Contributed by J. Thomas Molina, MD, PhD, Larry Nichols, MD, and Bernard Klionsky, MD
Published on line in March 2000
Fig 1. Representative EKG, January 2000.
The patient is a 37-year-old white female who was in excellent health until three years prior, when she developed flu-like symptoms. She took Alka-Seltzer for about four days, but sought medical help when the symptoms persisted. A chest x-ray showed pulmonary venous congestion. She was also noted to be in atrial fibrillation. An echocardiogram showed a normal size left ventricle. The overall left ventricular systolic function was severely reduced with an estimated ejection fraction of 25%. Mild to moderate mitral regurgitation was present. Cardiac catheterization showed normal coronary arteries. She was treated with digoxin and intravenous diuretic therapy, with much improvement. She developed nonsustained ventricular tachycardia. With amiodarone therapy, she converted to normal sinus rhythm.
The patient' s mother died at age 65 of congestive heart failure. Her father died at age 48 of a myocardial infarction. One of her sisters died at age 46 from a cardiomyopathy. This sister had heart failure for about two years and died suddenly. Another sister had congestive heart failure at age 38 and a history of rapid ventricular tachycardia which prompted automatic implantable defibrillator placement, and later a heart transplantation.
A follow-up echocardiogram one year later showed a mildly dilated left ventricular cavity, a moderate decrease in overall left ventricular systolic function, an estimated ejection fraction of 35-40%, marked global hypokinesis most severe in the septum, mildly dilated right ventricle, a mild decrease in right ventricular systolic function, mild to moderate mitral regurgitation, mild left atrial enlargement, moderate to severe tricuspid regurgitation and severe right atrial enlargement.
Evaluation for cardiac transplantation one year later included an echocardiogram which showed moderate left ventricular systolic dysfunction with an estimated left ventricular ejection fraction of 40%. The right ventricle was severely dilated and severely hypokinetic. Severe tricuspid regurgitation was present. Right heart catheterization showed a right atrial pressure of 16 mmHg, pulmonary artery pressure of 21/16 mmHg and pulmonary wedge pressure of 13 mmHg. The cardiac output was 4.3 liters/min and the pulmonary artery oxygen saturation was 62%.
The patient' s clinical status deteriorated rapidly over the next two months. She had increasing dyspnea and fatigue and a profound decline in exercise tolerance. She also developed intermittent fevers and a non-productive cough. She had intermittent diarrhea and abdominal bloating, as well as persistent nausea. Appetite was quite poor. She had 3-4 pillow orthopnea and frequent paroxysmal nocturnal dyspnea. She had intermittent palpitations but denied dizziness, lightheadedness or pre-syncope. She had no angina. The patient was hospitalized.
While hospitalized, she became critically hypotensive with multiple runs of non-sustained ventricular tachycardia. She also had respiratory failure which required intubation and "septic shock like" hemodynamics. She became anuric with marked hypernatremia and acidosis. On hospital day 4, the patient became unresponsive with severe anasarca, no pupillary response and no corneal reflexes. The patient proceeded to suffer a cardiac arrest from which she could not be resuscitated and she was pronounced dead.