Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities.

Congenital malformations are a known cause of intrauterine death; of them, congenital heart diseases (CHDs) are accountable for the highest fetal and neonatal mortality rates. They are strongly associated with other extracardiac malformations and an early fetal mortality. Two hundred and twenty fves cases of CHDs are presented. Of them, 155 were isolated CHDs (group A) and 70 were associated with extracardiac malformations, chromosomal disorders, or genetic syndromes (group B). The overall mortality in group B was higher than that observed in group A (p <0.01). Prenatal mortality was similar in both groups: A: 8.4% (13 out of 155); B: 15.7% (11 out of 70). Postnatal mortality was A: 16.8% (26 out of 155) (p <0.01), OR: 0.52 (95% CI: 0.16-1.7); B: 32.9% (23 out of 70) (p <0.01), OR: 0.41 (95% CI: 0.20-0.83). Heart diseases associated with extracardiac abnormalities had a higher mortality rate than isolated congenital heart diseases in the period up to 60 weeks of postmenstrual age (140 days post-term). No differences were observed between both groups of patients in terms of prenatal mortality.

Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities / Mortalidad fetal y neonatal en pacientes con cardiopatías congénitas aisladas y asociadas a anomalías extracardíacas

Congenital malformations are a known cause of intrauterine death; of them, congenital heart diseases (CHDs) are accountable for the highest fetal and neonatal mortality rates. They are strongly associated with other extracardiac malformations and an early fetal mortality. Two hundred and twenty fves cases of CHDs are presented. Of them, 155 were isolated CHDs (group A) and 70 were associated with extracardiac malformations, chromosomal disorders, or genetic syndromes (group B). The overall mortality in group B was higher than that observed in group A (p <0.01). Prenatal mortality was similar in both groups: A: 8.4% (13 out of 155); B: 15.7% (11 out of 70). Postnatal mortality was A: 16.8% (26 out of 155) (p <0.01), OR: 0.52 (95% CI: 0.16-1.7); B: 32.9% (23 out of 70) (p <0.01), OR: 0.41 (95% CI: 0.20-0.83). Heart diseases associated with extracardiac abnormalities had a higher mortality rate than isolated congenital heart diseases in the period up to 60 weeks of postmenstrual age (140 days post-term). No differences were observed between both groups of patients in terms of prenatal mortality.

Las malformaciones congénitas son una causa reconocida de muerte intrauterina. De ellas, las cardiopatías congénitas (CC) ocasionan la mayor mortalidad fetal y neonatal. La asociación con otras malformaciones extracardíacas es elevada y se acompaña de una mortalidad fetal temprana. Se presentan 225 casos de CC. En 155 casos fueron CC aisladas (grupo A) y en 70 se asociaron con malformaciones extracardíacas, cromoso-mopatías o síndromes genéticos (grupo B). La mortalidad global en el grupo B fue mayor con respecto al grupo A (p < 0,01). La mortalidad prenatal fue similar en ambos grupos: A: 8,4% (13 de 155) y B: 15,7% (11 de 70). La mortalidad posnatal fue A: 16,8% (26 de 155) (p < 0,01), OR 0,52 (IC 95% 0,16 a 1,7) y B: 32,9% (23 de 70), (p <0,01), OR 0,41 (IC 95% 0,20 a 0,83). Las cardiopatías asociadas a anomalías extracardíacas presentaron mayor mortalidad que las cardiopatías congénitas aisladas en el período comprendido hasta las 60 semanas de edad posmenstrual (140 días postérmino). No hubo diferencia en la mortalidad prenatal entre ambos grupos de pacientes.

Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities / Mortalidad fetal y neonatal en pacientes con cardiopatías congénitas aisladas y asociadas a anomalías extracardíacas

Congenital malformations are a known cause of intrauterine death; of them, congenital heart diseases (CHDs) are accountable for the highest fetal and neonatal mortality rates. They are strongly associated with other extracardiac malformations and an early fetal mortality. Two hundred and twenty fves cases of CHDs are presented. Of them, 155 were isolated CHDs (group A) and 70 were associated with extracardiac malformations, chromosomal disorders, or genetic syndromes (group B). The overall mortality in group B was higher than that observed in group A (p <0.01). Prenatal mortality was similar in both groups: A: 8.4% (13 out of 155); B: 15.7% (11 out of 70). Postnatal mortality was A: 16.8% (26 out of 155) (p <0.01), OR: 0.52 (95% CI: 0.16-1.7); B: 32.9% (23 out of 70) (p <0.01), OR: 0.41 (95% CI: 0.20-0.83). Heart diseases associated with extracardiac abnormalities had a higher mortality rate than isolated congenital heart diseases in the period up to 60 weeks of postmenstrual age (140 days post-term). No differences were observed between both groups of patients in terms of prenatal mortality.(AU)

Las malformaciones congénitas son una causa reconocida de muerte intrauterina. De ellas, las cardiopatías congénitas (CC) ocasionan la mayor mortalidad fetal y neonatal. La asociación con otras malformaciones extracardíacas es elevada y se acompaña de una mortalidad fetal temprana. Se presentan 225 casos de CC. En 155 casos fueron CC aisladas (grupo A) y en 70 se asociaron con malformaciones extracardíacas, cromoso-mopatías o síndromes genéticos (grupo B). La mortalidad global en el grupo B fue mayor con respecto al grupo A (p < 0,01). La mortalidad prenatal fue similar en ambos grupos: A: 8,4% (13 de 155) y B: 15,7% (11 de 70). La mortalidad posnatal fue A: 16,8% (26 de 155) (p < 0,01), OR 0,52 (IC 95% 0,16 a 1,7) y B: 32,9% (23 de 70), (p <0,01), OR 0,41 (IC 95% 0,20 a 0,83). Las cardiopatías asociadas a anomalías extracardíacas presentaron mayor mortalidad que las cardiopatías congénitas aisladas en el período comprendido hasta las 60 semanas de edad posmenstrual (140 días postérmino). No hubo diferencia en la mortalidad prenatal entre ambos grupos de pacientes.(AU)

Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities. / Fetal and neonatal mortality in patients with isolated congenital heart diseases and heart conditions associated with extracardiac abnormalities.

Congenital malformations are a known cause of intrauterine death; of them, congenital heart diseases (CHDs) are accountable for the highest fetal and neonatal mortality rates. They are strongly associated with other extracardiac malformations and an early fetal mortality. Two hundred and twenty fves cases of CHDs are presented. Of them, 155 were isolated CHDs (group A) and 70 were associated with extracardiac malformations, chromosomal disorders, or genetic syndromes (group B). The overall mortality in group B was higher than that observed in group A (p <0.01). Prenatal mortality was similar in both groups: A: 8.4

(13 out of 155); B: 15.7

(11 out of 70). Postnatal mortality was A: 16.8

(26 out of 155) (p <0.01), OR: 0.52 (95

CI: 0.16-1.7); B: 32.9

(23 out of 70) (p <0.01), OR: 0.41 (95

CI: 0.20-0.83). Heart diseases associated with extracardiac abnormalities had a higher mortality rate than isolated congenital heart diseases in the period up to 60 weeks of postmenstrual age (140 days post-term). No differences were observed between both groups of patients in terms of prenatal mortality.

[Superior mesenteric artery syndrome (Wilkie syndrome): case report]. / Síndrome de la arteria mesentérica superior (síndrome de Wilkie): Caso clínico.

Superior mesenteric artery syndrome is an uncommon cause of upper gastrointestinal tract obstruction. The syndrome results from compression of the third portion of duodenum as it crosses underneath the superior mesenteric artery, related to conditions that reduce the aortomesenteric angle (acute weight loss) or after scoliosis surgery. Patients may present symptoms of gastrointestinal obstruction, such as upper abdominal distension and epigastric tenderness, usually relieved by posture changing. Diagnose must be complemented with an upper gastrointestinal barium-contrast radiography. Conservative treatment is usually effective with early diagnosis. Surgery is needed when conservative measures are ineffective. We present the case of a 18 year-old patient with Wilkie's syndrome secondary to scoliosis surgery. The patient presented symptoms of gastrointestinal obstruction, and diagnosis was confirmed with upper gastrointestinal barium-contrast radiography. The patient started conservative treatment with proper positioning after eating and nutritional support to provide optimal calories supply.

Síndrome de la arteria mesentérica superior (síndrome de Wilkie): caso clínico / Superior mesenteric artery syndrome (Wilkie syndrome): case report

El síndrome de la arteria mesentérica superior (síndrome de Wilkie) es una causa poco frecuente de obstrucción duodenal de origen vascular. Se asocia con situaciones que disminuyen elángulo entre la arteria mesentérica superior y la aorta (compásaortomesentérico) secundarias a pérdida de grasa retroperitoneal (descenso brusco de peso o caquexia) o procedimientos que determinan hiperextensión dorsal, especialmente enpostquirúrgicos de cirugía correctora de escoliosis. Clínicamente, se manifiesta por obstrucción intestinal alta. El diagnósticoes radiológico y el tratamiento es inicialmente médico. El tratamiento quirúrgico se reserva para casos refractarios. Se describe el caso de un paciente de 18 años con síndrome de Wilkie, secundario a cirugía correctora de escoliosis dorsolumbar. Con la sospecha clínica se realiza seriada gastroduodenal que confirma el diagnóstico. Inicia tratamiento médico con dieta hipercalórica fraccionada y medidas posturales con buena respuesta.