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Tetralogy of Fallot

The combination of pulmonary stenosis, right ventricular hypertrophy, over-riding aorta, and ventricular septal defect creates a congenital anomaly call Tetralogy of Fallot. When the pulmonic stenosis is severe enough to elevate right-sided systolic pressures beyond those found within the left ventricle and systemic circulation, the blood will flow from right to left through the ventricular septal defect. Not all animals with Tetralogy of Fallot however have right to left shunting, and acyanotic tetralogies can occur. Several canine breeds are predisposed to the development of this congenital defect including Keeshonds and bulldogs (28,130). Tetralogy of Fallot is also a reported complex congenital defect in horses and cats (13,33,111,131,132).

Tetralogy of Fallot

Components

Right ventricular hypertrophy

Pulmonary stenosis

VSD

Over riding aorta

The pulmonic stenosis in animals with Tetralogy of Fallot is similar to isolated pulmonic stenosis. The defect may be valvular, subvalvular, or both. The majority of dogs appear to have valvular stenosis (133). Hypoplasia of the pulmonary artery is often present, and a poststenotic dilation is absent in most of these cases (Figure 10.55) (134).

Figure 10.55 Hypoplasia of the pulmonary artery is a typical form of stenosis in tetralogy of Fallot, and a poststenotic dilation is absent in most of these cases. The ventricular septal defect (small arrow) is seen in this imaging plane. Plane = right parasternal transverse heart base, RVW = right ventricular wall, RV = right ventricle, PV = pulmonary valve, PA = pulmonary artery, RA = right atrium, TV = tricuspid valve.

Spectral Doppler of pulmonary flow can document the degree of obstruction (53). Pressure gradients in excess of 100 are consistent with right to left shunting or bidirectional shunting. When severe subvalvular stenosis or hypoplasia of the pulmonary valve annulus is present, it is sometimes difficult

to obtain an adequate image of the pulmonary outflow tract and valve. Spectral Doppler of pulmonary artery flow is also often difficult to obtain in these animals. Color-flow Doppler will delineate the boundaries of the outflow tract and may help position the Doppler cursor. Examine all four views for obtaining the pulmonary artery. If pulmonary flow cannot be obtained, check for tricuspid insufficiency as an alternative way to derive right ventricular pressures.

Severe right ventricular hypertrophy is always present as a consequence of the pressure overload generated by the pulmonic stenosis (34,133). The right ventricular cavity is usually small unless valvular regurgitations are present. Left ventricular chamber size is also small secondary to poor preload when Tetralogy of Fallot is present regardless of whether the shunt is right to left or left to right. The left ventricle will appear to be concentrically hypertrophied, and invasive studies of left ventricular pressures in dogs with Tetralogy of Fallot reveal left ventricular pressures as low as 36 in some (34,133). A left to right shunt would typically volume overload the left ventricle and sometimes the right ventricle, but when a moderate to severe pulmonic stenosis is present, there is decreased volume flowing through the pulmonary circulation and into the left side of the heart. Although logically a right to left shunt should volume overload the left ventricle, the overriding aorta causes the shunted blood to flow directly into the aorta, and the left ventricle never receives the volume. The severe pulmonic stenosis, which must be present with right to left shunts, additionally results in decreased preload within the left ventricular chamber.

Tetralogy of Fallot

LV concentric hypertrophy pattern

Pseudohypertrophy

Due to decreased preload

The ventricular septal defect is usually very obvious and large. The interventricular septum is aligned with the center of the aorta (Figure 10.56). Only when septal hypertrophy is very severe will the defect become less obvious. Manipulating the transducer and sound beam in slightly different directions can help define whether the septum is indeed intact and whether the aorta is overriding. Color-flow and spectral Doppler will show shunting of blood across the defect no matter how hard it is to elucidate on real-time images. The direction of shunting may be left to right and positive on spectral displays, bidirectional low velocity flow, or right to left negative flow on spectral tracings (Figure 10.57). Pressure gradients are derived just as they are for isolated ventricular septal defects.

Figure 10.56 The ventricular septal defect of tetralogy of Fallot is usually very obvious. (A) The walls of the aorta (arrows) will straddle or override the ventricular septum. Right ventricular hypertrophy is severe. (B) The ventricular septum is lined up with the center of the aortic valves (arrow) in this dog with tetralogy of Fallot. There is severe right and left ventricle hypertrophy. Septal hypertrophy is also creating obstruction to left ventricular outflow. Plane = right parasternal long-axis left ventricular outflow view, RV = right ventricle, LV = left ventricle, VS = ventricular septum, AO = aorta, LA = left atrium.

Figure 10.57 (A) Flow through the ventricle septal defect of a tetralogy of Fallot is typically reversed and a negative flow profile is recorded. (B) Flow may be bidirectional, however, when right and left ventricular pressures are fairly equal.

When a pressure gradient can be obtained from both the pulmonic stenosis and the ventricular septal defect, they should make sense in that the gradient across the septal defect should reflect right-sided pressures and the degree of pulmonic stenosis. A pulmonary stenosis gradient of 130 mm Hg for instance suggesting a right ventricular pressure of approximately 140 to 150 mm Hg should result in right to left shunting, and a pressure gradient across the septum should be approximately 20 to 30 if systemic systolic pressure is 120 mm Hg. A dog with a blood pressure of 120 mm Hg and a gradient of 60 mm Hg across a left to right flowing septal defect suggests that right ventricular pressures are about 60 mm Hg, and the pulmonic stenosis would therefore have a gradient of approximately 40 to 50 mm Hg if pulmonary pressures are assumed to be about 10 to 20 mm Hg. Pulmonary pressure will typically be lower than the 20 to 25 mm Hg present in healthy dogs because of the pulmonary stenosis however (133). When multiple defects are present, always cross-check the derived gradients. They should make sense, and if they do not, one of the gradients is in error.

Bubble studies can document the presence of a right to left shunt across a ventricular septal defect. Inject a milliliter or 2 of saline that has been shaken vigorously into a peripheral vein. The

microbubbles will appear echodense on echocardiographic images, and they will be seen flowing into the left ventricular outflow tract when a reverse shunt is present. When a left to right shunt is present, the defect is harder to document with bubble studies unless an area of negative contrast is seen (33,131,133).

Significant septal hypertrophy may at times create a secondary obstruction in the left ventricular outflow tract. Look for the following M-mode signs of obstruction: systolic anterior mitral valve motion and early systolic aortic valve closure (Figure 10.58). Spectral and color-flow Doppler may show aliased and high velocity signals when left-sided obstruction is present. A left-sided obstruction, if present, is usually proximal to the ventricular septal defect, and analysis of pressure gradients should reflect this. In other words, systemic pressures are used in assessing the shunt and right ventricular pressures, not the left ventricular pressures as is done with isolated ventricular septal defects.

Figure 10.58 Significant septal hypertrophy may create a secondary obstruction in the left ventricular outflow tract. Look for systolic anterior mitral valve motion (arrow). RVW = right ventricular wall, RV = right ventricle, VS = ventricular septum, LV = left ventricle, MV = mitral valve.

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