Download Complete thoracic ectopia cordis

European Journal of Cardio-thoracic Surgery 23 (2003) 426–428
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Case report
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
Abstract
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.
q 2003 Elsevier Science B.V. All rights reserved.
Keywords: Thoracic ectopia cordis
1. Introduction
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).
2. Case report
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
* Corresponding author. 24 Andace Park, Widmore Road, Bromley BR1
3DH, Kent, UK. Tel.: 144-20-8325-3140; fax: 144-20-7955-4858.
E-mail address: [email protected] (N. Alphonso).
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.
The child was weaned off the ventilator on the fifth postoperative 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).
3. Discussion
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 supraumbilical abdominal wall defect, a defect of the lower
1010-7940/03/$ - see front matter q 2003 Elsevier Science B.V. All rights reserved.
doi:10.1016/S 1010 -79 40(02)00811-4
N. Alphonso et al. / European Journal of Cardio-thoracic Surgery 23 (2003) 426–428
Fig. 1. Complete thoracic ectopia cordis.
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
427
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.
References
Fig. 2. The heart relocated within the thoracic cavity covered with a GoreTex patch overlaid with cadaveric skin.
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