Cavopulmonary Shunt Research Articles

Integrating Novel Physiologic Data into Decision-Making in Congenital Heart Surgery.

Optimal decision-making to determine the type and timing of surgical intervention for various congenital heart disease (CHD) requires adequate understanding and interpretation of anatomic and physiologic data obtained from various imaging modalities. Cardiac magnetic resonance (CMR) has revolutionized the way we evaluate the anatomy and physiology of CHD. In addition to 2- and 3-dimensional anatomic data and volumetry, phase-contrast CMR allows quantitative measurements of cardiac output, pulmonary blood flow, pulmonary-to-systemic flow ratio, the amount of intracardiac shunt, valve regurgitation, and aortopulmonary collateral flows. This review article describes the utilization of CMR-derived flow data in surgical decision-making in three distinct subgroups: (1) patients with borderline left ventricle (LV) with emphasis on the ascending aortic flow and other physiologic parameters, (2) single ventricle patients who undergo bidirectional cavopulmonary shunt with emphasis on the impact of superior vena cava blood flow on postoperative physiology, and (3) patients with pulmonary atresia and major aortopulmonary collateral arteries with emphasis on the impact of total pulmonary blood flow and systemic-to-pulmonary flow ratio on clinical outcomes.

Narrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives.

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

Combined Norwood and cavopulmonary shunt as the first palliation in late presenters with hypoplastic left heart syndrome and single-ventricle lesions

ObjectiveA primary cavopulmonary shunt as a component of the initial Norwood palliation could be an option in patients with hypoplastic left heart syndrome and single-ventricle lesions. We present our initial experience with this approach in carefully selected patients with unrestricted pulmonary blood flow and low pulmonary vascular resistance. MethodsThe study included 16 patients; the mean age was 137.9 ± 84.2 days. All patients underwent a Norwood palliation consisting of atrial septectomy, Damus-Kaye-Stansel connection, and arch augmentation in addition to the cavopulmonary shunt as the initial palliation. ResultsThe mean preoperative pulmonary to systemic blood flow (Qp/Qs) ratio on room air (n = 9) and with 100% oxygen (n = 8) was 5.3 ± 3.2 and 8.6 ± 4.3, respectively. The mean pulmonary vascular resistance on room air (n = 10) and 100% oxygen (n = 9) was 4.8 ± 3.1 and 1.7 ± 0.97 WU/m2, respectively. Delayed chest closure was needed in 12 patients, and 6 patients required postoperative inhaled nitric oxide. One patient underwent takedown of the cavopulmonary shunt and construction of the right ventricle to pulmonary artery conduit after 1 month. The mean intensive care unit stay was 18.9 ± 15.4 days. There were 2 in-hospital deaths (48 hours and 8 days after surgery) and 2 postdischarge deaths (6 months and 2 years after hospital discharge). Seven patients have undergone the Fontan completion successfully, and 5 patients await further surgery. ConclusionsFirst-stage Norwood palliation with cavopulmonary shunt for patients with hypoplastic left heart syndrome or single-ventricle lesions is feasible in late presenters with low pulmonary vascular resistance.

Abstract 10996: Predictors of Surgical Reintervention Before Bidirectional Cavopulmonary Shunt in Children with Single Ventricle Physiology Who Underwent Main Pulmonary Artery Banding

Introduction: Unplanned intervention before stage 2 palliation (S2P) in patients with single ventricle (SV) lesions with dual unobstructed outflows palliated with pulmonary artery banding (PAB) is common. The factors contributing to intervention and its association with successfully undergoing S2P are unclear. Hypothesis: Early intervention is more common when PAB is placed at a younger age and with a lower initial PAB pressure gradient. Methods: Retrospective single center study of patients with SV lesions undergoing PAB between Jan 2000 and Dec 2020. Association with reintervention and successful S2P was modeled using exploratory cause-specific hazard regression. A multivariable model was developed adjusting for clinical and statistically relevant predictors. The cumulative proportion of patients undergoing intervention were summarized using a competing risk model. Results: A total of 77 patients - 53% female, 86% term and 12% syndromic - underwent PAB at median (IQR) 47 (24-66) days and 3.73 (3.2-4.5) kg. Within 18 months of PAB, 60 (78%) reached S2P, 9 (12%) died, 1 (1%) transplanted and 7 (9%) were alive without S2P. Intervention occurred in 18 (23%) prior to S2P (10/18) or death (4/18); 16 (21 %) occurred within 6 months of PAB. Intervention included PAB adjustment (n=7), conversion to DKS/BTT shunt (n=5), balloon atrial septostomy (n=4) and addition of a BTT shunt (n=2). Repeat intervention was required in 4 patients. Intervention was associated with genetic syndrome (p= 0.03) and lower weight at PAB (p=0.02) but not with age at PAB, pre/postoperative oxygen saturation, PAB length or gradient. Presence of genetic syndrome was the only risk factor associated with not reaching S2P (p=0.02). Conclusions: Intervention is common following PAB and associated with presence of genetic disorder or smaller patient size. Children with genetic syndrome aiming to undergo S2P are at higher risk for intervention and death and may benefit from alternate treatment strategy.

Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report

BackgroundCOVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion.Case presentationWe report the first case of an adult with single-ventricle physiology and bidirectional cavopulmonary shunt who suffered from severe COVID-19 ARDS. Treatment strategies were successfully adopted, and pulmonary vascular resistance was reduced, both medically and through prone positioning, leading to a favorable outcome.ConclusionARDS treatment strategies including ventilatory settings, prone positioning therapy and cannulation techniques for extracorporeal oxygenation must be adopted carefully considering the passive venous return in patients with single-ventricle physiology.

Cyanosis in a Previously Well Child.

Cyanosis in a Previously Well Child.

Prominent Neck and Pinna Pulsations

A young man presented with visible neck and pinna pulsations after a palliative bidirectional cavopulmonary (Glenn) shunt with open antegrade flow. This resulted in giant C-V waves in the jugular venous pulse. Giant C-V waves resulting from a Glenn procedure have not previously been described and represent a novel entity to recognize. (Level of Difficulty: Advanced.)

Cohort study of intervened functionally univentricular heart in England and Wales (2000–2018)

ObjectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular...

VP22.23: Fetal complex rare cardiac malformation

A 27-year-old gravida 1 para 0 attended for anomaly scan with a singleton pregnancy. There were no significant events of interest nor consanguinity between both parents. Second-trimester ultrasound scan at 22+3 weeks showed a single live fetus in changing lie. Fetal Echo revealed: Situs solitus. The 4- chamber view showed univentricular heart, left handed ventricular topology, right sided main ventricle of left ventricular morphology, hypoplastic left sided (morphological right) ventricle, left sided AV valve (likely tricuspid valve) atresia, VSD (subaortic), AV & VA discordance - congenitally corrected transposition. Foramen ovale was largely restrictive with significant a-wave reversal. Aorta was located to right and anterior to pulmonary artery - D malposition. Aortic arch hypoplasia, mild preductal aortic coarctation, main pulmonary arterial dilatation, bilateral branch pulmonary artery origin stenosis was noted. No arrhythmias, no disturbances in the heart rate. Fetal cardiac function was normal. Amniocentesis and microarray offered but refused by patient. At 39+3 weeks of gestation a 3.05 kg male infant was born by spontaneous vaginal delivery, required PPV and CPAP at birth, due to poor respiratory effort then recovered. Postnatal echocardiography confirmed the diagnosis and on second day after birth the newborn underwent Norwood surgery with right modified Blalock-Taussig shunt and recovered uneventfully. The second stage surgery (Glenn superior cavo pulmonary shunt) and PA plasty was performed at 6 months of age and the patient recovered uneventfully. The patient will be followed in multidisciplinary fashion along with pediatric cardiac critical care and pediatric cardiology teams. He will proceed along the univentricular pathway, with a Fontan procedure (at a weight of 15 kg or greater) at 3 to 4 years of age, preceded by cardiac catheterisation. He should remain on aspirin (5 mg/kg once daily) until his Fontan (if feasible).

Primary central pulmonary artery plasty for right atrial isomerism with pulmonary coarctation.

Patients with unbalanced pulmonary artery growth resulting from pulmonary coarctation are considered unsuitable candidates for the Fontan procedure. Particularly, patients with right isomerism pose a challenge. We aimed to investigate the use of primary central pulmonary artery plasty at initial palliation in patients with right isomerism. We recruited 34 right isomerism patients with pulmonary atresia and pulmonary coarctation who underwent modified Blalock-Taussig shunt with or without primary central pulmonary artery plasty between 1998 and 2014. We classified them into the primary central pulmonary artery plasty (group P) and no primary central pulmonary artery plasty (group N) groups. We retrospectively analyzed reintervention for pulmonary artery after initial palliation, difference in size between the left and right pulmonary arteries, overall survival, success of the Fontan procedure. The group P and group N included 25 and 9 patients, respectively. Five (20%) and six (67%) patients in group P and group N, respectively, required reintervention for pulmonary artery after initial palliation (p = 0.017). No patient underwent reintervention for the pulmonary artery before bidirectional cavopulmonary shunt in group P. There was a significant difference in the bilateral pulmonary artery size balance between the groups before bidirectional cavopulmonary shunt (p = 0.041). The two-lung Fontan procedure was successful in 14 (56%) and 1 (11%) patient in group P and group N. Primary central pulmonary artery plasty may contribute toward improving the balance in the size of the PA and preclude the need for reintervention for PA.

Comparison of shunt types in the neonatal Norwood procedure for single ventricle.

The ideal shunt for pulmonary blood flow, modified Blalock-Taussig shunt (MBTS) or right ventricular-pulmonary artery conduit (RVPAC) is yet to be determined. This study aimed to evaluate outcomes after the Norwood procedure according to the type of shunt. A total of 322 neonates with hypoplastic left heart syndrome and related anomalies who underwent the Norwood procedure at our institution between 2001 and 2019 were divided into MBTS and RVPAC groups and the outcomes after the Norwood procedure were compared between the groups with respect to mortality after each staged procedure. We identified 322 consequent patients who underwent neonatal Norwood procedure for hypoplastic left heart syndrome (271 patients, 84.2%) and its variant (51 patients, 15.8%). RVPAC was performed in 163 (50.6%) patients and MBTS was performed in 159 (49.4%). There were no differences in the rate of early death (11.0% vs 12.6%, P = 0.69) or late death (7.4% vs 6.9%, P = 0.87) between the 2 groups after the Norwood procedure, and no significant difference in the number of patients who reached bidirectional cavopulmonary shunt (77.9% vs 76.1%, P = 0.69), and there was no difference in mortality after bidirectional cavopulmonary shunt (12.3% vs 7.5%, P = 0.15) or Fontan completion rate (54.0% vs 52.2%, P = 0.42) between the 2 groups. Survival at 0.5, 1, 3 and 6 years after the Norwood procedure was 81.0%, 73.8%, 67.9% and 67.0% in patients with RVPAC and 77.1%, 73.3%, 69.1% and 67.9% in patients with MBTS. There was no significant difference in the survival between the 2 groups during the median follow-up of 2.6 (interquartile ranges: 0.3-8.4, maximal 18.8) years (P = 0.97). In neonates undergoing the Norwood procedure, our available data of maximal 18.8 years follow-up showed no significant difference in early mortality, inter-stage attritions, or overall survival, between MBTS and RVPAC.

Risk factors for the development of arrhythmias after the fontan surgery

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Fontan surgery or total cavopulmonary connection is a palliative procedure for complex congenital heart disease with univentricular physiology, without the possibility of biventricular surgical correction. This surgery has a high mortality rate (70%), with heart failure and arrhythmias being the main causes of death. Objective Identify the pre, intra and post-surgical factors that may predispose to arrhythmias in patients undergoing to Fontan surgery. Methods We conducted a retrospective review of the patients who underwent a total cavopulmonary connection from January 2003 to December 2018. We performed an analysis employing the Chi-square test to determine which variables were associated with the development of arrhythmias. Results During the mentioned period, 115 patients underwent to Fontan surgery, of which 51.3% were women, the average age at the time of surgery was 8 years. The most frequent diagnosis was tricuspid atresia in 44 patients (38.2%), followed by pulmonary atresia in 18 patients (15.7%) and double outlet right ventricle in 14 patients (12.2%). Regarding the type of the procedures performed, 93.9% (n = 108) were total extracardiac cavopulmonary connection and 6.1% (n = 7) were intracardiac. Also, 8.6% (n = 10) were taken to fenestration, the mean size of the fenestration was 7 mm (7 ± 3 mm) After Fontan surgery, there was a high incidence of arrhythmias which was 38% (n = 44). 22 patients (50%) presented them in the late postoperative period, being this, the most frequent time for the appearance of arrhythmias. We found that the intracardiac surgery presented an OR of 4.423 (95% CI 0.819 - 23.879, p> 0.05), being not statistically significant. Instead, the bidirectional cavopulmonary shunt previous to the Fontan surgery presented an OR of 0.255 (95% CI 0.081 - 0.806, p <0.05) and on the other hand the QRS duration > 120 ms was significantly associated with the appearance of arrythmias with an OR 2.99 (95% CI 1.2-4.2, p <0.05). Conclusions In patients undergoing to Fontan surgery, the previous bidirectional cavopulmonary bypass is a protective factor for the development of arrhythmias. And an QRS duration > 120 ms is a parameter that predicts the apparence of arrhythmias. Abstract Figure. QRS INTERVAL POST FONTAN SURGERY

Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome.

Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p < 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9 ± 1.3 to -9.7 ± 1.3 (p < 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC >35% and TAPSE Z-score > -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p < 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.

Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia

ObjectiveTricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS–Fontan interstage death/transplantation versus transition to Fontan in TA. MethodsA total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan. ResultsCPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation. ConclusionsThe mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation.

Impacts of stage 1 palliation and pre-Glenn pulmonary artery pressure on long-term outcomes after Fontan operation.

The present study was aiming to determine whether high mean pulmonary artery pressure before bidirectional cavopulmonary shunt is a risk factor for late adverse events in patients with low pulmonary artery pressure before total cavopulmonary connection (TCPC). We retrospectively reviewed the medical records of all patients undergoing both bidirectional cavopulmonary shunt and TCPC with available cardiac catheterization data. A total of 316 patients were included in this study. The patients were divided into 4 groups according to mean pulmonary pressure: those with pre-Glenn <16 mmHg and pre-Fontan <10 mmHg (Group LL, n = 124), those with pre-Glenn ≥16 mmHg and pre-Fontan <10 mmHg (Group HL, n = 61), those with pre-Glenn <16 mmHg and pre-Fontan ≥10 mmHg (Group LH, n = 66) and those with pre-Glenn ≥16 mmHg and pre-Fontan ≥10 mmHg (Group HH, n = 65). Group HL showed significantly higher rate of adverse events after TCPC than Group LL (P = 0.02). In univariate linear analysis, a history of atrial septectomy at stage 1 palliation was associated with low pre-Glenn mean pulmonary artery pressure (Coefficient B -1.38, 95% confidence interval -2.53 to -0.24; P = 0.02), while pulmonary artery banding was a significant risk factor for elevated pre-Fontan mean pulmonary artery pressure (Coefficient B 1.68, 95% confidence interval 0.81 to 2.56, P < 0.001). High mean pulmonary artery pressure before bidirectional cavopulmoary shunt (≥16mmHg) remains a significant risk factor for adverse events after TCPC even though mean pulmonary artery pressure decreased below 10 mmHg before TCPC.

Factors influencing length of intensive care unit stay following a bidirectional cavopulmonary shunt.

The goal of this study was to identify the risk factors for prolonged length of stay (LOS) in the intensive care unit (ICU) after a bidirectional cavopulmonary shunt (BCPS) procedure and its impact on the number of deaths. In total, 556 patients who underwent BCPS between January 1998 and December 2019 were included in the study. Eighteen patients died while in the ICU, and 35 died after discharge from the ICU. Reduced ventricular function was significantly associated with death during the ICU stay (P = 0.002). In patients who were discharged alive from the ICU, LOS in the ICU [hazard ratio (HR) 1.04, 95% confidence interval (CI) 1.02-1.06; P < 0.001] and a dominant right ventricle (HR 2.41, 95% CI 1.03-6.63; P = 0.04) were independent risk factors for death. Receiver operating characteristic analysis identified a cut-off value for length of ICU stay of 19 days. Mean pulmonary artery pressure (HR 1.03, 95% CI 1.01-1.05; P = 0.04) was a significant risk factor for a prolonged ICU stay. Prolonged LOS in the ICU with a cut-off value of 19 days after BCPS was a significant risk factor for mortality. High pulmonary artery pressure at BCPS was a significant risk factor for a prolonged ICU stay.

Risk Factors for Thrombus Formation at Stage 2 Palliation and Its Effect on Long-Term Outcome in Patients With Univentricular Heart

Thrombus formation is a feared complication following bidirectional cavopulmonary shunt (BCPS). We aimed to investigate the effect of thrombus formation on outcome. BCPS was performed in 525 patients at our center between 1998 and 2018. The impacts of thrombus formation on survival and probability of Fontan completion were analyzed, and risk factors for thrombus formation were examined. Thrombus formation occurred in 30 patients (5.7%). Compared with the remaining 495 patients, there was no significant difference in the median age at BCPS (4.9 vs 4.7 months; P = 0.587). However, unbalanced atrioventricular septal defects (17 vs 5%; P = 0.008) and preoperative ventricular dysfunction (23.3 vs 8%; P = 0.004) were more frequent in patients who developed a thrombus. Thrombolytic therapy was performed in all patients and surgical thrombus removal was required in 13 patients. In-hospital mortality was higher in patients with thrombus (30.0 vs 2.2%; P < 0.001). Of 505 hospital survivors, an estimated survival at 1 year after hospital discharge following BCPS was 84.4% (95% CI, 76.1-92.7%) in patients with thrombus and 96.8% (95% CI, 96.0-97.6%) in those without (P < 0.001). Cumulative incidence of Fontan completion at 3 years after BCPS was 52.8% (95% CI, 30.3-75.2%) in patients with thrombus and 90.1% (95% CI, 87.2-92.9%) in those without (P = 0.004). Higher left atrial pressure (OR = 1.165; P = 0.029) and longer cardiopulmonary bypass time (OR = 1.013, P = 0.001) at BCPS were independent risk factors for thrombus formation after BCPS. Thrombus formation after BCPS poses a significant risk for survival and Fontan completion. Preoperative higher left atrial pressure and longer cardiopulmonary bypass time are significant risk factors.

Glenn surgery: a safe procedure in the path of univentricular correction.

This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery. The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality. From 1980 to 2015, 204 Glenn surgeries were performed. The most common heart disease was tricuspid atresia IB (19.2%). In 48.1% of the cases, the procedure was performed with antegrade flow. A bilateral Glenn procedure was performed in 12.5% of the cases and 10.3% were carried out without using a cardiopulmonary bypass pump. Reported complications included infections, bleeding, arrhythmias, chylothorax, neurological alterations, and pleural effusion. The mortality rate was 2.9%. Glenn surgery is a palliative surgery with good results. It significantly improves patient quality of life over a long period until a total cavopulmonary shunt is performed. The complications observed are few, and the mortality rate is low. Therefore, it is a safe surgery that should be used for univentricular congenital heart disease.

Purpose-Built Transcatheter Cavopulmonary Anastomosis Device Requirements: Multi-Modality Imaging Evaluation

Background/purposePatients with a functional single ventricle undergo multiple, palliative open-heart surgeries. This includes a superior cavopulmonary anastomosis or bidirectional Glenn shunt. A less-invasive transcatheter approach may reduce morbidity. Methods/materialsWe analyzed pre-Glenn X-ray contrast angiography (XA), cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) studies. ResultsOver an eleven-year period (1/2007 – 6/2017), 139 Glenn surgeries were performed at our institution. The typical age range at surgery was 59 – 371 days (median = 163; IQR = 138 – 203). Eight-nine XA, ten CT, and ten CMR studies obtained from these patients were analyzed.Cephalad SVC measurements (millimeters) were 7.3 ± 1.7 (XA), 7.7 ± 1.6 (CT) and 6.9 ± 1.8 (CMR). RPA measurements were 7.3 ± 1.9 (XA), 7.4 ± 1.6 (CT) and 6.6 ± 1.9 (CMR). Potential device lengths were 10.9 ± 6 – 17.4 ± 6.4 (XA), 10.1 ± 2.1 – 17.7 ± 2.4 (CT) and 17.3 ± 4. - 23.7 ± 5.5 (CMR). SVC-RPA angle (degrees) was 132.9 ± 13.2 (CT) and 140 ± 10.2 (MRI).Image quality of all CT (100%), almost all XA (SVC 100%, RPA 99%), and most MRI (SVC 80%, RPA 90%) were deemed sufficient. Parametric modeling virtual fit device with 10 mm diameter and 20 – 25 mm length was ideal. ConclusionsIdeal transcatheter cavopulmonary shunt device for the typical patient would be 10 mm in diameter and 20–25 mm in length.

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow.

Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increased in group 1. There was a significant difference in the pre-Fontan and follow-up Nakata index between groups. There was a significant increase in the Nakata index in group 1 between the pre-Glenn and pre-Fontan assessments as well as the Nakata index between the pre-Fontan and midterm follow-up. There was no significant change in the Nakata index in group 2 between assessments. A pulsatile Glenn shunt is associated with better pulmonary artery growth which continues long after the additional pulsatile flow is eliminated. It is possible that the effects of anterograde pulmonary blood flow on pulmonary artery growth in early life continue long after the Fontan completion.