J Thorac Cardiovasc Surg 2006;131:469-471
© 2006 The American Association for Thoracic Surgery
Neonatal stenotic Ebstein's anomaly: A novel technique of right ventricular exclusion
Tae-Jin Yun, MD, PhD
*
,
Seung-Hyun Lee, MD,
Jae-Kon Ko, MD, PhD
Division of Pediatric Cardiac Surgery, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
Received for publication September 9, 2005; accepted for publication September 15, 2005.
* Address for reprints: Tae-Jin Yun, MD, Division of Pediatric Cardiac Surgery, Asan Medical Center, 388-1 Poongnap-Dong, Songpa-Ku, Seoul, Republic of Korea, 138-736 (Email: tjyun{at}amc.seoul.kr).
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Dr Yun
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A 16-day-old baby with stenotic Ebstein's anomaly underwent an emergency operation to relieve progressive hypoxia, which was unresponsive to prostaglandin E1 infusion and nitric oxide inhalation. Preoperative cardiac magnetic resonance imaging showed severe enlargement of the right cardiac chambers compressing the bilateral lungs (Figure 1). Operative findings showed a hugely dilated right atrium; a large, thin-walled atrialized right ventricle (aRV); and normal size and branching pattern of the pulmonary arteries. After aortic and bicaval cannulation through a median sternotomy, the ductus was divided, and cardioplegic solution was administered. Septal and posterior leaflets of the tricuspid valve were distally displaced down to the right ventricular outflow tract. The anterior leaflet was adherent to the right ventricular anterior free wall, leaving a small functional right ventricle (fRV) in the right ventricular outflow tract. There was a small opening (5 mm in diameter), or displaced tricuspid valve mechanism, between the aRV and the fRV. Given the severe wall thinning of the aRV and the small-sized fRV, biventricular repair was deemed unattainable. After the atrial septum was widely excised, the pulmonary and tricuspid valves were closed primarily. The tricuspid sac was suture obliterated from the apex to the base by suturing the right ventricular free wall to the septum, leaving the area 10 mm apart from the membranous septum to avoid injury of the conduction pathway (Figure 2). Once the right atrium was closed and the heart was reperfused, a 3.5-mm polytetrafluoroethylene vascular graft was interposed between the innominate artery and the main pulmonary artery. Cardiopulmonary bypass and aortic crossclamping times were 192 minutes and 71 minutes, respectively. The baby was extubated on postoperative day 8 and discharged on postoperative day 17. After 6 months of follow-up, the patient was readmitted and underwent bidirectional cavopulmonary shunt uneventfully. Chest computed tomography before bidirectional cavopulmonary shunt showed decreased right atrial size and obliterated aRV, which resulted in the compensatory growth of the lungs on both sides (Figure 1).
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Figure 1. Preoperative chest x-ray (A) and cardiac magnetic resonance image (B) showing severe cardiomegaly with a huge right atrium and atrialized right ventricle. Postoperative chest radiograph (C) and cardiac computed tomogram (D) at 6 months postoperatively showing decreased heart size and an effectively obliterated atrialized right ventricle.
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Figure 2. Schematic illustration showing the novel technique of atrialized right ventricle obliteration. RA, Right atrium; MS, Membranous septum; aRV, atrialized right ventricle; fRV, functional right ventricle; MPA, main pulmonary artery; LV, left ventricle.
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Discussion
It is generally believed that approximately 10% of patients with Ebstein's anomaly are not amenable to biventricular repair. After being introduced in 1991
1
and followed by several case reports,
2-5
the Starnes operation has been regarded as the procedure of choice for neonatal stenotic Ebstein's anomaly. The original Starnes procedure, however, has several pitfalls: the need for a prosthetic patch to close the tricuspid annulus, the risk of systemic thromboembolism of the prosthetic materials filled in the tricuspid sac, the risk of conduction injury on suturing the patch along the tricuspid valve annulus, and exposure of the coronary sinus to high pressure when it is left beneath the right ventricular exclusion patch. Our technique of right ventricular exclusion is simple and safe in that (1) it avoids the use of foreign materials, (2) it preserves the conduction pathway, and (3) it leaves the coronary sinus draining to the right atrium. Furthermore, it can be inferred from this case that effective reduction in the size of the right ventricle, as well as the right atrium, might be important to promote the appropriate growth of the once-hypoplastic lungs, which are compressed preoperatively by the severely enlarged right cardiac chambers in Ebstein's anomaly.
References
- Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. a new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]
- Van Son JA, Falk V, Black MD, Haas GS, Mohr FW. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiothorac Surg 1998;13:280-285.[Abstract/Free Full Text]
- Endo M, Ohmi M, Sato K, Tanaka T, Kakizawa H, Tabayashi K. Tricuspid valve closure for neonatal Ebstein's anomaly. Ann Thorac Surg 1998;65:540-542.[Abstract/Free Full Text]
- Maeda M, Sai N, Miyahara K, Sakuri H, Nakayama M. Two staged Starnes operation for Ebstein's anomaly with pulmonary atresia in early infancy—a report of a success case. Jpn J Thorac Cardiovasc Surg 1998;46:1200-1205.[Medline]
- Adachi I, Ishzaka T, Ichikawa H, Kagisaki K, Kokai S. Midterm result of a case after Starnes operation. Jpn J Thorac Cardiovasc Surg 2004;52:272-275.[Medline]
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