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Ann Thorac Surg 2001;72:2178-2179
© 2001 The Society of Thoracic Surgeons


Update

Update 2001: Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair"

Hiroo Takayama, MD*a, Akihiko Sekiguchi, MDa, Masahide Chikada, MDa

a Division of Cardiovascular Surgery, National Children’s Hospital, Tokyo, Japan

* Address reprint requests to Dr Takayama, Division of Cardiovascular Surgery, National Children’s Hospital, 3-35-31 Taishido Setagaya-ku, Tokyo 154-8509, Japan
e-mail: hirofu2{at}hotmail.com

Abstract

As Originally Published in 1995


One and a half ventricular repair can be applied to a small or poorly functioning right heart, but the long-term results of this operation remain unclear. Between 1982 and 1984, we successfully performed this procedure on 3 patients with pulmonary atresia with intact ventricular septum. In 1995, we reported the short-term and intermediate-term follow-up of these 3 patients [1]. In 2001, the follow-up period ranges from 17 to 19 years, and the present age of patients ranges from 20 to 27 years old.

All patients remain in New York Heart Association functional status class I. Oxygen saturation ranges from 91% to 96%. Exercise treadmill tests show relatively good functional capacities: 70.3%, 96.4%, and 110.7% of normal capacity. Exercise electrocardiograms show supraventricular arrythmias (paroxysmal atrial contraction and paroxysmal atrial fibrillation) in 2 patients, and depression of the ST segment in 1 patient. One patient developed paroxysmal atrial flutter, which was treated with electrocardioversion and anti-arrhythmic medication. No other patient takes any medication.

Echocardiograms at the last follow-up demonstrated good right and left ventricular function. The Z scores of the TV diameters increased from preoperatively -6.5, -5.2, and -5.2 to -3.2, -2.6, and -1.0, respectively. Right ventriculograms showed increased right ventricular end-diastolic volume. These findings indicate that antegrade flow maintained by one and a half ventricular repair contributes to growth of the right ventricle. Echocardiograms demonstrate mild tricuspid regurgitation in only 1 patient, suggesting that enlargement of the right ventricles is due to growth rather than pathological dilation. However, development of supraventricular arrhythmias may imply subclinical dysfunction of these ventricles.

All patients have pulmonary insufficiency. In the presence of pulmonary insufficiency, right ventricular systolic function and end-diastolic volume are less likely to be normal, and are likely to correlate with the amount of regurgitation [2]. Persistent pulmonary regurgitation may result in right ventricular dysfunction, subsequent elevation of right ventricular end-diastolic volume, dilation of the right atrium, and development of the supraventricular arrhythmias.

Right pulmonary arteriograms confirm development of pulmonary arteriovenous fistulas in 2 patients. It is now established that pulmonary arteriovenous fistula can develop long after a classical Glenn shunt [3], and use of this procedure is outdated. Kreutzer and colleagues suggest creating right pulmonary artery banding with bidirectional Glenn shunt in one and a half ventricular repair [4].

In conclusion, we consider the long-term results of one and a half ventricular repair for pulmonary atresia with intact ventricular septum almost acceptable, although the right ventricles develop mild dysfunction over term. Late complications include supraventricular arrhythmias and right-sided pulmonary arteriovenous fistula. Several op-erative modifications should be employed for better outcome.

References

  1. Miyaji K., Shimada M., Sekiguchi A., Ishizawa A., Isoda T., Tsunemoto M. Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair". Ann Thorac Surg 1995;60:1762-1764.[Abstract/Free Full Text]
  2. Redington A.N., Oldershaw P.J., Shinebourne E.A., Rigby M.L. A new technique for the assessment of pulmonary regurgitation and its application to the assessment of right ventricular function before and after repair of tetralogy of Fallot. Br Heart J 1988;60:57-65.[Abstract/Free Full Text]
  3. McFaul R.C., Tajik A.J., Mair D.D., Danielson G.K., Seward J.B. Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis. Report of four cases. Circulation 1977;55:212-216.[Abstract/Free Full Text]
  4. Kreutzer C., Mayorquim R.C., Kreutzer G.O., et al. Experience with one and a half ventricle repair. J Thorac Cardiovasc Surg 1999;117:662-668.[Abstract/Free Full Text]




This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
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Right arrow Author home page(s):
Hiroo Takayama
Akihiko Sekiguchi
Right arrow Permission Requests
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Right arrow Articles by Takayama, H.
Right arrow Articles by Chikada, M.
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Related Collections
Right arrow Congenital - cyanotic


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