Ann Thorac Surg 2009;88:1680-1683. doi:10.1016/j.athoracsur.2009.04.020
© 2009 The Society of Thoracic Surgeons
Case Reports
A Symptomatic Neonate With Tetralogy of Fallot, an Absent Pulmonary Valve, and a Single Coronary Artery With a Major Fistula
Noriyoshi Kajihara, MDa,*,
Toshihide Asou, MDa,
Yuko Takeda, MDa,
Yoshimichi Kosaka, MDa,
Minoru Matsuhama, MDa,
Yasuko Onakatomi, MDa,
Sadamitsu Yanagi, MDb,
Seiyo Yasui, MDb
a Department of Cardiovascular Surgery, Kanagawa Children's Medical Center, Yokohama, Japan
b Department of Pediatric Cardiology, Kanagawa Children's Medical Center, Yokohama, Japan
Accepted for publication April 2, 2009.
* Address correspondence to Dr Kajihara, 2-138-4 Mutsukawa, Minami-ku, Yokohama, 232-8555, Japan (Email: n-c.kaji{at}f6.dion.ne.jp).
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Abstract
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We present a case with a rare combination of tetralogy of Fallot with an absent pulmonary valve, and a single coronary artery with a major fistula to the main pulmonary artery. Myocardial ischemia developed in response to increasing shunt flow through the coronary fistula, resulting in heart failure. We ligated the coronary fistula and plicated the anterior wall of the dilated pulmonary arteries during the neonatal period. Complete repair through a transatrial-transpulmonary approach was performed at the age of 17 months. The postoperative course was excellent and the patient maintained a stable hemodynamic and respiratory state with no evidence of myocardial ischemia.
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Introduction
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Tetralogy of Fallot (TOF) in combination with an absent pulmonary valve (PV) syndrome is a disease that includes dysplasia and incompetence of the PV, annular stenosis, and dilatation of the pulmonary arteries, which lead to tracheobronchial compression of varying degrees [1]. A recent patient at our institution also had a single coronary artery with a major fistula to the main pulmonary artery. Although congenital coronary arterial fistulas have occasionally been described in patients with TOF [2], we believe that the combination of coronary arterial fistula and TOF with an absent PV has not been reported in English literature. We present a patient who had TOF with an absent PV, and a single coronary artery with a major fistula to the main pulmonary artery. This patient showed major symptoms of myocardial ischemia during the neonatal period.
A fetus at the gestational age of 27 weeks was referred to our institute because congenital heart disease was suspected. At 40 weeks of gestation, a 3,108 g baby girl was born without dyspnea. After birth, she was diagnosed with TOF with an absent PV, and a single coronary artery with a major fistula to the main pulmonary artery.
Echocardiography and computed tomographic scan (Fig 1) revealed that a remarkably dilated single coronary artery crossed the right ventricular outflow tract (RVOT) branches of the right and left anterior descending and circumflex arteries. The diameter of the main trunk was 10 mm. Continuous blood flow of the fistula poured proximally into the main pulmonary trunk. A PV was not detected, and the diameter of the pulmonary annulus was 5 mm (Z value, –3.7). Her pulmonary regurgitation was mild. The pulmonary artery branches were moderately dilated; the diameters of the right and left pulmonary arteries were 13 and 10 mm, respectively. The ventricular septal defect was the perimembranous outlet type with anterior misalignment of the conus septum.
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Fig 1. Three-dimensional computed tomographic scan shows (A) a remarkably dilated coronary artery crossed the right ventricular outflow tract (RVOT), which was bulged, and (B) the coronary arterial fistula, which joined the main pulmonary trunk (white arrow). (Asc. Ao. = ascending aorta; CA = coronary artery; LAD = left anterior descending artery; Lt. PA = left pulmonary artery; MPA = main pulmonary artery; RCA = right coronary artery; RVOT = right ventricular outflow tract.)
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Tachypnea and tachycardia appeared gradually in the late neonatal period. The chest roentgenogram revealed cardiomegaly with a cardiothoracic ratio of 73% and an electrocardiogram showed a significant ST depression at V4–6. Echocardiography confirmed increasing shunt flow through the coronary fistula.
At 28 days of age, surgery was performed, and no major obstructions of the trachea or bronchial trees were found using bronchofiberscopy. After performing a median sternotomy, a strikingly dilated coronary artery was observed with a bulged, thin RVOT wall beneath the pulmonary annulus. The coronary fistula joined with the main pulmonary trunk at its left-posterior aspect. We ligated the fistula at its distal end and plicated the anterior wall of the dilated right and left pulmonary arteries. Immediately after ligation of the fistula, the diastolic blood pressure increased by 10 mm Hg, and the unstable ST electrocardiogram profiles stabilized.
The patient's condition improved considerably in terms of hemodynamic and respiratory state after the initial operation. She was monitored while on anticoagulation therapy, because we had concerns about potential thrombus formation after ligation of the fistula. The results of cardiac catheterization at 1 year old are shown in Table 1. There was no drainage into the pulmonary artery from the coronary artery, the diameter of which had decreased to 3 mm at the main trunk (Fig 2). Right ventriculography showed stenotic infundibulum and post-stenotic dilatation beneath the pulmonary annulus, creating a double-chambered right ventricle (Fig 3).
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Fig 2. Coronary angiography at 1 year old. There was no drainage into the pulmonary artery from the coronary artery, which had returned to a normal diameter. (LAD = left anterior descending artery; LCX = left circumflex artery; RCA = right coronary artery.)
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Fig 3. Right ventriculography. Stenotic infundibulum and a dilated sinus portion were observed. The white arrow shows the RV pulmonary trunk junction. (LPA = left pulmonary artery; RPA = right pulmonary artery; RV = right ventricle.)
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At 17 months of age, she underwent a transatrial-transpulmonary TOF repair. Ascending aortic and bi-caval cannulation were performed, and cardiopulmonary bypass was maintained. Cardiac arrest was obtained using cold crystalloid cardioplegic solution. The PV leaflets were severely hypoplastic. The perimembranous ventricular septal defect was closed with a polytetrafluoroethylene patch (GoreTex patch; W. L. Gore & Associates, Flagstaff, AZ), the muscle was sliced from the RVOT [3] after a minimum ventriculotomy and the RVOT was reconstructed using a monocusp-valved transannular patch with a bulging sinus [4]. The postoperative course was excellent; the patient had a stable hemodynamic and respiratory state, and she was discharged 5 days after the operation. At 5 months after the operation, her condition remains stable with trivial pulmonary insufficiency.
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Comment
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Congenital coronary arterial fistula is classified into two subgroups according to the associated cardiac anomalies. One group of patients has an isolated lesion. Most patients in this group present late in life, occasionally in childhood, and rarely in infancy. The early and late results after surgical treatment are excellent [1]. Another group has major associated cardiac anomalies such as TOF, transposition of the great arteries or ventral septal defect [2]. Holzer and colleagues [2] reported 106 of 426 cases of coronary fistula were associated with congenital cardiac disease with symptoms of varying degrees from dyspnea in early infancy to asymptomatic findings; surgical outcomes in this group were dependent on these associated anomalies.
We used staged surgical repair in this case for the following reasons: (1) There were no major obstructions of the trachea or bronchial trees; (2) after ligation of the fistula, her hemodynamic condition improved and her myocardial ischemia disappeared; (3) our standard surgical strategy for TOF involves transatrial-transpulmonary repair during infancy, beyond the neonatal period, to limit or avoid a transmural right ventriculotomy. The timing for complete repair of TOF is controversial [5, 6]. Pigula and colleagues [5] reported that 99 infants aged less than 90 days old underwent complete repair of TOF through a right ventriculotomy with excellent early results. Of note, however, 25 reoperations were required for 22 children between 1 month and 5 years after the primary repair. In 15 of these patients who required reoperations, the complete repair was performed in the neonatal period. On the other hand, Karl and colleagues [6] reported outcomes for 366 patients who underwent a non-neonatal transatrial-transpulmonary repair of TOF at a median age of 15.3 months. In that study, the actuarial survival rate was 97.5% at 42 months and freedom from reoperation was 95% after 10 years of follow-up. They concluded that transatrial-transpulmonary repair would result in better long-term preservation of right ventricular function.
Coronary anomalies occur in between 5% and 12% of patients with TOF. Brizard and colleagues [7] reported that most patients with surgically important coronary anomalies in TOF can be managed through the transatrial-transpulmonary approach. Furthermore, the combination of a ventriculotomy with pulmonary insufficiency is implicated in the deterioration of right ventricular function and the development of arrhythmias after the repair of TOF. In the present case with a dysplastic PV and a hypoplastic pulmonary annuls associated with absent PV syndrome, we performed a minimum ventriculotomy using a well-designed polytetrafluoroethylene monocusp patch with a bulging sinus [4] through a transatrial-transpulmonary approach to reduce the risk of pulmonary regurgitation and maintain right ventricular function for the long term.
In conclusion, we have presented a case of TOF with an absent PV and a single coronary artery with a major coronary fistula. We performed staged surgical repair and the postoperative course was excellent with no evidence of myocardial ischemia.
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References
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- Kouchouskos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Congenital anomalies of the coronary arteriesIn: Kirklin JW, editor. Kirklin/Barratt-Boys Cardiac Surgery. 3rd ed.. Philadelphia: Churchill Livingstone; 2003. pp. 1240.
- Holzer R, Johnson R, Ciotti G, Pozzi M, Kitchiner D. Review of an institutional experience of coronary arterial fistulas in childhood set in context of review of the literature Cardiol Young 2004;14:380-385.[Medline]
- Asou T, Rachmat J. Slicing technique of the RV outflow tract in transatrial-transpulmonary repair for tetralogy of Fallot J Cardiovasc Surg (Torino) 2001;42:639-642.[Medline]
- Miyazaki T, Yamagishi M, Nakashima A, et al. Expanded polytetrafluoroethylene valved conduit and patch with bulging sinuses in right ventricular outflow tract reconstruction J Thorac Cardiovasc Surg 2007;134:327-332.[Abstract/Free Full Text]
- Pigula FA, Khalil PN, Mayer JE, del Nido PJ, Jonas RA. Repair of tetralogy of Fallot in neonates and young infants Circulation 1999;100:II157-II161.[Medline]
- Karl TR, Sano S, Pornviliwan S, Mee RB. Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair Ann Thorac Surg 1992;54:903-907.[Abstract/Free Full Text]
- Brizard CP, Mas C, Sohn YS, Cochrane AD, Karl TR. Transatrial-transpulmonary tetralogy of Fallot repair is effective in the presence of anomalous coronary arteries J Thorac Cardiovasc Surg 1998;116:770-779.[Abstract/Free Full Text]
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Abstract
Introduction
