The results disclosed that complementary exams, such as MRI and echocardiography, are important to better define the observed defects. Some of them, such as congenital heart defects, may be more common than previously reported. This definition is essential for the proper management of the pregnancy and genetic counseling of the family. The birth of these children must be planned with caution and for the prognosis a long survival possibility, despite unlikely and rare, must be considered.

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Introduction: True knot of the umbilical cord is characterized by a cord loop with increased tension and hence higher risk for obstetric and neonatal outcome intervention. It is a finding observed in 1-2% of births. Aim: Review the etiology, physiopathogeny, diagnosis and prognosis of true knot of the umbilical cord. Methodology: We carried out a review searching for scientific articles present in the electronic database PubMed, using the descriptors "true knot", "umbilical cord", "Doppler sonography" and "prenatal diagnosis". Articles published until 2013 were included in the review. Discussion: True knot of the umbilical cord is a relatively common finding at birth. However, its diagnosis in uterus is still quite rare. Several factors have been related to its etiology, including the size of the umbilical cord. Its appearance occurs mostly between 9 and 28 weeks of gestation, in the fetal period of greater mobility. The major part of the true knot of the umbilical cord is not associated to an increased risk of obstetric intervention and neonatal outcomes because most are loose. However, the true knots can tighten during pregnancy due to fetal movement or during labor, leading to reduced blood flow and consequent fetal asphyxia and death. As a result, the close monitoring of pregnancy in cases of true knot of umbilical cord is required. The prenatal diagnosis of a true knot of the umbilical cord can be a challenge. Sonographic complementary techniques, including color Doppler and Dopplervelocimetry can improve the detection rate and help to differentiate true from false knots. And this has important implications for the management and prognosis of pregnancy, besides consequences for the fetus and newborn.

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