Paradoxical renal embolism in a patient with congenital cardiac malformation.

A 35-year-old man was admitted with sudden new-onset pain in his right flank. He had been born with a complex cardiac malformation consisting of double outlet right ventricle with malposition of the great arteries, straddling of the tricuspid valve and large atrial and ventricular septal defects. During the newborn period, pulmonary artery banding was conducted. Three years later corrective surgery was attempted; however, de-banding of the pulmonary artery resulted in pulmonary congestion. Thus, pulmonary artery banding was restored and a palliative Mustard procedure was performed to achieve an increase in systemic arterial oxygen saturation by changing streaming and reducing the amount of venous blood that reaches the aorta (Figure 1). After this surgery, the patient developed relatively normal with only minor physical limitations. At the age of 33 years, cardiac function progressively deteriorated resulting in repetitive cardiac decompensation. Two months before the current admission, the patient presented with numbness and tingling of his left arm and leg, which was interpreted as a transient ischemic attack. Thromboembolism due to intermittent atrial fibrillation was assumed to be the most likely source, because the patient reported palpitations, and atrial tachycardia is a common late arrhythmia after the Mustard procedure. The patient was therefore started on phenprocoumon and the target International Normalized Ratio (INR) was set between 2 and 3. At the current admission, the patient described a progressive excruciating pain in his right flank that had abruptly started during the night before. Several days prior, he had doubled the dose of his diuretic since he had noticed increasing leg edema. At about the same time he had experienced a new episode of transient numbness and tingling of the left side of his body which ceased spontaneously after several hours. On physical examination, there was severe cyanosis of the face and hands and clubbing of fingers and toes. The pulse was 77/min and regular. The blood pressure was 105/75mmHg. The jugular venous pressure was slightly increased. The lungs were clear. There was a holosystolic thrill over the anterior and posterior chest, which was maximal at the fourth left intercostal space. Heart sounds were normal. There was tenderness in the abdominal right upper quadrant and right costovertebral angle. All peripheral pulses were palpable. Routine