Case 3/2015 - 53-year-old female with cardiogenic shock 12 years after surgical correction of endomyocardial fibrosis.

A 53-year-old female, submitted to surgical correction of endomyocardial fibrosis (EMF) 12 years before, sought medical care with hypotension and bradycardia. She complained of palpitations since the age of 29 years. Her symptoms aggravated 5 years later, with the appearance of dyspnea on moderate exertion. After 5 more years, the dyspnea intensified, being triggered on mild exertion and in the dorsal decubitus position. The patient was then referred to a hospital. In 1994, the physical examination revealed irregular pulse, heart rate of 88 bpm, blood pressure of 104/80 mm Hg, and increased jugular venous pressure. The pulmonary exam was normal, and the heart auscultation showed arrhythmic heart sounds and mitral systolic murmur (+/4). The exam of the abdomen and limbs was within the normal range. In February 1994, the electrocardiogram (ECG) showed atrial fibrillation, left bundle-branch block and left ventricular hypertrophy (Figure 1). Figure 1 ECG: Atrial fibrillation, left bundle-branch block and left ventricular hypertrophy. In 1996, on echocardiogram, the dimensions of the cardiac chambers were: left atrium, 57 mm; left ventricle, 51 mm (diastole) and 33 mm (systole). Obliteration of the apical region of both ventricles was observed, with suggestive signs of calcification. The morphological aspect was considered suggestive of EMF. In 1996, a ventriculography showed obliteration of the apical region of left and right ventricles, in addition to mild mitral regurgitation. On coronary cineangiography, the circumflex branch of the left coronary artery originated from the right coronary artery. No obstruction of the coronary arteries was identified. The ventriculography findings were suggestive of EMF. Surgical treatment was indicated. In September 1996 the surgery was performed with incision in the apical region of the left ventricle and resection of a fibrous and calcified mass. Then, through the interatrial septum, annuloplasty with bovine pericardium was performed in the posterior portion of the mitral ring and in the anterior portion of the tricuspid ring. The control ventriculography one month after surgery showed mild left ventricular hypokinesia and competent valves. According to the patient, the dyspnea improved, but the palpitations remained. She reported a transient episode of speech difficulty and right hemiparesis in 2002. The dyspnea reappeared, initially on moderate exertion and then on mild exertion. A new echocardiographic assessment detected marked tricuspid regurgitation. The medication was adjusted, with the prescription of the following drugs: warfarin, 5 mg; losartan, 50 mg; furosemide, 80 mg; digoxin, 0.25 mg; and diltiazem, 180 mg. The symptoms improved, although episodes of aggravation occurred. In 2005, a new hemodynamic study revealed hypertension of the right chambers. Left ventricular hypokinesia of moderate intensity in the inferior and apical walls, and of mild intensity in the anterior wall, was identified. The pharmacological treatment was maintained, with adjustments according to symptoms. The patient was brought to the hospital after 24 hours of vertigo, malaise and dyspnea. The physical examination on November 13, 2008, revealed pulse of 30 bpm, inaudible blood pressure, crepitant rales in pulmonary bases, bradycardia, liver palpable 7 cm from the costal margin, ascitis, and mild lower limb edema. The ECG revealed atrial fibrillation, high atrioventricular block and ventricular rate of 30 bpm. Chest X-ray showed enlarged cardiac area (+++/4+) and no signs of pulmonary congestion. The laboratory findings on that occasion were as follows: potassium, 4.4 mEq/L; sodium, 134 mEq/L; urea, 100 mg/dL; creatinine, 1.45 mg/L (glomerular filtration rate, 40 mL/min/1.73m2); INR, 2.6; BNP, 250 pg/mL; hemoglobin, 12.6 g/dL; hematocrit, 40%; leukocytes/mm3, 7900 (78% neutrophils, 10% lymphocytes, and 12% monocytes); platelets/mm3, 183000; arterial lactate, 11 mg/dL; and blood digoxin level, 1.13 ng/mL. Atropine and dopamine were administered and blood volume, replaced, but with no heart rate increase. Dobutamine was then administered, but unsuccessfully. A temporary transvenous external pacemaker was implanted, after which, blood pressure increased to 106/60 mmHg. Permanent pacemaker was implanted on November, 19, 2008. The underlying disease posed technical difficulty, and a reserve electrode was implanted in the coronary sinus for an occasional capture loss of the right ventricular electrode. The laboratory findings on that occasion were as follows: hemoglobin, 9 g/dL; leukocytes, 4700/mm3; platelets, 114000/mm3; urea, 47 mg/dL; creatinine, 0.76 mg/dL; potassium, 4.6 mEq/L; sodium, 133 mEq/L; blood glucose, 122 mg/dL; hemoglobin, 11.8 g/dl; hematocrit, 36%; and lactate; 7 mg/dL. Oliguria and renal failure followed. On December 4, 2008, the laboratory findings were as follows: creatinine, 2.4 mg/dL; urea, 105 mg/dL; potassium, 5.3 mEq/L; sodium, 130 mEq/L; hemoglobin, 8.8 g/dL; hematocrit, 30%; leukocytes, 6200/mm3 (neutrophils 86%, lymphocytes 4%, and monocytes 10%); platelets, 99000/mm3. The patient had ventricular fibrillation and cardiac arrest, which did not respond to the resuscitation maneuvers, dying on December 5, 2008.