Case presentation

Diana Girnita, MD, PhD

The Christ Hospital

CC: shortness of breath

42 yo Caucasian F pregnant, (P2G2) 28 weeks, seen by cardiology for dyspnea on exertion, since her first trimester of pregnancy that got progressively worse.

▪ In the last month, DOE with walking the length of 2 rooms, walking up the stairs and sometimes at rest. 

▪ Orthopnea episodes in the middle of the night.

▪ Pressure in the mid -chest, rated 2/10, constant, not irradiating, not accentuated by inspiration.

▪ Not feeling comfortable to lay flat in bed and will use up to 3 pillows to sleep

▪ Denies palpitations, cough, flu like symptoms, sick contacts or recent travel.

▪ At 21 weeks of pregnancy was dg with Hodgkin lymphoma, but chemotherapy was currently postponed close to her delivery term. 

▪ Had no similar complains with previous pregnancy, although had pedal edema.

PMH  Allergy   Hodgkin Lymphoma stage IIA

PMS▪ Hx appendectomy  ▪  D&c after delivery  ▪ Cesarean delivery

Allergies : Ciprofloxacin    (Hives)  

SH: Former Smoker -- 0.5 packs/day

FH: father with LAD infarction (died in the 40’s)

Home Medication ▪ cetirizine (ZYRTEC) 10 mg PO ▪ PRENATAL VITAMINS-IRON-FA PO 

ROS▪ Constitutional: No fevers, chills, or weight loss.  

▪ Skin: no skin, hair, nail changes, rash or pruritis

▪ Neurologic: No syncope, weakness, seizure disorder, headaches or gait abnormalities.  

▪ Endocrine: No history of DM, HLD, thyroid disorder

▪ Eyes: No blurred vision  

▪ Cardiovascular: + SOB,  No palpitations. No claudication.  

▪ Respiratory: + SOB , no wheezing, coughing, asthma, COPD or chronic bronchitis

▪ Gastrointestinal: No change in appetite, dysphagia, N/V/D/C  

▪ Psychiatric: No depressed mood / anxiety/No memory loss or altered mental status

Vital signs

▪ BP 127/70

▪ Pulse 83

▪ Temp 97.7 °F (36.5 °C) (Oral)

▪ Resp 18

▪ SpO2 95%

PHYSICAL EXAM

▪ Constitutional:  NAD, Non-toxic appearance▪ HEENT: NC/AT, Bilateral external ears, oropharynx moist, nose

normal.  Eyes normal. L side submandibular lymphadenopathy

▪ Neck: Normal ROM, No JVD, carotid upstrokes are preserved without audible bruits.

▪ Cardiovascular: RRR,  Normal S1 and S2. No rubs, gallops, or murmurs.

▪ Lungs:  Clear to auscultation.▪ GI: BS normal, Soft, No tenderness, No masses, No pulsatile

masses. No spleno/ hepatomegaly▪ Extremities:  Intact distal pulses, No pedal edema, No

tenderness.▪ Neurologic:  Alert & oriented x 3, Normal motor function, Normal

sensory function, No focal deficits.▪ Skin:  Warm, Dry, No erythema, No rash.

Labs

▪ Hb 12, Ht 34, WBC 10, Platelets 235

▪ EP1: Na133, K 4.3, Co2 18, AG 10, Bun 6, Cr 0.52, GFR 138

▪ Alk phosp 96

▪ Albumin 3.4

▪ Chol 184, HDL 39, LDL 128, TG

▪ ESR 82

ECG

CXR

CXRCXR 8/17 Large mediastinal and hilar

lymphadenopathy ; does not appear to be significantly changed.

CXR 09/06 Little change since August, 2012 with a mediastinal and bilateral hilar lymphadenopathy consistent with clinically reported Hodgkin's lymphoma. No evidence of acute cardiopulmonary disease.

MRI abdomen

1. No definite abdominal or retroperitoneal lymphadenopathy.

2. Partial visualization of mediastinal lymphadenopathy.

3. Left cardiophrenic lymphadenopathy.

4. Borderline splenomegaly.

ECHO 9/5▪ Left ventricle: The cavity size was normal. Wall

thickness was normal.

▪ Systolic function was mildly reduced. The estimated ejection fraction was 45%. Hypokinesis of theanteroseptal and inferoseptal myocardium.

▪ Left ventricular diastolic function parameters were normal.

▪ Pericardium, extracardiac: A moderate pericardial effusion was identified circumferential to the heart. There was no evidence of hemodynamic compromise. There was no chamber collapse.

▪ Respirophasic change in stroke volume was normal. 

Cardiology was consulted

Diagnostic approach

▪ Pericardiocentesis.

▪ Impression: Moderate to large pericardial effusion

▪ The fluid was noted to be clear yellow- placement of the pericardial catheter

▪ A repeat echo demonstrated resolution of the effusion

Pericardial effusion features

9/7/2012 Clarity, Fluid CloudyLymphs, Fluid 85Macrophage count 4Neutrophil Count, Fluid 11Nucl Cell, Fluid 3286RBC, Fluid 2681

LDH 203

Glucose 83

CEA <0.5

MICRO

No AFB isolated

No fungus isolated

Routine Cx- no organism seen

Anaerobes- pending

Cytology pending

ECHO 9/8 at discharge

▪ Left ventricle: The cavity size was normal. Wall thickness was normal.

▪ Systolic function was normal.

▪ The estimated EF 55% to 60%.

▪ Wall motion was normal; there were no regional wall motion abnormalities.

▪ Pericardium, extracardiac: A small, free-flowing pericardial effusion was identified circumferential to the heart. 

Pericardial effusion

Pericardial effusion▪ is an accumulation of fluid in the pericardial space,

with or without associated pericarditis.

Pericardial effusion

▪ can develop in patients with acute pericarditis or in association with a variety of systemic disorders.

▪ important implications ▪ for prognosis (pts with intrathoracic

neoplasm)▪ for diagnosis (myopericarditis or acute

pericarditis)▪ or both (as in Ao ascending dissection).

Etiology▪ Idiopathic 

▪ Infections A. Viral - Coxsackie, echo, adeno, EBV, CMV, influenza,

varicella, rubella, HIV, hepatitis B, mumps, parvovirus B19

B. Bacterial - Staph, Strep, Haemophilus, Neisseria, Chlamydia, Legionella, TB, Salmonella, Lyme disease.

C. Mycoplasma

D. Fungal - Histoplasmosis, aspergillosis, blastomycosis, coccidiodomycosis, actinomycosis, nocardia, candida

E. Parasitic - Echinococcus, amebiasis, toxoplasmosis

Etiology▪ Cardiac 

A. Early infarction pericarditis B. Late postcardiac injury syndrome

(Dressler's syndrome) C. Myocarditis D. Dissecting aortic aneurysm

▪ Trauma 

▪ Iatrogenic - Catheter and pacemaker perforations, cardiopulmonary resuscitation, post-thoracic surgery

▪ Malignancy

Etiology▪ Autoimmune 

A. Rheumatic diseases – SLE, RA, MCTD,scleroderma

B. Wegener's granulomatosis, PAN, sarcoidosis, IBD (Crohn's, ulcerative colitis), Whipple's, giant cell arteritis, Behcet's disease, rheumatic fever

▪ Drugs  (Procainamide, isoniazid, or hydralazine,cromolyn sodium, dantrolene, anticoagulants, thrombolytics, phenytoin, penicillin, phenylbutazone, doxorubicin)

▪ Metabolic (Hypothyroidism - primarily pericardial effusion, Uremia, Ovarian hyperstimulation syndrome)

Diagnostic approach

▪History (suspect in all cases of acute pericarditis)

▪ECG

▪CXR/ CT chest

▪Echo is required for establishing dg

ECG - Electrical alternans.

▪ beat-to-beat shift in the QRS (and sometimes the P wave) axis, amplitude, and/or morphology.

▪ occurs in association with swinging of the heart, usually in a large pericardial effusion

ECG - Low voltage QRS

Low voltage

▪ is probably due to short-circuiting of cardiac potentials by the fluid surrounding the heart.

▪ Defined as

▪ QRS complexes ≤5 mm (0.5 mV) in all of the limb leads.

▪ +/-- low voltage in the chest leads, QRS amplitude of ≤10 mm in V1 to V6

CXR

CT chest

ECHO –confirms the diagnosis

▪ Small collections of pericardial fluid can be physiologic if visible during ventricular systole.

▪ Effusions > 25 to 50 mL are seen as an echo-free space throughout the cardiac cycle

ECHO

Hemodynamic compromise assesment

▪ Factors that determine the degree of hemodynamic compromise include

the volume of pericardial fluidthe rate at which the effusion accumulates

whether or not the pericardium is scarred or adherent.

Acute vs subacute cardiac tamponade

ACUTE

▪ Sudden onset

▪ chest pain and dyspnea, and is life-threatening if not promptly treated.

▪ JVD markedly elevated

▪ Hypotension due to the decline in CO

▪ heart sounds muted.

▪ effusion is relatively small.

SUBACUTE

▪ less dramatic process.

▪ asymptomatic or c/o dyspnea, chest discomfort or fullness, fatiguability.

▪ Peripheral edema in chronic tamponade.

▪ Hypotension with a narrow pulse pressure (limited stroke volume)/ preexisting HTN may remain hypertensive due to the increased sympathetic activity

Acute vs subacute cardiac tamponade

Question no. 1

▪ 67 yo M is evaluated for an asymptomatic large pericardial effusion that has persisted for 4 months. He denies chest pain, dyspnea, fever, chills, cough, edema, and weight loss. He has a 30-year history of HTN which he takes amlodipine. CXR performed 4 months ago to evaluate chronic cough demonstrated an enlarged cardiac silhouette. Lab results at that time included normal CBC, electrolytes, creatinine, BUN, TSH. ANA and PPD were negative. Chest CT scan was normal. Echo performed 4 and 2 months ago showed a moderate pericardial effusion, no evidence of tamponade, and normal left and right ventricular function and size.

▪ PE: temp is normal, BP 135/75 mm Hg, HR 76/min, RR 16/min. Estimated CVP less than 5 cm H2O. Cardiac examination reveals muffled heart sounds, an absent apical impulse, and no pericardial friction rub. Pulmonary auscultation reveals normal breath sounds.

▪ Echo shows a large circumferential pericardial effusion and no findings of tamponade, and is otherwise without change from 4 and 2 months ago.

Which of the following is the most appropriate next step in management?

A. Colchicine

B. Indomethacin

C. Pericardiectomy

D. Pericardiocentesis

E. Prednisone

Answer

▪ Correct : D

▪ A pericardiocenthesis should be done when an idiopathic pericardial effusion persists more then 3 months.

▪ An idiopathic pericardial effusion lasting less than 3 months in a stable patient requires no specific therapy, but serial echocardiography based on clinical status, as was done in this patient, is advisable.

Cardiac tamponade – clinical features to look for:

▪ sinus tachycardia, even in the absence of frank hypotension, may indicate significant hemodynamic compromise from tamponade and serve as an indication for immediate pericardiocentesis.

▪ Elevated jugular venous pressure

▪ Pulsus paradoxus —  defined as an abnormally large decrease in systolic blood pressure (>10 mmHg) on inspirations

▪ Pericardial rub 

▪ Kussmaul's sign —  is the absence of an inspiratory decline in jugular venous pressure

Pulsus paradoxus

Question no. 2

▪ A 45 yo M is evaluated in ED for 3-day history of progressively worsening dyspnea on exertion to the point that he is unable to walk more than one block without resting. He has had sharp intermittent pleuritic chest pain and a nonproductive cough with myalgias and malaise for 7 days and has had orthostatic dizziness for 2 days. He is taking no medications.

▪ On PE: temp- 37.7°C (99.9 °F), BP 88/44 mmHg, HR- 125/min, and RR 29/min; BMI is 27. O2 sats at RA is 95%. Pulsus paradoxus is 15 mm Hg. Estimated central venous pressure is 10 cm H2O. Cardiac examination discloses muffled heart sounds with no rubs. Lung auscultation reveals normal breath sounds and no crackles. There is 2+ pedal edema. BP and HR are unchanged after a 500-mL intravenous normal saline challenge.

▪ Laboratory studies:

▪ Prothrombin time 12 s

▪ Activated partial thromboplastin time28 s

▪ Creatinine1.3 mg/dL (99.2 µmol/L); BUN 26 mg/dL (9.3 mmol/L)

▪ ECG: sinus tachycardia, diffuse low voltage, no ST-segment shifts.

▪ ECHO: shows a large circumferential pericardial effusion, right ventricular and atrial free wall diastolic collapse, normal LV systolic function, and EF of 70%.

▪ CXR shows an enlarged cardiac silhouette and no pulmonary infiltrates.

▪ Which of the following is the most appropriate treatment?

A.Dobutamine

B.Levofloxacin and tobramycin

C.Pericardiocentesis

D.Surgical pericardiectomy

Answer

▪ Correct answer: C

▪ This patient has examination findings that indicate cardiac tamponade. Physical examination reveals tachycardia, reduced blood pressure, distended jugular veins, elevated pulsus paradoxus (>10 mm Hg), and an unremarkable lung examination.

Malignant pericardial effusion

Etiology

▪ Neoplasm  A. Metastatic – Lung, breast Ca, Hodgkin,

leukemia, melanoma

B. Primary - rhabdomyosarcoma, teratoma, fibroma, lipoma, leiomyoma, angioma

C. Paraneoplastic

▪ Radiation 

Relative incidence of metastatic heart tumors in autopsy studies for frequent primary neoplasms.

Reynen K et al. Ann Oncol 2004;15:375-381

European Society for Medical Oncology

▪ J Thorac Cardiovasc Surg. 2011 Jan;141(1):34-8

▪ Wagner PL et al “Pericardial effusions in the cancer population: prognostic factors after pericardial window and the impact of paradoxical hemodynamic instability”

▪ Retrospective review of 179 pericardial windows performed for pericardial effusion over a 5-year period (2004- 2009)

▪ RESULTS: The most common malignancies were ▪ lung (44%)▪ breast (20%)▪ hematologic (10%)▪ gastrointestinal (7%)

▪ Heart Fail Rev. 2012 May 26

▪ Karatolios K et al “Diagnostic value of biochemical biomarkers in malignant and non-malignant pericardial effusion”

▪ Biochemical parameters and tumor markers to discriminate malignant from benign effusion in 105 pts

▪ Malignant pericardial effusions had significantly higher pericardial fluid levels of the tumor markers CEA, CA 19-9, CA 72-4, SCC and NSE as well as higher pericardial fluid hemoglobin (p < 0.001), WBC, LDH

▪ None of the biochemical or cell-count parameters tested proved to be accurate enough for distinguishing malignant from benign effusions.

▪ However, measurement of pericardial CA 72-4 levels offered a high diagnostic accuracy for malignancy, particularly in bloody pericardial effusions.

Cytology in pericardial effusions

▪ Cytology and pericardial biopsy — whenever there is a reason to suspect malignancy.

▪ Especially important if the effusion is hemorrhagic, and there is no history of antecedent trauma

▪ Negative cytology should not be used to exclude the diagnosis of malignancy, particularly if the index of suspicion is high

▪ A positive cytology may be predictive of a poorer outcome

Outcome prediction

▪ Prognosis and status of the underlying malignancy — most patients with a symptomatic malignant pericardial effusion have a short life expectancy (median two to four months)

▪ Patients with breast cancer or hematologic malignancies had a better median survival (9 and 17 months, respectively) than those with lung cancer.

THANK YOU!

ALYSA

ANA

References

▪ MKSAP 15

▪ John Hopkins Internal medicine review book

▪ Up to date www.Uptodate.com

▪ Reynen K et al. Ann Oncol 2004;15:375-381

▪ Karatolios K et al “Diagnostic value of biochemical biomarkers in malignant and non-malignant pericardial effusion” Heart Fail Rev. 2012 May 26

▪ Wagner PL et al “Pericardial effusions in the cancer population: prognostic factors after pericardial window and the impact of paradoxical hemodynamic instability” J Thorac Cardiovasc Surg. 2011 Jan;141(1):34-8