue. It is most commonly located in the interventricular septum or left ventricular wall. Symptoms depend on the size of the tumor, its location relative to the conduction system, and whether it obstructs blood flow. Two-thirds of children with this tumor are asymptomatic, showing no signs and symptoms. Symptomatic cardiac fibromas may be treated by surgical resection. It is associated with Gorlin syndrome. Benign cardiac tumors are rare, 75% are histologically benign. Cardiac fibromas only occur 4-6%, which is less common compared to myxomas (75%) and rhabdomymoas (5-10%).

The diagnosis of these tumors require physical checkups, imaging studies on the heart, and specialized tests to evaluate the heart. Cardiac fibroma is considered a congenital tumor where an ultrasound prenatal scan may help detect during fetal stage. Surgery is the best treatment for an individual with cardiac fibroma. During this surgery, the tumor is completely removed by the surgeon. The overall prognosis is very good with a surgical removal. There have been 200 cases of cardiac fibroma recorded in the medical literature. Risk factors are still unidentified, but 1 in 30 individuals with Gorlin syndrome are known to be present with cardiac fibroma.

Myocardial rupture is a laceration or t

e ventricles or atria of the heart, of the interatrial or interventricular septum, or of the papillary muscles. It is most commonly seen as a serious sequela of an acute myocardial infarction (heart attack).

It can also be caused by trauma.

Onset may be rapid or more gradual. Symptoms typically include those of cardiogenic shock including shortness of breath, weakness, lightheadedness, and cough. Other symptoms may relate to the underlying cause.

An electrocardiogram helps establishing the exact diagnosis and guides treatment, it may reveal:

- Abnormal heart rhythms, such as bradycardia (slowed heart rate)

- myocardial infarction (ST-elevation MI, STEMI, is usually more dangerous than non-STEMIs; MIs that affect the ventricles are usually more dangerous than those that affect the atria; those affecting the left side of the heart, especially the left ventricle, are usually more dangerous than those affecting the right side, unless that side is severely compromised)

- Signs of cardiomyopathy

Cardiac tamponade, also known as pericardial tamponade, is when fluid in the pericardium (the sac around the heart) builds up and results in compression of the heart. Onset may be rapid or more gradual. Symptoms typically include those of cardiogenic shock including shortness of breath, weakness, lightheadedness, and cough. Other symptoms may relate to the underlying cause.

Common causes include cancer, kidney failure, chest trauma, and pericarditis. Other causes include connective tissue diseases, hypothyroidism, aortic rupture, and following cardiac surgery. In Africa, tuberculosis is a relatively common cause.

Diagnosis may be suspected based on low blood pressure, jugular venous distension, pericardial rub, or quiet heart sounds. The diagnosis may be further supported by specific electrocardiogram (ECG) changes, chest X-ray, or an ultrasound of the heart. If fluid increases slowly the pericardial sac can expand to contain more than 2 liters; however, if the increase is rapid as little as 200 mL can result in tamponade.

When tamponade results in symptoms, drainage is necessary. This can be done by pericardiocentesis, surgery to create a pericardial window, or a pericardiectomy. Drainage may also be necessary to rule out infection or cancer. Other treatments may include the use of dobutamine or in those with low blood volume, intravenous fluids. Those with few symptoms and no worrisome features can often be closely followed. The frequency of tamponade is unclear. One estimate from the United States places it at 2 per 10,000 per year.

Echocardiography may show poor ventricular function, signs of PED, rupture of the interventricular septum, an obstructed outflow tract or cardiomyopathy.

The classical triad of symptoms that defines 3C syndrome includes certain heart defects, hypoplasia (underdevelopment) of the cerebellum, and cranial dysmorphisms, which can take various forms. The heart defects and cranial dysmorphisms are heterogeneous in individuals who are all classed as having Ritscher-Schinzel syndrome.

Heart defects commonly seen with Ritscher-Schinzel syndrome are associated with the endocardial cushion and are the most important factor in determining a diagnosis. The mitral valve and tricuspid valve of the heart can be malformed, the atrioventricular canal can be complete instead of developing into the interatrial septum and interventricular septum, and conotruncal heart defects, which include tetralogy of Fallot, double outlet right ventricle, transposition of the great vessels, and hypoplastic left heart syndrome. Aortic stenosis and pulmonary stenosis have also been associated with 3C syndrome.

The cranial dysmorphisms associated with 3C syndrome are heterogeneous and include a degree of macrocephaly, a large anterior fontanel, a particularly prominent occiput and forehead, ocular hypertelorism (wide-set eyes), slanted palpebral fissures, cleft palate, a depressed nasal bridge, cleft palate with associated bifid uvula, low-set ears, micrognathia (an abnormally small jaw), brachycephaly (flattened head), and ocular coloboma. Low-set ears are the most common cranial dysmorphism seen in 3C syndrome, and ocular coloboma is the least common of the non-concurrent symptoms (cleft lip co-occurring with cleft palate is the least common).

Cranial dysplasias associated with 3C syndrome are also reflected in the brain. Besides the cerebellar hypoplasia, cysts are commonly found in the posterior cranial fossa, the ventricles and the cisterna magna are dilated/enlarged, and Dandy-Walker malformation is present. These are reflected in the developmental delays typical of the disease. 75% of children with 3C syndrome have Dandy-Walker malformation and hydrocephalus.

Signs and symptoms in other body systems are also associated with 3C syndrome. In the skeletal system, ribs may be absent, and hemivertebrae, syndactyly (fusion of fingers together), and clinodactyly (curvature of the fifth finger) may be present. In the GI and genitourinary systems, anal atresia, hypospadia (misplaced urethra), and hydronephrosis may exist. Adrenal hypoplasia and growth hormone deficiency are associated endocrine consequences of Ritscher-Schinzel syndrome. Some immunodeficiency has also been reported in connection with 3C syndrome.

Many children with the disorder die as infants due to severe congenital heart disease. The proband of Ritscher and Schinzel's original study was still alive at the age of 21.

A fetus with 3C syndrome may have an umbilical cord with one umbilical artery instead of two.

Constrictive pericarditis is a medical condition characterized by a thickened, fibrotic pericardium, limiting the heart's ability to function normally. In many cases, the condition continues to be difficult to diagnose and therefore benefits from a good understanding of the underlying cause.

Signs and symptoms of constrictive pericarditis are consistent with the following: fatigue, swollen abdomen, difficulty breathing (dyspnea), swelling of legs and general weakness. Related conditions are bacterial pericarditis, pericarditis and pericarditis after a heart attack.

Sinus pericranii typically present as soft palpable masses along midline skull, which may fluctuate in size depending on body positioning. Classically, these lesions are not associated with color change of the overlying skin, such as with other vascular lesions such as hemangioma.

3C syndrome, also known as CCC dysplasia, Craniocerebellocardiac dysplasia or Ritscher–Schinzel syndrome, is a rare condition, whose symptoms include heart defects, cerebellar hypoplasia, and cranial dysmorphism. It was first described in the medical literature in 1987 by Ritscher and Schinzel, for whom the disorder is sometimes named.