ABSTRACT
BACKGROUND: Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. METHODS AND RESULTS: We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. CONCLUSIONS: Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.
Subject(s)
Palliative Care , Reoperation , Humans , Male , England/epidemiology , Female , Retrospective Studies , Wales/epidemiology , Infant , Child, Preschool , Reoperation/statistics & numerical data , Heart Transplantation/statistics & numerical data , Registries , Fontan Procedure/mortality , Univentricular Heart/surgery , Univentricular Heart/mortality , Univentricular Heart/physiopathology , Heart Ventricles/abnormalities , Heart Ventricles/surgery , Heart Ventricles/physiopathology , Infant, Newborn , Heart Defects, Congenital/surgery , Heart Defects, Congenital/mortality , Time Factors , Treatment OutcomeABSTRACT
Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.
Subject(s)
Cardiac Catheterization , Fontan Procedure , Heart Defects, Congenital , Fontan Procedure/adverse effects , Cardiac Catheterization/instrumentation , Humans , Heart Defects, Congenital/surgery , Heart Defects, Congenital/physiopathology , Heart Defects, Congenital/diagnostic imaging , Pulmonary Artery/surgery , Pulmonary Artery/physiopathology , Pulmonary Artery/diagnostic imaging , Vena Cava, Inferior/diagnostic imaging , Vena Cava, Inferior/surgery , Vena Cava, Inferior/physiopathology , Polytetrafluoroethylene , Univentricular Heart/surgery , Univentricular Heart/physiopathology , Univentricular Heart/diagnostic imaging , Prosthesis Design , Treatment Outcome , Hemodynamics , Animals , Blood Vessel ProsthesisSubject(s)
Fontan Procedure , Humans , Female , Male , Child , Child, Preschool , Ethnicity , Sex Factors , Univentricular Heart/surgery , Univentricular Heart/physiopathology , Infant , Adolescent , Racial Groups , Heart Defects, Congenital/surgery , Heart Defects, Congenital/mortality , Survival RateSubject(s)
Extracorporeal Membrane Oxygenation , Norwood Procedures , Postoperative Care , Humans , Extracorporeal Membrane Oxygenation/methods , Norwood Procedures/methods , Postoperative Care/methods , Infant, Newborn , Heart Ventricles/surgery , Heart Ventricles/abnormalities , Univentricular Heart/surgeryABSTRACT
BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.
Subject(s)
Cardiac Catheterization , Hypoplastic Left Heart Syndrome , Norwood Procedures , Palliative Care , Humans , Hypoplastic Left Heart Syndrome/surgery , Hypoplastic Left Heart Syndrome/physiopathology , Retrospective Studies , Treatment Outcome , Female , Male , Time Factors , Risk Factors , Norwood Procedures/adverse effects , Infant, Newborn , Cardiac Catheterization/adverse effects , Cardiac Catheterization/instrumentation , Atrial Septum/diagnostic imaging , Atrial Septum/physiopathology , Atrial Septum/surgery , Infant , Univentricular Heart/surgery , Univentricular Heart/physiopathology , Univentricular Heart/diagnostic imagingABSTRACT
AIM: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec). METHODS: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study. RESULTS: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death. CONCLUSIONS: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage.
Subject(s)
Heart Transplantation , Humans , Heart Transplantation/methods , Male , Female , Hospital Mortality/trends , Infant , Retrospective Studies , Palliative Care/methods , Univentricular Heart/surgery , Norwood Procedures/methods , Survival Rate/trends , Infant, Newborn , Follow-Up StudiesABSTRACT
Although the terms "single ventricle" and "univentricular heart" are frequently used to describe a variety of complex congenital heart defects, in fact, nearly all hearts have two ventricles, although one of the two may be too small to be functional. A better term for these hearts would therefore be "functional single ventricle."
Subject(s)
Tricuspid Atresia , Univentricular Heart , Humans , Heart Ventricles/physiopathology , Heart Ventricles/abnormalities , Tricuspid Atresia/surgery , Tricuspid Atresia/physiopathology , Tricuspid Atresia/diagnostic imaging , Univentricular Heart/surgery , Univentricular Heart/physiopathology , Univentricular Heart/diagnostic imaging , Univentricular Heart/therapy , Univentricular Heart/geneticsABSTRACT
Background and aims of the study A functionally single ventricle (FSV) refers to a group of congenital heart defects that are not amenable for biventricular correction. The Fontan operation is utilized as surgical treatment for most of FSV patients. The evaluation of FSV function is extremely difficult due to its unique pathophysiology. This study aimed to explore the efficacy of speckle tracking echocardiography (STE) parameters measured at rest and during exercise for comprehensive assessment of univentricular heart. METHODS: We enrolled 37 patients with a functionally single ventricle after the Fontan operation, hospitalized in the Department of Congenital Heart Defects between years 2019 and 2021.The echocardiographic stress tests were performed in the Echocardiography Laboratory of the Congenital Heart Defects Department. The study was conducted on a bicycle ergometer in a semi-recumbent position. The parameters obtained by speckle tracking echocardiography (STE): the longitudinal strain of the FSV free wall (Æ) and the longitudinal strain of myocardial layers: subendocardial, medial and subepicardial were analyzed. A transmural longitudinal strain gradient (TG) was calculated as the difference between longitudinal deformation of the subendocardial and subepicardial layers. Current results of cardiac magnetic resonance imaging (CMR) and cardio-pulmonary test (CPET) were also incorporated. Demographic data, past interventions, pharmacological treatment and comorbidities were extracted from medical records. RESULTS: Æ at rest and during exercise were not related to the parameters of physical capacity obtained on CPET nor to the CMR results. The transmural strain gradient was dependent on physical performance parameter- peak oxygen uptake- and related to the FSV ejection fraction calculated by magnetic resonance imaging. CONCLUSIONS: The transmural strain gradient and FSV free wall strain are readily measurable and suitable for evaluating single ventricle function. The TG is positively correlated with peak oxygen uptake during the cardiopulmonary test and with the ejection fraction derived from cardiac magnetic resonance imaging. The applicability of these findings in patients undergoing the Fontan procedure warrants further exploration.
Subject(s)
Echocardiography , Fontan Procedure , Humans , Fontan Procedure/methods , Male , Female , Echocardiography/methods , Adolescent , Heart Ventricles/diagnostic imaging , Heart Ventricles/physiopathology , Heart Ventricles/abnormalities , Adult , Univentricular Heart/surgery , Univentricular Heart/diagnostic imaging , Heart Defects, Congenital/surgery , Heart Defects, Congenital/diagnostic imaging , Child , Young Adult , Exercise Test/methods , Palliative Care/methodsABSTRACT
OBJECTIVES: To describe and compare maternal and fetal comorbidities and obstetrical outcomes in pregnancies with hypoplastic left and right heart (HLHS and HRH) single ventricle cardiac defects (SVCD) from a single center under a multidisciplinary protocol. METHOD: A single center retrospective review of fetal SVCD from 2013 to 2022. Maternal-fetal comorbidities, delivery, and postnatal outcomes were compared between HLHS and HRH using descriptive statistics and univariate and multivariate analyses. RESULTS: Of 181 SVCD pregnancies (131 HLHS; 50 HRH), 9% underwent termination, 4% elected comfort care, 5 died in utero and 147/152 liveborns survived to the first cardiac intervention. Cesarean delivery occurred in 57 cases (37%), planned in 36 and unplanned in 21. Comorbidities, which did not differ between HLHS and HRH, included fetal growth restriction (FGR, 17%), prematurity (14%), maternal hypertension (9%), maternal obesity (50%), fetal extracardiac anomalies and chromosome anomalies (12%, 13%). In multivariate analysis, only earlier gestational age at delivery and oligohydramnios predicted decreased odds of survival at one year. CONCLUSION: Maternal-fetal comorbidities are common in both HLHS and HRH. Earlier gestational age at delivery and oligohydramnios predict lower postnatal survival. FGR, even with severe early onset, did not significantly impact short- or long-term neonatal survival in single ventricle conditions.
Subject(s)
Comorbidity , Pregnancy Outcome , Humans , Female , Pregnancy , Retrospective Studies , Adult , Pregnancy Outcome/epidemiology , Hypoplastic Left Heart Syndrome/epidemiology , Hypoplastic Left Heart Syndrome/surgery , Infant, Newborn , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/therapy , Pregnancy Complications/epidemiology , Univentricular Heart/surgery , Univentricular Heart/epidemiologyABSTRACT
OBJECTIVES: Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation. DESIGN: Multicenter retrospective cohort study. SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society. PATIENTS: We reviewed 99 children with single ventricle physiology who underwent tracheostomy before the Fontan operation at 21 institutions participating in Collaborative Research from the Pediatric Cardiac Intensive Care Society between January 2010 and December 2020, with follow-up through December 31, 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Death occurred in 51 of 99 patients (52%). Cox proportional hazard analysis was performed to determine factors associated with death after tracheostomy. Results are presented as hazard ratio (HR) with 95% CIs. Nonrespiratory indication(s) for tracheostomy (HR, 2.21; 95% CI, 1.14-4.32) and number of weeks receiving mechanical ventilation before tracheostomy (HR, 1.06; 95% CI, 1.02-1.11) were independently associated with greater hazard of death. In contrast, diagnosis of tricuspid atresia or Ebstein's anomaly was associated with less hazard of death (HR, 0.16; 95% CI, 0.04-0.69). Favorable outcome, defined as survival to Fontan operation or decannulation while awaiting Fontan operation with viable cardiopulmonary physiology, occurred in 29 of 99 patients (29%). Median duration of mechanical ventilation before tracheostomy was shorter in patients who survived to favorable outcome (6.1 vs. 12.1 wk; p < 0.001), and only one of 16 patients with neurologic indications for tracheostomy and 0 of ten patients with cardiac indications for tracheostomy survived to favorable outcome. CONCLUSIONS: For children with single ventricle physiology who undergo tracheostomy, mortality risk is high and should be carefully considered when discussing tracheostomy as an option for these children. Favorable outcomes are possible, although thoughtful attention to patient selection and tracheostomy timing are likely necessary to achieve this goal.
Subject(s)
Fontan Procedure , Tracheostomy , Humans , Retrospective Studies , Male , Female , Infant , Child, Preschool , Fontan Procedure/methods , Child , Intensive Care Units, Pediatric , Heart Defects, Congenital/surgery , Heart Defects, Congenital/mortality , Univentricular Heart/surgery , Treatment Outcome , Proportional Hazards Models , Respiration, ArtificialABSTRACT
AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8-50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [P = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [P = 0.06] with a higher incidence in Group 2 during the FU [P = 0.005]. CONCLUSION: Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU.
Subject(s)
Arrhythmias, Cardiac , Fontan Procedure , Heart Ventricles , Humans , Male , Female , Fontan Procedure/adverse effects , Incidence , Child , Adolescent , Adult , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/diagnosis , Middle Aged , Young Adult , Heart Ventricles/physiopathology , Heart Ventricles/diagnostic imaging , Heart Defects, Congenital/surgery , Heart Defects, Congenital/epidemiology , Retrospective Studies , Time Factors , Univentricular Heart/surgery , Univentricular Heart/epidemiology , Univentricular Heart/physiopathology , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Risk FactorsABSTRACT
BACKGROUND: Children 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. METHODS: Children 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. RESULTS: Overall, 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). CONCLUSIONS: In 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.
Subject(s)
Biomarkers , Natriuretic Peptide, Brain , Peptide Fragments , Univentricular Heart , Humans , Peptide Fragments/blood , Natriuretic Peptide, Brain/blood , Male , Female , Child, Preschool , Infant , Biomarkers/blood , Univentricular Heart/surgery , Univentricular Heart/blood , Child , Fontan Procedure , Age Factors , Heart Ventricles/physiopathology , Heart Ventricles/abnormalities , Heart Ventricles/diagnostic imagingABSTRACT
Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d). Multivariable logistic regression and Cox proportional hazards modeling were used. Results: Fifty-one percent (137/269) of patients met TO. Common reasons for TO failure were prolonged LOS (78/132, 59%) and ventilator duration (67/132, 51%). In multivariable analysis, higher weight [odds ratio, OR: 1.44 (95% confidence interval, CI: 1.15-1.84), P = .002] was a positive predictor of TO achievement while right ventricular dominance [OR 0.47 (0.27-0.81), P = .007] and higher preoperative pulmonary vascular resistance [OR 0.58 (0.40-0.82), P = .003] were negative predictors. After controlling for preoperative factors and excluding operative mortalities, TO achievement was independently associated with a decreased risk of death over long-term follow-up [hazard ratio: 0.50 (0.25-0.99), P = .049]. Textbook outcome achievement was also associated with lower direct cost of care [$137,626 (59,333-167,523) vs $262,299 (114,200-358,844), P < .0001]. Conclusion: Achievement of the Glenn TO is associated with long-term survival and lower costs and can be predicted by certain risk factors. As outcomes continue to improve within congenital heart surgery, operative mortality will become a less informative metric. Textbook outcome analysis may represent a more balanced measure of a successful outcome.
Subject(s)
Fontan Procedure , Heart Defects, Congenital , Heart Ventricles , Humans , Retrospective Studies , Female , Male , Heart Defects, Congenital/surgery , Heart Defects, Congenital/mortality , Heart Ventricles/surgery , Heart Ventricles/abnormalities , Fontan Procedure/mortality , Fontan Procedure/methods , Infant , Child, Preschool , Heart Bypass, Right/mortality , Treatment Outcome , Univentricular Heart/surgery , Univentricular Heart/mortalityABSTRACT
BACKGROUND: In the Fontan palliation for single ventricle heart disease (SVHD), pulmonary blood flow is non-pulsatile/passive, low velocity, and low shear, making viscous power loss a critical determinant of cardiac output. The rheologic properties of blood in SVHD patients are essential for understanding and modulating their limited cardiac output and they have not been systematically studied. We hypothesize that viscosity is decreased in single ventricle circulation. METHODS: We evaluated whole blood viscosity, red blood cell (RBC) aggregation, and RBC deformability to evaluate changes in healthy children and SVHD patients. We altered suspending media to understand cellular and plasma differences contributing to rheologic differences. RESULTS: Whole blood viscosity was similar between SVHD and healthy at their native hematocrits, while viscosity was lower at equivalent hematocrits for SVHD patients. RBC deformability is increased, and RBC aggregation is decreased in SVHD patients. Suspending SVHD RBCs in healthy plasma resulted in increased RBC aggregation and suspending healthy RBCs in SVHD plasma resulted in lower RBC aggregation. CONCLUSIONS: Hematocrit corrected blood viscosity is lower in SVHD vs. healthy due to decreased RBC aggregation and higher RBC deformability, a viscous adaptation of blood in patients whose cardiac output is dependent on minimizing viscous power loss. IMPACT: Patients with single ventricle circulation have decreased red blood cell aggregation and increased red blood cell deformability, both of which result in a decrease in blood viscosity across a large shear rate range. Since the unique Fontan circulation has very low-shear and low velocity flow in the pulmonary arteries, blood viscosity plays an increased role in vascular resistance, therefore this work is the first to describe a novel mechanism to target pulmonary vascular resistance as a modifiable risk factor. This is a novel, modifiable risk factor in this patient population.
Subject(s)
Blood Viscosity , Erythrocyte Aggregation , Erythrocyte Deformability , Fontan Procedure , Humans , Child , Heart Defects, Congenital/surgery , Heart Defects, Congenital/physiopathology , Male , Female , Hematocrit , Univentricular Heart/surgery , Univentricular Heart/physiopathology , Child, Preschool , Heart Ventricles/physiopathology , Heart Ventricles/abnormalities , Cardiac Output , Adolescent , ErythrocytesABSTRACT
INTRODUCTION AND OBJECTIVES: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. METHODS: SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. RESULTS: We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). CONCLUSIONS: The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.
Subject(s)
Atrial Fibrillation , Atrial Flutter , Fontan Procedure , Heart Defects, Congenital , Univentricular Heart , Humans , Adult , Univentricular Heart/surgery , Heart Defects, Congenital/surgery , Heart Ventricles/surgery , Treatment Outcome , Retrospective Studies , Pulmonary Artery/surgeryABSTRACT
OBJECTIVES: Atrioventricular valve (AVV) replacements in patients with single-ventricle circulations pose significant surgical risks and are associated with high morbidity and mortality. METHODS: From 1997 to 2021, 16 consecutive patients with functionally single-ventricle physiology underwent mechanical AVV replacement. Primary outcome was transplant-free survival. Secondary outcomes included major postoperative morbidity. RESULTS: The median age of AVV replacement was 2 years old (interquartile range 0.6-3.8 years). All AVV replacements were performed with a St. Jude Medical mechanical valve, median 24 mm (range, 19-31mm). Extracorporeal membrane oxygenation (ECMO) was required in 4 patients. Operative mortality was 38% (6/16). There were 2 late deaths and 3 transplants. Transplant-free survival was 50% at 1 year, 37.5% at 5 years, and 22% at 10 years. Transplant-free survival was higher for patients with preserved ventricular function (P = 0.01). Difference in transplant-free survival at 1 year was 75% vs 25%, at 5 years was 62.5% vs 12.5% and at 10 years was 57% vs 0%. Three (19%) patients had complete heart block requiring permanent pacemaker insertion. 6 of 13 patients (46%) patients reached Fontan completion (3 patients operated at/after Fontan). Significant bleeding events occurred in 8 patients (50%) with 3 patients suffering major cerebrovascular accidents. There were 6 events of valve thrombosis in 5 patients, resulting in 2 deaths and 2 heart transplants. CONCLUSIONS: Mechanical valve replacement carries significant morbidity and mortality risk. While it successfully salvages about half of patients with preserved ventricular function, careful consideration of alternative options should be made before embarking upon mechanical valve replacement.
Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital , Univentricular Heart , Humans , Infant , Child, Preschool , Treatment Outcome , Retrospective Studies , Univentricular Heart/surgery , Heart Defects, Congenital/surgeryABSTRACT
Background The impact of neighborhood socioeconomic status (SES) on outcomes following first-stage palliation of single ventricle heart disease remains incompletely characterized. Methods and Results This was a single-center, retrospective review of consecutive patients who underwent the Norwood procedure from January 1, 1997 to November 11, 2017. Outcomes of interest included in-hospital (early) mortality or transplant, postoperative hospital length-of-stay, inpatient cost, and postdischarge (late) mortality or transplant. The primary exposure was neighborhood SES, assessed using a composite score derived from 6 US census-block group measures related to wealth, income, education, and occupation. Associations between SES and outcomes were assessed using logistic regression, generalized linear, or Cox proportional hazards models, adjusting for baseline patient-related risk factors. Of 478 patients, there were 62 (13.0%) early deaths or transplants. Among 416 transplant-free survivors at hospital discharge, median postoperative hospital length-of-stay and cost were 24 (interquartile range, 15-43) days and $295 000 (interquartile range, $193 000-$563 000), respectively. There were 97 (23.3%) late deaths or transplants. On multivariable analysis, patients in the lowest SES tertile had greater risk of early mortality or transplant (odds ratio [OR], 4.3 [95% CI, 2.0-9.4; P<0.001]), had longer hospitalizations (coefficient 0.4 [95% CI, 0.2-0.5; P<0.001]), incurred higher costs (coefficient 0.5 [95% CI, 0.3-0.7; P<0.001]), and had greater risk of late mortality or transplant (hazard ratio, 2.2 [95% CI, 1.3-3.7; P=0.004]), compared with those in the highest tertile. The risk of late mortality was partially attenuated with successful completion of home monitoring programs. Conclusions Lower neighborhood SES is associated with worse transplant-free survival following the Norwood operation. This risk persists throughout the first decade of life and may be mitigated with successful completion of interstage surveillance programs.
Subject(s)
Norwood Procedures , Univentricular Heart , Humans , Aftercare , Treatment Outcome , Patient Discharge , Univentricular Heart/surgery , Norwood Procedures/adverse effects , Risk Factors , Social Class , Retrospective StudiesABSTRACT
OBJECTIVES: Approximately 0.2% to 2.7% of children with congenital heart disease require a tracheostomy after cardiac surgery with the majority having single ventricle (SV) type heart lesions. Tracheostomy in SV patients is reported to be associated with high mortality. We hypothesized that short- and long-term survival of patients with SV heart disease would vary according to tracheostomy indication. METHODS: This is a single center, 20-year, retrospective review of all patients with SV heart disease who underwent tracheostomy. Demographic, cardiac anatomy, surgical, intensive care unit, and hospital course data were collected. The primary outcome was survival following tracheostomy. Secondary outcome was the completion of staged palliation to Fontan. RESULTS: In total, 25 patients with SV heart disease who underwent tracheostomy were included. Indications for tracheostomy included one or more of the following: tracheobronchomalacia (n = 8), vocal cord paralysis (n = 7), tracheal/subglottic stenosis (n = 6), primary respiratory insufficiency (n = 4), diaphragm paralysis (n = 3), suboptimal hemodynamics (n = 2), and other upper airway issues (n = 1). Survival at six months, one year, five years, and ten years was 76%, 68%, 63%, and 49%, respectively. Most patients completed Fontan palliation (64%). Patients who underwent tracheostomy for suboptimal hemodynamics and/or respiratory insufficiency had a higher mortality risk compared to those with indications of upper airway obstruction or diaphragm paralysis (hazard ratio 4.1, 95% confidence interval 1.2-13.7; P = .02). CONCLUSIONS: Mortality risk varies according to tracheostomy indication in patients with SV heart disease. Tracheostomy may allow staged surgical palliation to proceed with acceptable risk if it was indicated for anatomic or functional airway dysfunction.
Subject(s)
Fontan Procedure , Heart Defects, Congenital , Respiratory Insufficiency , Univentricular Heart , Child , Humans , Infant , Tracheostomy , Treatment Outcome , Heart Defects, Congenital/surgery , Univentricular Heart/surgery , Paralysis/surgery , Retrospective Studies , Heart Ventricles/surgery , Heart Ventricles/abnormalitiesABSTRACT
INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
Subject(s)
Fontan Procedure , Heart Bypass, Right , Heart Defects, Congenital , Tricuspid Atresia , Truncus Arteriosus, Persistent , Univentricular Heart , Pregnancy , Male , Humans , Female , Infant , Univentricular Heart/surgery , Heart Defects, Congenital/surgery , Heart Bypass, Right/methods , Pulmonary Artery/surgery , Pulmonary Artery/abnormalities , Tricuspid Atresia/surgery , Truncus Arteriosus, Persistent/surgery , Retrospective Studies , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Heart Ventricles/abnormalities , Treatment OutcomeABSTRACT
BACKGROUND: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS. METHODS: Transplant-free survivors of the SVR cohort were enrolled at 10 to 15 years of age for multifaceted in-person evaluation of cardiac function (cardiac magnetic resonance [CMR], echocardiogram and exercise test) and neurodevelopmental evaluation. Right ventricular ejection fraction measured by CMR served as the primary outcome. Development of arrhythmias, protein losing enteropathy, and other comorbidities were assessed through annual medical history interview. Through the course of SVR III, protocol modifications to engage SVR trial participants were designed to enhance recruitment and retention. CONCLUSIONS: Evaluation of long-term outcomes will provide important data to inform decisions about the shunt type placed at the Norwood operation and will improve the understanding of cardiovascular and neurodevelopmental outcomes for early adolescents with HLHS.