April

Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty

Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty Patel ND, Nageotte S, Ing FF, Armstrong AK, Chmait R, Detterich JA, Galindo A, Gardiner H, Grinenco S, Herberg U, Jaeggi E, Morris SA, Oepkes D, Simpson JM, Moon-Grady A, Pruetz JD. Catheter Cardiovasc Interv. 2020 Mar 26. doi: 10.1002/ccd.28846. [Epub ahead of print] PMID: 32216096 Similar articles Select item 32215649   Take Home Points: Fetal aortic valvuloplasty offers promise in patients with congenital aortic stenosis to prevent progression to hypoplastic left heart syndrome It is a high risk intervention and center experience is crucial to maximizing success Further investigation of outcomes in patients who undergo fetal aortic valvuloplasty but still require single ventricle palliation is warranted     Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Fetal aortic valvuloplasty (FAV) was first reported in 1991 as an option to treat congenital aortic stenosis in mid-gestation fetuses with the goal to prevent progression to hypoplastic left heart syndrome (HLHS). The process involves using ultrasound guidance to puncture through the maternal abdomen and uterus, then into the fetal thigh to deliver analgesia and a muscle relaxant. A different needle is then passed through the fetal chest and into the left ventricle. A wire is advanced through the needle across the aortic valve. A balloon is advanced over the wire and inflated across the aortic valve. Most centers performing fetal aortic valvuloplasty perform a small number of cases. Boston Children’s Hospital has performed the largest number of these cases and have previously reported their procedural outcomes (Freud LR, McElhinney DB, Marshall AC, et al. Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients. Circulation. 2014;130(8):638-645 and Friedman KG, Sleeper LA, Freud LR, et al. Improved technical success, postnatal outcome, and refined predictors of outcome for fetal aortic valvuloplasty. Ultrasound Obstet Gynecol. 2018;52(2):212-220), as have several other smaller single center studies. Procedure related fetal mortality rates are variable across centers (6.5-32.1%). The International Fetal Cardiac Interventions Registry (IFCIR) was created in 2010 to collect data on fetal interventions from multiple centers (19 centers actively entering data) and improve outcomes. The goal of this study was to look at several technical and procedural aspects of FAV (cannula size, balloon size, number of cardiac punctures) to determine if they were related to procedural and pregnancy outcomes.   Patel et al. report on their analysis of patients from 15 centers in the IFCIR who were candidates for fetal cardiac intervention from 2002-2018. The median center volume was 5 (range 1-21). Patients from Boston Children’s Hospital were excluded as data on those patients had already been presented in the previously mentioned studies. A total of 128 fetuses with a mean gestational age (GA) of 26.1 ± 3.4 weeks were deemed candidates. 108 of these fetuses underwent cardiac puncture and had adequate data in the database to be included for analysis. The indication for FAV was evolving HLHS in 103 fetuses (95.4%). The needle used to puncture the LV was 17 gauge in 15.7%, 18 gauge in 63.9%, and 19 gauge in 29.6%. A single puncture was needed in 77.1%, two punctures in 19.4%, and three punctures in 5.6%. 100 fetuses had an aortic balloon valvuloplasty performed, 90 (83.3%) of which were technically successful (defined as increased forward flow across aortic valve or new aortic regurgitation). The mean aortic valve Z-score was -2.5 ± 1.1 for all fetuses and the median balloon: aortic valve ratio was 1.1. 52 fetuses (48.1%) had at least one intraprocedural complication including bradycardia requiring treatment (37, 34.3%), pericardial effusion requiring treatment (24, 22.2%), pleural effusion (3, 2.8%), balloon rupture (6, 5.6%), and intraprocedural death (9, 8.3%). The overall procedural related mortality was 16.7% (9 intraprocedure deaths, 9 additional deaths within 48 hours). More than one puncture was associated with higher rates of procedural complications (specifically bradycardia, pleural effusion, and intraprocedural death). On multivariate analysis, later GA at intervention and technical success of the procedure were independently associated with live birth.   The outcomes of the pregnancies and infants born is shown below. The 81 fetuses born alive had a median GA of 38.1 weeks, with 26 of them being born prematurely (<37 weeks GA).     This study shows that fetal intervention is often technically successful and offers promise for a biventricular circulation. However, it has significant associated risk with a complication rate of nearly 50% and procedure related mortality of 16.7% (Boston Children’s Hospital’s most recent report had a mortality rate of 6.5%). The higher mortality in this series is multifactorial and likely involves the known learning curve that has been seen in performing this procedure. The authors highlight the importance of center experience and appropriate fetal positioning to minimize the number of punctures. Also, the authors discuss that later GA at the time of the procedure decreased risk likely due to larger fetus size. However, fetuses that were candidates later in gestation may also have less severe disease and waiting for many fetuses could lead to missing the window for prevention of HLHS. Interestingly, of the 81 patients born alive, 22 (27.1%) died prior to hospital discharge. Data from the Single Ventricle Reconstruction trial showed hospital mortality or need for transplantation at 18% (Pasquali SK, Ohye RG, Lu M, Kaltman J, Caldarone CA, Pizarro C, Dunbar-Masterson C, Gaynor JW, Jacobs JP, Kaza AK, Newburger J, Rhodes JF, Scheurer M, Silver E, Sleeper LA, Tabbutt S, Tweddell J, Uzark K, Wells W, Mahle WT, Pearson GD; Pediatric Heart Network Investigators. Variation in perioperative care across centers for infants undergoing the Norwood procedure. J Thorac Cardiovasc Surg. 2012; 144:915–921. doi: 10.1016/j.jtcvs.2012.05.021). While it is unknown if this difference is statistically significant and how many of those patients who died underwent attempted single ventricle palliation, the outcomes of patients that have had a fetal intervention and still require single ventricle palliation versus those that have not warrants further future investigation.    

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Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing? Ronai C, Freud LR, Brown DW, Tworetzky W. Prenat Diagn. 2020 Apr 20. doi: 10.1002/pd.5715. [Epub ahead of print] PMID: 32314369 Similar articles Select item 32354651] Take Home Points Despite routine prenatal cardiac screening ultrasounds, congenital heart disease lesions with normal four chamber view has low sensitivity in prenatal detection. Prenatal diagnosis of pulmonary valve stenosis maybe improved with the use of cine clips to define the valve mobility and color flow Doppler to detect valvar flow acceleration. Unlike pulmonary atresia and intact ventricular septum, fetuses with pulmonary stenosis has near normal appearing tricuspid valve and pulmonary valve size making the diagnosis of pulmonary stenosis without cine clips and Doppler imaging challenging.  Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:  This is a single center retrospective chart review of all neonates who underwent cardiac catherization for critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum.  The cohort was evaluated for prenatal diagnosis of congenital heart disease and those patient’s fetal echocardiograms were reviewed. Methods:  Data was collected on all neonates who underwent cardiac catheterization from 2000-2014 at Boston Children’s Hospital with the diagnosis of critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum. Results:  A total of 178 patients met inclusion criteria with 91 patients having critical pulmonary valve stenosis and 87 patients have pulmonary atresia intact ventricular septum.   Prenatal diagnosis was significant lower in those neonates with critical pulmonary valve stenosis at 37% compared to those with pulmonary atresia intact ventricular septum 60% (p=0.003). Tricuspid and pulmonary valve measurements were evaluated for those patients with fetal echocardiograms and all patients with postnatal transthoracic echocardiograms.  The tricuspid and pulmonary valve z score was significantly higher in those patients with critical pulmonary stenosis compared to those with pulmonary atresia intact ventricular septum (p = <0.001 and p =0.004).  Comparison of the transthoracic tricuspid and pulmonary valve z score of those patients prenatally and postnatally diagnosed showed no statically significance.   Discussion: In this cohort, as the authors proposed the prenatal detection of critical pulmonary valve stenosis would be significantly lower than those patients with pulmonary atresia intact ventricular septum.   The decreased detection for those patients with critical pulmonary valve stenosis was thought to be linked to a normal appearing tricuspid valve and right ventricular on the obstetric screening four chamber view, obstetric imaging of the right ventricular outflow tract was not routinely recommended until 2013 and the decreased obstetric use of cine/color flow mapping imaging.  Lastly, critical pulmonary valve stenosis may progress in severity over pregnancy therefore being undetected in routine obstetric ultrasound. Limitations:  Single center, retrospective study. Next Steps:  It would be of interest to see how many of the critical pulmonary valve stenosis patients had progressive pulmonary valve disease with a normal screening obstetric ultrasound at 18-20 weeks of gestation.  

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Telediagnosis system for congenital heart disease in a Japanese prefecture.

Telediagnosis system for congenital heart disease in a Japanese prefecture. Mabuchi A, Waratani M, Tanaka Y, Mori T, Kitawaki J.J Med Ultrason (2001). 2020 Apr 23. doi: 10.1007/s10396-020-01020-y. Online ahead of print.PMID: 32328807 Take Home Points Fetal diagnosis via telehealth using 3D images transferred via an optical fiber network system is feasible and reliable. In 161 cases screened, cardiac defects were noted in 24 fetal scans. Though there were some discrepancies in prenatal cardiac diagnosis versus post-natal diagnosis, no significant cardiac abnormalities were missed. Appropriate post-natal planning was performed using the telehealth fetal images with significant cardiac defects birthed at a tertiary pediatric hospital whereas minor cardiac defects were birthed at the home hospital. Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   In this retrospective study from a single institution, Kyoto Prefectural University of Medicine, a spatio-temporal image correlation (STIC) based tele-diagnosis system was created for remote fetal diagnostic purposes.  STIC is a 3D technique of acquiring structural and temporal data using a single scan.  Data are digitized and cross-sectional images can be reconstructed to be reviewed at a later date.  This system was created to help screen fetal cardiac exams from distant locations from the main pediatric cardiac hospital to maximize care and minimize unnecessary travel for the families. A total of 182 fetal scans over a nine-year period (2009 – 2018) were referred for evaluation from six hospitals within the Kyoto Prefecture.  21 cases were excluded (10 – error in transmission, 11 – no postnatal diagnosis available), therefore 161 cases were studied.  Images were obtained via a Voluson E7, E8, or E10 (GE Medical Systems) and transferred images were reviewed by a pediatric cardiologist within 24 to 72 hours from transmission.  Images were evaluated using a standard protocol to assess the cardiac anatomy. Cardiac disease was noted 14.9% of cases (24/161) and accuracy of diagnosis was 95.0% (153/161).  In four cases, the cardiac diagnosis changed from prenatal to post-natal: (1) double outlet right ventricle (DORV) to ventricular septal defect (VSD); (2) DORV and pulmonary artery stenosis (PS) to single ventricular and tricuspid atresia; (3) transposition of the great arteries (TGA) to DORV and PS; and (4) total anomalous pulmonary venous connection to normal heart after the visit.  In another four cases, the prenatal diagnosis was thought to be normal, but postnatally, two patients had a VSD, one had an ASD, and one patient an aneurysm of the PDA.  Seven cases suspected of having severe cardiac issues were delivered at the tertiary pediatric hospital whereas the other patients birthed at their respective home hospitals experienced no adverse outcomes. This study is limited by its retrospective nature, single center evaluation, and relatively small cases evaluated.  That being said, this paper adds more evidence that tele-health for fetal cardiac screening purposes is feasible and allows for efficiency of medical care for both the family and health professional.                        

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Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure.

Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure. Tomasulo CE, Gillespie MJ, Munson D, Demkin T, O'Byrne ML, Dori Y, Smith CL, Rome JJ, Glatz AC....

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