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Introduction: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) with pulmonary blood flow supplied by the ductus arteriosus require early intervention. This may be accomplished by: initial palliation (IP) followed by complete repair (CR) or initial primary repair (PR). The optimal approach for patients with TOF/PA has not been established. Methods: Neonates with TOF/PA who underwent IP or PR from 2005-17 were retrospectively reviewed from the Congenital Catheterization Research Collaborative. The primary outcome was mortality. Outcomes were compared as IP vs PR and IP+CR vs PR. Secondary outcomes included hospital and procedural complications and are listed in table 1. Propensity scoring was used to adjust for baseline differences between strategies. Results: Of 282 neonates with TOF/PA, 106 underwent PR and 176 underwent IP (144 surgical, 32 transcatheter). Prior to initial intervention, IP patients had higher rates of mechanical ventilation (83.9% vs 72.2%, p=0.023) and DiGeorge syndrome (14.77% vs 4.72%, p=0.009). Mortality was greater in the IP cohort (HR 2.7, 95% CI 1.02 - 7.1, p = 0.046), with no mortality in the PR cohort after 6 months post-repair. After adjustment, differences in survival were no longer significant (HR 1.4, 95% CI 0.7 - 3.1, p=0.39). Both cohorts had similar mechanical ventilation duration and inotrope use as well as procedural and hospital complications. Intensive care and hospital length of stay, cardiac bypass (CPB) and anesthesia time favored PR when compared to IP+CR (p= Conclusions: In neonates with TOF/PA, the IP approach is more often utilized in higher-risk patients. Accounting for this difference, IP and PR strategies have similar adjusted survival rates. Perioperative morbidities and lower risk for reintervention generally favor PR.

Introduction: Maternal exposure to various pathogens during pregnancy has been well established as a cause of congenital heart defects (CHD). Suggestive, although inconclusive, data exists of an association between Coxsackievirus B (CVB) infection during pregnancy and CHD. Therefore, this study was undertaken to examine association between perinatal infection with CVB and CHD. Hypothesis: CVB infection during pregnancy may play a role in the pathogenesis of CHD. Methods: In a prospective study of 122 pregnant women with pregnancies affected with CHD, circulating CVB IgG and IgM antibodies in the maternal serum were quantified. To determine specific CVB serotypes involved, Neutralizing antibodies (NA) were measured against three CVB serotypes: CVB1-Chi07, CVB3, Nancy, and CVB4, J.V.B. Results: A significant number of the women were positive for CVB antibodies; 31.1% for IgG and 6.6% for IgM. Elevated levels of IgG were seen in 50% of women carrying babies with Pulmonary Atresia (PA) (p=0.04). In addition, 55.6% (p=0.100) of moms carrying babies with Hypoplastic Left Heart Syndrome (HLHS), aortic atresia/mitral stenosis (AA/MS) variant and 47.1% (p=0.127) of HLHS AA/mitral atresia (MA) had positive IgG levels. Among babies of women with positive IgM titers, 75% (p=0.001) had isolated Ventricular Septal Defect (VSD), comprising 26.1% (6/23) of all babies with isolated VSD in the cohort.We also show a possible association between CVB4 and CHD in the cohort. All mothers with positive IgM titers whose babies were born with a VSD had elevated CVB4 NA (p=0.001). Among women with elevated IgG having babies with PA, 40.0% (p=0.061) had elevated CVB1 NA, 70.0% (p=0.014) had elevated CVB3 NA, and 40.0% (p=0.876) had elevated CVB4 NA. Conclusion: We present data from a clinical study suggesting that CVB infection during pregnancy may contribute to the burden of CHD in affected babies. Our data demonstrates not only an association of CVB with CHD in pregnant women but this is the first report showing significant association between in-utero CVB infection and PA. Our study has broad implications for the future understanding and potential management of CHD as well as introduces a new avenue of research for cardiac defects.

Introduction: Pulmonary atresia with intact ventricular septum (PAIVS) is surgically managed with biventricular repair (2V), 1.5 ventricle palliation (1.5V), or single ventricle palliation (1V). Cardiopulmonary exercise testing (CPET) has been increasingly utilized in these patients. However, prognostic ability of CPET is limited by achievement of maximal exercise effort [respiratory exchange ratio (RER) > 1.1]. Even during submaximal exercise, the VE/VCO2 can impact peak VO2. This study aims to identify CPET differences in 1V, 1.5V, and 2V PAIVS patients. Methods: A retrospective, cross-sectional study was performed, identifying PAIVS patients undergoing CPET. Contemporaneous echocardiography and CMR data was collected. CPET measures were compared by treatment group using ANOVA, Kruskal-Wallis, and chi-squared test, as appropriate. Comparisons of VE/VCO2 between individual groups were performed using Wilcoxon test. Univariate associations with VE/VCO2 were determined using Pearson correlation. Results: Nineteen PAIVS patients were identified (age 12.4 ± 0.68; seven 1V, five 1.5V, seven 2V). Only 7/19 (36.8%) patients achieved RER > 1.1. Sex, age, RER, peak VO2, ventilatory anaerobic threshold, O2 pulse, BSA-adjusted O2 pulse, peak HR, and HR reserve did not differ between treatment groups. The VE/VCO2 ratio was different (p=0.037), with lower VE/VCO2 in 1.5V vs. 1V (p=0.021). Across all PAIVS patients, univariate associations with lower VE/VCO2 were male sex, higher BSA, hematocrit, and O2 pulse, and lower mitral inflow A wave velocity. In the 1.5V and 2V patients, higher RVEF and RV stroke volume by CMR were associated with lower VE/VCO2. Conclusions: The 1.5V palliation of PAIVS may be associated with better gas exchange efficiency compared to 1V palliation, while 2V patients were not different from either 1V or 1.5V. BSA-adjusted O2 pulse did not vary between treatment pathways, suggesting similar stroke volume response across all patients.

Survival into adulthood of patients with unrepaired cyanotic congenital heart defects (CHDs) is possible when cyanotic CHDs are deemed unsuitable for radical surgical repair but are compatible with survival. These situations include, for example, complex pulmonary atresia with aortopulmonary collaterals and single-ventricle hearts (with and without earlier palliation). This is also the case when patients with cyanotic CHDs reach adulthood without serious symptoms requiring surgery, such as those with mild tetralogy of Fallot, Ebstein’s anomaly, and some cases of corrected transposition of the great arteries with pulmonary stenosis and ventricular septal defect. Many women with these heart conditions wish to become pregnant, which creates a situation of high maternal and fetal risks of complications.1 Management of these patients before, during, and after pregnancy has improved, with an earlier recognition of the underlying disease, improved understanding of cardiopulmonary physiopathology, better prenatal and peri-partum obstetric/anesthetic management, and the introduction of a multidisciplinary approach.2 We retrospectively reviewed the charts of all pregnant women with cyanotic CHDs (n=51) who were followed in 11 adult CHD referral …

Background— Aspirin (ASA) often is used to prevent thrombosis in infants with congenital heart disease after placement of a systemic-to–pulmonary artery shunt, but its effect on outcomes is unknown. Methods and Results— The present multicenter study prospectively collected data on 1-year postoperative rates of death, shunt thrombosis, or hospitalization age P =0.008) and death (closed shunt: hazard ratio, 0.41, P =0.057; open shunt: hazard ratio, 0.10, P P P =NS) compared with those not receiving ASA. Conclusions— The morbidity and mortality for infants after surgical placement of a systemic-to–pulmonary artery shunt are high. ASA appears to lower the risk of death and shunt thrombosis in the present observational study.

Introduction: The naturally poor survival of pulmonary atresia with ventricular septal defect (PA+VSD) has improved due to evolved perioperative and surgical treatment. Studies including PA+VSD patients, both with and without major aortopulmonary collateral arteries (MAPCAs), with extensive follow-up are scarce. This nationwide study aimed to investigate survival and surgical treatment in PA+VSD patients with and without MAPCAs. Methods: Study comprised 109 PA+VSD patients born in Finland between 1970 and 2007. We reviewed retrospectively medical records and operative reports through December 2011, as well as first available angiograms and preoperative angiograms prior to repair attempt. Results: The median follow-up time for the total study population, including also patients who died during the follow-up, was 11.4 years (IQR 0.8 - 21.1). The incidence of PA+VSD, which could be determined reliably from 1995 to 2007, was 6.1 per 100 000 live births. Although the patients with (n = 43) or without MAPCAs (n = 66) showed no difference in survival (p = 0.74), the patients without MAPCAs had better probability to achieve repair (64% vs. 28%, p < 0.0001). The bigger size of true central pulmonary arteries assessed by McGoon index at first angiogram [HR 0.66 (CI95% 0.49 - 0.88) per 0.5 McGoon index units, p = 0.006] and achievement of repair [HR 0.07 (CI95% 0.03 - 0.17), p < 0.0001] improved the overall survival. After successful repair survival was 93% at 1 year and 91% from 2 years on. Palliated patients, instead, had survival at 1, 5, 10, and 20 years of age of 55%, 42%, 34%, and 20% respectively. However, patients with right ventricle - pulmonary artery connection and septal fenestration had better survival than rest of the palliated patients (p = 0.001). Palliation with a systemic-pulmonary artery shunt increased McGoon index by 41% (p < 0.0001). Conclusions: The patients with MAPCAs had higher risk to remain palliated than patients without, although their survival was similar. Survival of PA+VSD was influenced by the initial size of true central pulmonary arteries and whether repair was achieved. Although palliative procedures may not improve the final outcome of PA+VSD, palliative surgery may have a role in its treatment.

Background— This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease. Methods and Results— Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively. Diagnoses were pulmonary atresia and ventricular septal defect in 11, tetralogy of Fallot in 6, and other forms of pulmonary stenosis or atresia in 6. Median age and weight at diagnosis were 9 years (range, 6 days to 43 years) and 24 kg (range, 2.6 to 60 kg). Fourteen patients had had a previous surgery. The occluded pulmonary artery was visualized at angiography by wedge injection or injection into the collateral circulation. The left pulmonary artery was occluded in 20 patients and the right pulmonary artery in 3. Criteria for reconstruction were estimated duration of occlusion 0.2. Twelve patients fulfilled these criteria and underwent pulmonary artery reconstruction at a mean interval of 2 months (range, 6 days to 6 months) from evidence of occlusion. Six patients had pericardial patch reconstruction, 3 terminoterminal anastomosis, 2 thrombectomy, and 1 a Blalock-Taussig shunt. There was 1 late death. At a median follow-up of 4 years (2 months to 5 years), all patients underwent cardiac catheterization: in 8 patients the reconstructed artery was patent, in 3 reoccluded. Hypoplasia of the occluded artery was reversed in 6 patients. Conclusions— Our data show that in selected patients, reconstruction of an occluded pulmonary artery can restore pulmonary vascularization and reverse hypoplasia. Strict surveillance is mandatory to prevent pulmonary artery loss.

Background— Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve. Methods and Results— Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1 ½-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time. Conclusions— Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle–dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.

A 59-year-old woman was admitted because of cyanosis and dyspnea on exertion and at rest. In her childhood, she was suspected of having ventricular septal defect (VSD), but she refused to undergo cardiac catheterization and operation. Dyspnea on exertion gradually developed after adolescence. On admission, chest roentgenography demonstrated enlarged cardiac silhouette with elevated cardiac apex, a right aortic arch, and enlargement of the main pulmonary arteries and their major branches with increased pulmonary arterial vascularity (Figure 1). Echocardiography revealed a large VSD which lay beneath the dilated aorta that overrides the interventricular septum, hypertrophied right ventricle, and the blind outflow tract of the right ventricle …

Hybrid cardiac procedures are a result of synergistic cooperation between the cardiologist and surgeon. They are a combination of surgical repair and intraoperative catheter-based interventions, and may provide an effective option for the repair of complex congenital heart lesions. We present a 30-year-old man with pulmonary atresia and a ventricular septal defect. He previously had a Waterston shunt placed at 10 days of age and a right pulmonary artery shunt at 4 years of age. He had ventricular septal defect closure with a 23-mm homograft conduit connecting the right ventricular outflow tract to the main pulmonary artery confluence at 9 years of age. The patient presented to us 21 years later with pulmonary conduit obstruction, right pulmonary artery stenosis, and an occluded left pulmonary artery (LPA; Figures 1, 2A, and 2B). The catheterization demonstrated right ventricular pressure (70% of systemic arterial pressure) at 89/14 mm Hg. There was a 28-mm Hg gradient to the right pulmonary artery, and the LPA was occluded. A 7-mm distal LPA was demonstrated by left pulmonary vein wedge angiogram. Given the long occlusion between the conduit and the lower LPA, we were concerned that the LPA would not be accessible for direct patch augmentation, and hence decided to perform a hybrid procedure; …

Introduction: Adults with congenital heart disease (ACHD) are at increased risk for early adverse outcomes following heart transplantation (Htx). Despite the need for improved risk stratification, small cohorts have constrained identification of patient-specific factors associated with poor prognosis. We hypothesized that type of CHD, number of sternotomies and prior end-organ dysfunction would be associated with an increased risk for mortality post-HTx. Methods: We performed a retrospective, observational cohort study of all patients with ACHD who underwent HTx at our institution from 1/1997 to 1/2014. The primary endpoint was death. Exposures of interest included type of CHD, number of sternotomies and hepatic dysfunction secondary to passive congestion, measured as high Models for End-Stage Liver Disease Excluding INR (MELD-XI) score. Results: 48 ACHD patients were followed (mean age at HTx: 36±1.7 years). Diagnoses included: Tetralogy of Fallot (TOF)/pulmonary atresia/double outlet right ventricle in 15 (31%), D-transposition of the great arteries in 10 (21 %), tricuspid atresia/double inlet left ventricle in 9 (19%), VSD/ASD in 4 (8%), heterotaxy in 3 (6%), congenitally corrected transposition of the great arteries in 2 (4%), and 5 (10%) with other diagnoses. Pre-transplant cardiac surgeries included: Fontan operation in 12 (25%), Mustard/Senning repair in 9 (19%), TOF repair in 5 (11%), Rastelli repair in 5 (11%), VSD/ASD closures in 3 (6%), other another congenital heart surgery in 8 (17%) and no prior cardiac surgery in 5 (11%). Over a median follow-up time of 3.9 years, 14 patients died (29%) and 10 (71%) deaths occurred within 30 days of HTx. Survival at 1 and 5 years was 77%. Deaths within 30 days of surgery were secondary to hemorrhage in 4 (40%), graft failure in 3 (30%) and multi-system organ failure in 3 (30%). By multivariate analysis, ≥3 sternotomies (HR 8.5, p=0.02), MELD-XI score >18 (HR 6.2, p=0.009) and heterotaxy (HR 9.8, p=0.01), were significant predictors of mortality. Conclusion: In our cohort of patients with CHD undergoing Htx, ≥3 sternotomies, MELD-XI score >18 and heterotaxy syndrome were significantly associated with death. These findings may be important in patient selection and timing of Htx in this population.

A 20-year-old man was admitted to our hospital for evaluation of his coronary arteries. His daily life activities were well established without any symptoms indicating myocardial ischemia. He had a history of repeat cardiac surgeries as a result of cyanosis consisting of a right Blalock-Taussig shunt 3 months after birth and a Fontan procedure at 2 years of age for pulmonary atresia with an intact ventricular septum (PA-IVS). Then, dilation of the middle left anterior descending (LAD) coronary artery without collaterals, an elevation of the right ventricular (RV) systolic pressure (86/8 mm Hg), and a decrease in the aortic pressure (81/51 mm Hg) were detected. Because there were no findings of the formation of coronary aneurysms of the dilated LAD coronary artery, the physicians carefully followed them up. Moreover, because of the RV-dependent coronary circulation, no surgical or catheter interventional repair of the PA to reduce the RV pressure, which could lead to myocardial ischemia in the LAD …

Background Recent reports have implicated mutations in the transcription factor NKX2.5 as a cause of tetralogy of Fallot (TOF). To estimate the frequency of NKX2.5 mutations in TOF patients and to further investigate the genotype-phenotype correlation of NKX2.5 mutations, we genotyped 114 TOF patients. Methods and Results Patients were recruited prospectively (November 1992 through June 1999) and tested for a 22q11 deletion; those with 22q11 deletion or recognized chromosomal alteration were excluded from the present study. Patients were screened for NKX2.5 alterations by conformation-sensitive gel electrophoresis and sequencing of fragments with aberrant mobility. Four heterozygous mutations were identified in 6 unrelated patients with cases of TOF, including 3 with pulmonary atresia and 5 with right aortic arch; none had ECG evidence of PR interval prolongation. Three of 4 mutations (Glu21Gln, Arg216Cys, and Ala219Val) altered highly conserved amino acids, of which 2 mapped in the conserved NK2 domain. The fourth mutation (Arg25Cys) was identified in 3 unrelated probands in the present study and has been previously reported. No homeodomain mutations were identified. Conclusions NKX2.5 mutations are the first gene defects identified in nonsyndromic TOF patients. NKX2.5 mutation is present in ≥4% of TOF patients. Mutations identified in the present study mapped outside of the homeodomain, were not associated with atrioventricular conduction disturbances, and were not fully penetrant, in contrast to mutations previously reported that impair homeodomain function.

Background —Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. Methods and Results —Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo–pulmonary arteries was performed in 24 patients. Conclusions —Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

Background —Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. Methods and Results —From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100 000 live births). The incidence was 4.1 cases per 100 000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland ( P =0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100 000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths ( P =0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. Conclusions —PAIVS is rare, occurring in 1 in 22 000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.

Background The bidirectional cavopulmonary shunt has become an important intermediate step in the treatment of pediatric patients with single ventricle physiology who are ultimately destined for palliative surgery. We wanted to know whether there would be risks or benefits if an additional source of pulmonary blood flow was left after a bidirectional cavopulmonary shunt. Methods and Results We retrospectively reviewed the medical and surgical records of all patients who underwent a bidirectional cavopulmonary shunt at the Children’s Hospital of Wisconsin between January 1991 and December 1993. A total of 43 patients were identified. Anatomic diagnoses included double inlet left ventricle (14 patients), tricuspid atresia (8 patients), pulmonary atresia with intact septum (6 patients), single right ventricle (5 patients), hypoplastic left heart (3 patients), unbalanced atrioventricular septal defect (3 patients), and other complex lesions (4 patients). We then divided the patients into two groups for purposes of analysis. Group 1 had only the cavopulmonary shunt as a source of pulmonary flow (22 patients); group 2 had an additional source of pulmonary flow (21 patients). Patient age at the time of cavopulmonary shunt ranged from 6 months to 12 years, with group 1 patients being younger (31 versus 45 months, P =.05). Group 2 patients had higher postoperative central venous pressures (17.8 versus 14.1 mm Hg, P P P Conclusions We conclude that patients with an additional source of pulmonary blood flow after bidirectional cavopulmonary shunt have higher postoperative central venous pressures, have higher oxygen saturations, and are at risk for the late development of a chylothorax.

Background Total cavopulmonary connection (TCPC) to repair functional single ventricle involves the sinus node area, in contrast to the Fontan procedure. We compared ECG findings after TCPC and Fontan to evaluate the impact of the cavopulmonary connection on sinus rhythm postoperatively. Methods and Results The Fontan group consisted of 17 patients repaired at 7.8±3.1 years of age (mean±SD): 11 for tricuspid or pulmonary atresia (TA/PA) and 6 for single ventricle. The TCPC group consisted of 19 patients repaired at 5.1±3.2 years of age (mean±SD) ( P P P 2 test). By 2 years postoperatively, only 6 of 15 TCPC patients available for follow-up (40%) were in SR, with 7 of 15 in JR and 2 of 15 in NSAR. By contrast, 13 of 17 Fontan patients (76%) remained in SR, with 1 in NSAR and 3 in JR ( P 2 test). TCPC patients with loss of SR did not differ from other patients in the group in age at repair, preoperative diagnosis, or surgeon performing the procedure. Conclusions This significant incidence of loss of SR temporally related to surgery suggests that operative compromise of the sinus node area is common with TCPC.

BACKGROUND Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) are frequently born with hypoplastic right heart structures that must grow after right ventricular decompression (RVD) procedures for a complete two-ventricle physiology to be achieved. Previous authors have asserted that neonatal right heart size or morphology will predict right heart growth potential. Since 1983, our bias has favored early RVD regardless of initial right heart size. In 1986, we recognized a subset of patients with coronary artery abnormalities associated with poor outcome after RVD and have defined these patients as having a right ventricular-dependent coronary circulation (RVDCC). METHODS AND RESULTS To assess the influence of right heart size on outcome independent of the presence of RVDCC, we measured echocardiographic right ventricular (RV) dimensions in 37 neonates with adequate studies presenting between 1983 and 1990. Coronary artery anatomy was adequately assessed by angiography in 36. RV volume and tricuspid valve (TV) diameter were significantly smaller in patients with RVDCC than in those without. However, there was no statistically significant association between RV volume or TV diameter and survival among patients with or without RVDCC: Among 29 patients without RVDCC, 23 of 24 (95.8%) who achieved RVD are alive compared with 1 of 5 (20%) who did not achieve RVD (P = .001). Twenty-one of the 23 survivors have a complete two-ventricle physiology with low right atrial pressure. Among 7 patients with RVDCC, 2 patients who underwent RVD died early of left ventricular failure, whereas 4 of 5 who did not undergo RVD have survived single ventricular palliation. CONCLUSIONS Small right heart size is associated with RVDCC but is not associated with survival in PA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heart size.

BACKGROUND The literature is ambiguous as to the question of the developmental background of systemic-pulmonary collateral arteries. These are found in combination with various congenital heart malformations such as pulmonary atresia. From a clinical point of view, it is of interest to know whether we are dealing with the persistence of transient embryological vessels such as ventral segmental arteries or parts of pharyngeal arch arteries or with the prenatal or postnatal recruitment of the bronchial vasculature that normally supplies the lung. This study of the embryology of the extrapulmonary and intrapulmonary vasculature aims at a better understanding of the variations in origin, course, branching pattern, and histology of collateral arteries. METHODS AND RESULTS Serial sections of quail embryos ranging between stage HH11 and stage HH28 were incubated with a monoclonal antibody (alpha MB1) against endothelial cells and their precursors. Additional series of chick embryos were injected with india ink to study the lumenized vascular patterns. A splanchnic plexus consisting of endothelial cells and precursors is present around the foregut before the lung buds develop. This plexus expands and gives rise to the pharyngeal arch arteries, the ventral pharyngeal veins, the pulmonary vessels, and the bronchial vessels, including the intrapulmonary vessel network. During two subsequent periods, the splanchnic plexus is transiently connected to the systemic arteries and veins. The bronchial arteries and veins develop in the second period from these transient vessels. The expansion and extension of the splanchnic plexus to many organs during the formation of the bronchial vessels explains the varying course and branching pattern of the bronchial vasculature. CONCLUSIONS These results show that we are not dealing with two or more individual vascular systems that contribute to the developing vessels of the lungs but with one vascular plexus that normally gives rise to the pulmonary and bronchial vasculature but has the potential to give rise to other systemic-pulmonary connections.

BACKGROUND The Fontan operation is the usual goal of therapy for children with tricuspid atresia. The influences of morphology and different management strategies on survival and subsequent suitability for this procedure are crucial but unstudied in an unselected population during the Fontan era. METHODS AND RESULTS The fates of 237 consecutive infants with tricuspid atresia were reviewed (1972-1987; median follow-up, 8.0 years). Overall actuarial survival was 72% at 1 year, 53% at 5 years, and 46% at 10 years. Univariate risk factor analysis established that discordant ventriculoarterial connections (24% of the group; relative risk, 2.7), pulmonary atresia (14%, 2.3), aortic arch obstruction (7%, 2.9), and subaortic stenosis (8%, 4.2) were associated with poor survival, whereas pulmonary stenosis (60%, 0.52), balanced pulmonary blood flow (9%, 0.25), and older age at presentation (33%, 0.42) were beneficial. Multivariate analysis allowed the creation of predictive patient-specific survival curves and two additive indexes. Survival was worse for patients who underwent banding of the pulmonary trunk with aortic arch repair than for other individual palliative procedures (p < 0.001). On retrospective review, 204 patients (86%) were judged suitable for a future Fontan procedure at presentation. However, 99 (48%) of these are known to have died before a Fontan operation or became unsuitable for such surgery during follow-up, mostly because of death after palliative surgery (23 patients, 11%), sudden death (18 patients, 9%), and new adverse features (32 patients, 16%) such as subaortic stenosis, pulmonary arterial distortion, and ventricular dysfunction. CONCLUSIONS Management in infancy must aim to ensure survival and maintain suitability for a Fontan-type operation. The accumulating incidence of adverse events with increasing age would argue in favor of undertaking definitive surgery in early childhood in most patients.

Background— The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. Methods and Results— The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8±12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. Conclusions— The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.

Background— Infants with functional single ventricle have a high risk of death during the early years of life. Studies have reported improvement in postoperative survival, but they do not include preoperative deaths or those occurring before transfer. The purpose of this population-based study was to estimate 5-year survival in infants with functional single ventricle, to define factors associated with survival, and to estimate improvement in outcome. Methods and Results— Patients with hypoplastic left heart syndrome, pulmonary atresia intact ventricular septum, single ventricle, and tricuspid atresia born in 1996 to 2003 were identified from the Texas Birth Defects Registry and linked to state and national birth and death vital records. We examined the effects of defect type, birth era, birth weight, gestational age, maternal race/ethnicity, extracardiac anomalies, sex, and maternal age and education on survival. Five-year survival varied by defect type: hypoplastic left heart syndrome, 38.0% (95% confidence interval, 32.6 to 43.5); single ventricle, 56.1% (95% confidence interval, 49.9 to 61.7); pulmonary atresia intact ventricular septum, 55.7% (95% confidence interval, 45.8 to 64.4); and tricuspid atresia, 74.6% (95% confidence interval, 62.4 to 83.4). The presence of extracardiac defects increased the adjusted risk of death by 84%. Non-Hispanic blacks had an adjusted risk of death that was 41% higher than that for non-Hispanic whites, and Hispanics had a 26% higher risk. Patients born in 2001 to 2003 had a 47% lower risk than those born in 1996 to 2000. Conclusions— This population-based study demonstrates significant improvement in overall 5-year survival, particularly in cases of hypoplastic left heart syndrome and single ventricle. Additional studies are needed to determine the factors causing racial/ethnic and regional differences in outcome.

BACKGROUND Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses. METHODS AND RESULTS Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction. CONCLUSIONS 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.

Background— The objective was to determine if intraoperative pulmonary artery (PA) flow studies after complete unifocalization correlate with postrepair hemodynamics for pulmonary atresia (PA), ventricular septal defects (VSD), and major aortopulmonary collaterals. Methods and Results— Twenty patients (median age, 8 months; weight, 7.9 kg) underwent unifocalization between 2003 and 2008. A functional PA flow study was achieved by cannulating the unifocalized central PA before intracardiac repair and increasing flow incrementally to 2.5 L/min per m 2 . Mean PA pressure (mPAP) was measured. The intent was to close the VSD for a mPAP of P =0.0027) and RVSP/systemic systolic pressure (ρ=0.67; P =0.0063). Total neopulmonary artery index had a nonsignificant negative correlation with RVSP (ρ=−0.42; P =0.079). Total incorporated pulmonary segments and pulmonary segment artery ratio were not correlated. Flow study mPAP had the highest accuracy in predicting successful VSD closure: area under the receiver-operator curve (0.83) versus total neopulmonary artery index (0.42), pulmonary segments (0.35), and pulmonary segment artery ratio (0.33). Conclusions— The intraoperative pulmonary flow study predicted postoperative physiology significantly better than did standard anatomic measures. Conventional measures should be used with caution when determining the possibility for complete repair.

The total right ventricular (RV) exclusion procedure for isolated congestive RV failure, in which most of the dilated right atrium (RA) and RV wall is resected, was first reported by Sano et al in 2002.1 This procedure improves the cardiac index2 and suppresses supraventricular/ventricular tachyarrhythmia. Using this procedure with an extracardiac conduit, we successfully treated severe heart failure characterized by a markedly dilated right heart lacking tricuspid leaflets and by recurrent tachyarrhythmia, 17 years after the patient had undergone initial definitive intracardiac repair including RV outflow tract plasty. A 22-year-old woman presented with general fatigue, dyspnea, palpitations, and loss of appetite of 6 months’ duration caused by low cardiac output. She was admitted to the University of Tokyo Hospital to undergo surgery to improve her low cardiac output. She was cyanotic at birth after an uneventful pregnancy and caesarian delivery. Cardiac catheterization performed when she was 47 days old revealed pulmonary atresia with atrial septal defect and patent ductus arteriosus. A Blalock-Taussig anastomosis was established. Subsequently, she was diagnosed with absent tricuspid valve, pulmonary atresia, and atrial septal defect. These were definitively treated surgically in 1990 when the patient was 5 years old via RV outflow tract plasty and atrial septal defect closure. Intraoperative findings included weak RV contraction and an anterior RV wall almost totally lacking myocardium. Five years after the operation, she suffered from ventricular tachycardia originating from the RV wall, which was treated by electrical cardioversion and subsequent administration of oral antiarrhythmic medication. Six months before admission, dyspnea …

A 25-year-old man with pulmonary atresia and ventricular septal defect had a right and a left Blalock Taussig shunt during infancy. At the age of 12 he had a 3rd surgical operation, consisting of unifocalization of systemic collateral arteries to the right lung, and complete intracardiac repair by ventricular septal defect closure and interposition of a 25 mm homograft between the infundibulum and the pulmonary trunk. No complications were observed in the postoperative period. The patient led a normal life for 13 years, but after this period he started to refer dyspnea at exercise. There was no evidence of active or previous infection. At clinical examination the patient was in good general condition and had a systolic murmur on the left sternal …

A neonate was admitted at the age of 8 days with a history of increasing cyanosis and tachydyspnea after a normal pregnancy and delivery at 42 weeks’ gestation. A transthoracic echocardiography appeared to indicate pulmonary atresia with an intact ventricular septum. The child was intubated, intravenous prostaglandin E1 was started, and the patient was transferred to our hospital. The newborn presented with central cyanosis while being ventilated with 100% oxygen. No heart murmur was perceptible. Transthoracic echocardiography revealed marked apical displacement of the tricuspid valve leaflets (Figure 1 and Movie I). Linear attachments of the anterior and the mural leaflets resulted in complete separation of the inlet portion from the apical trabecular and outlet portions of the right ventricle (Figure 2 and Movie II). On color Doppler, we were unable to demonstrate either antegrade or regurgitant flow across the valve …

Background— Early outcomes after tricuspid valve replacement in young children are ill defined. The experience of the Pediatric Cardiac Care Consortium (45 centers, 1984 to 2002) was reviewed to evaluate the results of tricuspid valve replacement in children Methods and Results— Ninety-seven patients who underwent initial tricuspid valve replacement are included in the present analysis. The most frequent cardiac diagnoses were Ebstein’s anomaly (40%), pulmonary atresia (11%), and tetralogy of Fallot (8%). Age at tricuspid valve replacement was 2.9±1.7 years (mean±SD). Mean patient weight was 12.7±6.1 kg. The major outcome was survival to discharge. Associations among age, diagnosis, valve type/size, and outcome were evaluated through the use of χ 2 analysis and logistic regression model fitting approaches. Hospital mortality was 26% and was very high (64%) in patients P 2.5. Other complications included heart block requiring a pacemaker (13%) and thrombosis (5%). Pacemaker implantation was associated with the use of a mechanical valve (23% versus 6% bioprosthetic valve; P =0.01) Conclusions— Tricuspid valve replacement in young children is associated with high mortality, especially in infants

Imaging of the fetal heart to diagnose congenital heart disease has become commonplace during the past 30 years, to the point that institutions such as the American College of Obstetrics and Gynecology, the American College of Radiology, and the American Institute of Ultrasound in Medicine have established, as a standard of care, routine screening for congenital heart disease in the scanning protocol of fetuses undergoing ultrasound study for any reason during the second or third trimester of pregnancy. Article p 1401 Until recently, the parents of fetuses diagnosed with congenital heart disease have had to face the limited but difficult decisions of continuation or termination of the pregnancy for these offspring and, when the pregnancy was continued, the decision of where, when, and how to deliver and whether to seek aggressive medical and surgical therapy during the neonatal period. More recently, however, the option of catheter intervention to alter the natural history of fetal aortic stenosis or pulmonary atresia has been offered at several medical centers.1–5 Although attempting to prevent the progression of aortic stenosis into hypoplastic left heart syndrome (HLHS) on its surface might appear to be an obvious choice, it should be noted that by engaging in fetal therapy, the pediatric cardiology community as a group is entering a field with complex and unique legal and ethical underpinnings that have been formulated during a period of more than 35 years. Unless the pediatric cardiology community takes note of the experiences of our colleagues in maternal-fetal medicine and pediatric surgery in the field of fetal intervention, it is likely that we and our patients will be doomed to revisit the errors of judgment that were made in well-intentioned efforts to treat fetal conditions such as diaphragmatic hernia,6–8 obstructive uropathy,9,10 and hydrocephaly.11–13 In those situations, frustration …

A 41-year-old man with untreated tetralogy of Fallot and pulmonary atresia was referred to our department for imaging. His diagnosis had been established in early infancy, but cardiac surgery was repeatedly refused over the years. In addition to central cyanosis and clubbing of the fingers, the patient started experiencing worsening exertional dyspnea, cough, and pain in the back and left shoulder within the last 2 years. A transthoracic echocardiogram revealed, in addition to the findings consistent with his congenital …

Magnetic resonance imaging data of a 20-year-old male patient was used to produce, to the best of our knowledge, the first cast model of a beating heart. Imaging was performed at the German Heart Institute Berlin, Department of Congenital Heart Disease and Pediatric Cardiology; data were transferred to the Department of Medical and Biological Informatics (German Cancer Research Center, Heidelberg). The patient had the diagnoses of dextrocardia, atrial and visceral situs inversus, L-looped ventricles, and L-malposition of the great arteries, with a large perimembranous ventricular septal defect and pulmonary atresia. A 3D whole-heart, navigator-corrected MRI data set …

Background— Dilatation of the aortic root is a known feature in tetralogy of Fallot (TOF) patients with pulmonary stenosis (PS) or pulmonary atresia (PA). We hypothesized that intrinsic histological abnormalities of the aortic wall present since infancy are an important causative factor leading to aortic root dilatation. Methods and Results— We examined the aortic histology of 17 cases with TOF and PS/PA from our cardiac morphology archive and compared them with a control group of normal aortas. Measured circumference of the aortic root at the sinotubular junction and at the ascending aorta was indexed to the left ventricle. Aortic walls were studied by light microscopy with the use of various stains. Seventeen TOF cases (7 with PS, 10 with PA) including 7 infants, 2 children, and 8 adults were compared with 11 hearts with normal aorta. Aortic root circumference to left ventricular index and ascending aortic circumference to left ventricular index were 1.24±0.25 and 1.37±0.24, respectively, in the TOF group versus 0.89±0.10 and 0.88±0.11, respectively, in the control group ( P r =0.525, P =0.03). Conclusions— There are marked histological abnormalities in the aortic root and ascending aortic wall of patients with TOF present from infancy, suggesting a causative mechanism for subsequent aortic root dilatation.

A 12-year-old boy with pulmonary atresia and ventricular septal defect was initially treated with 2 modified Blalock-Taussig shunts. Total repair with closure of the ventricular septal defect and placement of a nonvalved 18-mm conduit from the right ventricle to the pulmonary artery had been performed when he was 5 years old. …

A 55-year-old woman with congenital heart disease and increasing dyspnea at low levels of exertion was referred to our department for imaging. Clinical findings were cyanosis of the lips and clubbing of the fingers. The past medical history included several hospitalizations for dyspnea, tachycardia, recurrent pulmonary embolism, and suspected cerebral embolism. Despite several conventional angiographic studies, the exact classification of the congenital heart disease and the vascular abnormalities had not been made …