Vladimiro L. Vida

Scimitar Syndrome A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study

Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used. # Clinical Perspective {#article-title-44}Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Surgical outcome for patients with the mitral stenosis–aortic atresia variant of hypoplastic left heart syndrome

OBJECTIVE We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure. METHODS From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Childrens Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis-aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan-Meier method was used to assess survival. RESULTS Thirty-eight (23%) of 165 patients had mitral stenosis-aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis-aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle-subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis-aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate. CONCLUSIONS With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis-aortic atresia variant and left ventricle-subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.

Ventricular Function Deteriorates With Recurrent Coarctation in Hypoplastic Left Heart Syndrome

BACKGROUND Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function. METHODS Retrospectively reviewed were HLHS patients surviving stage I Norwood palliation (stage I) and cavopulmonary shunt (CPS) between January 2004 and February 2007. Echocardiographic right ventricular fractional area change (RV-FAC) was used to evaluate ventricular systolic function after stage I, before CPS, and before Fontan procedures. Cardiac catheterization and magnetic resonance imaging data before CPS were reviewed to assess re-CoA, using a coarctation index (CI = isthmus diameter/descending aortic diameter). RESULTS Fifty-one patients were included, and 21 had a CI of less than 0.75 (mean, 0.82 +/- 0.19; 21). Twelve patients required arch balloon dilation between CPS and Fontan. The change of RV-FAC for all patients between stage I and CPS was -2.2% +/- 9.6%. Pearson correlation coefficient demonstrated a significant correlation between lower CI values and lower RV-FAC at the pre-CPS echocardiogram (r = .35, p = 0.03); and lower CI values and greater decrease in RV-FAC between stage I and pre-CPS evaluation (r = 0.40, p = 0.018). At follow-up pre-Fontan, RV-FAC for patients who underwent balloon dilation for re-CoA recovered to a level that was inferior but not significantly different from that of patients who did not need balloon dilation. CONCLUSIONS Recurrent aortic arch obstruction after stage I for HLHS is associated with worse RV systolic function at the time of stage II operation. Timely intervention on the re-CoA results in recovery of RV function.

Congenital cardiac disease in children with Down's syndrome in Guatemala

BACKGROUND Congenital cardiac disease is the greatest cause of death in patients with Downs syndrome during the first two years of life, with from two-fifths to two-thirds of those with Downs syndrome also having congenital cardiac malformations. The lesions within the heart can be single or multiple. Our objective was to evaluate the frequency and type of such congenital cardiac malformations in patients born with Downs in Guatemala, and to provide baseline information for further research. METHODS We reviewed all patients with Downs syndrome who underwent a cardiologic screening examination between January, 1997, and December, 2003, in the only department dealing with Paediatric Cardiology in Guatemala. RESULTS Of the 349 patients reviewed, 189 (54.1 per cent) also had an associated congenital cardiac malformation. The median age at diagnosis was 6 months, with a range from 2 to 13 months. In 152 patients (80.4 per cent), the cardiac lesion was isolated, while 37 patients (19.6 per cent) had multiple defects. The most common single defect was patency of the arterial duct, found in 54 of the 189 patients (28.6 per cent), followed by ventricular septal defect in 27.5 per cent, atrial septal defect in 12.7 per cent, and atrioventricular septal defect with common atrioventricular junction in 9.5 per cent. The most frequent concomitant malformation found co-existing with other congenital cardiac lesions was patency of the arterial duct, found in 17.5 per cent. CONCLUSIONS As far as we are aware, ours is the first epidemiologic study concerning the frequency and type of congenital cardiac disease found in Guatemalan children with Downs syndrome. The high frequency of patency of the arterial duct, and the differential distribution of the cardiac malformations associated with Downs syndrome among Guatemalan children, differ from what has been reported in the United States of America, Europe, and Asia. This difference warrants further research.

Repair of congenital mitral valve dysplasia in infants and children: is it always possible?

OBJECTIVES Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. METHODS Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days-18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). RESULTS Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months-12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. CONCLUSIONS Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses.

Is there an optimal timing for surgical ligation of patent ductus arteriosus in preterm infants

BACKGROUND We sought to define the variables associated with hospital outcome in preterm infants with patent ductus arteriosus (PDA) and identify the optimal timing for PDA closure to improve hospital outcome. METHODS Included were 201 premature babies (< or = 32 weeks gestational age), from January 2001 to June 2007, with PDA who received primary medical treatment with ibuprofen. Number of ibuprofen cycles, gestational age, body weight, and presence of symptomatic hypotension requiring vasoactive/inotropic drugs were related to hospital outcome, including hospital mortality, presence of necrotizing enterocolitis, acute renal failure, intraventricular hemorrhage, retinopathy and bronchopulmonary dysplasia at week 36. Data were analyzed with a logistic regression model. RESULTS Medical treatment was effective in 149 patients (75%), but 52 (25%) required surgical ligation after medical treatment failed. They had younger gestational age (25 weeks [IQR, 24 to 27 weeks] vs 27 weeks [IQR, 25 to 28 weeks], p < 0.0001), lower body weight at birth (730 g [IQR, 595 to 915 g] vs 840 g [IQR, 670 to 1016], p = 0.05), and a higher incidence of symptomatic hypotension (38 of 52 [73%] vs 56 of 149 [38%], p < 0.0001) than patients who responded to ibuprofen. More than two cycles of ibuprofen was significantly associated with an increased risk for bronchopulmonary dysplasia (odds ratio [OR], 2.81; p = 0.03) and acute renal failure (OR, 3.81; p = 0.09). CONCLUSIONS The prolonged patency of the ductus arteriosus in preterm infants is related to an increased morbidity. Surgical ligation of PDA is a safe and effective treatment and should be done soon after two complete cycles of ibuprofen, especially in selected patients, to improve clinical outcome.

Double crisscross sternal wiring and chest wound infections: a prospective randomized study.

OBJECTIVE We sought to assess the efficiency of 2 different sternal wiring techniques in preventing deep sternal wound infection or sternal instability. METHODS Seven hundred patients were randomized to 2 different groups according to chest-closure techniques. Three hundred fifty patients who underwent a peristernal double crisscross wire closure were included in group X, whereas 350 patients who underwent a standard transsternal closure were included in group T. After sternal closure, the technique for wound suturing was the same for both groups, namely triple-layer sutures up to the intracutaneous skin. All data were prospectively collected and entered in our institute database. RESULTS The 2 groups of patients were comparable for sex, age, preoperative risk factors, and operative procedures. The overall mortality rate was 4.3% in group X and 4.6% in group T. Postoperative morbidity and mortality were comparable between the 2 groups, unlike for sternal wound complications. None of the patients included in group X had superficial or deep wound complications, whereas in group T 7 (2%) patients presented with a superficial sternal wound infection, 6 (1.7%) presented with a deep chest wound infection with sternal instability requiring re-exploration (P <.05), and 3 presented with a sternal instability caused by sternum disruption without infection. Among patients with deep wound infection and sternal instability, 1 patient died, resulting in a mortality rate of 16.7%. CONCLUSIONS The peristernal double crisscross wiring technique achieved a greater sternal stability, resulting in a lower incidence of wound infection in association with triple-layer closure of suprasternal tissues.

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study

Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate ( P= 0.006). Overall mortality was associated to malignancy ( P =0.0008), and adverse events during follow-up ( P =0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. # Clinical Perspective {#article-title-51}Background— To evaluate indications and results of surgery for primary cardiac tumors in children. Methods and Results— Eighty-nine patients aged ⩽18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). Conclusions— Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.

HeartWare Ventricular Assist Device as Bridge to Transplant in Children and Adolescents

Limited options do exist for mechanical circulatory support as a bridge to transplantation in the pediatric population. This is especially true when it comes to intracorporeal technologies. We describe our successful experience with the use of HeartWare ventricular assist devices (HeartWare, Inc., Framingham, MA, USA) in three patients <16 years of age, as a successful and feasible bridge to transplant in patients weighing greater than 20 kg.

Evolving strategies for preserving the pulmonary valve during early repair of tetralogy of Fallot: mid-term results.

BACKGROUND The aim of the study was to evaluate our results with pulmonary valve (PV) preservation in selected patients with tetralogy of Fallot (TOF). METHODS From January 2007, 69 patients who underwent early transatrial TOF repair were enrolled in the study. The patients were divided into 2 groups: PV preservation by PV annulus balloon dilation (group 1) and PV cusp reconstruction after annular incision (group 2). RESULTS Thirty-four patients underwent a successful PV annular preservation (49%). Median age at surgery was 113 days (range, 36-521 days) (group-1 vs group-2, P = not significant). Median preoperative PV Z score was -3.15 (range, -0.95 to -5.62) (group-1 vs group-2, P = .03). Median intensive care unit and hospital stays were 3 and 10 days, respectively (group 1 vs group 2, P = not significant). Median follow-up time was 580 days (range, 189-1940 days) (group 1 vs group 2, P = .08). Two patients were reoperated for residual right ventricular outflow tract (RVOT) obstruction (1 in group 1 and 1 in group 2). The remaining patients are alive and well. Median peak RVOT gradient was 25 mm Hg (range, 8-60 mm Hg) (group 1 vs group 2, P = not significant). The degree of PV regurgitation in group 1 was none/mild in 24 patients (80%) and moderate in 6 (20%) and was none/mild in 8 patients (25%), moderate in 11 (34.4%), and severe in 13 (28.6%) in group 2 (P = .001). Median right ventricular fractional area change was 55% (range, 42%-70%) in group 1 and 50% (range, 40%-63%) in group 2 (P = .003). CONCLUSIONS The integrity and function of the PV can be preserved in selected patients during early repair of TOF by concomitant balloon dilation, leading to a better mid-term right ventricular function.