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J Thorac Cardiovasc Surg 1997;113:620-621
© 1997 Mosby, Inc.

LETTERS TO THE EDITOR

Median sternotomy for prolonged resuscitation in neonatal tetralogy of Fallot with absent pulmonary valve

Margit Kadletz, MD, Robert M. Freedom, MD, FRCPC, Michael D. Black, MD, FRCSC

The Hospital for Sick Children
University of Toronto
Toronto, Ontario, Canada

To the Editor:

In a recent brief communication, van Son and MohrGo 1 discussed specific preoperative strategies designed to stabilize the condition of neonates who have tetralogy of Fallot with absent pulmonary valve syndrome.

We recently encountered a neonate with tetralogy of Fallot and absent pulmonary valve who failed to respond to the "usual" preoperative maneuvers designed specifically for the associated bronchial compression and severe respiratory distress. Preoperative nursing in the prone position and median sternotomy may be advantageous, as previously described by Heinemann and HanleyGo 2 and van Son and Mohr.Go 1 However, depending on the associated anatomic lesions, these selected maneuvers may be useless, as described below.

A female neonate (2.6 kg) was intubated 2 hours after birth because of cyanosis and respiratory insufficiency and was transferred to our institution. Because of the patient's instability a limited transthoracic echocardiogram was performed, demonstrating tetralogy of Fallot with absent pulmonary valve. A massively dilated right pulmonary artery (15 mm) and an enlarged left pulmonary artery (8 mm) were identified. Additional anomalies were a right aortic arch and an atrial septal defect.

Even after the administration of paralytics it was difficult to obtain satisfactory oxygen saturation. Importantly, the carbon dioxide tensions remained near normal. The baby was supported by continuous-mode ventilation with 100% oxygen. Prone positioning was attempted without significant improvement. Within the next 24 hours, increasingly large amounts of inotropic support were required. The patient remained critically ill with bradycardia and severe cyanosis. Repair of the cardiac malformation was considered urgent for survival and was not delayed.

Cardiopulmonary bypass was established and the lesion was repaired under low flow with moderate hypothermia. Exploration of the right atrium revealed a large inferior sinus venosus defect (posterior defect) extending deep into the inferior vena cava. An autologous pericardial patch closure was performed in addition to ventricular septal defect closure and pulmonary arterial reduction (Fig. 1). The combination of early takeoff of the massively enlarged right pulmonary artery and an anomalous left anterior descending coronary artery originating from the right coronary artery (crossing the pulmonary anulus) made insertion of a valved conduit impossible. Therefore right ventricular outflow tract reconstruction was accomplished with creation of a monocusp outflow patch. The patient was weaned successfully from cardiopulmonary bypass with an oxygen saturation of 100% on an inspired oxygen fraction of 40%.



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  		Fig. 1. Anterior view of the intraoperative repair. The right atrium has been opened. The large inferior sinus venosus defect (posterior defect) extending deep into the inferior vena cava has been closed with an autologous pericardial patch. Note the aneurysmal enlargement of the pulmonary arteries and the anomalous origin of the left anterior descending artery.

 
Decreased oxygen saturation combined with a near normal carbon dioxide tension in this infant cannot be explained by airway obstruction alone. Oxygen saturations did not improve with either prone positioning before the operation or median sternotomy in the operating room. The hypoxemia can now be explained by disadvantageous streaming at the atrial level. The high pulmonary resistance immediately after birth, augmented by impaired diastolic filling of the right ventricle as a result of free pulmonary valve insufficiency, right ventricular dilation, and decreased ventricular compliance, all increased the degree of atrial admixture.Go 3 We agree that surgical correction of this defect should not be delayed, for sometimes when one hears hoof steps it really is a zebra!

12/8/78754

References

  1. van Son JA, Mohr FW. Median sternotomy for prolonged resuscitation in neonatal tetralogy of Fallot with absent pulmonary valve. J Thorac Cardiovasc Surg 1996;112:185-6.[Free Full Text]
  2. Heinemann MK, Hanley FL. Preoperative management of neonatal tetralogy of Fallot with absent pulmonary valve syndrome. Ann Thorac Surg 1993;55:172-4.[Abstract]
  3. Lakier JB, Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve: natural history and hemodynamic consideration. Circulation 1974;50:167-75. [Abstract/Free Full Text]




This Article
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