Outcomes for Ectopia Cordis

Smith, Benjamin J.; Flyer, Jonathan N.; Edwards, Erika M.; Soll, Roger F.; Horbar, Jeffrey D.; Yeager, Scott B.

doi:10.1016/j.jpeds.2019.09.014

Cited by 9 publications

(11 citation statements)

References 15 publications

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“…By the end of the 20 th century, the estimated survival rate of the Pentalogy of Cantrell was 37,3%, but scientific advances in neonatal intensive care and pediatric surgery have increased it up to 61% 4,17 . Nonetheless, the syndrome remains a surgical challenge, given the variety and severity of the associated malformations 6 .…”

Section: Discussionmentioning

confidence: 99%

“…The prognosis of the Pentalogy of Cantrell is reserved, since most newborns are unable to make it through their first days of life. Patients still present considerable mortality and morbidity, even after initial stabilization and surgical correction, especially when there is significant ectopia cordis, due to complications such as impaired cardiac function, chronic lung disease and developmental delay 6 . In the reported case, the patient went through surgical intervention but progressed severely, presenting PCR in PEA and then proceeding to cardiogenic shock regardless of the reversion of the PCR with neonatal reanimation maneuvers, resulting in his death mere hours after the procedure.…”

Section: Discussionmentioning

confidence: 99%

“…The syndrome consists of a thoracoabdominal variety of ectopia cordis associated with five main abnormalities which affect the supraumbilical midline of the ventral abdominal wall (omphalocele); the anterior portion of the diaphragm; the lower section of the sternum; the parietal pericardium; and the cardiovascular system 1 . Therefore, the affliction presents with an elevated lethality rate, which gets higher the more severe and widespread the extent of the anomalies 6 .…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Pentalogy of Cantrell with a double-outlet right ventricle: Prenatal Diagnosis, Delivery and Immediate Postnatal Surgical Repair / Pentalogia de Cantrell com um ventrículo direito de saída dupla: Diagnóstico Pré-Natal, Parto e Reparação Cirúrgica Pós-Natal Imediata

Bringel¹,

Gadelha²,

Santos³

et al. 2021

BJDV

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A Pentalogia de Cantrell é uma doença congênita rara, havendo apenas cerca de 250 casos relatados. A síndrome consiste em uma variedade toracoabdominal de ectopia cordis, associada a cinco defeitos principais em: linha mediana anterior supraumbilical de parede abdominal, porção anterior do diafragma, parte inferior do esterno, pericárdio parietal e malformações cardiovasculares. A sua letalidade é alta, conforme gravidade e extensão das alterações, de forma que a assistência pré-natal se faz fundamental ao diagnóstico precoce e à investigação das anomalias, a fim de proporcionar um melhor prognóstico. Nesse sentido, objetiva-se relatar o caso de um neonato com variante completa da síndrome. O diagnóstico foi realizado no período intrauterino por meio de avaliação ultrassonográfica, identificando-se defeito de fechamento de paredes torácica e abdominal (onfalocele), ectopia cordis e anomalia estrutural cardíaca. Na ecocardiografia fetal, foram evidenciados: dupla via de saída de ventrículo direito, estenose pulmonar, aorta cavalgando septo interventricular e persistência de veia cava superior esquerda. Houve interrupção da gestação por parto cesariano, com idade gestacional de 36 semanas e 3 dias. O recém-nascido foi submetido a cuidados intensivos e correção cirúrgica, mas faleceu horas depois.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Pentalogy of Cantrell with a double-outlet right ventricle: Prenatal Diagnosis, Delivery and Immediate Postnatal Surgical Repair / Pentalogia de Cantrell com um ventrículo direito de saída dupla: Diagnóstico Pré-Natal, Parto e Reparação Cirúrgica Pós-Natal Imediata

Bringel¹,

Gadelha²,

Santos³

et al. 2021

BJDV

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“…It is commonly associated with an anterior diaphragmatic hernia, omphalocele, sternal and pericardial defects, and CHD. It can be classified according to the position of the heart into the cervical, cervicothoracic, thoracoabdominal (associated with pentalogy of Cantrell), or abdominal area 2,3 . The thoracic and thoracoabdominal EC are most common.…”

Section: Introductionmentioning

confidence: 99%

“…It can be classified according to the position of the heart into the cervical, cervicothoracic, thoracoabdominal (associated with pentalogy of Cantrell), or abdominal area. 2,3 The thoracic and thoracoabdominal EC are most common.…”

Section: Introductionmentioning

confidence: 99%

Prenatal diagnosis of fetal ectopia cordis by fetal cardiovascular magnetic resonance imaging

2022

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Objective:The purpose of this retrospective study was to report our cases of fetal ectopia cordis (EC) and to evaluate the utility of fetal cardiovascular magnetic resonance imaging (MRI) for the diagnosis of this rare anomaly.Method: This retrospective study included 11 fetuses with EC. The multiplane steady-state free precession (SSFP) sequence, single-shot turbo spin-echo sequence and non-gated SSFP cine cardiovascular magnetic resonance were used to evaluate the fetal heart and abdomen. Results:The 11 fetal cases with EC were examined by fetal cardiovascular MRI and confirmed by postnatal or post-mortem findings. Of these 11 cases, two were isolated thoracic EC, six had pentalogy of Cantrell, and three had an omphalocele and EC. Among all 11 fetuses, nine were associated with congenital heart defects. In four cases, fetal MRI added additional information compared to fetal ultrasound, however, in two cases, fetal MRI missed the diagnosis of a ventricular septal defect noted by echocardiography. Conclusion:Fetal MRI combined with prenatal echocardiography can improve the accuracy of the prenatal diagnosis of EC. Key points What's already known about this topic?� Ectopia cordis is a rare congenital anomaly associated with cardiac and/or extracardiac malformations and the prognosis is generally very poor.� Echocardiography is the first choice to assess fetal ectopia cordis, but it can be technically limited by certain factors. What does this study add?� We present findings of ectopia cordis diagnosed by fetal cardiovascular magnetic resonance in a single center.� Fetal cardiovascular magnetic resonance can add additional information to fetal echocardiography in the diagnosis of fetal ectopia cordis.

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The importance of designing a protector for a preterm and low birth weight infant with ectopia cordis

Mukai,

Ito,

Shitara

et al. 2024

Clinical Case Reports

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Key Clinical MessageEctopia cordis is a rare condition with expected low survival rate based on past studies. We encountered a case of a preterm and low birth weight infant with ectopia cordis. When the infant cried, the prolapse of the heart, liver, and intestinal tract worsened. A pressure‐applying protector was used to protect the organs and reduce the prolapse. Upon application, the infant's tachypnea and desaturation worsened. Fluoroscopic examination suggested that the pressure from the prolapsed regions was impeding pulmonary expansion and negatively affecting circulation. It is essential to carefully design a protector that accommodates the infant's growth.

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Outcomes for Ectopia Cordis

Cited by 9 publications

References 15 publications

Pentalogy of Cantrell with a double-outlet right ventricle: Prenatal Diagnosis, Delivery and Immediate Postnatal Surgical Repair / Pentalogia de Cantrell com um ventrículo direito de saída dupla: Diagnóstico Pré-Natal, Parto e Reparação Cirúrgica Pós-Natal Imediata

Pentalogy of Cantrell with a double-outlet right ventricle: Prenatal Diagnosis, Delivery and Immediate Postnatal Surgical Repair / Pentalogia de Cantrell com um ventrículo direito de saída dupla: Diagnóstico Pré-Natal, Parto e Reparação Cirúrgica Pós-Natal Imediata

Prenatal diagnosis of fetal ectopia cordis by fetal cardiovascular magnetic resonance imaging

The importance of designing a protector for a preterm and low birth weight infant with ectopia cordis

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