SEVERE RIGHT VENTRICULAR FAILURE FOLLOWING PERICARDIOCENTESIS: A CASE
REPORT OF PERICARDIAL DECOMPRESSION SYNDROME
Sergio A Pereza, MD; Jennifer
Amasthaa MD; Louis Vincenta, MD;
Carlos E Alfonsoa, MD; Eduardo de
Marchenaa, MD
a University of Miami, Division of Cardiovascular
Medicine, University of Miami Hospital, Miami, Florida.
Total word count: 1570
Funding: This research received no specific grant from anyfunding agency in the public, commercial, or not-for-profit
sectors
This study does not include any HIPPA patient identifier information.
IRB approval and written informed consent were not required.
Disclosures: All authors have reported having no relationships relevant
to the contents of this paper to disclose.
Address for Correspondence: Sergio A Perez, MD; 1400 NW 10th Avenue
Dominion Tower Suite 206A. Miami Florida 33136. Phone 305-987-7915.
Email:
Sergio.andres.perez@gmail.com
ABSTRACT.
Pericardial decompression syndrome is a rare but potentially fatal
complication following needle or surgical pericardiocentesis and should
be recognized as paradoxical hemodynamic deterioration. The exact
pathophysiology is unknown but is likely that several mechanisms
involving hemodynamic, ischemic and autonomic imbalance are involved.
There is no specific treatment other than supportive care, however,
early interventions such as aggressive heart failure therapy, inotropic
medications and sometimes mechanical circulatory support should be
implemented as mortality can be as high as 30%. We report a patient
presenting with right ventricular failure and cardiogenic shock
secondary to pericardial decompression syndrome.
INTRODUCTION
Pericardiocentesis is a lifesaving therapeutic procedure for patients
presenting with cardiac tamponade. It is a relatively safe procedure
however physicians must be aware of potential post-procedural
complications. The risk ranges from 4% to 10%, the most common being
arrhythmias, coronary artery or cardiac puncture, pneumothorax and
pneumopericardium [1]. Pericardial decompression syndrome (PDS) is a
rare but potentially fatal complication after pericardiocentesis defined
as paradoxical hypotension often with pulmonary edema and ventricular
dysfunction following fluid drainage in patients with cardiac tamponade.
Early recognition and aggressive initiation of supportive measures are
essential. Morbidity and mortality remain elevated. Here, we report a
case with an ominous outcome of profound cardiogenic shock after
percutaneous drainage of a large pericardial effusion causing cardiac
tamponade.
CASE PRESENTATION
73-year old woman presents to the hospital with 5-days of worsening
dyspnea and fatigue. Examination was noticeable for heart rate of 110
beats per minute, blood pressure of 90/50 mmHg, jugular venous
distention and pulsus paradoxus. Computed Tomography (CT) scan showed
multiple hepatic and pulmonary lesions; enlarged mediastinal and
abdominal lymph nodes and pleural and pericardial effusions concerning
for metastatic cancer of unknown primary origin (Figure 1). She had
elevated serum tumor markers including CA 19-9 and alpha feto-protein.
An echocardiogram showed a large pericardial effusion with right
ventricle (RV) diastolic collapse and 25-30% respiratory variation in
Doppler mitral inflow concerning for cardiac tamponade (Figure 2, Video
1).
She was taken to the catheterization laboratory for emergent needle
pericardiocentesis. Approximately 750 ml of sanguineous fluid was
drained and the patient was transferred to the ICU with the drain in
place. She experienced brief improvement in her symptoms and the drain
was removed 1 day later.
Repeat echocardiogram showed massive RV dilation and dysfunction and
persistent ventricular interdependence (Figure 3 and 4).
Electrocardiogram showed no changes suggestive of myocardial ischemia.
On day 2, patient developed hypotension. On exam she had cold
extremities with jugular venous distention. Laboratory showed metabolic
acidosis with elevated lactic acid. Hemodynamics from bedside right
heart catheterization using a Swan-Ganz catheter showed: right atrial
pressure of 30 mmHg, right ventricular diastolic pressure of 31 mmHg,
pulmonary artery pressure of 48/29 mmHg and pulmonary capillary wedge
pressure of 31 mmHg, consistent with right ventricular failure and
elevated pulmonary capillary wedge pressure likely secondary to
ventricular interdependence from RV enlargement. Cardiac output was 2.7
L/min with a cardiac index of 1.7 L/min/m². IV inotropic support was
initiated with dobutamine. CT angiography demonstrated a small sub
segmental pulmonary embolus in a right lower segmental branch. The
pulmonary embolism was not felt to be large enough to explain the degree
of right ventricular compromise.
She remained stable on dobutamine over the next 48 hours. Subsequently
she acutely deteriorated with refractory hypotension, hypoxemia,
lethargy and multi organ failure despite pharmacologic support.
Echocardiogram showed persistent RV enlargement with abnormal septal
motion (video 2-4). During emergent endotracheal intubation she had a
pulseless electrical activity cardiac arrest. Despite resuscitative
efforts she remained pulseless and was declared dead. Postmortem
examination was declined by the family.
DISCUSSION
Our patient illustrates a case of PDS, a rare but potentially fatal
complication after pericardiocentesis [2]. It is defined as
worsening of hemodynamics after an uncomplicated pericardial drainage in
patients with effusions and cardiac tamponade when hemodynamics are
expected to improve. Other names used in the past include “low cardiac
output syndrome” and “paradoxical hemodynamic instability” [3,
4]. Since the first description by Vandyke et al. in 1983 [5],
other reports allowed wider recognition of this complication among
clinicians.
The exact incidence of PDS is not precisely known given the wide
variability in occurrence rates in different small case series but is
estimated to be approximately 5%. Clinical factors associated with an
increased risk include history of malignancy or radiation therapy,
preexisting cardiomyopathy with decreased systolic function and
connective tissue disorders. In a study, surgical pericardiocentesis was
the only variable associated with increased mortality when compared with
needle pericardiocentesis in patients with PDS [6].
The onset of PDS after pericardial drainage is also variable. It can
occur immediately after a brief initial improvement in patient’s
hemodynamics or within a couple of days and most commonly presents as
acute congestive heart failure, often with pulmonary edema. In up to one
third of the cases, PDS may present with development of cardiogenic
shock.
The exact pathophysiology is not well understood but several mechanisms
involving hemodynamic, ischemic and autonomic imbalance have been
suggested. It is believed that right-sided chambers expansion resulting
from increased venous return after removal of the compressing
pericardial fluid can affect left ventricular filling and the effective
cardiac output. Simultaneously the net increase in pulmonary venous
return with an increased systemic vascular resistance can cause a
preload/afterload mismatch and result in congestive heart failure. An
additional contributing factor may be myocardial ischemia and stunning
caused by impaired coronary artery perfusion, as it is known that
increased intra-pericardial pressure affects maximal hyperemic coronary
flow [7]. Lastly, the acute withdrawal of sympathetic stimulus after
removal of effusion, has been hypothesized to provoke autonomic
imbalance, which is theorized to occur either because of an unmasking of
pre-existing myocardial dysfunction that was not apparent in the
hyperadrenergic state with increased circulating catecholamines or
because of induction of new myocardial dysfunction due to overwhelming
autonomic stress via a mechanism similar to that of stress-induced
cardiomyopathy. In fact, some have suggested that stress-induced
cardiomyopathy and PDS may not be distinct entities but rather belong to
the same spectrum of disease given the overlapping clinical presentation
seen in some cases [8]. Our case however, lacks typical features of
stress-induced cardiomyopathy: global RV involvement as opposed to
apical ballooning with preserved basal function, no chest pain and no
subsequent improvement of myocardial function.
There is no specific treatment for PDS other than supportive care. The
exact mortality rate is not well known, but has been estimated to be
around 30% based on case reports. Although ventricular dysfunction is
transient and is expected to recover in survivors of PDS, patients will
require advanced support measures such as aggressive heart failure
therapy, inotropic medications and the use of mechanical circulatory
support [9]. In cases of profound shock, the ideal type of
mechanical support depends on the degree of myocardial dysfunction as
well as the pattern of ventricular involvement, whether left, right or
bi-ventricular dysfunction is noted.
Currently there are no proven measures known to prevent PDS. Despite the
recommendation to drain fluid in < 1L steps to avoid acute
right-ventricular dilatation made by European Society of Cardiology 2004
guidelines [1], PDS may occur with drainage volumes < 500
ml. A reasonable strategy is to remove only enough fluid to alleviate
tamponade physiology and slowly remove the remaining fluid by leaving
the pericardial drain in place, especially in patients with
cancer-related effusions or impressive chamber collapse.
CONCLUSION.
This uncommon case of PDS highlights the high morbidity and mortality
associated with this complication, the possibility of instituting
preventative strategies in high-risk cases, and the importance of prompt
recognition of PDS and for close clinical monitoring and aggressive
supportive care.
REFERENCES
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LEGENDS.
FIGURE 1. (A) CT scan showing a large pericardial effusion. (B) CT scan
after pericardiocentesis showing significant RV enlargement (asterisk).
FIGURE 2. 2D-echocardiography four-chamber (A) and short-axis (B) views
revealing massive pericardial effusion (asterisk)
FIGURE 3. 2D-echocardiography after pericardiocentesis. Four-chamber (A)
and short-axis (B) views showing massive RV enlargement. Note the
diameter of the RV (solid arrow) in comparison to that of the left
ventricle (dashed arrow).
FIGURE 4. 2D-echocardiography after pericardiocentesis revealing
decreased TAPSE of 6 mm on M-Mode (A) and S‘ velocity of 6 cm/sec on
tissue Doppler (B) consistent with RV dysfunction. Pulsed-wave Doppler
> 25% mitral flow variation consistent with ventricular
interdependence before (C) and after (D) pericardiocentesis. (TAPSE:
tricuspid annular plane systolic excursion)
VIDEO 1. 2D-echocardiography, apical 4-chamber view revealing a large
pericardial effusion
VIDEO 2. 2D-echocardiography, apical 4-chamber view
VIDEO 3. 2D-echocardiography, parasternal short axis view
VIDEO 4. 2D-echocardiography, apical 4-chamber view with agitated saline
injection