Ann Thorac Surg 2008;86:1937-1940. doi:10.1016/j.athoracsur.2008.07.093
© 2008 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Tetralogy of Fallot With Total Anomalous Pulmonary Venous Drainage
Sachin Talwar, MCh,
Shiv Kumar Choudhary, MCh*,
Mukkannavar Babu Shivaprasad, MS,
Anita Saxena, DM,
Shyam Sunder Kothari, DM,
Rajnish Juneja, DM,
Balram Airan, MCh
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Accepted for publication July 29, 2008.
* Address correspondence to Dr Choudhary, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India (Email: shivchoudhary{at}hotmail.com).
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Abstract
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Background: The association of tetralogy of Fallot with total anomalous pulmonary venous drainage (TAPVD) is rare. We report our experience with this condition and review the literature.
Methods: Between January 1997 and May 2008, 6 patients (aged 3 months to 5 years; median weight, 10 kg) with combined tetralogy of Fallot with TAPVD underwent complete primary repair at All India Institute of Medical Sciences, New Delhi, India. Their records were retrospectively reviewed.
Results: A correct preoperative diagnosis was available in 5 patients by echocardiography and cardiac catheterization. Four patients had supracardiac TAPVD, and 1 each had TAPVD to the coronary sinus and right atrium. There were no early or late deaths. Median follow-up was 37.5 months ± 46.8 months (range, 2 to 112). All patients are in New York Heart Association class I. Follow-up echocardiograms have revealed no significant abnormalities and have documented normal biventricular function and pulmonary artery pressures. One patient underwent a 24-hour Holter examination at 68 months of follow-up for investigation of a new-onset 2:1 atrioventricular block, for which a permanent pacemaker implantation is planned.
Conclusions: When diagnosed accurately, complete primary repair is possible in patients with tetralogy of Fallot and TAPVD and the outcomes are satisfactory with planned surgery.
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Introduction
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The association of tetralogy of Fallot (TOF) with total anomalous pulmonary venous drainage (TAPVD) is rare and has been reported in the medical literature in the form of scattered case reports [1–22]. The pathophysiology, diagnosis, surgical challenges and postoperative outcomes are in the process of evolution. We reviewed the records of all patients with TOF and TAPVD managed by us surgically in the past 10 years. We also reviewed the available literature on this condition with special reference to the pathophysiology, clinical presentation, and methods of diagnosis, surgery and results.
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Patients and Methods
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Between January 1997 and May 2008, 2,292 patients with TOF underwent total repair at the All India Institute of Medical Sciences, New Delhi, India. Of these, only 6 patients had combined TOF with TAPVD (Table 1), giving an incidence of 0.26%. Their case records were retrospectively reviewed. The need for a formal Institutional Review Board approval was waived for this study as informed consent had been obtained and patient confidentiality was maintained.
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Table 1 Profile of Patients With Combined Tetralogy of Fallot (TOF) and Total Anomalous Pulmonary Venous Drainage (TAPVD)
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Only patients with a confirmed diagnosis of classic TOF and TAPVD confirmed at operation were included. Patients with TOF physiology associated with TAPVD with structurally or functionally univentricular hearts were excluded.
The clinical features of our patients were otherwise indistinguishable from those of a patient with "classical" TOF. All presented with cyanosis and had varying degrees of clubbing on physical examination. Chest radiographs were typical for TOF in 3 patients (patients 1, 2, and 6). All patients had a right ventricular (RV) type of apex, 1 (patient 5) had typical figure-of-8 appearance, 1 patient (patient 4) had evidence of severe pulmonary arterial hypertension, and the remaining patient (patient 3) had evidence of normal pulmonary blood flow.
Echocardiography was performed in all patients. In 5 of 6 patients, the diagnosis was apparent on echocardiography. As a matter of institutional policy, preoperative cardiac catheterization is performed in all patients with TOF at our institution, and it confirmed the abnormal pulmonary venous connections in 5 patients, whereas in 1 patient (patient 6) it was an intraoperative finding. All these patients underwent planned complete primary repair.
Operative Technique
After median sternotomy, the pericardium was opened to the right of the midline and was reflected on the left side. Cardiopulmonary bypass (CPB) was established by aortobicaval cannulation with core cooling to 28°C. In patients with supracardiac TAPVD and the vertical vein ascending on the left side, the vertical vein was dissected extrapericardially and was looped. The aorta was now cross clamped, and cold (4°C) hyperkalemic antegrade cardioplegia was delivered into the aortic root. The right pleura was completely opened in patients with supracardiac TAPVD, and the pericardial reflection was cut down just short of the right phrenic nerve to facilitate displacement of the heart into the right pleural cavity, after which a wide anastomosis was made between the common pulmonary venous chamber and the left atrial appendage by the standard posterior approach. After this, the heart was repositioned and a standard right atriotomy was made.
Repair of TAPVD to the superior vena cava (SVC) and to the coronary sinus and the right atrium was performed after TOF repair. In 2 patients, complete repair of TOF was performed using the pure transatrial approach, whereas a combined transatrial and transventricular approach with placement of a transannular patch was performed in 4 patients. In patients receiving a transannular patch, a monocusp pulmonary valve was placed in the pulmonary position. The TAPVD to coronary sinus was repaired by cutting back the coronary sinus toward the left atrium, thus creating a large atrial septal defect, which was closed by placing a Dacron (C. R. Bard, Haverhill, Pennsylvania) patch.
In 1 patient with TAPVD to the right atrium opening near the entry of the superior vena cava, this opening was enlarged by communicating it with the atrial septal defect, after which the closure of this defect directed the pulmonary venous return toward the left atrium. In patient 4, who had supracardiac TAPVD to the SVC, the right atriotomy was extended laterally into the SVC to just short of the innominate vein, avoiding the area of the sinoatrial node. The large opening of the common chamber was visualized above the opening of the azygous vein. The atrial septal defect was enlarged, and a Dacron patch was sutured around its edges and around the opening of the common chamber to baffle the pulmonary venous return toward the left atrium. A patch of untreated autologous pericardium was used to augment the SVC. A small 4-mm interatrial communication was left open in 5 patients to enable right-to-left shunting in the event of raised right side pressures in the early postoperative period. In patient 4, a unidirectional valved patch was used to close the atrial septal defect because of concerns about the elevated pulmonary vascular resistance in this patient. All patients were uneventfully weaned off CPB after routine maneuvers. Elective inotropic support consisted of dopamine, 5 µg · kg–1
· min–1, and sodium nitroprusside, 0.5 µg · kg–1
· min–1. Patient 4 also received milrinone, 0.5 µg · kg–1
· min–1.
The entire medical literature was searched for similar reports in the following databases: Medline, PubMed, Scopus, Index Medicus South-east Asia region (IMSEAR), IndMED, and Index Copernicus. The Keywords employed for the search were as follows: tetralogy of Fallot/complications/surgery; pulmonary veins/abnormalities/surgery; tetralogy of Fallot and anomalies of pulmonary veins. Upon finding a relevant report; its related articles were searched; and so on. A total of 25 such cases were identified in the entire medical literature.
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Results
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Of the 6 patients, 5 were male (Table 1). Ages ranged from 3 months to 5 years (mean, 26.8 ± 18 months); mean weight was 8.5 ± 3.1 kg. Four patients had supracardiac TAPVD, and 1 each had TAPVD to the coronary sinus and right atrium. The mean CPB and aortic cross-clamp times were 124 ± 24.7 minutes and 93 ± 18.9 minutes, respectively. All patients had an uneventful postoperative course, and there were no early deaths. The median duration of mechanical ventilatory support was 24 ± 10.6 hours. Median duration of inotropic support was 48 ± 16.4 hours. Median follow-up was 37.5 ± 46. 8 months (range, 2 to 112). All patients are in New York Heart Association class I. Patients 1, 2, and 3 are off medication, whereas the last 3 patients are on a regimen of oral digoxin, captopril, and diuretics. Their prehospital discharge and follow-up echocardiograms have revealed no significant abnormalities and have documented normal biventricular function and pulmonary artery pressures. One patient (patient 3) underwent a 24-hour Holter examination at 68 months of follow-up for investigation of a new-onset 2:1 atrioventricular block, for which a permanent pacemaker implantation is planned.
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Comment
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This study describes the clinical outcome of 6 patients of TOF with TAPVD of varying types. Tetralogy of Fallot is rarely associated with TAPVD [1–22]. An extensive search of the entire medical literature identified only 25 cases (Table 2). The incidence has been reported to be 0.25% (3 of 1,183) [5], to 0.34% (2 of 592) [1] in all patients with TOF. In the majority of patients with TOF and TAPVD, the presenting manifestations appear to be those of TOF, as seen in most patients in this series. A combination of TOF and TAPVD results in chronic volume and pressure load of the right side of the heart, leading to enlargement of the right atrium and right ventricle and underfilling of the left atrium and left ventricle. The only determinant of the volume load on the left side of the heart is then the shunt across the interatrial communication, which is invariably present. Many of these patients may only have cyanosis, and symptoms related to TAPVD never occur because of the right ventricular outflow tract obstruction [9]. Pulmonary venous pressures are almost always never elevated unless there is obstructed TAPVD or a pharmacologic manipulation such as prostaglandin infusion [6] or a systemic to pulmonary artery shunt is carried out [1]. Hence, the diagnosis is rarely suspected clinically because the reduction of pulmonary blood flow dominates the clinical presentation [5].
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Table 2 Profile of Patients With Combined Tetralogy of Fallot (TOF) and Total Anomalous Pulmonary Venous Drainage (TAPVD) in the Medical literature [1–22]
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We found a presentation similar to TAPVD in 1 patient (patient 4). This patient had cyanosis and had features of increased pulmonary blood flow on radiographs with typical figure-of-8 appearance. Cardiac catheterization revealed increased pulmonary arterial pressure with moderate pulmonary stenosis. Hence, presentation may not always be like that of classical TOF. Patients with mild pulmonary stenosis may present more like TAPVD spectrum.
Diagnosis
In the earlier reported cases, the diagnosis of TOF and TAPVD was either made at autopsy [1, 3–5] of patients who had an incorrect surgical procedure or made intraoperatively [9, 14, 15], as in patient 6 in the present series.
Although some studies have highlighted difficulties in identifying these lesions on echocardiography [3, 4], it is an important tool in identifying this anomaly [14]; and the presence of dilated coronary sinus with improper visualization of the pulmonary veins should raise the suspicion of associated cardiac type of TAPVD. In the earlier days, the diagnosis was often suspected by echocardiography but was confirmed only at cardiac catheterization [1, 2, 5, 16, 19, 20–22]. Computed tomography angiography [12], magnetic resonace imaging [10], and transesophageal echocardiography [8] have also been used to delineate pulmonary venous drainage. Antenatal diagnosis is possible by fetal echocardiography [11], which may be subsequently confirmed on postnatal echocardiograms.
A correct and complete diagnosis was established in 5 of 6 cases in our study on echocardiograms, and was subsequently confirmed on cardiac catheterization. In patient 6, the diagnosis was missed probably because of the presence of a large atrial septal defect. Although in present day practice most cases are diagnosed accurately, such a situation may be encountered occasionally.
Surgery
There are a number of concerns in these patients. If the abnormal pulmonary venous drainage is not identified and corrected, there may be difficulty in weaning the patient off CPB, which may be fatal. When a shunt procedure alone is performed, it results in intractable and fatal pulmonary edema [1]. We therefore believe that complete primary repair should be performed in all patients with this diagnosis, and the shunt procedure should be limited to patients with an anatomically small left ventricle, in whom a systemic to pulmonary artery shunt should be combined with repair of the TAPVD.
Another concern with these patients is that the pulmonary arterial tree is not immune to vascular changes despite decreased pulmonary flow. Muster and colleagues [1] have showed pulmonary vascular changes on histopathologic examination of lung specimens from their patient who presented with TOF with partially obstructed mixed TAPVD. However, the pulmonary vascular resistance was not measured in this patient, and the only evident sign was a raised pulmonary artery diastolic pressure. One of our patients had pulmonary vascular resistance of 12.67 units; however, we did not obtain lung biopsies for histopathologic evaluation. We advocate leaving behind a small interatrial communication in such patients because they may have significant right ventricular dysfunction in the immediate and late postoperative phase due to pulmonary vascular changes and the placement of a transannular patch. For those patients in whom a transannular patch is placed, a monocusp pulmonary valve may reduce the pulmonary regurgitation and improve at least the early postoperative outcomes.
As is apparent from Table 2, there have been only 11 reported survivors after surgery so far in the entire medical literature. Only 6 patients in the entire literature were 1 month of age or less [1, 3, 5, 7]. Of these, 3 patients died immediately after birth [3, 5, 14], and a definitive diagnosis was made on autopsy in 2 of these, other than the 1 with antenatal diagnosis; 2 neonates died after a systemic to pulmonary artery anastomosis [1], and successful correction was undertaken in only 1 neonate.
Strangely, the majority of our patients presented at a later age for surgery, with a mean age of 26 months. It is possible that these patients were a highly naturally selected group of patients. Only 2 patients presented in infancy, at 3 and 8 months of age. All of them have undergone successful one-stage corrective repair. With present day advances in surgery, myocardial protection techniques, and improvement in postoperative care of neonates, who may require prolonged intensive care, early primary repair should be the preferred surgical strategy.
Post-CPB and immediate postoperative management of these patients is critical, and the problems are not well defined. The majority of them are probably due to a small left atrium and left ventricle that has to handle an increased blood volume. Right ventricular failure is not uncommon owing to pulmonary regurgitation from a transannular patch. These patients require elective inotropic and ventilatory support until hemodynamic stability is reached. However, once this phase is tided over, their subsequent course is expected to be smooth with good long-term results as observed in our patients.
Our results indicate that the complete primary is possible in these patients, and the outcomes are satisfactory with planned surgery.
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Acknowledgments
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The authors thank Nobuyuki Ishibashi, MD, Department of Cardiovascular Surgery, Children's National Medical Center, Washington, DC, for providing us with the English translation of the papers in Japanese.
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References
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Abstract
Introduction
