Ann Thorac Surg 2012;93:e101-e103. doi:10.1016/j.athoracsur.2011.11.014
© 2012 The Society of Thoracic Surgeons
Case Reports
Alternate Method for Hybrid Fontan Completion
Ramón Arreola-Torres, MDa,
Alberto García-y Otero, MDb,
Alberto Valencia-Castellanos, MDc,
Eliseo Portilla-de Buen, CCP, DSca,
Fernando Carrillo-Llamas, MDd,*
a Surgical Research Division, Centro de Investigación Biomédica de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Mexico
b Pediatric Cardiology and Interventionism, Hospital Puerta de Hierro, Zapopan, Mexico
c Pediatrics Department, Hospital Real San José, Guadalajara, Mexico
d Cardiosurgery Department, UMAE Hospital de Especialidades, CMNO, Instituto Mexicano del Seguro Social, Guadalajara, Mexico
Accepted for publication November 7, 2011.
* Address correspondence to Dr Carrillo-Llamas, Av Vallarta 1670, Guadalajara, Jal. 44130, Mexico (Email: drfdocarl{at}hotmail.com).
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Abstract
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A Fontan completion with a hybrid approach was performed on a 27-month-old girl with a univentricular heart. A large covered stent was placed between the inferior vena cava and the cavopulmonary anastomosis through a pericardial patch in the intracardiac fenestrated tunnel, circumventing the need for an occluder device for baffle closure. The child's progress has been good and she displays normal growth and acceptable clinical, ultrasonographic, and laboratory results.
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Introduction
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Multiple staged surgical procedures are usually required in preparation for a Fontan repair in complex congenital heart diseases with a univentricular physiologic type. To reduce the number of surgical procedures required, several hybrid approaches have been developed by combining interventional strategies [1–5], with reportedly positive results. A simple alternative hybrid approach to Fontan completion, with very satisfactory results, is presented.
An 8-month-old cyanotic girl with a univentricular heart, body weight of 5.6 kg, 76% systemic oxygen saturation, mean pulmonary artery pressure of 40 mm Hg, and positive vascular reactivity to oxygen was treated surgically with a left thoracotomy and pulmonary banding for flow reduction and the creation of a systemic pulmonary shunt to elevate her pulmonary oxygen saturation. At 16 months of age, a hyperfunctional shunt with cardiomegaly was identified and was closed with a 6-mm Amplatzer vascular plug (AGA Medical, Golden Valley, MN), reducing her pulmonary arterial pressures to 25/10 mm Hg (Fig 1). Three months later, the patient had 78% arterial oxygen saturation, with systemic arterial pressure of 85/52 mm Hg and pulmonary artery pressure of 18/8 mm Hg. A bidirectional cavopulmonary anastomosis was performed, and a lateral intraatrial tunnel was created using two thirds of the circumference of a 16-mm polytetrafluoroethylene elastic graft, with a pericardial patch 1 cm below the anastomosis. A 14-mm fenestration, larger than the diameter of the inferior vena cava, was created in its middle portion, as described previously [3]. This was followed by closure of the pulmonary artery trunk at the level of the previous banding. Cardiopulmonary bypass lasted 172 minutes, with 63 minutes of aortic clamping. The surgical procedure was uneventful, and the patient retained sinus rhythm and satisfactory cardiac output. Early extubation was performed. Mild facial edema developed 2 days after surgery. A hemodynamic study showed minimal residual permeability through the trunk of the pulmonary artery, and infundibular reactivity was suspected. The trunk was then completely surgically occluded with transfixion silk sutures.
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Fig 1. Preoperative images. (A) Rudimentary left ventricle (black arrow) and an Amplatzer vascular plug closing the systemic-pulmonary shunt (white arrow). (B) Systemic ventricle.
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At 27 months of age, the patient weighed 10 kg. A new catheterization was performed (Fig 2
A). A Mullins transseptal needle (Medtronic, Inc, Minneapolis, MN) was inserted through the right internal jugular vein and used to perforate the pericardial patch. This was followed by dilation through a right femoral vein. No contrast material was detected on the inferior side of the pericardial patch extending 1 cm into the lateral intracardiac tunnel (Fig 2B). This was compatible with the possible presence of fibrosis or a thrombus, and the risk of a thromboembolic event was considered. Length calculations indicated that the use of a short covered stent, together with an occluder device, was not feasible. With these considerations in mind, a large covered stent (Advanta V12, Atrium Medical Corp, Hudson, NH) was implanted percutaneously between the cavopulmonary anastomosis and the inferior vena cava (Fig 2, C and D). The approach was made through the right femoral and jugular veins, with no complications. The informed consent of the patient's parent was obtained before implantation.
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Fig 2. Fontan completion. (A) Pulmonary circulation after hemi-Fontan procedure. (B) Lack of contrast filling in the upper portion of the intracardiac tunnel (black arrow). Fibrosis or thrombus formation in this location was suspected. Permeable fenestration (white arrow). (C) Insertion of an Advanta V12 stent connecting the cavopulmonary anastomosis and inferior vena cava, passing through the pericardial patch, and closing the fenestration. (D) Fontan procedure completed.
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The course after intervention included no adverse events (pleural effusions, arrhythmias, or thromboembolism) and the patient had systemic and pulmonary pressures of 92/57 and 19/9 mm Hg, respectively, with 96% systemic blood oxygen saturation.
After 5 months' follow-up, the patient's progress is good, with adequate growth and no hepatomegaly or thromboembolic events. Her medications include full oral anticoagulation with aspirin (10 mg/kg per 24 h orally [PO]) and acenocoumarol (1 mg per 48 h PO to achieve an international normalized ratio of 2–3), sildenafil (1 mg/kg per 8 h PO), and enalapril (0.1 mg/kg per 24 h PO). Her abdominal ultrasonogram and liver function test results have been unremarkable.
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Comment
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The case presented here demonstrates the feasibility of using a single device for hybrid Fontan completion, avoiding the use of an ocludder device for baffle closure. Different hybrid strategies have been reported for the management of patients with univentricular heart, and shorter recovery periods, less morbidity, early hemodynamic improvement, and better general echocardiographic and clinical progress have been seen [1–3]. We believe our technique contributes a shorter intervention time and a more economical approach to the Fontan completion phase. Although this is not a clinical series, the fact that none of the complications usually associated with Fontan procedures was observed in our patient is compelling.
We consider that the possibility of an embolic event due to the probable presence of a thrombus or fibrosis in the intracardiac tunnel was reduced by pressing it with the covered stent wall against the polytetrafluorethylene graft and simultaneously closing the fenestration.
Another source of concern has been the risk of compromised hepatic drainage caused by the presence of the device in the suprahepatic vena cava [6], particularly during the initial period after implantation. So far no liver congestion has been documented clinically, ultrasonographically or based on liver function test results in our patient, and we hope that her normal growth will reduce this risk.
An advantage of the device we used is the possibility of further balloon dilation (up to 22 mm) to adjust for the patient's growth, preferably without the need for additional surgical intervention. In our patient, the stent ends extend slightly into the right pulmonary artery and inferior vena cava. We expect that the ability of the device to shorten up to 30% with total dilation will allow final adjustments for size and termination of anticoagulation.
The investigation of other factors should be considered when this approach is used in more patients, including the associated general morbidity, length of hospital stay, cost saving, and long-term function. The good results achieved with hybrid approaches have led to the development of devices designed specifically for the resolution of this kind of pathologic condition [7] and have so far been evaluated in a sheep model pending clinical validation. With further similar technologic advances, we agree that the hybrid approach will be clinically accepted in the near future, as has been earlier proposed [8], and may become the gold standard technique for the management of complex heart diseases.
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Acknowledgments
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We thank the surgical and interventional groups for their support.
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References
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- Hausdorf G, Schneider M, Konertz W. Surgical preconditioning and completion of total cavopulmonary connection by interventional cardiac catheterisation: a new concept Heart 1996;75:403-409.[Abstract/Free Full Text]
- Alsoufi B, Alfadley F, Al-Omrani A, et al. Hybrid management strategy for percutaneous Fontan completion without surgery: early results Ann Thorac Surg 2011;91:566-572.[Abstract/Free Full Text]
- Sallehuddin A, Mesned A, Barakati M, Fayyadh MA, Fadley F, Al-Halees Z. Fontan completion without surgery Eur J Cardiothorac Surg 2007;32:195-200.[Abstract/Free Full Text]
- Sidiropoulos A, Ritter J, Schneider M, Konertz W. Fontan modification for subsequent non-surgical Fontan completion Eur J Cardiothorac Surg 1998;13:509-512.[Abstract/Free Full Text]
- Konertz W, Schneider M, Herwig V, Kampmann C, Waldenberger F, Hausdorf G. Modified hemi-Fontan operation and subsequent nonsurgical Fontan completion J Thorac Cardiovasc Surg 1995;110:865-867.[Free Full Text]
- Konstantinov IE, Benson LN, Caldarone CA, et al. A simple surgical technique for interventional transcatheter completion of the total cavopulmonary connection J Thorac Cardiovasc Surg 2005;129:210-212.[Free Full Text]
- Metton O, Calvaruso D, Stos B, Ben AW, Boudjemline Y. A new surgical technique for transcatheter Fontan completion Eur J Cardiothorac Surg 2011;39:81-85.[Abstract/Free Full Text]
- Konstantinov IE, Alexi-Meskishvili VV. Intracardiac covered stent for transcatheter completion of the total cavopulmonary connection: anatomical, physiological and technical considerations Scand Cardiovasc J 2006;40:71-75.[Medline]
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Abstract
Introduction
