Ann Thorac Surg 2001;71:725-727
© 2001 The Society of Thoracic Surgeons
Case report
Cavopulmonary connection after repair of pulmonary vein stenoses
Thierry Bove, MDa,
Hélène Demanet, MDa,
Hughes Dessy, MDa,
Pierre Viart, MDa,
Frank E. Deuvaert, MDa
a Department of Cardiac Surgery and Pediatric Cardiology, Hôpital Universitaire des Enfants Reine Fabiola, Free University of Brussels, Brussels, Belgium
Accepted for publication March 2, 2000.
Address reprint requests to Dr Bove, Department of Cardiac Surgery, A.Z.-V.U.B Laarbeeklaan 101, 1090 Brussels, Belgium
e-mail: thierry.bove{at}az.vub.ac.be
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Abstract
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We report a case of heterotaxia with single-ventricle physiology undergoing successively pulmonary artery banding (PAB) at 8 months of age, repair of pulmonary vein (PV) stenoses at 15 months of age, and a fenestrated total cavopulmonary connection (TCPC) combined with repair of a regurgitant common atrioventricular (AV) valve and a Damus-Stansel operation for a restrictive subaortic ventricle at 5 years of age, followed by percutaneous closure of the fenestration. The child is now 10 years old and is, to the best of our knowledge, the only reported long-term survivor of a Fontan type procedure done after repair of PV stenoses.
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Introduction
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Heterotaxia is characterized by failure of right-left differentiation, leading to ambiguities of the visceral and atrial situs and anomalies of the systemic or pulmonary venous pathways. Since there is often a functional single ventricle, definitive surgical repair will be directed to a Fontan type procedure.
Over the past years, increasing experience and several technical modifications have significantly extended the limits for application of the Fontan principle. Mayer and associates [1] have clearly demonstrated that the presence of anomalies of the systemic or pulmonary venous connection are no longer absolute contra indication for TCPC. However, the outcome of the modified Fontan procedure can still be hazardous in complex single ventricle hearts with heterotaxia variants, especially when anatomic features are detrimental to the pulmonary hemodynamics. To the best of our knowledge, this is the first case reported of a successful TCPC for a complex univentricular heart in association with PV stenoses.
The patient, a girl, was born in March 1990 from inbred Turkish parents, in a neighboring institution. Because her cardiac disease was initially thought to be incurable, she was transferred to our unit for eventual further management. Actually she had a complex cardiopathy with dextrocardia and heterotaxia syndrome, presenting a large atrioventricular (AV) septal defect with a double inlet left-sided ventricle of left morphology, a common moderately incompetent AV valve that was straddling a rudimentery rightsided ventricle of right morphology. The ventriculo-arterial connection was discordant while both arterial outlets were unobstructed. Completing anatomic features of heterotaxia suggesting mainly right isomerism were asplenia, a median liver, bilateral short eparterial bronchi, and an left-sided uninterrupted inferior vena cava.
At 6 months of age she underwent a pulmonary artery banding (PAB), resulting in systemic arterial pressure increase (57/38/46 to 74/48/59 mm Hg) and pulmonary artery pressure decrease (53/30/42 to 38/21/38 mm Hg). Persisting pulmonary congestion 6 months after adequate PAB led to a new catheterization, disclosing a long segment atresia of the right inferior PV and stenoses of the right superior, left superior, and left inferior PV (Fig 1). Therefore, at the age of 15 months, a second correction consisted of the repair of the three connected stenosed PV by a VY-plasty in two and an autologuous pericardial patch plasty in one vein, performed by sternotomy and using deep hypothermic circulatory arrest. The fourth atretic right inferior PV could not be reconnected. However, this procedure allowed the confirmation of the unusual atrial anatomy: a single morphologically right appendage and a left-sided morphologically right atrium receiving, respectively, all three anomalously connected PV, the leftsided SVC and IVC, the coronary sinus, and a suprahepatic venous trunk, draining separately from the IVC.
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Fig 1. Angiogram of the pulmonary venous drainage showing the stenosis of the right superior, left superior and left inferior PV, and the atretic connection of the right inferior pulmonary vein.
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At the age of 5 years, increasing cyanosis developed (SaO2 70% to 75%) and the child was reevaluated for the feasibility of a Fontan type procedure. Echocardiography and catheterization revealed satisfactory pulmonary artery pressure (20/11/17 mm Hg) and a pulmonary wedge pressure of 12 mm Hg in the presence of moderate common AV valve regurgitation. The drainage of the three connected PV was unobstructed. By redo sternotomy, a left bidirectional Glenn anastomosis was constructed. A patch of the atrial floor around the ostia of the left-sided inferior vena cava and suprahepatic venous trunk was connected to the obliquely cut arch end of an adult-sized cryopreserved descending thoracic aorta homograft. The other end of the homograft was sewn end to end to the mobilized leftsided and posterior distal pulmonary trunk after resection of the banded segment. The incompetent common AV valve was repaired by plication of the annulus with a PDS suture around a 22-mm Hegar dilator (Pilling-Weck Inc, Mariakerke, Belgium).
Because of the known potential for restriction by a small subaortic ventricle, a modified Damus-Stansel systemic arterial outlet was performed. Finally, a side-to-side 4-mm fenestration was created between the extra-atrial homograft and the pulmonary venous atrium. The early postoperative course was characterized by prolonged mechanical ventilation (20 days) and temporary NO inhalation therapy because of pulmonary and systemic venous hypertension, aggravated by a pulmonary infection. After 6 weeks, the child left the hospital with a SaO2 of 85% to 90%. However, the saturation progressively declined to 70%. In March 1996 a balloon occlusion test of the fenestration was well tolerated and raised the SaO2 to 95%. Definitive closure was obtained by launching an inverted Sideris device (Custom Medical Devices, Athens, Greece).
The child is now a 10-year-old active fourth grader. She receives only amiodarone and digoxin to control paroxysmal supraventricular arrhythmia. Last echocardiography shows a normal ventricular function and a competent AV valve. Despite extensive calcifications of the inferior cavopulmonary extraatrial conduit, its flow is unrestricted.
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Comment
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The anatomy of this child comprises a complex single-ventricle heart, which is probably best labeled as right isomerism, cumulating features as asplenia, inferior P-wave axis on ECG, single appendage of the right morphologic type, bilateral short eparterial bronchi, and anomalous pulmonary venous drainage. However, a few features are commonly observed in left isomerism anomalies as an initially unobstructed pulmonary arterial inflow, individual drainage of the pulmonary veins into the atrium, and a suprahepatic venous drainage separate from the inferior vena cava [2].
The presence of a combination of variant systemic and pulmonary venous connection patterns may require specifically planned and timed technical adaptations to obtain an optimal Fontan procedure [3]. Hence, encouraging results of the modified Fontan operation have been reported, even after exceeding the originally proposed physiological and anatomical limits [1]. In this child, several conditions notoriously deleterious for the success of a Fontan-type procedure were treated in a sequential although not fully nor ideally planned fashion. These included, respectively, correction of the originally unobstructed pulmonary arterial inflow, atresia of one and stenoses of the three other PV, incompetence of the common AV valve, and potential subaortic stenosis by a diminutive subaortic right ventricle.
Given the grim prognosis of pulmonary vein stenosis in general, this case reports a long-term successful outcome of a TCPC after repair of pulmonary vein stenoses and emphasizes the importance of clearly delineating, as soon as possible, all potential anatomic and hemodynamic obstacles, especially in complex single-ventricle hearts with heterotaxia variants, to ensure a satisfactory outcome of the Fontan operation.
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References
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Mayer J.E., Helgason H., Jonas R.A., et al. Extending the limits for modified Fontan procedures. J Thorac Cardiovasc Surg 1986;92:1021-1028.[Abstract]
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Anderson R.H., Macartney F.J., Shinebourne E.A., Tynan M. Atrial isomerism. . Paediatric cardiology. Edinburgh: Churchill Livingstone, 1987:473-495.
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Michielon G., Ghazagozloo F., Julsrud P.R., Danielson G.K., Puga F.L. Modified Fontan operation in the presence of anomalies of systemic and pulmonary venous connection. Circulation 1993;88:141-148.
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Cavopulmonary connection after repair of no confluent pulmonary atresia and total anomalous pulmonary venous connection
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March 1, 2003;
125(3):
731 - 733.
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Abstract
Introduction
