23-1. Ectopia Cortis
Ectopia cortis is a dramatic anomaly even to the experienced heart surgeon.
Infants born with this anomaly always have associated midline defects, including an omphalocele and sternal and/or rib deficiencies. Associated intracardiac prob- lems are common. Initial surgery is aimed at covering the heart and abdominal wall defect with skin. Ideally, repair of intracardiac defects is delayed until a later age, as is chest wall reconstruction.
heart
FIGURE
23-1. The ectopic heart is outside the chest wall and uncovered and unprotected.
There is a large associated omphalocele.
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464 Color Atlas of Congenital Heart Surgery
omphalocele
FIGURE
23-2. In the worst form of ectopia cortis, the heart is tipped up with the apex point- ing toward the baby's chin. It is very difficult to manage this problem because shifting the heart caudad often kinks the great vessels, which significantly alters hemodynamics.
area over heart area of previous omphalocele
skin graft
FIGURE
23-3. The omphalocele has been repaired primarily and there is skin coverage
over the cardiac structures. To accomplish this, adjacent releasing incisions have been
made bilaterally. These areas are closed with skin grafts. The baby had a ventricular septal
defect (VSD), which was later repaired. Chest wall reconstruction was also carried out
late by filleting the lower anterior rib cage and shifting rib grafts immediately.
FIGURE
23-4. Another infant was born with ectopia cortis and a large omphalocele with a short sternum and anterior rib deficiencies. There was 75% skin coverage at birth and the remaining coverage occurred naturally by ingrowth of surrounding skin. The infant returned at this time for definitive intracardiac repair and repair of the omphalocele. The baby has Pentalogy of Cantrell, which includes a large left ventricular diverticulum with a smaller right ventricular diverticulum associated with tetralogy of Fallot.
R ^
attaching tissue
FIGURE
23-5. The short upper sternum was incised and the heart and omphalocele
exposed through a longitudinal incision. There is a large lower pericardial and anterior
midline diaphragm deficiency. The upper pericardium is well developed, however, in the
lower area of absent pericardium there were adhesions between the heart and surround-
ing tissue.
466 Color Atlas of Congenital Heart Surgery
ceph R « - - > L
caud
area of pericardial and diaphragm deficiency
ectopia cordis or diverticulum
FIGURE 23-6. The large left ventricular diverticulum, which extended into the abdominal space, is exposed and exteriorized.
ceph R < > L
caud
diverticulum
FIGURE 23-7. The child is placed on cardiopulmonary bypass and excision of the diver- ticulum is carried out.
apical ventriculotomy
diaphragm deficiency
FIGURE
23-8. A two-layer closure of the diverticulum is performed with interrupted felted mattress sutures followed by an epicardial continuous stitch.
ceph R « -
caud - > L '£'
apical ventriculotomy
FIGURE 23-9. The ventriculotomy closure is complete.
468 Color Atlas of Congenital Heart Surgery
ceph R<- - • L
t caud
outflow tract ventriculotomy
muscle bundles
FIGURE
23-10. Because the heart is rotated severely to the right, transatrial repair of the tetralogy was not possible. A short high right ventriculotomy has been made and obstruct- ing muscle bundles exposed and excised.
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outflow tract patch
FIGURE
23-11. The VSD was closed with a Dacron® patch, after which the outflow tract below the pulmonary valve was reconstructed with a homograft pulmonary artery wall.
After completion of the cardiac operation, primary repair of the omphalocele was per-
formed, including abdominal wall repair. The diaphragm deficiency was repaired with a
Dacron® patch.
R«*
lower rib cage
area of heart
FIGURE
23-12. One year following the cardiac-omphalocele repair, the same child returned for definitive chest wall reconstruction. The sternotomy scar was entered and subcutaneous tissue dissected bilaterally across the existing lower rib cage to the mid cla- vicular line. Anterior chest wall muscle was dissected free of the ribs in the same area.
filleted ribs
FIGURE
23-13. The lower anterior ribs were filleted in the longitudinal plane of the rib or
coronal body plane leaving the anterior perichondrium intact and attached to the ante-
rior filleted rib sections.
470 Color Atlas of Congenital Heart Surgery
ceph R < - - • L
caud
left filleted rib patch
right filleted rib patch
FIGURE
23-14. Patches of the anterior filleted ribs were moved centrally to position them for anterior wall reconstruction. In other cases, these filleted ribs may be inverted to allow more effective use of these patches; here, it was more appropriate to leave the patches uninverted.
R ^
left and right sided filleted rib patches
FIGURE
23-15. The anterior rib patches were placed in the midline over the area of defi-
cient chest wall and sutured in place. Leaving perichondrium attached posteriorly to the
native ribs and anteriorly to the filleted portions allows regrowth of all rib segments.
ant caud <-
post -^•cept
epicardial lead
FIGURE
23-16. A short submammary incision in the fifth intercostal space is made. The pericardium is incised transversely. Ventricular and/or atrial steroid-emitting tip leads are attached to the surface of the heart. The lead is positioned with the tip of the foot plate pointed caudad and the wire looped toward the head. This loop is placed inside of the pericardium and helps to avoid later tension on the foot plate with growth of the baby.
The pericardium is closed over the wire.
ant caud^- ->ceph
post
leads in thorax leads in abdominal
wall
FIGURE
23-17. A short transverse incision is made in the left upper abdominal quadrant
and a pocket created between the external and internal oblique muscles. Placing the
battery pack deep to abdominal wall muscle helps to prevent erosion of the box. If the
battery back is placed between the internal oblique and transversalis muscles, there is a
greater danger that the abdominal cavity will be entered at a later date when the battery
pack is changed.
472 Color Atlas of Congenital Heart Surgery
ant caud^- -•ceph
post
FIGURE
23-18. The wires pass from the thoracic cavity to the abdominal wall after pierc- ing the diaphragm and passing them under the lowest anterior rib. The wires should not be passed between ribs because a kink here increases the risk of wire fracture.
ant caud<- -^•ceph
post
box
FIGURE
