Two-dimensional echocardiographic identification of sinus of Valsalva-right heart fistula due to infective endocarditis.

A sinus of Valsalva fistula with left-to-right shunting from the aorta to the right, atrium (RA) or right ventricle (RV) is an unusual complication of infective endocarditis (IE). These fistulas have been detected in the past only by angiography and have been definitively localized only at the time of surgery or postmortem examination.‘,” Because sinus of Valsalva listulas that occur during IE may serve as an indication for operative intervention,,’ localizing them noninvasively would be highly desirable. Echocardiographic techniques hold the greatest promise for identification of such fistulas. M-mode echocardiography (MME), lacking integration of spatial information, would make demonstration of these hstulas improbable. However, two-dimensional echocardiography (2DE) provides spatial anatomic information and thus has the potential for fistula identification. This is the first report of’ a patient with IE in whom 2DE demonstrated a vegetation that extended via a fistulous tract from the noncoronary cusp of t,he aortic valve through the pars membranosa onto the septal leaflet of’ the tricuspid valve. HR, a Wyear-old black male farmer with a history of rheumatic fever at age 25 years, was first noted by auscultation to have aortic regurgitation (AR) in 1975. At that time he was asymptomatic and MME was normal. He was otherwise well until -July, 1980, when he was admitted to a local hospital for prostatitis. During this hospitalization he had seven of seven blood cultures positive for Pswdomona.s alcaligcncs. These were attributed to prostatitis. He was treated with oral tetracycline for 3 weeks and his symptoms completely resolved. In June, 1981, he presented to another hospital wit,h increasing fatigue and weight loss. Three blood cultures grew F’. alcaligenes and IE was diagnosed, whereupon he was transferred to the Durham Veterans Administration Hospital. On initial presentation on ,July 7. 1981. HR was alert, and appeared chronically ill. His temperature was 100.fi” F orally, respirations were Wmin, heart rate was 88 bpm and regular, and hlood pressure was 120/,50 mm Hg. Splinter hemorrhages were present in the nail beds and petechiae were noted on the lower extremities, conjunct.ivae, and palate. The internal jugular venous pulsation was normal. Auscultation of’ the lungs revealed hibasilar rales. The apical impulse was enlarged and displaced to the sixth intercostal space in the anterior axillary line. The hrst heart sound was increased and the second heart sound was singular. A protodiastolic gallop was audible at the apex. A II/VI systolic ejection murmur and a III/VI early diastolic decrescendo murmur were heard along the left sternal border. The abdominal examination was normal. Trace pretibial edema was noted. The neurologic examination was normal. Initial laboratory data showed an erythrocyte sedimentation rate of 130 mm/hr, hematocrit of 23.5’,#, and white blood cell count of 11,800/~1 with a normal differential; the platelet count was lOO,OOO/~l. The blood urea nitrogen was 159 mg/dl and creatinine was 5.0 mg/dl. Urinalysis showed numerous red blood cells with granular and red hlood cell casts. Arterial blood gasses on room air showed a pH of 7.48, a pC0, of 20 mm Hg, and a p0, of 106 mm Hg. The prothrombin time was 13.5 seconds with control of 11.2 seconds. The partial thromboplastin time was 32.2 seconds with control of 32.3 seconds. The ECG showed first-degree AV block with left axis deviation. Cardiomegaly with clear lung fields was seen on chest x-ray. Five of five blood cultures grew J’s. alcaligww A Swan-Ganz catheter was placed shortly after admission and showed pulmonary artery (PA) pressure of 26/20 mm Hg, PA capillary wedge pressure of 12 mm Hg, mean RA pressure of 9 mm Hg, and arteriovenous oxygen content difference of’ 3.3 vol ’ ( MME revealed vegetations on the aortic valve and on the septal leaflet of the tricuspid valve. Left ventricular (LV) size was normal and there was no preclosure of the mitral valve (MV). The MV leaflets were thickened and high frequency diastolic oscillations of the anterior MV leaflet were present. 2DE was performed using a dynamically focused phased array imaging system and a 3.5 MHz transducer (Figs. 1 and 2). An asymmetric. “dumbbell-shaped” vegetation was demonstrated to extend from the noncoronary cusp of the aortic valve through the pars membranosa and onto the tricuspid septal leafiet. The presence of a tistulous tract was suggested by absence of echoes between the lesion and the int,erventricular septum. The mass lesion on the noncoronary cusp juxtaposed the orifice of the right coronary artery (RCA). Three days after arrival, the patient rapidly developed ascites and disseminated intravascular coagulation. Cardiac catheterization was performed confirming the presence of a sinus of Valsalva Jistula between aorta and RV. In addition, severe AR and mild mitral regurgitation were demonstrated. The RA A wave was 16 mm Hg and the RA V wave was 33 mm Hg with a mean of 24 mm Hg. The RV pressure was 46/18 mm Hg. PA pressure was 50/16 mm Hg. Mean PA capillary wedge pressure was I.5 mm Hg. An oximetry study verified a left-to-right shunt.. PA blood flow was 3.9 l/min/m’; systemic blood flow was 2.1 l/mm/m:’ with a ratio of 1.84 pulmonary-to-systemic flow. Arterial-venous oxygen content difference was 6.2 vol ’ , Despite vigorous medical therapy, the patient suddenly and rapidly deteriorated, became anuric. and required emergency dialysis. Surgery was impossible because of marked clotting abnormalities. Hepatic failure ensued