Ann Thorac Surg 1999;68:570-571
© 1999 The Society of Thoracic Surgeons
Case Reports
Combined rastelli and atrial switch procedure: a 10-year follow-up
Marshall L. Jacobs, MDa,
Mortimer J. Buckley, MDb,
Richard L. Liberthson, MDc
a Department of Surgery, Deborah Heart and Lung Center, Browns Mills, New Jersey>, USA
b Department of Surgery, Massachusetts General Hospital, Boston, Massachusetts>, USA>
c Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA
Address reprint requests to Dr Jacobs, Section of Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Erie Ave at Front St, Philadelphia, PA 19134
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Abstract
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We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.
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Introduction
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Numerous surgical strategies have been used to achieve optimal physiology for patients with discordant atrioventricular connection, ventricular septal defect, and left ventricular outflow tract obstruction. Important considerations in planning surgical therapy include intrinsic abnormalities of the right ventricular structures, including varying degrees of ventricular hypoplasia or abnormal atrioventricular valve morphology, anatomic variations and functional fragility of the conduction system (particularly in hearts with L-loop ventricles), and the risk of deterioration of systemic right ventricular function associated with tricuspid regurgitation. Among the most ambitious strategies to address all of these issues is what has become known as the double switch operation. First described in 1990 by Ilbawi and associates [1], this approach addresses atrioventricular discordance by means of a Mustard baffle or Senning procedure and addresses ventriculoarterial discordance with left ventricular outflow tract obstruction by means of a Rastelli operation. This concept is applicable to patiets with visceroatrial situs solitus and cardiac segmental anatomy {SLL}, or visceroatrial situs inversus and cardiac segmental anatomy {IDD}, together with either discordant ventriculoarterial connection or double outlet right ventricle with subpulmonary obstruction or atresia. In the original report, Illbawi and associates reported satisfactory functional status of 2 initial patients at follow-up of 6 and 18 months. DiDonato and associates [2] in 1992 added short-term follow-up of 2 additional patients, both with corrected transposition in situs inversus. The largest series of patients who had such operations (n = 18) was reported in 1994 by Imai and associates [3]. Of 16 survivors followed up between 4 and 46 months, 14 were in New York Heart Association functional class I and 2 were in class II. Two patients required reoperations, and none had more than mild right or left atrioventricular valve regurgitation.
We used that operative approach 10 years ago for a patient with cardiac anatomy {IDD}, double outlet right ventricle with pulmonary atresia, and right aortic arch. The patient, who early in life had carried the presumptive diagnosis of tricuspid atresia, had had two prior palliative operations. In 1958, when the patient was 9 years old, Dr Robert Gross had performed a modification of a Blalock-Taussig shunt, extending the length of the right subclavian artery by using a banked homograft taken from the descending thoracic aorta of a small child and anastomosing it to the right pulmonary artery. During the same operation, the intra-atrial communication was enlarged using a punch device through the lateral wall of the right atrium, followed by digital dilation of the resulting defect. In 1969 at age 20 years, the patient had severe cyanosis and hemoptysis. Cardiac catheterization delineated the cardiac anatomy, and the right Blalock Taussig shunt was the only apparent source of pulmonary blood flow. A left Blalock Taussig shunt was constructed.
In January 1988, when the patient was 39 years old, he presented with severe cyanosis, a 1- to 2-year history of progressive dyspnea on exertion, and a recent left sided hemiparesis which had lasted 12 to 18 hours. Hematocrit was 68%. The neurologic event was consistent with either an embolic phenomenon or a transient ischemic attack related to hyperviscosity. Cardiac catheterization revealed findings of {IDD}, double outlet right ventricle with pulmonary atresia, and moderate hypoplasia of the right ventricle. The right modified Blalock Taussig shunt was visualized, and the status of the left-sided shunt was uncertain. Two months later, operative repair was undertaken utilizing hypothermic cardiopulmonary bypass at 22°C. Both shunts were ligated. The homograft portion of the right-sided shunt was heavily calcified. Atrial inflow inversion by means of a Mustard baffle of autologous pericardium was accomplished through the left-sided morphologic right atrium. The Rastelli portion of the repair consisted of intraventricular baffling from the left ventricle through the ventricular septal defect to the aorta using a Dacron baffle, with all suturing done on the right ventricular side. Continuity between the anterior right ventricle and the pulmonary arteries was established using a cryopreserved 23-mm aortic valved homograft together with a 24-mm Dacron velour graft extension at the right ventricular end. When the confluence of the pulmonary arteries was opened, there was a moderate amount of return into the right pulmonary artery despite ligation of the right-sided shunt. Separation from cardiopulmonary bypass after 2 hours and 33 minutes was accomplished with moderate inotropic support. The postoperative course was complicated by renal dysfunction, prolonged respiratory failure, and supraventricular and ventricular arrhythmias. Nonetheless, the patient made a full recovery.
In 1996, 8 years after operation, the patient had cardiac catheterization. Oxygen saturation in the left ventricle and the aorta was 92%. Saturation in the superior vena cava was 63% with a step-up to 70% in the branch pulmonary arteries. Left ventricular pressure was 120/8 mm Hg with aortic pressure 120/60 mm Hg. Right ventricular pressure was 50/5 mm Hg with pressure in the pulmonary arteries distal to the conduit of 30/12 mm Hg. There was no systemic venous or pulmonary venous pathway obstruction or evidence of interatrial shunting. The intraventricular baffle was intact with a pinhole residual ventricular septal defect and no gradient between the left ventricle and the aorta. As noted, a 20-mm gradient was present between the right ventricle and the branch pulmonary arteries. Additionally, a tiny patent ductus was visualized angiographically with flow into the right branch pulmonary artery. Device closure of the ductus was considered, but the ductus could not be crossed and was left patent. Echocardiography revealed mild insufficiency of the right and left atrioventricular valves. There were segmental wall motion abnormalities of the left ventricle, particularly in the apical region. Overall left ventricular systolic function was mildly impaired.
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Comment
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The durability of this complex operative approach is exemplified by the satisfactory functional status of this patient at 10-year follow-up. Having been severely limited by cardiac-related disability throughout early adult life before repairative surgery, the patient is now 49 years old and handles a full work load and travels extensively. We adopted this approach contemporaneously with Ilbawi and others who sought alternatives to the suboptimal results encountered with use of the right ventricle and right atrioventricular valve in the systemic circulation of patients who have biventricular repair for malformations with atrioventricular discordance. This late follow-up of a patient adds to the numerous reports documenting satisfactory functional status of patients early after double switch operations [1–4]. There can be little doubt that patients who have combined atrial inversion and Rastelli operations are at risk for the entire spectrum of late problems associated with either of those operative approaches undertaken in isolation. Nonetheless, this combined approach, which enables the left ventricle and left atrioventricular valve to be used in the systemic circulation, might provide the most satisfactory option for some patients with atrioventricular discordance and ventricular septal defect combined with pulmonary stenosis.
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References
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Ilbawi M., DeLeon S., Backer C., et al. An alternative Approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia. J Thorac Cardiovasc Surg 1990;100:410-415.[Abstract]
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DiDonato R., Troconis C., Marino B., et al. Combined Mustard and Rastelli operations. J Thorac Cardiovasc Surg 1992;104:1246-1248.[Abstract]
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Imai Y., Sawatari K., Hoshino S., Ishihara K., Nakazawa M., Momma K. Ventricular function after anatomic repair in patients with atrioventricular discordance. J Thorac Cardiovasc Surg 1994;107:1272-1283.[Abstract/Free Full Text]
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Delius R., Stark J. Combined Rastelli and atrial switch procedure. Eur J Cardiothorac Surg 1996;10:551-555.[Abstract]
Accepted for publication January 13, 1999.
This article has been cited by other articles:
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M. Koh, T. Yagihara, H. Uemura, K. Kagisaki, and S. Kitamura
Functional biventricular repair using left ventricle-pulmonary artery conduit in patients with discordant atrioventricular connections and pulmonary outflow tract obstruction--does conduit obstruction maintain tricuspid valve function?
Eur. J. Cardiothorac. Surg.,
October 1, 2004;
26(4):
767 - 772.
[Abstract]
[Full Text]
[PDF]
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Abstract
Introduction
