Bidirectional Cavopulmonary Connection Research Articles

Fluid accumulation in the staged Fontan procedure: the impact of colloid osmotic pressures.

Despite Fontan surgery showing improved results, fluid accumulation and oedema formation with pleural effusion are major challenges. Transcapillary fluid balance is dependent on hydrostatic and colloid osmotic pressure (COP) gradients; however, the COP values are not known for Fontan patients. The aim of this study was to evaluate the COP of plasma (COPp) and interstitial fluid (COPi) in children undergoing bidirectional cavopulmonary connection and total cavopulmonary connection. This study was designed as a prospective, observational study. Thirty-nine children (age 3 months-4.9 years) undergoing either bidirectional cavopulmonary connection or total cavopulmonary connection procedures were included. Blood samples and interstitial fluid were obtained prior to, during and after the preoperative cardiac catheterization and surgery with the use of cardiopulmonary bypass (CPB). Interstitial fluid was harvested using the wick method when the patient was under general anaesthesia. Plasma and interstitial fluid were measured by a colloid osmometer. Baseline values were compared with data from healthy controls. Baseline COPp was 20.6 ± 2.8 and 22.0 ± 3.2 mmHg and COPi was 11.3 ± 2.6 and 12.5 ± 3.5 mmHg in the bidirectional cavopulmonary connection group and the total cavopulmonary connection group, respectively. These values were significantly lower than in healthy controls. The COPp was slightly reduced throughout both procedures and normalized after surgery. The COPi increased slightly during the use of CPB and significantly decreased after surgery, resulting in an increased COP gradient and was correlated to pleural effusion. Fluid accumulation seen after Fontan surgery is associated with changes in COPs, determinants for fluid filtration and lymphatic flow. NCT 02306057: https://clinicaltrials.gov/ct2/results?cond=&term=NCT+02306057.

Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality.

We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.

Accuracy of computed tomography in detection of great vessel stenosis or hypoplasia before superior bidirectional cavopulmonary connection: Comparison with cardiac catheterization and surgical findings

Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r=0.98 for both) and a low mean bias (0.71mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between -0.9 and 2.3mm and between -0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P=0.13). All computed tomography scans were performed without sedation. Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

Elevated Pulmonary Artery Pressure, Not Pulmonary Vascular Resistance, is an Independent Predictor of Short-Term Morbidity Following Bidirectional Cavopulmonary Connection.

Single ventricle palliation relies on the pulmonary vasculature accommodating non-pulsatile systemic venous return. Mean pulmonary artery pressure (MPAP) and indexed pulmonary vascular resistance (PVRi) are two measures that impact pulmonary blood flow following bidirectional cavopulmonary connection (BCPC). The purpose of the study was to determine which hemodynamic features are associated with adverse outcomes after BCPC. Pre-operative hemodynamic data and post-operative morbidity and mortality in 250 patients undergoing BCPC at a single center from 2008 to 2014 were reviewed. Patients were then separated into 5 physiologic states based on MPAP, PVRi, and degree of pulmonary to systemic blood flow (Qp:Qs). There were 9 (3.6%) deaths, and 49 patients (20%) sustained major morbidity. An ROC curve identified MPAP > 16mmHg as an inflection point. Pre-BCPC sildenafil and oxygen use, ventricular dysfunction, and MPAP > 16mmHg (OR 4.1 [95% CI 1.8-9.2]) were independently associated with morbidity. MPAP > 16mmHg (OR 6.7 [95% CI 1.6-28]) and pre-BCPC oxygen use were associated with hospital mortality. PVRi was not associated with morbidity or mortality. Of the five physiologic states, patients with high MPAP, low PVRi, and low Qp:Qs fared the worst, with the highest risk of major morbidity (OR 8.6 [3.0-24.9]) and highest risk of mortality (OR 8.0 [1.5-41.3]) when compared to their reference groups (low MPAP, low PVRi). Elevated MPAP, need for pre-operative oxygen support, sildenafil use, and systemic ventricular systolic dysfunction predict morbidity following BCPC. Specifically, patients with elevated MPAP not due to elevated PVRi or pulmonary blood flow had the highest risk of morbidity and mortality.

Undiluted Contrast Media in the Pulmonary Veins and Left Atrium After Cavopulmonary Connection: CT Sign of a Right-to-Left Shunt

Pulmonary arteriovenous malformations and systemic-to-pulmonary venovenous shunts are right-to-left shunts that can compromise the superior bidirectional cavopulmonary connection (BCPC). Computed tomography is indicated in critically ill patients or when noncardiac issues are the primary indication

Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis†.

This study aimed to investigate the indications for and the results of axillary arterio-venous fistula in patients with a functionally single ventricle at mid-term follow-up after bidirectional cavopulmonary connection. From 2012 to 2017, 20 patients aged 21.1 ± 8.7 years (median 20.5) underwent axillary arterio-venous fistula after bidirectional cavopulmonary connection in our institution. The interval between bidirectional cavopulmonary connection and axillary arterio-venous fistula was 13.9 ± 7.2 years. The clinical and haemodynamic parameters of the patients were assessed before and after surgery. There was no in-hospital mortality. Paraesthesia of the arm persisted for 1-7 days. The mean arterial oxygen saturation increased from 72.4 ± 7.1% pre-surgery to 81.6 ± 5.5% (P < 0.001) after surgery, and was 83.8 ± 6.5% at discharge (P = 0.002 Wilcoxon matched pairs test). The haemoglobin, haematocrit, McGoon index and ejection fraction values did not change significantly. The speed and minute volume of the blood flow in the superior vena cava increased from from 21.1 ± 8.3 cm/s to 28.8 ± 14.9 cm/s (P < 0.001) and from 10.6 ± 3.7 ml/s to 12.5 ± 2.9 ml/s (P < 0.001), respectively. At the mid-term follow-up (1-5 years), 2 patients died from concomitant pathology. Stable improvement in the state and tolerance of physical activity was noted: before surgery, 17 patients were classified as New York Heart Association functional capacity (FC) III and 3 patients as FC IV; after surgery, 9 patients were FC II and 3 patients were FC III. One patient underwent a Fontan operation within 3 years. Axillary arterio-venous fistula can be used as the stage of surgical treatment and a bridge to Fontan and/or heart transplantation for this category of patients.

Pediatric Fontan patients are at risk for myocardial fibrotic remodeling and dysfunction

BackgroundPatients with single ventricle (SV) circulations are at risk for ventricular dysfunction. This study investigates whether there is evidence of increased myocardial fibrosis and myocardial dysfunction in children after the Fontan operation. MethodsConsecutive children after the Fontan operation who underwent cardiac magnetic resonance (CMR) T1 relaxometry with a modified look-locker inversion recovery approach were included in this retrospective study. Native T1 times (T1) and extracellular volume fractions (ECV) in the free wall of the dominant ventricle (left, SLV; right, SRV) were compared with controls and correlated with hemodynamic and clinical parameters. ResultsTwenty-one SV patients (9.7±4.6years; 13 SLV; 8 SRV) and 24 healthy control children (13.9±2.6years, p=0.002) were included. T1 and ECV were higher in SRV patients than in controls (1036±46ms vs 974±27ms, p<0.001; 28±4% vs 22±3%, p=0.002) and SLV patients (978±39ms, p=0.002; 23±5%, p=0.012) while there was no difference between SLV patients and controls. Age at bidirectional cavopulmonary connection was correlated with T1 (R=0.55, p=0.015), while systolic blood pressure (R=−0.68, p<0.001) and body weight (R=−0.54, p=0.012) inversely correlated with ECV. T1 negatively correlated with radial and circumferential strain by CMR feature tracking. ConclusionsFontan patients with a SRV show increased CMR markers of diffuse myocardial fibrosis, which are associated with decreased myocardial contractility. Whether their increased fibrosis burden conveys a greater risk for long-term complications in this population remains to be investigated.

24 Accuracy of computed tomography for detecting great vessel stenosis or hypoplasia in complex congenital heart disease prior to superior bidirectional cavopulmonary connection: comparison with cardiac catheterization and surgical findings

Aim This study sought to investigate diagnostic accuracy and safety of computed tomography (CT) in assessing great vessel stenosis/hypoplasia prior to superior bidirectional cavopulmonary connection (BCPC) compared with cardiac catheterisation and surgical findings. Method and results Twenty-seven patients (37% after Norwood operation) who were assessed by CT prior to BCPC between January 2010 and June 2016, at median age of 229 days (range 96 days–3.2 years), were included. Median weight at the time of BCPC was 8.3kg (4.0–13.4kg), median time from previous surgery 222days (97–885 days). Sixteen of the patients also underwent cardiac catheterisation. Patients received significantly higher radiation dose at cardiac catheterisation than at CT (median 2.6, IQR 1.6,3.5 mSv versus 1.2, IQR 1.0, 1.9 mSv; respectively;p=0.040). Four patients (25%) suffered minor complications from cardiac catheterisation. There were no complications from CT scan. All cardiac catheterisations were performed under general anaesthesia. No sedation was required for any of the patients for CT. Based on surgical findings, the accuracy of CT for detecting stenosis/hypoplasia of either pulmonary artery was 99.7%. Nine right and 11 left pulmonary artery branches were enlarged at the time of surgery. CT did not miss any stenosis/hypoplasia of branch pulmonary arteries and reported 2 cases of branch pulmonary artery hypoplasia which were considered at the time of BCPC surgery to be normal. Conclusion CT may replace cardiac catheterisation for identification of great vessel stenosis or hypoplasia in patients before BCPC. This requires less radiation, carries less morbidity and can be performed without sedation in this fragile group of patients.

The Impact of Systemic-to-Pulmonary Collateral Flow (SPCF) in Patients after Bidirectional Cavopulmonary Connection (BCPC) or Fontan Operation Assessed with 4D Flow MRI

The Impact of Systemic-to-Pulmonary Collateral Flow (SPCF) in Patients after Bidirectional Cavopulmonary Connection (BCPC) or Fontan Operation Assessed with 4D Flow MRI

Hepatic stiffness in the bidirectional cavopulmonary circulation: The Liver Adult-Pediatric-Congenital-Heart-Disease Dysfunction Study group

ObjectivesWe hypothesized that hepatic injury in single-ventricle CHD has origins that predate the Fontan operation. We aimed to measure hepatic stiffness using ultrasound and shear wave elastography (SWE) in a bidirectional cavopulmonary connection (BCPC) cohort. MethodsSubjects were prospectively recruited for real-time, hepatic, ultrasound-SWE for hepatic stiffness (kPa) and echocardiography. Doppler velocities, a velocity-time integral, flow volume, and resistive index, pulsatility index, and acceleration index were measured in celiac and superior mesenteric arteries, and in the main portal vein (MPV). Comparisons were made among subjects who had BCPC, subjects who were healthy, and a cohort of patients who had undergone the Fontan procedure. ResultsForty subjects (20 patients who had BCPC; 20 age- and gender-matched control subjects) were studied. The hepatic stiffness in BCPC was elevated, compared with that in control subjects (7.2 vs 5.7 kPa; P = .039). Patients who had BCPC had significantly higher celiac artery resistive index (0.9 vs 0.8; P = .002); pulsatility index (2.2 vs 1.7; P = .002); and systolic-diastolic flow ratio (10.1 vs 5.9; P = .002), whereas the superior mesenteric artery acceleration index (796 vs 1419 mL/min in control subjects; P = .04) was lower. An elevated resistive index (0.42 vs 0.29; P = .002) and pulsatility index (0.55 vs 0.35; P = .001) were seen in MPV, whereas MPV flow was reduced (137.3 vs 215.7 mL/min in control subjects; P = .036). A significant correlation was found for hepatic stiffness with right atrial pressure obtained at catheterization (P = .002). Comparison with patients who underwent the Fontan procedure showed patients who had BCPC had lower hepatic stiffness (7.2 vs 15.6 kPa; P < .001). ConclusionsHepatic stiffness is increased with BCPC physiology, and this finding raises concerns that hepatopathology in palliated, single-ventricle CHD is not exclusively attributable to Fontan physiology. Hepatic stiffness measurements using SWE are feasible in this young population, and the technique shows promise as a means for monitoring disease progression.

Prior Innominate Vein Occlusion Does Not Preclude Successful Bidirectional Superior Cavopulmonary Connection

Low superior vena cava (SVC) blood flow has recently been identified as a marker for bidirectional superior cavopulmonary connection (SCPC) failure and death. Prior innominate vein occlusion is considered a significant anatomic risk factor for SCPC failure. We therefore evaluated the outcomes of infants who underwent SCPC with known upper-body venous obstruction. Between February 1995 and June 2014, SCPC was carried out in 8 patients who had either a single SVC with known prior occlusion of the innominate vein (n = 6) or bilateral SVCs without a bridging vein with occlusion of one SVC (n = 2). The cause of the occlusion was an indwelling catheter in 5 patients. These patients were compared with 8 patients with normal upper-body venous drainage who underwent SCPC. Patients were evaluated for preoperative risk factors (including SVC size, pulmonary artery size, Nakata index, pulmonary vascular resistance), operative factors, and clinical outcomes to determine the impact of prior upper-body venous occlusion on SCPC failure or death. There were no significant differences in preoperative risk factors between the two groups, except for a significantly lower Nakata index in the study group with a trend toward smaller pulmonary artery branch size. There were no SCPC takedowns or mortalities. There was no significant difference in postoperative length of stay (median of 7 days [range, 5 to 32 days] versus 5 days [range, 4 to 32 days]; p = 0.17. Study patients had a lower mean systemic oxygen saturation at discharge, 81% versus 85% (p = 0.05). In the study group, at a median follow-up of 42 months, 3 patients underwent successful Fontan completion and 5 are still awaiting Fontan completion. Although patients with prior upper-body venous obstruction may have lower systemic oxygen saturations at hospital discharge, they do not demonstrate an increased SCPC failure or mortality rate. Innominate vein occlusion or its equivalent in patients with bilateral SVCs should not preclude the performance of SCPC. Physiologic rather than anatomic evaluation of preoperative systemic venous return may be more useful to predict outcome after SCPC.

Atrio-ventricular valve regurgitation in univentricular hearts: outcomes after repair†.

The aim was to describe the early and mid-term outcome after atrio-ventricular valve (AVV) repair in patients with univentricular hearts (UVHs) and to identify risk factors for AVV reoperation and death. This study is a retrospective review of patients undergoing valve repair for AVV regurgitation at any stage of univentricular palliation from 1998 to 2014. Patient- and procedure-related variables were analysed. A total of 31 consecutive patients underwent 38 procedures for ≥ moderate AVV regurgitation at a median age of 3.6 years. Thirty-two percent of patients had a common AVV, 26% had two AVVs, 22% had a dominant tricuspid valve and 19% had a dominant mitral valve. All patients underwent valve repair as a first procedure without early mortality. At discharge, patients preserved their ventricular function (fractional shortening <30%: preoperative 16% vs postoperative 22.5%, NS). In 19% (n = 6) of patients, the procedure was considered as failed because of significant residual regurgitation. There were three late deaths [median delay: 1 year (range 0.7-13.6)] and three heart transplantations. Six patients underwent seven AVV reoperations [median delay: 2 years (range 0.2-7.6)]. Longer intensive care stay (P = 0.022), longer total postoperative hospital stay (P = 0.039), higher total number of surgeries (P = 0.039), lower body mass index (P = 0.042) and higher preoperative mean pulmonary pressure (P = 0.047) were univariate risk factors for death/transplantation. Failed first AVV repair (P = 0.01), higher total number of surgeries (P = 0.026), lower body mass index (P = 0.031), male gender (P = 0.031) and need for valve repair before bidirectional cavopulmonary connection (P = 0.036) were univariate risk factors for AVV reoperation. In multivariate analysis, no univariate risk factor reached statistical significance. Freedom from death/transplantation was 84% (CI 95%: 70%-98%) at 5 and 10 years. Survival free from AVV reoperation was 72% (CI 95%: 52%-92%) at 5 years and 62% at 10 years (CI 95%: 36%-88%). Mean follow-up of survivors was 4.7 years (SD ± 4.3; range 0.2-15.6). At last visit, 96% of survivors were in NYHA Class I-II. Ninety-two percent had a ≤ mild residual regurgitation. In patients with a UVH and ≥ moderate AVV regurgitation, AVV repair is feasible without postoperative deterioration of their ventricular function. Nevertheless, these patients remain at increased risk for death/transplantation and AVV reoperation.

The modern Fontan operation shows no increase in mortality out to 20 years: A new paradigm

Dating back to the first published report of the Fontan circulation in 1971, multiple studies have examined the long-term results of this standard procedure for palliation of single-ventricle heart disease in children. Although the technique has evolved over the last 4 decades to include a polytetrafluorethylene (PTFE) conduit for a large percentage of patients, the long-term outcome has not yet been established. The aim of the current study was to investigate the possibility of a late increasing risk for death after 15 years among patients with a modern Fontan operation and to evaluate late morbidity. Between January 1, 1988, and December 31, 2011, 207 patients underwent the Fontan procedure using an internal or external PTFE conduit plus a bidirectional cavopulmonary connection. Survival and late adverse events were analyzed. Risk factors for early and late mortality were examined using hazard function methodology. At 1, 10, and 20 years, survival for the entire cohort was 95%, 88%, and 76%, respectively, with no deaths in the last 6 years of the study. Hazard modeling showed a 1.3% risk of death per year 24 years after the Fontan procedure, with no late increasing hazard phase. Freedom from reoperations was greater than 90% at 20 years and freedom from thrombotic complications was 98% at 20 years (with greater than 80% of patients on aspirin alone). Survival curves were superimposable for 16- to 20-mm conduits, and the freedom from any reoperation including transplantation was greater than 90% after 20 years. Multivariable risk factor analysis identified only earlier date of operation as a predictor of early and late mortality. By era of surgery, the 10-year predicated survival is 89% for patients undergoing surgery in 2000 and 94% for patients in 2010. Early and late survival after a Fontan operation with a PTFE conduit is excellent, with no late phase of increasing death risk after 20 years. Late functional status is good, the need for late reoperation is rare, and thrombotic complications are uncommon on a standard medical regimen including aspirin as the only anticoagulation medication.

Effect of mechanical assistance of the systemic ventricle in single ventricle circulation with cavopulmonary connection

Previous attempts to support single ventricle circulation mechanically have suggested that acustom-built assist device is needed to push, rather than pull, through the pulmonary circulation. We hypothesized that using a conventional ventricular assist device, with or without conversion of a total cavopulmonary connection to a bidirectional Glenn cavopulmonary connection, would allow assistance by pulling blood through the circuit and improve the cardiac index (CI). Cavopulmonary connections were established in each of 5 Yorkshire pigs (25 kg) using ePTFE conduits in a Y configuration with appropriate clamping of the limbs of the Y to achieve a total cavopulmonary Fontan connection (TCPC), superior vena cava cavopulmonary connection (SVC Glenn), and inferior vena cava cavopulmonary connection (IVC Glenn). A common atrium had been established previously by balloon septostomy. Mechanical circulatory assistance of the single systemic ventricle was achieved using a centrifugal pump with common atrial inflow and proximal ascending aortic outflow. The CI was calculated using an ultrasonic flow meter placed on the distal ascending aorta and compared between the assisted and nonassisted circulation for 3 conditions: TCPC, SVC Glenn, and IVC Glenn. The mean pulmonary artery pressure, common atrial pressure, arterial oxygen saturation, partial pressure of arterial oxygen, and oxygen delivery were calculated. The unassisted SVC Glenn CI tended to be greater than the TCPC or IVC Glenn CI. Significant augmentation of total CI was achieved with mechanical assistance for SVC Glenn (109% ± 24%, P=.04) and TCPC (130% ± 109%, P=.01). The assisted CI achieved at least a mean baseline biventricular CI for all 3 support modes. Oxygen delivery was greatest for assisted SVC Glenn (1786 ± 1307 mL/L/min) and lowest for TCPC (1146 ± 386 mL/L/min), with a trend toward lower common atrial and pulmonary artery pressures for SVC Glenn. SVC bidirectional Glenn circulation might allow optimal augmentation of the CI and oxygen delivery in a failing single ventricle using a conventional pediatric ventricular assist device. The results from our model also suggest that the Fontan circulation itself can be supported with systemic ventricular assistance of the single ventricle.

The effects of different ventilator modes on cerebral tissue oxygen saturation in patients with bidirectional superior cavopulmonary connection

We used near-infrared spectroscopy to document changes in cerebral tissue oxygen saturation (SctO2) in response to ventilation mode alterations after bidirectional Glenn (BDG; superior cavopulmonary connection) procedure. We also determined whether spontaneous ventilation have a beneficial effect on hemodynamic status, lactate and SctO2 when compared with other ventilation modes. 20 consecutive patients undergoing BDG were included. We measured SctO 2 during three ventilator modes (intermittent positive-pressure ventilation [IPPV]; synchronized intermittent mandatory ventilation [SIMV]; and continuous positive airway pressure + pressure support ventilation [CPAP + PSV]). We, also, measured mean airway pressure (AWP), arterial blood gases, lactate and systolic arterial pressures (SAP). There was no change in SctO2 in IPPV and SIMV modes; the SctO2 measured during CPAP + PSV and after extubation increased significantly (60.5 ± 11, 61 ± 10, 65 ± 10, 66 ± 11 respectively) (P < 0.05). The differences in the SAP measured during IPPV and SIMV modes was insignificant; the SAP increased significantly during CPAP + PSV mode and after extubation compared with IPPV and SIMV (109 ± 11, 110 ± 12, 95 ± 17, 99 ± 13 mmHg, respectively) (P < 0.05). Mean AWP did not change during IPPV and SIMV modes, mean AWP decreased significantly during CPAP + PSV mode (14 ± 4, 14 ± 3, 10 ± 1 mmHg, respectively) (P < 0.01). The SctO2 was higher during CPAP + PSV ventilation and after extubation compared to IPPV and SIMV modes of ventilation. The mean AWP was lower during CPAP + PSV ventilation compared to IPPV and SIMV modes of ventilation.

Tricuspid Atresia Associated with Truncus Arteriosus versus Aortopulmonary Window: Combining Fetal and Postnatal Echocardiography to Make the Diagnosis

The coincident occurrence of tricuspid atresia and aortopulmonary window (APW) is exceedingly rare, with one previous case reported in the literature. We present a patient with tricuspid atresia, pulmonary atresia, and APW. Postnatal echocardiograms demonstrated no visible pulmonary valve, and additional defects including a bicuspid aortic valve, right aortic arch and anomalous coronary arteries raised suspicion for tricuspid atresia with persistent truncus arteriosus. However, fetal echocardiography and direct visualization of the anatomy confirmed the alternate diagnosis. The patient underwent successful palliation consisting of APW repair, atrial septectomy and a 3.5mm modified Blalock-Taussig shunt, followed by a bidirectional cavopulmonary connection.

Persistent Left Superior Vena Cava Remnant Causing Cyanosis in a Post-Fontan Patient

We report the successful surgical closure of a persistent left superior vena cava remnant draining into the pulmonary venous circulation causing cyanosis in a post-Fontan patient who had previously undergone Damus-Kaye-Stansel and bidirectional superior cavopulmonary connection followed by a transcatheter coil occlusion of his persistent left superior vena cava.

Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation

The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multi-stage palliation of various single ventricle anomalies. We aimed to report current outcomes following BCPC and to examine risk factors affecting survival and progression to next palliation stage. 227 consecutive children with variable single ventricle pathologies underwent BCPC from 2002 to 2007. Competing risks analyses were performed to model events after BCPC (death and transition to Fontan) and subsequently after Fontan (death and cardiac reoperation); and to examine associated risk factors for poor outcomes. There were 139 males (61%) with median age of 7.6 months (interquartile range (IQR) 6.0–10.8) and median weight of 6.2 kg (IQR 5.2–7.4). Forty-three patients (19%) had primary BCPC and 184 (81%) had prior palliation: aortopulmonary shunt (n = 83), Norwood (n = 55), pulmonary artery band (n = 48), atrial septectomy (n = 25), pulmonary artery reconstruction (n = 14), anomalous pulmonary venous connection repair (n = 7), other (n = 8). Predominant ventricle was left morphology (n = 122, 54%), right morphology (n = 95, 42%), two equally developed ventricles (n = 10, 4%). Twenty-six patients (12%) had bilateral SVC. Concomitant surgery included atrioventricular valve repair (n = 18), pulmonary artery augmentation (n = 80), percutaneous Fontan preparation (n = 34), other (n = 24). Competing risks analysis showed that 5-years following BCPC, approximately 17% have died, 76% have undergone Fontan and 7% were alive awaiting or not qualifying for Fontan. On multivariable analysis, risk factors for death prior to Fontan were PVRI > 3 WU/M2 (HR 3.9, p = 0.001), dominant right ventricle (HR 2.1, p = 0.03), prior palliation other than aortopulmonary shunt (HR 0.4, p = 0.03). Competing risks analysis showed that 3-years following 172 Fontan operations, approximately 10% have died, 6% have undergone further cardiac surgery and 84% were alive and free from reoperation. Overall, 8-year survival following BCPC was only 74%. Despite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, DILV) having the best survival while those with dominant right ventricle (i.e. HLHS, DORV with heterotaxy) having the worst survival. Increased pulmonary vascular resistance remains a significant factor affecting mortality.

Older children at time of norwood operation have ongoing mortality vulnerability that continues after the cavopulmonary connection

Background Delayed 1st stage palliation of children with hypoplastic left heart syndrome (HLHS) and related pathologies can be associated with poor outcomes due to development of progressive pulmonary vascular disease) and volume load effects on systemic ventricle and atrioventricular valve (AVV). We examine current era survival of this patients’ subgroup. Methods Fifty-five infants >2 weeks old underwent Norwood surgery (2003-07). Separate competing risks analyses were performed to model outcomes (death, transition to next stage) after Norwood and after bidirectional cavo-pulmonary connection (BCPC). Results Median age was 32 days (range:15-118). 47% had HLHS, 53% had other complex univentricular variants. Mean ascending aorta was 4.4 ± 1.9 mm, 10% had impaired ventricle function, 11% moderate AVV regurgitation, 32% restrictive pulmonary venous return. Pulmonary blood flow was established via aortopulmonary shunt (30) or Sano shunt (25) Following Norwood, patients required longer ventilation, more oxygen & nitric oxide and had higher inotropic scores compared to traditional management protocol. Competing risks analysis showed that 2-years after Norwood 39% had died and 57% underwent BCPC. 4-years after BCPC, 15% had died and 85% underwent Fontan operation. Overall 3 years survival post-Norwood was 53%. Factors associated with mortality were age, lower weight at Norwood, impaired ventricular function, longer circulatory arrest and lower pre-BCPC saturation. Conclusions Children >2 weeks old undergoing Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multi-stage palliation. Elevated pulmonary vascular resistance, volume load effects such as systemic ventricle impairment and AVV regurgitation are commonly evident in patients who fail or don’t qualify to proceed to next stage palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

807 Aortopulmonary Collateral Flow Volume Impacts Early Postoperative Outcome After Fontan Completion: A Multimodality Study

Aortopulmonary collaterals (APCs) are a frequent phenomenon in patients after bidirectional cavopulmonary connection (BCPC). APC flow volume can be quantified using cardiac magnetic resonance (CMR) imaging. However, the significance of APC flow for the postoperative outcome after total cavopulmonary connection (TCPC) is unclear and was sought to be determined.