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Ann Thorac Surg 2001;71:2039-2041
© 2001 The Society of Thoracic Surgeons

Case report

Staged unifocalization and anatomic repair in a patient with right isomerism

Hideki Uemura, MDa, Toshikatsu Yagihara, MDa, Youichi Kawahira, MDa, Yoshiro Yoshikawa, MDa

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan

Accepted for publication April 13, 2000.

Address reprint requests to Dr Uemura, Department of Cardiovascular Surgery, National Cardiovascular Surgery, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
e-mail: huemura{at}hsp.ncvc.go.jp


   Abstract
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 Abstract
Introduction
 Comment
 References
 
Anatomic biventricular repair was successfully achieved subsequent to bilateral unifocalizations of the pulmonary blood supply using heterologous pericardial rolls in a patient with isomeric right appendages and major aortopulmonary collateral arteries.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
Reports of successful biventricular repair in patients with isomeric right appendages have been made, particularly focusing on intracardiac rerouting and division of the common atrioventricular valve [1]. We report a successful achievement of bilateral unifocalizations of the pulmonary blood supply followed by anatomic repair in a patient having right isomerism together with major aortopulmonary collateral arteries (MAPCAs).

A female patient had abnormal arbolization of the pulmonary arteries (PA) (Fig 1A). The superior caval veins were bilaterally present. The inferior caval vein was connected to the left-sided atrium. The pulmonary veins formed the common pulmonary venous chamber [2], connected to the left-sided atrium. The morphologically right ventricle (RV) was located left-anteriorly to the morphologically left ventricle (LV), the apex pointing to the right. There were separate atrioventricular valves present without orificial obstruction nor regurgitation. The aorta arose from RV, with the PA trunk atretic. The aortic arch was located leftward to the trachea. No intrapericardial PA was identified.



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  		Fig 1. The pulmonary arteries and pulmonary perfusion. (A) Before surgical interventions, several major aortopulmonary collateral arteries were present, with the intrapericardial pulmonary arteries entirely lacking. (B) Subsequent to staged unifocalizations and definitive repair, the reconstructed pulmonary arteries had no obvious obstruction on angiography, although pulmonary perfusion scintigraphy demonstrated mild segmental hypoperfusion. (UF = unifocalization; R = right; L = left).

 
No interventions had been employed until the patient was 19 years old. Because general fatigue got worse, she decided to undergo surgical treatments. Unifocalization was initially carried out for the right intrapulmonary PA via a right thoracotomy, and followed, 2 months later, by the left-sided procedure via a left thoracotomy. To unify the intrapulmonary PA, 18 mm diameter equine pericardial roll tubes were used, and 6 mm diameter knitted Dacron (C. R. Bard, Haverhill, MA) tubes were interposed between the subclavian arteries and the rolls [3]. Postoperative courses were uneventful except for body weight loss because of severe appetite loss. Seven months after completion of unifocalizations, tuberculosis proved active within the upper lobe of the right lung. It took 6 months for the patient to be cured of tuberculosis.

Anatomic repair was established at the age of 20 years and 9 months. The atrial pectinate muscles showed an unequivocal feature of right isomerism [2]. The atrial septum was almost entirely lacking. The coronary sinus was absent. Via the left-sided atrium, a bovine pericardial baffle was placed for intraatrial redirection of blood to drain the pulmonary veins through the right-sided atrioventricular valve. A Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) patch was attached via a right ventriculotomy for intraventricular rerouting from the LV to the aorta. The aortic orifice was remote from the perimembranous inlet ventricular septal defect, the distance being greater than 30 mm. Confluence of PA was reconstructed interposing a 20 mm diameter bovine pericardial roll between the prosthetic rolls previously used for unifocalization. Reconstruction of RV outflow tract was completed using a 24 mm diameter bovine pericardial roll bearing three handmade leaflets [4]. Postoperative course was smooth.

Catheterization was carried out 13 months after repair (Table 1). Cardiac index was calculated as 2.8 L/min/m2. No significant pressure gradients were present across the ventricular outflow tracts. Extensively reconstructed PA had no obvious obstruction (Fig 1B), although mean PA pressure was 31 mm Hg. Pulmonary perfusion scintigraphy showed mild segmental hypoperfusion (Fig 1B). Consecutive echocardiography illustrated no significant obstructions across the ventricular outflow tracts and no significant regurgitation through the atrioventricular valves. With the follow-up of 6 years, the patient is currently doing well, working at an office, without medication. Exercise testing showed that anerobic threshold and maximal intake of oxygen were 14.1 and 17.7 mL/kg/min, respectively. Several episodes of nonsustained supraventricular tachyarrhythmia were noted on Holter electrocardiograph recordings.


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  		Table 1. Changes in Body Weight and Data Derived From Catheterization

 

   Comment
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References
 
In our previous morphologic study [5], abnormal arbolization of PA was present in four of 125 autopsied specimens with right isomerism (3%), and separate atrioventricular valves were identified morphologically as the tricuspid and the mitral valves in five (4%). In our clinical experiences, 134 patients with right isomerism underwent surgical interventions. Of these, 3 patients (2%) had MAPCAs, and 4 (3%) possessed the tricuspid and the mitral valves. These incidences indicate that the circumstances seen in this particular patient were extremely rare.

Malnutrition after unifocalization was obviously a clinical problem, related to tuberculosis. Presumably, the amount of pulmonary perfusion was excessive after the palliative procedures for the ventricles that had been deleteriously influenced by prolonged cyanosis and pressure overload. Exercise tolerance was considerably reduced. This might also reflect delayed definitive repair [6]. Earlier repair could have provided better functional results. This patient, nonetheless, is doing much better now than before these consecutive surgical procedures. Possible problems in the future may include obstruction and infection of the prosthetic materials used for the pulmonary circulation [4]. Arrhythmia would be another functional aspect we should pay attention to.


   References
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 Abstract
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 Comment
 References
 

  1. Uemura H., Yagihara T. Surgical treatment in patients with isomeric atrial appendages. In: Jacoub M., Carpentier A.F., eds. Annual of cardiac surgery, 10th ed. London: Rapid Science Publishers, 1997:105-112.
  2. Uemura H., Ho S.Y., Devine W.A., Kilpatrick L.L., Anderson R.H. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995;60:561-569.[Abstract/Free Full Text]
  3. Yagihara T., Yamamoto F., Nishigaki K., et al. Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1996;112:392-402.[Abstract/Free Full Text]
  4. Uemura H., Yagihara T., Kawashima T., Yamashita K., Kamiya T. Intrapulmonary reconstruction of pulmonary arteries using a heterologous pericardial roll. Ann Thorac Surg 1995;59:1464-1470.[Abstract/Free Full Text]
  5. Uemura H., Ho S.Y., Anderson R.H., Yagihara T. Ventricular morphology and coronary arterial anatomy in hearts with isomeric atrial appendages. Ann Thorac Surg 1999;67:1403-1411.[Abstract/Free Full Text]
  6. Uemura H., Yagihara T., Ishizaka T., Yamashita K. Pulmonary circulation after biventricular repair in patients with major systemic-to-pulmonary collateral arteries. Eur J Cardiothorac Surg 1997;12:581-586.[Abstract]



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