2023 年 5 巻 9 号 p. 365-366
A 68-year-old man with hypertrophic cardiomyopathy (HCM), chronic atrial fibrillation, and prior pacemaker implantation (at 50 years of age) was referred to the Sakakibara Heart Institute complaining of dyspnea. Computed tomography and transthoracic echocardiography showed significant circumferential pericardial effusion (Figure A,B). The heart presented a swinging motion (Supplementary Movie). The early diastolic mitral flow velocity presented inspiratory decrease and expiratory increase. Furthermore, the early diastolic mitral septal annular velocity (e’) was increased compared with lateral e’, indicating constriction of the heart (Figure C,D). The patient underwent subxiphoid pericardiocentesis, with 700 mL exudate drained; however, the constrictive echocardiographic findings persisted. The patient remained hemodynamically unstable and eventually died due to multi-organ dysfunction on Day 10 of hospitalization. The autopsy revealed cardiomegaly (heart weight 550 g) and a thickened pericardium (Figure E,F). Myocardial hypertrophy with disarray and interstitial fibrosis was observed, but no other cardiomyopathies were indicated. Notably, both the visceral and parietal pericardium had inflammatory cell infiltration and fibrous thickening (Figure G,H). There was no evidence of infection, autoimmune disease, or malignancy. The autopsy could not clarify the etiology of the pericarditis and its association with HCM; however, the presence of significant pericardial effusion, a thickened pericardium, and persistent constrictive echocardiographic findings suggested effusive-constrictive pericarditis (ECP).
(A) Computed tomography and (B) transthoracic echocardiography showing significant pericardial effusion (asterisks). (C) Septal e’ was greater than (D) lateral e’. (E) Autopsy revealed a thickened pericardium. (F) Thickening of the visceral pericardium (arrows; Masson’s trichrome stain). (G) Visceral and (H) parietal pericardium showing infiltration of inflammatory cells (white arrows; hematoxylin and eosin stain).
Although most cases of symptomatic pericardial effusion resolve after pericardiocentesis, some patients deteriorate even after the procedure.1 In such cases, ECP is a differential diagnosis that should be assessed by cardiac catheterization or Doppler echocardiography. Furthermore, the combination of HCM and ECP severely impaired biventricular blood filling, leading to rapid progression of severe systemic congestion and low output syndrome. Pericardiectomy including removal of the visceral pericardium should be considered to address hemodynamic crisis.1
This study complies with the Declaration of Helsinki.
Supplementary Movie. Transthoracic echocardiography.
Please find supplementary file(s);
https://doi.org/10.1253/circrep.CR-23-0061
