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1
Question:

A 54-year-old man comes to the emergency department due to severe fatigue and dyspnea.  He has a long history of progressively worsening heart failure that has been resistant to treatment with medications, including diuretics.  He was treated with chest radiation 10 years ago for non-Hodgkin lymphoma and has been in remission since then.  The patient is admitted to the hospital, but his condition continues to deteriorate despite aggressive therapy.  He dies 3 days later, and an autopsy is performed.  Gross inspection of the heart shows dense, thick, fibrous tissue in the pericardial space between the visceral and parietal pericardium.  Which of the following signs would most likely have been detected during a physical examination of this patient just prior to his death?

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Explanation:

Constrictive pericarditis

Etiology

  • Idiopathic or viral pericarditis
  • Cardiac surgery or radiation therapy
  • Tuberculosis (in endemic areas)

Pathogenesis

  • Rigid pericardium prevents ventricular expansion and restricts diastolic filling
  • Predominantly right-sided manifestations

Physical examination

  • ↑ Jugular venous pressure
  • Pericardial knock
  • Pulsus paradoxus
  • Kussmaul sign

The autopsy finding of thick, fibrous tissue in the pericardial space is consistent with constrictive pericarditis, a potential complication of chest radiation therapy for non-Hodgkin lymphoma.  This dense, rigid pericardial tissue encases the heart and restricts ventricular filling, causing low cardiac output (manifesting with fatigue and dyspnea on exertion) and progressive right-sided heart failure (manifesting with hepatomegaly and peripheral edema).

Physical examination in constrictive pericarditis typically shows elevated jugular venous pressure (JVP) with prominent x and y descents and a pericardial knock (early diastolic sound that occurs before S3) and may also demonstrate pulsus paradoxus (>10 mm Hg drop in systolic blood pressure during inspiration).  In addition, Kussmaul sign may be present.  Under normal circumstances, the decrease in intrathoracic pressure during inspiration increases venous return to the right side of the heart and lowers JVP.  However, in constrictive pericarditis, the rigid pericardium prevents the right side of the heart from accommodating increased venous return, which leads to a paradoxical rise in JVP during inspiration, referred to as Kussmaul sign.

(Choice B)  A loud pulmonic component of the second heart sound (P2) is heard in patients with pulmonary hypertension.

(Choice C)  During inspiration, venous return to the right side of the heart is increased, resulting in later closure of the pulmonic valve and physiologic splitting of the aortic (A2) and pulmonic components of S2.  Paradoxical splitting occurs when cardiac pathology (eg, aortic stenosis, left bundle branch block) delays closure of the aortic valve, causing A2 to occur noticeably later than P2.

(Choice D)  An S3 gallop occurs due to the sudden deceleration of blood as it enters a dilated ventricle; it is typically heard in patients with dilated cardiomyopathy or severe mitral regurgitation.

(Choice E)  A sustained left parasternal lift is most commonly a sign of right ventricular hypertrophy, such as occurs with chronic right ventricular pressure overload (eg, pulmonary hypertension, pulmonic stenosis) or volume overload (eg, tricuspid regurgitation).

Educational objective:
In constrictive pericarditis, normal pericardium is replaced by dense, rigid pericardial tissue that restricts ventricular filling, leading to low cardiac output and progressive right-sided heart failure.  Physical examination findings in such patients include elevated jugular venous pressure (JVP), pericardial knock, pulsus paradoxus, and a paradoxical rise in JVP with inspiration (Kussmaul sign).