Bidirectional Cavopulmonary Shunt Research Articles

Impact of veno-venous collaterals on outcome after the total cavopulmonary connection

ObjectiveTo evaluate the prevalence of veno-venous collaterals (VVCs) after total cavopulmonary connection (TCPC) and analyze their impact on outcomes. MethodsPatients undergoing TCPC between 1994 and 2022 were evaluated. VVCs were identified using angiograms of cardiac catheterizations and their impact on outcomes was analyzed. ResultsA total of 635 patients were included. Median age at TCPC was 2.3 (interquartile ranges (IQR): 1.8–3.3) years. The most frequent diagnosis was hypoplastic left heart syndrome in 173 (27.2%) patients. Prior bidirectional cavopulmonary shunt was performed in 586 (92.3%) patients at a median age of 5.3 (3.6–9.9) months. VVCs were found in 94 (14.8%) patients at a median of 2.8 (0.1–11.8) years postoperatively. The prevalence of VVCs was similar between the dominant right and left ventricle (14.7 vs. 14.9%, p = 0.967). Mean pulmonary artery pressure (16.2 vs. 16.0 mmHg, p = 0.902), left atrial pressure (5.5 vs. 5.7 mmHg, p = 0.480), transpulmonary gradient (4.0 vs. 3.8 mmHg, p = 0.554) and oxygen saturation (81.4 vs. 82.6%, p = 0.103) before TCPC were similar between patients with and without VVCs. The development of VVCs did not affect survival after TCPC (p = 0.161). Nevertheless, VVCs were a risk for the development of plastic bronchitis (PB, p < 0.001). Interventional closure of VVCs was performed in 60 (9.4%) patients at a median of 8.9 (0.6–15.1) years after TCPC, and improvement of oxygen saturation was observed in 66% of the patients. ConclusionsThe prevalence of VVCs after TCPC was 15%. VVCs had no impact on survival following TCPC but were associated with a high prevalence of PB.

Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%). The interventional closure of aortopulmonary collaterals was performed before stage II in 12 (4%) patients, before Fontan completion in 38 (13%), and afterwards in 39 (14%). Presence of aortopulmonary collaterals before stage II was not associated with the pulmonary artery index (129 vs. 150 mm2/m2, p = 0.176) at stage II. In contrast, aortopulmonary collaterals before the Fontan completion were associated with lower pulmonary artery index (154 vs. 172 mm2/m2, p = 0.005), and right pulmonary artery index (99 vs. 106 mm2/m2, p = 0.006). Patients who underwent interventional closure of aortopulmonary collaterals before total cavopulmonary connection had lower pulmonary artery index (141 vs. 169 mm2/m2, p < 0.001), lower right pulmonary artery index (93 vs. 106 mm2/m2, p = 0.007), and left pulmonary artery index (54 vs. 60 mm2/m2, p = 0.013) at Fontan completion. The presence of aortopulmonary collaterals did not influence pulmonary artery size by the time of stage II. However, presence of aortopulmonary collaterals was associated with under-developed pulmonary arteries at Fontan completion, especially in patients who needed interventional closure of aortopulmonary collaterals.

Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS)

Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, encompassing approximately 1%–3% of congenital heart diseases. Patients with PA-IVS have functional single-ventricle physiology. The ultimate possible outcomes for patients with PA-IVS are biventricular circulation, 1.5-ventricle or single-ventricle palliation, or cardiac transplantation. The bidirectional cavopulmonary shunt (BCPS) procedure directs flow from the superior vena cava into both the right and left pulmonary arteries, permitting flow to both lungs. The shunt is considered to be the second stage of palliation and is generally preparative for the third-stage Fontan procedure. The BCPS improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance.Case: A 10-month-old, 25-day-old boy, weight 9.93 kg, body length 72 cm, diagnosed with PA-IVS, restricted persistent foramen ovale (PFO), right ventricle hypoplastic, and turtuous patent ductus arteriosus (PDA), underwent BCPS, atrial septectomy, and PDA stent evacuation surgery. The patient underwent a PDA stenting and ballooning atrial septectomy (BAS) at 17 days of age at the cathlab. The patient was cyanotic with stable hemodynamics and a saturation of 72% preoperatively.Discussion: Preoperative fasting must be observed to maintain the patient's hydration state. Pulmonary blood flow and systemic blood flow must be balanced. An adequate analgetic can prevent pain stimuli that increase pulmonary vascular resistance. Drugs to reduce the afterload, such as milrinone, are needed. Mechanical ventilation was set to get PaCO2 between 40 and 45 mmHg. Maintain the normal heart beat, preload, and contractility to maintain cardiac output (CO) with saturation 80–85%. Wean from mechanical ventilation as soon as possible.Conclusion: Anesthetic management for BCPS in patients with single ventricles from the preoperative period, intraoperative period, and postoperative period. Understanding single ventricle phsiology is important in order to treat the patient.

Quantification of ventricular stress in univentricular hearts during early childhood using age-independent zlog-NT-proBNP

BackgroundChildren with univentricular hearts (UVH) undergo up to three palliative surgical procedures to achieve complete circulatory separation (Fontan circulation). As a marker of cardiac wall stress, NT-proBNP is a promising tool to assess systemic ventricular load in these patients. However, different reference intervals (RI) apply to each stage, as NT-proBNP is highly age-dependent. MethodsChildren undergoing systemic-to-pulmonary (SP) shunt placement (stage 1), bidirectional cavopulmonary shunt (BCPS, stage 2) or total cavopulmonary connection (TCPC, stage 3) between 2011 and 2021 with NT-proBNP measurement within 7 days before surgery were included. Furthermore, outpatients after TCPC with NT-proBNP measurement were enrolled. Biomarker levels were evaluated using its age-adjusted z-score (“zlog-NT-proBNP”; age-independent RI, −1.96 to +1.96), allowing comparison between different stages and revealing changes in systemic ventricular load independent of the marked physiological decline in RI with age. ResultsOverall, 289 children (227 before, 62 after TCPC) met the eligibility criteria. Median time between blood sampling and surgery (SP shunt/BCPS/TCPC) was 2 [1–3] days and 3.2 [2.0–4.5] years after TCPC. Age-adjusted zlog-NT-proBNP levels were 3.47 [2.79–3.93] in children with native UVH (before SP shunt), 3.10 [1.89–3.58] at stage 1 (before BCPS), 1.08 [0.51–1.88] at stage 2 (before TCPC), and 1.09 [0.72–1.75] at stage 3 (after TCPC/Fontan completion). Consequently, BCPS revealed the strongest decrease (median − 2.02 logarithmized standard deviations, p < 0.001). ConclusionsIn children with UVH undergoing staged Fontan palliation, zlog-NT-proBNP is a highly promising tool for course assessment of systemic ventricular load, independent of the age-related decline in physiological NT-proBNP concentration.

Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure.

To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection. Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis. A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8-2.7) years and 11.7 (10.7-13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2-5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13-15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events. Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.

Impact of calorie intake and weight gain after Norwood procedure on the outcome of stage II palliation.

This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt. A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed. Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< -3.0) at 2 months of age. Patients with weight-for-age-Z-score < -3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of -3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types. Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < -3) was a risk for survival after stage II.

Development of weight and height age z-score after total cavopulmonary connection.

We aimed to analyze somatic growth of patients after total cavopulmonary connection (TCPC) as well as to identify factors influencing postoperative catch-up growth. A total of 309 patients undergoing TCPC at 4 years old or less between 1994 and 2021 were included. Weight-for-age z-score (WAZ) and height-for-age-z-score (HAZ) at TCPC and at postoperative time between 1 to 3 years were calculated. Factors influencing somatic growth were analyzed. Most frequent diagnosis and initial palliation were hypoplastic left heart syndrome (HLHS) (34%) and the Norwood procedure (51%), respectively. Median age and weight at TCPC was 2.0 (IQR: 1.7-2.5) years and 11.3 (10.5-12.7) kg, respectively. Median 519 days after TCPC, significant increase in WAZ (-0.4 to -0.2, p<0.001) was observed, but not in HAZ (-0.6 to -0.6, p=0.38). Older age at TCPC (p<0.001, OR: 2.6) and HLHS (p=0.007, OR: 2.2) were risks for low WAZ after TCPC. Older age at TCPC (p=0.009. OR: 1.9) and previous Norwood procedure (p=0.021, OR: 2.0) were risks for low HAZ after TCPC. Previous bidirectional cavopulmonary shunt (BCPS) was a protective factor for both WAZ (p=0.012, OR: 0.06) and HAZ (p=0.028, OR: 0.30) at TCPC. In patients undergoing TCPC at the age of 4 years or less, a significant catch-up growth was observed in weight-for-age z-score after TCPC, but not in height-for-age-z-score. Previous BCPS resulted to be a protective factor for a better somatic development at TCPC. HLHS undergoing Norwood were considered as risks for somatic development after TCPC.

Impact of home monitoring program on interstage mortality after the Norwood procedure.

While early outcome after the Norwood operation for hypoplastic left heart syndrome has improved, interstage mortality until bidirectional cavopulmonary shunt (BCPS) remains a concern. Our aim was to institute a home monitoring program to (HMP) decrease interstage mortality. Among 264 patients who survived Norwood procedure and were discharged before BCPS, 80 patients were included in the HMP and compared to the remaining 184 patients regarding interstage mortality. In patients with HMP, events during the interstage period were evaluated. Interstage mortality was 8% (n = 21), and was significantly lower in patients with HMP (2.5%, n = 2), compared to those without (10.3%, n = 19, p = 0.031). Patients with interstage mortality had significantly lower birth weight (p < 0.001) compared to those without. Lower birth weight (p < 0.001), extra corporeal membrane oxygenation support (p = 0.002), and lack of HMP (p = 0.048) were risk factors for interstage mortality. Most frequent event during home monitoring was low saturation (<70%) in 14 patients (18%), followed by infection in 6 (7.5%), stagnated weight gain in 5 (6.3%), hypoxic shock in 3 (3.8%) and arrhythmias in 2 (2.5%). An unexpected readmission was needed in 24 patients (30%). In those patients, age (p = 0.001) and weight at BCPS (p = 0.007) were significantly lower compared to those without readmission, but the survival after BCPS was comparable between the groups. Interstage HMP permits timely intervention and led to an important decrease in interstage mortality. One-third of the patients with home monitoring program needed re-admission and demonstrated the need for earlier stage 2 palliation.

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.

Reintervention Before Bidirectional Cavopulmonary Shunt and Intermediate Outcomes in Children with Single Ventricle Who Underwent Main Pulmonary Artery Banding.

Unplanned reinterventions following pulmonary artery banding (PAB) in single ventricle patients are common before stage 2 palliation (S2P) but associated risk factors are unknown. We hypothesized that reintervention is more common when PAB is placed at younger age and with a looser band, reflected by lower PAB pressure gradient. Retrospective single center study of single ventricle patients undergoing PAB between Jan 2000 and Dec 2020. The 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 reintervention were summarized using a competing risk model. 77 patients underwent PAB at median (IQR) 47 (24-66) days and 3.73 (3.2-4.5) kg. Within18 months of PAB, 60 (78%) reached S2P, 9 (12%) died, 1 (1%) transplanted and 7 (9%) were alive without S2P. Within 18months of PAB 10 (13%) patients underwent reintervention related to pulmonary blood flow modification: PAB adjustment (n = 6) and conversion to Damus-Kaye-Stansel/Blalock-Taussig-Thomas shunt (n = 4). 6/10 (60%) reached S2P following reintervention. A trend toward higher intervention in patients with a genetic syndrome (p-0.06) and weight < 3kg (p-0.057) at time of PAB was noted. Only genetic syndrome was a risk factor associated with poor outcome (p-0.025). PAB has a reasonable outcome in SV patients with unobstructed systemic and pulmonary blood flow, but with a high reintervention rate. Only a quarter of patients with genetic syndromes reach S2P and further study is required to explore the benefits from an alternative palliative strategy.

The Efficacy of Sildenafil in Children with Uni-Ventricle Congenital Heart Disease Post Bidirectional Cavopulmonary Shunt: An Evidence-Based Case Report

BACKGROUND: Univentricular congenital heart disease (CHD) is an anatomical heart defect where one of the ventricles does not develop. Management of univentricular defects is performed gradually; bidirectional cavopulmonary shunt (BCPS) is one of the surgical approaches conducted before the definite treatment in the Fontan procedure. Therefore, the average rate of pulmonary artery pressure and vasculature resistance is critical factors in determining good post-surgical outcomes. However, studies exploring the evidence that sildenafil administration can reduce pulmonary pressure in patients with univentricular defects are currently limited. AIM: This evidence-based case report aims to investigate whether sildenafil administration toward post-BCPS patients reduces mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) before undergoing the Fontan procedure. METHODS: Available evidence was screened through four databases in PubMed, Cochrane, Embase, and ProQuest on October 9, 2022. The keywords used were (((((single Ventricle) OR univentricular heart) AND BCPS) OR Glenn) AND sildenafil) AND hemodynamic. A result of three cohorts and one clinical trial was identified and critically appraised. RESULTS: Analytical testing of the two studies by Park I and Jeremiasen et al. shows that sildenafil significantly reduces mPAP from 19.5 ± 5.5 mmHg to 14.3 ± 3.0 mmHg (p = 0.023) and from 19 mmHg (SD = 3) to 14 mmHg (SD = 2) (p &lt; 0.01). The studies from Hill KD and Mori et al. illustrate that sildenafil significantly reduces the PVR index by as much as 24% (p &lt; 0.01) and from 3.2 ± 0.5 wood unit to 1.6 ± 0.6 wood unit (p &lt; 0.0001). CONCLUSION: The administration of sildenafil decreases pulmonary artery pressure and vasculature resistance in post-BCPS pediatric patients, allowing patients to undergo the Fontan procedure.

Evaluation of Portal, Splenic, and Hepatic Vein Flows in Children Undergoing Congenital Heart Surgery

Little is known about changes in portal, splenic, and hepatic vein flow patterns in children undergoing congenital heart surgery. This study aimed to determine the characteristics of portal, splenic, and hepatic vein flow patterns using ultrasonography in children undergoing cardiac surgery. Single-center, prospective observational study. Tertiary children's hospital, operating room. Children undergoing cardiac surgery. The authors obtained ultrasound data from the heart, inferior vena cava, portal, splenic, and hepatic veins before and after surgeries. In the biventricular group, which included children with atrial and ventricular septal defects and pulmonary stenosis (n=246), the portal pulsatility index decreased from 38.7% to 25.6% (p < 0.001) after surgery. The preoperative portal pulsatility index was significantly higher in patients with pulmonary hypertension (43.3% v 27.4%; p < 0.001). In the single-ventricle group (n=77), maximum portal vein flow velocities of Fontan patients were significantly lower (13.5 cm/s) compared with that of patients with modified Blalock-Taussig shunt (19.7 cm/s; p=0.035) or bidirectional cavopulmonary shunt (23.1 cm/s; p < 0.001). The cardiac index was inversely correlated with the portal pulsatility index in the bidirectional cavopulmonary shunt and Fontan circulation. (β=-5.693, r2=0.473; p=0.001) The portal pulsatility index was correlated with splenic venous pulsatility and hepatic venous atrial reverse flow velocity in biventricular and single-ventricle groups. The characteristics of venous Doppler patterns in the portal, splenic, and hepatic veins differed according to congenital heart disease. Further studies are required to determine the association between splanchnic venous Doppler findings and clinical outcomes in this population.

Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation

BackgroundPatients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear. MethodsRetrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality. ResultsMedian age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O2 saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02). ConclusionsPatients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.

Changes in pulmonary artery index and its relation to outcome after stage II palliation in patients with hypoplastic left heart syndrome.

This study aimed to prove if pulmonary artery (PA) size influences survival and if an additional aortopulmonary shunt (APS) promotes left PA growth after bidirectional cavopulmonary shunt (BCPS) in patients with hypoplastic left heart syndrome. The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and BCPS between 2007 and 2020 were reviewed. Right, left and total (right + left) PA indices were calculated according to Nakata and colleagues. A total of 158 patients were included in this study. The median age at Norwood and BCPS was 8 (7-11) days and 3.6 (3.1-4.6) months, respectively. There were 7 hospital deaths and 12 late deaths. Survival after BCPS was 90.3% at 1 year and 86.2% at 2 years. Total, right and left PA indices were 238 (195-316), 136 (101-185) and 102 (75-130) mm2/m2 at the time of BCPS, and they were 237 (198-284), 151 (123-186) and 86 (69-108) mm2/m2 at the time of Fontan. Left PA index decreased significantly between the time of BCPS and Fontan (P < 0.01). Nine patients needed partial takedown and additional APS due to failing BCPS, but the additional APS did not promote the PA growth significantly. Preoperative PA index did not affect the mortality after BCPS. The partial takedown and additional APS for failing BCPS were unable to improve left PA size.

Review of surgical experience in 188 infants and young children with Ebstein anomaly.

This study sought to review our single-institutional surgical experience in paediatric Ebstein anomaly (EA). We retrospectively reviewed the paediatric patients with EA undergoing operation between 2004 and 2020. The time-to-event analysis was studied using Kaplan-Meier estimates. Cox regression model was used to identify risk factors for recurrent moderate-severe or greater tricuspid regurgitation (TR). A total of 188 patients at a median age of 3.0 [interquartile range (IQR), 1.6-5.6] years were included, among whom 108 (57.4%) underwent cone reconstruction (CR). Bidirectional cavopulmonary shunt was required in 53 patients (28.2%). There were no in-hospital deaths. The median follow-up time was 5.6 (IQR, 2.9-8.9) years. Twenty-three (12.2%) developed recurrent moderate-severe or greater TR, among whom 9 required reoperation and 1 had late death. There was a lower incidence of recurrent TR (P = 0.006) and reoperation for TR (P = 0.037) in the CR group compared with the non-CR group. There was no difference in the incidence of recurrent TR (P = 0.61), reoperation (P = 0.9) and death (P = 0.48) among patients aged <1, 1-4 and 4-18 years. Acceptable outcomes can be anticipated in paediatric EA undergoing CR in terms of freedom from TR of > moderate degree at a mid-term follow-up.

Current state of the art in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.

The impact of pulmonary artery size on midterm outcomes after nonfenestrated Fontan operation

ObjectiveWe sought to identify the impact of pulmonary artery size on outcomes after nonfenestrated total cavopulmonary connection. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the impact of each branch pulmonary artery size was individually determined. MethodsThe medical records of all patients who underwent nonfenestrated total cavopulmonary connection between 2009 and 2021 were reviewed. The pulmonary artery index was calculated using angiography before the operation. ResultsA total of 247 patients were included in this study. A right-sided bidirectional cavopulmonary shunt was performed in 217 patients (88%). Median pulmonary artery index was 162 (133-207) mm2/m2 before total cavopulmonary connection. Chylothorax occurred in 55 patients (22%). Pulmonary artery index was an independent factor for chylothorax (odds ratio, 0.98, 95% confidence interval, 0.97-0.99, P < .001) with a cutoff value of 170 mm2/m2. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the left pulmonary artery index was identified as an independent risk factor for longer stay in the intensive care unit (coefficient B –0.02, 95% confidence interval, –0.04 to –0.002, P = .034) and for adverse events (hazard ratio, 0.98, 95% confidence interval, 0.96-0.99, P = .011) with a cutoff value of 56 mm2/m2. ConclusionsThe pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm2/m2. In patients with right-sided bidirectional cavopulmonary shunt, left pulmonary artery index has a significant predictive value for longer stay in the intensive care unit and adverse events with a cutoff value of 56 mm2/m2.

Modified biatrial heart transplantation after bilateral bidirectional cavo-pulmonary shunts.

We describe a patient who underwent modified biatrial anastomosis heart transplantation after the bilateral Glenn procedure. We introduced a new surgical technique to use the native central pulmonary artery as systemic venous return, which was anastomosed to right atrium, and then, biatrial anastomosis was performed.

Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation

BackgroundTotal anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC’s impact on staged palliation outcomes. MethodsIn a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. ResultsPrimary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P < .001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P < .001). ConclusionsOverall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients.

Modified cone procedure along with bidirectional cavopulmonary shunt for Ebstein anomaly in adult patients

Ebstein anomaly is an uncommon congenital cardiac disease affecting the tricuspid valve and right ventricle. Da silva pioneered the cone reconstruction of tricuspid valve for surgical repair of this anomaly in 2007. We describe our experience of 5 adult patients who underwent cone repair along with a bidirectional cavopulmonary shunt and tricuspid annuloplasty ring placement at our institute. Five adult patients with Ebstein anomaly with progressive disease and symptoms were taken up cone reconstruction from 2019 to 2021. Along with cone reconstruction of tricuspid valve, we routinely added an end to side superior vena cava to right pulmonary artery shunt and a tricuspid annuloplasty ring in the newly formed tricuspid annulus in all cases. All patients tolerated the procedure well and easily weaned off cardiopulmonary bypass. There were no complications regarding bidirectional cavopulmonary shunt and only one patient had a temporary heart block which was managed conservatively. This series shows routine addition of a cavopulmonary shunt and an annuloplasty ring improves outcomes and can be performed at other experienced cardiac surgery centres.