Ann Thorac Surg 2002;74:916-917
© 2002 The Society of Thoracic Surgeons
Case report
Modified Starnes operation for neonatal Ebstein’s anomaly
Manabu Watanabe, MD*a,
Yorikazu Harada, MD, PhDa,
Takamasa Takeuchi, MD, PhDa,
Gengi Satomi, MD, PhDa,
Satoshi Yasukouchi, MD, PhDa
a Department of Cardiovascular Surgery and Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan
Accepted for publication May 1, 2002.
* Address reprint requests to Dr Watanabe, Department of Cardiovascular Surgery, Chiba Children’s Hospital, 579-1 Heta-cho, Midori-ku, Chiba-city, Chiba-ken, Japan
e-mail: dookuman{at}beige.ocn.ne.jp
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Abstract
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We report the case of a severely symptomatic neonate with Ebstein’s anomaly. A modified Starnes operation was performed, but insufficient drainage of venous blood returning through thebesian veins caused overdistention of the right ventricle and severe left ventricular dysfunction. Urgent reestablishment of right ventricular-right atrial communication successfully resolved these problems.
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Introduction
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Results of surgical interventions for Ebstein’s anomaly in severely symptomatic neonates had been uniformly poor [1, 2] until Starnes and associates [3] proposed an innovative procedure. Several modifications have been developed, one of which is the establishment of a small communication between the diminutive right ventricle (RV) and the plicated right atrium (RA) to drain venous blood returning through both the coronary sinus and the thebesian veins [4]. We report the case of a patient in whom insufficient right ventricular (RV) drainage caused overdistention of the RV and severe left ventricular dysfunction. Urgent reoperation to reestablish communication between the RV and the RA was successful.
Cardiomegaly was noted at 29 weeks of gestation by fetal echocardiography, and the mother was referred to our hospital. The fetal echocardiogram at our hospital demonstrated a giant RA, massive tricuspid regurgitation, and lung hypoplasia. The septal leaflet was severely displaced, and RV outflow tract obstruction was caused by a large anomalous anterior leaflet.
At 39 weeks of gestation, the mother gave birth to a boy weighing 3,248 g and with an Apgar score of 6/8. The delivery was normal. A chest roentgenogram demonstrated severe cardiomegaly with a cardiothoratic ratio of 1. On the basis of the echocardiographic grading by severity of the defect (1 to 4 in order of increasing severity proposed by Celermajer and associates [2]), the patient was classified in grade 4. Because of intractable respiratory failure, he was intubated immediately after birth and managed with high-frequency oscillatory ventilation to prevent pulmonary barotrauma and extensive atelectasis in the preoperative period.
Palliative operation was performed when he was 13 days of age. After institution of cardiopulmonary bypass and aortic cross-clamping, we vertically opened the giant RA and enlarged the interatrial communication by resecting the septum primum. The atrialized RV was quite large, and a severely displaced septal leaflet and a rudimentary posterior leaflet were observed. The pulmonary valve was patent, but a large, anomalous anterior leaflet was firmly attached to the RV outflow tract and was causing functional pulmonary atresia. After plicating the big RA, we closed the tricuspid annulus using autologous pericardium. Blood flow through the coronary sinus was directed to the RA.
To drain venous blood returning to the RV through thebesian veins, the membranous portion of the tricuspid annulus was left open. After the aortic cross-clamp was released, the RV immediately became overdistended. The main pulmonary artery was ligated, and the patent ductus arterios was left open. Weaning from cardiopulmonary bypass was uneventful, and an intraoperative echocardiogram showed trivial regurgitation through a slitlike opening in the pericardial patch. The operation was completed with the sternum left open.
Soon after arrival in the intensive care unit, the patient’s hemodynamic status deteriorated, and an echocardiogram revealed compression of the left ventricle by overdistended RV. Increased blood flow through the patent ductus arteriosus was also observed. There was expanding thrombus inside the RV, and no flow signal was detected through the pericardial patch (Fig 1).
Urgent thrombectomy and reestablishment of the communication between the RV and the RA were performed. After reinstituting cardiopulmonary bypass, we opened the RA and found the pericardial patch bulging into it. The slitlike opening in the membranous portion was stuck to the tricuspid annulus. We made a hole 4 mm in diameter in the center of the pericardial patch to reestablish the RV-right atrial communication. Massive fresh thrombus inside the RV was removed, and the RV cavity was washed with saline solution. Then we constructed a modified right Blalock-Taussig shunt with a 3-mm expanded polytetrafluoroethylene tube graft and ligated the patent ductus arteriosus. Weaning from cardiopulmonary bypass was uneventful, and an intraoperative echocardiogram showed sufficient communication between the RV and the RA through the newly made hole.
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Fig 1. Parasternal long-axis echocardiogram demonstrating the overdistended right ventricle (RV) filled with massive thrombus (TH). The compressed left ventricle (LV) is also seen.
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The patient was extubated a month later and discharged in good condition. Follow-up echocardiography demonstrated good left ventricular wall motion and a mildly dilated RV. He underwent a bidirectional Glenn operation at the age of 10 months and is scheduled to have a Fontan operation.
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Comment
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Results of surgical interventions for Ebstein’s anomaly in severely symptomatic neonates had been uniformly unsuccessful [1, 2] until Starnes and associates [3] proposed an innovative procedure. This procedure has been used in many institutions, but the importance of ensuring effective drainage of thebesian venous blood has not been emphasized. Although the precise proportion remains to be determined, a certain amount of coronary venous blood returns to the cardiac chambers through thebesian veins. Gates and coworkers [5] demonstrated that 67.2% ± 6.4% of retrograde cardioplegia was shunted through thebesian veins. Ansari [6] demonstrated that a greater number of thebesian veins are distributed in the RV than in the left ventricle. When we closed the tricuspid annulus with autologous pericardium in our patient, we directed blood flow through the coronary sinus to the RA. We also made a slitlike opening in the membranous portion of the tricuspid annulus, but it was insufficient for effective drainage of the RV and resulted in critical overdistention of that ventricle and left ventricular compression. Ligation of the pulmonary artery could also have played a part in the overdistention of the RV. This result indicates the importance of effective drainage of not only coronary sinus blood, but also thebesian venous blood. Good hemodynamics were obtained after reestablishment of RV-right atrial communication.
Because of the persistence of bidirectional blood flow through the pericardial patch, the diminutive RV has enlarged to a mild degree. Although this has caused no hemodynamic disturbance to date, careful follow-up is necessary.
Fetal echocardiography was instrumental in obtaining the prenatal diagnosis of severe Ebstein’s anomaly and enabled a multidisciplinary approach including prenatal diagnosis, induction of a high-risk delivery, and perinatal intensive care.
In the case of this neonate, insufficient drainage of thebesian venous blood caused overdistention of the RV and severe left ventricular dysfunction. Urgent reoperation to reestablish communication between the RV and the RA successfully relieved the distention and resulting compression.
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References
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- Roberson D.A., Silverman N.H. Ebstein’s anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 1989;14:1300-1307.[Abstract]
- Celermajer D.S., Cullen S., Sullivan I.D., Spiegelhalter D.S., Wyse R.K., Dranfield J.E. Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 1992;19:1041-1046.[Abstract]
- Starnes V.A., Pitlick P.T., Bernstein D., Griffin M.L., Choy M., Shumway N.E. Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]
- Sakamoto T., Harada Y., Takeuchi T., et al. A surgical case of severe Ebstein’s anomaly, pulmonary atresia in the neonate: experience of modified Starnes operation. Kyobu Geka 1997;50:487-491.[Medline]
- Gates R.N., Laks H., Drinkwater D.C., et al. Gross and microvascular distribution of retrograde cardioplegia in explanted human hearts. Ann Thorac Surg 1993;56:410-417.[Abstract]
- Ansari A. Anatomy and clinical significance of ventricular Thebesian veins. Clin Anat 2001;14:102-110.[Medline]
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Abstract
Introduction
