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Ann Thorac Surg 1995;60:1762-1764
© 1995 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Pulmonary Atresia With Intact Ventricular Septum: Long-Term Results of ``One and a Half Ventricular Repair''

Departments of Cardiovascular Surgery and Pediatric Cardiology, National Children's Hospital, Tokyo, Japan

Accepted for publication August 8, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. Between 1982 and 1984, we successfully performed ``one and a half ventricular repair'' using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results.

Methods. In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years postoperatively.

Results. All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient.

Conclusions. Although the lower limits of the tricuspid valve diameter for ``one and a half ventricular repair'' using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Pulmonary atresia with intact ventricular septum (PA + IVS) is an uncommon congenital cardiac anomaly, and only a minority of patients undergo successful primary biventricular repair because of hypoplastic right ventricles (RVs). From 1982 to 1984, 3 patients whose tricuspid valve diameters (TVDs) were less than 66% of normal and whose tricuspid valve to systemic (QsRV) blood flow ratios [1] were less than 50% underwent a so-called one and a half ventricular repair using a Glenn shunt at National Children's Hospital, Tokyo. We review the 10-year results of the repair in patients with PA + IVS and severely hypoplastic RVs.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Patient Population
Three patients were diagnosed as having PA + IVS, patent ductus arteriosus, and atrial septal defect (Table 1). Previous palliative operations included a pulmonary valvotomy in all 3 patients and an aortopulmonary shunt in 1 patient because of closure of the patent ductus arteriosus. Age at palliation ranged from 1 month to 4 months. The follow-up from palliation to definitive operation ranged from 2 to 8 years.

In our series, the TVDs just prior to one and a half ventricular repair ranged from 51.1% to 56.0% of normal, less than 66% of normal in all 3 patients. The right ventricular end-diastolic volumes ranged from 30.3% to 37.4% of normal. The percent QsRV flows in 2 patients were 46.0% and 47.5% and hence, less than 50%. The Z score of the TVDs in all patients, calculated as described by Hanley and colleagues [5] in 1993, ranged from -5.2 to -6.5, indicating severely hypoplastic RVs.

Surgical Procedures and Postoperative Courses
Because of the presence of the severely hypoplastic RV in each patient, we decided to perform a one and a half ventricular repair instead of biventricular repair. Under cardiopulmonary bypass, the superior vena cava was divided and anastomosed end-to-end to the right pulmonary artery, and the azygos veins were ligated. Right ventricular outflow tract reconstruction was performed using equine pericardial patches, and the atrial septal defects were closed. The patent ductus arteriosus in 2 patients and the aortopulmonary shunt in the other patient were ligated.

There were no operative deaths. All 3 patients had an uneventful postoperative course without episodes of low cardiac output or elevated right atrial pressure.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
No patient died during the 10-year follow-up, and all have maintained New York Heart Association functional class I status. Cardiac catheterization was performed in all 3 patients at least 10 years after one and a half ventricular repair. In patient 1, right ventricular angiograms could not be obtained because of bilateral femoral vein occlusions, and therefore, the postoperative TVD and right ventricular end-diastolic volume could not be determined. The TVDs of patients 2 and 3 increased from 52.5% to 74.8% of normal and from 56.0% to 71.9% of normal, respectively. The Z scores and the right ventricular end-diastolic volumes also improved in these 2 patients (see Table 1). The arterial oxygen saturation of patients 1 and 2 remained 96.3% and 94.3%, respectively, but in patient 3, it had decreased from 95.4% to 89.2% 10 years after repair. A pulmonary arteriovenous fistula was detected in this patient on the right pulmonary arteriograms.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Various guidelines for deciding on the appropriate definitive repair for patients with PA + IVS and hypoplastic RVs have been reported. In 1985, de Leval and colleagues [6] presented a revised classification system using the tripartite morphology of the RV and the tricuspid valve size to help select the optimal repair of PA + IVS. In 1993, Hanley and co-workers [5] established that the value of the Z score of the TVD correlates well with the ability to perform a biventricular repair in patients with PA + IVS.

Since 1982, we have chosen the definitive surgical procedure for patients with PA + IVS and hypoplastic RVs on the basis of the TVDs and percent QsRV flows. Patients with TVDs greater than 66% of normal and percent QsRV flows greater than 50% have undergone biventricular repair. In 1982, we successfully performed one and a half ventricular repair using a Glenn shunt for a patient with a TVD of less than 66% of normal and a percent QsRV flow of less than 50% [1]. By 1984, 2 additional patients had undergone this repair and by 1990, 1 more patient.

The Z values of the TVDs in the 3 patients seen between 1982 and 1984 ranged from -5.2 to -6.5. For patients with such severely hypoplastic RVs, a Blalock-Taussig shunt or, if possible, a Fontan procedure had been used as a definitive procedure. In 1989, Billingsley and colleagues [7] reported that a bidirectional Glenn shunt had been applied successfully in the definitive repair of PA + IVS. Last year, Gentles and associates [8] reported excellent results of one and a half ventricular repair using cavopulmonary and atriopulmonary anastomoses in 6 patients with PA + IVS and severely hypoplastic RVs with TVD Z scores ranging from -2.0 to -3.5.

The long-term results of one and a half ventricular repair using cavopulmonary shunts have not yet been reported. To address this issue, we performed cardiac catheterization in 3 patients and right ventricular angiography in 2 of the 3 patients at least 10 years after one and a half ventricular repair. In the latter 2 patients, these studies confirmed that there is sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction without elevated right ventricular end-diastolic pressures. The Z scores of the TVDs increased over the 10 years. A pulmonary arteriovenous fistula has developed in 1 of these patients. It is now established that pulmonary arteriovenous fistula has developed long after a Glenn shunt [9]. Therefore, we believe one and a half ventricular repair using a bidirectional Glenn shunt should be performed for this group of patients.

The lower limits of the TVD for one and a half ventricular repair using a cavopulmonary shunt have not yet been determined. Nevertheless, we successfully performed this repair in patients with severely hypoplastic RVs with TVD Z scores lower than -5.0, and the 10-year results have been acceptable.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr Miyaji, Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo 113, Japan.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

1. Utsunomiya H, Tsunemoto M, Ohta Y, et al. 2-step operation of pulmonary atresia with intact ventricular septum and hypoplastic right ventricle. Nippon Kyobu Geka Gakkai Zasshi 1983;31:2209–13.[Medline]
2. Rowlatt UF, Rimoldi HJA, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963;10:499–588.
3. Bull C, de Leval MR, Mercanti C, Macartney FJ, Anderson RH. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation 1982;66:266–72.[Abstract/Free Full Text]
4. Graham TP, Jarmakani JM, Atwood GF, Canent RV Jr. Right ventricular volume determination in children. Circulation 1973;47:144–53.[Abstract/Free Full Text]
5. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcome in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993;105:406–27.[Abstract]
6. De Leval MR, Bull C, Hopkins R, et al. Decision making in the definitive repair of the heart with a small right ventricle. Circulation 1985;72(Suppl 2):52–60.
7. Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1989;97:746–54.[Abstract]
8. Gentles TL, Keane JF, Jonas RA, Marx GE, Mayer JE. Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle. Circulation 1994;90(Suppl 2):2–6.[Free Full Text]
9. McFaul RC, Tajik AJ, Mair DD, Danielson GK, Seward JB. Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis-Report of four cases. Circulation 1977;55:212–6.[Abstract/Free Full Text]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Kagami Miyaji Akihiko Sekiguchi Minoru Tsunemoto Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Miyaji, K. Articles by Tsunemoto, M. Search for Related Content PubMed PubMed Citation Articles by Miyaji, K. Articles by Tsunemoto, M.