A 60-year-old woman presented to another hospital with shortness of breath on exertion. Her family history was unremarkable. She had undergone chemo-radiation for non-Hodgkin’s lymphoma 40 years earlier and achieved complete remission. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy did not induce cardiac toxicity. Twenty years earlier, she had an anterior myocardial infarction and underwent percutaneous coronary intervention. Moderate combined valvular diseases (aortic regurgitation, mitral regurgitation, and tricuspid insufficiency) were also followed. She had received standard treatment for chronic heart failure. However, she had suffered from acute exacerbation of congestive heart failure several times beginning 2 years before and was referred to our hospital because of refractory repeated congestive heart failure.
Her vital signs were blood pressure 106/43 mmHg, heart rate 72/min, and SPO2 97% on room air, with a body mass index of 15.8 kg/m2. The conjunctiva showed no signs of anemia or jaundice. The first and second heart sounds were normal, but the third one was increased. There was a pan-systolic murmur at the cardiac apex (Levine III/VI). There were no rales in the lungs. The abdomen was flat and soft and the liver was palpated two finger widths below the ribs. There was no edema, ascites or coldness of the limbs. She had hepatomegaly and jugular vein distention remarkable at inspiration, also known as “Kussmaul’s sign”.
Laboratory tests revealed mild renal and liver dysfunction, and the brain natriuretic peptide (BNP) level was 492 pg/mL. There were no signs of an inflammatory response, anemia, or thyroid dysfunction. Chest X-ray showed a cardiothoracic ratio of 47% and no evidence of pleural effusion, pulmonary congestion, or pericardial calcification. The electrocardiogram showed a pulmonary P wave, high left-side voltages, poor R progression, and ST changes in V5 and V6.
Echocardiography showed decreased motion of the left anterior septum and the left ventricular ejection fraction (LVEF) was 42%. There was bilateral atrial enlargement, but no left ventricular dilatation. No pericardial effusion was seen. Although moderate mitral regurgitation, and tricuspid insufficiency were seen at the end of a previous hospitalization (Fig. 1a), severe mitral and tricuspid regurgitation were detected at our hospital admission (Fig. 1b). The septal leaflet of the tricuspid valve showed decreased mobility. The tricuspid regurgitation pressure gradient was 45 mmHg. Systolic flow reversal in the hepatic veins was observed, suggesting severe tricuspid regurgitation. Pulse doppler flow showed that the ratio of mitral peak velocity of early filling (E: 1.45 m/s) to late filling (A: 0.98 m/s) was 1.5, and the deceleration time was 211 ms. The ratio of mitral peak velocity of early filling (E) to early diastolic mitral annular velocity (E’: 6.9 cm/s) was 21. The early diastolic left ventricular filling velocity was reduced by 27% during inspiration. These findings indicate the possibility of CP and/or restrictive cardiomyopathy. The inferior vena cava was enlarged to 22 mm and the respiratory fluctuation was decreased. We diagnosed acutely developed congestive heart failure due to severe mitral and tricuspid regurgitation, which was not observed at the end of hospitalization at the previous hospital. The patient was prescribed additional diuretics to reduce volume overload.
Computed tomography revealed a mild pericardial thickening of 4 mm in front of the right ventricle (Fig. 2). Considering the history of radiation therapy, it was likely that the patient was suffering from concealed pericardial disease.
Prior to the cardiac catheter examination, normal saline was infused at a rate of 50 mL/h for 12 h to manifest the pericardial disease and to protect renal function from the contrast medium. Coronary angiography showed that the coronary arteries were intact and there was a stent in the left anterior descending artery. The right atrial, end diastolic right ventricular, diastolic pulmonary artery, and pulmonary capillary wedge pressures were 15 mmHg. This finding was not seen in a right heart pressure study performed previously in hospital about 6 months ago (Fig. 3a). In the right atrial pressure waveform there were prominent x and y descents. The right ventricular systolic pressure exceeded 40 mmHg and showed a dip-plateau pattern (Fig. 3b). In the simultaneous pressure measurement of the left and right ventricle, the difference in end-diastolic pressure was within 5 mmHg. At maximum inspiration, the systolic pressure of both ventricles was dissociated, and showed mirror-image discordance (Fig. 4). Based on these changes in hemodynamics after loading of the patient with fluids, we revealed the diagnosis of OCPD.
Pericardiectomy, aortic valve replacement, and mitral and tricuspid valvuloplasty were performed. At surgery, there was a hard, thickened pericardium, and very mild pericardial adhesion. Histological examination of the resected pericardium revealed hyaline-like fibrous hypertrophy.
Twelve months postoperatively, there was no acute exacerbation of the congestive heart failure. The New York Heart Association functional class had also improved from III to II. On echocardiography, although mild tricuspid and mitral regurgitation remained, the size of both the left and right atria decreased. The percent difference in E-velocity between expiration and inspiration was improved to 12% after pericardiectomy, compared to 27% before.