Case Report Received: February 3, 1992 Accepted: February 5, 1992
Cardiology 1992;80:438-440
Physiological Features of Pericardial Constriction in the Absence of Pericardial Disease A. S.
Owena Trotterb
Departments of aCardiology and bPathology, Royal Brompton National Heart and Lung Hospital, London, UK Key Words Pericardial constriction x-descent
Abstract To sustain a clinical diagnosis of constriction it is classically held that the fibrous pericardium must be thickened and adherent to the surface of the heart. A case is presented in which leukaemic infiltration of the fat overlying the myocardium resulted in the physiological features of constriction, although all layers of the pericardium itself were normal. Constriction is thus a physiological diagnosis; it may develop in the absence of the classical anatomical findings. A. Owen, PhD, MRCP, Department of Cardiology, Royal Brompton National Heart and ung Hospital, Sydney Street London, SW3 6NP (UK)
A 66-year-old woman with a two-year history of prolymphocytic T-cell leukaemia presented with massive ascites and pitting oedema to the chest. The jugular venous pressure was raised to at least 15 cm, and no wave form was discernable. The heart was clinically normal. External pulse recording of the jugular venous pressure demonstrated a remarkable dominant xdescent (fig. la). Pulsed Doppler echocardiography demonstrated dominant systolic superior vena caval flow towards the right atrium (fig. lb). Tricuspid ring motion was accentuated on M-mode with the transducer at the apex and the beam directed towards the ring (fig. lc). No pericardial effusion was present. The cardiac chambers were of normal size with no evidence of impaired function. The cardiac valves were normal. She became anuric and died 2 days later. At necropsy, the fibrous pericardium was mildly thickened and was overlaid by large deposits of tumour, but there was no evidence of infiltration. The fibrous pericardium did not tightly invest the heart, and there was a space between this and the underlying heart. The only macroscopic suggestion of tumour infiltration of the heart was roughening of its exterior surface. Microscopy showed no evidence of infiltration of the pericardium. The roughened surface of the heart showed also nodules of tumour which overlaid, but did not infiltrate the underlying myocardium (fig. 2). The clinical features of the case presented are entirely consistent with a diagnosis of constriction. The combination of an x-descent in the jugular venous pressure with forward flow in the superior vena cava, indicating an increase in the volume of blood in the great veins and right atrium, is very characteristic. An
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ECG ⅜c
PCG Fig. 1.a External pulse recording of the jugular venous pressure, demonstrating a dominant xdescent. ECG = Electrocardiogram; PCG = phonocardiogram; x = x-descent. b Doppler echocardiographic recording of superior vena cava velocity, demonstrating systolic flow toward the atrium. ECG = Electrocardiogram; PCG = phonocardiogram. c M-mode of right A-V ring, demonstrating well-preserved right ventricular long axis function. TR = Tricuspid ring. 439 r W Fig. 2. Photomicrograph showing leukaemic infiltrate involving the epicardium only (arrow). No tumour is seen infiltrating the myocardium. HE × 50. thus appear that the leukaemic infiltration of the surface of the heart was responsible for the characteristic physical findings of constriction. A similar case has been reported [1] in which pericardial constriction was diagnosed clinically and by haemodynamic measurements, but was not confirmed at thoracoto-my. A diagnosis of restrictive cardiomyopa-thy was made, and he died 14 months later. At necropsy the superficial epicardium was replaced by an acellular focally mineralised hyaline scar of uniform thickness, that had partially separated from the underlying left ventricular myocardium. It was felt that this epicardial scar would have been capable of causing myocardial constriction. The case presented here, and probably that presented previously [1], demonstrate that the classical clinical and haemodynamic findings of pericardial constriction may occur in the presence of a normal fibrous pericardium; the abnormalities then residing in the surface of the heart which is capable of constricting the myocardium in the same way that the fibrous pericardium can. The more general term constriction would therefore seem appropriate until an anatomical diagnosis has been established. It should thus be recognised that abnormalities of the surface of the heart as well as the pericardium can result in the syndrome of constriction.
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increase in volume accompanied by a fall in pressure can only occur if the capacity of the right atrium increases; this is mediated by motion of the tricuspid ring towards the apex of the right ventricle during ejection. The accentuated ring motion reflects the changes in the right ventricular function, with transverse axis excursion being reduced by the presence of constriction. No other cardiac abnormality could account for these findings. At necropsy, however, the pericardium was unremarkable and was not constricting the heart. It would Acknowledgements The authors are grateful to Dr. D.G. Gibson for his helpful comments during the preparation of this report. Reference 1 Fowler N, Bove KE, Dunba S, Mayer R: Fatigue, Dyspnea and abdominal swelling in a 13 year old boy. Am Heart J 1978;96:533-542. 440 Owen/Trotter Physiological Features of Pericardial Constriction in the Absence of Pericardial Disease
Cardiology 1992;80:438-440
Physiological Features of Pericardial Constriction in the Absence of Pericardial Disease A. S.
Owena Trotterb
Departments of aCardiology and bPathology, Royal Brompton National Heart and Lung Hospital, London, UK Key Words Pericardial constriction x-descent
Abstract To sustain a clinical diagnosis of constriction it is classically held that the fibrous pericardium must be thickened and adherent to the surface of the heart. A case is presented in which leukaemic infiltration of the fat overlying the myocardium resulted in the physiological features of constriction, although all layers of the pericardium itself were normal. Constriction is thus a physiological diagnosis; it may develop in the absence of the classical anatomical findings. A. Owen, PhD, MRCP, Department of Cardiology, Royal Brompton National Heart and ung Hospital, Sydney Street London, SW3 6NP (UK)
A 66-year-old woman with a two-year history of prolymphocytic T-cell leukaemia presented with massive ascites and pitting oedema to the chest. The jugular venous pressure was raised to at least 15 cm, and no wave form was discernable. The heart was clinically normal. External pulse recording of the jugular venous pressure demonstrated a remarkable dominant xdescent (fig. la). Pulsed Doppler echocardiography demonstrated dominant systolic superior vena caval flow towards the right atrium (fig. lb). Tricuspid ring motion was accentuated on M-mode with the transducer at the apex and the beam directed towards the ring (fig. lc). No pericardial effusion was present. The cardiac chambers were of normal size with no evidence of impaired function. The cardiac valves were normal. She became anuric and died 2 days later. At necropsy, the fibrous pericardium was mildly thickened and was overlaid by large deposits of tumour, but there was no evidence of infiltration. The fibrous pericardium did not tightly invest the heart, and there was a space between this and the underlying heart. The only macroscopic suggestion of tumour infiltration of the heart was roughening of its exterior surface. Microscopy showed no evidence of infiltration of the pericardium. The roughened surface of the heart showed also nodules of tumour which overlaid, but did not infiltrate the underlying myocardium (fig. 2). The clinical features of the case presented are entirely consistent with a diagnosis of constriction. The combination of an x-descent in the jugular venous pressure with forward flow in the superior vena cava, indicating an increase in the volume of blood in the great veins and right atrium, is very characteristic. An
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/12/2019 2:48:28 AM
ECG ⅜c
PCG Fig. 1.a External pulse recording of the jugular venous pressure, demonstrating a dominant xdescent. ECG = Electrocardiogram; PCG = phonocardiogram; x = x-descent. b Doppler echocardiographic recording of superior vena cava velocity, demonstrating systolic flow toward the atrium. ECG = Electrocardiogram; PCG = phonocardiogram. c M-mode of right A-V ring, demonstrating well-preserved right ventricular long axis function. TR = Tricuspid ring. 439 r W Fig. 2. Photomicrograph showing leukaemic infiltrate involving the epicardium only (arrow). No tumour is seen infiltrating the myocardium. HE × 50. thus appear that the leukaemic infiltration of the surface of the heart was responsible for the characteristic physical findings of constriction. A similar case has been reported [1] in which pericardial constriction was diagnosed clinically and by haemodynamic measurements, but was not confirmed at thoracoto-my. A diagnosis of restrictive cardiomyopa-thy was made, and he died 14 months later. At necropsy the superficial epicardium was replaced by an acellular focally mineralised hyaline scar of uniform thickness, that had partially separated from the underlying left ventricular myocardium. It was felt that this epicardial scar would have been capable of causing myocardial constriction. The case presented here, and probably that presented previously [1], demonstrate that the classical clinical and haemodynamic findings of pericardial constriction may occur in the presence of a normal fibrous pericardium; the abnormalities then residing in the surface of the heart which is capable of constricting the myocardium in the same way that the fibrous pericardium can. The more general term constriction would therefore seem appropriate until an anatomical diagnosis has been established. It should thus be recognised that abnormalities of the surface of the heart as well as the pericardium can result in the syndrome of constriction.
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/12/2019 2:48:28 AM
increase in volume accompanied by a fall in pressure can only occur if the capacity of the right atrium increases; this is mediated by motion of the tricuspid ring towards the apex of the right ventricle during ejection. The accentuated ring motion reflects the changes in the right ventricular function, with transverse axis excursion being reduced by the presence of constriction. No other cardiac abnormality could account for these findings. At necropsy, however, the pericardium was unremarkable and was not constricting the heart. It would Acknowledgements The authors are grateful to Dr. D.G. Gibson for his helpful comments during the preparation of this report. Reference 1 Fowler N, Bove KE, Dunba S, Mayer R: Fatigue, Dyspnea and abdominal swelling in a 13 year old boy. Am Heart J 1978;96:533-542. 440 Owen/Trotter Physiological Features of Pericardial Constriction in the Absence of Pericardial Disease
