Staged surgical repair for infants with interrupted aortic arch.

BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital cardiac anomaly, which necessitates surgical treatment. There are several surgical strategies for corrective repair of IAA, such as one-stage repair, rapid two-stage repair and two-stage repair. Here, we reported our surgical result of staged-repair policy for the patients with IAA. METHOD: From November 2003 to July 2015, there were 14 patients (8 boys, 6 girls) with IAA treated by us. Except one teenager patient, we routinely used intravenous infusion of prostaglandin E1 for all the infant patients (n = 13) to keep adequate end organ perfusion before the first surgical intervention. Surgical repair was performed after the perfusion of end organs recovered. RESULT: Two patients (1 teenager and 1 infant with one-stage surgery) were excluded from this study. At the time of the first surgery, we did the first-stage surgery with anastomosis in between aortic arch and descending aorta, division of patent ductus arteriosus and banding of pulmonary trunk through left thoracotomy. The overall surgical survival rate of the first surgery was 100% (12/12). At the time of the second surgery, corrective repair was done under cardiopulmonary bypass through median sternotomy. The surgical survival rate of the corrective surgery was also 100%. There is no late death during follow-up for 9 years (range 4.2-15.0 years). CONCLUSION: Out of several surgical strategies for the infants with IAA, staged repair still could be a treatment option to achieve satisfied surgical result.

Repair of a quadricuspid truncal valve by tricuspidization and reconstruction of right ventricular outflow tract with the excised truncal cusp.

OBJECTIVE: Quadricuspid truncal valves are susceptible to regurgitation, and tricuspid configuration is considered more durable after repairing the truncal valve. We report a new method by excising the excessive truncal cuspid to reconstruct a new competent aortic valve. METHODS: Bilateral pulmonary banding failed to stabilize a 4-month-old baby with type I truncus arteriosus and quadricuspid truncal valve with severe regurgitation, so surgery was performed. The aorta was transected, and the pulmonary arteries was separated from the aorta. There was an excessive cusp between the right coronary and left coronary cusp. A transannular incision was carried into the right ventricular outflow tract through the left border of the right coronary cusp. We performed truncal valve repair by translocating the excessive cusp, its annulus, and its wall to the right ventricular outflow tract side and reconstructing the neoaortic valve to tricuspid. Ventricular septal defect was repair through a right ventricular outflow tract approach. The floor of the right ventricular outflow tract was made from the excised cusp, including its wall and the resected posterior aortic wall. Then the anterior wall was reconstructed with bovine pericardial patch with a 19-mm porcine valve. RESULTS: Postoperative echocardiography confirmed a tricuspid neo-aortic valve with trivial aortic regurgitation, no residual ventricular septal defect, and a patent right ventricular outflow tract. CONCLUSIONS: We present the surgical technique to repair a quadricuspid truncal valve insufficiency by using the excessive anterior cusp and its pedicled flap for reconstruction of the pulmonary pathway and restoration of the tricuspid truncal valve.

Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy.

BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3). RESULTS: All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). CONCLUSIONS: Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.

Influence of apical position on the left ventricular outflow tract obstruction in congenitally corrected transposition.

BACKGROUND: The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval ipsilaterality, which may influence the outcome of the double switch operation (DSO). The goal of this study was to determine if the LVOT is compressed by the right ventricle in this setting. METHODS: A total of 103 patients with CCT were divided into four groups according to ventricular looping and apical position, including Group A (D-loop and levocardia), Group B (L-loop and dextrocardia), Group C (D-loop and dextrocardia), and Group D (L-loop and levocardia). Computed tomography was used to define left-right laterality and ventro-dorsal relationship of the LVOT. RESULTS: Apicocaval ipsilaterality was found in 57 patients (Group A, n=25; Group B, n=32), in whom the right ventricle was found to wrap around the LVOT. Among them, 49 (86%) had LVOT obstruction. In 46 patients without apicocaval ipsilaterality (Group C, n=10; Group D, n=36), 31 had LVOT obstruction (67.4%). LVOT obstruction was more prone to occur in patients with apicocaval ipsilaterality compared with those without (p=0.025), and was more significant in the situs solitus (p=0.058) than in situs inversus (p=0.547). CONCLUSIONS: LVOT obstruction was prone to occur in CCT patients with situs solitus and apicocaval ipsilaterality (Group B). The ventricular outflow patency was influenced by apical position, which should be considered to avoid a posterior ventricular outflow tract from compression after DSO.

The Concept of the Arch Window in the Spiral Switch of the Great Arteries.

When the arterial switch operation includes the Lecompte maneuver, the arterial trunks are reconnected in parallel, rather than the spiral fashion observed in the normal heart. Thus, although the ventriculo-arterial connections are hemodynamically corrected, the anatomic arrangement cannot be considered normal. We hypothesized that, if feasible, it would be advantageous to restore a spiral configuration for the arterial trunks. In 58 patients, we reconstructed the arterial trunks such that, postoperatively, the pulmonary channel spirals round the aorta, passing to either the right or the left, and branches posteriorly. We compared the outcomes with those in 95 patients undergoing a standard non-spiraling operation over the same period. Average follow-up was 8.2 ± 4.5 years. The estimated 10-year survival was similar in the cohorts, at 94.7 % for those with spiraling trunks, as compared to 90.4 % for those with parallel outflow tracts. Reoperation-free survival at 10 years was not significantly different (87.6 vs. 90.5 %). Supravalvar pulmonary stenosis, aortic neo-coarctation, or left bronchial stenosis, however, was encountered in one-eighth of those undergoing a standard operation. None of these complications occurred in those patients who, postoperatively, had spiraling outflow tracts (P = 0.002). Reconstruction of spiraling trunks after the arterial switch has, thus far, avoided the complications of supravalvar pulmonary stenosis, neo-aortic kinking, or bronchial stenosis. The spiraling arrangement prevents compression of the pulmonary vessels and bronchial tree by the aorta, since it provides a wide window in the new aortic arch.

Outcome of Tricuspid Valve Plasty in Norwood Stage I Operation.

BACKGROUND: Significant tricuspid valve regurgitation (TR) is considered a poor prognosis factor for patients with hypoplastic left heart syndrome (HLHS). Performing a tricuspid valve plasty (TVP) during Norwood Stage I palliation (S1P) has rarely been reported. We report mid-term results of performing TVP during Norwood S1P. METHODS AND RESULTS: Between December 2004 and July 2013, 48 patients with HLHS or variants underwent Norwood S1P and of them 11 (23%) with TR of a moderate degree or above underwent TVP. The estimated 1- and 5-year survival rates for Norwood S1P were 61% and 54%, respectively. Among the 11 patients with TVP, there were 2 early deaths. Nine patients had a Stage II operation and 3 of them died late. Four patients completed a Fontan operation, and 2 were waiting. Using Cox regression analysis, lower body weight, presence of intact atrial septum, and preoperative cardiopulmonary resuscitation were factors associated with increased risk for death. Patients with significant TR and undergoing TVP during Norwood S1P had a similar survival curve to those without significant TR. CONCLUSIONS: Our results for TVP performed during Norwood S1P were encouraging. They suggested that aggressive TVP is warranted in Norwood S1P when primary heart transplantation is not available. Further study is required to determine if the strategy does improve the results for patients with HLHS and TR initially. (Circ J 2016; 80: 1362-1370).

Platypnea-Orthodeoxia Syndrome Two Decades after Definitive Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum.

A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.

17-year experience in surgical management of congenitally corrected transposition of the great arteries: a single-centre's experience.

OBJECTIVES: We report our surgical experience in congenitally corrected transposition of great arteries (CCTGAs) and the long-term follow-up result. METHODS: From January 1995 to February 2012, 56 patients with CCTGA received definite surgical repair; 15 patients received conventional repair (Group I), 18 patients received anatomical repair (Group II) and 23 patients received single ventricular palliation (Group III). They were followed for early and late mortality, long-term survival, postoperative morbidity and reintervention or reoperation. RESULTS: The overall survival rate was 80% at 16 years in Group I, 53% at 13 years in Group II and 100% at 13 years in Group III. After excluding the early surgical mortality, the long-term survival rate was 92% at 16 years in Group I, 64% at 13 years in Group II and 100% at 13 years in Group III. Patients with significant tricuspid valve regurgitation showed the worst outcome after surgery. CONCLUSIONS: Our series showed good results with single ventricular palliation (SVP) in CCTGA with complex anatomy, but the long-term result should be followed. Anatomical repair is the choice of operation only for those with favourable anatomy. The more complicated intracardiac repair may result in late left ventricular outflow tract obstruction, various degrees of atrioventricular block, systemic or pulmonary venous return obstruction and the lack of an ideal conduit (e.g. homograft) for Rastelli reconstruction. Therefore, we preferred SVP in patients with complex and unfavourable anatomy.

Ventricular geometric characteristics and functional benefit of mild right ventricular outflow tract obstruction in patients with significant pulmonary regurgitation after repair of tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) outflow tract obstruction (RVOTO) might protect the RV from adverse remodeling caused by significant pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (rTOF), but the underlying mechanisms and influences on exercise tolerance remain unclear. This study sought to investigate the impacts from mild RVOTO on ventricular remodeling and exercise capacity in rTOF. METHODS: Eighty-five rTOF patients with a PR fraction ≥20% were assessed with cardiac magnetic resonance, cardiopulmonary exercise test, and echocardiography. Patients with a peak RVOT pressure gradient 20-50 mmHg were considered to have mild RVOTO (n = 29), while those with a gradient <20 mmHg had isolated PR (n = 56). RESULTS: Comparing to patients with isolated PR, patients with combined PR and mild RVOTO had smaller RV and RVOT dimension, better RV and left ventricular (LV) ejection fraction (EF), and superior exercise capacity. PR severity and RV mass/volume ratio were similar between these 2 groups. LVEF coupled with RVEF only in patients with isolated PR. In multivariate analysis, smaller RVOT dimension was independently related to smaller RV dimension (P < .001) and higher RVEF (P = .005). Furthermore, mild RVOTO was independently associated with higher peak oxygen consumption (P = .014) and oxygen uptake efficiency slope (P = .005). CONCLUSIONS: Patients with combined PR and mild RVOTO had better RV remodeling and exercise capacity compared to those with isolated PR. Our findings confirm the benefits from mild residual RVOTO support a policy of conservative RVOTO relief at repair.

Risk stratification and outcome of cardiac surgery for patients with body weight <2 ,500g in an Asian center.

BACKGROUND: Cardiac surgery performed in patients with low body weight is a challenge for surgeons. Currently, such outcomes are mainly reported from European or North American centers. In this study, we review our cardiac surgery experience with neonates and infants weighing <2,500g. METHODS AND RESULTS: We included patients with a body weight <2,500g who received cardiac surgery between January 2008 and December 2012. The survival outcome was compared to that of patients with large body weight, and then the Risk Adjusted Classification for Congenital Heart Surgery (RACHS-1) categorization was used for operative risk stratification. In the 1,245 index operations, 53 patients (4.3%) were <2,500g. The mean body weight was 2,232g (range 1,320-2,500g). The hospital mortality rate was 20.7% (11/53). Most (85%) of the procedures were in RACHS-1 category ≥3. The risk ratio was significantly higher in RACHS-1 category 3 (relative risk [RR]:6.2; 95% confidence interval [CI]:1.6-23.9) and 4 (RR:4.6; 95% CI:1.4-15.0), respectively, while it was not significantly different in category 2 (RR:1.02; 95% CI:1.01-1.02) and category 6 (RR:2.9; 95% CI:0.36-13.3). CONCLUSIONS: Cardiac surgery performed on infants with low body weight is generally a complex procedure, but the results are acceptable. The risk was higher than that for patients with higher body weight in RACHS-1 category 3 and 4. Further investigation to improve the outcome of this high-risk group is needed.

Prognostic value of submaximal exercise data for cardiac morbidity in Fontan patients.

INTRODUCTION: Submaximal exercise parameters are associated with an increased risk of hospitalization in patients with heart failure, but the implication in patients with Fontan circulation remains unknown. We investigated the prognostic value of these parameters in a Fontan cohort, in whom maximal exercise effort is often limited. METHODS: Fifty-two Fontan patients received cardiopulmonary exercise tests at least 12 months after Fontan completion. We evaluated two maximal parameters (peak oxygen consumption (V˙O2) and HR reserve) and two submaximal parameters (oxygen uptake efficiency slope (OUES) and minute ventilation (V˙E) to carbon dioxide elimination (V˙CO2) slope). RESULTS: The peak V˙O2 and OUES were 58.0% ± 11.2% and 56.6% ± 14.5% of the age- and sex-related predicted values. In the subsequent follow-up (median, 22.7 months), 11 patients (21%, including one death) exhibited cardiac morbidity, defined as cardiac-related hospitalization. Time-dependent receiver operating characteristic curve analysis demonstrated that only submaximal parameters were related to 2-yr cardiac morbidity (area under the curve for OUES 0.781, P = 0.018; for V˙E/V˙CO2 slope 0.714, P = 0.04), even in the subgroup achieved maximal exercise effort. The optimal threshold value for OUES was 45%, and for the V˙E/V˙CO2 slope, it was 37. Furthermore, the OUES conveyed independent prognostic information beyond resting oxygen saturation and a history of heart failure or protein-losing enteropathy. CONCLUSION: Submaximal exercise parameters provide superior prognostic information to maximal exercise data for predicting cardiac morbidity in Fontan patients. Moreover, the association between the OUES and cardiac morbidity is independent of relevant baseline clinical information.

Two-stage endovascular balloon angioplasty and stent implantation for supravalvular pulmonary stenosis in a 33-month-old boy incurring congestive heart failure following arterial switch operation.

We report a 33-month-old boy, who had undergone arterial switch operation (ASO) for simple transposition of the great arteries (TGA) since birth, presenting with exercise intolerance with dyspnoea, tachypnoea, tachycardia, cardiomegaly and hepatomegaly. Doppler echocardiography showed severe tricuspid regurgitation and dilatation of the right atrium. At 28 months, cardiac catheterization tracked down the culprit to be right ventricular outflow tract obstruction (RVOTO) manifested at the mid-portion of the main pulmonary artery (MPA) as supravalvular pulmonary stenosis (SVPS) and measuring 2.98 mm in diameter. There was a pressure gradient of 76 mmHg between the right ventricle and the distal portion of the MPA. The lumen was initially dilated to 6.79 mm by balloon angioplasty (BA), after which the pressure gradient dropped to 51 mmHg. However, cardiomegaly and hepatomegaly lingered for 5 months. Stent implantation (SI) was performed at 33 months, after which the lumen was dilated to 9.99 mm and the pressure gradient dropped to 20 mmHg. Liver span decreased from 10 cm to 6 cm.There was a recrudescence of physical activity. Echocardiography showed regression of tricuspid regurgitation and dilatation of the right atrium at the 12-month follow-up.

Comparison of bilateral pulmonary arterial level and diameter in transposition of the great arteries.

In normal anatomy, the left pulmonary artery (LPA) is usually situated higher than the right pulmonary artery (RPA); however, transposition of the great arteries (TGA), the LPA is not always situated higher than the RPA. This study was performed to clarify the relative position of the RPA and the LPA in transposition of the great arteries (TGA) as well as the implications. We reviewed 101 angiograms of patients with TGA (age 4.1 ± 1.2 months). The width of the RPA, the LPA, and the pulmonary trunk (PT) were measured just before their first branch in the frontal view. They were classified into four groups according to the ratio between the RPA and the PT (RPA/PT). The initial courses of the LPA and the RPA were compared and defined according to their height in the frontal view, and the preferential flow (or not) to the RPA was recorded. The equation of hydrodynamics was applied to evaluate the bifurcation angle. Both PAs were the same size in all cases. Forty-eight patients (47.5 %) had a RPA/PT diameter ratio < 0.49. The LPA coursed higher than the RPA in the majority of cases (81 [80.2 %]); in a minority of cases the LPA and RPA were at the same level (6 [5.9 %]); and in some cases the RPA coursed higher than the LPA (14 [13.9 %]). Patients with a high degree of PA hypoplasia tended to have both PAs at the same level or a higher-positioned RPA. Autopsy (1 of 3 cases) showed a posterior ridge against the bronchus in the higher RPA. Hydrodynamic calculation showed that the greater the angle between the RPA/PT, the greater the preferential flow. Preferential flow to the RPA in TGA did not necessarily result in LPA hypoplasia before its first branch. Higher RPA position relative to the LPA was associated with greater flow in it against the posterior bronchus. This situation was more prevalent in patients with severe PA hypoplasia.

Circulating biomarkers of collagen type I metabolism mark the right ventricular fibrosis and adverse markers of clinical outcome in adults with repaired tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) fibrosis is common in patients with repaired tetralogy of Fallot (rTOF). Although accumulating evidence indicates the role of circulating biomarkers of collagen metabolism in left ventricular fibrosis, rTOF data are lacking. This study examined the expression profile and clinical relevance of circulating biomarkers of collagen type I metabolism in rTOF patients. METHODS: Serum biomarkers of collagen type I synthesis (carboxy-terminal propeptide of procollagen type I, PICP), degradation (carboxy-terminal telopeptide of collagen type I, CITP), and enzymes regulating collagen degradation (matrix metalloproteinases, and type I tissue inhibitor, TIMP-1) were measured in 70 rTOF and 91 control adults. All patients had complete clinical data and received cardiovascular magnetic resonance scans with late gadolinium enhancement (LGE). RESULTS: Compared to the controls, rTOF patients had higher PICP levels (p<0.001), PICP:CITP ratios (p<0.001), and TIMP-1 concentrations (p<0.001). Increasing PICP levels correlated with higher RV LGE scores (r=0.427, p<0.001), lower VO2max (r=-0.428, p=0.002), and larger RV volumes. Furthermore, stepwise multivariate linear regression analysis identified RV end-diastolic volume index >150mL/m(2) (ß=40.52, p=0.016), RV LGE score (ß=3.94, p=0.008), and age (ß=-1.77, p=0.011) as independent correlates of circulating PICP levels. CONCLUSIONS: Patients with rTOF exhibited a profibrotic state with excessive collagen type I synthesis and dysregulated degradation. Elevated circulating PICP levels might reflect RV fibrosis, and link to adverse markers of clinical outcome.