Brief relevant presenting history
A 23-year-old man was admitted to our institution with dyspnoea and fatigue of sudden onset. He was asymptomatic until the day of the admission to our hospital, at which time he did not complain of syncope, chest pain or haemoptysis, but he had progressive and severe dyspnoea and fatigue. He had no history of cocaine abuse.
Background or previous history
Six years before admission the patient had been diagnosed with myocarditis, and presented with an episode of syncope without chest pain. The electrocardiogram (ECG) showed inferior ST segment elevation, with a peak value of cardiac troponin I 2.9 ng/dl. Left ventricular ejection fraction at echocardiography was 55% without segmentary motility abnormalities, and coronary angiography was normal. The ECG changes and cardiac troponin I normalised and he was discharged.
A month later, the patient was admitted to another hospital because he had an episode of spontaneous tetraparesis and generalised parethesias from which he recuperated completely in a few hours. Brain computed tomography (CT), magnetic resonance imaging (MRI), electroencephalogram (EEG) and Doppler echography of the supra-aortic vessels were normal. He was discharged without neurological sequelae.
He also had a long history of hypotonia and recidivant spontaneous dislocation of the right knee. Surgical history included removal of a pilonidal cyst and varicotomy in the right leg.
One month before admission the patient presented with substernal chest pain, dyspnoea and haemoptysis. The ECG was normal. A diagnosis of left haemopneumothorax was achieved and he was treated with a chest tube. A high resolution CT scan of the chest, undertaken 3 days after admission, demonstrated small cavitated nodules in the right upper lobe (RUL) and micronodules in right intermediate lung (RIL) that were radiologically interpreted as tuberculous nodules. The patient was discharged 15 days later.
The patient was a thin, white man with a narrow face, but not “marfanoid habitus”. It was not noticed that he had translucent skin. He was conscious, cyanotic and diaphoretic. His body temperature was 37°C, pulse 138 beats/min and blood pressure 70/40 mm Hg. Peripheral O2 saturation was 70% on pulsoximetry.
Chest examination revealed tachypnoea, dyspnoea, and severe respiratory distress with use of intercostal and accessory respiratory muscles. On chest auscultation there were basal bilateral rales with left basal hypoventilation. Heart sounds were normal, without murmurs or gallops.
Neurological examination was normal; the strength of the four extremities was 5/5, with no sensory or deep tendon reflexes abnormalities.
Relevant clinical investigations and results
The ECG showed sinus tachycardia and generalised ST segment elevation in leads I, aVL, II, avF and V2–V6 ().
Electrocardiogram showing generalised ST segment elevation in leads I, aVL, II, avF and V2–V6.
The cardiothoracic ratio was normal on the chest radiograph and there was left pleural effusion without signs of pulmonary congestion. Cardiac troponin I value at admission was 0.06 ng/dl, An echocardiogram showed generalised severe hypokinesis and ruled out pericardial tamponade.
The patient was in severe cardiogenic shock and underwent urgent orotracheal intubation, mechanical ventilation, pressor support and admission to the intensive care unit (ICU). A few minutes after ICU admission the patient presented pulseless electrical activity arrest (ECG monitoring showed persistent sinus tachycardia and ST segment elevation), requiring advanced cardiopulmonary resuscitation and intra-aortic balloon pump support. In spite of resuscitation efforts the patient did not recover and died 1 h after hospital admission. Because of the rapidly fatal outcome, a necropsy was performed with the presumptive diagnosis of tuberculosis and myocarditis versus myocardial infarct of toxic (cocaine) aetiology.