Staged Repair of Pentalogy of Cantrell with Ectopia Cordis and Ventricular Septal Defect

Sakasai, Yoshie; Thang, Bui Quoc; Kanemoto, Shinya; Takahashi-Igari, Miho; Togashi, Shinji; Kato, Harumi; Hiramatsu, Yuji

doi:10.1111/j.1540-8191.2012.01456.x

Cited by 20 publications

(17 citation statements)

References 8 publications

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“…PoC including complete thoracic ectopia cordis is a rare congenital disorder of unknown pathogenesis with sporadic incidence [2]. Especially, complete thoracic ectopia cordis in addition to complex heart disease, is known to have poor prognosis [4,5]. Surgical internalization of the heart into the thoracic cavity is associated with high risk of cardiovascular compromise andischemia [5].…”

Section: Discussionmentioning

confidence: 99%

“…Especially, complete thoracic ectopia cordis in addition to complex heart disease, is known to have poor prognosis [4,5]. Surgical internalization of the heart into the thoracic cavity is associated with high risk of cardiovascular compromise andischemia [5]. The paucity of survivors with this entity demonstrates the need for extreme caution in preserving hemodynamic stability when the heart is covered, whether the repair is performed as a primary or a staged repair.…”

Section: Discussionmentioning

confidence: 99%

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Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report

Delgado¹,

Matongo²,

Dumo³

et al. 2019

JPP

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Ectopia cordis (EC) is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen. It can be defined as 0.1% of congenital heart diseases, and it could present isolated or could belong to the spectrum of the Pentalogy of Cantrell (PoC), which is a rare congenital disorder first described in 1958 by Cantrell. We are reporting a rare case of total ectopia cordis, associated to a major omphalocele, total agenesis of the sternum, anterior diaphragmatic deficiency, absence of pericardium, and persistence of the Ductus arteriosus, making therefore these features compatible with a full spectrum of the Pentalogy of Cantrell, encouraging us to report this case.Key words: Ectopia cordis, major omphalocele, sternum agenesis, anterior diaphragmatic deficiency, absence of pericardium, persistence of the Ductus arteriosus.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report

Delgado¹,

Matongo²,

Dumo³

et al. 2019

JPP

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“…(Sadlecki et al 2011;Sakasai et al 2012) Surgical internalization of the heart into the thoracic cavity is associated with high risk of cardiovascular compromise and ischemia. (Sakasai et al 2012) We present a case of Pentalogy of Cantrell with total ectopia cordis surgically covered with musculocutaneous flaps and corrected double outlet right ventricle of Fallot type after 11 years of follow up. Today we see ourselves confronted with the 11-year-old boy in very good general condition without severe subjective compromise.…”

Section: Discussionmentioning

confidence: 99%

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle

Harring

Weil

Thiel

et al. 2015

Congenital Anomalies

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Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.

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“…An abnormal closure of the ventral body wall by the lateral body wall folds and an arrest of cardiac descent during this stage (3 th -4 th embryonic week) may results in disorders varying from isolated ectopia cordis to complete ventral evisceration [2,3,5,7,9]. Although genesis of ectopia cordis has not been fully explained, several theories are suggested: early rupture of the chorion and/or the yolk sac or amniotic band syndrome [1,3,5].…”

Section: Discussionmentioning

confidence: 99%

Ectopia Cordis: A Case Report and Review of Literature

Habou¹,

Amadou²,

Adamou³

et al. 2017

JCR

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Background: Ectopia cordis is a rare congenital anomaly characterized by partial or complete displacement of the heart outside the thoracic cavity. Here, we report a case of two-day-old black neonate born with an ectopia cordis associated with an omphalocele. Case Report: A full-term, barely responsive, black newborn presented at the pediatric surgery department with a heart totally outside the thoracic cavity without pericardium protection and an omphalocele. The baby was transferred immediately to the intensive care unit but died a few hours later before any surgical treatment. Conclusion: Ectopia cordis is an uncommon congenital anomaly. Its management represents a challenge even in developed country, and its prognosis is poor despite advances in neonatal cardiac surgery.

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Staged Repair of Pentalogy of Cantrell with Ectopia Cordis and Ventricular Septal Defect

Cited by 20 publications

References 8 publications

Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report

Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele—A Case Report

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle

Ectopia Cordis: A Case Report and Review of Literature

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