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J Thorac Cardiovasc Surg 2005;129:1450-1451
© 2005 The American Association for Thoracic Surgery


Brief Communication

Novel management strategy for severe cyanosis after Sano modification of the Norwood procedure

Janet M. Simsic, MDa,*, Angel Cuadrado, MDa, Paul M. Kirshbom, MDb, Kirk R. Kanter, MDb, Donna Ramaswamy, MPH, MSa, Martha Clabby, MDa, Joseph M. Forbess, MDb,*

a Sibley Heart Center Cardiology/Children’s Healthcare of Atlanta, Atlanta, Ga.
b Division of Pediatric Cardiothoracic Surgery, Emory University, Atlanta, Ga.

Received for publication December 7, 2004; accepted for publication December 15, 2004.

* Address for reprints: Janet M. Simsic, MD, Sibley Heart Center Cardiology, 52 Executive Park South, Suite 5200, Atlanta, GA 30329 (Email: simsicj{at}kidsheart.com).

Sano modification of the Norwood procedure has resulted in a more favorable distribution of systemic, pulmonary, and coronary blood flow. However, frequently these patients present with severe cyanosis caused by dynamic proximal shunt stenosis. The purpose of this review was to evaluate a management strategy for severe cyanosis in this patient population.

Methods

Between September 2003 and September 2004, 22 newborns underwent Sano modification at Children’s Healthcare of Atlanta. One patient died before hospital discharge and was excluded from further analysis. Four (19%) of the 21 remaining patients presented at 53 ± 26 days of age with oxygen saturations of less than 70%. These 4 patients (group 1) were compared with the remaining 17 (group 2). Management strategy to treat cyanosis in group 1 patients consisted of increasing preload and ß-blockade. Age-matched hemodynamic data were evaluated between group 1 before therapy and group 2; variables before and after therapy in group 1 were also evaluated.

Results

Sano shunt size was 6 mm (n = 3; birth weight, >3 kg) and 5 mm (n = 1; birth weight, <2 kg; group 1) versus a shunt size of 6 mm (n = 13; 4 patients with a birth weight <3 kg; 9 patients with a birth weight >3 kg) and 5 mm (n = 4; birth weight, <3 kg; group 2). Hemodynamic variables before and after therapy in group 1 are shown in Table 1. In addition, comparison was made between hemodynamic variables at the time of presentation with cyanosis group 1 and group 2 at the same age (Table 2).


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TABLE 1. Hemodynamic variables before and after therapy in group 1
 

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TABLE 2. Hemodynamic variables at the time of presentation with cyanosis in group 1 and patients in group 2 at the same age
 
This management strategy prolonged the timing to bidirectional Glenn shunt by 70 ± 40 days in group 1. A bidirectional Glenn shunt was performed at 123 ± 32 days in group 1 (4/4 patients) versus 133 ± 28 days in group 2 (11/13 patients, P = .6).

Discussion

Secondary to the variable pulmonary and systemic vascular resistance ratio, the net effect of the Norwood procedure with a systemic-pulmonary artery shunt is runoff from the systemic to the pulmonary bed during diastole, resulting in a low diastolic blood pressure, a wider pulse pressure, and a change in the pattern of coronary perfusion.1 Sano modification, substituting a right ventricle-pulmonary artery shunt in place of the systemic-pulmonary artery shunt, provides a more favorable distribution of systemic, pulmonary, and coronary blood flow. Several studies have reported a hemodynamic profile with higher diastolic pressure,2,3 narrowed pulse pressure,2,3 and higher coronary perfusion pressure2 after a right ventricle-pulmonary artery shunt compared with that after a systemic-pulmonary artery shunt.

In our recent experience, 4 (19%) of 21 patients after Sano modification presented at 53 ± 26 days of age with systemic oxygen saturation of less than 70% caused by dynamic proximal shunt stenosis. The proximal shunt Doppler gradient determined by transthoracic echocardiography was 92 ± 12 mm Hg. The gradient was due to dynamic obstruction below the ventricular anastomosis site of the right ventricle-pulmonary artery shunt. We believe that this mechanism of significant cyanosis after the Sano modification is similar to that of hypercyanotic episodes in tetralogy of Fallot: dynamic outflow obstruction. Therefore, we adopted a management strategy of increased preload and ß-blockade similar to the treatment strategy for the hypercyanotic episode in patients with tetralogy of Fallot.

Increasing preload was achieved by volume bolus (3 patients) and decreasing diuretic therapy (4 patients). ß-Blockade therapy was with propranolol (1 mg · kg–1 · d–1) in 3 patients and esmolol in 1 patient. This patient was converted to sotalol instead of propranolol because of atrial dysrhythmias not responsive to propranolol. This combination therapy resulted in a 22% increase in systemic oxygen saturation (from 62% ± 9% to 80% ± 3%), with a 15% decrease in heart rate (from 151 ± 13 to 129 ± 9 beats/min).

Increased preload in these single-ventricle patients results in increased systemic resistance, allowing more blood flow into the pulmonary circuit. Increased cardiac output and pulmonary blood flow leads to increased systemic oxygen saturations. ß-Blocker therapy has been shown to increase systemic saturation in patients with tetralogy of Fallot through a variety of complex mechanisms, including increases in peripheral resistance,4 prevention of tachycardia, and shift in the hemoglobin-oxygen dissociation curve.5 Increased peripheral resistance again allows for increased pulmonary blood flow in this single-ventricle patient. A slower heart rate allows for increased ventricular filling time and thus increased preload. Shifting the hemoglobin-oxygen dissociation curve results in increased availability of oxygen delivery to the tissues. The overall result is improvement in systemic oxygen saturations.

Conclusion

The medical management strategy of increasing preload and initiating ß-blockade therapy is effective in the treatment of severe cyanosis caused by dynamic proximal shunt stenosis after Sano modification of the Norwood procedure.

Footnotes

* Present address: University of Texas Southwestern Medical Center, Dallas, Tex. Back

References

  1. Vogel MA, Rychik J, Vetter J, Donofrio MT, Jacobs M. Effect of volume unloading surgery on coronary flow dynamics in patients with aortic atresia. J Thorac Cardiovasc Surg 1997;113:718-727.[Abstract/Free Full Text]
  2. Maher KO, Pizarro C, Gidding SS, Januszewska K, Malec E, Norwood Jr WI, et al. Hemodynamic profile after the Norwood procedure with right ventricle to pulmonary artery conduit. Circulation 2003;108:782-784.[Abstract/Free Full Text]
  3. Bradley SM, Simsic JM, McQuinn TC, Habib DM, Shirali GS, Atz AM. Hemodynamic status following the Norwood procedure. a comparison of right ventricle to pulmonary artery versus modified Blalock-Taussig shunt. Ann Thorac Surg 2004;78:933-941.[Abstract/Free Full Text]
  4. Honey M, Chamberlain DA, Howard J. The effect of beta-sympathetic blockade on arterial oxygen saturation in Fallot’s tetralogy. Circulation 1964;30:501-506.[Abstract/Free Full Text]
  5. Oski FA, Miller LD, Dellvoria-Papadopoulos M, Manchester JH, Shelburn JC. Oxygen affinity in red cells. changes induced in vivo by propranolol. Science 1972;175:1372-1373.[Abstract/Free Full Text]



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