Early extubation is associated with improved outcomes after complete surgical repair of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries in pediatric patients.

BACKGROUND: The aim of this study was to investigate the impact of an early extubation strategy on outcomes following complete repair of pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary artery. METHODS: One hundred thirteen patients undergoing complete repair surgery of pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary artery between 2016 and 2018 were included in our retrospective propensity-score matched study. Propensity score matching was conducted in 1 to 2 ratio to balance the covariables impacting on clinical outcomes between groups. The primary outcomes were defined as length of intensive care unit stay, postoperative length of hospital stay and in-hospital medical cost. The secondary outcomes included postoperative complications such as re-intubation, re-exploration, in-hospital mortality, arrhythmia and etc.. In addition, blood product consumption were also abstracted. RESULTS: Compared with matched controls, patients in the early extubation group were demonstrated with a significant reduced length of intensive care unit stay (Median: 1.9 d νs. 4.1 d, p = 0.039), postoperative length of hospital stay (Median: 9.0 d νs. 17.0 d, p = 0.007) and in-hospital medical cost (Median: 69.5 × 1000CNY νs. 113.6× 1000CNY, p = 0.041). As for the postoperative complications, the occurrence of re-intubation, re-exploration, in-hospital mortality, arrhythmia and renal replacement therapy was similar between groups. However, pulmonary complications (p = 0.049) were with a significantly lower rate in the early extubation group. In addition, fresh frozen plasma (p = 0.041) transfusion volume were significantly reduced in the early extubation group rather than packed red blood cells and platelets. CONCLUSIONS: Early extubation following complete repair of pulmonary atresia improved clinical outcomes and reduced in-hospital medical cost without increasing any postoperative complications.

Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden.

BACKGROUND: Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years. METHODS: A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group). RESULTS: The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group (P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010. CONCLUSION: The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.

The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions.

BACKGROUND: The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. METHODS: The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. RESULTS: We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations. CONCLUSIONS: The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.

Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants.

BACKGROUND: The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. METHODS: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before 90 days of age were identified and enrolled in this retrospective study. The cohort was divided into two groups: group-SR, who underwent initial palliation with staged repair (n = 50), and group-PR who underwent primary repair (n = 15). RESULTS: There were three post-palliation in-hospital mortalities, four inter-stage mortalities, and one post-repair in-hospital mortality in group-SR. In group-PR, there was one in-hospital death and one late death. Five-year survival rates were comparable between the two groups (group-SR: 83.6%; group-PR: 86.7%; p = 0.754). During the median follow-up duration of 44.7 months (Inter-quartile range, 19-109 months), 40 post-repair re-interventions (22 in group-SR, 18 in group-PR) were performed in 26 patients (18 in group-SR, 8 in group-PR). On Cox proportional hazards model, primary repair was identified as the only risk factor for decreased time to death/1st post-repair re-intervention (Hazard ratio (HR): 2.3, p = 0.049) and death/2nd post-repair re-intervention (HR 2.91, p = 0.033). CONCLUSIONS: A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.

Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand.

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005-2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0-13.8) years. The patients' PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

Neonatal Pulmonary Atresia With Intact Ventricular Septum-8-Year Surgical Experience at One Center.

BACKGROUND: Surgical treatment of pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is challenging because of the broad variations of right ventricular (RV) malformations. In this retrospective study, we summarized our 8-y experience in surgical management for neonatal PA/IVS patients. METHODS: Thirty-four neonates with PA/IVS between July 1, 2006 and June 30, 2014, were reviewed. Patients were categorized into three groups: mild, moderate, and severe RV hypoplasia according to RV morphology and development. Patients were on regular follow-up for at least 5 y. Overall survival, complications, reinterventions, risk factors for mortality, and health status were evaluated. RESULTS: 21 patients (61.8%) were treated with biventricular repair, eight patients (23.5%) with Fontan procedure, and one patient (2.9%) with bidirectional Glenn procedure. There were four postprocedural mortalities and one late death. The 5-y survival rates after final surgical repair for mild, moderate, and severe RV hypoplasia groups were 100%, 100%, and 88.9%, respectively. The reintervention rates were 0% (0/4), 21.4% (3/14), and 55.6% (5/9) for the subgroups, respectively. At the latest follow-up, most patients had a status characterized as New York Heart Association class I (88.9%, 24/27). CONCLUSIONS: Surgical management for PA/IVS in neonates should be individualized. Favorable early and long-term outcomes can be achieved in neonatal PA/IVS patients by individualized surgical strategies, regardless of the degree of RV hypoplasia. In spite of potential RV catch-up development, the degree of RV hypoplasia is a factor of paramount importance to assess PA/IVS in neonates.

Surgical Management of Fallot's Tetralogy With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries: Multistage Versus One-Stage Repair.

A strict and rational approach to Fallot's tetralogy with pulmonary atresia and major aortopulmonary collateral arteries allows to achieve optimal results. Rehabilitative and unifocalization strategies do not constitute separate philosophies; instead the surgical strategy should be tailored to each individual patient. Based on our previous experience, the ability to achieve definitive intracardiac repair is the real determinant of both improved survival and adequate systolic right ventricular performance on mid-term follow-up.

Right Ventricle-Dependent Coronary Circulation: Location of Obstruction Is Associated With Survival.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium. METHODS: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC. RESULTS: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217). CONCLUSIONS: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC.

Midline one-stage complete unifocalization early outcomes from a single center.

OBJECTIVE: This study aims to present our experience with single-stage complete unifocalization and intraoperative flow study for the repair of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This study was conducted through retrospective chart review of all the patients who underwent complete single-stage midline unifocalization in a single tertiary-care institution. RESULTS: Twenty-two patients underwent midline single-stage unifocalization. The median age was 11 months (IQR: 5-21 months). The number of collateral arteries unifocalized was between one and three (median two). In-hospital mortality was 5%. Follow-up was complete; and the median follow-up regarding survival was 20 months (IQR: 10-28 months). There were three late deaths, and the estimated survival rate was 80% at 10 months and on. Out of 22 patients, ventricular septal defect was closed in the first surgery in three patients (14%) and the second surgery in four patients (19%). Total seven patients underwent surgical total repair (32%). Additionally, one out of four patients whose ventricular septal defects were closed with a fenestrated patch is under follow-up with a small ventricular septal defect, while two are waiting for ventricular septal defect closure. Therefore, total eight patients (36%) have reached total correction. CONCLUSION: Single-stage unifocalization is a feasible treatment option in ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. This cohort had unfavorable results regarding the rate of complete repair. The pitfalls encountered were related to problems with meticulous surgical technique, complete unifocalization, and correct implementation of the flow study.

Long-term outcomes in adults with repaired tetralogy of Fallot and pulmonary atresia.

BACKGROUND: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia. METHODS: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic. RESULTS: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031). CONCLUSION: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.

Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

BACKGROUND: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited. METHODS: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies. RESULTS: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I2 = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I2 = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia. CONCLUSIONS: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX.

Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals.

BACKGROUND: Our institutional approach to tetralogy of Fallot with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR and mid-term outcomes have not been assessed. METHODS: Clinical data were reviewed. PA diameters were measured offline from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions. RESULTS: From November 2001 to March 2018, 352 patients with tetralogy of Fallot/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age, 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median, 8 months) after APW. There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (range, 0.22 to 0.74). CONCLUSIONS: Most patients with tetralogy of Fallot/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.

Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum.

OBJECTIVE: Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy. METHODS: Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network. RESULTS: Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6-22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3-1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052). CONCLUSIONS: Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.

Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum.

BACKGROUND: Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) or duct-dependent pulmonary valve stenosis (DD-PVS) has become a reasonable alternative to surgical right ventricle decompression. AIM: To investigate mid-term outcomes following pulmonary valvuloplasty. METHODS: Sixty-five neonates with PA-IVS (n=29) or DD-PVS (n=36) (median age 4 days; mean weight 3.0kg) undergoing pulmonary valvuloplasty were reviewed retrospectively. Procedural data and clinical outcomes were assessed. RESULTS: Pulmonary valvuloplasty was successful in 59 patients (90.8%). Preterm birth, larger tricuspid valve annulus diameter and PA-IVS correlated with procedural failure. Eleven patients (18.6%) required a Blalock-Taussig shunt during early follow-up, despite valvuloplasty. These neonates had smaller tricuspid and pulmonary valve annulus Z-scores (-1.9 vs. -0.8 [p=0.04] and -2.5 vs. -0.9 [P=0.005], respectively) and a higher incidence of "bipartite" right ventricle (P=0.02). Mean follow-up was 5.4±3.3 years. Mortality after successful valvuloplasty was 8.5% (n=5). Among the 54 survivors, biventricular repair was achieved in 52 patients (96.3%), including nine with a previous Blalock-Taussig shunt. The cumulative rate of subsequent surgery (excluding Blalock-Taussig shunt) was 13.7% (95% confidence interval 6.8-26.7%) and 16.4% (95% confidence interval 8.5-30.4%) at 2 and 4 years, respectively. Secondary surgery was significantly more frequent in PA-IVS compared with DD-PVS, and in neonates with a Blalock-Taussig shunt (P=0.003 and 0.01, respectively). CONCLUSIONS: Selected neonates with DD-PVS or PA-IVS managed by transcatheter pulmonary valvuloplasty had a good mid-term outcome. In neonates with a borderline small right ventricle, a hybrid strategy with a supplementary source of pulmonary blood flow can be efficient to achieve biventricular repair.

Simultaneous Systemic to Pulmonary Shunt and Pulmonary Artery Banding is a Viable Option for Neonatal Palliation of Single Ventricle Physiology.

A subset of neonates with single ventricle (SV) physiology has antegrade pulmonary blood flow that is deemed unlikely to be reliable until Glenn. We have used systemic to pulmonary shunt (SPS) with pulmonary artery banding (PAB) to optimize pulmonary blood flow while maintaining reserve antegrade flow. We hypothesize that this is an effective strategy that can be accomplished without the routine need for cardiopulmonary bypass. We retrospectively reviewed the records of 60 neonates who underwent combined SPS + PAB between 2004 and 2015. Data are presented as median with quartiles. Children were 8 (4-19) days old at surgery and included 38 (63%) boys. Atresia or severe stenosis of the subpulmonary atrioventricular (AV) valve associated with pulmonary blood flow across a bulboventricular foramen was present in 37 (62%). In 20 (33%), heterotaxy-associated unbalanced AV canal with pulmonary stenosis with or without anomalous pulmonary venous drainage was present. First-stage palliation was accomplished without cardiopulmonary bypass in 44 patients (73%). There were 7 (12%) hospital deaths, 4 among the 20 (20%) with heterotaxy. Fifty-three children were followed for a median 5.1 (1.8-8.2) years. Three early reinterventions were required after initial palliation (1 PAB adjustment, 2 SPS balloon angioplasties). Five additional heterotaxy patients experienced late mortality during follow-up. There were no early or emergent Glenn. Thirty-nine patients have reached Fontan circulation with a median pre-Fontan PA pressure of 14 (12-18) mm Hg. One patient converted to biventricular physiology and the remaining await completion Fontan. Heterotaxy was the only independent predictor of mortality (hazard ratio 10 (2.3-44, P < 0.001). In SV patients with unreliable antegrade PA flow, SPS + PAB is an effective first-stage palliation. SV patients with heterotaxy are at increased risk for mortality.

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ±â€¯8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Analysis of achieving an "ideal" outcome following midline unifocalization.

OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex form of congenital heart disease. Midline unifocalization has been developed for the surgical treatment of this condition. There are 3 outcome measures that determine long-term success: patients are alive, patients have achieved complete repair (i.e. ventricular septal defect closure), and patients have a relatively low right ventricle-to-aortic pressure ratio (<0.45). However, to date, no studies have combined these 3 outcome measures to analyze the likelihood of achieving an ideal outcome. METHODS: This was a retrospective review of 255 patients who underwent midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The median age at unifocalization was 4.5 months. RESULTS: Two hundred thirty-five (92%) patients were alive at a mean follow-up of 5.3 years. Two hundred and seventeen (85%) patients underwent single-stage complete repair, and 38 (15%) had an initial unifocalization and shunt. Twenty-four of the 38 palliated patients have subsequently undergone repair. Thus 241 (94%) patients ultimately achieved complete repair. Of the 241 patients who were repaired, 219 (86%) had a right ventricle-to-aortic peak systolic pressure ratio <0.45. Combining these outcome measures, 77% of patients achieved an ideal outcome at one month, 73% at 6 months, 76% at one year, and 80% at 4 years. CONCLUSIONS: Most (80%) patients can achieve all 3 measures of favorable outcome at 4 years following midline unifocalization. We speculate that this will be a favorable portend for the future of these patients.

Myocardial strain abnormalities in fetuses with pulmonary atresia and intact ventricular septum.

OBJECTIVES: Global and regional myocardial deformation have not been well described in fetuses with pulmonary atresia and intact ventricular septum (PA/IVS). Speckle-tracking echocardiography (STE), an angle-independent technique for assessing global and regional strain, may be a more sensitive way of determining ventricular systolic dysfunction compared with traditional 2D echocardiography. The aim of this study was to assess myocardial deformation in fetuses with PA/IVS compared with control fetuses and to determine if, in fetuses with PA/IVS, strain differs between those with and those without right ventricle-dependent coronary circulation (RVDCC). METHODS: This was a retrospective analysis of fetuses with PA/IVS examined at two medical centers between June 2005 and October 2017. Left ventricular (LV) and right ventricular (RV) regional and global longitudinal strain (GLS) and strain rate were obtained using STE, and comparisons were made between fetuses with PA/IVS and gestational age (GA)-matched controls. Postnatal outcome was assessed, including the presence of RVDCC. RESULTS: Fifty-seven fetuses with PA/IVS and 57 controls were analyzed at a mean GA of 26.5 ± 5 weeks. LV-GLS was significantly decreased in fetuses with PA/IVS compared with controls (-17.4 ± 1.7% vs -23.7 ± 2.0%, P < 0.001). LV strain rate was also significantly decreased (-1.01 ± 0.21/s vs -1.42 ± 0.20/s, P < 0.001). Fetuses with PA/IVS had decreased strain in all segments. Similarly, RV strain was significantly decreased in fetuses with PA/IVS (-11.6 ± 3.8% vs -24.6 ± 2.5%, P < 0.0001). Thirty-six patients had postnatal cardiac catheterization performed to define coronary anatomy; 10 fetuses had RVDCC. Fetuses with RVDCC had decreased LV strain compared with those without (-15.8 ± 1.2% vs -17.9 ± 1.7%, P = 0.009). RV strain was also decreased in fetuses with RVDCC vs those without (-7.0 ± 2.9% vs -12.1 ± 3.2%, P = 0.0004). CONCLUSIONS: Fetuses with PA/IVS have decreased global and regional LV and RV strain compared with controls. The finding of decreased LV strain may be due to altered ventricular mechanics in the context of a hypertensive right ventricle and/or abnormal coronary perfusion. Moreover, fetuses that were found to have RVDCC postnatally had decreased LV and RV strain compared with those that did not. These results encourage further investigation to assess whether fetal ventricular strain could be a prenatal predictor of RVDCC. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch.

BACKGROUND: The prognosis of tetralogy of Fallot with absent pulmonary valve (TOF/APV) without operation is poor. We evaluated the surgical outcome of TOF/APV in a single center. METHODS: Twenty-two TOF/APV patients underwent complete surgical correction in our hospital. Right ventricular outflow tract reconstruction was performed using bovine jugular vein (BJV)-valved conduit implantation (n = 10), homograft-valved conduit implantation (n = 2), or monocusp-valve patch (n = 10). Health-related quality of life (QOL) was evaluated during follow-up. RESULTS: The overall survival at 5 and 10 years was 86.4 ± 7.3% (confidence interval 69.4-97.2%). The survival rates were significantly different between patients with and without bronchial stenosis (40 and 100%, P = 0.0003, log-rank test). The survival of patients aged > 6 months was higher than those ≤ 6 months (100 vs. 40%, P = 0.0003, log-rank test). Patients with BJV-valved conduits had higher systolic gradients from the right ventricle to the pulmonary artery (RV-PA) compared to those with monocusp-valve patches. BJV-valved conduit implantation was a risk factor for post-operative pulmonary-valve stenosis. The QOL score for patients with BJV-valved conduits was lower than those with monocusp-valve patches (P < 0.05). No reoperation was performed during follow-up. CONCLUSIONS: Bronchial stenosis and lower age (≤ 6 months) were the main factors influencing post-operative survival. The use of a BJV-valved conduit was a main reason for RV-PA restenosis; thus, the use of a BJV-valved conduit may increase the need for repeat intervention and decrease the post-operative quality of life.

Midterm Echocardiographic Assessment of Right Ventricular Function After Midline Unifocalization.

BACKGROUND: Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function. METHODS: Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study-aided repair of PA/VSD/MAPCAs. For patients who underwent either concomitant or delayed intracardiac repair, echocardiographic RV systolic function was retrospectively calculated to assess (1) RV fractional area change (RVFAC) and (2) two-dimensional RV longitudinal strain (RVLS) of the free wall of the right ventricle. QLAB cardiac analysis software version 10.3 (Philips Medical Systems, Andover, MA) was used for analysis. RESULTS: A total of 64 patients underwent concomitant VSD closure at the time of unifocalization, and 16 patients underwent delayed VSD closure at a median of 2.3 years (range: 3 days to 7.4 years). At a median follow-up of 8.1 years (range: 0.1 to 19.5 years) for the concomitant repair group versus 7.4 years (range: 0.01 to 15.3 years) for the delayed repair group, no differences in RVFAC and RVLS were observed (RVFAC: 41.0% ± 6.2% versus 41.2% ± 7.6%, p = 0.91; RVLS: -18.7 ± 4.3 versus -18.9 ± 4.0, p = 0.87). CONCLUSIONS: Patients (83%) with PA/VSD/MAPCAs underwent complete repair at intermediate-term follow-up with preserved RV function. Delayed VSD closure was accomplished in 50% of the patients initially deemed unsuitable for repair. Delayed VSD closure did not affect survival and did not portend impaired RV systolic function.