Asian Cardiovasc Thorac Ann 2006;14:235-238
© 2006 Asia Publishing EXchange Ltd
Outcome of Glenn Anastomosis for Heterotaxy Syndrome with Single Ventricle
Mohammed Koudieh, FRCS,
E Dean McKenzie, MD1,
Charles D Fraser, Jr, MD1
Division of Congenital Heart Surgery, Michael E DeBakey Department of Surgery, Baylor College of Medicine
1 Congenital Heart Surgery Service, Texas Children’s Hospital, Houston, USA
For reprint information contact: Mohammed Koudieh, FRCS Tel: 966 50 623 0589 Fax: 966 1 465 6666 Email: mkoudieh{at}yahoo.com, Cardiac Surgery Department, Cardiac Center, King Fahad Medical City, PO Box 59046, Riyadh 11525, Saudi Arabia.
 |
ABSTRACT
|
|---|
A retrospective study was undertaken of 157 patients who underwent a Glenn anastomosis between January 1996 and May 2001. Of these, 33 had heterotaxy syndrome: 20 males and 13 females, with a mean age of 1.26 ± 2.8 years. Twenty-five had right atrial isomerism and 5 had left isomerism. A common atrioventricular valve was found in 24 patients, 18 had bilateral superior venae cavae, and 18 had anomalous pulmonary venous return. Repair was carried out in 8 patients with anomalous pulmonary venous return, and pulmonary artery augmentation was performed in 11. Compared to the 124 patients who had a Glenn operation for single ventricle without heterotaxy, there were significantly longer durations of mechanical ventilation, intensive care unit stay, and inotropic support, as well as higher mortality in the heterotaxy group. Heterotaxy syndrome with single ventricle still has a high rate of morbidity and mortality. Patients with severe atrioventricular valve regurgitation are at risk of early death. Complete Fontan circulation may not be possible in all patients, and Glenn anastomosis may be their final palliation.
|
INTRODUCTION
|
|---|
Heterotaxy syndrome is a disorder of laterality with a mismatch or imbalance of right- and left-sided structures. The right- and left-sided atria (which normally are morphologically different) are similar in this condition. It accounts for approximately 3% of all congenital heart defects and often results in complex congenital heart disease. Autosomal dominant transmission has been reported.1,2 Gene mutation has been suggested as the cause.3 Left atrial isomerism (polysplenia syndrome) and right atrial isomerism (asplenia syndrome) are the two types usually described. As asplenia or polysplenia may exist without atrial isomerism, Anderson and colleagues4 prefer to use the term heterotaxy and describe the morphologic details for each patient. Patients with heterotaxy syndrome and single ventricle are a difficult group to manage, and their outcome is worse than those with hypoplastic left heart syndrome. Heterotaxy syndrome is a known factor for failure of the Glenn operation.5 The main aim of this study was to compare the outcome of patients with heterotaxy and single ventricle who underwent palliation with a bidirectional cavopulmonary anastomosis, to those with no heterotaxy who underwent this procedure during the study period, to determine whether advances in postoperative cardiac intensive care management have resulted in improved outcomes.
|
PATIENTS AND METHODS
|
|---|
After approval by the Ethics Committee on Human Research, a database search was carried out in the department of congenital heart surgery in Texas Children’s Hospital for all patients who underwent Glenn anastomosis between January 1996 and May 2001. There were 157 patients of whom 33 had heterotaxy syndrome and single ventricle: 20 males and 13 females, aged 0.16–16.7 years, weighing 4–38.9 kg. Twenty-five patients had right atrial isomerism and 5 had left isomerism. Details of the morphology in these patients are shown in Table 1
. Atrioventricular (AV) valve regurgitation was moderate in 7 patients and severe in 1. Preoperative arrhythmias were present in 12 patients, mainly atrial arrhythmia; there were 4 patients with supraventricular tachycardia, one with sinoatrial node dysfunction, one with AV node dysfunction, one with junctional tachycardia, 3 with atrial ectopy, and 3 with complete heart block. Associated diseases were: gut malrotation in 5, spina bifida in 1, hypothyroidism in 1, imperforate anus with colostomy in 1, history of seizures in 2, and history of femoral osteomyelitis in one.
The operations were performed under standard cardiopulmonary bypass; cardioplegic arrest was used in 13 patients and deep-hypothermic circulatory arrest in 4, mainly to repair total anomalous pulmonary venous return (TAPVR) or for complex pulmonary artery reconstruction. Sixteen patients had bilateral bidirectional cavopulmonary anastomoses, and two had ligation of a small right superior vena cava in the presence of an innominate vein. Cardiopulmonary bypass was needed for associated procedures in more than half of the cases: repair of TAPVR in 8, AV valve repair in 3, and permanent pacemaker implantation in 5 (for 3rd degree heart block in 3 and for atrial arrhythmia in 2). Pulmonary artery augmentation was carried out in 11 patients and take down of a previous shunt in 14.
Statistical analysis was performed with SPSS software (SPSS, Inc., Chicago, IL, USA) using paired Student t tests. Values are expressed as mean ± standard deviation.
|
RESULTS
|
|---|
In the 33 patients with heterotaxy syndrome, intensive care unit stay ranged from 1 to 31 days, the duration of mechanical ventilation was 3–336 hr, postoperative hospital stay was 3–36 days, and O2 saturation on discharge was 70% – 90%. The mean values are given in Table 2. There were 3 early and 2 late deaths. Severe AV valve regurgitation was present in 2 of the early deaths; the 3rd patient died from sepsis and diffuse thrombosis. The two late deaths were due to sepsis and a gastrointestinal complication. Postoperative sepsis occurred in 8 patients, arrhythmia in 11, and 4 had prolonged pleural effusion of more than 14 days. Only 5 patients had final palliation with Fontan circulation at the end of the study; 2 with a lateral tunnel and 3 with an extracardiac conduit. In the 124 patients with single ventricle and without heterotaxy, intensive care unit stay was 1–22 days, and postoperative hospital stay was 3–56 days (Table 2). There was one early and 2 late deaths. When comparing results in the two groups, it was noted that postoperative intensive care unit stay, mechanical ventilation, and hospital stay were significantly longer in the heterotaxy group (Table 2). Postoperative mortality was significantly higher in the heterotaxy group (15.1%) compared to patients without heterotaxy (2.4%).
Follow-up ranged from one month to 5 years, with a mean of 15 months, and it was complete in the 28 survivors, their mean O2 saturation was 78.5%. Some patients with complex systemic and pulmonary venous return were doing well on follow-up and it was felt that Fontan completion would be difficult and risky, so they were left with the Glenn anastomosis as possibly their final palliation.
|
DISCUSSION
|
|---|
Patients with heterotaxy syndrome and single ventricle have very high mortality in the neonatal period after initial palliation (25%–60%), which may reach 95% if associated with obstructed TAPVR.6–8 However, Heinemann and colleagues9 reported a mortality of 33% in 12 patients with heterotaxy and obstructed TAPVR in the neonatal period, and they concluded that repair of TAPVR does not increase the mortality risk. Those who survive the initial palliation continue to be at risk, and the 5-year survival is 40% – 60% in cases of left isomerism, 35% in right isomerism, 80% in patients with a normal heart, and 0% in those without surgical intervention.6,7 The perioperative course is usually stormy with a high incidence of complications. Being asplenic or polysplenic infers greater vulnerability to sepsis. The association of TAPVR and an AV septal defect increases the incidence of pulmonary hypertension that works against the Glenn circulation. Those patients may need measures in the immediate postoperative period to reduce their pulmonary vascular resistance, including prolonged ventilation and the use of nitric oxide. A poor outcome has been reported for these patients, with a 5-year survival of 23%.8 We believe that those with severe AV valve regurgitation, severe pulmonary hypertension, and poor function of the morphologic right ventricle with multiple extracardiac anomalies are a very high risk group and it is better to leave them on a shunt or forward flow, as their prognosis is very poor. McElhinney and colleagues10 reported that neither heterotaxy nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome, although they had 5/18 deaths.
Arrhythmia constitutes a major pre- and postoperative problem in this group of patients; absent sinoatrial node or more than one sinoatrial node is a feature of this syndrome (right or left isomerism, respectively). Among our patients, 12 had preoperative arrhythmias: 9 had atrial arrhythmia and 3 had complete heart block; 11 had rhythm problems immediately after surgery, in the form of supraventricular tachycardia, junctional tachycardia, or sinus bradycardia. Anomalous systemic and pulmonary venous return is quite common in this disease and constitutes a surgical challenge. Bilateral superior venae cavae were present in 18 patients, 13 with TAPVR and 5 with partial anomalous pulmonary venous return. Five patients had previous repair of their pulmonary veins and 8 needed repair with the Glenn operation under deep-hypothermic circulatory arrest with its well-known risks.
Due to the associated procedures required in more than half of our patients, cardiopulmonary bypass was used, although Glenn anastomosis can be performed off-pump, especially in those with bilateral superior venae cavae, and this is our current practice. Antegrade flow was maintained in 7 patients and 13 had associated pulmonary atresia. Forward flow was based on the surgeon’s judgment of the anatomy and risks in each individual case. It is known that right isomerism carries a dismal prognosis and the outcome of surgical repair of left isomerism is better.11 Only 5 of our patients had left isomerism: one of them with high pulmonary pressure, severe AV valve regurgitation, and sustained supraventricular tachycardia needed multiple cardioversions and was included in the early mortality. The other 4 deaths were in asplenic patients. One patient, who had a history of gut malrotation, died on the 10th postoperative day from gram-negative septic shock. Another died on the 5th postoperative day with diffuse thrombosis of his systemic veins including the Glenn shunt; he also had gut malrotation. The 2 late deaths were in a patient who died 83 days postoperatively of septic shock, and another who died at 8 months postoperatively after 3 months of hospitalization with sepsis, arrhythmias, heart failure, and high inotropic support; this patient had poor right ventricular function and moderate tricuspid valve regurgitation preoperatively, with gut malrotation and cystic encephalomalacia. We did not notice any change in patient outcome over the period of the study. Although most patients die before reaching the Fontan stage, recent reports show improved outcomes after the Fontan operation, especially using an extracardiac conduit and fenestration.12–16
In spite of marked advances in intensive care management, heterotaxy syndrome with single ventricle still represents a challenge, with high morbidity and mortality. Patients with severe AV valve regurgitation are at risk of early death after surgery, even after valve repair. Complete Fontan circulation is not possible in all patients, and Glenn anastomosis may be their final palliation.
|
REFERENCES
|
|---|
- Casey B, Cuneo BF, Vitali C, van Hecke H, Barrish J, Hicks J, et al. Autosomal dominant transmission of familial laterality defects. Am J Med Genet 1996;61:325–8.[Medline]
- Alonso S, Pierpont ME, Radtke W, Martinez J, Chen SC, Grant JW, et al. Heterotaxia syndrome and autosomal dominant inheritance. Am J Med Genet 1995;56:12–5.[Medline]
- Aylsworth AS. Clinical aspects of defects in the determination of laterality. Am J Med Genet 2001;101:345–55.[Medline]
- Anderson RH, Webb S, Brown NA. Defective lateralisation in children with congenitally malformed hearts. Cardiol Young 1998;8:512–31.[Medline]
- Alejos JC, Williams RG, Jarmakani JM, Galindo AJ, Isabel-Jones JB, Drinkwater D, et al. Factors influencing survival in patients undergoing the bidirectional Glenn anastomosis. Am J Cardiol 1995;75:1048–50.[Medline]
- Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998;31:1120–6.[Abstract/Free Full Text]
- Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000;36: 908–16.[Abstract/Free Full Text]
- Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999;117:506–14.[Abstract/Free Full Text]
- Heinemann MK, Hanley FL, Van Praagh S, Fenton KN, Jonas RA, Mayer JE Jr, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg 1994;57:88–91.[Abstract]
- McElhinney DB, Reddy VM, Moore P, Hanley FL. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997;63:1676–84.[Abstract/Free Full Text]
- Freedom RM, Hashmi A. Total anomalous pulmonary venous connections and consideration of the Fontan or one-ventricle repair. Ann Thorac Surg 1998;66:681–2.[Abstract/Free Full Text]
- McElhinney DB, Reddy VM. Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects. Ann Thorac Surg 1998;66:683–7.[Abstract/Free Full Text]
- Stamm C, Friehs I, Duebener L, Zurakowski D, Mayer JE Jr, Jonas RA, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 2002;74:1967–78.[Abstract/Free Full Text]
- Azakie A, Merklinger SL, Williams WG, Van Arsdell GS, Coles JG, Adatia I. Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 2001;72:1636–40.[Abstract/Free Full Text]
- Mainwaring RD, Lamberti JJ. Extracardiac conduit Fontan for children with heterotaxy and functionally single ventricle. Cardiol Young 1998;8:479–85.[Medline]
- Cetta F, Feldt RH, O’Leary PW, Mair DD, Warnes CA, Driscoll DJ, et al. Improved early morbidity and mortality after Fontan operation: the Mayo Clinic experience, 1987 to 1992. J Am Coll Cardiol 1996;28:480–6.[Abstract]
TOP
ABSTRACT
INTRODUCTION
