Completion Of Total Cavopulmonary Connection Research Articles

Serial assessment of right ventricular function and deformation in patients with hypoplastic left heart syndrome: a cardiovascular magnetic resonance study

Abstract Background In patients with hypoplastic left heart syndrome (HLHS), right ventricular (RV) dysfunction and failure is a major cause of adverse outcome. Most studies have assessed RV function and size during staged palliation but serial data about RV deformation and function after completion of the Fontan circulation are rare. We assessed RV function and deformation by analysing serial cardiovascular magnetic resonance (CMR) studies in a large cohort of HLHS patients after completion of the total cavopulmonary connection (TCPC). Methods CMR examinations of 105 HLHS patients (female: 31, male: 74) were retrospectively analysed. Short axis cine images were used to measure RVEF and end-diastolic myocardial mass indexed to body surface area (RVMMi). 2-dimensional CMR feature tracking (2D-CMR-FT) was performed using long-axis and short axis cine images to measure myocardial global longitudinal, circumferential and radial strain (GLS, GCS, GRS) values. Results From 105 patients, all had at least two CMR examinations after TCPC completion (mean age at TCPC: 2.7±0.8 years; mean interval between examinations 5.4±1.6 years). 36 patients had three examinations (mean interval between first and third scan 9.9±2.2 years). The first scan was performed at a mean age of 5.6±2.9 years. Global strain values and RVEF decreased from the first to the third examination with a significant decline in GLS from the first examination to the second examination (median and interquartile range (IQR): −18.6%, IQR −20.4 to −16.3 vs. −16.8%, IQR −19.2 to −14.7) and from the first to the third examination in 36 patients (−18.6%, IQR −20.8 to −16.1 vs. −15.9%, IQR −18.3 to −12.4) (all p-values <0.004). RVEF decreased significantly from the first to the third examination (55.9%, IQR 50.2–59.8 vs. 50.4%, IQR 45.0–57.6; p<0.002) and from the second to the third examination (55.1%, IQR 47.1–58.7 vs. 50.4%, IQR 45.0–57.6; p<0.0004). The decrease in RVEF between the first and second scan and the changes in GCS and GRS across the examinations did not reach statistical significance. Median RVMMi increased significantly between the first and second scan (47.4 g/m2, IQR 39.0–55.3 vs. 50.2 g/m2, IQR 42.6–61.7; p<0.0007) and slightly but not significantly between the first and third scan in patients with three examinations (47.4 g/m2, IQR 39.5–56.2 vs. 50.1 g/m2, IQR 45.3–60.4). Negative correlations between GCS and RVEF (r=−0.57 to −0.76; p all <0.0001) as well as between GLS and RVEF (r=−0.31 to −0.59; p all <0.002) at all three time points could be demonstrated. Conclusion Serial assessment of CMR studies in HLHS patients after TCPC completion demonstrates a significant reduction in global strain values and RVEF at mid-term follow-up. The significant reduction in GLS between the first two examinations with non-significant changes in RVEF suggest that 2D-CMR-FT might be a suitable technique for the detection of early myocardial dysfunction. Funding Acknowledgement Type of funding sources: None.

Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study.

The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile-third quartile) at the 3 examinations was 4.2 (3.3-6.1), 9.4 (6.1-11.4) and 14.6 (11.8-16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P < 0.05). There was a slight decrease in RVEF and LAS throughout the examinations, but this was not statistically significant. Correlations were found between RVEF and LAS (r = -0.23; P < 0.01). Both RVEF and LAS correlated with RVEDVi and RVESVi (r = -0.17 to 0.43; P < 0.05). Serial assessment of CMR studies in HLHS patients after total cavopulmonary connection completion demonstrate an increase in indexed RV volumes in older HLHS patients but only mild reduction in RVEF and LAS. The correlation of indexed RV volumes with RVEF and LAS together with the significant increase in RV volumes over time suggests that indexed RV volumes might be superior to RV functional markers to monitor the RV in HLHS patients.

Serial right ventricular assessment in patients with hypoplastic left heart syndrome patients (HLHS): a cardiovascular magnetic resonance study

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with hypoplastic left heart syndrome (HLHS) are at risk for right ventricular (RV) dysfunction over the course of the three-stage surgical palliation with the final step being the completion of the total cavopulmonary connection (TCPC). However, less is known about RV function during follow-up after TCPC completion. We assessed RV function by analysing serial cardiovascular magnetic resonance (CMR) studies in a large cohort of HLHS patients. Methods CMR studies from 95 HLHS patients (67 males) were retrospectively analysed. Short axis cine images were used to measure RV end systolic and end diastolic volumes and ejection fraction (RVEF). Oblique cine images showing the atria and right ventricle in a similar manner like a standard "4-chamber view" were used to measure tricuspid annular plane systolic excursion (TAPSE) and long axis strain (LAS). Results From the 95 patients, all had at least two and 32 patients had three CMR scans. The first scan was performed at a mean age of 4.9 ± 2.8 years, the second scan at a mean age of 9.3 ± 4 years and the third at a mean age of 14.3 ± 3.7 years. The mean values of RV end diastolic and end systolic volume indexed to body surface area (REDVi, RVESVi) as well as RV ejection fraction (RVEF) at the three time points were: 1) REDVi 92.6 ± 21.9 ml/m2, RVESVi 43 ± 15.1 ml/m2, RVEF 54.2 ± 7.1%; 2) REDVi 93.9 ± 25.6 ml/m2, RVESVi 44.6 ± 18.3 ml/m2, RVEF 53.6 ± 7.8%; 3) REDVi 110.9 ± 41.9 ml/m2, RVESVi 58.1 ± 35 ml/m2, RVEF 50.1 ± 10.1%. There was a statistically significant increase in RVEDVi and RVESVi from the first and the second scan to the third scan (p &amp;lt; 0.01). RVEF was lower at the time of the third scan compared to the first and second scan, but this difference was not statistically significant. TAPSE increased slightly from the first to the third scan (p &amp;lt; 0.05). There was no change in stroke volume and LAS from the first to the third scan. Strong correlations were found between RVEF and LAS as well as between RVEF and TAPSE (r = 0.49 and r=-0.50; p &amp;lt; 0.001, respectively). Conclusion Serial assessment of CMR studies in HLHS patients after TCPC completion could demonstrate an increase in indexed RV volumes over time, whereas RV stroke volume, RVEF and LAS largely remain stable.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

Background:Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs.Methods:In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included.Results:Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity.Conclusions:PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Aortopulmonary Collaterals in Single Ventricle Physiology: Variation in Understanding Occlusion Practice Among Interventional Cardiologists.

Although aortopulmonary collaterals (APCs) frequently develop in patients with single ventricle palliation, there is a lack of understanding of pathophysiology, natural history, and outcomes with no universal guidelines for management and interventional practice. We conducted a study to assess the views held by interventional congenital cardiologists regarding the hemodynamic impact of APCs in patients with single ventricle palliation, and their embolization practice. An electronic survey using the Pediatric Interventional Cardiology Symposium (PICS) mailing list was conducted between February and March 2019 with one reminder sent 2weeks after initial invitation for participation. Of the 142 interventional cardiologist respondents, 95 (66.9%) reside in North America and 47 (33.1%) worldwide. We elected to exclude the data from interventionalists outside North America in this analysis as it was not representative of worldwide practice. Hypoxemia was considered to be the most common trigger for development of APCs by 56 (58.9%) respondents. After completion of total cavopulmonary connection, 30 (31.6%) respondents reported the APC burden stays the same while 31 (32.6%) feel it decreases. In evaluating the burden of APC flow, only 4 (4.2%) reported measuring oxygen saturation at different pulmonary artery segments, 21 (22.1%) perform segmental aortic angiograms, and 18 (19%) perform selective bilateral subclavian artery angiograms. A majority of respondents, 71 (74.7%), occlude the feeder vessel at different locations, while 10 (10.5%) occlude only the origin of the vessel. Our study demonstrates significant variation in the understanding of the cause and prognosis of APCs in patients with single ventricle palliation. Furthermore, there is variation in the approach for diagnosis and management among interventional cardiologists. Further studies are required to improve understanding of APCs and develop universal management guidelines.

Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

Cardiovascular complications in patients with total cavopulmonary connection: A nationwide cohort study

BackgroundLong-term outcomes are not well established at the population level after completion of the total cavopulmonary connection (TCPC) among patients with functional univentricular hearts. PurposeTo evaluate the incidence of cardiovascular events after TCPC completion. MethodsFrom a validated population-based cohort, we identified 178 patients with TCPC circulation completed after January 1, 1995. We established a comparison cohort by frequency-matching patients 1:200 on sex, and month and year of birth to the general Danish population (n = 35,600 population controls). We started follow-up at date of TCPC completion for cases and, for controls, at the index date of their matched case. The risks of cardiovascular events were assessed using cumulative incidence rates and Poisson regression models adjusted for age, sex, and calendar year. ResultsThe median age at TCPC completion was 3.3 years (interquartile range 2.6 to 5.3 years). Over a median follow-up of 12.5 years, 10 (5.6%) TCPC patients died and 7 (4.5%) had a heart transplantation compared with a 0.2% mortality in the matched population. In TCPC patients, 15.7% had an arrhythmia (11.8% supraventricular tachycardia), 3.4% had a stroke (all ischemic), and 21.4% of TCPC patients initiated a combination of a diuretic and a renin-angiotensin system (RAS)-inhibitor. These rates were >50-fold higher than in the comparison cohort: 0.2% arrhythmia, 0.06% stroke, and 0.04% starting a diuretic and a RAS-inhibitor. ConclusionsPatients with TCPC circulation face a high risk of cardiovascular events during intermediate term follow-up.

Invited Commentary

In the Fontan circulation, variation in the negative intrathoracic pressure associated with normal respiration enhances venous return and pulmonary blood flow. This influence is now well known, with further recognition that phrenic nerve palsy leading to diaphragmatic paralysis is poorly tolerated in Fontan patients. It is rare for a Fontan patient with phrenic nerve palsy to be able to go home without surgical diaphragm plication. However, there is an awareness growing that despite fixing the diaphragm to prevent its paradoxical motion, Fontan patients with phrenic nerve palsy will have more restrictive ventilatory physiology and potentially worse hemodynamics, at least in the short term, than those with normal diaphragmatic function.1Ovroutski S. Alexi-Meskishvili V. Stiller B. et al.Paralysis of the phrenic nerve as a risk factor for suboptimal Fontan hemodynamics.Eur J Cardiothoracic Surg. 2005; 27: 561-565Crossref PubMed Scopus (30) Google Scholar Because a significant proportion of children with phrenic nerve palsy following congenital heart surgery will eventually recover diaphragm function, the logical questions are what happens to those paralyzed diaphragms in Fontan patients, and how they are coping later in life. The difficulty with answering these questions, however, is also a blessing: not many Fontan patients had paralyzed diaphragms. Thanks to Komori and colleagues2Komori M. Hoashi T. Shimada M. et al.Impact of phrenic nerve palsy on late Fontan circulation.Ann Thorac Surg. 2020; 109: 1897-1903Abstract Full Text Full Text PDF Scopus (1) Google Scholar from the highly respected group in Osaka, Japan, we are able to gain valuable insights in this issue of The Annals of Thoracic Surgery. In an elegant study that included spirometry, exercise testing, and cardiac catheterization, they delineated some of the long-term effects of phrenic nerve palsy in Fontan patients 10 years after total cavopulmonary connection completion. Despite the unavoidable imbalance of patient cohort to the control group (those who never had phrenic nerve palsy), their findings were quite interesting, and surprising: (1) nearly half of the patients with phrenic nerve palsy recovered diaphragmatic function and (2) despite worse restrictive ventilatory impairment, those with persistent diaphragmatic paralysis did not have deleterious hemodynamic outcomes or exercise capacity, even when compared with patients who never had diaphragm problems. These findings are of course contrary to what we have expected. Although it might not be surprising that half of the paralyzed diaphragms recovered, the discovery that Fontan patients with persistent phrenic nerve palsy have essentially equivalent hemodynamics and exercise tolerance (eg, anaerobic threshold and peak oxygen consumption) as those with normal diaphragm function went against conventional (and unsupported) supposition. One would expect that losing the valuable respiration driven flow benefits would significantly affect how they cope with the obligatory and unrelenting adversities of the Fontan physiology. Nonetheless, Komori and colleagues’ data2Komori M. Hoashi T. Shimada M. et al.Impact of phrenic nerve palsy on late Fontan circulation.Ann Thorac Surg. 2020; 109: 1897-1903Abstract Full Text Full Text PDF Scopus (1) Google Scholar suggest that they are indeed coping well. It is worth noting that instead of relying on one single test, such as spirometry alone, the authors were able to engender a more complete picture and gather accurate answers with concomitant cardiac catheterization and cardiopulmonary exercise testing. For complex physiologies such as the Fontan circulation, this study further validates the importance of multidisciplinary and multimodality follow-up. Impact of Phrenic Nerve Palsy on Late Fontan CirculationThe Annals of Thoracic SurgeryVol. 109Issue 6PreviewAlthough adverse effects of phrenic nerve palsy (PNP) on early Fontan circulation have been reported, detailed late impact remains unclear. Full-Text PDF

Predictors of Bidirectional Glenn Shunt Failure and Fontan Completion Rate in Patients with Single Ventricle

Background: The introduction of bidirectional Glenn (BDG) procedure in patients with functional single ventricles improved the clinical outcomes for all candidates undergoing total cavopulmonary connection (TCPC). We tried to evaluate the obstacles that prevent patients to achieve TCPC after BDG. Methods: All patients who underwent BDG at Leipzig Heart Centre from April 2003 to November 2013 were included in this study, except cases preceded by Norwood procedure. Results: 82 patients were included in this study. 59 patients underwent TCPC (72%), while 12 patients were waiting for TCPC (14.6%). There were two in-hospital deaths (2.4%) after BDG. In two patients (2.4%), completion of TCPC was not possible, while 6 patients were lost during follow-up (7.3%). One patient had his BDG taken down (1.3%). Therefore the survival rate till completion of Fontan was 72% with another 14.6% waiting for TCPC. A univariate analysis revealed that patients with unbalanced atrioventricular septal defect (AVSD), previous repair of total anomalous pulmonary venous connection (TAPVD), elevated mean pulmonary arterial pressure and long operation time were significant predictors of death, take-down, or failure to progress to Fontan Operation. Thrombosis was found to be the main mechanism for morbidity and mortality. Conclusion: The staged plan for single ventricle candidates provides excellent clinical results. The main risk factors for death, take-down, or failure to progress to Fontan Operation were elevated pulmonary arterial pressure, unbalanced AVSD, TAPVD and long operation time.

Approaches to Establish Extracardiac Total Cavopulmonary Connections in Animal Models-A Review.

Long-term survival of patients with a single ventricle palliated with a Fontan procedure is still limited. No curative treatment options are available. To investigate the pathophysiology and potential treatment options, such as mechanical circulatory support (MCS), appropriate large animal models are required. The aim of this review was to analyze all full-text manuscripts presenting approaches for an extracardiac total cavopulmonary connection (TCPC) animal model to identify the feasibility and limitations in the acute and chronic setting. A literature search was performed for full-text publications presenting large animal models with extracardiac TCPCs on Pubmed and Embase. Out of 454 reviewed papers, 23 manuscripts fulfilled the inclusion criteria. Surgical procedures were categorized and hemodynamic changes at the transition from the biventricular to the univentricular condition analyzed. Surgical procedures varied especially regarding coronary venous flow handling and anatomic shape of the TCPC. In most studies (n = 14), the main pulmonary artery was clamped and the coronary venous flow redirected by additional surgical interventions. Only in five reports, the caval veins were connected to the right pulmonary artery to create a true TCPC shape, whereas in all others (n = 18), the veins were connected to the main pulmonary artery. An elevated pulmonary vascular resistance was identified as a limiting hemodynamic factor for TCPC completion in healthy animals. A variety of acute TCPC animal models were successfully established with and without MCS, reflecting the most important hemodynamic features of a Fontan circulation; however, chronic animal models were not reported.

Cardiovascular anatomy in children with bidirectional Glenn anastomosis, regarding the transcatheter Fontan completion

Transcatheter stent-secured completion of total cavopulmonary connection (TCPC) after surgical preparations during the Glenn anastomosis procedure has been reported, but complications from this approach have precluded its clinical acceptance. To analyse cardiovascular morphology and dimensions in children with bidirectional Glenn anastomosis, regarding the optimal device design for transcatheter Fontan completion without special surgical "preconditionings". We retrospectively analysed 60thoracic computed tomography and magnetic resonance angiograms performed in patients with a median age of 4.1years (range: 1.8-17.1years). Additionally, we simulated TCPC completion using different intra-atrial stent-grafts in a three-dimensional model of the representative anatomy, and performed calculations to determine the optimal stent-graft dimensions, using measured distances. Two types of cardiovascular arrangement were identified: left atrium interposing between the right pulmonary artery (RPA) and inferior vena cava, with the right upper pulmonary vein (RUPV) orifice close to the intercaval axis (65%); and intercaval axis traversing only the right(-sided) atrial cavity, with the RUPV located posterior to the atrial wall (35%). In the total population, the shortest median RPA-to-atrial wall distance was 1.9mm (range: 0.6-13.8mm), while the mean intra-atrial distance along the intercaval axis was 50.1±11.2mm. Regardless of the arrangement, 83% of all patients required a deviation of at least 5.9±2.4mm (range: 1.2-12.7mm) of the stent-graft centre at the RUPV level anteriorly to the intercaval axis to avoid covering or compressing this vein. Fixing the anterior deviation of the curved stent-graft centre at 10mm significantly decreased the range of bend angle per every given RUPV-RPA distance. For both types of cardiovascular arrangement, after conventional bidirectional Glenn anastomosis, the intra-atrial curved stent-graft seemed most suitable for achieving uncomplicated TCPC completion percutaneously without previous surgical "preconditionings" in the majority of children. Experimental study is necessary to validate this conclusion.

Early to mid-term results after total cavopulmonary connection performed in the second decade of life.

Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.

Looks Do Matter! Aortic Arch Shape After Hypoplastic Left Heart Syndrome Palliation Correlates With Cavopulmonary Outcomes

Aortic arch reconstruction after hypoplastic left heart syndrome (HLHS) palliation can vary widely in shape and dimensions between patients. Arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch three-dimensional shape features with functional and short-term outcome data after total cavopulmonary connection (TCPC). Aortic arch shape models of 37 patients with HLHS (age, 2.89 ± 0.99 years) were reconstructed from magnetic resonance data before TCPC completion. A novel, validated statistical shape analysis method was used to compute a three-dimensional anatomic mean shape from the cohort and calculate the deformation vectors of the mean shape toward each patient's specific anatomy. From these deformations, three-dimensional shape features most related to ventricular ejection fraction, indexed end-diastolic volume, and superior cavopulmonary pressure were extracted by partial least-square regression analysis. Shape patterns relating to intensive care unit and hospital lengths of stay after TCPC were assessed. Distinct deformation patterns, which result in an acutely mismatched aortic root and ascending aorta, and a gothic-like transverse arch, correlated with increased indexed end-diastolic volume and higher superior cavopulmonary pressure but not with ejection fraction. Specific arch morphology with pronounced transverse arch and descending aorta mismatch also correlated with longer intensive care unit and hospital lengths of stay after TCPC completion. Independent of hemodynamically important arch obstruction, altered aortic morphology in HLHS patients appears to have important associations with higher superior cavopulmonary pressure and with short-term outcomes after TCPC completion as highlighted by statistical shape analysis, which could act as adjunct to risk assessment in HLHS.

Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection.

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP<17mmHg (acceptable) and >=17mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9±6.6years and mean follow-up time of 3.4±1.9years were included in this study. Mean PAP was 14.6±3.97 (range 8-22mmHg). Thirteen (68%) patients had PAP<17mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50% vs. 7.7% P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P<0.001). IVC-CI<21.8% can predict PAP>=17mmHg with 83% sensitivity and 100% specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg)=20.2097-0.1796×(IVC-CI), (r2=0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg)=0. 8675×PVP, (r 0.90, P<0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.

Does functional health status predict health-related quality of life in children after Fontan operation?

It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents. We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form. In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains. Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.

Cardiac MR-derived indices are stronger predictors of resource use and risk than jugular venous pressure, in paediatric patients with functionally single ventricles, prior to completion of total cavopulmonary connection (TCPC)

Methods Outcome data for all patients undergoing inter-stage pre-TCPC CMR between 2007-2014 was analysed. Predetermined outcome measures included survival, duration of peri-TCPC hospitalisation and TCPC fenestration. MRI was performed under general anaesthetic with simultaneous transduction of jugular venous pressure (JVP), as per our Unit protocol. CMR-derived data included volumes and function of single ventricle, through-plane phase contrast flow volumes, contrast-enhanced angiography and 3D SSFP images. Flow was measured in ascending aorta, SVC, IVC, pulmonary arteries and pulmonary veins bilaterally. Thus systemic to pulmonary (S-P) collateral flow, net PA flow and SVC:IVC flow was quantified. 3D images allowed quantitative scoring of systemic veins off-loading the SVC system into IVC : 0= no SVC-IVC offloading veins seen, 1= few, small, low calibre veins, 2= large, obvious veins.

Exercise capacity in children after total cavopulmonary connection: Lateral tunnel versus extracardiac conduit technique

In patients with univentricular heart disease, the total cavopulmonary connection (TCPC) is the preferred treatment. TCPC can be performed using the intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) technique. The purpose of the present study was to evaluate exercise capacity in contemporary TCPC patients and compare the results between the 2 techniques. A total of 101 TCPC patients (ILT, n=42; ECC, n=59; age, 12.2±2.6 years; age at TCPC completion, 3.2±1.1 years) underwent cardiopulmonary exercise testing. The patients were recruited prospectively from 5 tertiary referral centers. For the entire group, the mean peak oxygen uptake was 74%±14%, peak heart rate was 90%±8%, peak workload was 62%±13%, and slope of ventilation versus carbon dioxide elimination (VE/VCO2 slope) was 127%±30% of the predicted value. For the ILT and ECC groups, patient age, age at TCPC completion, body surface area, peak workload, and peak heart rate were comparable. The percentage of predicted peak oxygen uptake was lower in the ILT group (70%±12% vs 77%±15%; P=.040), and the percentage of predicted VE/VCO2 slope was greater in the ILT group (123%±36% vs 108%±14%; P=.015). In a subgroup analysis that excluded ILT patients with baffle leak, these differences were not statistically significant. Common exercise parameters were impaired in contemporary Fontan patients. Chronotropic incompetence was uncommon. The peak oxygen uptake and VE/VCO2 slope were less favorable in ILT patients, likely related to baffle leaks in some ILT patients. These results have shown that a reduced exercise capacity in Fontan patients remains an important issue in contemporary cohorts. The ECC had a more favorable exercise outcome at medium-term follow-up.

Nontraditional placement of an implantable cardioverter-defibrillator in a heterotaxy patient after the completion of total cavopulmonary connection

A 15-year-old boy with heterotaxy syndrome developed a prolonged QT interval and intractable torsade de pointes after the administration of sodium channel blockers for atrial tachyarrhythmia. Although this situation called for the placement of an implantable cardioverter-defibrillator, a conventional transvenous approach was not available since the patient had previously undergone a nonfenestrated extracardiac total cavopulmonary connection. We were urged to carry out the surgical placement of an epicardial lead for an implantable cardioverter-defibrillator using a single coil transvenous shock lead through re-do midline sternotomy. Here we describe the details of this nontraditional surgical procedure for the placement of a lead for an implantable cardioverter-defibrillator in a case without venous access into the heart.

Total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation†

Single ventricle with apicocaval juxtaposition (ACJ) is a rare, complex anomaly, in which the optimal position of the conduit for completion of total cavopulmonary connection (TCPC) is still controversial. The purpose of this study was to identify a preoperative method for optimal conduit position using the IVC anatomy and computational fluid dynamics (CFD). Twenty-four patients with ACJ (5.3 ± 5.7 years) who underwent TCPC were enrolled. A conduit was placed ipsilateral to the cardiac apex in each of 11 patients, of which 9 were intra-atrial and 2 extracardiac (group A) and, in a further 13 patients, extracardiac on the contralateral side (group B). As control, 10 patients with tricuspid atresia were also enrolled (group C). The location of the IVC in relation to the spine was evaluated from the frontal view of preoperative angiogram, using the following index: IVC-index = IVC width overlapping the vertebra/width of the vertebra × 100%. Energy loss was calculated by CFD simulation. IVC-index of group B was larger than groups A and C (45 ± 26 vs. 20 ± 21 and 28 ± 19%, P = 0.03). Postoperative catheterizations showed that, due to its curvature, conduit length in group B was significantly longer than the others (65 ± 12 vs. 36 ± 14 and 44 ± 10 mm, P < 0.001), although there was no statistical difference in central venous pressure or cardiac output. CFD studies revealed less energy loss in group A conduits compared with group B (1.6 ± 0.3 vs. 3.6 ± 0.6 mW, P = 0.05), although this did not appear to be clinically significant. Moreover, CFD simulation showed significant energy loss within the Fontan circulation when the conduit was either compressed or kinked: 4.9 and 18.2 mW respectively. In patients with ACJ, placement of a straighter and shorter conduit on the ventricular apical side provides better laminar blood flow with less energy loss. However, conduit compression and kinking are far more detrimental to the Fontan circulation. A preoperative IVC-index is pivotal for avoiding these factors and deciding the optimal conduit route.

Presurgical Evaluation of Fontan Connection Options for Patients With Apicocaval Juxtaposition Using Computational Fluid Dynamics

Apicocaval juxtaposition (ACJ) is a rare congenital heart defect associated with single ventricle physiology where optimal positioning of the Fontan conduit for completion of total cavopulmonary connection (TCPC) is still controversial. In ACJ, the cardiac apex is ipsilateral with the inferior vena cava (IVC), risking kinking and collapse of the Fontan conduit at the apex of the heart. The purpose of this study is to evaluate two viable routes for Fontan conduit connection in patients with ACJ, using computational fluid dynamics. Internal energy loss evaluations were used to determine contribution of conduit curvature to the energy efficiency of each cavopulmonary anastomosis configuration. This percentage of energy loss contribution was found to be greater in the case of a curved extracardiac conduit connection (44%, 4.1 mW) traveling behind the ventricular apex, connecting the IVC to the left pulmonary artery, than the straighter lateral tunnel conduit (6%, 1.4 mW) installed through the ventricular apex. In contrast, net energy loss across the anastomosis was significantly lower with extracardiac TCPC (9.3 mW) in comparison with lateral tunnel TCPC (23.2 mW), highlighting that a curved Fontan conduit is favorable provided that it is traded off for a superior cavopulmonary connection efficiency. Therefore, a relatively longer and curved Fontan conduit has been demonstrated to be a suitable connection option independent of anatomical situations.