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CASE (Phila)
CASE (Phila)
CASE : Cardiovascular Imaging Case Reports
2468-6441
Elsevier

S2468-6441(24)00121-X
10.1016/j.case.2024.05.005
Pericardial Pathologies
Effusion, Constriction, and/or Tamponade
To Drain or Not to Drain? Pericardial Decompression Syndrome
Mathias Isadora MD isandemathias@houstonmethodist.org
∗
Narasimhan Bharat MD
Guha Ashrith MD
Nagueh Sherif F. MD
Zoghbi William A. MD
Houston Methodist DeBakey Heart & Vascular Center, Houston, Texas
∗ Correspondence: Isadora Mathias, MD, Houston Methodist DeBakey Heart and Vascular Center, 6550 Fannin Street, Suite 1901, Houston, TX 77030. isandemathias@houstonmethodist.org
11 6 2024
8 2024
11 6 2024
8 8 428431
2024 by the American Society of Echocardiography. Published by Elsevier Inc.
2024
American Society of Echocardiography
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Graphical abstract

Highlights

• PDS is a potential complication of pericardiocentesis.

• Apply caution when considering a pericardiocentesis for effusions of unknown chronicity.

• Serial assessment with bedside TTE can guide slow pericardiocentesis to avoid PDS.

Keywords

Pericardial decompression syndrome
Pericardiocentesis
Right ventricle failure
Tricuspid regurgitation
Point-of-care echocardiography
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pmcIntroduction

Pericardial decompression syndrome (PDS) is a rare condition, mostly described in patients with known pulmonary hypertension (PH) and right ventricular (RV) failure.1 Pericardial decompression syndrome can result from drainage of a pericardial effusion of unknown chronicity, leading to acute pericardial decompression, loss of pericardial constraint, and potential RV failure and tricuspid regurgitation (TR), with worsening hemodynamic compromise. Bedside transthoracic echocardiography (TTE) has a pivotal role in preventing and diagnosing this condition. This case depicts a patient who presented with a circumferential pericardial effusion of unknown chronicity that was fully drained, resulting in an unexpectedly persistent shock state.

Case Presentation

A 59-year-old female patient, recently diagnosed with COVID-19, presented with worsening symptoms of malaise, generalized weakness, and lower-extremity pain. Medical history was significant for nonischemic cardiomyopathy with improved ejection fraction (biplane left ventricular ejection fraction 49% prior to admission), reportedly normal RV function (tricuspid annular plane systolic excursion of 2.1 cm), moderate mitral regurgitation, and moderate TR. The patient had ventricular tachycardia arrest 13 years prior with a cardiac resynchronization therapy-defibrillator (CRT-D) in situ, chronic atrial fibrillation with warfarin use, stage 4 chronic kidney disease, renal cell carcinoma, status post radical right nephrectomy a year before current admission, and obesity, status post Roux-en-Y gastric bypass 3 years before admission.

On the initial physical exam, the patient was in no acute distress, vital signs were heart rate of 70 beats per minute (bpm), blood pressure of 93/76 mm Hg, peripheral oxygen saturation 95% on room air, and temperature 96.6 °F. Focused cardiovascular exam showed regular rate and rhythm and diminished heart sounds with no audible murmurs, rubs, or gallops. The jugular venous pulse was elevated. Lungs were clear, and extremities were cold with no discoloration or edema.

Initial workup was significant for acute on chronic kidney injury with profound lactic acidosis and hyperkalemia. Furthermore, the international normalized ratio was found to be 11. Chest x-ray showed an enlarged cardiac silhouette and a CRT-D device (Figure 1), while electrocardiogram showed a ventricularly paced rhythm at 70 bpm and a low-voltage QRS (Figure 2).Figure 1 Chest x-ray, anterior-posterior projection, demonstrates clear lung fields, enlarged cardiac silhouette, and a CRT-D device in situ with leads visible in the right ventricle and coronary sinus.

Figure 2 Twelve-lead electrocardiogram demonstrates ventricular-paced rhythm at a rate of 70 bpm with diffusely low-voltage QRS.

Because of the worsening hypotension, the patient was admitted and started on vasopressors and empiric antibiotics for possible sepsis, had coagulopathies reversed, and had urgent continuous dialysis initiated to improve acidosis, hyperkalemia, and azotemia, with ultrafiltration rate cautiously monitored to avoid further hypotension.

Limited bedside TTE revealed normal left ventricular systolic function of 60% (single-plane method), reduced RV systolic function (fractional area change of 32%; Videos 1 and 2), moderate PH (RV systolic pressure 45 mm Hg, assuming a mean right atrial [RA] pressure of 15 mm Hg), and a large circumferential pericardial effusion with diastolic interventricular septal bounce and RV diastolic collapse (Figure 3, Videos 1 and 2).Figure 3 Two-dimensional TTE, parasternal long-axis end-diastolic view, demonstrates a large circumferential pericardial effusion with maximal echo-free diameter >2.0 cm (yellow arrows).

While planning possible pericardial drainage, the patient developed pulseless electrical activity cardiac arrest. Return of spontaneous circulation was achieved after 10 minutes of resuscitation measures. Given the persistent hypotension and hemodynamic instability in the context of echocardiographic features of tamponade, pericardiocentesis was performed, with drainage of 600 mL of bloody pericardial fluid, which was later confirmed by pericardial fluid analysis. Vital signs after drainage were unchanged, and the patient did not demonstrate any significant improvement. We reprogrammed the lower rate limit on the CRT-D device from 70 to 100 bpm to improve cardiac output by increasing the heart rate, with temporary improvement in the blood pressure. Despite these measures, the patient continued to deteriorate precipitously. A pulmonary artery (PA) catheter was placed and showed mean RA pressure of 17 mm Hg, with tall V waves, PA pressure 34/23 mm Hg, pulmonary capillary wedge pressure 19 mm Hg, transpulmonary pressure gradient 8 mm Hg, diastolic pulmonary gradient 4 mm Hg, SvO2 45%, CO 3.7 L/min, PA pulsatility index 0.64, and RA/pulmonary capillary wedge pressure ratio 0.9.

Repeat TTE shortly after drainage and 24 hours later did not show pericardial fluid reaccumulation but revealed severe biventricular failure and severe TR (Figure 4, Videos 3 and 4).Figure 4 Two-dimensional TTE, subcostal, early systolic view with color flow (left panel) and pulsed-wave (right panel) Doppler, demonstrates a dilated inferior vena cava and systolic flow reversal in the hepatic vein (red arrow); the noted “bubbles” represent continuous infusions from a femoral central venous catheter. Pulsed-wave spectral Doppler display of the hepatic vein demonstrates systolic flow reversal (yellow arrow) due to severe TR.

A decision was made to pursue mechanical circulatory support with an RV assistance device combined with an intra-aortic balloon pump. Unfortunately, the patient continued to decline and was transitioned to comfort care, passing away a few days later. The family declined postmortem evaluation.

Discussion

This patient’s precipitous and paradoxical clinical decline after pericardiocentesis appears to have been an unfortunate consequence of acute pericardial decompression (Videos 5 and 6). Pericardial decompression syndrome is a rare condition, mostly described in patients with known PH and RV failure,1 unlike our patient, who had no history of prior PH and only mild RV dysfunction and mildly elevated PA pressures.

Pericardial decompression syndrome presents as a paradoxical hemodynamic deterioration after uncomplicated pericardial drainage and is believed to have 3 possible mechanisms:2 (1) in patients with RV dysfunction, the pericardium performs an integral role in “constraining” the right ventricle, and abrupt removal of this increased intrapericardial pressure leads to unchecked enlargement of the ventricle; (2) RV overdistention with increased RV end-diastolic pressure after drainage can lead to reduced perfusion pressure for RV coronary bed and thus RV ischemia; (3) there is reduced sympathetic stimulation after resolution of tamponade physiology. The overloaded right ventricle enlarges further, promoting more septal displacement toward the left ventricle and decreasing cardiac output.3,4 About 60% of the patients have a history of malignancy, and 40% of cases had a neoplastic etiology of the effusion.2 This patient had a history of renal cell carcinoma a year prior, and although pericardial fluid analysis did not reveal a malignant etiology, a neoplastic effusion remained a possibility. The clinical presentation suggested that the pericardial effusion itself was not fully responsible for the initial deterioration and cardiac arrest. Additionally, we cannot exclude the possibility of effusive-constrictive pathology contributing to the clinical presentation, despite the absence of mitral and tricuspid inflow variation. Furthermore, the dilated inferior vena cava with minimal collapse is most likely due to marked elevation in RA pressures with RV dysfunction, and the interpretation of septal bounce is confounded by TR causing the volume overload pattern of septal movement.

The European Society of Cardiology5 recommends performing pericardiocentesis in a stepwise manner and limiting the amount of fluid removed to 1 L at a time; however, much less than that may be enough to provoke PDS and hemodynamic compromise in susceptible patients. A case series done by Thabet et al. in 20216 proposes a manometric approach to pericardiocentesis by measuring intrapericardial pressure and removing fluid just until intrapericardial pressure reaches 10 mm Hg. This has not been validated and is not part of the guidelines, but it can be a simple and promising approach to avoid PDS in patients at high risk, such as cases of PH or malignant effusions.

Conclusion

Pericardial decompression syndrome is a rare but potentially fatal complication of pericardiocentesis. Reasonable approaches to perform pericardiocentesis for large effusions of unknown chronicity, especially in patients with PH or neoplastic-related etiology, include (1) performing the procedure in a staged manner by removing the minimum amount of fluid necessary to achieve hemodynamic stability, based on both clinical and echocardiographic criteria; (2) not exceeding 1 L at a time; and (3) placing a pericardial drain for serial removal of additional fluid in the following hours during close monitoring of intrapericardial pressure.

Ethics Statement

The authors declare that the work described has been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans.

Consent Statement

The authors declare that since this was a non-interventional, retrospective, observational study utilizing de-identified data, informed consent was not required from the patient under an IRB exemption status.

Funding Statement

The authors declare that this report did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Disclosure Statement

The authors have no conflicts of interest to disclose.

Supplementary Data

Video 1

Two-dimensional TTE, parasternal long-axis view, demonstrates a large circumferential pericardial effusion with maximal echo-free diameter >2.0 cm and normal left ventricular systolic function. There is interventricular septal bounce but no clear RV diastolic collapse. The RV wall appears thickened.

Video 2

Two-dimensional TTE, apical 4-chamber view, demonstrates a large circumferential pericardial effusion with interventricular septal bounce and mildly reduced RV systolic function. An old pacemaker lead is visualized in the right ventricle.

Video 3

Two-dimensional TTE, apical 4-chamber view postpericardiocentesis, demonstrates resolution of the large effusion with progressive RV chamber and tricuspid annulus dilation, reduced RV systolic function, interventricular septal flattening consistent with RV volume overload, and poor coaptation of the tricuspid valve leaflets. Also noted is the interatrial septum, which now deviates toward the left atrium.

Video 4

Two-dimensional TTE, apical RV-focused view with color-flow Doppler postpericardiocentesis, demonstrates resolution of the large effusion with severe TR.

Video 5

Two-dimensional TTE, apical 4-chamber view before (left) and RV-focused view after (right) pericardiocentesis, demonstrates the abrupt anatomic and functional changes for direct comparison.

Video 6

Two-dimensional TTE with color-flow Doppler, apical 4-chamber view before (left) and RV-focused view after (right) pericardiocentesis, demonstrates the abrupt changes in TR for direct comparison.

Supplementary data related to this article can be found at https://doi.org/10.1016/j.case.2024.05.005.
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