Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review.

This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocardiogram. Comparisons between groups were performed with Mann-Whitney U-test and Fisher´s exact test. A systematic literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34 (11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no definitive golden standard treatment.

Pulmonary Atresia with Intact Ventricular Septum: Correlation of Preoperative Computed Tomography-Derived Parameters with Echocardiographic Tricuspid Valve Z-Score and Surgical Outcomes.

The role of preoperative cardiac computed tomography (CT) in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) remains unclear. This study was aimed to elaborate the role of preoperative CT-derived anatomical and functional findings in planning treatment strategies in neonates with PA-IVS. The presence of ventriculocoronary arterial connections was evaluated by CT. CT-derived ventricular volumetric parameters were compared and correlated with echocardiographic tricuspid valve (TV) z-score in 12 neonates with PA-IVS. Cardiac CT and echocardiographic findings were compared between definite surgical types (median follow-up, 4 years). Ventriculocoronary arterial connections were identified with CT in 58.3% of cases (7/12) and associated with higher incidence of Fontan procedure (42.9%, 3/7) and high mortality (28.6%, 2/7). The CT-derived and echocardiographic TV z-scores exhibited a high correlation (R = 0.924, p < 0.001). The CT-derived right ventricle (RV) volume and RV-left ventricle volume ratio also displayed high correlations (R = 0.875 and 0.867, respectively; p < 0.001) with echocardiographic TV z-score. More positive echocardiographic TV z-score, high CT-derived RV end-diastolic volume and RV-left ventricle volume ratio, and low CT-derived left ventricular end-diastolic volume were observed in biventricular surgery group (N = 2), compared to Fontan operation (N = 3) and 1.5 ventricular surgery (N = 3) groups, and mortality cases (N = 3). Preoperative CT-derived coronary artery anatomy and ventricular volumetric parameters may supplement treatment planning in neonates with PA-IVS especially when multifactorial decision including echocardiographic TV z-score is in a gray zone.W.

Percutaneous transient occlusion of the transtricuspid flow: a new method to evaluate the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with an intact ventricular septum is characterised by heterogeneity in right ventricle morphology and coronary anatomy. In some cases, the presence of ventriculocoronary connections may promote coronary artery stenosis or interruption, and aortic diastolic pressure may not be sufficient to drive coronary blood flow. This requires a correct evaluation (currently done by angiography) which depends on whether the patient can be offered decompression of the right ventricle. To date, there is no objective method to do so, so we designed a percutaneous, transitory technique with the purpose of occluding the transtricuspid anterograde flow. The manoeuverer was performed in a 25-day-old female with pulmonary atresia with intact ventricular septum, right ventricle at suprasystemic level, and selective coronarography was not conclusive, the anterior descendant with stenosis in its middle third and from this point, thinner with to-fro flow. Occlusion was performed with a balloon catheter. We re-evaluated the coronary flow and the normalised anterior descendant flow. We hope that with this new method, we can give a more accurate diagnosis and determine the cases in which the coronary circulation is truly not right ventricle dependent to offer a greater number of patients biventricular or 1.5 ventricular repairs and thereby improve their quality of life and survival, the ones that turn out to be right ventricular dependant; offer them an early reference for cardiac transplant or in case it is not available to consider univentricular palliation knowing that this probably would not reduce the risk of ischaemia and/or death over time.

Anaesthesia management of interventional cardiac catheterisation for pulmonary atresia with intact ventricular septum and critical pulmonary stenosis: a retrospective analysis.

OBJECTIVE: Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis usually have to undergo treatment in the neonatal period. Compared to traditional surgical intervention, catheter-based cardiac interventions may achieve similar or superior outcomes for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, there is limited literature on anaesthesia techniques, challenges, and risks associated with cardiac catheterisation in this population. METHODS: This article retrospectively analysed the clinical data of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis neonates who were treated with interventional cardiac catheterisation in our hospital from January 2015 to October 2022. Clinical outcomes considered were haemodynamic or pulse oxygen saturation instability, vasoactive requirements, prolonged intubation (>24 h postoperatively), and cardiovascular adverse events. RESULTS: A total of 63 patients met the inclusion criteria. All patients survived the intervention. Among the patients with critical pulmonary stenosis, 40 successfully received percutaneous balloon pulmonary valvuloplasty, while three patients received ductal stenting due to moderate right ventricular dysplasia at the same time. For patients with pulmonary atresia with intact ventricular septum, 17 of the 23 patients successfully underwent percutaneous pulmonary valve perforation and percutaneous balloon pulmonary valvuloplasty. Of these, five patients underwent ductal stenting due to unstable pulmonary blood flow. Three patients only underwent ductal stenting. In addition, three patients received hybrid therapy. CONCLUSIONS: There are various clinical techniques and risk challenges in the interventional cardiac catheterisation of neonatal pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, by mastering the physiological and pathophysiological characteristics of the disease, adequately preparing for the perioperative period, and predicting the procedure process and potential complications, anaesthesia and surgical risks can be effectively managed.

Cardiac magnetic resonance parameters associated with successful conversion from a single ventricular to a one-and-a-half or biventricular circulation in patients with a hypoplastic right ventricle.

BACKGROUND: Some patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.5V or 2V circulation. METHODS: In this single center retrospective study, subjects with PA/IVS or LDAVC and no conotruncal abnormalities were included if they had a 1 V circulation at the time of CMR followed by a surgical intervention intended to convert them to a 1.5 V or 2 V circulation. Conversion failure was defined as any of the following: (1) oxygen saturation < 90% at the most recent follow-up, (2) conversion back to a 1.5 V or 1 V circulation, or (3) death. RESULTS: In the PA/IVS cohort (n = 15, median age 1.32 years), 10 patients underwent surgical conversion to a 1.5 V circulation and 5 to a 2 V circulation. In the attempted 1.5 V group, there were 2 failures, and these cases had a lower RV mass (p = 0.04). In the attempted 2 V group, there was 1 failure, and no CMR parameters were significantly different compared to the successes. Among the successful 2 V group patients, the minimum RV end-diastolic volume (EDV) was 27 ml/m2. In the LDAVC cohort (n = 15, median age 1.0 years), 1 patient underwent surgical conversion to a 1.5 V circulation and 14 patients to a 2 V circulation. In the attempted 1.5 V group, the 1 conversion was a failure and had an RV EDV of 15 ml/m2. In the attempted 2 V group, there were 2 failures, and these cases had a smaller RV:LV stroke volume ratio (p = 0.05) and a lower RV ejection fraction (p = 0.05) compared to the successes. Among the successful 2 V group patients, the minimum RV EDV was 22 ml/m2. CONCLUSIONS: We identified multiple CMR parameters associated with successful conversion from 1 V circulation to 1.5 V or 2 V circulation in patients with PA/IVS and LDAVC. This information may improve patient selection for conversion procedures and encourage larger studies to better define the role of CMR.

Prediction of postnatal circulation in pulmonary atresia/critical stenosis with intact ventricular septum: systematic review and external validation of models.

OBJECTIVE: A favorable postnatal prognosis in cases of pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS) is generally equated with the possibility of achieving biventricular (BV) repair. Identification of fetuses that will have postnatal univentricular (UV) circulation is key for prenatal counseling, optimization of perinatal care and decision-making regarding fetal therapy. We aimed to evaluate the accuracy of published models for predicting postnatal circulation in PA/CS-IVS using a large internationally derived validation cohort. METHODS: This was a systematic review of published uni- and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28 weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver-operating-characteristics curves (AUCs) and proportion of cases with true vs predicted outcome were calculated. RESULTS: Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (n = 3), non-BV (n = 3), BV (n = 3), right-ventricle-dependent coronary circulation (n = 1) or tricuspid valve size at birth (n = 1)) and in terms of the included predictors (single parameters only (n = 6), multiparametric score (n = 4) or both (n = 1)), and were developed on small sample sizes (range, 15-38). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter Z-score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80-0.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models. CONCLUSIONS: Current prenatal models for the prediction of postnatal outcome in PA/CS-IVS are heterogeneous. Multiparametric models for predicting UV and non-BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non-BV postnatal circulation is certain. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

The Utility of CT Angiography in Neonates with Pulmonary Atresia with Intact Ventricular Septum and Concern for Right Ventricular Dependent Coronary Circulation: Case Series.

Up to one third of patients with pulmonary atresia with intact ventricular septum (PA-IVS) will have inadequate anterograde coronary blood flow and rely on fistulous connections from the right ventricle (RV) for myocardial perfusion, known as RV-dependent coronary circulation (RVDCC). Historically, identification of the extent of ventriculocoronary connections and coronary stenosis has required invasive imaging with cardiac catheterization and angiography. Cardiac computed tomography (CCT) potentially provides a less invasive imaging option for therapeutic planning in this group of patients. We describe six neonates with PA-IVS who underwent both CCT and cardiac catheterization at our institution prior to any surgical or transcatheter intervention between 2009 and 2019. Imaging was concerning for RVDCC in all six patients. The average tricuspid Z-score was - 4.19 (2.1 to - 5.34). Two patients underwent cardiac transplantation and two patients underwent ductal stenting. The overall mortality rate was 50%. CCT findings closely mirrored the findings of invasive cardiac catheterization and identified important morphological variations. The average radiation exposure (DLP) per CCT was (10.5 mGy cm, range 6-20). Technological improvements in CCT have enabled adequate visualization of coronary anomalies in children with comparable accuracy to cardiac catheterization, but considerably less radiation exposure. However, diagnosis of RVDCC requires direct right ventricular angiography. Therefore, the potential benefit of obtaining a CCT prior to catheterization for infants with PA-IVS is the ability to risk stratify, assist with procedural planning, and improve family counseling.

Type I and II pulmonary atresia with intact ventricular septum in infants: a 10-year experience in initial surgery at one center.

BACKGROUND: To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS). METHODS: 50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled. Children with Type I were divided into groups A (n = 25) and B (n = 25). Group A had received BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, whereas group B had undergone BT shunt combined with PDA ligation and pulmonary valve incision. Children with type II were divided into groups C (n = 25) and D (n = 25). Group C had received BT shunt combined with PDA ligation, right ventricular outflow tract (RVOT) incision and transannular patch. Group D had undergone BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation. The differences in mechanical ventilation time, length of ICU stay, mortality rate, tricuspid Z value, tricuspid regurgitation, oxygen saturation, pulmonary regurgitation, McGoon ratio, pulmonary artery transvalvular pressure, survival rate were compared between groups A and B, between groups C and D respectively. RESULTS: The ventilator assistance time and length of ICU stay were greater in group C than in group D (80.96 ± 8.42 h vs. 65.16 ± 4.85 h, P = 0.045; 222.00 ± 11.72 h vs. 162.48 ± 7.91 h, P = 0.048). The pulmonary artery transvalvular pressure was significantly higher in group A than in group B at 3, 6, 12, 24 and 36 months after surgery (64.86 ± 4.13 mmHg vs. 53.04 ± 5.64 mmHg, P = 0.045; 69.47 ± 1.93 mmHg vs. 55.95 ± 4.04 mmHg, P = 0.005; 80.16 ± 3.76 mmHg vs. 73.24 ± 2.34 mmHg, P = 0.035; 62.95 ± 5.64 mmHg vs. 48.47 ± 7.44 mmHg, P = 0.04; 53.69 ± 4.89 vs. 45.77 ± 3.26, P = 0.02). Furthermore, the tricuspid Z value was significantly greater in group B than in group A at 3 and 24 months after surgery (- (1.37 ± 0.04) vs. - (1.43 ± 0.06), P = 0.03; - (0.41 ± 0.06) vs. - (0.51 ± 0.11), P = 0.02). CONCLUSIONS: The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and the effect of BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch.

Pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries associated with hypoplastic left ventricle and severe mitral regurgitation.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Right ventricle-dependent coronary circulation diagnosed by non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum is a complex cyanotic congenital heart lesion with the potential for myocardial ischaemia due to the presence of coronary artery anomalies. We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy.

Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.

BACKGROUND: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. METHODS: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. RESULTS: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). CONCLUSIONS: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

Successful Total Management of Multi-Causative Sleep-Disordered Breathing Complicated with Patient with Adult Congenital Heart Disease.

Sleep-disordered breathing is one of the complications commonly seen in patients with adult congenital heart disease (ACHD) due to multiple causes including complex underlying cardiac defects, cardiomegaly, previous thoracotomies, obesity, scoliosis, and paralysis of the diaphragm. It is often hard to determine its main cause and predict the efficacy of each treatment in its management. We herein report a 30-year-old woman after biventricular repair of pulmonary atresia with intact ventricular septum diagnosed as sleep-related hypoventilation disorder. Simultaneous treatment targeting obesity, paralysis of the diaphragm, and cardiomegaly followed by respiratory muscle reinforcement through non-invasive ventilation resolved her sleep-related hypoventilation disorder. Such management for each factor responsible for the hypoventilation is expected to provide synergetic therapeutic efficacy and increase daily activity in a patient with ACHD.

Modified wire atrial septostomy with a reverse transseptal puncture in an infant: Case report.

We report a modified technique of wire atrial septostomy (WAS) with a reverse transseptal puncture (TSP) in an infant case of pulmonary atresia with intact ventricular septum. A radiofrequency (RF) wire was advanced to the septum through a 4 Fr pigtail catheter hooked on the left side of atrial septum and RF energy was applied while advancing the wire across the septum. Following that reverse TSP, WAS was performed to cut the septal tissue using a 0.010 microwire and RF wire. The atrial septum defect (ASD) was enlarged to a size of 15 mm. WAS with a reverse TSP could be a useful and safe method to enlarge ASD in infants with congenital heart diseases.

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome.

OBJECTIVES: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). METHODS: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks). RESULTS: 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p < 0.001). Tricuspid insufficiency (TI)-Vmax > 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax < 2.5 m/s, TV/MV ratio < cutoff) to a score, non-BV outcome was correctly predicted when > 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention. CONCLUSION: TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

Myocardial infarct late after Fontan-type surgery in pulmonary atresia and intact ventricular septum: a must-know complication! About a case report.

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

Left ventricular outflow tract obstruction in a patient with pulmonary atresia with intact ventricle septum following Fontan procedure: a rare complication.

Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators.

BACKGROUND: Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery. METHODS: We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital. RESULTS: We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure. CONCLUSION: Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

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.

Absent pulmonary valve or pulmonary atresia with intact ventricular septum: Which is it?

A fetal echocardiogram noted valvar pulmonary atresia vs critical pulmonary valve stenosis, confluent pulmonary arteries, patent ductus arteriosus, and an apical muscular ventricular septal defect vs coronary sinusoid. Postnatal echocardiogram documented an absent pulmonary valve, coronary artery fistula to the right ventricle, confluent pulmonary arteries, and a patent ductus arteriosus. Catheterization confirmed the postnatal echocardiographic findings except there was no antegrade flow from the right ventricle to the pulmonary arteries. Close evaluation of all imaging modalities revealed the final diagnosis of absent pulmonary valve with a double-chambered right ventricle with no egress from the right ventricle to pulmonary artery.

Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum.

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.