An Interesting Diagnosis in a Patient With Adult Congenital Heart Disease
A 33-year-old male, history of congenitally corrected transposition of the great vessels, bicuspid aortic stenosis (status post valvotomy, homograft, and ultimately mechanical valve replacement) complicated by complete heart block (status post pacemaker with upgrade to defibrillator in the setting of reduced ventricular function) and atrial tachycardia, initially presented for further evaluation due to 15 months of persistent shortness of breath and right-sided chest/abdominal pain. Patient had already undergone extensive diagnostic cardiovascular workup over a 6-month course, between his local hospital and our institution, due to his symptoms.
Left and right heart catheterization was negative for obstructive coronary artery disease or significant valvulopathies but noted mild to moderate biventricular combined systolic and diastolic dysfunction on ventriculogram with the illustrated hemodynamics (Figure 1).
*Estimated RA pressure was (mean) of 17 mmHg and LA pressure is estimated to be 23 (mean). LV/RV pressures are reported as SBP/DBP and end diastolic pressures.
Liver biopsy, performed due to intermittent ascites, showed normal hepatic architecture with mild patchy centrilobular sinusoidal dilatation and congestion, without any signs of cirrhosis. Computed tomography (CT) chest scan (Figure 2) showed a 10 x 3 x 1.6 cm loculated pericardial effusion running vertically and overlying the anterior atrioventricular groove with distortion of the base of the anterior, morphologic left ventricle, as well as mild to moderate calcifications involving the visceral pericardium.
Given the above findings, patient was referred to pericardial disease clinic for our opinion regarding further management. Repeat transthoracic echocardiogram was done, showing mildly dilated systemic ventricle with hypertrophy and mildly decreased systolic function (ejection fraction [EF] 45%); well-positioned aortic prosthesis without regurgitation and stable peak/ mean gradients 36/20 mm Hg; normal subpulmonic ventricle with 1-2+ atrioventricular valve insufficiency; small pericardial effusion; septal bounce; and these additional findings (Figure 3).
Which of the following is the most accurate statement regarding the diagnosis in this patient?