A case of recurrent pericardial constriction presenting with severe pulmonary hypertension
A 78-year-old man was admitted to the pulmonary hypertension service at Stanford Hospital in December, 2010 with elevated pulmonary arterial pressures and progressive right-sided heart failure. He reported progressive shortness of breath over a period of two months, culminating in dyspnea at rest. This was associated with a 20-pound weight gain, abdominal bloating, and leg edema.\nHe was first evaluated for dyspnea several years prior to admission, where he was diagnosed with bilateral pleural effusions and extensive pericardial calcification on chest computed tomography (CT). Echocardiography and cardiac catheterization were consistent with constrictive pericarditis. He subsequently underwent uneventful complete pericardiectomy in 2008. The pericardium was stripped from phrenic nerve to phrenic nerve and the diaphragmatic and posterior pericardium was removed as well.\nThe patient enjoyed symptomatic improvement for approximately one year but then developed recurrent dyspnea. An echocardiogram demonstrated right ventricular (RV) pressure overload and a right ventricular systolic pressure of 66 mmHg. A trial of sildenafil was initiated but was discontinued after the patient developed a marked worsening of his dyspnea.\nHis medical history was remarkable for pericarditis diagnosed in 1978, which was presumed to be viral in origin. He had permanent atrial fibrillation/atrial flutter which did not respond to an attempted atrial flutter ablation in 2003. He had a history of chronic hypertension and gradually progressing stage 3-4 chronic renal impairment.\nOn admission, his jugular veins were distended to angle of his jaw when upright. Pitting edema was noted up to the mid thighs bilaterally. Crepitations were heard on auscultation of his lungs. N-terminal pro-brain natriuretic peptide was measured to be greater than 30,000 pg/mL. Chest x-ray showed blunting of the costophrenic sulci with coarse reticular linear basilar parenchymal opacities ().\nPrior to admission, ventilation-perfusion scan was low probability for pulmonary embolism. Neither pulmonary function tests nor screening blood work revealed any other potential cause for his pulmonary hypertension.\nEchocardiogram on admission was technically difficult. Moderate RV enlargement and dysfunction were identified. Right ventricular systolic pressure was severely elevated at 82 mmHg. No findings of pericardial constriction were reported.\nCardiac catheterization was performed two days post admission after the patient had received intravenous diuresis. Pulmonary artery pressures were elevated at 70/13 mmHg with a mean of 35 mmHg. The pulmonary capillary wedge pressure was 13 mmHg. Cardiac output as calculated by the Fick equation was preserved at 5.3 L/min and pulmonary vascular resistance was calculated to be 4.3 WU. The pulmonary pressures did not respond to vasodilator challenge with inhaled nitric oxide at a dose of 20 ppm. The right atrial pressure tracing () showed abrupt x and y descents, and no fall in right atrial pressure with inspiration (positive Kussmaul's sign). Simultaneous pressure measurements of the RV and left ventricle (LV) revealed an early diastolic “dip and plateau” and equalization of end diastolic pressures (). However, no evidence of discordant change in RV and LV pressures was seen with respiration. Thus, the catheterization was supportive but not diagnostic for pericardial constriction. Volume challenge was not performed. Cardiac biopsies were performed at the time of the catheterization and showed only nonspecific changes.\nCardiac magnetic resonance imaging (MRI) was performed on a 1.5 T GE scanner, with no contrast given secondary to the patient's renal insufficiency. The main pulmonary artery, inferior vena cava and right atrium were found to be dilated. Residual thickened pericardium was identified in the basolateral and basal posterolateral segments (), with adhesive segments, most prominent in the basal anterior segment of the RV and the basal posterolateral segment of the LV. Septal flattening occurred on inspiration () and resolved completely on expiration (), revealing heightened ventricular interdependence. These findings confirmed the diagnosis of recurrent pericardial constriction.\nGiven his significant comorbidity and the high-risk nature of repeat pericardiectomy, the patient was managed medically with diuresis. He responded well to furosemide and was clinically euvolemic with improvement in his dyspnea on discharge.