Incidental Diagnosis After a Car Accident: A Rare Case of Asymptomatic Uncorrected Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is a heterogeneous congenital heart disease that is occasionally diagnosed during adulthood. However, although they are often asymptomatic, adult patients with uncorrected TOF often have a poor prognosis. Poor outcomes indicate the importance of the identification and management of these patients, especially in the context of intercurrent disease or noncardiac surgery. We describe a case of clinically silent TOF in a 51-year-old woman. TOF was unmasked during a major noncardiac surgery for a polytrauma and successfully treated with the cooperation of a multidisciplinary team. (Level of Difficulty: Advanced.).

[Efficacy of eculizumab in a case of pregnancy-associated aHUS]. / Efficacia di eculizumab in un caso di SEU atipica associata alla gravidanza.

Pregnancy-associated thrombotic microangiopathy (TMA) is a rare condition, but it is burdened by a significant perinatal and maternal morbidity as well as mortality. We describe the case of a 33-year-old woman, who developed a TMA at the 36th week of gestation characterized by increased LDH, haptoglobin consumption, schistocytes, thrombocytopenia and acute renal failure requiring dialysis. There were not gestational hypertension nor proteinuria until the day of hospitalization. ADAMTS 13 deficiency was ruled out and the patient did not have diarrhea. She was initially treated with caesarean section, plasma infusion and plasmapheresis with no benefit. Five days after the onset of TMA, a temptative diagnosis of atypical uremic syndrome (aHUS) was made and the patient was switched to eculizumab. Antibiotic prophylaxis and anti-meningococcal A,B, C, W135 and Y vaccination was performed. TMA rapidly resolved and renal function completely recovered. The newborn had a normal perinatal course. A complement dysregulation was ruled out by testing for mutations on CFH, CFHR3-R1, CFI, MCP, CFB, C3 and for anti CFH antibodies. In conclusion the differential diagnosis of aHUS with HELLP syndrome is often not straightforward. The severity and persistence of TMA, the high mortality associated to peripartum TMA and the risk for irreversible kidney failure require an early therapeutic decision as to the use of eculizumab.