Table of contents :

Epidemiology : in 2002 62 million Americans (32 million females and 30 million males > 20%) - had a cardiovascular disease (including hypertension). The prevalence rises progressively with age from 5% at age 20 to 75% at age > 75 years. 8% or 22 million adults in the US have heart disease. In the US, the prevalence rate for those who have angina pectoris is 17.5 per 1000 people
Cardiovascular diseases (CVD) represent the first leading cause of death in Westernized countries, accounting for 45-50% of all deaths: 35% are coronary artery diseases (CAD). Contradicting conventional wisdom, the largest-ever worldwide collaborative study of heart disease has found that women are slightly more likely to die from CVD than men and that heart attacks and stroke kill twice as many women as all cancers combined. Out of the total 16.5 million CVD deaths annually, 8.6 million are of women. Although > 80% of the global burden of cardiovascular disease occurs in low-income and middle-income countries, knowledge of the importance of risk factors is largely derived from developed countries. Therefore, the effect of such factors on risk of coronary heart disease in most regions of the world is unknown.
Risk factors significantly related to acute myocardial infarction (p<0.0001 for all risk factors and p=0.03 for alcohol) include :

• smoking (odds ratio 2.87 for current vs never, PAR 35.7% for current and former vs never)
• raised ApoB/ApoA1 ratio (3.25 for top vs lowest quintile, PAR 49.2% for top four quintiles vs lowest quintile)
• history of hypertension (1.91, PAR 17.9%)
• diabetes mellitus (2.37, PAR 9.9%)
• abdominal obesity (1.12 for top vs lowest tertile and 1.62 for middle vs lowest tertile, PAR 20.1% for top 2 tertiles vs lowest tertile)
• psychosocial factors (2.67, PAR 32.5%)
• daily consumption of fruits and vegetables (0.70, PAR 13.7% for lack of daily consumption)
• regular alcohol consumption (0.91, PAR 6.7%)
• regular physical activity (0.86, PAR 12.2%)

• family history of cardiovascular accident in ancestor females before age 40 and males before age 50

Heart diseases

• Web resources :
• Heart disease at CDC
• Journals
• Texas Heart Institute Journal [free at PubMed Central (PMC); archive starts with Vol. 27(1); 2000]

• congenital heart diseases / cardiopathies

Epidemiology : prevalence = 0.8% of all (alive) newborns (1 per 200).
Aetiology :
• unknown (85%)
• intrinsic aetiology
• chromosome alterations (6%)
• single gene mutations (4%)
• Holt-Oram syndrome (HOS) / heart-hand syndrome : autosomal heart disease of varying severity, usually an atrial or ventricular septal defect, associated with skeletal malformation (hypoplastic thumb and short forearm)
• Noonan syndrome
• Kartagener syndrome
• malformations of the septum, outflow tract and aortic arch are the most common congenital cardiovascular defects and occur in mice lacking Cited2, a transcriptional coactivator of TFAP2. Cited2^-/- mice also develop laterality defects, including right isomerism, abnormal cardiac looping and hyposplenia, which are suppressed on a mixed genetic background. Cited2^-/- mice lack expression of the Nodal target genes Pitx2c, Nodal and Ebafin the left lateral plate mesoderm, where they are required for establishing laterality and cardiovascular development. CITED2 and TFAP2 were detected at the Pitx2c promoter in embryonic hearts, and they activate Pitx2c transcription in transient transfection assays. An abnormal Nodal-Pitx2c pathway represents a unifying mechanism for the cardiovascular malformations observed in Cited2^-/- mice, and that such malformations may be the sole manifestation of a laterality defect^ref.
• extrinsic aetiology (< 5%)
• rubella virus (vaccine for women not infected yet)
• thalidomide(once used to treat gravidic emesis)
• ethanol
• Li
• diabetes mellitus
Perloff's classification according to the following criteria :
• presence or absence of cyanosis
• normal, increased or decreased pulmonary blood flow
• normal, increased or decreased pulmonary blood pressure
• right or left anomaly
• right or left dominant ventricle
• general cardiopathies
• innocent murmurs
• double-inlet ventricle : a congenital anomaly in which both atrioventricular valves or a single common atrioventricular valve open into a single ventricle, which usually resembles the left ventricle morphologically (double inlet left v.) but may resemble the right (double inlet right v.) or neither or both ventricles.
• double-outlet left ventricle : a rare anomaly in which both great arteries arise from the left ventricle; it is often associated with a hypoplastic right ventricle, ventricular septal defect, valvular or subvalvular pulmonic stenosis, and a variety of associated malformations.
• double-outlet right ventricle : incomplete transposition of the great vessels in which both the aorta and the pulmonary artery arise from the right ventricle, associated with a ventricular septal defect. The defect may be remote from or close to either or both semilunar valves; it may be related to the aorta (subaortic), to the pulmonary trunk (subpulmonic), to both vessels (doubly committed), or to neither (uncommitted); and it may be associated with pulmonary stenosis.
• congenital complete heart block (CHB)
• corrected transposition of great vessels / congenitally or physiologically corrected transposition of the great arteries / mixed levocardia (0.5% of congenital cardiopathies) : a developmental cardiac anomaly characterized by transposition of the great vessels with inversion of the ventricles and atrioventricular valves; termed “corrected” because the inverted ventricles compensate for the transposition, producing a mirror-image blood flow in the heart, ensuring that blood flow continues in its usual physiologic pathway. However, because in > 99% of cases this condition usually is associated with other abnormalities, many have commented that it should not be called "corrected" .The most common anatomic associations include a ventricular septal defect (VSD) (80%) and the presence of pulmonary valve stenosis (50%), and tricuspid valve anomalies (including dysplasia, straddling, or Ebstein-like malformation (with or without regurgitation), 14-56%). The presence of a VSD causes a systemic-to-pulmonary shunt; however, this usually is balanced because of the "protective" effect of coexisting pulmonic stenosis.
• Taussig-Bing syndrome : a rare congenital malformation of the heart characterized by transposition of the great vessels and a ventricular septal defect straddled by a large pulmonary artery; hemodynamically it is characterized by pulmonary hypertension, pulmonary plethora, cyanosis, and greater O₂ saturation of blood in the pulmonary artery than in the aorta.
• microcardia : smallness of the heart
• cardiac or heart malposition / ectocardia : congenital displacement of the heart, either inside or outside the thorax.
• dextrocardia : location of the heart in the right hemithorax, with the apex pointing to the right, occurring with transposition (situs inversus) of the abdominal viscera, or without such transposition (other organs are normally placed).
• isolated dextrocardia : mirror-image transposition of the heart without accompanying alteration of the abdominal viscera.
• mirror-image dextrocardia : location of the heart in the right side of the chest, the atria being transposed and the right ventricle lying anteriorly and to the left of the left ventricle, usually associated with complete situs inversus.
• secondary dextrocardia : displacement of the heart to the right as a result of disease of the pleura, diaphragm, or lungs
• levocardia : a term denoting the normal position of the heart, used when other viscera are transposed
• isolated levocardia : levocardia associated with transposition (situs inversus) of the abdominal viscera, congenital structural anomaly of the heart, and sometimes with absence of the spleen.
• mixed levocardia / corrected transposition of the great vessels
• situs inversus viscerum / situs transversus : lateral transposition of the viscera of the thorax and abdomen; a familial pattern and consanguineous parents have been reported. Complete transposition of the viscera in the absence of other defects is structurally sound

Aetiology : Klinefelter's syndrome
• dextroversion : location of the heart in the right hemithorax, the left ventricle remaining on the left as in the normal position, but lying anterior to the right ventricle.
• ectopia cordis : congenital displacement of the heart outside the thoracic cavity because of maldevelopment of the pericardium and sternum
• pectoral ectopia cordis : location of the heart outside the thoracic wall, through a cleft in the lower sternum
• ectopia cordis abdominalis : a rare anomaly in which the heart is located in the abdominal cavity.
• acyanotic cardiopathies without a shunt
• right cardiopathies
• pulmonic valve stenosis
• congenital pulmonic valve failure
• idiopathic dilatation of the pulmonary trunk
• PPH
• acyanotic Ebstein anomaly of the tricuspid valve
• right ventricle hypoplasia
• arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC) : a congenital right-sided cardiomyopathy

Epidemiology : predominantly in young males
Aetiology :
• hereditary (30%; autosomal dominant) : heterozygous mutations in PKP2, which encodes plakophilin-2, an essential armadillo-repeat protein of the cardiac desmosome^ref
• sporadic (70%)
Pathogenesis : replacement of cardiomyocytes by fibrous or adipose tissue (transmural in right ventricle and subepicardiac in left ventricle) resulting in weakness, dilatation of the right ventricle, and pseudoaneurysmal bulging of the infundibulum, apex, and posterior basilar region of the right ventricle during systole. Cardiomyocytes entrapped within fatty tissue are electrically unstable and can occasionally lead to palpitations, syncope, and sometimes ventricular tachycardia arising in the right ventricle =>
• sudden arrhythmic cardiac death
• dangerous tachyarrhythmias
• contractile failure
Symptoms & signs : ARVD patients present between the second and fifth decades of life either with symptoms of palpitations and syncope associated with ventricular tachycardia or with SCD. There is a wide variation in presentation and course of ARVD patients, which can likely be explained by the genetic heterogeneity of the disease^ref
Laboratory examinations : white tissue at cardio-MRI. Diagnosis is often delayed
Therapy : once diagnosed and treated with an implanted cardioverter/defibrillator (ICD), mortality is low
• left cardiopathies
• aortic valve stenosis
• aortic valve failure
• congenital obstruction to left atrial inflow
• pulmonary vein stenosis
• mitral stenosis
• intraatrial septum in left atrium due to (cor triatriatum)

Treatment : perforation.
• mitral valve failure
• congenitally corrected trasnposition of the great arteries
• anomalous origin of the left coronary artery from the pulmonary trunk
• miscellaneous
• double-orifice mitral valve
• congenital perforations
• accessory commissures with anomalous chordal insertion
• congenitally short or absent chordae
• cleft posterior leaflet
• parachute mitral valve
• bicuspid aortic valve : a congenital anomaly of the aortic valve, caused by incomplete separation of 2 of the 3 cusps; it is generally asymptomatic early in life but is predisposed to calcification and stenosis later on. This condition, which narrows the passage through which blood exits the heart, can require surgery at birth. In severe cases the heart does not develop properly in the fetus and the child is born with an illness called hypoplastic left heart syndrome

Aetiology : calcification of the aortic valve is the third leading cause of heart disease in adults. The incidence increases with age, and it is often associated with a bicuspid aortic valve present in 1-2% of the population. Despite the frequency, neither the mechanisms of valve calcification nor the developmental origin of a 2, rather than 3, leaflet aortic valve is known. Mutations in the signalling and transcriptional regulator NOTCH1 cause a spectrum of developmental aortic valve anomalies and severe valve calcification in non-syndromic autosomal-dominant human pedigrees. Consistent with the valve calcification phenotype, Notch1 transcripts were most abundant in the developing aortic valve of mice, and Notch1 repressed the activity of Runx2, a central transcriptional regulator of osteoblast cell fate. The hairy-related family of transcriptional repressors (Hrt), which are activated by Notch1 signalling, physically interacted with Runx2 and repressed Runx2 transcriptional activity independent of histone deacetylase activity. These results suggest that NOTCH1 mutations cause an early developmental defect in the aortic valve and a later de-repression of calcium deposition that causes progressive aortic valve disease^ref.
Complications :
• infectious endocarditis
• aortic dissection
• aortic stenosis
• aortic failure
• endocardial fibroelastosis : diffuse patchy thickening of the mural endocardium, particularly in the left ventricle, due to proliferation of collagenous and elastic tissue. It usually occurs in infants without other cardiac defects, but may occur in adolescents and adults, usually in association with congenital cardiac malformations. Thickening and incompetence of mitral and aortic valves is often associated. It is usually classified as dilated if the left ventricle is enlarged and hypertrophied or contracted if the left ventricle is of normal or reduced size.
• primary endocrine endocardial fibroelastosis : a congenital form of endocardial fibroelastosis, manifest in infancy and occurring unassociated with other cardiac defects.
• coarctation of aorta, infantile type : a form usually seen in infants, characterized by cyanosis and diffuse involvement of the aortic isthmus, associated with other anomalies such as a patent ductus, due to dysharmonic body development (robust trunk and weak lower limbs) : hyposphigmia of femoral artery (compared to radial artery), upper arterial hypertension, costal incisions.
• pseudocoarctation of the aorta : an uncommon congenital anomaly of the arch of the aorta that simulates coarctation radiographically but does not produce occlusion of the vessel.
• cervical aortic arch : a rare, usually asymptomatic, congenital anomaly in which the aortic arch has an abnormally superior location, occasionally extending to the thoracic inlet or into the neck
• double aortic arch : a congenital anomaly in which the aorta divides into 3 branches which embrace the trachea and esophagus and reunite to form the descending aorta
• right aortic arch : a congenital anomaly in which the aorta is displaced to the right and passes behind the esophagus, thus forming a vascular ring that may cause compression of the trachea and esophagus
• acyanotic cardiopathies with left-to-right shunt => pulmonary arterial hypertension (PAH)
• endocardial cushion defects : a spectrum of septal defects resulting from imperfect fusion of the endocardial cushions :
• persistent ostium primum : an endocardial cushion defect characterized by a cleft in the basal portion of the atrial septum, usually associated with cleft mitral valve.
• persistent complete common atrioventricular canal
• septal defect : a defect in one of the cardiac septa, resulting in an abnormal communication between the opposite chambers of the heart
• atrioseptal or atrial septal defect (ASD) (8-13%) : congenital cardiac anomalies in which there is persistent patency of the atrial septum due to failure of fusion between either the septum secundum or the septum primum and the endocardial cushions
• isolated septal defect
• in septum primum
• atrioventricularis communis / persistent common atrioventricular canal : a congenital cardiac anomaly in which the endocardial cushions fail to fuse, the ostium primum persists (producing a low-lying atrial septal defect), sometimes a single atrioventricular valve occurs which has anterior and posterior cusps, and there is commonly a defect of the membranous interventricular septum
• ostium primum defect (severe) is a incomplete form of atrioventricularis communis, there is no septum at the base of the defect, between the mitral and tricuspid valves; it is usually associated with a cleft mitral cusp and occasionally with a cleft tricuspid valve or a ventricular septal defect.
• ostium secundum defect : there is a rim of septum all around the defect
• complete absence : common atrium or trilobated heart
• premature closure of foramen ovale => left atrium hypoplasia => death at birth
• patent foramen ovale (PFO)
• probe patency of foramen ovale : incomplete physical closure of the foramen ovale postnatally, although functional closure occurs, so that a probe may be passed between the atria in the adult. It occurs in 20 to 25% of all hearts.
Epidemiology : prevalence = 26%, but reduces with advancing age (34.3% until third decade (3.3 mm), 20.2% at ninth decade); commonly associated with
Aetiology : by maintaining an embryonic right atrial flow pattern into adult life and directing the blood from the inferior vena cava preferentially toward the interatrial septum, Chiari's network (found in 1.3% to 4% of autopsy studies and 2% at TEE) may favor persistence of a patent foramen ovale (associated in 83% of cases) and formation of an atrial septal aneurysm and facilitate paradoxic embolism^ref
Pathogenesis : interatrial right-to-left shunting during those periods of time when right atrial pressure exceeds left atrial pressure (Valsalva maneuver, occurring physiologically during defecation, weight lifting or pulling, strong repeated cough) =>
• embolism
• thromboembolism
• paradoxical embolism
• direct arterial embolism at the level of foramen ovale
• occult AF paroxysms => atrial thrombosis => cardioembolism
• gaseous or fatty embolism
• refractory hypoxemia in patients with right ventricular infarction or severe pulmonary disease
• orthostatic desaturation in the setting of the rare platypnea-orthodeoxia syndrome
• neurological decompression illness in divers
• migraine headache with aura^{ref1, ref2}
• transient global amnesia
• OSAS
• COPD
Therapy : despite the growing recognition of the PFO, particularly when associated with an atrial septal aneurysm (ASA), as risk factor for paradoxical embolism, the optimal treatment strategy for symptomatic patients remains undefined.
• surgical closure with prosthetic device
• anticoagulant drugs
Laboratory examinations :
• contrast-enhanced transesophageal echocardiography (TEE) + Valsalva maneuver
• contrast-enhanced transcranial echocolordoppler (TCD) (supine patients with horizontal arms, antecubital access, 9 ml NS + 1 ml air mixed just before injection => monitor 5 seconds after injection for 10 seconds; transit > 1 microbubble within 40") + Valsalva maneuver. It doesn't allow differential diagnosis with shunt at other level. Higher sensitivity with femoral vein access. Repeat if negative : the shunt may be permanent or latent (unmasked after Valsalva maneuver)^ref
• mild shunt : 1-10 microbubbles
• moderate shunt : 11-20 microbubbles
• severe shunt : > 20 microbubbles (tent effect)
Prognosis : if not treated, the likelihood of a relapse of ischemic stroke increases by 3%/yr
• septal defect associated with ...
• ... pulmonic valve stenosis
• Fallot trilogy
• Fallot pentalogy
• ... transposition of pulmonary veins
• total transposition : the 4 pulmonary veins join into a single trunk which drains into ...
• supracardiac variety
• ... azygos vein
• ... brachiocephalic trunk
• ... upper cava vein
• ... left pulmonary vein, which then drains into upper cava vein
• cardiac variety :
• ... right atrium
• ... coronary sinus
• subcardiac variety :
• ... lower cava vein
• ... suprahepatic veins
• mixed variety : 2 of the abovementioned varieties.
• partial transposition
• venous sinus-type partial transposition : the 2 right pulmonary veins drain into upper cava vein
• ... mitral valve stenosis (Lutembacher disease)
Epidemiology : 30% of congenital lesions in adults
Symptoms & signs :
• doubling of S₁ sound
• fixed doubling of 2^nd tone
• opening click
• filling or mesodiastolic or delayed diastolic murmur
Therapy :
• antithrombotic drugs
• percutaneous transcatheter closure
• surgical closure
• baffle fenestration or adjustable atrial septal defect in a modified Fontan operation. Hemodynamic benefits include increased cardiac index and systemic oxygen transport, as well as lower systemic venous pressure. The incidence and duration of pleural effusions is also reduced by this approach. Potential complications include those associated with the closure mechanism (snare or umbrella) as well as the possibility of paradoxical embolism.
• partial anomalous pulmonary venous connection
• ventricular septal defects (VSD) (25% of all congenital cardiopathies) : congenital cardiac anomaly in which there is persistent patency of the ventricular septum in either the muscular or fibrous (membranous) portions, most often due to failure of the bulbar septum to completely close the interventricular foramen.
• isolated septal defect (Eisenmenger disease)
• inlet septum
• pars perimembranosa
• over the crista supraventricularis (less frequent).
• below the crista supraventricularis (more frequent).
• pars muscularis / septum inferius (Roger disease / maladie de Roger) : single or multiple => Roger murmur
• infundibular septum
• cushions
• complete asbsence : univentricular heart
• septal defect associated with ...
• aortic regurgitation
• left ventricular to right atrial shunt
• ...
• Fallot tetralogy
• Fallot pentalogy
Symptoms & signs :
• systolic regurgitation murmur
• 3^rd tone
• aortic root to right heart shunt
• ruptured sinus of Valsalva aneurysm
• coronary arterovenous fistula
• anomalous origin of the left coronary artery from the pulmonary trunk
• aortopulmonary level shunt
• patent Botallo ductus arterious (PDA) : while in foetus blood flows from left pulmonary artery into aortic arc, in adult (when it doesn't undergo obliteration to create the arterial ligament) it flows from aorta into left pulmonary artery. Signs :
• differential cyanosis
• continuos murmur
• aortic or aorticopulmonary septal defect / aorticopulmonary fenestration or window : a congenital anomaly in which there is abnormal communication between the ascending aorta and pulmonary artery just above the semilunar valves
• multiple level shunts
• complete common atrioventricular canal
• ventricular septal defect (VSD) with atrial septal defect (ASD)
• ventricular septal defect (VSD) with patent ductus arteriosus (PDA)
• Fallot pentalogy / pentalogy of Fallot : the 4 defects of the Fallot tetralogy of Fallot + patent foramen ovale (PFO) or atrial septal defect (ASD)
• Cantrell's pentalogy / pentalogy of Cantrell : a cleft in the inferior part of the sternum associated with midline abdominal defects such as omphalocele, defective pericardium and diaphragm with communication between the pericardial and peritoneal cavities, and cardiac anomalies such as ventricular septal defect or less often atrial septal defect, tetralogy of Fallot, or left ventricular diverticulum.
• cyanotic cardiopathies with right-to-left (RLS) shunt
• normal or decreased pulmonary blood flow
• overriding aorta : a congenital anomaly occurring in tetralogy of Fallot and Eisenmenger disease, in which the aorta is displaced to the right so that it appears to arise from both ventricles and straddles the ventricular septal defect.
• normal or decreased pulmonary blood pressure
• dominant right ventricle : Fallot tetralogy / "blue disease"

Pathogenesis : anomaly of truncoconic septum => pulmonic valve stenosis & ventricular septal defect (VSD) => aorta communicating with both ventricles (overriding aorta, "knight aorta") & hypertrophy of right ventricle
• pseudotruncus arteriosus : the most severe form of tetralogy of Fallot with associated pulmonary atresia in which outflow is through a single major vessel, the aorta, accompanied by the remnant of the atretic pulmonary artery.
• Corvisart's disease : tetralogy of Fallot associated with right aortic arch
Symptoms & signs : early cyanosis, squatting (decreases blood return from lower limbs and increases pulmonary arterial hypertension (PAH) which limits left-to-right shunt), bronchitis (due to pulmonary hyperemia), doubling of 2^nd tone.
• dominant left ventricle
• Ebstein anomaly : congenital low dysplacement of tricuspid valve with right-to-left atrial shunt
• tricuspid valve atresia + ASD or VSD
• complete transposition of the great arteries with pulmonic valve stenosis
• double-outlet right ventricle with pulmonic valve stenosis
• single ventricle with pulmonic valve stenosis
• pulmonary arteriovenous fistula
• vena caval to left atrial communication
• normal ventricles
• increased pulmonary blood pressure
• Eisenmenger syndrome : ventricular septal defect with pulmonary hypertension and cyanosis due to right-to-left (reversed) shunt of blood. Sometimes defined as pulmonary arterial hypertension (pulmonary vascular disease) and cyanosis with the shunt being at the atrial, ventricular, or great vessel area. It occurs in patients with large ...
• ... congenital cardiac ...
• Eisenmenger disease : ventricular septal defect (VSD) (=> hypertrophy of right ventricle) and aorta communicating with both ventricles (overriding aorta, "knight aorta"). Therapy : heart transplantation.
• aortopulmonary window
• patent ductus arteriosus (PDA)
• ... surgically created extracardiac ...
• 10% of Blalock-Taussig anastomosis (subclavian artery to pulmonary artery)
• 30% of Waterston anastomosis (ascending aorta to pulmonary artery)
• 30% of Potts anastomosis (descending aorta to pulmonary artery)
... left-to-right shunts. These shunts initially cause increased pulmonary blood flow. Subsequently, usually before puberty, pulmonary vascular disease causes pulmonary arterial hypertension, ultimately resulting in reversed (right-to-left) or bidirectional shunt flow with variable degrees of cyanosis.
Symptoms & signs : hypoxia => cyanosis, dyspnea, syncope
• increased pulmonary blood flow
• complete transposition of great arteries or vessels (5-7% of all congenital cardiopathies, the commonest) : a congenital cardiovascular malformation in which the aorta arises entirely from the morphologic right ventricle and the pulmonary artery from the morphologic left ventricle, so that the venous return from the peripheral circulation is recirculated by the right ventricle via the aorta to the systemic circulation without being oxygenated in the lungs. It is incompatible with prolonged life unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level (atrial septal defect, ventricular septal defect, patent ductus arteriosus (PAD))

Treatment : surgical atrial septectomy => balloon atrial septostomy (Blalock-Hanlon operation : a palliative operation for transposition of the great vessels, consisting of the creation of an interatrial septal defect) => atrial switch (Senning operation : surgical creation of two interatrial channels for crossing the systemic and pulmonary venous circulations in transposition of the great vessels) => arterial switch operation / Mustard operation : correction of transposition of great vessels by construction of an intra-atrial baffle, composed of pericardial tissue or synthetic material, to direct the systemic and pulmonary venous blood into the left and right ventricles, respectively (survival rate > 90%)
• double-outlet right ventricle of the Taussig-Bing type
• truncus arteriosus
• total anomalous pulmonary venous connection
• single ventricle wthout pulmonic stenosis
• common atrium
• tetralogy of Fallot with pulmonary atresia and increased collateral arterial flow
• tricuspid atresia with large ventricular septal defect and no pulmonic stenosis
• hypoplastic left heart syndrome (HLHS) : any of a group of congenital anomalies consisting of hypoplasia or atresia of the left ventricle and of the aortic or mitral valve or both and hypoplasia of the ascending aorta; it is characterized by respiratory distress and extreme cyanosis, with cardiac failure and death in early infancy. It accounts for nearly 25% of deaths among neonates with congenital heart disease. A hypertrophic frenulum separating the alveolar portion of the maxillary palatine suture is a marker^ref. A hypothesis is proposed implicating an immune mechanism involving maternal antibodies produced in response to pharyngitis caused by group A b-hemolytic streptococci (GABHS) (“strep throat”). After crossing the placenta, the antibodies injure the developing fetal heart, leading to HLHS either because of direct injury to the LV or secondary to reduced blood flow through affected aortic and mitral valves. Analogy is drawn to rheumatic heart disease (RHD), a known sequela of strep throat^ref.
Complications : hyperuricemia, cholelithiasis, renal failure, impaired hemostasis
Aetiology : SNPs in BMP4 can lead to atrioventricular canal defects (AVCD)
• cardioptosis : downward displacement of the heart.
• cardiocoele : protrusion of the heart through a fissure of the diaphragm or through a wound.
• cardioplegia : arrest of contraction of the myocardium, as may be induced by the use of
• chemical compounds
• cold (cryocardioplegia)
... in the performance of surgery upon the heart.
• carditis : inflammation of the heart.
• Lyme carditis : cardiac involvement, generally transient, in Lyme disease; it usually manifests as some degree of atrioventricular block but ventricular tachycardia and left ventricular dysfunction (LVD) may occur.
• rheumatic carditis / rheumatic heart disease (RHD) : cardiac involvement in rheumatic fever, which when severe may be manifested by congestive heart failure, progressive cardiac enlargement, pericarditis, and significant murmurs due to valvular dysfunction.
• streptococcal carditis : carditis occurring as a result of streptococcal sore throat.
• diseases of endocardium
• endocarditis : exudative and proliferative inflammatory alterations of the endocardium, usually characterized by the presence of vegetations or verrucae on the surface of the endocardium (vegetative or verrucous endocarditis) or in the endocardium itself. It may occur as a primary disorder or as a complication of or in association with another disease.

Localizations :
• valvular endocarditis (most commonly) : endocarditis affecting the membrane over the valves of the heart, rather than the mural, chordal, trabecular, or papillary tissue
• native valve endocarditis : infective endocarditis involving one or more of the natural heart valves. The most affected is the mitral valve.
• prosthetic valve endocarditis : infective endocarditis as a complication of implantation of a prosthetic valve in the heart; the vegetations usually occur along the line of suture
• ulcerative endocarditis : infective endocarditis characterized by rapid ulceration of the valvular lesions.
• mural or parietal endocarditis : a form affecting the lining of the walls of the heart chambers, rather than the valvular, chordal, trabecular, or papillary tissue
• endocarditis chordalis : endocarditis affecting particularly the chordae tendineae.
• right-side endocarditis : primary acute endocarditis of the right side of the heart.
Aetiology :
• infectious or infective endocarditis (IE) / malignant or septic endocarditis : endocarditis caused by infection with microorganisms. It has been classified according to course. Because underlying causes and available therapies have changed, this division has little current clinical validity and has been largely replaced by classification on the basis of etiology or underlying anatomy.

Epidemiology : incidence = 1.6÷6 cases / 100,000 / yr; male-to-female ratio 1:5; average onset at age 60; mortality has declined since half of 1970's
Localizations : as for rheumatic endocarditis, but right endocarditis is more common in intravenous drug addicted
• acute bacterial endocarditis (ABE) involves a normal heart valve, and has a short history and rapid course (incubation : 2-5 weeks). RF in 25-50%.
• Staphylococcus aureus (staphylococcal endocarditis : infective endocarditis caused by staphylococcal invasion of the heart valves; lasting 1-2 weeks, expecially in immunocompromised hosts)
• Neisseria gonorrhoeae
• Streptococcus pneumoniae
• subacute endocarditis / endocarditis lenta (affects damaged heart valves, and lasting 3-4 weeks or months)
• subacute bacterial endocarditis (SBE) :
• streptococcal endocarditis : infective endocarditis caused by streptococcal invasion of the heart valves
• fecal streptococci (in feces of both children and adults; nowadays renamed Enterococcus spp.)
• Enterococcus avium (feces of children)
• Enterococcus faecalis
• Enterococcus faecium
• viridans endocarditis : a subacute form of infective endocarditis due to infection with viridans streptococci.
• Streptococcus bovis (feces of children)
• Streptococcus intermedius (feces of adults)
• Streptococcus milleri
• Streptococcus mitis (feces of adults)
• Streptococcus mutans
• Streptococcus salivarius (feces of adults)
• Streptococcus sanguinis
• fungal or mycotic endocarditis : infectious endocarditis, usually subacute, due to various fungi, most commonly
• Candida spp. (Candida endocarditis / endocardial candidiasis)
• Aspergillus spp.
• Ajellomyces capsulatus
• HACEK group :
• Haemophilus spp.
• Actinobacillus actinomycetemcomitans
• Cardiobacterium hominis
• Eikenella spp.
• Kingella kingae
• Brucella melitensis
• Yersinia spp.
• Listeria monocytogenes
• Coxiella burnetii (rickettsial endocarditis : endocarditis caused by invasion of the heart valves with Coxiella burnetii; it is a sequela of Q fever, usually occurring in persons who have had rheumatic fever)
• anaerobic bacteria^ref :
• anaerobic cocci
• Propionibacterium acnes
• Bacteroides fragilis group
• Veillonella spp.
• Acinetobacter spp.
• Chlamydia spp.
• Mycoplasma spp.
• Treponema pallidum subsp. pallidum (syphilitic endocarditis : endocarditis resulting from extension of syphilitic infection from the aorta)
• Mycobacterium tuberculosis (tuberculous endocarditis : a rare form of endocarditis in which the endocardium is involved by extension of a tuberculous perimyocarditis or of miliary tuberculosis)
• Suttonella indologenes
• Tropheryma whippelii
Predisposing conditions :
• local conditions increasing pressures and slowing blood flow => deposition of microorganisms and platelets
• high risk
• aortic valve stenosis
• calcification of mitral valve ring
• valve prostheses (prosthetic valve endocarditis)
• ventricular septal defect (VSD)
• patent ductus arteriosus (PAD)
• coarctation of aorta
• Marfan's syndrome
• intermediate risk
• prolapse of mitral valve
• mitral valve stenosis
• right heart valvulopathies
• previous fibroplastic endocarditis
• asymmetric septal hypertrophy
• calcified aortic sclerosis
• vascular catheters
• low risk
• atrial septal defect (ASD)
• AMI
• atherosclerosis
• immunodepression
Source of infectious organisms :
• direct implantation during diagnostic or therapeutic invasive techniques
• hematogenous route
• endovenous drug addiction (=> endocarditis on tricuspid valve => tricuspid valve failure)
• tooth extraction
• abdominal or urinary surgery
• respiratory infections
• skin infections
Symptoms & signs :
• due to systemic infection
• long-lasting, remitting or intermittent fever with chills, diaphoresis
• malaise, lethargy
• myoarthralgia and tenosynovitis => focal embolic glomerulonephritis => proteinuria and hematuria
• delirium, headache
• pallor
• weight loss
• splenomegaly
• anemia
• leukocytosis
• Litten's sign
• splinter hemorrhages
• Janeway's lesions
• due to local infection
• dyspnea, chest pain, abdominal and limb pain
Morphology : few large polypoid thrombotic vegetations consisting of fibrin, platelets, and granulocytes) : that can embolize in early stages, ulcerations => cuspid perforation, and colonies of microorganisms, Bracht-Wächter bodies (nonspecific inflammatory foci of lymphocytic and mononuclear cells in the myocardium) (differential diagnosis with nonbacterial thrombotic endocarditis (NBTE))
Definition of infective endocarditis according to
• Duke criteria, 1994, proposed by Durack et al.^ref. These criteria have been validated by the authors of reports of numerous studies during the past 4 years in a wide spectrum of patients, including children, adults, the elderly, prosthetic valve recipients, injection drug users, nondrug users, patients in tertiary care settings, patients in primary hospitals, and patients treated outside of the United States. When pathologically confirmed cases were considered to be the gold standard for assessing the Duke criteria, the collective sensitivity of the Duke criteria in numerous studies was >80%^ref. In addition, the high specificity^ref and negative predictive value^ref of the Duke criteria have been confirmed. Despite these data, the Duke criteria have shortcomings. An often-heard criticism of the Duke criteria is the overly broad categorization of the group "possible IE." For example, in the original Duke criteria, an individual patient could be classified as having "possible IE" if only 1 minor criterion was present and if the patient did not meet requirements for "rejected IE." To "reduce the size of this possible group and to amplify the size of the rejected group," Bayer^ref recently called for a "diagnostic floor" for the group of patients listed as "possible IE." Other critics have suggested the modification of current^ref or the inclusion of additional minor criteria^ref to increase the sensitivity of the Duke criteria. Finally, issues such as the relative risk of IE in cases of Staphylococcus aureus bacteremia^{ref1, ref2}, the poor diagnostic sensitivity in suspected cases of Q-fever IE^ref, and the relative role of transesophageal echocardiography (TEE) in the diagnosis of IE^{ref1, ref2, ref3} remain unresolved.
• modified Duke criteria, 2000, with modifications shown in Italics^ref :
• definite infective endocarditis
• pathologic criteria
• microorganisms demonstrated by culture or histologic examination of a vegetation, a vegetation that has embolized, or an intracardiac abscess specimen; or
• pathologic lesions; vegetation or intracardiac abscess confirmed by histologic examination showing active endocarditis
• clinical criteria
• 2 major criteria; or
• 1 major criterion and 3 minor criteria; or
• 5 minor criteria
• possible infective endocarditis
• 1 major criterion and 1 minor criterion; or
• 3 minor criteria
• rejected :
• firm alternate diagnosis explaining evidence of infective endocarditis; or
• resolution of infective endocarditis syndrome with antibiotic therapy for 4 days; or
• no pathologic evidence of infective endocarditis at surgery or autopsy, with antibiotic therapy for 4 days; or
•  does not meet criteria for possible infective endocarditis, as above
Major criteria :
• blood culture positive for IE (time for PCR introduction ?^ref)
• typical microorganisms consistent with IE from 2 separate blood cultures:
• viridans streptococci, Streptococcus bovis, HACEK group, Staphylococcus aureus; or
• community-acquired enterococci, in the absence of a primary focus; or
• microorganisms consistent with IE from persistently positive blood cultures, defined as follows:
• at least 2 positive cultures of blood samples drawn >12 h apart; or
• all of 3 or a majority of 4 separate cultures of blood (with first and last sample drawn at least 1 h apart)
• single positive blood culture for Coxiella burnetii or antiphase I IgG antibody titer >1 : 800
• evidence of endocardial involvement
• echocardiogram positive for IE (transesophageal echocardiography recommended in patients with prosthetic valves, rated at least "possible IE" by clinical criteria, or complicated IE [paravalvular abscess]; transthoracic echocardiography as first test in other patients), defined as follows :
• oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation; or
• abscess; or
• new partial dehiscence of prosthetic valve
• new valvular regurgitation (worsening or changing of pre-existing murmur not sufficient)
Minor criteria :
• predisposition, predisposing heart condition or injection drug use
• fever, temperature >38°C
• vascular phenomena, major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway's lesions
• immunologic phenomena: glomerulonephritis, Osler's nodes, Roth's spots, and rheumatoid factor
• microbiological evidence: positive blood culture but does not meet a major criterion as noted above (excludes single positive cultures for coagulase-negative staphylococci and organisms that do not cause endocarditis) or serological evidence of active infection with organism consistent with IE
• echocardiographic minor criteria eliminated
Complications :
• septic embolism => Osler's nodes, multiple cerebral and renal infarction or abscess
• perforation => acute valvular failure => acute heart failure
• myocardial abscess
• cardiac aneurysm
• focal membranous glomerulonephritis
• diffuse proliferative glomerulonephritis
Therapy & prophylaxis before minor and major surgery : antibacterial drugs.
Prognosis :
• lethality : 40%.
• recovery when treated
• organization of thrombi => joining of edges => nastriform calcified masses on edges after years
• rheumatic endocarditis : endocarditis associated with rheumatic fever. Involvement may be mural but is usually valvular and involves the entire valve; it is then more accurately termed rheumatic valvulitis, characterized by valvular edema, numerous small, erosions and translucent vegetations (1-3 mm "warts" or protrusions of connective tissue covered by fibrin and platelets) located as a rosary chain on the edges of the valve cusps along the lines of closure and chordae tendinae. As vegetations are well-anchored, they don't embolize

Localizations : mitral valve (alone or with aortic valve) (90%) > isolated aortic valve > mitral, aortic and tricuspid valves > mitral and tricuspid > tetravalvulitis
Pathogenesis : after 1 year => retracting fibrosis (fibroplastic endocarditis) => stiffening and joining of commissurae
• nonbacterial thrombotic endocarditis (NBTE) / marantic endocarditis : endocarditis characterized by noninfected vegetations composed of fibrin and other blood elements, generally located on the line of closure of the mitral and aortic valves and susceptible to embolization.

Aetiology : chronic debilitating disease
• cancers
• Libman-Sacks disease or endocarditis / atypical or nonbacterial verrucous endocarditis or carditis / endocarditis benigna : NBTE associated with systemic lupus erythematosus and occasionally in scleroderma, thrombotic purpura, and other collagen diseases; the vegetations (whose size is intermediate between that of rheumatic and infectious endocarditis) consist of necrotic nuclear debris (hematoxylinophilic bodies), fibrinoid degeneration, and trapped, disintegrating, fibroblastic and inflammatory cells and are located on the edges of the valve cusps along the lines of closure, on chamber walls and chordae tendinae

Localizations : tricuspid valve > tricuspid + mitral valves > tricuspid + pulmonary valves
Pathogenesis : => fibrosis (as for rheumatic endocarditis)
• thromboendocarditis : a term formerly used for nonbacterial thrombotic endocarditis or sometimes incorrectly for nonbacterial verrucous endocarditis.
• Löffler's parietal fibroplastic endocarditis / constrictive endocarditis / eosinophilic endomyocardial disease : endocarditis associated with eosinophilia, marked by fibroplastic thickening of the endocardium, and resulting in congestive heart failure, persistent tachycardia, hepatomegaly, splenomegaly, serous effusions into the pleural cavity, edema of the legs, and edema and ascites of the arms
Laboratory examinations :
• transesophageal echocardiography (TEE) => polypoid endocardial vegetations on valve edges or chamber walls (and rarely on endothelium of large arteries)
• hemoculture
• variation of preexisting cardiac murmurs
• increased ESR
• sometimes alterated CSF
• diseases of heart valves / valvulopathies
• cardiovalvulitis : inflammation of the valves of the heart.
• failure
• stenosis
• restenosis : recurrent stenosis, especially of a valve of the heart, after surgical correction of the primary condition.
• false restenosis : stenosis recurring after failure to divide either commissure of the cardiac valve beyond the area of incision of the papillary muscles.
• true restenosis : restenosis occurring after complete opening of one or both of the commissures of the cardiac valve involved.
• diseases of atrioventricular valves
• diseases of mitral valve^ref
• mitral stenosis (MS)
• buttonhole or fishmouth mitral stenosis / mitral buttonhole : an advanced state of mitral stenosis in which adhesion and shortening of the mitral cusps produces a diaphragmatic slit resembling a buttonhole
Epidemiology : female:male ratio 3:1.
Aetiology :
• rheumatic fever : complete calcification of a rheumatic left atrium was first described in 1898, in association with chronic rheumatic mitral disease, and is more common in women, most of whom have symptoms for > 20 years. The condition is assumed to be the end result of extensive rheumatic pancarditis. The calcification may be confined to the left atrial appendage or, rarely, to the posterior left atrial wall — owing to a regurgitant mitral jet — in which case the calcified patch is called the MacCallum's patch. Massive calcification usually spares the interatrial septum, but when the septum is affected, any further surgery near the mitral valve is hazardous. Radiography of the left lateral side of the chest is recommended to assess long-standing rheumatic mitral-valve disease. Complete calcification has been described as a "coconut atrium" or "porcelain atrium"^ref
• thromboembolism
• atrial myxoma
• bronchial carcinoid
Pathogenesis => symptoms : fusion of valve commissures, fibrotic and calcified cuspids, shortened chordae tendinea =>
• A normally 4 cm²
• A = 2-4 cm² (mild stenosis)
• A = 1.3-2 cm² (moderate stenosis)
• A < 1.3 cm² (severe stenosis) => surgery
symptomatic if A < 1 cm² => decreased AV flow => atrial hypertension => left heart failure (exercise intolerance (tachycardia), AT and AF => arterial thromboembolism, bitonal voice due to compression of left lower laryngeal / recurrent nerve, cyanotic mitralic facies; rarely myocardium ischemia) => pulmonary arterial hypertension (PAH) (=> hemoptysis, dyspnea, acute pulmonary edema if A < 0.7 cm²).
Signs :
• heart auscultation
• opening click of mitral valve
• filling or mesodiastolic or delayed diastolic murmur
• presystolic or atriosystolic murmur
• increased intensity of S₁ sound
• normal or increased intensity of S₂ sound
• hyposphygmic radial pulse
• EKG
• camel-shaped P wave
• atrial tachycardia and atrial fibrillation
• chest X-ray
• enlarged left atrium auriculae
• increased lung vascularization
• if age < 50 : transesophageal echocardiography (TEE) (doming opening and atriomegaly)
• if age > 50 : cardiac catheterism + coronarography to exclude CAD
Therapy:
• asymptomatic (NYHA dyspnea class I or II) : echocardiography every year and medical therapy
• symptomatic (NYHA dyspnea class III or IV, thromboembolism, AF, A < 1.2 cm², tachycardia) :
• diuretics for heart failure; digoxin, b-blockers, and rate-limiting calcium antagonists for rate control in AF
• conservative surgery (divulsion, commissurotomy or ballooned valvuloplasty) or valve replacement
Secondary prevention : penicillin prophylaxis against recurrent episodes of rheumatic fever; anticoagulants to prevent systemic thromboembolism
• mitral failure or regurgitation (MR)

Aetiology :
• acute => atrial compliance doesn't increase
• acute myocardial infarction (AMI)
• trauma
• infectious endocarditis(vegetations, fissuration)
• left atrial myxoma
• left ventricle aneurysm
• myocardium abscess
• chronic => increased atrial compliance
• congenital : fenestrated mitral valve
• inflammatory
• rheumatic fever
• systemic lupus eryhtematosus (SLE)
• systemic scleroderma (SSc)
• rheumatoid arthritis (RA)
• ankylosing spondylitis (AS)
• Takayasu arteritis
• degeneration
• myxomatous degeneration (mitral valve prolapse)
• Marfan's syndrome
• Ehlers-Danlos disease
• valvular ring calcifications
• infections : bacterial endocarditis in carriers of valve prostheses or after cardiosurgery in elderlies
• structural impairment
• dilatation of left ventricle
• dyskinesia of papillary muscles and chordae tendinea
• thromboembolism
• annular dilatation
• thickened leaflets
• flail mitral valve : a mitral valve having a cusp that has lost its normal support (as in ruptured chordae tendineae) and flutters in the blood stream
• redundant leaflets with abnormal coaptation
• papillary muscle rupture
• papillary muscle dysfunction
• annular calcification
• paraprosthetic leak
Symptoms are those due to AF, pulmonary arterial hypertension (PAH), dyspnea, acute pulmonary edema, and right ventricle failure
Signs :
• acute
• protomesosystolic regurgitation murmur
• chronic
• pansystolic regurgitation murmur
• large doubling of 2^nd tone
• 3^rd tone
Carpenter classification :
• type I (fig.a) : normal leaflet, normal chordal motion; annular dilatation due to dilatated cardiomyopathy
• type II (fig. b) : leaflet prolapse, excessive chordal motion = mitral valve prolapse (MVP) syndrome / Barlow's disease or syndrome / floppy mitral valve syndrome / midsystolic click–late systolic murmur syndrome / systolic click–murmur syndrome

Epidemiology : prevalence = 5% of population (females > males)
Pathogenesis : myxomatous proliferation of the leaflets of the mitral valve
• too large leaflets
• too long chordae tendinea
• ischemia of papillary muscles
Symptoms : often asymptomatic. Palpitations and chest discomfort may occur
Signs on auscultation :
• mesotelesystolic regurgitation murmur
• midsystolic clicks
Treatment : in some cases progressive mitral regurgitation necessitates valve replacement (redundancy or hooding of mitral valve leaflets so that they prolapse into the left atrium, often causing mitral regurgitation), conservative (asportation of part of the strip(s) and suture of the remaining part, shortening of chordae tendinea by inserting them back into papillary muscles, solid ring) or prosthetic surgery.
• type III (fìg. c) : restricted leaflet or restricted chordal motion

Therapy :

	0	1	2
symptoms (NYHA class)	absent	I/II	III/IV
EF%	> 60	50-60	< 50
ESD [mm]	< 40	40-45	> 45
factibility	absent	possible	sure

Overall score :
• 0-1 : dilatation, clinical follow-up, echocardiography every 12 months
• 2 : borderline, clinical follow-up, echocardiography every 6 months
• 3-6 : surgery
• asymptomatic (NYHA dyspnea class I or II) :
• normal heart => echocardiography every 6-8 months
• dilated or hypokinetic heart => cardiac catheterism and coronarography if age > 40
• EF > 25-30% => conservative (valvuloplastic with insertion of solid ring or suture) or prosthetic surgery.
• EF < 25-30% => medical therapy (diuretics and vasodilators (usually ACEIs) for heart failure; nitrates and b-blockers for angina), valve replacement, or heart transplantation
• symptomatic (NYHA dyspnea class III, tachyarrhythmia, AF) => transesophageal echocardiography (TEE) (echocolordoppler may be expecially useful)
Secondary prevention : no proven treatment
• mitral valve stenofailure

Aetiology :
• rheumatic fever
• senile degeneration
• diseases of tricuspid valve
• tricuspid stenosis (TS)

Signs :
• doubling of S₁ sound
• filling or mesodiastolic or delayed diastolic murmur
• tricuspid failure or regurgitation (TR)

If mild it can be considere paraphysiological.
Aetiology : gastrointestinal carcinoid
Signs :
• systolic regurgitation murmur
Treatment : cardiac catheterism + right ventriculography =>
• functional failure =>
• mild => no need for surgery
• severe =>
• organic failure => conservative surgery or valve replacement
• combined disease
• diseases of semilunar valves
• diseases of aortic valve^ref
• aortic stenosis (AS)

Aetiology :
• malformative : bicuspid aortic valve
• calcific or calcifying aortic stenosis : atherosclerosis
• post-inflammatory : rheumatic fever
• rarely bacterial endocarditis
Grading :
• normal transvalvular pressure gradient < 4 mmHg
• 50-60 mmHg : moderate stenosis
• > 80 mmHg : severe stenosis
Varieties  :
• valvular aortic stenosis : congenital, bicuspid aortic valve, rheumatic fever, senile fibrocalcification
• supravalvular aortic stenosis : a rare form of aortic stenosis occurring above the aortic valve, usually caused by a complete circumferential fibrous ring of constricting tissue at the level of the sinus of Valsalva => Williams-Beuren syndrome / elfin facies syndrome
• subaortic or subvalvular aortic stenosis : aortic stenosis due to an obstructive lesion in the left ventricle below the aortic valve, causing a pressure gradient across the obstruction within the ventricle
• membranous
• nodular
• due to hypertrophic cardiomyopathy
Sudden onset.
Symptoms & signs :
• effort symptoms : fatigue, dyspnea, lipothymia, angina, resting arrhthmias (increased afterpotential QT dispersion)
• edemas
• diamond-shaped systolic ejection murmur irradiated to carotid arteries
• paradoxical doubling of S₂ sound
• S₃ sound
• S₄ sounds
Laboratory examinations : widespread use of catheterisation to assess the extent of aortic-valve stenosis could increase the risk of cerebral blood clotting and brain damage
Therapy :
• A < 0.75 cm² / m²_BSA => DP_{transvalvular} > 50 mmHg : aortography, coronarography if age > 40 years
• A > 0.75 cm² / m²_BSA => DP_{transvalvular} < 50 mmHg
• asymptomatic => echocardiography
• symptomatic => diuretics for heart failure, nitrates and b-blockers for angina
=> conservative (divulsion, commissurotomy or ballooned valvuloplasty) or valve replacement (indicated also for women planning a pregnancy) + ventriculoplasty (if post-ischemic) to prevent aCHF
Complications : type 2 acquired von Willebrand's disease / von Willebrand's syndrome => slatentization of intestinal angiodysplasia (IA) (Heyde's syndrome)
Secondary prevention : no proven treatment but lipid-lowering therapy may slow progression of calcific aortic stenosis
• aortic failure, insufficiency or regurgitation (AR)

Aetiology :
• alterations of cuspids
• rheumatic fever
• rheumatoid arthritis (RA)
• infectious endocarditis
• trauma
• annuloaortic ectasia : dilatation of the proximal aorta and the fibrous ring of the heart at the aortic orifice, marked by aortic regurgitation and, when severe, by dissecting aneurysm
• Marfan's syndrome
• Turner syndrome
• Ehlers-Danlos disease
• ascending aorta aneurysm
• ankylosing spondylitis (AS)
• aortitis
• Treponema pallidum subsp. pallidum
• Reiter syndrome
• ulcerative colitis
• loss of support : subaortic ventricular septal defect (VSD)
• quadricuspid aortic valve
Pathogenesis : myocardial hypertrophy compensatory of left ventricular volume overload
Symptoms & signs :
• diastolic regurgitation murmur
• presystolic or atriosystolic murmur
• Traube's sign / pistol-shot sound : a loud sound like a pistol shot heard in auscultation over the femoral arteries in aortic regurgitation
• Hill's sign : disproportionate femoral systolic hypertension, seen in aortic regurgitation and certain other conditions involving increased stroke volume.
• results of markedly increased stroke volume :
• Quincke's pulse :
• Müller's sign : a sign of aortic insufficiency consisting of pulsation, or bobbing, of the uvula and redness of the tonsils and velum palati during systole, infrequently seen with severe or sudden-onset aortic regurgitation. In some patients, the pulsation extended to the soft palate and posterior oropharynx^ref
• Musset's sign : rhythmical jerking movement of the head; seen in cases of aortic aneurysm and aortic insufficiency.
Treatment : carotideal doppler if age > 65 =>
• normal heart, asymptomatic, intermediate failure => control every 6-8 months
• enlarged heart, severe failure =>
• asymptomatic
• normal EF => control every 3 months
• decreased EF =>
• symptomatic => aortography, cardiac catheterism, coronarography =>
• EF > 25 % => surgery
• EF < 25 % => echocardiography with dobutamine
• negative => diuretics and vasodilators (usually ACEIs) for heart failure; valve replacement and eventually heart reduction or transplantation
• positive => surgery
Secondary prevention : vasodilators (nifedipine or ACEIs) to protect the left ventricular myocardium and delay the need for surgery
• combined disease
• diseases of pulmonic valve
• pulmonary stenosis
• subinfundibular
• infundibular stenosis : stenosis below the pulmonary valve, within the infundibulum (conus arteriosus) of the right ventricle of the heart.
• valvular
• supravalvolar (stenosis of pulmonary artery and its branches))
Aetiology : gastrointestinal carcinoid
Signs :
• systolic ejection murmur
• large doubling of 2^nd tone
• paradoxical doubling of 2^nd tone
• pulmonary failure

If mild it can be considere paraphysiological.
Symptoms & signs : diastolic regurgitation murmur
• combined disease
• multiple valvulopathies
• diseases of myocardium / cardiomyopathies : a general diagnostic term designating primary noninflammatory disease of the heart muscle, often of obscure or unknown etiology and not the result of ischemic, hypertensive, congenital, valvular, or pericardial disease.

Diagnosis : EKG.
Aetiology and pathogenesis
• myocardial contusion : contusion of the heart, most frequently due to impact against an automobile steering wheel or other blunt object; the trauma may cause arrhythmias, conduction disturbances, or clinical signs of acute myocardial infarction such as electrocardiographic abnormalities.
• idiopathic or primary cardiomyopathies : those disorders in which the pathological process involves solely the myocardium and in which the cause is unknown and not part of a disease affecting other organs
• idiopathic
• familial or intrinsic aetiology (20-50%; 68% between age 1 and 19)
• mutations in cardiomyocyte-expressed genes (=> direct damage)
• sarcomere proteins
• cytoskeleton proteins
• energy metabolism proteins
• mutations in modifier genes
• ACE
• altered expression of normal genes
• secondary cardiomyopathies : any form that is due to another cardiovascular disorder (e.g., systemic arterial hypertension) or is a manifestation of systemic disease (e.g., sarcoidosis).
• sporadic
• arrhythmias

HR varies physiologically during respiration (up to 5% during normal respiration, up to 30% during deep respiration).
If HR < 60 bpm in an adult : bradycardia
• central bradycardia : bradycardia dependent on disease of the CNS
• fetal bradycardia : a fetal heart rate (FHR) < 120 beats per minute, generally associated with hypoxia; it is usually due to placental insufficiency; it may also result from placental transfer of local anesthetics or beta-adrenergic blocking agents, and rarely from heart block associated with congenital heart disease or maternal collagen vascular disease.
• nodal bradycardia : bradycardia in which the stimulus of the heart's contraction arises in the atrioventricular node or common bundle.
• postinfective bradycardia : bradycardia occurring after infectious disease.
• essential bradycardia : bradycardia occurring without discoverable cause.
• Branham's bradycardia or sign : bradycardia produced by digital closure of an artery proximal to an arteriovenous fistula.
If HR > 100 bpm in an adult : tachycardia
• atrioventricular reciprocating tachycardia (AVRT) : a reentrant tachycardia in which the reentrant circuit contains both the normal atrioventricular nodal to His bundle pathway and an accessory pathway as integral parts
• antidromic atrioventricular (AV) reciprocating tachycardia : a reentrant tachycardia in which the reentrant circuit involves anterograde conduction over the accessory pathway and retrograde conduction over the normal AV node to His bundle pathway
• orthodromic atrioventricular (AV) reciprocating tachycardia : a nodal reentrant tachycardia in which the reentrant circuit involves anterograde conduction over the usual AV node to His bundle pathway and retrograde conduction over an accessory pathway
• double tachycardia : the occurrence of 2 types of ectopic tachycardia, e.g., nodal and ventricular tachycardia, at the same time.
• ectopic tachycardia : abnormally rapid heart action in response to impulses arising outside the sinoatrial node.
• orthostatic tachycardia : disproportionate rapidity of the heart rate on rising from a reclining to a standing position.
• pacemaker-mediated tachycardia / endless loop tachycardia : in patients with dual chamber pacemakers, tachycardia caused by retrograde conduction of ventricular impulses, either premature ventricular complexes or impulses triggered by ventricular pacing which are sensed by the atria and trigger a subsequent ventricular impulse; an endless loop may develop
• paroxysmal tachycardia : a condition marked by attacks of rapid action of the heart having sudden onset and cessation. It is usually qualified by the locus of impulse origin as either ventricular or supraventricular; some classifications subdivide the latter into atrial and junctional tachycardias.
• reciprocating tachycardia : a tachycardia due to a reentrant mechanism and characterized by a reciprocating rhythm
• reentrant tachycardia / circu