Single Ventricular Physiology Research Articles

Long-Term Outcomes of the Non-fenestrated Strategy for Extracardiac Total Cavopulmonary Connection

BackgroundThe discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy. MethodsBetween August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy. Primary outcomes were Fontan-related events, including death, reoperation, catheter intervention, and postoperative complications such as arrhythmias, protein-losing enteropathy, plastic bronchitis, thrombosis, bleeding, and drainage of pleural effusion after discharge. ResultsThe median follow-up duration was 15.7 years (interquartile range, 15.3–18.4). The 1-, 2-, 3-, 5-, 10-, and 15-year occurrence of Fontan-related events was 24%, 43%, 44%, 55%, 67%, and 76%, respectively. The 1-, 2-, 3-, 5-, and 10-year occurrence of venovenous collaterals was 16%, 60%, 65%, 72%, and 81%, respectively. Ventricular end-diastolic pressure was higher in patients with venovenous collaterals (median, 5.0 mm Hg; interquartile range, 4.0-7.0 mm Hg) than in those without (median, 3.5 mm Hg; interquartile range, 3.0-4.25 mm Hg) (P = .01). Multivariable Cox regression analysis showed that higher ventricular end-diastolic pressure and younger age at Fontan completion significantly increased the risk of developing venovenous collaterals, with hazard ratios of 1.22 for each (95% CI, 1.052–1.41; P = .0085 and .016, respectively). ConclusionsThe occurrence rate of Fontan-related events was acceptable with the non-fenestrated Fontan strategy, whereby venovenous collateral development was common. The results suggest that patients with high ventricle end-diastolic pressure and young patients might benefit from fenestration.

Intravenous infusion of cardiac progenitor cells in animal models of single ventricular physiology.

The goal of this study was to identify the practical applications of intravenous cell therapy for single-ventricle physiology (SVP) by establishing experimental SVP models. An SVP with a three-stage palliation was constructed in an acute swine model without cardiopulmonary bypass. A modified Blalock-Taussig (MBT) shunt was created using an aortopulmonary shunt with the superior and inferior venae cavae (SVC and IVC, respectively) connected to the left atrium (n = 10). A bidirectional cavopulmonary shunt (BCPS) was constructed using a graft between the IVC and the left atrium with an SVC cavopulmonary connection (n = 10). The SVC and the IVC were connected to the pulmonary artery to establish a total cavopulmonary connection (TCPC, n = 10). The survival times of half of the animal models were studied. The other half and the biventricular sham control (n = 5) were injected intravenously with cardiosphere-derived cells (CDCs), and the cardiac retention of CDCs was assessed after 2 h. All SVP models died within 20 h. Perioperative mortality was higher in the BCPS group because of lower oxygen saturation (P < 0.001). Cardiac retention of intravenously delivered CDCs, as detected by magnetic resonance imaging and histologic analysis, was significantly higher in the modified Blalock-Taussig and BCPS groups than in the TCPC group (P < 0.01). Without the total right heart exclusion, stage-specific SVP models can be functionally constructed in pigs with stable outcomes. Intravenous CDC injections may be applicable in patients with SVP before TCPC completion, given that the initial lung trafficking is efficiently bypassed and sufficient systemic blood flow is supplied from the single ventricle.

Aortic arch repair with extended end-to-side anastomosis in neonates and infants with transverse arch hypoplasia

Background/Aim: The use of patches to repair the aortic arch is believed to have a positive effect on long-term morbidity. In this study, perioperative and follow-up data of patients who underwent transverse arch repair with a patch were compared with the data of patients who underwent end-to-end anastomosis (ESA). Methods: In this retrospective cohort study, the data of 27 patients (including 18 newborns) who underwent aortic arch repair at the Gazi Yasargil Education and Research Hospital between January 2018 and April 2023 were analyzed. The inclusion criteria included a diagnosis of proximal and distal transverse aortic arch hypoplasia, an age younger than 12 months of age, and the completion of aortic arch repair using cardiopulmonary bypass. Patients who underwent recoarctation repair due to residual obstruction, patients with single ventricular physiology, and patients who underwent aortic arch repair via a lateral thoracotomy without undergoing cardiopulmonary bypass were excluded from the study. The patients were divided into two groups. Group 1 included individuals who underwent aortic anterior wall expansion with autologous pericardium in addition to ESA; Group 2 included patients who underwent ESA only. Results: The median age of the patients was 21 days (range: 6–365 days), and the median body weight of the cohort was 3.5 kilograms (range: 2.4–8.9 kilograms). Enlargement with autologous pericardial patch was applied to 11 patients (40.7%). Surgical procedures performed in addition to arch repair included eight ventricular septal defect closures, six instances of pulmonary banding, three atrial septal defect closures, and one subvalvular pulmonary stenosis repair. The in-hospital mortality rate was 11.1% (n=3). Those three patients died due to sepsis. The median follow-up period was 152 days (range: 10–1316 days). Recoarctation requiring re-intervention did not occur in any of the studied patients. The antegrade selective cerebral perfusion time was statistically significantly longer in patients who underwent aortic arch repair using a patch (P=0.03). Conclusion: Repair of the arch with a patch may contribute to a reduction in long-term mortality and morbidity. However, there is a need for more comprehensive and long-term follow-up studies to verify these findings.

Growth and risk of adverse neuro-developmental outcome in newborns with congenital heart disease: A single-centre retrospective study

BackgroundMortality in infants with CHD is decreasing and focus has shifted to improving long-term outcomes. Growth and neurodevelopmental outcomes are important long-term endpoints for both clinicians and parents. ObjectiveTo evaluate growth and determine the impact of growth on neurodevelopmental outcomes at one year of age in infants who had an operative or therapeutic catheter procedure for CHD in neonatal period. MethodsThis was a single-centre retrospective cohort study of infants born at term gestation with CHD. Demographic details, measurements of growth, and scores of Bayley Scales of Infant and Toddler Assessment (third edition), were collected. Enrolled study participants were categorised in subgroups based on the procedures required prior to one-year assessment. Regression analysis was performed to explore predictive ability of anthropometric measurements on mean scores of developmental assessment. ResultsA total of 184 infants were included in the study. Mean z-scores for weight and head circumference at birth were age-appropriate. Mean scores for various developmental domains ranged from borderline to normal except for infants with single ventricular physiology who had gross motor delay concomitant with growth failure. In this group, z-score of weight at one-year-assessment predicted mean score of cognition (p-value 0.02), fine motor (p-value 0.03) and gross motor nearly (p-value 0.06). ConclusionsInfants born at term gestation, with CHD, without genetic diagnosis had normal fetal growth. Postnatal growth restriction and developmental delay was most marked in infants with single ventricle physiology, suggesting careful nutritional and developmental monitoring.

Clinical Applications of Pressure-Volume Assessment in Congenital Heart Disease.

Ventricular pressure-volume (PV) loops offer unique insights into cardiovascular mechanics. PV loops can be instrumental in improving our understanding of various congenital heart diseases, including single ventricular physiology, heart failure, and pulmonary hypertension, as well as guiding therapeutic interventions. This review focuses on the theoretical and practical foundations for the acquisition and interpretation of PV loops in congenital heart disease and discusses their clinical applications.

Extracorporeal Cardiopulmonary Resuscitation in Infants: Outcomes and Predictors of Mortality

Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

The Relationship Between Placental Pathology and Neurodevelopmental Outcomes in Complex Congenital Heart Disease.

Complex congenital heart disease (CCHD) is associated with impaired neurodevelopmental outcomes. Peri- and post-operative factors are known contributors while the impact of the prenatal environment is not yet delineated. Variations in fetal circulation, seen in transposition of the great arteries (TGA) and single ventricular physiology (SVP), are associated with placenta abnormalities. These abnormalities may be associated with placental insufficiency, a risk factor for poor neurodevelopmental outcomes. We hypothesized there is a correlation between placental pathology and impaired neurodevelopmental outcomes in patients with CCHD. We performed a single center retrospective cohort study with patients with TGA and SVP from 2010 to 2017 at Children's Wisconsin. Patient variables were obtained from the medical record. Bayley Scales of Infant Development Third Edition standard scores for cognitive, motor, and language performance were collected from neurodevelopmental visits. Placenta pathology reports were reviewed with tabulation of predetermined anatomical and pathological characteristics. We identified 79 patients in our cohort and 61 (77.2%) had abnormal placentas. There was no significant difference between the two groups in any demographic or clinical variables. For cognitive and motor performance, without adjusting for the covariates, infants with placental abnormalities had significantly lower scores compared to infants without (p = 0.026, p = 0.045 respectively). Conversely, there was no significant difference in language scores between the two groups (p = 0.12). Placenta abnormalities are common in patients with CCHD, and placenta abnormalities are associated with impaired neurodevelopmental outcomes. These results underscore the complex causal pathways of neurodevelopmental impairment in infants with CCHD and offer opportunities for targeted postnatal developmental interventions after discharge.

Hypoxic Gas Therapy in Neonatology: Considerations in Practice.

Adequate oxygenation is essential for sick newborns. Each disease determines the target of oxygenation. Nevertheless, hyperoxia and hypoxia are related to adverse outcomes. Most studies related to this aspect have been conducted in preterm infants or term babies with pulmonary pathology. Congenital heart diseases may also require careful oxygenation control and management of oxygen supply. Presurgical stabilization of complex heart diseases (CHD) may be difficult, especially after the physiological decrease of pulmonary resistance, which generates pulmonary edema (due to overcirculation) and systemic hypoperfusion. Several strategies have been described to avoid this phenomenon, such as prostaglandin, vasodilators, inotropes, positive airway pressure, and even hypoxic mixture (inspired fraction of oxygen (FiO2) below 21%). The latter therapy is mainly used in single ventricular physiology heart diseases, such as the hypoplasic left heart syndrome (HLHS) or systemic ductus-dependent flow CHD (interruption of the aortic arch and coarctation of the aorta). Alveolar oxygen affects pulmonary vascular resistance modifying lung flow. This modification could help the stabilization during the presurgical period of complex CDH. Many centers use hypoxic therapy to avoid hypotension, metabolic acidosis, coronarycerebral ischemia, and liver, renal and intestinal injury. Despite the theoretical benefits, there are doubts about how tissue oxygen supply would change during hypoxic gas ventilation. It is worrisome that FiO2 < 21% causes a decrease in brain oxygenation, adding neurological injury as a complication to the already established disease of CHD and other not modifiable factors. Brain monitoring through near-infrared spectroscopy (NIRS) during hypoxic gas therapy is mandatory. Recent studies have shown that hypoxic gas ventilation therapy in patients with HLHS in the preoperative period decreases the ratio between systemic and pulmonary circulation (Qp/Qs) but does not improve regional oxygenation delivery. The use of hypoxic gas ventilation therapy continues to be controversial. It could be an option in some complex CHD, mainly HLHS.

Laparoscopic Cholecystectomy in a Patient with Glenn Shunt-Aided with Erector Spine Block.

The bidirectional (BD) Glenn shunt or hemi-Fontan procedure has been used to temporarily improve cardiac function in patients with severe structural heart disease which feature single ventricular physiology. Subsequently, more of these patients present for noncardiac surgical interventions, which present as an anaesthetic challenge. Erector spinae block (ESP) is an effective analgesic modality used in various thoraco abdominal surgeries, which can also be given safely in such patients. A case of a 17-year-old female patient with complex congenital heart disease (CHD) who underwent BD Glenn shunting with main pulmonary artery ligation in childhood, presently admitted for laparoscopic cholecystectomy done under general anaesthesia. With detailed preoperative workups, close haemodynamic monitoring and use of ESP for analgesia during perioperative period, the patient had successful outcome. We outline the anaesthetic management and the concerns of laparoscopy in such patients. A comprehensive understanding of physiology of Glenn's shunt and implications of the proposed surgical procedure is necessary to plan the anaesthetic and analgesic agents, cardiovascular drugs and ventilation strategies in patients with CHD with shunt procedure done.

The effect of hypocapnia on systemic perfusion in patients with single ventricle after surgery

The objective:the aim of the study was to identify the relationship between arterial hypocapnia and systemic hypoperfusion in newborns with single ventricular physiology after hemodynamic correction of congenital heart disease. Subjects and methods.125 newborns with congenital heart defects operated from 2014 to 2018 were examined retrospectively. Arterial and central venous blood gases were collected in the postoperative period. A total of 670 pairs of laboratory results were selected.Results.Based on the presence/absence of hypocapnia (PaCO2 less than 35 mm Hg), 2 groups were formed. Group G-0 (the hypocapnic variant of the single-ventricular circulation) comprised 44 observations. Group G-1 (PaCO2 more than 35 mm Hg) included 40 observations. In 32 (38%) cases the level of systemic perfusion was within the normal range, in 52 (62%) cases, systemic hypoperfusion was detected. In samples corresponding to Group G-1, signs of DOS were observed in 20 cases. The study showed that the most pronounced intergroup difference in parametric data was observed among indicators reflecting oxygen consumption and, as a consequence, the system flow rate (РO2 in mixed venous blood, saturation in mixed venous blood, arterio-venous difference in saturation, O2 content in venous blood, O2 extraction ratio, arterio-venous difference in РCO2). In addition, the HF markers such as arterio-venous difference in saturation, O2 extraction ratio, arterio-venous difference in РCO2 had a strong correlation with the signs of systemic hypoperfusion. In the hypocapnic group, the tendency for more pronounced desaturation of venous blood was determined, and a higher arterio-venous difference in saturation, O2 content in venous blood, O2 extraction ratio, and arterio-venous difference in РCO2 parameters were also noted.Conclusions.Arterial hypocapnia may be a sign of pulmonary overflow and reduction of systemic blood flow in newborns with single ventricular physiology, after hemodynamic correction of congenital heart disease. When managing newborns with parallel circulation, hypocapnia should be avoided as a factor contributing to the redistribution of blood flow from left to right and the development of systemic hypoperfusion.

O045 / #673: EVALUATION OF THE PERFORMANCE OF VASOACTIVE VENTILATION RENAL SCORE IN PREDICTING THE DURATION OF MECHANICAL VENTILATION AND INTENSIVE CARE HOSPITALIZATION AFTER PEDIATRIC CARDIAC SURGERY

Aims & Objectives: Various methods have been used to evaluate the recovery process after cardiac surgery in children. In here, we aimed to evaluate the vasoactive ventilation renal (VVR) score. Methods: Patients who underwent congenital heart surgery between November 2016 - March 2019 were evaluated prospectively. VVR score was calculated as follows: VIS + ventilation index + (change in serum creatinine level based on baseline value X 10). The VVR score, VIS and serum lactate levels of all patients were recorded at the time of admission, the highest value in the first 24 hours and 48th hour. The relationship between the duration of mechanical ventilation monitoring and ICU stay was evaluated by ROC analysis and threshold values were calculated. Results: The median age of the 94 patients included in the study was 1.1 (range 0.2-17) year, and 12 patients had single ventricular physiology. In the time period corresponding to each study point, the VVR score was higher in area under the ROC curve (AUC) compared to VIS and serum lactate levels, and the 48th hour VVR score was highest in the ROC analysis (AUC 0.851, 95% confidence interval). 0.761-0942 / ICU hospital stay; AUC 0.946, 95% confidence interval 0.885-1 / mechanical ventilation time). The 48th hour VVR score was 9.1 (sensitivity 87%, specificity 97.1%) for the duration of mechanical ventilation, and 6.7 (sensitivity 70%, specificity 92%) for ICU stay. Conclusions: The VVR score is a new and effective predictor of the duration of mechanical ventilation and ICU stay in postoperative congenital heart surgery patients.

Risk Factors for Mid-Term Liver Disease After the Fontan Procedure.

The Fontan procedure is a palliative surgery performed for patients with complex congenital heart disease who exhibit functional single ventricular physiology. Although clinical outcomes of the Fontan procedure have improved in recent years and most patients who undergo the procedure reach adulthood, Fontan-associated liver disease (FALD) is a noncardiovascular complication that has become increasingly common; its risk factors remain unknown.A total of 95 patients who underwent the Fontan procedure and who were followed up for at least three years at Gunma Children's Medical Center and Kitasato University Hospital between 1996 and 2015 were retrospectively enrolled in this study.The mean age of the patients at the time of Fontan procedure was 2.3 ± 1.4 years. Overall, 21 patients (23.1%) experienced FALD. All Fontan procedures were performed with extracardiac total cavopulmonary connection using 16-mm expanded polytetrafluoroethylene grafts. The presence of systemic right ventricle, requirement of pulmonary vasodilator, application of a non-fenestrated Fontan procedure, and absence of fenestration flow at the time of follow-up catheter examination were identified as predictors of FALD using univariate analysis. All these factors, except the requirement of pulmonary vasodilator, remained significant predictors of FALD in multivariate logistic regression analysis.Patients with a systemic right ventricle who undergo the Fontan procedure are at a high risk of FALD in the mid-term. Creating fenestration at the time of Fontan and maintaining the fenestration flow may reduce the mid-term risk of FALD.

In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease

IntroductionRisk factors contributing to heightened thrombosis in pediatric congenital heart disease (CHD) patients are not fully understood. Among the neonatal CHD population, those presenting with single ventricular physiology are at the highest risk for perioperative thrombosis. The von Willebrand factor and ADAMTS13 interactions have emerged as causative risk factors for pediatric stroke and could contribute to heightened thrombosis in CHD neonates. MethodsThis study investigates a cohort of children with single ventricle physiology and undergoing cardiac surgery, during which some patients developed thrombosis. In this cohort, we analyzed the relationship of several molecular features of ADAMTS13 with the plasma and activity levels in patients at risk of thrombosis. Additionally, in light of the natural antithrombotic activity of ADAMTS13, we have sequenced the ADAMTS13 gene for each patient and evaluated the role of genetic variants in determining the plasma ADAMTS13 levels using a series of in silico tools including Hidden Markov Models, EVmutation, and Rosetta. ResultsLower ADAMTS13 levels were found in patients that developed thrombosis. A novel in silico analysis to assess haplotype effect of co-occurring variants identified alterations in relative surface area and solvation energy as important contributors. Our analysis suggested that beneficial or deleterious effect of a variant can be reasonably predicted by comprehensive analysis of in silico assessment and in vitro and/or in vivo data. ConclusionFindings from this study add to our understanding the role of genetic features of ADAMTS13 in patients at high risk of thrombosis related to an imbalanced relation between VWF and ADAMTS13.

Use of extracorporeal membrane oxygenation in postcardiotomy pediatric patients: parameters affecting survival

We aimed to investigate the risk factors affecting survival after extracorporeal membrane oxygenation use in pediatric postcardiotomy patients. One hundred thirty-three consecutive patients who underwent surgery for congenital heart disease who needed extracorporeal membrane oxygenation support were retrospectively analyzed. In all, 3,082 patients were operated, of which 140 patients (4.54% of the total number of operations) needed extracorporeal membrane oxygenation. Eighty (60.1%) patients were successfully weaned and 51 (38.3%) patients were discharged. Of the 50 patients discharged during the mean follow-up period of 34.8 (0-192.4) months, 6 (12%) patients died. The extracorporeal membrane oxygenation support was instituted in 29 (21.8%) patients for extracorporeal membrane oxygenation cardiopulmonary resuscitation, in 44 (33.1%) patients due to the inability to be separated from cardiopulmonary bypass, in 19 (14.3%) patients due to respiratory failure, and in 41 patients due to low cardiac output syndrome. Eighty patients (60.2%) were successfully weaned from extracorporeal membrane oxygenation support. The remaining 53 (39.8%) patients died on extracorporeal membrane oxygenation. Mortality was observed in 29 (21.8%) of the 80 patients in the successful weaning group, while the remaining 51 (38.3%) patients were discharged from the hospital. Multivariate analysis showed that double-ventricular physiology increased the rate of successful weaning (odds ratio: 3.4, 95% confidence interval lower: 1.5 and upper: 8, p = 0.004) and prolonged extracorporeal membrane oxygenation durations were a risk factor in successful weaning (odds ratio: 0.9, 95% confidence interval lower: 0.8 and upper: 0.9, p = 0.007). The parameters affecting mortality were the presence of syndrome (odds ratio: 3.8, 95% confidence interval lower: 1.0 and upper: 14.9, p = 0.05), single-ventricular physiology (odds ratio: 5.3, 95% confidence interval lower: 1.8 and upper: 15.3, p = 0.002), and the need for a second extracorporeal membrane oxygenation (odds ratio: 12.9, 95% confidence interval lower: 1.6 and upper: 104.2, p = 0.02). While 1-year survival was 15.2% and 3-year survival was 12.1% in patients with single-ventricular physiology, the respective survival rates were 43.9% and 40.8%. Parameters affecting mortality after extracorporeal membrane oxygenation support in pediatric postcardiotomy patient group were the presence of a syndrome, multiple runs of extracorporeal membrane oxygenation, and single-ventricular physiology. Timing of extracorporeal membrane oxygenation initiation, appropriate patient selection, appropriate reintervention or reoperation for patients with correctable pathology, the use of an appropriate cannulation strategy in single-ventricle patients, management of shunt flow, and appropriate interventions to reduce the incidence of complications play key roles in improving survival.

Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound.

Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly important. At present, little is known about the potential mechanisms of cognitive dysfunction. In this cross-sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology. Ten adults with single ventricular physiology (age 18-40years) and 12 age- and gender-matched controls underwent transcranial Doppler ultrasound and accompanying cognitive assessment. Patients underwent neuropsychological testing that assessed differing cognitive domains, with subjective cognitive decline determined from a 24-question survey. Transcranial Doppler ultrasound was used to assess baseline cerebral blood flow as well as change in cerebral blood flow velocities from baseline and during cognitive testing. Age, ethnicity, individual, and parental education levels were considered in the multivariate analyses. On assessment of cognitive function, the patient group performed more poorly across each of the measured domains. The control group had a significantly greater increase in cerebral blood flow in response to cognitive stimuli compared to the patient cohort; these differences in response to cognitive stimuli were seen to a similar extent across each of the measured cognitive domains. Adults with Fontan physiology are underperforming in assessments of executive function with associated abnormalities in cerebral perfusion potentially contributing to cognitive deficits.

Evaluating the response to cardiac resynchronization therapy performed with a new ventricular morphology-based strategy for congenital heart disease.

In cardiac resynchronization therapy (CRT) for patients with congenital heart disease (CHD) and a ventricular morphology other than a systemic left ventricle (LV), we previously proposed pacing sites that are different from those used for a systemic LV. The leads should be placed laterally on opposite sides of both ventricles in patients with short-axis dyssynchrony and a single ventricular physiology with two ventricles, whereas they should be placed at the farthest sites along the longitudinal direction in the right ventricle (RV) in patients with long-axis dyssynchrony of the RV. Moreover, in patients with interventricular dyssynchrony and a biventricular physiology with a systemic RV, they should be placed at sites that both ventricles can contract simultaneously. We retrospectively investigated 27 consecutive procedures in 24 patients with CHD who underwent CRT to evaluate the effectiveness of a new ventricular morphology-based CRT strategy. The responder rate was 63% (17/27). The reasons for a non-response to CRT in 10 cases were as follows: non-optimal lead positions during CRT, 4; no systemic ventricular conduction delay or heart failure symptoms before the CRT, 5; short follow-up periods after the CRT, 2; and an extremely dilated systemic RV, 1. The responder rate became 88% (14/16), after excluding the procedures without a ventricular conduction delay or heart failure symptoms and those with non-optimal lead positions. This new strategy for CRT can provide favorable results for CHD patients with a systemic ventricular conduction delay and heart failure.

Single ventricle: Diagnosis and prognosis

Between October 2011 and June 2019 we detected 2787 new heart defects in fetuses in the Referral Centre for Perinatal Cardiology in Warsaw, Poland, Agatowa US Clinic. 393 had univentricular hearts and 144 critical aortic stenosis. The term: SINGLE VENTRICLE is a very wide definition. If the heart is “functionally univentricular” one ventricle is too small or its function is too poor to maintain systemic or pulmonary cardiac output. It is already known that cardiac lesions are dynamic. It is likely that in some circumstances growth of cardiovascular structures depends on the volume of flow. There are hearts which will always be univentricular. There are: tricuspid atresia (67 cases), double inlet left ventricle (31), mitral atresia (9), hypoplastic left heart syndrome (222), and more complicated cardiac defects in which only one ventricle is developed. In such babies final treatment is to obtain Fontan circulation. During prenatal period it is important to plan the perinatal management and to decide what kind of treatment will be necessary in the newborns. Stenosis or hypoplasia of the pulmonary artery or aorta can occur, so the lesion will be ductal dependent. In other - restriction of the foramen ovale can cause the neonatal problem. In rare cases neonates will not need any treatment, if both arteries are well developed. On the other hand there are “evolving lesions”, like critical aortic stenosis (evolving HLHS) or pulmonary atresia and intact ventricular septum. In both we suspect that outflow tract obstructions caused underdevelopment of the ventricle. So fetal opening of the left or right outflow tract should be beneficial. Basing on this theory fetal cardiac interventional program developed. My team performed 90 aortic balloon valvluoplasties in 84 fetuses and 12 pulmonary artery valvuloplasties in 11 fetuses. In the recent period we achieved 48% of biventricular circulation of live born babies with critical aortic stenosis and 90% in pulmonary atresia or critical pulmonary stenosis. Criteria for prenatal intervention are still under development. The main rule is that prenatal cardiac interventions should be performed only if predictive outcome without intervention will be worse than without it. It must be remembered that fetal valvuloplasty in the aortic stenosis is the first step of long and complicated postnatal treatment. In conclusion: univentricular heart can be successfully treated for life long single ventricular physiology. In some lesions natural history can be changed by prenatal cardiac intervention.

205: Predictors of adverse outcomes in infants with single ventricle physiology

We aimedto investigate factors that are associated with adverse outcomes in newborns with prenatally diagnosed functional single ventricular heart physiology. This is a retrospective case-control study of 336 pregnancies diagnosed with congenital heart disease (CHD) between 2011 and 2016. Groups consisted of newborns who survived beyond age of four (control), and those who encountered postnatal mortality prior to that (study). Data included maternal and fetal characteristics, and postnatal outcomes.A p value of <0.05 was considered statistically significant. We identified 53/336 (15.7%) pregnancies with single ventricle physiology during the study period. Most common anatomical defects contributing to the single ventricle physiology were: hypoplastic right heart (HRH) (45.2%), hypoplastic left heart syndrome (HLHS) (32 %), pulmonary atresia and intact interventricular septum (PA-IVS) (9.4 %), unbalanced atrioventricular septal defect (AVSD) (9.4%), and double inlet left ventricle (DILV) (3.7 %). Significant factors associated with poor adverse outcomes included: hypoplastic left heart (P= 0.006), and delivery at less than 37 weeks (P= 0.03). Significant postnatal predictors of poor outcomes included pre-operative vasopressors use (P= 0.03) and post-operative Extracorporeal Membrane Oxygenation (ECMO) (P= 0.0001). Hypoplastic right heart was significantly associated with better postnatal outcomes (p= 0.0001). We found higher rate of postnatal mortality among newborns with single ventricle physiology in pregnancies delivered prior to 37 weeks gestation.

The Effect of the Superior Cavopulmonary Anastomosis on Ventricular Remodeling in Infants with Single Ventricle

Infants with single ventricular physiology have volume and pressure overload that adversely affect ventricular mechanics. The impact of superior cavopulmonary anastomosis (SCPA) on single left ventricles versus single right ventricles is not known. As part of the Pediatric Heart Network placebo-controlled trial of enalapril in infants with single ventricularphysiology, echocardiograms were obtained before SCPA and at 14 months and analyzed in a core laboratory. Retrospective analysis of the following measurements included single ventricular end-diastolic volume (EDV), end-systolic volume (ESV), mass, mass-to-volume ratio (mass/volume), and ejection fraction. Qualitative assessment of atrioventricular valve regurgitation and assessment of diastolic function were also performed. A total of 156 participants underwent echocardiography at both time points. Before SCPA, mean ESV and mass Z scores were elevated (3.4±3.7 and 4.2±2.9, respectively) as were mean EDV and mass/volume Z scores (2.1±2.5 and 2.0±2.9, respectively). EDV, ESV, and mass decreased after SCPA, but mass/volume and the degree of atrioventricular valve regurgitation did not change. Subjects with morphologic left ventricles demonstrated greater reductions in ventricular volumes and mass than those with right ventricles (mean change in Zscore: left ventricular [LV] EDV, -1.9±2.1; right ventricular EDV, -0.7±2.5; LV ESV, -2.3±2.9; right ventricular ESV, -0.9±4.6; LV mass, -2.5±2.8; right ventricular mass, -1.3±2.6; P≤.03 for all). Approximately one third of patients whose diastolic function could be assessed had abnormalities at each time point. Decreases in ventricular size and mass occur in patients with single ventricle after SCPA, and the effect is greater in those with LV morphology. The remodeling process resulted in commensurate changes in ventricular mass and volume such that the mass/volume did not change significantly in response to the volume-unloading surgery.

Surgery for total anomalous pulmonary venous connection: primary sutureless repair vs. conventional repair.

Despite recent advances in surgical technique and perioperative care, the surgical correction of total anomalous pulmonary venous connection (TAPVC) remains a challenge. The major complication and the main cause of reoperation in TAPVC surgery are the occurrence of pulmonary venous obstruction (PVO). In the 1990s, sutureless repair was introduced as a technique to relieve PVO after TAPVC repair. Following the favorable outcomes for postoperative PVO, the indications for sutureless repair as a primary operation have been expanded to include infants who have preoperative PVO or those at risk of developing PVO after the repair of TAPVC. However, the indications of "prophylactic" primary sutureless repair still remain controversial. Recent studies have shown that normal-risk patients have excellent early and long-term outcomes and a low incidence of reoperation for postoperative PVO. Most patients who survived beyond 2years after TAPVC surgery were in NYHA class I and offered good outcomes. Although favorable early and mid-term outcomes of primary sutureless repair are reported, the long-term outcomes of this technique are still unclear. The influence of non-contractile pericardial tissue interposed between the PV vessel wall and LA myocardium on the atrial function is also unclear in patients who undergo sutureless repair. Another disadvantage of primary sutureless repair is potential bleeding from the gap between the confluence and pericardium into the posterior mediastinum or pleural cavity. Thus, it might be best for primary sutureless repair to be indicated for high-risk infants, such as those with TAPVC associated with single-ventricular physiology, mixed-type TAPVC, or small PV confluence.