RESUMEN

BACKGROUND: Pregnant patients with single ventricle (SV) physiology carry a high risk of spontaneous pregnancy loss (SPL), yet the clinical factors contributing to this risk are not well defined. METHODS: Single-centre retrospective study of pregnant patients with SV physiology seen in cardio-obstetrics clinic over the past 20 years with chart review of their obstetric history. Patients without a known pregnancy outcome were excluded. Univariable Bayesian panel-data random effects logit was used to model the risk of SPL. RESULTS: The study included 20 patients with 44 pregnancies, 20 live births, 21 SPL and 3 elective abortions. All had Fontan palliation except for two with Waterston and Glenn shunts. 10 (50%) had a single right ventricle (RV). 14 (70%) had moderate or severe atrioventricular valve regurgitation (AVVR). Atrial arrhythmias were present in 16 (80%), Fontan-associated liver disease (FALD) in 15 (75%) and FALD stage 4 in 9 (45%). 12 (60%) were on anticoagulation. Average first-trimester oxygen saturation was 93.8% for live births and 90.8% for SPL. The following factors were associated with higher odds of SPL: RV morphology (OR 1.72 (95% credible interval (CrI) 1.0008-2.70)), moderate or severe AVVR (OR 1.64 (95% CrI 1.003-2.71)) and reduced first-trimester oxygen saturation (OR 1.83 (95% CrI 1.03-2.71) for each per cent decrease in O2 saturation. CONCLUSION: Pregnant patients with SV physiology, particularly those with RV morphology, moderate or severe AVVR, and lower first-trimester oxygen saturations, have a higher risk of SPL. Identifying these clinical risk factors can guide preconception counselling by the cardio-obstetrics team.

Asunto(s)

Aborto Espontáneo , Ventrículos Cardíacos , Complicaciones Cardiovasculares del Embarazo , Humanos , Femenino , Embarazo , Estudios Retrospectivos , Adulto , Factores de Riesgo , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Aborto Espontáneo/etiología , Aborto Espontáneo/epidemiología , Aborto Espontáneo/diagnóstico , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Procedimiento de Fontan/efectos adversos , Medición de Riesgo , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/complicaciones , Adulto Joven

RESUMEN

PURPOSE OF REVIEW: Patients with a functionally single ventricle (SV) are palliated with a series of procedures leading to a Fontan circulation. Over the life span, a substantial proportion of SV patients develop heart failure that can arise from circulatory or ventricular failure. Diastolic dysfunction (DD) is an important determinant of adverse outcomes in SV patients. However, assessment and categorization of DD in the SV remains elusive. We review recent literature and developments in assessment of DD in the SV and its relation to clinical outcomes. RECENT FINDINGS: DD is prevalent in the SV and associated with worse outcomes. Occult DD can be exposed with provocative testing by exercise or preload challenge during catheterization. Likewise, sensitivity to detect DD may be increased via assessment of atrial function and strain imaging. Recent studies revisiting previous concepts such as incoordinate diastolic wall motion show that these are associated with SV end-diastolic pressures and post-Fontan recovery, yielding accessible DD assessment. Emerging technologies such as ultrafast ultrasound (UFUS) can provide noninvasive assessment of myocardial stiffness, inefficient diastolic flow patterns and intraventricular pressure gradients, thereby yielding new tools and insights into diastolic myocardial and hemodynamic properties. SUMMARY: Characterizing DD in the SV continues to have substantial limitations, necessitating synthesis of multiple parameters into an overall assessment, accounting for their change over time, and in the context of the patient's clinical status. New and emerging techniques may help advance DD assessment and the ability to track response to treatment of new targets.

Asunto(s)

Diástole , Procedimiento de Fontan , Humanos , Niño , Corazón Univentricular/fisiopatología , Corazón Univentricular/cirugía , Corazón Univentricular/diagnóstico , Ventrículos Cardíacos/fisiopatología , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/complicaciones

RESUMEN

BACKGROUND: Despite a rigorous screening process, including cardiac catheterization, a subset of patients with a single right ventricle (SRV) demonstrates suboptimal short-term outcomes after the Fontan operation. The goal of this study was to perform a comprehensive assessment of diastolic function in pre-Fontan patients with an SRV using invasive reference-standard measures and determine their associations with post-Fontan outcomes. METHODS AND RESULTS: Children aged 2 to 6 years with SRV physiology undergoing pre-Fontan heart catheterization were recruited prospectively. Patients were divided into those who had an optimal or suboptimal outcome. A suboptimal outcome was defined as length of stay ≥14 days or heart transplant/cardiac death in first year after Fontan. Patients underwent pressure-volume loop analysis using reference-standard methods. The measure of ventricular stiffness, ß, was obtained via preload reduction. Cardiac magnetic resonance imaging for extracellular volume and serum draws for matrix metalloproteinase activity were performed. Of 19 patients with an SRV, 9 (47%) had a suboptimal outcome. Mean age was 4.2±0.7 years. Patients with suboptimal outcomes had lower ventricular stiffness (0.021 [0.009-0.049] versus 0.090 [0.031-0.118] mL-1; P=0.02), lower extracellular volume (25% [28%-32%] versus 31% [28%-33%]; P=0.02), and lower matrix metalloproteinase-2 (90 [79-104] versus 108 [79-128] ng/mL; P=0.01) compared with patients with optimal outcomes. The only invasive measure that had an association with suboptimal outcome was ß (P=0.038). CONCLUSIONS: Patients with an SRV with suboptimal outcome after the Fontan operation had lower ventricular stiffness and evidence of maladaptive extracellular matrix metabolism compared with patients with optimal outcome. This appears to be a novel phenotype that may have important clinical implications and requires further study.

Asunto(s)

Procedimiento de Fontan , Ventrículos Cardíacos , Fenotipo , Humanos , Procedimiento de Fontan/efectos adversos , Preescolar , Masculino , Femenino , Niño , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Estudios Prospectivos , Resultado del Tratamiento , Cateterismo Cardíaco , Función Ventricular Derecha/fisiología , Trasplante de Corazón , Metaloproteinasa 2 de la Matriz/sangre , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Factores de Tiempo

RESUMEN

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.

Asunto(s)

Cateterismo Cardíaco , Procedimiento de Fontan , Cardiopatías Congénitas , Procedimiento de Fontan/efectos adversos , Cateterismo Cardíaco/instrumentación , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/diagnóstico por imagen , Arteria Pulmonar/cirugía , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/diagnóstico por imagen , Vena Cava Inferior/diagnóstico por imagen , Vena Cava Inferior/cirugía , Vena Cava Inferior/fisiopatología , Politetrafluoroetileno , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Diseño de Prótesis , Resultado del Tratamiento , Hemodinámica , Animales , Prótesis Vascular

RESUMEN

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.

Asunto(s)

Cuidados Paliativos , Reoperación , Humanos , Masculino , Inglaterra/epidemiología , Femenino , Estudios Retrospectivos , Gales/epidemiología , Lactante , Preescolar , Reoperación/estadística & datos numéricos , Trasplante de Corazón/estadística & datos numéricos , Sistema de Registros , Procedimiento de Fontan/mortalidad , Corazón Univentricular/cirugía , Corazón Univentricular/mortalidad , Corazón Univentricular/fisiopatología , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/fisiopatología , Recién Nacido , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Factores de Tiempo , Resultado del Tratamiento

Asunto(s)

Procedimiento de Fontan , Humanos , Femenino , Masculino , Niño , Preescolar , Etnicidad , Factores Sexuales , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Lactante , Adolescente , Grupos Raciales , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Tasa de Supervivencia

RESUMEN

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.

Asunto(s)

Trasplante de Corazón , Humanos , Trasplante de Corazón/métodos , Masculino , Femenino , Mortalidad Hospitalaria/tendencias , Lactante , Estudios Retrospectivos , Cuidados Paliativos/métodos , Corazón Univentricular/cirugía , Procedimientos de Norwood/métodos , Tasa de Supervivencia/tendencias , Recién Nacido , Estudios de Seguimiento

Asunto(s)

Arteria Pulmonar , Humanos , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Arteria Pulmonar/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Corazón Univentricular/cirugía , Masculino , Femenino

RESUMEN

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."

Asunto(s)

Atresia Tricúspide , Corazón Univentricular , Humanos , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Atresia Tricúspide/cirugía , Atresia Tricúspide/fisiopatología , Atresia Tricúspide/diagnóstico por imagen , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen , Corazón Univentricular/terapia , Corazón Univentricular/genética

RESUMEN

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 (Ɛ) 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: Ɛ 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.

Asunto(s)

Ecocardiografía , Procedimiento de Fontan , Humanos , Procedimiento de Fontan/métodos , Masculino , Femenino , Ecocardiografía/métodos , Adolescente , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Adulto , Corazón Univentricular/cirugía , Corazón Univentricular/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/diagnóstico por imagen , Niño , Adulto Joven , Prueba de Esfuerzo/métodos , Cuidados Paliativos/métodos

RESUMEN

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.

Asunto(s)

Cateterismo Cardíaco , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Cuidados Paliativos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Estudios Retrospectivos , Resultado del Tratamiento , Femenino , Masculino , Factores de Tiempo , Factores de Riesgo , Procedimientos de Norwood/efectos adversos , Recién Nacido , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Tabique Interatrial/diagnóstico por imagen , Tabique Interatrial/fisiopatología , Tabique Interatrial/cirugía , Lactante , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Corazón Univentricular/diagnóstico por imagen

Asunto(s)

Oxigenación por Membrana Extracorpórea , Procedimientos de Norwood , Cuidados Posoperatorios , Humanos , Oxigenación por Membrana Extracorpórea/métodos , Procedimientos de Norwood/métodos , Cuidados Posoperatorios/métodos , Recién Nacido , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/anomalías , Corazón Univentricular/cirugía

RESUMEN

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: - 4.1 ~ - 1.0) at pre-PAB to -2.8 (range: - 3.6 ~ - 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: - 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches.

Asunto(s)

Aorta Torácica , Procedimientos Quirúrgicos Cardíacos , Ventrículos Cardíacos , Arteria Pulmonar , Humanos , Estudios Retrospectivos , Masculino , Arteria Pulmonar/cirugía , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/anomalías , Femenino , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/anomalías , Procedimientos Quirúrgicos Cardíacos/métodos , Lactante , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Ventrículos Cardíacos/fisiopatología , Resultado del Tratamiento , Ecocardiografía , Preescolar , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/diagnóstico , Corazón Univentricular/cirugía , Corazón Univentricular/fisiopatología , Niño , Recién Nacido

RESUMEN

BACKGROUND: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. METHODS: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. RESULTS: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). CONCLUSIONS: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Asunto(s)

Ventrículos Cardíacos , Arteria Pulmonar , Humanos , Lactante , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Recién Nacido , Femenino , Masculino , Estudios Retrospectivos , Preescolar , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Resultado del Tratamiento , Corazón Univentricular/cirugía , Conducto Arterial/cirugía , Conducto Arterial/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Anomalías Múltiples/cirugía

RESUMEN

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.

Asunto(s)

Comorbilidad , Resultado del Embarazo , Humanos , Femenino , Embarazo , Estudios Retrospectivos , Adulto , Resultado del Embarazo/epidemiología , Síndrome del Corazón Izquierdo Hipoplásico/epidemiología , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Recién Nacido , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/terapia , Complicaciones del Embarazo/epidemiología , Corazón Univentricular/cirugía , Corazón Univentricular/epidemiología

RESUMEN

BACKGROUND: Infants with single ventricle heart disease (SVHD) suffer morbidity from insufficient pulmonary blood flow, which may be related to impaired arginine metabolism. No prior study has reported quantitative mapping of arginine metabolites to evaluate the relationship between circulating metabolite levels and outcomes. METHODS: Prospective cohort study of 75 SVHD cases peri-Stage 2 and 50 healthy controls. We targeted pre- and post-op absolute serum quantification of 9 key members of the arginine metabolism pathway by tandem mass spectrometry. Primary outcomes were length of stay (LOS) and post-Stage 2 hypoxemia. RESULTS: Pre-op cases showed alteration in 6 metabolites including decreased arginine and increased asymmetric dimethyl arginine (ADMA) levels compared to controls. Post-op cases demonstrated decreased arginine and citrulline levels persisting through 48 h. Adjusting for clinical variables, lower pre-op and 2 h post-op concentrations of multiple metabolites, including arginine and citrulline, were associated with longer post-op LOS (p < 0.01). Increased ADMA at 24 h was associated with greater post-op hypoxemia burden (p < 0.05). CONCLUSION: Arginine metabolism is impaired in interstage SVHD infants and is further deranged following Stage 2 palliation. Patients with greater metabolite alterations experience greater post-op morbidity. Decreased arginine metabolism may be an important driver of pathology in SVHD. IMPACT: Interstage infants with SVHD have significantly altered arginine-nitric oxide metabolism compared to healthy children with deficiency of multiple pathway intermediates persisting through 48 h post-Stage 2 palliation. After controlling for clinical covariates and classic catheterization-derived predictors of Stage 2 readiness, both lower pre-operation and lower post-operation circulating metabolite levels were associated with longer post-Stage 2 LOS while increased post-Stage 2 ADMA concentration was associated with greater post-op hypoxemia. Arginine metabolism mapping offers potential for development using personalized medicine strategies as a biomarker of Stage 2 readiness and therapeutic target to improve pulmonary vascular health in infants with SVHD.

Asunto(s)

Arginina , Citrulina , Óxido Nítrico , Humanos , Arginina/análogos & derivados , Arginina/sangre , Arginina/metabolismo , Estudios Prospectivos , Masculino , Femenino , Lactante , Óxido Nítrico/metabolismo , Óxido Nítrico/sangre , Citrulina/sangre , Tiempo de Internación , Ventrículos Cardíacos/metabolismo , Hipoxia/sangre , Estudios de Casos y Controles , Recién Nacido , Cuidados Paliativos , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/metabolismo , Corazón Univentricular/cirugía , Morbilidad

RESUMEN

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.

Asunto(s)

Procedimiento de Fontan , Traqueostomía , Humanos , Estudios Retrospectivos , Masculino , Femenino , Lactante , Preescolar , Procedimiento de Fontan/métodos , Niño , Unidades de Cuidado Intensivo Pediátrico , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Corazón Univentricular/cirugía , Resultado del Tratamiento , Modelos de Riesgos Proporcionales , Respiración Artificial

RESUMEN

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.

Asunto(s)

Arritmias Cardíacas , Procedimiento de Fontan , Ventrículos Cardíacos , Humanos , Masculino , Femenino , Procedimiento de Fontan/efectos adversos , Incidencia , Niño , Adolescente , Adulto , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/diagnóstico , Persona de Mediana Edad , Adulto Joven , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/epidemiología , Estudios Retrospectivos , Factores de Tiempo , Corazón Univentricular/cirugía , Corazón Univentricular/epidemiología , Corazón Univentricular/fisiopatología , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Factores de Riesgo

RESUMEN

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.

Asunto(s)

Biomarcadores , Péptido Natriurético Encefálico , Fragmentos de Péptidos , Corazón Univentricular , Humanos , Fragmentos de Péptidos/sangre , Péptido Natriurético Encefálico/sangre , Masculino , Femenino , Preescolar , Lactante , Biomarcadores/sangre , Corazón Univentricular/cirugía , Corazón Univentricular/sangre , Niño , Procedimiento de Fontan , Factores de Edad , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/diagnóstico por imagen

RESUMEN

BACKGROUND: Fenestrating a Fontan baffle has been associated with improved perioperative outcomes in patients with univentricular hearts. However, longer-term potential adverse effects remain debated. We sought to assess the impact of a fenestrated Fontan baffle on adverse cardiovascular events including all-cause mortality, cardiac transplantation, atrial arrhythmias, and thromboemboli. METHODS: A multicentre North American retrospective cohort study was conducted on patients with total cavopulmonary connection Fontan baffle, with and without fenestration. All components of the composite outcome were independently adjudicated. Potential static and time-varying confounders were taken into consideration, along with competing risks. RESULTS: A total of 407 patients were followed for 10.4 (7.1-14.4) years; 70.0% had fenestration of their Fontan baffle. The fenestration spontaneously closed or was deliberately sealed in 79.9% of patients a median of 2.0 years after Fontan completion. In multivariable analysis in which a persistent fenestration was modelled as a time-dependent variable, an open fenestration did not confer a higher risk of the composite outcome (hazard ratio, 1.18; 95% confidence interval, 0.71-1.97; P = 0.521). In secondary analyses, an open fenestration was not significantly associated with components of the primary outcome: that is, mortality or transplantation, atrial arrhythmias, or thromboemboli. However, sensitivity analyses to assess the possible range of error resulting from imprecise dates for spontaneous fenestration closures could not rule out significant associations between an open fenestration and atrial arrhythmias or thromboemboli. CONCLUSIONS: In this multicentre study, no significant association was identified between an open fenestration in the Fontan baffle and major adverse cardiovascular events.

Asunto(s)

Procedimiento de Fontan , Cardiopatías Congénitas , Humanos , Procedimiento de Fontan/métodos , Procedimiento de Fontan/efectos adversos , Masculino , Femenino , Estudios Retrospectivos , Cardiopatías Congénitas/cirugía , Niño , Complicaciones Posoperatorias/epidemiología , Preescolar , Adolescente , Estudios de Seguimiento , Tromboembolia/etiología , Tromboembolia/epidemiología , Tromboembolia/prevención & control , Trasplante de Corazón/métodos , Corazón Univentricular/cirugía