Three Decades of Follow-up After Surgical Closure of Subarterial Ventricular Septal Defect.

We determined the occurrence of aortic regurgitation (AR), AR progression and risk factors in patients followed up for up to three decades after closure of subarterial VSD. We reviewed the outcomes of 86 patients categorized into three groups: group I comprised 37 patients without AR and had VSD closure alone, group II comprised 40 patients with AR and had VSD closure without aortic valvoplasty, and group III comprised 9 patients with AR and required both VSD closure and aortic valvoplasty. Patients were followed up for 18.9 ± 7.3 years (median 19.5 years, range 3.5-36.6). At latest follow up, 54.7% (47/86) of patients had AR. The prevalence of progression of AR from any one grade to the next one higher was 37.2% (32/86). Freedom from AR progression was 75.6%, 52.1%, and 22.2% at 20 years of follow-up for groups I, II and III, respectively (p < 0.05). On the other hand, progression to moderate to severe AR occurred only in 4.7% (4/86). Group I and II patients were free from progression to significant AR, while only 33.3% of group III patients were free from progression on follow-up (p < 0.001). Multivariate Cox regression analysis showed that severity of preoperative AR was the significant risk factor for persistence and progression of postoperative AR after VSD closure. In conclusion, aortic regurgitation is common and may progress even after surgical repair of subarterial VSD. Severity of preoperative AR is the most significant predictor of persistence and progression of AR after surgical closure of subarterial VSD.

Frontal QRS-T angle and ventricular mechanics in congenital heart disease.

BACKGROUND: The QRS-T angle has been associated with adverse cardiovascular events and sudden cardiac deaths. We determined frontal QRS-T angle in patients with complete transposition of the great arteries (TGA) after atrial switch operation and repaired tetralogy of Fallot (TOF) and explored its relationships with ventricular mechanics. METHODS: Thirty TGA patients aged 32.3 ± 4.4 years after atrial switch operation and 47 repaired TOF patients aged 28.7 ± 6.0 years were studied. The frontal planar QRS-T angle and QRS duration were measured from 12-lead electrocardiograms. Right (RV) and left ventricular (LV) strain parameters were determined using speckle tracking echocardiography. RESULTS: Compared with TOF patients, TGA patients after atrial switch operation had significantly greater frontal QRS-T angle (136.3° ± 43.5° vs 74.5° ± 59.6°, p < 0.001), greater prevalence of QRS-T angle ≥ 100° (83.3% vs 29.8%, p < 0.001), and showed progressive increase in QRS-T angle over a duration of 3.3 ± 1.0 years (p = 0.035). The QRS-T angle correlated positively with QRS duration in both the TGA (r = 0.61, p < 0.001) and TOF (r = 0.30, p < 0.043) groups. Among TGA patients, QRS-T angle was found to correlate negatively with systemic RV global longitudinal strain (r = - 0.49, p = 0.007), early diastolic strain rate (r = - 0.41, p = 0.026), and fractional area change (r = - 0.38, p = 0.045), but not subpulmonary LV strain indices. By contrast, among repaired TOF patients, there were no significant correlations between QRS-T angle and systemic and subpulmonary ventricular strain indices (all p > 0.05). CONCLUSION: Increased frontal QRS-T angle is prevalent in TGA patients after atrial switch operation and is related to worse systemic RV mechanics.

Tricuspid Regurgitation in Adults after Repair of Right Ventricular Outflow Obstructive Lesions.

We determined the prevalence and factors associated with tricuspid regurgitation (TR) in adults with repair of right ventricular (RV) outflow obstruction. A total of 256 patients (128 males) were studied at 25.7 ± 7.2 years after surgery, of whom 179 had repaired tetralogy of Fallot (TOF), 31 had pulmonary atresia with intact ventricular septum (PAIVS), and 46 had pulmonary stenosis (PS). The mitral and tricuspid annulus diameters, maximum right atrial (RA) area, RV end-systolic and end-diastolic areas, and tricuspid and pulmonary regurgitation were assessed using echocardiography. The prevalence of moderate-to-severe TR was 20.7%. Subgroup analysis revealed that prevalence was greater in patients with repaired TOF (20.7%) and PAIVS (35.5%) than PS patients (10.9%). As a group, severity of TR was found to be correlated with RA area (r = 0.35, p < 0.001), RV end-diastolic (r = 0.28, p < 0.001) and end-systolic (r = 0.22, p = 0.001) areas, and tricuspid valve annulus diameter (r = 0.15, p = 0.022). Moderate-to-severe TR was associated with development of cardiac arrhythmias with an odds ratio of 2.9 (95% CI 1.1 to 8.1, p = 0.031). Multivariate analysis revealed maximum RA area (ß = 0.36, p = 0.016) as an independent determinant of severity of TR. Moderate-to-severe TR occurs in about one-fifth of adults with repaired TOF, PAVIS, and PS and is associated with RA dilation and risk of development of cardiac arrhythmias.

Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome.

BACKGROUND: Idiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease's impact on a child's cardiac function. CASE PRESENTATION: A Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6 years of age. His second attack developed at 8 years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient's echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS. CONCLUSION: ISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction.

Fifty-Five Years Follow-Up of 111 Adult Survivors After Biventricular Repair of PAIVS and PS.

There is paucity of long-term data on adult survivors after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS). This study aimed to determine the cardiac and non-cardiac outcomes of adult survivors after biventricular repair of PAIVS and PS. The cardiac, neurodevelopmental and liver problems of 111 adults, 40 with PAIVS and 71 with PS, were reviewed. The median follow-up duration of our patients was 26.5 years (range 14.8-55 years). The freedom from reintervention at 30 years was 17.4% and 73.3% for PAIVS and PS patients (p < 0.001), respectively. Compared with PS patients, PAIVS patients had significantly greater prevalence of right atrial and right ventricular (RV) dilatation, and moderate to severe tricuspid and pulmonary regurgitation (all p < 0.05), and cardiac arrhythmias (22.5% vs. 8.5%, p = 0.047). The freedom from development of cardiac arrhythmias at 30 years of 68.4% and 91.6%, respectively, in PAIVS and PS patients (p = 0.03). Cox proportional hazards model identified PAIVS as an independent risk factor for reintervention (HR 4.0, 95% CI 2.1-7.6, p < 0.001) and development of arrhythmias (HR 4.1, 95% CI 1.1-14.4, p = 0.03). Neurodevelopmental problems were found in 17.5% of PAIVS patients and 7.0% of PS patients (p = 0.11). Liver problems occurred in 2 (5%) PAIVS patients, both of whom required conversion to 1.5 ventricular repair. In conclusion, long-term problems, including the need for reinterventions, cardiac arrhythmias, RV dilation, pulmonary regurgitation, and neurodevelopmental and liver issues are more prevalent in adult PAIVS than PS survivors.

Circulating MicroRNA in patients with repaired tetralogy of Fallot.

BACKGROUND: Emerging data suggest that heart-related microRNAs (miRs) may serve as circulating biomarkers of myocardial injury. We aimed to determine the circulating profile of miRs in patients with volume-overloaded right ventricles after repair of tetralogy (TOF). MATERIALS AND METHODS: A total of 104 TOF patients and 70 controls were recruited. The study was conducted in two phases: (1) determination of circulating heart-related miRs described in left heart diseases (miR-1, miR-133a, miR-208a, miR-208b and miR423-5p) by quantitative real-time PCR in 49 patients and 30 controls and followed by validation in an independent cohort of 55 patients and 40 controls; (2) expression profiling of serum samples from eight patients and eight controls, followed by validation. Alteration in circulating miRNA expression was related to cardiac functional indices as assessed by 2D speckle tracking and 3D echocardiography. RESULTS: No significant differences in serum levels of left heart-associated miRNAs were found between patients and controls. Of the candidate 19 miRNAs identified by profiling, upregulation of miR-99b and down-regulation of miR-766 were validated. However, no correlations were found between miRs levels and echo indices. CONCLUSION: In young adults with repaired TOF and volume-overloaded right ventricles, circulating levels of miR-99b and miR-766, but not left heart-associated miRNAs, were significantly altered.

Apoptosis of cardiomyocytes in children with right ventricular pressure overload with and without hypoxemia.

BACKGROUND: Cardiomyocyte apoptosis has been implicated in ventricular remodeling and initiation of cardiac failure. We sought to determine the severity of right ventricular (RV) cardiomyocyte apoptosis in cyanotic and acyanotic children with RV pressure overload. METHODS: Fourteen patients, seven with tetralogy of Fallot (group I) and seven with pulmonary stenosis and ventricular septal defect (group II), undergoing open-heart surgery were studied. Right ventricular biopsies were examined for cardiomyocyte apoptosis by terminal deoxynucleotide transferase-mediated dUTP nick-end labeling. The magnitude of cardiomyocyte apoptosis was related to preoperative oxygen saturation and postoperative inotrope use and hospital stay. RESULTS: Compared with group I patients, group II patients were significantly older at operation (p = 0.002) and had a larger body size (p < 0.01) and higher preoperative oxygen saturation (p = 0.01). The prevalence of cardiomyocyte apoptosis in both group I and II patients as a whole was 0.24 ± 0.29% (range, 0% to 1.10%). The prevalence was similar between group I (median 0.30%, range 0% to 1.10%) and group II (median 0.20, range 0% to 0.40%, p = 0.65). The prevalence of cardiomyocyte apoptosis correlated positively with preoperative oxygen saturation on room air (r = -0.69, p < 0.005) and postoperative inotrope score (r = 0.67, p = 0.001). A higher postoperative inotrope score (r = 0.68, p = 0.001) was associated with a significant longer duration of postoperative stay in the hospital. CONCLUSIONS: The prevalence of cardiomyocyte apoptosis in the pressure-overloaded right ventricle is related to the severity of hypoxia and may have an impact on postoperative course in terms of early postoperative use of inotropes and duration of hospital stay.

Circulating microRNA expression profile and systemic right ventricular function in adults after atrial switch operation for complete transposition of the great arteries.

BACKGROUND: Data on the use of circulating microRNAs (miRNAs) as biomarkers of cardiovascular diseases are emerging. Little, however, is known on the expression profile of circulating of microRNAs in congenital heart malformations with a systemic right ventricle that is prone to functional impairment. We aimed to test the hypothesis that circulating miRNA profile is altered in patients late after atrial switch operation for complete transposition of the great arteries (TGA) and further explored possible relationships between alteration of circulating miRNAs and systemic ventricular contractility. METHODS: Circulating miRNA expression profiling of serum samples from 5 patients and 5 healthy controls was performed. The results were validated in 26 patients and 20 controls using real-time quantitative reverse-transcription polymerase chain reaction for candidate miRNAs with fold changes >3 by expression profiling. Systemic ventricular myocardial acceleration during isovolumic contraction (IVA) was determined by colour tissue Doppler echocardiography. RESULTS: Compared with controls, patients had significantly lower systemic ventricular IVA (p = 0.002). Of the 23 upregulated miRNAs identified by profiling, 11 were validated to be increased in patients compared with controls: miR-16, miR-106a, miR-144*, miR-18a, miR-25, miR-451, miR-486-3p, miR-486-5p, miR-505*, let-7e and miR-93. Among the validated 11 miRNAs, miR-18a (r = -0.45, p = 0.002) and miR-486-5p (r = -0.35, p = 0.018) correlated negatively with systemic ventricular IVA for the whole cohort. CONCLUSIONS: A distinct serum miRNA expression signature exists in adults with complete TGA after atrial switch operation, with serum miR-18a and miR-486-5p being associated with systemic ventricular contractility.

Evaluating High-Confidence Genes in Conotruncal Cardiac Defects by Gene Burden Analyses.

Background In nonsyndromic conotruncal cardiac defects, the use of next-generation sequencing for clinical diagnosis is increasingly adopted, but gene-disease associations in research are only partially translated to diagnostic panels, suggesting a need for evidence-based consensus. Methods and Results In an exome data set of 245 patients with conotruncal cardiac defects, we performed burden analysis on a high-confidence congenital heart disease gene list (n=132) with rare (<0.01%) and ultrarare (absent in the Genome Aggregation Database) protein-altering variants. Overall, we confirmed an excess of rare variants compared with ethnicity-matched controls and identified 2 known genes (GATA6, NOTCH1) and 4 candidate genes supported by the literature (ANKRD11, DOCK6, NPHP4, and STRA6). Ultrarare variant analysis was performed in combination with 3 other published studies (n=1451) and identified 3 genes (FLT4, NOTCH1, TBX1) to be significant, whereas a subgroup analysis involving 391 Chinese subjects identified only GATA6 as significant. Conclusions We suggest that these significant genes in our rare and ultrarare burden analyses warrant prioritization for clinical testing implied for rare inherited and de novo variants. Additionally, associations on ClinVar for these genes were predominantly variants of uncertain significance. Therefore, a more stringent assessment of gene-disease associations in a larger and ethnically diverse cohort is required to be prudent for future curation of conotruncal cardiac defect genes.

Circulating levels of biomarkers of collagen synthesis and ventricular function and dyssynchrony in adolescents and young adults after repair of tetralogy of Fallot.

BACKGROUND: Circulating carboxy-terminal propeptide of type I procollagen (PICP) and amino-terminal propeptide of type III procollagen (PIIINP) are biomarkers of collagen synthesis. We tested the hypothesis that circulating PICP and PIIINP are altered and may correlate with ventricular volume load and function in patients with repaired tetralogy of Fallot (TOF). METHODS AND RESULTS: Serum PICP and plasma PIIINP levels were determined in 39 patients with repaired TOF aged 17.7 ± 4.1 years and 25 healthy controls and correlated with right ventricular (RV) and left ventricular (LV) volumes, functional indices, and mechanical dyssynchrony as assessed by 3-dimensional and tissue Doppler echocardiography. Compared with controls, patients had significantly higher circulating PICP (P = .016) and PIIINP (P = .008) levels, worse RV function with intra-RV mechanical delay (all P < .001), impaired LV systolic functional indices (all P < .05), and greater LV systolic dyssynchrony index (SDI) (P < .001). For the whole cohort, circulating PICP and PIIINP levels correlated with age (P = .001 and P < .001, respectively), body mass index (P = .033 and P = .012, respectively), LV eccentricity (P = .035 and P = .046, respectively), RV end-diastolic volume (P = .029 and P = .047, respectively), and LV SDI (both P < .001). In addition, PICP levels correlated negatively with RV and LV isovolumic acceleration and RV ejection fraction. Multiple linear regression analysis identified LV SDI as a significant independent correlate of circulating levels of PICP (ß = .31, P = .045) and PIIINP (ß = .37, P = .004). CONCLUSION: Circulating levels of PICP and PIIINP correlate positively with LV mechanical dyssynchrony in patients after TOF repair, implicating a possible role of increased collagen synthesis in its pathogenesis.

Interplay between right atrial function and liver stiffness in adults with repaired right ventricular outflow obstructive lesions.

AIMS: This study determined the associations between right atrial (RA) and right ventricular (RV) mechanics and liver stiffness in adults with repaired tetralogy of Fallot (TOF), pulmonary atresia with intact ventricular septum (PAVIS), and pulmonary stenosis (PS). METHODS AND RESULTS: Ninety subjects including 26 repaired TOF, 24 PAIVS, and 20 PS patients and 20 controls were studied. Hepatic shear wave velocity and tissue elasticity (E), measures of liver stiffness, were assessed by two-dimensional shear wave elastography, while RA and RV mechanics were assessed by speckle tracking echocardiography. Deformation analyses revealed worse RV systolic strain and strain rate, and RA peak positive and total strain, and strain rates at ventricular systole and at early diastole in all of the patient groups compared with controls (all P < 0.05). Compared with controls, all of the patient groups had significantly greater shear wave velocity and hepatic E-value (all P < 0.05). Shear wave velocity and hepatic E-value correlated negatively with RV systolic strain rate, and RA positive strain, total strain, and strain rate at ventricular systole and at early diastole (all P < 0.05). Multivariate analyses revealed RA strain rate at early diastole (P = 0.015, P < 0.001), maximum RA size (P < 0.001, P < 0.001), and severity of pulmonary regurgitation (P = 0.05, Pp = 0.014) as significant correlates of shear wave velocity and hepatic E-value. CONCLUSION: In adults with repaired TOF, PAIVS, and PS, RA dysfunction and pulmonary regurgitation are associated with liver stiffness.

Differential myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle after atrial switch operation for complete transposition of the great arteries.

BACKGROUND: This study aimed to assess diffuse myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle in patients after Senning or Mustard operation for complete transposition of the great artery (TGA) using cardiac magnetic resonance (CMR) T1 mapping. METHODS: Thirty-one adult TGA patients after Senning (n = 24) or Mustard (n = 7) operation were studied at the age of 33.3 ± 4.0 years. Systemic right ventricular (RV) and subpulmonary left ventricular (LV) volumes, ejection fraction, and myocardial T1 values and extracellular volume fraction (ECV) were determined using CMR. RESULTS: The RV and LV ejection fractions were 47.0 ± 10.9% and 61.3 ± 7.4%, respectively. Compared to published normative values, patients had significantly greater RV and LV native T1 and ECV values (all p < 0.001). For each of the basal, mid, and apical segments, the LV native T1 and ECV values were significantly greater in the left than the right ventricle (all p < 0.05). There is a significant trend on progressive increase in ECV value from the basal towards the apical segments in both the right (p = 0.002) and the left (p < 0.001) ventricle. Modestly strong correlations were found between RV and LV native T1 (r = 0.60, p < 0.001) and ECV (r = 0.49, p = 0.005) values but not with ejection fractions of the respective ventricles. CONCLUSIONS: Differential myocardial fibrosis, with greater involvement of the subpulmonary left ventricle than the systemic right ventricle, is present in patients with TGA after atrial switch operation. Associations between the magnitude of RV and LV fibrosis suggests adverse ventricular-ventricular interaction at the cardiac extracellular matrix level.

Aortic dissection in a Chinese patient 31 years after surgical repair of tetralogy of Fallot.

Aortic dilation has been increasingly recognized in congenital heart diseases, and aortic dissection is one of the important complications. We report a case of aortic dissection in a patient 31 years after repair of tetralogy of Fallot (TOF) and review reported cases. While aortic dissection is uncommon, aortic dilation is common among patients with repaired TOF and it appeared progressive in some patients. Based on the reported cases, progressive aortic dilation appeared as the pre-requisite for aortic dissection, although other factors might be involved. Regular surveillance and monitoring for aortic complications should be incorporated into clinical practice. .

Monoallelic Mutations in CC2D1A Suggest a Novel Role in Human Heterotaxy and Ciliary Dysfunction.

BACKGROUND: Human heterotaxy is a group of congenital disorders characterized by misplacement of one or more organs according to the left-right axis. The genetic causes of human heterotaxy are highly heterogeneous. METHODS: We performed exome sequencing in a cohort of 26 probands with heterotaxy followed by gene burden analysis for the enrichment of novel rare damaging mutations. Transcription activator-like effector nuclease was used to generate somatic loss-of-function mutants in a zebrafish model. Ciliary defects were examined by whole-mount immunostaining of acetylated α-tubulin. RESULTS: We identified a significant enrichment of novel rare damaging mutations in the CC2D1A gene. Seven occurrences of CC2D1A mutations were found to affect 4 highly conserved amino acid residues of the protein. Functional analyses in the transcription activator-like effector nuclease-mediated zebrafish knockout models were performed, and heterotaxy phenotypes of the cardiovascular and gastrointestinal systems in both somatic and germline mutants were observed. Defective cilia were demonstrated by whole-mount immunostaining of acetylated α-tubulin. These abnormalities were rescued by wild-type cc2d1a mRNA but not cc2d1a mutant mRNA, strongly suggesting a loss-of-function mechanism. On the other hand, overexpression of cc2d1a orthologous mutations cc2d1a P559L and cc2d1a G808V (orthologous to human CC2D1A P532L and CC2D1A G781V) did not affect embryonic development. CONCLUSIONS: Using a zebrafish model, we were able to establish a novel association of CC2D1A with heterotaxy and ciliary dysfunction in the F2 generation via a loss-of-function mechanism. Future mechanistic studies are needed for a better understanding of the role of CC2D1A in left-right patterning and ciliary dysfunction.

Circulating Transforming Growth Factor-ß and Aortic Dilation in Patients with Repaired Congenital Heart Disease.

This study determined the circulating levels of TGF-ß1 and its association with aortic dilation and elastic properties in congenital heart patients. Forty-six patients after tetralogy of Fallot (TOF) repair, 21 patients post arterial switch and 15 patients post atrial switch for transposition of the great arteries (TGA), 27 patients post Fontan procedure, and 36 controls were studied. Aortic dimensions and elastic properties and ventricular function were assessed by echocardiography. Serum TGF-ß1, metalloproteinase (MMP)-2 and MMP-9 levels were quantified. Compared with controls, all groups of patients had significantly larger ascending aortic dimensions and worse elastic properties (all p < 0.05). Aortic stiffness correlated positively with sinus dimension (r = 0.48, p < 0.001) and negatively with indices of ventricular deformation (all p < 0.001). Patients with repaired TOF had significantly higher levels of TGF-ß1 (p = 0.005), MMP-2 (p = 0.001) and MMP-9 (p < 0.001) than controls, while patients after atrial switch operation (p = 0.034) and Fontan procedures (p < 0.001) had higher MMP-2 levels. In patients as a group, circulating TGF-ß1 levels correlated with MMP-9 (r = 0.44, p < 0.001) and aortic sinus dimension (r = 0.22, p = 0.035). In conclusion, increased circulating TGF-ß1, MMP-2, and MMP-9 levels were found in patients with repaired TOF, and increased circulating MMP-2 levels were also evident in patients after atrial switch operation and Fontan procedure.

Porcine Versus Pericardial Pulmonary Valve Replacement in Adults With Prior Congenital Cardiac Surgery: Midterm Outcomes.

BACKGROUND: Postcongenital heart surgery pulmonary regurgitation requires subsequent pulmonary valve replacement. We sought to compare the outcomes of pulmonary valve replacement after using bioprosthetic valves, porcine versus pericardial bioprosthesis. METHOD: Retrospective single-center study of consecutive pulmonary valve replacement in patients with pulmonary regurgitation following initial congenital cardiac surgery. From 2004 to 2016, 82 adult patients (53 males, 29 females) underwent pulmonary valve replacement at a mean age of 28.7 ± 8 years (range 18-52 years) with a mean time to pulmonary valve replacement of 24 ± 7 years (range 13-43 years). Porcine bioprosthetic valves (group 1, n = 32) and pericardial valves (group 2, n = 50) were used. Cardiac magnetic resonance imaging was performed (n = 54) at a mean of 18 ± 13 months before and 24 ± 21 months after pulmonary valve replacement. RESULTS: No significant difference was seen between the groups except that the mean follow-up was longer for group 1 (5.02 ± 2.06 vs 4.08 ± 3.21 years). In-hospital mortality was 1.1%. Follow-up completeness was 100% with no late death. Mean right ventricular end-systolic and end-diastolic volumes reduced significantly in both the groups ( P < .001), whereas right ventricular ejection fraction remained unchanged (group 1, P = .129; group 2, P = .675) . Only the left ventricular end-diastolic volume increased in both the groups, but the increase was significant for group 2 only (group 1, P = .070; group 2, P = .015), whereas the left ventricular end-systolic and ejection fraction remained unchanged in both the groups. There was no reoperation for pulmonary valve replacement. Freedom from intervention was 93.8% (group 1) and 100% (group 2) at eight years after pulmonary valve replacement ( P = .407). CONCLUSION: Midterm outcomes of pulmonary valve replacement in our adult cohort were satisfactory. Both types of bioprosthetic valves performed comparably for eight years and were a good option in adults.

Mechanical right ventricular dyssynchrony in patients after atrial switch operation for transposition of the great arteries.

Recent data suggest potential benefits of cardiac resynchronization therapy in the management of right ventricular (RV) dysfunction in congenital heart disease. The aim of this study was to determine the nature, prevalence, and functional implications of mechanical RV dyssynchrony in patients after Senning or Mustard procedures for transposition of the great arteries. Twenty-eight patients (mean age 21.1 +/- 3.5 years) at 19.9 +/- 3.2 years after atrial switch operations and 29 healthy controls were studied. The times from the onset of QRS to peak systolic strain (T epsilon) at the base of and the mid RV free wall, the ventricular septum (VS), and the left ventricular (LV) free wall were determined using tissue Doppler echocardiography. Intraventricular mechanical delay was defined as Delta T epsilon(RV-VS) and interventricular mechanical delay as Delta T epsilon(RV-LV). In patients, the magnitude of RV intra- and interventricular mechanical delay was correlated with cardiac magnetic resonance-derived RV volumes and ejection fractions (n = 26) and treadmill exercise testing parameters (n = 20). Compared with controls, patients had significantly longer Delta T epsilon(RV-VS) (48.1 +/- 50.9 vs 17.0 +/- 16.1 ms, p <0.001) and Delta T epsilon(RV-LV) (63.1 +/- 49.5 vs 19.0 +/- 12.9, p <0.001). Nine patients (32%) exhibited RV dyssynchrony (Delta T epsilon(RV-VS) >49 ms, control mean +/- 2SD), and 16 patients (57%) showed interventricular dyssynchrony (Delta T epsilon(RV-LV) >45 ms). In patients, RV intra- and interventricular mechanical delay was correlated negatively with the RV ejection fraction (both r = -0.42, p = 0.03) and percentage predicted maximum oxygen consumption (r = -0.50, p = 0.03, and r = -0.52, p = 0.02, respectively) and positively with minute ventilation/carbon dioxide production slope (r = 0.49, p = 0.03, and r = 0.56, p = 0.01, respectively). In conclusion, RV dyssynchrony is common in young adults after atrial switch operations and is associated with RV systolic dysfunction and impaired exercise performance.

Oxidative stress in children late after Kawasaki disease: relationship with carotid atherosclerosis and stiffness.

BACKGROUND: Persistent arterial dysfunction in patients with a history of Kawasaki disease (KD) and an integral role of oxidative stress in the development of cardiovascular disease are increasingly recognized. We sought to test the hypothesis that oxidative stress is increased in KD patients and related to carotid atherosclerotic changes and stiffness. METHODS: We compared the serum levels of oxidative stress biomarkers, carotid intima-media thickness (IMT), and carotid stiffness index among KD patients with coronary aneurysms (n = 32), those without coronary complications (n = 19), and controls (n = 32). RESULTS: Compared with controls, patients with coronary aneurysms had significantly higher serum levels of malonaldehyde (2.62 +/- 0.12 microM vs 2.22 +/- 0.07 microM, p = 0.014) and hydroperoxides (26.50 +/- 1.13 microM vs 22.50 +/- 0.62 microM, p = 0.008). A linear trend of the magnitude of oxidative stress in relation to inflammatory damage was observed for malonaldehyde (p = 0.018) and hydroperoxides (p = 0.014) levels. Serum malonaldehyde and hydroperoxide levels correlated positively with carotid IMT (p < 0.001 and p = 0.034, respectively) and stiffness index (p = 0.001 and p = 0.021, respectively). Multiple linear regression analysis identified serum malonaldehyde level as a significant determinant of carotid IMT (beta = 0.31, p = 0.006) and stiffness (beta = 0.27, p = 0.008). CONCLUSION: Our findings suggest oxidative stress is increased in KD patients with coronary aneurysms and is associated with carotid intima-media thickening and stiffening.

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation.

OBJECTIVE: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long-term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure. DESIGN, SETTINGS, AND PATIENTS: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. INTERVENTION AND OUTCOME MEASURES: Demographic and procedural data were correlated with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biventricular circulation. RESULTS: The procedural success rate was 92% (33 out of 36) in this group with moderate right ventricular hypoplasia (tricuspid valve z score -4.2 ± 3.0, 69.4% of patients with z score <-2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock-Taussig shunt. Overall reintervention-free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch-up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circulation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%) CONCLUSION: Encouraging long-term results with biventricular circulation and functional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.