Congenita heart disease: ATRIAL AND VENTRICULAR SEPTAL DEFECTS: Ettinger Flashcards
(45 cards)
ATRIAL AND VENTRICULAR SEPTAL DEFECTS
During cardiac embryonic development the atria and ventricles begin as a common chamber. The heart is subsequently partitioned into the normal four-chambered heart by the growth of cardiac septa.15a The atria are partitioned by a wall formed mainly from two septa: the septum………….., which forms first, and the septum ………….., which develops to the ……………t of the septum primum. The ……………, a slitlike passageway, which persists between these septa, permits ………..to……….. atrial shunting in the fetus, but functionally and anatomically closes in the neonate when ……………. pressure rises.
The atria are partitioned by a wall formed mainly from two septa: the septum primum, which forms first, and the septum secundum, which develops to the right of the septum primum. The foramen ovale, a slitlike passageway, which persists between these septa, permits right-to-left atrial shunting in the fetus, but functionally and anatomically closes in the neonate when left atrial pressure rises.
The major portion of the ventricular septum forms by inward growth from the ventricular walls. The area of atrioventricular confluence, including the upper ventricular septum, lower atrial septum, and atrioventricular valves, is formed primarily by growth and differentiation of the ………………………..
The major portion of the ventricular septum forms by inward growth from the ventricular walls. The area of atrioventricular confluence, including the upper ventricular septum, lower atrial septum, and atrioventricular valves, is formed primarily by growth and differentiation of the endocardial cushions.
Defects in the development of the embryonic ventricular septum, the primum or secundum atrial septa, or the endocardial cushions, may result in atrial and/or ventricular septal defects. Congenital septal defects are common in both dogs and cats as isolated lesions and as components of more complex lesions such as tetralogy of Fallot.4a-13a,62a-79a
Defects in the development of the embryonic ventricular septum, the primum or secundum atrial septa, or the endocardial cushions, may result in atrial and/or ventricular septal defects. Congenital septal defects are common in both dogs and cats as isolated lesions and as components of more complex lesions such as tetralogy of Fallot.4a-13a,62a-79a
PATHOGENESIS
Except for the proven genetic basis of ventricular septal defect in Keeshonden with conotruncal malformation,124a there are no data on the cause(s) of spontaneous septal defects in dogs or cats.[28],4a Atrial septal defects (ASD) are usually classified based on the anatomic region of the malformation.15a,16a Defects at or near the foramen ovale are referred to as ostium (or septum) ………….. defects (Figure 249-11), while defects of the lower atrial septum are called ostium primum defects. Rarely observed ………………. ASD are found dorsocranial to the fossa ovalis near the entrance of the cranial vena cava.
PATHOGENESIS
Except for the proven genetic basis of ventricular septal defect in Keeshonden with conotruncal malformation,124a there are no data on the cause(s) of spontaneous septal defects in dogs or cats.[28],4a Atrial septal defects (ASD) are usually classified based on the anatomic region of the malformation.15a,16a Defects at or near the foramen ovale are referred to as ostium (or septum) secundum defects (Figure 249-11), while defects of the lower atrial septum are called ostium primum defects. Rarely observed sinus venosus ASD are found dorsocranial to the fossa ovalis near the entrance of the cranial vena cava.
Since the endocardial cushions are responsible for partitioning the lowermost atrial septum, defects in the region immediately adjacent to the AV valves (septum primum) are often included in the term “endocardial cushion defects.” A defect in this area may also include anomalous development of the atrioventricular valves such as a “cleft” in the septal leaflet of the mitral valve.
A complete endocardial cushion defect is a large defect of the lower atrial septum and upper ventricular septum with fusion of the septal leaflets of both AV valves. This is also referred to as an AV canal defect, since the embryonic atrioventricular canal area never partitions and there is communication between all four cardiac chambers.
Since the endocardial cushions are responsible for partitioning the lowermost atrial septum, defects in the region immediately adjacent to the AV valves (septum primum) are often included in the term “endocardial cushion defects.” A defect in this area may also include anomalous development of the atrioventricular valves such as a “cleft” in the septal leaflet of the mitral valve.
A complete endocardial cushion defect is a large defect of the lower atrial septum and upper ventricular septum with fusion of the septal leaflets of both AV valves. This is also referred to as an AV canal defect, since the embryonic atrioventricular canal area never partitions and there is communication between all four cardiac chambers.
Patent foramen ovale is not a true ASD inasmuch as the atrial septum forms normally but the walls of the foramen are pushed apart, usually by conditions which increase right atrial pressure. A patent foramen ovale achieves clinical significance when it allows right-to-left shunting, as may occur with severe pulmonic stenosis or tricuspid valve dysplasia.
Patent foramen ovale is not a true ASD inasmuch as the atrial septum forms normally but the walls of the foramen are pushed apart, usually by conditions which increase right atrial pressure. A patent foramen ovale achieves clinical significance when it allows right-to-left shunting, as may occur with severe pulmonic stenosis or tricuspid valve dysplasia.
Most ventricular septal defects (VSD) are located in the upper ventricular septum (Figure 249-12). Muscular apical or mid-ventricular septal defects are uncommon in small animals.
On the left side, the typical location of a VSD is just below the ……………, most often centered between the ………. ……………. and …………………. cusps.
Most ventricular septal defects (VSD) are located in the upper ventricular septum (Figure 249-12). Muscular apical or mid-ventricular septal defects are uncommon in small animals.
On the left side, the typical location of a VSD is just below the aortic valve, most often centered between the right coronary and noncoronary cusps.
On the right side, the opening is often described by its position relative to the crista supraventricularis muscular ridge. A subcristal (infracristal) VSD is located proximal to the crista supraventricularis near the cranial aspect of the septal leaflet of the tricuspid valve, which may partially cover it. A supracristal VSD is located distal to the crista supraventricularis just below the pulmonic valve.
On the right side, the opening is often described by its position relative to the crista supraventricularis muscular ridge. A subcristal (infracristal) VSD is located proximal to the crista supraventricularis near the cranial aspect of the septal leaflet of the tricuspid valve, which may partially cover it. A supracristal VSD is located distal to the crista supraventricularis just below the pulmonic valve.
Large defects may obliterate the crista and are usually associated with additional defects, as in tetralogy of Fallot (see Figure 249-12).2a,4a The right side of the root of the aorta, including the right coronary and noncoronary cusps, may be displaced to the right so that the aorta straddles the defect. The altered geometry of the aortic root that accompanies many VSDs sometimes results in substantial aortic valve regurgitation.17b
Large defects may obliterate the crista and are usually associated with additional defects, as in tetralogy of Fallot (see Figure 249-12).2a,4a The right side of the root of the aorta, including the right coronary and noncoronary cusps, may be displaced to the right so that the aorta straddles the defect. The altered geometry of the aortic root that accompanies many VSDs sometimes results in substantial aortic valve regurgitation.17b
PATHOPHYSIOLOGY
Shunting across small (resistive) defects depends primarily on the size of the communication and the pressure difference between the ……………., while shunting across large (nonresistive) defects depends primarily on …………..
Shunting across small (resistive) defects depends primarily on the size of the communication and the pressure difference between the two chambers, while shunting across large (nonresistive) defects depends primarily on relative resistances of the systemic and pulmonary circulations.
In the absence of other abnormalities, left heart pressures exceed those on the right and the direction of shunting is …………………
Cardiac chambers in the circuit of the shunt enlarge to accommodate the excess blood volume and the pulmonary vasculature is overcirculated. Large volume left-to-right shunts eventually result in myocardial failure, elevated filling pressures, and the development of overt congestive heart failure.
In the absence of other abnormalities, left heart pressures exceed those on the right and the direction of shunting is left-to-right.
Cardiac chambers in the circuit of the shunt enlarge to accommodate the excess blood volume and the pulmonary vasculature is overcirculated. Large volume left-to-right shunts eventually result in myocardial failure, elevated filling pressures, and the development of overt congestive heart failure.
Right-to-left shunting occurs via a septal defect when …………,…………,or…………….. (3) raise pressures on the right side of the heart. The consequences of “reversed” shunting include cyanosis from arterial ……………., ………………… ……………….., and sudden death.
Right-to-left shunting occurs via a septal defect when pulmonic stenosis, tricuspid dysplasia, or pulmonary arterial hypertension raise pressures on the right side of the heart. The consequences of “reversed” shunting include cyanosis from arterial hypoxemia, polycythemia, hyperviscosity, and sudden death.
Atrial Septal Defect
Flow across an ASD occurs primarily during ……………. The pressure difference across the defect is ……. and the direction and magnitude of the shunt is determined mainly by the relative ……….. resistance to inflow for each ventricle.
Normally, the right ventricle is more compliant than the left and offers little resistance to filling, causing blood to preferentially shunt from the left atrium into the right atrium and ventricle. The result is ………….. of the right atrium, …………… hypertrophy of the right ventricle, and pulmonary …………………
Atrial Septal Defect
Flow across an ASD occurs primarily during diastole. The pressure difference across the defect is low and the direction and magnitude of the shunt is determined mainly by the relative diastolic resistance to inflow for each ventricle.
Normally, the right ventricle is more compliant than the left and offers little resistance to filling, causing blood to preferentially shunt from the left atrium into the right atrium and ventricle. The result is dilation of the right atrium, eccentric hypertrophy of the right ventricle, and pulmonary overcirculation.
ASD: …………… saturation in the right heart and pulmonary arteries is increased. The …………atrium receives the shunted blood, but most of the increased pulmonary venous return is shunted immediately into the right ………….., resulting in minimal ………………..
Oxygen saturation in the right heart and pulmonary arteries is increased. The left atrium receives the shunted blood, but most of the increased pulmonary venous return is shunted immediately into the right atrium, resulting in minimal left atrial dilatation.
If considerable left atrial enlargement is observed in an animal with an ASD, an additional defect, such as an …………………………… defect with mitral regurgitation, should be suspected. ………………………….. defects are more commonly detected in cats and may cause left sided or bilateral congestive heart failure.
If considerable left atrial enlargement is observed in an animal with an ASD, an additional defect, such as an endocardial cushion defect with mitral regurgitation, should be suspected. Endocardial cushion defects are more commonly detected in cats and may cause left sided or bilateral congestive heart failure. Endocardial cushion defects are reported in dogs, but are rare.
The flow across an ASD does not usually generate an audible heart murmur because the pressure gradient and flow velocity across the defect are …….. When the shunted blood joins with blood entering from the ……………, the volume and velocity of flow through the right heart is increased resulting a murmur of relative …………….(common) or …………………..(uncommon). Delayed closure of the ……………… (and ………………… closure of the aortic valve) causes splitting of the second heart sound.
Since the volume overload affects the…… ventricle and not the ….. large shunts culminate in the development of …….. heart failure.
The flow across an ASD does not usually generate an audible heart murmur because the pressure gradient and flow velocity across the defect are low. When the shunted blood joins with blood entering from the vena cava, the volume and velocity of flow through the right heart is increased resulting a murmur of relative pulmonic stenosis (common) or tricuspid stenosis (uncommon). Delayed closure of the pulmonic valve (and early closure of the aortic valve) causes splitting of the second heart sound.
Since the volume overload affects the right ventricle and not the left, large shunts culminate in the development of right heart failure.
Ventricular Septal Defect
Flow across VSD occur primarily during ventricular ……….. In the absence of other cardiovascular defects LV systolic pressure is …………….. times that of the right ventricle and flow proceeds from the left to the right ventricle. The magnitude of left-to-right shunting with small (resistive) defects is mainly determined by the …………….. of the defect and the systolic ……………… (…………….) between the …………….
Ventricular Septal Defect
Flow across VSD occur primarily during ventricular systole. In the absence of other cardiovascular defects LV systolic pressure is 4 to 5 times that of the right ventricle and flow proceeds from the left to the right ventricle. The magnitude of left-to-right shunting with small (resistive) defects is mainly determined by the diameter of the defect and the systolic pressure difference (gradient) between the ventricles.
The peak pressure difference across the defect can be estimated noninvasively by Doppler echocardiography. Using the peak flow velocity (meters/second) of the blood flow passing through the defect, the pressure gradient is calculated using the simplified Bernoulli equation (ΔP = 4V2). A resistive defect with normal right and left ventricular pressures (approximately 20 mm Hg and 100 mm Hg, respectively) is expected to have a peak jet velocity of …………. than …….m/s, corresponding to a peak pressure gradient across the defect of greater than ……mm Hg.
The peak pressure difference across the defect can be estimated noninvasively by Doppler echocardiography. Using the peak flow velocity (meters/second) of the blood flow passing through the defect, the pressure gradient is calculated using the simplified Bernoulli equation (ΔP = 4V2). A resistive defect with normal right and left ventricular pressures (approximately 20 mm Hg and 100 mm Hg, respectively) is expected to have a peak jet velocity of greater than 4.5 m/s, corresponding to a peak pressure gradient across the defect of greater than 80 mm Hg.
If the peak velocity is lower than predicted, right ventricular ………………. pressure is most likely increased, caused either by the presence of ……………….. or increased ……………………… (pulmonary arterial hypertension).
If the peak velocity is lower than predicted, right ventricular systolic pressure is most likely increased, caused either by the presence of pulmonic stenosis or increased pulmonary vascular resistance (pulmonary arterial hypertension).
When a small VSD is located high in the membranous septum, blood is ejected by the left ventricle directly into the RV outflow tract and out the main pulmonary artery: the right heart experiences only a very modest volume load and right heart enlargement is minimal.
When a small VSD is located high in the membranous septum, blood is ejected by the left ventricle directly into the RV outflow tract and out the main pulmonary artery: the right heart experiences only a very modest volume load and right heart enlargement is minimal.
Right heart enlargement is more prominent when the VSD is …………… or located in the …………….. region of the ………………. septum.
Right heart enlargement is more prominent when the VSD is large or located in the muscular region of the interventricular septum.
With a typical, small, high VSD, oxygen saturation in the right ventricular outflow tract (RVOT) and pulmonary artery is higher than in the ………………..
Large shunts (pulmonary to systemic flow ratio greater than … : …) may overload the left and/or right heart enough to increase ventricular …………….. pressures and cause signs of left, right or biventricular failure. Very large, nonresistive VSDs cause the pressures in both ventricles to equilibrate and the two ventricles behave as a common pumping chamber.
Unless the pulmonary circulation is protected by a …………………. valve, the development of pulmonary hypertension is unavoidable.
With a typical, small, high VSD, oxygen saturation in the right ventricular outflow tract (RVOT) and pulmonary artery is higher than in the right atrium or apex of the right ventricle. Large shunts (pulmonary to systemic flow ratio greater than 3 : 1) may overload the left and/or right heart enough to increase ventricular diastolic pressures and cause signs of left, right or biventricular failure. Very large, nonresistive VSDs cause the pressures in both ventricles to equilibrate and the two ventricles behave as a common pumping chamber.
Unless the pulmonary circulation is protected by a stenotic pulmonic valve, the development of pulmonary hypertension is unavoidable.
Eisenmenger’s Syndrome
In newborn animals with a large VSD, pulmonary …………….may not decline after birth, resulting in sustained pulmonary arterial hypertension. If right ventricular ……………..and ………………. pressures equilibrate at or above systemic (left ventricular) levels, bidirectional or right-to-left shunting may occur.
The development of pulmonary hypertension associated with a shunting cardiac defect is known as Eisenmenger’s physiology or syndrome. When the defect is a VSD, the term “Eisenmenger complex” is often employed.
Eisenmenger’s Syndrome
In newborn animals with a large VSD, pulmonary vascular resistance may not decline after birth, resulting in sustained pulmonary arterial hypertension.13b,14b If right ventricular systolic and diastolic pressures equilibrate at or above systemic (left ventricular) levels, bidirectional or right-to-left shunting may occur. The development of pulmonary hypertension associated with a shunting cardiac defect is known as Eisenmenger’s physiology or syndrome. When the defect is a VSD, the term “Eisenmenger complex” is often employed.
Sustained pulmonary hypertension is characterized by progressive and irreversible changes in the pulmonary ………….. similar to those mentioned in the section on reversed PDA.
As in dogs with a PDA, reversed (right to left) shunting through a VSD is usually established before ……. months of age. In contrast to human patients, gradual development of pulmonary vascular disease and progressive pulmonary hypertension is relatively common in dogs and cats.
Sustained pulmonary hypertension is characterized by progressive and irreversible changes in the pulmonary arteries similar to those mentioned in the section on reversed PDA.
As in dogs with a PDA, reversed (right to left) shunting through a VSD is usually established before 6 months of age. In contrast to human patients, gradual development of pulmonary vascular disease and progressive pulmonary hypertension is relatively common in dogs and cats.