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Objectives We compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. Methods This retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan–Meier method. Results The hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight Conclusions Postoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.

Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth.A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm.Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation.Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model.We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151).The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0.The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

Objective The Damus–Kaye–Stansel operation sometimes results in deteriorating semilunar valve insufficiency. We verified the semilunar valve function after the Damus–Kaye–Stansel operation and compared the end-to-side Damus–Kaye–Stansel with the double-barrel Damus–Kaye–Stansel. Methods Forty-seven patients who underwent the Damus–Kaye–Stansel operation between June 1993 and August 2008 were retrospectively reviewed. Any patient who underwent a Norwood-type operation was excluded. The median age at operation was 19 months (range, 0–276 months). Forty-five patients were Fontan candidates. Thirty-nine patients underwent pulmonary artery banding before the Damus–Kaye–Stansel operation. Twenty-two patients had undergone an arch repair previously. The semilunar valve function was evaluated by echocardiography. Results Thirteen patients underwent the end-to-side Damus–Kaye–Stansel operation, and 34 patients underwent the double-barrel Damus–Kaye–Stansel operation. The mean follow-up period was 71 ± 50 months (range, 1–188 months). Although there were 4 deaths, no death was related to the Damus–Kaye–Stansel procedure. Two of the patients with early death could not undergo a postoperative evaluation of the semilunar valves. The semilunar valve regurgitation mildly deteriorated in 7 patients (pulmonary regurgitation in 5 patients and aortic regurgitation in 2 patients). Pulmonary regurgitation deteriorated from none to mild in 1 patient, none to trivial in 2 patients, and trivial to mild in 2 patients. Both deteriorations in aortic regurgitation ranged from none to trivial. Semilunar valve regurgitation did not affect patients' circulatory condition. The end-to-side Damus–Kaye–Stansel operation more frequently caused a deterioration in pulmonary regurgitation than the double-barrel Damus–Kaye–Stansel operation (4/11 vs 1/34, P = .001). No surgical intervention for a systemic ventricular outflow obstruction was observed in the follow-up period. Conclusions The double-barrel Damus–Kaye–Stansel operation was found to be superior to the end-to-side Damus–Kaye–Stansel operation for the prevention of postoperative pulmonary regurgitation.

Objective Tricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with intact ventricular septum. Lack of right ventricle–pulmonary artery continuity is associated with poor tricuspid valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth of the tricuspid valve after establishment of right ventricle–pulmonary artery continuity. Methods We studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter (Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with the changes of Z-values (Zt2 − Zt1) were analyzed. Results The mean initial tricuspid Z-value (Zt1) was −6.2 ± 3.5. After treatment (Zt2), the mean Z-value was −6.0 ± 3.4 (n = 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 − Zt1) was larger than +2 in 11 (32%) patients and smaller than −2 in 6 (18%) patients. Increases in Z-value (Zt2 − Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured after initial palliation ( r = −0.54; P = .001) and the initial tricuspid valve Z-value (Zt1) ( r = −0.40; P = .02). Conclusions Disproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery.

Objective The aim of this study was to evaluate right and left ventricular functions in patients with pulmonary arterial hypertension after living-donor lobar lung transplantation compared with those without hypertension. Methods Thirty-three recipients of living-donor lobar lung transplantation were divided into two groups: those with pulmonary arterial hypertension (PAH group; n=12) and those without (non-PAH group; n=21). Their systolic pulmonary artery pressure was 93.1 ± 6.7 mm Hg versus 31.4 ± 2.9 mm Hg, respectively. Right and left ventricular ejection fractions, systolic pulmonary artery pressure, and cardiac index were serially measured by radionuclide ventriculography and right heart catheterization, respectively. Results Pretransplant right and left ventricular ejection fractions were lower in the PAH group (29.8% ± 7.0%, 49.9% ± 6.6%) than in the non-PAH group (49.7% ± 3.3%, 65.2% ± 1.9%) ( P = .010, .068). Two months after living-donor lobar lung transplantation, right ventricular ejection fraction and systolic pulmonary artery pressure in the PAH group (57.3% ± 5.1%, 25.7 ± 1.8 mm Hg) improved dramatically, equal to those in the non-PAH group. In contrast, left ventricular ejection fraction and cardiac index in the PAH group (50.9% ± 3.7%, 2.66 ± 0.12 L · min −1 · m −2 ) were still significantly lower than in the non-PAH group (65.4% ± 2.8%, 3.13 ± 0.15 L · min −1 · m −2 ) ( P = .0038, .037). At 6 to 12 months, the PAH group demonstrated a significant rise in left ventricular ejection fraction and cardiac index that reached similar values in the non-PAH group measured at 2 months. These values were stable for up to 3 years. Conclusions Right ventricular function recovered early after living-donor lobar lung transplantation in the PAH group. In contrast, recovery of left ventricular function required 6 to 12 months. Improved cardiac function was sustained for up to 3 years, suggesting long-term durability of cardiac function recovery after living-donor lobar lung transplantation.

Objective Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle–pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome. Methods Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle–pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14. Results All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle–pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%). Conclusion Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle–pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.

From the Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. Disclosures: Authors have nothing to disclose with regard to commercial support. Received for publication Dec 7, 2010; revisions received May 2, 2011; accepted for publication May 23, 2011; available ahead of print July 1, 2011. Address for reprints: Takuya Kawabata, MD, PhD, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata, Okayama, 700-8558, Japan (E-mail: ktakuya@pop01.odn.ne.jp). J Thorac Cardiovasc Surg 2011;142:1582-4 0022-5223/$36.00 Copyright 2011 by The American Association for Thoracic Surgery doi:10.1016/j.jtcvs.2011.05.019

of the 16th Asian Pacific Congress of Cardiology, Taipei, Taiwan, 13–16 December, 2007 99 ventricle (SV), and SV s/p bidirectional Glenn shunt (BDG) (p

Objectives Our aim was to assess midterm safety and clinical outcomes of intracoronary infusion of cardiosphere-derived cells (CDCs) after staged palliation in patients with hypoplastic left heart syndrome (HLHS). Methods In this prospective, controlled study, 14 consecutive patients with HLHS who were undergoing 2- or 3-stage surgical palliations were assigned to receive intracoronary CDC infusion 1 month after cardiac surgery (n = 7), followed by 7 patients allocated to a control group with standard care alone. The primary end point was to assess procedural feasibility and safety; the secondary end point was to evaluate cardiac function and heart failure status through 36-month follow-up. Results No complications, including tumor formation, were reported within 36 months after CDC infusion. Echocardiography showed significantly greater improvement in right ventricular ejection fraction (RVEF) in infants receiving CDCs than in controls at 36 months (+8.0% ± 4.7% vs +2.2% ± 4.3%; P = .03). These cardiac function improvements resulted in reduced brain natriuretic peptide levels ( P = .04), lower incidence of unplanned catheter interventions ( P = .04), and higher weight-for-age z score ( P = .02) at 36 months relative to controls. As independent predictors of treatment responsiveness, absolute changes in RVEF at 36 months were negatively correlated with age, weight-for-age z score, and RVEF at CDC infusion. Conclusions Intracoronary CDC infusion after staged procedure in patients with HLHS is safe and improves RVEF, which persists during 36-month follow-up. This therapeutic strategy may enhance somatic growth and reduce incidence of heart failure.

Objective: To prevent possible deleterious effects of right ventricular volume overload on cardiorespiratory function, we developed a total right ventricular exclusion procedure for the treatment of end-stage isolated congestive right ventricular failure. Methods: Since 1996, this procedure has been performed in 5 patients in New York Heart Association functional class IV: 2 adults with arrhythmogenic right ventricular dysplasia and 3 children with Ebstein anomaly. The entire right ventricular free wall was resected along the atrioventricular groove and then parallel to the interventricular septum, sparing the pulmonary valve and a skeletonized right coronary artery. The orifice of the tricuspid valve was closed with either a polytetrafluoroethylene patch or with its leaflets. The defect of the right ventricular free wall was covered with a polytetrafluoroethylene patch in the 2 patients with arrhythmogenic right ventricular dysplasia and directly closed with the remnant of the free wall in the 3 children with Ebstein anomaly. After resection of a redundant right atrial wall, coronary sinus blood flow was rerouted into the left atrium through an atrial septal defect. A total cavopulmonary connection was constructed in 4 patients and a bidirectional superior cavopulmonary anastomosis in 1 infant. The heart was controlled with a DDD pacemaker in 3 patients. Results: The patients were extubated at a mean of 14 hours postoperatively (range, 1-38 hours). There were no early or late deaths. At follow-up, ranging from 8 to 57 months, the mean cardiothoracic ratio had decreased from 74% ± 7% before the operation to 52% ± 6% (P

DOI:€10.1016/j.jtcvs.2006.04.042 J Thorac Cardiovasc Surg 2006;132:21-22 Date Hisayuki Shigematsu, Yoshifumi Sano, Shingo Kasahara, Hiroyuki Yanai and HiroshiCalcifying fibrous tumor arising from the heart http://jtcs.ctsnetjournals.org/cgi/content/full/132/3/e21 located on the World Wide Web at: The online version of this article, along with updated information and services, is