Management of Moderate Functional Tricuspid Valve Regurgitation at the Time of Pulmonary Valve Replacement: Is Concomitant Tricuspid Valve Repair Necessary?

Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve. Patients often undergo subsequent pulmonary valve replacement (PVR) to protect the heart from the consequences of pulmonary regurgitation. In the presence of associated moderate functional tricuspid valve (TV) regurgitation, it is unclear what factors contribute to persistent TV regurgitation following PVR. In particular, it is unknown whether PVR alone will reduce the right ventricular (RV) size and restore TV function or whether concomitant TV annuloplasty is required as well. Thirty-five patients were analyzed. Each patient underwent initial palliation of congenital pulmonary stenosis or tetralogy of Fallot, underwent subsequent PVR between 2002 and 2008, and had at least moderate TV regurgitation at the time of valve replacement. Serial echocardiograms were analyzed. Pulmonary and TV regurgitation, along with RV dilation and dysfunction, were scored (0, none; 1, mild; 2, moderate; 3, severe). RV volume and area were also calculated. Potential risk factors for persistent postoperative TV regurgitation were evaluated. One month following PVR, there was a significant reduction in pulmonary valve regurgitation (mean, 3 vs. 0.39; P < 0.0001) and TV regurgitation (mean, 2.33 vs. 1.3; P < 0.0001). There were also significant reductions in RV dilation, volume, and area. There were no significant further improvements in any of the parameters at 1 and 3 years. There was no difference in the degree of TV regurgitation postoperatively between those patients who underwent PVR alone and those who underwent concomitant tricuspid annuloplasty (mean, 1.29 vs. 1.31; P = 0.81). We conclude that following PVR, improvement in TV regurgitation and RV size occurs primarily in the first postoperative month. TV function improved to a similar degree with or without annuloplasty.     

Mitral Valve Replacement with the Pulmonary Autograft in Children: A Word of Caution

A 4½-month-old patient who underwent mitral valve replacement for congenital mitral stenosis using a pulmonary autograft is reported. Failure of the autograft resulted in pulmonary hypertension, leading to pulmonary regurgitation in the reconstructed right ventricular outflow tract, then tricuspid regurgitation, refractory right heart failure, and death. Caution should be exercised in applying this procedure with children, particularly those at risk for pulmonary hypertension.     

Completion Angiography After Cardiac Surgery for Congenital Heart Disease: Complementing the Intraoperative Imaging Modalities

Residual structural pathology after cardiac surgery for congenital heart disease may complicate postoperative recovery. Completion angiograms obtained in the operating room may facilitate early detection and therapy of residual structural abnormalities. Our objective here is to report our institutional experience performing completion angiograms after cardiopulmonary bypass surgery. Between October 2007 and August 2008, 31 patients underwent completion angiograms after 32 cardiac surgical procedures. The median age was 7.5 months (range, 50 days to 31.2 years) and the median weight was 6.5 kg (range, 3.1–153 kg). Type of procedure, angiographic findings, and therapeutic decision were retrospectively reviewed. Procedures (proc) evaluated through completion angiography included comprehensive stage II or Glenn (n = 13), aortic arch reconstruction/conduit (n = 3), repair/palliation of tetralogy of Fallot or pulmonary atresia with ventricular septal defect (n = 4), PVR or conduit replacement (n = 5), and others (n = 7). Unexpected pathology was identified in 18 of 32 (56.3%) proc, which included left pulmonary artery (LPA) stenoses (n = 15), right pulmonary artery (RPA) stenoses (n = 11), and stenosis impairing coronary blood flow (after DKS; n = 1). In 9 of 32 (28.1%) proc, findings may have led to a change in therapeutic management. This included surgical revision (n = 1), ‘Hybrid’ therapy in the same setting (n = 2: LPA stent, 1; RPA balloon, 1), early catheterization within 3 months (n = 4), and change in medical management (n = 2: change in anticoagulation, 1; early CT, 1). Complications related to completion angiography were seen in only a single procedure (LPA staining). In conclusion, completion angiograms using a dedicated Hybrid cardiac operating suite may aid in early diagnosis and therapy of postsurgical abnormalities. They complement other methods of intraoperative imaging and may reduce the potential need for early surgical or transcatheter reintervention.     

Management of Congenital Aortic Stenosis

72 patients with congenital aortic stenosis whose ages ranged from 1 month to 15 years were under supervision for periods ranging from several months to more than 9 years. The obstruction was subvalvar in 3. Additional malformations were aortic regurgitation, persistent ductus arteriosus, coarctation of the aorta, ventricular septal defect, partial anomalous pulmonary venous drainage, and mitral stenosis. Bacterial endocarditis complicated 3. Of 5 deaths unrelated to operation, 3 were sudden. Surgical treatment was carried out in 12 patients (commissurotomy, valve replacement with or without previous commissurotomy, subvalvar resection) with 2 deaths. 52 patients remained symptom-free. Detailed investigation to assess the patient for surgical treatment is indicated if symptoms are present or electrocardiographic evidence of severe left ventricular hypertrophy appears. Commissurotomy is, where possible, deferred until an age at which valve replacement can be undertaken if the stenosis cannot be adequately relieved without causing substantial regurgitation.     

Midterm Results of the Ross Procedure in a Pediatric Population: Bicuspid Aortic Valve Is Not a Contraindication

Our objective was to analyze retrospectively the short- and midterm results of the Ross operation in children and the impact of bicuspid aortic valve (BAV) disease on outcome. From 1991 to 2003, 41 patients (26 male, 15 female) underwent a Ross procedure. Aortic disease was congenital in all but one. Sixty-six percent had BAV. Mean age at operation was 10.13 ± 5.6 years (range, 0.4–18.3 years). Root replacement technique was performed in all but two (inclusion technique). There were two early deaths. Mean follow-up was 6 ± 3.8 years (range, 0.1–14 years). The autograft (neo-aorta) presented absent, trivial, mild, and moderate regurgitation in 42%, 46%, 10%, and 2%, respectively, at latest follow-up. Root dilation was seen in 64% of the patients (mean Z-score, +3.53 ± 0.04). Four patients (9%) required allograft replacement, two for endocarditis and two for stenosis. Allograft stenosis (gradient >20 mmHg) was detected in 44% of the remaining patients, without symptoms or the need for reintervention. Estimated freedom from allograft replacement at 5 and 10 years was 97% and 89%. Left ventricular dimensions and function were normal in all patients. No difference was found between patients with BAV and those with tricuspid aortic valves in aortic regurgitation or root dilation. BAV was not identified as a risk factor for root dilation. In conclusion, the Ross operation remains an excellent option for aortic valve replacement in children. BAV is not associated with a worse outcome and is not a risk factor for aortic root dilation at medium-term follow-up. Long-term follow-up is, however, required.     

Clinical Experience of Chest Pain in Adults with Congenital Heart Disease in a Single Tertiary Center

The aim of this study was to investigate clinical manifestation and causes of chest pain in adults with congenital heart disease. Of 966 patients in our Adult Congenital Heart Clinic, 50 patients had cyanosis (cyanosis [+]), and 916 patients did not have cyanosis (cyanosis [−]). Fifty-four patients (16 male and 38 female) with a history of chest pain requiring medical care were selected: 33 were in the cyanosis (+) group, and 21 were in the cyanosis (−) group. Medical records were reviewed. The causes of chest pain were (no. cyanosis [+], no. cyanosis [−], respectively) as follows: idiopathic (n = 19, n = 10), pulmonary hemorrhage (n = 6, n = 0), pulmonary tuberculosis (n = 2, n = 0), pulmonary thromboembolism (n = 4, n = 0), localized musculoskeletal (n = 1, n = 1), myocardial ischemia (n = 1, n = 1), and arrhythmia (n = 2, n = 9). Patients with cyanosis had much higher frequency of chest pain compared with patients without cyanosis (33 of 50 vs. 21 of 916, p < 0.0001). Chest pain in adults with congenital heart disease has diverse causes. Meticulous evaluation of chest pain in adults with congenital heart disease is required to differentiate correctable causes from idiopathic or incurable ones.     

婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

Quantitative Assessment of Pulmonary Vascular Resistance and Reactivity in Children with Pulmonary Hypertension Due to Congenital Heart Disease Using a Noninvasive Method: New Doppler-Derived Indexes

We assessed the usefulness of transthoracic Doppler-derived indexes obtained in the proximal pulmonary artery (PA) branch for estimating pulmonary vascular resistance (PVR) in 45 children with congenital heart disease (CHD) and 23 normal control subjects. The acceleration time, inflection time (InT), deceleration index, and peak velocity, which were measured from the systolic PA flow velocity curve obtained at the sites of the main PA, and right and left PA, were compared with the PVR in patients with CHD. In addition, changes in either Doppler-derived indexes or PVR during 100% oxygen administration were compared in 22 patients showing a baseline PVR ≥4.6 U/m² (high PVR). The heart-rate-corrected InT (InTc) values obtained in the left PA in the high PVR group were significantly lower than those in the main PA (4.7 ± 1.5 vs. 7.5 ± 3.0; p < 0.001). The InTc obtained from the left PA separated patients with high and low PVR (4.7 ± 1.4 vs. 9.9 ± 2.4; p < 0.001) and no significant differences in InTc were found between the low PVR and the control groups. An increase in InTc to >6 during 100% oxygen administration for the high PVR group indicated good PA reactivity with a sensitivity of 93%, specificity of 100%, and agreement of 95% (κ = 0.83). Moreover, this InTc index correlated inversely with PVR (r = –0.80). In conclusion, our method can noninvasively separate high and low PVR and assess the PA reactivity for high PVR in children with CHD.     

Isolated Congenital Absence of a Single Pulmonary Valve Cusp

Isolated absence of a single pulmonary valve is extremely unusual. We present a 16-year-old male with the absence of one cusp resulting in significant pulmonary regurgitation, right ventricular enlargement, and dilatation of the main pulmonary artery and its branches. Surgery consisted of creating a valve cusp from the posterior wall of the pulmonary artery at the base of the pulmonary trunk. Follow-up at 3.5 years revealed good results with only mild pulmonary stenosis and regurgitation.     

Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve

Deleterious long-term effects of pulmonary regurgitation after tetralogy of Fallot (TOF) repair have become evident during the last two decades. Subsequently, different groups have developed strategies aimed at preserving the pulmonary valve function. However, the results of these approaches are not well known. From July 2009 through March 2012, 38 patients underwent primary repair of TOF at the authors’ institution. Of these, 12 children (7 boys) underwent attempted pulmonary valve-sparing surgery with intraoperative dilation of the pulmonary valve. The technical details as well as the echocardiographic preoperative and follow-up data for this repair were recorded, with a special focus on the feasibility of the technique and the effects on pulmonary valve function. No patient in the series died. At repair, the median age was 6 months (range 3.4–126 months), and the median weight was 7.6 kg (range 4.7–47 kg). Intraoperative dilation of the pulmonary valve was technically feasible for all the patients. Two patients had unsuccessful dilation and underwent a transannular patch procedure. During a median follow-up period of 22 months (range 6–30 months), the pulmonary valve diameter and z-score improved significantly. Moreover, the annular size normalized, whereas the mean right ventricular outflow tract (RVOT) gradient remained at the mild level (median, 24 mmHg; range 12–36 mmHg). At the most recent follow-up evaluation, three patients showed moderate pulmonary regurgitation. Intraoperative dilation of the pulmonary valve in patients undergoing TOF repair is feasible and provides good relief of obstruction. Moreover, the pulmonary valve annulus grows through the follow-up period. Longer follow-up studies are needed to evaluate the exact role of this strategy in this population.     

Congenital Muscular Ventricular Septal Aneurysm: Report of Four Cases and Review of the Literature

We describe a rare association between congenital muscular ventricular septal aneurysm (MuVSA) and right heart lesions, with emphasis on echocardiographic diagnosis, clinical presentation, and outcome. Our four cases of MuVSA occurred in combination with either pulmonary atresia with intact ventricular septum (PAIVS; three patients) or absent pulmonary valve syndrome with severe tricuspid stenosis (one patient). MuVSA and right heart lesions were detected in utero in three patients. The three patients with PAIVS and MuVSA survived with no significant morbidity from the septal aneurysm at 3- to 5-year follow-up. The presence of MuVSA did not negatively affect the outcome of our patients.     

The Use of High-Resolution Computed Tomography in the Evaluation of Pulmonary Hemodynamics in Patients with Congenital Heart Disease: In Pulmonary Vessels Larger Than 1 mm in Diameter

High-resolution computed tomography (HRCT) was carried out in 36 patients with congenital left-to-right shunt disease and 10 normal control subjects to assess the feasibility of CT in the evaluation of pulmonary hemodynamics. The patients had a left-to-right or a bidirectional shunt and the hemodynamic data obtained by cardiac catheterization in these patients were compared to the information obtained by CT imaging. The pulmonary/systemic blood flow (Q _p/Q _s) ratio and pulmonic/systemic resistance (R _p/R _s) ratio had a significant correlation with the pulmonary artery/bronchus (PA/Br) ratio (r= 0.54 and r=−0.37, respectively) and pulmonary vein/bronchus (PV/Br) ratio (r= 0.66 and r=−0.66, respectively), and the R _p/R _s and mean PA pressure also showed a significant correlation with the PA/PV ratio (r= 0.53 and r=−0.61, respectively) in the mid-lung field when accompanying bronchi were 4.0–5.9 mm in diameter. There was no correlation between the hemodynamic data and the size of the central and hilar PA or with the rate of PA tapering. With HRCT, it is possible to evaluate pulmonary hemodynamics in patients with congenital heart disease with a left-to-right or bidirectional shunt, particularly R _p/R _s and mean PA pressure, which have been very difficult to obtain noninvasively. The small-sized pulmonary vessel/Br ratio or the small-sized PA/PV ratio could offer very useful information, but the dimension of the central PA provided the least useful information.     

Porcine Bioprosthetic Valve in the Pulmonary Position: Mid-Term Results in the Right Ventricular Outflow Tract Reconstruction

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors’ experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18–64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7–48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6–132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries.     

Tetralogy of Fallot and Aortic Root Dilation: A Long-Term Outlook

Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1–48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥4 cm in 39 % (42 of 109), ≥4.5 cm in 21 % (23 of 109), ≥5 cm in 8 % (9 of 109), and ≥5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.     

Progression of Aortic Regurgitation After Different Repair Techniques for Congenital Aortic Valve Stenosis

We sought to characterize the incidence of AR progression and determine risk factors for AR progression in a consecutive series of infants and children after surgical correction of congenital aortic valvular and supravalvular stenosis. N = 30 patients underwent repair of the aortic valve for isolated congenital aortic valve stenosis (n = 14, 47 %) or combined with aortic regurgitation (AR, n = 16, 53 %). N = 27 (90 %) had a valvular and n = 3 patients (10 %) presented with supravalvular pathology of their aortic valve. In n = 16 patients (53 %) a bicuspid and in n = 2 (6 %) patients, a unicuspid valve was present. Comparative survival was analyzed using the Cox model and log-rank calculations. Log-rank calculations were performed for variables reaching statistical significance in order to identify differences in survival between groups. Commissurotomy was performed in n = 20 patients, patch implantation in n = 4, cusp shaving in n = 8, cusp prolapse correction in n = 3, and cusp augmentation in n = 4 patients. In patients with combined dysfunction and preoperative AR, AR was successfully reduced by the initial procedure, and postoperatively the overall median AR grade was 1+ (range 0–2.5+, p = 0.001, for AR reduction among patients with any grade of preoperative AR). By the time of follow-up echocardiography, the median AR grade had significantly progressed toward 1.5+ (p = 0.004). At the time of mid-term follow-up at 3.2 years, none of the patients had moderate or severe AR grades >2.5+. Patients with a monocuspid aortic valve and patients who had some kind of patch implantation into their cusps or commissures or shaving of thickened cusps were more likely to present with progression of aortic regurgitation. Monocuspid aortic valve and patch implantation, as well as cusp shaving, are probably linked to AR progression. The standard procedure of commissurotomy results in an absolute rate of AR progression of 40 % over a medium-term follow-up period.     

Prevalence of Congenital Heart Diseases in Oral Cleft Patients

To establish the prevalence of congenital heart diseases (CHDs) in cleft patients, the type of cleft and the presence of a syndrome were coded in 220 patients. A Doppler echocardiogram with color-flow mapping (DE) was obtained in all patients. Mean age was 112.0 ± 101.2 months (range, 1–576 months), and 56.8% (125) were males. Cleft lip and palate occurred in 144 patients (65.5%), cleft lip in 40 (18.2%), and cleft palate in 36 (16.4%). Cleft palates were more frequent among females. Twenty-four CHDs were diagnosed in 21 of 220 patients (9.5%): 7 mitral valve prolapses, 6 atrial septal defects, 4 patent ductus arteriosus, 3 ventricular septal defects, 2 cases of tetralogy of Fallot, 1 pulmonary stenosis, and 1 bicuspid aortic valve. The presence of CHD did not correlate with the type of clef. Syndromes occurred in 28 patients (12.7%), and this association was higher among patients with a cleft palate.     

Utility of Intraoperative Transesophageal Echocardiography in the Assessment of Residual Cardiac Defects

To investigate the accuracy of immediate postbypass transesophageal echocardiography in the assessment of residual cardiac defects, we compared intraoperative transesophageal echocardiograms with intra/postoperative data in 86 patients, aged 4 days to 30.7 years (median = 1.4 years), at risk for a total of 174 postoperative lesions: right (n= 55) or left (n= 26) ventricular outflow tract obstruction, ventricular septal defect (n= 65), aortic (n= 12) or mitral regurgitation (n= 8), or mitral stenosis (n= 8). Accuracy of intraoperative transesophageal echocardiography was evaluated based on comparison with (1) immediate post-bypass left (n= 4) or right (n= 9) ventricular outflow tract pressure gradients by pullback in the operating room, (2) direct surgical inspection of residual ventricular septal defects (n= 3), (3) pulmonary artery oxygen saturation (n= 49), (4) right ventricular outflow tract pullback gradient (n= 24), and (5) transthoracic echocardiogram (n= 51) performed within 40 days of surgery. The results indicate that intraoperative transesophageal echocardiography agreed with intra/postoperative data in 87% of patients at risk for right ventricular outflow tract obstruction, 96% at risk for left ventricular outflow tract obstruction, 97% at risk for ventricular septal defect, and 100% at risk for aortic regurgitation, mitral regurgitation, or mitral stenosis. Significant residual lesions led to immediate surgical revision in 11 cases: 3 ventricular septal defects, 6 right and 2 left ventricular outflow tract obstructions. Of these, intraoperative transesophageal echocardiography confirmed and quantified suspected residual lesions in 7 and identified unsuspected lesions in 4 cases. Immediate postbypass transesophageal echocardiography proved reliable for assessing residual ventricular septal defect, mitral stenosis, and mitral or aortic regurgitation. Although accurate for assessment of the left and right ventricular outflow tracts in most patients, transesophageal echocardiography may not reliably reflect the severity of obstruction in all cases.     

Eisenmenger Ventricular Septal Defect: Classification, Morphology, and Indications for Surgery

This study aimed to examine the definition and indications for surgery, to elucidate the morphologic substrate of aortic regurgitation, and to extrapolate the pathologic mechanisms of subpulmonary stenosis in Eisenmenger ventricular septal defect (EVSD). The study enrolled 160 patients. Preoperative respiratory symptoms and poor growth were present in 41 patients (26%), and 21 patients (13%) required mechanical ventilation. Perimembranous ventricular septal defect (pVSD) had been diagnosed previously for 136 of the patients (85%) at other institutions. Of the 160 patients, 51 (32%) had muscular posteroinferior rims. Aortic regurgitation was experienced by 36 patients (23%), found to be mild in 31 cases (19%) and moderate in 5 cases (3%). None of the patients had severe regurgitation. No aortic valvuloplasty was performed. The significant risk factors for aortic regurgitation were subpulmonary stenosis (p = 0.001) and a muscular posteroinferior rim (p = 0.000). Subpulmonary stenosis was seen in 57 patients (35%), found to be mild to moderate in 42 cases (26%) and severe in 15 cases (9%). Adequacy of the stenosis band was repaired through the tricuspid valve for 57 of these patients. The definition of EVSD should identify it as a subgroup different from pVSD, and it should be closed as soon as it is identified in developing countries. Aortic regurgitation occurs rarely, and aortic valvoplasty should be performed if it exceeds a moderate level. The subpulmonary stenosis can be repaired through the tricuspid valve.     

Results of Surgical Treatment of Congenital Heart Defects in Children with Down's Syndrome

We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations. The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients. These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.     

Outcomes for Patients with Unbalanced Atrioventricular Septal Defects

Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors’ institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period (P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies.