Table 2.

Overview of case reports on cardiovascular TMA manifestations in aHUS.

Reference	Age at onset/sex	Defect	Characteristics	Imaging/biopsy results	Specific therapy	Outcome
CARDIAL TMA
(34)	1 month/f	CFB-mut.	Anephric patient (Ktx; aHUS recurrence, graft loss, nephrectomy) with sudden onset of neurovascular symptoms (further details see Table 1)	CT-angiography and cardiac catheterization: stenosis of all branches of pulmonary arteries, moderate pulmonary arterial hypertension, and stenosis of distal anterior interventricular, right and marginal coronary arteries, right humeral artery, celiac trunk, and splenic artery	After onset of cerebrovascular symptoms attempt of carotid siphon angioplasty	Attempt of carotid siphon angioplasty, complicated by dissection and massive infarction leading to death
(48)	1 year/f	CFH-mut.	PE and HD after aHUS onset, ongoing hemolysis and severe hypertension. Two months after disease onset with worsening proteinuria, arterial hypertension, renal insufficiency, anemia, thrombopenia, and dilated cardiomyopathy leading to cardiorespiratory arrest with subsequent resuscitation and mechanical ventilation; under ongoing PE the clinical condition slightly improved but cardiac dysfunction did not resolve; thus eculizumab was introduced	On echocardiography signs of cardiomyopathy; ejection fraction decreased (around 31%)	PE leading to slight improvement of cardiac function; after introduction of eculizumab normalization of cardiac function	Normalization of cardiac function
(47)	43 years/f	CFH-mut; fam	Onset of aHUS including nephrotic syndrome and pulmonary edema with low C3; kidney biopsy showed typical TMA lesions. PE led to hematologic recovery. On day 15 after beginning of PE the patient developed sudden circulatory arrest with pulse rates under 25/min and the patient died despite immediate resuscitation attempts	Cardiac ultrasound during circulatory arrest: pericardial effusion with tamponade; Necroscopy: myocardial infarction without obstruction of the coronary arteries; multiple microscopic cardiomyocyte necrosis were present; no coronary thrombi or atherosclerosis; the small vessels showed microscopic features of TMA; immunochemistry revealed activation of final pathway of complement	PE leading to hematology remission	Sudden cardio circulatory arrest at day 15 of PE followed by death

aHUS, atypical hemolytic-uremic syndrome; CFB, complement factor B; CFH, complement factor H; CT, computer tomography; f, female; fam., familial; HD, hemodialysis; Ktx, kidney transplantation; m, male; MRI, magnetic resonance imaging; MRI/A, MRI angiography; Mut., mutation; n/a, not available; PD, peritoneal dialysis; PE, plasma exchange; PI, plasma infusion; PT, plasma therapy (PE and/or PI); TMA, thrombotic microangiopathy.