Fontan Revision: Presurgical Planning Using Four-Dimensional (4D) Flow and Three-Dimensional (3D) Printing.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

Decline of Systolic and Diastolic 2D Strain Rate During Follow-Up of HLHS Patients After Fontan Palliation.

Accurate assessment of ventricular function is particularly important in children with hypoplastic left heart syndrome (HLHS) after completion of the total cavopulmonary connection (TCPC). For this purpose, two-dimensional speckle tracking (2DST) is a promising technique as it does not depend on the angle of insonation or the geometry of the ventricle. The objective of this study was to assess changes in systolic and diastolic right ventricular (RV) function within a 5-year follow-up period of HLHS patients after TCPC using conventional and 2DST echocardiography. RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), E/A, E/e' and 2DST parameters [global longitudinal peak systolic strain (GS) and strain rate (GSRs), global strain rate in early (GSRe) and late (GSRa) diastole] of 40 HLHS patients were compared at 1.6 and at 5.1 years after TCPC. RVFAC, E/A, E/e' and GS did not change, whereas TAPSE (13.7 ± 3.2 vs. 10.5 ± 2.4 mm/m(2), p < 0.001), GSRs (-1.56 ± 0.28 vs. -1.35 ± 0.31 1/s, p < 0.001), GSRe (2.22 ± 0.49 vs. 1.96 ± 0.44 1/s, p = 0.004) and GSRa (1.19 ± 0.39 vs. 0.92 ± 0.39 1/s, p < 0.001) decreased significantly. Systolic and diastolic RV function parameters of HLHS patients decreased from 1.6 to 5.1 years after TCPC in our patients. Changes in global strain rate parameters may be signaling early RV dysfunction that is not detectable by traditional echocardiography. Further study is needed to verify this and to determine whether these changes are clinically relevant.

Two-dimensional global longitudinal strain rate is a preload independent index of systemic right ventricular contractility in hypoplastic left heart syndrome patients after Fontan operation.

BACKGROUND: Assessment of systemic right ventricular (RV) function in patients with hypoplastic left heart syndrome is important during long-term follow-up after Fontan repair. Traditional echocardiographic parameters to evaluate systolic ventricular function are affected by loading conditions. The only generally accepted load-independent parameter of systolic function, end systolic elastance (Ees), requires invasive catheterization. Therefore, we sought to determine if parameters obtained by 2-dimensional speckle tracking (2DST) were affected by acute changes in preload and correlated with catheterization-derived indices of RV contractility in hypoplastic left heart syndrome patients after Fontan palliation. METHODS AND RESULTS: Fifty-two patients with hypoplastic left heart syndrome (median age, 6.6; range 2.9-22.2 years) were prospectively enrolled to have echocardiography and conductance catheter studies performed simultaneously. We compared traditional echo, 2-dimensional speckle tracking and catheterization-derived parameters during different states of preload at baseline and during dobutamine infusion. Global longitudinal strain (S) showed a tendency to decrease with preload reduction, whereas global longitudinal strain rate (SR) did not change (S: -17.7 ± 3.4% versus -16.9 ± 3.8%, P=0.08; SR: -1.30 ± 0.29 versus -1.34 ± 0.34 s(-1), P=0.3). S did not change with dobutamine infusion (-17.7 ± 3.4% versus -18.4 ± 3.9%, P=0.24), whereas SR increased significantly (-1.30 ± 0.29 versus -2.26 ± 0.49 s(-1), P<0.001). RV Ees correlated with SR (rs= -0.47, P<0.001), but not with S (rs=0.07, P=0.5) or other echocardiographic parameters. CONCLUSIONS: In contrast to S, SR was not affected by preload and correlated with Ees of the systemic RV. SR may be a useful noninvasive surrogate of RV contractility and suitable for follow-up of patients with hypoplastic left heart syndrome after Fontan palliation.

Arterial elastance and its impact on intrinsic right ventricular function in palliated hypoplastic left heart syndrome.

BACKGROUND: Systemic right ventricular (RV) function is a major determinant of long-term outcome in patients with palliated hypoplastic left heart syndrome (HLHS). Abnormal elastic properties of the reconstructed aorta may negatively impact on ventricular function. We therefore aimed to assess arterial elastance and its relationship to systemic RV function in these patients. METHODS: Fifty-six HLHS patients (median age 5.4 years; range 2.9-14.2 years) were studied at a median of 2.6 years (range 0.8-12.7 years) after completion of the Fontan circulation with the pressure-volume conductance system. RESULTS: Arterial elastance (Ea) was abnormally high and correlated inversely with RV ejection fraction (r=-0.42, P=0.001). However, end systolic elastance (Ees) - a load independent measure of intrinsic systolic ventricular function - and more so end diastolic stiffness (Eed) were positively correlated with Ea (Ees vs. Ea: r=0.44, P=0.001: Eed vs. Ea: r=0.62, P<0.0001). Patients who were treated for significant aortic arch obstruction after surgical palliation showed higher Ea and Eed even four years after successful treatment compared to the remainder of the group (Ea: 3.4 ± 1.2 vs. 2.8 ± 1.0 mmHg/ml, P=0.04 and Eed: 0.67 ± 0.44 vs. 0.45 ± 0.3 mmHg/ml, P=0.04). CONCLUSIONS: Arterial elastance is abnormally high in palliated HLHS patients and negatively impacts on ejection fraction but not on intrinsic systolic RV function early after completion of the Fontan circulation. Increased arterial elastance, however, is associated with increased RV diastolic stiffness with potential adverse effects on long-term outcome. Furthermore, arterial elastance and diastolic stiffness are particularly high in patients who needed treatment for aortic arch obstruction.

Implications of early aortic stiffening in patients with transposition of the great arteries after arterial switch operation.

BACKGROUND: The elastic function of the aorta in patients with transposition of the great arteries after arterial switch operation (ASO) is suspected to be important for long-term prognosis. METHODS AND RESULTS: Fifty-one patients and 34 controls were studied at 3.0 Tesla with MRI. Forty-three patients (12.8±6.9 years) underwent 1-stage ASO, 8 patients (23.8±6.9 years) had prior pulmonary artery banding (2-stage ASO). Aortic dimensions, distensibility, pulse wave velocity, aortic arch angle, left ventricular (LV) mass, LV systolic function and left atrial (LA) volumes, and LA passive emptying function as marker of LV diastolic function were assessed. Compared with controls, patients had increased aortic root areas (602.6±240.5 versus 356.8±113.4 mm(2)/m; P<0.01) and reduced distensibility of the thoracic aorta most pronounced at the aortic root (3.2±2.0 versus 9.1±4.7×10(-3) mm Hg(-1); P<0.01). Aortic distensibility correlated negatively with the aortic areas (P<0.01). Pulse wave velocity was higher in adults after ASO (5.0±1.0 versus 3.8±1.3 m/s; P<0.01). In contrast to controls pulse wave velocity and distensibility correlated with age in patients (P=0.04 to <0.01), LV mass was higher in patients (P=0.02). LA volumes correlated negatively with aortic root and ascending aortic distensibility and positively with pulse wave velocity (P<0.05). In patients, LA passive emptying function was lower (27.3±8.9 versus 41.1±6.0; P<0.01) and correlated with aortic root distensibility (P=0.004). CONCLUSIONS: Reduced aortic bioelasticity and aortic root dilatation are present in transposition of the great artery patients post ASO and are likely to contribute to LV diastolic dysfunction. Impaired aortic bioelasticity was strongly associated with age, suggesting the usefulness of follow-up studies for early onset of degenerative cardiovascular disease.

Does left ventricular size impact on intrinsic right ventricular function in hypoplastic left heart syndrome?

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index ≤ 1.33 cm(2)/m(2), n=29; group 2: LV area index>1.33 cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70 ± 1.92 vs. 3.68 ± 2.68 mmHg/ml; Mw: 52.75 ± 14.98 vs. 51.09 ± 16.63 mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40 ± 0.26 vs. 0.52 ± 0.45; group 2: 0.68 ± 0.44 vs. 0.47 ± 0.38 mmHg/ml, P<0.01). Furthermore Eed was lowest in patients with mitral atresia/aortic atresia, the anatomic subgroup with the smallest LV remnant. CONCLUSIONS: Intrinsic systolic RV function is not affected by the size of the hypoplastic LV in survivors of surgical palliation of HLHS. Diastolic stiffness, however, was higher in patients with a larger LV remnant.

Fetal hypoplastic left heart syndrome and maternal liver transplantation for Wilson's disease: a case report.

INTRODUCTION: Liver transplantation currently represents the only curative treatment for Wilson's disease. A lifelong immunosuppressive therapy is mandatory. In spite of increased maternal and fetal risks, pregnancies after liver transplantation have been reported with favorable perinatal outcomes. Hypoplastic left heart syndrome is a spectrum of congenital heart defects that results in the inability to support the systemic circulation. Although its etiology remains elusive, the prognosis of this previously fatal condition has dramatically improved over the last 2 decades mainly due to advances in prenatal diagnosis, surgical technique and perioperative care. CASE PRESENTATION: We present a case of a Caucasian 26-year-old woman, gravida 2, para 1 at 36+0 weeks of gestation who had received a liver transplantation due to Wilson's disease and subsequently delivered a child with hypoplastic left heart syndrome. CONCLUSIONS: This coincidence of medical conditions has not been described in the literature so far and its implications for mother and child as well as the pathophysiological mechanisms are discussed on the basis of a literature review.

Normal values of aortic dimensions, distensibility, and pulse wave velocity in children and young adults: a cross-sectional study.

BACKGROUND: Aortic enlargement and impaired bioelasticity are of interest in several cardiac and non-cardiac diseases as they can lead to cardiovascular complications. Cardiovascular magnetic resonance (CMR) is increasingly accepted as a noninvasive tool in cardiovascular evaluation. Assessment of aortic anatomy and bioelasticity, namely aortic distensibility and pulse wave velocity (PWV), by CMR is accurate and reproducible and could help to identify anatomical and bioelastic abnormalities of the aorta. However, normal CMR values for healthy children and young adults are lacking. METHODS: Seventy-one heart-healthy subjects (age 16.4 ± 7.6 years, range 2.3-28.3 years) were examined using a 3.0 Tesla CMR scanner. Aortic cross-sectional areas and aortic distensibility were measured at four positions of the ascending and descending thoracic aorta. PWV was assessed from aortic blood flow velocity measurements in a aortic segment between the ascending aorta and the proximal descending aorta. The Lambda-Mu-Sigma (LMS) method was used to obtain percentile curves for aortic cross-sectional areas, aortic distensibility and PWV according to age. RESULTS: Aortic areas, PWV and aortic distensibility (aortic cross-sectional areas: r = 0.8 to 0.9, p < 0.001; PWV: r = 0.25 to 0.32, p = 0.047 to 0.009; aortic distensibility r = -0.43 to -0.62, p < 0.001) correlated with height, weight, body surface area, and age. There were no significant sex differences. CONCLUSIONS: This study provides percentile curves for cross-sectional areas, distensibility and pulse wave velocity of the thoracic aorta in children and young adolescents between their 3rd and 29th year of life. These data may serve as a reference for the detection of pathological changes of the aorta in cardiovascular disease.

Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome.

OBJECTIVE: The Norwood procedure, the first surgical step of staged palliation for hypoplastic left heart syndrome, is also applied for other complex single-ventricle lesions with systemic outflow tract obstruction or aortic arch hypoplasia. We reviewed our 15-year institutional experience with the Norwood procedure for patients with and without hypoplastic left heart syndrome. METHODS: A total of 41 patients without hypoplastic left heart syndrome and 212 patients with hypoplastic left heart syndrome who underwent a Norwood procedure between January 1996 and December 2010 were enrolled. Full medical records were reviewed to assess the determinants of outcome. RESULTS: Early failure (death or cardiac transplantation) was 7% in patients without hypoplastic left heart syndrome and 13% in patients with hypoplastic left heart syndrome (P = .29). Frequency of postoperative complications, duration of postoperative ventilation, and length of vasoactive drug treatment were not different between groups. Transplant-free survival until the second operative step trended to be higher for patients without hypoplastic left heart syndrome (92% vs 80%, P = .067). Recurrent aortic arch obstruction was more common in patients without hypoplastic left heart syndrome (15/39 vs 32/171, P = .008), but there were 4 patients with stenosis of the proximal aortic arch. In subsequent procedures, 31 patients without hypoplastic left heart syndrome underwent superior cavopulmonary anastomosis and 5 biventricular repair. Overall transplant-free survival was not different between groups (P = .119) but trended to be higher in patients with a systemic or substantial left ventricle remnant contributing to cardiac output (P = .082). CONCLUSIONS: Early and long-term survivals and postoperative complications were similar between patients with and without hypoplastic left heart syndrome undergoing a Norwood operation. Recurrent aortic arch obstruction was common in both groups but more prevalent in patients without hypoplastic left heart syndrome.

Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome.

OBJECTIVES: While hospital mortality after the Norwood operation for hypoplastic left heart syndrome (HLHS) has decreased steadily, interstage mortality until the superior cavopulmonary anastomosis (SCPA) remains a major concern. Our aim was to institute a home surveillance programme to decrease interstage mortality. METHODS: We enrolled 45 HLHS patients surviving the Norwood operation into our home surveillance programme and compared them with 97 patients treated before the initiation of the programme and with a third group of 20 patients not discharged between the first- and the second-stage operation. While still in hospital, parents were taught to record weight and fluid intake as well as oxygen saturations with the help of a vital sign monitor. During the last week of the hospital stay, the following criteria had to be met: oxygen saturation >75%, weight gain of at least 20-30 g in 3 days and a maximum weight loss of 30 g in a day. After discharge, these criteria had to be maintained at all times or the parents were supposed to call our hospital. Additionally, an experienced paediatric cardiologist from our centre called the parents at home once a week. RESULTS: Interstage mortality was reduced significantly from 12.4% (12/97) to 2.2% (1/45) (P = 0.042). The number of patients, who were not discharged before the SCPA, was significantly higher after the start of the home surveillance programme (12/57 vs. 8/105, P = 0.022). After discharge, 14 (31%) infants breached the surveillance criteria. Of these, one patient died and eight patients were operated earlier (SCPA, n = 6; shunt replacement, n = 2). The remaining five patients could be discharged home after observation. Children in the home surveillance programme were younger [102 (67-299) vs. 152 (77-1372) days, P = 0.001] and weighed less (5.09 ± 0.79 vs. 5.75 ± 1.22 kg, P = 0.001) at the SCPA compared with the remainder. Early survival after SCPA was not different. CONCLUSIONS: The home surveillance programme led to an important decrease in interstage mortality. The adherence to the surveillance criteria before discharge resulted in a larger number of patients receiving inpatient treatment until SCPA. Earlier SCPA in the surveillance group had no negative impact on early survival after SCPA.

Comparison of longitudinal myocardial deformation and dyssynchrony in children with left and right ventricular morphology after the Fontan operation using two-dimensional speckle tracking.

OBJECTIVE: Two-dimensional speckle tracking can assess ventricular deformation independent of ventricular geometry. Using this technique, the aim of our study was therefore to compare global and regional longitudinal deformation and intraventricular dyssynchrony between children with left and right ventricular morphology after Fontan operation. DESIGN AND PATIENTS: In this retrospective study, we examined 29 children with systemic right (group 1: age 7.7 ± 2.7 years, time after Fontan 5.3 ± 3.0 years) and 22 children with systemic left (group 2: age 7.8 ± 4.8 years, time after Fontan 4.6 ± 4.2 years) ventricles after Fontan surgery using two-dimensional speckle tracking. We compared global and regional longitudinal strain and strain rate (SR) as well as time to peak strain in basal lateral and septal segments. RESULTS: Global strain (-18.5 ± 3.5 vs. -17.9 ± 3.2%, P= NS) and global SR (-1.0 ± 0.2 vs. -1.0 ± 0.2/s, P= NS) did not differ between groups. Regional strain (-8.7 ± 8.6 vs. -14.7 ± 6.7%, P= .008) and SR (-0.7 ± 0.4 vs. -1.0 ± 0.3%, P= .002) in the basal septal segment were lower in group 1, while regional strain was higher in group 1 in the apical septal segment (-23.5 ± 8.0 vs. -18.4 ± 5.9%, P= .02). Time to peak strain was higher in the basal septal segment in group 1 (410 ± 78 vs. 338 ± 90 ms, P= .004) but not different in the basal lateral segment. CONCLUSIONS: Despite minor regional differences in longitudinal deformation and dyssynchrony, overall ventricular longitudinal deformation was not different between morphologic right and left ventricles. These findings may reflect similar adaptation of longitudinal function of both ventricular morphologies to the single-ventricle circulation in our cohort, albeit relatively early after Fontan surgery.

Changes of right ventricular function and longitudinal deformation in children with hypoplastic left heart syndrome before and after the Norwood operation.

BACKGROUND: The purpose of this study was to investigate changes in right ventricular (RV) function and deformation parameters before and at steady state after the Norwood operation in neonates with hypoplastic left heart syndrome. A further aim was to delineate factors that affected these changes. METHODS: On echocardiograms before and 21 days (range, 10-35 days) after the Norwood operation, the two-dimensional speckle-tracking parameters global and regional peak systolic longitudinal strain and strain rate were retrospectively compared in 33 patients with hypoplastic left heart syndrome. In addition, RV functional assessment included RV fractional area change and tricuspid annular plane systolic excursion. The associations between postoperative echocardiographic findings and preoperative or postoperative complications, prenatal diagnosis, postoperative heart rate, oxygen saturation, and medication use as well as cardiopulmonary bypass and aortic cross-clamp times were tested. RESULTS: Global strain (-18.3 ± 3.6% vs -16.8 ± 3.8%, P = .02) and global strain rate (-1.6 ± 0.3 vs -1.2 ± 0.3 sec(-1), P < .0001) decreased significantly. Regional strain decreased significantly in the apical and mid lateral segments, while regional strain rate decreased significantly in all but the basal septal segments. Tricuspid annular plane systolic excursion of the lateral annulus decreased significantly, while RV fractional area change remained the same. No significant associations were found between postoperative RV function and potential impact factors. CONCLUSIONS: Two-dimensional global and regional longitudinal strain and strain rate as well as tricuspid annular plane systolic excursion were reduced in patients with hypoplastic left heart syndrome after the Norwood operation. None of the examined preoperative and postoperative patient or surgical factors was found to explain this decrease.

Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking.

BACKGROUND: The left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome. We aimed to investigate differences in right ventricular deformation and intraventricular dyssynchrony between hypoplastic left heart syndrome patients with different anatomical subtypes and left ventricle sizes after Fontan surgery using two-dimensional speckle tracking. PATIENTS AND METHODS: We examined 29 hypoplastic left heart syndrome patients aged 5.4 plus or minus 2.8 years after Fontan surgery and compared 15 patients with mitral and aortic atresia with the remaining 14 patients with other anatomic subtypes. We used two-dimensional speckle tracking to measure the global and regional systolic longitudinal strain and strain rate as well as intraventricular dyssynchrony. RESULTS: Global strain (-19.5, 2.8% versus -17.4, 3.9%) and global strain rate (-1.0, 0.2 per second versus -0.9, 0.3 per second) were not different between groups. The mitral and aortic atresia group had higher strain in the basal septal (-13.0, 5.0% versus -3.9, 9.3%, p = 0.003) and mid-septal (-19.4, 4.7% versus -13.2, 6.5%, p = 0.009) segments, and higher strain rates in the mid-septal segment (-1.14, 0.3 per second versus -0.95, 0.4 per second, p = 0.047), smaller left ventricle area (0.18, 0.41 square centimetre versus 2.83, 2.07 square centimetre, p = 0.0001), and shorter wall-to-wall delay (38, 29 milliseconds versus 81, 57 milliseconds, p = 0.02). CONCLUSION: Significant differences in regional deformation and intraventricular dyssynchrony exist between the mitral and aortic atresia subtype with small left ventricles and the other anatomic subtypes with larger left ventricles after Fontan surgery.

Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic left heart syndrome after Fontan surgery.

BACKGROUND: There is some evidence that hypoplastic left heart syndrome (HLHS) survivors with a larger left ventricular (LV) cavity may have poorer long-term outcome than those with mitral and aortic valve atresia (MA/AA) and negligible LV. A negative impact of the LV remnant on right ventricular (RV) function may contribute to this. METHODS: We retrospectively evaluated RV function echocardiographically using 2D, Doppler and colour tissue Doppler techniques in 42 children with HLHS after Fontan surgery. Patients diagnosed with MA/AA at birth (group 1, n = 20) were compared to all the remaining anatomical subgroups of HLHS (group 2, n = 22). RESULTS: The MA/AA group had a smaller diameter of the ascending aorta at birth (p<0.0001), smaller LV area (p<0.0001) and larger RV area at end-diastole (p = 0.004) and end-systole (p = 0.01) after Fontan. All parameters of RV function including the myocardial performance index, tricuspid annular plane systolic excursion, RV fractional area change and all regional colour Doppler derived myocardial velocities were not different between groups. CONCLUSION: In our cohort, RV function in HLHS after Fontan surgery was not different for the subgroup with MA/AA when compared to the remaining subgroups.

Assessment of ventricular function and dyssynchrony before and after stage 2 palliation of hypoplastic left heart syndrome using two-dimensional speckle tracking.

Two-dimensional (2D) speckle tracking (2DST) is a new technique independent of ventricular geometry but not independent of preload and afterload. Using 2DST, this study aimed to investigate differences in right ventricular (RV) function and intraventricular dyssynchrony in patients with hypoplastic left heart syndrome (HLHS) before and after preload-reducing stage 2 palliation. For 31 HLHS patients, this study compared global longitudinal strain (S) and strain rate (SR) as well as regional peak systolic longitudinal S, SR, and velocity (V) in six RV segments on echocardiograms before and after stage 2 surgery. Intraventricular dyssynchrony was assessed by calculating the standard deviation of the intervals from the beginning of systole to peak S, SR, and V. Global S (-16.7 ± 5.0 vs -15.6 ± 5.5%) and global SR (-1.2 ± 0.3 vs -1.2 ± 0.3 s(-1)) did not change after surgery. After surgery, V decreased in the mid lateral segment (2.3 ± 1.3 vs 1.7 ± 0.9 cm/s; p = 0.01) and the basal lateral segment (3.6 ± 1.1 vs 2.8 ± 1.0 cm/s; p = 0.001), whereas S was lower in both of these segments (-19.9% ± 6.0% vs -17.4% ± 6.3%; p = 0.01 and 20.0 ± 5.1 vs 15.8 ± 7.1%; p = 0.002, respectively). Segmental SR and dyssynchrony did not change. Decreased V and S in the RV free wall could be explained by reduced preload of the systemic RV after stage 2 palliation.

Echocardiographic evaluation of children with systemic ventricular dysfunction treated with carvedilol.

Echocardiography is used to measure the therapeutic effectiveness of heart failure therapy in adults and children. The purposes of this study were (1) to assess baseline echocardiographic predictors of clinical outcome, (2) to investigate changes in echocardiographic parameters, and (3) to compare these echocardiographic changes with changes in plasma levels of b-type natriuretic peptide (BNP) in a population of children with systemic ventricular dysfunction and symptomatic heart failure treated with carvedilol or placebo. All available baseline and 6-month echocardiograms from Pediatric Carvedilol Trial (PCT) participants (carvedilol n = 161; placebo n = 55) were reviewed. Systolic and diastolic sphericity index (SI; n = 110), TEI index (n = 145), and systemic ventricular dP/dt (n = 70) were measured. The PCT composite definition of clinical outcome (i.e., worsened, improved, or unchanged) was used. For all patients, baseline TEI index was a predictor of worsened outcome. Only children treated with carvedilol showed a significant decrease in systolic SI (P B 0.0001), diastolic SI (P B 0.0001), and TEI index (P = 0.02). An inverse correlation between changes in BNP and changes in dP/dt (r = -0.45, P = 0.04) was found only in the carvedilol group. In conclusion, TEI index predicted outcome in children with systemic ventricular dysfunction and heart failure. Carvedilol may have a beneficial effect on reversal of left ventricular remodeling and global ventricular function in pediatric heart failure.

Amplatzer occlusion of accessory ventriculopulmonary connections.

BACKGROUND: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. OBJECTIVE: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. METHODS: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. RESULTS: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. CONCLUSIONS: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease.

Congenital long QT syndrome in children identified by family screening.

The diagnosis of congenital long-QT syndrome (LQTS) in the relatives (nonprobands) of index patients (probands) is increasing because of screening. This report documents the clinical courses and outcomes of nonproband pediatric patients with LQTS. All patients aged <18 years with LQTS were identified at 3 pediatric centers. Demographic data, personal and family histories, electrocardiographic data, and genetic diagnoses (if available) were obtained. Probands were defined as the first patients in their families diagnosed with LQTS and nonprobands as those diagnosed by screening. Of 144 patients with LQTS, 84 (58%) were nonprobands aged 6.5+/-5.4 years with QTc intervals of 479+/-34 ms. No nonproband presented with resuscitated sudden death, atrioventricular block, or ventricular arrhythmia, but 7 (8.3%) had histories of syncope at presentation. All nonproband patients were treated. During a follow-up period of 4.7+/-3.9 years, there were no deaths in the nonproband group, but device implantation was performed in 13 (15%), 4 of whom had appropriate shocks. As expected, compared with probands, nonprobands were less symptomatic. Additionally, nonprobands were younger and had shorter QTc intervals. Although device implantation was more common in probands, there was no difference in appropriate implantable cardioverter-defibrillator shocks or mortality between the probands and their affected relatives. In conclusion, children are increasingly identified with LQTS as a result of family screening. Although phenotypic differences exist between probands and nonprobands, survival is excellent in the 2 groups with therapy. Appropriate implantable cardioverter-defibrillator discharges in the nonproband group underscore the importance of follow-up in this relatively asymptomatic population.