Asian Cardiovasc Thorac Ann 2001;9:65-67
© 2001 Asia Publishing EXchange Pte Ltd
Extrathoracic Heart (Ectopia Cordis)
Balram Airan, MCh,
Harpreet Wasir, MCh,
Shiv Kumar Choudhary, MCh,
Shyam Sunder Kothari, DM,
Anita Saxena, DM,
Panangipalli Venugopal, MCh
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Department of Cardiothoracic and Vascular Surgery Cardiothoracic Sciences Centre All India Institute of Medical Sciences New Delhi, India
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For reprint information contact: Balram Airan, MCh Tel: 91 11 686 4851 Fax: 91 11 686 2663 email: airanbalram{at}hotmail.com Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.
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Abstract
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Two cases of ectopia cordis are described. One patient underwent an initial procedure to cover the heart using homologous pericardium and a homograft pulmonary artery patch but did not survive. The other baby died before any surgical intervention could be performed.
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Introduction
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Ectopia cordis is a rare congenital cardiac anomaly in which the heart is located outside the confines of the chest cavity. Surgical correction of this defect is complex and generally requires a staged closure. This involves coverage of the bare or "naked" heart, placement of the heart into the thoracic cavity, and repair of associated anomalies, particularly intracardiac defects and omphaloceles.
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Case Reports
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Patient 1
A full-term baby boy (birth weight, 2.4 kg) was delivered at home after an uncomplicated pregnancy and transferred to our institute 6 hours later because of an extrathoracic heart. There was a midline defect in the thoracoabdominal wall, which extended from the manubrium of the sternum to the umbilicus. Overlying pericardium was absent. The abdominal portion of the defect was partially covered by skin and the peritoneum was intact. The apex of the heart was upturned cranially towards the chin and the vascular pedical was exposed. He had a heart rate of 164 beats per minute and a respiratory rate of 78 breaths per minute. There was mild central cyanosis and pulse oximetry showed oxygen saturation of 68%. There was no evidence of omphalocele or any other congenital abnormality (Figure 1
). Arterial blood gas analysis indicated gross metabolic acidosis. The patient was intubated and his metabolic parameters were corrected. Echocardiography revealed pulmonary atresia and a ventricular septal defect. The baby died before any surgical intervention could be performed.
Patient 2
A full-term 2.5-kg female neonate was brought to the hospital 4 hours after birth with stable vitals. Her heart was situated outside the chest cavity. There was no cyanosis, oxygen saturation was 90%, and there was good peripheral perfusion. The heart protruded through a midline sternal defect with the apex pointing towards the chin. The pericardium was absent and there was no skin cover. The heart was rotated through 90 degrees in the frontal plane so that the diaphragmatic surface was anterior and the anterior surface faced the thoracic cavity. The diaphragm was exposed but there was no omphalocele and no craniofacial abnormalities. The sternum was completely absent. Metabolic parameters were corrected and the heart was covered with saline-soaked sterile gauze pads to prevent dehydration. Chest radiography revealed an upturned heart, a small chest cavity, and a large liver pushing into the chest cavity. Detailed echocardiography was not possible but a large ventricular septal defect was noticed on a partial study.
Surgical repair was undertaken. Intubation was difficult due to the upturned heart. Via midline exposure, bilateral pectoral flaps were adequately raised. The enlarged thymus gland was completely excised. The great vessel pedicles were cleared up to the base of the heart. After proper identification, the left phrenic nerve and left internal mammary artery were preserved. Longitudinal partial excision of the left costal cartilages was performed to create space in the chest cavity to accommodate the heart. Even after extensive mobilization of skin flaps and excision of costal cartilages, there was bradycardia and hypotension when an attempt was made to place the heart in the chest cavity. As efforts to give primary skin cover also resulted in bradycardia and hypotension, an initial repair was carried out in which the heart was covered using homologous pericardium and a homograft pul-monary artery patch. However, the patient's hemodyna-mics slowly deteriorated and in spite of adequate ventilation and inotropic support, she died 36 hours after surgery. Permission for autopsy was refused.
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Discussion
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Ectopia cordis is a very rare but dramatic congenital malformation (< 0.1% of all congenital heart defects) that presents a great surgical challenge. The incidence ranges from 5.5 to 7.9 per one million live births. Two-thirds of patients are male and one-third are premature or stillborn. Ectopia cordis is usually found in the presence of various other anomalies (Table 1).1 The condition can be classified into 4 types, depending upon the position of the heart: cervical, thoracic, thoracoabdominal, or abdominal. The most common variety is thoracic ectopia in which the heart lies partially or completely outside the chest with the ventricular apex pointing cephalad, as seen in both of our patients. This type carries the worst prognosis with < 5% surviving beyond the first month of life.2 Equally common is the thoracoabdominal type that is frequently associated with Cantrell's pentad.3 This comprises bifid sternum, deficiency of the diaphragm, diaphragmatic pericardium, anterior abdominal wall defects, and intra-cardiac defects. A number of patients with the abdominal type live until adulthood but the cervical type appears to be incompatible with life. Although the exact etiology is unknown, various reports have shown an association with chromosomal abnormalities and mechanical teratogenesis.4,5
Improved techniques of antenatal screening have facilitated the diagnosis of congenital heart disease including all varieties of ectopia cordis. A swinging movement of the heart in the amniotic fluid readily gives a clue to extrathoracic heart.6 Detailed antenatal examina-tion of the fetal heart may suggest a favorable prognosis but elective termination of pregnancy might be indicated in other cases. Caesarean delivery may be decided in cases of ectopia cordis that appear to be amenable to surgical repair.1
Hornberger and colleagues7 in one of the largest single institutional series, reported that survival beyond infancy and successful surgical repair of ectopia cordis with definitive palliation was possible. Ten of 13 cases (4 thoracic, 9 thoracoabdominal ectopia cordis) were man-aged postnatally; 3 patients with significant extracardiac defects died without any surgical intervention, 5 of the remaining 7 patients without extracardiac defects were alive at 3.5 to 9.5 years after successful surgical repair. Toyoma8 reported a survival rate beyond the first month of life of < 30% in cases of ectopia cordis with structural heart defects.
Attempts at staged correction have been made as early as possible after birth but results have mostly been unfavorable. Prognosis is related to the severity of the intracardiac defects. The type of ectopia cordis, capacity of thoracic cavity, hemodynamic instability due to kinking of great vessels, absence of skin over the heart, and associated extracardiac defects pose various surgical problems. Immediate treatment should begin with covering the heart with warm saline-soaked sterile gauze pads, maintaining optimal temperature, and preventing dehydration and desiccation of the ectopic heart and omphalocele, if present. Harrison and colleagues1 reported using adherent plastic drapes to cover an omphalocele in a case of ectopia cordis. Primary coverage can also be accomplished by mobilizing skin over the chest wall, as attempted in patient 2, using split-thickness skin grafting or a polytetrafluoroethylene membrane and skin flaps.
Although the available literature is scant, various issues regarding the management of ectopia cordis have been highlighted. Having decided to go ahead with the pregnancy rather than opt for medical termination, the neonate should be referred promptly to a specialist pediatric cardiac surgical team. After hemodynamic stabilization, temporary coverage of the heart should be attempted. Complete diagnosis of intracardiac defects should be ascertained and the decision to proceed with palliative or complete correction should be made according to these findings.
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References
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Harrison MR, Filly RA, Stanger P, de Lorimier AA. Prenatal diagnosis and management of omphalocele and ectopia cordis. J Pediatr Surg 1982;17:64–6.[Medline]
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Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg 1990;5:156–64.
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Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602–14.[Medline]
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Klingensmith WC III, Cioffi-Ragan DT, Harvey DE. Diagnosis of ectopia cordis in the second trimester. J Clin Ultrasound 1988;16:204–6.[Medline]
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Van Allen MI, Mytire S. Ectopia cordis thoracalis with craniofacial defects resulting from early amnion rupture. Teratology 1985;32:19.[Medline]
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Todros T, Presbitero P, Montemurro D, Levis F. Prenatal diagnosis of ectopia cordis. J Ultrasound Med 1984;3: 429–31.[Medline]
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Hornberger LK, Colan SD, Lock JE, Wessel DL, Mayer JE Jr. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation 1996;94 (Suppl II):32–7.
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Toyoma WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: a case report and review of the syndrome. Pediatrics 1972;50:778–92.[Abstract/Free Full Text]
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Abstract
Introduction
