Advances in cardiovascular care and cardiothoracic surgical expertise have enabled many patients with congenital heart disease to live well into adulthood. With lengthened life expectancies, congenital heart disease patients are more frequently undergoing noninvasive nuclear cardiac imaging to exclude perfusion abnormalities. We present the Tc-99m sestamibi SPECT findings of a patient with an uncommon congenital malformation. Case history. A 45-year-old man with doubleinlet left ventricle, pulmonary stenosis, pulmonary hypertension, status postmultiple systemic-pulmonary shunt procedures, and, most recently, a Potts anastamosis (descending aorta to the left pulmonary artery anastamosis) at age 31, presented to the cardiology clinic complaining of burning epigastric pain on exertion. The patient had known pulmonary artery hypertension but had improved his oxygen saturation and exercise tolerance on a regimen of bosentan, coumadin, digoxin, and verapamil. Initially, the patient underwent a modified walk test and was able to walk on a level surface at increasing speed for 15 minutes, achieving a peak speed of 1.8 miles per hour, HR 97, BP 148/69, when he complained of progressive chest burning. His pulse oximetry at rest was 84% and dropped to 72% with exercise. The electrocardiogram showed no change from baseline (sinus rhythm, left ventricular hypertrophy with strain, and ST elevation in the right precordial leads). In light of the reproducible symptoms and the patient’s strong preference for noninvasive evaluation, he underwent adenosine Tc-99m sestamibi SPECT to evaluate for coronary artery disease. The patient was infused with 140 mcg per kilogram per minute of adenosine over a 6-minute period; at 3 minutes into the infusion, 99mTc sestamibi was injected. There were no reported electrocardiogram changes or symptoms during adenosine infusion. Images were acquired with 180° SPECT approximately 30 minutes later. Findings. The adenosine sestamibi SPECT images showed a large left-ventricle cavity with no definite myocardial perfusion abnormality. Low counts were seen along the middle inferior wall because of adjacent tracer activity in a loop of small bowel. On both stress and rest images, the short-axis projection showed a smaller, rounded structure with a central lumen from the middle anterior to basal ventricular cavity, consistent with a rudimentary right ventricle (Figure 1). Because this is essentially a single functioning chamber, absent were the typical-appearing interventricular septum or right ventricle (compare with the normal right ventricle and septum in Figure 2). Despite the unusual anatomy, the gated SPECT perfusion images showed normal left ventricular myocardial wall motion and systolic thickening with an ejection fraction of 51%. The cardiac chambers were further clarified by viewing the patient’s transesophageal echocardiogram to evaluate mitral insufficiency (Figures 3 and 4).
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