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Innovations: Technology & Techniques in Cardiothoracic & Vascular Surgery:
doi: 10.1097/IMI.0b013e3182810e08
Case Reports

Extracardiac Off-Pump Fontan Completion for Single Ventricle With Dextrocardia

Goksel, Onur S. MD; Gok, Emre MD; Tireli, Emin MD; Dayioglu, Enver MD

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Author Information

From the Department of Cardiovascular Surgery, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey.

Accepted for publication November 22, 2012.

Presented at the Annual Scientific Meeting of the International Society for Minimally Invasive Cardiothoracic Surgery, May 30–June 2, 2012, Los Angeles, CA USA.

Disclosure: The authors declare no conflicts of interest.

Address correspondence and reprint requests to Onur S. Goksel, MD, Department of Cardiovascular Surgery, Istanbul University, Istanbul Medical Faculty, 34093 CAPA, Aksaray, Istanbul, Turkey. E-mail: onurgokseljet@gmail.com.

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Abstract

Abstract: Extracardiac off-pump is reported to result in better early hemodynamics and shorter mechanical ventilation periods. We present a case report of extracardiac off-pump Fontan in a 5-year-old girl with single ventricle and dextrocardia.

Extracardiac conduit Fontan procedure (ECFP) has been performed with increasing frequency in patients with functional single ventricle as a result of optimal midterm results and reduced incidence of early and late arrhythmias.

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CASE REPORT

The advantages of ECFP, including optimal laminar flow in the systemic venous pathway, avoidance of myocardial ischemia, atriotomy, and intra-atrial suture lines, as well as the simplicity of the surgical technique, could contribute to a low incidence of atrial arrhythmia and better ventricular function.1 However, anatomical considerations with regard to dextrocardia as well as the need for resternotomy may hinder a satisfactory adjustment of the extracardiac conduit. We present our surgical technique of extracardiac off-pump Fontan in a 5-year-old girl with single ventricle and dextrocardia.

After resternotomy, all mediastinal structures, including previous bidirectional Glenn anastomosis (Figs. 1A, B), bilateral pulmonary arteries (PAs), and inferior vena cava (IVC) as inferior as hepatic veins, are extensively freed of adhesions from previous operation. Dissection of the midline-situated IVC was necessary from cavoatrial junction as far as the hepatic veins, which was possible by tilting the heart upward with care not to disturb optimal filling and by pulling the diaphragm. A left-sided ECFP was anticipated for the shortest distance from the IVC to the PA branch due to dextrocardia (Figs. 1A and 2A). A side-biting clamp was applied on the left PA as the right-sided Glenn anastomosis continued perfusing the lungs. After a longitudinal arteriotomy, the proximal end of the 20-mm Gore-Tex graft (W.L. Gore & Associates Inc, Flagstaff, AZ USA) was anastomosed to the left PA with continuous suture technique. For the distal anastomosis of the graft to the IVC, a Y-shaped passive venous decompression cannula system was used as described previously.2 The IVC is cannulated as distally as possible for the one arm of the Y-shaped system and the atrium is cannulated for the other so that the IVC drainage continues during anastomosis to avoid cardiopulmonary bypass and cardiac arrest. The IVC was then separated from the cavoatrial junction, except for the posterior one-third circumference. This technical maneuver is critical for correct configuration and diameter of the lower anastomosis because the IVC wall has a great tendency to shrink and rotate.

Figure 1
Figure 1
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Figure 2
Figure 2
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The patient was tilted in the Trendelenburg position to promote IVC decompression. Distal venous pressure is allowed to be 25 to 30 mm Hg during passive drainage. After completion of the distal anastomosis with continuous technique and the decannulation, PA pressure was 17 mm Hg, and a 5-mm direct fenestration was constructed between the conduit and the left-sided right atrium. The final saturation of the patient was 99% at 50% of ventilated oxygen.

The patient was extubated within 12 hours. After 2 days of intensive care unit stay, she had 15 mm Hg of central venous pressure, base excess of −2.4, and oxygen saturation of 87% in room air. Duration of chest tube drainage was 3 days, and she was discharged on postoperative day 9 with 85% of oxygen saturation. There were no early or late arrhythmias. On the 3-year follow-up, she had a mean PA pressure of 16 mm Hg and was free of arrhythmia, thromboembolic complications, and systemic ventricular dysfunction.

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DISCUSSION

The advantages of ECFP, including optimal laminar flow in the systemic venous pathway, avoidance of myocardial ischemia, atriotomy, and intra-atrial suture lines, as well as the simplicity of the surgical technique, could contribute to a low incidence of atrial arrhythmia and better ventricular function. Surgical management of congenital heart pathologies with single ventricle is composed of a series of palliative operations culminating in the Fontan procedure.3 Patients with heterotaxy syndromes constitute a very difficult group of patients, particularly those with right isomerism. The latter group represents one of the worst forms of contemporary heart disease, with an overall 5-year survival of 30% to 74%.4 These patients are more likely to receive a single ventricle repair and a right ventricle morphology as the systemic ventricle further complicates the outcome.3–5

The anatomical features of the patient, in addition to adhesions from previous sternotomy, made the procedure more complicated. However, the presence of a midline-situated IVC enabled the authors to prefer a practical approach to create a left-sided ECFP while the right-sided Glenn perfused the lungs and provided optimal oxygenation and hemodynamics.

For extracardiac Fontan, correct orientation of the conduit is a major determinant of the outcome; an intact posterior caval wall during inferior anastomosis is the cornerstone. The benefit of performing this surgical step with or without the use of cardiopulmonary bypass is still controversial. The Fontan procedure can be performed with no significant differences in operative mortality, morbidity, or use of resources, with or without cardiopulmonary bypass support. Long-term survival after this procedure is high with both strategies. Mortality in the modern era is rare, whereas postoperative pleural drainage remains the dominant morbidity. The extracardiac off-pump Fontan operation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, minimal early and late rhythm disturbance, and less postoperative pleural effusion.6,7

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CONCLUSIONS

Patients with heterotaxy syndromes constitute a very difficult group of patients, particularly those with right isomerism. The ECFP technique, in addition to the well-known advantages mentioned above, allowed a practical and quicker approach for Fontan completion as venous cannulation and control of the Glenn anastomosis would have otherwise been cumbersome for the surgeon. This technique of ECFP for Fontan completion in patients with dextrocardia is an acceptable option in selected patients.

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REFERENCES

1. Ochiai Y, Imoto Y, Sakamoto M, et al.. Mid-term follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure. Eur J Cardiothorac Surg. 2009; 36: 63–68.

2. Tireli E, Ugurlucan M, Basaran M, et al.. Extracardiac Fontan operation without cardiopulmonary bypass. J Cardiovasc Surg (Torino). 2006; 47: 699–704.

3. McGuirk SP, Winlaw DS, Langley SM, et al.. The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection. Eur J Cardiothorac Surg. 2003; 24: 37–46.

4. Anagnostopoulos PV, Pearl JM, Octave C, et al.. Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg. 2009; 35: 871–877.

5. Lim JS, McCrindle BW, Smallhorn JF, et al.. Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes. Circulation. 2005; 112: 2454–2461.

6. LaPar DJ, Mery CM, Peeler BB, Kron IL, Gangemi JJ. Short and long-term outcomes for bidirectional Glenn procedure performed with and without cardiopulmonary bypass. Ann Thorac Surg. 2012; 94: 164–170.

7. Shikata F, Yagihara T, Kagisaki K, et al.. Does the off-pump Fontan procedure ameliorate the volume and duration of pleural and peritoneal effusions? Eur J Cardiothorac Surg. 2008; 34: 570–575.

Keywords:

Off-pump Fontan; Single ventricle; Dextrocardia

Copyright © 2013 by the International Society for Minimally Invasive Cardiothoracic Surgery. Unauthorized reproduction of this article is prohibited.

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