Congenital heart disease: PDA-Ettinger Flashcards
(52 cards)
DEFECTS CAUSING PRIMARILY VOLUME OVERLOAD
SYSTEMIC TO PULMONARY (LEFT-TO-RIGHT) SHUNTS:
The circulation in the fetus differs from that in the adult. In fetal animals the ductus arteriosus develops from the left sixth embryonic arch and extends from the pulmonary artery to the descending aorta where it functions to divert blood from the nonfunctional fetal lungs back into the systemic circulation.
Before birth, the ductus diverts approximately 80% to 90% of the right ventricular output back to the left side of the circulation. Following parturition and the onset of breathing, pulmonary vascular ………….. falls, flow in the ductus ……………, and the resulting rise in arterial ……………….. inhibits …….. …………. release causing …………… of the vascular smooth muscle within the vessel wall and functional closure of the ductus arteriosus.
While the ductus may be probe-patent in puppies less than ….. days of age, it is usually closed securely …….to……… days after birth. a Persistence of a PDA beyond the early neonatal period is the first or second most commonly diagnosed congenital cardiac defect in dogs.
DEFECTS CAUSING PRIMARILY VOLUME OVERLOAD
SYSTEMIC TO PULMONARY (LEFT-TO-RIGHT) SHUNTS:
The circulation in the fetus differs from that in the adult. In fetal animals the ductus arteriosus develops from the left sixth embryonic arch and extends from the pulmonary artery to the descending aorta where it functions to divert blood from the nonfunctional fetal lungs back into the systemic circulation.
Before birth, the ductus diverts approximately 80% to 90% of the right ventricular output back to the left side of the circulation. Following parturition and the onset of breathing, pulmonary vascular ………….. falls, flow in the ductus ……………, and the resulting rise in arterial ……………….. inhibits …….. …………. release causing …………… of the vascular smooth muscle within the vessel wall and functional closure of the ductus arteriosus.
While the ductus may be probe-patent in puppies less than ….. days of age, it is usually closed securely …….to……… days after birth. a Persistence of a PDA beyond the early neonatal period is the first or second most commonly diagnosed congenital cardiac defect in dogs.
PATHOGENESIS
Failed ductal closure in dogs is characterized by distinct histologic abnormalities within the ductal wall. The normal fetal ductal wall contains a loose branching pattern of circumferential …………… throughout its length. In prenatal puppies bred to have a high probability of PDA, varying portions of the ductal wall are comprised of ………………….. rather than contractile smooth muscle fibers.
According to the work of Patterson and Buchanan et al.,4a increasing genetic liability to PDA results in “extension of the noncontractile wall structure of the aorta to an increasing segment of the ductus arteriosus, progressively impairing its capacity to undergo physiologic closure.” It is tempting to speculate that some defect prevents one or more of the series of processes that permits smooth muscle cells to proliferate in the wall of ductus before birth.
PATHOGENESIS
Failed ductal closure in dogs is characterized by distinct histologic abnormalities within the ductal wall. The normal fetal ductal wall contains a loose branching pattern of circumferential smooth muscle throughout its length. In prenatal puppies bred to have a high probability of PDA, varying portions of the ductal wall are comprised of elastic fibers rather than contractile smooth muscle fibers.
According to the work of Patterson and Buchanan et al.,4a increasing genetic liability to PDA results in “extension of the noncontractile wall structure of the aorta to an increasing segment of the ductus arteriosus, progressively impairing its capacity to undergo physiologic closure.” It is tempting to speculate that some defect prevents one or more of the series of processes that permits smooth muscle cells to proliferate in the wall of ductus before birth.
In its mildest form, the ductus closes at the pulmonary arterial end only and there is a blind, funnel-shaped outpouching of the ventral aspect of the aorta, called a …………………… This hidden form (forme fruste) of incomplete ductal closure can only be diagnosed by angiography or necropsy, but it indicates that the dog possesses genes for this defect.4a,39a-40a
In its mildest form, the ductus closes at the pulmonary arterial end only and there is a blind, funnel-shaped outpouching of the ventral aspect of the aorta, called a ductus diverticulum. This hidden form (forme fruste) of incomplete ductal closure can only be diagnosed by angiography or necropsy, but it indicates that the dog possesses genes for this defect.4a,39a-40a
Increasing genetic liability results in a tapering, funnel-shaped ductus arteriosus that remains patent after the early natal period and allows blood to flow from the high-pressure aorta to low-pressure pulmonary artery (Figure 249-2) and varying amounts of left-to-right shunting. The most severe, but least common, form is the cylindrical, nontapering ductus with persistent, postnatal pulmonary hypertension (Eisenmenger syndrome) and bidirectional or right-to-left shunting (see Figure 249-2).
Increasing genetic liability results in a tapering, funnel-shaped ductus arteriosus that remains patent after the early natal period and allows blood to flow from the high-pressure aorta to low-pressure pulmonary artery (Figure 249-2) and varying amounts of left-to-right shunting. The most severe, but least common, form is the cylindrical, nontapering ductus with persistent, postnatal pulmonary hypertension (Eisenmenger syndrome) and bidirectional or right-to-left shunting (see Figure 249-2).
Based on the breeding studies of Poodle-type dogs, Patterson et al. concluded that the mode of transmission of PDA in dogs is most likely polygenic but other mechanisms of inheritance are possible. For additional information regarding the morphology and pathogenesis of PDA, the reader is referred to several outstanding reviews of the subject.[12-14]
Based on the breeding studies of Poodle-type dogs, Patterson et al. concluded that the mode of transmission of PDA in dogs is most likely polygenic but other mechanisms of inheritance are possible. For additional information regarding the morphology and pathogenesis of PDA, the reader is referred to several outstanding reviews of the subject.[12-14]
PATHOPHYSIOLOGY
The direction of flow through the PDA is determined by the relative …………….. of the pulmonary and systemic vascular beds and is, in the vast majority of cases, directed from left-to-right (from the aorta to pulmonary artery). This results in a continuous cardiac murmur, increased pulmonary blood flow, and volume overloading of the ….. atrium and left ventricle.
PATHOPHYSIOLOGY
The direction of flow through the PDA is determined by the relative resistances of the pulmonary and systemic vascular beds and is, in the vast majority of cases, directed from left-to-right (from the aorta to pulmonary artery). This results in a continuous cardiac murmur, increased pulmonary blood flow, and volume overloading of the left atrium and left ventricle.
Because of the relatively …………………….. of the systemic circulation after birth, ………………..blood pressure is greater than pulmonary pressure, and blood shunts continuously across the PDA during both systole and diastole.
For a given pressure gradient, the magnitude of shunting is determined by the …………………….. (effective …………………) of the ductus arteriosus. In the majority of cases the ductus is widest at the aortic end and tapers to its narrowest (flow limiting) region at the point of attachment to the pulmonary artery. Increased left ventricular ………………. and rapid run-off of blood from the aorta to the low-pressure pulmonary circulation via the PDA causes increased aortic ………… and decreased aortic …………….. pressures. The resulting widened arterial …………….. offers a hemodynamic explanation for the bounding or hyperkinetic (waterhammer, Corrigan’s) arterial pulse detected in dogs with substantial shunts.
Because of the relatively high resistance of the systemic circulation after birth, aortic blood pressure is greater than pulmonary pressure, and blood shunts continuously across the PDA during both systole and diastole.
For a given pressure gradient, the magnitude of shunting is determined by the morphology (effective resistance) of the ductus arteriosus. In the majority of cases the ductus is widest at the aortic end and tapers to its narrowest (flow limiting) region at the point of attachment to the pulmonary artery. Increased left ventricular stroke volume and rapid run-off of blood from the aorta to the low-pressure pulmonary circulation via the PDA causes increased aortic systolic and decreased aortic diastolic pressures. The resulting widened arterial pulse pressure offers a hemodynamic explanation for the bounding or hyperkinetic (waterhammer, Corrigan’s) arterial pulse detected in dogs with substantial shunts.
All vascular structures involved in the transport of the shunted blunt enlarge to accommodate the extra volume flow. Increased volume flow causes dilatation of the ………………. aorta, main ………………., and overcirculation of the …………………..bed.
Dilation of the left ………… and eccentric hypertrophy of the …………………. develop in proportion to the volume flow of the shunt.
This mechanism permits compensation for a variable period of time but if the shunt is large, myocardial failure (cardiomyopathy of volume overload) develops together with progressive elevation of left ventricular ……………-………….. and overt pulmonary …………..
All vascular structures involved in the transport of the shunted blunt enlarge to accommodate the extra volume flow. Increased volume flow causes dilatation of the proximal aorta, main pulmonary artery, and overcirculation of the pulmonary vascular bed. Dilation of the left atrium and eccentric hypertrophy of the left ventricle develop in proportion to the volume flow of the shunt. This mechanism permits compensation for a variable period of time but if the shunt is large, myocardial failure (cardiomyopathy of volume overload) develops together with progressive elevation of left ventricular end-diastolic pressure and overt pulmonary edema.
Because the left-to-right shunt occurs at the level of the great vessels, the right ventricle and atrium never handle the shunted blood and these structures remain normal unless …………………… and ………………………. increase.
Because the left-to-right shunt occurs at the level of the great vessels, the right ventricle and atrium never handle the shunted blood and these structures remain normal unless pulmonary vascular resistance and pulmonary arterial pressure increase.
In a small percentage of cases, the lumen of the PDA remains wide open after birth. The absence of a …………….ductal orifice allows aortic pressure to be transmitted to the pulmonary circulation, precluding the normal postnatal ………………. in pulmonary vascular resistance. In this circumstance, the aortic and pulmonary artery pressures equilibrate and the right ventricle remains ………………………… hypertrophied after birth.
In a small percentage of cases, the lumen of the PDA remains wide open after birth. The absence of a restrictive ductal orifice allows aortic pressure to be transmitted to the pulmonary circulation, precluding the normal postnatal decline in pulmonary vascular resistance. In this circumstance, the aortic and pulmonary artery pressures equilibrate and the right ventricle remains concentrically hypertrophied after birth.
In Patterson’s colony of dogs, this pattern of pulmonary hypertension and reversed (right-to-left) shunting developed within the first few weeks of life.39a These observations fit the usual clinical presentation of most dogs diagnosed with a (reversed) PDA, in which there is usually no history of a continuous murmur and no evidence of left ventricular enlargement or large left-to-right shunt earlier in life..
Most dogs with reversed PDA flow exhibit ………………..pulmonary blood flow, a normal to small……………..ventricle, and marked …………………. hypertrophy of the right ventricle.
On rare occasions, dogs with a moderate to large left-to-right shunting PDA will experience a gradual increase in pulmonary resistance and gradual reversal of the direction of shunting, typically at several months to several years of age.
Most dogs with reversed PDA flow exhibit diminished pulmonary blood flow, a normal to small left ventricle, and marked concentric hypertrophy of the right ventricle.
On rare occasions, dogs with a moderate to large left-to-right shunting PDA will experience a gradual increase in pulmonary resistance and gradual reversal of the direction of shunting, typically at several months to several years of age.
In these dogs there is often a history of prior left heart failure. Substantial residual LV enlargement is evident on thoracic radiographs and by echocardiography. Pulmonary blood flow is reduced but right ventricular hypertrophy is less pronounced than in dogs that reverse the direction of shunting at an early age.
The precise pathogenesis of pulmonary hypertension is not completely understood, but anatomic descriptions of the pulmonary vasculature are similar in humans and animals. Histologic changes within small pulmonary arteries include hypertrophy of the ………., thickening of the ………. and reduction of lumen …………….., and development of ………………….lesions of the vessel wall. Most of these changes are considered to be ………………, precluding surgical correction of the reversed PDA.
The precise pathogenesis of pulmonary hypertension is not completely understood, but anatomic descriptions of the pulmonary vasculature are similar in humans and animals. Histologic changes within small pulmonary arteries include hypertrophy of the media, thickening of the intima and reduction of lumen dimensions, and development of plexiform lesions of the vessel wall. Most of these changes are considered to be irreversible, precluding surgical correction of the reversed PDA.
CLINICAL FINDINGS
The clinical features of PDA have been thoroughly characterized in both a breeding colony and in clinic populations.[16],1a-4a,37a-53a,10c Compared with male dogs, female dogs have substantially greater risk (2.49/1000 versus 1.45/1000) of developing a PDA.2a The Chihuahua, Collie, Maltese, Poodle, Pomeranian, English Springer Spaniel, Keeshond, Bichon Frise, and Shetland Sheepdog are most frequently affected, although other breeds such as the Cavalier King Charles Spaniel may also be predisposed. Many other breeds, including larger dogs such as the German Shepherd Dog, Newfoundland, and Labrador Retriever may also be prone to PDA in some regions. While severely affected pups and kittens may appear stunted, thin, or tachypneic from left heart failure, most are reported to be asymptomatic and developing normally at the time of their initial recognition. Clinical signs are rarely recognized within the first few weeks of life, and most dogs are not diagnosed until the time of initial examination at 6 to 8 weeks of age.
CLINICAL FINDINGS
The clinical features of PDA have been thoroughly characterized in both a breeding colony and in clinic populations.[16],1a-4a,37a-53a,10c Compared with male dogs, female dogs have substantially greater risk (2.49/1000 versus 1.45/1000) of developing a PDA.2a The Chihuahua, Collie, Maltese, Poodle, Pomeranian, English Springer Spaniel, Keeshond, Bichon Frise, and Shetland Sheepdog are most frequently affected, although other breeds such as the Cavalier King Charles Spaniel may also be predisposed. Many other breeds, including larger dogs such as the German Shepherd Dog, Newfoundland, and Labrador Retriever may also be prone to PDA in some regions. While severely affected pups and kittens may appear stunted, thin, or tachypneic from left heart failure, most are reported to be asymptomatic and developing normally at the time of their initial recognition. Clinical signs are rarely recognized within the first few weeks of life, and most dogs are not diagnosed until the time of initial examination at 6 to 8 weeks of age.
CLINICAL FINDINGS
The clinical features of PDA have been thoroughly characterized in both a breeding colony and in clinic populations.[16],1a-4a,37a-53a,10c Compared with male dogs, female dogs have substantially greater risk (2.49/1000 versus 1.45/1000) of developing a PDA.2a The Chihuahua, Collie, Maltese, Poodle, Pomeranian, English Springer Spaniel, Keeshond, Bichon Frise, and Shetland Sheepdog are most frequently affected, although other breeds such as the Cavalier King Charles Spaniel may also be predisposed. Many other breeds, including larger dogs such as the German Shepherd Dog, Newfoundland, and Labrador Retriever may also be prone to PDA in some regions. While severely affected pups and kittens may appear stunted, thin, or tachypneic from left heart failure, most are reported to be asymptomatic and developing normally at the time of their initial recognition. Clinical signs are rarely recognized within the first few weeks of life, and most dogs are not diagnosed until the time of initial examination at 6 to 8 weeks of age.
CLINICAL FINDINGS
The clinical features of PDA have been thoroughly characterized in both a breeding colony and in clinic populations.[16],1a-4a,37a-53a,10c Compared with male dogs, female dogs have substantially greater risk (2.49/1000 versus 1.45/1000) of developing a PDA.2a The Chihuahua, Collie, Maltese, Poodle, Pomeranian, English Springer Spaniel, Keeshond, Bichon Frise, and Shetland Sheepdog are most frequently affected, although other breeds such as the Cavalier King Charles Spaniel may also be predisposed. Many other breeds, including larger dogs such as the German Shepherd Dog, Newfoundland, and Labrador Retriever may also be prone to PDA in some regions. While severely affected pups and kittens may appear stunted, thin, or tachypneic from left heart failure, most are reported to be asymptomatic and developing normally at the time of their initial recognition. Clinical signs are rarely recognized within the first few weeks of life, and most dogs are not diagnosed until the time of initial examination at 6 to 8 weeks of age.
Left-to-Right Shunting PDA
A thorough physical examination and chest radiographs usually suffice to suggest the diagnosis. Mucous membranes are pink in the absence of heart failure. The precordial impulse is often exaggerated and more diffuse than normal because of left ventricular enlargement. A thrill may be palpated at the heart base and a continuous murmur is best heard in the same location (Figure 249-3). The murmur’s point of maximum intensity is located over the main pulmonary artery at the dorsocranial left heart base, and it may radiate cranially to the thoracic inlet and to the right base, where it is almost always softer.[16],5a,39a,47a Often, only a systolic murmur is audible over the mitral area. This murmur may simply represent radiation of the loudest portion of the continuous murmur from the heart base to this location or may indicate that secondary mitral regurgitation has developed as a consequence of severe left ventricular dilatation.
Left-to-Right Shunting PDA
A thorough physical examination and chest radiographs usually suffice to suggest the diagnosis. Mucous membranes are pink in the absence of heart failure. The precordial impulse is often exaggerated and more diffuse than normal because of left ventricular enlargement. A thrill may be palpated at the heart base and a continuous murmur is best heard in the same location (Figure 249-3). The murmur’s point of maximum intensity is located over the main pulmonary artery at the dorsocranial left heart base, and it may radiate cranially to the thoracic inlet and to the right base, where it is almost always softer.[16],5a,39a,47a Often, only a systolic murmur is audible over the mitral area. This murmur may simply represent radiation of the loudest portion of the continuous murmur from the heart base to this location or may indicate that secondary mitral regurgitation has developed as a consequence of severe left ventricular dilatation.
In cats, the continuous murmur of a PDA may be heard best somewhat more caudoventrally than in affected dogs. Increased LV stroke volume and rapid diastolic run-off through the PDA combine to produce peripheral arterial pulses that are hyperkinetic (bounding).
In cats, the continuous murmur of a PDA may be heard best somewhat more caudoventrally than in affected dogs. Increased LV stroke volume and rapid diastolic run-off through the PDA combine to produce peripheral arterial pulses that are hyperkinetic (bounding).
Figure 249-3 Phonocardiogram recorded at the left heart base from a dog with left-to-right patent ductus arteriosus. The lead II electrocardiogram is recorded simultaneously for timing purposes (ventricular mechanical systole is approximately the period from the middle of the QRS complex to the end of the T wave; the remainder of the time is diastole). The recorded murmur is continuous, increasing in intensity during systole, peaking near the end of systole, and decreasing in intensity during diastole.
Figure 249-3 Phonocardiogram recorded at the left heart base from a dog with left-to-right patent ductus arteriosus. The lead II electrocardiogram is recorded simultaneously for timing purposes (ventricular mechanical systole is approximately the period from the middle of the QRS complex to the end of the T wave; the remainder of the time is diastole). The recorded murmur is continuous, increasing in intensity during systole, peaking near the end of systole, and decreasing in intensity during diastole.
Electrocardiography typically indicates left ventricular enlargement (increased R-wave voltages in leads II, III, aVF and in the left chest leads, V2 and V4) and normal mean electrical axis (Figure 249-4). Widened P waves may also be found, indicating left atrial enlargement.
Electrocardiography typically indicates left ventricular enlargement (increased R-wave voltages in leads II, III, aVF and in the left chest leads, V2 and V4) and normal mean electrical axis (Figure 249-4). Widened P waves may also be found, indicating left atrial enlargement.
Chest radiographs indicate left atrial and left ventricular enlargement and pulmonary hypervascularity in proportion to the magnitude of the left-to-right shunt (Figure 249-5). On the dorsoventral projection, the aortic arch, left auricle, and main pulmonary artery may be abnormally prominent. The most specific radiographic finding is the appearance of an aortic bulge (“ductus bump”) near the origin of the ductus, which is caused by abrupt narrowing of the descending aorta just caudal to the ductus origin (see Figure 249-5). Moderate to severe LV enlargement sometimes causes the cardiac apex to shift to the right (common in cats).
Chest radiographs indicate left atrial and left ventricular enlargement and pulmonary hypervascularity in proportion to the magnitude of the left-to-right shunt (Figure 249-5). On the dorsoventral projection, the aortic arch, left auricle, and main pulmonary artery may be abnormally prominent. The most specific radiographic finding is the appearance of an aortic bulge (“ductus bump”) near the origin of the ductus, which is caused by abrupt narrowing of the descending aorta just caudal to the ductus origin (see Figure 249-5). Moderate to severe LV enlargement sometimes causes the cardiac apex to shift to the right (common in cats).
Figure 249-4 Electrocardiogram from a dog with left-to-right patent ductus arteriosus. The mean QRS axis is normal (+80%), but the QRS voltage (amplitude) is increased in several leads, including leads II, III, aVF
Figure 249-4 Electrocardiogram from a dog with left-to-right patent ductus arteriosus. The mean QRS axis is normal (+80%), but the QRS voltage (amplitude) is increased in several leads, including leads II, III, aVF
Figure 249-5 Thoracic radiographs from a dog with a left-to-right shunting patent ductus arteriosus. A, Lateral projection shows left atrial enlargement and enlarged cranial pulmonary arteries and veins. B, Dorsoventral projection shows moderate cardiomegaly, a characteristic bulge in the descending aorta and dilation of the main pulmonary artery. The pulmonary vasculature is prominent.
Figure 249-5 Thoracic radiographs from a dog with a left-to-right shunting patent ductus arteriosus. A, Lateral projection shows left atrial enlargement and enlarged cranial pulmonary arteries and veins. B, Dorsoventral projection shows moderate cardiomegaly, a characteristic bulge in the descending aorta and dilation of the main pulmonary artery. The pulmonary vasculature is prominent.
The diagnosis of a PDA can be confirmed by echocardiography in almost all cases. 2D and M-mode echocardiography demonstrate eccentric LV hypertrophy and dilatation of the left atrium, ascending aorta, and pulmonary artery (Figure 249-6).11c Reduced myocardial contractility is often observed and is reflected by reduced fractional shortening and/or increased LV end-systolic dimension and EPSS measurements.
The diagnosis of a PDA can be confirmed by echocardiography in almost all cases. 2D and M-mode echocardiography demonstrate eccentric LV hypertrophy and dilatation of the left atrium, ascending aorta, and pulmonary artery (Figure 249-6).11c Reduced myocardial contractility is often observed and is reflected by reduced fractional shortening and/or increased LV end-systolic dimension and EPSS measurements.
The ductus can usually be imaged from the left cranial parasternal window (see Figure 249-6). Doppler interrogation of the pulmonary artery consistently demonstrates high-velocity continuous ductal flow directed toward the pulmonic valve (see Figure 249-6). In the typical case, the peak velocity of this jet is about 4.5 to 5.0 m/s and occurs at end-systole. Other common echocardiographic findings include mildly increased LV outflow velocity (1.8 to 2.3 m/s) and modest secondary mitral and pulmonary valve insufficiency.
The ductus can usually be imaged from the left cranial parasternal window (see Figure 249-6). Doppler interrogation of the pulmonary artery consistently demonstrates high-velocity continuous ductal flow directed toward the pulmonic valve (see Figure 249-6). In the typical case, the peak velocity of this jet is about 4.5 to 5.0 m/s and occurs at end-systole. Other common echocardiographic findings include mildly increased LV outflow velocity (1.8 to 2.3 m/s) and modest secondary mitral and pulmonary valve insufficiency.
In dogs with PDA, associated cardiac defects are uncommon; nonetheless, it is still worthwhile to exclude, via a carefully performed echocardiographic examination, the concurrent presence of other common congenital defects such as subaortic stenosis.4a,44a
In dogs with PDA, associated cardiac defects are uncommon; nonetheless, it is still worthwhile to exclude, via a carefully performed echocardiographic examination, the concurrent presence of other common congenital defects such as subaortic stenosis.4a,44a