Long-Term Survival of Uhl’s Anomaly With Total Cavopulmonary Conversion
Koki Takizawa, MD,
Shoji Suzuki, MD,
Yoshihiro Honda, MD,
Shigeaki Kaga, MD,
Hidenori Inoue, MD,
Masahiko Matsumoto, MD
Department of Surgery, University of Yamanashi, Chuo-city, Yamanashi, Japan
Koki Takizawa, MD, Tel: +81 55 273 9682, Fax: +81 55 273 6767, Email: koki0610jp{at}yahoo.co.jp, University of Yamanashi, Chuo-city, Yamanashi, 409-3898, Japan.
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ABSTRACT
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We report a long-term survivor with Uhl’s anomaly who underwent one and a half ventricle repair combined with a partial right ventriculectomy in infancy, followed by successful total cavopulmonary conversion with right ventricular exclusion 5 years later. The combination of total cavopulmonary connection and right ventricular exclusion could be the optimal surgical option for a critically ill infant with Uhl’s anomaly.
Key Words: Heart Bypass • Right • Heart Defects • Congential
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INTRODUCTION
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Uhl’s anomaly is a rare cardiac disease characterized by complete absence of the right ventricular (RV) myocardium. It is very difficult to achieve long-term survival, especially in symptomatic infants. Since the original description by Uhl1 in 1952, there have been only 4 reports of successful surgical treatment in infancy (Table 1
). Ikari and colleagues2 described successful cardiac transplantation, and various right heart bypass procedures were selected in the other cases.3,5,6 We focus on the optimal surgical approach for a critically ill infant with Uhl’s anomaly.
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CASE REPORT
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A 6-year-old boy, weighing 20 kg, with Uhl’s anomaly had undergone one and a half ventricle repair combined with a partial right ventriculectomy at 9 months of age because of intractable right heart failure and low output from the left ventricle (LV) which was markedly compressed by a huge RV, with RV end-diastolic volume 378% of normal. He had shown good recovery postoperatively. However, despite remodeling the RV, it had not been functioning. We found that RV ejection could be barely maintained as an accompaniment to LV contraction. Over the years, the RV had gradually re-dilated, and the patient’s general condition had deteriorated somewhat. He had also developed severe tricuspid regurgitation. A catheter study showed hemo-dynamics similar to that of a failed classical Fontan in which the pulmonary artery, RV, and right atrium showed the same mean pressure of approximately 16 mm Hg. Therefore, we decided to perform total cavopulmonary connection (TCPC) and RV exclusion. At surgery, the previously created atrial septal fenestration of 4 mm diameter was found and closed. The tricuspid valve had a moderately dilated annulus and smaller leaflets with poor coaptation. The RV free wall was extremely thin (parchment-like). The pulmonary valve was closed from inside, the RV free wall was mostly removed (Figure 1A), and the remnant flaps were sutured together in double layers. The tricuspid valve was closed with an expanded polytetrafluoroethylene patch, and the intraatrial septum was completely resected. Finally, an extracardiac TCPC was made using an 18-mm expanded polytetrafluoroethylene graft. A fenestration, 4 mm in diameter, was created between the graft and the right atrium (Figure 1B). The postoperative course was uneventful. A follow-up catheter study at 15 months postoperatively showed acceptable mean pulmonary arterial and right atrial pressures of 10 and 7 mm Hg, respectively. During 2 years of follow-up, the patient has been doing well without any episode of arrhythmia.
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Figure 1. (A) The extremely thin right ventricular free wall was resected. (B) After exclusion of the right ventricle, a fenestrated total cavopulmonary connection was constructed.
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DISCUSSION
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Since we applied one and a half ventricle repair combined with a partial right ventriculectomy for a 9-month-old infant,3 2 other case reports of successful surgical treatment for Uhl’s anomaly in infancy have been published. Azhar and colleagues5 described surgical repair in a 5-month-old infant, using exclusion of the RV by patch closure of the tricuspid valve, atrial septectomy, and a bidirectional Glenn shunt; the patient underwent TCPC completion 3 years later. Tanoue and colleagues6 documented a 2-month-old infant who had developed critical ventricular arrhythmia related to Uhl’s anomaly. They performed one and a half ventricle repair combined with semi-total resection of the RV free wall and tricuspid annuloplasty. These reports strongly suggested that the combination of a right heart bypass operation for univentricular physiology and RV volume reduction or exclusion would be one of the surgical options and an alternative to cardiac transplantation for a critically ill infant with Uhl’s anomaly. However, the selection of the right heart bypass procedure is still a controversial issue.
When we carried out the initial operation, we believed that one and a half ventricle repair would be superior to Fontan circulation, even in Uhl’s anomaly,3 as Hoffman and colleagues4 reported the capability of the LV to generate satisfactory cardiac output when the non-contracting RV is relatively small. Unfortunately, the outcome was somewhat disappointing because of re-dilatation of the RV. Probably we should not have expected preserved RV function in a critical case of Uhl’s anomaly, even if we made a Glenn anastomosis part of one and a half ventricle repair. It is also important to recognize that the markedly dilated RV could easily lead to impairment of LV function in terms of distensibility and contractility. Exclusion of the RV, which was introduced by Sano and colleagues,7 combined with a fenestrated TCPC was certainly effective as a rescue operation in our patient. Straightforward TCPC combined with RV exclusion, skipping one and a half ventricle repair, seems to be the optimal surgical option for long-term survival.
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REFERENCES
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- Uhl HS. A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle. Bull Johns Hopkins Hosp 1952; 91:197–209.[Medline]
- Ikari NM, Azeka E, Aiello VD, Atik E, Barbero-Marcial M, Ebaid M. Uhl’s anomaly. Differential diagnosis and indication for cardiac transplantation in an infant. Arq Bras Cardiol 2001;77:69–76.[Medline]
- Yoshii S, Suzuki S, Hosaka S, Osawa H, Takahashi W, Takizawa K, et al. A case of Uhl anomaly treated with one and a half ventricular repair combined with partial right ventriculectomy in infancy. J Thorac Cardiovasc Surg 2001; 122:1026–8.[Free Full Text]
- Hoffman D, Sisto D, Frater R, Nikolic S. Left-to-right ventricular interaction with a noncontracting right ventricle. J Thorac Cardiovasc Surg 1994;107:1496–502.[Abstract/Free Full Text]
- Azhari N, Assaqqat M, Bulbul Z. Successful surgical repair of Uhl’s anomaly. Cardiol Young 2002;12:192–5.[Medline]
- Tanoue Y, Kado H, Shiokawa Y. Uhl’s anomaly complicated with critical ventricular arrhythmia in a 2-month-old infant. Eur J Cardiothorac Surg 2003;24:1040–2.[Abstract/Free Full Text]
- Sano S, Ishino K, Kawada M, Kasahara S, Kohmoto T, Takeuchi M, et al. Total right ventricular exclusion procedure: An operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002;123:640–7.[Abstract/Free Full Text]
Asian Cardiovasc Thorac Ann 2009;
17:203-205
© 2009 by SAGE Publications
DOI: 10.1177/0218492309103328
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ABSTRACT
INTRODUCTION
