Chapter 19

### Key Points

1. The nomenclature for ectopia cordis is confusing and inaccurate; only a few survivors of “true” ectopia cordis have ever been reported.

2. Thoracic ectopia cordis or “true” ectopia cordis is an extremely rare condition in which the bare heart beats outside the body on the newborn's chest, and is frequently tilted so that the apex is near the patient's chin.

3. Thoracoabdominal ectopia cordis is an omphalocele variant involving major deficiencies of the abdominal wall (superior to and including the umbilicus), diaphragm, and lower sternum. Although the heart may protrude (and may be visible), it is typically covered with attenuated pericardium or skin, and is properly oriented.

4. Sternal cleft is a condition in which there is a deficit in the superior sternum. In some patients with large clefts, the heart appears to protrude out of the chest when the patient performs a Valsalva maneuver.

5. Patients with ectopia cordis often have structural abnormalities of the heart, whereas patients with sternal clefts usually have normal hearts.

6. Sternal clefts can usually be repaired in a single stage with primary closure or autologous tissues. In contrast, repairs of ectopia cordis generally require staged operations and the use of prosthetic materials.

### Introduction

Ectopia cordis refers to a rare congenital anomaly, sometimes considered bizarre or even horrifying, in which the heart is located outside the thoracic cavity. The exposed heart is frequently tilted so that the apex of the heart is near the patient's chin. Sternal clefts, however, can range in severity from relatively minor defects in the sternum to large sternal deficiencies through which the heart, although covered by pericardium and skin, herniates out of the chest with crying or straining. This condition is so dramatic and it has at times mistakenly been called “partial ectopia cordis” or “thoracocervical ectopia cordis” (because the protruding heart appears to rise through the sternal cleft into the neck), even though the heart is structurally normal and in the anatomically correct position.

The first reported case of ectopia cordis is attributed to Niels Stensen in 1671 (“the sternum was split, and the heart, liver, and spleen, most of the intestines and right kidney have passed out through the slit being thus uncovered…”). Others, however, credit Haller or Martinez, who both reported cases in 1706. Operative repair was first described in 1925 by Cutler and Wilens, who attempted unsuccessfully to create a subcutaneous pocket over the left chest to cover the exposed heart. The infant died 11 hours after surgery. An early and celebrated case of a sternal cleft involved E. A. Groux of Hamburg, who, in the 1850s, exhibited himself at many universities and claimed that more than 2000 physicians had examined his heart through the V-shaped defect in his sternum. When Groux performed a Valsalva maneuver, his heart thrust forward, allowing direct palpation of its activity. Investigators made numerous studies on Groux and were able to determine, for example, that contraction ...

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