Congenital Heart Disease Christian Weder DVM, MS, DACVIM (Cardiology) Great Lakes Veterinary Conference
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Congenital Heart Disease Christian Weder DVM, MS, DACVIM (Cardiology) Great Lakes Veterinary Conference Introduction Congenital heart disease (CHD) refers to a set of cardiovascular defects that are present since birth. The overall prevalence of CHD in the general population of mixed breed dogs and cats is reported to be 0.13% and 0.14%, respectively.1 Pure-bred dogs and cats have a significantly greater predilection for CHD. Most animals have a single defect, however, multiple concurrent defects can also occur.2 Although exact percentages vary somewhat by study, the most common congenital defects in dogs are pulmonic stenosis (31%), patent ductus arteriosus (17%), subaortic stenosis (15%), ventricular septal defect (14%), mitral valve dysplasia (8%), tricuspid valve dysplasia (2%) and atrial septal defect (2%).1 The most common congenital defects in cats are ventricular septal defect (21%), aortic stenosis (valvular and subvalvular, 17%), pulmonic stenosis (valve and branch pulmonary arteries, 14%), atrioventricular septal defect (7%), hypertrophic obstructive cardiomyopathy (5%), patent ductus arteriosus (5%), mitral valve dysplasia (3%), and peritoneal-pericardial diaphragmatic hernia (3%).1 Innocent Murmurs Nonpathological heart murmurs are commonly detected in young animals. These are often referred to as puppy and kitten murmurs and are not due to a congenital defect. Common characteristics of these murmurs are that they are generally soft (grade I or II), of short duration, are predominately heard during early or mid-systole, and are typically located over the heart base.3 These murmurs can also be variable with heart rate and position of the patient. They often resolve after 6 months of age, however, they can persist for longer periods in some animals. Although the cause of these murmurs has not been definitively determined, they are thought to be due to increased blood flow velocity and turbulence through the outflow tracts or great vessels. It is also important to realize that young animals can also have physiologic hearts murmurs secondary to anemia, fever, and other systemic causes. Congenital Heart Disease in Dogs Pulmonic Stenosis Pulmonic stenosis (PS) is a congenital defect that results in an obstruction to outflow of blood from the right ventricle. PS can be anatomically classified as subvalvular, valvular and supravalvular based on the level of the obstruction. Valvular is the most common form of PS in dogs. Valvular PS can be further classified into type A and type B based on the valve and annulus morphologies.4 PS has been shown to be a heritable defect in beagles and keeshonds, but can occur in any breed.5 A somewhat distinct form of PS with coronary artery anomalies has been described in both English Bulldogs and Boxer dogs.6,7 Dogs with PS typically have a systolic ejection murmur of variable intensity heard best over the heart base. They often have a normal femoral pulse quality. PS is typically diagnosed on echocardiography. Dogs with PS have an increased velocity of flow across their pulmonary valve, variable right ventricular hypertrophy and right atrial enlargement, post-stenotic dilation of their main pulmonary artery, and some degree of pulmonary insufficiency. The severity of the PS can also be assessed based on the estimated pressure gradient across the pulmonary valve as well as the degree of secondary changes. A pressure gradient of greater than 80mmHg across the pulmonary valve is consistent with a severe lesion, whereas 50- 80mmHg is moderate and <50mmHg is mild. Mild PS is generally associated with a favorable long-term prognosis with most dogs have a normal life expectancy and intervention is generally not recommended.8 The prognosis and treatment recommendations for dogs with moderate PS is less certain. Many dogs with moderate PS still have a normal life expectancy without intervention. However, balloon valvuloplasty (BVP) is often considered in dogs with moderate PS of they have associated clinical signs, severe secondary changes, or significant concurrent tricuspid regurgitation. Dogs with severe PS are at risk for the development of clinical signs such as exercise intolerance, syncope, and those associated with right sided congestive heart failure. There is also some risk of sudden cardiac death. Given these risks, BVP is generally recommended for dogs with severe PS at the time of diagnosis. This procedure has been well described and is generally well tolerated in dogs.9 BVP has been shown to be successful both in alleviating clinical signs and in prolonging survival in dogs with severe pulmonic stenosis.10 While BVP is an effective treatment option, there is some risk of re-stenosis and the possibility of sudden death is never fully eliminated. Dogs with moderate-severe PS are also often treated with a beta blocker (atenolol). Atenolol (1-2mg/kg BID) is used in this disease process to decrease the myocardial oxygen demand, alleviate dynamic outflow tract obstructions, and prevent/treat ventricular arrhythmias. Patent Ductus Arteriosus The ductus arteriosus is a vessel present during fetal life and allows blood flow to bypass the lungs. When the ductus fails to close at birth, it is termed persistently patent and the defect is usually referred to as a patent ductus arteriosus (PDA). This typically results in shunting of blood from the aorta (left) to the pulmonary artery (right), although the opposite can also occur (right-to-left shunt). PDA is a hereditary defect in dogs and is likely related to varying degrees of hypoplasia and asymmetry of ductus-specific smooth muscle as well as the presence of abnormal elastic tissue in the ductus.11 PDA is more prevalent in female dogs.12 Dogs with PDA typically have a characteristic continuous heart murmur heard best over the heart base. It is important to note that the murmur may primarily sound systolic in its timing when auscultation is performed only over the left apex. The femoral pulse quality is usually hyperdynamic (often describes as “bounding”) due to the large difference between the systolic and diastolic pressures. While a presumptive diagnosis can often be made of physical exam, echocardiography is recommended to obtain a definitive diagnosis and also allows for the identification of any concurrent congenital defects. Chronic left-to-right shunting through a PDA leads to volume overload of the left side of the heart and pulmonary vasculature. If left untreated, approximately 65% of dogs experience congestive heart failure and death by one year of age.12 As such, PDA closure is recommended in all dogs with a left-to-right shunting defect at the time of diagnosis. PDA closure has been shown to be associated with an excellent long-term survival with most dogs having a normal life expectancy.13 PDA closure can be performed either via surgical ligation or interventional closure. Both methods have been shown to be highly successful with low complication rates.14, 15 The most commonly performed interventional closure is performed using an Amplatz Canine Ductal Occluder (ACDO). Medical therapy is generally not required or recommended unless congestive heart failure is present. Subaortic Stenosis Subaortic stenosis (SAS) is a congenital narrowing of the left ventricular outflow tract that results in a pressure overload on the left ventricle.17 SAS is a heritable defect to which large and giant breed dogs are predisposed. A specific gene associated with the development of SAS has been identified in the Newfoundland but the condition is also commonly diagnosed in Rottweilers, Boxers and Golden retrievers.18 SAS results in varying degrees of outflow tract obstruction and a grading system for the lesion does exist.19 Chronic pressure overload often leads to left ventricular hypertrophy and variable degrees of left atrial enlargement. Dogs with SAS typically have a systolic ejection murmur of variable intensity heard best over the heart base. In dogs with a moderate-severe obstruction, the femoral pulse quality is generally decreased. SAS is usually diagnosed on echocardiography. The severity of SAS is typically based on the estimated pressure gradient across the left ventricular outflow tract. Dogs with a pressure gradient of less than 50mmHg are categorized as having mild SAS, whereas, moderate SAS is defined as a pressure gradient between 50-80mmHg. A pressure gradient greater than 80mmHg is considered severe. The prognosis for dogs with mild-moderate SAS is generally good, with most dogs having a normal or near-normal life expectancy without treatment.20 Unfortunately, the long-term prognosis for dogs with severe SAS is often considered poor. A median survival time of 56 months has been reported for dogs with severe SAS that are treated with atenolol. However, a more recent study found no survival benefit for dogs treated with a beta blocker as compared to those that were untreated.21 The same study showed a marked survival difference in dogs with a pressure gradient greater than 133mmHg (2.8 years) as compared to those less than 133mmHg (8.3 years). Potential complications of SAS include left sided congestive heart failure, syncope, arrhythmias, sudden death, and infectious endocarditis. Treatment options for dogs with severe SAS are very limited. Both standard balloon valvuloplasty (BVP) and open surgical correction have been reported but have not been shown to prolong survival in dogs with severe SAS.20,22 Recently, a new procedure has emerged as a potential treatment option for dogs with severe SAS. This procedure is a modification on balloon valvuloplasty using combined cutting and high-pressure balloons and has been described in the veterinary literature.23 The procedure has been shown to significantly decrease the pressure gradient across the stenosis and improve clinical signs in symptomatic dogs, however, no long-term survival data is currently available. Given that the long-term outcome of this procedure is unknown, it is not routine recommended. However, it does provide an additional option for this often devastating disease.