Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum

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1 Mid-term Result of One and One Half Ventricular Repair in a Patient with Pulmonary Atresia and Intact Ventricular Septum Kagami MIYAJI, MD, Akira FURUSE, MD, Toshiya OHTSUKA, MD, and Motoaki KAWAUCHI, MD SUMMA A 10-year-old girl was referred to us with a diagnosis of pulmonary atresia and intact ventricular septum with a severely hypoplastic right ventricle and atrial septal defect. Her tricuspid valve diameter (TVD) was 48.5% of normal, with a TVD Z-score of -6.7, while right ventricular end-diastolic volume was 53.4% of normal. As a definitive procedure, one and one half ventricular repair using a bidirectional cavopulmonary shunt (BOPS) was performed instead of a Fontan procedure. The postoperative course was uneventful without episodes of low cardiac output or elevated right atrial pressure. She has been doing well during a follow-up period of 3 years. (Jpn Heart J 1996; 37: ) Key words: Pulmonary atresia and intact ventricular septum Hypoplastic right ventricle Z-score of tricuspid valve diameter Fontan procedure ULMONARY 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 their hypoplastic right ventricles. In 1993, Hanley and colleagues1) established that the Z score of the tricuspid valve diameter (TVD) correlates well with the ability to perform a biventricular repair in patients with PA: IVS. The Blalock-Taussig shunt or the Fontan procedure have been used previously as a definitive procedure for patients with severely hypoplastic right ventricles. Recently, "one and one half ventricular repair"2) has been advocated for this group of patients. This procedure allows superior vena caval blood to flow directly into the pulmonary artery, while the hypoplastic right ventricle pumps inferior vena caval blood through the right ventricular outflow tract also to the pulmonary artery. The concept of "one and one half ventricular repair" is the incorporation of a small right ventricle into From the Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan. Address for correspondence: Kagami Miyaji, MD, Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, T-3-1 Hongo, Bunkyo-ku, Tokyo 113, Japan. Received for publication March 25, Accepted April 15,

2 510 MIYAJI ET AL Jpn Heart J July 1996 the pulmonary circulation. Here, we report a case of successful "one and one half ventricular repair" using a bidirectional cavopulmonary shunt (BCPS) for a patient with PA: IVS and a severely hypoplastic right ventricle. CASE REPORT A 10-year-old girl was referred to us with a diagnosis of PA: IVS with a severely hypoplastic right ventricle and atrial septal defect (ASD). She had undergone pulmonary valvotomy and aortopulmonary shunt at the age of 2 months. At 5 years of age, the tricuspid valve diameter (TVD) had been 39.1% of normal, the Z-value -7.8, and the right ventricular end-diastolic volume (RVEDV) 34.7% of normal. Right ventricular outflow reconstruction using an equine pericardial patch and a partial biventricular repair using a snared adjustable ASD3) was performed. However, ASD closure could not be performed because of elevated right atrial pressure (greater than 18mmHg). Cardiac catheterization at 10 years of age revealed that the right ventricle was still severely hypoplastic, the TVD was 48.5% of normal, and the Z-value However, the RVEDV had increased significantly from 34.7% to 53.4% of normal because of mild pulmonary valve regurgitation (Table). The pulmonary to systemic blood flow ratio was 0.61, the mean pulmonary artery pressure 11mmHg and the pulmonary vascular resistance 2.2 units Em2. As a definitive operation in the presence of the small TVD, one and one half ventricular repair using a BCPS was performed instead of a Fontan procedure. Under cardiopulmonary bypass, the superior vena cava was divided and anastomosed end-to-side to the right pulmonary artery, and the ASD was closed. The postoperative course was uneventful without episodes of Table. Patient Data RVOTR=right ventricular outflow tract reconstruction; 1 and 1/2 ventricular repair=one and one half ventricular repair; TVD=tricuspid valve diameter; RVEDV=right ventricular end-diastolic volume; RVEP=right ventricular ejection fraction; RVEDP=right ventricular end-diastolic pressure; Qp/ Qs=pulmonary to systemic blood flow ratio; Rp=pulmonary vascular resistance; SaO2=arterial oxygen saturation.

3 Vol 37 No 4 ONE AND ONE HALF VENTRICULAR REPAIR 511 Figure. Representative wave tracing from cardiac catheterization 3 years after one and one half ventricular repair. Pulsatile flow tracings in the left and right pulmonary arteries proved the significant contribution of right ventricular contraction to pulmonary blood flow. A: Superior vena caval pressure wave. B: Right pulmonary arterial pressure wave. C: Main pulmonary arterial pressure wave. D: Right ventricular pressure wave. low cardiac output or elevated right atrial pressure. The patient has since been doing well during follow-up, and has maintained New York Heart Association functional class 1 status. Repeat cardiac catheterization was performed 3 years after the one and one half ventricular repair. This revealed that the TVD had increased from 48.5% to 61.4% of normal, the Z-value from -6.7 to -5.6 and the RVEDV increased from 53.4% to 64.1% of normal (Table). The right ventricular ejection fraction (RVEF) had decreased slightly from 0.54 to Pulsatile flow could be seen in the right and main pulmonary arteries during the catheterization (Figure). DISCUSSI Various guidelines for deciding the most appropriate definitive repair for patients with PA: IVS and hypoplastic right ventricle have been reported. In 1985, de Leval and colleagues4) reported a revised system of classification using the tripartite morphology of the right ventricle and the size of the tricuspid valve to help select the optimal repair for PA: IVS. In 1993, Hanley and colleagues1)

4 512 MIYAJI ET AL Jpn Heart J July 1996 established that the values of the Z scores of TVDs correlate well with the ability to perform a biventricular repair in patients with PA: IVS. For patients with severely hypoplastic right ventricles, and whose TVDs are less than the 99% CL, a Blalock-Taussig shunt or, if possible, the Fontan procedure, had been advocated previously as a definitive procedure.5) In 1982, Utsunomiya and colleagues reported the first one and one half ventricular repair using a classical Glenn shunt for a patient with a TVD less than 66% of normal.6) Recently, it was reported that the long-term results over 10 years for 3 patients who underwent this procedure were acceptable, although a pulmonary arteriovenous fistula developed in one of them.7,8) Since 1985, a number of reports have emphasized the utility of BCPS, which is an end-to-side anastomosis between the superior vena cava and the undivided right pulmonary artery, for cyanotic congenital heart disease.9,10) It is now well known that pulmonary arteriovenous fistulae can develop long after a classical Glenn shunt.11,12) Therefore, one and one half ventricular repair using BCPS should be performed for this group of patients. Billingsley and colleagues13) reported that BCPS has been applied successfully for definitive repair of PA: IVS. Gentles and colleagues14) also reported excellent results of one and one half ventricular repair using BCPS and atriopulmonary anastomoses for 6 patients with PA: IVS and hypoplastic right ventricles whose TVD Z values ranged from -2.0 to While the lower limits of TVD for one and one half ventricular repair using cavopulmonary anastomosis have not yet been determined, we successfully performed this repair in a patient with a hypoplastic right ventricle with a TVD Z-score of less than The long-term results of one and one half ventricular repair using BCPS have not yet been reported. However, the present case shows that the mid-term outcome of this procedure up to 3 years is acceptable. REFERENCES 1. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcome in neonatal pulmonary atresia with intact ventricular septum. A multi-institutional study. J Thorac Cardiovasc Surg 1993; 105: Jonas RA. Letter to the editor. J Thorac Cardiovasc Surg 1993; 105: Laks H, Pearl JM, Drinkwater DC, et al. Partial biventricular repair of pulmonary atresia with intact ventricular septum. Use of an adjustable atrial septal defect. Circulation 1992; 86 (Suppl 2): de Leval MR, Bull C, StarkJ, Anderson RH, TaylerJFN, Macartney FJ. Pulmonary atresia and intact ventricular septum. Surgical management based on a revised classification. Circulation 1982; 66: 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 II): 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: Miyaji K, Shimada M, Sekiguchi A, et al. One and one-half ventricular repair for pulmonary atresia

5 Vol 37 No 4 ONE AND ONE HALF VENTRICULAR REPAIR 513 and critical pulmonary stenosis with intact septum. Ann Thorac Cardiovasc Surg 1995; 5: 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: Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham HN. Physiological rationale for a bidirectional cavo-pulmonary shunt: a versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 1985; 90: Mazzera E, Corno A, Picardo S, et al. Bidirectional cavopulmonary shunt: clinical applications as staged or definitive palliation. Ann Thorac Surg 1989; 47: 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: Cloutier A, Ash JM, Smallhorn JF, et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure. Risk of development of arteriovenous fistulae. Circulation 1985; 72: 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: Gentles TL, Keane JF, Jonas RA, Marx GE, Mayer JE. Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle. Circulation 1994; 90 [part 2]: II-2-6.