RESUMEN

Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR.

RESUMEN

There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of infundibular obstruction is seen in more severe PS cases and in older subjects. Reappearance of PS was observed in approximately 10 % of patients following BPV. The reasons for recurrence were found to be balloon/annulus ratio less than 1.2 and immediate post-BPV pulmonary valve peak gradients greater than 30 mmHg. Recurrent stenosis is successfully addressed by repeating BPV with lager balloons than used initially. Long-term results revealed continue relief of obstruction, but with development of pulmonary insufficiency, some patients requiring replacement of the pulmonary valve. It was concluded that BPV is the treatment of choice in the management valvar PS and that balloon/annuls ratio used for BPV should be lowered to 1.2 to 1.25. It was also suggested that strategies should be developed to prevent/reduce pulmonary insufficiency at long-term follow-up.

Asunto(s)

Valvuloplastia con Balón , Estenosis de la Válvula Pulmonar , Humanos , Valvuloplastia con Balón/métodos , Estenosis de la Válvula Pulmonar/terapia , Estenosis de la Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/diagnóstico , Válvula Pulmonar/cirugía , Resultado del Tratamiento

RESUMEN

Vascular access-related complications are an important consideration in patients undergoing cardiac catheterisation. Patients with CHD are increasingly undergoing percutaneous treatment for suitable procedures as an alternative, less invasive option to surgical intervention. As such, recognition and treatment of these complications are becoming increasingly important. We present a case of a patient with repaired Tetralogy of Fallot who developed a femoral arteriovenous fistula and femoral artery pseudoaneurysm arising from an accessory arterial branch following percutaneous Harmony valve implantation, both of which were treated endovascularly with placement of a stent.

Asunto(s)

Aneurisma Falso , Fístula Arteriovenosa , Humanos , Fístula Arteriovenosa/diagnóstico , Fístula Arteriovenosa/etiología , Fístula Arteriovenosa/cirugía , Arteria Femoral/cirugía , Cateterismo Cardíaco/efectos adversos , Enfermedad Iatrogénica , Resultado del Tratamiento

RESUMEN

Balloon dilatation techniques became available to treat congenital obstructive lesions of the heart in the early/mid-1980s. The purpose of this review is to present the author's experiences and observations on the techniques and outcomes of balloon dilatation of pulmonary stenosis (PS), aortic stenosis (AS) and aortic coarctation (AC), both native and postsurgical re-coarctations. Balloon dilatation resulted in a reduction of peak pressure gradient across the obstructive lesion at the time of the procedure as well as at short-term and long-term follow-ups. Complications such as recurrence of stenosis, valvar insufficiency (for PS and AS cases) and aneurysm formation (for AC cases) have been reported, but infrequently. It was recommended that strategies be developed to prevent the reported complications.

RESUMEN

The "Avalon Elite®" cannula is a double-lumen cannula used to establish veno-venous extracorporeal membrane oxygenation support. The reported advantages are that extracorporeal circulation can be established by cannulating through the right internal jugular vein only, and there is less re-circulation than with a two-cannula technique. It is available in a wide range of cannula sizes and can be used in a variety of patients, from children to adults. We herein report three pediatric cases in which an Avalon Elite® cannula was useful. The first was a case of acute mitral regurgitation due to idiopathic chordal rupture for postoperative severe lung injury and atelectasis due to cardiogenic pulmonary edema. The second was a case of end-stage radiation pneumonitis for safe transfer to facility of lung transplantation. The third was a convalescent case of fulminant myocarditis with severe atelectasis due to cardiogenic pulmonary edema. In each case, veno-venous extracorporeal membrane oxygenation using an Avalon Elite® cannula was established, the expected sufficient support was secured, and a good clinical course was obtained without major complications associated with an Avalon Elite® cannula.

RESUMEN

BACKGROUND: Postoperative pulmonary insufficiency (PPI) is an important contributor to morbidity and mortality after thoracic surgery. Lung ultrasound is a reliable tool for assessing respiratory function. We sought to determine the clinical value of the early lung ultrasound B-line score for predicting changes in pulmonary function after thoracic surgery. METHODS: Eighty-nine patients undergoing elective lung surgery were included in this study. The B-line score was determined 30 min after removal of the endotracheal tube, and the PaO2/FiO2 ratio was recorded 30 min after extubation and on the third postoperative day. Patients were divided into normal (PaO2/FiO2 ≥ 300) and PPI (PaO2/FiO2 < 300) groups according to their PaO2/FiO2 ratios. A multivariate logistic regression model was used to identify independent predictors of postoperative pulmonary insufficiency. Receiver operating characteristic (ROC) analysis was performed for significantly correlated variables. RESULTS: Eighty-nine patients undergoing elective lung surgery were included in this study. We evaluated 69 patients in the normal group and 20 in the PPI group. Patients conforming to NYHA class 3 at administration were significantly more represented in the PPI group (5.8 and 55%; p < 0.001). B-line scores were significantly higher in the PPI group than in the normal group (16; IQR 13-21 vs. 7; IQR 5-10; p < 0.001). The B-line score was an independent risk factor (OR = 1.349 95% CI 1.154-1.578; p < 0.001), and its best cutoff value for predicting PPI was 12 (sensitivity: 77.5%; specificity: 66.7%). CONCLUSIONS: Lung ultrasound B-line scores 30 min after extubation are effective in predicting early PPI in patients undergoing thoracic surgery. Trial registration This study was registered with the Chinese Clinical Trials Registry (ChiCTR2000040374).

Asunto(s)

Pulmón , Procedimientos Quirúrgicos Torácicos , Ultrasonografía , Humanos , Pulmón/diagnóstico por imagen , Pulmón/cirugía , Procedimientos Quirúrgicos Torácicos/efectos adversos , Complicaciones Posoperatorias , Insuficiencia de la Válvula Pulmonar , Pronóstico , Masculino , Femenino , Persona de Mediana Edad , Anciano

RESUMEN

Experimental data on pulmonary regurgitation has linked right ventricular longitudinal function to left ventricular filling pressure in animals with induced and treated pulmonary regurgitation but this relationship has not been investigated in patients with repaired Tetralogy of Fallot (rToF). The aim of this study was to determine if right ventricular longitudinal function assessed using cardiovascular magnetic resonance (CMR) is associated with left ventricular filling pressure in patients with rToF. A second objective of this study was to determine if direction of septal movement is related to right ventricular pressure load in rToF. Eighteen patients with rToF undergoing CMR and heart catheterization prior to pulmonary valve replacement were retrospectively included and catheter-based pressure measurements were compared with CMR-derived RV regional function. Left ventricular filling pressure was measured as precapillary wedge pressure (PCWP). Longitudinal contribution to RV stroke volume correlated with PCWP (r = 0.48; p = 0.046) but not with RV EF or pulmonary regurgitation. Neither RV longitudinal strain nor TAPSE showed correlation with PCWP. Longitudinal contribution to stroke volume was lower for the RV compared to the LV (49 vs 54%; p = 0.039). Direction of septal movement did not show a correlation with RV end-systolic pressure. Right ventricular longitudinal pumping is associated with left ventricular filling pressure in rToF-patients and this inter-ventricular coupling may explain LV underfilling in patients with pulmonary regurgitation and rToF and may be of value to determine right ventricular dysfunction. RV systolic pressure, however, cannot be assessed from the direction of septal movement, in these patients.

Asunto(s)

Insuficiencia de la Válvula Pulmonar , Tetralogía de Fallot , Disfunción Ventricular Derecha , Humanos , Estudios Retrospectivos , Valor Predictivo de las Pruebas , Imagen por Resonancia Magnética , Función Ventricular Derecha

RESUMEN

Extremely preterm birth is associated with a high risk of chronic pulmonary insufficiency of prematurity (CPIP). Lung ultrasound score (LUS) proved capable to characterise CPIP progression beyond the acute setting, but still, post-discharge data remain lacking. We hypothesised a priori that LUS in both BPD and no-BPD infants declines with postnatal age from birth through early infancy. This observational retrospective cohort study included preterm infants < 32 gestational weeks, who underwent the follow-up for CPIP. LUS was assessed from birth to 8 months postnatal age, over antero-lateral (LUS) and posterior (pLUS) pulmonary areas, placing the transducer longitudinally over the midclavicular and midaxillary lines and medial to the scapular line respectively. Extended LUS (eLUS) including LUS and pLUS was calculated. The primary outcome was LUS time course. Secondary outcomes included the correlation between LUS and pLUS. Sixty-two infants were included: 22 (35.5%) in the BPD group and 40 (64.5%) in the no-BPD group. BPD group infants were smaller (weight 841 g (± 228) vs 1226 (± 328), p < 0.001) and younger (26.8 weeks (± 2.0) vs 28.9 (± 1.9), p < 0.001). LUS declined over time in the entire population (ß = - 1.75, p < 0.001) and in both no-BPD and BPD groups (ß = - 1.64, p < 0.001 and ß = - 1.93, p < 0.001, respectively). eLUS declined correspondingly (p < 0.001). LUS and likewise eLUS were significantly different between BPD and no-BPD groups over time (p < 0.001).  Conclusion: LUS trajectory progressively decreased from birth to early infancy. BPD cohort tracked higher, implying a worse respiratory status. pLUS had a similar timepoint course, adding no further information to LUS. To the best of our knowledge, this is the first study that describes preterm LUS time course after discharge. LUS may help track the CPIP progression. What is Known: • Extremely preterm birth is associated with high risk of chronic pulmonary insufficiency of prematurity (CPIP). • Several studies investigated the ability of lung ultrasound score (LUS) to characterize CPIP progression beyond the acute setting, still post-discharge data remain lacking. What is New: • LUS trajectory progressively decreases from birth to early infancy; BPD cohort tracks higher, implying a worse respiratory status. pLUS has similar timepoint course, adding no further information to LUS. • The use of LUS may contribute to better characterising and monitoring CPIP in BPD and no-BPD infants.

Asunto(s)

Displasia Broncopulmonar , Enfermedades del Recién Nacido , Nacimiento Prematuro , Lactante , Femenino , Recién Nacido , Humanos , Displasia Broncopulmonar/diagnóstico por imagen , Displasia Broncopulmonar/complicaciones , Recien Nacido Prematuro , Estudios Retrospectivos , Cuidados Posteriores , Alta del Paciente , Pulmón/diagnóstico por imagen , Edad Gestacional

RESUMEN

Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. The surgical approach to TOF continues to evolve, with many centers now favoring early repair for TOF. This study aimed to determine the prevalence of postsurgical complications such as pulmonary insufficiency, residual pulmonary stenosis, and interventricular communication. Methodology We conducted a cross-sectional, observational study with a descriptive and analytic focus involving 61 patients who were operated on for TOF. Results In total, 39 (63.9%) patients had a regular TOF and 22 (36.1%) had an irregular TOF. In our study, 32 (56.14%) patients had a pulmonary insufficiency, of whom 20 had a minimal pulmonary insufficiency (62.5% of pulmonary insufficiency cases), and 79.4% of patients with right ventricular outflow tract enlargement had pulmonary insufficiency (p < 0.005). Among patients who did not have a right ventricular outflow tract enlargement, three cases of pulmonary insufficiency were identified with a prevalence of 16.6%. Six (10.52%) patients had residual pulmonary stenosis. In addition, two (3.2%) cases of minimal residual ventricular septal defects were identified in this study. Conclusions Postoperative complications of TOF treatment are frequent and require medical care throughout the lifetime of patients.

RESUMEN

The coexistence of tetralogy of Fallot and Ebstein's anomaly is extremely rare. There are only a few case reports in the literature, and surgical options for the treatment are controversial. There is insufficient data on long-term follow-up of patients and management of complications. In this case report, we present a 20-year-old adult with operated tetralogy of Fallot, Ebstein's anomaly, and Glenn anastomosis who underwent transcatheter pulmonary valve implantation for severe pulmonary insufficiency.

Asunto(s)

Anomalía de Ebstein , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Adulto , Anastomosis Quirúrgica , Anomalía de Ebstein/complicaciones , Anomalía de Ebstein/cirugía , Humanos , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/etiología , Insuficiencia de la Válvula Pulmonar/cirugía , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/cirugía , Adulto Joven

RESUMEN

OBJECTIVES: The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS: The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS: The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS: The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

Asunto(s)

Apéndice Atrial , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Tetralogía de Fallot , Animales , Bovinos , Humanos , Lactante , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Estudios Retrospectivos , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/cirugía , Resultado del Tratamiento

RESUMEN

Chronic pulmonary and respiratory conditions associated with preterm birth are incompletely characterized, complicating long-term treatment and development of more effective therapies. Stakeholders face challenges in the development of validated, clinically meaningful endpoints that adequately measure morbidities and predict or represent health outcomes for preterm neonates. We propose in this paper a research agenda, informed by the input of experts from a 2018 workshop we convened on this topic, to advance endpoint and treatment development. We discuss the necessity of further evaluation of existing endpoints and the improved characterization of disease endotypes. We also discuss key steps to the development of optimized short- and long-term endpoints that can be linked to meaningful health outcomes. Finally, we discuss the importance of limiting variability in data collection and the application of new clinical trial endpoints as well as the critical nature of multi-stakeholder collaboration to advancing therapeutic development for this vulnerable patient population.

Asunto(s)

Nacimiento Prematuro , Displasia Broncopulmonar , Femenino , Humanos , Recién Nacido , Recien Nacido Prematuro , Morbilidad , Embarazo

RESUMEN

OBJECTIVES: The aim of this study was to test the hypothesis that narrowing the landing zone using commercially available endografts would enable transcatheter pulmonary valve replacement (TPVR) using commercially available transcatheter heart valves. BACKGROUND: TPVR is challenging in an outsized native or patch-repaired right ventricular outflow tract (RVOT). Downsizing the RVOT for TPVR is currently possible only using investigational devices. In patients ineligible because of excessive RVOT size, TPVR landing zones were created using commercially available endografts. METHODS: Consecutive patients with native or patch-repaired RVOTs and high or prohibitive surgical risk were reviewed, and this report describes the authors' experience with endograft-facilitated TPVR (EF-TPVR) offered to patients ineligible for investigational or commercial devices. All EF-TPVR patients were surgery ineligible, with symptomatic, severe pulmonary insufficiency, enlarged RVOTs, and severe right ventricular (RV) enlargement (>150 ml/m2). TPVR and surgical pulmonary valve replacement (SPVR) were compared in patients with less severe RV enlargement. RESULTS: Fourteen patients had large RVOTs unsuitable for conventional TPVR; 6 patients (1 surgery ineligible) received investigational devices, and 8 otherwise ineligible patients underwent compassionate EF-TPVR (n = 5 with tetralogy of Fallot). Three strategies were applied on the basis of progressively larger RVOT size: single-barrel, in situ fenestrated, and double-barrel endografts as required to anchor 1 (single-barrel and fenestrated) or 2 (double-barrel) transcatheter heart valves. All were technically successful, without procedure-related, 30-day, or in-hospital deaths. Two late complications (stent obstruction and embolization) were treated percutaneously. One patient died of ventricular tachycardia 36 days after EF-TPVR. Compared with 48 SPVRs, RV enlargement was greater, but 30-day and 1-year mortality and readmission were no different. The mean transvalvular pressure gradient was lower after EF-TPVR (3.8 ± 0.8 mm Hg vs. 10.7 ± 4.1 mm Hg; p < 0.001; 30 days). More than mild pulmonary insufficiency was equivalent in both (EF-TPVR 0.0% [n = 0 of 8] vs. SPVR 4.3% [n = 1 of 43]; p = 1.00; 30 days). CONCLUSIONS: EF-TPVR may be an alternative for patients with pulmonic insufficiency and enlarged RVOTs ineligible for other therapies.

Asunto(s)

Procedimientos Quirúrgicos Cardíacos , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Adulto , Cateterismo Cardíaco , Femenino , Humanos , Masculino , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Estudios Retrospectivos , Stents , Volumen Sistólico , Resultado del Tratamiento , Función Ventricular Izquierda , Función Ventricular Derecha , Adulto Joven

RESUMEN

Noonan syndrome is a relatively common genetic disorder and the second most common cause of congenital heart disease after trisomy 21. The spectrum of cardiac anomalies in Noonan syndrome typically involves pulmonary valve stenosis occasionally in conjunction with hypertrophic cardiomyopathy. Mitral valve involvement is a rare finding in Noonan syndrome and is most commonly associated with either mitral valve prolapse or abnormal valvular insertion causing left ventricular outflow tract obstruction. Patients with Noonan syndrome typically have preserved fertility and, given the success of cardiac surgery and medical management of heart failure in this population, are beginning to present more commonly as parturients in adulthood. Maternal physiologic changes during pregnancy introduce an added complexity to hemodynamic management and anesthetic considerations during labor and delivery. In this article, we present a case of a patient with Noonan syndrome with severe mitral stenosis, pulmonary valve insufficiency, and severe restrictive and obstructive pulmonary disease who presented preterm for delivery due to increased dyspnea at rest. Here we review the pathophysiology behind Noonan syndrome and peripartum management strategies in a patient with severe combined cardiac and pulmonary disease.

Asunto(s)

Cardiomiopatía Hipertrófica/complicaciones , Enfermedades Pulmonares Obstructivas/complicaciones , Estenosis de la Válvula Mitral/complicaciones , Síndrome de Noonan/complicaciones , Síndrome de Noonan/fisiopatología , Complicaciones del Embarazo/fisiopatología , Insuficiencia de la Válvula Pulmonar/complicaciones , Adulto , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/fisiopatología , Cesárea , Disnea/complicaciones , Disnea/fisiopatología , Ecocardiografía Transesofágica/métodos , Femenino , Humanos , Enfermedades Pulmonares Obstructivas/diagnóstico por imagen , Enfermedades Pulmonares Obstructivas/fisiopatología , Estenosis de la Válvula Mitral/diagnóstico por imagen , Estenosis de la Válvula Mitral/fisiopatología , Embarazo , Nacimiento Prematuro , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/fisiopatología , Ultrasonografía/métodos

RESUMEN

Various degrees of pulmonary insufficiency (PI) (PaO2 ≤60 mm Hg, SaO2 ≤90%) are diagnosed in most of patients with severe acute stroke (AS). Frequency and severity of PI positively correlates with the severity of AS. PI worsens patient's condition, prolongs the hospitalization period, and increases the probability of fatal outcome. Early clinical signs of PI may be undiagnosed due to the severity of stroke and thus not treated. The initiating pathogenic mechanism of PI is stress-related activation of sympathetic nervous system (SNS) and systemic immunosuppression. In severe stroke with mass effect, the rapid and significant increase in intracranial pressure may additionally activate the SNS. Risk factors of PI include older age, previous pulmonary disease, prolonged supine position, respiratory muscle dysfunction, apnea, and concomitant somatic diseases. Decompensation of somatic diseases leads to multiple stage reactions with facilitation of functional and morphologic changes in the pulmonary system, hypoxemia and hypoxia, promotes infectious complications and multiple organ failure and worsens neurological outcome. Diagnosis and treatment of PI in AS decreases mortality and improves rehabilitation prognosis.

Asunto(s)

Síndrome de Dificultad Respiratoria , Accidente Cerebrovascular , Anciano , Humanos , Hipoxia , Factores de Riesgo

RESUMEN

Almost half of all preterm births are caused or triggered by an inflammatory process at the feto-maternal interface resulting in preterm labor or rupture of membranes with or without chorioamnionitis ("first inflammatory hit"). Preterm babies have highly vulnerable body surfaces and immature organ systems. They are postnatally confronted with a drastically altered antigen exposure including hospital-specific microbes, artificial devices, drugs, nutritional antigens, and hypoxia or hyperoxia ("second inflammatory hit"). This is of particular importance to extremely preterm infants born before 28 weeks, as they have not experienced important "third-trimester" adaptation processes to tolerate maternal and self-antigens. Instead of a balanced adaptation to extrauterine life, the delicate co-regulation between immune defense mechanisms and immunosuppression (tolerance) to allow microbiome establishment is therefore often disturbed. Hence, preterm infants are predisposed to sepsis but also to several injurious conditions that can contribute to the onset or perpetuation of sustained inflammation (SI). This is a continuing challenge to clinicians involved in the care of preterm infants, as SI is regarded as a crucial mediator for mortality and the development of morbidities in preterm infants. This review will outline the (i) role of inflammation for short-term consequences of preterm birth and (ii) the effect of SI on organ development and long-term outcome.

Asunto(s)

Corioamnionitis , Trabajo de Parto Prematuro , Nacimiento Prematuro , Corioamnionitis/etiología , Femenino , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Inflamación/etiología , Embarazo , Nacimiento Prematuro/etiología

RESUMEN

Timing and indication for pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rToF) and pulmonary regurgitation (PR) are uncertain. To improve understanding of pumping mechanics, we investigated atrioventricular coupling before and after surgical PVR. Cardiovascular magnetic resonance was performed in patients (n = 12) with rToF and PR > 35% before and after PVR and in healthy controls (n = 15). Atrioventricular plane displacement (AVPD), global longitudinal peak systolic strain (GLS), atrial and ventricular volumes, and caval blood flows were analyzed. Right ventricular (RV) AVPD and RV free wall GLS were lower in patients before PVR compared with controls (P < 0.0001; P < 0.01) and decreased after PVR (P < 0.0001 for both). Left ventricular AVPD was lower in patients before PVR compared with controls (P < 0.05) and decreased after PVR (P < 0.01). Left ventricular GLS did not differ between patients and controls (P > 0.05). Right atrial reservoir volume and RV stroke volume generated by AVPD correlated in controls (r = 0.93; P < 0.0001) and patients before PVR (r = 0.88; P < 0.001) but not after PVR. In conclusion, there is a clear atrioventricular coupling in patients before PVR that is lost after PVR, possibly because of loss of pericardial integrity. Impaired atrioventricular coupling complicates assessment of ventricular function after surgery using measurements of longitudinal function. Changes in atrioventricular coupling seen in patients with rToF may be energetically unfavorable, and long-term effects of surgery on atrioventricular coupling is therefore of interest. Also, AVPD and GLS cannot be used interchangeably to assess longitudinal function in rToF.NEW & NOTEWORTHY There is a clear atrioventricular coupling in patients with Tetralogy of Fallot (ToF) and pulmonary regurgitation before surgical pulmonary valve replacement (PVR) that is lost after operation, possibly because of loss of pericardial integrity. The impaired atrioventricular coupling complicates assessment of ventricular function after surgery when using measurements of longitudinal function. Left ventricular atrioventricular plane displacement (AVPD) found differences between patients and controls and changes after PVR that longitudinal strain could not detect. This indicates that AVPD and strain cannot be used interchangeably to assess longitudinal function in repaired ToF.

Asunto(s)

Implantación de Prótesis de Válvulas Cardíacas , Válvula Pulmonar/cirugía , Tetralogía de Fallot/fisiopatología , Tetralogía de Fallot/cirugía , Función Ventricular Izquierda , Función Ventricular Derecha , Adulto , Prueba de Esfuerzo , Femenino , Pruebas de Función Cardíaca , Humanos , Estudios Longitudinales , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Válvula Pulmonar/diagnóstico por imagen , Volumen Sistólico , Tetralogía de Fallot/diagnóstico por imagen , Resultado del Tratamiento

RESUMEN

OBJECTIVE: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). BACKGROUND: Long-term data following BPV is limited to small, single center studies. METHODS: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. RESULTS: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. CONCLUSION: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.

Asunto(s)

Valvuloplastia con Balón/efectos adversos , Hemodinámica , Insuficiencia de la Válvula Pulmonar/epidemiología , Estenosis de la Válvula Pulmonar/terapia , Válvula Pulmonar/fisiopatología , Adolescente , Adulto , Anciano , Peso Corporal , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , New York/epidemiología , Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/fisiopatología , Estenosis de la Válvula Pulmonar/diagnóstico por imagen , Estenosis de la Válvula Pulmonar/fisiopatología , Recuperación de la Función , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven

RESUMEN

Rates of bronchopulmonary dysplasia (BPD) are increasing. After preterm birth, there are important developmental periods in which neonates are more vulnerable to stressful events. These periods are opportunities for pharmacologic interventions. Many drugs remain inadequately tested and no new drugs have been approved in more than 25 years for BPD prevention or therapy. More progress is needed in defining appropriate end points based on the pathophysiology of BPD and postdischarge chronic pulmonary insufficiency of prematurity and to develop effective new drugs. In addition, much work is needed to better define perinatal factors, early postnatal findings, and physiologic phenotypes or endotypes.

Asunto(s)

Displasia Broncopulmonar/tratamiento farmacológico , Displasia Broncopulmonar/prevención & control , Administración por Inhalación , Corticoesteroides/uso terapéutico , Broncodilatadores/uso terapéutico , Cafeína/uso terapéutico , Diuréticos/uso terapéutico , Femenino , Humanos , Recién Nacido , Recien Nacido Prematuro , Factor I del Crecimiento Similar a la Insulina/uso terapéutico , Trasplante de Células Madre Mesenquimatosas , Óxido Nítrico/uso terapéutico , Terapia por Inhalación de Oxígeno , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Inhibidores de Fosfodiesterasa/uso terapéutico , Embarazo , Atención Prenatal , Progesterona/uso terapéutico , Progestinas/uso terapéutico , Surfactantes Pulmonares/uso terapéutico , Citrato de Sildenafil/uso terapéutico , Vitamina A/uso terapéutico , Vitaminas/uso terapéutico

RESUMEN

Bronchopulmonary dysplasia (BPD) remains the most prevalent long-term morbidity of surviving extremely preterm infants and is associated with significant health care utilization in infancy and beyond. Recent advances in neonatal care have resulted in improved survival of extremely low birth weight (ELBW) infants; however, the incidence of BPD has not been substantially impacted by novel interventions in this vulnerable population. The multifactorial cause of BPD requires a multi-pronged approach for prevention and treatment. New approaches in assisted ventilation, optimal nutrition, and pharmacologic interventions are currently being evaluated. The focus of this review is the current state of the evidence for pharmacotherapy in BPD. Promising future approaches in need of further study will also be reviewed.