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Constrictive pericarditis in a patient with Turner's syndrome and liver cirrhosis: coincidence or part of the syndrome?

To the Editor: We observed a woman with Turner syndrome (TS) in whom suspected idiopathic liver cirrhosis (ILC) preceded the appearance of constrictive pericarditis (CP) by 7 years. Although coexistent ILC has been rarely described, CP in TS has not been reported, to our knowledge. We discuss various clinical aspects and propose a possible connection between them. A 48-year-old woman with TS was admitted for exacerbation of dyspnea, ascites, and leg edema. She had received a diagnosis of ILC 21 years earlier after investigation for shortness of breath, ascites, leg edema, cirrhotic liver (detected in surgery for inguinal hernia), mild anemia, thrombocytopenia, elevated liver enzymes, and serum-ascites albumin gradient >1.1 g/dL. Her chest radiograph was normal except for small bilateral pleural effusion. Abdominal ultrasonography demonstrated ascites and mild hepatic enlargement. The results of further evaluation, including serologic tests for viral hepatitis and autoimmune diseases, were negative. Two years later the patient was lost to follow-up. On admission, her blood pressure was 110/60mm Hg, her heart rate was 110 beats/min, and her respiratory rate was 32/min. Her jugular veins were distended, heart sounds were distant, and crackles over both lungs were noted. An electrocardiogram showed sinus tachycardia with low voltage. Computed tomography demonstrated typical signs of CP, including thickened calcified pericardium, pulmonary congestion, and bilateral pleural effusion. Echocardiographic examination showed thickened pericardium. The systolic pulmonary artery pressure was 70mm Hg. On meticulous reevaluation of chest radiographs, performed in an ambulatory setting 7 years after the diagnosis of ILC, pericardial calcifications could be detected. Pericardial resection was recommended. The patient and her family repeatedly refused this procedure. She died 4 months later of respiratory failure. In this woman, ILC preceded the appearance of frank CP by 7 years. CP in TS has not been described, to our knowledge. We propose that the association between ILC and CP was not incidental. Abnormal liver function has been reported in as many as 80% of female individuals with TS. Intrahepatic cholestasis, fatty infiltration, vascular intimal thickening or obstruction, portal thrombosis, sinusoidal dilatation, nodular regenerative hyperplasia, and fibrosis have been described. Estrogen deficiency, hepatic vascular disorders, hormone replacement therapy, autoimmune disease, and fatty liver have been implicated. In our patient it is possible that cirrhosis was not idiopathic but secondary to undiagnosed CP, which had possibly existed long before. CP is a chronic fibrous thickening of the pericardial sac, resulting in abnormal diastolic filling and abnormal myocardial relaxation. CP should be suspected in any patient with jugular venous distention, pleural effusion, ascites, anasarca of unknown origin, and diastolic heart failure. The course is typically slowly progressive. Its symptoms and signs are not specific. As a result, there is generally a long delay (20±6 months) between the onset of symptoms and the diagnosis. For this reason, CP is often misdiagnosed as primary hepatic disease, ILC, or nephrotic syndrome, with which CP shares some common features. Even in the current era, CP is frequently under diagnosed. In the past, tuberculosis was the leading cause. Presently, 30% of cases are idiopathic, followed by complication of cardiac surgery and viral pericarditis. Computed tomography or magnetic resonance imaging is useful for visualization of a thickened pericardium and small calcifications. These findings may be missed by echocardiography. Pericardial calcification often is associated with idiopathic and long-standing disease. Our proposal that ILC in our patient was in fact the result of longstanding undiagnosed CP, is based on the following data: A case of slow progression of unrecognized CP culminating in a full-blown clinical picture more than 20 years later has been documented. At presentation, our patient manifested signs of right-sided heart failure, common to both CP and LC. These included dyspnea, jugular venous distention, pleural effusion, ascites, and leg edema. These findings may result from occult CP if no other valid diagnosis is confirmed. In fact, diagnosis of CP may be difficult, and radiographic findings may appear long after clinical findings are manifested, as in our patient. Finally, the cirrhotic appearance of the liver could be the result of right-sided heart failure, which may cause liver fibrosis and cirrhosis. We propose that some cases of ILC reported in association with TS were in fact the result of occult CP. It is possible that early death (TS is associated with a 3-fold increase in overall mortality) prevented definition of the correct chain of events initiated by CP. We therefore recommend appropriate work-up for CP in TS patients with diopathic chronic liver disease or cirrhosis.