J Thorac Cardiovasc Surg 2005;130:1207-1208
© 2005 The American Association for Thoracic Surgery
One-stage definitive repair of pulmonary atresia with intact ventricular septum and hypoplastic right ventricle
Takeshi Shinkawa, MD
*
,
Masaaki Yamagishi, MD,
Keisuke Shuntoh, MD,
Keitarou Koushi, MD,
Takahiro Hisaoka, MD,
Hitoshi Yaku, MD
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Received for publication May 12, 2005; accepted for publication June 7, 2005.
* Address for reprints: Takeshi Shinkawa, MD, Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamigyo-ku, Kyoto, 602-8566 Japan. (Email: newriver{at}koto.kpu-m.ac.jp).
The right ventricle (RV) overhaul technique is an effective method for enlarging the RV cavity on the way to biventricular repair for pulmonary atresia and intact ventricular septum with hypoplastic RV. We describe two successful cases of one-stage definitive repair with the RV overhaul technique in early infancy.
Clinical Summaries
The first case was that of a 1-month-old boy weighing 3.3 kg. His preoperative right ventriculogram showed tripartite RV with end-diastolic volume 50% of the normal value,
1
a tricuspid valve with annular diameter 67% of the normal value (Z value –2.2),
2
and minor sinusoidal communications. The second case was that of a 1-month-old girl weighing 3.1 kg. Her preoperative right ventriculogram showed tripartite RV with end-diastolic volume 67% of the normal value, a tricuspid valve with annular diameter 101% of the normal value (Z value +0.3), and no sinusoidal communication (Figures 1, A, and 2, A). The pulmonary circulation was ductus dependent, and the intervention to perforate atretic pulmonary valve was unsuccessful in both cases.
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Figure 1. Preoperative (A) and postoperative (B) right ventriculograms from case 1 showing enlarged RV (126% normal end-diastolic volume) and grown tricuspid valve (79.3% normal annular size).
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Figure 2. Preoperative (A) and postoperative (B) right ventriculograms from case 2 showing enlarged RV (128% normal end-diastolic volume) and competent tricuspid valve (80.0% normal annular size).
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In both cases cardiopulmonary bypass was established through a median sternotomy and the ductus was divided. Severe tricuspid valvular dysplasia was not detected in either case. In case 1, the pulmonary arterial incision was extended to the ventricular outflow portion because the pulmonary annular diameter was too small. In case 2, pulmonary valvotomy was performed because the pulmonary annular diameter was of approximately normal size. The hypertrophied muscles in the outflow and the trabecular portions were resected as possible (RV overhaul). In case 1, the outflow tract incision was patched with a monocuspid pericardial patch. A purse-string suture was placed around the foramen ovale and extended to the outside of the heart. After weaning from bypass, the foramen ovale was closed after test snaring of the purse-string suture. Postoperative right ventriculograms showed competent tricuspid valves and adequately enlarged right ventricles (Figures 1, B, and 2, B). The right atrial pressures were 11 and 7 mm Hg in cases 1 and 2, respectively.
Discussion
Most patients with pulmonary atresia and intact ventricular septum with a tripartite RV and an absence of RV-dependent coronary circulation are initially treated with pulmonary valvotomy, irrespective of RV volume.
3,4
However, it has been reported that the tricuspid valve diameter does not change after the initial valvotomy,
4
and the size of the tricuspid valve would be a risk factor for not receiving biventricular repair.
2
We have therefore formulated a new strategy, consisting of the RV overhaul technique and concomitant RV outflow tract reconstruction in early infancy to achieve greater tricuspid valve and RV development for patients who cannot undergo successful catheter intervention. We believe that the postoperative tricuspid valve and RV can receive more venous return and have greater growth potential with this strategy than with simple valvotomy. If vital signs became unstable after test snaring of the interatrial communication, the communication should be left open. This strategy seems to be appropriate for patients with tripartite RV and tricuspid annulus larger than 60% of normal value without severe valvular dysplasia.
References
- Rowlatt UF, Rimoldi HJ, Lev M. The quantitative anatomy of the normal child's heart. Pediatr Clin North Am 1963;10:499-588.
- Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993;103:406-427.
- Pawade A, Capuani A, Penny DJ, Karl TR, Mee RB. Pulmonary atresia with intact ventricular septum. surgical management based on right ventricular infundibulum. J Card Surg 1993;8:371-383.[Medline]
- Sano S, Ishino K, Kawada M, Fujisawa E, Kamada M, Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg 2000;70:1501-1506.[Abstract/Free Full Text]
