Review Question - QID 217639

This patient with a history of pulmonary embolism, worsening dyspnea on exertion, pitting edema, and echocardiographic evidence of right heart failure (elevated RVSP, enlarged right ventricle, low TAPSE) most likely has right heart failure due to pulmonary arterial hypertension as a result of chronic thromboembolic pulmonary hypertension (CTEPH). Hepatojugular reflux (elevation of the jugular venous pressure with compression of the right upper quadrant) can be seen in patients with right heart failure.

Signs and symptoms of right heart failure include dyspnea, exercise intolerance, palpitations, lower extremity edema, jugular venous distention, pulsus paradoxus, and hepatojugular reflux. In right ventricular dysfunction, elevated back pressure can lead to liver congestion. Subsequently, manual pressure to the right upper quadrant of the abdomen leads to backflow of blood through the right ventricle to the jugular vein. A positive result is usually defined as a sustained rise in the jugular venous pressure of at least 3 cm. Causes of isolated right heart failure include pulmonary arterial hypertension, chronic pulmonary thromboemboli, and right ventricular myocardial ischemic disease. Treatment of right heart failure includes optimization of volume status, restoration of perfusion pressure, improvement of myocardial contractility, and correction of the underlying etiology if possible. For patients with CTEPH in particular, pulmonary thromboendarterectomy is the definitive therapy.

Arrigo et al. reviewed the pathophysiology, diagnosis, and treatment of right ventricular failure. The authors found that the optimal treatment of right ventricular failure remains challenging. They recommended that in refractory right ventricular failure, mechanical circulatory support be considered.

Incorrect Answers:
Answer 1: Arterial pulse waveform showing alternating strong and weak beats (pulsus alternans) can be seen in severe left ventricular systolic impairment due to heterogeneity of the refractory period between healthy and diseased myocardial tissue. Patients with left ventricular failure present with bibasilar lung crackles and would show impaired left ventricle contractility on echocardiogram. Pulsus alternans should not be confused with pulsus paradoxus, in which there is an abnormally large decrease in stroke volume during inspiration. Pulsus paradoxus can be seen in right heart failure.

Answer 2: Diffusely diminished breath sounds with crackles bilaterally are indicative of pulmonary edema, which may be seen in left heart failure. Patients with left heart failure show impaired left ventricular contraction and ejection fraction on echocardiogram. In this patient with isolated right heart failure, pulmonary edema is unlikely. This patient’s dyspnea is more likely due to impaired oxygen delivery to the left heart secondary to pulmonary arterial hypertension.

Answer 4: A holosystolic murmur best heard at the cardiac apex with radiation to the left axilla may be seen in patients with mitral regurgitation. Mitral regurgitation (MR) is a cause of left heart failure, which can, in turn, cause right heart failure. However, this patient does not have echocardiographic evidence of MR or left heart failure.

Answer 5: Lateral displacement of the point of maximal impulse is indicative of left ventricular hypertrophy. This is a common finding in left heart failure and but is less likely in this patient with echocardiographic evidence of a normal left ventricle.

Bullet Summary:
Jugular venous distention with the exertion of manual pressure on the liver (hepatojugular reflux) is a sign of right heart failure.