Impact of Surgical Strategy and Post-Repair Transverse Aortic Arch Size on Late Hypertension After Coarctation Repair During Infancy.

OBJECTIVE: Late HTN after CoA repair contributes to higher morbidity and mortality. An association between TAA hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA. METHODS: We retrospectively reviewed patients who underwent surgical repair of CoA during infancy with at least 10 years follow-up, excluding those with atypical coarctation, major associated heart defects, and residual isthmic narrowing. TAA diameter z-score immediately post-repair was measured as a marker of surgical strategy. Systemic HTN at latest follow-up was assessed using standard criteria. RESULTS: A total of 130 patients underwent surgical repair of CoA (76% via thoracotomy, 24% via sternotomy; type of repair - resection and end-to-end anastomosis 62%, extended end-to-end anastomosis 30%, subclavian flap 5%, arch repair with patch 4%), at a median age of 14 (IQR 7-62) days. Median post-repair TAA diameter z-score was -2.04 (IQR -2.69, 1.24). After a mean follow-up of 17.3 years, 43/130 (33%) patients developed HTN. After controlling for age at repair, gender, and presence of a genetic syndrome, HTN was not associated with immediate post-repair TAA diameter z-score, (p=0.41), type of surgical incision (p=0.99), or type of surgical repair (p=0.66). CONCLUSIONS: In patients undergoing surgical repair of CoA during infancy, late HTN was not associated with immediate post-repair TAA size or surgical strategy pertaining to the TAA. These results suggest that factors other than surgical strategy, such as differential growth of the TAA during childhood, may be important.

Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Aortic growth after arch reconstruction with patch augmentation: a 2-decade experience.

OBJECTIVES: Optimal aortic sizing during aortic arch reconstruction remains unknown. Negative effects of arch under- or oversizing are well-published. We aimed to characterize longitudinal aortic growth after patch-augmented arch reconstruction to identify the initial reconstructed arch size that results in normal mid-term arch dimensions. METHODS: Single-centre, retrospective review of infants undergoing Damus-Kaye-Stansel (DKS) or non-DKS patch-augmented aortic arch reconstruction between 2000 and 2021. Ascending aorta, proximal and distal transverse arch, aortic isthmus (AIsth) and descending aorta dimensions were measured in postoperative echocardiograms (<3 months from index operation) and cross-sectional imaging (>12 months). Longitudinal changes to aortic dimensions and z-scores were analysed. Secondary outcomes included reintervention, valve and ventricular function, mortality and transplantation. RESULTS: Fifty-four patients (16 DKS, 38 non-DKS) were included. At 6.3 [2.2, 12.0]-year follow-up, all aortic segments grew significantly in both groups, while z-scores remained unchanged except for non-DKS proximal and distal transverse arch z-scores, which significantly increased (P < 0.05 each). When stratified by initial postoperative z-score (z < -1, -1 ≤ z ≤ 1, z > 1), non-DKS patients with initial AIsth z-score <-1 had a final z-score significantly smaller than both the targeted z-score zero (P = 0.014) and final z-score in a group with initial postoperative z-score ±1 (P = 0.009). Valve and ventricular function remained stable. Eighteen patients required reintervention, 1 died and 1 underwent transplant. CONCLUSIONS: Over mid-term follow-up, aortic growth after arch reconstruction with patch augmentation was proportional when repaired to normal z-score dimensions, aside from proximal transverse arch, which disproportionately dilated. AIsth undersizing prevailed mid-term and trended towards a higher reintervention rate. Initial reconstruction between z-score 0 and +1 resulted in maintenance of that z-score size at mid-term follow-up. Overall, it is crucial to achieve targeted aortic sizing at index operation to maintain appropriate aortic dimensions over time and reduce reintervention risk with specific focus on the AIsth.

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation.

BACKGROUND: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. METHODS: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. RESULTS: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2-9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (- 0.61%/year vs. - 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). CONCLUSIONS: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

Utility of cardiac CT in infants with congenital heart disease: Diagnostic performance and impact on management.

BACKGROUND: Advances in cardiac CT (CCT) scanner technology allow imaging without anesthesia, and with low radiation dose, making it an attractive technique in infants with congenital heart disease. However, the utility of CCT using a dual-source scanner with respect to diagnostic performance and impact on management has not been systematically studied in this population. METHODS: Retrospective review of infants who underwent CCT to determine the utility of CCT with respect to the following: answering the primary diagnostic question, providing new diagnostic information, prompting a change in management, and concordance with catheterization or surgical inspection. RESULTS: A total of 156 infants underwent 172 scans at a median age of 64 days, (IQR 4-188) from Jan 2016-Dec 2019. The most frequent diagnostic question was related to the pulmonary arteries (43%), followed by the aortic arch (30%), pulmonary veins (26%), coronary arteries (17%), patent ductus arteriosus (10%) and others (9%). A high-pitch spiral scan was frequently used (90%). The median effective radiation dose was low (0.66 â€‹mSv) and general anesthesia was used infrequently (23%). CCT answered the primary diagnostic question in 168/172 (98%) and added to the diagnostic information already available by echocardiography in 161/172 (96%) scans. CCT led to a change in management following 78/172 (53%) scans and had an impact on management following 167/172 (97%) scans. On follow-up, after 107/172 (62%) scans, subjects underwent cardiac surgery, and after 55/172 (32%) scans, they had cardiac catheterization. CCT findings were concordant with catheterization and/or surgical inspection in 156/159 (98%) scans. CONCLUSIONS: In infants with complex congenital heart disease, CCT was accurate, answered the diagnostic questions in nearly all cases, and frequently added diagnostic information that impacted management. Radiation exposure was low, and anesthesia was needed infrequently.

Cytotoxic potentials of S. cumini methanolic seed kernel extract in human hepatoma HepG2 cells.

Syzygium cumini (Myrtaceae) is commonly called as Jamun or Jambolan. It has antidiabetic, anti-inflammatory, antipyretic, and antioxidant activities. Hepatocellular carcinoma is the most frequent and deadliest cancers worldwide. We investigated the cytotoxic potentials of S. cumini methanolic seed kernel extract against human hepatoma HepG2 cells. HepG2 cells were treated with 10, 20, and 40 µg/mL of seed kernel extract for 24 hours and cytotoxic analysis was performed by MTT assay. S. cumini induced apoptosis related morphological changes in HepG2 cells were analyzed by annexin V and propidium iodide double staining. Nuclear fragmentation and chromatin condensation were analyzed by Hoechst nuclear staining. Mitochondrial membrane potential (MMP) was investigated by 5,5',6,6'-tetrachloro-1,1',3,3'-tetraethylbenzimidazolyl-carbocyanine iodide (JC-1) staining. Protein expressions of hepatocyte nuclear factor-1α (HFN-1α) was performed using western blotting. S. cumini treatments caused a significant and a concentration-dependent increase in the cytotoxicity of HepG2 cells. S. cumini treatments increased the percentage of cells in an early and late apoptosis stage. This treatment also caused chromatin condensation and nuclear fragmentation. Further, S. cumini treatments decreased MMP and also caused a significant downregulation of HFN-1α protein expression. The present study demonstrated that S. cumini seed extract induced apoptosis in HepG2 cells through decrease in MMP and downregulation of HFN-1α.

Impact of Treatment Modality on Vascular Function in Coarctation of the Aorta: The LOVE - COARCT Study.

Background Optimally treated patients with coarctation of the aorta remain at risk for late vascular dysfunction. The effect of treatment modality on vascular function is unknown. The LOVE-COARCT (Long-term Outcomes and Vascular Evaluation After Successful Coarctation of the Aorta Treatment) study was done to compare vascular function in patients with coarctation of the aorta treated with surgery, balloon dilation (BD), or stent implantation. Methods and Results In treated coarctation of the aorta patients without residual coarctation, we prospectively compared aortic stiffness by applanation tonometry and cardiac magnetic resonance; endothelial function by endothelial pulse amplitude testing; blood pressure ( BP ) phenotype by office BP , ambulatory BP monitoring, and BP response to exercise; left ventricular mass by cardiac magnetic resonance; and blood biomarkers of endothelial function, inflammation, vascular wall function, and extracellular matrix. Participants included 75 patients treated with surgery (n=28), BD (n=23), or stent (n=24). Groups had similar age at enrollment, coarctation of the aorta severity, residual gradient, and metabolic profile, but differed by age at treatment. Prevalence of systemic hypertension, aortic stiffness, endothelial function, and left ventricular mass were similar among treatment groups. However, BD patients had more-distensible ascending aortas, lower peak systolic BP during exercise, less impairment in diurnal BP variation, and lower inflammatory biomarkers. Results were unchanged after adjustment for potential confounders, including age at treatment. Conclusions In our cohort of patients without residual coarctation, treatment modality was not associated with major vascular outcomes, even though there were some favorable vascular characteristics in the BD patients. Although this suggests that choice of treatment modality should continue to be driven by likelihood of achieving a good anatomical result, more long-term studies are required to assess the clinical significance of the more-optimal results of secondary markers of vascular function in BD patients. Clinical Trial Registration URL : http://www.clinicaltrials.gov . Unique identifier: NCT 03262753.

Rationale and design of Long-term Outcomes and Vascular Evaluation after Successful Coarctation of the Aorta Treatment study.

BACKGROUND: Coarctation of the aorta (CoA) can be treated using surgery, balloon angioplasty, or stent implantation. Although short-term results are excellent with all three treatment modalities, long-term cardiovascular (CV) morbidity and mortality remain high, likely due to persistently abnormal vascular function. The effects of treatment modality on long-term vascular function remain uncharacterized. The goal of this study is to assess vascular function in this patient population for comparison among the treatment modalities. METHODS: We will prospectively assess vascular Afunction in large and small arteries fusing multiple noninvasive modalities and compare the results among the three groups of CoA patients previously treated using surgery, balloon angioplasty, or stent implantation after frequency matching for confounding variables. A comprehensive vascular function assessment protocol has been created to be used in 7 centers. Our primary outcome is arterial stiffness measured by arterial tonometry. Inclusion and exclusion criteria have been carefully established after consideration of several potential confounders. Sample size has been calculated for the primary outcome variable. CONCLUSION: Treatment modalities for CoA may have distinct impact on large and small arterial vascular function. The results of this study will help identify the treatment modality that is associated with the most optimal level of vascular function, which, in the long term, may reduce CV risk.

Extracellular vesicle-mediated transfer of processed and functional RNY5 RNA.

Extracellular vesicles (EVs) have been proposed as a means to promote intercellular communication. We show that when human primary cells are exposed to cancer cell EVs, rapid cell death of the primary cells is observed, while cancer cells treated with primary or cancer cell EVs do not display this response. The active agents that trigger cell death are 29- to 31-nucleotide (nt) or 22- to 23-nt processed fragments of an 83-nt primary transcript of the human RNY5 gene that are highly likely to be formed within the EVs. Primary cells treated with either cancer cell EVs, deproteinized total RNA from either primary or cancer cell EVs, or synthetic versions of 31- and 23-nt fragments trigger rapid cell death in a dose-dependent manner. The transfer of processed RNY5 fragments through EVs may reflect a novel strategy used by cancer cells toward the establishment of a favorable microenvironment for their proliferation and invasion.

Persistent Aortic Arch Hypoplasia After Coarctation Treatment Is Associated With Late Systemic Hypertension.

BACKGROUND: Mild transverse aortic arch (TAA) hypoplasia is common after coarctation treatment, but is considered benign in the absence of an arm-leg systolic blood pressure (SBP) difference. Hypertension (HTN) is a common long-term morbidity after successful coarctation treatment. We examined whether mild TAA hypoplasia after coarctation treatment is associated with late systemic HTN. METHODS AND RESULTS: We retrospectively reviewed 92 patients (median age, 19.9 years; range, 4.9 to 57.8; 60% male) 14.1±10.3 years after successful coarctation treatment (surgery in 63, stent in 16, and balloon dilation in 13), excluding those with resting right arm-leg blood pressure gradient >20 mm Hg, atypical coarctation, and major associated heart defects. Minimum body-surface area (BSA)-adjusted TAA cross-sectional area (CSA) was calculated from cardiac magnetic resonance (CMR) images. On follow-up, 38 of 92 (41%) patients had systemic HTN using standard criteria. Systemic HTN was independently associated with smaller TAA CSA/BSA (P=0.006; odds ratio [OR], 6.41 per 0.5 cm(2)/m(2) decrease), higher age at CMR (P=0.03; OR, 1.57 per 5-year increase), and in a subset (n=61), higher arm-leg SBP difference during exercise (P=0.05; OR, 1.03 per 1-mm-Hg increase). Lower ratio of TAA diameter/descending aorta diameter was associated with a larger increase in right arm SBP during peak exercise (P=0.006; r(2)=0.11). CONCLUSIONS: Persistent mild aortic arch hypoplasia, even in the absence of an arm-leg SBP difference at rest, is associated with late systemic HTN. Further studies should be undertaken to determine whether more-aggressive arch reconstruction at initial repair can reduce the incidence of systemic HTN.

Risk factors for profuse systemic-to-pulmonary artery collateral burden in hypoplastic left heart syndrome.

Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown. We performed a retrospective case-control study to identify risk factors for developing a profuse SPC burden in HLHS. Angiograms of 439 patients with HLHS (performed <2 years of age) were reviewed using a previously published angiographic grading scale to identify cases (profuse SPC burden, n = 20) and controls (no or minimal SPC burden, n = 35). In univariate analyses, profuse SPC burden was associated with mitral atresia and aortic atresia subtype (MA/AA) (65% vs 14%, p <0.0001), use of a Sano shunt (70% vs 37%, p = 0.03), longer log-transformed durations of intensive care unit stay (p = 0.02), hospital stay (p = 0.002), pleural drainage (p = 0.008) after stage 1 palliation, lower oxygen saturation at discharge after stage 1 palliation (82 ± 4 vs 85 ± 4%, p = 0.03), and a history of severe shunt obstruction (37% vs 11%, p = 0.04). In a multivariate logistic regression model, profuse SPC burden was associated with MA/AA subtype (odds ratio 6.6), Sano shunt type (odds ratio 8.6), and log-transformed duration of hospital stay after stage 1 (odds ratio 7.9, model p <0.0001, area under the curve 0.88). Nonassociated parameters included fetal aortic valve dilation, severe cyanotic episodes, number of days with open sternum or number of additional exploratory thoracotomies after stage 1 palliation, pulmonary vein stenosis, and restrictive atrial septal defect. In conclusion, in the present case-control study of patients with HLHS, the development of a profuse SPC burden was associated with MA/AA subtype, Sano shunt type, and longer duration of hospital stay after stage 1 palliation.

Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum.

OBJECTIVE: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). BACKGROUND: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. METHODS: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. RESULTS: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. CONCLUSIONS: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Relation of systemic-to-pulmonary artery collateral flow in single ventricle physiology to palliative stage and clinical status.

Systemic-to-pulmonary collateral arteries (SPCs) are common in patients with single-ventricle physiology, but their impact on clinical outcomes is unclear. The aim of this study was to use retrospective cardiac magnetic resonance data to determine the relation between SPC flow and palliative stage and clinical status in single-ventricle physiology. Of 116 patients, 78 were after Fontan operation (median age 19 years) and 38 were at an earlier palliative stage (median age 2 years). SPC flow was quantified as aortic flow minus total caval flow or total pulmonary vein flow minus total branch pulmonary artery flow. Median SPC flow/body surface area (BSA) was higher in the pre-Fontan group (1.06 vs 0.43 L/min/m(2), p <0.0001) and decreased nonlinearly with increasing age after the Fontan operation (r(2) = 0.17, p <0.0001). In the Fontan group, patients in the highest quartile of SPC flow had larger ventricular end-diastolic volume/BSA (p <0.0001) and were older at the time of Fontan surgery (p = 0.04), but SPC flow/BSA was not associated with heart failure symptoms, atrial or ventricular arrhythmias, atrioventricular valve regurgitation, the ventricular ejection fraction, or peak oxygen consumption. In multivariate analysis of all patients (n = 116), higher SPC flow was independently associated with pre-Fontan status, unilateral branch pulmonary artery stenosis, a diagnosis of hypoplastic left-heart syndrome, and previous catheter occlusion of SPCs (model r(2) = 0.37, p <0.0001). In conclusion, in this cross-sectional study of single-ventricle patients, BSA-adjusted SPC flow was highest in pre-Fontan patients and decreased after the Fontan operation with minimal clinical correlates aside from ventricular dilation.

Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience.

OBJECTIVES: The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type. BACKGROUND: Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested. METHODS: An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses. RESULTS: Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy. CONCLUSIONS: Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Late pulmonary valve replacement in patients with pulmonary atresia and intact ventricular septum: a case-matched study.

BACKGROUND: Pulmonary valve replacement (PVR) is a common therapy for chronic pulmonary regurgitation. However, the use of this strategy is mostly based on the studies performed on patients with tetralogy of Fallot (TOF) and not in patients with pulmonary atresia/intact ventricular septum (PA/IVS). The aim of this study is to evaluate our experience with PVR in patients with PA/IVS and compare them with a matched cohort of TOF patients. METHODS: Between 1995 and 2009, 13 patients with PA/IVS underwent a late PVR. Matched TOF control subjects were identified for 12 of these patients. Before and after PVR echocardiographic, magnetic resonance imaging, exercise test, Holter, and electrocardiographic data were compared between groups. RESULTS: There was no mortality in either group. The PVR improved pulmonary regurgitant fraction and right ventricular volumes in all patients. Patients with PA/IVS had more significant tricuspid regurgitation (TR [at least moderate]) by echocardiography and magnetic resonance imaging before PVR (n = 11 [85%] versus n = 1 [8%]; p = 0.003) and had more tricuspid valve repairs than TOF patients (n = 9 [69%] versus n = 1 [8%]; p = 0.004). Repair was undertaken by a combination of techniques. Although TR was improved early postoperatively, only 2 of 9 patients (22%) were free from significant TR at most recent follow-up (median 2.5 years; range, 0.1 to 10.9). No patient underwent reoperation at latest follow-up. CONCLUSIONS: Patients with PA/IVS can undergo a late PVR with excellent results. Significant TR and repair are more commonly observed among patients with PA/IVS compared with TOF patients. Although tricuspid valve repair improves regurgitation early, TR tends to recur, suggesting the need for further refinement of current surgical techniques.

Partial and transitional atrioventricular septal defect outcomes.

BACKGROUND: Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort. METHODS: We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change. RESULTS: Median age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt. CONCLUSIONS: Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.

A new diagnostic algorithm for assessment of patients with single ventricle before a Fontan operation.

OBJECTIVES: Cardiac catheterization has a low diagnostic yield before a Fontan operation, and magnetic resonance imaging and computed tomography are reliable alternatives to invasive angiography. A new diagnostic algorithm to avoid cardiac catheterization in "low-risk" subjects before a Fontan operation is proposed. METHODS: The proposed algorithm would identify "high-risk" subjects on the basis of risk factors on medical history, echocardiography, and noninvasive angiography. The efficacy of this algorithm in screening for subjects deemed to be inoperable after catheterization was evaluated retrospectively in 151 children. For this analysis, results of conventional angiography (assumed to be equivalent to noninvasive angiography) were used. RESULTS: According to the algorithm, 95 (63%) of 151 subjects had no risk factors ("low risk") whereas 56 (37%) of 151 had 1 risk factor or more ("high risk"). Nine (6%) of 151 subjects were found to be inoperable after catheterization and all 9 were correctly classified as high risk by the algorithm. In the 135 of 151 subjects who underwent a Fontan operation, the algorithm predicted an adverse postoperative outcome with a sensitivity of 51% and specificity of 78%. However, this prediction was not improved by including elevated pulmonary artery pressure or ventricular filling pressure as additional risk factors. CONCLUSIONS: The proposed algorithm effectively screened for subjects who were deemed unsuitable for a Fontan procedure. In addition, omitting preoperative invasive hemodynamic assessment did not impair prediction of adverse postoperative outcomes. Prospective evaluation of such a noninvasive diagnostic strategy before the Fontan operation is warranted.

Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure.

OBJECTIVE: The initial palliative procedure for patients born with hypoplastic left heart syndrome and related single right ventricle anomalies, the Norwood procedure, remains among the highest risk procedures in congenital heart surgery. The classic Norwood procedure provides pulmonary blood flow with a modified Blalock-Taussig shunt. Improved outcomes have been reported in a few small, nonrandomized studies of a modification of the Norwood procedure that uses a right ventricle-pulmonary artery shunt to provide pulmonary blood flow. Other nonrandomized studies have shown no differences between the two techniques. METHODS: The Pediatric Heart Network designed a randomized clinical trial to compare outcomes for subjects undergoing a Norwood procedure with either the right ventricle-pulmonary artery or modified Blalock-Taussig shunt. Infants with a diagnosis of single, morphologically right ventricle anomaly who are undergoing a Norwood procedure are eligible for inclusion in this study. The primary outcome is death or cardiac transplant 12 months after random assignment. Secondary outcomes include postoperative morbidity after Norwood and stage II palliation procedures, right ventricular function and pulmonary arterial growth at stage II palliation, and neurodevelopmental outcomes at 14 months old. Incidence of adverse events will also be compared between treatment groups. CONCLUSION: This study will make an important contribution to the care of patients with hypoplastic left heart syndrome and related forms of single, morphologically right ventricle. It also establishes a model with which other operative interventions for patients with congenital cardiovascular malformations can be evaluated in the future.