ABC | Volume 112, Nº2, February 2019

Anatomopathological Correlation Arq Bras Cardiol. 2019; 112(2):204-210 Favaratto & Aiello Heart failure after surgical correction of aortic dissection Figure 3 – Aortography. Ascending aortic aneurysm with dissection and catheter in the true lumen. A Doppler ultrasonography of the lower limbs carried out due to suspicion of pulmonary thromboembolism (recent RV dysfunction) showed no signs of thrombosis. The patient then underwent a chest tomography, which identified a small consolidation on the right side, with right pleural effusion. He developed refractory shock, despite the use of antibiotics. The right pleural effusion was punctured, and the presence of transudate was identified. All blood cultures and urine cultures were negative. He required orotracheal intubation for ventilatory support on August 29, 2012, developing an increasing need for noradrenaline, and the antibiotics were replaced by daptomycin, micafungin, and rifampicin. Laboratory reassessment (September 4, 2012) showed: hemoglobin: 10 g/dL; hematocrit: 33%; leukocytes: 5,450/mm³ (neutrophils 85%, lymphocytes 10% and monocytes 5%); platelets: 313,000/mm³; C-reactive protein: 221.38mg/L; urea: 44 mg/dL; creatinine: 2.21 mg/dL; phosphorus: 2.2 mg/dL; magnesium: 1.6 mEq/L. The patient remained in shock and died (September 4, 2012). Clinical aspects This was a male patient, who presented with ascending aortic dissection, and who, even after surgery for dissection repair, developed a picture of severe heart failure and died. The International Registry of Acute Aortic Dissection (IRAD) showed that patients with aortic dissection were older than our patient, 61 years; and a majority of males (63%). Regarding the diseases related to the dissection, they were: Marfan syndrome (6.7%), hypertension (69.3%), atherosclerosis (24.4%), previously known aortic aneurysm (12.4%), previous aortic dissection 3.9%) and diabetes mellitus (4.3%). Also, 15.9% had a history of previous cardiac surgery and iatrogenic cause in 4.8% (1.7% coronary angiography and 3.1% after cardiac surgery). 1 The 2015 update of the same registry, with a ten-fold higher number of patients, showed an increase in hypertension (75.5%) and a decrease in the presence of Marfan syndrome (4.5%), (75.5%), atherosclerosis (19.6%) and previous cardiac surgery (10.6%). 2 Genetic tests can be performed in the presence of aortic aneurysm in younger patients. The syndromes related to the presence of aortic aneurysms are Marfan, Loeys-Dietz, Ehler‑Danlos syndromes, the cutis laxa or elastolysis and that related to a defect in the transforming growth factor beta (TGF β ). Classically, the genetic alterations found inMarfan syndrome are related to the fibrillin-1 gene. 3 Changes in the physical examination involve ocular (myopia, ectopia lentis, and risk of retinal detachment), skeletal (exaggerated growth and joint laxity, exaggerated growth of the extremities) and cardiovascular alterations (dilation of the aorta at the level of the sinuses of Valsalva, predisposing to dissection). In the present case, we do not have a description of these phenotypic changes that might suggest such diagnosis. Loeys-Dietz syndrome includes several manifestations similar to those of Marfan Syndrome, but they also include hypertelorism, broad or bifid uvula, cleft palate and generalized arterial tortuosity, aneurysms, and arterial dissection. Generally no increase of extremities and ocular alterations are observed. 4 As well as for Marfan syndrome, we do not have evidence in the present case to suggest such a diagnosis. The Ehler-Danlos syndrome is characterized by fragility of the connective tissue and the manifestations occur in the skin (hyperelasticity, atrophic scars and easy ecchymosis), joints (hypermotility, frequent dislocations and arthralgias) and vessels (aneurysms and spontaneous vessel ruptures). The vascular form, in which dissection occurs most frequently, occurs through a mutation in the alpha-1 gene of type III collagen, with a silent substitution that leads to the replacement of glycine in the collagen chain. 5 206