Tomohiro Nakata

Feasibility of the extracardiac conduit Fontan procedure in patients weighing less than 10 kilograms

OBJECTIVE The extracardiac conduit Fontan procedure has led to improved outcomes. We performed the procedure in patients weighing less than 10 kg and evaluated its feasibility. METHODS Since January 1999, 72 patients weighing less than 20 kg underwent extracardiac conduit Fontan procedure with polytetrafluoroethylene conduits. The patients were divided into 2 groups: 36 patients weighing less than 10 kg in group S and 36 weighing more than 10 kg in group L. Mean weight, median age, and median follow-up period in groups S and L were 8.5 +/- 1.1 and 14.0 +/- 3.0 kg, 18.9 and 42.0 months, and 29.2 (1.7-79.7) and 42.1 (2.8-94.2) months, respectively. Postoperatively, most patients received peritoneal drainage catheters. We reviewed data precatheterization and postcatheterization and postoperative course. RESULTS Conduit sizes in groups S and L were 17.0 +/- 1.3 and 17.9 +/- 1.9 mm, respectively (P = .03). Five patients required fenestrations. There were 2 hospital deaths, 1 in each group, and 2 late deaths in group S. The postoperative course was identical in both groups, except for median length of stay in the intensive care unit and peritoneal drainage volume. Group S versus L: ventilator support, 11 versus 7 hours; pleural drainage, 9 days each; pleural drainage greater than 14 days, 6 versus 5 cases; peritoneal drainage, 8 versus 7 days; intensive care unit stay, 7 versus 4 days (P = .01), peritoneal drainage volume, 26.1 versus 14.1 mL x kg x d(-1) (P = .0007). CONCLUSIONS The early outcome of the extracardiac conduit Fontan procedure was satisfactory in patients weighing less than 10 kg. However, the required size of the conduit remains debatable.

Functional single ventricle with extracardiac total anomalous pulmonary venous connection

OBJECTIVE The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). METHODS Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0-744) days; median weight 3.2 (range 2.0-9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. RESULTS The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39-77%) and 54% (95% CI, 34-73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34-78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p=0.001, hazard ratio, 13.4; 95% CI, 2.8-64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p=0.024, hazard ratio, 23.4; 95% CI, 1.5-363.4) was a risk factor for recurrent PVS. CONCLUSIONS The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved.

Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome.

OBJECTIVES Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown. METHODS From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Childrens Hospital Japan with significant (3-4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome. RESULTS Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0-2) and those with a poor outcome (3-4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage. CONCLUSION Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.

Norwood procedure combined with the Starnes procedure.

We describe the case of 6-day-old baby girl with congenitally corrected transposition of the great arteries, Ebstein anomaly with severe tricuspid valve regurgitation, aortic atresia, hypoplastic aortic arch, and patent ductus arteriosus. She underwent Starnes procedure combined with the Norwood procedure. Postoperative echocardiography demonstrated good left ventricular function, reduced size of the right ventricle, and an unobstructed aortic arch. She was discharged and is currently awaiting a bidirectional Glenn operation.

Right atrial isomerism with infracardiac total anomalous pulmonary venous connection complicated by hiatal hernia

A 31-day-old girl with right atrial isomerism, a single atrium, a single right ventricle, pulmonary atresia, patent ductus arteriosus, coarctation of the pulmonary artery, infracardiac total anomalous pulmonary venous connection, and a large hiatal hernia was referred to our institution. We successfully performed a first-stage open palliation comprising the creation of a systemic-to-pulmonary arterial shunt, pulmonary artery angioplasty, and correction of the total anomalous pulmonary venous connection. During cardiopulmonary bypass, the hiatal hernia was also repaired through the median sternotomy itself without another laparotomy. The postoperative course was uneventful, and we avoided the potential complications of pulmonary venous obstruction and enteral feeding. She underwent a bidirectional Glenn operation and Fontan completion at 5 and 19 months of age, respectively.

Application of hybrid Stage I palliation for patients with two ventricular cavities and hypoplastic left heart structures

OBJECTIVES To assess the feasibility of hybrid Stage I palliation consisting of bilateral pulmonary artery bandings and ductal stenting for patients with 2 ventricular cavities and hypoplastic left heart structures. METHODS Eleven consecutive patients who underwent hybrid Stage I palliation between 2010 and 2017 were enrolled. The diagnoses were interrupted aortic arch/coarctation of the aorta, ventricular septal defect and significant left ventricular (LV) outflow tract obstruction in 5 patients, critical aortic stenosis and reduced LV contraction in 3 patients and hypoplastic left heart complex in 3 patients. RESULTS The median age at definitive surgery was 12 months (range 6-22 months). During the mean follow-up period of 24 months (range 9-83 months) following the definitive surgery, there was 1 death. Two patients with interrupted aortic arch/coarctation of the aorta did not undergo the Yasui operation but underwent arch repair and ventricular septal defect closure after the growth of the aortic valve and LV outflow tract. For 2 of the 3 patients with critical aortic stenosis, biventricular repair was performed. Of the 3 patients with hypoplastic left heart complex, 2 patients showed growth of the mitral valve and left ventricle following LV rehabilitation by balloon pulmonary artery dilatation or surgical debanding of the banded pulmonary arteries and subsequently underwent biventricular repair, which resulted in 1 death. CONCLUSIONS Hybrid Stage I palliation would be a safe and beneficial treatment for patients with 2 ventricles, as a bridge to decide whether and how to achieve a biventricular repair and whether it should be preceded by a preliminary LV rehabilitation.

Utility of a super-flexible three-dimensional printed heart model in congenital heart surgery†

OBJECTIVES The objective of this study was to assess the utility of 3D printed heart models of congenital heart disease for preoperative surgical simulation. METHODS Twenty patient-specific 3D models were created between March 2015 and August 2017. All operations were performed by a young consultant surgeon who had no prior experience with complex biventricular repair. All 15 patients with balanced ventricles had outflow tract malformations (double-outlet right ventricle in 7 patients, congenitally corrected transposition of great arteries in 5, transposition of great arteries in 1, interrupted aortic arch Type B in 1, tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries in 1). One patient had hypoplastic left heart complex, and the remaining 4 patients had a functional single ventricle. The median age at operation was 1.4 (range 0.1-5.9) years. Based on a multislice computed tomography data set, the 3D models were made of polyurethane resins using stereolithography as the printing technology and vacuum casting as the manufacturing method. RESULTS All but 4 patients with a functional single ventricle underwent complete biventricular repair. The median cardiopulmonary bypass time and aortic cross-clamp time were 345 (110-570) min and 114 (35-293) min, respectively. During the median follow-up period of 1.3 (0.1-2.5) years, no mortality was observed. None of the patients experienced surgical heart block or systemic ventricular outflow tract obstruction. CONCLUSIONS Three-dimensional printed heart models showed potential utility, especially in understanding the relationship between intraventricular communications and great vessels, as well as in simulation for creating intracardiac pathways.

A Case of Total Right Heart Bypass Operation for Polysplenia with Diffuse Bilateral Pulmonary Arteriovenous Fistula and Left Pulmonary Vein Obstruction by Anterior Translocation of the Hemiazygos Vein and Direct Anastomosis of the Hemiazygos Vein to the Hepatic Vein

症例は1歳7ヵ月,女児,診断は多脾症候群,単心房,右室系単心室,左側上大静脈,下大静脈(IVC)欠損.Total cavopulmonary shunt (TCPS)術後に両側肺動静脈瘻(PAVF)が発生し,同時に,拡張した半奇静脈と心房の間で圧迫されて,左肺静脈狭窄(PVO)も合併していた.PVOの解除を目的として半奇静脈前方転位を行い,両側肺循環への肝静脈血流の付与を目的として半奇静脈-肝静脈直接吻合を行う完全右心バイパスを施行した.術後9ヵ月のカテーテル検査でPAVFは消失しており,また,通路狭窄もなかった.本術式の利点は,1)半奇静脈経由で肝静脈血を肺に流すことで左右均等な血流分布が構築できること,2)半奇静脈の拡張によるPVOが解除できること,3)自己組織の成長も期待できることであり,IVC欠損の単心室群に適応可能な術式と考えられた.