Which Heart Disease Do I Have and Will I Get Better with the Surgery?
A 60-year-old male with prior history of mediastinal radiation for treatment of Hodgkin's lymphoma, coronary artery disease (CAD), bioprosthetic aortic and mitral valve replacement, and paroxysmal atrial fibrillation presented to clinic with exertional dyspnea. His symptoms have been ongoing for 6 months along with abdominal distention, weight gain of 24 pounds and lower extremity swelling. He has recently noticed a decline in his exercise tolerance. He denied chest pain, orthopnea or paroxysmal nocturnal dyspnea. His cardiac history is significant for right coronary artery (RCA) drug-eluting stent (DES) insertion and aortic and mitral valve replacements with 23 mm and 29 mm bioprosthetic valves respectively (a year ago). He was recently started on prednisone 40 mg daily for possible constrictive pericarditis due to post-pericardiotomy syndrome. Patient developed acute kidney injury previously with use of ibuprofen and was unable to tolerate colchicine. His other medications were aspirin, clopidogrel, carvedilol, warfarin, digoxin, furosemide, and pitavastatin.
On physical exam he was afebrile with a blood pressure of 134/68 mmHg, pulse 74/min, respiratory rate 12/min, saturating 98% on room air. He appeared comfortable at rest. He had an elevated jugular venous pressure (8cm H2O), regular heart rhythm, holosystolic murmur over the left lower sternal border, diminished basal lung sounds, distended abdomen, and 1+ pitting edema of the bilateral lower extremities. Serum creatinine was 1.5 mg/dl [normal range: 0.58-0.96 mg/dL], which was at his baseline. Erythrocyte sedimentation rate (ESR) was 12 mm/hr [normal range: 0-15 mm/hr], ultra-sensitive C-reactive protein (us-CRP) was elevated at 8.1 mg/L [normal range: <3.1mg/L), NT pro-BNP was elevated at 899 pg/mL [normal range: <125pg/mL). Complete blood count, liver function tests, electrolyte panel and urinalysis were unremarkable. Electrocardiogram showed normal sinus rhythm with first-degree atrioventricular block (PR interval 284 milliseconds) without any ST elevation or PR depression.
The patient underwent transthoracic echocardiogram which showed normal left ventricular size and ejection fraction of 60%, mildly decreased right ventricular systolic function and moderate tricuspid regurgitation. Also showed tethering of the right atrial and right ventricular free wall, dilated inferior vena cava (3 cm), respirophasic septal shift, medial e' velocity of 5 cm/s, and hepatic vein expiratory diastolic reversal velocity ratio of 1.08. Prosthetic aortic and mitral valves were normally functioning (Image 1). Gated computerized tomography (CT) scan of the chest showed a thickened pericardium (Image 2). Cardiac magnetic resonance (CMR) imaging showed tubular/conical ventricular deformities, septal bounce, mild abrupt cessation of diastolic filling and prominent respirophasic septal shift on free breathing sequence (Video 1). On delayed enhancement imaging there was no pericardial late gadolinium enhancement or increased pericardial signal intensity to suggest pericardial inflammation/active pericarditis. A dual-transducer constriction study showed elevated right and left sided filling pressures with equalization of diastolic pressures, but concordant changes in right and left ventricular systolic pressures during inspiration. Systolic area index was not calculated (Table 1).
|Cardiac catheterization pressure recordings (mmHg)|
|Right atrial mean pressure||20|
|Right ventricular end-diastolic pressure||24|
|Pulmonary artery diastolic pressure||25|
|Pulmonary capillary wedge pressure (mean)||24|
|Left ventricular end-diastolic pressure||24|
What is the most likely diagnosis?