1、Congenital Heart Diseases,Caridovascular lab #3,Congenital heart diseaseGeneral considerations,Abnormalities of the heart and/or great vessels that are present from birth. Incidence- Approximately 6-8 per 1,000 live births Genetic and/or environmental factors play a role, but only 10% of cases have

2、associated identifiable contributing factors.,Congenital heart diseaseAge of Presentation,Variable depending on type The minority are incompatible with intrauterine survival May present soon after birth ( right to left shunts ) Later in life, even adulthood ( ASD ),Three Functional Categories,Left t

3、o right shunts (“l(fā)ate cyanosis”) Right to left shunts (cyanotic congenital heart disease) Obstructions to flow (obstructive congenital heart disease),Left to Right Shunts “Late Cyanosis”,Initially Left sided pressure right sided pressure ASD, VSD, or patent ductus arteriosis (PDA) -these may later b

4、ecome right to left shunts,Obstructions to Flow “Obstructive Congenital Heart Disease”,Abnormal narrowing (stenosis) or complete occlusion (atresia) of chambers, valves or blood vessels e.g. coarctation of the aorta, aortic valvular stenosis, pulmonary stenosis May have combinations of obstructive l

5、esions and shunts- e.g. tetralogy of Fallot has pulmonary stenosis associated with a shunt,Right to Left Shunts “Cyanotic Congenital Heart Disease”,Less common than L to R shunts (Late cyanosis) Right sided pressure left sided pressure Blood is shunted away from pulmonary circulation,Right to Left S

6、hunts the five Ts,Tetralogy (8%) Transposition of great vessels(5%) Tricuspid atresia (2%) Total anomalous pulmonary venous connection (2%) Truncus arteriosis (1%),( relative %s of congenital Heart dz),Tetralogy of Fallot,Most common cause of right to left shunts Cyanosis is present from birth or so

7、on after in most cases Hypoxia is variable and directly depends on the amount of pulmonary obstruction Pulmonary obstruction relates directly to amt shunted away from the lungs Correctable with surgery Some anatomic variations are harder to correct than others Surgery is not emergent as long as chil

8、d can handle level of hypoxia (pulmonary obstruction protects the lungs from high pressures so unlike a big VSD, these pts dont need to be correctly quickly to avert pulmonary hypertension),Tetralogy of FallotTreatment,Correctable with surgery Some anatomic variations are harder to correct than othe

9、rs Surgery is not emergent as long as child can handle level of hypoxia Pulmonary vessels protected from VSD by pulmonic stenosis- alleviates need for immediate surgical intervention,Tetralogy of Fallot Four Anatomic Alterations “PROVe”,Pulmonary stenosis Right ventricular hypertrophy (RVH) Overidin

10、g aorta (overriding VSD) VSD,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Diagramatic representation of the anatomic abnormalities and typical shunt of Tetralogy of Fallot. Notice the RV hypertrophy, large VSD, over-riding aorta and the pulmonary stenosis. The shunt is a result of

11、 the large VSD (which serves to equalize pressures between the right and left ventricles) and the pulmonic stenosis which directs more blood to the systemic circulation.,Tetralogy of Fallot Pulmonary Stenosis,Obstruction of pulmonary outflow tract Variable anatomic reasons- most commonly narrowing o

12、f infundibulum (subpulmonary stenosis Pulmonary vavle or arteries may be narrowed This causes the right to left shunting VSD by itself will cause left to right shunts Pulmonary obstruction actually protects pulmonary vasculature from the great pressures of the left ventricle,Gross example of Tetrolo

13、gy of Fallot. The interior of the right ventricle is exposed showing a large VSD (D) and an aorta (A)straddling (ie over-riding) the VSD. A probe (arrow) is positioned inside of the stenotic subpulmonary infundibular channel.,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Tetralogy

14、of Fallot Right Ventricular Hypertrophy,Surprisingly the RVH rarely leads to right heart failure RV failure prevented by abililty to decompress into left ventricle.,This is an example of tetralogy of Fallot opened sagittally. The aorta and its valve (AV) clearly straddle the VSD (D) such that the ao

15、rta appears to arise equally from each ventricle. The right ventricular (RV) is clearly hypertrophic as it is nearly as thick as left ventricle (LV).,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Tetralogy of Fallot Overriding Aorta,Location of VSD puts aorta over both ventricles V

16、SDs upper border is made by aortic valve- Aorta then overrides both chambers ( see next diagram),This is an example of tetralogy of Fallot opened sagittally. The aorta and its valve (AV) clearly straddle the VSD (D) such that the aorta appears to arise equally from each ventricle. The right ventricu

17、lar (RV) is clearly hypertrophic as it is nearly as thick as left ventricle (LV).,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Tetralogy of Fallot VSD,Usually big- size of aortic orifice Located just below aortic valve,Gross example of Tetrology of Fallot. The interior of the righ

18、t ventricle is exposed showing a large VSD (D) and an aorta (A)straddling (ie over-riding) the VSD. A probe (arrow) is positioned inside of the stenotic subpulmonary infundibular channel.,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,The “boot-shaped” heart, an x-ray finding associ

19、ated with tetralogy of fallot (arrow at the “toe end” of the boot). A right sided aorta is present in 1/3 of tetralogy pts and when seen on chest films is virtually diagnostic of tetralogy.,Transposition of the great vessels,Definition- Ventriculoarterial discordance, such that the aorta and pulmona

20、ry artery origins are switched Produces seperation of the systemic and pulmonary circulations,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,An anterior view of the heart and lungs of a 2 month-old infant with transposition of the great vessels. The aorta and pulmonary trunk are eas

21、ily seen to be reversed here, with the aorta emanating from the right ventricle and the pulmonary trunk from the left. Also notice the widely patent ductus arteriosis (arrow) acting as an unstable shunt to allow some oxygenated blood to get from the pulmonary artery to the systemic circulation.,Edwa

22、rds, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Transposition of the Great VesselsPresentation,Present very early in life (may present later if good shunt, ie VSD) Cyanosis, may rapidly develop CHF ( dont see CHF in tetralogy ) Classic chest x-ray finding “EGG ON A STRING” Narrowed media

23、stinum ( the string ). Anterior-posterior relationship of the great vessels plus small thymus (stress),Transposition of the great vessels,All patients with transposition also have a shunt of some type-Transposition without a shunt is incompatible with life- Without a shunt there is no mixing of bloo

24、d ( no oxygenated blood would get into the systemic circulation),Transposition of the great vessels Shunts- ( two types ),Stable- 35% of pts. VSD Unstable (ie. They tend to close)- 60% of pts Patent foramen ovale Patent ductus arteriosis (PDA) These require speedy surgical shunt creation (balloon at

25、rial septoplasty),Diagrammatic representation of transposition of great vessels, with two shunts; a patent ductus arteriosis, and a large VSD. The presence of two shunts, particularly the stable VSD shunt will portend a better prognosis as there is better mixing of blood and less systemic hypoxia. D

26、espite having two shunts the patient with this abnormality would still require prompt surgery to correct the defect.,Transposition of the great vessels,Outlook All patients need surgery! Without surgery pts die within first few months of life in the vast majority of cases. Patholgic changes seen rel

27、ate to altered demands on each ventricular chamber Left ventricle will become atrophic- pumps into low-resistance pulmonary system Right ventrile hypertrophies- pumps into high-resistance systemic circulation,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,A sagittal section through

28、the ventricles of a heart with complete transposition of the great vessels. The anatomic switch of the aorta (A) and the pulmonary trunk(PT) is clearly evident. Also seen here is the damage done to the ventricles as a result of the altered demands placed on them. The RV has hypertrophied in response

29、 to its role in supplying the systemic circulation. The left ventricle in contrast has a very thin atrophic appearance. A patent ductus arteriosis (arrow) is present, and appears to be the sole supplier of oxygenated blood to the systemic circulation.,Left to Right shunts(Late cyanotic heart disease

30、s),VSD (32%) ASD (8%) PDA (8%),Left to Right shunts(Late cyanotic heart diseases),Malformations all overload right side of the heart resulting in problems. Pulmonary hypertension- Most important problem once this happens irreversible damage to the lung vasculature is done Surgical correction must be

31、 done before this happens!). Right ventricular hypertrophy (RVH) Shunt reverses (due to increased pulmonary resistance)-becomes a right to left shunt- thus the name “l(fā)ate cyanotic heart disease”.,ASD,3 Major types: Secundum type: 90% of ASDs, located in the mid-portion of the atrial septum. Primum t

32、ype: 5% of ASDs, located low in the atrial septum Sinus venosus type: 5% of ASDs, located high in the atrial septum, near the entrance of the superior vena cava.,Atrial Septal Defect(ASD),An abnormal opening in the atrial septum that allows free communication of blood between the right and left atri

33、a. The most common congenital cardiac anomaly presenting in adulthood Physical exam findings include: Widely split S2 (secondary to delayed pulmonic valve closure because of increased blood flow) May hear a systolic ejection murmur,ASD,ASDs result in a left to right shunt Pulmonary blood flow may in

34、crease up to 2-4 times normal Irreversible pulmonary hypertension develops in only 10% of patients lungs are tolerant of an increase in blood flow in contrast to an increase in pressure (as with a VSD).,ASDSymptoms,Small ASDs are virtually always asymptomatic. Isolated large ASDs are usually not sym

35、ptomatic before 30 years of age. Possible complications: Heart failure Irreversible pulmonary vascular disease Paradoxic embolization,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,Diagramatic representation of an atrial septal defect. Notice well oxyngenated blood shunting into the

36、 right atrium. From this diagram it is easy to see why people with an ASD may only present later in life.,Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965,This is a atrial septal defect(D.) of the fossa ovalis type (ostium secundum). This fossa ovalis type is by far the most common ty

37、pe of ASD. This picture is taken looking into the right atrium with the defect right in the mid-portion of the atrial wall. Strands of tissue may occasional cross an ASD as is seen here. You can see the defects location relative to the right and left pulmonary veins (R and L).,VSD,An abnormal openin

38、g in the ventricular system that allows free communication between the right and left ventricles. The most common congenital cardiac anomaly. Frequently associated with other structural defects (30% occur as isolated abnormalities). Symptoms may be present at birth, or not until later in life. Funct

39、ional significance depends on the size of the defect, and the presence or absence of pulmonary stenosis (these components determine pulmonary vascular pressure and resistance).,VSD,An abnormal opening in the ventricular system that allows free communication between the right and left ventricles. The most common congenital cardiac anomaly. Frequently associated with other structural defects (30% occur as isolated abnormalities). Symptoms may be present at birth, or not until later in life (depends on the size).,VSD