Complete Thoracic Ectopia Cordis With DoubleOutlet Right Ventricle: Neonatal Repair Kevin G. Watterson, FRACS, J. L. Wilkinson, FRCP, L. Kliman, FRACOG, and Roger B. B. Mee, FRACS Royal Children’s Hospital, Melbourne, Australia
A case of total thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect is presented. Prenatal diagnosis allowed single-stage correction immediately after birth. This approach proved to be technically feasible. Death occurred on the twelfth postoperative day owing to sepsis unrelated to the repair. (Ann Thorac Surg 1992;53:146-7)
E
ctopia cordis is a very rare congenital defect commonly associated with intracardiac defects and can be seen in either partial or complete forms [1-6]. Survival has been difficult to achieve in the latter condition. We report a case of complete thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect in which a one-stage correction was attempted immediately after birth. A 38-year-old woman was referred after a routine second trimester ultrasound (17 weeks’ gestation) revealed ectopia cordis. Further serial ultrasound examinations demonstrated a ventricular septal defect, but the great vessel relationships were not clear. A one-stage correction was planned, and because of developing hydrops the fetus was delivered by classic cesarean section at 36 weeks gestation in an adjacent operating room. There was a tear in the epicardium across the right atrium and extending onto the right ventricle. The heart was covered with sterile plastic and the infant resuscitated. The entire heart was protruding through a large defect involving the front of the chest wall and through which both lungs were also partially protruding (Fig 1). Sterile echocardiography demonstrated double-outlet right ventricle with ventricular septal defect. A persistent left superior vena cava was visible. Pressure lines were inserted and the patient was redraped and a one-stage repair embarked upon. A midline incision was made up into the neck above the thoracic defect and down into the abdomen. There was a single cartilaginous bar crossing the midline inferiorly but no xiphisternum. This bar continued in a horseshoe fashion, and the front ends of the ribs and clavicles were attached to it. There was no sternum superiorly. Cardiopulmonary bypass was instituted using a single venous Accepted for publication June 18, 1991. Address reprint requests to Mr Mee, Victorian Paediatric Cardiac Surgical Unit, Royal Children’s Hospital, Flemington Road, Parkville, Melbourne, Australia 3052.
0 1992 by The Society of
Thoracic Surgeons
cannula with core cooling to 16°C. During cooling, the ductus was divided and the lungs fully mobilized from the edges of the defect in the thoracic wall. The diaphragm was detached anteriorly from the inferior cartilaginous bar, which was then excised, allowing the liver to move posteriorly. After circulatory arrest a right atriotomy was performed. A noncommitted ventricular septal defect was visualized in association with double-outlet right ventricle, and so a long Dacron patch was inserted to connect the left ventricle to the aorta. During rewarming, thoracic expansion was performed and involved the following steps: (1) The anterior diaphragm was divided down to and to the right of an anteriorly placed inferior vena cava to allow the inferior vena cava to settle posteriorly when the heart was placed in situ. (2) An incision was made in the pleura in both costophrenic angles for the complete circumference to allow the diaphragm to move down about 0.75 cm and increase the thoracic cavity on each side. (3)Right-sided multiple rib slide was performed after the intercostal spaces had been exposed. Each rib was split longitudinally for 2 cm and half transected at medial and lateral ends. A rib slide was then carried out with a 4-mm overlap, moving the ventricle cartilage bar 1.5 cm toward the midline. (4)On the left side the ribs were transected in a staggered fashion, allowing the left vertical cartilage bar to move 0.5 cm toward the midline. The heart was inserted inside the thoracic cavity with the apex to the left, and the patient was weaned from bypass with satisfactory hemodynamics. The vertical cartilage bars were sutured together followed by direct skin closure. Peak lung inflation pressure was 32 mm Hg. Over the next 36 hours the circulation deteriorated. Sutures to the midportion of the sternal bars were removed, and the elliptical gap was closed with a thin Gore-Tex patch. The skin edges were reclosed. Subsequently the child‘s condition steadily improved, and by the ninth postoperative day he had stable hemodynamics with satisfactory lung fields radiologically (Fig 2) and a peak lung inflation pressure of 21 mm Hg. One day later hemodynamic collapse occurred with a clinical picture of sepsis (fever, low blood pressure, dilated periphery, rising white blood cell count [lo to 32 X 109/L]and falling platelet count). Use of antibiotics and increased inotropes led to some clinical improvement, but the general course was downhill with death on the twelfth postoperative day. Autopsy confirmed the diagnosis with an associated moderately hypoplastic left ventricle and mitral valve. 0003-4975/92/$3.50
CASE REPORT WAITERSON ET AL COMPLETE THORACIC ECTOPIA CORDIS
Ann Thorac Surg 1992;53:14&7
147
B Fig 2 . Plain chest roentgenograms taken (A) immediately and ( B ) 9 days postoperatively.
Fig 1. Patient after resuscitation.
Comment Survival after correction has been documented in the incomplete form of ectopia cordis [ 1 4 ] and also in the total thoracic form with no intracardiac defects [5] but not when the combination [6] exists. We reasoned that the best chance for survival would be immediate correction after birth. Sterility was maximized by using cesarean section in an adjacent operating room and covering the heart in sterile plastic immediately after birth. Sterile direct echocardiography was of use for preoperative planning. The intracardiac repair was performed in the usual manner under profound hypothermia and circulatory arrest. Expansion of the thoracic cavity proved to be technically feasible but depended on (1) widening the lateral and anteroposterior diameters by a rib slide maneuver as well as rib transection and detachment of the diaphragm from the inferior cartilaginous bar and (2) lengthening the cavity by lowering the diaphragm. The external orientation of the heart at right angles to the chest wall in our patient predisposed it to flow obstruction with
minimal interference and the technical impossibility of in situ skin cover; thus placement back into the thoracic cavity was mandatory. Cardiopulmonary bypass was needed for this, and hence total internal cardiac repair was done at the same time. Although our patient did not survive, death was due to sepsis (with no focus being located in the thoracic cavity) rather than directly related to the repair itself.
References 1. Cantrell JR, Haller JA, Ravitch Mh4. A syndrome of congenital
2. 3.
4.
5. 6.
defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107602-14. Jones AF, McGrath RL, Edwards SM, Lilly JR. Immediate operation for ectopia cordis. Ann Thorac Surg 1979;28:484-6. Crittenden IH, Adams FH, Mulder DG. A syndrome featuring defects of the heart, sternum, diaphragm and anterior abdominal wall. Circulation 1959;20:396404. Tachibana H, Gan K, Oshima Y, Ohashi H, Hosokawa Y, Yamaguchi M. Thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. J Jpn Assoc Thorac Surg 3 7148-53. Dobell ARC, Williams AHB, Long RW. Staged repair of ectopia cordis. J Pediatr Surg 1982;17353-8. Poulias GE, Raftopoulos J, Polernis L, Skoutas B, Katsouli R. Total thoracic ectopia cordis with complete absence of sternum and pericardium and double diverticulum. J Cardiovasc Surg 1982;23:75!3.
A case of total thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect is presented. Prenatal diagnosis allowed single-stage correction immediately after birth. This approach proved to be technically feasible. Death occurred on the twelfth postoperative day owing to sepsis unrelated to the repair. (Ann Thorac Surg 1992;53:146-7)
E
ctopia cordis is a very rare congenital defect commonly associated with intracardiac defects and can be seen in either partial or complete forms [1-6]. Survival has been difficult to achieve in the latter condition. We report a case of complete thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect in which a one-stage correction was attempted immediately after birth. A 38-year-old woman was referred after a routine second trimester ultrasound (17 weeks’ gestation) revealed ectopia cordis. Further serial ultrasound examinations demonstrated a ventricular septal defect, but the great vessel relationships were not clear. A one-stage correction was planned, and because of developing hydrops the fetus was delivered by classic cesarean section at 36 weeks gestation in an adjacent operating room. There was a tear in the epicardium across the right atrium and extending onto the right ventricle. The heart was covered with sterile plastic and the infant resuscitated. The entire heart was protruding through a large defect involving the front of the chest wall and through which both lungs were also partially protruding (Fig 1). Sterile echocardiography demonstrated double-outlet right ventricle with ventricular septal defect. A persistent left superior vena cava was visible. Pressure lines were inserted and the patient was redraped and a one-stage repair embarked upon. A midline incision was made up into the neck above the thoracic defect and down into the abdomen. There was a single cartilaginous bar crossing the midline inferiorly but no xiphisternum. This bar continued in a horseshoe fashion, and the front ends of the ribs and clavicles were attached to it. There was no sternum superiorly. Cardiopulmonary bypass was instituted using a single venous Accepted for publication June 18, 1991. Address reprint requests to Mr Mee, Victorian Paediatric Cardiac Surgical Unit, Royal Children’s Hospital, Flemington Road, Parkville, Melbourne, Australia 3052.
0 1992 by The Society of
Thoracic Surgeons
cannula with core cooling to 16°C. During cooling, the ductus was divided and the lungs fully mobilized from the edges of the defect in the thoracic wall. The diaphragm was detached anteriorly from the inferior cartilaginous bar, which was then excised, allowing the liver to move posteriorly. After circulatory arrest a right atriotomy was performed. A noncommitted ventricular septal defect was visualized in association with double-outlet right ventricle, and so a long Dacron patch was inserted to connect the left ventricle to the aorta. During rewarming, thoracic expansion was performed and involved the following steps: (1) The anterior diaphragm was divided down to and to the right of an anteriorly placed inferior vena cava to allow the inferior vena cava to settle posteriorly when the heart was placed in situ. (2) An incision was made in the pleura in both costophrenic angles for the complete circumference to allow the diaphragm to move down about 0.75 cm and increase the thoracic cavity on each side. (3)Right-sided multiple rib slide was performed after the intercostal spaces had been exposed. Each rib was split longitudinally for 2 cm and half transected at medial and lateral ends. A rib slide was then carried out with a 4-mm overlap, moving the ventricle cartilage bar 1.5 cm toward the midline. (4)On the left side the ribs were transected in a staggered fashion, allowing the left vertical cartilage bar to move 0.5 cm toward the midline. The heart was inserted inside the thoracic cavity with the apex to the left, and the patient was weaned from bypass with satisfactory hemodynamics. The vertical cartilage bars were sutured together followed by direct skin closure. Peak lung inflation pressure was 32 mm Hg. Over the next 36 hours the circulation deteriorated. Sutures to the midportion of the sternal bars were removed, and the elliptical gap was closed with a thin Gore-Tex patch. The skin edges were reclosed. Subsequently the child‘s condition steadily improved, and by the ninth postoperative day he had stable hemodynamics with satisfactory lung fields radiologically (Fig 2) and a peak lung inflation pressure of 21 mm Hg. One day later hemodynamic collapse occurred with a clinical picture of sepsis (fever, low blood pressure, dilated periphery, rising white blood cell count [lo to 32 X 109/L]and falling platelet count). Use of antibiotics and increased inotropes led to some clinical improvement, but the general course was downhill with death on the twelfth postoperative day. Autopsy confirmed the diagnosis with an associated moderately hypoplastic left ventricle and mitral valve. 0003-4975/92/$3.50
CASE REPORT WAITERSON ET AL COMPLETE THORACIC ECTOPIA CORDIS
Ann Thorac Surg 1992;53:14&7
147
B Fig 2 . Plain chest roentgenograms taken (A) immediately and ( B ) 9 days postoperatively.
Fig 1. Patient after resuscitation.
Comment Survival after correction has been documented in the incomplete form of ectopia cordis [ 1 4 ] and also in the total thoracic form with no intracardiac defects [5] but not when the combination [6] exists. We reasoned that the best chance for survival would be immediate correction after birth. Sterility was maximized by using cesarean section in an adjacent operating room and covering the heart in sterile plastic immediately after birth. Sterile direct echocardiography was of use for preoperative planning. The intracardiac repair was performed in the usual manner under profound hypothermia and circulatory arrest. Expansion of the thoracic cavity proved to be technically feasible but depended on (1) widening the lateral and anteroposterior diameters by a rib slide maneuver as well as rib transection and detachment of the diaphragm from the inferior cartilaginous bar and (2) lengthening the cavity by lowering the diaphragm. The external orientation of the heart at right angles to the chest wall in our patient predisposed it to flow obstruction with
minimal interference and the technical impossibility of in situ skin cover; thus placement back into the thoracic cavity was mandatory. Cardiopulmonary bypass was needed for this, and hence total internal cardiac repair was done at the same time. Although our patient did not survive, death was due to sepsis (with no focus being located in the thoracic cavity) rather than directly related to the repair itself.
References 1. Cantrell JR, Haller JA, Ravitch Mh4. A syndrome of congenital
2. 3.
4.
5. 6.
defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107602-14. Jones AF, McGrath RL, Edwards SM, Lilly JR. Immediate operation for ectopia cordis. Ann Thorac Surg 1979;28:484-6. Crittenden IH, Adams FH, Mulder DG. A syndrome featuring defects of the heart, sternum, diaphragm and anterior abdominal wall. Circulation 1959;20:396404. Tachibana H, Gan K, Oshima Y, Ohashi H, Hosokawa Y, Yamaguchi M. Thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. J Jpn Assoc Thorac Surg 3 7148-53. Dobell ARC, Williams AHB, Long RW. Staged repair of ectopia cordis. J Pediatr Surg 1982;17353-8. Poulias GE, Raftopoulos J, Polernis L, Skoutas B, Katsouli R. Total thoracic ectopia cordis with complete absence of sternum and pericardium and double diverticulum. J Cardiovasc Surg 1982;23:75!3.
