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Ann Thorac Surg 2005;79:1042-1044
© 2005 The Society of Thoracic Surgeons

Case report

Successful Single-Lung Fontan Operation in an Adult: 5-Year Follow-Up

Abdulaziz Al-Khaldi, MDa, Edgar G. Chedrawy, MDa, Christo I. Tchervenkov, MDa,*, Dominique Shum-Tim, MDa

a Division of Cardiovascular Surgery, The Montreal Children's Hospital, McGill University Health Center, Montreal, Quebec, Canada

Accepted for publication September 8, 2003.

* Address reprint requests to Dr Tchervenkov, Division of Cardiovascular Surgery, Room C-829, The Montreal Children's Hospital, 2300 Tupper St, Montreal, QC H3H 1P3, Canada
christo.tchervenkov{at}muhc.mcgill.ca


   Abstract
Top
 Abstract
Introduction
 Comment
 References
 
We report a case of a 20-year-old man with a functional single left ventricle who underwent a successful Fontan operation into a single left pulmonary artery, the right pulmonary artery being atretic. The literature of a single-lung Fontan procedure is also reviewed.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
The Fontan operation is considered a major advance in the palliation of patients with a functional single ventricle. The flow of systemic venous blood directly through the lungs in the absence of active pump is highly dependent on the status of the pulmonary vascular bed. The performance of a Fontan operation in a patient with a single lung is a rare and high-risk situation because of the major loss in the pulmonary vascular bed. Significant alterations of the pulmonary vascular bed are generally considered relative contraindications for the Fontan operation.

The male patient was born in November 1977 with a diagnosis of situs inversus, dextrocardia, single left ventricle, common atrium, and a single atrioventricular (AV) valve. A restrictive bulboventricular foramen leading into a subpulmonary outlet chamber caused reduced pulmonary blood flow. The systemic venous drainage consisted of the left superior vena cava to the left-sided right atrium, the separate return of the inferior vena cava, and the hepatic veins entered the inferior surface of the common atrium.

Because of progressive cyanosis, he underwent a right Blalock-Taussig shunt in November 1978. Persistent cyanosis required the shunt to be revised 1 month later. Massive intraoperative bleeding during the procedure could only be controlled by ligation of the right pulmonary artery. In March 1980, at 2 years of age, a left classic Blalock-Taussig shunt was done (Fig 1A) because of progressive cyanosis and polycythemia. In December 1981, he underwent an enlargement of the bulboventricular foramen to further increase blood flow to the left lung. The Fontan operation was not considered at that time because the right pulmonary artery could not be visualized.



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  		Fig 1. (A) Schematic representation of pre-Fontan procedure anatomic findings (the right pulmonary artery is not visualized). Note the restrictive bulboventricular foramen and the separate drainage of hepatic veins. (B) Ligation of the MPA, and Blalock shunt and anastomosis of the superior and inferior SVC stump to the LPA. A Gor-Tex graft was used to divert blood from the IVC and HV to the SVC and then to the LPA. (Ao = aorta; HV = hepatic vein; IVC = inferior vena cava; LPA = left pulmonary artery; MPA = main pulmonary artery; RPA = right pulmonary artery; SVC = superior vena cava.)

 
The patient remained stable for many years. His case was rediscussed from time to time, and a Fontan operation into a single lung was thought to be prohibitively risky. At the age of 18 years, he was again investigated because of increasing fatigue on exercise. Cardiac catheterization showed a left ventricular end-diastolic pressure of 10 mm Hg and a peak gradient across the bulboventricular foramen of 93 mm Hg. The systemic oxygen saturation (SaO2) was 90%. The mean left pulmonary artery pressure was 17 mm Hg, and the mean main pulmonary artery pressure was 23 mm Hg. Neither cardiac catheterization nor magnetic resonance imaging visualized the right pulmonary artery. The calculated Nakata index was 159 mm2/m2. The high SaO2 with the slightly elevated pulmonary artery pressure suggested a low pulmonary vascular resistance and that he might be a candidate for a single-lung Fontan operation, particularly because of a good-sized left pulmonary artery feeding the predominant left lung as a result of the dextrocardia.

In 1997, at the age of 20 years, he underwent a modified Fontan operation (Fig 1B). The main pulmonary artery was ligated twice before its transition into the left pulmonary artery. A left bidirectional Glenn anastomosis was performed by dividing the left-sided superior vena cava 2 cm above the junction with the atrium and anastomosing the cephalic end to the superior surface of left pulmonary artery. Although an extracardiac Fontan is our preferred approach, it was not possible to do an extracardiac repair because of the wide separation of the orifices of the inferior vena cava and the hepatic veins. Therefore, we decided to proceed with intracardiac repair. The atrial end of the divided superior vena cava stump was anastomosed to the inferior surface of the left pulmonary artery. A 22-mm Gore-Tex (WL Gore & Assoc, Flagstaff, AZ) tube was sutured to the inside surface of the right atrium around the cavopulmonary anastomosis. Inferiorly, it was sutured around the hepatic venous orifice. Since the inferior vena cava was too far to be included in this anastomosis, a separate Gore-Tex patch was used to include the inferior vena cava drainage and carry it to an opening on the lateral surface of the tube graft. A 4-mm fenestration was made in the Gore-Tex tube. The left Blalock shunt was ligated.

The patient tolerated the procedure well. He was extubated the next morning and had an uneventful postoperative course. Oxygen saturation measured by oximetry was 90% on room air. He was discharged home 22 days postoperative. Five years later, he leads a fairly normal life with mild fatigue on exertion (New York Heart Association class I to II) on a drug regimen of digoxin, enalapril (Vasotec), metoprolol (for paroxysmal sinus tachycardia), and warfarin (Coumadin). His most recent hemoglobin level is 15.4 g/L, and he has a systemic arterial saturation of 96% on room air.


   Comment
Top
 Abstract
 Introduction
 Comment
References
 
The status of the pulmonary circulation is of the utmost importance to the success of the Fontan operation in patients with a functional single ventricle. Among the risk factors, pulmonary artery distortion, elevated pulmonary artery pressure ( > 15 mm Hg), elevated pulmonary vascular resistance ( > 4 Wood units), significant AV valve incompetence, heterotaxy, systemic obstruction, and ventricular dysfunction (both systolic and diastolic) have all been shown to be predictors of early or late failure of Fontan operations [1–3].

Pulmonary artery distortion is a well-recognized risk factor for the early failure of a Fontan operation that carries with it an ominous prognosis [2, 3]. The ultimate pulmonary artery distortion is the complete irreversible loss of one pulmonary artery. In our patient, the right pulmonary artery was lost as a complication of a failed Blalock shunt. This left the patient with a single left pulmonary artery.

Single-lung Fontan operations have been reported in the literature. The first involved a patient with a double-inlet left ventricle with subpulmonic stenosis and a severely hypoplastic left pulmonary artery who underwent a Fontan operation at 10 years of age. A Dacron (DuPont, Wilmington, DE) conduit was used between the right atrium and right pulmonary artery, and the right AV valve and patent foramen ovale were closed with Dacron patches [4]. Follow-up 9 years postoperatively showed the patient was doing well clinically and hemodynamically. A recent case series by Zachary and colleagues [5] reported encouraging surgical results of Fontan operations performed in patients with one lung. The only difference was noted in the postoperative arterial oxygen saturation compared with the two-lung control group (87% vs 91%).

The complete loss of one pulmonary artery represents an almost 50% reduction in the cross-sectional area of pulmonary vasculature, leading not only to an anatomic distortion but also to a physiologic deficit. The pulmonary artery index (pulmonary artery cross-sectional area indexed to the body surface area) has been shown to predict early failure and hospital death in Fontan patients [6]. The observed success of the Fontan procedure in selected cases of single-lung physiology is likely related to the well-described functional and anatomic compensation by the remaining lung. Preclinical studies showed that pulmonary vascular resistance decreases significantly in the remaining lung postpneumonectomy over time (2 to 12 months) with an increase in maximal cardiac output [7]. The degree of pulmonary functional compensation postpneumonectomy is greater in young animals than in adults, suggesting a relationship between developmental and adaptive potentials [8].

The increased risk of performing a Fontan operation in patients with a functional single ventricle and a single lung requires that careful preoperative assessment must be done to determine whether these patients are acceptable surgical candidates. A single, functional pulmonary artery should not, in itself, be a contraindication to a successful Fontan operation.


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Mayer JE, Helgason H, Jonas RA, et al. Extending the limits for modified Fontan procedures. J Thorac Cardiovasc Surg. 1985;92:1021–1028
  2. Gentles TL, Mayer JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg. 1997;114:376–391[Abstract/Free Full Text]
  3. Mayer JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castaneda AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg. 1992;103:444–452[Abstract]
  4. Sade RM, Gillette PC. Fontan operation in a case of single functional pulmonary artery. J Thorac Cardiovasc Surg. 1989;98:153–154[Medline]
  5. Zachary CH, Jacobs ML, Apostolopoulou S, Fogel MA. One-lung Fontan operation: hemodynamics and surgical outcome. Ann Thorac Surg. 1998;65:171–175[Abstract/Free Full Text]
  6. Knott-Craig CJ, Julsrud PR, Schaff HV, Puga FJ, Danielson GK. Pulmonary artery size and clinical outcome after the modified Fontan operation. Ann Thorac Surg. 1993;55:646–651[Abstract]
  7. Hsia CC, Herazo LF, Ramanathan M, Johnson RL Jr, Wagner PD. Cardiopulmonary adaptations to pneumonectomy in dogs. II. VA/Q relationships and microvascular recruitment. J App Physio. 1993;74:1299–1309
  8. Johnson RL Jr, Cassidy SS, Grover R, et al. Effect of pneumonectomy on the remaining lung in dogs. J App Physio. 1991;70:849–858



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Edgar G. Chedrawy
Christo I. Tchervenkov
Dominique Shum-Tim
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