April

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.  

READMORE

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.                        

READMORE

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....

read more