Characteristics and long-term outcome for congenital left main coronary artery atresia.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

Conservatively treated coronary malperfusion associated with acute type A dissection before aortic root replacement.

Coronary malperfusion associated with aortic dissection usually requires aggressive surgical treatment or catheter revascularization. Here, we report a case of conservatively treated coronary malperfusion associated with acute type A dissection before aortic root replacement. An 81-year-old woman was rushed to our hospital in a state of circulatory shock after developing chest pain. She was severely hypotensive on admission, and the electrocardiogram (ECG) revealed anterior and lateral ST elevation. However, the initial fluid resuscitation increased her blood pressure to a normal level, and the ischemic ECG changes disappeared in about 20 min. ECG-gated cardiac multidetector computed tomography showed a type A aortic dissection complicated with left main trunk dissection. A primary entry tear was located 5 mm below the left coronary ostium. The patient successfully underwent composite graft replacement of the aortic root in a stable hemodynamic condition. .

Abdominal aortic aneurysm with periaortic malignant lymphoma differentiated from aneurysmal rupture by clinical presentation and magnetic resonance imaging.

Abdominal aortic aneurysm (AAA) associated with periaortic malignant lymphoma is difficult to differentiate from aneurysmal rupture because of similarities in their clinical presentation and appearance on computed tomography images. We here report a case of AAA associated with periaortic malignant lymphoma diagnosed preoperatively with an absence of typical symptoms, showing that AAA in periaortic malignant lymphoma can present without any clinical correlates. Magnetic resonance imaging was used to confirm the diagnosis. The patient was treated by endovascular repair, which may be safer and more effective than open surgery for AAA associated with malignant lymphoma because of the tight adhesion between the aneurysm and the lymphoid tissue.

Early clinical outcomes of right ventricular outflow tract reconstruction with small caliber bovine jugular vein conduit (Contegra®) in small children.

This study reviewed early clinical outcomes of right ventricular outflow tract reconstruction with Contegra® valved conduits in pediatric patients. Between April 2013 and July 2014, thirteen pediatric patients underwent right ventricular outflow tract reconstruction with Contegra valved conduits. The size of the implanted conduits were 12 mm in 5 patients, 14 mm in 3, 16 mm in 3, and then 2 patients were implanted with bicuspidized conduits for downsizing the conduit to 9 and 10 mm in each. Follow-ups were completed in all patients. One conduit was explanted 7 days after a neonatal biventricular repair for Ebstein's anomaly and pulmonary atresia, timed to be at the point of conversion to a single ventricular palliation. Among the 5 patients who developed significant pulmonary insufficiency and/or conduit stenosis, 3 patients exhibited persistent pulmonary hypertension. Both the bicuspidized conduits resulted in early pulmonary insufficiency. One patient implanted with a ring-supported conduit developed coronary artery stenosis, caused by suppression between the ring of the implanted conduit and the annulus of the mechanical vale. Although the small caliber Contegra valved conduit might be an alternative to RVOT reconstruction, the indication should be carefully considered. More than mild pulmonary hypertension, and low body weight at operation of less than 3.0 kg caused early conduit dysfunction.

Impact of Ross Operation on Outcome in Young Female Adult Patients Wanting to Have Children.

BACKGROUND: The most appropriate valve substitute at aortic valve replacement (AVR) for young female adult patients wanting to have children is unclear. METHODS AND RESULTS: Between 1992 and 2013, 12 consecutive female patients aged >18 (median, 22.5 years; range, 18-34 years) underwent Ross operation (Ross group). Between 1984 and 2013, 9 consecutive female patients aged >18 (median, 30 years; range, 22-39 years) underwent AVR with bioprosthesis (bioprosthesis group). There was 1 late mortality in the bioprosthesis group, due to prosthetic valve endocarditis (PVE). Freedom from reoperation for aortic valve at 15 years was 90.0% in the Ross group, and 57.1% in the bioprosthesis group (log-rank, P=0.098). One in the Ross group underwent reoperation for aortic regurgitation (AR), whereas 4 in the bioprosthesis group did so for aortic stenosis (AS) in 2, combined AS and AR in 1, and PVE in 1. Five patients in the Ross group and 3 in the bioprosthesis group had 7 and 4 uneventful pregnancies, respectively. AR progressed during the perinatal period in a total of 7 of 11 pregnancies. No AS was seen at discharge, after 5 years, or during pregnancy in the Ross group. CONCLUSIONS: The long-term outcome of Ross operation for female patients wanting to have children is excellent. Although subclinical pulmonary autograft valve regurgitation during pregnancy was often observed, pulmonary autograft stenosis did not occur, therefore it would be an ideal option for patients wanting to have children.

Adipose-derived stem cell sheet transplantation therapy in a porcine model of chronic heart failure.

Adipose-derived stem cells (ASCs) are a promising resource for cell transplantation therapy for damaged heart tissue. Cell death in the graft early after transplantation represents the main cause of unsatisfactory therapeutic efficacy, but tissue-engineered cell sheets grown in temperature-responsive cell culture dishes may enable improved engraftment of transplanted cells. We investigated the therapeutic potential of this method in chronic myocardial ischemia in swine. We created a porcine model of chronic heart failure by implanting an ameroid constrictor around the main trunk of the left anterior descending artery, just distal to the circumflex branch. Simultaneously, ASCs were obtained from a piece of subcutaneous adipose tissue and expanded to form ASC sheets using temperature-responsive dishes. Four weeks after ameroid constrictor placement, triple-layered ASC sheets were transplanted onto the area of the ischemic myocardium (sheet group, n = 7). Controls (n = 7) received no sheet. Just before and 4 weeks after transplantation, left ventriculography (LVG) and coronary angiography (CAG) were performed. LVG revealed a significant improvement in the left ventricular ejection fraction of the sheet group compared with controls (47.6 ± 2.9% vs 41.4 ± 2.8%, P < 0.05). Furthermore, development of collateral vessels was only detected in the sheet group with right CAG. Histologic analysis demonstrated that engrafted ASC sheets grew to form a thickened layer that included newly formed vessels. ASC sheet transplantation therapy is an intriguing therapeutic method for ischemic heart failure.

Long-term outcomes after definitive repair for tetralogy of Fallot with preservation of the pulmonary valve annulus.

OBJECTIVES: The aim of our study was to evaluate the long-term outcomes after definitive repair of tetralogy of Fallot with preservation of the pulmonary valve (PV) annulus. METHODS: From 1989 to 2000, 84 of 222 patients (37.8%) with tetralogy of Fallot and PV stenosis underwent definitive repair with preservation of the PV annulus without right ventriculotomy. PV commissurotomy was concomitantly performed in 74 patients (88.1%). The PV was bicuspid in 56 patients (66.7%); the mean Z value was -1.2 ± 1.5 (range, -4.9 to 2.4). The mean follow-up period was 15.8 ± 5.7 years (maximum, 22.8), and follow-up data were complete for 75 patients (89.3%). RESULTS: The actuarial survival and freedom from reoperation rates at 20 years was 98.6% and 95.8%. The freedom from ventricular arrhythmia at 5, 10, 15, and 20 years was 98.7%, 89.6%, 74.1%, and 58.0%, respectively. All detected ventricular arrhythmias were isolated monofocal premature ventricular contractions. Freedom from moderate or greater pulmonary regurgitation at 5, 10, 15 and 20 years was 50.4%, 44.9%, 38.4%, and 35.7%, respectively. A bicuspid PV (hazard ratio, 2.910; 95% confidence interval, 1.404-6.204, P = .004) and a Z-value of less than -2 (hazard ratio, 1.948; 95% confidence interval, 0.915-5.857; P = .034) were the risk factors for developing moderate or greater pulmonary regurgitation. CONCLUSIONS: The long-term outcomes after definitive repair of tetralogy of Fallot with preservation of the PV annulus were excellent. Although isolated, monofocal premature ventricular contractions were frequently observed, fatal ventricular arrhythmia was not. The indication should not only be decided by the PV annulus size, but also by the valvular morphology to maintain long-term PV competency.

Long-term outcomes of common atrioventricular valve plasty in patients with functional single ventricle.

OBJECTIVES: Common atrioventricular valve (CAVV) regurgitation is widely known as a risk factor for mortality and Fontan completion in patients with functional single ventricle. Hence, we reviewed our surgical experience with CAVV plasty in Fontan candidates. METHODS: Staged Fontan strategy and extracardiac total cavopulmonary connection as Fontan modification were our principal approaches in 1995. Since then, 38 consecutive Fontan candidates (21 males, median weight at operation was 7.0 kg and median age was 17 months old) underwent CAVV plasty. Right atrial isomerism was associated with 24 patients. The initial CAVV plasty was performed before inter-stage bidirectional Glenn (BDG) in 3 patients, at BDG in 23, before Fontan in 4 and during Fontan in 8. Since 1995, the modified Alfieri technique with a tailed, expanded, polytetrafluoroethylene tube as a bridging strip was the procedure for repair and 27 patients underwent the procedure. The mean follow-up period was 7.1 years (range 0-17 years). RESULTS: Actuarial survival and freedom from CAVV replacement rates at 1, 5 and 10 years were 81, 70 and 67% and 89, 85 and 75%, respectively. Seven patients ultimately underwent CAVV replacement with one death. Twenty-three of the 38 patients completed Fontan operation (61%). Association with total anomalous pulmonary venous connection (P= 0.01) and CAVV plasty before BDG (P= 0.05) were risk factors for mortality. CONCLUSIONS: CAVV plasty for patients with functional single ventricle is still challenging; however, the aggressive and repeated surgical intervention may contribute to provide better life-prognosis. The ventricular volume unloading effect of BDG without additional pulmonary blood flow or Fontan operation did not contribute to maintain CAVV function. Therefore, there would not be any hesitation for CAVV replacement to control CAVVR in the setting of systemic ventricular failure. Although the statistically significant therapeutic superiority of the modified Alfieri technique was not shown so far, further follow-up may reveal the advantage of this easy and simple technique.

Midterm outcomes of sutureless technique for postoperative pulmonary venous stenosis.

BACKGROUND: The efficacy of a sutureless technique for postoperative pulmonary venous stenosis (PVS) following repair of total anomalous pulmonary venous connection (TAPVC) has been reported, though detailed clinical advantages remain unclear. We retrospectively reviewed our surgical experience, and compared outcomes between conventional procedures and a sutureless technique. METHODS: For relief of postoperative PVS after TAPVC repair, five patients underwent a conventional procedure, such as orifice cutback or resection of a proliferated intima, from 1999 to 2004 (Conventional group, 4 males, median 93 days old, 3.6 kg), then seven underwent a sutureless technique (Sutureless group, 5 males, 119 days old, 3.4 kg) from 2005 to 2011. Patients with a functional single ventricle were excluded. There were no significant differences regarding patient characteristics. Follow-up examinations were completed in all patients. RESULTS: The rate for cumulative survival at 5 years was 60 % in the Conventional group and 71.4 % in the Sutureless group. Re-stenosis after relief of PVS occurred in 100 % (10/10) of patients in the Conventional group and 31.6 % (6/19) of patients in the Sutureless group (p = 0.0088). For bilateral venous stenosis patients, the survival rate was 66.7 % (4/6) in the Sutureless group and 0 % (0/2) in the Conventional group (p = 0.10). Out of three patients who developed whole 4-vein stenosis, only one in the Sutureless group survived. CONCLUSIONS: Although overall survival rate was similar in both groups, the Sutureless technique for postoperative PVS following TAPVC repair successfully rescued more pulmonary veins without re-stenosis than conventional procedures. Further follow-up may demonstrate therapeutic advantages.

Anatomic repair for corrected transposition with left ventricular outflow tract obstruction.

BACKGROUND: We investigated the long-term outcomes of anatomic repair for congenitally corrected transposition of great arteries (ccTGA) and its variant associated with left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD). METHODS: From 1987 to 2011, 47 patients (27 with pulmonary atresia and 20 with pulmonary stenosis) with ccTGA and its variant associated with LVOTO and VSD underwent anatomic repair. The mean operative age was 5.5 ± 3.7 years old (range, 1.6 to 21.3). The preoperative right ventricular end-diastolic volume was 133% ± 31% (81 to 222) of their normal size. The atrial switch procedure was Mustard in 31 patients and Senning in 16, with the latter used in all from 2002. RESULTS: The mean follow-up period was 11.6 ± 7.3 years (maximum, 22.7). The VSD was concomitantly enlarged in 4 patients and Damus-Kaye-Stansel (DKS) anastomosis was added in 9 patients with pulmonary stenosis and restrictive VSD. The overall survival rate at 20 years was 70.2% and no mortality has been observed in 21 consecutive patients since 1997. No patient required reoperation for the postoperative systemic ventricular outflow tract obstruction. A surgical heart block developed in 1 patient (2.1%) who underwent concomitant VSD enlargement. None of the patients developed a moderate or greater aortic or neo-aortic regurgitation. CONCLUSIONS: Recent outcomes after anatomic repair for congenitally corrected transposition of great arteries associated with LVOTO and VSD were excellent. For patients with pulmonary stenosis and restrictive VSD, additional DKS anastomosis seems to be an effective approach to avoid postoperative systemic ventricular outflow tract obstruction and surgical heart block.

Late left ventricular function after successful Ross-Konno operation.

BACKGROUND: The changes of left ventricular function after undergoing a Ross-Konno operation for pediatric patients with multiple-level left ventricular outflow tract obstruction are still unclear. METHODS: From 1996 to 2006, 14 patients younger than 20 years underwent a Ross-Konno operation. The mean age at the time of the operation was 6.6 ± 5.7 years. A postoperative catheter examination was performed at early (1.0 ± 0.4 years after the operation, n = 13), mid (3.3 ± 1.6 years, n = 9), and late (8.5 ± 2.5 years, n = 6) periods. The mean follow-up period was 10.1 ± 6.0 years (range, 0.1 to 17.2 years). RESULTS: There was 1 early death and no late deaths. The pressure gradient across the left ventricular outflow tract was well relieved from 62.4 ± 22.9 mm Hg at the preoperative period to 4.5 ± 7.2 mm Hg at the early postoperative period, 4.1 ± 3.5 mm Hg at the mid postoperative period, and 3.8 ± 3.7 mm Hg at the late postoperative period. The left ventricular ejection fraction significantly decreased from 0.73 ± 0.12 at the preoperative period to 0.60 ± 0.10 at the early postoperative period (p = 0.00035), then improved to 0.64 ± 0.14 at the late postoperative period. The left ventricular end-diastolic volume normalized to 103 ± 37% of the normal volume at the late postoperative period. However, the left ventricular end-diastolic pressure did not decrease from 15.2 ± 6.1 mm Hg at the preoperative period, 12.5 ± 3.3 mm Hg at the early postoperative period, 13.1 ± 5.1 mm Hg at the mid postoperative period, or 12.7 ± 5.6 mm Hg at the late postoperative period. CONCLUSIONS: The left ventricular contraction and volume normalized long after the Ross-Konno operation. However, the reduced ventricular compliance did not recover, although left ventricular outflow tract obstruction was well relieved.

Indication of Takeuchi technique for patients with anomalous origin of the left coronary artery from the pulmonary artery.

BACKGROUND: Intrapulmonary tunnel repair, called the Takeuchi technique, is a unique procedure for repairing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Since 1986, we have clearly defined the indication for the Takeuchi technique based on the location of the left coronary artery (LCA) orifice. METHODS AND RESULTS: From 1986 to 2011, 19 consecutive patients with ALCAPA underwent surgical repair; the dual-coronary system was reconstructed in 16 of these patients with either Takeuchi (n=9: 1 male; median age 14.7 years; median weight 42.7 kg) or a translocation procedure (n=7, 3 males; 0.4 years old, 5.6 kg). Takeuchi was performed in patients whose LCA arose far from the aorta (middle of posterior facing sinus in 3 patients, left side of posterior facing sinus in 2, non-facing sinus in 4). The mean postoperative follow-up period was 7.4±6.1 years in the Takeuchi group and 9.3±8.5 years in the Translocation group. Actuarial survival rate at 10 years was 87.5% and 71.4%, respectively, and the freedom from either reoperation or re-intervention rate at 10 years was 67.7% and 85.7%, respectively. All the patients who died had a preoperative left ventricular ejection fraction≤30%. CONCLUSIONS: The long-term outcome of the Takeuchi technique was acceptable. Although late reoperation and/or re-intervention cannot be disregarded, Takeuchi can be recommended when the LCA arises far from the aorta.

The incidence and characteristics of supraventricular tachycardia in left atrial isomerism: a high incidence of atrial fibrillation in young patients.

BACKGROUND: In left atrial isomerism (LAI), both atria show left atrial morphology. Although bradyarrhythmias are frequent and highly complex in LAI patients, previous studies have reported a low incidence of supraventricular tachycardia (SVT). METHODS: To evaluate the incidence and characteristics of SVT in LAI, we retrospectively evaluated the clinical characteristics of SVTs in 83 patients with LAI (age at last follow-up, 15.3±10.5 years). RESULTS: There were 27 SVTs in 19 patients (23%), including nine episodes of atrial fibrillation (AF) and eight non-reentrant SVTs. Sixteen of the 19 patients with SVT had histories of atriotomy, but the three patients with AF or non-reentrant tachycardia had no history of atriotomy. The rates of freedom from SVT were 66% and 59% at ages of 20 and 30 years, respectively; the corresponding rates for freedom from AF were 89% and 74%. In multivariate analysis, the predictors of SVT were age (OR, 1.14; 95% CI, 1.06-1.26; p=0.003) and sinus node dysfunction (SND) (OR, 3.88; 95% CI, 1.57-13.34; p=0.01). CONCLUSIONS: In patients with LAI, SVTs are common, and AF and non-reentrant SVTs are the major type of SVTs. The incidence of AF was high in young patients with LAI. The lack of anatomical barriers in the atria that allow the formation of macro-reentrant circuits may account for the higher incidence of AF and non-reentrant SVT than macro-reentrant tachycardia. Moreover, the increasing prevalence of SND with age should contribute to a higher incidence of SVT.

Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection.

OBJECTIVES: Surgical outcomes of patients with functional single ventricle have improved, though those for patients whose condition is complicated by extracardiac type total anomalous pulmonary venous connection (TAPVC) remain poor. We retrospectively reviewed our 21 years of surgical experiences with this challenging group. METHODS: From 1990 to 2010, 48 consecutive patients with functional single ventricle complicated by extracardiac TAPVC (26 males, 46 with right atrial isomerism) underwent initial surgical palliation at our centre. The median age and body weight at surgery were 69 days and 3.5 kg, respectively. The type of TAPVC was supracardiac in 31 patients, infracardiac in 14 and mixed type in 3. TAPVC was repaired in 25 patients before bidirectional Glenn (BDG) and 18 at BDG, while it remained in 3 patients. Since 2007, stent implantation for obstructive drainage veins for patients with preoperative pulmonary venous obstruction and sutureless marsupialization for relief of postoperative pulmonary venous stenosis (PVS) have been initiated. The mean follow-up period was 4.2 ± 5.1 years. RESULTS: The overall survival rates at 1, 3 and 5 years after the initial surgical intervention were 58.3, 41.1 and 31.3%, respectively. Sixteen patients achieved the Fontan operation (33.3%). The freedom from postoperative PVS rates at 1 and 3 years after repair was 68.7 and 63.4%, respectively. Univariate analysis detected that infracardiac TAPVC (P = 0.036), coexisting major aortopulmonary collaterals (P = 0.017), and TAPVC repair before BDG (P = 0.036) all reduced survival, and multivariable analysis indicated the repair of TAPVC before BDG as the only risk factor (P = 0.032). Whereas the occurrence of postoperative PVS did not reduce survival, which had a significant negative impact on achieving the Fontan operation (P = 0.008). The cumulative survival rate did not improve by surgical era. CONCLUSIONS: Surgical outcomes of patients with functional single ventricle undergoing the repair of extracardiac TAPVC in the neonatal period due to obstruction of the venous drainage pathway remain poor. Stent implantation for obstructive drainage veins to delay the timing of surgical correction and sutureless marsupialization as relief of postoperative PVS are expected to improve the late outcomes; however, the effect is still limited.

Late clinical features of patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum after biventricular repair.

BACKGROUND: We aimed to reveal late clinical features of patients with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (cPS) after biventricular repair (BVR) based on preoperative right ventricular (RV) end-diastolic volume (RVEDV) findings. METHODS: Since 1985, 23 of 73 patients with PA/IVS (n=22) or cPS (n=1) with a tripartite RV and without major sinusoidal communication underwent BVR with a hybrid approach. The mean age and weight at BVR were 1.4±2.1 years and 6.9±5.9 kg, respectively. Mean follow-up was 10.1±6.4 years (range, 1.1 to 24.6 years). RESULTS: Overall survival, reintervention-free, and arrhythmia-free rates at 20 years were 90.6%, 75.4%, and 50.4%, respectively. In 19 patients with preoperative RVEDV of 60% to 120% of normal, echocardiography at 10 years after BVR showed well-maintained RV systolic function. However, RV volume was quantitatively dilated in 16 (88.9%) due to moderate or greater tricuspid regurgitation in 8 (44.4%), pulmonary regurgitation in 12 (66.7%), or both, which caused arrhythmia in 3 patients more than 10 years after BVR. Two patients with preoperative RVEDV of greater than 120% of normal required tricuspid valve replacement after BVR, after which refractory atrial tachyarrhythmia developed in both patients. Furthermore, 2 patients with preoperative RVEDV of less than 60% of normal showed a cardiac index value within 2.5 L/min/m2 at 1 year after BVR, which did not improve. CONCLUSIONS: Patients with PA/IVS or cPS and adequately sized RV showed good late clinical features after BVR. However, long-term follow-up examinations are necessary for RV dilatation and late-onset arrhythmia.

Long-term results of mitral valve repair for severe mitral regurgitation in infants: fate of artificial chordae.

BACKGROUND: The experience with mitral valve repair for severe mitral regurgitation in infants was retrospectively reviewed. METHODS: From 1978 to 2009, 15 infant patients (4 boys, 7.2±3.2 months old) underwent mitral valve repair for severe mitral regurgitation. The etiology of mitral regurgitation according to Carpentier classification was type I in 1 patient, type II in 10 patients, and type III in 4 patients. Artificial chordal replacement was performed in 11 patients. The follow-up course was completed in all patients, with a median follow-up period of 10.2 years (range, 2.2 to 33.4 years). RESULTS: There were no operative or in-hospital mortalities. The actuarial survival and freedom from reoperation rates at 10 years were 89% and 65%, respectively. Five patients required redo mitral valve surgery, including 4 patients needing mitral valve replacement. Three of 4 patients with type III mitral regurgitation, caused by congenital subvalvar structural abnormality, required redo mitral valve surgery. In 11 patients who underwent artificial chordal reconstruction, 2 (18%) required mitral valve replacement during the early postoperative period, whereas the remaining patients showed no recurrent mitral regurgitation during the entire study period. The latest echocardiography findings at 7.3 years after the operation showed that the left ventricular diastolic diameter, mitral valve diameter, and ejection fraction were 99.2%±5.0% of normal, 101.7%±13.3% of normal, and 0.741±0.058, respectively. CONCLUSIONS: Long-term durability of mitral valve repair for severe mitral regurgitation in infants was satisfactory. Artificial chordal reconstruction is an effective procedure without an elevated risk of late reoperation.

Alternative to pulmonary allograft for reconstruction of right ventricular outflow tract in small patients undergoing the Ross procedure.

OBJECTIVES: Small pulmonary allografts are difficult to obtain, thus we now use a tailor-made right ventricle to pulmonary artery (RV-PA) conduit for the Ross procedure, consisting of a fresh non-treated autologous pericardial (AP) patch for the posterior wall and expanded polytetrafluoroethylene (ePTFE) monocusp patch for the anterior wall. Long-term durability and RV function were assessed. METHODS: Between 1997 and 2011, tailor-made conduits were used for right ventricular outflow tract (RVOT) reconstruction in 38 consecutive Ross procedures. Patients were divided into two groups by type of material used for reconstruction of the RVOT anterior wall: Group A (n = 11), pedicled AP patch with ePTFE monocusp valve; Group B (n = 27), ePTFE patch with the ePTFE monocusp valve. The posterior wall was reconstructed with an AP patch in both. We examined survival and freedom from re-intervention, haemodynamic indices by cardiac catheterization, efficacy of the RVOT by ultrasound cardiography (UCG) and exercise capacity at 3 years after the operation. The mean follow-up period was 6.0 ± 0.5 years. RESULTS: No patients required re-intervention for neo-aortic valve. Overall survival and freedom from re-intervention for RVOT reconstruction at 10 years were 100 and 100%, respectively, in Group A, and 92.6 and 89.4%, respectively, in Group B. No patients showed an RVOT pressure gradient greater than 25 mmHg by cardiac catheterization at 1 year after the operation. All showed less than 2.5 m/s of RVOT flow estimated by Doppler UCG at 6 years. RV function in both groups was preserved at normal in spite of a higher incidence of free RVOT insufficiency in Group A (P = 0.018). Exercise capacity was also preserved at normal in both groups. CONCLUSIONS: In paediatric patients undergoing the Ross procedure, a tailor-made conduit might be helpful to avoid growth-related RVOT obstruction. The incidence of free RVOT insufficiency was lower than with an anterior ePTFE patch, thus our method may be a better option to preserve RV function for a longer period.

Clinical outcomes of prophylactic Damus-Kaye-Stansel anastomosis concomitant with bidirectional Glenn procedure.

OBJECTIVE: We evaluated prophylactic Damus-Kaye-Stansel (DKS) anastomosis in association with the timing of a bidirectional Glenn (BDG) procedure as second-stage palliation aiming at Fontan completion to prevent late systemic ventricular outflow tract obstruction. METHODS: Between 1996 and 2005, 25 patients (14 boys; median age, 12 months) underwent a BDG procedure concomitant with DKS anastomosis. All had a systemic ventricular outflow tract through an intraventricular communication or morphologically developed subaortic conus and had previously undergone pulmonary artery banding. Enlargement of intraventricular communication and/or resection of a subaortic conus were not performed before or during the operation. RESULTS: Twenty-one (84%) patients subsequently underwent a Fontan operation, with a follow-up period of 6.8 ± 1.9 years (range, 4-11 years), with no mortalities after the Fontan operation. Cardiac catheterization showed that systemic ventricular end-diastolic volume was significantly decreased from 187% ± 74% of normal before BDG to 139% ± 35% after (P = .038) and to 73% ± 14% at 4.3 years after the Fontan operation (P < .001). However, the pressure gradient across the systemic ventricular outflow tract remained at 0.5 ± 0.8 mm Hg after DKS anastomosis and 0.6 ± 2.3 mm Hg at 4.6 years after the Fontan operation. None of the patients showed more than moderate aortic or neoaortic regurgitation, except 1 who progressed to pulmonary regurgitation after DKS anastomosis and required a reoperation for a systemic ventricular outflow tract. No anatomic properties affected late neoaortic valve function. CONCLUSIONS: Regardless of a significant reduction in systemic ventricular volume, DKS anastomosis concomitant with a BDG procedure shows promise for a nonobstructive systemic ventricular outflow tract after a Fontan operation.

The applications of non-ECG-gated MSCT angiography in children with congenital heart disease.

BACKGROUND: To minimize radiation exposure during a multi-slice computed tomography (MSCT) examination in children with congenital heart disease (CHD), we assessed diagnostic value and the various applications of MSCT without electrocardiography (ECG)-gated image acquisition. METHODS: Three-dimensional (3D) images were reconstructed to include volume rendering and differential color-coding of the arteries and veins. The vessel diameter in MSCT was measured with CT attenuation profiles and compared with that of angiography. Ventricular volumetry was initially validated by phantom experiment and followed by comparison with that of cine-angiography (n=15). Simulation for surgical operation was performed to evaluate the possibility of an intraventricular conduit in patients diagnosed with a double outlet right ventricle. RESULTS: Differential color-coding was able to provide accurate and understandable anatomical structure in CHD. The diameter of the descending aorta measured in MSCT correlated well with the values obtained by angiography (r(2)=0.86). According to the results of the phantom experiment, ventricular volume was studied in patients whose heart rate was faster than 120 beat/min. The left and the right ventricular volume in MSCT correlated well with values obtained by cine-angiography (r(2)=0.98 for RV, r(2)=0.94 for LV). Simulative operation of intraventricular rerouting provided appropriate indication for biventricular repair in patients diagnosed with a double outlet right ventricle. CONCLUSIONS: Non-ECG-gated MSCT angiography is applicable for precise anatomical diagnosis, ventricular volumetric study, and simulation surgery in children with CHD.

Clinical characteristics of acute mitral regurgitation due to ruptured chordae tendineae in infancy-experience at a single institution.

In infants, acute mitral regurgitation resulting from ruptured chordae tendineae is very rare, but often fatal. There are a few case reports, but the characteristics and etiology of chordae tendineae rupture have not been elucidated. Our aim was to determine the clinical characteristics of idiopathic acute mitral regurgitation due to chordal rupture in infancy. A retrospective analysis was performed on ten consecutive patients, with a mean onset age of 4.6 ± 1.3 months. Despite nonspecific initial symptoms, all patients developed respiratory distress and four required resuscitation within a few days (mean, 1.8 ± 1.8 days). Chest radiographs showed pulmonary congestion with a normal or mildly increased cardiothoracic ratio in all ten patients. Laboratory data and electrocardiograms showed nonspecific findings. Echocardiography revealed ruptured chordae in all patients; locations were anterior (50%), posterior (20%), and both (30%). Surgical intervention was performed within 24 h of admission in eight patients (mean, 3.6 ± 5.1 h). Pathological findings included inflammatory cells in six specimens and myxomatous degeneration in two. No bacteria were isolated from preoperative blood cultures, pathological tissues, or excised tissue cultures. Autoantibody levels were insignificant. Three preoperatively resuscitated patients developed neurological sequelae and arrhythmias occurred in four after mitral valve replacement. Acute onset and rapid deterioration in patients with ruptured chordae tendineae necessitates early surgical intervention to improve outcomes. Though the etiology remains unknown, onset is in infants approximately 4 months of age, suggesting a definite disease entity.