Eur J Cardiothorac Surg 2003;24:1040-1042
© 2003 Elsevier Science NL
Uhl's anomaly complicated with critical ventricular arrhythmia in a 2-month-old infant
Yoshihisa Tanoue,
Hideaki Kado*,
Yuichi Shiokawa
Department of Cardiovascular Surgery, Fukuoka Children's Hospital Medical Center, 2-5-1 Tojin-machi, Chuo-ku, Fukuoka 810-0063, Japan
Received 21 June 2003;
received in revised form 13 September 2003;
accepted 16 September 2003.
* Corresponding author. Tel.: +81-92-713-3111; fax: +81-92-713-3122
e-mail: kado.h{at}fukuoka-child.jp
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Abstract
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We report a case of Uhl's anomaly in a 2-month-old infant who presented with critical ventricular arrhythmia. The patient underwent a successfully surgical repair by semitotal resection of the right ventricle free wall, tricuspid annuloplasty, and one and one-half ventricular repair.
Key Words: Uhl's anomaly • Ventricular arrhythmia • One and one-half ventricular repair
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1. Introduction
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Uhl's anomaly, a very rare anomaly with unknown etiology, is characterized by congenital hypoplasia of the right ventricular myocardium [1,2]. The prognosis in the case of an infant is uniformly poor; though cases surviving surgery have been reported recently [3–6]. We report a 2-month-old infant undergoing surgical treatment for Uhl's anomaly with critical ventricular arrhythmia. The infant underwent semitotal resection of the right ventricular free wall, tricuspid annuloplasty, and a one and one-half ventricular repair.
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2. Case report
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A 51-day-old boy was admitted to our hospital for progressive cyanosis and hepatomegaly. Chest radiography showed cardiomegaly (cardiothoracic ratio, 65%). Echocardiography showed that the right ventricle and atrium were enlarged and the right ventricular free wall was thin and akinetic. Cardiac catheterization was performed urgently 2 days after admission. Pulse waves of right ventricular pressure and pulmonary arterial pressure were almost flat at 9 mmHg. Right ventriculography demonstrated an enlarged and akinetic right ventricle (Fig. 1A)
. Nitric oxide inhalation therapy was started due to progressive cyanosis, which was alleviated and so the patient's general status also improved. However, multiple ventricular premature contractions and ventricular tachycardia begun to occur frequently at 65 days of age. Medical antiarrhythmic therapy was not effective, and the patient was urgently operated on at 72 days when weighing 5.1 kg.
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Fig. 1. Right ventriculography before and after operation. (A) Before operation. (B) 21 days after operation. (C) 14 months after operation.
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A standard median sternotomy was performed. The right ventricle was found to be markedly dilated, and its free wall extremely thin and hardly contracting; suggestive of fibrous change from the white surface. The right atrium was also dilated. Cardiopulmonary bypass was quickly instituted due to the high frequency of ventricular arrhythmia. A left ventricular venting tube was inserted through the right upper pulmonary vein. After cross-clamping the ascending aorta, cardiac arrest was achieved by cold crystalloid cardioplegia [7,8]. Through a right atriotomy, a small atrial septal defect and a dilated tricuspid annulus were observed. The free wall of the right ventricular outflow was resected in a triangular fashion and sutured in linearly. The anterior papillary muscle of the tricuspid valve was preserved. The right ventricular outflow was plicated to 10 mm diameter; and DeVega's tricuspid annuloplasty (12 mm) was performed. The aortic cross-clamping was released after the direct closure of the atrial septal defect, and then the right atrium was plicated and closed. The superior vena cava was divided and a bidirectional cavopulmonary shunt made by direct end-to-side anastomosis between the superior vena cava and the right pulmonary artery. Weaning from cardiopulmonary bypass was smooth under conditions of nitric oxide inhalation. Mean radial arterial pressure was around 60 mmHg and right atrial pressure was 8 mmHg. We decided at this point that fenestration was unnecessary. The patient was extubated 1 h after the operation in the intensive care unit.
Histological examination of the resected right ventricular free wall revealed irregular disappearance of cardiac muscle bundles and replacement by fibrous tissue. There was no fatty or fibrofatty tissue, which is a morphological feature of arrhythmogenic right ventricular dysplasia [9]. Pulse waves of right ventricular pressure and pulmonary arterial pressure appeared on postoperative cardiac catheterization. Right ventricular pressure was 13/3 mmHg, and pulmonary arterial pressure was 9/4 mm Hg (mean 6 mmHg). Right ventriculography showed the reduction of the right ventricle (Fig. 1B). Ventricular arrhythmia was recorded once after operation. Medical follow-up continues, and chest radiography, electrocardiogram, and echocardiography are performed periodically. Angiotensin converting enzyme inhibitor (imidapril hydrochloride) and an antiarrhythmic agent (amiodarone hydrochloride) are administered to prevent the dilatation of the right ventricle and recurrence of arrhythmia. The patient continues to do well 16 months after the operation without recurrence of arrhythmia. Most recently echocardiography and cardiac catheterization data showed one and one half ventricular repair circulation was in a stable condition. The inferior vena cava was not dilated and right atrial pressure was low (2 mmHg); right ventricular pressure was 12/2 mmHg, and pulmonary arterial pressure was 9/4 mmHg (mean 6 mmHg). Right ventriculography showed there was no dilatation of the right ventricle (Fig. 1C).
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3. Discussion
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The operation in this case was performed urgently when the patient was 2 months old due to uncontrollable ventricular arrhythmia. Complete exclusion of the free wall of the right ventricle would be necessary to prevent recurrence of ventricular arrhythmia; and the total exclusion of the right ventricle means the creation of a single ventricle. In the present case, we performed semitotal resection of the right ventricular outflow free wall in hopes of the improving right ventricular contractility. The plication of the right ventricular outflow and tricuspid annuloplasty effectively recovered the right ventricular contraction, which was manifested by the emergence of right ventricular pressure waves after the operation. If ventricular arrhythmia remained, total exclusion of the right ventricle and a bidirectional Glenn shunt would had to have been performed. One and one-half ventricular repair was seen to be the best operation in the circumstances because a Fontan procedure could not be performed because of the patient's age.
Recently, Azhari and associates have reported the surgical repair of 5-month-old infant with a Uhl anomaly, who underwent exclusion of the right ventricle by tricuspid valve patch closure and a bidirectional Glenn shunt [3]. Yoshii and colleagues documented the surgical treatment of 9-month-old infant with Uhl's anomaly, who underwent one and one-half ventricle repair combined with partial right ventriculectomy [4]. They made a 4 mm atrial septal fenestration; whereas we completely closed the atrial septal defect. A fenestration would have been made if the right atrial pressure had elevated. These three reports of successful surgical repair of Uhl's anomalies are becoming common with the application of right heart bypass operations, which are part of one of the most progressive fields in cardiac surgery; this experience should contribute to the surgical management of Uhl's anomaly.
Residual right ventricular free wall has the possibility of recurrence of ventricular arrhythmia and right ventricular dilatation. If a critical ventricular arrhythmia and right ventricular dilatation occur again, the residual right ventricular free wall would need to be excluded completely, and a total cavopulmonary connection performed [10]. However, a relatively good prognosis regarding arrhythmia is expected because this case is not one of arrhythmogenic right ventricular dysplasia.
A right heart bypass operation would be an effective and promising adjunct to treat Uhl's anomaly. Surgical intervention for Uhl's anomaly is not so much of a challengeable anymore, but is a recommendable operation, although long-term follow-up and further investigations are necessary.
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References
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Abstract
1. Introduction
