Eur J Cardiothorac Surg 2003;23:426-428
© 2003 Elsevier Science NL
Complete thoracic ectopia cordis
N. Alphonso*,
P.S. Venugopal,
R. Deshpande,
D. Anderson
Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas'Hospitals NHS Trust, St Thomas' Street, London, SE1 9RT, UK
Received 24 October 2002;
accepted 28 November 2002.
* Corresponding author. 24 Andace Park, Widmore Road, Bromley BR1 3DH, Kent, UK. Tel.: +44-20-8325-3140; fax: +44-20-7955-4858
e-mail: nelson.a{at}bigfoot.com
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Abstract
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Thoracic ectopia cordis is a rare congenital defect with very few reported survivors after surgical correction. We report a case of complete thoracic ectopia cordis with double outlet right ventricle. The diagnosis was established antenatally and a repair was undertaken soon after birth. The child remained stable and was extubated on the fifth post-operative day. Forty-eight hours later the child succumbed to an unexplained respiratory arrest. Also presented is a review of the different surgical strategies for this unusual condition.
Key Words: Thoracic ectopia cordis
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1. Introduction
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Ectopia cordis is a rare congenital defect commonly associated with intra-cardiac lesions [1]. The thoracic subtype involves partial or complete displacement of the heart outside the thoracic cavity through a sternal defect. Despite advances in neonatal cardiac surgery, complete thoracic ectopia cordis remains a surgical challenge with only few long-term survivors [1–4].
We report a case of complete thoracic ectopia cordis with double outlet right ventricle (DORV).
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2. Case report
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The diagnosis of complete thoracic ectopia cordis with DORV was made by antenatal ultrasound screening. An elective caesarean section was performed in an adjacent operating theatre. The sternum was entirely deficient and the heart was completely extra-thoracic oriented almost at right angles to the chest wall (Fig. 1
). There was a normal arrangement of the great vessels and an intact diaphragm.
We decided to adopt a staged surgical approach to this defect with the initial goal being replacement of the heart in the thoracic cavity.
The skin was incised down to the costal margin. The sternum was separated from the skin edges and the pericardial reflections were freed from the posterior halves of the sternum. The pericardium and pleura were incised down to the great veins and the left pulmonary artery. The diaphragm was reflected off the undersurface of the left costal margin and both pleura was laid open.
A traction suture was placed in the apex of the heart and brought through the left chest wall. The heart could now be relocated within the thoracic cavity without haemodynamic compromise with the apex directed into the left pleural cavity. The heart was covered with a patch of Gore-Tex sutured to the edges of the defect (Fig. 2
) overlaid with a patch of cadaveric skin.
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Fig. 2. The heart relocated within the thoracic cavity covered with a Gore-Tex patch overlaid with cadaveric skin.
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The child was weaned off the ventilator on the fifth post-operative day. Forty-eight hours later the child suffered an unexpected respiratory arrest and could not be resuscitated. We are unsure as to the exact cause of the patient's demise. Possibilities include carbon-dioxide retention due to flail behaviour of the anterior chest wall, pulmonary hypoplasia [2], and sepsis (despite the absence of corroborating evidence).
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3. Discussion
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Failure of fusion of the paired cartilage bars of the embryonic sternum leads to a sternal cleft. When associated with the heart being positioned outside the chest wall it is known as ectopia cordis [5]. It is classified into cervical, cervico-thoracic, thoracic and more commonly the thoraco-abdominal or abdominal types [2,6]. Cantrell in 1958 described a pentalogy characterized by a midline supra-umbilical abdominal wall defect, a defect of the lower part of the sternum, a deficiency of the anterior diaphragm, a defect of the diaphragmatic pericardium and a congenital heart malformation [7].
Thoracic ectopia cordis is extremely rare with a reported incidence of 5.5–7.9/million live births [1]. In partial thoracic ectopia cordis the heart can often be seen to pulsate through the skin. In complete thoracic ectopia cordis the naked heart is displaced outside the thoracic cavity without pericardial coverage [2]. Many cases of these (80.2%) have an associated intra-cardiac defect [6] including ventricular septal defect (VSD, 100%), atrial septal defect (ASD, 53%), tetralogy of Fallot (TOF, 20%), left ventricular diverticulum (LVD, 20%) and pulmonary hypoplasia [2].
Partial thoracic ectopia cordis can often be repaired electively when the sternal cleft is closed primarily after freshening the edges and making oblique relaxing incisions in the costal cartilages bilaterally [5].
Complete thoracic ectopia cordis left untreated is universally lethal [1]. There are only few reported survivors following surgical correction [1–4,6,8,9]. Of the four reported survivors beyond 1 month [1–4], only one patient has had an intra-cardiac lesion corrected successfully [1]. Three patients who survived neonatal surgical correction, died from unrelated causes [6,8,9]. Though all long-term survivors had a staged repair, we feel that in view of the rarity of this condition, no uniform surgical strategy can be suggested.
The aims of surgical treatment are: (1) to provide soft tissue cover of the heart; (2) to reduce the heart into the thoracic cavity; (3) palliation or repair of any intra-cardiac defect; and (4) reconstruction of the chest wall.
There is only one report of a neonatal single stage repair of complete thoracic ectopia cordis with correction of an intra-cardiac defect [9]. Unfortunately, the baby succumbed to sepsis on the twelfth post-operative day.
In a staged repair, the initial intervention is aimed at providing soft tissue coverage to the heart [1,2,8]. If primary approximation with skin flaps cannot be achieved or if there is any haemodynamic compromise, then a split-thickness skin graft, cadaveric skin graft or prosthetic material can be sutured to the skin edges. The defect can then be reduced slowly over the ensuing weeks [2].
The timing for palliation of haemodynamically significant intra-cardiac defects remains unclear. Morales [2] suggested that the placement of a Blalock–Taussig shunt or a pulmonary artery band should be deferred for a few weeks until the haemodynamic effects of chest coverage have stabilized.
Reduction of the heart into the thoracic cavity is carried out at a subsequent stage [1,2,8]. Simple stable intra-cardiac defects like ASD, VSD, LVD and TOF can be repaired at this time. Moreover, defects like ASD or VSD may have resolved spontaneously.
Reduction of the heart into a small thoracic cavity in the neonatal period often produces compression, kinking of the great vessels and a low cardiac output [2,8]. However, Amato has suggested that it is worthwhile making an attempt at placing the heart even partially within the thoracic cavity at the first stage [6]. This would make subsequent procedures easier and also avoid the obvious physical deformity. The heart can be returned to the left or right chest depending on which way the apex points. Additional techniques have been suggested to enlarge the thoracic cavity [9].
Chest wall reconstruction is carried out at a later date in collaboration with a plastic surgeon [6,8].
Finally, in patients with unstable complex haemodynamically intra-cardiac defects a one-stage repair may have to be attempted in the neonatal period despite a poor outcome.
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References
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- Hornberger L.K., Colan S.D., Lock J.E., David L.W., Mayer J.E. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation 1996;94(Suppl II):II-32-II-37.
- Morales M.J., Patel S.G., Duff J.A., Villareal R.L., Simpson J.W. Ectopia cordis and other midline defects. Ann Thorac Surg 2000;70:111-114.[Abstract/Free Full Text]
- Saxena N.C. Ectopia cordis child surviving: prosthesis fails. Pediatric News 1976;10:3.
- Dobell A.R.C., Williams H.B., Long R.W. Staged repair of ectopia cordis. J Pediatr Surg 1992;17:353-358.
- Fonkalsrud E.W. Chest wall abnormalities. In: Bove A.E., Geha A.S., Hammond G.L., Laks H., Naunheim K.S., eds. Glenn's thoracic and cardiovascular surgery. East Norwalk, CT: Appleton and Lange, 1991:581-591.
- Amato J.J., Jonathan Z., Talwalkar N.G. Single stage repair of thoracic ectopia cordis. Ann Thorac Surg 1995;59:518-520.[Abstract/Free Full Text]
- Cantrell J.R., Haller J.A., Ravitch M.M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602.[Medline]
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- Watterson K.G., Wilkinson J.L., Kliman L., Mee R.B.B. Complete ectopia cordis with double outlet right ventricle: neonatal repair. Ann Thorac Surg 1992;53:146-147.[Abstract]
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Abstract
1. Introduction
