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PubMed | University of Bonn, Medical University of Warsaw, Masaryk University, Royal Brompton and Harefield Hospital and 5 more.
Type: Journal Article | Journal: Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology | Year: 2016

Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV.From a European multicenter retrospective study of 214 fetuses with aortic stenosis (2005-2012), 107 fetuses in ongoing pregnancies that did not undergo FV were included in this study and their natural history was reported. We examined longitudinal changes in Z-scores of aortic and mitral valve and left ventricular dimensions and documented direction of flow across the foramen ovale and aortic arch, and mitral valve inflow pattern and any gestational changes. Data were used to identify fetuses satisfying the Boston criteria for emerging HLHS and estimate the proportion of these that would have been ideal FV candidates. We applied the threshold score whereby a score of 1 was assigned to fetuses for each Z-score meeting the following criteria: left ventricular length and width>0; mitral valve diameter >-2; aortic valve diameter >-3.5; and pressure gradient across either the mitral or aortic valve>20mmHg. We compared the predicted circulation with known survival and final postnatal circulation (BV, UV or conversion from BV to UV).Among the 107 ongoing pregnancies there were eight spontaneous fetal deaths and 99 livebirths. Five were lost to follow-up, five had comfort care and four had mild aortic stenosis not requiring intervention. There was intention-to-treat in these 85 newborns but five died prior to surgery, before circulation could be determined, and thus 80 underwent postnatal procedures with 44 BV, 29 UV and seven BV-to-UV circulatory outcomes. Of newborns with intention-to-treat, 69/85 (81%) survived 30days. Survival at median 6 years was superior in cases with BV circulation (P = 0.041). Those with a postnatal UV circulation showed a trend towards smaller aortic valve diameters at first scan than did the BV cohort (P=0.076), but aortic valve growth velocities were similar in both cohorts to term. In contrast, the mitral valve diameter was significantly smaller at first scan in those with postnatal UV outcomes (P=0.004) and its growth velocity (P = 0.008), in common with the left ventricular inlet length (P = 0.004) and width (P = 0.002), were reduced significantly by term in fetuses with UV compared with BV outcome. Fetal data, recorded before 30 completed gestational weeks, from 70 treated neonates were evaluated to identify emerging HLHS. Forty-four had moderate or severe left ventricular depression and 38 of these had retrograde flow in the aortic arch and two had left-to-right flow at atrial level and reversed a-waves in the pulmonary veins. Thus 40 neonates met the criteria for emerging HLHS and BV circulation was documented in 13 (33%). Of these 40 cases, 12 (30%) had a threshold score of 4 or 5, of which five (42%) had BV circulation without fetal intervention.The natural history in our cohort of fetuses with aortic stenosis and known outcomes shows that a substantial proportion of fetuses meeting the criteria for emerging HLHS, with or without favorable selection criteria for FV, had a sustained BV circulation without fetal intervention. This indicates that further work is needed to refine the selection criteria to offer appropriate therapy to fetuses with aortic stenosis. Copyright 2016 ISUOG. Published by John Wiley & Sons Ltd.

Skippen P.,Pediatric Intensive Care Unit | Sanatani S.,Childrens Heart Center | Froese N.,British Columbia Childrens Hospital | Gow R.M.,Childrens Hospital of Eastern Ontario
Pediatric Critical Care Medicine | Year: 2010

Objective: To summarize the practical operation of temporary pacemakers in common use pertinent to the intensivist caring for the postcardiac patient. Pacemaker therapy is commonly required in the postoperative period after congenital cardiac surgery. Data Synthesis: Monitoring the hemodynamic status and availability of equipment for resuscitation is always important in any patient requiring a temporary pacemaker. Two important scenarios to consider in the pediatric intensive care unit are: 1) the patient in whom pacing has been initiated to optimize cardiac function; and 2) the patient without demonstrable spontaneous electrical activity or with extreme bradycardia. A number of different models of temporary pacemaker are available. Management of the child requiring cardiac pacing requires an understanding of the indications for pacing, a thorough knowledge of the available pacemaker, and an ability to troubleshoot problems. Conclusions: As the most common arrhythmias post congenital cardiac surgery involve either rate or conduction abnormalities, temporary pacemaker systems are a common form of electrical therapy in the postoperative period. © 2010 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.

PubMed | University of California at Los Angeles and Childrens Heart Center
Type: Comparative Study | Journal: Echocardiography (Mount Kisco, N.Y.) | Year: 2015

The aim of this study was to determine and quantify the relationship between Doppler echocardiography and cardiac catheterization measurements of the peak right ventricle-pulmonary artery (RV-PA) pressure gradient in patients within 24 hours of Melody valve implantation for treatment of a dysfunctional RV outflow tract (RVOT) conduit or bioprosthetic valve (BPV).Patients with a dysfunctional RVOT conduit or BPV are now routinely treated percutaneously with implantation of a Melody valve. However, often the postimplantation catheter measurements of the RV-PA peak gradient do not match the postimplant echo-derived gradients obtained after completion of the procedure. Importantly, these echo gradients are commonly used to monitor patients after implantation over time.Medical records of 42 patients with Melody valve implantation were reviewed (men: 25/42; mean age: 22.4 11.2 years; RVOT conduit: 23/42; BPV: 18/42; pulmonic stenosis: 6/42, pulmonic regurgitation: 3/42, both: 33/42).The postimplantation RV-PA peak gradient measured by Doppler echocardiography (within 24 hours of valve implantation) was significantly higher than the measurements acquired by catheterization immediately following implantation (24.2 16.3 mmHg vs. 11.6 8.5 mmHg, P < 0.0001). The relationship showed a moderate-strong correlation (r = 0.65, P < 0.0001) with regression analysis suggesting a linear association in both directions (echo gradient = 1.24 cath gradient + 9.8, P<0.0001 vs. cath gradient = 0.34 echo gradient + 3.4, P<0.0001).The Doppler echocardiography-derived RV-PA peak gradient within 24 hours of valve implantation is predictably higher than the catheter measured peak gradient immediately following valve implantation. A regression equation was derived to define this important relationship.

Evans W.N.,Childrens Heart Center | Evans W.N.,University of Nevada, Las Vegas | Kogut K.,653 wn Center Dr Ste 507 | Acherman R.J.,Childrens Heart Center | Acherman R.J.,University of Nevada, Las Vegas
Pediatric Surgery International | Year: 2014

This report describes an infant born with esophageal atresia, tracheoesophageal fistula, right-sided diaphragmatic hernia, and an interrupted inferior vena cava (IVC) that underwent successful repair. The report emphasizes the importance of diagnosing an interrupted IVC prior to tracheoesophageal repair. © 2013 Springer-Verlag Berlin Heidelberg.

Alhuzaimi A.,Childrens Heart Center | Roy N.,British Columbia Childrens Hospital | Duncan W.J.,Childrens Heart Center
Cardiology in the Young | Year: 2011

Cardiac strangulation from epicardial pacemaker leads is a rare event that can be difficult to recognise and can cause serious complications such as cardiac failure or death. We describe a 3-year-old girl who received an epicardial pacing system as a neonate for complete congenital cardiac block and developed cardiac strangulation from the leads. The clinical presentation modes are reviewed and technical aspects for lead and generator positioning are discussed. © 2011 Cambridge University Press.

Friedman D.,Childrens Heart Center | Szmuszkovicz J.,Childrens Hospital Los Angeles | Rabai M.,Childrens Hospital Los Angeles | Detterich J.A.,Childrens Hospital Los Angeles | And 2 more authors.
Journal of Heart and Lung Transplantation | Year: 2012

Background: Idiopathic pulmonary arterial hypertension (IPAH) is a life-threatening disease manifested by progressive pulmonary vascular remodeling, compromised pulmonary blood flow and right heart failure. Most studies have explored how pulmonary endothelial function modulates disease pathogenesis. We hypothesize that IPAH is a progressive panvasculopathy, affecting both pulmonary and systemic vascular beds, and that systemic endothelial dysfunction correlates with disease severity. Recent studies have demonstrated systemic endothelial dysfunction in adults with pulmonary hypertension; however, adults often have additional comorbidities affecting endothelial function. Systemic endothelial function has not been explored in children with IPAH. Methods: In this single-center, prospective, cross-sectional study we examined brachial artery flow-mediated dilation (FMD), a nitric oxide-mediated, endothelial-dependent response, in children with IPAH and matched controls. FMD measurements were compared with clinical and echocardiographic measures of IPAH severity. Results: Thirteen patients and 13 controls were studied, ranging in age from 6 to 20 years. FMD was decreased in IPAH subjects compared with controls (5.1 ± 2.1% vs 9.7 ± 2.0%; p < 0.0001). In IPAH subjects, FMD correlated directly with cardiac index (R2 = 0.34, p = 0.035), and inversely with tricuspid regurgitation velocity (R2 = 0.57, p = 0.019) and right ventricular myocardial performance index (R2 = 0.44, p = 0.028). Conclusions: The presence of systemic endothelial dysfunction in children with IPAH and its strong association with IPAH severity demonstrate that IPAH is a global vasculopathy. Although morbidity in IPAH is typically associated with pulmonary vascular disease, systemic vascular changes may also relate to disease pathogenesis and progression. Further study into shared mechanisms of systemic and pulmonary endothelial dysfunction may contribute to future therapies for IPAH. © 2012 International Society for Heart and Lung Transplantation.

Rothman A.,Childrens Heart Center | Rothman A.,University of Nevada, Las Vegas | Galindo A.,Childrens Heart Center | Evans W.N.,Childrens Heart Center
Catheterization and Cardiovascular Interventions | Year: 2015

We describe a 29 mm Sapien XT valve implantation in a 10-year-old girl with repaired Tetralogy of Fallot, severe pulmonary regurgitation, and right ventricular dilatation, using the NovaFlex+ delivery system and the expandable introducer eSheath, without pre-stenting of the right ventricular outflow tract. © 2015 Wiley Periodicals, Inc.

Robbers-Visser D.,Erasmus MC Sophia Childrens Hospital | Miedema M.,University Utrecht | Nijveld A.,UMC St Radboud | Boersma E.,Erasmus University Rotterdam | And 4 more authors.
European Journal of Cardio-thoracic Surgery | Year: 2010

Objectives: This study aims to compare the outcome of the two co-existing modifications of staged total cavopulmonary connection (TCPC) - the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). Methods: We included 209 patients after staged TCPC (102 ILT and 107 ECC), operated on between 1988 and 2008. Medical and surgical records were reviewed for (1) patient demographics and cardiac anatomy; (2) pre-Fontan procedures; (3) pre-Fontan haemodynamics and cardiac functional status; (4) operative details; (5) postoperative hospital course; (6) follow-up information on arrhythmias and thrombo-embolic events; (7) post-Fontan interventions; and (8) clinical status at last follow-up until June 2008. Results: Median follow-up duration was 4.3 years (interquartile range 1.5-7.4 years). At 6-year follow-up, freedom from Fontan failure (i.e., mortality or re-operations for Fontan failure) was 83% for the ILT and 79% for the ECC groups (p = 0.6); freedom from late re-operations (other than re-operations for Fontan failure) was 79% for the ILT and the ECC groups and freedom from arrhythmias was 83% for the ILT, and 92% for the ECC groups (p = 0.022). Multivariable Cox regression analysis identified intensive care unit stay and cardiopulmonary bypass time as risk factors for Fontan failure, but they were not strong predictors. Right ventricular morphology was identified as a risk factor for arrhythmias. The occurrence of thrombo-embolic events was low with no difference between the ILT and the ECC groups, and irrespective of the postoperative use of anticoagulant or anti-platelet aggregation therapy. At most recent follow-up, sinus rhythm was present in 70% of patients; in 23% of the patients, ventricular function was found to be moderately or severely impaired at echocardiography. Conclusions: Outcome after staged ILT- and ECC-type Fontan operations is good, with comparable freedom from late re-operations and freedom from Fontan failure at 6-year follow-up. The incidence of arrhythmias was significantly lower in the ECC group. Right ventricular morphology was identified as a risk factor for arrhythmias. © 2009 European Association for Cardio-Thoracic Surgery.

Wohlmuth C.,Childrens Heart Center | Wohlmuth C.,Paracelsus Medical University | Tulzer G.,Childrens Heart Center | Arzt W.,Womens and Childrens Hospital | And 4 more authors.
Ultrasound in Obstetrics and Gynecology | Year: 2014

Objectives Fetal cardiac interventions have the potential to alter natural disease progression and reduce morbidity and mortality in children. Although there are already encouraging data on fetal outcome, information on maternal morbidity and mortality after intervention is scarce. The aim of the present study was to assess maternal aspects, pregnancy-associated risks and adverse events in 53 intrauterine cardiac interventions.Methods Between October 2000 and December 2012, 53 fetal cardiac interventions were performed in 47 patients (43 aortic valve dilations in 39 patients, seven pulmonary valve dilations in six patients and three balloon atrioseptostomies in two patients). Median gestational age was 26 + 4 (range, 20 + 3 to 33 + 1) weeks. Interventions were performed by an ultrasound-guided percutaneous approach under general anesthesia. All medical records and patient charts were analyzed retrospectively.Results All women were considered to be healthy in the preoperative assessment; 39 (83%) patients continued pregnancy until term and eight of 47 patients had an intrauterine fetal death (IUFD) and were induced. Postoperative nausea was reported in 29.8% of patients and abdominal pain in 36.2% of patients on the day of surgery. Preterm contractions were observed in two patients; no preterm prelabor rupture of membranes occurred. One severe postpartum hemorrhage was observed in a patient with IUFD and subsequent induction; however, this was unrelated to the balloon valvuloplasty. No intensive care unit admission and no major anesthesia-associated complications (aspiration, anaphylactic reaction, cardiovascular collapse, damage to teeth, laryngeal damage, awareness or hypoxic brain damage) were observed. Maternal mortality was zero. A significant learning curve was observed in terms of duration of intervention.Conclusion In our experience, percutaneous needle-guided fetal cardiac intervention seems to be a safe procedure for the mother. In 53 procedures no major maternal complication directly related to the intervention was observed. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.

Kubus P.,Childrens Heart Center | Vit P.,Childrens University Hospital Brno | Gebauer R.A.,University of Leipzig | Materna O.,Childrens Heart Center | Janousek J.,Childrens Heart Center
Circulation: Arrhythmia and Electrophysiology | Year: 2014

Background-Data on the results and clinical effect of an invasive risk stratification strategy in asymptomatic young patients with the Wolff-Parkinson-White electrocardiographic pattern are scarce. Methods and Results-Eighty-five consecutive patients aged <18 years with a Wolff-Parkinson-White pattern and persistent preexcitation at maximum exercise undergoing invasive risk stratification were retrospectively studied. Adverse accessory pathway (AP) properties were defined according to currently consented criteria as any of the following: shortest preexcited RR interval during atrial fibrillation/rapid atrial pacing =250 ms (or antegrade effective refractory period =250 ms if shortest preexcited RR interval was not available) or inducible atrioventricular re-entrant tachycardia. Age at evaluation was median 14.9 years. Eighty-two patients had a structurally normal heart and 3 had hypertrophic cardiomyopathy. A single manifest AP was present in 80, 1 manifest and 1 concealed AP in 4, and 2 manifest APs in 1 patient. Adverse AP properties were present in 32 of 85 patients (37.6%) at baseline and in additional 16 of 44 (36.4%) after isoproterenol. Ablation was performed in 41 of these 48 patients. Ablation was deferred in the remaining 7 for pathway proximity to the atrioventricular node. In addition, 18 of the low-risk patients were ablated based on patient/parental decision. Conclusions-Adverse AP properties at baseline were exhibited by 37.6% of the evaluated patients with an asymptomatic Wolff-Parkinson-White preexcitation persisting at peak exercise. Isoproterenol challenge yielded additional 36.4% of those tested at higher risk. Ablation was performed in a total of 69.4% of patients subjected to invasive risk stratification. © 2014 American Heart Association, Inc.

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