CardioExchange Archive

The patient is a 57-year-old woman with a history of Hodgkin disease of the anterior mediastinum. Upon being diagnosed at age 26, she was treated with a staging laparotomy, splenectomy, and 36 Gy of radiation to the mantle and para-aortic areas. She did well until September 2009, when she developed increasing dyspnea on exertion, weight gain, and lower-extremity edema. She was hospitalized four times within 3 months for decompensated heart failure.

Other Comorbidities

• morbid obesity (body-mass index, 44.2)
• diabetes mellitus (HbA1c, 10.8%)
• hyperlipidemia
• hypothyroidism
• moderate restrictive and obstructive lung disease
• pseudotumor cerebri

Physical Exam

• heart rate, 95 bpm
• blood pressure, 124/82 mm Hg
• jugular veins distended to the angle of jaw sitting upright
• brisk carotid upstrokes without bruits
• bilateral expiratory wheezes in the lungs
• distant heart sounds, with audible S1 and S2 and an early peaking II/VI systolic, crescendo–decrescendo murmur at the base
• morbid obesity without palpable hepatomegaly
• 1–2+ bilateral pitting edema, with chronic venous stasis changes in both lower extremities

Notable Chemistry Findings

• sodium, 139 mmol/L
• potassium, 4.4 mmol/L
• blood urea nitrogen, 22 mg/dL
• creatinine, 1.1 mg/dL

Echocardiography

• left-ventricular ejection fraction of 65%, with no regional wall-motion abnormalities
• flattening of the interventricular septum in both systole and diastole, consistent with right-sided pressure and volume overload
• enlarged right ventricle, with mildly decreased systolic function
  trileaflet, calcified aortic valve (mean gradient, 16 mm Hg; calculated aortic valve area, 1.1 cm2)
• mild aortic insufficiency
• heavily calcified mitral valve (mean gradient, 13 mm Hg; calculated mitral valve area, 1.9 cm2)
• risk-stratification score for balloon valvuloplasty, 13 (after review of mitral anatomy)
• moderate tricuspid regurgitation, with an estimated pulmonary-artery systolic pressure of 62 mm Hg + right-atrial pressure
• small circumferential pericardial effusion

Catheterizations
After inpatient diuresis, right-heart catheterization confirmed elevated filling pressures: right-atrial pressure, 11 mm Hg; right-ventricular pressure, 54/13 mm Hg; pulmonary artery pressure, 60/25 (40) mm Hg; and pulmonary capillary wedge pressure, 22 mm Hg. The patient’s mixed venous saturation was 65%, with a calculated cardiac output of 5.1 L/minute and a cardiac index of 2.7 L/minute/m2. Her mean aortic valve gradient was calculated as 10 mm Hg, with an aortic valve area of 1.7 cm2. Her mean mitral valve gradient was calculated as 12 mm Hg, with a mitral valve area of 1.6 cm2.

Left-heart catheterization revealed 2-vessel coronary artery disease, with a 60% proximal left-anterior descending artery stenosis and an 80% proximal right coronary artery stenosis.

Questions

• Would you medically manage this patient or recommend surgical correction?
• If you recommend medical therapy, which treatment would you suggest? If you recommend surgery, what should the operation fix?
• How would you counsel this patient about her prognosis, whether or not you advise surgery?

Response:
James Fang, MD

This unfortunate woman is suffering from radiation heart disease and its manifestations, including CAD, heart failure, and valve dysfunction. Her concomitant mitral and aortic stenosis make it difficult to assess pericardial disease using echocardiography and catheterization; CT or MRI may be better options. The high radiation dose she received is consistent with radiation-induced cardiac dysfunction, which is notoriously difficult to treat and, in this patient, is complicated by radiation lung disease and morbid obesity.

Therapy in this clinical context is typically limited to intense sodium/fluid restriction and judicious use of diuretics. The patient’s persistently elevated filling pressures despite diuresis are worrisome. Vasodilators are usually poorly tolerated, and hemodynamic assessment rarely documents a high resistance state. Given the patient’s mitral stenosis, maximization of cardiac output will be difficult to achieve; it requires calibrating a heart rate that is slow enough to allow LV filling through the mitral valve but not so slow as to prevent adequate stroke volume per minute. Selective pulmonary vasodilators would probably be tolerated poorly because of the mitral valve disease and restrictive physiology. I also wonder whether the patient has hepatic fibrosis from her right-heart failure.

Unless constriction could be demonstrated, I would not be enthusiastic about cardiac surgery, especially given the patient’s morbid obesity and radiation lung disease. An intervention for the epicardial coronary disease would not be likely to mitigate her heart failure and, given the need for dual antiplatelet therapy, would probably complicate her subsequent care. Her other comorbidities make advanced therapies such as transplantation or an LV assist device untenable.

This patient should be informed of her poor prognosis early in her course. She needs to know that she will experience progressive disability and is likely to require multiple hospitalizations. Eventual onset of atrial fibrillation is almost certain. Patients in her condition often have central sleep apnea, which nocturnal or continuous oxygen (not CPAP) may help to treat as it also provides relief of her symptoms.


Follow-Up:

Anju Nohria, MD

Given the patient’s high risk for morbidity and mortality from surgery, she was advised to pursue medical therapy. Instead, however, she insisted on surgery, citing a desire not to “suffer” from progressive heart failure and recurring hospitalizations.

She underwent mitral valve replacement, aortic valve replacement, subtotal pericardiectomy, and coronary artery bypass grafting with both a mammary-artery graft to the left-anterior descending artery and a saphenous-vein graft to the posterior descending artery.

Postoperatively, the patient was significantly vasoplegic and required multiple pressors at very high doses. As a consequence, she developed severe peripheral vasoconstriction, bilateral foot discoloration and necrosis, and multiorgan failure. When she could not be weaned from her pressors, her physicians and family discussed the futility of continued treatment and agreed to withdraw care. The patient died on postoperative day 5.

Conclusion:
James Fang, MD

This case describes what is becoming a common predicament in advanced heart disease: patients are presenting later and later for therapy, leaving physicians torn between our great desire to help and our edict to “first do no harm.” I would argue that, no matter how desperate a patient is, we are not obligated to perform interventions that we believe are futile or that put the patient at exceptionally high risk. In this particular case, I would not have offered surgery at our center and, if pressed, would have recommended that the patient get a second opinion elsewhere.