Radiation-Associated Cardiac Disease

A 46-year-old woman is admitted with cardiogenic shock. She has a history of nonischemic cardiomyopathy secondary to remote chemotherapy and radiation-related cardiotoxicity following treatment of Hodgkin's lymphoma 25 years earlier. Due to radiation-induced valve injury, she underwent mitral and tricuspid valve repair a decade ago. She was hospitalized several times in recent months for decompensated heart failure (HF) despite escalation of oral diuretic dosing, suffering a progressive decline in her functional status (New York Heart Association Class IV). Attempts at uptitration and optimization of outpatient HF guideline-directed medical therapy was limited by symptomatic hypotension and neurohormonal blockade intolerance.

Right heart catheterization performed on presentation demonstrated reduced cardiac index, severely elevated filling pressures with increased pulmonary artery pressures, and evidence of a "square root sign" on the right ventricular pressure tracing (Figure 1). Transthoracic echocardiogram was significant for the following:

  • Calcified aortic valve with limited leaflet mobility (Videos 1-2) and increased peak aortic valve velocities (Figure 2A)
  • Restrictive left ventricular filling pattern (Figure 2B)
  • Reduced septal and lateral mitral annular tissue Doppler velocities (Figure 3) with significantly increased E/e' ratio of 26
  • Moderate right ventricular dysfunction
  • A nondilated left ventricle
  • Left ventricular ejection fraction of 49%

A computed tomography (CT) angiogram was notable for substantial aortic root and ascending aorta calcification (Figure 4) without significant pericardial calcification. The patient was stabilized in the cardiac intensive care unit with continuous inotropic infusion and intravenous diuretics. She had good augmentation in urine output but remained volume overloaded with ongoing low cardiac output despite attempts at optimization and inotropic support.

Figure 1: Right Heart Catheterization

Figure 1

Video 1: Transthoracic Echocardiogram

Video 2: Transthoracic Echocardiogram

Figure 2: Transthoracic Echocardiogram (Spectral Doppler)

Figure 2

Figure 3: Transthoracic Echocardiogram (Medial and Lateral Mitral Annular Tissue Doppler)

Figure 3

What is the next best step in the evaluation of this patient?

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