Progressive Right Ventricular Obstruction Caused by a Double-Chambered Right Ventricle Resulting in Shunt-Reversal via a Concomitant Congenital Ventricular Septal Defect and Subsequent Erythrocytosis in a Dog

SIMPLE SUMMARY: A 3-year-old Chihuahua was presented because of exercise intolerance, respiratory distress, and collapsing episodes. The dog was known to have a congenital heart disease, a ventricular septal defect. This anomaly was diagnosed using echocardiography at 10 weeks of age when the dog showed no clinical signs but had a murmur. The defect was considered unimportant then because only a small amount of blood flowed from the left to the right ventricle. However, at 3 years of age, a repeated echocardiogram revealed shunt reversal via the ventricular septal defect, allowing deoxygenated blood from the right ventricle to enter the systemic arteries, resulting in insufficient oxygen supply to all organs. The reason why the direction of blood flow through the ventricular septal defect reversed was the development of an obstructive band of tissue in the right ventricle, which caused the right ventricular pressure to exceed the left ventricular pressure. The most likely cause for the development of the abnormal band was thought to be the continuous mechanical irritation of the right ventricular wall caused by the high-velocity jet arising from the left ventricle. Because of the poor prognosis, the dog was euthanized, and a post-mortem examination took place. ABSTRACT: A 3-year-old Chihuahua was presented because of exercise intolerance, respiratory distress, and syncopal episodes. At the age of 10 weeks, the dog was diagnosed with a congenital small left-to-right shunting ventricular septal defect and a mild right ventricular outflow tract obstruction via echocardiography. At that time, the dog was asymptomatic, but the breeder’s veterinarian heard a murmur. Both cardiac defects were judged to be clinically non-relevant at that time. However, at 3 years of age, echocardiography revealed a severe right ventricular obstruction, known as a double-chambered right ventricle, along with right-to-left shunting via the ventricular septal defect. Because of chronic hypoxemia due to the right-to-left shunting, erythrocytosis developed. Flow reversal via the shunt was caused by a progressively worsening right ventricular obstruction leading to a supra-systemic right ventricular systolic pressure. Because of the poor prognosis, the dog was euthanized, and the heart was submitted for post-mortem examination. Gross pathologic findings revealed the close proximity of the right ventricular obstructive lesion to the ventricular septal defect. Histopathology revealed localized muscular hypertrophy and severe endocardial fibrosis. The suspected pathogenesis of the progressive obstruction was infiltrative myocardial fibrosis due to turbulent blood flow from the left-to-right shunting ventricular septal defect, as described in humans.