|INTERESTING CASE REPORT
|Year : 2020 | Volume
| Issue : 1 | Page : 53-57
Pericardial Constriction with Severe Mitral Regurgitation: An Uncommon Association
Saurabh Ajit Deshpande, A. George Koshy, Mathew Iype, K. Sunitha Viswanathan
Department of Cardiology, Government Medical College, Thiruvananthapuram, Kerala, India
|Date of Submission||05-May-2019|
|Date of Decision||31-Oct-2019|
|Date of Acceptance||24-Nov-2019|
|Date of Web Publication||11-Apr-2020|
Dr. Saurabh Ajit Deshpande
Apurva Building, 3rd Floor, Opposite Shardashram Society, Bhavani Shankar Road, Dadar (West), Mumbai - 400 028, Maharashtra
Source of Support: None, Conflict of Interest: None
Pericardial constriction is a condition, in which there is a lack of transmission of changes in intrathoracic pressures to the structures encased in pericardium due to thickened, fibrosed, and most of the cases, calcified pericardium. A 24-year-old male presented to us in right heart failure. There was a history of diagnosis of acute rheumatic fever with carditis in childhood. X-ray and echocardiogram gave us a probable diagnosis of rheumatic constrictive pericarditis (CP) with mitral regurgitation (MR). The diagnosis of CP was confirmed with computed tomography chest and invasive hemodynamic assessment. He had significantly elevated pulmonary artery (PA) pressures and elevated left ventricular dip diastolic pressures, which were unexpected findings. We have discussed the probable causes of associated MR and elevated PA pressures in this case report. We have also tried to differentiate CP from restrictive cardiomyopathy, specifically endomyocardial fibrosis, which is common in the state of Kerala.
Keywords: Constriction, endomyocardial fibrosis, mitral regurgitation, rheumatic
|How to cite this article:|
Deshpande SA, Koshy AG, Iype M, Viswanathan KS. Pericardial Constriction with Severe Mitral Regurgitation: An Uncommon Association. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:53-7
|How to cite this URL:|
Deshpande SA, Koshy AG, Iype M, Viswanathan KS. Pericardial Constriction with Severe Mitral Regurgitation: An Uncommon Association. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2020 Jun 1];4:53-7. Available from: http://www.jiaecho.org/text.asp?2020/4/1/53/282198
| Introduction|| |
Constrictive pericarditis (CP) is a condition, in which there is a lack of transmission of changes in intra-thoracic pressures to the structures encased by the pericardium due to thickened, fibrosed, and in most of the cases, calcified pericardium. Any cases presenting with acute inflammation of the pericardium, can go into chronic constriction, and there are many suspected etiologies, but most cases are idiopathic. Among the other common causes are cardiac trauma and surgery, tuberculosis and other infectious diseases, neoplasms (particularly lung and breast), radiation therapy, renal failure, connective tissue diseases, etc. There has been a controversy regarding rheumatic fever and rheumatic pericarditis going into constriction, but a few authors still do consider it a possible causal association.
| Clinical Presentation|| |
The patient is a 24-year-old gentleman, a mason worker by occupation and a resident of Varkala, Kerala. He presented with insidious onset heart failure at the age of 8 years and was evaluated by cardiologist and was diagnosed to have acute rheumatic fever with carditis and pericardial effusion. As he had cardiac tamponade, the pericardial effusion was aspirated. He was treated as an inpatient for almost a month and was discharged on secondary prophylaxis and diuretics.
He remained in the New York Heart Association functional Class II till 5 months ago, when his functional class worsened to Class III, with a few episodes of paroxysmal nocturnal dyspnea. He was admitted to our hospital due to significant worsening of symptoms and history of recent onset hemoptysis.
On admission to our hospital, he was in atrial fibrillation (AF) with a heart rate of 88/min and blood pressure of 120/84 mmHg. Jugular venous pressure was markedly raised. Apex beat was palpable in the 6th left intercostal space and was forceful in character. Grade 2 left parasternal heave was palpable. P2 was loud and a grade 3/6 pan systolic murmur was audible over the apex with an audible left ventricular (LV) third heart sound. Breath sounds were bilaterally equal and with no adventitial sounds. Liver margin was felt 8 cm below the costal margin and was mildly tender. Free fluid was present in the abdomen.
Electrocardiogram showed AF with controlled ventricular rate. Chest X-ray posteroanterior view [Figure 1] and lateral views [Figure 2] were taken, which showed calcification in the area of atrioventricular (AV) groove on LV side (mainly) and some part along LV border.
Transthoracic two-dimensional echocardiography showed markedly dilated left atrium, measuring 7 cm. Mitral valve appeared normal with no thickening and no evidence of rheumatic involvement. There was no evidence of prolapse or chordal rupture. Parasternal short-axis view showed dilated LV with good LV systolic function (LV ejection fraction of 70%). A hyperechoic structure was identified in relation to the inferior wall with evidence of acoustic shadowing. Apical four-chamber view showed all four chambers were dilated. Mitral and tricuspid valves were morphologically normal. The hyperechoic structure was more prominent on the LV side, and it was stationary with respect to myocardial movement [Figure 3]. This indicated that the structure was not a part of the myocardium and mostly was involving the pericardium. Since it looked hyperechoic, it was presumed to be calcium. There was no diastolic gradient across the mitral valve.
As per clinical suspicion and the presence of calcification, a likely diagnosis of calcific pericarditis was made. Hence, the other signs of CP were sought. Medial annular tissue velocity [Figure 4] (0.25 m/s) was found to be more than lateral annular tissue velocity [Figure 5] (0.17 m/s). Spectral Doppler interrogation of hepatic veins, in the subcostal view, showed a diastolic reversal of the flow. The mitral and tricuspid inflow showed increased velocity on the right side in inspiration and associated decreased velocity on the left side. On expiration, there was decreased tricuspid inflow velocity [Figure 6] and increased mitral inflow velocity [Figure 7]. There was right-sided overload and dilated inferior vena cava with lack of inspiratory collapse.
On color Doppler interrogation, tricuspid regurgitation was noted. On the left side, color flow across the mitral valve showed regurgitant jets, two central and one eccentric, which was hugging the interatrial septum [Figure 8]. The cause of mitral regurgitation (MR) was not certain. Hence, the patient was taken up for transesophageal echocardiography (TEE). On TEE, mitral leaflets were morphologically normal. There was evidence of three jets, similar to ones seen on transthoracic echocardiography. Tricuspid annular diameter was 30–34 mm.
To know the location and extent of calcification, the patient was taken up for computed tomography (CT) chest with angiography. It was confirmed that the calcification was more in the left-sided AV groove and was seen to be extending into the myocardium [Figure 9].
|Figure 9: Three-dimensional reconstruction of the contrast-enhanced computed tomography scan showing pericardial calcification extending into myocardium|
Click here to view
Even after all these investigations, there was confusion regarding the diagnosis-restrictive cardiomyopathy (especially endomyocardial fibrosis [EMF]) or CP. As per the Shafer's Classification (1965), Type III EMF can show focal perivalvular involvement. So to confirm the diagnosis, the patient was then taken up for invasive hemodynamic evaluation, i.e., right heart catheterization (RHC).
On RHC, there was raised right atrial (RA) mean pressure (34 mmHg). RA waveform showed the absence of a wave and prominent y descent. Right ventricular (RV) pressure tracing [Figure 10] showed increased RV systolic and diastolic pressure. The ratio of RV end-diastolic pressure and RV systolic pressure was 40/70 = 0.57 (>0.33). Mean pulmonary artery pressure and pulmonary capillary wedge pressure were elevated [Figure 11], indicating postcapillary pulmonary hypertension. Simultaneous LV/RV tracing showed diastolic equalization of LV and RV pressure and ventricular interdependence [Figure 12]. Although the dip diastolic pressures were elevated, the typical “square root sign” of the ventricular filling pattern could still be demonstrated. LV rapid filling wave was found to be more than 7 mmHg, which favored the diagnosis of CP.
|Figure 11: Right heart catheterization: Pulmonary capillary wedge pressure at rest|
Click here to view
| Discussion|| |
The diagnostic investigations for CP in most of its parts are to differentiate it from restrictive cardiomyopathy. If we go through the evolution of diagnosis of these conditions, the earliest studies were based on autopsy-based diagnoses. The diagnosis of CP was earlier based on dyspnea and intermittent pulse by Richard Lower et al. (1669), Kussmaul's paradoxical pulse (1873), and the entity known by the eponym “Pick's disease.” RHC for CP was first described by Paul Dudley White (1935) and RV dip and plateau sign by Hansen et al. Use of CT and magnetic resonance imaging in the diagnosis of CP was first described by Isner et al. and Soulen et al. (1985), respectively. In our case, we have tried to use the basic available investigations to come to the diagnosis of CP, which are similar to those used by most authors.
Our case being from the state of Kerala, the closest differential for us, was the most common form of restrictive cardiomyopathy found in this part of India – EMF. The disease can present as both left and right heart failure depending on the predominant ventricle affected in the disease process. LVEMF can lead to significant pulmonary arterial hypertension. There is decreased medial annular velocity and medial to lateral annular velocity ratio on echocardiogram. In constriction, the velocity as well as ratio increase. The transmitral flow shows normal respiratory variation, as against exaggerated variation in the case of constriction. Pericardial calcification is not seen in EMF. Imaging and hemodynamic correlates of ventricular interdependence are also not seen in the case of EMF.
Although it is commonly described that pericardial constriction affects all parts of the pericardium equally, there have been instances where the constriction was found to be localized., The signs and symptoms of these localized pericardial disease forms depend on the parts affected by the constriction. They can be asymptomatic or present as RV inflow tract obstruction or mid-ventricular obstruction (similar to mid-ventricular hypertrophic cardiomyopathy). Our case has features of generalized pericardial constriction with associated possible affection of functioning of mitral valve apparatus causing MR.
We postulate that MR is due to abnormal function of the AV fibrous ring. The mitral annulus, though in itself a relatively simple structure, has a complex and varying shape during the cardiac cycle due to its attachment to the muscle of the left ventricle below in its posterior two-thirds, circumferentially to the muscle of the left atrium above, and the aortic root anteriorly. The area of the mitral annulus is reduced by about 25% during late diastole due to the contraction of circular muscle fibers in the left atrial wall. Hence, it forms a valley like structure during systole to promote proper leaflet apposition. Furthermore, LV contraction contributes to a 10%–15% reduction in the annular area. We think that this part of the normal physiology was diseased in our case, which resulted in MR. The above mentioned atrial contribution will be lost in the presence of AF. MR associated with pericardial constriction has been earlier reported by Mittal.
There have been many etiological factors considered in patients with CP. In the Indian subcontinent, where tuberculosis is endemic, the most common cause of chronic pericardial constriction is considered to be tuberculosis.
| Conclusion|| |
There have been a few instances where there was a positive history of acute rheumatic fever in cases of CP. Most of these patients were found to have rheumatic affection of the cardiac valve(s), most commonly the mitral valve. Instances of acute rheumatic fever with pericardial effusion in childhood progressing to chronic CP in later life continues to be controversial. In our case, the patient had a history of rheumatic fever with pericardial effusion at a young age which had led to cardiac tamponade necessitating aspiration. Hence, we propose that this could be a case of “rheumatic CP.” This should not be confused with rheumatoid CP, which is pericardial affection in a diagnosed case of rheumatoid arthritis.
In the typical hemodynamics of CP, RV dip diastolic pressure is found to be normal (<5 mmHg); but, in our case, it was found to be elevated. This can be due to associated RV systolic failure. Furthermore, in our case, there was some involvement of the myocardium in the calcification process. This might have contributed to some restrictive-like physiology with an increase in RV diastolic pressure.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Tse G, Ali A, Alpendurada F, Prasad S, Raphael CE, Vassiliou V. Tuberculous constrictive pericarditis. Res Cardiovasc Med 2015;4:e29614. [Full text]
Przybojewski JZ. Rheumatic constrictive pericarditis. A case report and review of the literature. S Afr Med J 1981;59:682-6.
Gupta PN, Kunju SM, Vishwanathan S, Thomas JM, Kumar BR. An uncommon picture of endomyocardial fibrosis: No embolism yet. Heart Asia 2013;5:71-3.
Fowler NO. Constrictive pericarditis: Its history and current status. Clin Cardiol 1995;18:341-50.
Hansen AT, Eskildsen P, Gotzsche H. Pressure curves from the right auricle and the right ventricle in chronic constrictive pericarditis. Circulation 1951;3:881-8.
Isner JM, Carter BL, Bankoff MS, Konstam MA, Salem DN. Computed tomography in the diagnosis of pericardial heart disease. Ann Intern Med 1982;97:473-9.
Soulen RL, Stark DD, Higgins CB. Magnetic resonance imaging of constrictive pericardial disease. Am J Cardiol 1985;55:480-4.
Geske JB, Anavekar NS, Nishimura RA, Oh JK, Gersh BJ. Differentiation of constriction and restriction: Complex cardiovascular hemodynamics. J Am Coll Cardiol 2016;68:2329-47.
Krakulli K, Prifti E, Gjergo H, Hasimi E. Localized constrictive pericarditis compressing and obstructing the right ventricular inflow tract due to a giant anterior calcified cardiac mass. A case report. Int J Surg Case Rep 2017;39:276-9.
Gautam MP, Gautam S, Sogunuru G, Subramanyam G. Constrictive pericarditis with a calcified pericardial band at the level of left ventricle causing mid-ventricular obstruction. BMJ Case Rep 2012;2012. pii: bcr0920114743.
Lawrie GM. Structure, function, and dynamics of the mitral annulus: importance in mitral valve repair for myxamatous mitral valve disease. Methodist Debakey Cardiovasc J 2010;6:8-14.
Mittal SR. Mild mitral and tricuspid regurgitation secondary to pericardial constriction. J Assoc Physicians India 2014;62:529-30.
Thould AK. Constrictive pericarditis in rheumatoid arthritis. Ann Rheum Dis 1986;45:89-94.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]