|CURRICULUM IN CARDIOLOGY - CASE DISCUSSION
|Year : 2019 | Volume
| Issue : 2 | Page : 111-115
Faraz Ahmed Farooqui
Department of Cardiology, Cardiothoracic and Neurosciences Centre, All Institute of Medical Sciences, New Delhi, India
|Date of Submission||23-Jul-2019|
|Date of Decision||28-Jul-2019|
|Date of Acceptance||02-Aug-2019|
|Date of Web Publication||19-Aug-2019|
Dr. Faraz Ahmed Farooqui
Department of Cardiology, Cardiothoracic and Neurosciences Centre, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
A patient presented with Ascites for 3 months. Clinical examination showed ascites , presence of atrial fibrillation and a raised JVP. Further evaluation helped make a diagnosis of constrictive pericarditis. The questions that arise at each step of evaluation of such a patient starting from the history to examination and investigation is discussed in this bedside case discussion.
Keywords: Ascites, bedside case discussion, constrictive Pericarditis, restrictive cardiomyopathy
|How to cite this article:|
Farooqui FA. Constrictive pericarditis. J Pract Cardiovasc Sci 2019;5:111-5
| Clinical Presentation|| |
A 20-year-old male patient, resident of Bihar, presented with the following:
- Abdominal distension and facial puffiness for 3 months
- Bilateral lower limb swelling for 1 month
- Exertional dyspnea for 1 month.
The patient was apparently asymptomatic 3 months ago when he began to develop abdominal distension, which was insidious in onset and gradually progressive. There was associated facial puffiness. Two months later, he developed swelling of bilateral lower limbs. He also gives a history of the New York Heart Association (NYHA) Class II exertional dyspnea which was insidious in onset. However, dyspnea has not shown any progressive worsening. There was no associated orthopnea or paroxysmal nocturnal dyspnea (PND). The patient gives a history of easy fatigability. The patient would have intermittent relief in his symptoms with diuretics. However, there would be a recurrence of symptoms on stopping medications. There is a history of early satiety; however, appetite has remained normal. There was no history of palpitations, syncope, or cyanosis. There was no history of jaundice, hematemesis, or melena. The patient does not give any history of previous blood transfusions or high-risk behavior.
Past history was significant for intake of antitubercular therapy (ATT) 1 year ago for pleural effusion. He had taken a complete course of ATT. There was no history of trauma to the chest, prior chest surgeries, or radiation therapy.
Examiners: Please summarize the case history.
Examinee: A 20-year-old male patient has presented with a 3-month history of progressive abdominal distension followed by pedal edema and NYHA II dyspnea without associated orthopnea/PND. There is a history of past extrapulmonary tuberculosis with a history of ATT intake.
Examiners: What are your differential diagnoses based on your history?
Examinee: I would like to consider the following differentials based on the history.
- Constrictive pericarditis – In the context of systemic venous congestion, a history of abdominal distension preceding pedal edema (ascites precox) and a past history of extrapulmonary tuberculosis make constrictive pericarditis the most likely diagnosis
- Restrictive cardiomyopathy (RCM) – Prominent systemic venous congestion associated with dyspnea may be a presenting feature of RCM. RCM is more likely in a patient with a predisposing disease such as diabetes mellitus or amyloidosis. Uncommon causes of RCM include endomyocardial fibrosis, iron-overload cardiomyopathy, and storage disorders such as Fabry disease, radiation heart disease, and idiopathic RCM,
- Severe tricuspid regurgitation (TR) – Organic TR is an important differential diagnosis that closely mimics pericardial constriction in its clinical presentation. Organic TR may be congenital or acquired. Ebstein's anomaly is an important cause of organic TR. Other causes of organic TR include tricuspid valve (TV) prolapse, carcinoid syndrome, trauma, dilated cardiomyopathy, infective endocarditis (IE), or following surgical excision of the TV in patients with IE unresponsive to the medical management.
Examiners: What is the normal thickness of the pericardium?
Examinee: The normal thickness of the pericardium is ≤2 mm. A thickened pericardium on computed tomography is defined a pericardial thickness >4 mm.,
Examiners: What is the most common cause of constrictive pericarditis?
Examinee: The most common cause of constrictive pericarditis in the developed world is postsurgical followed by idiopathic constrictive pericarditis. Tuberculosis continues to be the most common cause of constrictive pericarditis in India.
Examiners: What percentage of patients develop constrictive pericarditis after cardiac surgery?
Examinee: Overall, 0.2%–2.4% of patients develop constrictive pericarditis after cardiac surgery.
Examiners: What is the average duration of presentation of postsurgical constrictive pericarditis?
Examinee: After cardiac surgery, the interval to presentation with constrictive pericarditis is on average 2 years but can range from as little as 1 month to >15 years.,,
Examiners: Describe the pathophysiology of constrictive pericarditis?
Examinee: Constrictive pericarditis arises after a cycle of injury and inflammation, which might be initiated several days, months, or years before clinical presentation, although the precise event often remains elusive. Damage to pericardial mesothelial cells is associated with an acute reduction of tissue-type plasminogen activator and reduced fibrinolytic activity, with fibrinous inflammation and adhesion formation, which is a possible mechanism for pericardial fibrosis, although this mechanism is yet to be proven. Most patients (>80%) with constrictive pericarditis who undergo pericardiectomy have nonspecific fibrocalcific thickening of the pericardial tissue, with evidence of ongoing acute or chronic inflammation of the pericardium. In contrast to late presentations, reduced pericardial compliance early in the disease course (usually <3 months) is more often caused by inflammation than by fibrosis.,
Examiners: What is your understanding of the hemodynamics of constrictive pericarditis?
Examinee: The principal hemodynamic abnormality in constrictive pericarditis is loss of pericardial compliance, with a reliance on elevated ventricular pressures to maintain cardiac filling and output, which eventually leads to primary diastolic heart failure. The constricting pericardium insulates the heart from respiratory intrathoracic pressure changes, which make the atrioventricular pressure gradient decrease on the left during inspiration, and increase during expiration. In healthy individuals, intrathoracic pressure decreases by approximately 5–10 mmHg during inspiration, and this pressure change is fully transmitted to the intracardiac cavities. However, in patients with a noncompliant or thick pericardium, the intrathoracic pressure change is not entirely transmitted to the intracardiac cavities. The extrapericardial components of the vena cavae and pulmonary veins are still subjected to these intrathoracic pressure changes, leading to enhanced variation between left and right filling pressures. Consequently, the pressure difference between the pulmonary veins and the left ventricle during diastole decreases with inspiration and increases on expiration, with opposing changes between the vena cavae and right ventricle. In patients with constrictive pericarditis, increased pericardial constriction leads to a fairly fixed combined volume of the left and right ventricles, such that atrioventricular filling of the right ventricle increases with inspiration and decreases with expiration. Ventricular interdependence is a characteristic hemodynamic feature of constrictive pericarditis.
Examiners: What is the mechanism of dyspnea in constrictive pericarditis?
Examinee: Exertional dyspnea in constrictive pericarditis is the result of limited or no increase in cardiac output with physical exertion. Orthopnea and PND are exceedingly rare in cardiac catheterization procedure (CCP). This is because pulmonary venous pressure rarely rises high enough to cause interstitial edema of the lung.
Examiners: What is ascites precox and what is the underlying mechanism?
Examinee: Ascites in constrictive pericarditis is out of proportion to pedal edema. It usually occurs earlier than pedal edema. This is referred to as ascites precox. Suggested mechanisms include:
- Partial constrictor effect on at least one of the hepatic veins, as it enters the right atrium
- Relatively high atrial pressures compared to other causes
- Hypoalbuminemia resulting from protein-losing enteropathy
- Cardiac cirrhosis
- Increased capillary permeability
- Impedance to lymph flow.,
| Examination|| |
On examination, the patient is thin built with a body mass index of 17. He is afebrile. The pulse rate is 96 beats/min. The pulse is irregularly irregular with apex pulse deficit of 20 bpm. No pulsus paradoxus is noted. The blood pressure is 104/70 mmHg in the right upper limb and 106/72 mmHg in the right lower limb. There is bilateral lower limb pedal edema. The jugular venous pressure (JVP) is raised (12 cm above the sternal angle at 45°) with absent “a” wave and prominent “y” descent. There is no pallor, icterus, or lymphadenopathy. The oxygen saturation by pulse oximetry is 100%. There are no peripheral signs of chronic liver disease.
| Cardiovascular Examination|| |
The precordium is symmetrical with no deformity or bulge. The apex is visible in the fifth intercostal space about 1 cm medial to the midclavicular line. On palpation, the apex is tapping and is of a left ventricular (LV) type. There is no parasternal heave. On auscultation, the first and second sounds are normal. A pericardial knock is present. No murmur is heard.
| Other Systems Examination|| |
The abdomen is soft. The liver is palpable 4 cm below the costal margin. The liver dullness is in the fifth right intercostal space, and the liver span is 14 cm. The liver is nontender and nonpulsatile. Shifting dullness is presented suggestive of ascites. The spleen is not palpable.
There is a dull percussion note with decreased breath sounds in the right infrascapular, infra-axillary, and mammary areas.
Examiners: Enumerate the positive findings in your examination.
- Irregularly irregular pulse with a variable volume
- B/L lower limb pitting pedal edema
- Elevated JVP with prominent y descent
- The presence of a high-pitched pericardial knock
- Shifting dullness on abdominal examination
- Dull percussion note with decreased breath sounds in the right infrascapular, infra-axillary, and mammary areas.
Examiners: What percentage of patients with constrictive pericarditis have atrial fibrillation?
Examinee: In a previous study, atrial fibrillation has found to be present in 22% of patients with constrictive pericarditis. Presence of pericardial calcification and higher duration of disease have been shown to be associated with a higher chance of developing atrial fibrillation.
Examiners: How common is pericardial knock-in constrictive pericarditis?
Examinee: Approximately half of the patients with constrictive pericarditis demonstrate an audible pericardial knock.
Examiners: How frequent is pericardial calcification in constrictive pericarditis?
Examinee: Nearly 25%–30% of patients with constrictive pericarditis demonstrate calcification on a chest radiogram. Patients with tuberculous constrictive pericarditis have a higher frequency (35%–50%) of radiologic calcification. Calcification in constrictive pericarditis is best observed in a lateral chest projection.
Examiners: What is transient constrictive pericarditis?
Examinee: Transient constrictive pericarditis is characterized by improvement and resolution of clinical features of constriction spontaneously or with anti-inflammatory therapy. Recurrence of constriction is not seen in patients who respond to anti-inflammatory therapy.
Examiners: Enumerate the Mayo Clinic Echocardiographic Criteria for constrictive pericarditis.
Examinee: Shown in [Figure 1].
|Figure 1: The Mayo Clinic Criteria for Echocardiographic Diagnosis of cardiac catheterization procedure|
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Examiners: Describe the cardiac catheterization findings in constrictive pericarditis.
- Significantly elevated right atrial pressure
- Prominent “y” descent in the right atrial waveform, commonly referred to as “Friedrich's sign” along with prominent “x” descent
- “M” or “W” pattern of the right atrial waveform due to the combination of elevated mean pressure, inconspicuous positive waves, and prominent descents
- Elevation and equalization of diastolic pressures (within 5 mmHg) in all cardiac chambers
- “Dip and plateau” or “square root sign” in the ventricular pressure waveforms which refer to the pattern of accentuated early dip in diastolic pressure followed by plateauing in mid-late diastole
- Elevation in the right ventricular systolic pressure (RVSP) (generally limited to <50 mmHg)
- A marked increase in the right ventricular end-diastolic pressure to levels more than one-third RVSP
- Failure to decline or increase in right atrial pressure with inspiration (Kussmaul's sign)
- Decrease in early diastolic gradient between pulmonary capillary wedge pressure and minimum LV diastolic pressure is during inspiration
- Ventricular interdependence as demonstrated by systolic area index (SAI) >1.1 × (SAI is then calculated as the ratio of RV area [mmHg × s] to the LV area [mmHg × s] in inspiration versus expiration).,,
Examiners: What is the role of contrast-enhanced magnetic resonance imaging (MRI) in a patient with suspected constrictive pericarditis?
Examinee: MRI provides an accurate assessment of pericardial thickness in patients with constrictive pericarditis. In addition, delayed gadolinium enhancement of the pericardium (especially ≥3 mm) is suggestive of ongoing pericardial inflammation and anti-inflammatory therapy may reverse the constriction in such patients.,
Examiners: Describe the outcomes after pericardiectomy surgery in patients with constrictive pericarditis.
Examinee: Pericardiectomy is associated with a 30-day mortality rate ranging from 5% to 10%. The prognosis is worse in patients with radiation-associated and postsurgical constrictive pericarditis. One-third of patients may develop recurrent symptoms due to the progression of underlying myocardial disease or less often due to recurrent constrictive pericarditis.,
Chest X-ray [Figure 2] shows calcification of the cardiac border. There is no pulmonary hypertension or cardiomegaly. Echo finds were suggestive of CCP with a dilated inferior vena cava and Doppler flow patterns suggest of CCP [Figure 3].
|Figure 2: Chest X-ray in cardiac catheterization procedure showing calcification.|
Click here to view
|Figure 3: Echocardiogram showing a dilated inferior vena cava, abnormal movement of the interventricular septum, increased respiratory variation of the mitral and tricuspid Doppler signals, and enhanced hepatic vein flow reversals.|
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[Figure 4] shows the catheterization findings with a rapid Y descent on the atrial waves, diastolic equalization, and a dip and plateau wave.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]