Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect: 15-year experience of a single center / 中国胸心血管外科临床杂志

@#Objective    To explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD). Methods    Clinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time. Results    In the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001). Conclusion    The effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.

A Case of Biventricular Repair with Damus-Kaye-Stansel (DKS) Take-Down after DKS Anastomosis for Interruption of the Aortic Arch and Ventricular Septal Defect with Subaortic Stenosis / 日本心臓血管外科学会雑誌

The patient in this case was a boy aged 2 years and 9 months. The patient was transferred to our hospital with ductal shock, and bilateral pulmonary artery banding was performed on the 9th day after the diagnosis of interruption of the aortic arch, ventricular septal defect, subaortic stenosis, and bicuspid aortic valve. Left ventricular outflow tract stenosis due to aortic annulus diameter and subaortic stenosis after repair was suspected. Damus-Kaye-Stansel (DKS) anastomosis, extended aortic arch anastomosis, and a right modified Blalock-Taussig operation were performed. Preoperative examination of the intracardiac repair showed growth of the aortic annulus and confirmed that biventricular repair was possible after DKS take-down. The patient's native aortic and pulmonary valves were preserved, and an intracardiac repair was performed without using an extracardiac conduit. The postoperative course was uneventful, and the patient is currently in a good condition at the age of 6 years. Three and a half years after surgical intervention, echocardiography and cardiac catheterization showed improvement of subaortic stenosis and enlargement of the aortic annulus. Our findings indicate that the most appropriate surgical procedure can be selected by detailed examination of the preoperative condition at each stage of the staged operation.

Prediction of biventricular repair by echocardiography in borderline ventricle / 中华医学杂志(英文版)

Objective@#In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle.@*Data sources@#Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including "borderline ventricle," "congenital heart disease," "CHD," "echocardiography," and "biventricular repair."@*Study selection@#Original articles and critical reviews relevant to the review’s theme were selected.@*Results@#Borderline left ventricle (LV): (1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair. (2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair. (3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV: (1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted. (2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair.@*Conclusions@#Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.

Assessment of Potential Renal Dysfunction in Patients with Congenital Heart Disease after Biventricular Repair

BACKGROUND AND OBJECTIVES: There are few reports on renal dysfunction in the remote period after biventricular repair, and biomarkers for early detection of renal dysfunction are not well understood. We examined whether early fluctuation of biomarkers of renal function occurs in the remote period after biventricular repair in patients with congenital heart disease (CHD). METHODS: Fourteen patients with CHD after biventricular repair were included. The examination values obtained by cardiac catheterization test and renal function indices based on blood and urine sampling were compared. RESULTS: The median estimated glomerular filtration rate (eGFR) of creatinine was 113 mL/min/1.73 m2, and the median eGFR of cystatin C was 117 mL/min/1.73 m2. A urine albumin-to-creatinine ratio (UACR) ≥10 mg/gCr was considered a risk factor for cardiovascular disease in 6 (43%) patients. There was a significant difference in right ventricular ejection fraction and deviation in right ventricular end-diastolic volume from the normal value between the 2 groups divided by UACR. Cyanosis before biventricular repair was noted in 2 (25%) patients with UACR < 10 mg/gCr and in 4 (67%) patients with UACR ≥10 mg/gCr. CONCLUSIONS: Increased UACR was noted in 43% of patients. In patients with UACR ≥10 mg/gCr, right heart system abnormality was observed, and several patients had cyanosis before radical treatment. Measurement for UACR may be able to detect renal dysfunction early in the postoperative remote period.

Computed Tomography-Based Ventricular Volumes and Morphometric Parameters for Deciding the Treatment Strategy in Children with a Hypoplastic Left Ventricle: Preliminary Results

OBJECTIVE: To determine the utility of computed tomography (CT) ventricular volumes and morphometric parameters for deciding the treatment strategy in children with a hypoplastic left ventricle (LV). MATERIALS AND METHODS: Ninety-four consecutive children were included in this study and divided into small LV single ventricle repair (SVR) (n = 28), small LV biventricular repair (BVR) (n = 6), disease-matched control (n = 19), and control (n = 41) groups. The CT-based indexed LV volumes, LV-to-right-ventricular (LV/RV) volume ratio, left-to-right atrioventricular valve (AVV) area ratio, left-to-right AVV diameter ratio, and LV/RV long dimension ratio were compared between groups. Proportions of preferred SVR in the small LV SVR group suggested by the parameters were evaluated. RESULTS: Indexed LV end-systolic (ES) and end-diastolic (ED) volumes in the small LV SVR group (6.3 ± 4.0 mL/m² and 14.4 ± 10.2 mL/m², respectively) were significantly smaller than those in the disease-matched control group (16.0 ± 4.7 mL/m² and 37.7 ± 12.0 mL/m², respectively; p < 0.001) and the control group (16.0 ± 5.5 mL/m² and 46.3 ± 10.8 mL/m², respectively; p < 0.001). These volumes were 8.3 ± 2.4 mL/m² and 21.4 ± 5.3 mL/m², respectively, in the small LV BVR group. ES and ED indexed LV volumes of < 7 mL/m² and < 17 mL/m², LV/RV volume ratios of < 0.22 and < 0.25, AVV area ratios of < 0.33 and < 0.24, and AVV diameter ratios of < 0.52 and < 0.46, respectively, enabled the differentiation of a subset of patients in the small LV SVR group from those in the two control groups. One patient in the small LV biventricular group died after BVR, indicating that this patient might not have been a good candidate based on the suggested cut-off values. CONCLUSION: CT-based ventricular volumes and morphometric parameters can suggest cut-off values for SVR in children with a hypoplastic LV.

A Case of Cone Reconstruction at Primary Biventricular Repair for Neonatal Ebstein Anomaly / 日本心臓血管外科学会雑誌

<p>There are few reports of cone reconstruction for neonatal Epstein's anomaly. We report 14-day-old girl with symptomatic Ebstein's anomaly with prenatal diagnosis prenatally and who was born by caesarean section. Continuous infusion of prostaglandin was started immediately and she was transferred to our institution. The cardiothoracic ratio (CTR) was 74% on chest roentgenograph. The echocardiograph showed severe tricuspid valve regurgitation (TR) and functional pulmonary atresia. Despite aggressive medical therapy, congestive heart failure had advanced and early surgical intervention was required. The anterior leaflet was severely plastered to free the wall, like the Carpentier type C. After cone reconstruction, the intraoperative echocardiography showed mild to moderate TR, antegrade flow to the pulmonary arteries and good function of both ventricles. The patient was extubated on the 6th postoperative day and stayed in the intensive care unit for another 2 days. The CTR was 58%, and the echocardiography showed a well-functioning RV with mild to moderate TR and a right to left transatrial shunt flow with an arterial saturation level of over 90%. Cone reconstruction might even be effective in such border line cases of biventricular repair with dysplastic anterior leaflet of tricuspid valve.</p>

Biventricular Repair after Bilateral Pulmonary Artery Banding as a Rescue Procedure for a Neonate with Hypoplastic Left Heart Complex

Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.

Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review / 华中科技大学学报（医学）（英德文版）

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.