Address for correspondence:
Ammar A. Hasnie, MD
1720 2nd Ave S
BDB 327
Birmingham, AL 35233
Email:
ammarhasnie@uabmc.edu
Phone: 205-934-2490
Fax: 205-975-6424
Abstract: Constrictive pericarditis is an uncommon cause of
right-sided heart failure. We present a case of idiopathic constrictive
pericarditis presumably following a viral episode of acute pericarditis
several months prior to the patient’s presentation. This case highlights
the necessity for a high clinical index of suspicion. Importantly, a
series of imaging modalities were required to confirm the diagnosis and
lead to a successful surgical intervention.
Clinical Vignette: A 31-year-old man presented to the emergency
department with progressively worsening abdominal swelling and
unintentional weight loss for five months. He denied any fever, chills,
night sweats, chest pain, shortness of breath, palpitations, orthopnea,
paroxysmal nocturnal dyspnea, or syncope. The patient had a history of
generalized anxiety and major depression. He was not on any medications
and denied drug or alcohol use. He was adopted and unable to provide any
family history.
On presentation his blood pressure was 105/68 mmHg and heart rate was
106 beats per minute. On physical examination his abdomen was non-tender
but distended with shifting dullness. His cardiac exam revealed normal
S1/S2 heart sounds with no murmur, rub, or gallop. His lungs were clear
to auscultation. There was trace pitting edema of his lower extremities
and jugular venous distension (JVD) 3 centimeters above his clavicle. We
were unable to appreciate prominent waveforms or x/y descents and an
electrocardiogram showed a normal sinus rhythm.
Abdominal ultrasound was suggestive of cirrhosis with large volume
ascites. A diagnostic and therapeutic paracentesis was performed with 11
liters removed. His peritoneal fluid studies yielded an albumin of
2.8g/dL and serum ascites albumin gradient (SAAG) of 1.5g/dL. While we
initially suspected cirrhosis his high protein, high SAAG ascitic fluid
shifted our differential to include a cardiac cause of his ascites as
well.
A transthoracic echocardiogram showed a left ventricular ejection
fraction (LVEF) of 50-55% and thickened pericardium (Figure 1). There
was evidence of ventricular interdependence with resulting respiratory
variation in aortic flow velocity (Video 1). Medial mitral annular e’
velocity was 27.4 cm/s and hepatic vein expiratory diastolic reversal
ratio was 0.82(Figure 2A and 2B).
Cardiac MRI was subsequently completed which redemonstrated a thickened
pericardium, measuring 6mm, without evidence of a pericardial effusion
or calcification. A prominent diastolic interventricular septal bounce
and LVEF of 53% was noted on Cardiac MRI as well (Video 2). He was
diagnosed with chronic constrictive pericarditis and scheduled for
pericardiectomy. However, the etiology for his pericardial disease was
unclear. A purified protein derivative (PPD) skin test was non-reactive,
and he had no previous surgeries or radiation.
In the operating room his thickened pericardium was directly visualized,
and pericardial stripping was performed (Figure 3). Specimens were then
sent for histopathologic examination (Figure 4) which ultimately
revealed the cause of his constrictive pericarditis to be idiopathic
(most likely post-viral).
The patient recovered rapidly after pericardial stripping and was
discharged home four days later. At three-month follow-up he had
complete resolution of his ascites.
Discussion: Constrictive pericarditis arises secondary to
chronic inflammatory changes resulting in fibrous thickening of the
pericardium. Scarring can progressively restrict ventricular filling
past early diastole.1 Clinical presentation can vary
but patients tend to complain of chronic symptoms secondary to volume
overload including peripheral edema, dyspnea on exertion, or worsening
abdominal distension as seen in this case.
The first line imaging modality for patients with suspected constrictive
pericarditis is transthoracic echocardiogram according to 2015 European
Society of Cardiology Guidelines.2 Key imaging
findings include ventricular interdependence – a result of
non-compliant pericardium preventing diastolic ventricular filling.
Normally inspiration decreases intrathoracic pressure allowing inflow of
blood into both ventricles. In constrictive pericarditis, inspiration
results in decreased intrathoracic pressure without significant change
in the intracardiac pressures. Intrathoracic-intracardiac dissociation
leads to right ventricular (RV) expansion and shifts the
interventricular septum to the left as the stiff pericardium limits left
ventricular (LV) filling. In expiration the septum shifts towards the
right lowering the RV’s ability to fill. Additional echocardiographic
findings suggestive of constrictive pericarditis include expiratory
hepatic vein diastolic flow reversal ratio (≥ .79 cm/s) and elevated
medial e’ velocities (≥ 9cm/s). Each of these findings are independently
associated with the diagnosis of constrictive pericarditis and were seen
in our patient. The Mayo Clinic Criteria for echocardiographic evidence
of constrictive pericarditis found the combination of these three
variables had a diagnostic specificity of 97% for constrictive
pericarditis.3
Accordingly, ventricular interdependence seen on echocardiogram can be
demonstrated on cardiac catheterization. Simultaneous RV and LV pressure
tracings with discordant respiratory changes are pathognomonic, and
remain the most sensitive and specific finding, for constrictive
pericarditis.4 As compared to a restrictive
cardiomyopathy, or even a normal heart, concordant respiratory variation
is seen instead.
Although direct visualization of pressure tracings is classically the
gold standard when confirming constrictive pericarditis, it no longer
remains a first-line method for diagnosis. Cardiac MRI has evolved into
a more comprehensive imaging modality. Not only does it allow for
visualization of the pericardium, but also the impact on the structure
and function of the myocardium. Real-time imaging during free breathing
can evaluate hemodynamics during cardiac filling which emulates what we
elicit with cardiac catheterization. Over time Cardiac MRI has presented
a viable non-invasive test to contrast catheterization as the gold
standard for diagnosis. Another novel approach, Biderman et al.
described the utility of a tagged myocardium study measuring
visceral-parietal pericardial adherence to determine constriction
offering another alternative method when trying to confirm an elusive
diagnosis such as constrictive pericarditis.5
Constrictive pericarditis remains a difficult, but important diagnosis
given surgical interventions available for treatment. The use of
multiple imaging modalities allowed us to confirm the diagnosis of
idiopathic chronic constrictive pericarditis followed by successful
surgical intervention.